May 2016

Since my diagnosis last November, I have kept private about going public with my disease. I’m not really comfortable talking with strangers about it. And I was able to hide it for awhile. But I think if you tell someone, it breaks your denial a little bit . And I still have some denial. I also think I needed time to process it. And then it took a few months to get up the courage to tell our son. Then we started telling close friends and neighbors. So, it’s been a gradual process.

But I took a big step at the beginningof May and announced it on Facebook. This went against my instinct for privacy and almost felt nauseous for several hours after posting. I have never talked about my health before on Facebook. But I’m glad I did. The response was really great. The warm wishes I received from people, some complete strangers was overwhelming. And what surprised me was people thanked me for sharing it. That was baffling.

I have been dealing with difficulty with eating for some time and as a result have lost almost 20 pounds since February. This is also due to loss of muscle mass which is contributing to the weight loss.

However, the Dr. McC______ started me on a medication to improve my appetite and it seems to be working. I suddenly have a big appetite. And I’m eating big meals again and can taste food, which I haven’t in several months. I’m greatly relieved and grateful

I also had Botox injections in my jaws that are helping reduce some of the spasticity which is one of the issues I was having with chewing. No sign that’s its working yet, but apparently it may take a few weeks.

I seem to waver between two extremes: thinking, schemeing and looking for alternative treatments for my ALS and then conversely, by being completely passive and surrendering to the inevitable. Maybe both viewpoints are necessary. Much as I like and respect my neurologist and his team at the Clinic I find it difficult to accept that they’re there to keep me comfortable until I die. I'm really pissed off about that.

I don’t know how Dr. McC______ does it, going into work every day knowing that he can’t really do anything to change the course of his patients' disease. It must be both frustrating and unrewarding.

But the truth is I’m not improving. I have days that are better than others, but gradually I’m growing weaker, although since I’ve started using the Trilogy my breathing has improved considerably.

Whenever I hear of someone looking into an alternative treatment I think to myself: “they are completely deluded!…why can’t they accept that we’re doomed”? But then I spend a lot of time online looking into alternative therapies myself! But them I’ve always been a hypocrite.

For example, I took the Igenex blood test for Lyme disease. Even though I don’t believe that there is a Lyme-ALS connection…well, I’m curious.It came back negative anyway.

I just seem to can’t give up, although my body is telling me something else. 

My son had a very emotional reaction the other night to my ALS situation. Quiet tears. I think he’s seeing that I’ve slowed down even more in the past few months

April 2016

April 

We went to the Bahamas on a vacation in mid March. I didnt think it'd be possible for me, but there I was, being whisked through the airport, and found myself a few hours later in the pool! (water therapy is great for ALS. I wish the Clinic offered it). Going away was both pleasurable and a big step for me  because it was the first time I had to rely using on electric mobility to get around the resort. (I leased a scooter for the week) I also had to rely on wheelchairs at the airports. This was a first for me. It took a little psychological adjusting to deal with it. The first day was a little rough. My pride took a wounding. But after a short while I saw that there was no other way and the practicality of using a scooter won me over. I was also amazed to learn that so many people were willing to help.

More challenging was witnessing other father's with their sons doing all the things I used to do with my son. I find that this does not get easier. This morning was the opening day of little league. I was grateful to be present, but the contrast between this year, with me now partially disabled compared to last year is when I was "normal" is painful. But still I had a great time, and I find that I adjust to my new reality pretty readily.

Since February I have noticed that I am continuing to decline. Hate to admit it, but facts is facts. I notice it most notably in my hands. I was an accomplished guitarist. I was still able to play a little guitar in February. Today, that is not the case. I can barely hold a pick. Gripping objects in my hands is very challenging. My handwriting is terrible. On the nutritional front, I experience a fluctuating appetite.  I have lost 10-15 pounds since then as well, mostly in muscle mass. Swallowing has become harder.  I am scheduled to receive a feeding tube soon. I was firmly against this earlier, but it's funny how when staring the possibility of a quicker demise you suddenly become very open to other options. 

On the plus side, I continue with the Deanna protocol. I think it works in some ways, in some ways I think it's just fattening the wallet of the  pharmaceutical company. Who knows. I continue to do light yoga courtesy of my yoga teacher who comes to our house once a week, I do stretching and have an excellent masseuse. I purchased a rollator and have gotten over my manly ego to use it. In fact I used it to go to the little league game this morning and it made a huge difference. It's black and I'm going to put skull and crossbones decals on it.

In late February and March, my voice suddenly, inexplicably improved somewhat. I was able to have short conversations and be understood! Unfortunately this improvement has ended and it's worse as it ever was. Totally garbled. Oh well.

