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

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

Really?

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

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

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

What is Spinal Muscular Atrophy – SMA?

SMA is a genetic disease. Both my mom and dad were carriers and did not know it. Approximately one in 400 people are carriers. Even if both parents are carriers there is only a one in four chance that each child would have the disease. Of course that’s just a percentage over the long haul. You can flip a coin 10 times, have it always come up heads and the next one is still 50/50.

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

I have Type 2 which is not as severe but it generally means that I never walked (which I did not). Type 3 people can generally walk until their early teens sometimes later. There is a type 4 which actually is a significantly different disease but is still called SMA and I don’t know much about it. Even within the types there is a great variation. On various Facebook groups related to SMA I hear people describe themselves as “a strong Type 2” or “a weak Type 2”. I have a Facebook friend who lives in Canada and we are both Type 2 but her current physical ability at age 45 is about where I was at 21 or 22.

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

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

What Went Wrong?

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

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

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

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

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

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

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

How Does the Inheritance Work?

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

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

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

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

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

But what about me?” Pardon me quoting the old joke “I have no children… That I know of :-)” and at age 63 with no romantic involvement in my life I doubt that I will. But there’s no reason I couldn’t have children. What about them? People who have the disease of course pass it on to their offspring and so technically they are carriers but usually we reserved the word “carrier” for someone who carries the gene but does not exhibit the disease. But anybody with the disease does automatically pass it on. I’ve got two bad copies of the gene so at minimum, all of my kids would be carriers. If my wife was not a carrier then that’s all the risk we would have. If she was a carrier, half of our kids would have SMA and anyone who did not have it would be a carrier. If my wife had SMA, we would both have 2 bad copies and therefore ALL of our children would have two bad copies and have SMA.

How Do You Test for SMA?

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

How to Cure SMA

Short version… Become a Nazi.

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

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

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

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

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

Pardon me if I take that personally.

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

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

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

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

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

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

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

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

Life finds a way.

How Do You Treat SMA?

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

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

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

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

Pray That They Listen to the Man with No Voice

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

Tough to Swallow

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

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

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

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

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

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

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

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

The Cost of Life-Saving Drugs

How does Spinraza Work?

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

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

What Else Can Be Done?

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

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

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

What Do You Want to Know?

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

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

More Resources

  • Cure SMA -– Charitable organization that funds research and supports families
  • Biogen — Markets SMA treatment Spinraza
  • Spinraza — Treatment marketed by Biogen
  • SMA News Today — Website with SMA news and information.
  • Invitae Corporation — Free genetic testing for SMA

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

The Cost of Life-Saving Drugs

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

Christmas Gifts — Holiday Hospital Part 8

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

Big Surprise

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

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

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

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

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

Christmas Eve

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

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

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

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

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

Christmas Day

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

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

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

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

After Christmas

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

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

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

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

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

A Dry Heat — Holiday Hospital Part 5

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

Vertical at Last

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

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

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

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

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

As Dry as Arrakis

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

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

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

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

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

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

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

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

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

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

Skills Lab

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

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

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

Miscellaneous Events

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

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

An Uneventful Weekend — Holiday Hospital Part 4

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

Recap

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

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

An Uneventful Weekend

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

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

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

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

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

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

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

About Ableism and Other Imposition of Worldviews

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

Afterward

This note is added April 14, 2018.

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

A Personal Reflection on My Role Model Stephen Hawking

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

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

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

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

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

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

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

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

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

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

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

Nobody could tell me.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

Links of interest.

Having a Prophet for a Friend

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

Where did we leave off?

It’s been quite a while since my previous installment because I had a major computer crash that took a week or so to recover. Then I got distracted with other things. We are now up to Friday, December 16 which is the first weekend that I spent in Seton Specialty Hospital. I had a bit of a rough night the night before. Here was my early morning Facebook post at 8:17 a.m.

The plans for Friday were to try to get me sitting up in the wheelchair. Dad brought my wheelchair with him that morning because the physical therapist said I should be getting up. It turns out the therapists were pretty busy that day and they decided they couldn’t help. Dad didn’t really need much of any help getting me dressed and in the chair except that we didn’t have any experience operating their patient lift equipment. As it turned out since I had had a kind of rough night the night before I decided to just stay in bed and we would get me in the wheelchair on Monday when the therapists could help us. Here is another Facebook post from later in the day at 5:06 p.m.

My friends Rich and Kathy Logan were planning to come by to visit me that evening. I had Rich bring me a print out of my communication board because I had a quick update I wanted to get and I didn’t want to wait until dad came the next day. But before they came, I kept myself busy throughout the day working on a 3D printing project.

