[mike-huddleston]

This is a tough one, Alyssa. I’m much older, so my 4 nieces now range from 19 to mid 30s. My 8 nephews now range from about 20 to early 40s. When they were in the range you’re asking about, I was still ambulatory and able to be more involved. I was no longer able to run, but could stand and throw a frisbee or play catch with baseball/glove or lacrosse sticks. I’ve been in the chair since 2015, so most of them were already older by this time. As they’ve gotten older, we go to baseball games and dinner, etc., but that type of activity may not serve an under 7 year old very well.

So, my suggestion would be to think through things you can do, even if those stretch you slightly – slightly – out of your comfort zone while still being safe for you. Like, are you comfortable going to the movies? If so, maybe go to a movie with one or both of them. Do they like to play sports of any kind? If so, maybe watching them play games or practice and encouraging them might work. I think seeing you in different settings will be helpful to them and to you.

Please let us know how your relationship with them progresses.

[mike-huddleston]

My neuro at Hopkins prescribes Mestinon (Pyridostigmine) for me. Has to be taken with meals, so caution if GI issues. I take 1 60 mg tablet with breakfast, and then split one in half for lunch and dinner. This allows the muscles to contract longer before fatiguing. Does it work for me? I’d have to say yes. On a recumbent stepper (NuStep) used at PT for a 10 minute warmup, I went from level 1 to level 6 (out of a total of 10) and my reps per minute were more stable toward the end of the 10 minutes than before I started taking it. IOW, as an example, before taking it, I was not able to increase level of resistance and would average 75 steps per minute for the first 5 minutes than about 60 steps per minute for the last 5 minutes. After taking, increased resistance and average of 75 steps per minute the first 5 minutes and close to 70 steps per minute for the last 5. Similar consistency at each increased level of resistance.

Last week, I also just started taking nitric oxide supplements (OTC, not prescription). An ambulatory type 3 mentioned it to me. Basically, it increases blood flow as it’s a vasodilator. Highly recommend discussing with doc before taking this as there are potential side effects. I’m taking less (one) than the suggested amount (three), and will see if I notice any benefit.

Of note, I exercise a LOT. Weekly clinic and aqua PT sessions, and about 2 hours per day in my home exercise program – even on PT days.

[mike-huddleston]

Sorry, maybe it’s where I live, but I don’t get this impression at all. And I’m not a fan of trying to make myself a victim. Access is not going backwards just because businesses that had to make accommodations for EVERYONE to even stay in business during COVID, like curbside delivery, eliminated those options. It sounds like you may not have a lot of options, and I understand the frustration with that. but I sincerely find this whole topic and some of the responses difficult to process. Try calling the business or better, going by when convenient for you and actually talk with the owner or manager. Explain your situation and see if some kind of compromise is an option. You say they don’t do takeout on the weekends, is a weekday a possibility. Staffing someone, or several people, to handle takeout on their busiest days may not be feasible for them. It’s hard for people to hire and retain in the service industry, so maybe this was a decision they made to remain viable.

There is the ADA and the subsequent updates that have made things considerably better for all of us. That was preceded by the Architectural Barriers Act in 1968. Is it perfect? Nope, but that’s why revisions happen.

Stop being a victim. Don’t allow others to make you one either. Be your own best advocate as it benefits you and others. And just because changes made during a once in a century or lifetime pandemic are not persisted, it certainly doesn’t mean things are going backward.

[mike-huddleston]

Hey Alyssa –

To me, it boils down to making sure the doctor doesn’t look at you as an SMA patient disguised in a human body, but rather a person living with SMA (among many other things). Just like interactions with folks who don’t, won’t, or can’t see beyond our condition, especially if you’re using a wheelchair, it’s the lazy view or discomfort that results in such a definition. It’s up to us to help them overcome that.

I had a PCP that had nothing to do with my SMA related care. I should preface this by stating I NEVER went to the doctor unless it was absolutely necessary. But, he was my wife’s PCP so after we married, she encouraged me to start going annually. I gave him the usual rundown, but also note that I was ambulatory at the time and not even using a cane or other assistive device. But obviously, my SMA was progressing (more labored walking, difficulty getting out of chairs, etc.). For my annual visit, the diagnosis code every time for over 15 years was, “Spinal Muscular Atrophy”. I dismissed this as either ignorance or just a need for insurance billing purposes. He had another doctor in the office I saw once for the flu or similar issue and I saw him as I was leaving. He said, “Hey Mike, how are the muscles? Still getting weaker?” I was astonished, angry, and so disappointed. I said, “Gee, doc, after being my PCP for almost 20 years, I would have thought you’d have pulled the PDR just once or Googled SMA. That’s kind of how this condition works. How can you not know that? I will never be back.”

