Day 374: A year plus in perspective:

So, it’s been quite some time since I’ve written here. I feel like I say that a lot and its something I need to work on. It helps to write, and it helps to let others know what this life is like.

I spent the morning in a pretty good funk. In fact, I’ve spent that last few weeks in a big funk. Its hard to watch yourself change and its even harder when you lose something in total. This disease is like a 200-round fight with Mike Tyson in his prime. When you are first diagnosed your life in a funk fighting between denial, bargaining with God, and did I mention denial? You waste so much time feeling sorry for yourself and those were the best times you had left. You miss things because of it. Later you try to grab it all by the horns and you realize you aren’t running marathons anymore but instead trying to run one lap. But when you lose a leg, arm, or some form of function fully the grim reality rains down on you. That’s when Mike takes his hardest swing. You’ve dropped your hand and left yourself open for a massive cross.

So, the last few months have been that time. I’ve lost my ability to walk and partial ability to stand. My wife must yank me up to my feet and it’s a game of Russian roulette just to hold on for dear life to a car door. My right leg has become nothing, and my left arm has lost half its strength. You haven’t lived life until you’ve been stuck on a toilet for 45 minutes without the ability to lift yourself one inch into a wheelchair. Maybe that’s too much information but its ALS. ALS in its mild stage is wondering how you are going to transfer into bed, how can you sit up, and getting stuck in the middle of the night held down by a blanket that weighs ounces.

The only way I know how to describe it is that someone is loading up your body with weights every day. You start off with a 2lb weight around the ankle and eventually it feels like a bus is holding your leg down. Those weights are my Mike Tysons I must fight.

So, I’d hit the wall of whining. The self-pity train was parked at the station and I was sitting in first class. Then this morning we ran out the door to go speak at a charity event. I used to love being the center of attention, but it becomes harder every day. I look at it all wrong and feel like the room is full of pity looking at me. Sometimes I dread it, but people need to see what ALS is. It most certainly must rank high on the list of asshole diseases (excuse my language) but its rare enough most people have never seen it or been close to it.

Today was a bit different today. I talked to a few classes at Orange Therapy who is fundraising for Augie’s Quest. Fantastic charity and a ton of money raised…hey why not. This one was a bit harder for me. I used to love the gym. I’d come home from work, immediately change, and head to the Y. The feeling was fantastic to lay it all out there to see if I could better myself. These people were doing what I used to love, and I felt a bit empty. The love I felt though really caught me off guard. There were several people there who had been affected by ALS and several more just wanting to hear my story. It felt special to me and I honestly had to fight back some tears. I felt like I was putting a lot out there and maybe it helped shed some light. Maybe it didn’t but it felt good and for the first time in awhile I felt productive again. A few people even brought up reading my blog and hugged me. I really thought I was just talking to myself ha-ha.

So, in overall this year has been crazy. We’ve been through 2 drug trials, traveled all over the east coast, and continue to fight. I maybe losing right now but I want to throw some more victories in the mix this year. I’m fighting emotions daily but getting closer to stable and I still have some punches to throw. It may only be round 8 but I’m preparing myself for the 9th.

If anyone is reading this who participated in the OTF class this morning thanks you for taking time to listen to me. Thank you for your time and donation. What you give myself and others is hope. Maybe next year we can be summarizing a cure or new treatment because of your donations.

Day 258: What is Pride

It seems like forever since I’ve posted anything here. Not sure if I’ve just been busy or felt more comfortable in life but I have shifted a bit in where I am. By shifting I mean that I’m not an emotional cluster all the time. I don’t feel the need to vent as much and have been trying to find new outlets for my energy whether it be work, fundraising, etc. Not sure I have it nailed down or not but I feel like I’m closer than ever to finding a real outlet for what I want to do.

One thing I do want to talk about though is pride. We all have our different feelings and definitions of it. Some of us especially myself saw or see pride as being independent and achieving goals. By far I will say this has been my biggest roadblock in my journey. How do you have pride while having to rely on others constantly. It’s not something you can change at the flip of a switch but I’m getting there.

