My Toledo Vacation — August 1986

In my previous blog post I talked about my friend Barbara Alkema who might be my new roommate along with her adult son Josh and their 4 cats. You can read the details about that here. I mentioned in that blog that in 1986 Barb drove from her home in Toledo down to Indianapolis, picked me up in my van, and drove me back to Toledo to spend a week vacation with her and my friend Jim Grubs. I had met Jim online on CompuServe on a disability forum. He had a type of muscular dystrophy different from mine. Barb was his live-in caregiver. We all became friends online. This blog is the story about that vacation that I spent with her and Jim.

The Adventure Begins

I don’t remember the exact dates but it was in August 1986 that I went to visit my friends Jim and Barb who lived in Toledo. Granted one doesn’t typically think of Toledo as a big vacation destination. John Denver even wrote a silly song about what a boring place it is. YouTube
Actually Jim lived in Sylvania which is a suburb of Toledo. Anyway this was a really big deal for me because it was the first time I had been anywhere overnight or under the care of anyone other than my parents. It was a big milestone for me in my independence. It served as proof of concept that let me take other out-of-town trips with other friends years later.

The drive to Toledo was uneventful. I don’t really remember any details of it. Google maps (which didn’t exist in those days) tells me today that it is a 224 mile journey that should take three hours 48 minutes.

Barb rearranged some furniture in Jim’s bedroom and they rented a hospital bed for me for the week. We took my Hoyer lift and my oxygen machine. This was decades before I had a CPAP, ventilator, trach or G-tube. I just had oxygen that I used at night. Jim did as well although I think he may have had a CPAP I don’t recall. Barb did double duty taking care of both of us. We visited with friends of theirs and went out to dinner a couple of times.

Catching A Ferry

The big event of the week was our trip to South Bass Island about 5 miles into Lake Erie. There is a little resort town called Put-in-Bay on the island. They have a fancy marina with lots of expensive boats to look at and tourist shops you can browse. The main attraction on the island was the “Perry’s Victory and International Peace Memorial”. It is a National Park Service facility and the website (which did not exist in 1986) describes it as follows “Perry’s Victory and International Peace Memorial was established to honor those who fought in the Battle of Lake Erie, during the War of 1812, and to celebrate the long-lasting peace among Great Britain, Canada and the U.S. The Memorial, a Doric column, rising 352 feet over Lake Erie is situated 5 miles from the longest undefended border in the world.” Link to park service website for Perry’s Victory Memorial

We would drive from Toledo to Catawba Island just north of Sandusky. There you catch a ferry boat out to South Bass Island. You can click on the maps in this blog for a larger version and each has a link where you can to go to Google maps to see more details.

When we got to the ferry landing they told us that we could not take our vehicle with us because they had already taken over a large number of vehicles for the day. There’s only so much room for cars on the boat and there was a chance that there would not be room for us for the return trip. So we parked the van at a parking lot about a block away and got on the boat just in our wheelchairs with Barbara on foot.

I had my VHS camcorder with me and I shot video along the way. You can see the YouTube version at the end of this blog. Some of the images in this blog are screen grabs from that video. Unfortunately the railing on the ferry had a bar exactly at the height of my video camera so it was a little bit difficult to shoot video out across the water. Most of the passengers road up high on an upper deck that gave them a great view but we stayed on the main deck with the vehicles that were going across. A couple of seagulls flew alongside us on the journey. We could see other boats out on Lake Erie and another ferryboat passed us going the other way.


Growing up severely landlocked here in Indianapolis, being able to look out to the horizon and see endless water is a strange experience for me. I’ve never been to the ocean. I have been to Chicago on a couple of occasions and seen Lake Michigan but I had never been out on it in a boat. South Bass Island is only 2.9 miles from shore so we could see it from the shore where we departed. But there were directions you can look out into the lake and it was water all the way to the horizon. For most people it wouldn’t be a big deal but it was a new experience for me.

I don’t know how Google maps computed that ferry transit time in the map above. It says 20 minutes on foot and 31 minutes by car. Maybe it takes longer to load your car onto the ferry than to walk on 🙂

When we got to the landing at the south tip of South Bass Island we realized we were in trouble. We were at the wrong end of the island. We had no idea how far we would have to walk. Most people who got off the ferry went to the bicycle rental stand but that wasn’t going to work for us. As this Google map below shows, it’s 2.2 miles. Had we been in the van it would’ve been a six minute drive from one end of the island to the other. On foot it’s 46 minutes. And with two electric wheelchairs and an aide on foot it was probably longer than that. Of course we didn’t have Google maps in those days or smart phones with which to check Google maps. Not knowing what we were up against we had no choice but to set out on foot.

We passed by a small airport on the island and sat and watched a couple of private planes land and take off. There is also an air service called Island Airlines that will take you out to the island. Jim saw one of them flyover while we were on the ferryboat. Somewhere along the way we came across a little hamburger stand with some picnic tables sitting in front of it so we stopped and had lunch. You can see the Perry Monument off in the distance and it looked like it was just over the hill a bit. But I had no idea just how tall the thing really was. (Again we couldn’t Google it.) We were still over a mile away.

When we finally got to the town of Put-in-Bay it was very nice. There was a nice marina with lots of expensive looking boats. One of them looked like a big offshore racing boat. Others were fancy cabin cruisers. We didn’t really spend any time in the tourist trap shops. We went on to the big Memorial to get a good look at it. Now we could see just how huge it really was. There was a small shack that was sort of like a mini museum. I shot some video of a really cool scale model of the USS Lawrence which was Commodore Perry’s flagship during the battle of Lake Erie. His motto and battle flag bore the phrase “Don’t Give up the Ship”. This was a paraphrasing of the dying words of Capt. James Lawrence who was a friend of Perry and who had died in an earlier battle in the war.

We hung out in the park for a while and it was very peaceful. The island has a very narrow Isthmus right at that point and you can see water on either side. The bay itself is very calm compared to the waters of Lake Erie.

We Are Stranded

The problem now was how to get home. There was no way we could make it back to the other end of the island where we had landed in time to catch the last boat home. Fortunately there is a ferryboat that leaves directly from the bay. Had we known what we were doing, we would’ve taken the boat that landed there. So we purchased a ticket to go back on a different ferry from this different departure point.

There was another problem however. This ferry did not go back to the Catawba Island Terminal where we left the van. Instead it went back to a place called Port Clinton which is 9 miles away from where the van was. We didn’t have any other choice. We had to take this ferry home.

We depart from the north side of the isthmus and had to go around the east end of the island to get back to the shore. It gave us a nice scenic view of the island. On the trip out there were seagulls that flew alongside us but on the trip home there were many more birds and they would fly up to the rear of the ship and the passengers would throw breadcrumbs to them. You could buy a package of breadcrumbs on the fairy. The birds would swoop down and catch them in midair. One person tried holding out their hand palm up with food in it but I never saw a bird swooped down to take it. I wouldn’t have done that.

When we landed at Port Clinton we called a taxi and Barb left Jim and me alone in the parking lot while she went to get the van 9 miles away. Then she drove the van back to pick us up. There was another guy there in a wheelchair with his own van but there would be no room for three wheelchairs in his van so we didn’t even bother to ask him if we could bum a ride. There was some sort of fast food restaurant across the street and we thought about going to get something to eat while we waited but we decided it was best to just stay put. We felt safe where we were.

There are actually 2 different fairy routes that leaves the north side of the island and they go to 2 different locations on the shore. One of them went to Marblehead and the other to Port Clinton. I couldn’t remember which route we went but I did remember that Barb said the taxi ride was 9 miles. So in researching for this blog I used Google maps and discovered that it was Port Clinton and not Marblehead where we returned. Marblehead is 11 miles from Catawba Island according to Google maps. Recalling this whole experience from 1986 has really opened my eyes to how dependent we are in this day and age on the Internet and all of the resources it provides. It makes me wonder how we ever survived without it.

The journey back to Toledo was uneventful. When we got home we ordered some pizza. We were all pretty exhausted from our adventure especially Barb who had walked the length of the island and still had to put Jim and me to bed. We had plans for the next day to drive to Detroit to visit a CompuServe friend of mine but we decided to cancel because we had already had enough adventure for now.

Home Again via Chicago

Barb and I left Jim in the capable hands of his backup aide Dede and we drove onto Chicago to visit other friends there. We had a great time in Chicago for the weekend. I don’t remember why but I did not shoot any video for this leg of the trip. Now I wish I had. Didn’t take any photos either.We got in a huge traffic jam coming through the interstate in Chicago. We also drove by Soldier Field where the Indianapolis Colts were playing the Bears in a preseason football game. I thought it was ironic that I was in town for a Colts game without tickets to see it. We got lost once along the way trying to find the hotel where I had booked us a room. We eventually made it to the right place and checked in. I had my laptop with me and I logged into CompuServe to touch base with friends. They started teasing me about spending a weekend in a hotel room with a nurse. Barb didn’t appreciate that. Barb met up with a friend of hers who lived in Chicago and he hung out with us and spent the night. I don’t remember his name or how she knew him. I had dinner with a friend from CompuServe. Sadly I don’t remember her name either.

This was my third trip to Chicago. The first was a family vacation when I was 13. I always tell people “Yeah… I was in Chicago in ’68. The scene with all the hippies in Grant Park was wild.” The truth was that vacation was the week before the infamous 1968 Democratic National Convention with all of its riots and controversy. And I neglect to tell people I was only 13 at the time and was on vacation with my mommy and daddy.

My second trip to Chicago had been just a few years prior in 1981 when I was invited to exhibit some software in the “John’s Hopkins First National Search for Computer Applications for the Handicapped“. My software VersaScan won seventh place in the Midwest regional and was one of the top 100 in the country. (See page 276 of this PDF “Communicating with Tokens” by Chris E. Young) That event was held in the Museum of Science and Industry so I got to spend lots of time there as well as my other favorite museums.

Whenever I’m in Chicago I have to visit the museums. My favorite place is the Adler Planetarium which sits out on a little peninsula on Lake Michigan. I also like the Shedd Aquarium. I’m pretty sure we went to the Planetarium but I don’t think we visited the aquarium this time. [Update: After reading this, Barb reminded me we did go to the aquarium and now that I think about it more, I do remember parts of that adventure as well.]

Getting My Eyes Maxed at the OmniMax

I definitely wanted to go to the Museum of Science and Industry because they had just installed a new OmniMax Theater. If you’ve never heard of it, OmniMax is a domed version of IMAX. Some theaters refer to it as IMAX Dome while others continue to use OmniMax. The film was a NASA documentary called “The Dream Is Alive“. This 1985 film was made during space shuttle mission STS 41C aboard the space shuttle Challenger. Ironically in January 1986 a tragic accident destroyed the Challenger and its crew of seven and the dream was almost destroyed with it. It would be two years before shuttles flew again. It was bittersweet to see the Challenger in such a magnificent format.

We didn’t have time to tour the entire museum. We just went directly to the OmniMax theater. Unfortunately when we got there, the show was sold out. Some nice man who was in line with his family gave me his ticket for free. Barb waited in the lobby for me. The show was only about 45 minutes. It was the first IMAX film I had ever seen. The OmniMax Theater screen is huge. It’s like you took a dome and tilted it on its side and suspended it above the stadium seating. If you would stare at the center of the screen and did not look side to side, the image would fill your entire field of vision. The multichannel digital surroundsound was state-of-the-art. You can feel the floor shake when the shuttle took off. I’ve got a DVD of that film but of course watching it at home even in HD with my home surround system is nothing like IMAX.

I was instantly hooked and I’ve been an IMAX addict ever since. I wished we had IMAX theaters in Indianapolis. In 1996 my wish came true. The Indianapolis Children’s Museum opened a theater called the CineDome. While not technically IMAX nor OmniMax, it was much like a smaller version of the OmniMax in Chicago with a domed screen. Although built by iWerks and not IMAX it would still show 1570 format films some of which were IMAX films. In 2002 the Indiana State Museum opened an IMAX with a traditional flat screen but it had 3D capability. The CineDome closed in 2003 and the museum turned it into a dinosaur exhibit called Dinosphere which is pretty cool but I still miss the CineDome. There are now 3 other IMAX theaters in the central Indiana area but they are smaller digital IMAX facilities as part of a multiplex of other theaters. Purists call these smaller theaters lie-Max but I still like them. Still they don’t compare to the large IMAX at the Indiana State Museum or the Chicago OmniMax. In 2017 the Chicago OmniMax theater was renovated and the IMAX film projector removed. It was replaced by a laser digital projection system and was renamed the Giant Dome Theater. Maybe someday I will go back and see how the upgrade looks. But I still have fond memories of the original OmniMax and my first IMAX experience.

Cherished Memories and More to Come

The journey home to Indianapolis the next day was uneventful. Barb drove back to Toledo. We stayed in touch but I did not see her again until 1990. By then she had a boyfriend and a baby. She was leaving Jim to move to Texas. She did return to Toledo a few years later to care for Jim but sadly he passed away in 1995. She then went to school and got a job doing the social work. We’ve stayed in touch via Christmas cards, email, and an occasional phone call. I will see her again in a couple of weeks when she will come visit and we will decide if she’s capable of helping out as my caregiver. If so she and Josh and their cats will move here. I really hope it works out. Regardless of what happens, I will always cherish these great memories we’ve made together for over 30 years.

YouTube Video of Trip to South Bass Island

I May Have Found a Roommate

I may have found a roommate to move in with me.

The day I posted my previous blog saying that I was looking for one or two people to move in with me and live here rent-free in exchange for being my part-time caregiver, I got a Facebook messenger message from my old friend Barbara Alkema saying “How physical is the job?”. I totally freaked out.

I’ve known her for over 30 years. She currently lives in West Virginia with her adult son Joshua and 4 cats. I had thought about asking her if she would consider coming to Indiana but I thought it was very arrogant of me to presume that someone would pick up their life and move to a different state for me. Barb points out that I sent her a link to the blog hoping she would “take the bait”. Well maybe yes but I sent the link everywhere I could think of. In addition to posting on Facebook I sent emails to lots of people including her. Unfortunately I had an old email address that was no good so when I asked her about her current email then I sent her the link. The idea that she might respond positively seemed like a ridiculous fantasy.

This was much better because she was volunteering based on my open casting call and I didn’t look like I was an egotistical ass asking her to uproot her life and move to a different state.

Who Is She?

