Users Manual for Chris Young

Ninth in a series. Click here for the first installment.

Taking Notes

Thursday, December 8th was my first full day of having the trach tube. I was once again speechless as I had been when I was intubated. I learned lots of lessons from those first days. I wish I had had more time being able to talk before having the trach and more time to prepare for losing my voice again. But I had spent much of the past day or so going over things in my head about how I would adapt to the situation.

When dad came in Thursday morning I had him set up the iPhone so that I could type messages to him. I reported to him I had had some pain overnight but the pain medication they gave me took good care of it. I then told him that I was going to write some messages to the nurses and I wanted him to transcribe them on a piece of paper. I had already planned that it would be three iPhone screens worth of messages and I would have him write them all down on a single page. I told him to sit down and relax while I typed away. When I finished soon after, here is what the three messages said.

How to talk to me
Eyebrows up and down =Yes
Mouth side-to-side=no
Wink=I’m okay
Spit swishing =need mouth suction
Click tongue =Need to use message paper. You point to row then I do click then you point to letters and I click to select

I’m quoting these messages from my iCloud archives. I’m not sure that the phrase “Wink= I’m okay” was originally part of that page. I may have added it later and had dad write it in to the paper version later. I don’t really know when I added that particular communication gesture. I remember at the beginning I had the yes and no but on several occasions I tried to mouth the words “I’m okay” and many of the nurses were unable to read my lips. I thought that the words were distinct enough that you should have been able to understand me. All I know is that there were sufficient number of nurses who could not get it from lip reading so I invented the wink as a new way to communicate that fact. Once I had instructed them how to communicate with me, the next message talked about my specialized nurse call button.

My call button is in my right hand and is very touchy! Keep covers, tubing , and everything away.
Put rolled up washcloth under my wrist. My first finger on long metal part, thumb atop shorter part, blue part toward palm

Those particular instructions probably were very difficult to follow. We always tried to have the nurse take a good look at how my hand was positioned and how I was holding the button before moving it. Dad or Carol always put the button in my hand before they left me alone for the night. As I mentioned earlier, one of the problems was that I could not operate the iPhone and told the nurse call button at the same time. So my only means of communicating at night was by having the nurse point to the message paper row by row, column by column and spelling out words one letter at a time. The final message was instructions on how to safely reposition me.

How to move me
DON’T touch knees! The kneecap dislocate easy. Keep knees apart when lying flat but put side-by-side when rolling me. Roll me using hips and shoulders

The issue with my sensitive knees was one that really worried me. I had always relied on my ability to speak to that I could warn people not to grab my kneecaps. It must’ve been divine providence but I spent nearly an entire month in this hospital and another one and never once did my knees get kinked.

Once needs instructions were written down, I would leave this piece of paper lying on my chest throughout the night so that anyone who walked in might see it. I would tell each nurse to be sure to point it out to the next nurse during the shift change. Although this “User’s Manual” on how to operate me wasn’t always effective, it was better than nothing at all.

Bodily Functions

I’m going to issue a TMI warning at this point. (Too Much Information). This next section deals with some rather gross stuff.

Ever since I had the G-tube installed last May, I’ve had lots of trouble with constipation. After consulting with the gastroenterologist he suggested I take a dose of Mira-Lax as needed to keep things going. Through experimentation we had found that about one dose per week was enough to keep me going reasonably well. The problem was with all of the confusion and lung problems I couldn’t remember when I had had my last dose. I had to now been in the hospital five days and still had not had a bowel movement.

One of the things that concerned me was they had been giving me antibiotics. Traditionally that gives me diarrhea so I was a little bit reluctant to take a laxative if the antibiotics were already going to make me loose. Dad and I talked about it via my iPhone message and I decided to delay taking the laxative till later that day or perhaps the following day. As things turned out I took a dose of the Mira-Lax early the next morning.

Speaking of antibiotics, I don’t think I fully addressed the issue of whether I did or did not have pneumonia. Some of the Facebook messages posted by my sister Carol said I did or did not have pneumonia at various points. Some of my messages may also have been confusing. But here is the bottom line on the pneumonia issue. When I first went into the ER on December 3 they took an x-ray and said that my lungs looked clear. They later took a sputum sample and did a culture and discovered bacteria so based on that they concluded it was technically pneumonia. There was a second chest x-ray at one point but I don’t think I ever heard any of the results from it. So based on that sample, they gave me a few days of antibiotics but I really wasn’t on anything for very long.

The other topic of conversation with the nurses and doctors was my urinary output. I have an absolutely enormous bladder. It rarely empties completely. They can do an ultrasound scan on your bladder to estimate how much is in there. I was reluctant to let them do a scan because I knew they would find 700-800cc or more which would be very uncomfortable for the average person. I knew if they did a scan then they would want to catheterize me. I don’t even start to get uncomfortable till we approach 1100cc. They were not giving me all my usual medication and so I wasn’t peeing as much as I usually do. The problem is, those medications lower your blood pressure and they were already concerned about my blood pressure being low especially while I was sleeping.

I communicated some of that to the doctors by iPhone message but I found one other message shortly after that which I thought was kind of funny and worth quoting here.

Message for doctors
I peed right after you left.
I couldn’t pee while you were all standing around threatening me with catheter. Also note that I wear diapers at night which can’t be measured. I know when I need catheter trust me.

I ended up winning this argument. Despite their continued concern about my bladder, I never did let them catheterize me. This may seem like a minor victory but I needed a win to boost my spirits.

Weaning off the Ventilator

The plan was to try to have me off of the ventilator and just breathing normally through the trach as much as possible. This first day I was able to stay off the ventilator about five or six hours. From time to time I would start to get congested. The trach would make a rattling noise and at times it would get difficult to breathe when the congestion would appear.

The solution is to suction out the trach using a catheter. It had to be done by an RN. A regular patient care technician was not allowed to do it. They would open up a little kit that contained a pair of sterile latex gloves. Most of the latex gloves they just pull out of a box hanging on the wall are clean but not completely sterile. Their purpose is to protect the nurse or caregiver from my bodily fluids. However because you’re going to be sticking tubes into the trach directly into the lungs you need to be completely sterile.

The gloves in the kit have the cuffs turned up so that as you pull them on you are actually grabbing the cuff from the inside and only rolled the cuff back after it’s on your hand. They make a big deal out of the fact that one of your hands (your right hand if you’re right-handed, left otherwise) is your clean hand. Once you get that glove on you never touch anything except for the catheter tube itself. The other hand is your dirty hand and it can touch just about anything.

Once you get the gloves on, you hookup the catheter to a suctioning tube. There is a little hole at the top of the catheter that you can put your thumb over to either turn a suction on or release your thumb from the hole so that it leaks and doesn’t suck. With the suction off they stick the tube down your trach until they feel resistance. Basically the tube is hitting the Y branch in your windpipe where it splits to go to each lung. Then they put their thumb over the hole and slowly pull the catheter out while making a circular motion like you were stirring a cup of coffee.

Below is a YouTube video that demonstrates the process. In this video they set up a small container of sterile water to rinse out the catheter however they were not doing that part of the process at St. Vincent. This particular video was the only one I could find that was closest to what they did at St. Vincent.

All of the setup of the suction that they describe in the video was already in place. So it goes much faster than this particular video implies. But it would still take a considerable amount of time first of all to round up a nurse, she had to get the kit open, put on the gloves, connect the tubing, and do the procedure. Although they could make multiple passes of the tubing if the first one didn’t clear out completely, after one session they always threw away the tube. That means the next time they had to open a new kit and start from scratch. We asked if they could re-use the catheter to speed up the process. There were a couple of times where I was getting a little bit of distress waiting for them to get things set up. It took for a while to get a feel for when I really needed it. I didn’t want to ask for it at the first sign of a little rattle in my breathing. But I did not want to wait until I was in real distress because it took time to get things going.

Throughout the first day, I think I only needed suctioning two or three times over the five or six hours that I was off the ventilator. So it wasn’t too bad. By the time dad was ready to leave for the day I was ready to get back on the vent. I didn’t want to be off of it at this point at least unless there was somewhere on there with me who could call the nurse quickly if I got in trouble.

That pretty much describes my first full day of having the trach. The next day would be a very eventful day that included a visit from friends, surprise support from hundreds of miles away, and the dreaded “second incident”.

To be continued…

A Short-Lived Conversation

Eighth in a series. Click here for the first installment.

Getting Caught Up

On December 5th I was complaining that the respiratory people were not doing enough suctioning to get my lungs cleared out in preparation for being extubated. But later that day and throughout the night they did several suctioning’s and my lungs were feeling much better. Everyone seemed anxious to get me extubated and since things were going better I agreed on the morning of the 6th to go ahead and remove the tube.

It’s not exactly painful to be extubated but it definitely is an uncomfortable experience having a long tube yanked out of your nose. It was good to be able to talk again although my throat was a little sore and my voice a little weak.

Shortly after they removed the tube one of the nurses looked in the hallway and said “There is a priest outside your room.” I told her to invite him in. I figured it was the St. Vincent chaplain. Much to my surprise it was my good friend Fr. Paul Landwerlen who had been a pastor of St. Gabriel for many years and was partially responsible for guiding me back into the Catholic Church after being away from a years. Fr. Paul had transferred to St. Vincent de Paul parish in Shelbyville Indiana but a couple of years ago had retired. He still lives in Shelbyville and fills in for other priests and parishes around the diocese.

He was surprised that I was extubated and was able to talk. That’s why he was just kind of lurking around outside praying for me and not disturbing me. We had a really nice conversation and I told him a little bit of what I’ve been going through. Naturally he was happy that I was off the vent and talking normally. He told me he had been having some problems with pneumonia lately but was treating it aggressively I was doing okay. We also talked about sports and he was looking forward to going to the next Colts game where he had been given tickets to watch the game and one of the luxury suites.

If I had seen him even a day earlier I would’ve asked him to give me the sacrament of anointing of the sick. As I mentioned before that isn’t necessarily the old-fashioned “Last Rites”. You don’t have to be on your deathbed to get that sacrament. However considering that I was now off the ventilator, my lungs felt good, I didn’t really feel like I needed anointing. I could see myself getting out of the hospital in a couple of days. I thought about having him hear my confession (not that I had any huge sins weighing on my conscience). But about that time dad came in and we talked. Soon after Fr. Paul left.

I brought dad up to speed with everything that happened since he left the previous night. Of course he was happy to see me off the ventilator and doing well.

At one point I was visited by the resident Dr. Heaven. She was the one who had tried to communicate with me using the message board with me pointing to it with a straw in my mouth. It was great to actually have a real conversation with her. I apologized to her for making her a guinea pig in my failed attempts to communicate. We both had a really good laugh over the whole crazy experience.