The biggest mistake I’ve made over the past few years is thinking that I was better off by not asking for help. For fighting through things that I didn’t have to and instead hurting myself along the way. As many of you know I lost the use of my right leg first. I have slowly progressed into my left leg. Over the years I fought using a cane or walking stick when I needed the support. Then further down the line I fought using a walker. By the time I got to needing a wheelchair I had realized what I had done. By holding onto my pride I had fallen and hurt myself so many times that I most likely accelerated the damage. By refusing to let go I had pushed my other leg so hard that I had hurt another limb. By refusing to accept who I am I had slipped 3 discs in my back and caused myself a life of permanent discomfort.

So, sitting back and looking at it was what I was doing really pride or just the disillusion that I could fight this disease? Even more likely is the fear of change and acceptance. The real key to this disease that I have since seen is not fighting it but trying to stay level or ahead of it. Every day we lose a bit of functionality and the key is to find out how to counteract that. If you can’t walk comfortably and without pain can you function better with a manual wheelchair? If you can’t push the chair manually anymore should you get a power chair? These decisions are hard but aren’t negotiable without pain of some sort whether it be mental or physical.

The moral of the story is that grunting through pain and doing things the hard way isn’t showing pride. Pride is best shown by acceptance of yourself and the limitations in your life. Pride is not only doing that but being strong enough to accept the help. Pride is pulling up your big boy pants and fighting to enjoy your life in lieu of fighting to stay at the status quo.

There is so much more to life than walking through it alone and when you learn that you can truly enjoy what you have left. Don’t be a victim of yourself and a disease. Being a victim of one is hard enough by itself.

Day 165: Still kicking (literally)

165 days really seems like a big number. 764 seems even bigger. It’s been so long since this all started that you just become a bit numb to it. I spend 75% of my day forgetting something is wrong with me, 20% of the time thinking about if it will ever be better, and a 5% mix of anger/sadness/worry/etc. My pet peeve in life has become the simple daily question of “how are you doing?”. The question is relentless and I really don’t have the answer. Don’t get me wrong in the fact that I am blessed to be slowly progressing but I honestly have no idea how I’m doing anymore or how I am progressing. On paper, I’m the same person I was in February but in life it’s an inconsistent mess.

Some days I find myself in full stride and fine. Most days it feels like an adventure to walk 25ft. I know I wasn’t there a year ago but it’s so hard to pinpoint when it just went to crap. I feel like I’m on this bubble of being converted to a chair and some days I just feel like giving in. I don’t want to but it looms over me like a dark cloud. I’ve been so lax in my activity that I wonder if I’m just making it worse. My goal is to try and work on that right now and take steps to get more exercise. The atrophy is my right leg is severe and my right leg just doesn’t want to compensate anymore. Heck I wonder if I would be happier with a set of wheels and just accepting the future. How do you shut the door on walking when some days you don’t even need a cane?

The irony of it all is after 20 years of abusing my lungs they are the one consistent thing holding on. Still ranging between 113% and 117% on my FVC tests and still blowing all the other breathing tests out of the water. Still no substantial bulbar issues either. So, I can talk, I can breathe, and I can still work my arms although a bit weaker now. Heck even my grip strength is at the top of my age range. It’s just so hard to go from going on an elliptical full bore for an hour 2 years ago to finding 20ft to be a daunting task.

The other thing on my mind right now is the trial I’m in. Obviously its double blind and I have no idea if I’m getting the drug or not. Most likely never will unless I go open label at some point. I spend 15 hours a month sitting in a clinic getting IV’s with this great drug and have no idea if I’m getting it. No idea if I’m spending 7 months wasted on extending life. Heck we just went “right to try” and I could basically have my choice of drugs to try. Not trying to be overdramatic but it keeps me awake at night. Sometimes I feel like I move a bit better and hope it’s the drug improving my situation. Then I wonder if I could move my leg more if I did more PT.

