November 14, 2017

MRI for Growth Hormones

One of the requirements we had to meet before starting growth hormones was to get a brain MRI to make sure there isn't a tumor on Azer's pituitary gland.

He wasn't a big fan of the over-sized gown. 
It was a day where is lungs sounded a bit junky. Luckily, he was able to lay still for the majority of the scan, and they allowed him to cough in between the frequency scans. I was able to sit near him where he could see me, to try to relieve the claustrophobic feeling. When it was over, he sat up and he said he felt dizzy. The technician said this was from the magnetic field from the MRI. The feeling passed quickly.

We had a meeting a few hours later, so there as no point in driving home. To help pass the time, Azer decided to stand near the glass windows where you can see people on the lower floors, and "dab". He was seriously delighted when a man dabbed back!

The results of the MRI came in, and the doctor reviewed it. It shows that his pituitary gland is smaller than normal. The doctor isn't concerned about this (we have questions), and has given the go-ahead for growth hormones, pending insurance approval!

October 31, 2017

Physical Therapy Visit

It has been about a month since his last PT session, so Azer had a few things to work on today. (I snitch on Azer and tell her what he has and has not been doing.)  As punishment, for extra fun, he did plenty of core exercises and some knee exercises for his Osgood-Schlatter Disease which has been bothering him.

Since it is Halloween, they decided to do their planks wearing, what else, but Poo Emoji hats! Lol

This last pic is from a few months ago, but I think it demonstrates his therapy sessions the best haha. She does a fantastic job motivating him, which is a job in itself to motivate a teenager!

October 14, 2017

CF Clinic

Thursday was another normal CF Clinic day. Azer blew about 5% lower than he did when he was discharged from the hospital in September. Ever since we had a cold front, his cough has increased as well as mucus production. About a week ago, his shoulder pain returned, but it only lasted about a day. The doctor didn't feel like it was time for antibiotics yet. She said we should restart his Nasonex to help his allergies out a little bit more.

His weight was exactly the same as well as his height. He hasn't grown in height for the majority of the year. The results of his Growth Hormone Test showed that his hormones for growth are too low to allow his body to grow. Azer wants to pursue the Growth Hormones, so we will be started them soon, despite the increased risk of CF related diabetes. We talked with the dietitian and GI doctor about the risk, and all people with CF have a 60% of getting CFRD at some point.

They retested his potassium as well as the rest of his electrolyte panel and it showed everything is right where it should be. The dietitian assured us that worrying about his potassium should be on the lower end of things to obsess over. So, as long as he does his night feeds, it should stay stable.

September 22, 2017

Growth Hormone Stimulation Test

Last night, Azer felt good enough to play baseball. He stayed in positions that were less physically demanding. He pitched to the very last batter and got the last out of the game.

So Azer ran his G-tube feed on a fast setting last night so he could get his 3 cans in before midnight (really pushing it since Sunday). After we got to the hospital this morning, they told him he could have as much water as he wanted.

I had to remind a few nurses about putting gowns and gloves on before they worked with him. I told them it protects him as well as the other patients.

The nurse was able to get an IV started very quickly. Then she gave him a Clonidine tablet which is usually for blood pressure. She said this is supposed to neutralize something in his body and that it can also make him sleepy. He was in a recliner with a warm blanket and pillow. They said they would come back and test him in a half an hour.

He said his mouth and eyes felt dry from the Clonidine. He was also very sleepy, and was able to nap during much of the time.

They came in 30 minutes after they gave him the pill to take a blood sample. About 30 minutes after that, they started l-Argenine by IV, after which the IV pump switched over to saline solution to help keep him hydrated. They took his vitals and blood every half an hour.

We will probably know the results and what possible direction we can take in a week. 

September 19, 2017


So, last night his potassium was getting just a little higher than normal, so they took him off the IV potassium and the levels have returned to normal.

They have had to poke him many times to test his potassium through these last few days.