One of the challenges Dr. Heaven had was in the area of nursing skills. For example occasionally I needed to spit something out into the Yankaur but she had been having difficulty operating it. It’s just a plastic tube connected to a suction device like you spit into at the dentist office. But this one had sort of a plastic sheath over the end of it that for lack of a better word look like a foreskin. You had to pull back the plastic sheath in order to put it in your mouth. She didn’t know how to operate it. When I finally was able to explain to her how it works, she said “You been waiting for days to try to explain that to me haven’t you?” I laughed and said “Doc you are going to be a great doctor someday but I hate to tell you this that you make an absolutely lousy nurse.” and laughed heartily. She agreed with me completely. I told her that she should speak to the head of the residency program and tell them that my recommendation was that they take a day or a day and a half and have all the residents go to a CNA (Certified Nursing Assistant) in-service training for basic patient care skills. Like how to operate the suction, operating the hospital beds, how to turn a patient etc. She chimed in saying “I totally agree and on top of my list is how the hell do you get an IV pump to stop beeping!”

It was such a relief just to be able to talk to the woman and laugh and joke and relive all of the frustrations that we had in our working relationship up until that point.

I was also visited by the young man I spoke of a couple of installments ago who was the one person who was able to read my lips or understand what I was saying as they were trying to intubate me. It turns out he is a medical student named Brady Hansen. I congratulated him on being most focused on what was going on with me when he was in a room full of more educated and experienced people.

We joked about my communication problems with Dr. Heaven. They were both in the room at the same time as we were talking about having the residents learning nursing skills. In regards to my communication problems I said it reminded me of the famous line from the old Paul Newman movie “Cool Hand Luke” where the prison boss beats the crap out of Paul Newman and then on a thick southern drawl says “What we have here is failure to communicate”. Even though this young man was probably in his mid to late 20s likely not even 30 he knew the reference. I was very impressed. Anybody his age who can quote classic movie lines is pretty cool in my book.

I was reminded of a few months earlier when they were putting in my G-tube I was talking to radiology surgeon and telling him a joke about old-time radio and vaudeville comedian George Burns. He had never heard of him. The guy had to be 40 and 45. I realize that’s not 61 like me but I would’ve thought he had heard of George Burns. Yet another example of how student Dr. Brady was in my book ahead of his older and supposedly more experienced colleagues.

By the way several weeks later when I was finally out of the hospital I was browsing through the on-screen guide on my DVR and noticed that “Cool Hand Luke” was playing on Turner Classic Movies. I recorded it and watched it again. Below is a YouTube clip of the famous scene. I don’t know how long that clip will be valid because it probably violates copyright.

While talking to student Dr. Brady and resident Dr. Heaven I told them that part of the problem I had with using the communication board was that the alphabet was in alphabetical order instead of normal keyboard QWERTY order. A lot of times I couldn’t find the letter I was looking for. It reminded me of the old Stephen Wright joke where he asks “Why is the alphabet in that order?… Does it have something to do with that song?”. They both got a laugh out of that joke even though they’d never Stephen Wright.

Success was Short-lived

I don’t have a lot of notes about what else happened December 6th. For the most part it was uneventful day. I believe it was probably the day that I spoke to the nursing supervisor about the so-called “first incident” in which the nurse tried to maneuver me against my will and refuse to communicate with me. I don’t recall if Carol visited that afternoon after dad left or not.

Somewhere late in the evening of the sixth my lungs started going bad again. It was just like it had been three days earlier in the ER and when I first brought me into ICU. Sometimes I could breathe just fine. Then a minute later it felt like my windpipe was closing up from a spasm of some kind. It seemed to me like I was going to have to be intubated again. They paged whoever was on call from the pulmonary doctors. Again a large contingent of residents began to gather around. Among them was resident Dr. Heaven and Student Dr. Brady.

I don’t recall the name of the pulmonary doctor. He went up my nose with the scope and try to suction out some of the gunk. He was looking at a video screen when he noted that something was wrong with my vocal cords. They seem to be stuck together. The doctor who was operating the scope asked if there was a way to record this video. Student Dr. Brady whipped out his iPhone and begin videoing the screen. About that time one of the other doctors figured out that there was a record button on the scope itself and they began recording properly. Brady gave me a heads up that he had the video on his iPhone and would show me later.

I begin to think that whatever this vocal cord problem was had been a major part of my breathing difficulty all along. What felt to me like my entire windpipe spasming closed may have just been my vocal cords not opening properly. Below is a medical diagram illustrating how vocal cords are supposed to work.

In the video, mine were not completely closed but they weren’t as far open as they should have been. Also about one third of the way up there was a place where it looks like they were stuck together. The opening between the vocal cords look like a tall skinny figure 8 or perhaps an infinity symbol standing its end.

In the end they did not put the intubation tube back in me. They said what I really needed was a tracheostomy. They did clear out a bunch of gunk and I was able to breathe okay again using my CPAP and some oxygen. That evening and early the next morning I had several conversations with a variety of doctors about what it would mean to have a trach.

What Are My Options?

For many years I had known there was a possibility that I could end up on a ventilator permanently. I was beginning to wrestle with the fact that today might be the day that that happened. I had lots of questions for the doctors and in summary I learned the following… Doing a tracheostomy did not mean that I would be on a ventilator permanently. Having the hole in my throat would make it easier to suction out any congestion that developed in my lungs. I would not have to worry about the spells of respiratory distress that I had been experiencing.

A just recently watched a political dinner on C-SPAN where Hillary Clinton and Donald Trump spoke at some Catholic fundraiser. Clinton made some really good self-deprecating jokes and as usual Trump made a total ass of himself. But the guy who was the head of the organization look to be in his mid 60s and he had a trach tube in his throat. He was dressed in a white tie tuxedo just like all the other people in this very formal affair. He was the head of the organization presenting the event. However when he talked, he had to put his finger on the trach tube. I had seen other people with a trach and knew that in order to talk they either had to put their finger over the hole or press a button that would temporarily close the hole. This guy looked like he was pushing a button.

While it was reassuring to see a political and religious bigwig leading a relatively normal life despite a trach, there was no way I was going to be able to push the button like he was. The doctors who assured me that there was a special kind of talking valve that you can use instead. It was like a butterfly valve that when you inhale through the trach, the flaps collapsed together and let the air go past into your lungs. When you exhale, the flaps fly open and seal off the trach so that the air goes out you windpipe, past your vocal cords, and you can talk normally. He called it a “passing mirror valve”. He said something about the flaps popping open like a mirror.

I later learned that was not at all what was called or why it was called that. It is actually called a “Passy-Muir” valve after the two inventors named Patricia Passy and David Muir. More about them later…

They said that I would need to get used to the trach for a few days. I would be conducted to ventilator initially. Then they would gradually wean me off of the ventilator to breathe normally to the trach. Once I had established that I could do that well then they would try the Passy-Muir talking valve.

The bottom line was I would still be able to talk even with the trach. I would not be permanently on a ventilator at least not yet. And the fact that we could easily suction out any congestion that might accumulate would be a big plus. At this point it was a real no-brainer for me. I told them I would give consent to have the trach. They said that they would do it tomorrow (Wednesday, December 7).

Surgery by a Familiar Face

Wednesday morning we went over the details of the surgery again and I gave my consent. I don’t recall if I called dad at home and told him they were going to do the surgery or how he found out about it. I don’t believe I called him the night before because all of that suctioning and vocal cord issues were in the middle of the night. I’m pretty sure I called him early Wednesday morning to let him know what was going on so he could get there in time for the surgery. Karen was already planning to visit me that day. When dad told Carol about the surgery he said she did not need to come especially since Karen would be there. However Carol took off work to be there anyway.

I took a look at Facebook to see if I told everyone about the trach surgery. I can’t find any message where I told everyone that the surgery was going to happen. I did find this message that Carol posted. She told everyone I had called her and told her about the surgery. I don’t remember calling her directly. I thought dad had called her. Oh well…

Much to my surprise, the ear, nose, and throat doctor who was going to do my surgery was a familiar face. Dr. Robert Youkilis was my regular ENT doctor. I have problems with tinnitus or ringing in my ears. He also periodically cleans the buildup of wax out of my ears. He has consulted with me on my chronic sinus problems as well. It seems you never really know how specialized a specialist will be within their own field. Even though he is an ear, nose and throat doctor, I had only seen him for ears and slightly nose issues. I wondered if perhaps he was more of an ear specialist then the others. But apparently he does throats as well and would be doing my surgery. I never did find out if it was coincidence that he was the guy on call that day or if they called him specifically because I had him listed as my ENT doctor. I think it was probably just lucky coincidence or divine providence that he was the guy to do my surgery.

I was able to speak with him briefly before the surgery. We talked about my inability to be intubated normally and that we would have to do a nasal intubation up the left nostril. I believe it was about noon when they carted me off to the surgery. They transferred me from the hospital bed onto a gurney and I gave them warnings about being careful with my loose kneecaps. They said they could do the surgery on that gurney and I would not need to transfer again.

Dad followed me down to the surgery area but then eventually went to the surgery waiting room. They must’ve given me a really good dose of Versed because I don’t remember anything about going into the operating room. The next thing I knew, I was waking up back in my same room in the ICU. Dad and Carol and Karen were there.

Karen said “You probably think you are in bad shape for me to show up.” But I knew that she was coming anyway that day. I was slightly more surprised to see Carol but then again not really because I knew she would want to be there once she learned I was having surgery.

There was no pain in my throat where the actual trach was. By now I was getting used to waking up being on a ventilator so that wasn’t such a weird experience. My main problem was my mouth and tongue especially were very sore. Carol explained to me that the surgery had been a bit difficult. She said that Dr. Youkilis told them they had to put my tongue in a clamp and pull it out for some reason. I explained why my tongue was so sore. In fact there was an area on the tip of my tongue in the very center that was actually numb. It was well over a month until that numbness completely went away. I’m still not sure if it really went away or I just got used to it and my brain just sort of fills in whatever information it can to make my tongue feel normal.

I made the following post to Facebook via my iPhone at 2:41 p.m. saying that the surgery was over. Carol made a similar post to her Facebook page a few minutes later.

I just now browsed through my iPhone notes containing all of the messages I wrote to family and the nurses on my iPhone that day. Most of it was asking them to tell me more details about why the doctor had to put a clamp on my tongue. I also talked about what kind of pain medication I was getting and how often. The rest of that day I was just trying to recover from the surgery and dealing with the pain issues.

There was one note where I told Carol that I had talked to a chaplain and asked about getting anointed. I don’t recall when that happened but it must’ve been that morning before the surgery. Until I had the bad spell the night before and had decided to have the trach, I would not have requested the anointing. The chaplain told me that the priest would be in on Thursday and could do it then. I had told her there was no hurry and that was okay.