Remote 3D Printing

Although my specialized nurse call button was working okay at the new hospital, my hand holding that button wasn’t doing as well. At one point a few years ago I thought I had glued a metal ring on the side of it that I would stick my finger into that would help me hold that in the proper position. Somewhere along the way I lost the metal ring. It’s extremely difficult to get the button positioned exactly the way I needed it. Anytime someone moved me or had to take the button away and put it back it was always difficult to get it put back in exactly the right position. Rather than trying to have my dad come up with a new piece of metal to glue onto the side of the switch, it was going to be easier to design and 3D print a new piece.

My St. Vincent Call Button

I do all my 3D modeling in a program called Blender 3D. That program isn’t specifically designed for 3D printing. It’s used mostly for rendering and creating video games and doing photorealistic 3D models similar to what I create in POV-Ray. It’s free and open source and rather than learn one CAD program for rendering and another one for 3D printing I decided to just stick with Blender bback when I first got my 3D printer. So I downloaded a copy onto my laptop and begin designing a little ring that would fit on the side of the nurse call button. Once I had a prototype designed, I transferred it from my laptop to my desktop at home using Team Viewer file transfer features. I told dad when he got home that he should turn on the 3D printer. I would type messages to him on the screen of my desktop and I would initiate the 3D print. I have a WebCam pointed at the 3D printer so I could remotely monitor the progress of the print.

For many months I’ve had difficulty getting parts to reliably stick to the build plate. However just prior to going to the hospital I had installed a new Zebra Plate build plate on the printer that had been working wonderfully. It worked beautifully again when I was printing the part. I printed the part Friday night and dad brought it in Saturday morning. It turned out that I needed to put a little twist in the model so that the switch would be in the proper position. I actually printed 2 different versions at different angles but even the one with the greatest twist wasn’t quite enough. I printed a third version Saturday night and dad brought it in Sunday morning. The final version worked just fine. Here is a screen grab showing the part that I modeled in Blender 3D. It illustrates 2 different versions with a slightly different angle on the slot that fits onto the original switch.

Here are some photos of the final part attached to my nurse call button. The 3D printed part is in green plastic.


The use of a closed ring to put my finger through worked so well that when I got home I redesigned the way I use my pushbutton switches on my iPhone and my bedroom IR remote/alarm button. Prior to this, I had a 3D printed part that help to position the buttons my hand but they were just a couple notches to position my fingers. After my experience with the closed ring, I redid the part on my iPhone and remote switches to completely enclose one of the rings around my finger. I tried using 2 rings. I tried the ring around the index finger. But ultimately it works best with only one ring around my middle finger and just a notch for my index finger. Here are some photos that show the old and new versions of that switch. And some photos of it in use. A friend of mine from Facebook once described this as a Doctor Strange “sling ring”. It really made me mad when he said that. I wasn’t offended. I was mad that I didn’t think of the name first 🙂


Although this revised system worked fairly well throughout the rest of my stay at Seton, the way that the wire comes off of the switch would often get in the way of my wrist or my forearm. After I got home from the hospital I decided I would take the switch apart. And I would change the way the wire extends from the switch. I would redesign the ring. As I am writing this blog I’m also working on a new 3D printed part for the hospital call button. It will look more like the ones I use on my remote at home in that it will have a middle finger ring and a first finger notch. As I mentioned above, I could not hold both the call button and my remote buttons at the same time. Ultimately I hope to be able to resolve that problem as well for the next time I am in the hospital.

Having a Prophet for a Friend

That evening I was visited by my great friends Rich and Kathy Logan. I’ve known them since my college days and I was the best man at their wedding years ago. I took the opportunity to tell the story of everything that had happened while I was in St. Vincent’s Hospital getting the trach. They had visited me at St. Vincent but I was unable to talk during those visits so I had lots of stories to tell them. Basically they got a condensed version of my entire 14 part blog where I wrote about the St. Vincent stay.

When you lay around the hospital for days and you contemplate the friendships you have and legacy that you have built, you tend to think of things that had been left unsaid. While there really wasn’t anything significant unsaid between me and the Logans, there was one story I have been meaning to tell them for a long time and had not gotten around to it.

When I teach religion classes for the RCIA program at St. Gabriel, I teach a class about the Old Testament prophets. Normally you think of a prophet is a person who predicts the future. But that really isn’t the primary role of a prophet. Prophets are actually spokespersons for God. I described them as similar to the White House Press Secretary. Of course I used to use that example when we had decent people in that job. I would hate to equate a prophet of God with someone like Sean Spicer 🙂 Instead think of C.J.Cregg from The West Wing. But not only are prophets spokespersons for God, specifically their role is to call us back to God when we go astray. To remind us of our core principles when we tend to forget them ourselves. Typically a prophet says if you don’t shape up, things will go bad for you and if you do shape up things will go well for you. Whichever happens… their prophecy tends to come true.