Back in the early 1980s before Al Gore invented the Internet (okay I know he really didn’t) there was an online service called CompuServe. You would use dial-up modems just like AOL which appeared a few years later. There were live chat rooms, discussion forums, news pages, and you could book travel just like you can on the Internet today. But it was all plaintext with no pictures and you connected at 300 baud. To compare that when AOL and other Internet services stopped using dial-up modems they were running at 56,000 baud. Today’s Internet is thousands of times faster.

There was a disability discussion group and that’s where I met a man named Jim Grubs. He was 50 years old, lived in Toledo Ohio, and had some kind of muscular dystrophy. It wasn’t the kind that I have. Jim’s parents were in their 80s and could not properly care for him but they had some money and so they hired a full-time live-in caregiver named Barbara.

I communicated with them both online through CompuServe and occasionally by phone. One time Barbara took the weekend off from caring for Jim and left him in the hands of a backup aide named Dede. She drove down from Toledo to Indianapolis to spend a few days with me. We had a great time together. We went out to dinner with my friends Rich and Kathy Logan and then went on to a comedy club where we saw a new rising star in the comedy world. You might’ve heard of him… His name was Jerry Seinfeld. This of course was years before his TV show. We had a great time. The next day Barb and I went over to the Speedway to tour the museum and who did we run into? Jerry Seinfeld! We told him we really enjoyed his show the night before. I wish I had the presence of mind to ask for an autograph.

In August 1986, Barb came back to Indianapolis and picked me up in my van and drove me to Toledo to visit with her and Jim for a few days. We took a trip on a ferryboat out into Lake Erie to a place called South Bass Island and to a resort town called Put-in-Bay. There is a huge lighthouse on the island that commemorates Commodore Perry’s victory in the battle of Lake Erie during the war of 1812. We also had a great time visiting with several of their friends and then backup aide Dede took over and Barb and I drove from Toledo to Chicago where we each visited some friends there. Then she drove me back to Indianapolis. I’m going to do an entirely other blog post about that whole adventure including a YouTube video of some VHS tape I shot on her trip out to the island.

The last I saw Barb in person was in 1990. She had quit working for Jim and was on her way to move to Texas with her seven-month-old baby boy Joshua. I remember she was here the day that my Grandma Osterman went to the hospital for the last time before she passed away.

Barb later returned to Toledo to work for Jim briefly but he passed away in 1995. Barb and I stayed in touch over the years. By email, Facebook, and the occasional phone call.

She went to school and got a job doing social work. A few years ago she developed health problems and had to go on Social Security Disability, Medicare and Medicaid. I wasn’t really sure if she was in good enough physical shape to help take care of me or if she would be interested in moving here. I did know that they were struggling financially but like I said, I thought I would be an idiot to presume she might move here even if she could.

What Is the Plan?

Over the past couple of weeks we’ve talked over all of the ins and outs of her moving here. She of course discussed it with her son Josh who has been a Facebook friend of mine for some time. He is in his late 20s. He’s a bit of a nerd like me. Loves sci-fi. Hates Trump. In a way more nerdy than me. He plays Dungeons & Dragons which I don’t. I’m sure we will get along just fine. Josh currently has a crap job working in a drugstore making crap wages. He was already looking for a better job so he will have to find one here. He has friends in West Virginia that he plays D&D with once a month but they say they can Skype with him or he can come visit sometimes. He also plays online with other people which you can do anywhere. He is on board with the plan.

I’m not wild about the idea of 4 cats coming with them because I’m not much of a cat person but given a choice between A) Living with a longtime friend, her son who is cool, and four cats versus B) Living in a nursing home… I will take “Plan A” any day.

You might think that we live in the United States of America but actually we live in a bunch of egotistical local jurisdictions that each think they know what’s best for their constituents especially when it comes to healthcare. While Medicare is completely federal, Medicaid varies state-by-state. We will have some hoops we need to jump through to get her eligible for Indiana Medicaid but after consulting with my lawyer we think we’ve got that one figured out.

Barb has plans to go visit a friend in a different part of West Virginia the week of April 6. The current plan is that she will spend a week with that friend that then take a bus here to Indianapolis and hang out with me for a week or so. It will probably be too strenuous for her to get me up and bathed and dressed in the morning but we are hopeful she will be able to put me to bed at night which is much easier. Her visit will verify just what she is and is not able to do. If that goes okay then she will go back home and begin to pack. Josh may come before her to start looking for a job.

I currently get nursing services from 8 AM- 6 PM Monday through Friday but that’s only because Carol is living here as my primary caregiver and she has to work. With Barbara living here full-time and not working I will no longer be eligible for that kind of nursing support. I will end up going back to having a home health aide come in for a couple of hours every day to get me up and dressed. We can also get respite nursing to come in and take care of me if Barb needs to go somewhere like a doctor appointment. We would essentially be back to the way things were with me and my dad before he got sick this past summer. My weekend aide has that she would love to come back and work for me seven days per week the way that she did for the past two years before we got nursing help.

In my original blog posts I said that I would need more than one roommate so that they could share responsibilities. However because Barb doesn’t have any friends around here and doesn’t work it will not be a burden for her to be here full-time. Josh can help out around the house as well as he already does where they live in West Virginia.

I took a tour of my house shooting video on my iPhone and sent it to them to show them what the house looks like. Barb will take over the main bedroom that used to be my parents room and Carol is currently using. We have a junk room which contains my mom’s old sewing machine, a worktable that my dad used to build my gadgets, and a closet full of my ventilator supplies. We will clean that room out and it will be for Josh. We’ve already cleaned out my office closet in anticipation of moving stuff out of the junk room. Karen will probably take mom’s old sewing machine. We are going to get rid of one of the sofas in the family room and the organ which nobody plays anymore. That will give us room to put stuff in the family room.

Barb will bring some furniture of her own and some she will put in storage here. They will bring their beds. She has a hutch she likes that we can probably put in the family room. She has her own sewing machine. She sells stuff she makes on esty.com at a store called MakeItSewByBarb. We just priced what it will cost to rent a U-Haul truck and it’s going to be expensive but it’s cheaper than hiring a real mover.

She is excited that we have a dishwasher. Josh is excited that I have a 3D printer he can use to make D&D figurines and map pieces. I’m excited that they have a 60 inch TV they are bringing. Mine is only 47 inches but it will do 3D so we will keep them both. We both subscribe to Netflix, Hulu, CBS All Access and Amazon prime so we can consolidate those and save some money. I will probably cancel some of the premium channels on our cable. I will probably cancel our landline telephone since we all have cell phones. There are a lot of details to work out but I’m extremely hopeful that we can do it.

So send up a prayer or two that we can make this work. This is going to be a win-win situation for both of us if we can work out the details.

I need your help to find a roommate. Actually two roommates.

I need a roommate. Actually I need two but at least one to start with. I’m writing this blog directed towards friends, family, friends of friends, friends of family, family of friends etc. in hopes that someone knows someone who can move in here and help be my caregiver. The idea is that they would live here rent-free with free utilities, free Internet, free cable and use of the house in exchange for part-time responsibility of taking care of me.

I currently can get nursing care from 8 AM until 6 PM on weekdays. I also have a home health aide who comes on Saturday and Sunday morning for 2 to 3 hours. She gets me bathed and dressed and up in my chair like the nurses do. But because of the rules and because I have a trach in a G-tube she cannot be alone with me. So these hypothetical roommates would be required to be here evenings and weekends. I may also be able to get some additional nursing hours called respite hours but they might only be 10 or 12 hours per week. These respite hours are not flexible so we would have to come up with a regular schedule

I need two people because it’s a full-time endeavor. If one person does it, they wouldn’t have a life beyond going to work while my nurses are here and taking care of me the entire rest of the time. But if I have 2 people sharing the duty it becomes a much more manageable job. Currently my sister Carol is doing all of this on her own. The idea is that we will recruit a roommate who will share the responsibilities with her and then if it works out we would recruit a second roommate and Carol would be free to move on with her life

Anyone with normal physical ability who can follow simple instructions can do the job. It doesn’t have to be a nurse or a trained caregiver. None of this is rocket science. I can train them to suction my trach and do G-tube feedings. I can train them to put me to bed using my Hoyer lift. They would have to undress me and put a diaper on me. Is a simple task to put me on the ventilator overnight. While it’s quite a job to get me up and dressed, the nurses and aides will do that. Putting me to bed is relatively easy. It’s a law of physics that is easier to tear things apart that it is to put them together 🙂 The only nasty part of the job would be getting me on and off a bedpan and getting me cleaned up. While wiping my ass is not a fun job, as the children’s book says “Everyone Poops“. You wipe your own ass and it’s no different than wiping mine.

So what I’m asking you to do is to look around and see if you know of someone who might be interested in the job. At some point I will probably do some advertising to recruit a college student or perhaps even a nursing student. It doesn’t have to be a nursing student but they might be more inclined to take the job. It would be much better if it was someone that I knew or someone that you knew and could say that they were a decent reliable person.

That’s the short version of the story. But anyone who knows me, knows that I can’t tell a short story so I invite you to continue reading while I give you all the background on how I came to this point and why I need these roommates.

This idea is fraught with problems. Where do I recruit such people? How do I trust them? Sure we can do a background check or a drug test but that doesn’t mean I will find the right people or that I will find two of them and all three of us get along. What do I do when one of them quits? What are the legal issues? Can I write a roommate agreement that is not 80 pages long like Sheldon Cooper on “The Big Bang Theory”? These are all questions I can’t answer right now. But I have to pursue this idea because it is the only way I’m going to be able to avoid ending up in a nursing home.

What did he say? Nursing home? Oh no!

For many years whenever I mentioned to someone that at some point in my life I may end up living in a nursing home, the most common reaction has been a very quick “We can’t let that happen.” It takes a variable length of time however before the person saying that realizes there really isn’t a whole lot they can do to prevent it if it comes down to that. Let’s face it I am an extremely high maintenance person 🙂

In recent years I’ve become even more so. Basically I need someone to take care of me 24/7. It is not at all safe for me to be alone for any length of time. At one time it was common for mom or dad to leave me alone for up to an hour or two while they would run an errand. But in recent years it became less and less safe for me to be alone. Dad began scheduling his trips to the grocery or to the doctor at a time when my home health aide was available to take care of me.

Once I got my trach, things changed significantly. According to the rules, a home health aide or certified nursing assistant (CNA) is not allowed to deal with my trach or G-tube. In has to be a real nurse. A CNA is not allowed to be alone with me. That meant that dad had to change his schedule and cannot leave the house while the aide was here. Fortunately we were able to recruit a nurse who was permitted to care for me. He would come regularly one day a week and had some flexibility that he could come on other days as well.

My need to have someone here ALL of the time was clearly illustrated in an incident about a year ago. Dad went outside to do some yard work. He was only gone about 15 minutes before I found myself in real distress needing my trach suctioned. This was despite the fact I had told him before he went out the door that I was okay. I really thought I was.

Dad was able to do it these past few years basically because he didn’t have a life beyond caring for me. When he did go to a retirees meeting or go out to lunch with a friend on occasion we had to recruit other friends to stay with me. But dad didn’t have much of a social life so he didn’t have to give up much to be here all the time. In my eulogy of him last month I told the story of him coming to visit me in the hospital despite terrible weather that should’ve kept him home. His response was simply “I didn’t have anything better to do.” And that was the truth. I could rely on his care 24/7 because he didn’t have anything else to do.

That’s what it takes to keep me out of a nursing home. And that’s why all the people who say “We can’t let that happen” really can’t replace him. My other friends and family have a life. They have jobs and friends and commitments. They can’t devote their entire life to me and my care no matter how much they don’t want to see me in a skilled nursing facility.

When dad became ill this summer, my sister Carol moved in with us to help care for the both of us. She did this even though she herself was battling some very difficult health problems. She had surgery for throat cancer which was completely successful however the doctor still recommended an extensive round of radiation treatments which were devastating to her. Although she is back at work she still has a long road of recovery ahead of her. She’s been unable to eat enough to keep her healthy and recently had a G-tube installed so that she can get sufficient nutrition to heal. Her sacrifices for dad and I over the past many months have been phenomenal and I am totally lost for words to express how much it has meant to me.

Even when dad was doing well and Carol had not yet begun her difficult radiation treatments, I found myself at times overwhelmed at the thought of what she was going through to take care of us. I’ve needed pretty much this level of care my whole life. While I understand that mom and dad had made sacrifices to care for me for 60+ years, they are my parents. Taking care of your kids is part of the job. I don’t want to say that I ever took them for granted because I didn’t. But it just seemed natural that we had the kind of relationship that we had. On the other hand although Carol is family, what she has done for me in these past months has been way above and beyond the call of duty.

When all of this started, Carol expressed her commitment to stick with us to the end that we knew was coming from my dad but not necessarily for my entire life. We always knew that this would be a temporary situation for me. I intend to do everything I can to honor that concept that she wouldn’t be here forever.

In the late 1980s my grandma Osterman lived with us through all of her final health issues for about five years. The stress on my mom being a caregiver for grandma and I was overwhelming. Perhaps you didn’t see it but during those years it changed her personality. Much of the time she was on the verge of a nervous breakdown. We all made sacrifices in those days but we understood that it was important to grandma to be here among family and it was important to my mom that she be able to care for her. After grandma passed away in 1990, mom recovered and became again the happy, kind, giving person that she had been prior. Dad and I spoke many times that we wanted to make things as easy as possible on Carol having seen what it cost my mom to take on such heavy responsibility.

Throughout the process, we’ve been exploring possibilities for my future once dad was gone and it was time for Carol to move on with her life. Although a skilled nursing facility was at the bottom of my list of options, unfortunately we had to explore it first because it was a bit of an emergency. It was questionable whether Carol would be able to continue to help out while recovering from her surgery and radiation. At one point she did move back home and dad and I were on our own. Fortunately dad was still in good enough shape that we could get by with the nursing help for several weeks. If dad had gotten worse before Carol was ready to come back, I needed somewhere to go.

When we weren’t certain that Carol was going to be able to continue to help out, I went looking for an SNF that could handle me. We had help from my caseworker from an agency called CICOA who handles my Medicaid paperwork. It turns out that finding a facility that will take someone who uses a ventilator is not easy. Most SNFs don’t have the staff to handle it. While anyone could do the job in my home, because of liability and other issues such a facility has to have highly trained people. I don’t really use the ventilator to keep me alive. It’s really just like using a CPAP or BiPAP to help me sleep at night. Before I had the trach I used a CPAP. But once you say “the patient is on a ventilator” that triggers all sorts of problems.