The plan was that they would gradually wean me off of the ventilator and let me breathe through the trach normally. I told them my iPhone message that I wanted to relax the rest of the day and try to get off the ventilator on Thursday. Apparently they agreed.

To be continued…

The IPhone to the Rescue!

Seventh in a series. Click here for the first installment.

Why I Have an iPhone

I’ve never been a big fan of Apple products. I always thought it was ridiculous that people would stand in line for hours to get the newest iPhone when they already had the previous model in their pocket. Why not just order the thing in the comfort of your home and wait for UPS or FedEx to deliver it. I’ve been very loyal to brands like HP printers, Gateway 2000 and Dell Computers but nothing like the fanaticism of Apple fans. Back in the day I had an Apple ] [ but I only had one because I thought it would give me more programming jobs. I never have owned a Mac of any kind. I’ve always been an MS-DOS and Windows and Intel-based PC kind of guy.

Many years ago I took a chance and bought an iPod touch version 1. It’s sort of like an iPhone without the phone. It was a touchscreen device for playing video, games, Internet access using Wi-Fi. I had a lot of fun with it and later upgraded to the iPod touch 4. However eventually the newer versions of iOS would not work with the iPod touch 4. I hated to buy a touch 5 at the time when the latest iPhone was a 6. I kept thinking they would come out with an iPod touch 6 but they never did. At one point I did a Google search for “Android alternative to iPod touch”. The recommendation was to buy a cheap android phone and don’t activate it. A few years ago I did just that. I purchased a Motorola Moto G android phone and did not activate it. I just used it like an iPod and was really happy with it.

Eventually I discovered a cell phone company called “Ting” which has a really cheap pay-as-you-go plan. You start with nine dollars per month. You get up to 100 minutes of talk for an additional three dollars per month. You get up to 100 MB data for additional three dollars per month. And you get an additional 100 text messages for three dollars per month. In most months I would need no data because I’m always at home on Wi-Fi. Also I don’t very often use text messaging. That means for $9+ $3 per month I could make a few phone calls and that would be it. Worst case scenario it was $9+$3+$3+$3=$15 per month if I talked, texted, and used some data. I couldn’t pass up that kind of deal.

I had no intention of going back to Apple or iPhone even if they did ever come out with another iPod touch. But then I discovered something amazing available in iOS. It was an but accessibility feature called “switch control”. Basically allows you to use a Bluetooth device with a couple of pushbuttons to have complete access to the phone without having to use the touchscreen. Although Android has a similar feature, it is so limited that it is basically useless. Here’s an article and a YouTube video that I created that shows how it works on my nephew’s iPad 2. It’s a little easier to see on a big screen like that than to demonstrate it on an iPhone.

Switch control of iPad 2 using Adafruit Bluefruit Micro

When I was no longer able to use the touchscreen because my disability was getting worse, I absolutely had to switch to iPhone. I ended up getting an iPhone 6 and I was able to activate it using my existing inexpensive Ting plan. Of course I had to pay full price for the iPhone up front which was about $675 but all of you who are buying your phones on contract are paying that much for them anyway. You’re just spreading it out over a two-year contract.

I can’t really do everything with the iPhone that I used to be able to do with my old Android device actually using the touchscreen. I miss playing some of the games like Zuma, Where’s My Water, and at one time I was admittedly an Angry Birds addict. But for practical things like Facebook, IMDb, messaging etc. it’s been great to have full access to the iPhone. But I never thought that the phone would be such an essential item until I started using it in the hospital on December 5.

I normally keep my iPhone mounted on a metal bracket that attaches to a swingarm on my left arm rest. On the back of the bracket is a battery pack that I call a Printy-Boost that I designed for the Adafruit Learning System. And next to it is my “Ultimate Remote” which serves not only as a universal TV remote but as a Bluetooth device for operating the iPhone.

When dad brought the iPhone, we put it on one of those hospital bed trays that you can roll around and it extends out over the bed. We propped it up on its side but we needed something in back of it to hold it up. The nurses had brought in a pump to give me my G-tube feedings but for some reason somebody brought in two of them. We used the spare pump to prop up the phone. It was probably a $1000 piece of equipment we were using like a brick 🙂

Then we put the pushbuttons in my right hand and I called up the “Notes” app on the phone where I could type messages. Then the doctors or nurses or my family could look at the phone and read the messages. It’s interesting that all messages typed in the “Notes” app get archived in the iCloud. I’ve never thought I had much use for iCloud or Microsoft cloud or any other kind of cloud storage. But in the long run I was glad it did archive them. Even if you delete the messages, they remain in your trashcan for 30 days. So about 15 days into this endeavor, I decided that that was an excellent record of some of the things I had been through. I copied everything out of the trashcan into a special folder called “deleted messages”. Now as I write this blog I can go back and see every message that I wrote to the doctors, nurses etc. All the messages are dated however it doesn’t appear to have a timestamp or at least not one that I can find.

The first message that I typed on the iPhone was “Raise head”. Not exactly “Come here Mr. Watson I need you” or “What hath God wrought?” But it was a very practical first message. I couldn’t see the phone very well with the head of the bed lying flat. The next several messages were much more significant. They were notes that I wrote for the doctors and the nurses to try to explain some issues that I was having.

Avoiding the Trach

The next note that I typed on my iPhone gives a lot of insight into what I was worried about at that particular moment. I knew from experience with my mom that you can only be on a ventilator via an intubation tube about a week or so. Even though I had only been on this one a couple of days, I was already thinking way ahead. I wanted to make sure that I was not on the vent so long that they eventually had to do a tracheostomy. I was worried that I wasn’t getting the congestion cleared quickly enough. The ventilator tube had a built in suctioning device that they could stick down the inside of it and suck the gunk out of my lungs. They had done it once or twice in the beginning and it was extremely uncomfortable but it helped a lot. I was concerned they weren’t doing it enough. When I would spit into the Yankaur, all they were getting was spit and sinus drainage. I wasn’t coughing up anything and spitting out of my mouth. Here was the message…

Message to doctor
My breathing is good on vent but congestion is no better for the past 24 hours. Oral suction is getting saliva and sinus drainage only. I hate suctioning from vent but I’m willing,and requesting frequent vent suction because I think that it’s the only way I can get better. I want to know how long I can keep vent before I need trac. I want to avoid trac unless 100% necessary so I want to treat aggressively to avoid it. Tell nurse to tell doctor.

My next message was for the nurses to explain a little bit of a change procedure for using the message board. It read…

Message for nurse
I am only using the phone for long messages. Continue using paper for quick message about what I need. When I make click noise it means I want to use message board. You point at row. My eyebrows =yes. I twitch mouth= no. You point to each letter. I click to select.
Please explain this to next nurse at shift change.

This explains that rather than going letter by letter to the entire alphabet, we had determined it was easier to figure out which row the letter I wanted was in and then after picking the proper row going across the row and picking the letter. With the shift changing every 12 hours I needed to train a new nurse with all my signals and procedures. So I wanted to make sure that they didn’t just pass on my medical information but that they also pass on how to communicate with me.

Sometime after I wrote these messages the doctors must have come in and read the first one addressed to them. While they answered my question saying that I could be on vent for many, many days without having to get a trach, they didn’t get my point that I wanted to aggressive suctioning through the vent so that when I came off of it I would not have to go right back on or I would not have to go to the trach. The suctioning worked to clear my lungs but they weren’t doing it often enough. They kept coming in and giving me breathing treatments but they weren’t doing anything to clear the congestion. Apparently they were talking about taking me off the vent very soon. I wanted to be much better before they did even though I was anxious to be off of it. They seemed impressed that I wasn’t rely on the ventilator to breathe. There I was essentially breathing all my own even though I was connected to the machine. They were also impressed that my oxygen saturation level was consistently in the upper 90s. All that is great but if we don’t get the gunk out my lungs we are going to be in trouble again very soon. I wrote to them…

New message for doctor
They not understand my first message. I am certain that I can breathe okay if they removed vent right now but I am very certain I can’t cough up congestion if the vent is removed. That is why I requested that they suction through the vent. I understand that it would be easier to suction though a Trac but why cut a hole in throat if we haven’t tried suction thru the vent? The respiratory therapist have given me many treatments but not any vent suction in well over 24 hours. Please have nurse call doctor and read this message word for word.

This really illustrates one of the most frustrating parts about using the iPhone to communicate. It looked to me like they were skip reading what I wrote. They would just glance at it to get the gist of what I was trying to say. It took a lot of effort to type out a paragraph of text like this and I made every word count. This happened multiple times. I kind of felt like an ass telling them to “read it word for word” but it was obvious that they were not.

This type of aggressive most argumentative discussion with doctors is normal for me but had been impossible when all I could use was the message board. The iPhone really got me back in my game and got me actively involved in my own healthcare decisions. However in a few days those decisions would become so complicated that even the iPhone messaging system wasn’t enough. But for the time being I was extremely happy to be able to communicate more than a word or two.

Most of the messages were things like how to move my arm or leg to get more comfortable. A reminder to dad and Carol that if they saw the nurse move me around, to make sure that I got my call button back in my hand. One problem about using the iPhone was that I could either hold the iPhone buttons or the nurse call button but not both. So the plan was that anytime dad or Carol was with me, I would have the phone in front of me and if I needed the nurse they could call for me. But once Carol left at about 7 PM, she had to take the phone away and make sure that I had the nurse call button. I would not get back on the phone again until dad arrived in the morning.

One of the last messages on December 5 was to tell them to leave the TV on ESPN so I could watch the Colts game.

I did finally get the respiratory therapists to do more suctioning to get the gunk out of my lungs every time they came in every six hours to do a breathing treatment. It seemed to help. The plan was that I would probably be extubated and get of the vent tomorrow (the 6th).

There were absolutely no archived notes for December 6th. That’s because they did indeed pull the tube early that morning and I was able to talk normally all day.

To be continued…

The Non-Incident

Sixth in a series. Click here for the first installment.

Getting Out the Word

The night that I went to the ER dad called both my sisters Carol and Karen to let them know what was going on. Carol began posting Facebook messages starting with this one that she posted on my timeline and 8:41 PM on the night I went to the ER. She continued posting updates on my page and hers so that all of my friends and all her friends would know what was going on.

Our normal routine over the next several days was that dad would come sometime around 9-10 AM in the morning and stay until around 5 PM when Carol would show up and stay with me until the 7 PM shift change. Visiting hours are normally until 8:30 PM however in the ICU they don’t allow family members in the room between 7:00 and 8:00. That’s because they don’t want family members overhearing the doctors and nurses giving reports between shifts. That means if you really wanted to stay until visiting hours are over, you have to kill that hour between 7-8 and then only get additional half hour of visiting time. So Carol would just leave at 7:00 PM.

