I am 72 years old, nonambulatory due to a rare disease (inclusion body myositis), and am living in an assisted living facility. It took me a lot of searching before I could even find one that would accept me. Most said that they would not take care of someone who wasn’t able to stand or walk. My “neighbors” are almost exclusively people 20 years older than me and most are incapable of carrying on a conversation. (no fault of their own, disease and age have damaged important cognitive functions.) The caregivers here are very conscientious, but like most, they are overworked. I am fortunate that I can be my own advocate and thus I get very good treatment.
Every so often, I will check the surrounding area of Southern California to see if something new has arrived that might provide a more stimulating environment. At the end of each search I always return to my room and feel grateful for what I have, because it is fully wheelchair accessible including an enormous roll in shower. Most of the facilities have tiny tub-showers with fiberglass enclosures and little built-in seats that could not possibly accommodate me. As for their “activities” the larger facilities seem to focus on the needs of the least common denominator and provide “sing-alongs” featuring songs from the 40s, bingo, and various childish games. For this, they charge anywhere from $5000 – $7000 per month. By comparison, I like to spend my time studying computer programming via the Stanford University lectures on iTunes. (Or writing this blog.) I know that I am not the only person who is older or disabled but also has a continued desire to learn.
It is obvious that we need a new paradigm for the care of an aging population. As we live longer, more and more of us will develop chronic illnesses that reduce our mobility or our cognitive facilities. In my own case, the ideal solution would be to live in my own home with visiting care givers to get me out of bed and shower and into my wheelchair in the morning and then get me back into bed at night. For most of the day I can be independent except for meals, which I could obtain at a local restaurant or my local microwave. The problem comes from not knowing exactly when I might need help. If I were in bed at night and an emergency arose, what would I do? If, during the day, I dropped something important and couldn’t pick it up, how would I get help? Right now, I can press the pendant that is always around my neck and someone will be around to help me. At home alone there is no such system. The cost of round the clock in-home care is prohibitive and would be a terrible waste of human resources anyway.
My concern is that the movement of for-profit corporations into this field means that any rational and humane solutions will be forever blocked.
Getting around when your legs aren’t much help.
My wife had myotonic dystrophy. It was the underlying cause of her early death. Although we knew of her condition for the past 7 years, we were unaware just how life-threatening it could be. Unfortunately, her doctors were also ill informed since myotonic dystrophy is not a common illness. I am presenting some information here in the hopes that it could be useful, and even life extending, for others who may have myotonic dystrophy.
Myotonic dystrophy is caused by a defect in a specific area of chromosome 19 called DMPK (dystrophia myotonica-protein kinase). It is normal for protein sequences to repeat a few times, however when a particular sequence in this region repeats more than 35 times, a person is said to be affected by myotonic dystrophy. This was discovered in the early 1990s and since then, suspected cases can be confirmed by a genetic test. Myotonic dystrophy is an inherited disease. It is passed from parent to child in an autosomal dominant fashion. This means that if one parent has the disease, their offspring each have a 50% chance of also being affected. Moreover, the disease tends to become worse with each successive generation. Thus, if a parent had a mild form of the disease, their child could have a classic or even a congenital form.
Myotonic dystrophy is classified based on the number of times the protein sequence is repeated. Fewer than 35 repeats–normal, no disease indicated. 35 -100 repeats–mildly affected by myotonic dystrophy. (However, their offspring have a 50% chance of developing the illness, possibly in a stronger form.) 100 – 1000 repeats–the person has the classic form of myotonic dystrophy. Beth had 212 repeats and was seriously affected..
Until 1992, myotonic dystrophy was one of many neuromuscular diseases that was difficult to diagnose with certainty. Since that time, the availability of genetic testing means that a person can be determined to either have the disease or not. Unfortunately, having this diagnostic capability for such a short time means that there have been many undiagnosed cases and therefore there are large numbers of people today who may be at risk for the illness and not know it. In my wife’s case, we will never know for certain which of her parents had the disease, although we are fairly certain it must have been her mother. For those with the mild form, they may have a normal life span with little physical symptoms. The most typical symptom would be early-onset cataracts, the possibility of diabetes, and the typical myotonia (the inability to relax one’s grip easily).For those with the classic form, there are many symptoms affecting various muscles and organs. These include weakening of muscles in the forearms and hands, calves and feet, shoulders back and face, and diaphragm. Those weaknesses can affect a person’s ability to walk, keep their balance, perform fine motor skills with their hands, and breathe. Men may experience infertility. Women with this form of myotonic dystrophy frequently have difficulty with childbirth. (My wife suffered through many hours of unproductive labor before eventually giving birth via cesarean section to our first child.)