We finally found a facility called Greenwood Health Care in Greenwood. My friends Rich and Kathy drove me down there one afternoon for a tour. It was a reasonably nice place. I would have to share a room with another person and it would be a small living space but there is ample common area that I can hang out with the other residents. The minute you say “nursing home” it immediately conjures up all sorts of bad images. Someone like me doesn’t want to end up stuck in some wing with a bunch of Alzheimer’s patients.

One of the key things that I asked the staff in Greenwood was “Are there other people here like me? Are there people that, for the lack of a better term, I would consider peers? Are the people who are here because of their disability not because of age or medical condition?” She said that in the respiratory unit there were naturally lots of sick people. However in the unit adjacent to that there are people like me and I would have ample opportunity to make friends and interact with them in the common areas. That was a great relief to me.

I came away from the tour feeling greatly relieved that even if I had to take this worst-case scenario of a nursing home that I could get by reasonably well.

Obviously a nursing home was not my first choice. What I really would like to do is be able to stay in this home which I’ve lived in for 60 years ever since I was three years old. We talked about the possibility of my sister Karen, her husband Terry, their son Cole and their friend/roommate Dawn moving here. Even though it would be a little bit easier on them because there are more people to share the load, uprooting their family and moving here is just too much to ask.

I talked to my case manager and asked her what is the maximum amount of nursing hours she believed I could possibly get. She indicated that I might get 45-50 hours per week of what is called “preauthorization” or PA hours such as I was getting from 8 AM – 6 PM currently. And additionally 20-30 respite hours per week. That just isn’t enough to get me 24/7 care in my home.

Another option is to find one or two other disabled people each of whom could get authorization for maybe 10-12 hours per day. Then between all of us combined together we could get 24/7 care by utilizing each of our authorizations sequentially. That has worked in other cases. The problem is we would have to find someone very much like me who needed nursing care for a trach and a ventilator and not just a regular home health aide. Finding two or three such people has pretty much proven to be an impossible task.

Another option is what’s called a residential group home. This is a regular house managed by some agency that would have three or four disabled residents sharing caregivers 24/7. Again the problem is finding one that would be staffed by people who can handle the trach and the ventilator. To the best of our ability we have not been able to find such a group home. I had other reservations about the group home setting. I’ve heard some bad stories about them that actually made a nursing home sound like a better place. But given that there aren’t any group homes that can provide the level of care that I need, this one is off the table as well.

I have been exploring other Medicaid programs beyond the one that I currently use. But I don’t think any of them are going to qualify me for 24/7 care simply because if there was such a program, you wouldn’t need group homes to exist or at least not in the numbers that they do. I’m still looking into alternatives but I’m not at all hopeful that there are such programs.

All of our efforts have been directed at trying to get more nursing hours. We explored the possibility of trying to get private funding to hire more nurses. All of those people who said “We won’t let that happen” might be able to contribute something financially to hire more help. But I keep going back to the fact that I don’t really need a nurse. The only reason it has to be a nurse is because that’s the only way Medicaid will pay for it. I just need a reasonably able-bodied average strength person who won’t freak out at the idea of wiping my ass. And recognizing that one person can’t do it all alone… I need two of them

One of my nurses told me the story of another client she once served who had a college student living with her. The student would take care of her when the nurses were not on duty. That was the inspiration for the plan that we are pursuing.

I don’t need a place to live. This house is paid for.

I don’t need nurses. Any able-bodied person can do it.

I don’t need other disabled roommates.

I just need someone to be here when my nurses are not and to do some basic care.

While looking for the exact proper solution to the problem it occurred to me that what I was looking for was a unicorn… A beautiful mythical creature that probably didn’t exist. But the odds of finding and able-bodied roommate to come here and sharing the responsibility of caring for me doesn’t seem like such a long shot. I think I’m now looking for a horse instead of a unicorn.

That doesn’t mean this is going to be easy. Finding the right people will be hard. Finding someone who can get along with me and the other caregiver will be hard. Whoever we get is likely not going to stay forever so there will be turnover. These are the issues that Carol seemed most concerned about when I proposed the idea to her. They concern me as well. But I always have Greenwood as a fallback position. And I really can’t see going to Greenwood until and unless I have given this my best shot.

So look around… Think about your friends and neighbors and coworkers. What about nieces and nephews and cousins and their friends. Put out the word and help me find someone to be my roommate.

Sometime soon I’m going to put up a webpage with perhaps a YouTube video designed for the general public to view and you can direct them to that page. By the way if anyone is interested and it makes a difference, I live in the Eagledale area just north of Speedway. It is a four bedroom house and the caregiver would get their own bedroom.For now this is just directed to people I know. I’m hoping you can help.

Q & A about Spinal Muscular Atrophy – Celebrating SMA Awareness Month

Note: this blog was originally written in August 2018 but was updated in 2019 with more current information and corrected some errors.

This month has been designated by someone as “SMA Awareness Month”. As someone who has had Spinal Muscular Atrophy or SMA his entire life I don’t think I could be more aware 🙂 I always wonder about people who have breast cancer or who have family members who have had breast cancer if they ask the same question whenever they say it is “Breast Cancer Awareness Month”?

Really?

Is there anybody out there who has never heard of breast cancer?

But seriously folks… Back to the topic at hand. SMA is considerably more obscure. Even if you’ve known me many years you may not know why I’ve been in a wheelchair my whole life. What put me here? What is SMA?

So in the spirit of SMA Awareness Month” I thought I would spread a little knowledge and invite questions.

What is Spinal Muscular Atrophy – SMA?

SMA is a genetic disease. Both my mom and dad were carriers and did not know it. Estimates are that approximately one in 40 to 50 people are carriers. Even if both parents are carriers there is only a one in four chance that each child would have the disease. Of course that’s just a percentage over the long haul. You can flip a coin 10 times, have it always come up heads and the next one is still 50/50.

There are a variety of types of SMA. Type 1 is extremely severe and without intervention those kids are lucky to see their second birthday. With respiratory support, G-tube feeding etc. they can live several years. There is a new treatment that can prolong that. More on that later.

I have Type 2 which is not as severe but it generally means that I never walked (which I did not). Type 3 people can generally walk until their early teens sometimes later. There is a type 4 which actually is a significantly different disease but is still called SMA and I don’t know much about it. Even within the types there is a great variation. On various Facebook groups related to SMA I hear people describe themselves as “a strong Type 2” or “a weak Type 2”. I have a Facebook friend who lives in Canada and we are both Type 2 but her current physical ability at age 50 is about where I was at 21 or 22.

The disease is an overall weakening of your muscles. It does not involve any loss of sensation or paralysis. The muscles just gradually get weaker a.k.a. “atrophy”. It is actually a neurological disease however not necessarily a muscular one although it is often lumped into the general category “muscular dystrophy”. There are nerves in your spine called motor neurons. They are responsible for making your muscles move. They have nothing to do with your sense of touch or sensation. The motor neurons quit working and eventually die. That causes the muscles to atrophy.

The disease is in some ways similar to ALS or Amyotrophic Lateral Sclerosis. Although the root causes are very different and the progression of the disease is different, both of them involve the loss of function of motor neurons. ALS is often referred to in this country as Lou Gehrig’s disease because of the famous baseball player who had it. In Europe especially the UK is referred to as Motor Neuron Disease. Its most famous patient was the late Stephen Hawking. So while there are some similarities, they are completely different diseases.

What Went Wrong?

Okay how does it work? Why did I end up like this?

In every cell of your body you have 23 pairs of chromosomes. Chromosomes are coiled up strands of DNA. Sections of the DNA strands called “genes” are strands of instructions that tell your body how to do different things. DNA is made up of 4 molecules chained together like a twisted ladder. You’ve probably seen drawings or models of it. Each rung of the ladder is made up of two molecules linked together. The 4 molecules that make up DNA are called A and T or C and G. The order of these four letters is a kind of code. The strings of code are divided into sections called exons and introns. The exons are the code and the introns are just sort of filler material.

In your number 5 chromosome at a location called 5q13.1 is a gene called SMN 1. SMN stands for “Survivor Motor Neuron” gene. It produces a protein called the SMN protein. Without a sufficient quantity of this protein, your motor neurons die and then your muscles weaken and presto… You’ve got SMA.

In people with SMA like me, there’s a mistake in the SMN gene. The seventh exon is missing. Think of the chromosome is a strip of movie film where you cut out a section and spliced it back together. When DNA gets copied when your cells duplicate, it tends to get copied in chunks. The ends of the chunks are like pieces of a puzzle that only fit together in certain ways. You have probably worked a jigsaw puzzle where there were pieces that almost fit even though they weren’t the right ones. It just so happens that the ends of the chunk around that particular exon are similar enough that under the right conditions (I guess I should say wrong conditions) they can stick to one another forming a small loop. That leaves a gap in the film strip and even though they aren’t quite right, those pieces end up sticking to each other and the little ring of DNA gets lost.

Once a mistake occurs, it’s passed down generation after generation through carriers of the disease. But it is technically possible that that mutation can spontaneously occur and someone with no history of SMA in their family could get the mutation when that little ring accidentally forms.

Once you got a screwed up SMN1 gene, it’s not going to make the proper protein, and so you’re screwed. Almost…

As Jeff Goldblum famously says in all of the Jurassic Park movies “Life finds a way”. Mother Nature or God or natural selection or whatever you believe in… Has a backup plan. There is another gene that everyone has further down the fifth chromosome called the SMM2 gene. It’s a backup copy. In fact some people have multiple backup copies. But there’s a problem… The backup got corrupted. In the long strings of A, T, G, and C there is a one letter mistake. Think of it as a single bit error in a computer code. Everybody’s SMN 2 genes have this mistake. The end result is that SMN2 only works on average about 20% of the time. So even if your SMN1 gene is totally screwed like mine, the SMN2 occasionally makes up for it. But it’s just not enough.

How Does the Inheritance Work?

Now that we’ve established that the cause is a missing chunk out of your SMN1 gene, how does that get passed on?

As mentioned previously everyone has 23 pairs of chromosomes. You have 2 number 1, 2 number 2 etc. You’ve got one of each from each parent. The problem is in my fifth chromosomes. We each have two of them. One came from mom and one from dad. My mom and dad were both “carriers” of SMA. That means that in their fifth chromosomes they had one that was normal and one that was damaged by having that number 7 exon missing. They don’t exhibit the disease because their good copy on their other number 5 chromosome covers it up.

When I was conceived, I had a 50-50 chance of getting either a good copy or a bad copy from each of them. If I got the good one from both mom and dad (25% chance) I would’ve been okay as would my children and children’s children etc. If I had gotten a good one from mom and a bad one from dad or vice versa then I would be a carrier as well. Each of those has a 25% chance for a total of 50% chance of being a carrier. The remaining 25% chance is that I got the bad copy from both. Lucky me!

My sister Karen obviously does not have the disease so one of three things has happened. Either she got 2 good copies, she got a good one from dad and a bad one from mom, or she got a good one from mom a bad one from dad. That means there is a 66% chance she is a carrier. If her husband is not a carrier as well, her kids are at no risk of actually getting the disease. Her only son Cole is fine.

Because we know that my mom and dad were both carriers, that means that at least one grandparent on each side was a carrier. That means that my Uncle Keith on my dad’s side or my late Aunt Jody on my mom’s side have a 50-50 chance of also being a carrier. If they are not carriers, none of my cousins are at risk nor will their descendants carry it. If Keith or Jody are carriers, my cousins could not exhibit the disease unless Aunt Barbara or Uncle John were also carriers. None of my cousins nor their kids have exhibited the disease. If my Aunt or Uncle were carriers, my cousins have a 50-50 chance of being a carrier themselves.

But what about me?” Pardon me quoting the old joke “I have no children… That I know of :-)” and at age 64 with no romantic involvement in my life I doubt that I will. But there’s no reason I couldn’t have children. What about them? People who have the disease of course pass it on to their offspring and so technically they are carriers but usually we reserved the word “carrier” for someone who carries the gene but does not exhibit the disease. But anybody with the disease does automatically pass it on. I’ve got two bad copies of the gene so at minimum, all of my kids would be carriers. If my wife was not a carrier then that’s all the risk we would have. If she was a carrier, half of our kids would have SMA and anyone who did not have it would be a carrier. If my wife had SMA, we would both have 2 bad copies and therefore ALL of our children would have two bad copies and have SMA.

How Do You Test for SMA?

They just take a blood sample and send it to a genetic testing lab. They sequence your number five chromosome and look for the missing chunk. If you’ve got a double deletion like me you have the disease. If only one of your two number five chromosomes has a deletion they can tell that you are a carrier. These tests can also be performed prenatally using amniocentesis or by testing in vitro cells prior to implantation. Which brings us to our next topic…

How to Cure SMA

Short version… Become a Nazi.

Either kill or sterilize everyone who is a carrier. They will never pass on their defective qualities and in one generation SMA will be virtually wiped from the planet. We become a super race of genetic perfection and rule the world. There is the possibility of spontaneous mutation by creating that little DNA ring by mistake that I talked about earlier. We can kill those people off as we find them.

Alternate solution only slightly less drastic… You can test for SMA by doing amniocentesis. If it turns out that your unborn child will have SMA, abort it.

Further solution only slightly less drastic… If you do in vitro fertilization (otherwise known as a test tube baby) conception occurs outside the womb. The fertilized egg is allowed to grow until it is a small clump of cells. You then extract a cell and test it for SMA. If it has SMA, you flush the embryo down the toilet and only implant the healthy ones. Sorry… that toilet comment was just speculation for dramatic effect. In reality the embryo is probably incinerated in an oven. We are Nazis after all. Right?

While I don’t know of anyone actually going around sterilizing or killing SMA carriers these days, I’m sad to report that abortions and selective in vitro procedures are being done to avoid having children with SMA.

In short… These people believe that people like Chris Young ought to have never been born.

Pardon me if I take that personally.

I’ve never been opposed to abortion just because the Catholic Church says so. I’ve always felt it was a really bad idea through my own innate conscience and sense of morality. Then I am especially opposed to abortions which implement such selective breeding practices. It turns human life into an animal commodity for breeding purposes rather than the sacred thing that it is.