I mentioned earlier that I told dad to bring my iPhone. Now that I think about it, I seem to recall that dad had already left when I thought of the idea. I use the message board to tell Carol to call dad and have him bring the iPhone. I hoped that I could find a way to type messages on it to communicate easier.

I knew that Carol was posting Facebook messages and that would cover most people. But I know there are some people who don’t check in Facebook very often especially my friend Judy Chapman. So using the message board on the night of the fourth, I told Carol to call Judy and let her know what was going on with me. I was 99.99% sure that her daughter Anne had probably seen the messages on Facebook and would have told Judy. But I just wanted her to get a personal call from us.

I later heard that my request “Call Judy and let her know I’m here” got delivered as “Chris wants to see you.” Well okay… That wasn’t exactly a lie. I really did want to see Judy even though that’s not what I attempted to communicate. This was one case where someone who knew me well (Carol) knew what I really wanted despite my limited ability to express it. Even though she didn’t deliver the message I was trying to deliver, she got the real message right by reading between the lines.

The Second Morning

As a result of my request to inform Judy where I was, December 5 I woke to find Judy walking into my hospital room in the ICU. It brought back memories of a previous visit described here in an older blog. I was very happy to see her. She had taken off work that morning to visit me. It was a bit difficult at first because she didn’t know my yes/no signals but we figured them out pretty quickly. She kept trying to pat my hand to comfort me but unfortunately she was hitting my hand that had the call button in it. I didn’t have an easy way to tell her she could pat anything but that hand.

While Judy was in the room but there was no nurse around, two guys came in lab coats and said they wanted to put in a central line in my arm. It’s called a PIC line which I think stands from Peripheral Inserted Central Line. I had had one before in my neck and they said this one would go in my arm which was easier. I like the idea. However they wanted to put it in my right arm. I talked them into doing the left one because I was worried it would interfere with me using my call button in my right hand. Judy was able to tell them my signals for yes and no. She later said she didn’t feel like she had helped much but I explained just having her able to tell these guys how to interpret my facial movements as yes or no was a big big help.

In the end they agreed and put the line in my left arm despite the fact that the right arm was according to them “the usual way”. It must have something to do with your heart on one side or the other. As it turned out, the PIC line would not have interfered with my right arm. It was nice that these guys were paying attention to me. Much better than I had the day before.

Judy visited for a while. I don’t really remember how long but she determined that I was doing okay despite the circumstances. She went ahead and went on to work the rest of the day. I’m glad my message to her was interpreted wrong. I was really happy she came.

“Only a space…”

I went to a special education school from kindergarten all the way through high school. They had a really nice physical therapy department that included lots of mats for working out, weights of all sizes, and a swimming pool. I never used any of it most of the time I was there. When I did, it wasn’t so much for therapy as it was to get a break from the depressing high school situation we had there. For kids with polio, cerebral palsy or spina bifida physical therapy really had a positive impact. But for guys with muscular dystrophy there really wasn’t anything you could do to help the situation.

When I was about three or four years old when my parents took me to a clinic at Riley Children’s Hospital. They had physical therapists there who would do all sorts of range of motion exercises on me and encourage my mom to do them on a regular basis. I would come away from a clinic visit there with all my joints terribly sprained. The contractures in my joints had already started an early age and all of the exercises they did to try to loosen me up did nothing but hyper extend my joints and tear my tendons.

I will plead guilty to being a “mama’s boy”. I was an only child in the first eight years of my life an incredibly spoiled not only by my parents but grandparents and great aunts. But there were only 2 times in my life that I ever got so needy that I wouldn’t sleep in my own bed and wanted to sleep near mommy. One was after I fell out of my wheelchair at a day camp at Crossroads Rehabilitation Center. And the other one was after an especially torturous physical therapy session that severely sprained my ankles, knees, and elbows. So needless to say I was not a big fan of physical therapy. I later learned the adage that “Only a space separates the therapist from the rapist”.

Sometime after Judy left on the morning of December 5 with no nurse in the room, a man in a woman came in saying they were physical therapists who had been called in to evaluate me. I was absolutely petrified! These people were going to start twisting and turning me in painful ways and I was going to be unable to even grunt or moan in pain. They quickly caught on to my yes and no signals and started asking me about things that were enormously beyond my capability and had they been attempted would’ve caused serious injury. Things like pivoting into a chair. That is a process where someone helps you to sit on the edge of the bed, grab you under the armpits putting about half of your weight on your feet, and then pivoting you into a chair. I vigorously signaled my “NO” signal to all of these suggestions such as sitting on the edge of the bed.

Although they did gently move my arms and legs a bit to see what my range of motion was, they did not try to in any way forcibly extend that range has had the therapists done to me when I was a toddler.

In a nutshell… They listened to me. They respected me. They respected my “NO”. And in the end left me alone absolutely none the worse for the wear. Had things gone differently I would have labeled this “The Second Incident” but in fact I now refer to it as “The Non-Incident”.

I told you in the last installment about my interview with the nursing supervisor and the way I felt disrespected and maneuvered against my will by an impatient nurse. I also spoke with this nursing supervisor about this non-incident with the physical therapist. First I said that I didn’t appreciate them calling in the therapist without at least discussing it with me or my family. We could’ve explained to them a number of things that would’ve made the therapy visit unnecessary and certainly less risky. I would have preferred to have a friend or family member present during such an encounter. So I was pretty firm with my displeasure on how the therapist got there in the first place. But I countered that with the highest praise I could muster for the way those therapists conducted themselves. I told her how scared I was and then how relieved I was when they listened to my “no” and respected my wishes. I asked her to please thank them for their professionalism.

Shortly thereafter, dad showed up with my iPhone and that turned be a real game changer in my ability to communicate with people. The challenges were still huge but at least we were putting a pretty good dent in those challenges. More on that in the next installment.

To be continued…

The First Incident

Fifth in a series. Click here for the first installment.

A Failure to Communicate

When I woke the next morning I was still intubated of course and on a ventilator. My initial reaction when waking up and finding myself in such condition is I often feel like the ventilator is breathing a little bit shallower than I would like. However this time it wasn’t as bad as my previous experiences and I was more ready for it. The first time I woke on a ventilator several years ago I felt like the settings were way too low and I had that “I can’t breathe” situation where that one respiratory therapist was the only one who understood my problem.

St. Vincent nursing staff does 12 hour shifts with a shift change at 7 AM and 7 PM. There was a new nurse in the room so it had to be after the 7 AM change. Also in the room was a young resident named Dr. Heaven although she pronounces it Haven. She’s kind of petite with her hair pulled back in a ponytail and kind of nerdy glasses. She somewhat resembled my maker friend Limor “LadyAda” Fried except the doctor didn’t have pink hair like LadyAda.

Before I had gone on the vent I had asked if they had some sort of message board that I could point to to communicate while on the ventilator. The new nurse had a piece of paper with the alphabet displayed in rows and columns and some other symbols and icons around the edge like daytime, nighttime, pain I forget what else. I had told the previous nurse that I would try to point to the message board with a straw in my mouth. So we attempted to do that.

Note: after I posted this blog earlier today I went through some old paperwork that I brought back with me from the hospital and found this original message board. There are penciled in sections that we added several days later so for purposes of this blog entry assume that the pencil writing wasn’t there. You can click on the image for a larger version.

Dr. Heaven held the paper up in front of my face and I tried to type out some messages. The first thing that I noticed was that it was difficult for me to point accurately because she was holding the paper too far away. I couldn’t get the far end of the straw to actually touch the page so it was really difficult to tell where I was pointing. The whole thing was just a regular 8.5″ x 11″ piece of paper held up landscape orientation. Each square containing a letter was probably at most a square 1 inch. Try pointing at that with a stick at a distance of even two or 3 inches and it’s difficult to precisely tell which letter you are pointing at.

One of the problems with even an accurate way of spelling out messages is that it’s hard for the recipient of the message to connect the letters into words. For example my Uncle Keith is severely hearing impaired and my Aunt Barbara is totally deaf. I learned the finger spelling alphabet at an early age and when I have a little more dexterity with my fingers I could spell words using finger spelling sign language. But I never could read sign language. Even if they spell a word slowly I could recognize each letter but somehow I always perceived it as individual letters in my brain. I couldn’t ever learn to form words. I always thought it gave me a little insight into what might be like to have dyslexia or some other learning disability.

When mom had been in intensive care for 19 days with her pancreas problems and finally was in the St. Elizabeth Seton specialty wing rehabilitating, she would try to use finger spell sign language to talk to me or dad. The only way we could do it is if I would read the letters out loud as she was spelling things and dad would write it down and then we would try to read it back by inserting spaces where we thought they might go.

People were having the same problem understanding me. By the time I pointed to one letter or another they had forgotten what I had already pointed to. They had to write it down as I typed it. I don’t really recall what I tried to type initially. But I was far enough into the process and realizing that I needed the paper to be held closer to me that I decided to forget about whatever else it was I wanted to say and to try to tell them to hold the paper closer.

The very first message that I successfully communicated to Dr. Heaven was “paper closer”. I don’t recall if she wrote it down or the nurse wrote it down or how they managed to get my sequence of letters to reveal those two words but I did successfully communicate those two words. Unfortunately the young resident was clueless as to what I was trying to communicate with those two words. She didn’t understand that I wanted her to hold the paper closer to me so that I could more accurately communicate. As I tried to type out another message… I don’t recall what it was I was trying to say… The way she was holding the paper was slowly drifting further away and making accurate typing even more difficult. If she had understood what I meant by “paper closer” the system might have worked.

At some point after Dr. Heaven left, I tried communicating with one of the nurses with her just pointing at the letters. I had already established with them that wiggling my eyebrows up and down was yes and twitching my mouth left and right was no. Somewhere along the way I also established that making a clicking sound with my tongue who get their attention. And I could use the clicking sound when they pointed to letters on the message board as if I was clicking on it with the mouse. Of course the tongue clicking originated with my late friend Christopher Lee who could not talk because of his severe cerebral palsy. I built the computer for him where he could click into a microphone and use it as a single switch scanning communication system. More on that later.

A Refusal to Communicate

While I could laugh in frustration at my inability to get the resident to understand what “paper closer” meant, there was another communication problem that was not the tiniest bit funny. The new nurse for this first dayshift was extremely impatient with the slowness of my communication.

She was also extremely insistent that she repositioned me as is their usual protocol. I fully understand why they have rules that you should turn the patient every few hours. For the most part it is to protect your skin from bedsores. On the other hand I know my body better than anyone else. Unlike many “quadriplegic” patients, my inability to move does not preclude me from feeling everything. My motor neurons are shot but my sensory nerves are completely intact. I know when I’m feeling pressure and what to do about it. For me if I lie flat on my back without my head elevated even the tiniest bit, all of the pressure equalizes over the widest area of my body arms and legs. If you try to jam a pillow under one side or the other, everything gets imbalanced and it actually creates pressure points. Of course it’s difficult to communicate that using slow typing message board and an inpatient nurse. The best I could do was when they would try to starts a pillow under me I would give them an emphatic “NO” signal.