I found another PCP and have been seeing him since 2015 when I started using a power chair. That means I went over 10 years without a PCP. The experience has been much better, but I took the time to lay out basic SMA related info on my first visit and he’s actively engaged and treats me like a person.

And I know we’re discussing PCPs, but just to show it’s not all been negative, while I was still ambulatory, I tried acupuncture with a traditionally trained acupuncturist. She and her husband had moved here from China and were both medical doctors. At the time (not sure if it’s different now) the US had requirements of several additional years of medical training to practice here in the States, but they had started a family, so they opened an acupuncture clinic (it was part of their medical training in China). Between my first and second appointment, she had done a ton of research, reached out to several former colleagues in China about SMA, and developed a comprehensive treatment plan for me. What a great experience!

[mike-huddleston]

First of all, congratulations on getting close to be able to obtain treatment! That’s exciting. That we have a choice is also amazing!

I’ve been treated with Spinraza since May of 2018 and mostly have had positive experiences and results. However, I do seem to have plateaued and have considered switching to Evrysdi. I have a PT assessment in early April and if I’ve declined as I suspect I have especially in the arms/shoulders, I have little to lose by switching and trying. Also of note, it is now much easier to switch between the two treatments than previously, so trying the alternative isn’t as horrible of an experience as it used to be.

For Everysdi, the primary side effect I’ve heard about and echoing the comments of those here who use it, it’s the potential GI issues.

For Spinraza, you do 4 loading doses in the first 8 weeks (0, 2, 4, and 8 weeks), then every 4 months for maintenance. Some folks have stated they feel much more energetic immediately after the injection, and more lethargic the last month or so before the next one. I have been moderately stable in the way I feel throughout the 4 month intervals. Another factor you might want to consider is the method, as Spinraza is administered with a lumbar puncture. That may cause pain or discomfort, and if it leaks, it may require a follow-up blood patch. This is rare. Also, for me, the lumbar puncture requires me to lay flat for nearly 2 hours after the injection to reduce the possibility of a headache. The one time I didn’t adhere to this, I had a headache for 6 days. YAMMV. The facility you have the injection done may use live fluoroscopy or may just rely in the anesthesiologist to guide the needle. You may want to know that going in if that’s a concern for you.

And finally, since you asked, there is not a single thing that any doctor can point to and say definitively one will work better for you than the other. Some people respond better to Spinraza, some better to Evrysdi, and some who have switched have said they felt no noticeable difference. So, it’s going to boil down to your preference. That said, the ability of Evrysdi to distribute throughout your body as it’s oral and goes through the bloodstream is viewed by some as a better distribution mechanism. In autopsies, the only time when this can currently be determined, the highest concentration of Spinraza is near the injection site.

From a cost perspective, it’s likely best top discuss with each company. Spinraza is typically considered a medical procedure and covered as such if you have insurance. Evrysdi is often considered a prescription drug, and thus often covered by your prescription coverage rather than medical. Also, BioGen seems to have a much better co-pay assistance program if Spinraza is not covered 100%. In discussions with Genetech reps, I don’t have a warm and fuzzy about whatever may not be covered for Evrysdi.

Best wishes in your treatment journey. I took the time to write all of this because it’s not a simple answer.

[mike-huddleston]

I wish awareness months historically were not needed, but if you’re an able bodied, white, Christian, (most often) male, sometimes a reminder that the country and world has moved beyond every single thing being about you isn’t necessarily a bad thing. That said, like many things, these often seem to be “preaching to the choir” and not as helpful as we’d like. However, if they serve in some way as a reminder that people within a given community matter, then that’s a good thing. One of the reasons I enjoy volunteering within and mentoring for the disability community is to serve as an example that everyone is more than just their disability. It doesn’t define me; it’s part of who I am, but it’s not what I am. So often kids can’t see beyond their current situation and that they matter and that they can have a fulfilling life.

However, to answer your question, let me say that if awareness months help with this, to me, no harm no foul. But advocacy, for self and for others without voice – or a forum to express it – seems to be the most important area of work that needs to be done.