He is holding down food and all his formula. Hopefully he can continue to keep himself well hydrated.

Other than working on hydration and nutrition, hospital stays can become boring very quickly. So, here are some pictures and videos of hospital fun XD.

The tree with legs outside is always kind of creepy lol. 

Azer won a hat from a game he played through the Children's Network TV. The clowns even came by and tried to barter with him using some cold, hard, giant sized cash. 

We just saw the doctor, and he gave the all clear for going home. It will be great to be home tonight. We will be going home with oral antibiotics to finish the course he started Tuesday. The doctor wants Azer to not exert himself too much for a few days to help his body recover. The doctor agreed it was just a combination of vomiting, antibiotics, being out in the heat, and being unable to keep down much formula that caused this to happen.  We really have to push for hydration now more than ever. The doctor we saw has had a running joke with Azer through the years that when Azer is a famous MLB pitcher, there's going to be Mom running onto the field, halting the game to remind him to drink his Gatorade. But, truly, I think Azer can see now the worst than can happen from being dehydrated. 

We are outta here!

September 18, 2017


So the dietician felt like this incident is unlikely to occur again, but gave us a list of foods we can add to his diet to add potassium. Apparently, with everything he normal eats and drinks, formula included, he is meeting half of his potassium requirements. The frustrating thing is he would need about 5 servings of fruits and vegetables a day to meet it. She basically said push it more especially on days he's going to be outside and active.

He did his PFT's a little while ago and he blew higher than he did Tuesday and his lungs are clear. 

The funny thing (not really funny) is through the period of this day, Azer has had a full bottle of Gatorade that everyone keeps urging him to drink. The dietician came in, and noticed it had maybe a sip taken out of it after hours had passed. She told Azer, your mom is just going to have to put it through your G-tube! 

Day 2 in the hospital

Azer ended up having to get stuck multiple times in the fingers and had several butterflies in his hands. The poor nurse was trying to get blood to test his potassium, but he kept clotting and she was having a difficult time.

He finished his night feed for the first time in a few days and held it down. He also did his morning feed and feels pretty good. 

We were a little disappointed that we asked for hand CPT last night, and the RT still used the Vest. So, this morning the RT for today was questioning why we did hand CPT. Both Azer and I said the Vest does almost nothing to help him clear his lungs. The RT had me show him how I do CPT and was impressed. He recommended something called a IPV as something Azer could possibly use in college. I've read about it before, and sounds great, but when I asked a doctor about it, they said usually it's not prescribed for home because of the risk of lung bleeds. 

Our dietician came in and will give us a list of foods that are high in potassium and also give us a daily fluid gole. She believes this incident might have been from "the perfect storm" conditions. We had a cold front, and then it's been suddenly hot for the past week. The dehydration probably started from last Thursday's baseball game, Friday he had a tough athletic period and had to run 8 laps in the afternoon heat, and then Saturday he pitched almost the entire game. He then played outside for quite a bit of the day. For a few days, he hasn't been able to do much of his formula feeds, has been nauseous and has vomited a few times as well. All these factors likely contributed to his dehydration. 

One of the nurses came in and said that Azer's blood work from today compared to yesterday's is strikingly improved.  The results of the EKG weren't too concerning, but his QT was prolonged. The nurse also said Azer's creatinine and BUN was high yesterday, making his kidneys work hard. They said that his blood counts were high because of it being so concentrated. So, Azer was definitely in a dangerous place, and I'm glad we brought him in. We might be able to go home tomorrow if he keeps looking good, but we are definitely going to have to work on keeping his potassium and hydration up especially if he is going to be outdoors. 

September 17, 2017

Feeling better!

He's feeling a lot better, was able to urinate, and ate dinner and seems to be holding it down!

We're not sure how long he'll be here, but we'll probably find out in the morning after PFT's. They will test his potassium and electrolytes periodically through the night to make sure they stay stable.

