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.


June 10, 2016

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.

May 29, 2016

Thank you!!!

Dear Donors and walkers for “Joggers for Azer”,

Thank you so much for what you have done. This is the first time ever that we have raised over $600 and it's all thanks to all of you who contributed. Thank You to everybody who showed up to the 2016 CF walk this year to support me and many other people with this rare and genetic disease. We could not have done it without everybody's help. Thank you so much for all you support and help.

Sincerely, Azer Russell

May 26, 2016

Endocrinology Appointment and Results

The endocrinology appointment went pretty fast. We talked about some of the concerns over him gaining weight, but not growing in height. We reviewed the GI medications he's on. I was also able to ask about the bone density test he had a few months ago. It was on the lower end in the range of acceptable. She did a physical exam, and concluded that based on her observation, his hormones are likely normal. Just in case, we were sent down for X-rays and labs. 

She tested for many different hormones that help growth and puberty.

Our number or "place in line" at labs. Azer noted it was his lucky number. 

In X-ray, he had a Bone Age scan, which looks at how his bone age matches up with his actual age. The result was it was about two deviations from his actual age, which apparently is still within the normal range. 

All of his hormones came back as normal for his age and stage in puberty. She noted where he was on the growth chart, and it looks like his height is finally starting to creep upwards. She said if he continues to follow this trend, he should reach his genetic height without the need for growth hormones. 

They also did a random glucose test. In the past, his fasting glucose has been normal, but his random glucose tests have been a little high. So, for the next month, they want us to test his blood sugar twice a day, twice a week. This seems so sudden. Luckily, I was able to go back to the clinic and get hands on instructions from one of the awesome nurses there. They told me to let them know right away if his blood sugar is too high. I am hoping he doesn't have CFRD, but if he does, I am grateful that we have the opportunity to treat it soon. At the same time, every time something new is added, it always feels like a huge burden for a while until we get used to it. So, I am still hoping this is only for a month!