[mike-huddleston]

Some of this is incremental improvements. Personally, I don’t view an ASL interpreter as “media representation”, but rather a necessary enhancement to the experience of those in attendance – or watching. And, unlike some, I don’t really think this is an “equal access” issue. They don’t need to do a split screen, but a PiP option would be a nice and easy thing to do. Technology is way past this capability – so use it. As far as in the stadium experience, I haven’t been able to confirm it online, but perhaps they had the ASL displayed on the jumbotrons, meaning anyone further away from the field could see this…games I’ve been to (never the Super Bowl) often display something on the in stadium broadcasts not easily seen in the entire stadium? Additionally, there were streaming options available that showed the entire ASL performance of each of the songs.

Was it perfect? No. Will it improve? Yes, thus my incremental improvement comment above. I mean, since the US Barriers Act and the ADA were implemented, access has improved – dramatically. Curb cutouts, ramps, dedicated public seating, public transportation access, accessible restrooms, online mechanisms to determine accessibility for those concerned (have you seen this option in Google maps??), and the list goes on for folks with non-mobility related issues. No, it’s not perfect, but this whole uproar is making perfect the enemy of good. Be constructive, advocate for yourself and for improvements when and where deficiencies are identified.

Some of this, and certainly not all of it, fake outrage is what gives DEI and being “woke”, which truly just means being aware and empathetic that others have a different life experience than your own (and not the lies and garbage being portrayed negatively in some political circles), a bad rep.

[mike-huddleston]

FWIW, MDA Quest is seeking speakers within their local ambassador program (I’m one) to talk specifically about this very topic of finding and securing caregivers. I’d imagine since they’re seeking speakers now, they will have this as either a live discussion or podcast soon. Keep an eye out, but I will try to remember to respond here once I see something scheduled.

[mike-huddleston]

Like Alyssa, I have a FAM for Spinraza. Not sure that it’s critical to have a person in this role, but I find she is a good resource for initial questions. Better to have and not need them, than to find out you don’t have one and no initial point of contact. I suppose I do have another question: what is the consent for? In other words, what happens if you don’t sign it?

[mike-huddleston]

I agree wholeheartedly with what both Micaela and Alyssa said. Painting a picture of your experience is critical to educating them. They apparently are seeking this type of information; glossing over it so it doesn’t sound like you’re complaining may make it seem like less of an issue than it is. Micaela’s “here’s what I need, here’s what I get and this is the gap” is amazingly powerful. Folks have no idea what the experience is, so lay out the facts and see where it goes. It may also be worth asking what they’re planning to do with this information. In other words, is it actionable or lip service? Offer your willingness to participate in ongoing discussions. And finally, as far as feeling like you may not be comfortable speaking, this is what I told myself when nervous in preparation for presentations when I served as a BioGen Spinraza ambassador: This is my story – mine – no one knows it better than me. You’ll be fine!

[mike-huddleston]

There are many people dealing with arthritis that don’t have SMA or other similar conditions, so would imagine they are separate. That said, without load bearing and a lot of muscle mass supporting the bones, there could be an increased chance of arthritis or other bone related issues. As an example, I have bone density related issues and take prescription Fosamax, which has actually improved my bone density per the last two annual DEXA scans. So, I guess my take is SMA doesn’t cause arthritis, but the lack of load bearing and muscle mass may allow those types of issues to be more likely? Sorry, don’t know if that makes any sense whatsoever!! 🤨

[mike-huddleston]

Nice and great location. FYI, I volunteer at an accessible sailing facility near there, CRABSailing.org, on Bembe Beach Rd (Bay Ridge Rd to Edgewood to Bembe Beach Rd). They’re closed for the season, but it’s a wonderful organization and if anyone wants to get out on a sailboat on the Chesapeake Bay, they have 6 accessible 22′ sail boats (transfer via slide box or Hoyer lift) and a power catamaran with in-floor locking mechanisms for those who don’t want to transfer out of their chair. If you’re not local, stay at this Air BnB and come see me at CRAB starting next spring!

[mike-huddleston]

I turned 60 earlier this year. As there are no guarantees for anyone on this planet, I think I’m mostly just grateful that I’ve had a rewarding life and a wonderful 36 year career that I retired from last year. And even more importantly, my wife and I celebrated our 40th anniversary just a little over a week ago! I know I’m 60, but I feel closer to about 25 – 30 mentally. And aside from the SMA, I feel amazingly good, health wise, so thankful for that as well.

