Jun 282016
 

I was diagnosed with inclusion body myositis 20 years ago. Throughout this blog I have recorded the various stages of weakness and disability that I have experienced. I have always wondered how the story would end. Now I know. I hope this will help others suffering from my condition.

The past few months I have noticed the effects of my weakness to a much greater extent. Especially with respect to being able to hold myself upright when sitting, the ability to breathe, and most importantly, the ability to swallow. People have suggested a feeding tube however that would have meant moving to a different type of facility and a much lower quality of life. Therefore I decided that would be something I would never do.

Two weeks ago I woke up with pain in my left side that greatly restricted my ability to take a deep breath. I also seemed more congested than usual and attributed it to a cold. However after a week the cold had not gone away and I was spiking high temperatures at night. Eventually I decided I had to go to the urgent care.

At urgent care they took a chest x-ray and said I had aspiration pneumonia in three places in my lungs: upper right, upper left and lower left. I was immediately transported to the hospital where they began giving me their strongest antibiotics IV.

Their speech therapists evaluated my ability to swallow which was made much worse by the hospital bed that did not give me the lateral support I need in order to sit upright and swallow. So she said they would not be able to give me food or water. After two days the doctor came in and said I was not getting better and that I would never get better because they could not get nutrition into me. He suggested a feeding tube but added that even that might not be any difference. Of course I refused the feeding tube. He then told me my best option was to return to my assisted-living facility under hospice care. They would continue the antibiotics and I would be able to eat and have a chance to fight off the pneumonia. However he cautioned that under even the best of circumstances there was a good chance that this would be the end of my life. He was a very good doctor and went on to say that I had been fighting this inclusion body myositis for many years and that now it just might be time for me to stop.

I am back at Huntington Manor now and despite the antibiotics and being able to eat (although not wanting to) I am back to running high fevers at night which is a sure sign the pneumonia is continuing to worsen.

I am on hospice and they will keep me comfortable as I complete this journey. I have made many online friends over these years and I value those friendships deeply. I simply couldn’t leave without saying goodbye.

Jun 112016
 

mapI don’t know how often pulse oximetry and seismology come together but it happened to me a couple of nights ago. I use a ventilator at night because inclusion body myositis has dramatically lowered my ability to breathe. During the day I get by okay, but when I lay down at night, I don’t do a good job of getting air in and out. Recently I noticed that I was more fatigued in the late afternoon and even dozing off to sleep when I didn’t want to. This made me wonder whether my settings were still good on my ventilator or if they needed to be increased. I knew from past experience that this would be a multi step process. First I needed an appointment with my pulmonologist to get an order for overnight pulse oximetry. Then go to the sleep clinic, another two hour bus ride (four hours round trip) to pick up all of the equipment and bring it home. After spending the night with the oximeter recording my pulse oxygen level I would have to pack it up and make the same bus trip once again to the sleep clinic. Altogether this would cost me $27 in bus fares, plus 3 lost days.It would also cost Medicare a lot of money to pay for the doctor visit and the equipment rental. Surely there must be a better way.

Once again I turned to Amazon. I purchased an oximeter, the iOX, that would plug into my iPhone microphone jack. During the night as I charged my iPhone, I would also be recording the oximetry data. Cost? About $45. The next morning I emailed the data to myself, opened the data file in Excel and reviewed it.

I enjoyed being able to have access to the data for myself rather than just waiting for a phone call from the clinic to tell me if my machine was working okay. I learned that my heart rate slowly dropped as I fell into a deeper sleep, starting with my normal resting rate of 60 beats per minute and then eventually reaching the low 50’s to high 40’s. My respiration rate showed a similar pattern, starting at around 15 and then stabilizing at about 12. My pulse oxygen remained consistently around 97 to 99% and only briefly dropped into the mid 90s for a total of about 30 seconds.These are all good numbers and give me confidence that I can continue to rely on my ventilator.

Chart copyNow here is the seismology part. There was a 5.2 earthquake at 1:04:39 that morning with its epicenter near Borrego Springs, about 50 miles from here. The shaking in my location began about 20 seconds later (seismic waves travel through the Earth’s surface at about 2.5 mi./s). You can see my response on the oximetry data. It woke me slightly after 1:05 AM, although I did not stay awake. In fact by morning I had forgotten about it until I saw it mentioned on the news and then remembered being awakened. My heart rate briefly rose to my normal waking rate of 60 and my respiration rose from 12 to 18, but within less than a minute both returned to sleeping levels.

