I'd love to say that there has been no changes in the past seven months but that wouldn't be true. Writing these blogs are difficult, primarily because they force me to consider what changes have occurred and where we are in this journey. I don't think often about how far we've come, I find the this line of thinking depressing, so I stick to the here and now and contemplate the future. I completely appreciate being in "denial" - it's a really warm, fuzzy and comforting place, but in the ALS world, denial for any length of time will create tremendous hardships. So, for most of the time I try to stay in the "now" and prepare to face challenges and changes as they occur.
The easiest way to consider the ALS progression is to look at the various areas affected by the disease. At this point ALS has impacted most regions of Patrick's body - some worse than others. The initial onset area of his hands being the worst and the bulbar region being the best.
Bulbar Changes
The term "bulbar" is used quite often by ALS medical professionals. Until Patrick's diagnosis, I don't think I'd ever heard the term, "bulbar" with regards to ALS. "Bulbar" refers to the region of the body that includes the head and torso. The bulbar region involves primarily swallowing, speaking, and breathing. This is the most critical part of the body and ultimately why ALS is a terminal disease; we have to eat and breath to live.
Patrick's breathing is down from over 100% in December 2013 to 70-80% (two different tests) this past week, from a clinic standpoint he is bordering on "normal range." He doesn't seem to notice much difference, but he has started using a breathing machine at night to help while he sleeps. Breathing tends to be more difficult when you lay on your back so the machine helps throughout the night.
There are various mechanical devices available to help with breathing and as he progresses he will need to consider these options. Currently, the discussion at his clinic appointments revolve around considering a surgery that that will implant a diaphragm pacing system (DPS). The DPS stimulates the diaphragm muscles with electrical impulses. These impulses help the diaphragm with the patients breathing. The DPS has been implanted for a number of years and was approved by the FDA in 2011. Here is a link to the FDA information.
At this point, I'm happy to report that his breathing is too good to have this surgery. But we know that the progression continues and eventually he will need to decide whether to get the DPS implant.
I'm not aware of any debate on the DPS system for easing breathing comfort, but there is debate on whether it extends the life of an ALS patient. The FDA does not require the same trial studies for mechanical devices as it does for drug approval. The ALS medical community has some concerns over the claims the device manufacturer makes in extending an ALS patients life expectancy. The Forbes Norris ALS Clinic, where Patrick is a patient, is doing a trial study on the pacing system. He does not have to join the study to get the implant since it is already FDA approved but it is still a consideration. He is now deciding whether or not to get the DPS system or joining the study and allowing the study to determine whether he gets the system (2/3 get the DPS System Implanted, 1/3 do not).
Speech
If you've seen Patrick lately you know that his speech has been impacted by the disease. Over the past few months we've seen a significant decline in his speech. The words come out slowly and occasionally he needs to repeat words for understanding. We are working with a local speech therapist to consider what technology to use to augment his ability to communicate.
At the beginning of the ALS journey Patrick did "voice banking." Voice banking is recording 1,000s of phrases and words, so that the voice you hear through the electronic device is your own instead of a mechanical one. The clinic encourages patients to voice bank early in their diagnosis while their voice is strong to be used later if the ALS patient loses their ability to speak. Here is an article on voice banking.
There are a couple of different devices available for speaking and we are just in the beginning stages of looking at them. Sadly, there have been some drastic changes with Medicare coverage in the case of speech devices. In the past, Medicare covered the cost of a speech device on a monthly rental basis. The patient used it to speak, respond to emails and surf the Internet through eye movements. Medicare recently changed this benefit and is "locking down" some of the features on the device such as email and Internet. Sadly, Medicare is not even allowing the patient to pay for this features to be unlocked. ALSA and others are battling this decision on a federal level and there is a bill going through Congress that would revise this decision, but even that bill is controversial in the ALS community. The wheels of change in government move slowly, but Patrick's ALS progression does not. So, we will make a decision based on the way things are and hope things change in the future.
If you've read my past blogs you know that ALS is an expensive disease. The cost of these speech augmented computers are over $10,000. At this point we are looking at alternatives and I'll keep you posted.
Mobility
Patrick's mobility is limited. His hands, where his ALS started, are greatly impacted. The left hand is better than the right and he uses one finger on his left to click the mouse on the computer. His hands become fatigued so any extended time on the computer is difficult. At this point, emailing, texting or Facebook posts are 98% of the time done by me, so don't send him anything you don't want his wife to see:) Eating, dressing, showering, brushing teeth, etc. etc. all require help at this stage in the journey.
His legs are impacted but still allow him to stand and move a few feet which helps to transfer or stand over the sink. He is able to control his power chair with a "T" shaped control that moves by rolling the hand back and forth. Many ALS patients opt for a joystick type device which for him would not be usable. In the future, he will likely be set up to use his head to control his power chair. The technology of today is amazing and has made our lives much easier.
Odds and Ends
There are other symptoms of ALS, which are common among ALS patients but because they seem trivial in comparison to other challenges are often ignored. I'm constantly applying moisturizer to Patrick's skin, his skin has changed drastically which makes him itchy and blotchy. After mentioning this to an ALS neurologist he said "yeah, that is common among ALS patients." For someone who can't "itch" when necessary - the skin irritation can be quite annoying.
Recently, Patrick also complained after yawning that his tears were burning his eyes. Apparently this too is an ALS symptom. ALS is a strange disease.
The Decisions on this Journey are Personal
As we move forward in this journey, I think it is important to acknowledge the decision making process with ALS. There is no cure for ALS, but there are mechanical interventions a patient can choose to prolong their lives. These decisions are personal and an ALS patient considers these options based on their own quality of life, personal beliefs and religion. Patients can chose absolutely no mechanical interventions (from feeding tubes to tracheotomy), utilize all interventions available and frankly everything in between. There are no wrong or right answers.
I have tremendous respect for ALS patients making these decisions, they are not easy. I have one recommendation to you, don't be a passive participant, we often don't like uncomfortable conversations so we avoid them. I recommend that you put together a will that includes medical directives and end of life decisions. Select someone close to you to be your voice. Make sure your love ones know what you want, don't leave them to "figure it out" or you may find decisions made for you that are not of your choosing.
And So it Goes
Throughout this journey, our life continues; we have good days and bad days like everyone else. We continue to find the joy in the day and often simple things make us laugh. Patrick is his usual self with his sense of humor intact.
I'll leave you with a smile, here is Patrick and Jake... They ride the lift together, and fight over the same blanket on the couch. Patrick says Jake is worth his weight in gold...