My mindset has always been that getting older isn’t a problem as birthdays are only a problem when they stop coming around. Embrace getting older. Live and love the experience. Focus on the beauty of life, not the challenges presented.

[mike-huddleston]

When I had a Quantum Q6 Edge, I had to replace the joystick several times. I had one die on me in the restroom at work. I was able to get it to stay in the slowest speed, but still drivable, like that for a few days by taping it in position. I also had an attendant joystick on the back for when it died completely another time. I was able to have someone pull that out of its holder and drive it from where I was resting it near my chair arm. I realize that may not be an option. I’ve had the wires fray completely on the current Permobil M3. After that was fixed, I found and bought another replacement wire online to have as a backup. If you tell the DME that you’re basically unable to move your chair, they should escalate that and resolve more quickly. I have found that they keep used parts and often have temporary replacements available until your new ones come in. This is usually not inventoried, and may come down to some of the technicians and what they have in their vans. And finally, I keep all of my parts when something is replaced and have actually MacGyvered a joystick. If your DME tells you they need to take the parts with them, that’s only true if it’s something being replaced under warranty.

[mike-huddleston]

Hey Matt –

Very interesting and I appreciate you posting that. Do you mind if I ask if you are also being treated with either Evrysdi or Spinraza and if so, any difference in combo with it?

[mike-huddleston]

Hey Lisa – definitely worth a shot. I know two other type 3s, both ambulatory (I’m not since 2015) who tried it as well. One remains on it the other didn’t. As far as the side effect, it’s to be taken with meals. When I do that, I haven’t had any issues. And without re-reading what I wrote above, apologies if this is repeated info. The Rx calls for 60 mg and it comes in tablets, each being one dose. When I ramped up with it, I cut each in half and took a half with breakfast for a few days to see how it went. Then increased to 1/2 at breakfast and lunch for a few days, then added a 1/2 at dinner for a while. I now take one whole one at breakfast and a 1/2 at lunch and dinner. So, ramping up helped for me and something to keep in mind. That will also tell you when you may cross into the side effect area.

I hope you find it helpful, but please keep us in the loop on any progress.

[mike-huddleston]

Interesting study. Thanks for sending Lisa. I read it quickly, but it would be good if repeated in conjunction with treatment (I started Spinraza in mid-2018, before starting Mestinon). And reported outcomes are hard to measure (“How do you feel?”) and more susceptible to the placebo effect. They should also break down the results better between type 2s and 3s as well as copies of SMN2, all potentially meaningful to how one responds to treatment of any kind. I’m a 3 with 4 copies of SMN2.

My experience was documented and displayed at the 2021 CureSMA Conference as we had weekly hard, documented, measured data over a nearly 2 year period. So, my baseline measures were established while already being treated with Spinraza, so the biggest gains for me were with Mestinon. IOW, I was measuring results on the NuStep for nearly 2 years before starting Spinraza, and then being treated with Spinraza at least 1 1/2 years before starting Mestinon. There was a slight and consistent uptick in resistance levels and steps per minute with Spinraza, but a much sharper increase in both measures after starting Mestinon.

[mike-huddleston]

Thanks Tracy. I value and appreciate your response, thoughts, and perspective.

That said, I think I have a fundamental disagreement with framing it as “going backward”. As mentioned in my response to Eric, not moving forward as fast or as much as we’d like is not the same thing as moving backward. Framing the removal of necessary conveniences that were implemented for everyone, not just those in the compromised or disabled community during the pandemic as moving backward and an afront to the disabled community, is in fact making oneself a victim. I simply don’t agree with that and refuse to be a victim or allow others to place me in that category. When we do that, it reinforces the stereotype or bias people have with those of us within our community. It’s my opinion, and I hope you respect that as much as I respect yours.

One main thing that makes this difficult is the umbrella that we are under in our community. I was the chair for the employee resource group for employees with disabilities at the company I retired from at the end of 2022. There were over 42,000 employees. One thing unique in our community that was not evident on other ERGs is the broad range of disabilities. We have mobility related concerns, as well as vision, hearing, mental, neurodiversity, etc. There is simply no one size fits all approach to our community. From a business perspective, that can be overwhelming when trying to accommodate everyone. The ADA is nowhere near perfect, but it lays a solid foundation. Companies that meet the bare minimum requirements present a whole slew of issues for those of us with accessibility difficulties.

