Living with ALS

[Bill]

I’m over 3 years since diagnosis. I was a year before that getting diagnosed. At first I did more more time researching. I read all I could and actively researched trials. Nowadays, I spend only 15-30 minutes per day on line either keeping abreast with pALS or reading the news from the several news letters and alerts that I get. Luckily for me with my slower progression I do not need to spend time dealing with my ALS. Yep, things are more difficult but it’s part of my life.

[Marianne Opilla]

I was diagnosed about 18 months ago.  At first, I devoured everything I could find, and tried many things.

Now, I rarely read about research, watch webinars, or visit ALS websites anymore.  It is depressing to learn of so many efforts only to realize they are not available.  I only want to know about FDA approved treatments that I can access.

But, alas, I continue to progress and lose function.  Nothing seems to be helpful.  I just try to focus on one day at a time and forget about ALS.  I enjoy family, friends and my dogs and try to stay positive.

 

[Aloha Frank]

I was diagnosed with ALS one year ago (March 2020) but had obvious symptoms six months earlier when I noticed slight difficulty speaking and a small, but noticeable loss of muscle in my left arm and hand. I did some research and realized that it was most likely ALS and so began a four-month long process involving four different specialists and every medical test known to man. Finally a neurologist confirmed what I had already suspected – ALS. My wife had no idea that I suspected anything so she took it very hard when we were given the news while I took it in stride, knowing the obvious. At first I spent countless hours reading everything I could get my hands on about the disease and eventually came to the realization that there was nothing anyone could do about it. Even the neurologist said I had 24 to 36 months to live, and that my future was going to be extremely difficult as the disease progressed, which I already knew. He told me about one medicine that would, at best, extend my life by 60 days, but said it came with significant side effects and went so far as to not recommend it. I decided not to bother taking it. Why prolong the terrible suffering any longer than I have to? Makes no sense to me. I spend much of my time communicating with relatives and friends via e-mail using my ‘good’ right hand as I can no longer speak. I follow ALS patients here on the Forum often and learn how they manage to cope from day to day. Some comments are interesting and helpful about how they’re dealing with their lives. I’ve learned more from them than from most published articles on the subject. I think it’s a waste of time constantly reading about ALS trials, potential new treatment options or any possible cures on the horizon. If they haven’t found an effective treatment, let alone a cure, in over 100 years, I seriously doubt they’ll suddenly find any during my limited lifetime. I live in the present one day at a time with my loving wife and family, and frequent visits from my many friends. I get online every day and watch TV a lot while waiting for the elevator we’re having installed to be completed. We live across the street from the ocean in Haleiwa, Hawaii with a great view of the ocean. I miss going outside to enjoy our beautiful weather and environment. We live in a flood zone that requires our living space be 10 feet above sea level. As a result, I can’t get up and down the stairs unless someone carries me, something I try not to bother anyone with. I spend nearly all of my time in the house. Hopefully, when the elevator is completed, I’ll spend much of my remaining days outside in my electric wheelchair, enjoying the beautiful Hawaii weather and awesome surroundings.

[Marianne Opilla]

Aloha Frank,

I, too, live on the water. We live on Smith Mountain Lake in Virginia.  We have lots of porch space so I can see the water.   It is the  #1 thing that seems to sooth my soul.

Take care and enjoy the outside time once your elevator is available.

Marianne

[David Crellin]

Thanks for all the helpful comments. We’re all so different in how we respond emotionally, mentally and physically. Vive la difference.

I have c9orf72 fALS diagnosed 3 March 2020. I’m in the invidious position of having as a child nursed my gran through FTD and as a teenager nursed my mum through ALS, then nearly 50 years later being diagnosed with ALS and a year later my 28 yr old son being told he has an 80% chance of developing ALS (my 30 yr old son is waiting).  So a year after diagnosis and two years after noticing first signs (you can guess I was more aware than my GP of the signs and probabilities), I keep on with research. If not for my benefit, then for my sons’ and wider family’s.

I also question the competence of many doctors (my sister & her husband are both doctors and several of my friends are consultants). The neurologist mentioned by Frank above is a case in point. Riluzole prolongs life by an average of 90 days. I’ve been taking it for a year with no side effects. My consultant  suggested Riluzole would not pass current clinical trial evidence of efficacy. He also said that in my position he would do what I’m doing: research all possible therapies and get them by any means. So after taking Metformin for several months when a consultant friend sent me a supply from Singapore (!), I sent recent evidence of it’s efficacy with c9ALS to my GP and now get it free on the NHS (free health care here in the UK). Similarly with TUDCA.

If my mother’s disease progression is my benchmark, then I am  progressing more slowly than she did. She showed first symptoms four years before death, but was diagnosed 5 months before dying aged 49.

And I keep on reading, posting, talking and campaigning. That’s just the sort of person I am. It’s what I did before ALS, and  probably what I’ll keep doing until I no longer can.

Like Marianne and Frank, I’m privileged to live in a beautiful place with wide vistas all around in an area known as England’s last wilderness  (a marketing slogan!). So I’m preparing my garden and land to make it accessible and have bought an all-terrain mobility scooter. Ain’t no stopping me now.