I have included a link to the oximeter I’ve used in case anyone is curious. A word of caution: I do not have any heart or lung conditions that I know of and so I feel confident with relying on the data I obtained. Others might want to consult their medical professional.

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Apr 142016
 
Back where I belong.

Huntington Manor assisted living in Poway, California.

I recently returned to Huntington Manor assisted living after spending 15 months at a much larger, more expensive facility which I will refer to as “The Other Place.” People have wondered why I came back to Huntington Manor and, if I like it better here, why did I move in the first place? These are good questions and my answers may interest you.

Huntington Manor is a 27-bed assisted living facility located on the outskirts of Poway, California. My wife and I moved here in the spring of 2012 because it was the only place we could find that would accept my overhead lift system and was priced low enough that we could afford two rooms. My wife died a few months later but I remained here for another two years. I liked the location, had become friends with the owner, had gotten to know the staff well and enjoyed working on their website and blog.

Is this all there is?

My family felt that I was not taking advantage of some of the extra amenities that were available in larger facilities. So every once in a while I would check out a few of them, however I always got the same answer, “Sorry, but we can’t take care of someone as disabled as you and we definitely don’t want your overhead lifts in our facility.”

One day I was told about a place near the coast that might accept my physical requirements and my lifts so I checked them out. Like the other large facilities, they promised an extensive activities program, restaurant–style dining, frequent excursions, and several common areas where residents could mingle. When I asked about my overhead lifts, they said they would have to check it out with “corporate.” At the time I didn’t know exactly what that meant but I certainly do now. After several weeks they told me I was approved. But could I afford to live there?

Large assisted living facility

Could I afford to live this large?

Sticker shock.

Do you ever see those reports about assisted living that show the average cost by state and think “that’s not so bad.” It wouldn’t be if those were the actual costs, but sadly they are not. When the larger facilities publish costs or give them out over the telephone they are quoting the base rate for a room and the most standard care level available. (Smaller facilities are less likely to use this tactic.) There are very few people entering assisted living who could live with that amount of care. If you have difficulty transferring or walking or keeping track of appointments and medicines or dressing or feeding yourself, you will pay a lot more. The way it works is the facility will do an “assessment” and assign points for each type of care you need. Would you like help taking a shower? Do you have difficulty cutting meat? Do you have other bathroom issues? Based on the number of points, they will assess you an additional daily rate. In my case, the basic charge for the room was $140 per day, but the added charge for providing me assistance was $80 per day. That meant I would be paying $6600 per month for those months with 30 days — much more than I was paying at Huntington Manor. Nevertheless, they were advertising so many extra services that I felt I should try it out.

Corporate greed trumps senior need.

If I had known what was going on behind the scenes, I would have made a different decision. I had been given the standard story –a married couple started this chain of facilities out of a desire to create lodging for seniors that felt more like homes and less like hospitals. This was true, and from the stories I heard from people who had worked there for many years, life used to be very good for both the staff and the residents. But then came the economic downturns and the big corporations smelled blood in the water. Today The Other Place is owned by a real estate investment trust (REIT) that packaged several hundred senior living facilities together and offers shares to investors. They have no interest in the business or the residents — but they certainly worry about their shareholders. They contract with large property management corporations to run each of the facilities as profitably as possible. How do you get more profits? You cut costs and raise rates.

I experienced the cost-cutting first. This showed up in the number and quality of the activities offered, the quality of the food, and the number of caregivers available.

Activities – not my idea of good times.

The main reason my family wanted me to move to a large facility was for the activities. Even before the cost-cutting, I discovered that most of the activities were designed around the needs of people who were much older and especially for those who were suffering from early stages of memory loss. I’m sure my children picture me have a lively conversations, going to movies, developing close friendships, etc. Sadly, that did not turn out to be the case. Most of the activities focused on either playing bingo, or playing simple word games. There were crafts every couple of weeks but they were usually at the level of early elementary school. I understand that I was not the target audience for their program. Assisted living activities programs are mostly for people who are over the age of 80, female and with some form of cognitive difficulties.