Overall, I’m a head case. The other hot topic in my mind right now is work. Anyone who knows me knows I was a workaholic for years. I wanted to work hard, play hard, and retire early. All meaningless things to be honest. Work means very little in life to be honest and I’ve accepted the fact that I take it too personally. With that said it makes me immune to my disease. When I’m work it doesn’t matter that I walk like a duck or that I twitch. None of it matters and my mind can still work. It’s almost therapeutic to forget what’s actually happening for 8 hours at a time. Heck now with “right to try” I need to work. I need to have that extra income for drugs that simply would be out of reach on disability. The flipside is I take it all too personally. It upsets me half the time and I stress myself out. It’s nothing new but stress is a bad thing in my state. It’s probably killing me faster to be frank. So, what do you do? Do you quit and live your life bored out of your skull on a back porch smoking cigars and drinking gin and tonics? Do I quit and focus on my health with PT and healthy eating? Heck do I go full blown hippie and do yoga for 2 hours a day or fish all day long? Those decisions aren’t easy and every day is a day that has passed. How many do I really have? Am I going to look back in 6 months and ask why I did it? Am I going to look back in 6 months when I can afford drugs and thank myself? I don’t think there’s an answer and those are the hardest questions to ponder.

So, to flip from my emotional babblings to real eventful things “right to try” passed in the senate. Yes, the thing I’ve mentioned 8 times already and am actually pumped about. I cannot try and wait 5 years for an SOD1 ASO to clear phase 3, I cannot try and wait for a cocktail of meds, and I cannot just cruise the web for a new trial. It’s all there and all accessible shortly (pending the companies allowing it). I still hope that these companies offer some assistance beyond “cost” for patients. If I hear one more drug costs 150k a year I’m going to puke. I love the thought of being a human Guinea pig to stay alive but I have to drop 300k a year to try 2? We just hate dying people in America!

For the most part I think the entire act is great. I think it’s going to hurt trial participation but I guess if they keep the costs high enough people will be forced into them. I don’t dislike the trial process as a whole but for ALS it’s a crock of crap. The limitations are garbage and most trials are for patients under 2 years since onset. It takes a doctor 2 year to blow his nose in the US if you aren’t seeing a top specialist. It took me forever to be diagnosed and in fact for the first year+ I was told there was zero chance I had ALS. So, you expect to get a truckload of folks with those parameters……………pshhhh.

Anyways I’m jacked about that which is offsetting the bad. Hope is awesome you just have to have enough to get through the crap moments of the day!

Day 152: The Future

So, I try not to get technical and use this as my venting post but I’m particularly excited right now. So as some of you know I’m SOD1 FALS which is good and bad all wrapped into one. It’s good because I’m the most popular kid in the class. I was the first jacked up ALS gene discovered, I’m the basis of most research in SOD1 mice, and most of the gene therapy and ASO’s right now are aimed at SOD1. Basically, I have hope that if anyone is going to get figured out its going to be my cool group of PALS. The bad side is that I have ALS haha.

So, I’ve been in a trial and while I have no idea if I am getting the drugs I have felt better this month. Basically, the trial was screened through CRP (C-reactive Protein) which is now being touted as an ALS biomarker. CRP is essentially a protein that is released into the bloodstream to fight an illness or inflammation. Inflammation sounds fun, doesn’t it? So, I’m going to discuss a few drugs but not say what trial I am in and where I see the pipeline going. Many have the theory that ALS will eventually be treated through a cocktail of drugs just like HIV. Not everyone with ALS has the same cause, same reactions, or same gene abnormalities so it’s going to be confusing to figure out.

A few months ago, Edavarone was approved by the FDA as the first drug to “treat” ALS in 22 years. Personally, I think the drug makes a lot of claims and doesn’t have a lot of back up. It was never trialed in the US and was used in Japan. It’s an antioxidant free radical scavenger. The point is for it to attempt to scrap our unpaired, unstable, and deadly molecules. Free radicals basically cause cell death. So Edavarone doesn’t “fix” the problem. I compare it to dropping a bottle of red wine on white carpet but trying to blot it with a single paper towel. It’s going to clean up some wine but your carpet still looks like crap.

Now there are something like 64 other drugs in trial stages for ALS. I’m particularly excited about a few of them. One of them is Biogen and ISIS’s SOD1 ASO. An ASO is an Antisense Oligonucleotide. Big words that mean the reduction of the production of superoxide dismutase 1 (SOD1). OH, THAT’S ME! So, a few things get me pumped up about this. Numero Uno being that Biogen is a legit worldwide pharma power. There aren’t enough big pharma companies pushing ALS drugs and quite frankly they don’t mess around. I’ve done work on the building side with Biogen and nothing moves slow and nothing stands in their way. We’ve built filler lines in three months so it won’t be “Waiting years for production”. Number two is that this is a phase 1 / 2 trial. They are pushing on this hard. Phase 1 evaluates safety and phase 2 to is to evaluate effectiveness. So, this drug could possibly be pushed right into phase 3 afterwards and into a NDA (new drug application). I just honestly don’t believe they would be perusing it this hard if it didn’t merit excitement. So now the cause is being addressed.

