Our Family's Journey: May 2017

Anthony and Patrick

I've been asked, "why do you write a blog, its so personal." My experience is that in the absence of information, people make things up. So, I continue to share this blog, to make sure we tell our own story - including all the awkward, uncomfortable and difficult stuff. My hope is that our story will help bring understanding to an awful disease and how families manage through all the changes.

Throughout this journey both Patrick and I run through the five stages of grief, in no particular order. This includes denial, which is a comforting stage of grief and I believe gets a bad rap. Denial is simply a coping mechanism that allows the mind to process things more slowly and when ready. The tough part of writing "How is Patrick Doing?" blogs is that I must shed what comfortable denial blanket I'm wrapped up in and assess how the disease has progressed.

Recently, I completed forms to continue Patrick's long term disability claim. Yes, it seems a bit silly to prove his medical condition at this stage, but they require it annually. This year the ALS Clinic completed their section and sent it back to us for submission. On the form, under "Nature of Medical Impairment, i.e. loss of function" the doctor wrote "quadriplegia, chronic respiratory failure, dependent for all ADLs" (activities of daily living). I read this a few times and found myself pondering all that it means. When you are involved with daily living it's hard sometimes to step back and consider the disease progression.

Whether I live in denial or not, the ALS continues to move forward and force adjustments to our regular routine. So, I continue this blog with as little denial as possible...

Mobility
Patrick has lost most mobility including his hands, legs and arms. When he is getting dressed his arms often fall to his sides like dead weight. His legs have some movement which allows him to lift a bit and help when rolling over in bed, but not much else.

One issue that happens with a lack of mobility is a concern for bedsores and pressure points. Patrick has lost much of his muscle tone, he has bony parts on his back that can be very uncomfortable. We had the back pad changed out for one that is more comfortable and allows the back to "breath." Additionally, they added "wings" to the sides of the chair to keep Patrick from falling out - they keep him propped up.

His neck muscles have weakened making the head array controls for his chair too difficult. This leaves him dependent on others to move his chair. A tough change, first he had to give up driving a car and now he's lost control over his power chair. This created a problem in using the lift in our house - how do I manage the power chair by myself. We worked with the power chair company and they were able to add a controller to the front and back making it available to me both on and off the lift.

Over the past four years, Patrick has gone from cane, to walker, to scooter, to power chair, truly a reflection of the disease progression. So much of the equipment we use is temporary which can be expensive and creates a constant need for training and adjustment.

Feeding Tube, Eating and Medications
Patrick receives his nutrition, medication and water through a feeding tube. He can still manage some foods by mouth, but it is quite limited and primarily he only eats for enjoyment. Ice cream is a favorite!

We've learned how to use the feeding tube, including taking medications; medications must be completely crushed, broken open or liquid to put through the tube. Patrick calls all the syringes, supplies and equipment my chemistry lab and it sort of has that feel. You learn as you go; from managing the tube under clothing, dressing the wound, flushing the tube and avoiding stomach acid that often comes back up. Just like everything else we adjust and create our own processes. There are things that you discover and routines you create that make life much easier.

Respiratory Challenges
The biggest changes we've seen over the past five months is his respiratory decline. It seems like a rapid decline, but I'm not completely sure that is true. The ALS Clinic has been unable to get accurate respiratory readings in over two years because of his severe bulbar decline. It's likely that his respiratory has been declining for the last few years, but the only test is in observation. Patrick is using the respiratory equipment more often throughout the day and when he's not using it you can hear how his breathing is quite shallow. Over time ALS patients lose their ability to breath deeply enough to exhale the CO2 and require the assistance of respiratory equipment.

Jake still finds a way!

Over the past few months, the respiratory therapist has come over to adjust settings, provide new supplies and equipment, and assure that everything is working well for Patrick.

Patrick has some of the classic symptoms of respiratory decline; the increased use of respiratory equipment, headaches and difficulty lying flat. These symptoms have become more apparent over the past few months.

Other Stuff: Acid Tears, Spasticity, and Getting Comfortable
One strange symptom of ALS is acid tears, which burn and are quite painful. Recently, they have become more prevalent and seem very uncomfortable. So, we do quite a bit of eye wiping.

Throughout this journey Patrick has suffered spasticity. Spasticity is the stiffness' or 'tightness' in his already weakened muscles. This continues to cause him discomfort and amongst other things is triggered by coughing, which unfortunately has increased. Recently, we went to the movies and Patrick was coughing and struggling with spasticity. Because there was a counter in front of us, he banged his leg repeatedly against it. His leg was bleeding and pretty sore by the end of the movie.

With most of his muscles compromised, getting comfortable is a challenge. When you settle into a chair or bed you do a lot of fidgeting. With Patrick unable to fidget and move around he has a hard time getting comfortable. A considerable time is spent helping him adjust his arms & hands, pushing on his shoulder because of the way he leans and adjusting the chair back and forth. It's also critical to get him from the Hoyer lift to the chair making sure that he is straight in the middle of the chair. It can be quite frustrating at times, but if he is placed incorrectly he will be uncomfortable for the rest of the day.

He continues to deal with constipation issues. One of the greatest difficulties with not walking or moving around is that the body struggles with its bowels. Body movement is what keeps things working in the digestive track and without this movement things don't quite work right. We continue to try to find balance in his diet and supplements to lessen this problem.

With the increase of water in his diet, it also increases the need to urinate. This can be difficult when we are outside the house for several hours. We spent a "lovely" day watching Men's Liberty videos on external male catheters. You know I love movies:). Daringly, we went to the movies for the first time with the Trilogy (respiratory equipment) and wearing a catheter. Honestly, I was worried, but it all seemed to go without incident.

And Life Continues...
Life continues through all things... good and bad. Here are some recent photos...