You could ask hypothetically what would a person be like if they had not caught some disease like polio or AIDS? You can ask what if they never suffered a spinal cord injury or had cerebral palsy due to anoxia during birth. But you can’t say what would someone with SMA be like without it. It’s genetic. It’s part of who we are.

Despite the harshness with which I have described this situation, I am sympathetic to parents who are facing tough decisions when they know that they have the potential to create SMA kids. The situation is especially complicated by the fact that there is no genetic test to determine which type of SMA a particular kid will have. As a Type 2 person who has lived 63 years and had a pretty good life in which I’ve made lots of effort to make the world a better place because I was in it, it’s easy to condemn anyone who doesn’t want people like me to be born. On the other hand Type 1 kids have it much, much harder. As I mentioned before, without serious intervention their life expectancy is 18-24 months. That intervention involves having a trach, ventilator, G-tube for feeding, 24/7 care, and a seriously impaired ability to do much constructive with their lives. Nobody wants to see their kids suffer.

I feel a strange connection to the Type 1 kids. Basically I’m in the same shape at age 63 as they are at age 2-3.

I have no problem whatsoever with parents who choose not to have children if they feel ill prepared to provide adequate care for a child with SMA. All parents should assess their capacity to deal with parenthood and all that it might entail before deciding to have a family. That is the responsible thing to do whether you have genetic risk or not. But I draw the line at selective breeding.

One also has to take into consideration that there now is a treatment for SMA if not a cure. The mortality of Type 1 is not as significant as it once was. More on that in the next section.

I recently encountered a family online who was considering preselection of in vitro embryos to avoid having a kid with SMA. They had already lost a Type 1 infant and could not bear the idea of suffering another such loss. They were opposed to the idea of abortion so I give them some credit for that. I can’t begin to imagine in my wildest dreams what they’ve been through. So it’s tough for me to sit in judgment of them. I really can’t.

On the other hand… I also met a family online who had a kid with SMA Type 1 and were inspired by the experience to adopt other special needs children.

Life finds a way.

How Do You Treat SMA?

Up until recently you couldn’t do jack shit. All you could do is try to mitigate the consequences of the disease.

I wore a back brace from the time I was five years old until my early 20s. It was basically a corset made of cloth and metal stays that we bought from the Spencer Corset Shop. Then in my 20s I switched to a plastic body cast the kind used for treatment of scoliosis. My spine has 2 curves in it 90° each. For various reasons at an early age I quit going to orthopedic doctors because they weren’t doing anything for me. That was a mistake. I should have had spinal fusion when I was a teenager. Most kids with SMA these days do get spinal fusion. They also get metal rods earlier than that that can be adjusted as they grow.

The biggest risk for people with SMA is respiratory issues. I’ve had pneumonia a couple of times. Most people with SMA use a device called a “Cough Assist” that helps them keep their lungs clear. They use it a couple times a day for prevention and every few hours if they have a cold or flu. Until recently I never knew such a device existed and I’ve never used one. I didn’t get my diagnosis until I was nearly 40 and none of my doctors knew what I had or what to do about it. When it comes to SMA specific medical when down care I’ve basically had none.

I started using oxygen at night to help me breathe better. That came after I developed congestive heart failure in my early 20s. Eventually I added a CPAP machine along with the oxygen. Then about 20 months ago I had to have a trach installed and the CPAP got replaced by a ventilator that I still use with oxygen but only at night. Here is the story of how I ended up with a trach.

Pray That They Listen to the Man with No Voice

Swallowing is extremely difficult. Two years ago in May I got to the point where I couldn’t swallow very well anymore and so I had to have a G-tube installed. As I mentioned before, the Type 1 kids end up on ventilators and with G-tubes at a very early age. I’ve encountered many Type 2 people who also got a G-tube much earlier than I did and is not unusual for them to need a trach and ventilator eventually as well. Here is a blog post about when I got my G-tube.

Tough to Swallow

The issue of not being able to directly treat the disease but only its side effects changed dramatically in December 2016 when the FDA approved a new drug called “Spinraza”. It has shown dramatic positive effect on the young severe Type 1 patients. It’s not a cure. It doesn’t always work. In fact in the clinical trials it only works in about 56% of the patients. But considering the severity and mortality of Type 1 patients, it is a real godsend. When it works, the kids stay off the ventilator, there are even signs that some of the effects of the disease are reversed.

The positive effects of the drug were so dramatic during clinical trials that they opened the test from its double-blind format. Typically half of the patients get the drug and the other half get a placebo. But because it’s got such high mortality, once they discovered positive benefits they could not morally withhold the drug from the other kids. For the remainder of the trials everyone got the real deal.

The clinical trials were only for Type 1 kids who were very young, the FDA approval was for all types and all ages. I can get the drug if I wanted. The problem is, I can’t see it having any positive effect on someone my age and my state of deterioration. It’s not just a simple pill. It involves spinal injections in an outpatient hospital procedure every few months for the rest of your life. One time an anesthesiologist looked at my spine to see if I could get an epidural for some surgery I was having. He took one look at me and said “There is no way I’m sticking a needle in that crooked thing”. The long-term side effects of the drug are yet to be determined but respiratory issues are one of the listed side effects.

Because this is a so-called “orphan drug” for which there is a very, very small market, the drug company has to charge exorbitant prices to recoup their development costs. The first year of treatment costs about $750,000 and every year after that $450,000 per year for life. Both government and private insurance have been very reluctant to cover it. If they do it is more likely only for the very young Type 1 patients although some older Type 2 and 3 are getting the drug. It also depends on what country you live in. My friend in Canada cannot get it. Much of the UK cannot get it although Scotland does support it. Australia does. Brazil doesn’t. It’s hit and miss around the world.

Keep in mind that the progression of the disease is that the motor nerves die and that makes the muscles atrophy. This does not regrow the motor neurons. It only prevents them from dying. So the best case scenario for older patients is that they get no worse. If I was in my 20s I would be beating down the door to wherever they had it to get it. But at age 63, with limited upside and potential downside, it just doesn’t make sense for me.

Here is a blog that I wrote in December 2016 the day after the FDA’s approval came. It recounts the story of why it took me so long to get a diagnosis that I did have SMA. It also tells a humorous story about my encounter with the head of the Genetics Department where I used to work as a computer programmer. Although it includes some of the information I have already discussed above, if you’ve not read it before I encourage you to check it out.

I’ve Got That One. It’s called SMA Type II. And now it finally matters!

Here is the link to another blog I wrote last month about the cost of Spinraza and those who seek to blame the drug company for its exorbitant price.

The Cost of Life-Saving Drugs

How does Spinraza Work?

I mentioned that everyone has an SMN 2 gene and possibly multiple copies of it but they don’t work very well. Because of that one bit error, the process of copying DNA to RNA and RNA creating the protein just doesn’t work. Spinraza makes it work better. It makes it so that the failure of SMN 2 to properly create the SMN protein is not as bad. There has been a big focus on how many copies of SMN 2 you have because that makes Spinraza potentially more effective.

I wondered what causes the difference between Type 1, Type 2 and Type 3 and speculated it was how many copies of SMN 2 that you have. But apparently there are other factors. There is a variability in how well SMN 2 works from person-to-person. So it’s a combination of a lot of factors. Some insurance companies will only approve Spinraza for people who have multiple copies of SMN 2.

What Else Can Be Done?

There is a new treatment in trials that basically involves what’s called gene therapy. It is a treatment that actually manipulates your DNA. I’m not sure the details but I doubt that it’s fixing the missing section of the SMN 1 gene. It’s more likely it’s patching that one bit error in SMN 2. The good news is that if it works, it’s a one time treatment. The bad news is, other forms of gene therapy for other diseases have proven to be even more expensive than Spinraza. That is because once you get the treatment, they can’t sell you treatment anymore. So they have even less opportunity to recover their development costs. Let’s of people are who do not have access to Spinraza are hanging their hopes on the new gene therapies. But I think they are going to be severely disappointed because gene therapy will be even costlier upfront even if it is cheaper over a lifetime. Who knows what the government or private insurance companies will think of that.

Okay I’ve edited this section in 2019 because I now have more information on the gene therapy it’s called “Zolgensma”. It has been recently approved by the FDA for children under 24 months. It is not yet approved for older patients. The children can get it as an IV injection but for older patients that might cause liver damage so it would have to be a spinal injection the same way as Spinraza is administered. As I predicted in this previous paragraph written in 2018, it is insanely expensive. A single dose is $2.1 million. But that’s all you need is a single dose. Compared to the fact that you have to take Spinraza for your entire life at about 1/2 million dollars per year it’s actually cheaper. But for one dose it is now the most expensive drug in the world. I also did not understand how the drug worked but I have a slightly better understanding now

It uses a virus to insert a good copy of SMN1 into your cells. Viruses don’t have any mechanism for reproducing. They infect yourselves and trick yourself into making copies of the virus. This gene therapy tricks yourselves into making copies of the SMN protein. It basically cures the disease. But you have to get it quick enough before the disease has done all its damage. Again it can’t regrow dead motor neurons.

Additionally we should report there is a third option on the table or it soon will be. It’s called “Risdiplam”. It is an oral treatment that works the same way as Spinraza in that it makes the SMN2 gene work better. It is still in clinical trials but is showing good progress in increasing the level of SMN protein in patients. It’s too early to tell if that will actually improve their condition but chances are it will. It is being tested on patients over a wide variety of ages and when it becomes available would likely be available for a wide age group unlike the gene therapy.

There was a recent article linked here https://smanewstoday.com/2018/07/23/motor-neuron-death-sma-linked-abnormal-rna-editing-study/ It explains why the motor neurons die off when they don’t have enough SMN protein. It has to do with the regulation of 2 other proteins called Mdm2 and Mdm4. If they could find a treatment that would regulate these other proteins then you can keep the motor neurons from dying without fixing the SMN problem itself.

There is also a lot of talk about using stem cells to regrow the dead motor neurons. Regrowing nerve cells from stem cells has been the goal of all sorts of spinal cord injury treatments. To date that has not come even close to working. Christopher Reeve expected to be walking in 10 years after his spinal cord injury and it didn’t happen. He died years ago waiting on it to happen any day and people are still waiting.

What Do You Want to Know?

Those who know me, know how long-winded I am and if you made it this far then I’ve proved that reputation. But there are a million other question we could answer. What do you want to know about SMA? About my life with SMA? Don’t hold back anything. If we are going to create awareness then let’s create some full awareness. I would rather you not respond to comments on this blog but ask me your questions on Facebook where I’m going to post the link to this article. I will also posted on my Google+ page so you can ask there but I would rather reply on Facebook. You can read my page on Facebook even if you are not a Facebook member. Or if you are a member you can follow me without friending me. Or go ahead and friend me. Find me at https://www.facebook.com/cyborg5

I mean it when I say ask me anything. It is EXTREMELY UNLIKELY you can offend me. (Never say never). There is a slim chance I will choose not to answer something personal. But there is a much greater risk that I will answer your question in very much more detail than you ever really wanted. So ask me anything but beware you might get an encyclopedia worth in reply. If it is a long answer I may make another blog out and it. If it’s a quickie I will just answer on Facebook.

More Resources

I’m also a member of five or six Facebook groups for families or patients with SMA. They are closed groups that I can get you links to request membership on request.

The Cost of Life-Saving Drugs

In this essay I want to explore the ethics and responsibilities of drug companies when it comes to pricing of lifesaving drugs. We’ve seen numerous news reports about drug companies increasing the prices of EpiPens, AIDS drugs and other lifesaving pharmaceuticals. In extreme cases the CEO of these companies have been coldly unapologetic and shown signs of unbridled greed as the driving force behind these price increases.

However it is my opinion that we cannot paint the entire pharmaceutical industry in such broad strokes. In the case of so-called “orphan drugs” that treat rare diseases, the cost of developing such drugs is as high or higher than any other pharmaceutical yet the potential market can be quite small. With a small market and a small potential return on investment, the prices on these drugs necessarily can be enormous.

Case in point Spinraza. About 18 months ago, the FDA approved Spinraza for the treatment of Spinal Muscular Atrophy. I have Type 2 of that particular disease. Although I will soon celebrate my 63rd birthday, those with the more severe Type 1 SMA have extremely short life expectancies. In clinical trials on Type 1 patients, the drug greatly reduced the effects of the disease or at minimum halted the progression of the disease in over 50% of patients treated. Because Type 1 has serious mortality, they opened up the double-blind study and took patients off of the placebo and gave them the real drug once they realized he was having a positive effect. It would’ve been unethical to continue to give placebo to other patients in the trial once a significant benefit had been demonstrated. So I have to give them a tip of the hat for that choice. Although clinical trials only targeted infant Type 1 patients, the FDA approved the drug for all Type 1, 2, and 3 patients of any age despite the lack of evidence of its efficacy for other types and ages.

The drug is marketed by a company called Biogen. Biogen, which is licensing Spinraza from Ionis Pharmaceuticals, has set the list price for one doss at $125,000. That means the drug cross $625,000 to $750,000 to cover the five or six doses needed in the first year, and about $375,000 annually after that, to cover the necessary three doses a year. Patients will presumably take Spinraza for the rest of their lives. https://www.nytimes.com/2016/12/30/business/spinraza-price.html

Administration of the drug requires an outpatient procedure to inject it directly into the spinal fluid. This procedure can be particularly difficult on older patients because many of them have had spinal fusion surgery which complicates things greatly. Biogen has been working hard to train a variety of hospitals around the world in the administration of the drug. They’ve also been working hard to work with government agencies and insurance companies to see to it that the cost of the drug is covered.

Getting coverage has been an uphill battle. The large expense, the incomplete data on its efficacy for older patients or for Type 2 and 3 patients has made this a tough sell.

Recently on Facebook in a number of groups related to SMA, I’ve seen several messages which are so critical of Biogen that they blame the company for the death of every SMA patient since the drug was approved. Week after week there are postings of obituaries of SMA patients with the accusation that their greed is responsible for the death of another victim. These posts have gone on to suggest in some cases that the drug should be given away for free. Note that these complaints against Biogen don’t ask that the drug be provided at cost. They seem to suggest that it ought to be completely free. If our entire healthcare system were to be held to this unreasonable standard, there would be no healthcare system at all. Doctors, nurses, and support staff could never be expected to work for free. Hospitals would never be built. Equipment could not be purchased to operate such facilities.

