July 20, 2017

Physical Therapy

So, back in April at CF clinic, Azer was evaluated for the first time by the team's new Physical Therapist. She noted that his posture was very poor. His back and shoulders were slouched similar to an elderly person, and his range of motion was limited. Azer also has had some issues because of overuse from playing baseball over the years. She said that poor posture in CF can begin with excessive coughing, and also that having a G-tube can attribute to poor core strength. She did a bunch of measurements, and a few exercises with him. She then sent us home with exercises to do everyday that help strengthen his core and back to help his Scoliosis, stretch out tight muscles, and strengthen his Osgood-Schlatter's disease knee.

Within a few weeks, Azer began to have back pain. Through communication with the Physical Therapist, she determined he would need to be worked on more frequently than just at clinic. We began seeing her once a week at first, and now it is every two weeks. She was able to clear up  his back pain within a few weeks and also make progress in his strength.

This physical therapy room is awesome. It has lots of space and the cushioned table is height adjustable. 

 It is challenging to do these exercises and stretches every single day. It takes up time, and sometimes  he is physically tired the next day depending on what we did the day before. However, I wish I had a picture of how his back and shoulders looked before we started PT. The change has been dramatic. She re-measured him a few weeks ago, and basically his scapulars are an inch closer together than they were before. An inch! Within a few weeks of starting exercises, even Azer noticed that he felt a little taller. He is visibly standing up straighter. His muscle definition is getting to be noticeable as well. His Osgood-Schlatter's (we joke about it and call it Schlotzky's) knee is also improving. He has been running 3x a week during the summer, and his knee hasn't been hurting. Azer ran 3.4 miles in 30 minutes yesterday for the first time. He is getting so strong, and it is incredible to watch this tiny, scrawny child grow into a strong young man.

The Physical Therapist has to fight with insurance companies and Medicaid constantly to get them to cover her treatments. They don't believe that people with CF should need physical therapy. She said the interesting thing she has noticed about treating kids with CF is that most of the time, they need her help for sports injuries. She tells the insurance companies that if CF kids get injured because their so active, and don't get help and can't exercise, they are going to have a much bigger problem on their hands than just PT!

June 27, 2017


Ever since Azer was put on antibiotics a few weeks ago, his sense of smell started returning. We were recently on vacation and stopped doing the nasal rinses then. We noticed he was breathing better out of his nose. On the way back home, it was almost like telling a blind person what they see. He kept asking what he was smelling. It's very encouraging.
Blue Hair Dye for the Summer!

So, we told the ENT doctor about what we haven't been doing. He said to keep not doing what we've been not doing. Lol.

Again, they squirted lidocain up his nose and waited a few minutes for it to work before looking up his nose. He drank some Gatorade after to get the taste out of his mouth.

The doctor still saw new polyps, but the inflammation was decreased, and he said it looked pretty good for a person with Cystic Fibrosis. 

After ENT, we had to go to labs again. On his blood work from CF Clinic, his IgE was elevated, and they wanted to make sure he hasn't developed ABPA again. However, his sputum culture showed Steno and Staph, so the antibiotics that he's been on have definitely helped this.

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! 

January 7, 2017

The Week After Sinus Surgery & ENT visit

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!

January 5, 2017

CF Clinic

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!