April 13, 2018

CF Clinic

Saturday night, Azer started to feel like he was getting sick. Sunday morning, he was very sick. He had a high fever, lower back pain, and a sore throat. Fearing the flu (again), I went to have him tested, and luckily he was negative for the flu, but positive for Strep throat. He was prescribed an antibiotic, and initially felt better, but Monday evening, the back pain returned with vomiting. He was able to stay hydrated thanks to the G-tube, and by Tuesday, most of his symptoms were gone. As with just about any virus or bacteria, it made his lungs a little junky, so his PFT's were down yesterday. His lungs sounded pretty clear otherwise, so most of it was drainage from his sinuses.

Speaking of sinuses: It seems like the nasal rinses with the added vial of Pulmocort are working. He noticed he was able to smell a little bit a few days ago. He also noticed that he's been flushing out some blood from his sinuses, but apparently, while steroids reduce inflammation, oddly enough, sometimes they can cause a little as well. The doctor explained that the tissue in the sinuses is very thin and fragile and it doesn't take much to cause them to bleed, and not to be alarmed, that this is very normal, and to keep going!

None of the doctors were able to really explain the back pain for sure but, sometimes Strep can irritate the kidneys. It could have also been pancreatitis, which apparently does not occur often with Azer's CF mutations, but it does happen. Even though pancreatitis typically causes abdominal pain, the doctor said some CF kids only feel it in their back. The doctor said it also could be from kidney stones, which are much more common in CF.

Yesterday, they measured Azer's height, and he is actually growing! The growth hormones are working, and he is also putting on muscle. Unfortunately, he isn't putting on weight to go with it, so his BMI went down. The GI doctor said we can try adding Periactin to spur on his appetite a little bit. The Pulmonologist also agreed it would be great, as Periactin is also a type of allergy pill. When it comes to allergies, Azer is pretty much symptomatic to most things during the spring. The GI doctor said we can also try to add a calorie additive to his overnight feeds to try to increase caloric intake without increasing volume (which sometimes can cause his reflux to worsen). Azer has been wondering lately what he would have to do so he wouldn't have a G-tube anymore, so he spoke with the GI doctor about it. She said he would definitely have to eat three meals a day, and make sure to add fatty additives to them (like butter, heavy cream, extra cheese, ranch). It's also difficult to consume thousands of calories through food alone, and he'd likely have to supplement with several shakes a day. She also said that right now is a critical time to keep his nutrition high because of the growth hormones, and if he's not taking in enough calories, the growth hormones just won't work. This is something we can revisit in a few years.

Azer told the Pulmonologist that his ears have been blocked and hurting since Monday. She looked in his ears, and saw that he has double ear infections. Because of this, she is changing the antibiotic he is currently on for the Strep, to a different one that should be better at targeting his ear infections. Azer had a baseball game yesterday evening, and he reminded me of a game he played when he was 6 years old and he also had a double ear infection that day. That day, he was standing on the pitcher's mound crying because his ears hurt so bad, but he wouldn't get off the field because he wanted to play so bad!

Azer also brought up a concern he's had since December. He told the doctor that when he sprints, he has pain in his right lung, and that he was worried the pain was from the partial collapse he had from the flu. The doctor assured him that from his last X-ray, that area has recovered, and that the pain may be from allergies. She said she was fine with him doing Dulera and Qvar twice a day (two inhaled steroids) for the next 6 weeks. She says she has some asthmatics on this combination and they do well (she said the Endocrinologist might not be as pleased with this, as steroids can inhibit growth).

Azer met the clinic's genetic counselor for the first time yesterday. She discussed the genetic side of CF, how someone acquires it, and also what the chances are of his possible children having CF. We talked about what his CF mutations are. He has one common gene, and the other is very rare, and isn't even in the database. With all this talk of treating CF on the DNA level, I asked her about the possibilities of treating his common gene, but unfortunately, there isn't any drugs in the pipeline to treat his variance at the moment.

It's been about a year, since Azer was first evaluated by the clinic's physical therapist. She measured his form in several places, and saw that he was digressing again. She said he may be degrading from puberty and growing. In order to correct this, he will be needing to see her again frequently this summer.

Azer's G-tube has been bothering him for a few months now. The skin around the stoma has been cracking and irritated. An ostomy nurse came and looked at it. She found he only had a little over 1 cc of water in the balloon. Azer had removed some water for comfort a few months ago, but since then, he's lost a little weight making the button fit loser. The nurse but the recommended amount of water back in, and so far, it seems to be fitting much better!

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