Her only explanation in refusing to follow my “no” was to say “we have to”.


Timeout for a minute. When I type the word “REALLY?” like that, you need to read it in the same tone of voice used by Seth Meyers and Amy Poehler in their famous SNL sketch titled “Really?” If you’re not familiar with that sketch, stop reading right now and check out this YouTube video for an example. You really need to see this sketch if you’re going to appreciate the rest of this blog.


Do you think you could be more condescending? I recall numerous arguments between my mother and my sisters when they were little kids and they would give mom a very defiant “why?” whenever mom would insist upon something they didn’t want to do. Mom would reply to them “Because I’m the mom and what I say goes”. When this nurse said to me simply “We have to” all I could hear was my mother saying “Because I’m the mom”. Another resident or nurse I’m not sure which also gave me a similar condescending explanation. I only saw this other woman this one time but her explanation of why they had to stuff pillows under me was “We just have to. We wouldn’t be taking good care of you if we didn’t”. Did these people really think I had been in a wheelchair my entire life and didn’t understand skincare issues? I’ve been wrestling with this longer than they’ve been alive.

While I was 99.999% sure that this was totally motivated by my skincare concerns, there was a possibility that they were concerned about my lungs. If that was the case then I would put up with it. I would suffer discomfort and risk pressure points on my skin in order to help my lungs. On the other hand I had been in the ICU less than 24 hours and there was no way my skin was going to start breaking down that quickly. I wanted to stay comfortably flat without being twisted and propped into awkward positions for my own good.

I wanted to ask the nurse if her insistence on repositioning me was based on my skincare or my lungs. I tried to get her to hold up the communication board and she refused! What had been a frustrating situation about my comfort at suddenly turned into a battle of wills in which she could not only enforce her will upon me as if I were a little child, she now had a means to basically enforce a gag order on me by not allowing me to use the only method of communication available to me.

My St. Vincent Call Button

Next this inpatient nurse with whom I’ve been having a battle of wits noticed my special called button in my right hand. She picked it up, stared at it in amazement, and said “WHAT is this?” It is a rather bizarre looking thing. She commented that it had these strange metal prongs sticking out of it that look like they should be plugged into something. She basically ripped it out of my hand and laid aside and went about doing whatever she was doing.

Now not only was she refusing to communicate with me, she removed the only remaining method I had of communicating with anyone else which was to be able to press the call button. So for example if she left the room and I hit the button I might be able to get a more cooperative nurse to come. But now that option was unceremoniously deprived of me. Not to mention if there was something medically wrong with me and I actually needed a nurse I had no way to call.

What I thought was going to be “the last straw” was one final refusal of hers to communicate with me when she said “You need to stop trying to talk”.


There have been times when I tried to talk and I shouldn’t have. At 3 AM the previous night when they were trying to get the intubation tube up my nose and in my throat I was trying to ask what was going wrong and why was it taking so long. They told me don’t talk and that was a legitimate request. But this was in no way medically motivated. She sort of implied that I needed to calm down and shut up because trying to talk with getting me agitated. Talking wasn’t upsetting me… Her refusal to allow me to communicate was what was upsetting me. If she wanted me to calm down maybe she would treat me like an adult human being and listen to what the fuck I was trying to say to her.

I couldn’t do anything but just lay there and be pissed off. I kept thinking about how I’ve always been able to be such an active participant in my own health care. Dozens of nurses have praised me saying how much they enjoy having me for a patient because I can tell them exactly what I need. I can explain to them how to grab me, how to roll me, how to prop up a urinal between my legs so I can pee comfortably. By the way they all have great things to say about my skin. Even this horrible nurse who was so concerned about protecting me from bedsores at one point ended up saying what remarkable skin I have. When she told me what great skin I had I wanted to say to her “Then why the hell won’t you trust me to manage it?”

By the way I’m not in any way discounting the need to protect myself from bedsores. My mother had a really good friend who was a paraplegic that she worked with in various advocacy groups. He had pressure sores that went septic and it killed him and he was probably in his late 40s and otherwise medically stable besides his spinal cord injury. My cousin Nancy struggled with pressure sores on her feet that ended up with her needing amputation of one of her legs below the knee. So I take this stuff seriously. But I understand my own body. I’ve been living with this situation for 61+ years.

I’ve always been able to win these battles because I can communicate. I was laying there stewing over all of this and thinking things like “I’m not some Alzheimer’s or dementia patient or stroke victim who doesn’t know where they are or what they are doing. I’ve got an IQ well above 120 and only missed passing the Mensa exam by 2 percentage points. I know what the fuck I’m talking about. They just can’t talk down to me like this.”

So there I sat with nothing to do but think about “The Questionnaire” that I had been given less than 24 hours prior. It occurred to me that in the situation I was in now, I would actually answer some of those questions very differently.

I need to be 100% clear about what was really going on here. Not in this incident nor in another one I will describe later was I ever physically harmed. Although I was a bit emotionally shaken by the whole thing, I can’t really say that it rose to the level of verbal or emotional abuse. I suppose I would feel differently about it if I was in a permanent situation with a caregiver such as this and had to endure it every day. This was really a battle of wits. A kind of pissing contest in which I was getting both pissed off and pissed on. But there were serious principles at stake. I have the right to refuse any treatment whatsoever. There was no reason to shut off communication with me and in fact that cut off of communication put me at risk. It was clearly the first time in my life where I felt totally helpless and slightly endangered at the hands of a caregiver. It’s exactly the kind of thing the questionnaire was trying to uncover.

At some point they had let me know that they had called my dad that morning to let him know that I had been intubated. I doubt that it came to any surprise because I think we both anticipated it was going to happen. I was more surprised that they didn’t try to do it in the ER or shortly after I arrived in the ICU but as I explained this breathing difficulty would come and go in waves and every time he got bad it would clear up again. So in some respects dad wasn’t going to be surprised to see me on the vent because they had told him in advance.

When dad arrived he talked to this nasty nurse and she gave him a reasonable update about my condition. That I was doing okay on the vent. But at some point she says to him “He shouldn’t be talking right now.”







R E A L L Y !!!????

My Father walks in the door having recently learned I was intubated. He really has no idea what my medical condition is or isn’t, even though you’ve told him I’m doing relatively well.

And then you tell him that I shouldn’t be talking!


What on God’s green earth would motivate you to say that to my Father? This was the absolute last straw! What the fuck is he supposed to think now? Not only does it conjure up all sorts of medical complications that are nonexistent and probably leave him with 100 questions about why I shouldn’t be talking. Now she’s trying to impede my communication with the only person in the universe who can help me in this particular situation. It’s bad enough that she doesn’t have the time or the patience or the concern to talk to me. Now she’s trying to limit my ability to communicate with a man who loves me most of the world. He is the most absolute patient human being ever created by God Almighty. And he has nothing to do except sit and try to communicate with me. And she’s telling him that I shouldn’t be talking.


Sweetheart you had better hope I die before I regain the ability to talk. Because when I can talk. I’m going to have a fucking encyclopedia worth of things to say about you.

By the way I later asked my dad what he thought when she said I should not be talking. It really was of no concern. It was like… Well he’s got a tube in his throat. He can’t talk. So what’s the big deal? He did not take it to mean there should be no attempt to communicate with me at all. He took it to be literally “talking” which of course I couldn’t do. So my concern that it unnecessarily worried him turned out not to be true. It still doesn’t excuse the situation.

I Get My Chance to Communicate After All

Normally as I write these blogs I try to do them chronologically and I leave cliffhangers for dramatic effect as in the foreshadowing of both “The First Incident” and the fact that there is at least one other. But I’m going to jump to the end of this particular story and tell you how it resolved 2 days later.

This was the morning of Sunday, December 4th but on Tuesday, December 6th I was doing well enough that they removed the intubation tube and I could talk normally. And it so happened that a nursing supervisor stopped by sometime during the day and asked me how things were going. I had a similar supervisor visit during my hospitalization back in May when I had the G-tube placed and I was fighting a UTI. I’m really not a complainer. I don’t get pissed if the nurse doesn’t come within 30 seconds of me pushing the call button. I don’t care if my bath comes late. I’m a very patient patient. But when the supervisor asked me back in May if there was anything wrong way decided to tell her about a problem with respiratory therapist. The RT didn’t want to hook up the humidifier to my CPAP machine. In the end it was a good thing I did complaint. The respiratory therapist was wrong. I should’ve complained to someone the first day. So having that incident under my belt made it very easy to jump right in and tell the supervisor what had happened with the nurse refusing to communicate in treating me in such a condescending way.

Not that I would’ve needed the experience back in May to voice my opinion this time around. I would tell it stranger on the street about this. It reminds me of the joke about the guy who goes in to confession and tells the priest about all of the women that he’s had sex with. The priest says to him “My son you don’t sound like you have any regrets. Why are you confessing?” The guy replies “I’m not Catholic. I’m not here to confess. I’m just telling everybody!” So it’s a little bit disingenuous of me to say that the experience back in May made it easier for me to complain this time around.

So I told the nurse supervisor the whole thing in approximately the same amount of detail that I’ve related here. I made it clear to the woman that I had not been physically nor emotionally harmed. But that I did feel violated. That I did feel disrespected. That I did feel that I had clearly tried to exercise my right to refuse “treatment” and that those wishes had not been met. I told her that I respected their protocols about skincare and could understand why they would be insistent upon them but that I felt I had a legitimate reason to refuse. I gave her the “I’m not an Alzheimer’s or dementia patient I’ve got an IQ of blah blah blah blah” speech.

And then I made it clear to her that while I could possibly get over that disappointment of losing this little battle of wits. That this whole deal “You shouldn’t be talking” was way, way, way out of bounds since there was no medical reason whatsoever for it. And that the worst most unforgivable aspect of the entire incident was to say that to my Father again for no medical reason and at a time within minutes of him walking in the door and not really understanding my condition. That wasn’t a battle of wits or an argument over skincare protocol. That was medical misinformation delivered to my family member who is my designated medical representative.

Because it was two days later a lot of the fury, anger, hatred had faded but the determination to correct the situation of course had not and to some extent still hasn’t. This issue is going to become a crusade for me. This blog isn’t just to exercise my writing muscles and to entertain friends and family. This is a big issue and is going to be one that I continue to pursue as an advocate.

I told the supervisor that things got better later in the day and I may have had her as my daytime nurse the second day without further incident, But because things had gone a little smoother, I told the supervisor that I really didn’t want anyone reprimand or fired or punished. But we certainly have one hell of a teachable moment.