Weakness in the facial muscles can cause a person’s appearance to change rapidly. There can be loss of hair on the front of the scalp, drooping eyelids and an open mouth. These weaknesses can also cause frequent jaw dislocation, and difficulty swallowing.
Cataracts are very common, and an alert ophthalmologist can be the first to raise the possibility of myotonic dystrophy with the patient because the types of cataracts have a distinctive appearance when they are caused by myotonic dystrophy.
There is an increased likelihood of diabetes and cancer among those who have the classic form of myotonic dystrophy. They will also be more likely to have digestive and intestinal issues.
There are numerous hormonal consequences, including reduced sex drive, early infertility, insulin resistance, and thyroid problems. There can be increased chance of gallbladder inflammation, problems with the pancreas, and chronic constipation.
The most common causes of death for those with myotonic dystrophy are respiratory failure and cardiac arrest. Most articles you will read about myotonic dystrophy recommend yearly EKGs because there is the likelihood that cardiac arrhythmia may develop. What they don’t say, is that there should also be a 24 hour Holter study done from time to time, as this is more likely to catch an arrhythmia that comes and goes. Thanks to having an autopsy performed following my wife’s death, we now know that she had been having episodes of insufficient blood flow to her brain for some time that had not been detected by either the annual EKG or MRIs. The MRIs would have shown more if we could have used contrast medium with her but unfortunately her kidneys had been damaged to the point where contrast medium was not possible. She died when a combination of factors overwhelmed her.
It is especially important that an anesthesiologist know about a patient’s status with myotonic dystrophy as the patient will be at much higher risk of respiratory issues.
Here are some things to look for:
An unusual walking gait where the foot seems to slap down on the ground, preventing the normal flowing motion of a healthy walking motion. There may also be an increased likelihood of falling.
Early-onset cataracts, especially “Christmas Tree” cataracts on the back of the lens.
Difficulty relaxing ones grasp on an object.
Important disclaimer: I am not a medical professional and what is on this page should not be considered medical advice. I have read a great deal about my wife’s illness and have attempted to present the information in a more readable fashion here. I am providing you with the links to my sources. You should also know that I’m only covering the mild and classic forms of type I myotonic dystrophy. There is also the congenital form which can affect infants at birth and type II myotonic dystrophy where a different gene is affected and the disease is generally not as severe.
I went from being a low handicap golfer, avid hiker and runner, enthusiastic traveler, to being confined to a wheelchair and now being confined to an 11′ x 15′ room, especially when the weather is too bad to go out for a wheelchair ride. My wife of 38 years died in October, far too young, due to her own rare disabling disease (myotonic muscular dystrophy).
I definitely grieve the loss of my wife and loss of strength and loss of the life I once had. At least once a day that grief will be strong enough to make me stop whatever I’m doing and shed a tear. In fact, during the first couple of months after my wife died, the grief was so intense that it was quite painful physically. Rather than avoid it, I chose to accept that pain, experience it as deeply and fully as possible, and in that way let it help me heal. Years ago, a meditation instructor taught our class how to get rid of a headache. He said when you felt it coming on to focus on the pain as intensely as you could. Try to describe it, determine its focal point, trace it throughout your body. The remarkable thing was that once a person focused intensely on the pain it usually went away. It seems that pain is trying to send us a message and if we can pay attention, it won’t have to shout so loud. So that is Part One of how I deal with grief and loss; I embrace the pain and let it heal. In fact, I pick a fight with it by writing this blog and the new blog I have created celebrating Beth’s life.
Part Two is realizing that my life is about so much more than what I have lost. I have the comfort of feeling surrounded by family, even though many of them live far away. Beyond that, life is so exciting right now due to the advances of technology. My computer provides a window into a world that I could never have imagined when I was growing up. I can use Google Earth Street View and visit nearly any city or countryside in the world just by moving my mouse. One moment I can be “walking” beside the Eiffel Tower, the next I can be riding along the Pacific Coast Highway at Big Sur. Recently I have developed a keen interest in computer programming and have discovered the wonderful online courses offered by Stanford University. In fact I was just watching a lecture (on iTunes) by a Stanford computer professor and marveling at how much I felt like I was right in the room. That prompted me to add this article to my blog.
