Post 26 - State of the Union

Since this promises to be a slow weekend, (happy Memorial Day weekend to all) I’ll try and slip this post in, since it is not shaping up to be a particularly uplifting one. Consider that your warning. It has been a while since I've given an update on my physical status. My own kind of "can and can't do" list. So without any further ado, here goes nothing.

The good news is that I'm still breathing and swallowing. My arms and legs are still taking the brunt of the disease. It almost feels like I am giving you battle coverage from the front, but I guess in some ways, this is a war. The atrophy in my muscles has become very apparent throughout my body. The most notable area, to me is my neck and shoulders, but it is very obvious in my arms, hands and legs as well. I attached some recent pictures at the bottom of the post, so you can judge for yourself. I estimate that I only have about 5% usage of my left arm and maybe even less of my left hand. There only two ways that I can move my left arm. The first is to swing it back and forth, and kind of fling it where I want it to go. The second way is to grab my left arm or hand with my right hand and move it to where I want it to go. My right arm and hand, although considerably better than my left side, is starting to falter as well. I estimate that I have less than 50% usage of my right arm and hand and I'm starting to fling at arm a little bit more now as well, in order to get it where I want it to go. I can still bend my arm at the elbow, but it is very difficult. I am unable to lift either arm anything more than a shoulder shrug.

My legs, which seemed to have held their strength a little longer, have now caught up to my arms. I am unable to walk without my leg braces more than 5 feet or so, and I can assure you it is not a pretty sight. I look a little like a drunk Frankenstein. Even with the braces on, the maximum distance I could walk, maybe is about 20 or 30 feet before becoming extremely fatigued. Even then, it must be on a flat, smooth surface with no impediments. Because of my leg weakness, I will almost always rely on using my walker. Without the walker I am very unsteady and unbalanced and a danger to fall with each step. The last time I went for an extended walk without my walker, was at work a little more than two weeks ago. I'd walked out of my office to see my secretary, and then decided to continue my walk around the office to stretch my legs. As I was beginning to take a step, the tip of my shoe caught the carpet, and down I went. In ALS terms, this is known as "foot drop," and is often the cause of many an ALS patient falls. Foot drop is basically the dropping of the forefoot due to weakness or paralysis of the muscles down the front of the lower leg. It is characterized by the inability or difficulty in moving the ankle and toes upward. Thankfully, I was able to break the fall with my head and ended up with a little egg above my right eye, in addition to a nasty case of rug burn. When I fell, I also had my right arm caught underneath me, which caused me to land on my elbow and temporarily knocked the wind out of me, in addition to bruising my elbow. I also banged up my left knee and ankle... yes, that same ankle. Luckily, there were only about a dozen people in the office at the time, instead of the usual 13 or 14, so it wasn't too embarrassing. This fall earned me a round-trip wheelchair ride to the nurses’ office. I tell you, everyone should be so lucky to work in a place that has a nurse's office.

Since this fall, I have been very tentative to attempt walking without my Walker. Even with the Walker, it feels as though both my strength and stamina have decreased significantly. I assume that this is both partly mental and physical since each fall does cause me to lose a little bit of confidence but also takes a physical toll on me. At the rate that I am progressing, I would estimate that I would almost be fully wheelchair-bound within the next three months.

There is some good news out of all of this. As I mentioned at the beginning, both my breathing and swallowing appeared to be normal, although I have begun to experience shortness of breath with almost all physical activity. Also, I believe I have detected some slight slurring of words, although nobody else has or will confirm that. Even though people may not be able to hear it, I know I am starting to struggle with saying certain words. It is therefore something I'll have to keep an eye on.

Over the past several years, my weight has remained somewhat consistent. Over the last six months, however, I have lost 11 pounds. That may not seem like a lot, but when you think about it, it has obviously not been through exercise. Weight has always been something my clinical team is concerned about, and they have always encouraged me to try and keep my weight up. A recent study of over 400 ALS patients conducted at Massachusetts General Hospital (MGH) found that those who were mildly obese survived longer than patients who were normal weight, underweight or even overweight. "We have long known that being underweight shortens survival for ALS patients, and several studies in an animal model have shown that weight gain is associated with increased survival," says Anne-Marie Wills, MD, MPH, of the MGH Neurology Clinical Trials Unit, senior author of the report. "Our study was designed to investigate how cholesterol levels affect survival. We were surprised to find that body mass index or BMI – a measure of weight adjusted for height – made a large difference in survival. Patients with a BMI of 30 to 35, who would be considered mildly clinically obese, lived the longest; and patients who were overweight, with a BMI of 25 to 30, lived the second longest." For better or for worse, my current BMI places me in the "overweight" category. I guess I have some eating to do. This is also why my clinical team at the University of Pennsylvania hospital has been urging me to quit my job for the last three years and just sit on my couch and eat potato chips and drink milkshakes.

My Functional Vital Capacity (FVC), which is a measure of my ability to breathe, was slightly down at my last clinic visit, although it was still well within normal range. According to my neurologist and the pulmonary specialist, the drop in score was insignificant. Insignificant to them, of course, because they don't have ALS. As my stomach muscles weaken, which is evident by the fact that I can no longer do certain things, it is expected that my breathing will become more difficult. Typically, the first sign of breathing distress usually occurs at night while you are laying down. Luckily for me, this has not been a problem and my FVC score while lying down is almost identical to my score in a seated position, which is unusual, even for a healthy person.

Several things that I am unable to do at this point, that I was able to do 3 to 6 months ago, includes: sitting up in bed, getting out of bed, getting up from a seated position, showering, getting dressed, brushing my teeth, shaving, drinking without a straw and driving. It has now been almost 6 weeks since I had last driven a car. Not that I still couldn't, but it would probably require me to expend a lot of physical energy to do so. Besides, I would be unable to get up out of the car without assistance, and even if I could, I would be so thoroughly exhausted, that I would probably take one or two steps and then be on the ground. Therefore, Tracey has been driving me to work every morning, and picking me up almost every afternoon. Several coworkers have graciously provided me with a ride home on occasion.

I can still hold a pen to scribble something that kind of looks like my signature, but I have to do this with my right hand. Other than that, I am unable to write. Typing on the computer is also extremely difficult. Oddly enough, I am unable to straighten my pinky, index finger and middle finger on my left hand, and they remain in an almost perpetual fist. My pointer finger, however, does what it's supposed to do, in that it points straight out. That finger, I am unable to bend. My thumb is similar to the first three fingers. On my right hand, I pretty much have full use and control of all my fingers, although they lack strength. Go try and figure that one out. After four years, things like this are still mind boggling to me.

Ironically, I am actually feeling very well today. Had I felt the same as I did today, three months ago or even a month ago, I would have told you that I was feeling very poor. I rarely notice a difference from day to day, and occasionally notice a difference week to week. However, I rarely feel as good as I do on any given day, than I did a few months ago. For the most part, I can say that more often than not, my spirits remain up.  As always, you can follow my status anytime by checking out my "PatientsLikeMe" profile on the right.  I typically update my status to coincide with my clinic visit to University of Pennsylvania.  My next scheduled visit is for July 21.




Trying to walk without my braces or Walker.

  

Dressed up for a night out on the town!