The one program I truly enjoyed was the music. They brought in professional musicians who performed and encouraged residents to join in singing. Of course that cost money and it was one of the the first programs to be affected by budget cuts. The number of acts was cut in half and some performers just quit coming because the pay was too low. There were monthly excursions and weekly trips to local restaurants but I preferred my wheelchair and public transit for the independence it gave me. Besides, many of the residents needed to be watched carefully, so any trips away from the facility required at least one caregiver for every two residents to make sure no one wandered off. This limited the number of participants to about ten residents.

Dining

I had heard stories about the elegant dinners they used to serve, and you still see pictures of food from those events posted on their website. I never saw any personally however because by the time I arrived all menus were being planned from the central headquarters back east and the local chef was given almost no authority to change the menu or improve the quality of food being offered. In fact, the menus were printed a month in advance and shipped to the facility to be displayed for each meal. After a few weeks, everything seemed to taste the same. That was because many of the foods were based on the same packaged ingredients. For example soups were made from one of four stock bases. Very few items were cut from scratch in the kitchen.

No turkey loafing at Huntington Manor.

No turkey loafing at Huntington Manor.

At Huntington Manor, the first step in making chicken soup is to boil some chicken. At The Other Place, most turkey or ham dinners come from deli loafs of turkey or ham. Here, they come from turkeys and hams.

Cuts in caregiving.

Perhaps the saddest result of corporate greed is what happened to the caregivers and their relationships with residents. When I arrived there were seven or eight caregivers on duty during the daytime hours. They were charged with caring for approximately 70 residents. By the time I left, many days there were only five caregivers for the same number of residents. If you are doing the math you’ll see this works out to one caregiver for every 14 residents. Subtract from that the several hours per day they must spend on other tasks such as working in the dining room and there is obviously little time to get to know a resident well. By contrast, at Huntington Manor we have five caregivers on duty during the daytime for 27 residents – – an average of about one caregiver for every five residents. At night, the contrast is even more dramatic. The Other Place has two caregivers on duty at night for all 70 residents. Huntington Manor has three on duty for 27 residents.

At both facilities, the people who actually provide the care are competent and hard-working. Many of them truly enjoy what they do and like to help people. Because the pay levels are so low they typically work two or more jobs to support their families. They deserve our respect. Unfortunately many of the residents have personality disorders that go along with dementia, making them difficult to care for. In my four years of living in facilities, I have never experienced any kind of abuse but I have certainly seen caregivers on the receiving end of it.

Where does the payroll go?

Something else that comes along with bloated corporate ownership is bloated staff. It’s wasteful but may be necessary to keep up with the record-keeping and regulatory demands of a big corporation. Where I am now we have four staff who are seldom involved in direct patient care: the owner, the administrator, the cleaning person and the gardener. At The Other Place most of the common areas are filled with offices. There is the executive director plus people in charge of the business office, sales, assisted living, intermediate memory care, health services, activities, food service, maintenance, and reception. Many of these have their own staff. Every two months or so a carload of impeccably dressed executives descends on the managers and hovers over them making sure every procedure is carried out according to the corporate book. I’m sure the regional managers fly back to headquarters every so often for some of the same treatment. Almost lost from sight are the five or so caregivers themselves.

The big price increase.

When I was negotiating the price for The Other Place, I was given a chart showing price increases during the past several years. They averaged about 2% which made me feel comfortable that I could manage them. After a year I got my first actual price increase. It was just under 10%! Doing some investigating, I learned that a dollar increase of about the same amount was likely for the following year. There was no way I could afford to live here unless I changed to one of the smallest rooms. To do that I would need to reinstall my overhead lifts – an expense of about $2000 and adjust to a cramped lifestyle and wait for the next round of cost cutting and price increases. It so happened that I heard from Huntington Manor the next day asking if I would like to return. I really didn’t have to think twice. So now I am back here and far happier. I miss my favorite caregiver at The Other Place but I am back with good friends that I had here before. If only I had known all of this beforehand, it would have saved me tens of thousands of dollars.

Conclusion:

Despite my experience, I am not recommending people avoid larger facilities. For their target audience, they are providing the services those people seek and can afford to pay for. If someone has Alzheimer’s disease, it could be a very good choice. However, for those of us whose challenge is mostly loss of mobility, we may need to be more creative in our selection process. The big chains now control the largest portion of assisted living beds, but there are many intermediate and small privately owned facilities. Here in California, six-bed Residential Care For the Elderly (RCFE) properties are everywhere. They are less strictly regulated and can offer personally tailored types of care sometimes at significantly less cost. I was unable to find one that could meet my needs, but not many people are as mobility-challenged as I am. If you are facing the prospect of needing more care then you can receive at home, don’t be discouraged. During these past four years the good moments have far outweighed the bad and I’m looking forward to more.

