I received some surprising news today that may put me into another funk if I don't try to nip it in the bud. Every case of ALS is different but all PALS "Person with ALS" suffer some degree of paralysis of their voluntary muscle movement which includes the breathing muscles. The main muscle for that is the diaphragm which contracts to create a vacuum in the chest cavity. This allows air to rush into the lungs causing them to expand fully and take in oxygen. I have taken this simple act for granted for the better part of fifty years now without a hitch. But over the past few months my diaphragm has begun to to weaken. The options I have are few but unavoidable. They range from simply breathing on my own. Breathing with a bi-level positive airway pressure, also known as BiPAP, which is a machine with a hose and a mask. It takes over my breathing when I sleep by forcing air into my lungs as I begin each breath and then reducing pressure allowing my lungs to deflate naturally. I use both of these presently and, on occasion, I even use the BiPAP during the day when I'm feeling short of breath (by the date of this post I am needing BiPAP 18-20 hours a day). Another option is to have a tracheostomy which is to have a ventilator "Vent" tube permanently installed into my throat to supply air to my lungs This is an invasive surgical procedure which for me, at this moment, is not an option period! The key impacts being the complete loss of my speech and the need for twenty four medical support.
A last option, that I was seriously considering, is a diaphragm pacing system DPS. The DPS requires the surgical implantation of multiple electrodes into the diaphragm muscle which connect to a power unit. The power unit then sends impulses to the diaphragm making it contract a specified amount of times every minute. This procedure, though invasive, seemed like it would have allowed me the best opportunity to maintain a good quality of life that's equivalent to the life I had before my lungs began to weaken. Yes, not much to cheer about but for a PALS to get back something they have lost it's a tremendous event to say the least. Last week I underwent pulmonary function test which I sent to the the Cedars Sainai ALS clinic in Los Angeles. They are the only doctors who perform the procedure in my area. I was informed that I am too ill to undergo the procedure. I learned from my Pulmonologist that my lungs are functioning at 50% as compared to my last pulmonary function test from sixteen months ago. That test was normal. The most serious news was that my normal breathing has diminished by 2/3's to 1.7 liters. When I drop to 1.0 liter I will no longer be able to breath on my own. Meaning I will be dependent on technology to live. This means that the nerve that controls my diaphragm has now begun to die. Resulting in the death of too many of the diaphragm's individual muscle fibers for the pacemaker to work effectively. This now leaves me with only two technological options. The first is to use my BiPAP more and more until I am on it full time for the remainder of my life. Or a tracheotomy to which I say no thanks! I'll stick with the BiPAP
Yet another setback in my life which leaves me with the thought of what setback is next. ALS has been nothing but these things and it's not that easy to just brush them off anymore. Just when we adapt to a problem another one pops up and we're back to playing catch up again. It is frustration to say the least. I figured I would try writing about this most recent setback in the hope that it would help feel better which it hasn't so far. I think I'll sleep on it and write again in the morning.
This morning I woke up around 4:30 a.m. (I know this because my neighbor starts his truck at 4:30 a.m. everyday). I laid there trying to fall back to sleep but my mind began to race and sure enough I was awake. Soon after that my thoughts turned to the bad news from yesterday. I kept hearing the words they used yesterday "You are too ill for the procedure." My thoughts were becoming consumed by them. Then I heard another engine start, It was my other neighbor, Bob, heading to work meaning it was 5:30 a.m. I glance at the blinds to see if it was still dark outside. It was. My mind focused on the remaining time I have to live. Soon I realized I may not see the year 2015 at the rate my capacity to breath is reducing. After a few minutes of this I felt a tingling sensation throughout my body that was pure feelings of sadness. I was falling into despair when a thought crossed my mind "Why don't I try to focus these feelings into the area of my chest over my heart. So I began concentrating on a spot over my heart. After a minute or so I had accomplished the task "Now what do I do?" I thought. "You have to lose it!" was the answer I heard. I had laid there unsuccessfully trying to do just that for a bit when Dee's alarm went off. "Six a.m.!" So Dee gets up and I tell her that I am sad. She comes over to my bedside, sits down, puts her hand on the very spot that I had been focusing on and started to gently rub my chest. Then they came! The tears of sadness. They flowed forth along with all of the sadness in my soul. "I'm sad and afraid!" I say to her. "So am I" she whispers to me as she lays her head on mine in the darkness. Through my tear filled eyes I look at her and say, "You are all I have ever wanted or needed since the first day I saw you!" She sniffles and rubs my chest some more. I continued on, "I am so thankful that you are here at the end of my life!" She continued to hold me as she said, "So am I." "I love you!" "I love you too!" We cry a bit more before I say "Kiss me!" Without hesitation, she touches her warm, soft lips to mine and we kiss, pause for a brief moment and kiss some more. As our lips parted, I felt the pressure of the sadness leave my chest. She hugged me and I told her to go get ready for work. As I lay there with the pressure lifted from my chest I became aware that the sun was up. I began to hear the birds singing and the world outside starting to come to life. It was a good moment for the both of us and that kiss may be the best one since our first! I will never forget it.
What I called setbacks twelve hours ago are not that at all. They are actually milestones on my life's journey. Milestones which, as I pass in front of them, seem so ugly, full of decay and sadness. But as I look back at them from the other side they are shining beacons that show me the work of art that my life has become. It's a good life I've led. A bit shorter than I thought it would be but a beautiful one indeed. I may be ill but I am whole! It then occurs to me that life goes on no matter what. My life still goes on too!