Another trial is NP001 which is an anti -inflammatory which most believe is based on sodium chlorite. The drug failed to meet phase 2 goals but they found something promising. Out of the small percentage of people that either improved in condition or stabilized without further progression all people had elevated CRP. This is a twofold win for me. For one I hate the trial process for ALS. We are all different and all trials lump everyone together (except for gene trials). Now a drug has specifically addressed a subset of ALS patients which is obvious yet brilliant and innovative. So, I think this could be used in conjunction with other drugs adding another step to the cocktail.

My only odd feeling about NP001 is that in my opinion Edavarone would need to be strategically combined. From what I’ve gathered NP001 does not play well with antioxidants and reduces the effectiveness. None of this is official but assumes that NP001 is sodium chlorite based. Could be completely wrong there but it could also possibly be staggered. Edavarone is meant to be a lifetime drug of a rotation consisting of 10 days of infusions and 10 days off. I’m not sure how they would play together in the pool but who knows. If nothing else there is going to be another process required to figure that out.

Lastly is masitinib. This drug is a repurposed drug used by vets to treat cancer in dogs. Say what? The drug has a target is to reduce inflammation and it is a kinase inhibitor. I would be shocked if this wasn’t FDA approved by the end of year. There have been numerous questions involving AB and the clinical protocols but to me the backup seems more substantial than what was provided with edavarone. Also, not sure who and how is treated by masitinib. For instance, NP001 was found to work effectively only in people with elevated CRP levels. This is where I get a bit lost and need to research a bit. I’m not sure if the goal is to reduce the inflammatory response itself or to remove the proteins that may be misfolded due to the accumulation. Kind of a chicken or the egg deal. Now a kinase inhibitor such as masitinib are enzymes that group to a protein and change the function. The reason that the drug was previously marketed as a cancer treatment is it inhibits overexpressed kinases in cancer. I also know that many trials are opening up participants to taking edavarone now. Will be interesting to see if the new masitinib or NP001 trial include that option.

The idea of an inhibitor and drug to reduce the inflammation itself would seem to be a double whammy combined. Now the kicker is that a large percentage of PALS DO NOT have elevated CRP levels but do have elevated CPK levels. So, in theory the combination of NP001 and masitinib would really only be beneficial to those having a high CRP level. The high CPK level maybe a biomarker for masitinib which is common amongst most PALS. Again, this is a possible cocktail with riluzole also for some. Both drugs were trialed with the inclusion of those taking riluzole. I do worry that NP001 has been around for 5 years and hasn’t made it to phase 3 but do feel there are too many miracle stories to ignore it.

So basically, if you throw all those kids into the pool you have addressed the source for SOD1, a kinase inhibitor, a possible free radical scavenger, a glutamate regulator, and an anti-inflammatory. Wow! I’ve stayed cautiously hopeful about ALS treatments but all of this is extremely exciting.

If none of this made sense or no one cares I get it but it gives me chills. For the last 22 years we’ve had one halfcocked drug to “treat” ALS which offered a possible 10% extension of life. Now we are using figures like 25-30% on each drug. It’s obviously not a “add them all up” situation but it’s becoming clear that life is and will be extended shortly.

Day 134: Renewed Hope

Not sure where to start. It was a long week with a ton going on. Started my trial on Monday and continued through Friday. Made for extremely long days with being in the clinic for 4-5 hours at a time. Also had my standard clinic visit on Tuesday which added a few hours to the whole ordeal. Luckily, I haven’t had much progression over the last 3 months so it was fairly uneventful.

PT rep took my standard readings such as hand grip, foot test for driving, general strength ratings. My hand grip has remained relatively the same over the last 4 ½ months at 110lb in each. My right hip seemed a bit weaker but I also had an iv line in at the same time and was uncomfortable with movement. Overall (minus 1 person) everyone felt that I’ve held up well. One wanted to put me in full orthotics and a walker or wheelchair. I typically dismiss this person immediately regardless.

