To preface, I know this is a long blog but I want to provide a real life experience of what we are going through. When I first starting hunting through information and blogs it was hugely beneficial to me to read exactly what others were doing and experiencing. Things like the first ALS clinic seem daunting and overwhelming and if someone reading this helps alleviate their anxiety about their first clinic, then I want to do that. I also know lots of people are interested in the details and we are more than happy to share! 

We went to our first ALS multidisciplinary clinic on Wednesday. What exactly does that mean? We headed up to UW to check in at 7:45am and met with eight different doctors or therapists by noon.  The reason for doing this is so we don’t have to do all of those appointments on separate days and instead can meet with them all on one day once every 3 months unless otherwise needed. They work as a team and meet before and after they meet with patients to go over each patient together to make sure they all know the same information and can provide the best support. We met for the most part with 2 doctors at a time as many of them had similar questions so it was quite convenient. 

The different practitioners included:

• Respiratory Therapist
• Physical Therapist 
• Speech Therapist 
• Occupational Therapist 
• Nutritionist 
• Palliative care doctor
• ALS association representative 
• His main doctor (new to us)

Most of the doctors did not have much for him as he is still very early in diagnosis and symptoms and/or there isn’t much they can do to help with the symptoms. 

The nutritionist wants him to be eating 3000 calories a day with 120 grams of protein to maintain his weight. Keeping weight on as an ALS patient is very important as often once you lose it, you can’t get it back. Also he is slowly losing muscle mass and isn’t able to gain muscle. Once the muscle is lost, it is gone. Studies show ALS patients that can maintain weight do better. So we will be making sure to introduce things into his diet to make sure he maintains. He has only lost 3 pounds since his first visit at UW which is great.

We did talk about the future of getting a feeding tube with multiple of his practitioners and at what point we will pursue that option. A feeding tube is considered supplemental at first and not a complete replacement of food. Once he gets to the point where eating a meal takes over an hour to complete, he is losing weight or his desire to take the time to eat decreases enough then we will get a feeding tube placed. That way he can still enjoy eating meals with his family and what he loves to eat most and we can supplement with the feeding tube. Other key things to watch out for is if he starts choking on food (aspiration causing pneumonia is a huge risk), if he is losing weight or if his lung functions decrease enough that performing surgery would be dangerous. The UW highly encourages placing a feeding tube before you need one. 

The physical therapist didn’t have much for him as his strength and balance is still fine. He feels he is getting weaker in his arms and left hand but from a diagnostic perspective they can’t tell a difference yet. She has him working on his posture though as eventually the neck muscles will go and so the better his posture is now, the better it will be for him in the long run. She also wants him doing 30 minutes of medium to moderate aerobic activity per day. ALS patients cannot build muscle but keeping active is very important and so things like riding the stationary bike without tiring himself out is ideal. 

The occupational therapist (for those who are confused by what that is – they essentially help with mobility or daily life tasks like buttoning shirts and wheel chair friendly homes…etc) also didn’t have much for him besides us starting to think ahead about planning for mobility issues. She did give him a few hand exercises as he is feeling slight weakness in his left hand. She also talked to him about pacing himself each day as he is going to have new normals and wont’ be able to recoup as quickly as he did before. 

The speech therapist gave us some apps for his phone that he can use to communicate in situations that are starting to be hard like ordering food in a drive through. For the most part his speech is still good enough to be understood without asking him to repeat himself on good days. We also talked about pacing himself in terms of his speech so on days he has a busy evening, to try to talk less and rest more during the day so he saves that energy for the evening. She also connected him with her tech guy to talk about how to assist with work presentations to make sure he is understood or have something speak for him. We also talked a bit about the future and other devices that are available as his speech deteriorates more.

We already spoke to the palliative care doctor earlier this week and so she had some paperwork and documents for us to read through to assist with creating an advance directive, making sure his voice is heard with his wishes and also how to start talking to the kids about grief. She also gave us a reference for what is called rehab therapy and they help with transitioning from quitting working and going on disability…etc. 

The respiratory therapist did another lung test and we were surprised by his results. His previous tests showed 99% capacity and -80 MIP (Maximal inspiratory pressure – essentially diaphragm strength) in October. Yesterday he had 78% capacity and a -65 MIP score. He hasn’t noticed any physical changes in his lungs or capabilities and we were wondering if maybe having COVID in November might be contributing still to a decreased lung capacity. But this means we need to be keeping an eye out for symptoms of him not getting enough oxygen while sleeping between now and his next lung test in March. If he starts getting morning headaches, having restless sleep, vidid dreams or nodding off during the day then we need to take him back in for another lung test. If his scores get below 50% capacity and a MIP of -50 then we will get him a BIPAP machine for while he sleeps. Essentially a BIPAP (similar to a CPAP) just helps the diaphragm rest while sleeping so that it has more energy to work during the day.

The ALS association representative was super helpful in showing us resources that are available to us for borrowing equipment and other support systems in our local area. 

We also met with his new main doctor. We switched doctors due to getting into the ALS clinic so we will consistently see him every three months. He is one of the most sought after ALS doctors at UW that does the clinics so we were pleased. He doesn’t think he needs to see him more frequently than once every 3 months at this point since his progression is slow. He was pleased that his ASL score remained at 44. Most patients decrease 1 point per month. So for Dirk to remain 44 since October is great news and the hope is that the ALS drugs will help slow it down even more. We are still awaiting the Kennedys disease test but his doctor believes it will come back negative due to the symptoms and progression he sees in Dirk. They also tested his liver functions as Riluzole can cause increased liver functions and it came back normal.

He is currently taking Riluzole which he has been on since October. He has been on Relyvrio for 2 weeks and begins taking it twice a day on Monday. Radicava also arrives on Friday and he will begin taking that as well. Radicava can cause some balance issues though so prayers that he doesn’t experience that. Those are all the FDA approved drugs at the moment. His doctor has prescribed Nuedexta which treats the pseudo bulbar affect (essentially uncontrolled laughter) but said there are some studies it helps with the Bulbar symptoms as well. Both of those combined made us decided to try it. We weren’t going to try it just for the laughter as it hasn’t bothered him or us yet but based off of his new doctors recommendation he will start on that soon as well. 

Overall, we greatly appreciate the opportunity to do the ALS clinic so we don’t have to drive to Seattle 6-7 times per month. It was also nice to have a team of doctors who are all on the same page about his care and needs so we don’t have to communicate the same information to each of them! One of the hardest parts of all of this has been having to emotionally go through the same process with each provider. So by being able to combine them all it greatly eased that burden. They made it very clear they are all here to help us in whatever way we need help and we are very thankful for them and their support. 

Prayer requests:

• That his stomach would continue to tolerate all the drugs and he would have no adverse effects. 
• Prayers the drugs would work at slowing his progression and the plateaus would continue and get longer. 
• That he wouldn’t aspirate food or water into his lungs.
• Wisdom for us as we start working on paperwork like living wills and advance directives. 
• Energy for both of us as we navigate this disease daily. 
• Wisdom as we pursue things like wanting to move into a new more accessible home in the new year closer to our support systems. 
• That we would be able to maintain his weight.
• And that we would have wisdom in talking with Adeline at some point. So far she hasn’t noticed anything (or at least not enough to mention it) but we definitely need wisdom in how to eventually talk to her. 

And just for fun, a few photos from Adelines “Christmas tree” themed birthday party!