The Multiple System Atrophy (MSA) symptoms are increasing exponentially. A while ago I asked Leo to write an update of the blog “How are you doing?” The fact that he hasn’t gotten around to this yet indicates that he is getting more and more fatigued. ‘Central fatigue’ is one of the symptoms of MSA. In addition there is the fact that every single human activity becomes more and more difficult. Everything takes more effort. Things that were easy to do are now impossible to accomplish. Unfortunately this also seems to affect the laws of synergy in the opposite direction that is …
Also eating is becoming more and more difficult. Leo continues to feed himself as much as possible, sometimes I assist him. Chewing expends a lot of energy. We have already taken the blender out of storage so we can use it when the time comes. On the positive side Leo no longer gets tube feeding. His weight is at the right level now, which is why it could be stopped. But the PEG (a small tube into the stomach) is still in place. We use the PEG to hydrate Leo (like watering a plant). Drinking of course is very important. Leo likes a drink from time to time and he also has a sweet tooth.
The stoma functions well. I can tell you that it gives us a lot of pleasure. Like with the suprapubic catheter no more need to go to the toilet. The stoma allows Leo to eat everything that he is able to chew. All constipation issues have been resolved.
Leo’s is experiencing more problems with his airway and breathing. He is under treatment by the lung specialists of the Centrum voor Thuisbeademing (CTB) of The Erasmus University Medical Center in Rotterdam. At night he sleeps with a Bilevel Positive Airway Pressure (BiPap) machine. That is a device that regulates high and low diastolic pressure to ensure that the air will reach deep into the lungs. The BiPap does not deliver oxygen. But there is still a rattle in the lungs which can be a sign of phlegm. Leo is unable to cough out this phlegm.
For this reason we have a Cough Assist Machine at home, which Leo uses a couple of times a day. When he inhales, the machine gives him air (positive pressure) to help expand his lungs. When he exhales, the machine creates a sucking force (negative pressure) that pulls the air out of his lungs. This rapid change in pressure during the inhaling and exhaling loosens mucus in the lungs and causes a coughing reflex. The pressure of this machine is higher than the BiPap, which assists normal breathing.
With the Cough Assist Machine Leo must try to initiate coughing spells. That only works part of the time. Before he goes to sleep and in the mornings Leo’s uses a nebuliser to administer Budesonide (BUD) a medication that helps prevent infections of the lungs. After the nebuliser has operated for 10 minutes it is connected to the BiPap so that the mist containing the BUD can reach as far into the lungs as possible.
Despite all these measures things aren’t improving. Photos have been taken of the lungs, but everything looks normal. As an extreme measure we can consider a Tracheostoma, a surgically created hole into the windpipe (trachea). It provides an air passage to help you aspirate, but Leo refuses this. A tracheostomy involves another surgery from which to recover and with which you have to learn to live and it means an immediate need for more intensive care. Leo is of the opinion that there is a limit to the procedures he will undergo. He knows that his condition will worsen and that the quality of his life will deteriorate. It is highly likely that Leo will die of pneumonia, which he prefers over prolonging life with as many procedures as possible. In English “Old Man’s Friend” is a popular term for pneumonia in elderly patients with strokes or other debilitating illness, which allows them the dignity of a quiet death, often while asleep.
In the meantime it is also clear that the “M” in MSA means Multiple. Last Wednesday we visited the neurologist who explained that Leo has different kinds of pain. Like pulling a new rabbit out of a hat Leo has had extreme pains in his arms, hands, and legs, especially at night. It starts with prickles that develop into a stabbing pain and ends in a burning pain. The painkillers Leo now takes, Fentanyl patches and Ibuprofen, do not help. Leo wakes regularly at night because of the pain. Therefore he now gets Lyrica (pregabalin), the drug treatment for neuropathic pain. We hope this medication will help. If it does, of course I will be very grateful to Pfizer again …
By day light Leo actually looks quite well. But appearances are deceiving. Simple things such as sitting in his chair is often very uncomfortable and tiring for Leo. Also a wheelchair has its restrictions, even if it is totally shaped to his body with orthopaedic adjustments.
How about sleeping? When I wake Leo in the morning and remove his mask, I see a distorted, tensed up face. All his muscles are numb—in his arms, legs, face, everywhere. It takes time before Leo can speak. Everything is frozen. That is why in due time we will request muscle relaxants from the neurologist. Funny that I continue to use “we”, but that goes automatically.
Because Leo has difficulties falling asleep he will start using sleeping pills. We need the advice of the lung specialist of the CTB to determine which ones can be used. In short we are trying to find the correct cocktail for Leo with the intend to reduce the most pain and symptoms.
Leo now uses Sinemet (Carbidopa-Levodopa), Droxidopa (Japanese pills against orthostatic hypotension), Ibuprofen and Fentanyl against pain and Lyrica against neuropathic pain. Possibly soon to be supplemented with Zolpidem tartrate (sleeping medication) and a yet to be determined muscle relaxant (the neurologist first want to build up the Lyrica). Yes, it is true: I am detailing all these pills, but this blog will be read by other MSA patients and/or their family and friends. They often are curious to know what medications Leo uses and how he is doing.
Walking is becoming more laborious. Leo tries to walk with the Rollator once or twice a day. That takes a lot of effort. His posture is bent over and he walks slowly. But he manages. He is not capable of standing up for very long. He does not have the strength in his legs. The most difficult moment is when he has to move from the Rollator into his wheelchair. It is difficult to turn because so much coordination is required. If it takes Leo too long he loses the strength in his legs, turns even more slowly, and can no longer lift his feet. Then I have to act fast. And the word “fast” no longer applies to Leo. We are keeping a close eye on these developments. We have a patient lift at home for when the time comes.