With having an IV and being under observation for half the day I wasn’t running around the office like a chicken with its head cut off. I’m not sure if that’s what produced an energy boost this week or if the drug/placebo is working. It’s extremely hard to judge things when you throw your weekly schedule out the window.

On Monday, I felt relatively fine but developed a migraine and then extreme fatigue to the point that I passed out at home for a few hours. I never nap and can’t normally force myself to fall asleep during the day so this was extremely odd. Tuesday was the same and the migraine continued a few hours after the infusion. I never get headaches so this was tough to deal with. Also, I just felt weak. Ended up stumbling and falling a few times in the clinic. Trust me the last place you want to fall is in the clinic. After that I was thrown a walker (didn’t use it) or forced to hold my wife’s hand when walking around. That slightly irritated me. I’ve been independent forever and didn’t sit well with me to be told I couldn’t be. That’s one thing I’m struggling with in general.

Now Thursday got a bit interesting. I had some fasciculations and tingling in my left leg which has been “dead” below the knee for over a year. Also, I started cramping all over like I never have. When I say cramping I mean full toe/leg locks, stomach cramps, and everywhere. Friday, it took me about 45 min to put on a pair of socks for dinner. My toes were locked curled and when I bent over to try and pull them straight my stomach would lock. Magnesium drink to the rescue but wow what an uncomfortable moment.

Here is the weird thing though. Saturday and Sunday, I was wide open. I’d felt high energy a few days but thought it was from additional rest. This however was just overdrive. Sunday, I was out in the yard all day mowing, moving things, and even carried some bags of soil around. On a normal day, I struggle to walk with a laptop bag. I also noticed my leg movement to be a bit better. This maybe TMI but one thing I can never do is get my last pant leg off standing up. It’s like a battle where I just pray not to fall. Without thinking about it I just slung the pant leg off and kicked it 5 or 6 feet. Absolutely amazing. No falls for the weekend minus me trying to walk down a muddle hill after a beer and a happy pill. I knew I was going to fall I just wanted to see if I could do it.

Overall though I just feel better. I’ve had moments of walking without my can where I don’t feel off balance and even some longer walks where I didn’t feel the need for it. My appetite also skyrocketed. Ate 2-3 plates at dinner last night like I haven’t in forever. Normally I’m just not that hungry but the beast wanted to be fed. I’ll keep an update on how this week goes but I’m pleasantly surprised over my state and have some hope of a positive reaction to the meds/placebo.

Day 125: And it begins

I could make a nice excuse about why I haven’t posted but I’ve flat out just been busy and not focused on it. Things haven’t gone too downhill health wise. My FVC is still above normal and while I’m progressing it’s pretty slowly (knock on wood). It’s easy for me to say slowly progressing but I think back to a year ago and I was much different. I could still get around well without a cane and now I can’t get 10ft without it.

Good news I start a clinical trial on Monday. While I’m excited I’m also a bit petrified by it. There’s no real big health risk but mentally I’m not sure how to deal with a 50/50 shot at getting a drug that could change my life. Many people have stopped progression completely and some have actually gained strength. So how do you mentally prepare yourself for not getting it? Then take into account that if I do get it I’m taking that gift from someone else. It shouldn’t get to me but it’s definitely thrown me off keel.

The second part of this is that this drug currently doesn’t offer compassionate care after the fact. Basically, I would get the drug for months, be taken off to see my reaction for months, and then thrown back into the world. I get it but I think it’s a tragic flaw in our medical process. Personally, I feel that people with terminal diseases should have the choice. If a company is testing a drug past phase 1 it means that tolerance and safety has already been tested. Why not let those needing the drug just take it? I mean the world terminal is pretty definitive. A great flaw we seem to have is trying to protect pharma too much.

I work a lot with pharma companies and understand they are in it to make money but where do dollars vs life get compromised?

