Quarterly doctor's appointment

Our last big appointment was in February. We had an appointment in May, but were in the hospital for trach placement and saw (and this is just my opinion!) TOO many doctors, so we never rescheduled. We had a lot of questions/issues to address.

Fevers: Since the trach was placed, Jason has had low grade fevers. Even while in the hospital, his temperature every night got to 99.7 and 100.1, but no intervention was done and a good explanation was given-his lungs were getting used to a new foreign object! At home, the low grade fevers continued, but we honestly did not measure them, as he would sweat and it would go away. Also, it might would only happen once a week or every 5 days or so and we didn't have a good thermometer. This is no excuse and makes me a terrible nurse. Jason never complains, so we just never made too big a deal of it. Anyway, while in Maine, Jason's mom got us a thermometer, so we've been measuring since July 10. Back on July 21, the temperatures started coming daily, sometimes 99.9, but up to 101.7. They were relieved by some tylenol. We asked the doctor if poor temperature regulation was to blame and if this was part of the disease process. Being hot/cold, taking blankets on/off, fan use on/off, sweating at times are all normal for anyone, but an actual fever with it is NOT normal. (And "poor temperature regulation" is not a common side effect of the disease) So, we were sent for blood cultures, a urine culture, and routine labs just after the appointment was over-more on this later.

Nausea/upset stomach: Since the PEG was placed, Jason has always been on the same formula. And unfortunately, it can cause upset stomach because of the amount of fiber in it. There are formulas without fiber. I am just not going to go in detail, but we want to stay on the formula we are on. Although there is a goal of calorie intake daily to maintain weight and nutrition, there are ways to change frequency and amount of feeding each time that we will try. We got a new prescription for nausea medication to have on hand, just in case. Also, if you aren't eating by mouth, then saliva is just annoying! Jason joked while we were prepping questions to make sure to "ask the doctor's for something to stop saliva" There is a drug out there that we will try. I'm not sure how the drops will taste, but if it works, it works!

GREAT NEWS.....Since May 1, we have maintained weight. His weight was within a pound of what it was on May 1. This is another reason not to change what we've been doing.

Grants: We did get some information from the social worker about programs and grants out there. We were also told that it seemed we were doing great emotionally with one another and with all that is going on, which always makes one feel good during an appointment.

Poor sleeping: Neither of us sleep well. If you've looked up anything about ALS, not being able to move/adjust yourself can make one uncomfortable. We do use a wedge pillow all the time, but Jason just needs readjustment often and the time of day doesn't matter. He doesn't want to take a sleeping pill though because when he does wake up from a forced sleep, he will really be aching. So, we may try over the counter melatonin.

We also asked about poor circulation, arm exercises, and a possible pulled muscle/strain on the ribs-which there is not necessarily a treatment for.

Newly approved study drug Endaravone: Emory does not have this drug yet. It was FDA approved back on May 5 based on results in Japan, but we knew it would take some time to develop a protocol and get insurance approval. The first step is to fill out an insurance form to see if they will even cover it for Jason. This drug is not easy to take. My understanding is that it is given via IV for 14 days straight. Then, it is given 10 out of 14 days (M-F with the weekend off for 2 weeks in a row) every month. It is not set up for home health yet, which means an infusion center. Unfortunately, this drug was only tested on patients that were diagnosed less than 2 years ago (that is us) but with almost normal function-patients without PEG or trach (not us). In this population, only 30% of the people showed a decrease of disease progression versus the placebo. Will this drug be approved for us? Will it even work for us? Hope and reality are 2 different things. Anyway, we will fill out the paperwork.

On our way home from this appointment, we got a phone call from Dr. Fournier, one of our 2 neurologists that gave us bad news. The CBC (complete blood count) showed twice the amount of WBC (white blood cells or immune system cells) that should be circulating. Normal range (depending on the hospital) is 4.5-10.5. Jason was at 21. This means that if these cells are circulating in the blood stream, that an infection is brewing. It was recommended that we go to the ER. She gave us a choice of 2 places to go and she called Emory John's Creek (closer to us than the Main Campus) ahead of us arriving.

We just bought ourselves another ICU stay :(

UP NEXT:

*Monday, 7/31: appointment to see about a communication device

*Wednesday, 8/9: 3 month trach change out

*pending insurance: some changes to the power wheelchair to make driving easier