It seems to me that such accusations against Biogen are unsupported by any objective facts and display an unreasonable expectation on our healthcare system. We live in a world where healthcare is provided by either private insurance or by government programs. If one wanted to take the radical step of blaming someone for the death of these children, I would think that the blame would more correctly be placed on government agencies and insurance programs which refuse to pay for the treatment.

Let’s look at the cold truth about developing drugs in general especially orphan drugs.

Although there has been much SMA research supported by charitable efforts such as CureSMA.org and MDA, there is no way that these efforts have entirely paid for the development of Spinraza. In addition to the cost of search for such treatment, the clinical trials necessary to get such a drug approved are extremely expensive. As mentioned previously, Biogen has been working to get the drug approved in other countries around the world and to establish treatment centers capable of safely delivering the drug around the world. Naturally their initial focus has been on the US because that’s where the initial regulatory approval has been obtained. Expansion of these efforts to other countries with other regulatory procedures is no doubt a costly and time-consuming effort.

When they say that the cost of the dose is $125,000, that’s not manufacturing costs alone. The pharmaceutical company has to recoup tens or hundreds of millions of dollars it has invested in developing the drug, getting it approved, and getting it to market. The expansion of the market to other countries and other regulatory jurisdictions has to take a big piece of that as well. If we had to raise until the drug pays for itself in order to expand coverage beyond the US, there would be many more deaths then we are seeing now.

Although the effects of the drug have been phenomenal in many cases, it is only showing positive results in about 57% of those receiving the treatment. Many of these patients are only showing positive results after being on the drug a year or more. Once you get approved for the drug, it can be difficult to determine if you’re going to be one of those 43% for whom the drug has no apparent benefit. Only recently has there been any data to show its effectiveness in older patients or in Type 2 or 3 patients. This makes it a very tough sell for either private or government insurance programs. If we can believe statements from the company at various conferences and conventions, they are working hard to convince public and private insurance not only to approve the initial treatments but to continue treatments for a reasonable period of time to show that a particular patient will benefit.

Unfortunately we live in a world of limited medical resources. While we are arguing the ethical issues of paying for treatment for SMA, there are countless other medical conditions which face funding crises. We are faced with the tough choices of providing extremely expensive treatments with limited efficacy versus using those same dollars on other treatments for other diseases and conditions which have more proven track records. That is cold and cruel to say that but unfortunately we live with a healthcare system that has to impose triage to do the most good for the most people.

This is clearly not a case like the EpiPen or AIDS drugs that had been already marketed and priced and were later marked up hundreds of percent in price for no apparent reason. This is a brand-new treatment with an extremely limited market that is difficult to administer and for which there is only limited supporting data to justify its widespread use.

I don’t know what the actual costs of the drug are. I don’t know how rich Biogen and its shareholders are profiting from this new product. I do know that had Biogen and Ionis not invested the time and effort necessary to get the drug where it is today, there would be a lot more dead kids. I would think patients and their families would be grateful for the efforts that these companies have made and be less likely to paint them as greed driven coldhearted murderers.

Until I see hard evidence that there is some sort of price gouging going on with this treatment, I’m going to prefer to give them the benefit of the doubt and to be grateful for all of their efforts.

Now some disclaimers… I do not own stock in any company whatsoever. I have no ties to the pharmaceutical industry in any way. I have chosen not to pursue Spinraza treatment because of age 63 I seriously doubt it would do me any good. I have severe scoliosis which would make spinal injections extremely dangerous if not impossible. Other side effects such as respiratory infections would present a dangerous risk given my overall unstable health. If I were younger or in better shape I would most likely pursue the treatment aggressively. And if I had difficulty in getting approved for it, any anger I would have would be directed at government or private insurance and not the pharmaceutical companies.

Christmas Gifts — Holiday Hospital Part 8

This is the eighth in a multi-part blog about my 2 week stay at St. Vincent Seton Specialty Hospital. Here is an index to all of the entries in this series.

Big Surprise

In my previous installment I talked about going to a Christmas ceremony on December 23 but there were other things that happened that day that we ought to cover. I had a bit of a rough night so I tried to nap as much as I could in the morning. I spent some time finishing up the second installment of my blog about being in the ICU. Here was my early morning Facebook post. [12/23/2016 7:16 AM ]

I’m not sure whether it was by email or by a Facebook post that I got some amazing news. The FDA had approved a drug called Spinraza for the treatment of Spinal Muscular Atrophy which is the genetic neuromuscular disease that I have. SMA comes in about four different varieties. I have type 2 which is very early onset but only weakens you very gradually over your lifetime. Living into your 60s like I had was not unheard of but I was probably pushing the limits. However the more severe type 1 weakens the children very severely at birth and they are fortunate if they managed to live more than two years. In a clinical trial, this drug was so effective in not only stopping the disease may in many cases increasing the strength of patients, that they stopped giving the placebo to part of the participants. Given that this was a seriously fatal disease and they had already seen positive results, it would’ve been immoral to deny the treatment to the other participants.

Although the clinical trials only involved the extreme type 1 infants, the FDA had taken the unusual step of approving it for all varieties of SMA and all ages of patients. This was a treatment and not a really cure. Early results showed it to be effective in only about 40% of the patients. Later that number increased to around 50%. I had known for years that if they ever did come up with a treatment, it wasn’t what to do me any good. Over 60 years of the disease had done damage that cannot be reversed. For a variety of reasons I won’t go into here, I’ve not pursued the treatment. The major reason is that is unproven for older type 2 patients at highly unlikely to do me any good. The side effects of the treatment are not well known. It involves a spinal injection and with my severe scoliosis that would be either dangerous or impossible.

Still it was an amazing piece of news and a wonderful Christmas present. I put together the following blog item telling a long story about my relationship with SMA and a funny story about one of my former employers who was a geneticist who is a bit of an ass.

I’ve Got That One. It’s called SMA Type II. And now it finally matters!

Christmas Eve

For Christmas Eve I expected it to be an uneventful day. Dad was going to come and we were going to watch the Colts game. I had told Carol not to bring her grandkids to the hospital to see me because I knew they would be bored and rowdy. I had decided to take it easy and not get into the wheelchair today. Here was my early morning Facebook post.
[12/24/2016 9:45 AM ]

Fortunately nobody listened to me when I said don’t come and don’t bring the kids. Carol showed up with her daughter Alaina and grandkids Leighton and Keeleigh. Carol brought me a Christmas present that I have asked for. It kind of started out as a joke but actually it turned out to be a good idea for present. I had said “What do you get a guy for Christmas who has a trach? Of course the answer is scarves!” She got me a Colts and a Pacers scarf. There was a promise eventually we would get one for the Indy Fuel hockey team which we did eventually get. Here’s the live Facebook video of their visit at 2:13 PM.

Alaina and her kids did not stay long because as I predicted, they got a little bit rowdy. To my surprise, before Carol and the kids left, Karen and her boy Cole showed up for a visit as well. Here is the Facebook live video from 3:14 PM

My nephew Cole is a huge Chicago Cubs fan. When the Cubs won the World Series I built a special electronic hat for him. It has a strip of NeoPixels across the brim as well as a group of pixels underneath a 3D printed “C” for Cubs in the front. It runs off of an Adafruit Feather 32u4 BLE with a LiPo battery. You can control the pattern of lights using an app on your phone or tablet. Dad had brought it and left it in the car just in case we happen to see them. Unfortunately I could not demonstrate it because we ran down the battery playing with it. Here’s a video demonstration that I gave several weeks earlier on the Adafruit “Show-And-Tell”. My segment starts at about 5:35 into the video.

It turned out that the Colts lost their game and were eliminated from the possibility of making the playoffs. I spent the rest of the day working on a blog post about my Christmas card. If I wasn’t going to be able to mail out the Christmas cards, at least I could write about it and share the image with everyone on Facebook. I took time out to call my friend Judy and wish her Merry Christmas. Here was my final Facebook post of the day.
[12/24/2016 8:12 PM ]

Christmas Day

Christmas morning I put the final touches on my blog about my Christmas card. Usually I make a CGI rendered image using POV-Ray rendering software but this year for a change I took one of my old CGI designs, 3D printed it, and took a photo of it. Here is a blog post from my graphics blog with the details.

My 2016 Christmas Card: Reimagining a CGI image by photographing 3D printed models

I connected with my sister Carol via Skype and got to watch her kids and grandkids open Christmas presents that morning. I had hoped to do the same with Karen but we never were able to get together on that. I had a surprise visit from my friend Judy and from Fr. Paul. I had no idea they were coming so it was fun to spend time with them. I spent the rest of the day working on another installment of my blog about my time in the ICU. My only Facebook post other than the blog posts was the following note.
[12/25/2016 5:00 PM ]

In the end it was a much better Christmas than I expected it to be. I got to see all of the family that I usually see on Christmas plus visit from friends that I did not expect.

After Christmas

Because it was the holidays, they were still very shortstaffed. With Christmas falling on Sunday, people were considering Monday the holiday. Even the cafeteria downstairs was shut down. I was able to get up in my chair for over five hours. I got a visit from my friends Stu and Pat Byram which was fun.

While I was up in my chair, my throat got quite dry and my lungs got a bit congested. Dad was supposed to be learning how to do a trach suctioning but I had been doing so well that I didn’t really need one. He had done a couple of practices when there was nothing in my lungs to suck out. So when I got gunked up that day, Dad did his very first trach suctioning under “real world” circumstances where I really needed it. We did okay even though it maybe wasn’t the best one I ever had.

I had a talk with the nurse practitioner about taking me off some of the medication that they had put me on. I don’t recall what sort of extra meds I had been on but I didn’t really think I needed them anymore. We talked it over and she cleared it with the doctor and we cut me back to just my usual medication.

One of the meds I had been on for many years was Flomax which is supposed to help with prostate issues and to help you pee better. The warnings on that drug said do not crush it or chew it. It is a capsule that is only about half full of some sort of gritty powder. For months (perhaps years) I had been taking it by mouth and letting the capsule partially dissolve until I could smash it flat and swallow it. Many times the contents would come out into my mouth. Fortunately it did not taste bad so I had no trouble taking it by mouth. However under my new circumstances I wasn’t taking anything by mouth except plain water. So they had discontinued the Flomax and put me on something similar that was safe to grind up and put through the G-tube. With this exception everything else on my meds were back to normal. It turns out that substitute prostate medicine has continued to work well to this day. I’m not had a urinary infection in two years.

I managed to write another blog post about being in the ICU. I was really looking forward to Tuesday because that was the day that the respiratory therapist was going to bring in my new ventilator and to train my dad and Carol how to use it. Dad would then stay overnight Tuesday night to prove to everyone that he was capable of taking care of me. It was a ridiculous requirement but it was the only way they were going to let me out of the place. Theoretically I could be going home by Wednesday the 28th. Here are the two Facebook posts I made that day. [12/26/2016 6:58 AM ] [12/26/2016 6:14 PM ]

A Dry Heat — Holiday Hospital Part 5

This is the fifth in a multi-part blog about my 2 week stay at St. Vincent Seton Specialty Hospital. Here is an index to all of the entries in this series.

Vertical at Last

We are now up to Monday, December 19, 2016. Things were relatively quiet around the hospital over the weekend but starting Monday things were much more active. The staffing levels were higher. Therapist were back on duty. Although we didn’t really need the people from physical therapy to assist getting me out of bed and into the wheelchair, they wanted to be involved in that activity. They would need to round up a lift seat for me to use. Rather than a regular Hoyer patient lift like I use at home with a pump handle that you have to pump up and down and it rolls around on the ground, they had a motorized hoisting device hanging from a track on the ceiling. It was best we had them help because we really didn’t know how to work the gadget yet. I had had an uneventful night and my lungs were in pretty good shape according to this Facebook post.
[12/19/2016 11:06 AM ]

We eventually rounded up someone from therapy and they got us a lift seat and managed to get me into the chair without incident. I took the opportunity to drive around the hallway up and down once or twice. I went across the hall to visit my neighbor. It was a guy who looked like he was about 60 or so and had had some sort of physical problem I forget what. He was awaiting the opportunity to be transferred to a rehab hospital and ended up being discharged the same day that I was about two weeks later. We then set up my laptop on the hospital tray and I figured out how to use the WebCam to do a ride video on Facebook. Here’s the video I posted.

In the background of this video you can see the motorized lift device hanging over my dad’s head.

That evening I was visited by my friend Judy Chapman. It was the first opportunity I had had to talk with her since I had been in the hospital. During her previous visits in the St. Vincent ICU I had still been on the ventilator and could not talk. I was able to tell her the complete story of all the events that had brought me this far. We talked about the text message I sent her the content of which became the title of that 14 part blog series “Pray That They Listen to the Man with No Voice”. I talked about the two incidents with nurses who treated me so badly because I couldn’t talk and defend my position and about the therapists who did take the time to pay attention to my wishes. It was wonderful to get caught up with her and tell her all the stories I had not been able to tell her up until now.

The only other item I have from my notes for that day was that I had constipation and had to take a suppository. The bedpan that they had at the hospital was very uncomfortable so I had dad bring my bedpan from home. It was one I found on Amazon that had a very wide lip around it. The standard bedpans these days have a sharp edge that digs into your hips and can be uncomfortable.

As Dry as Arrakis

On Tuesday the 20th, they did a big team meeting. It included the case manager Michelle, people from all of the therapy departments, the nurse practitioner, and even the chaplain. It’s a weekly ritual where they evaluate how you are doing, what your goals are, and address whatever concerns any of the team has about your care plan. We made the conclusion that I did not need physical therapy, or occupational therapy so I was officially “discharged” from their programs.

One of the issues we addressed was my concerns about humidity. After being up in my wheelchair for a couple of hours the day before, I had ended up with a little more congestion in my lungs because my lungs had dried out somewhat. At times I had a little difficulty with coughing spells while trying to talk to Judy that evening.

As I had mentioned in the second installment of this blog “A Busy First Day”, they had me wearing a device called a trach mask. It’s a clear plastic cup that fits over your trach and is held around your neck with an elastic strap. A hose connects it to a source of heated humidified oxygen running at up to 10 liters per minutes. I didn’t really need the extra oxygen but I was pretty sure I needed humidity. Normally when you are breathing through your nose or mouth, the air is warmed and humidified by the moisture in your sinuses or mouth before it enters your lungs. However when you are breathing through a trach, the air goes straight into your windpipe and doesn’t have the opportunity to be humidified. They made a big deal about how I needed to be on this humidity all the time. It was very noisy and most of the time I would turn it down from 10 to about six or so.