Anyways everything else is fairly good. I’ve taken a step back in my compulsion to micro manage at work to limit my stress a bit. It might be causing more stress until everyone picks up the pieces but I’m going to force myself into setting life before work. That doesn’t mean I’m quitting my job. Contradictory to most other people I really enjoy work. I enjoy the rush, I enjoy the control, and I enjoy making things happen. On top of that I just like winning. What I do mean is I’m cutting out the 60 hours a week and the high stress. My body reacts so poorly to it that it takes days to recover. I’ve had a rough month or so and feel like it just drained me.

On the other hand, I fight to be energized outside of work. It’s one of the few things I have left that isn’t limited by what I can do physically. I struggle with yardwork which I loved and we just sold the boat due to it being too hard to use/maintain. Balance will be the key until I find a new hobby though.

Most likely I need something new to tinker with since golf, boating, etc. are out the window. Ideas welcome! I’ll try to update these coming weeks as I proceed with the trial. Doing a lot of praying between now and then so if anyone else wants to throw one out there I wouldn’t hate it.

Day 77: I’m back

 

I haven’t posted in a while. It’s been a pretty wild couple of weeks. I’ve written but not felt like sharing. So many things have been going through my mind and so many events have taken place.

I was hoping to have my next post be about starting a clinical trial. Everything was ready to go but it appears I have some liver issues right now. Heading to a GI this week to hopefully see some improvement but things got a bit scary last week with my ALT & AST levels going up over 500% in the last month. Just hoping it’s because of an adverse effect to the riluzole but regardless I’ve been taken out of eligibility of trials until I get within normal. Not going to lie it really knocked me down but I feel like it’s God’s plan and I just need to see where he is pointing me.

Thursday, we went to an ALS Jazz concert and while I was having a bad day it ended great. It was our dateaversary. Not sure that’s a word but 5 years before we met at a restaurant in Durham for our first date. We ended the night at the same restaurant. While my health is weak our love is stronger than ever. We’ve been through a lot in the last year and my wife is my rock. Without her I have no idea what I would do.

Then there was the talk of finally talking to my kids about the ALS. I vaguely remember that exact conversation with my father. I never really processed it and not sure the children really did either but they took it very well. Everyone in the house has a fighting attitude and I couldn’t ask for more. We all cried, we all hugged, and then the next day we had the ALS walk in Raleigh.

To be honest I dreaded the walk. Not because I don’t want to support the cause or others but because I hate feeling exposed or weak. We had a wonderful turnout and to the eyeball it appeared there were about 5000 people in attendance. Our team probably had 50 walkers some of which I knew and some of which I met for the first time. The outpouring of hope and positive attitudes were incredible. We made it probably 80% of the walk which is farther than I thought I’d ever make it. Somedays a trip to the mailbox seems daunting so doing a  1.5 mile plus walk up and down hills after 2 hours of standing felt great in a painful way. I think I wanted to prove to myself a lot and I felt good about it. Today however I feel like I could stab myself in the legs and feel better. I used to hate leg day when I was healthy and today is like that times 1000.

Regardless the event raised over $400,000 and our team pulled in a nice haul of over $14,000. I left this blog last time in a rather terrible mood and hoping I can get through these challenges and get a refresh. Seeing all my family together this weekend was definately a great start to that.

Day 57: Who Knows

 

I hate the barrage of questions that comes with this disease. The uncertainty can eat you alive. The waves of symptoms, the wondering if you are doing the right things, and the constant unknown clock that is ticking. It’s like I live in a world of borrowed time and have no idea what to do.

I spend so much time trying to figure out how to save myself, how to buy one more day, and how to survive for the possibility of a cure. For a long time, it was easy to ignore what was happening. I thought it would all be better one day and at the worst I would be without one working leg. Now I find myself obsessed with what is going next and how fast it’s going. Between the supplements, an eating schedule, a pill schedule, and the spasms/fasciculation’s it is a never-ending reminder that the clock is ticking.

Most days I can handle it but the last 2-3 weeks have been awful. My energy is down and when I try to distract myself with getting out in the yard or working on something I find myself more down by the fact that I can’t do something. I just put up a post about the cans and cants of my life but sometimes the cant’s weigh you down to a point where you feel like you are drowning.

Sometimes I dream about being normal and I don’t want to wake up. There’s a lot of things I’ve learned from dying but how to die isn’t one of them if that makes any sense. You can train yourself to be a better person and you can lean on your faith but how do you truly grasp the fact that its most likely going to happen?