I don’t recall the name of this nurse supervisor but she listened intently. Understood everything I was saying. Agreed with pretty much everything I said that this should not have happened. And she promised me that it would be addressed. I never saw that condescending nurse again.

As I said… I’m jumping ahead a couple of days from the 4th to the 6th. There was another I guess we will call it “non-incident” on the 5th that I discussed with the supervisor on the 6th. I will go back and tell those stories in context later.

Just to reassure readers… “The Second Incident” which occurred on Friday, December 9th and which we will tell you about later, was again more a battle of wits than any physical or emotional abuse. It had some unique elements we will discuss. But after all of the cliffhangers, I just want to let everyone know by except for the 2 incidents, I had magnificent care from everyone both at St. Vincent 86th Street and at St. Vincent Seton Specialty Hospital to which I was later transferred. And that in neither of these incidents was I in any way physically harmed. I was made physically uncomfortable and angered by lack of respect for my wishes and self determination. But I never was really in harms way. And I would tell you if I had been.

I was able to use the communication board to talk to my dad and to the nurses later in the day. I instructed dad to bring my iPhone when he came back the next day. So beginning on December 5th I was able to use my iPhone and my ultimate remote control gadget to type messages to family, nurses and doctors. More on that in the future installments.

To be continued…

Top 10 News Stories in My Life for 2016

In this time of year all the news organizations are recapping the top 10 stories of 2016. I like to take this opportunity to recap the top 10 stories of my life. In any other year items like getting sued in federal district court for copyright infringement would be the number one item but not in this very busy and eventful year. It comes in further down the list. Actually in any other year any of the top four or even five of these stories could have been number one .

So here are the top 10 news stories in my life counting down from 10 to number one.

10. Major new connections on Facebook

From time to time I do Google searches or Facebook searches for long lost friends. 2016 yielded three major finds that I had been seeking for a long time. My old college buddy Frank Williams who is still playing keyboards in bars and nightclubs just like he was when I knew him 40 years ago at IUPUI. We haven’t gotten together in person yet but we’ve exchanged emails and follow each other on Facebook. Ellie Vinci also from my college days was the first girl who wasn’t in a wheelchair that I ever took on a date. Among other things we did back in the day was see Jaws, and go to a hockey game. She taught me an appreciation for violence on ice. My friendship with taught me the value of a strong nonromantic friendship with a female and became the foundation for many other similar relationships throughout my life. And finally Cheryl Fayette, my date for the senior prom and the first girl I ever kissed. Sadly my reconnection with her on Facebook was postmortem.

RIP: My First Kiss


9. Release My First Non-CGI Christmas Card

After making Ray Traced CGI Christmas cards using POV-Ray every year since 1995, this year I created a Christmas card that was a photograph of 3D printed statues re-creating a look of my 1995 CGI rendered card. This was a major accomplishment to get the POV-Ray models converted to something that could be 3D printed. It was the culmination of 18 months of work since I first got my 3D printer in the summer of 2015.

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


8. Released IRLib2 Software Library

Several years ago I developed a library of software for Arduino based microcontrollers that sends, receives, and decodes infrared signals such as those used in TV remotes. In 2016 I did a major rewrite to version 2 that took me many months to complete. The new software is much more flexible, expandable, and includes a new 117 page user’s manual. Released as open source for the world to use 100% free. I cannot begin to count the hours I spent on this project in 2015 and 2016.

Announcing the Release of IRLib2


7. Built The Ultimate Remote

Somewhat related to item 9 but actually a separate development project I built a new ultimate remote control that not only controls every TV and cable box in the house but uses Bluetooth connection to my new iPhone 6 that allows me to use iOS accessibility switch control features to have full control of the iPhone without using the touchscreen. Both the IR portion and the Bluetooth switch control of the iPhone by using three micro switches that I hold in my right hand. This is the latest in a long series of adaptive technologies I built using Arduino based microcontrollers and represents the most powerful device I’ve ever built in this category. This device was an essential part of my ability to communicate with doctors and nurses during my December 2016 hospital stay.

The Ultimate Remote Control and Why I Built It


6. Revolving Door of Home Health Aides

After my friend Rick Ruiz had to quit as my home health aide in November 2015 I’ve spent more than half of the time since then without a home health aide and when I did have them they did not last very long. We’ve had approximately 15 different people. Our longtime agency Acme Home Care could no longer supply a person and we moved to a new agency First Horizon where we are getting much better service. This is been a real strain on dad having to spend so much time taking care of me. However we have recently expanded from six days per week to a full 7 days per week.


5. Returned to Teaching RCIA

After teaching RCIA for more than 30 years I had retired because the program had been taken over by St. Michael’s parish. However when the RCIA program returned to St. Gabriel I decided to come out of retirement and teach again. I taught three lessons at the beginning of the year and it was wonderful to return to my most treasured ministry. I’m not sure if I will teach next year but it was good to be back one more time.


4. Sued for Copyright Infringement in Federal Court

Was threatened with a lawsuit for the use of a photo of the Indianapolis skyline as the background of my 2013 Christmas Card. Retained a lawyer but negotiations failed and the suit was ultimately filed in June of this year. After spending thousands of dollars in lawyers fees it was finally settled out of court. The details of the case yet to be blogged.


3.Stopped Eating and got a G-tube Placed

As my spinal muscular atrophy has progressed my ability to swallow has been severely compromised. I made the decision to stop eating altogether because it was becoming way too difficult. I had a surgical G-tube inserted in my stomach in May of this year. The procedure was supposed to be done outpatient however I was hospitalized for a UTI few days prior to the scheduled procedure so we did it while I was still an inpatient. I still eat occasional soft foods such as ice cream and cake for birthday parties and pumpkin pie for Thanksgiving. I continued to drink Coke and take most of my medications orally although that may be changing soon.

Tough to Swallow


2. New Wheelchair Finally Arrives

This April my new reclining power wheelchair with a mouth control joystick finally arrived after a nearly year-long process of evaluating equipment, going through paperwork, having an evaluation from a physical therapist and doctors. Even after the chair arrived we had much difficulty getting me able to operate it and had to build a new joystick mount using my 3D printer. Software glitches and mechanical problems also delayed my initial use of the wheelchair but since then it has operated well and I regained much of the independence I lost in late 2015 when I could no longer drive my old wheelchair.

My New Wheelchair Finally Arrives


1. While Hospitalized for Most of December with Tracheostomy Learned of New Treatment for SMA approved by FDA

A simple viral head cold led to a call to 911, an ambulance trip to St. Vincent ER, a tracheostomy and a lengthy wait in Seton Specialty Hospital to get a new in-home ventilator approved. This is the longest continuous hospitalization of my life. The adventure of not being able to communicate while intubated or on the ventilator led to a emotional roller coaster experience that has inspired me to write what may become my next published work after beginning with an extensive blog version. While recuperating in the hospital and waiting on the vent approval learned that the FDA has approved the new drug treatment for Spinal Muscular Atrophy. While primarily aimed at infant Type I patients it has also been approved for use by my Type II children and adults. Although I will not likely benefit from the treatment it is an encouraging sign that others may not have to endure much of what I have lived through for 61 years. The entire hospital experience also illustrated the depth of support and friendship that I have in the maker community which has gone way beyond what I ever expected. This entire month of December could easily be described as the top story of the past decade of my life. The full ramifications of which are not yet known. Extensive blog coverage of these events is still being written.

Pray That They Listen to the Man with No Voice


Well that’s it folks. The top 10 news items in the life of Chris Young for 2016. Let’s hope 2017 is a little more calm but just as interesting 🙂

The Intubation Crowd

Fourth in a series. Click here for the first installment.

I anticipated that I was settled in for the night. They seem to have finished poking and prodding at me and I’ve answered all the questions. The BiPAP was continuing to be a little bit out of phase but I was compensating best I could. However somewhere around 2 AM the “come-and-go” congestion got really worse. I told the nurse I wasn’t doing very well and she could see that for herself. It wasn’t long before I was in yet another of these very serious spells of respiratory distress. We made the decision that I needed to be intubated.

They called for the pulmonary doctor who happened to be one that was familiar to me I guy named Dr. Pfeiffer. He was a partner to my regular pulmonary doctor whose name is Dr. Vohra. Pfeiffer does all of the in-hospital work for that particular partnership. I had had him before as had my mother on many occasions years ago when she was battling lung cancer. He would not remember me or her given the number of patients he sees on a regular basis but I remember him and knew I was in good hands.

It took a while to get everything organized. Residents and interns started showing up. The nurse said to me “You are drawing a crowd”. It reminded me of a running joke I have with my friend Anne Chapman about all of the artistic skills we have based on things we can “draw” such as: I can draw a bath. I can draw a glass of water. I can draw the wrong conclusion. At the time it seemed to me the phrase “I can draw a crowd” might not ever have been on the list that Anne and I had compiled. However prior to writing this blog I dug out the old list and “draw a crowd” was indeed already on it.

Because my mouth doesn’t open very far there’s no way to intubate me using the normal method down throat. I always have to be intubated in my nose. I explained to them that the left nostril was the best one. I told them that occasionally they have used pediatric size tubing. They go in with a scope so that they can see what they’re doing. One of the first times I had to be nasally intubated they just jammed into me blindly and it went really badly. Fortunately the scope method is much smoother. They spray a bunch of medicine in your nose to attempt to numb it. It never does seem to completely numb the pain for example in the way that a shot of Novocain deadens your mouth for dental procedures. Some of it they squirt in and some of it they dab in on the end of a Q-tip. I say it doesn’t work very well but who knows… Maybe it would be much worse if they didn’t do it.

They had some Versed anesthetic available that makes you forget procedures such as this and some other kind of mild anesthetic ready to inject into the IV that have been placed in the back of my right hand. But they didn’t want to give me any of that until they actually had the tube inserted.

While we were waiting for Dr. Pfeiffer to arrive I had a conversation with the nurse and told her that I would raise my eyebrows up and down for yes and wiggle my mouth left to right to indicate no. I can’t really move my head up and down or side to side so that was the best I can do. I asked if they had some sort of chart or paper with the alphabet on it so that we can spell out words. She said that she believed they did have something like that available. Because I can’t point to such a chart with my hand, I suggested that they put a straw in my mouth, hold the chart up in front of me, and I would try to point to it with the straw. I had never actually attempted this but I had been thinking about it many times since the last time I had to be intubated. She said we would try it.

Dr. Pfeiffer finally arrived and began the procedure. He would get the tube just to the point where it felt like it was about to enter my windpipe but he would not put it all away in. I couldn’t figure out what was holding him up. Of course your gag reflex kicks in and the entire process is quite unpleasant. I knew I shouldn’t be talking but at times I tried to ask why isn’t it working? Sometimes I would get really distressed and couldn’t breathe I would try to mouth words like “I can’t breathe”.

