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Prognosis vs. Reality
An acquaintance I knew with muscular dystrophy recently passed away at 36. In light of that, I’ve been thinking a lot about the prognosis I was given with my SMA diagnosis versus the reality I’ve lived. My parents were told I’d live until 3. I’m nearing my 30s myself now. That’s 10 times the years doctors thought I’d have.
I’m not sure what to think about the math. But I do think it’s good to contemplate it, from time to time. What about you guys? What was your prognosis? Did you live to exceed it? What do you think about that? Maybe, in sharing, we can strengthen our perspectives.
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18 Replies
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When I was diagnosed, prognosis was a tricky thing to pin down with SMA, they weren’t sure what to say to my parents. Someone mentioned Duchenne which, at the time, had a lifespan of 15. Even after they realized the mistake, they didn’t know what my lifespan could be. I have SMA II but I’ve got extra copies so that changes my outcome. The science has gotten better, there’s less misdiagnosing.
I’m 57 now, I never saw myself living past 30. While I’ve exceeded expectations, I also didn’t have plans for the second half of my life. At this point, my physicality has degraded so much that I can’t do a lot of things that I would’ve wanted to do if I knew I had the time.
Regret is a bitter pill. It’s one I’m trying to set aside.
The mantra of could’ve, would’ve, should’ve will eat you alive if you let it.
I’ve got a good gratitude practice, I read, I think about the imaginary book I’m going to write. But creating the habit of long-term goals when I never thought I would need them as proven… challenging.
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I can relate to your experience, Susana. I don’t have plans for the current phase of my life either. Which can be a little frustrating when I watch my peers zoom past me. Yet old habits and beliefs die hard.
I tell myself that mental health professionals recommend taking one day at a time for a reason. That helps.
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I have Type 1 and was given two years to live. I’m now about to turn 40, and with taking Everysdi my progression seems to have basically stopped. In fact, my swallowing has improved some over the last year. So I say hang in there and do whatever exercises you can to keep what you’ve got because we don’t know what other treatments will be developed in the near to mid future. When I was a child I was told that there would never be a cure for my disease, and now there are three treatments for it.
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Fantastic to hear your progression has stalled, Cam! Great that you’re so positive, too. Have you managed to re-try new or old activities since you started getting better?
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Hi Sherry and others,
Yes, I’ve greatly outlived the expectations of my childhood neurologist. She estimated I’d live to about age 45 as a type 3. I’m now age 70 and have fairly good health for a little old SMA lady!
(My Dad passed at 86 and my mom is now 96. Who knows what my future life will look like, but with all the advances in medicine, I just might surprise everyone, including myself!)
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Good for you, Robin. I’ve just turned 73 with type 3 too and am doing pretty well for an ‘oldie!’
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It’s nice to meet you. I don’t meet that many adults over 70, but I know there are probably quite a few. Have you ever attended a CureSMA conference? I just returned from WDW.
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I’m type III in my 30’s. I can still walk short distances with the help of crutches, but I’ve pretty much become homebound as I cannot walk any real distance without needing a rest. Every time I ask a neurologist about getting a wheelchair for long distances they turn me down and just keep saying I need to keep walking as long as possible. They wouldn’t even recommend any walking assistance, so I got the crutches myself online. I’m glad I did because I fall a lot less since I have had the crutches. How do other type III get their doctors to listen to their concerns or is it pretty normal to just get what you need yourself without help? I feel like since it is less severe, then I get ignored a lot. The last time I was there I said I could feel that I was getting a little worse as I couldn’t walk quite as far without needing a break and I had just broke my foot falling down some stairs, so after they briefly tested my legs they bluntly told me they were the same and not worse. It took me 10 years of complaining about my hip hurting because they said I was too young for hip issues before they would finally do an MRI, which found I have arthris, a torn labrum, and excess fluid on both sides. Even then they pretty much ignored my complaints of pain and finally I took pain care into my own hands when edibles became legal in my state.
I stopped going to the neuromuscular clinic all together about 4 years ago because of the lack of help. Last time I was there I had a worse than normal experience with a very rude doctor that was the final straw for me going there. The very same appointment I was told my legs were the same strength I was also told I would change my mind about getting pregnent, after a 15 minute speach about being able to have kids. I don’t want kids and tried to say that at least a half dozen times. I’m well aware I have the organs to carry a child, but have zero interest in doing so. My partner also tried to say we don’t want kids, which prompted the answer that my mind would change. The doctor kept interupting me and kept talking to my partner instead of me. I tried to ask politely to a nurse practicioner why they were changing my appointments from once a year to 6 every months and the doctor cut her off and said, “Because I said so. You cannot come and go as you please”. I got the feeling she did not like him at all either because she was in the room the whole time and made some faces at the things he said. After that experience I was so upset I haven’t been back since. Normally, I would just switch doctors if I did not like a particular doctor, but obvisiously that is not so simple for SMA. Normal neurologist in my area won’t see SMA patients as I have tried that and the two closet facilities that see SMA patients are both over 2 hours away. I feel like I am stuck between a rock and a hard place, but the stress of going to a bad neurologist was just not worth it anymore. I keep hoping that doctor switches hospitals, but no such hope. I honestly hated their SMA program, but tolerated it even before the bad doctor because I felt like they had nothing in place for type III patients.
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@Cait I’m sorry you’re not having the best experience with your neurologist. Even though the other neurologist is two hours away, is that something totally out of the question? Could you somehow arrange travel to and from if given the proper resources? Well, are they willing to do telehealth if you send your medical record over to them? I wonder if calling the office would get you some advice.