Hope has always been my saving grace. I had hope that my symptoms were just celiac and heck last week I had myself convinced that b6 toxicity from years of energy drinks maybe a culprit but memories of my dying father just drag me back into it. Ironically, I pulled up the stages of dying.

  1. Denial- Been there done that.
  2. Anger- I didn’t really have that phase but I think I got my anger out for this disease over the last 20 years.
  3. Bargaining- Yea I’m not going to lie I’ve tried it. I’ve prayed and pleaded with God to spare me. Not sure if that one worked yet but I’ll let you know in 5+ years if I’m still alive.
  4. Depression- Think I just fell face first into that one.
  5. Acceptance- It’s going to be ok.

Oddly I think I had both some denial and acceptance early. I always try to see through things and grab the reality and I still know I can’t control it. The depression is like a sledgehammer though. I’m not writing this for sympathy but maybe someone reads this who has someone struggling in one way or another.

I’ve never really been depressed. I’ve usually mocked depression because you see it in so many situations that are controllable. This is one of the first things in my life I can’t control that obviously has the ability to end my life. So, what do you do when you have no idea how much time you have? If I knew that I was dying tomorrow I would know exactly what to do. Maybe the key is to treat life that way? That would work out fantastic if life didn’t need to be planned. It would work out even better if things like bills and work weren’t involved. It’s so morbid to think about. I was just about to order football tickets for the upcoming season and I have no idea if I’ll be standing. Things were so much better two months ago, when all I had was a bum leg and couldn’t see past that extent of my sickness.

Maybe this is all a senseless rant and I’ll feel better tomorrow. Maybe I need to force that. Heck maybe I just need to double down on an anti-depressant and relax. All I know is it’s a wall I need to climb just like any other barrier in life. I’ve been dying since I was born someone just pointed it out for me. Mortality is an awful thing to grasp and I think that’s why I’d appreciate another 40 years to grasp it a bit better. Maybe I’ll back up to bargaining again and see if I can swing that one by the big man.

Day 54: Stepping back and slowing down

I keep wondering how I used to do it. Things are slowing down a bit and it brings a lot of mixed emotions. 3 years ago I was 100mph all day every day. I was a workaholic both in and out of the office. My goal every week was to build something, work on the yard, hit the lake, and in general just go go go. Last weekend we attached some yardwork. It really brought some clarity to me on where I am in my life and progression. I can’t pick up a 40lb bag of mulch and really struggled just to walk around the yard. When you struggle to carry a 1lb plant and walk it gets real.

To break it down I just can’t do it all anymore. It almost feels like I’ve aged 40 years in the past 3 months. It really had me down for a few days. The frustration brewed and I honestly just felt like quitting my job and curling up in a ball. My job has been a focus for me for as long as I can remember. More than that I’ve felt like the weight of that world fell on me. If I didn’t do my job the best I could people would go jobless. I mean that completely literally. Recently I hired another guy under me. I’ve known him for years and worked together previously. Reminds me a lot of myself a few years ago, and is a real go getter with a positive attitude. I have another guy working for me who is analytical and very composed. I thought the marriage of the two would help but it’s gone so much better than I thought.

To shorten this to the point I realized I don’t have to do it all anymore. I can truly start delegating and helping them grow into my position. I’m actually taking today off and working from home. It was a breath of fresh air. So, while I can’t do some things I’m discovering I don’t have to kill myself trying. Probably a lesson I could have learned much earlier in life. Its pulled an immense amount of stress off of me in the past few days just to know I can be flexible in my hours and where I work from.

So, per the usual it’s just ups and downs and trying to live somewhere in the middle. As far as my health goes it could be better and could be worse. My “good leg” is progressively going to crap. It’s rigid and feels like I’m in a 24-7 cramp. Going to try a new magnesium treatment to see if I can level out the spasticity a bit but it’s a bit depressing. I know the next step when this leg goes is a wheelchair and I don’t know how I’m going to handle that. My first leg completely went to garbage in 2 years but I feel like I don’t have that much time on this one. I guess only time will tell but I’m trying not to think about it.