One of my growing concerns was what to do once all of this was over and I was trying to get my life back to “normal” again. I knew I wasn’t going to need to be on a ventilator all of the time. The talking valve meant that I had my voice back again. But I didn’t want to be tied down to some big piece of hose connected to a humidifier all of the time.

One of the alternatives is something called an HME attachment for your trach. The letters stand for Heat and Moisture Exchange. Sometimes it’s called an artificial nose. It is simply a cap that fits over your trach and it has a small piece of foam in it. As you exhale, it captures the humidity from your breath. Then when you inhale, the humidity goes back into your lungs. It kind of reminds me of the stillsuit technology worn by the Freemen on the desert planet Arrakis in the Frank Herbert sci-fi classic “Dune”. The problem is, it doesn’t work with a talking valve.

The Passy-Muir talking valve has a butterfly flap inside it. When you inhale, the “wings” collapse and allows you to breathe in through the trach. When you exhale, the wings flap apart closing the passage and instead of exhaling through the trach, the air goes out your windpipe and past your vocal cords allowing you to talk normally. So the HME device never gets your exhaled breath to collect the humidity to be recycled. You already know how I feel about not having the ability to speak so the HME was not a viable option either.

The only other alternative is what is called a “red cap”. It is a solid cap made out of red plastic that completely closes off your trach. Then you just breathe normally in it out your mouth or nose through your windpipe as if you had no trach at all. After discussing this option with the respiratory therapist I tried to get the doctor or nurse practitioner to write orders to allow me to try it out. I got in a somewhat nasty argument with a nurse practitioner named Joy. She tried to convince me that because my particular type of trach had an inflatable cuff that it was not meant to be used with a red cap device.

The problem is that if for some reason the inflatable cuff doesn’t get fully deflated or if somehow the cuff would malfunction and block your airway, you wouldn’t be able to breathe at all. Without the red cap device, if the cuff would fail and block your airway, you would still be able to breathe through the trach. Even with a talking valve it might be difficult but you wouldn’t suffocate. I suppose that’s one of the reasons they make it red is because it is a somewhat dangerous device. You don’t want to accidentally put one on a person who shouldn’t have one.

I tried to convince the nurse practitioner in a very heated debate that I did not want to be tied down to a humidifier. I practically begged her to let me at least tried the device and she pretty much refused. Later in the afternoon I did some online research about trachs and how the cuff works. I believe at one point they even brought in an anatomical model to show me how a cuffed trach works. There were also some online videos that explained it. The doctor was going to be making rounds that evening and I had planned to appeal to him for the use of the red cap but by then I was beginning to understand the real risks and so I gave up on that idea. So it was a very heated debate… but it was a dry heat 🙂

This Facebook post inarticulately tries to explain all of the above. There are some dictation typos and errors that make it almost unreadable in places. I guess I didn’t proofread very well. [12/20/2016 6:05 AM ] I also got up in the wheelchair again for the second day in a row and made the following live Facebook broadcast.

Skills Lab

At some point dad was going to have to learn a lot of new skills about how to take care of the trach. I was receiving an albuterol breathing treatment every 6 hours around the clock (even at night). They were changing the gauze pad around my trach twice a day and the strap around my neck every other day. I also periodically needed my trach suctioned out with a catheter to clear congestion. So the respiratory therapist started giving my dad training sessions on how to do that.

The most complicated part is to suction the trach. The catheter comes in a little kit with its own pair of sterile latex gloves. These are not the kind of traditional latex gloves they pull out-of-the-box when dealing with you in the hospital. These are carefully wrapped in a paper envelope and are 100% sterile. Of course my dad has very large hands and even though the gloves will stretch to any size, it’s very difficult for him to put them on. They insist that you only touched the gloves by the cuff. One of your hands is considered the “clean hand” and the other is your “dirty hand”. You aren’t allowed to touch anything but the catheter itself with your clean hand.

This day was just the first of several training sessions they gave my dad. It wasn’t until the last day when we were talking to one of the nurses and she admitted “You realize, when you get home you probably aren’t going to go through all of that. As long as you don’t touch the end of the catheter and you keep it clean going in, you don’t need to worry about gloves or clean hand/dirty hand issues.” Dad and I looked at each other like “Then why the fuck did we have to go through all of that here?” The catheter kits supplied by my DME provider don’t include the sterile gloves and we never use even nonsterile gloves except when actually changing the trach itself. Although it took a bit of attitude adjustment on my part, I eventually trained friends and family how to do a trach suction. But that’s another story.

Miscellaneous Events

Carol came to visit that evening and gave dad a break. I told her the whole story of my arguments over what to do about humidity. The other thing that happened was we finally got the call from Home Health Depot to set up an in-home visit. That was part of the lengthy process we needed to get approved for an in-home ventilator. They set up an appointment to come to the house at 1:30 the next afternoon. Dad waited around on them and did not come until later in the day on Wednesday. This Facebook post shares my thoughts on all of the stuff we had to go through to get certified for the ventilator. [12/20/2016 9:51 AM ]

While I normally would not confess to illegal activity in a public blog, I will admit here that I downloaded a bootleg copy of “Rogue One: A Star Wars Story”. I never missed seeing any of the Star Wars movies since the first one. Most of them I’ve seen in a theater multiple times and that doesn’t include dozens of viewings on cable or DVD. I always go with my friends Rich and Kathy Logan. We saw the first one together. We went back and saw it again a year later for a one year anniversary. And I’ve seen every other episode with them. But when they visited over the weekend I told them they needed to go ahead and see Rogue One without me even though it really hurt to admit I wasn’t going to get to see it in theaters. The copy I watched was a really bad camera copy with bad audio. Not the best way to see a big action-adventure movie. In my defense, I did eventually purchase a 3D Blu-ray and I watched it as well on cable that I paid for legitimately. Under the circumstances I don’t feel bad about indulging myself under these conditions. Wednesday would be a day of more legitimate online activities. We will cover that in the next installment.

An Uneventful Weekend — Holiday Hospital Part 4

This is the fourth in a multi-part blog about my 2 week stay at St. Vincent Seton Specialty Hospital. Here is an index to all of the entries in this series.

Recap

In December 2016 I spent nearly the entire month in the hospital with respiratory problems. I chronicled the first 10 days or so of St. Vincent Hospital ICU in a series of blog posts titled “Pray That They Listen to the Man with No Voice”. The remaining two weeks were spent in a different hospital called St. Vincent Seton Specialty Hospital where I stayed through Christmas. I’m calling this second series “Holiday Hospital”

I didn’t get around to starting to write about those additional two weeks until over six months later in July 2017. I wrote 2 installments in July and then a third one in September but never got back to it until now April 2018. Even though it was well after a year since these events occurred, I do have some notes about what happened each day and I will have links to Facebook posts and videos that I made at the time. Who knows if I will finish it this time or I will take another couple months off. But anyway here goes. The link at the top of this page takes you to a table of contents for this entire series. Note: anywhere you see the little Facebook icons it will link you to a Facebook message I wrote at that date and time.

An Uneventful Weekend

We are now up to Saturday, December 17. I had been having a lot of problems with congestion and one of the things they had been doing supposedly to help it was giving me some sort of medicated patch to wear. I forget the name of it but it was supposed to help with my congestion. We concluded that perhaps it was making the congestion too thick so we decided to discontinue it and see if that helped. It really did. My lungs were much better without the patch. [12/17/2016 6:50 AM ]

Dad brought in my electric razor and he shaved me for the first time since I had moved to this facility. I had shaved once before in the ICU so this was only the second shave in about 10 days. I had developed a rash on my neck from not shaving, being sweaty, and having a trach collar around my neck. They brought in a roll of medically treated cloth in a little box that looked like a wax paper roll. The cloth is white, stretchy and had a bit of a sheen to it almost like a silk cloth. It was treated with some sort of silver solution. It was designed to wick away your sweat from creases like around my fat neck. It really worked well to clear up the rash over the next day or two. I took home the leftover material when I checked out and I’ve used it once or twice since then. It’s really good stuff.

Typically dad was arriving late morning and staying till about dinnertime when he would go home before dark and Carol would come and visit me in the evening. It was always handy to have a family member there who could make sure that I was laying comfortably and had my call button in my hand. They could do things for me so I didn’t need to bother the nurses or aides. But there was supposed to be an ice storm coming in and Carol canceled her visit. That was okay. By now I was settled in to my environment a little better than I had been originally. I also told dad not to come on Sunday because the roads would be terrible. [12/17/2016 6:34 PM ]

Sunday the 18th was mostly an uneventful day for me. My lungs were in good shape when I woke up however I did have some hip pain so I took a pain pill. Lying in bed so much and trying to set up in bed without my back brace on was messing things up for me. Dad came to visit despite the nasty weather. Not much goes on in the hospital on the weekend. [12/18/2016 8:27 AM ]

I did not sleep very well the night before but I was able to catch a nap along the way. I watched the Colts win their game but unfortunately Tennessee also won which made our playoff hopes much diminished. Dad brought with him the latest 3D printed parts for my nurse call button and they worked really well. I posted a photo of them on Facebook. [12/18/2016 4:08 PM ] [12/18/2016 6:58 PM ]

I said it was uneventful for me but from a news standpoint it was an eventful day. I was saddened to hear of the passing of former Indianapolis Mayor William Hudnut. He was a great Mayor who did a lot of good things for the city especially negotiating the transfer of the Colts NFL team here from Baltimore. He continued the work of former mayor Richard Lugar in maintaining a Mayor’s Advisory Council on the Handicapped. I was honored by him to be awarded with “Indianapolis’ Outstanding Handicapped Citizen Award”. Here’s a photo I posted on Facebook remembering him. Behind the left to right is the mayor, my mom, my grandma Osterman, my Aunt Jody Roell, and my dad. [12/18/2016 10:59 AM ]

One of the nice things about having good Wi-Fi in the hospital and having my laptop was that I was able to catch up on some online streaming TV shows. I got on Netflix and finished up watching Luke Cage that day. I thought the whole series started off a little slow because as an old white guy I had trouble getting into the Harlem aspects of the story. But once it got deeper into his origin story and became more of a superhero story and not just an inner-city gangster story I ended up really liking it a lot. I didn’t have any problem with the black urban portions of the story. I just had trouble relating to it.

About Ableism and Other Imposition of Worldviews

Recent tributes honoring the life and death of renowned physicist Stephen Hawking have reignited a long time debate on the topic of “ableism”. Images such as this one depicting Hawking free of his wheelchair and walking among the stars are among those sparking a lively debate about ableism.

For me there are really two parts to this debate. What is or is not appropriate in an expression of one’s feelings about the death of a celebrity? And separately does this particular tribute or other similar statements made in tribute to Stephen Hawking really represent ableism.

If you’re not familiar with the term “ableism”, it is a type of prejudice which denigrates those with physical disability in favor of those who are able-bodied. It is a type of prejudice that has parallels to racism and sexism.

Before addressing the claims of ableism, I’d like to address the issue of celebrity tributes in general. When a celebrity dies, we often see tributes to them which are not necessarily in tune with the deceased’s particular beliefs or wishes. We saw the same thing when Steve Jobs died. A number of cartoons depicted him in heaven and/or being in the presence of God which would have been contrary to his Buddhist beliefs. Here’s a page with a good sampling.

http://w99.suretech.com/16846/Steve_Jobs_Remembered_in_Cartoons

There was similar controversy during this year’s Super Bowl halftime concert by Justin Timberlake who projected an image of Prince onto a large screen. Prince had specifically said he did not want to appear as a hologram after his death. Although not technically a hologram, it did have a sort of ethereal holographic feel to it.

The Steve Jobs tributes were undoubtedly disrespectful of his religious beliefs. The criticism of the Super Bowl depiction of Prince was probably a little bit nitpicky especially since it wasn’t really a hologram. Furthermore it was not complicated by religious overtones. But was there anything similarly inappropriate about this particular tribute to Hawking?

This article from time.com documents his beliefs about God and an afterlife.

http://time.com/5199149/stephen-hawking-death-god-atheist/

In various statements, he made his atheist stance quite clear. This included his disbelief of any sort of afterlife. His beliefs about God confused people because he had written famously that if we understood how the universe worked that we would glimpse “the mind of God”. He later made it clear that he was speaking much more metaphorically. For him God was not a particular being with which one would have a personal relationship. Rather God was a metaphorical concept of the manner in which the universe worked. He did not believe in a being like God who was responsible for creating it. He was instead saying that to have knowledge of the universe would give you a godlike perspective.

Similar confusion surrounds statements from Albert Einstein when he famously said “God does not throw dice” in expressing his difficulty with the uncertainty principle in quantum mechanics. In a recent biographical TV series “Genius”. Einstein is not portrayed in any way as a religious person or a man of faith. His defense of the persecution of his own Jewish people appeared to be more based on human rights concern rather than devotion to his religious heritage. It’s pretty clear that Einstein was speaking metaphorically about God rather than from a position of faith.

While this image of Hawking walking among the stars isn’t as blatantly as religious as those depicting Steve Jobs in heaven talking to God, it does presuppose a type of afterlife which Hawking pretty clearly had rejected. The Time article quotes an interview in which he says

“I regard the brain as a computer which will stop working when its components fail,” he told the Guardian. “There is no heaven or afterlife for broken down computers; that is a fairy story for people afraid of the dark.”

Personally I consider myself a man of faith and a man of science even though many find these two disciplines to be incompatible and mutually exclusive. Without getting into that debate, I have to also say I have the deepest respect for those who are devout atheists or agnostics because at one point in my life I was very much an agnostic and I appreciated those who respected my beliefs.

On the other hand, such depictions are an expression of our own personal beliefs about the person. They are an artistic expression of the artist’s reaction to the death of someone they admired or respected. I believe in an afterlife despite the lack of scientific evidence. That is the nature of faith. I tend to believe that heroes of mine who happened to be atheists such as Stephen Hawking, Albert Einstein, and Isaac Asimov who have led exemplary lives and contributed to the good of the world are enjoying Paradise in an afterlife despite their disbelief in an afterlife while here on earth.

