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. 

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! 

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!

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! 

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.

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. 

ENT

So, Azer's sense of smell has still not returned. He says he can only smell Axe spray, and sometimes unpleasant odors. We were finally able to get an appointment with ENT. Our CF nurse ended up having to contact the manager of the ENT clinic after repeated requests for a consult.

When we met with the ENT doctor, he said usually his first step with sinus issues is to put the patient on an antibiotic. I asked if he could ask our CF doctors about it first, since Azer hasn't needed antibiotics for quite a while now. He agreed, and later on in our conversation, he decided that it probably wouldn't improve his sense of smell. We were sent down to get a CT scan about an hour later. The doctor told us he would compare the scan from today with CT scans Azer has had in the past: one in 2013, and one last year.

So, the doctor called this afternoon, and the CT scan from today was significantly worse than it was previously. He said it can be difficult to discern polyps on the scan, but he could make out definitive polyps and they are blocking quite a bit of his sinuses, especially on the right side. The doctor said the best thing that could be done to improve Azer's quality of life is to have sinus surgery to remove the polyps and to open up the sinuses. He said that it should improve his sense of smell, but it may never be quite as it was before. He also mentioned that this should be considered like "mowing the lawn"; that the polyps will return, and Azer will need periodic surgery to remove them. The doctor said usually the patient can go home the same day, but he will be coordinating admission with the CF clinic, to decide how long they want him admitted, and whether he will be on antibiotics during his stay.

13 Years Old

I am now officially the mother of a teenager! What better way to document 13 years of life than through pictures?



Happy Birthday Teenager!

Clinic Visit

So, the other day, I happened to be sitting next to Azer during his nebulizers. I noticed his "Bubbles the Fish" mask wasn't fitting on his face very well anymore. He actually outgrew Bubbles.

Aww... couldn't resist.

So, prior to our clinic visit, he has been using the mouth piece with the Pari cup instead. The Hyper-Sal has been causing him to cough quite a bit during his treatments, like when he first started using it.  He seems to have been sounding more junky because of this. Both of us were dreading what his PFT's would look like. Even the respritory therapist wasn't expecting very good numbers based on how his cough sounded when he was preparing to blow.

His first blow was 89%. His highest FEV1 was 93%. That has been all he's talked about since yesterday. He feels very motivated and encouraged to continue to work harder and get higher numbers! 

He weighed in at 116 lbs yesterday. He is now nearly 95 percentile in weight. Wow... His height did not improve, however. So, we will talk with the GI doctor about it next visit.

The doctor was very impressed in how he sounded and was feeling, especially because this is usually the worst allergy season for him. He mentioned to her that during and after athletics at school, he feels like his chest is heavy, as though something was pressing down on it. She thinks this is mainly due to the asthmatic component, and not necessarily the "CF". She then explained to Azer in a easy to understand way why he needs two inhaled steroids and what their specific purposes were. He also discussed with her his bowel troubles he's been having, and they figured out he needs to increase his enzyme dosage at a few meals based on how much fat he is consuming. It feels great to take the back seat at times, and let him take the reigns!

After clinic, we went over to the main building to do his yearly Scoliosis X-ray.

Big Tex was on the way haha.

On the way into the X-ray room, the tech asked Azer to verify his date of birth. The tech had the same birth date as Azer! What are the odds. The doctor saw the results and messaged me within the same day. I thought that was pretty incredible. She said that his scoliosis is still very mild and is unchanged from last time. She said it hasn't progressed with his growth and is stable.

So, all around, fantastic news!

Clinic

Yesterday was the appointment where they did all the annuals: annual bloodwork, X-rays, fasting glucose test, bone density, and a deep review of his care. We were there for about 5 hours. While exhausting, it is necessary.


So, he had been fasting since midnight. It seemed to take quite a while to get into the labs, so we went to X-rays first. After, they did a blood draw for his fasting blood sugar and many other levels, including vitamins.

Shortly after we got into our clinic room, he was instructed to drink several cups of the glucose drink.


His weight was 108, which is the same as last time. I told the GI doctor, it has been difficult to withhold the 5th can of formula, but his BMI went down a little, so she's happy with where it is. He grew an inch since February. The GI doctor wanted to know a little more about our Endocrinology visit. She said that the Dr. we saw is on the more conservative side of growth hormones. Our GI doctor said that in about 6 months, if he's still growing slowly, and not catching back up with his genetic goal, we can see another endocrinologist that is starting to take over more of the CF kids.


His G-button has been feeling tight - again! - so a few weeks ago I had to take some water out of the balloon. This is what the balloon looks like. This gave him a little more space between the button and the stomach wall. Since then, it has been feeling better, but the skin around the stoma has been very dry and itchy. They gave us what is essentially diaper rash ointment (zinc ointment) and some special gauze to use to protect his skin when he's swimming. They said perhaps the chlorine in the water is irritating it.



Since he's gained weight, his stools have been more fatty. When people with CF gain weight, they need a stronger dosage of enzymes, so we can increase his enzyme dosage for large meal times to see if his stools improve.