Mike Shirk

Feb 252016
 
On the left, the way we were in 1996. On the right, with some of my grandchildren and great grandchildren, late last year.

On the left, the way we were in 1996. On the right, with some of my grandchildren and great grandchildren, late last year.

Normally, my “Chronicles of Disability” consists of annual reports on the changes in my health over the previous 12 months. I forgot to post a report for the year 2014, but perhaps it’s just as well because there were very few changes – – just more weakness in general. So now we come to this major milestone. It has been 20 years since I was diagnosed with inclusion body myositis (IBM). This rare muscle wasting disease is described as “slowly progressing”. That may be true one month to the next or even one year to the next. But when the person I was in 1996 is compared to who I am today, the contrast is jaw-dropping.

Twenty years ago I didn’t think there was much wrong with me. Yes I was slowing down in my running, and my golf shots seemed to be getting shorter, and I did fall once in a while, so what? I was 55 years old, just normal aging? I could still hike mountain trails, jog (slowly), show up for work every morning, work around the house, go to parties with my wife, take long driving vacations. Life was very good.

Now, I very nearly meet the criteria of a quadriplegic. I can’t move either of my legs or my left arm. I can only raise my right arm a few inches above my waist. I cannot stand, walk, or transfer without the aid of an overhead lift system and a caregiver. This will probably be the last year that I am able to continue feeding myself, unless the new drug (BYM338) gets released and actually works. My fingers don’t bend and my speech is getting quite weak. This is making my writing avocation more challenging and I may need to give it up within a year or so. Unrelated to my disease, but definitely affecting my life, my wife died of her own rare muscle illness in 2012.

My current home features the ultimate "open floor plan". My wheelchair loves it.

My current home features the ultimate “open floor plan”. My wheelchair loves it.

At the time of my initial diagnosis, we were living in a two-story four-bedroom home overlooking the mountains of southern California and a little slice of the Pacific Ocean. Today I am living in 250 square feet in an assisted living facility. The room is comfortable, the view isn’t bad (mostly of an ancient olive grove), the caregivers are friendly and helpful, and the food is very good. My days are spent doing what writing I can, either for Huntington Manor or for my Life Disabled blog, but that work is getting more difficult every day. So instead I am catching up on a lot of movies and television and doing a little reading. I also like to take my wheelchair out on long jaunts through the countryside and down to the local business district of Poway. Huntington Manor is launching a major renovation of the facility and I have been promised one of the beautiful new rooms that will overlook the garden and the hills beyond. That is enough to keep me motivated to stick around until the project is finished in 2017.

When I first started this blog, and when I wrote “Rolling Back: Through a Life Disabled” I suggested that the newly diagnosed read about my experiences to be properly prepared for what lies ahead. Now with a new treatment on the horizon, it is quite likely they may never have to experience this severe of a decline.

I have reposted many of the pictures and captions from the past 20 years. I think they tell an interesting story about the effects IBM has had on one person’s life. As you’ll see, I have remained generally happy and hopeful throughout that time, but I must admit that my general mood has been declining. Recently, I saw a neurologist who lowered my expectations for the new drug by pointing out that it would not be of any use for the muscles that were already dead and that, in my case, most of the muscles are completely destroyed. The most I can hope for is maintaining the minimal capabilities I have now.

By the way, one of the special pleasures I get these days is when someone purchases my book. It’s available on Amazon — just click on the link on this page — seven dollars for paperback and three dollars for the Kindle edition, or free if you are using Kindle Unlimited.

Nov 192015
 

My new/old home is calling me back.

My new/old home is calling me back.

My former assisted-living facility has been tugging at my emotions for some time. Then my current facility gave me a swift kick in the wallet. So I am moving back to Huntington Manor in Poway, the place that originally welcomed my wife and me with all of our disabilities, where I was living when she died and just a mile from where her ashes are buried. Compared to my current facility, it is one fourth the size, 30 miles further inland, and more country then luxury. But it is also much closer to my eldest daughter and many of my friends, more flexible in the kind and amount of care provided and surrounded by the mountains and trails I have enjoyed for so much of my life. So when I received the letter from Sunrise telling me my rates were being raised by nearly 10%, the message was clear: “Go!”