So in some respects the image in question expresses my beliefs about the fate of Prof. Hawking whether that image reflects his beliefs or not. Despite his espoused atheism and his expression that religious beliefs were fairytales, I would not describe him as blatantly anti-religion or radically disparaging of those with religious beliefs along the lines of someone like comedian Bill Maher. I would hope that he would understand that such a depiction expressed the artist’s wishes for him rather than be offended by it.

Inappropriate tributes to the dead which disrespect their beliefs and desires are a mild form of prejudice. They are an imposition of one’s own worldviews onto that of another. I think they are relatively minor offenses but there are other forms of prejudice that are more destructive. Racism, sexism, religious intolerance, LGBT intolerance and ableism are all impositions of one’s own worldview one to another.

Perhaps it is disrespectful of Hawking’s atheist stance but is that image ablest? What constitutes ableism? Is it the same as other forms of prejudice such as sexism or racism?

Apart from this particular image, there have been other statements such as “He is finally free of his burden”. While I don’t have links to such statements, I don’t doubt that they exist. And in some respects claims of ableism about such statements are more credible than the charges against this particular piece of art.

I can understand how people who are especially sensitive towards ableism might see that expressed in this image but I do not. Let me explain upfront that anything I’m about to say should in no way be construed to discount ableism as a real and destructive thing. Ableism exists and it needs to be confronted in the same way as any form of prejudice. However I really believe some of what is labeled as ableism is unjustified.

Ableism, racism, and sexism have much in common. They are all distorted worldviews which unjustifiably proclaim the superiority of one group of people over another. They denigrate and dehumanize classes of people. They are born of ignorance. They are born of fear. In extreme cases the purveyors of these prejudices are blatant and unapologetic. But the most insidious forms of these prejudices are those in which the believers are unaware of their innate negative biases.

I believe that this subtle unconscious form of prejudice constitutes 99.9% of ableism. You would be hard-pressed to find someone who would openly speak out against people with disabilities in the same way that we see unapologetic denigration of races, religious beliefs, women, or sexual orientation. It takes a pretty hard core Nazi-like person to speak disparagingly of the disabled.

It is common for people with prejudices to deny them. We have seen obvious racists declare “I am the least racist person you’ll ever find.” The most misogynistic men will proclaim their love and respect for women. Religious bigots full of intolerance hypocritically express a devotion to love fellow all human beings.

In contrast, I believe that when most people have an ablest attitude, they are genuinely unaware of the mistake of their perspective. They are almost always uninformed well-intentioned people who lack the knowledge or perspective to see the mistake of their attitude. Unlike racism, sexism, or religious hatred, people expressing ableism are more often genuinely unaware of the hurtful nature of their misperceptions. Expressions of ableism rarely are an exposure of underlying core beliefs of the person in the way that other forms of prejudice are an expression of basic character flaws. They are more easily educated and converted away from their mistaken understanding of people with disabilities.

Ableism is most often expressed in the form of pity towards the disabled person. They feel sorry for us in our horrible condition. Despite the inappropriateness of the pity and the hurtful nature of being pitied, such expressions come from a legitimately well-intentioned motivation. The condescending attitude comes not from an egotistical sense of self superiority but from a genuine misunderstanding of what it’s like to have a disability.

All prejudice is driven by fear of loss. White supremacists fear the loss of their power and perceived superiority. Sexists fear the loss of their male dominance. Religious bigots fear that alternate belief systems challenge their own beliefs and sense of certainty.

But in the case of ableism, it is the genuine and legitimate fear that someday they will end up with a disability of their own. There is a sense of awe and amazement that someone can persist in spite of a disadvantage which they themselves believe unsurmountable. The fear driven amazement is expressed in condescending, hurtful, and even dehumanizing ways. So even though ableism is not as inherently evil in its origins as other forms of prejudice, the effects of it are no less destructive.

All forms of prejudice are harmful. That harm is very personal to its victims. Prejudice must be confronted, exposed, attacked, denounced and discredited. Although it is difficult to eradicate among its believers, through thoughtful education its spread can be halted. But in our zeal to do so, there is always the risk of seeing such prejudice were it doesn’t really exist.

I am reminded of the joke about the guy who goes to a therapist and the therapist administers an inkblot test. “What does this image remind you of?” the therapist asks. The patient replies “sex”. “What about this?” Again he replies “sex”. As each abstract image is shown to the patient he replies “sex”. The therapist says “Mr. Jones has it occurred to you that you’re obsessed with sex?” Jones replies “Me? You are the one with all of the dirty pictures!”

Sadly in the face of all the real sexism, racism, and ableism… Some people find it where it does not exist. In some respects I think that may be the case in this particular circumstance. I don’t think this particular image is an expression of even subconscious ableism.

It depicts Hawking walking among the stars out of his wheelchair. As evidence that Hawking might not have been offended by the image in that way, I offer up the fact that he had booked passage on a Virgin Galactic flight into outer space where he would experience zero-g. In his New York Times obituary linked here there is a photo of him about halfway down the page showing him out of his wheelchair floating in simulated zero gravity. He is on board a so-called “vomit comet” airplane which flies a parabolic arc inducing a freefall. The smile on his face and the fact that he did not go about this adventure while continuing to be strapped into his chair (which he could have done) tells me that an image of him floating free from his chair would have in no way offended him. While you might point out that the artwork in question depicts him upright rather than floating at an odd angle, I don’t believe that’s a significant difference.

While I am sympathetic and respectful of those whose anti-ableism sensitivities are triggered by such an image, I think there is a significant difference between ableism and other forms of prejudice that make some of it unjustified. I do not believe that all expressions of dislike of a disability are necessarily a bad thing. I think they represent the reality that having a disability is legitimately an undesirable situation.

That doesn’t mean that everything about having a disability is bad. In my own tribute to Stephen Hawking, I explained that the reason that I so admired him and considered him a role model despite my natural tendency to avoid role models was that he had made positive use of his disability. Because of the limitations imposed on him by his physical condition, he adapted his methods of reasoning to rely upon mental visualization techniques rather than writing out derived equations as is the usual strategy. This gave him insights to the cosmological questions he was pondering that had escaped notice by other physicists in his field. So there was an aspect of his disability that was undoubtedly an asset. Recognizing that in some ways my disability is a God-given gift or from a non-theological perspective simply asset in my life, I felt a connection to Stephen Hawking. I wrote in my tribute to him “Understanding that Hawking had similarly turned his disability to his advantage was a conformational data point to prove my hypothesis that having a disability wasn’t all bad.”

The clichéd proverb states “When life gives you lemons… make lemonade” acknowledges the fact that lemons are sour but you can still make something good from them. It doesn’t deny the fact that your life has taken a negative turn. It only proposes that such negatives can be turned around into positives.

All prejudice presupposes an illegitimate or insignificant difference between groups. One race is considered superior to another race for no justifiable reason. Any measurable differences between races can always be justifiably explained as being caused by the effects of institutional racism rather than being justifiable reasons for racism. Sexism against women is not based upon the legitimate biological differences between men and women but is rather a defense of institutional and cultural domination of men over women. Hate against different religious groups or directed towards sexual orientation have their roots in belief systems rather than measurable superiority. Such prejudices are inherently subjective rather than objective.

But in the case of ableism, there are objective, demonstrable, measurable differences between able-bodied people and people with disabilities. These differences should not be used to denigrate the value and basic humanity of people with disabilities. Ableism is wrong. It is evil. It is real and should be confronted and stopped. But because people with disabilities are measurably different than able-bodied people, not every attempt to discuss these differences or to describe a disability in a negative light is in fact evil ableism.

In order to live a productive life with a disability, it is absolutely essential that one come to terms with one’s condition and to accept it as a part of themselves. It is okay to identify intimately with one’s disability and to embrace the positive aspects of it. But to deny that there are negative aspects to it is to deny reality.

I offer the following challenge to people with disabilities. If someone presented you with a magic button that you could push that would instantly remove your disability without robbing you of the insights, perspective, and giftedness that your disability has provided you, would you not push that button?

No matter how accepting I am of my situation… No matter how much I view my disability as not only an asset but literally a gift from God above. I would push that fucking button in a millisecond. My guess is that the vast majority of people with disabilities would do so as well no matter how deeply they embraced their current condition.

The difference between having a disability or being of a particular race or gender is that by its very nature it has the power to enslave you. The enslavement of race or gender is externally imposed. The enslavement of disability comes from the disability itself.

Did black people like being slaves? Do they enjoy the economic disadvantages imposed them by their race? Do they embrace being presumed guilty and gunned down in the streets by racist police? Of course they do not. It doesn’t mean they don’t want to be black or can’t be proud to be black.

Do women enjoy making 70 cents on the dollar compared to men? Do they enjoy the degradation and sexual abuse? Recent events show that we are finally listening to their expressions about the extent of this degradation. While they fight to be free of such limitations it doesn’t mean they don’t want to be women.

The desire to be free of the disadvantages of a disability is not an assault on one’s identity as a disabled person. Much of what is mistakenly labeled as ableism is NOT an expression in the belief of the superior value of able-bodied people over disabled people. It is a legitimate recognition of the genuine disadvantages of disability.

Many of the negative aspects of having a disability can be mitigated by changing people’s attitudes. By changing society. By changing negative stereotypes. By educating people. But having a disability is inherently, objectively, measurably a disadvantage. It is legitimate and non-ablest to point out these differences. It is legitimate to want to be free from these inherent disadvantages. And it is not only legitimate but praiseworthy to hope for others to be free of those disadvantages.

Part of the problem comes down to evolving terminology. We have evolved our language in such a way that it is driven more by the forces of arbitrary political correctness rather than logical reasoning. Words are abused in such a way that they lose their legitimate meaning. I’ve talked about this before in other essays but I will try to summarize my beliefs here.

I have a “disease”. It is a genetic neuromuscular disease called Spinal Muscular Atrophy Type 2. It is part of who I am. I literally would be a different person without it because it is genetic. Other diseases are caused by bacteria or viruses such as polio or AIDS. Similarly there are medical conditions caused by outside forces. Cerebral palsy is typically a result of anoxia during childbirth. Spinal cord injuries come about by physical trauma. All of these are “medical conditions”. Short of a medical cure or some natural healing process, these don’t go away. They are an undeniable reality.

My disease and any other medical condition results in a “disability”. I am literally dis-able to do certain things. I can’t walk. I can’t take care of my personal needs. I cannot feed myself. I have virtually no use of my arms. These are things that objectively I cannot do. There is truth in the abused cliché “everyone has a disability.” You can’t fly without an airplane. You can’t lift 5000 pounds with your bare hands. You are literally dis-able to do these things. The difference is, you don’t expect to be able to do these things and nobody expects you to. Nobody else can either.

That’s where the word “handicap” comes into play. Unfortunately is a word that has fallen out in favor but it still has an important legitimate meaning and use. Your handicap is the way that your disability interacts with your environment. When a disability restricts you from doing the things you want to do and/or that the world around you expects you to be able to do then it becomes a handicap. Nobody expects you to lift 5000 pounds with your bare hands or fly like Superman. And even though it might be fun, that disability really doesn’t adversely affect your life. Being able to walk, care for myself, engage in the types of physical activities that most people is something that I could reasonably want to do and that the world about me expects me to be able to do.

People militantly declare “I’m not disabled”. Bullshit! That’s denying reality. You have a disease or medical condition. Willing it away or denying it doesn’t work. Short of a medical cure, you are stuck with it. Similarly that disease or medical condition gives you a disability. Again willing it away, denying it, ignoring it doesn’t work. Handicaps are different. Handicaps can be changed. You can overcome handicaps. Change my environment. Put me in an environment with curb ramps, elevators, accessible transportation. Provide me with affordable assistant technology such as wheelchairs, adaptive computers, alternative communication tools. Adjust society’s expectations of me. I can eliminate my handicap. These strategies will never cure my disease, remove my medical condition, or give me abilities that I will never have. They do eliminate handicaps.

One of my problems with the shift from the term handicapped to disabled is that it is applied to assistance programs. By establishing programs for disabilities rather than handicaps we are saying that the disability, in and of itself, entitles you to assistance. I know of people with disabilities who do not have a handicap and do not need or deserve any sort of assistance. Apart from medical research, no benefit or program is ever going to get rid of a disability. But appropriately administered government and private programs can and do eliminate handicaps.

Those who declare “I’m not disabled” or “don’t call me disabled” are denying reality. Scream that you are not handicapped and I will support you. On a good day neither am I. Deny your disability and you are living in fantasy land.

Denying a disability exists is one extreme. Denying the negative aspects of disability is another extreme. There has to be a middle ground in which you can embrace the giftedness of a disability and own it as part of your identity without denying the reality of the inherent disadvantages to it.

Anything that diminishes our humanity or is an expression of a lack of respect for our humanity and free will, regardless of what kind of -ism it is, must be challenged, intolerated, and eliminated. But crying wolf and unjustifiably accusing people of such denigrating attitudes where they don’t exist only serves to perpetuate that which we are trying to oppose.

Afterward

This note is added April 14, 2018.

Stephen Hawking was interviewed in an episode of Star Talk by host Neil Degrasse Tyson. The episode aired shortly before Hawking died. I just got around to watching it today. In it, Tyson asks “Stephen, you’ve been in that zero G airplane, I’ve always wanted to go. I wondered where it felt like for you.” Hawking replies (as they show photos of him floating in zero G smiling) “It was wonderful to float weightless free of my wheelchair. I could’ve gone on and on and on.” For me that definitively ends the debate on whether or not postmortem depictions of him free of his wheelchair are appropriate for not. The words “free of my wheelchair” are his words. And he didn’t say “it was a fun experiment one time”. Instead he said “I could’ve gone on and on and on.” Case closed.

A Personal Reflection on My Role Model Stephen Hawking

I have to share my thoughts on the passing of Stephen Hawking. The renowned physicist and pop culture icon who expanded our boundaries of knowledge of the nature of the universe did so while fighting a lifelong battle with ALS motor neuron disease. His iconic electronic voice is the most famous piece of assistive technology for the disabled ever created. He passed away today March 14, 2018.

I was born with a genetic neuromuscular disease known as Spinal Muscular Atrophy type 2. Like Hawking’s disease ALS, my own disease SMA affects the motor neurons. Also like Hawking, I’ve had a lifelong passion for science. I’ve always been a curious person but that curiosity was given an outlet somewhere around fourth or fifth grade when I discovered science. I have a logical mind and it was obvious that scientific pursuits were within my grasp despite my own physical disability.