He only blew a 85% on his PFT's. His chest X-ray was improved since last time. Even though his X-ray looks good to the Drs, it still makes me sad to read the radiologists report: that there is wall thickening and bronchiectasis permanently in his lungs. It seems that lately, there is plugging in the same places that just doesn't go away.




The pulmonologist showed Azer what his X-ray looked like before and now and he was able to see how there wasn't as much cloudiness as there used to be. She listened to him, and while he sounded clear, she made him blow hard a few times, and she heard some wheezing. She thinks that his lower PFT's might be due to his asthmatic components, so she wants to add an extra inhaled steroid to his treatments to see if his breathing improves.
Two hours later, they retested his blood sugar. They now do this two ways, one by the prick of the finger, and the second sample is taken from his arm which is sent down to the lab and is considered more accurate. The technician had tried digging around at his veins twice and couldn't get any blood. The GI doctor asked Azer if was possibly dehydrated. He was... She recommended that he over-hydrate the day before labs.
Nearly every doctor, nurse, and medical student that came in stressed to him the importance of keeping his sodium intake high during the summer. The pulmonologist told Azer how she has had to admit other people with seizures because of low sodium. While he didn't have muscle cramps this time, he did experience some about a week later, and they were very painful. When he doesn't drink a sufficient about of gatorade during the day, we run it through his G-tube, and he walks around with it in a backpack for a few hours.
It has been about a year since his last scoliosos X-ray, so the doctor put in another order for it to get before we left. When we tried to check in for the second X-ray of the day, we were told they only did it later in the evening. By then, we had already been there for 5 hours, and we were exhausted and hungry, so we decided to come back for those another day!

Since I was there, I was able to ask the pulmonologist more about the bone density test results. She said that, while it does appear he is on the lower end of the scale, they don't know yet if this is considered normal for a person with CF. They are still collaborating with other clinics for their data. This is a relatively new test they are doing, since they have noticed that since people with CF are getting older, they are having to address osteopenia and osteoporosis earlier. The dietitian came in and recommended that if his vitamin D levels are below 40, they will increase his dosage. They said he is getting more than enough calcium from his formula. The GI doctor noted that he is very active already and with his running, it should be enough exercise to maintain bone density. She said that repetitive exercises such as running and jumping help build and maintain bone density. She also said that the clinic is working on getting a physical therapist on to the staff to help addressing exercise.

Heat Exhaustion And CF

This past weekend, Azer's team had a tournament. On Saturday, they had two games. He pitched several innings. He felt alright at the end of the day, but hadn't drank enough fluids and felt very tired.

Sunday, they played one game. He had started his morning bolus a little later than usual because he slept a little later. By the time he needed to start warming up on the field, he said his stomach still felt full, and he sat in the dugout to avoid vomiting. He had pitched a little while, and drank mostly water in the dugout between innings.

That evening at home, he still hadn't drank much more. We started to run some Gatorade through his G-tube, and he vomited it all up. He said he felt very tired. He ate a small amount of food for dinner. In the middle of the night, he ended up vomiting up his night feed. He said he was still feeling physically exhausted.

After communicating with the CF clinic, we decided to skip a bolus feed, and run Gatorade through his G-tube throughout the day.

We have been in the ER before due to dehydration. Apparently, in Cystic Fibrosis you can have heat exhaustion caused by low sodium in the blood. So, by drinking water in addition to aggressively sweating in hot weather, the salt in the body is not being replaced and this can cause hyponatraemia, which is having a low sodium level in the blood.

http://understandingcysticfibrosis.blogspot.com/2007/07/understanding-and-preventing-heat.html

New Mic-key Button Size - Again!

So, for about a month, Azer continued to have pain around his stoma. He said it was in the muscles around it. The last time he felt like this, we had to put in a larger sized Mic-key button. We managed to battle the infection of the stoma itself with the oral antibiotics, and special gauze they gave us. However, the pain remained when he coughed, and when his stomach was not completely full. In May, the ostomy nurse didn't think he needed a bigger size. I could tell he was getting tired of dealing with this pain, so we were able to get a quick clinic visit with the ostomy nurse again.

Azer explained to her how it felt, even though the button could still turn freely. She left to go find the next size up, but it was unavailable, and the one she did have was a whole half size longer. She agreed to put it in him, and see if we have any leakage.

Within seconds, he was pain-free. The whole drive home, he was relaxed and kept commenting on how much better it felt. I guess he didn't realize how much discomfort he was in until it was relieved!

This is what happens when he sits down lol

Bigger Size

Last week, he ended up coming down with a virus that came with a fever and cough. He was feeling pretty bad. Luckily it was short lived, but as it frequently happens, the virus leaves, but the congestion stays. We have been doing short sessions of manual CPT 4-5 times a day to try to help him clear the extra mucus. It's hard to say at this point if he won't need antibiotics soon, but it definitely helps him breathe better.