I had an amazing sense of déjà vu because there were probably eight people standing around me working on me and some of them were trying to understand what I was saying to them but there it was one young guy standing in the back who was the only one out of the group that can either read my lips on makes sense of what I was trying to mumble at various times. I recall the first time I ever came out of anesthetic from my intestinal surgery and I was on the ventilator I tried to mouth the words “I can’t breathe”. A young male respiratory therapist on that particular day was the only one who understood me. I wonder is this young man was also a respiratory therapist.

In that previous incident years ago, I had the pleasure to talk to that guy later after I had the intubation tube out. I thanked him for being the only one in a group of supposedly more educated people who were standing around me clueless as to what was going on and that he was the one who understood me. Whoever this new young man was I hope to be able to later have a conversation with him and congratulate him for being the only one in the room who was aware of my needs. Spoiler alert… He was a medical student and I did have an opportunity to talk to him a few days later. We developed a great report and friendship throughout my stay in the ICU. We will have more about him later.

Somehow I became aware that the reason they were having such difficulty is that there was a huge mucous plug that he was having to suction out of the way before he could get the intubation tube into my lungs. The combination of that and my somewhat strange anatomy had made the whole process very difficult. Eventually the tube went in and the anesthetic followed and I was out for the night.

To be continued…Not in the next post but in the following post titled “The First Incident

The Questionnaire

Third in a series. Click here for the first installment.

We come now to “The Questionnaire”. No I’m not talking about the one that James Lipton uses in “Inside the Actors Studio” that he famously credits Bernard Pivot from his French talk shows Apostrophes and Bouillon de culture. This was a questionnaire I’d never heard before.

Alone with the nurse in ICU room 2615 she began to ask me a series of questions that she said were standard questions asked to incoming patients. Although I had been in St. Vincent Hospital a number of times, I don’t recall being asked these particular questions before. I can’t quote exactly each of the questions. There were perhaps 10 of them. At first I thought it was measuring my general emotional state. I thought they were standard psychological questions about how you feel about yourself and your situation. Are you depressed? etc. However that isn’t exactly what they were going for. They wanted to know if I felt safe in my home environment at the hands of family members and caregivers. The questionnaire I was receiving was designed to root out patient abuse.

I could be philosophical and analytical about being asked the question because they were so far outside my experience I didn’t have to stop and think about the answers. I’ve never felt at risk for abuse from anyone. We probably had 20 or 30 home health aides in the nearly 8 years that we been getting help and they’ve all been great people except for one guy who stole over $100 from my wallet. Even though he ripped me off, physically he was a good caregiver. Of course he didn’t want to call any attention to himself so he was going to behave as a perfect gentleman and a good friend to hide the fact that he was a fucking thief.

The one particular question that I do recall was “How are arguments resolved in your home?” I sort of chuckled to let them know I was joking and I said “I always win”. I wanted to reassure them that no one was acting on me against my will. I told Carol later I was being so emphatic about how I was in charge it was more likely they were going to arrest me for abusing dad then do anything to him for abusing me 🙂

I remember thinking it was a shame that they had to ask such questions but it made me feel good for the people who needed to answer those much differently than I did. Who knows it might just save someone from a very difficult situation. At least I hope that it would. Again this was so far beyond my experience that I didn’t know what was like to feel unsafe at the hands of a caregiver.

The huge irony behind all of this was that I’ve been dependent on other people for absolutely everything for over 61 years, would never have answered those questions in a way that would indicate that I had ever felt vulnerable or at risk, and less than 24 hours later had they asked me the same questions I would’ve had to answer differently. In the very room in which those questions were asked, I found myself in a situation where I felt helpless and at the mercy of a caregiver who was ignoring my explicitly stated wishes for no medically necessary reason whatsoever. We will describe what happened in a future blog post titled “The First Incident”.

To be continued in the next post…


I was going to end this blog post right here but I can’t resist the temptation to fill out James Lipton’s questionnaire so here goes…

Q. What’s your favorite word?

A. Understand


Q. What’s your least favorite word?

A. Hypocrisy


Q. What turns you on?

A. There’s this spot on my… No we’re not going there… okay let’s see… “Explaining things”


Q. What turns you off?

A. Duplicitousness


Q. What sound or noise do you love?

A. The sound of 33 IndyCars or 43 NASCAR Cup Cars going by a fence 20 feet in front of you on an opening lap.


Q. What sound or noise do you hate?

A. After a serious accident at the Indianapolis Motor Speedway when you suspect that a driver has been killed in an accident, the PA announcer (either the late Tom Carnegie or the current chief announcer Dave Calabro) comes on and says “Ladies and gentlemen may I have your attention please?” Hundreds of thousands of people go immediately silent knowing what they are about to hear next. The track announcer never asks for their attention in that way unless it is the ultimate bad news. He then goes on to say that he is sorry to report that the driver in a recent incident has succumbed to his injuries. It’s that literally deathly silence after the initial announcement and before he actually delivers the news. That’s the sound or noise that I hate. I’ve been there twice at such times.


Q. What profession other than your own would you like to attempt?

A. Although it’s totally beyond my physical capability my fantasy job would be a keyboard player in a rock band similar to Keith Emerson.


Q. What profession would you not like to do?

A. At one point before I discovered computing, I thought perhaps I would go to law school but in retrospect I realize it would’ve been a terrible mistake. I could never have a passion for “The Law” that exceeded “doing what’s right” but that’s what lawyers have to do all the time. They have to uphold the law even when it doesn’t always lead to the truth or to justice. I could not do that.


Q. Lipton asks “If heaven exists what would you like to hear God say when you arrive at the pearly gates?”

A. First of all my fear is he would say “Chris we have this computer virus we’d like you to take a look at.” But what I hope he says is “Well done.” Either that or “Go back… You’ve still got work to do.”

Q. Note however the original version from Pivot which was feared offensive to American audiences was “”If God exists, what would you like Him to tell you when you’re dead?”

A. I would say “Thanks for everything.”

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

Note: To those of you who have heard this story before, please keep reading to the end. There is a new ending and I don’t want to give it away. Please keep reading.

Approximately 40 years ago I applied for a job as a computer programmer at the Indiana University Department of Medical Genetics which in those days was housed in the research wing of Riley Hospital for Children. My mentor and computer professor Dr. John Gersting was a consultant to the project and helped me secure the job despite his insistence that he would not help and I had to earn it on my own. He attended the job interview which was conducted by the department chairman Dr. Donald Merritt MD, PhD.

To put it mildly, Dr. Merritt was an asshole. Somewhere during the course of the interview Merritt and my mentor Gersting exchange some snarky joke with one another and Merritt flipped the bird at Gersting. Dr. Gersting looked at him as if to say “Why the fuck would you behave in such a childish manner in front of one of my pet students? Are you really so inarticulate that you have to stoop to flipping me off in front of him?” But in fact he said nothing. He didn’t need to.

At one point during the job interview Dr. Merritt asked me “What’s your diagnosis?”

I didn’t understand. I thought he wanted me to diagnose something. He rephrased the question “With what were you diagnosed? What you got? Why are you in the wheelchair?”

“Oh now I understand” I replied. “It’s some kind of muscular dystrophy or genetic neuromuscular disease. As you well know there are a dozen or so different varieties. I’ve never bothered to figure out which one I have. It’s not Duchenne muscular dystrophy obviously. There is genetic history in my family. The people here at Riley described it as ‘amytonia congenita’ but that’s not really a diagnosis it’s more description of my symptoms.” That particular Latin phrase means congenital (since birth) low muscle tone. I always said it was like they took me into the doctors and said “hey this kid has had weak muscles since birth” and the doctor said “We’ve got a name for that… amytonia congenita” and we said “What’s that mean?” and they said “it’s Latin for this kid has had weak muscles since birth”. It sounds so much more formal in Latin it doesn’t it?

“But don’t you want to know?” He inquired.

“Well Doc… When you can tell me which of those dozen or so types of muscular dystrophy you can do anything to help… Then I’ll worry about whether or not I’ve got that one.”

He raised his eyebrows as if to say “okay you got me there.” He went on to explain there were other reasons one might want to know one’s diagnosis. If I have siblings they might want to know if they were at risk to bear children with my disability etc. I told him we would deal with that at the proper time.

He went on to explain very matter of fact that his true purpose was to see if I was comfortable discussing my own disability. He had experienced people who were shy about discussing such issues and considering the way I threw it back in his face I had alleviated any concerns about my shyness or timidity on the topic. The interview went on and I got the job. I worked there for two years until my disability brought on congestive heart failure and other issues that made it no longer possible for me to work a full-time job.

Approximately 25 years later while doing some Google searches about medical conditions for a friend, I decided to look up “amytonia congenita”. Among the websites found were my own blog where I explained my connection to the phrase. Another was a quote from a book jacket on for an autobiography titled “The Me in the Mirror” by Connie Panzarino. It said that the author had “Spinal Muscular Atrophy Type II, formerly known as amytonia congenita”. That peaked my interest. Whatever the hell “Spinal Muscular Atrophy Type II” was… It sounded much more like a real diagnosis than a symptom description like amytonia congenita.

I continued searching about Spinal Muscular Atrophy or SMA as it was abbreviated and as I read the descriptions of patients many in their own words, it sounded as though they could’ve been my twins. The course of their disease sounded identical to my own experiences.

One of the things that had made me reluctant to determine what type of muscular dystrophy I had was that the diagnosis process generally involves things like electrical stimulations of muscles and muscle biopsies neither of which sounded very unpleasant simply to answer a question that yielded no useful answers. However SMA was known to be caused by a missing chunk of DNA in my number 5 chromosome. Specifically it is missing from a gene known as the SMN1 gene. Genes are strands of DNA which create specific proteins. They are divided into coded sections called axions and non-coded sections called interons. The seventh axion of the SMN1 is missing in patients with SMA. It’s like you cut the tape and spliced it back together again. All they had to do was take a DNA sample in the form of a blood sample. They would send it off to a lab and sequence that gene and see if than chunk was missing.

Everyone has two copies of each chromosome with one coming from their mother and the other from their father. If you have a missing chunk from one parent but not the other you are a carrier but do not exhibit the disease. If both parents give you DNA with a missing chunk (a 1 out of 4 chance) then you hit the DNA lottery and end up with SMA like me. Whoops… Spoilers… The DNA test came back saying indeed I did have a missing 7 axion from both copies of my 5 chromosome.

Where did I go to get the test done? I went back to the Indiana University Department of Medical Genetics where I had worked as a computer programmer 25 years earlier. Dr. Merritt had died of skin cancer shortly after that job interview. After I left the department it moved to new buildings at the IU Medical Center. But some of the people who were there when I was were still working there and they had a vague recollections of the guy in the wheelchair who worked on the “Mega-dats” computer database all those years ago.