It might seem obvious that Stephen Hawking would be a hero and a role model for me but I was not always anxious to embrace role models. He earned that title with much difficulty. Let me explain.

With my passion for science and intellectual pursuits, I was especially fascinated by stories I heard about Albert Einstein. He passed away in 1955 just a few months before I was born but I knew that he was considered by consensus “The Smartest Man in the World”. In fact the name Einstein has become a synonym for genius. For example “Smartass! Do you think you are some sort of damned Einstein?”

I learned that not only was Einstein considered The Smartest Man in the World, he was actually a kind of a celebrity and pop cultural icon. He was the intellectual equivalent of a rock star in an era before rock ‘n’ roll.

In some ways I felt cheated that I was living in a time that did not have an obvious successor for both the roles of Smartest Man in the World and intellectual pop culture icon. While scientists such as Carl Sagan, of whom I was a great fan, achieved celebrity status and he was a man of great intellect, he did not rise to the level of Einstein as a renowned genius. I always felt there should’ve been some sort of committee to decide who was Smartest Man in the World. Oh sure they give out Nobel prizes in physics, chemistry etc. but even those people typically only occupied the news cycle for a few seconds after winning their awards. There was no heir apparent to Einstein and it didn’t seem to bother anybody except me.

I can’t say for sure when I first became aware of Stephen Hawking. But he immediately became a hero for me. Not only was he fast approaching (and eventually reached) the status of consensually the Smartest Man in the World, the fact that he did so with a severe disability was an added bonus for me. In more recent years he definitely has become the intellectual pop culture icon for which I longed. Others agree with me that he was as iconic as Einstein. In Hawking’s New York Times obituary it says:

“Not since Albert Einstein has a scientist so captured the public imagination and endeared himself to tens of millions of people around the world,” Michio Kaku, a professor of theoretical physics at the City University of New York, said in an interview.

It is speculation on my part because I did not experience the Einsteinian era myself but I would say he fulfilled that pop-culture role even more than Einstein himself. Not only was Hawking a best-selling author and the subject of multiple documentaries and dramatic biopics, he had cameo parts in TV shows such as The Simpsons, Star Trek: The Next Generation, and The Big Bang Theory. The latter of those might never have even had a title were it not for Hawking. His electronic voice is much impersonated by standup comedians. Although the times are different, I really don’t think Einstein achieved those levels of celebrity.

For some bizarre reason, most of my life I’ve not really found myself in need of an inspirational role model. I looked at other famous people with disabilities with a bit of cynicism. In my autobiographical magazine article “The Reunion” I told the story of a group discussion we had in my high school years in the early 70s where I expressed my cynicism about such role models.

“You hear all these stories about the ‘Super-Cripple’ types who make it in the world and are successful and are supposed to be our role models,” I said. “But I’m not FDR or Stevie Wonder or that girl from ‘The Other Side of the Mountain.’ Where did they get that strength? How did they overcome their handicaps? What do I do to tap into their magic that lets them cope or achieve or be somebody?”

Nobody could tell me.

And I then realized that nothing magic was going to happen. The way to do it was to just do it. Just be. There’s no magic.

I’ve also felt frustrated by such super cripples. I’ve often stated in conversations that when I was very young, all a kid with a disability had to do to get publicity was sit in a wheelchair and look cute. I had been a bit of a poster boy for United Way when I was 10 years old and for Goodwill Industries also at around that age. I have been the subject of TV news items and newspaper articles in the early 80s when I designed a special piece of communication software called VersaScan for my friend Christopher Lee who had severe cerebral palsy. The idea of a disabled software engineer developing something for another disabled person made for a unique human interest story.

But as the years rolled on, it became more and more difficult to mine publicity coins. There were paraplegic mountain climbers. Wheelchair users were an everyday part of marathon races. Paralympics and Special Olympics are well known and well covered institutions. When I was young there was no Make-A-Wish Foundation yet these days they have a monopoly on the “sit in a wheelchair and be cute” phenomena. Perhaps no one raised the publicity bar higher than NPR journalist John Hockenberry who was a paraplegic war correspondent in Afghanistan. I have been known to say jokingly “How the hell does the ordinary gimp get any ink these days an atmosphere like that?” Yet that joke thinly hides my jealousy and cynicism. I do consider people like Hockenberry a bit of a hero. I admire him for his accomplishments. But I never considered him or other disabled people of note to be role models. I never sought to pattern my life after them or draw strength from them.

Yet somehow, whether I felt I needed it or not, Stephen Hawking became not only a person whom I admired for his accomplishments, he truly became an important role model for me.

Sometime around 1995 I watched a documentary about Hawking. It gave me insights into the thought processes he uses to develop his hypotheses and theories. Because he’s physically unable to write out long equations on chalkboards, windows (like the beautiful minds do) or even with paper and pencil, he uses the strategy of visualizing the graphic representations of the equations in his mind. Those visualization techniques have given him insights into those equations that had escaped other physicists. In other words, his physical limitations were in fact an asset that made him a more insightful physicist than his peers.

This whole concept of a disability as a gift or an asset was a concept that was developing in me. I had a growing sense that in my own life, my perspective, my insights, my ability to contribute to society in my own unique ways had been enhanced by my lifelong disability. I won an award for my autobiographical magazine article “The Reunion”. It was voted by the Indiana Chapter of the Society of Professional Journalists Sigma Delta Chi as a “The Best Magazine Feature” in the state of Indiana in 1987. My simple story about coming of age with a disability was considered more newsworthy (or at least better written) than stories about famous AIDS patient Ryan White or the crash of a military jet into an Indianapolis airport hotel. Understanding that Hawking had similarly turned his disability to his advantage was a conformational data point to prove my hypothesis that having a disability wasn’t all bad.

Very shortly after seeing that documentary on Hawking, I found myself in the hospital with a severe case of pneumonia. Although I had wrestled my entire life with a shortened life expectancy and had often found myself obsessed with a fear of death, this particular incident was probably the first time I had felt I was imminently in peril. The doctors discussed with me the real possibility that I could end up on a ventilator. They noted that while it was intended as a temporary solution until I got over the pneumonia, there was a possibility that once I was on the ventilator I might not come off. At that point they started having those difficult discussions about what level of care you want. Living wills and “do not resuscitate” orders are discussed.

With my fear of death only barely under control to the point where I could live my life without obsessing over it, my natural reaction was of course to say that I wanted everything possible to keep me alive. But that standard response now had a new justification. I could say to myself and to others that if someone like Stephen Hawking could lead a productive life while on a ventilator then perhaps I could as well.

For the first time I was able to look at someone as a true role model and say “If they can do it… then so can I!” Previously I had mistakenly thought of a role model as someone whom I should attempt to equal in their accomplishments. Realizing that I could not be that person, I had rejected the whole concept of role models. What Stephen Hawking taught me was that a role model is not someone whom we should try to equal or surpass. Role models are people who empower us to be the best versions of ourselves.

This brings up the whole topic of “inspiration”. There is a trend these days among many people with disabilities to boldly and militantly declare “My purpose in life is not to inspire you!” I can understand the roots of this complaint and I partially agree with it. I think the problem is when someone says “you are an inspiration to me because of your disability” it often comes from a place of pity. People don’t necessarily understand that it comes from pity. They don’t intend for it to be. And even when it does not come from pity, it is often perceived that way by the disabled person. Not only does pity play a role in this equation… There is also an undercurrent of guilt at play. People are made to feel guilty for their good fortune. When they admire or are inspired by someone with a disability it often comes from misplaced guilt that they are healthy. There is also a sense that they underestimate their own ability to deal with such a challenge should they ever be unfortunate enough to end up with a disability of their own. I try to remind people that they underestimate their own capabilities when they feel that way.

When we hear that we are “an inspiration” it often comes across as a bit condescending. What we hear is “Oh look at the poor little handicapped kid. Isn’t it special that he even gets out of the house.” It’s a bit ironic and admittedly hypocritical that I lament the passing of the days when “sitting in a wheelchair and being cute” was sufficient to get publicity yet I decry the condescending attitude behind that faint praise.

It is the goal of every disabled person to be as normal as possible. So to be praised in a way that implies our disability, in and of itself, is a source of “inspiration” is easily rejected by us. We want to be known for our accomplishments and our abilities and not for our disability. So I sympathize with the philosophy of “I don’t exist to inspire you”.

On the other hand, in admitting that someone like Stephen Hawking is a legitimate role model and having a deeper understanding of the true purpose of a role model, I no longer tend to reject it when people say I am an inspiration to them. I don’t mind seeing myself as a role model. When people express feelings of admiration or inspiration attributed to Stephen Hawking, it doesn’t come out of a sense of “oh the poor guy in a wheelchair I feel so sorry for him”. He is admired and respected for his accomplishments in the disability is only one facet of that admiration.

It has been encouraging to me the way that people with disabilities have been recently portrayed on reality competition shows such as American Idol and Dancing with the Stars. While the judges of those events do tend to overuse the word “inspiration” in their commentary on these disabled contestants, they do also make it clear that they are forced to judge the person on their talent. They make sincere efforts not to be swayed by the disability or other inspirational natures of their personal stories.

So while I agree that I don’t exist solely to be your inspiration, if I do inspire you by my accomplishments then I have no difficulty with that. My evolution of attitude in this area is thanks to the inspiration of my role model Stephen Hawking.

One of the other interesting stories from that documentary in the mid-1990s had to do with Hawking’s position at Cambridge University. He told the story that there is an old ledger that has been signed by every member of the faculty of that institution. In 1997 he was appointed to the Lucasian chair of mathematics at Cambridge. It is a post once held by Isaac Newton. Someone realized he had never signed the ledger. So with great difficulty they placed a pen in his hand and he signed his name to that ledger recognizing himself as one of the legitimate successors of Isaac Newton. He then calmly stated “It was the last time I ever signed my own name to any document.”

I was in awe of that story. It brought tears to my eyes that the last time he signed his name was in such a momentous and meaningful way. I remember lying in the hospital in 1995 telling that story to my friend Judy. I wondered what would be the last time I was ever able to sign my name by myself. I already did it rarely. She speculated perhaps I would someday sign my marriage certificate to the love of my life. Perhaps that would be a fitting and momentous final signature.

That brings us to another way in which I admired Stephen Hawking who was married and had three children. I had long recognized that the odds of me finding a wife or being able to have a family and children would be quite a long shot. It led me to a mistaken belief that any time a woman took any interest in me, I had to do everything possible to try to direct that relationship into a romance that would someday lead to a happily ever after ending. It took me some time to appreciate that this wasn’t a good strategy. I eventually learned to focus my energy on building strong and meaningful friendships that would be fulfilling lifelong relationships even if they were not romantic or marital. My successful relationship with Judy is a testament to the success of that strategy.

But as I stated before, the goal of most disabled people is to be as normal as possible and so I admired Hawking for having a wife and children despite his disability. Although extramarital affairs, divorce, and remarriage are not typically thought of as admirable or praiseworthy, they are in fact very much normal. So when I heard that he divorced his wife Jane in 1995 and married his caregiver nurse Elaine, I had to give him an “attaboy“ for doing such a “normal” thing. He later divorced his second wife in 2006. He maintained a friendly relationship with his first wife, his children and grandchildren. His first wife’s memoir “Travelling to Infinity: My Life with Stephen” appeared in 2007, and was made into the Oscar-nominated film “The Theory of Everything” in 2014. It won the BAFTA award for Best British Film and Eddie Redmayne won best actor Oscar for his portrayal of Hawking.

As it turned out, the last time I ever signed my name was not on a marriage certificate. Instead it was a few years ago when I signed some legal documents establishing a special needs trust that hopefully will provide some financial assistance in the event of my father’s passing. I also signed power of attorney and designated medical representative authority to my dad. It includes an advanced life directive that indicates I want everything possible to keep me alive as long as I have mental capacity of some sort.

I’ve already mentioned there are similarities between Hawking’s disease ALS and my disease SMA. The parallels in our medical conditions became deeper 15 months ago when I ended up in the hospital again with severe respiratory problems. This resulted in me needing to have a tracheostomy and spending several days on a ventilator unable to speak. I found myself wishing that I had Hawking’s electronic voice. The inability to speak over several days was clearly the most difficult challenge I had faced in my over 60 years as a disabled person. I chronicled those events in my essay “Pray That They Listen to the Man with No Voice”. I still use a ventilator at night and have struggled to adapt ways to communicate while on the vent. I’m exploring various communication techniques such as open source speaking software known as CoughDrop and other assistive technology solutions.

I had not done much work in the area of assistive technology since my work in the early 1980s on VersaScan. However since that hospitalization a year ago last December, I’ve focused a great deal of my efforts on developing assistive technology not only for myself but for others. My efforts in the field of assistive technology have motivated others to take up the cause of open source solutions for the disabled. Many have said that my work in this field has been an “inspiration” to them to take up the cause of assistive technology as well. I have no problem with this. Again this is related to my concept of my disability as an asset. There are more talented engineers and programmers then I am working in the field. But they don’t have the perspective that I have as an actually user of the technology. I bring insights to the discussion that no other contributor can provide.

Stephen Hawking is yet again a role model and inspiration in this area. His iconic electronic voice is the most famous piece of assistive technology ever created. As technology has evolved, speech generation has improved to the point where the voices of Siri, Alexa, and other digital persona are every day experiences. Hawking resisted the opportunity to upgrade is rather robotic electronic digital voice to a more natural sounding one. He said that the outdated technology of his speech generation equipment had become so closely identified as “his voice” that he could not imagine changing it. To me this illustrates the intimate relationship that disabled people have with their assistive technology. It truly becomes a part of them. It’s one of the main reasons that my former software company was called “Cyborg Software Systems” and that I still go by the online handle of “cyborg5”. My relationship with my assistive technology is so intimate that it becomes part of me in the way that the mechanical aspects of the cyborg are united with its biological aspects.

I mourn the loss today of my hero and role model Prof. Stephen Hawking. But I really don’t feel very sad. He truly deserves the cliché description of “a life well lived”. Having been told in his 20s that he only had a few years to live, he worked his ass off to contribute something to the world and in the end proved the experts wrong by reaching the ripe old age of 76. I too was told I would not live very long but at age 62, I’m similarly proving the experts wrong.

This brings to mind the famous Vulcan salutation “Live long and prosper.” Certainly Stephen Hawking did so and the universe is a better place because he did.

Links of interest.