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I had to step away and collect my thoughts when I read this. Can’t even tell you how pissed I am on your behalf.
None of this is normal.
None of this is acceptable.
Your neurologist sounds awful. They’re supposed to be supporting you to stay engaged, not limiting your options. At this point, having one of those walkers with a seat on it would probably be very helpful for you. Can you talk to a Physical Therapist instead?
The whole pregnancy bit is outrageous. It’s not his decision and frankly, none of his business. If you and your partner have decided not to have kids that is YOUR CHOICE. It’s good to discuss your concerns and what reality might look like or what options are available but at the end of the day, you’re the one living the consequences of your decisions. By the way, you won’t change your mind. We didn’t have kids and don’t regret it.
And why do they want to monitor you every 6 months instead of once a year? That’s very unusual. “Because I said so“ is not an answer. That is condescending and insulting to you as a human being. You’re an adult and you can also say no. (You actually can come and go as you please) Unless they give you a real reason why they want to monitor you every 6 months make your appointment for once a year.
But I think you should make an appointment with a different clinic, even if it’s 2 hours away. I go for my eval once a year and it is a one day thing which takes several hours, but it’s very comprehensive and the staff is awesome. Other clinics are better, other doctors are better.
Finally, I want to encourage you to reach out to the patient experience department of your hospital. Explain what happened, all of it and be specific. This doctor needs a check on his bedside manner, although this goes far beyond bedside. If you’re not comfortable doing this for yourself, do this for the next patient who will be ignored and dismissed and unheard. You’re not the first and you won’t be the last, but you can do something about this. I had a similar situation with a doctor and the head of the department reached out to me personally. I never had to deal with that other doctor again. Your voice has power, don’t let this troll silence yours.
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Hey Cait. Like Alyssa and Susana, I’m appalled by the treatment you’ve received from your neurologists. I was at a talk recently where my neurologist’s senior did a presentation: in it, he said only half of all the SMA patients at my hospital have received Evrysdi due to things like cost, and he implied that result was a failure of the healthcare system. That’s what neurologists should be doing: ready to go to bat for patients. We deserve nothing less.
I think monitoring every 6 months instead of every year is worth it if you can afford it. As we get older, it’s better to catch bloodwork deficiencies and new needs sooner rather than later. But that’s something your doctor should have patiently discussed with you and respected your decision either way. You are definitely allowed to come and go as you please. He’s supposed to be serving you!
I won’t say you should report him, as I haven’t reported on healthcare professionals I was traumatised by. Still, trying to find ways to get the care you need and deserve may be worth it. It’s your health. Don’t give up.
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Your post got me thinking, Robin! Of course, living with SMA often means a shorter lifespan, but I wonder whether genetics plays a role as well. Both sides of my family have lived well into the 90s. My grandmother is one of nine, and although she lost a sibling due to the Spanish flu way back when, the rest of her siblings have lived into the 90s and even 100. The 100-year-old is still living independently, too!
The doctors told my parents I wouldn’t live past the age of two. Obviously, genetics aren’t the reason why I’ve gotten to 35 years old with SMA type 1, but I wonder if it’s a small factor.
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Alyssa, I’m embarrassed to admit that I never really thought about others’ genetics. How silly of me. Since scientists are still learning about SMA and the different ways it affects us, it’s definitely something to consider. We already know that SMA has a wide spectrum, and the number of copies of SMN2 is only a part of the equation (for severity). Hmmmm. Perhaps there will be interest in this topic soon. 🤔
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To be honest, I never really thought of it until your last post! The next time I have an appointment at the clinic, I will ask them this question. It kind of makes sense, right?
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My paternal grandmother is now 97. Just survived two operations for stage 2 colon cancer. Her mind is as lucid as ever and she’s still able to be mobile. Makes me really hope you’re right, Alyssa. 😂
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Wow, that is a good question,
The problem with the prognosis is nothing more than an educated guess sometimes based on as much factual data as you can have. The fact of the matter is that reality, maybe sometimes trumps the prognosis. Case in point. I was diagnosed with SMA III in 2005 and when I talked to the doctor, the prognosis was, I would be wheelchair bound by the age of 50 and would lose much of my strength and functionality due to what they knew back then today. Today I am still walking at 61 albeit sometimes hard and going up and down steps is a real challenge, but I’m able to lead a mostly normal happy life having an incredible wife. Have children pretty much live in the American dream other than the disability. I firmly believe that part of us have a hereditary inherited trait that’s passed down to us from our parents along with the mutated SNM2 Gene. I believe that a prognosis is something that may happen and sometimes it is what does happen, but it is not a 100% gurantee.
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Absolutely, a prognosis is simply an educated guess dependent on the data available at the moment. We have much better data now. When I was diagnosed, you would only be tested if you were showing symptoms which generally applied to type I or II, I’m guessing there’s a lot of type III who historically were never diagnosed.
But even so that doesn’t take into account our personalities, our drives, our goals, our passions. This condition brings barriers with it, but it doesn’t define who we are as individuals. I think that’s a tough sell to new parents who are given this devastating diagnosis. But it’s so important that these kids have all aspects of the human experience, including career and financial support
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Exactly and I have a saying that keeps me going. The saying is “Life doesn’t always follow the plan. What you do next matters”
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This reply was modified 1 day, 15 hours ago by JeffS.
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