I can’t have my old room back with its gorgeous view, but they are doing all they can to make me comfortable in a different arrangement. I will be occupying two rooms that were recently constructed as part of an overall improvement project. (You can see them in the background of the photograph.) In a few weeks, once I am settled, I will share some of the insights I have gained into the big business side of assisted living. One little preview – – instead of a family–owned chain of facilities, I have actually been living in a real estate investment trust, VENTAS, comprising many hundreds of properties, each being run by property management firms, with the primary emphasis on returns to investors. Exactly what that means to the residents, and the hard-working caregivers who serve them, will be the subject of my next blog post.

Aug 312015
 

The interest level and expectations surrounding BYM338 are evidenced here.

The interest level and expectations surrounding BYM338 are evidenced here.

All of us with inclusion body myositis are waiting anxiously for some sign of results from the Novartis BYM338 trials. After all, until now there has been no proven treatment available for this relentlessly progressive muscle wasting illness. The critical stage III worldwide trial is wrapping up later this year and so far all we have are anecdotal remarks from participants. Of course they can only guess because the study is blind, with a fourth of the participants getting a placebo and another fourth getting a dose that is very low. The good news is that some people are reporting results that to them seem significant enough they believe they must be getting either the middle or highest dose and it is working.

However there are places we can look to find real data. For example, a study report published in Current Rheumatology Reports and made available through Springer.com contains some actual results from the phase IIa trial on patients. This was a small trial of 14 participants and of them 11 received the drug. It states that the participants who received the drug were given a dose of 30 mg per kilogram of body weight in a single infusion. The results were then measured at two months and again at three months. After two months, the average person receiving the drug gained about 7 percent of thigh muscle volume. Walking distance in six minutes was measured another month later and the average drug recipient gained 15 percent in walking speed. Both results were statistically significant. The dose was three times greater than the maximum dose being used in the current stage IIb/III dose-finding trial, (although it was only a single dose while the trial consists of monthly doses.) According to the Brookings institute, these results were what prompted the FDA to grant Breakthrough Status to BYM338.

The results of an early study of BYM338 are reported here.

The results of an early study of BYM338 are reported here.

Another place we can look to obtain hints about the success of BYM338 is in the other trials that Novartis has been conducting. The ClinicalTrials.gov website lists eight studies that have been completed or are ongoing plus one that was withdrawn. Among the conditions for which the drug is being tested are IBM, sarcopenia, and cachexia related to chronic obstructive pulmonary disease, lung cancer and pancreatic cancer. Sarcopenia is the skeletal muscle wasting that occurs in most people beginning at age 30 and accelerates as we age. It is classified as an illness when the muscle wasting exceeds two standard deviations from the norm. Cachexia is the wasting of muscle plus fat that results from many serious illnesses. Considering the high cost of conducting all these trials, it is clear that Novartis has high hopes for the drug. As do we!

Questions:

1. In the Novartis quarterly report presentation for Q2 of 2015 they continue to state that FDA submission of BYM338 for sporadic inclusion body myositis will be in 2016. My physician was told by Novartis that they were accepting no requests for compassionate use for this drug prior to FDA approval. Might that policy change once the submission to FDA has been made? (Compassionate use, also called extended use, is an FDA program to make drugs available to seriously ill patients when no other medication is available. FDA states that compassionate use is available for drugs that have not been formally approved nor proven effective, so it is a mystery why Novartis has chosen to withhold this particular drug from the program.)

2. As I understand the mode of action of BYM338, it prevents myostatin from signaling muscle cells to stop growing. This allows the cells to resume their normal process of regeneration (myogenesis). Although it doesn’t cure the IBM, it hopefully will allow the body to build new muscle cells as fast or faster than IBM destroys them. Of interest to those of us where the disease process has resulted in near paralysis, how much muscle fiber must be remaining for myogenesis to take place?

3. The trial with mechanically ventilated patients was withdrawn. Does this mean there was some side effect related to mechanical ventilation? Unfortunately, some IBM patients may become too weak to breathe without mechanical assistance.

Jul 182015
 
"Point Pinos" is one of two of my paintings (along with one of my wife's) hanging in the permanent art collection of the Muscular Dystrophy Association.

