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.
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.
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 The ClinicalTrials.gov website lists eight studies that have been completed or are ongoing plus one that was withdrawn.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!
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.
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 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.
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.
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.
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.
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.
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.
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.
As a degenerative physical disease such as myositis progresses, our coping methods also progress. In the beginning there are inexpensive physical aids such as canes and walkers and often the help of a spouse or family member. Then come more expensive pieces of equipment such as wheelchairs, hospital beds, adaptive toilets, lifting mechanisms and modified vans. Next may come home renovations or moves to homes with more appropriate layouts. Next we may hire caregivers from home health agencies. Finally comes the really big decision of whether to enter a facility. Recently I have noticed more discussion about assisted living or skilled nursing facilities among the myositis community. I can only speculate that as awareness grows and diagnoses increase in number more of us have advanced to the point we can no longer live independently.
It is difficult to write a general article about the need for assisted living or the experience of residing in such a facility because there is no federal standard. It has been left to each state to create its own definitions and requirements. In some states such as California, assisted living facilities may approach the level of skilled nursing in the amount of care they are allowed to provide. In other states assisted living can only provide minimal assistance with activities of daily living. For those states with more restrictions, skilled nursing may be the only option. Of course that means more expense and less freedom.
I wrote a series of blog entries on the subject of assisted living and much of the content is still valid especially for those living in a state such as California. I have provided links to each of those blog entries below. I am continuing to do research on the subject and someday I might turn this into a book. For now I will expand on the series of articles I have already written, with special emphasis on the unmet needs of the physically disabled.
Follow these links to read more:
In my previous post, I explained that losing nearly all the muscles in my hands and arms and taken away my ability to hold the camera and press the shutter. Today I am happy to report that my occupational therapist has created a system that attaches to my wheelchair and restores my ability to do photography. Actually, it turns my wheelchair into a rolling tripod. Couple that with the ability to tilt, elevate, and roll, and my new system gives me more capabilities for taking stills and videos than before. Please watch the video below to see how it all comes together.
This photo shows why I haven’t been able to take photos lately.
Recently I have not had much to say. No, let me correct that. I have not been saying much. I do have a lot to talk about, however I am trying to make some more adaptations to keep up with the progress inclusion body myositis is making on my body. The effects are especially noticeable on my hands and fingers, shoulders, and the ability to speak, sing and swallow. I recently got approval to go on home health and have started receiving regular visits from a speech therapist, occupational therapist and physical therapist.
Not only are my hands and fingers week, they are nearly frozen in one position. this has caused me to temporarily give up photography altogether. Some of you may recall that in the beginning I was using either my iPhone camera or my GoPro camera which was controlled by my iPhone using the GoPro app. I can no longer hold the iPhone in my hand and use my other hand to touch the screen, so that rules out both of those methods of taking pictures. My occupational therapist is working with me to try to find a system that will solve this. When he does (and I am sure he will; he is very clever and persistent) I will do an article about the method used.
My occupational therapist is also working with me to expand upon a scheme I devised to restore some movement in my fingers. I played the piano from the age of three. It was one of my great pleasures and a favorite method of relaxation. IBM took that away from me several years ago. There is a piano in the common area at my assisted-living facility and occasionally I peck away with the one finger of my right hand that is still strong enough to press a key. So I decided to purchase an electronic keyboard, a Yamaha PSR E443, that would always be waiting for me in the “office” of my assisted-living apartment. My theory was that I would be so motivated to produce music that I would play it often and perhaps expand my ability to move the fingers on my right hand. Even more ambitious, I was hoping to be able to use at least one finger on my left hand to take advantage of the auto accompaniment function of the keyboard. However, the extreme weakness of my left shoulder prevents me from using my left hand unless I lean to the right and lock my shoulder in place. Doing that leaves me unable to use my right hand. After working with my keyboard about one month, my right hand acquired enough dexterity that I can play two notes at once using the index and middle finger and then add a third note with my thumb. This is a major increase in hand function and it is also paying off with things as simple as picking up an object from my desk. I am also now able to use two fingers on my left hand, although I have not been able to overcome the problem of lifting that hand and using it in conjunction with my right hand. My occupational therapist believes this is a therapy worth pursuing and he is now working on a system that might allow me to make more use of my left hand by supporting my left arm and leaving my hand free to move. If this works out, it will also be the subject of a blog post.
My physical therapist is trying to loosen up my neck muscles which are so tight that I can no longer turn my head enough to see behind me. This is a big problem when you need to back up a 350 pound wheelchair. My speech therapist is working with me on strengthening the muscles used for swallowing and is teaching me ways to avoid further damage to my weakened vocal cords.
Early next year, I will let you know how everything is going. Meanwhile I wish you all a good holiday season and an even better New Year.
Some more advantages to using an overhead lift together with a hygiene sling.In addition to lifting someone safely and easily, an overhead lift offers some additional benefits. You might compare it to taking your car in for an oil change. Just like they put your car up on the rack to have easy access, the caregiver also has easy access to otherwise hard-to-reach areas of the person being cared for. Besides post-toileting hygiene, this helps with skin checks, skin care and changing underwear.
Changing underwear? Here’s how: while the patient is being suspended by the lift, pull the underwear around the bottom and toward the knees as far as possible. Lower the patient back down to a seat. Unhook the leg straps from the overhead lift and then bring them back up, passing them between the underwear and the seat. Lift the patient again and let the shorts fall off. Put a clean pair over the feet and legs and lower the patient once again. Unhook the leg straps from the lift. Pull the shorts as far up as they will go. Put the leg straps on the lift again, being sure they are on the outside of the shorts. One last time, lift the patient and pulled the shorts the rest of the way on. Lower the patient back to the seat and the shorts have been changed. (The process takes a lot longer to describe that it actually takes to accomplish.)What about the outer wear? Some people use open-bottom garments that are specially made for wheelchairs. Personally I prefer the type of pant that I designed which simply lays flat on the wheelchair seat and I am lowered onto it. Then it Velcros in three places – along the legs and down the front to form a complete pair of shorts that look exactly like a regular garment.
If you are not yet convinced, maybe a demonstration will help. In the interest of public decency I decided not to be the model for this brief video.
Special thanks to Jeff Connor, President and owner of Pacific Mobility, who recently presented me with a new overhead lift mechanism, courtesy of Prism Medical. He also brought along his panda to help demonstrate the advantages of an overhead lift and a hygiene sling.