I am experiencing some breathing issues, mostly with intense coughing and wheezing. I am now using a Trilogy which is making a big difference in my overall energy level. Amazing device, although the sound of it is driving my wife crazy.

Speaking of my wife and driving her crazy, I am so incredibly lucky. Emma is amazingly helpful, I don't know how she finds the energy to take care of my increasing needs, and hold down a demanding job, a demanding kid, and a demanding husband. Thank you Emma.  My 11-yar-old son is also amazing. He’s very helpful in general, but what I find truly remarkable is that he’s not fazed by any other the mobility assistance devices or medical equipment hanging around. In fact, hes fully supportive of my using it. Did I say I’m lucky? Oh yeah, I did.

As I am getting more comfortable to share the news of my condition, I continue to reconnect with friends, some I have lost contact with over the years, all of which are willing to help. Its truly humbling.

I have many frustrations too, but I’m not going to go into that now.

this is what happended

I started having noticeable symptoms in May 2014. Out of nowhere, I suddenly started experiencing problems with speaking. On certain words, it was as if my tongue would glitch. At first I assumed it was stress. Then I thought it might be an allergy, as some of the symptoms mimicked allergies that I sometimes had in the spring and fall. It would come and go and eventually went away altogether so I didn't think much of it. 

The following November, it started again. This time accompanied by a hoarseness in my vocal chords. I remember being with my sibs Mark & Betsy at Thanksgiving and we all were singing Beatles tunes and I was really struggling to stay in pitch, something that I never had trouble with before. Still, I wasn’t alarmed, and it went away, again.

A few months later in January 2015, I was recovering from rotator cuff (shoulder) surgery and the symptoms suddenly became much more acute, more persistent. I began having trouble with chewing and sometimes had choking episodes. This was new and very scary. The symptoms would last for days then disappear, only to reappear again a few days or weeks later.  What made it all the more confusing as the symptoms were similar to the side effects from pain meds I was taking at that time for my shoulder. So, initially I thought it was the meds. But once I was done with them, and my shoulder straightened out, the symptoms with my voice remained and continued to worsen. In March, I sought out a speech therapist who urged me to see a neurologist. Right away. So I did. By mid-April my voice was almost an unintelligible garble and my body started to do weird things: feeling rubbery, loss of balance, dizzy, intense fatigue. Very scary. 

At first I assumed it was Parkinson's, as two of my cousins have it, but the neurologists ruled that out. After some testing, I received a preliminary diagnosis of motor neuron disease (MND) in April 2015. That's it. I'm done for. But my local neurologist thought there was a possibility that it might be something called Myasthenia Gravis, as some of the symptoms he was seeing didn’t add up. The symptoms between MND and myasthenia gravis are so similar and both are difficult to diagnose. Luckily, MG is treatable and I responded well to the meds for a few months. So well, that while I still felt lousy and rubbery, my voice got much better and we were able to travel to the UK and France in June and I planted a huge garden and continued to live fully, and felt strong. So at that point I thought I'm going to be ok. I started doing yoga, exercising, and riding my bike religiously. 

But as the summer progressed, I stated to experience weakness in my body. In early July I had these little indications that something was drastically wrong. I recall dragging a small kayak out of the water, something I had done countless times before, but this time it felt as if I was pulling a huge ship. My voice slid back into garble and most distressingly, I was having difficulty in playing guitar. This was devastating. I had severe weakness in my pinky and ring fingers and difficulty in gripping a pick. I'd exercise them, do scales, anything I could think of to try stay ahead of the symptoms. Despite this, I continued to ride my bike, swim, and do everything I like to do and stay active, although I could no longer deny that there was a compromise in nearly everything I'd did and after I'd expended myself, it would leave me exhausted and I'd collapse, having to rest for long periods. Yet, I was determined to fight it, keep hitting back, and live every day like it was my last. In some ways, I had never felt more alive at that time because I just had this feeling that something was closing in, hovering over me.

By September, despite my attempts using yoga, exercising, medications etc. I had to admit to myself that I had dramatically declined. There was no question now that my strength and abilities were not improving. For example, opening a lid on a jar was suddenly a difficult procedure whereas a few months before, I'd whip it off without thinking twice. Holding a pen proved challenging. Eating had become a potentially hazardous endeavor and had to be done carefully and slowly to prevent choking. Cutting our wee lawn would take an hour whereas before it used to take 20 minutes. I had to give up riding my bike due to the fact that my leg strength had seriously waned. In fact, walking became difficult and I developed a limp. I spent a good part of October in a recliner.