[Chuck Kroeger]

I was diagnosed with ALS about 2 years ago and initially it was really tough but I was still able to walk and use my arms and breathe without a breathing assist machine except at night.  as I progressed to the point that I can longer walk and I can no longer use my arms . barely use my hands and am on breathing machine full time, I doubt if I go 10 minutes without thinking about my situation and the difficulties that my wife has as my full time care giver. I am ready for the Lord to take me home before I progress further.  Winter weather and covid have certainly not helped the situation

[Tom Pritchard]

I am 69, living in my 70th year. I retired on my 55th birthday, 14 years and 8 months ago after I had a stent put in my heat. Four years later I got a second stent. My father and oldest brother had multiple cardiac bypasses my oldest brother has had several stents after his bypass. My mother was on her second pace-maker when she passed away after a series of mini-strokes. My father had to have his pacemaker turned off because he had cancer at the back of his tongue preventing him from eating. He passed away about 12 hours after his pacemaker was turned off. My oldest brother has just recently had a pacemaker installed. My other brother passed away (my #3 sibling, I was the 4th) several years ago he had a lot of medical issues. My sister had polio when I was born and she was cured. She has high cholesterol which is chronic in our family, including me. My heart is reliable now, no need for a pacemaker or any more stents.

!8 months ago my speech started slurring. My Primary Care Physician (PCP) tested me for strength, stability, and coordination, thereafter he ordered an MRI of my brain and the radiologic result indicated two strokes which I had been totally unaware of. He also referred me to a speech therapist who helped me as best as she could in four appointments. My Florida PCP also referred me to a neurologist who referred me to a psychologist for cognition, memory, and coordination, a sleep test for oxygen intake overnight, at home, and a sleep test at a hospital facility. I also went to an ENT physician because I had sinus issues. The Neurologist suspected Myasthenia gravis (MG) and ordered a blood test, which was specifically for MG, that test was negative. She also ordered a barium swallow, which was done in upstate NY (where we spend our summers).

During these 18 months, since my speech started slurring, my speech has degraded severely, my tongue is weaker, my swallowing is much more difficult, I cough a lot more due to mucus and saliva, my sinuses are much more bothersome. I have some arm and leg muscles twitching sporadically. My PCP in upstate NY is a Family Nurse Practitioner, he is professional and sensitive, he referred me to a neurologist and ordered another barium swallow for me and another blood test for MG. The blood test was still negative. THe Neurologist immediately suspected Bulbar ALS. I went through a nerve test of my left foot, leg, hand, arm, and the left side of my tongue. He also ordered a pulmonary test of my breathing capacity and strength. I still do not know what those results were. The nerve test did not confirm anything. I also went to an ENT physician who prescribed a nasal spray called Azesterol, which is helping. I am drooling now and I also use nasal strips before I go to sleep. I had two maternal first-cousins who were afflicted with ALS, the older of the two started with the same symptoms I am experiencing, he was diagnosed with sporadic ALS. His younger sister was diagnosed with familial ALS (Familial is 5% to 10% of victims, sporadic is diagnosed for the other 90% to 95%). The widower, of the second cousin victim, is encouraging me to be proactive and keeps sending me helpful information. I am working with another speech pathologist/therapist who is very professional, sensitive, helpful to my issues. My NY Neurologist prescribed Riluzole which I am now taking. I also went to the Lewis Golub ALS Center in Albany, NY a few days ago and met their team of a nurse, and physical therapist, two Occupational Therapists, and a Social Worker. They were also professional, sensitive, and helpful.

At this point, I suspect that ALS is caused by genetics and therefore all victims are familial. Unlike cancer, which can be caused by genetics as well as by environmental issues such as pollution, chemicals, occupational exposure, and food. The French Neurologist Dr. Jean Martin Charcot identified ALS in 1869. !52 years later, along with much more genetic research, we or they cannot necessarily track our ancestors back far enough to know what our ancestors may have passed onto us. They had shorter life spans than we experience now. Sanitization of Surgery. anti-biotics, which we have been overexposed to, and other modern technology has helped us last longer. But those of us who are afflicted with Muscular Dystrophy and ALS seems to me, anyway, to be genetic. Some other afflictions are probably genetic as well. The current pandemic has been dramatic and ut is more impactful on elderly people. Researchers and scientists suspect that there will be worse viruses that will attack us in the future.

[Tom Pritchard]

I just spent an hour and a half writing down my situation, experience, and thoughts and I had to edit it and it disappeared. I am not going to waste another hour and a half trying to put it in again.

[Deleted User]

“I just spent an hour and a half writing”

 

Hi Tom,

Just a tip. If you can use a program like Microsoft Word, you can save your writing before you post it. Copy and paste it when you are done by pushing Ctrl/C to copy, and Ctrl V to paste. If you dont have Word, you can use notepad, it is included in all Windows system free of charge.

[Kevin Schaefer]

Hi Tom, your post accidentally got filtered as spam, but I recovered it and it’s now posted. Apologies for the tech issues. WordPress often flags long posts, especially if they’re copied and pasted from a Word doc. Please let us know if you have any questions.