Related to this is the whole “anti woke” nonsense. Woke was always intended to be a raising of awareness of other’s circumstances, history, or current challenges, and to develop an understanding and potentially increase empathy. However, it has been co-opted and turned into a pejorative, which means anyone in ANY of the diversity groups, including the disability community, are lumped together. I still don’t think that means moving backward. I still won’t allow myself to be viewed as “less” or a victim.

And it’s unfair to imply that I’m saying that we should just accept accessibility issues. I don’t mean that and if I implied it, that certainly was not the intention. Raise issues and concerns…I clearly suggested directly communicating with the business, preferably in person if that is possible. Again, kind of what Eric was describing in his response to me. If a new business is not accessible, that concern can and should be raised.

TO ME: Going backward would be no medical/pharmaceutical companies pursuing treatments. It would mean eliminating building access. It would mean removing curb cut outs. It would mean accessibility was being removed rather than improved. It would mean airlines weren’t actively considering modifying their policies and accommodations for travelers within our community. These are not my experiences and observations, but it doesn’t mean things can’t be better. We should push forward, not give up. Does that mean everyone or every company is in compliance? Certainly not. Does it mean we’re better off than we were? Absolutely yes.

[mike-huddleston]

Thanks for your response and clarification, Eric. I think my issue is framing it as going backwards. Perhaps, and my opinion, perhaps we aren’t moving forward as quickly as we’d like, but that doesn’t mean we’re moving backward. Yes, by all means, point out issues with accessibility, and raising awareness is a part of that. I personally have not experienced things moving backward. That doesn’t mean things are perfect or where they should be.

[mike-huddleston]

Please explain what you mean by, “disability access is being repackaged as optional special access”? I have no idea what this means and would like to understand. And only part of the reason masks were required were to protect the elderly and those with compromised immune systems. If that was viewed as protecting everyone with a disability, well, there’s no cure for stupid. That was quickly determined to not be a very effective mechanism to stop the spread, but rather protected the wearer more than anything else. Again, a pandemic where no roadmap existed, so caution, perhaps sometimes taken too far, was taken. Malls going away are just the way things have been trending over the last 20 years or so. It has absolutely nothing to do with taking away an accessible space for the disability community. Again, being a victim is not a pretty way to go through life.

[mike-huddleston]

Hey Lindsay –

So glad to hear the sedation worked for you. That said, perhaps the radiologist is now comfortable with more than just lumbar injections so you might be able to revisit the injection site, cervical vs. lumbar. If you didn’t experience pain when having the injections in your neck and if the sedation becomes problematic (ineffective or giving it to you), I’d encourage you to discuss with your neurologist and the radiologist the possibility of relocating your injections back to your neck.

Hope this works out for you!

[mike-huddleston]

Sorry for the delayed response. So, my neuro at Hopkins suggested I schedule an appointment with an endocrinologist to discuss bone health. DEXA scans measure bone density, and mine was low. The endo’s recommendation was to take Fosamax, which can be an annual injection or a pill taken once per week. I chose the weekly pill option. One of the measures of bone density is called a T score or T count. Mine has improved on the medication. That means the bones are more dense, thus stronger, and less susceptible to breaks/fractures. I don’t feel any different taking it but appreciate the stronger denser bone structure. I’m not aware of any side effects, but am sure there are some.

Have a nice Thanksgiving, Alyssa!

[mike-huddleston]

That’s wonderful! Glad you enjoyed the experience and that you have it listed!

[mike-huddleston]

Honestly, it’s hit or miss with my DME (NuMotion). Typically, and especially more recently, when the chair is not working at all, they were pretty good about helping quickly to resolve it. The frayed controller wires on the Permobil was September 2022. I had my old chair, but that has unreliable batteries and the elevator seat no longer works. They came out to my house the next day and installed temporary wires until the ordered ones came in a few weeks later. I believe it’s hit or miss as it depends on what they have available. In my case, the tech happened to have a serviceable and exact replacement cable. Not sure how much you can count on something like that.

I would encourage you – and everyone – to not overstate the direness of your situation. If you can make due safely, don’t tell them it’s urgent as that will affect response time for everyone, including people who are genuinely in need of urgent attention. If you chair is DOA, tell them and let them know if you have a temporary replacement or need one. It also might be worth a call to your local MDA office to see if they have something temporary available in their lending closet. Doubtful for a power chair, but just another option.