Still Coughing A Lot

About a week into doing the inhaled steroids, Azer still was coughing so much that he was feeling very fatigued. He had another telemed visit with his Pulmonologist. There didn't seem to be a lot of options on what to try next. Azer suggested an inhaled antibiotic, Ceftaz, as this has helped in the past. The doctor said it was worth a try, even though his last culture didn't show any bacteria at all.

It seems impossible, but it appeared to be making his cough worse, and his cough turned wet. He said his lungs felt full. He contacted the clinic again, and they decided to test the aspiration theory. He started a modified dose of Trikafta, and restarted the EES. The first week of this, he had a lot of bloating, but this got better, and the fevers stopped. 

The coughing never got better. Azer had another telemed visit with the clinic. The doctor agreed with Azer that the coughing has been going on for far too long. She didn't think another course of antibiotics would help. He is starting a new sinus routine with steroids in his nasal rinse just in case there is some polyps that are causing mucus to drain into his lungs. She also felt like it was a good idea for him to try to be better hydrated, as this can only help hydrated his lungs and thin any secretions in his sinuses. 

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!

ENT

Today, Azer saw ENT a few months sooner that an already scheduled appointment, mainly because he cannot use a nose clip during his nebulizer treatments anymore. He says it feels like the nose clip is squishing something in his nose, and it hurts. He also has had no sense of smell for the past few months, and allergy season isn't making it any better.

 The ENT doctor looked with the scope today, and saw a few polyps, and some inflammation, but nothing bad enough to schedule surgery yet. She wants Azer to try to put a full vial of an inhaled steroid medication in his nasal rinse, and to do it consistently four days a week. She wants him to try this for the next 6 weeks, and if it hasn't worked by then, to try another course of steroids, to try to shrink the polyps.

Azer sent these screen shots to me the other day from Instagram. I thought it was so cool that this Rangers Fan's page shared Azer's website and is helping raise awareness for CF! Thank you #txrangersfanatics!

ENT

It's been about 6 months since Azer's last appointment with ENT. He has lost most of his sense of smell, and can only smell strong smells. It's also been nearly exactly a year since his surgery to remove polpys.

Whenever Azer does his nasal rinses with the Netipot squirt bottle, he says it is almost painful, as it feels like the water stays up in his sinuses instead of draining out.

The ENT doctor saw some polyps and edema. It was very swollen and irritated looking as well. The doctor said it was up to us to make the decision about the polyps surgery. He reminded us that it will be a regular, periodic thing. Azer felt like at this point, he wasn't ready to have surgery again, and said that being able to smell wasn't that important at the moment. Unfortunately, by waiting, it will only increase the number of polyps and may become painful at some point. 

The doctor said to wait 6 weeks and see how he feels about surgery then. In the mean time, Azer has to be vigilant with his nasal rinses and also needs to restart Pulmicort drops in each nostril. Hopefully this will cut down on the inflammation. 

ENT

Yesterday was another follow up with ENT. It was mainly to see if the inflammation from the last visit had gotten any better. Azer has been doing a sinus rinse twice a day, followed by a drop of Pulmicort in each nostril. 

Again, before they could scope him, they squirted a numbing medicine, which was Lidocaine mixed with something else. We requested they bring in some apple juice to sip after, since that medication tastes awful. 

It wasn't as inflamed as last time, so the doctor didn't have such a difficult time going in. He removed some thick mucus. Unfortunately, there were a few more new polyps that grew since last month. The doctor had told us before, and reminded us again, that this will just be an ongoing thing for Azer. Whenever Azer experiences headaches caused by the polyps blocking his sinus passages, or loses his sense of smell again, he can go ahead and have another surgery to have them removed. This is merely an effort to improve his quality of life. 

I asked the ENT doctor if we should continue to do Pulmozyme through the mask to loosen any mucus up in his sinuses. He told us that is a question left up to the Pulmonologist, but felt like it would be more effective on the mucus in his lungs, and the best way to clear out the mucus in his sinuses is with the nasal rinses. 

We will continue with follow up appointments with ENT to monitor the inflammation and the growing family of polyps! 

Follow up with ENT

Yesterday we had a follow up appointment with the ENT doctor. They made in sniff up some lidocaine solution again (he says it tastes horrible), and waited a few minutes before taking us to the procedure room.

This time the procedure was a little more painful than last time. The insides of his sinuses were very inflamed, red and bumpy, and much narrower than they were a few weeks ago. The doctor even saw what he called a polypoid, meaning that Azer could already be forming polyps. We expected this to happen, but perhaps not so soon. Azer has also been having a decreased sense of smell again, which the doctor said is likely from all this inflammation. 

A funny thing - when the doctor put the scope/light up Azer's right nostril, Azer said he could "see" the light from the inside. He said it was very weird and hard to explain. At first he thought the doctor was shining the light from the outside, but realized the light was inside of his sinuses. Strange! 

Because Azer had some infected looking mucus in his sinuses, the doctor prescribed an antibiotic for the next two weeks. For the inflammation, he prescribed Pulmicort, which is an inhaled steroid. The Pulmicort I picked up from the pharmacy is a liquid usually put into nebulizer cups. Instead of using it in a nebulizer, we are to put a drop in each nostril twice a day. This is to be done while he's lying on his back so it doesn't just drip down into his throat. We can also stop the Flonase for now, but are to continue with the sinus rinses. Our medication routines are always changing!