Cheers!

[Bill]

Tom, I got your update showing in my email from the blog site. You should look for a site to do your genome to find which gene affects your family. I noticed you mentioned Florida. University of Miami ran mine through the prefALS study. I am in Florida. Couldn’t find a mutation with me but would be good to know. Good luck.

[Tom Pritchard]

Thank you Bill. I retyped it into a document and I am reposting it now:

I am a 69-year-old Caucasian male. Fourteen years ago I was told I had a heart attack however the solution was a posterior stent. Four years later, I was asymptomatic but I got an anterior stent installed because of diagnostic test results. I was the youngest of four siblings. My mother experienced a series of mini-strokes and she also had cardiac issues. She lost a lot of weight and contracted into a fetal position. She could not speak but we could tell from her eyes that she was alert and sensitive until she finally died. She had a second pacemaker when she passed away. My father and the older of my two brothers (#2) had bypass cardiac surgeries and my #2 sibling has had several stents since the bypass. Our Father had to have his pacemaker turned off to allow him to pass away because he had cancer of his tongue, he was a long-time smoker, and that cancer blocked his ability to swallow food. My remaining oldest brother recently had a pacemaker installed. The #1 sibling was our sister who had polio when I was born. We all have had high cholesterol issues. The #3 sibling, another brother, had many health issues, including cardiac issues. My cardiac health seems to be under control with no more stents and no pacemaker.

Eighteen months ago my speech started slurring. My Primary Care Physician (PCP), in SW Florida, did a strength and coordination/balance exam on me and ordered an MRI of my brain. He referred me to a speech therapist with whom I had four appointments with. The result of the MRI had a radiological report which indicated that I had experienced two strokes. There had been no apparent consequences from those two strokes. My PCP also referred me to a Neurologist who ordered several tests for me. One was an oxygen test overnight at home, a referral to a psychologist who did a series of cognition tests on me. A blood test to test for Myasthenia gravis (MG), an overnight sleep test at a hospital clinic, and a barium swallow. She had suspected a loss of oxygen intake due to sleep apnea. And she was also focused on MG. The results of all of that testing displayed some degradation but no source was confirmed. The barium swallow was done in upstate NY where we spent our summers.

I contacted another Primary Care Practitioner, a Family Nurse Practitioner, in upstate NY and he referred me to a Neurologist, and ordered another Barium Swallow. The Neurologist came to a tentative diagnosis of Bulbar ALS and he ordered a Nerve test, a pulmonary test, another barium swallow and I went to an ENT specialist because I have had sinus issues for decades. The second Barium Swallow exam was not very different than the first one. The nerve test was on my left side from my foot to my leg, to my hand, to my arm and the left side of my tongue. The results did not confirm the tentative diagnosis, at least not at that point in time. I still do not know what the results of my pulmonary test were. I had another blood test for MG and is was negative

I have lost two maternal first cousins who succumbed to ALS. The first, a brother, started out with symptoms like I am experiencing. His younger sister had lower extremity issues. He was diagnosed with sporadic ALS, she was diagnosed with familial ALS. Her widower has been briefing me with a lot of information and advice. He has been a big help. Her twin sister passed away first, from Cancer.

I still do not have a prognosis of how much longer I will live. The more recent Neurologist prescribed Riluzole to slow down the spread of the ALS I am experiencing. He referred me to an ALS Center in Albany, NY. I met with a nurse, a physical therapist, a social worker, and two Occupational Therapists. They and all of my other practitioners, have been professional, sensitive, and helpful. The ALS Center’s Medical Director had just retired but she had evaluated all of my diagnostic tests and concurred with my Neurologist about his diagnosis of Bulbar ALS..

I have come to a conclusion that all ALS is genetic. According to clinical sources, 90% to 95% of ALS victims are sporadic and 5% to 10% are familial. In 1869 a French Neurologist, Dr. Jean-Martin Charcot, identified ALS and 152 years later, here we are. He is considered to be the “father of Neurology.” I don’t know if geneticists can trace our afflictions back to our ancestors. They had much shorter life spans back then than now, and medical practices did not yet have antiseptic surgical protocols, they did not have the technology to analyze genes, and they did not have antibiotics, which we have been overexposed to. The recent pandemic has had more devastation to elderly people. Researchers and scientists are anticipating more severe pandemics in the future. Consider what we have experienced in the past.

My current Neurologist has prescribed Riluzole for me to slow down the degradation and progress of the ALS destruction of Neurons. There is another medication, Radicava. A couple of possible cures have finally been developed. Researchers in the Northwestern University in Chicago have come up with NU-9 and the Wave Life Sciences corporation in Cambridge. MA, has developed WVE-004 dealing with C9orf72 gene mutations. NU-9 will not be tested until 18 to 24 months, WVE-004 may be tested on humans later this year. I am also curious about CRISPR and stem cells, some of which have been taken from afflicted people’s MSC in their bone marrow. (NeuroNata-R – NCT-01363401) which is injected into their Cerebral Spinal Fluid with helpful results.