One thing that did not change over those 25 years. There still wasn’t anything they could do for it. But it was fascinating to know they really did know what was wrong with me. In the years since my diagnosis I have followed the course of the research. For example they have developed a strain of lab rats that have the disease which is useful. They’ve come up with a variety of strategies for possibly treating the disease.

I mentioned that the problem is a missing chunk from SMN1 gene which encodes something called the SMN protein the lack of which causes my disease. I can go into more detail on how it works but that’s unimportant for the current discussion. Everyone, whether they have SMA or not also has in their 5 chromosome something called the SMN2 gene sometimes called a backup gene that should kick in if there’s something wrong with SMN1. The problem is everyone’s SMN2 gene has a glitch of its own. Just one base pair in the strand of DNA (I forget whether it’s a AG or a CT that is swapped) is wrong. Think of it as a one bit data error in a strand of data thousands of bits long. That one bit error means that the SMN2 gene only creates the proper protein about 20% of the time. The other 80% create some worthless molecule that does nothing useful.

One of the strategies has been to get SMN2 to turn itself on five times more than usual. That way when it only works 20% of the time you get 5×20% equals 100% of what’s needed. The other strategy is to introduce a drug that increases that percentage so that it works more often. It doesn’t correct the bad bit of data but it causes the protein to get properly manufactured despite the flipped bit. That strategy is one employeed by a drug known as “Spinraza”. But more on that later…

I said that I have SMA Type II. In my particular case I never walked as an infant and the disease has progressed relatively slowly throughout my 61+ years on the planet. A more severe and more common version is SMA Type I in which infants are born severely weakened and rarely survive more than a few months. It is the number one genetic killer of any disease known. There is also an SMA Type III which has a later onset than mine and therefore consequently less severe. The goal of course of most treatment strategies is to try to get those Type I babies to survive more than a few months. A successful treatment is easy to measure because they wouldn’t die. Measuring success in Type II or III is more difficult because the disease progresses more slowly.

Okay… I can’t stand it anymore.

I have buried the lead of the story as deep as I can.

Today, December 23, 2016, the FDA has approved the use of the drug Spinraza for the treatment of Spinal Muscular Atrophy Type I, II and III. In clinical trials with a double-blind study of 121 patients with SMA Type I, results showed that 40% of the patients receiving the drug showed measurable efficacy measured by a variety of standards including extended survival while those receiving a placebo had no benefit. The drug is so effective that all participants are now receiving the actual drug. It is been fast tracked and approved for all types of SMA including mine.

The treatment involves injection into the spinal fluid. I’m not sure how often such injections are required. If you can do it on their program they will cover the cost of the drug. This is also hot off the press there isn’t much information available right now.

Obviously for someone like me who has endured the effects of the disease for 61 years even if this was an all-out cure (and no one is calling it that… They are calling it a treatment) nothing is going to reverse the orthopedic effects of contracted joints, severe scoliosis, muscle deterioration, nerve damage etc. that can come with the course the disease throughout my life. I’ve always known that as I followed the research. To the parents of those Type I babies however it’s the miracle they’ve all been praying for.

But finally Dr. Merritt, wherever you are, they can do something about one of those types of muscular dystrophy. And guess what… It’s the one I got!

And I discovered the news by a Facebook post from while sitting alone in room 207 of St. Vincent Seton Specialty Hospital two days before Christmas while waiting on bureaucracy to get me a ventilator so I can go home.

Here are two articles with details on the above story.

FDA Approves Spinraza for SMA

FDA approves first drug for spinal muscular atrophy

This Ambulance Sucks… Not!

Second in a series. Click here for the first installment.

This health crisis began with an ordinary viral head cold. I first noticed it the evening of Thursday, December 1. It was a scratchy feeling in the back of my throat and the soft palate on the right side of my throat. Throughout the night and into the next day I could feel it migrate from one side of my throat to the other and then up into my sinuses. Usually I’m very aggressive in treating any kind of cold because my lungs are easily susceptible to pneumonia. For most people if it’s obviously a virus, doctors recommend you do not use antibiotics because over prescription can be dangerous. But my doctor has always made it clear we need to be aggressive in preventing pneumonia.

In this particular instance I really didn’t think it was necessary. I didn’t feel any congestion in my lungs at all. I had no sign of a fever. It just didn’t seem necessary to treat it at this point. I have some antibiotics that I keep on hand. I keep them handy in case of one of my recurring urinary infections pops up on a weekend when I can’t get to the doctor. I knew that I had that option as well as an immediate care facility in Speedway that I had used on weekends on a couple of other occasions. So I just wasn’t very worried about the whole thing.

Friday afternoon the drainage from my sinuses was getting a little bit excessive and I was also having some unrelated intestinal issues so I decided to go to bed in the middle of the afternoon to take care of my breathing and to be ready to use the bedpan at a moments notice. I wasn’t having any difficulty breathing. I did not put on my CPAP or oxygen until my usual bedtime around 11 PM. But for some reason I just couldn’t sleep Friday night. That happens occasionally and I don’t know why. Sometimes my brain gets to moving so fast I just can’t turn it off at night. I was wide awake until the early hours of the morning so I told my dad to cancel the home health aide who was scheduled to come in at 10 AM. I finally got to sleep around 6 AM and slept until nearly 1 PM Saturday afternoon.

Dad got me up and dressed and I started working on preparing the message for the inside of my Christmas cards. I had already had the outside of the cards printed at and I typically print custom inside messages on my laser printer. I also did some tinkering with my 3D printer making some little gifts I planned to give for Christmas.

The drainage from my sinuses started to make me feel a little congested in the throat. Because I use voice control on my computer I decided I needed to stop talking and relax a little bit. I went in the living room and turned on the TV and reclined my wheelchair slightly to help ease my breathing. That usually does the trick whenever I start to feel congested. The reclining wheelchair is new since April. Reclining has always helped in the past but this time it wasn’t working.

Dad was getting ready to cook himself some supper but I interrupted him and said I needed to lay down. Laying flat on my back always helps my breathing. That way I’m not fighting gravity to try to cough up any congestion. I could tell immediately however that this wasn’t helping as much as usual. I put on my CPAP as soon as he got me undressed. I rarely ever do that under such circumstances but I really felt I needed it. It was like my windpipe or bronchial tubes were having some sort of spasms and trying to close up. I was having difficulty getting my lungs to inflate and I was sure that the CPAP would do the trick

The breathing difficulty would come and go in spurts. Sometimes it felt like I was breathing fine. Other times it felt like everything was closing up and spasming. This was definitely an unusual experience. I finally called dad again interrupting his dinner and saying we needed to call 911. I simply could not get my lungs inflated properly.

Firefighter paramedics arrived first and the ambulance shortly thereafter. They picked me up by grabbing the contour sheet off my bed with me wrapped up in it and transferred me to the gurney. They put a mask over my nose and started blowing a high rate of oxygen into me very hard. Much harder than my CPAP pressure. It was still barely enough to keep me breathing well. My oxygenation level was good but I was panting and only breathing with a small portion of my upper lungs.

We headed off to St. Vincent at non-emergency speed which was okay. I was getting by okay and I had the ambulance EMT there if things got worse. Dad would follow in our van and meet me there.

Because of my usual swallowing difficulty I needed to spit the congestion and ordinary saliva from my mouth. I asked the EMT if he had a suction tube and he reached over to the wall and grabbed a Yankauer. He couldn’t get the suction to work. He reached to the other side of the ambulance for another device and it didn’t work either. He said the ambulance was brand-new and they had probably never used the suction devices. Somebody had failed to test them. He had a portable suctioning device that he quickly hooked up and was able to clear my mouth.

There is a ground-level entrance to St. Vincent ER where anyone can drive up and unload but ambulances go down a steep ramp to an underground ambulance bay that is great because it allows you to unload despite the weather. Once inside have to go up elevator to get to the ER.

St. Vincent ER is the only ER I’ve ever been in. They put you in a small enclosed room that looks nothing like the ER facilities you see on TV or movies. On TV it’s always a big open bay with nothing but a curtain between patients or the exam rooms have lots of windows that allow patients to see through to the next bay which allows for all sorts of dramatic TV interactions. I always wondered if other real ERs had that open layout like they do on TV or if that was just to make the stories more interesting or easier to tell. An ER TV show or movie shot in a room like St. Vincent’s ER certainly would not be as interesting. Then again this was not a trauma room so that might be different.

I don’t remember a lot of details of what we did in the ER that night. They put me on a BiPAP machine which is sort of like the CPAP that I use to help me sleep. CPAP provides constant positive air pressure hence the name. A BiPAP is a bilevel CPAP that supposedly senses when you are trying to breathe in and out. He gives you high-pressure on inhale and then lowers the pressure on exhale. For some reason that never works very well for me. I like to let the high-pressure fill my lungs and then squeeze out the air against that pressure so that my lungs deflate slowly. When wearing a BiPAP, the pressure drops quickly and I exhale too quickly. On this particular occasion I was having so much difficulty inhaling it seemed like the BiPAP was not sensing that I was attempting to inhale. That meant it wouldn’t kick in that high-pressure like I needed to do.

Dad had brought my CPAP and the mask that I use at some point, I don’t recall if it was in the ER or he was in the ICU later, but I got them to use my nasal pillow mask rather than the full face mask they had been using. That helped a little bit but not much. As I mentioned before my respiratory distress seemed to come and go in waves. Sometimes I could breathe almost normally. Other times I was in serious distress.

At one point they took a chest x-ray and said that I had no sign of pneumonia. That surprised me considering how much difficulty I was having breathing. But really it did not feel like congestion as much as it did spasms in my windpipe and bronchial tubes. When I would try to inhale I would make a loud rasping sound. It wasn’t until a couple of days later that we discovered I was having some sort of vocal cord dysfunction. My vocal cords were slamming shut and that was restricting my breathing.

I seem to recall being transferred to the ICU sometime around 11 or midnight. Dad had brought not only my CPAP and mask but my special nurse call button that I’ve been using for many years. I’m estimating it was perhaps 30 years ago I was at St. Vincent and could not operate their call button. The biomedical engineer built one for me after I told him the type of microswitch that I could operate. He just told us when you leave the hospital, take it with you and anytime you come back bring it again. We been doing that nearly 30 years now. More on that later.

Because my primary doctor is part of the St. Vincent network and because I been in St. Vincent Hospital on several occasions they already have my medical history and all the necessary information so there wasn’t the usual long patient history to be taken. Dad left and most of the doctors wandered out it was just me and my primary nurse in the room. It was at that point that she told me we went through the questionnaire. A series of questions I had never been asked before.

To be continued not in the next post but in this following one