In the mean time Leo is active on the English MSA Patients Forum (MSA Trust) where he is trying to give information and offers guidance to new patients and their caregivers about the forum and the grim MSA world. Leo lets them know that MSA is not immediately the end of the world. But we are constantly confronted with the deaths of MSA patients. I also have contact with some of the children and partners of MSA patients. (*)
We also try to occupy ourselves with other activities; for example, with the garden. And a while ago Leo suggested we start looking for a new kitchen, in order to work on a project that has nothing to do with illness. We make trips from time to time and receive visitors. Those are the good times, but it is becoming more and more difficult.
We have our setbacks. They include troubles with the wheelchair; such as interruptions in the computer navigation system; a short circuit in the wiring, which almost caused a fire; the new arm pads. There are the try outs of; the new Vicair mattress; the new sliding sheets for Leo’s bed. And the request for another new movable headrest and armrests for the wheelchair (see header photograph; uncomplicated brain surgery)
Other less routine items also keep us busy. For instance, we are researching the possibility of Leo becoming a brain tissue donor. If that is possible, it is important that the brain tissue be used in MSA-related research. Can Leo be an organ donor as well? And are both possible if Leo chooses euthanasia at home? Organ donation is only possible if you die in a hospital … Et cetera …
I understand that the next fraught sentence may be difficult for some to read. From the beginning it has been no secret that when the suffering becomes too much Leo will consider euthanasia.
Many things to discuss. And the conversation in itself is complicated as well since Leo has almost lost all speech. He uses a text-to-speech device. Slow but efficient. But you need to be able to summarise the things you want to say….
Yes from time-to-time we do get tired of everything. There is no moment to relax when you have to deal with a progressive illness. The only constant is change. In this way it is different from a chronic illness. Without minimizing chronic illnesses, I imagine that with such illnesses it is possible to optimize certain aspects. Although you can also experience setbacks and complications with chronic illnesses.
We’ll manage with the person-related budget for care at home (PGB), friends, family, and the wheelchair accessible van. Yesterday we went on an outing to Rotterdam with our van. We attended a performance by Gare Du Nord in our favourite theatre Lantaarn ’t Venster. Leo rested during the day, a nap of an hour and a half with the BiPap. It was worth the effort. And of course it was a late night.
Back home “nadieselen” (**) as Leo calls our little after talk with a nip of Cuarenta y Tres (a sweet spanish licor; we hope one of the 43 herbs does the trick). Then we call the taxi (stairlift) and I attend to Leo and get him into bed. The light is turned off at 1:30 a.m. We look back on a beautiful evening, tired but very satisfied.
And how does it all affect you? I tell Leo that this blog posting is again an enumeration of physical inconveniences. I deliberate with him. Shall we also post something about our states of mind? Leo considered this and says: …“No, that is too comprehensive”… .
I understand what he says. Thoughts and feelings. Where to begin….
(*) So last Saturday we met Milvia Luijendijk. She lost her father five years ago to MSA. Because she feels that not much is known about MSA, she started to search for other MSA patients after her father’s death. She made a photo journal about Jan Stoof, who died of MSA last May. Jan also wrote a blog. Milvia, who is a photographer, had followed him for a couple of years and documented the progression of his illness with her photos. Using crowd-funding she collected money to make a book of her photos of Jan, including Jan’s texts from his blog. We had a look in the photo book and recognised the similarities between Jan and Leo. The book has a meaningful title “Have you fallen yet?”. Here and on Facebook you will find more information.
(**) When the engine continues to run for a bit after you have turned it off.
This blogpost was translated into English since we imagine it could be of help to other MSA-patiënts or carers. Please let us know if you appreciate this. This will help us to decide whether or not to translate future blogs.
We thank you for sharing these personal details with us as well as anyone dealing with such a disease. What a challenge! You may wish to consider Zopiclone for sleep for Leo. I take it along with Lyrica. It is nice as it is a fast ‘to sleep’ and there is no lagging effect when you wake up.
We SO wish that we lived close so we could just pop in for 5 minutes for a quick cup of coffee and visit!
Pat’s sister was just diagnosed with a similar illness. I just can’t bring to memory the name of it right now but it affects her ability to walk, speak and eat etc.. It is very similar to ALS.
Please give each other a big hug from us.
Pat and Ria
I am glad we found friends who translate the blogposts for me. Glad you will be able to read and understand although you could probably get the rough lines when written in Dutch. But you miss a lot I pressume if written in Dutch.
Thank you for the suggestion about the Zopiclone. I will tell Leo. You see, that is also a benefit of this blog in English….. Good to exchance information.
I would love it if you live close. Please note that the holiday houses are very cheap nowadays!!!
I am sorry to learn about Pats sister. Must be so difficult. There are more devastating illnesses I am afraid.
I will hug Leo from you and from myself 😉
Hey Syl, I appreciate your updates so much. We think of you both very often and wish we had more time with you. Mary has Progressive Supranuclear Palsy. She is the youngest of us three kids, and so it has come as a shock to me. Thank you also for conveying hugs…we would have loved to give them in person.
Leo. You are an amazing man.We and a handful of others kept the MSAT Forum alive for several years. I could not have survived without your input and that is is how many others felt as well. You are always so positive in your fighting of The Beast
Love to you and sylvia, your lovely lady