How is Patrick Doing #8?, Part I - Speech and Other Bulbar Changes

Patrick's progression over the past six months continued to challenge us. We are now faced with a new set of declines involving the bulbar region (head and torso). These changes are related to his chewing and speaking They are sad and trying and we continue to work to find ways to adapt. I'll admit I've had some profound moments of frustration, where I just kind of throw up my arms. The disease never relents, it may slow or plateau but it never relents. Part I of this "How is Patrick Doing?" blog is a discussion of the challenges due to bulbar decline. Part II will go over some of the continued mobility problems and the medications we're using to combat these symptoms.

With ALS everything is about time and progression, you know that the changes will come but you don't know when. We've witnessed other patients progression, but they vary by individual so there is no road map to prepare for the things to come, you only know they will come. I'm not sure if it would be comforting to know what's next or how soon, sometimes I wish I knew, but it wouldn't really change anything. There has always been an overlap with Patrick's issues; for example we now struggle to understand Patrick's speech but in truth his voice hasn't sounded "normal" for over a year. So this is a current challenge but certainly not something that happened overnight.

The Importance of Communication
There are so many losses in this disease. An ALS caregiver described the losses as a deck of cards, every week a card is removed and you are left with the remaining cards. ALS is a relentless foe, so eventually it takes all the cards. By far the greatest loss over the past six months and perhaps throughout this entire journey is Patrick's ability to speak. At this point I would say I can understand 50% of what he's trying to say, but if I'm distracted or if he's tired I probably won't understand at all.

The loss of speech creates such a void for me and him. The conversations while driving in the car, while going to bed or while sitting outside on the porch are becoming less and it is so frustrating for him when I ask him for a third time to repeat what he just said. He has such a quick wit and always makes me chuckle but if I ask him to repeat it over and over, it simply loses its impact. He will sometimes give up trying to say something and I fight the urge to simply smile and nod which often folks do in response to not understanding. Frankly, he knows that they don't understand. Sadly, I've become the translator for him in most situations.

At a past support group I remember a conversation where a caregiver was complaining about taking over the driving responsibilities from her husband and she was frustrated because her husband was an awful backseat driver. She just wanted him to "be quiet." After she said that another caregiver responded sadly, "be careful what you ask for..." her husband had recently lost his ability to speak. Frankly, I felt sorry for both caregivers - I hate backseat drivers and I hate Patrick losing his speech.

Johannes assisting with SGD Setup

We are fortunate to live during this time, a hundred years ago there would be no real method to communicate with an ALS patient except perhaps yes or no questions through blinking. But today we have speech generating devices (SGD) that patients use to continue communicating. I'm sure you've seen them being used by Stephen Hawking or Steve Gleason. The device is attached to the front of the wheelchair and controlled by eye movement. Patrick received his Tobii Speech device in November and has been working to learn to use all its features.

To say using a speech device is the same as speaking would not be true. The device follows your eyes and you select phrases or words by allowing the cursor to dwell on a selected item. It takes some time to put a sentence together and makes it near impossible to contribute to an active conversation.  OJ Brigance, of the Baltimore Ravens, who was diagnosed in 2007, explains it well, "I have been able to communicate well with my DynaVox, even with the loss of my voice. The only frustration is the inability to communicate real time and conversations. By the time I finish typing, the topic may have changed, so it requires patience. With that being said, praise God I can still audibly communicate."

Moving your eyes in this manner has its own level of exhaustion, the eyes were simply not made for it. I find that conversations are becoming more directive: I need this, please do that etc. We both agree that loss of speech is the worst card that ALS removes from the deck. As with all his losses we are learning to adapt, but this is like removing the joker from the deck of cards. The joker fills in when something is missing so without it there is a big void.

Recently, Katherine Adraktas with the Sacramento ALS Association did a video of us to promote the speech workshops and loan closet services provided by the association. Patrick is using a Microsoft Surface and Quha Zono wireless gyromouse in the video. The mouse works by moving the head. He is now slowly making the change to the eye gaze technology but he's used the Zono mouse for about a year.

A word to fellow caregivers and ALS patients, the process of getting a speech generating device is long, for us two months from start to finish, so don't delay in getting the process started. 

Other Symptoms
Respiratory: Patrick continues to use a breathing machine at night called a Trilogy. This machine helps his diaphragm rest by forcing air in and out, a little different than a c-pap that only provides a constant flow of oxygen. Unfortunately, because his facial muscles have become so weak, Forbes Clinic does not have the equipment at the mobile Sacramento clinic to measure his breathing capacity so we don't know his current breathing levels. However, he doesn't need the machine during the day which would indicate a decline so we are still in the beginning stages of respiratory decline.

Chewing and Swallowing: We use our tongue and muscles to move food around, clean out our teeth and make things ready for swallowing. They work together to assure that food makes its way down safely. This is a challenging area for Patrick and it has slowed his ability to eat, as well as making him avoid certain foods that are simply too difficult. We combat choking by cutting bites that are not too big and medications are easier to digest with a thicker liquid - we use juice. Water can be a choking hazard for an ALS patient, which still sounds odd to me, but it is true.

Odds and Ends: Patrick has some odd ALS symptoms in the bulbar region. Recently, he developed difficulties breathing when I baked bread and there was a strong baking smell. After discussing this at clinic the respiratory therapist concluded that this was due to the salivary glands developing spasticity when they were stimulated by the smells. No, there was nothing she could really do, except to say "that's a bummer." I couldn't agree more...

Another symptom is called the pseudobulbar affect which causes amongst other things exaggerated emotional responses. It's an over reaction like excessive crying or laughing to something that does not warrant it.  This has been an ongoing issue for Patrick and is common in ALS patients. He does have medication that helps keep this under control. We got the medication when he started crying when people were eliminated on Dancing with the Stars.

A Note of Warning
Part II of this blog is primarily related to our journey navigating the use of cannabis for managing his symptoms (there is plenty of humor in it). If this insults your sensitivities or you simply think it's wrong you may want to skip it. For me I'm in this journey for Patrick, if it helps with some difficult symptoms, I have no problem with him using marijuana. With all the medications he takes, challenges he faces and severity of his diagnosis the moral issue of his use of marijuana is just not significant to me.

I wish you all the best as we start this new year! Here are a few recent photos:

Us

"Storytime"

Patrick and Christian at his 8th Birthday Party