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! 

December 22, 2016

The Day After Surgery

Yesterday started out pretty tough. He woke up in the middle of the night panting and unable to breathe easily. Some albuterol helped a little, but it seems that all the bleeding from his sinuses ended up in his lungs. At first, he sounded so bad, and was having such a hard time breathing, I was tempted to go ahead and bring him back to the hospital. We put in a lot of work yesterday, doing hand CPT every couple hours. It started out pretty rough. Coughing and laying down hurt his nasal area. Most of the day, he was coughing up a lot of old blood and mucus. Luckily, his nose wasn't bleeding much at all, so we were able to do his normal nebulizer medicines.

Because of those splints stitched inside to his septum, nasal rinses have been challenging, and painful. The doctor told us that if we didn't keep trying to do the rinses, the blood in his sinuses could scab over and dry and cause scarring. It took most of the day to use one bottle up, but it did flush a lot out.

He ate very little, so we did extra formula boluses to supplement the calories he wasn't eating. He felt nauseous after most boluses so he took it easy for most of the day.

Later on in the afternoon, the mucus he was coughing up was becoming less bloody, and more clear. He definitely was starting to feel more like himself. He slept sitting more upright last night, so while this is more comfortable for his head, all the drainage ends up in his lungs. So, we plan to continue with frequent hand CPT sessions for the next week.

December 20, 2016

Sinus Surgery

A Pre-op nurse called yesterday and let us know when and where to register and check in. We put in plenty of extra breathing treatments to prepare for the surgery. They also let us know they might be admitting him and keeping him overnight after the surgery.

As soon as we arrived at the Surgery Clinic, they put us in a room right away. The team was very upbeat, and kept the joke running about Azer getting a brain transplant. He was given lidocane using a  J-tip  before they put his IV in. A little while later, they gave him Versed through his IV, and he got very relaxed and giggly. The doctor said to expect the surgery to last about 2-3 hours. (I was surprised it was so long)

About an hour into the surgery, we were called into a Consultation room with the doctor. 



He apologized and said not to worry. He had already cleared some sinus cavities on one side, but was having great difficulty accessing the left side. Like many people, Azer has a deviated septum. However, in Azer's case, the inflammation, mucus, and polyps had further pushed the septum so far, that he probably hasn't been able to breath from that side for a long time. The doctor needed our signature and permission to continue with the surgery and to cut and file down the septum. The doctor put rubber splints into each nostril and secured them with a stitch.

The surgery was indeed close to three hours. When it was over, we were able to go be with Azer during his recovery. 


It took about an hour in recovery for him to be released and they let us take him home. They felt like he was breathing well enough to send him home. We were sent home with steroids and precautionary antibiotics. Tomorrow, we will start Neti pot sinus rinses. 

His breathing treatment tonight was shorter than usual. Coughing is very painful for him right now. His lungs sound very noisy. It is always shocking at how quickly his lungs and become congested. I tried to do some CPT for him while he did his best with his nebulizer. 



He is coughing up some blood, likely from drainage from his sinuses. When he sits up, he soaks through gauze pretty quickly. Obviously, his face is sore to the touch. Controlling all this bleeding and pain is challenging. Hopefully, this will get better quickly.