How's Patrick Doing? #11 - Part II: Respiratory, Speech and Bulbar Changes

Patrick and Christian

This second part of Patrick's update concerns his respiratory, speech and bulbar region (head & chest). As time progresses, ALS has affected all his muscles; from the ability to move legs and arms to swallowing, speaking and breathing. As I've mentioned before, ALS starts in one of the following places: limb onset (arms or legs), bulbar onset (speech or swallowing) or respiratory onset - most of the statistics I've seen are about 30% Leg, 30% Arms, 30% Bulbar and 10% Respiratory. For Patrick it started in his hands and now affects all the various areas to some degree.  His hands and arms are the most impacted because it is the place of onset.

Respiratory and Coughing
There is no way to know Patrick's actual rate of respiratory decline. As I've mentioned in prior blogs, they haven't been able to get respiratory readings in over three years. This is due to his extreme decline in the bulbar region specifically his facial muscles. Years back Patrick participated in a trial at UC Davis where they were looking at using ultra sound technology to determine respiratory decline in ALS. I never heard the outcome of the ultra sound trial, but it seemed like a good idea.

Without any measurable way, the only way to determine his status is through his dependency on respiratory equipment and observation. Over the past 8 months he has increased his typical use of the trilogy (Noninvasive ventilator) from only overnight (6-8 hours) to about 18-20 hours per day. However, he is not fully dependent on the ventilator and can go without it for a few hours if necessary. 

He struggles with saliva and coughing which subsides while using the ventilator. So, part of the increased use is related to assisting in the management of coughing. I don't totally understand how the ventilator assists with this issue, but it does help.

When he is not using the ventilator, coughing can become quite severe. He has the added bonus that his body reacts to coughing with spasticity and clonus. Essentially, he begins coughing and his entire body becomes rigid and starts to shake uncontrollably. It's not pleasant to watch. If this happens in the car or while sitting at a table he often hits his shins which have a number of bumps and bruises.

We've had some close calls during transfers where he almost slides out of his chair as he begins coughing and becomes rigid and shakes. These are tense moments for him and me. Fortunately, I've been able to manage to get him hooked into his sling or tilted back in his chair before he slides to the floor. Unfortunately, being stressed, further exasperates these symptoms. 

Much of his coughing comes from a difficulty in managing saliva/mucus. With throat and tongue muscles compromised it's becoming more difficult to manage the fluids in his mouth. We have two machines that help with these issues: the cough assist, which does exactly that, it forces a cough. A mask is placed over the mouth and nose, the machine is activated and it pushes air into the lungs and then sucks the air out; hence a cough. This helps to keep the airway clear of mucus and brings up fluids. Then we use a suction machine, which is very similar to what is used at the dentist's office, to suck up the fluids in his mouth.

I doubt it's pleasant for him and it's not particularly fun to be the administrator, but it does cut down on the coughing and makes him more comfortable.

Food and Medications

Patrick has had a feeding tube for over a year.  He eats, drinks and takes medication exclusively through a tube. He simply can't manage food or beverage orally. When he initially had the feeding tube placed he still occasionally ate regularly, but sadly those days have passed.

The Ultimate Pill Crusher - Silent Knight

All his medications are given through the feeding tube. I've been through a number of different pill crushers and settled on the Silent Knight (thank you, Justin). This pill crusher is the easiest to use and does a good job of crushing the pills into a fine powder. You have to be careful with putting ground medication in the feeding tube, you don't want to clog it - trust me. I'm a bit of a pill crusher and syringe connoisseur.

We've looked at his medications to determine if they can be ground into a powder. Time released formulas are not meant to be ground up so we had to change a few.  In addition to prescription drugs you have to stay away from any over the counter medications that are time released. I try to find uncoated medicines or liquid formulas if available, these are the easiest and grind up the best.

Communication
Patrick's ability to communicate is extremely limited. When he's not using the Tobii (speech generating device) we use the a-e-i-o-u method I've described in past blogs. I try to stick with questions that require only yes or no answers. With his neck muscles severely impacted his ability to nod has diminished so we use blinks to answer yes or no. I also ask him to "make a noise" to designate yes. Making a noise works at 4:00am when he needs something and I'm trying to figure it out in the dark. This can get confusing though because of his use of the ventilator, he sometimes just makes involuntary noises.

Out Getting Nails Done - We take the Tobii & Respirator

During the afternoon Patrick uses the speech generating device (Tobii). I'd love to report that the Tobii works well all the time, but that just hasn't been the case. It would be awful though without the Tobii, so even with its challenges it is such an amazing tool. The challenges are twofold;

Firstly, it's a Windows computer, so all the problems you have with your computer he has too. Occasionally it locks up or decides not to work requiring it to be rebooted. The software requires updates and he runs the risk of viruses just like everybody else.

Secondly, it uses eye gaze to control the computer which is precisely why we have it, but it has its problems. For someone who is losing his neck and core muscles it can be difficult to get his eyes in constant aliment with the computer eye gaze. If he happens to be placed in his chair at a bit different angle or he starts to slouch, his eyes move away from the computer eye gaze and he loses connection. We spend a bit of time daily, calibrating the eye gaze, moving him in his chair, and adjusting the Tobii.

The other limitation is that it's just not practical to have the Tobii in front of him all the time. Generally he spends his afternoons using the Tobii but the rest of the time we rely on our a-e-i-o-u system or the use of yes/no questions/answers.

Eyes
Since his communication relies on his eyes either through blinking or using the Tobii, keeping his eyes comfortable is a priority. He continues to struggle with acid tears. I don't know what actually causes acid tears but if you "google it" you'll find its a common problem with ALS. The body creates tears that burn and sting.

Jake

Another problem is the occasional item that gets caught in the eye, such as an eyelash. This is a delicate problem trying to remove it without scratching his eye. We use eye drops, but flushing doesn't always work. It can be a bit of a project to get something out of his eye - I'd say my success rate is about 80%. 

Final Thoughts
The life of a person with ALS and his caregiver are not always a "walk in the park." We do our best to find good moments. We read, watch movies and hang out with a funny white dog. 

Hope you are enjoying the start of spring (at least here in California)! Here are some photos from some recent family events.

Patrick and the grand kids - Valentine's Day

At the Movies

Valentine's Day Dinner 2018