“Point Pinos” is one of two of my paintings (along with one of my wife’s) hanging in the permanent art collection of the Muscular Dystrophy Association.

Ten years before this blog began, and several years before the term “blogging” came into general use, IBM had taken my life in a new direction. Besides forcing me to give up a career as a writer and advertising agency owner, it had herded me into a new career as a watercolor artist. Within months of closing the doors to my ad agency, I was opening books on painting techniques, design theory and color harmony. I joined an outdoor painting group, then became a member of the San Diego Watercolor Society – one of the largest groups of its kind in the world – and soon became a board member and eventually president.

This was   my booth at the Hotel Intercontinental in 2003 for The Myositis Association national conference.

This was my booth at the Hotel Intercontinental in 2003 for The Myositis Association national conference.

This new avocation gave me such pleasure and added so much meaning to my life that I had to share the news. I talked to members of our local Myositis Association (TMA) support group and they encouraged me to take my message to the national conference that was coming up in Houston. This was in 2003. The organizers of the conference also encouraged me and gave me a booth where I could display several paintings and hand out literature.

 "Monterey Bay" (15 " x 22 ") will be auctioned  at the 2015 national conference of the myositis Association to raise money for research.

“Monterey Bay” (15 ” x 22 “) will be auctioned at the 2015 national conference of the myositis Association to raise money for research.

Twelve years later, I have once again been approached by TMA to participate in their national conference in Orlando. Of course I cannot travel there, but one of my paintings is making the trip, along with this updated version of my literature (below). The painting will be auctioned with the proceeds going to benefit research into myositis. One session of the conference is about finding purpose in life following disability, so I am very pleased to participate in this small way.

 If you click on this image of the front and back cover it will make it large enough to read.

If you click on this image of the front and back cover it will make it large enough to read.

 This is the inside of the brochure where I tell the story of becoming an artist after being diagnosed with inclusion body myositis.

This is the inside of the brochure where I tell the story of becoming an artist after being diagnosed with inclusion body myositis.

They titled this session “Act II” but if I could be there in person I might add that myositis is not necessarily a two act play. It has been nearly 5 years since I was forced to give up painting due to seriously declining strength in my hands and arms. Of course that led to yet another exciting pursuit – – writing a blog and a book taking advantage of voice recognition technology and electronic publishing. (Which reminds me, my book has been published for nearly a year and a half now and sales have slowed down considerably after beginning with quite a flourish. I didn’t publish the book to make money, in fact I have given away nearly as many as I have sold, but I am curious why it seems to have been forgotten. It was written to help people with myositis, especially those newly diagnosed, because this is not a common illness and coping with it is not a common skill. You don’t even have to pay for the book if you don’t want to because Amazon Kindle owners can read it for free. I’ve included the link below.

Jul 042015
 

 This cutaway view shows how the pool noodle is placed under the fitted sheet at the side of the bed.

This cutaway view shows how the pool noodle is placed under the fitted sheet at the side of the bed.

As my legs continued to become weaker I started waking up with sprained ankles. My feet would simply fall over toward the outside, stretching the tendons. I knew there were special boots you could buy to wear in bed, but that would add just one more complication to an already very complicated nighttime routine. I discovered the solution while watching the activities director at our facility leading an exercise class.
Seeing an exercise class using these pool noodles gave me inspiration for solving my falling foot problem.

Seeing an exercise class using these pool noodles gave me inspiration for solving my falling foot problem.


She had purchased some “pool noodles” which they were using to strengthen their hands and arms. I realized that having one on either side of my bed would give me a comfortable place to rest my feet. One of the advantages of being nearly completely paralyzed from the waist down is that once a foot is placed in bed, it won’t go wandering around during the night.
 Before I made this modification, my feet were to  weak to rest in an upright position and would fall over during the night. By morning both ankles would be sprained.

Before I made this modification, my feet were to weak to rest in an upright position and would fall over during the night. By morning both ankles would be sprained.


So for three dollars, I bought one of the 3 inch diameter noodles, had it cut in half and placed at the foot of the bed on either side under the fitted sheet. I’ve been using it for several weeks and it works perfectly.

Jun 192015
 

There seems to be some optimism for the future of the new Novartis drug BYM338 currently under investigation for its efficacy and safety as a treatment of inclusion body myositis.There is nothing official, but anecdotal remarks from study participants and others who may have connections within the study point toward encouraging news.

