June 13, 2017

CF Clinic - Annual Visit

Today was Azer's "Big Clinic Day", which includes a mental health screening, blood draws, two sputum cultures, a fasting glucose test, and a glucose test 2 hours after drinking liquid glucose.

Azer has been able to maintain his weight since the last clinic. The GI doctor was very happy with his weight, and he has grown some, but his height is still plateauing. The GI doctor said that at our next clinic, they will bring in the Endocrinologist and we will discuss growth hormones again. Azer told the GI doctor that he is still willing to start growth hormone shots.

About a week ago, Azer started oral antibiotics after some sinus congestion seemed to move into his chest. He cleared up very quickly after starting the medication, and today his PFT's were 95%, and the doctor said he sounded clear. The doctor said that if the chest congestion returns after he's off of the antibiotics, they may consider putting him on inhaled antibiotics until winter time. Azer told the doctor about how physically active he is, and both the doctor and respiratory therapist were impressed with him, and agree that exercise is so often downplayed when it's one of the most effective airway clearance treatments.

They noted that his blood sugars have been a little high in the past, and that if this one turns out to be borderline again, we may do a month of additional blood sugar testing to ensure it isn't getting dangerously high.

Trying to raise a successful teenager is challenging. Trying to raise one with a chronic health condition definitely is more difficult! Everyone was impressed that Azer has been getting more independent with his care, medications and exercise.

Today, we were introduced to a new product called Relizorb for G-tube feeds. It is basically a cartridge of enzyme beads (Lipase only) that screws onto the bag of formula and gives continuous dosages of enzymes through the feed. Since this product only helps with the absorption of fat, they recommend still taking regular enzymes at the beginning of his night feed. They have already had some great results from other people, so we are looking forward to trying it out! 

May 13, 2017

Great Strides "Joggers for Azer" 2017

It's that time of year again! If you would like to walk with us, or donate, here's the link.

Event Location: Vitruvian Park
Date: 5/13/2017
Check-in: 7:30 AM
Event: 9:00 AM
Distance: 5K

Here is the map of the location: https://www.google.com/maps?q=3875+Ponte+Ave+Addison+TX

April 7, 2017

CF Clinic

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. 

March 17, 2017


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! 

February 2, 2017

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!