As examples in my class I cite people like Abraham Lincoln whose Gettysburg address was a prophetic message that reminded us what we were fighting for in the Civil War. I also include people like JFK with his “Ask not what your country can do for you… Ask what you can do for your country”. Similarly prophets are dreamers. People who dream about what the world would be like if we would stick to our core principles. The primary example of course is Martin Luther King Jr. and his “I have a dream” address.

But I also give biblical examples of prophets who are personal friends. My favorite story is the story of Nathan who is a prophet to King David and who called him out when he killed one of his generals Uriah to cover up the fact that he was sleeping with Uriah’s wife Bathsheba. I tell the class sometimes our friends are prophets for us who call us back to be the best versions of ourselves. To be the people that they know we can be. To be the people who made them want to be our friends in the first place. I explained that true friends are the people who can lovingly look us in the eye and say “You fucked up but I love you anyway. Now get your god damn shit together.”

Of course I don’t use that kind of language teaching the class… But you get the point.

So I told Rich and Kathy that when I teach that part of the class, they are among the friends I think of who are my prophets and to whom occasionally I have been a prophet. I explained that what I cherish most about our friendship was our ability to lovingly point out when we were being a real dick about something. I also confessed to them that I had not always exercised my role as a prophet to them out of the purest of motives. Sometimes I had been a bit self-serving in my role as a prophet and that was something which I regretted.

It wasn’t exactly a deathbed confession and I made it clear that it wasn’t. But it was nice to take the opportunity to thank them for being prophets for me and to say that I haven’t always been the purest of prophet for them even though I tried to be.

There is a parable about a guy who goes to heaven and asked Jesus “What in your opinion was the worst sin that I ever confessed?” Jesus replied “Once you had confessed your sin, I didn’t remember it anymore. So I can’t answer that.” Like the true friends that they are, the Logan’s didn’t recall the incidents that I had recounted until I recounted them.

Scripture says “Faithful friends are life-saving medicine…” Sirach 6:16

I have no doubt that they and other faithful friends had been life-saving medicine for me.

Hard Questions about Treating SMA

As I reported in this blog last December, the FDA has approved a new treatment for Spinal Muscular Atrophy or SMA. This is the genetic neuromuscular disease which I have. My particular variety would be considered Type 2. I never walked as an infant but the disease has progressed quite slowly and as I’m approaching my 63rd birthday I’m still alive. The most severe Type 1 makes infants so weak that their life expectancy without extreme measures is a year or two. Some children are living as much as three or four years using trachs, ventilators, and other extensive medical support systems.

The new drug which was approved was only tested on the most severe Type 1 individuals but has been approved for Type 2 and Type 3 as well. The drug is administered by a spinal injection and is extremely expensive costing up to $500,000 the first year and perhaps $300,000 each year thereafter. The disease is caused by a failure to create a necessary protein called the SMN protein. The lack of this protein causes a deterioration in motor neuron cells called anterior horn cells. One of the big questions I’ve had about the new drug is that if these motor neuron cells have completely deteriorated, it would seem to me the most is that any drug therapy could possibly accomplish is to maintain current health levels and not actually reverse the disease. Yet the initial clinical trials of these Type 1 patients have showed not just stabilization but actual improvement.

The big question is what about older patients. I’m not just talking someone in their 60s like myself but let’s suppose you are in your 20s. If you been living with the disease not long, one would expect that these motor neurons would be completely gone. This treatment is not going to regenerate those neurons. Could you really expect any actual improvement or are we just talking stabilization. Also given that the drug is administered by spinal injection and adult patients either have severe scoliosis like I do or they’ve had spinal fusion surgery (which I probably should have had years ago). Either of which make administering the drug more difficult.

It’s one thing to administer an extremely expensive drug to a Type 1 patient for which the disease is most certainly fatal and it’s something else to administer it to a Type 2 patient who could easily expect to live as long as I have or longer. Type 3 while it does leave them severely disabled could expect a normal life expectancy. Is it really worth it if there’s going to be no positive improvement or better outcomes?

In the video below a group of doctors discuss this new treatment as well as possible gene therapy treatments which are currently in phase 1 trials. They asked some of these tough questions about the advisability of using these treatments in adults for which improved outcomes are both unproven and in their opinion (and mine) unlikely.

They also asked a very disturbing yet appropriate question… If a Type 1 patient is not going to have a reasonable quality of life, should we use expensive and heroic measures to sustain that life or is it better to allow nature to take its course? These are very tough questions. I know people with Type 1 children and if I was in their shoes I could well appreciate their desire to do anything to keep their child alive. Still at some point, quality of life issues have to figure into the equation.

The bottom line is we still don’t know enough about how effective the new drug therapy is and we are a long way from knowing about the effectiveness and consequences of gene therapy treatments that are still in very early stage one trials.

Here is the video