November 10, 2016


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.

October 6, 2016

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!

October 5, 2016

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!

July 20, 2016


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.

June 28, 2016

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.