In a few minutes, the caregiver will deliver my dinner, attach my mobile arm support, place a bib around my neck, and I can enjoy my meal while watching the latest Batman movie on my computer. So yes, my life is populated by loss and grief, but they are joined by new sources of fulfillment. I hope that others in my situation are finding their own joy as we continue this surprising journey.
Inclusion body myositis is somewhat unpredictable. Although you can count on gradually becoming weaker you have no way of knowing which muscles will be affected the most. In my case, it was my legs and fingers that suffered the most damage for many years. I was still able to drive, using hand controls up until November of 2011. But during that year I noticed I was having more and more trouble turning the steering wheel and applying the brakes using the hand lever. By the time I moved to assisted living in May of this year, my left arm was almost completely useless and my right arm was quite limited. This gave me special trouble when eating as I could not bring a fork full of food from plate to my mouth unless I performed a rather bizarre maneuver of holding my right arm out perfectly straight, slinging it out and up and then bending it at the elbow to allow the fork to come near my mouth. Not only must this have looked weird to surrounding diners, it also had a tendency to throw food in a fairly wide arc as the fork took its orbital route from plate to mouth.
At the recommendation of a couple of occupational therapists, I looked into a mobile arm support. There are plenty of them out there – the issue is to find a mobility supplier who has the knowledge and is willing to spend the time to fit you with one. I found both qualities in Carlos who works for Access Medical in northern San Diego County. Carlos ordered the equipment from JAECO and brought it to me here at Huntington Manor Assisted Living. After making several adjustments, it is now working just fine.
You can compare the arm support to a counterbalanced garage door. Rubber bands provide upward torque on the part of the support that holds your forearm. When it’s adjusted properly your forearm will seem to float weightless in front of you. This makes it easy to pick up a forkful of food and bring it directly to the mouth. I am now eating better, throwing much less food around the room and I have Carlos and the JAECO mobile arm support to thank for it, along with Lynn, my occupational therapist at Scripps Rancho Bernardo.
We did a CANHR (California Advocates for Nursing Home Reform) search and it yielded nearly 700 facilities just for San Diego County. Most are converted single-family homes where they are able to keep six residents, two to a bedroom. Small facilities such as this do not have as restrictive licensing requirements as larger ones and they may be less expensive. For many people this might be a good alternative, however for us they were not appropriate.We needed wide hallways and doorways and adjoining separate rooms to accommodate our large wheelchairs — a physical layout rarely found in a single-family home. Since I cannot help with transfers, I needed a team of caregivers or an overhead lift system. I was also concerned whether a small facility would have the level of care we needed for Beth’s complex medication management and my challenging physical state due to inclusion body myositis.
There are several large assisted living facilities in the county, but they have fire code restrictions that prevent them from accepting non ambulatory residents. Most have associated skilled nursing facilities which is where they suggested I stay, but that would totally restrict my freedom and be very expensive. Also, because our needs were different, the large facilities planned to put my wife and I into two separate buildings.
After eliminating hundreds of facilities, we came upon Huntington Manor, just five miles east, in Poway, California. At 27 residents it was neither large nor small, which for our needs was just right. It came with good online recommendations and had beautiful surroundings. What really caught my eye was their statement that they accepted nonambulatory patients. Huntington Manor specializes in caring for the frail elderly and has done all of the necessary legal groundwork to be able to accept up to 21 residents who are unable to bear weight. Also, for the first time, I found a facility owner who was willing, even eager, to let me install an overhead lift system in my room.
I stopped by several times, unannounced, to observe the staff and sample the food (friendly, delicious).One drawback, which the owner made sure we understood, was the age of the other residents. Most are in their nineties, so we may need to look outside the facility for social interactions.
Price was an issue of course and although Huntington Manor was less expensive than some of the very large facilities, it is still more than we can afford indefinitely. Eventually we will need to sell our home and after that within a few years we’ll need to find some other living arrangement.
In the meantime I am looking for additional sources of income. (Any potential sponsors for this blog site, please take note.)
There were several aspects to this decision. Perhaps the most easy to evaluate was the financial. Perhaps the most difficult was the emotional.
And then there were family issues, especially children who were tired of worrying about us.