In early November 2015,  I went in for new round of testing. This is when I received a new diagnosis of ALS. It  wasn't entirely a surprise, as the likelihood of it had been hanging over my head since April, but still, hearing it being spoken by your doctor is surreal. And sickening. I'm still partly in shock. I think I’m going to wake up, and I’ll be normal again. But after having a six months to chew on it, I’m a little closer to accepting it. But some days, I’m seriously not. 

At first, I had the attitude: “damn it, we’re gonna fight this!”, and I was researching just about anything no matter how crazy or far-fetched as far as treatment options. But really, and this may sound defeatist, as far as I can see there’s just nothing out there right now. (I think they are decades away from a cure, hell, they don’t know what even causes it although different theories abound, all unproven). As time as has passed and observing the changes in my body I have to say; I’m really humbled by this disease. And while I do take many proactive steps (supplements, yoga, massage, lite exercise, etc), there’s just no stopping it. It just keeps on rolling along. 

The biggest challenge I’m dealing with is being a father of a 11-year-old child. My thinking is (right or wrong) if he were older, more formed, in college, etc. I think I would feel more secure about dying and his dealing with the repercussions. We held off telling Gavin until late January. I was hesitant for fear of traumatizing him. It was the most difficult thing I’ve ever done. The first 10 minutes were excruciating. Many tears. Then, it lightened up. Somehow, we survived. Emma had to do all the talking (as now my voice is about 100% gone), forcing me to write notes. His spirit is so strong and positive (go figure, him having a melancholic father!). Kids seem to live in the moment and compartmentalize. A Blessing. He’s level-headed, he’s resilient. But I do worry about the future. 

Emma is suddenly thrust into the role of caretaker, which she’s handling brilliantly on top of now being the sole breadwinner of a incredibly demanding and sometimes frustrating job. Luckily, she’s much more into living in the day or the moment, while I'm a month in the future, a year back in time and all places in between. Amazing, she still has a great sense of humor, in fact we all do despite this shitty, f’d-up, unfair situation. We all still laugh and have fun, and despite my mobility issues we went to the Bahamas in March and had an incredible time. My sister Betsy has been incredibly supportive, coming out several times just to hang out and help and networking through Facebook to raise ALS awareness and $. My brother Mark, I think is having difficulty dealing with it. He's well aware of it but hasnt come around to being able to talk about it. At least to me. I never told my Mother. (whom just passed away recently) She knew something was wrong with my voice, but I never elaborated on what was the cause. I just couldn’t bring myself to do it. As a parent, I know when something is wrong with your child, you suffer deeply and you feel that you have should've done something to have prevented it, no matter how illogical that seems. I didn’t want her to have to go through that. 

Currently, I’m continuing to decline which is ‘normal’ as it’s a progressive illness. I notice small changes week to week, loss of ability, less strength. For example, a month or so ago I was still able to cook without too much difficulty. Now, Its nearly impossible to prepare a meal. Even a simple one is hard. I leave a huge mess behind, drop things, and the difficulty I experience standing for any length of time makes it not really pleasurable. (I used to be the family shopper, cook, etc).

My breathing has improved slightly due to a BiPAP machine I use at night so I’m more rested when I get up.

 I held off telling people because I didn’t want to upset them and also because of my pride; I didn’t want to be thought of as a ‘tragic’ person. And frankly, it was kind of a bother telling people too. Now, I don’t care, no time to worry about peoples reactions. It’s none of my business anyway. I just got a bad hand dealt, that’s all and the last 11 years of my life have been perfect. 

The Clinic

Met with Dr. Xxxxx at Jefferson Hospital last Thursday. I knew already that I had ALS, but somehow hearing it from him had a finality. In the back of my mind, I keep hoping that there's a way out; perhaps a mistake was made, perhaps I've imagined the whole thing, maybe I'm being a hypochondriac, I made it up, I'm exaggerating my symptoms and really it's just stress or I breathed too much joint compound dust over the years.

No, it's final.

if I just get enough to sleep, I'll feel better.

I keep thinking if I just get enough to sleep, I'll feel better. Stronger. Able to do things. While this was true "before", it definitely is not longer true.

I also think if I have a really strong cup of coffee, it'll give me energy and I'll be able to do Things. Also no longer true.

Now when I get up, I'm immediately feel,like the ship,is listing and I'm in danger of falling.

****

The only time I feel normal is when I'm sitting down and doing absolutely nothing.

If I get up, I'm dragging the 50 pound weight being me.

Hands are weak, holding a kitchen knife and cutting vegetables my wrists feel flaccid. Thumbs are no longer firm, turning the key in a lock or starting the car has become a two handed procedure.

Right leg and right foot now pointing 20 degrees to the side

Voice is now almost completely unintelligible.