I have heard some people speculate that even if BYM338 is proven effective, it is too late for those of us with more advanced disease. I would like to express a contrary opinion.

My inclusion body myositis has progressed quite far yet I still see potential benefit that could come from BYM338 should it prove effective. After nearly 30 years with IBM (19 since diagnosis) all of my muscles have been affected to some extent. The earliest affected muscles are the worst, such as the quadriceps and finger flexors, but all the rest are gradually growing weaker. At this point, each loss of muscle results in a significant loss of function. For example, the weakness in my diaphragm and core muscles is significantly reducing my ability to breathe and sit upright. The last time my inspiration and expiration strength were measured two years ago, both were only 30% of the low limit of normal. In the past few years, the additional loss of strength in my biceps took away my ability to feed myself using normal motions and forced me to adapt to a slinging motion combined with tilting back in my wheelchair. The point is that once IBM has progressed far enough to cause the ability to walk or stand to be lost, this is far from an endpoint with the illness. I still am able to recruit other muscles to accomplish most of my crucial activities of daily living beyond walking through creativity and use of mechanical aids.

I can also tell that even my most seriously affected muscles still have enough living cells within them to produce tiny movements. After all, a muscle consists of many bundles of muscle fibers which themselves consist of many muscle cells. The point is that even a slight gain in strength and muscle that has been mostly destroyed could still contribute to an adaptation that is important to the patient. Or a slight additional loss of strength could cause that adaptation to be lost.

In my own case, preserving or strengthening certain shoulder muscles could allow me to continue feeding myself indefinitely. Preserving or strengthening remaining healthy muscle fibers in the diaphragm and rib cage could allow me to avoid full-time ventilation. Each of these would be benefits that could easily justify an expensive medication.

If anyone knows how to get this observation in front of any researchers or Novartis executives, please do.

Jun 162015
 
Here is my new mattress, following final inspection. You can see the individual air bladders that are inside. Once the cover is pulled over and zipped shot it looks just like a regular mattress. For the curious, the rest of my equipment consists of the electric bed, the pump unit, the DVR, Blu-ray, Roku, smart TV, emergency call button on the wall and one of the supports for the overhead lift system.

Here is my new mattress, following final inspection. You can see the individual air bladders that are inside. Once the cover is pulled over and zipped shut it looks just like a regular mattress. For the curious, the rest of my equipment consists of the electric bed, the pump unit, the DVR, Blu-ray, Roku, smart TV, emergency call button on the wall and one of the supports for the overhead lift system.

Inclusion body myositis usually begins with weakness in the legs, hands, or, sometimes, the swallowing muscles. Eventually it gets around to all of them and a lot more however. One of the results is you may lose the ability to roll over in bed. That happened to me several years ago leaving me vulnerable to skin problems. Eventually they got bad enough to need nursing help from a home health agency. One of the first things they did was order a low air loss alternating pressure mattress. It consists of 20 air filled chambers that take turns fully inflating. They also are constantly releasing a small amount of air to keep the skin supplied with oxygen. These two functions worked very well for me and also provided the best sleep I have had in years.

Unfortunately, as I reported in a post two years ago, once I was getting better, Medicare took away the mattress. As we all know, Medicare takes little interest in preventing illness, preferring to spend much more money once we get sick enough.You can read the entire original story here.

To get around their silly game I bought my own mattress on Amazon, exactly like the one they had provided. I paid just a few dollars more ($600) for the entire mattress and pump system than Medicare was paying each month.

After two years of good service, the pump that put air into the mattress gave out. (Actually, just a cheap little pressure gauge failed but that was enough to make the entire mattress stop working.) The point is what happened for the next four days until my replacement system arrived.

At first, I felt I had only two choices: don’t sleep on a completely deflated mattress, or don’t sleep on one so overinflated it feels like concrete. For the first two nights, I got no sleep whatsoever. Then on the third night I worked out a system where I could use a remote control in bed to turn the pump on and off. Of course I needed to be awake to do this effectivelyWhich turned out to be not difficult since there was almost always an alarm sounded. On those nights I got two or three hours of sleep each. Regardless, each morning my backside would be as sore as if I had been taken to the principal’s office several times.

I guess you can call this a backhanded testimonial to the efficacy of a low air loss alternating pressure mattress. In fact, you might want to buy two.