As far as other symptoms I’ve been lucky. My arms seem strong and while I have fasciculation’s I really haven’t noticed any upper body weakness outside of the random moments of speech problems. Some great things are popping up in research though. Edavarone is up for FDA approval in June which is promising and I’m looking at some trials. There are also 100 other promising things out there. It just seems like I’m on a timeclock. I’m praying they find something to help give us all suffering enough time for there to be a cure or way to stop progression. That time just keeps getting shorter for all of us. Until then enjoy what we have and live the best we can.

Day 42: More Ups, More downs

This week was a strange one. As some know I’ve been on a major crunch at work. It’s been almost a year into a project and made my presentation on Friday. For the week, I was on a jobsite which is less than convenient for someone like me. It was cold, lots of walking, and nothing was comfortable. This was going to be my last push before I go back to my comfortable office setting.

Ironically, I got out of my presentation Thursday and felt fantastic. Pretty much nailed it and it was just a giant sigh of relief. I went to check my phone and had missed a phone call from my doctor. I’ve been waiting since my diagnosis for my gene testing to come back. I really didn’t know what to hope for. If it came back blank it didn’t mean I didn’t have ALS it just meant I didn’t have the gene. If it came back with an abnormality there was more hope to treat in the future and a solid diagnosis so I could get into trials. In the back of my mind I’ve just been hoping its celiac gone wild. I’m only a month or so into gluten free and feel a bit better. I know that my body should be regaining its ability to process which is great.

The troubling part out of all of this was if it was genetic my kids could be dealing with the same issues hopefully far down the road. Well the call confirmed that I have the SOD1 gene abnormality which is familial. Hopefully by the time my children ever have to deal with this there will be a cure but it’s my youngest sons birthday this week. I haven’t really broken down throughout this process but I broke hard. For me it gives me hope and SOD1 is basically what all ALS research is built around. Per my wonderful doctor this is the gene abnormality they could have an answer for in a few years. Now I’ve been around ALS long enough to know they said this 10 years ago, but I’m keeping that hope in my back pocket. On the other hand, what and when do you talk to a child about this.

When my father was diagnosed, there was little known about the disease and no gene testing period. It was the dark ages of ALS research and all I knew is it was rare. Now I know my children are carrying a coin flip of having this gene. It pretty much floored me and I don’t know how to deal with it. I’ve felt like I was being strong but this is different. This doesn’t involve me or my wellbeing. We are going to talk to the kids soon and that scares me enough but I know the kids are going to investigate the disease further. I can’t imagine what will eventually cross their minds or if they will even comprehend it. It’s made the weekend tough.

I joined my wife out of town Friday and we had a nice night in Pinehurst but I just felt out of it. It felt great to get out but things are so crazy right now. To be honest my health is bipolar right now. One minute I feel great and better than I have in years and then the next hour I feel total weakness. I’m not talking weakness like after walking a mile I’m talking almost paralyzing weakness. My right leg has begun to cramp and have fasciculation’s much like my left leg did but it seems to be going so much faster than before. I had developed in my mind that I had 2 years before I’d lose my other leg. I’m blessed to not have many upper motor problems but it scares me to think I could be in a wheelchair soon. Pardon my language but it just scares the hell out of me. I’ve always been independent and losing the ability to walk, drive, etc. is my greatest fear. I think I fear that moment more than dying. Death happens to everyone but taking away the rest scares me.

Everything has just become so much more “real”. I feel like my coordination is better now and my PT says my strength is up but I just feel different. I’m trying to focus on the measurable things I can instead of “feelings”. I haven’t fallen in what seems to be months. For a year, I fell all the time. I could just be cooking and collapse. It all stopped when I changed my diet and overall I just feel more coordinated. Then I try to walk into an appointment and feel like after ten feet I can’t make it to the front door. The fear almost feels paralyzing. The weakness is just so random and the cramping scares me to death. My kids bought me a walking stick for my birthday last week and while I hated the idea of using it this week I’ve needed it far too much. I probably should have been taking it far easier on the other leg but just didn’t know better. Now it just feels too late.

The thought of clinical trials is exciting though. A few were brought up and I’m more than willing to try and help if it never brings a cure for me. Some of them do look promising though. That’s part of the highs that I’ve had. My goal for the week though is to make more highs and less low’s. All of this gets overwhelming and I have to learn to focus on the important things more!