We just had the most recent ALS clinic up at UW. As a reminder the ALS multidisciplinary clinic is a day where we spend the morning at UW and meet with all Dirk’s doctors in one day every 3 months rather than having to schedule out all of those appointments separately. We spend about 45 minutes per provider or per two providers who are paired up together.

The week before clinic we have a call with Winpei, the lovely ALS RN who manages the clinic and is our main point of contact for his medical team. She checks in on how we are doing, gathers any questions we have for his team and also does the ALS functional rating scale questions to determine Dirk’s progression. 

As a reminder, what is the ALSFRS? 

The Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS) is an instrument for evaluating the functional status of patients with Amyotrophic Lateral Sclerosis. It can be used to monitor functional change in a patient over time. It measures speech, salivation, swallowing, handwriting, cutting food and handling utensils, dressing and hygiene, turning in bed and adjusting bed clothes, walking, climbing stairs and breathing. A score of 48 means you are perfectly healthy with no issues. 

A regular persons score is 48. In October Dirk was a 44 and remained a 44 through December. As of now he is now a 39. Most ALS patients lose 1 point per month and so we were hoping for a slower progression than that, but it is also very up for interpretation. It is possible that in December we could have answered a few questions yes that were borderline as well so its possible that measurement was off. But nonetheless, he has overall progressed in many areas.

Our morning started off with his Speech Therapist and Nutritionist. They didn’t have too much new information for him as his progression in their areas of expertise has been slow. He is still eating fine, has gained weight since his last appointment and his swallowing hasn’t progressed much. We did review a few apps he can have on his phone to help communicate but for the most part the people around him on a regular basis can understand him well. They estimate he is 30-40% intelligible. If he had a job where he spoke more or had to be out in public more, then he would most likely need to use an alternative form of communication. But for now we still understand him with a little bit of repetition and context. We discussed pursuing additional communication devices at the next clinic in 3 months. But as long as his hands still work well, he can use apps on his phone or computer when the need arises. 

We then met with an ALS association member to review his progression and see if he is in need of borrowing any equipment. They have an awesome loaner equipment closet for anything above $200. They also gave us information on community groups and guidance on talking to children about ALS as well. At the moment he is not in need of any equipment which is great.

Respiratory therapy was our biggest concern as we were fearful of his lung scores dropping. She didn’t see any immediate cause of concern upon asking questions and examining him. After doing some lung tests his scores have IMPROVED slightly. As a reminder in October he was 99% capacity and -80 MIP (Maximal inspiratory pressure – essentially diaphragm strength). In December he had 78% capacity and a -65 MIP score. Yesterday he had a 80% capacity and -75 MIP score!! If his scores get below 50% capacity and a MIP of -50 then we will get him a BIPAP machine for while he sleeps. This was a huge encouragement to us that we were not expecting!

The Respiratory therapist did send us home with a cough assist machine. Not because he needs it (and doesn’t even qualify for one via insurance, so he got a loaner from UW) but because she wants him to become familiar with it and have it on hand in case he gets sick. Since his lungs are still good, we want to keep them that way. If he aspirates anything other than water or gets a cold, then he should use it to help clear his lungs to avoid infection. 

After that we met with his main doctor who overall agreed with all the other providers assessments. He saw overall progression throughout but said his arms and legs didn’t exhibit any strength changes that were obvious from their perspective despite Dirk feeling like they were weaker. Overall he is weaker and slower almost in all areas of his body but not enough to stop him from doing daily life. The biggest changes have been in speech and his energy levels. 

He did recommend starting a B12 injection twice a week after some positive trials on the supplement at slowing the disease progression. It doesn’t have any side effects and targets the disease in a different way from his current drugs. So I will be getting trained on how to do those for him and begin those soon. 

After that we met with Physical Therapy and Occupational Therapy. Again, not too much to report from them besides Dirk feeling like his walking has changed slightly. The PT observed his walking and recommended a few changes to be more aware of his core when walking which fixed the sloppy feeling he was having. But she didn’t deem him a fall risk or recommend anything else which is good. 

Overall, it was a pretty good clinic day and better than we anticipated. All around, he is weaker and slower at doing tasks but still able to do everyday tasks without assistance. He can still eat fine and isn’t avoiding foods and his lungs look great! The biggest thing is his speech and communication, but at the moment we don’t need any intervention on a daily basis but anticipate changes in the next 3 months. 

Prayers:

• That his progression would slow more!! And/or that the disease might jump to other areas of his body than his lungs.
• That his lungs would stay healthy an he wouldn’t aspirate food or drinks.
• Prayers that he can avoid sickness.
• Prayers that he wont have any falls or injuries.
• And that we would have wisdom in talking with Adeline at some point. She has started to have trouble understanding him once or twice a day and so we foresee the need to talk to her happening soon.
• Pray for energy for myself (Rebecca). Small tasks are slowly being added to my plate (like doing both the girls bedtime routines rather than just 1 girl…etc) that are draining and wedding season is also approaching quickly.
• Praise for his weight maintenance and even gaining!
• Praise his lungs haven’t progressed! 
• Praise he hasn’t had any reactions to his drugs! 

Some fun photos from the last few months.