Being the analytical type, I prepared numerous spreadsheets trying to decide whether the move to an assisted living facility made financial sense. I discovered that, to obtain adequate care within our home, we would need to spend about as much as it would cost to get assisted living outside the home. However, there were so many other emotional issues involved that no amount of tweaking the numbers on the spreadsheet seemed to fully resolve the issue in either of our minds.We had spent a lot of money on, and had a significant emotional attachment to, the changes we made to our existing home. It was single-story, easy to get around for us in wheelchairs, and had a very nice view out the living room window. We each had our individual art studios on either side of the spacious family room. We would have to say goodbye to all of that. In addition we would be downsizing dramatically from about 1600 ft.² to a little over 500 ft.²
Then there was the concern about our independence. Would we feel as though we were unable to live our own lives if we moved into a facility that had its own schedule and structure?
Eventually, the more I worked on the financial side of it, the more I realized that moving to some form of facility was going to become inevitable. If we remained where we were and continued to spend considerably more money than we had coming in, we would eventually reach the point where we had exhausted our savings and then what? We could sell the home, but then we would hardly have enough resources to maintain us in any other location for more than a few years. On the other hand if we moved and spent down our savings, we could retain our home and rent it, which would provide additional income during that time. Then, when the savings was depleted, we could sell the home and continue to live in the assisted living facility for several more years.
So, ultimately, the practical considerations and family concerns outweighed the emotional worries. How is it working out? Better than we expected. In the next article we will get into the details of how we chose Huntington Manor to be our home – conceivably for the rest of our lives.
Today I am reluctantly admitting that this is one battle that ultimately I could not win. IBM is too progressive, too relentless, too untreatable. The final straw came when my right shoulder and arm became so weak that I could no longer raise my arm much above my waist. This meant that it was no longer safe for me to drive. It also meant that I could no longer chop vegetables or stir a skillet. It meant that I could no longer hold a paintbrush and create art. And worst of all, it meant that I was no longer an appropriate caregiver for my wife, whose own battle with Myotonic Muscular Dystrophy was not going well.
The first thing I did was have my van converted once again, only this time in the opposite direction. I had the passenger seat moved over into the driver’s seat position and put the ez-lok on the passenger side. This meant that I could pull into the van and lock myself in on the passenger side — provided I had found a willing driver to take me where I needed to go. Fortunately, my friends and family and neighbor have pitched in and I still have been able to get around when absolutely necessary. Perhaps more importantly, my wife, who also can’t drive and who has more medical challenges than I, could get to her various doctor appointments. When drivers aren’t available we are able to get to appointments using the accessible bus transportation called MTS access. It gets the job done, but it is certainly not a convenience. I plan an article on the general subject of bus transportation (and lack there of) soon.
But the really big change I have made is to hire caregivers for the two of us morning and night. The loss of arm strength meant that I was at great risk of being stranded when trying to use my ceiling lift to get into or out of bed or onto and off the toilet. After several close calls and more than a few minutes of hanging suspended in a very painful and awkward position, I realized I simply couldn’t go it alone anymore. It is an expensive adaptation and one that we will not be able to afford indefinitely. But for now it is getting us through each day. In future articles I will talk more about the good points of having caregivers.
I am also searching for a “mobile arm support.” The right one might restore some of the functions of my right hand and could possibly let me try to paint again.
I apologize for the length of time that I have been absent from my blog site. Over the past few months, my upper body strength has deteriorated dramatically. I had always assumed that my illness (Inclusion Body Myositis) would continue to progress slowly, as that is how it is described in the medical literature. However, you can’t always believe what you read. Just since this spring, I have lost the ability to raise my right arm. This has also caused me to give up driving, abandon any thought of painting, and even give up using my iPad (can’t lift it). It has also forced me to develop a lot of additional tools that I need just to get through the day.
To be honest, I have been more depressed than at any point in my disease progression, and this has caused me to lose motivation. I am finally coming to grips with my new status and am ready to renew the fight. In fact, I am finishing up work on a song that hopefully will inspire me to persevere. It is called, “I won’t stand for that.” As soon as I finish production, I will be posting it here. (I am no singer, but I did have some musical ability in my previous life.)
I have also recently designed a unique pair of “shoes” that I can put on by myself and I will demonstrate those in the next couple of weeks.
Another major milestone has been the acquisition of a custom–molded back rest for my wheelchair which has made a huge difference in my general well-being. More to come on that as well.
Important update: when I lived at home, these lifts were attached to the ceiling. Now that I am in assisted living that is no longer a desirable solution. There are new types of systems that require only unobtrusive pieces attached to walls on opposite sides of the room–they don’t even need to be attached to studs as all of the weight is absorbed at the base.