Last week, Azer seemed to have come down with a virus that only seemed to last for a few days. The virus brought nasal drainage, so that on top of any allergies he has right now has caused all that mucus to end up in his chest. On Friday, he really seemed to be having trouble breathing through it all. We increased breathing treatments, and did manual CPT about 5 times a day.
His PFT's were 86% FEV1, which is lower than his usual baseline. We were expecting it to be lower the way he sounded. When the doctor listened to him, he had some wheezing that moved around, and some noisy spots on his lungs. We all agreed that a course of oral antibiotics would help a lot right now. Luckily he only cultured sensitive staph again.
We had a new member of our CF team come visit us. Our clinic now has a Physical Therapist. She said she loves working with the CF team, and thought it was kind of interesting that most of what she sees in the CF clinic is sports injuries. Although that's not necessarily a good thing, it means that the majority of our CF kiddos are very active and athletic.
Even though Azer is already very active, she did measurements on him in various places. She noted that his posture is incorrect, and is likely due to all the years of coughing and also having a G-tube. She came back a few times during our clinic visit. She had him do some movements after his PFT's, and then later on, she took him out into the hallway to try out some stretches. There was a lot more to it than I ever thought there could be. She hopes that in three months, his form will get better. After his form has improved, she hopes to have him start weight bearing exercises. She expects that this will help his pitching form, his Osgood-Schlatter's knee, and his scoliosis. There is also evidence that doing the proper PT could reverse low bone density.
Azer lost a few pounds, which put him down closer to 50th percentile for weight, which is a bit more normal. He also grew nearly an inch since his last clinic visit. The doctor is hopeful that his body will catch up in height now that his nutrition is more than sufficient though puberty.
After clinic we had chest X-rays just to see how everything looks. Overall, everyone thought he looked and sounded great, and is very happy with all the work we put in at home.
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!
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!
The first week was definitely challenging. CPT was difficult, as every 30 seconds or so, he had to sit up and cough out more blood and mucus. Just coughing in general hurt his nose and face quite a bit. He was constantly oozing blood for the first three days. Even though we were assured this was a normal consequence of the surgery, it still is concerning to see your child coughing up blood.
He wasn't eating very much, and it seemed he lost enough weight to cause his G-button to start leaking, so we upped the formula. After the Prednisone really got in his system, he started to become very hungry and ate.
Doing the nasal rinses were really hard the first week. He could only do a little bit at a time and the only way to finish the bottle of nasal rinse in a day was to do a little every few hours. But as the week went on, the swelling began to subside and he was able to do an entire bottle at a time. The best time for him to do it is in the shower. The hot water seemed to loosen the scabs enough for them to come out on their own.
I think we were all very grateful he was on antibiotics for the week, as all that blood was in his lungs, and it took almost a whole week of consistent treatments and hand CPT to get it out. After a week, he started to sound very clear. We had great weather around Christmas break, and so he spent a lot of time outdoors being super active. That also helped clear his lungs, and as a result, we could start backing off on the treatments.
As for the main reason for the surgery, his sense of smell which has been MIA for most of the year has come back! He has been enjoying food much more and it has definitely improved his quality of life.
Yesterday was the follow up visit. Thankfully, ENT noticed on his notes he had CF, so we were taken back to a room quickly. First the doctor removed the stitch. Apparently there was only one and it was on the left side. This didn't hurt at all. When he pulled the splints out, this was a little more painful, as his nose is still tender to the touch. Azer could immediately breath better.
The assistant then brought in a syringe with a nasal attachment at the end. She said it had numbing medicine in it. Azer had to stiff the medicine up each nostril. He said it tasted horrible, like hand sanitizer. She let him sip some apple juice periodically to help with the taste. They left for a few minutes to allow the medicine to take effect.
We were then taken to a different procedure room, which had a machine set up with a scope, suction, and camera. The doctor carefully fed the camera up each nostril and pulled out a large amount of scabs. This was painful mainly when the doctor had to push on the septum area, and also when the scabs were pulled loose. The doctor was pleased with how it looked, but said there was still a lot of inflammation, and wants to see us back in a few weeks for another round of suction.
This appointment ended up being in the late afternoon. We had had a bit of snow flurries which ended up sticking to some of the roadways. The roads out of Dallas didn't seem to be treated at all, and it took about 2 and a half hours to get home!
Our appointment today started out with a visit from the psychiatrist's assistant. She had several surveys for us as parents to fill out, and several similar surveys for Azer to fill out. She also did a verbal survey with Azer over the how he felt about his treatments, and how much annoyance they caused in his life. The surveys we took had to do with our family dynamic, how we function as a family, and how we cope with problems together. They are using these for research purposes. Just a good mental note to make, that not all CF research has to do with pills or medications!
Today, Azer's weight is stable. His height is still on the 10th percentile, so the doctor said she would talk to the endocrinologist again and see if growth hormones would help since he has already started puberty.
A few months ago, Azer had his teenage well visit with his pediatrician. She was very concerned with how much weight he had gained over the year, and put in an order to have his cholesterol tested. I mentioned this to the CF doctor today, who essentially said that if the pediatrician had even read the notes in the system, she would realize he is being followed closely by a whole team of doctors regarding his growth and weight. She said it would be very unlikely that any person with CF would have high cholesterol due to diet. She reminded Azer that the general goal is for his weight to be 50% percentile or better as research has shown this helps lung health. She also said that if as a doctor, if she only focused on the numbers, and treated a patient by what the numbers said (pft's, weight, etc.) the quality of the life of the patient might not be so great.
His lungs sounded completely clear, with no crackles and his PFT's were at 93%. He still has nasal drainage and has been complaining of a bad odor he smells when he breathes out. This hopefully will be resolved when the ENT doctor removes the splints stitched in his nose.
Since his sense of smell has returned (yay!), Azer's appetite has been improved. He has been showing a lot more interest in food, and has been enjoying the smells of foods being cooked. So, I believe the polyps removal and the reconstruction of his septum has been a big improvement for his quality of life.
Azer recently competed in cross-country with his school and did fantastic. Although he wasn't first, I reminded him he outran a whole bunch of boys that don't have Cystic Fibrosis!