Bottom fell out...

There were quite a few options for a title.

Some of our reading audience would find this inappropriate, so I'll leave this picture as a subtle hint to the alternative title....sometimes you just have to lighten the mood!

The last "bad" appointment that we had was back in December 2016. That was the start of needing respiratory intervention and Jason had major weight loss. This was the beginning of considering PEG placement and placing a trach, all of which happened about 4 months later... Within a few weeks, I had quit my job, Jason began working from home. This was when Jason was falling daily and started the wheelchair and van purchase process. It was truly a "bad" appointment that started a whole lot of interventions in the next few months....this may have been when we realized that things weren't as slow progressing as the disease started. Our optimism wained and we realized just the effect ALS would have on our life. Well-interventions were done, there was stress involved and we got through it, and since then we've labeled appointments as "routine."

Unfortunately, routine just stopped and we had another set of "bad" appointments. I planned all these appointments well before the baby is due, all jumbled together to get them checked off the list and taken care of. In hindsight-it was simply too much. If the appointments had gone well, it would have still been too much. The fact that they went bad really made for a stressful past 10 days or so. We were grateful that Jason's parents, sister, niece and nephew were here for part of the week to distract us from all we had going on ALS related and there was also a baby shower right after thrown at our house where spirits were lifted and we could ignore ALS for a few hours! But believe me-we napped some kind of good on Sunday!

On Thursday, April 19, Jason had a 3 month trach change. We informed the doctors that Jason did complete the 10 day antibiotics back in March, but has continuously had fevers. What comes up with the cough assist machine is obvious to anyone that it is infection, unsolved by the most recent antibiotics. After a year of on/off infection, infectious disease (ID) was going to be consulted. This is a team that specializes in infections. It is also the team that comes into play when "weird" and rare infections come to light-like when Ebola patients were being treated in the US-and YES, Emory did treat these patients. A broncoscopy was done as always, and the lung team send all these results from previous trach changes to Dr. Marshall Lyon. Because it could take 3 months to get in with ID, our lung doctors communicated with him and came up with a regimen to try to solve our ongoing problem. Jason will take antibiotics all the time, alternating between Bactrim twice a day for 2 weeks and doxycycline twice a day for 2 weeks. On top of this, he will get inhaled gentamicin via a nebulizer. As of today (4/30/18), we have the nebulizer machine and I've gotten education on how to give the treatment, but we are waiting on the antibiotic that needs to come from a specialty pharmacy-all the way from Texas! This makes 3 pharmacies that we deal with-UGH!

For those that like the extra education-Bactrim (a combination of 2 antibiotics sulfamethoxazole and trimethoprim, TMP/SMZ) is common for prevention regimens for chronic immunosuppressed populations (ie. bone marrow transplant patients take on Monday, Wednesday, Friday for YEARS!) to prevent pneumocystis pneumonia-see article. Inhaled gentamicin is commonly used in cystic fibrosis populations-see article.

On Friday, April 20, Jason had 2 appointments, a yearly PEG tube change done in the office of a GI doctor. Then we went to a 3 month neurology appointment with the nurse practitioner. The PEG change went great-no issues, it was done right in the office. I got a backup one in case we have any issues because of my nursing background. We were in and out in time for me to get some lunch! And have time for Jason to eat as well so that we wouldn't get behind.

The 3 month neurology appointment did not go so well.

-For starters, he got a fever while IN the doctor's office, I had no Tylenol, and there was no pharmacy in the building. When he gets a fever, he is bright red and does not look good at all. I see it every day, but going to the doctor looking like that when intervention to "fix" it is still in progress does not look good.

-On top of that, Jason has lost 20% of his body weight in 3 short months. I can't even type the number. I cried! These appointments for months have been supporting what we are doing. They have been routine. And now, all of a sudden, we have to change everything we've gotten used to, all the while he looks a hot mess with fever that I did not prepare for...Maybe exhaustion played a part in it, but I think it was that I felt that I hadn't done enough encouraging full meals. It is also hard being a wife that knows what is best medical wise, while trying to respect Jason's wants/needs when he only wants partial meals. Not only has he not wanted full meals as ordered (he only wants 75% most of the time), fevers for nearly a month eat away at calories quickly as well. I have to balance encouragement, respect for his decisions, nagging about it, getting upset about it....I could go on and on, but we decided that more nutrition was needed. If bolus feeding was causing side effects, then we would try a continuous feed at night. This means another machine and another addition to routine. Home health was ordered again so that a nurse could come by and offer support/answer questions concerning the continuous feeding. As of writing the blog, home health has still not been, but we've completed 4 nights of continuous feed, with 3 of the nights being full 100% meals as ordered! Jason is tolerating well.

-When the nurse practitioner, Annie, would ask a question, Jason also wouldn't answer-showing that after months of practice, the computer doesn't allow him to free type responses, that he only uses what I've prepped for him to say, and that it causes more frustration than help most of the time. He would instead type something that he needed (cough, move my arms...) or I would have to figure out what he wanted to say. We have several ways of communicate without the computer because it was not a life changing device like we anticipated 8 months ago.

-Finally, a concern that we planned to bring up was that because of lack of movement, there is a small sore on his back that I wanted her to take a look at. By this time, it was the end of the appointment. She did nothing but say that home health could look at it and give advice/treatment. As of today, as said before, home health has not been. I think I'm doing a pretty good job with Neosporin and a waterproof barrier bream, but they may provide/order/recommend a dressing for it when they assess. Also, the one area I was concerned about has turned into several small areas-about 5, all on various parts of the back and sizing anywhere from a nickel to quarter. Pressure Ulcer information: Caring for them, Home Remedies-adding this for my own reference!

At the end of the day, I felt like the worst caregiver on earth! When appointments are routine, everything we are doing is encouraged and supported. I feel adequate. When they are "bad," it seems like nothing is right and nothing I do is right. But what can you do? Just keep keeping on. The next trach change appointment will not wait 3 months-we will go for trach change on May 17. The next neurology appointment also will not wait 3 months-we will go in 2 months on June 22.