Cystic Fibrosis can be hard on little sisters too... 


So, here we are at the ER. While we've been here, his symptoms improved over a few hours. They did an x-ray and saw his lungs are slightly better than they were last week. 

His sodium levels were pretty good. Unfortunately his potassium was low, and they have started him on IV fluids with another bag of potassium. They have decided to admit him so they can continue to administer potassium. They have him hooked up to a heart monitor. The doctor said with his potassium so low, if we took him home and his levels stayed low, he could go into cardiac arrest. At this point, I don't know why it's so low. The only answer the doctor could give us was it might be from the vomiting. 

The potassium burns going through his iv. He was in a lot of pain and wanted them to stop it. The nurse brought in cold and heat packs to help. They also are running additional fluids through his IV to help dilute it. 

New Symptoms

He is still sweating profusely but shivering and cold. Tested blood glucose and it was 130 which is good I think, since he's had very little to eat. He's started to say his vision is blurry at a distance. He said his heart is pounding hard, and is weak to stand. His finger tips are numb. We are all a little scared at that right now. Called the CF doctor on call again but considering taking him to the ER. 

Nausea and reflux

CF is such a catch 22 sometimes. Tuesday, Azer started antibiotics to clear his lung infection that was bad enough to cause shoulder pain when he took a breath in. Now, this same antibiotic that is basically saving his lungs has caused him to lose 5 lbs in 5 days. That's a pound a day. Since he's gotten sick a few weeks ago, he's lost almost 10 lbs. He's almost the same weight he was a year ago. In an instant, all the weight gain we fight to put on and maintain is gone.

For the past 3 days, Azer has been experiencing nausea and reflux every time he coughs. Yesterday, he had a baseball game, and after we went out to lunch to celebrate and he wasn't able to eat much at all. He was able to drink two bottles of Gatorade and keep it down, but he felt so exhausted and weak by the evening. We couldn't finish his breathing treatment last night because every time he coughed with any force, he felt his stomach contents coming up. He had to stop his night feed early on last night. This morning, I gave him two Bethanechols after he threw up to try to help his stomach feel better so we could get his breathing treatment done, but we could only do one minute of CPT.  He said while he was throwing up this morning, stomach contents started leaking from around his G-tube stoma.

I looked into my past blog posts, and the last time he was on Bactrim, he was having a lot of vomiting issues as well. The last time, the doctors switched him to Levaquin. So, I called the On-call CF doctor to see if we can switch him to Levaquin again, which they will do. Hopefully, this helps, but he is suffering right now. He says he feels cold, but yet he is sweating profusely.

September 12, 2017

CF Clinic - Sick Visit

About two weeks ago, Azer seemed to have caught a virus. While the rest of his body recovered, it seemed like his lungs did not. In the evening, he was exhausted, after doing athletics at school and then baseball practice after that. It just felt like his breathing treatments weren't enough. He felt like his lungs were full of mucus, even though he sounded relatively clear when he coughed. His stomach has been full of mucus, which has been making him feel sick to his stomach, and definitely hasn't help his appetite.

We were waiting to see if treatments alone could clear this up, but three days ago, he ended up having shoulder pain when he took deep breaths, or coughed. We knew it was time for a clinic visit.

We were expecting low PFT's but he actually blew higher than he's been this year. He lost a few pounds, but it didn't affect his BMI too much. We saw a new doctor today, who noted that he really hasn't grown much in height this year, and hasn't grown at all since his last clinic visit. Azer will have a growth hormone stint next week and hopefully we will have some answers.

The doctor listened to him, and he sounded perfectly clear. He does have nasal drainage, so she wants us to go back to doing nasal rinses at least twice a day. Hopefully that will stop some of the mucus from ending up in his stomach and lungs.

Azer had two X-ray's today. One for his lungs, and another for his shoulder to see if the pain was possibly coming from an injury. It showed that his shoulder was fine, but that there is worsening in the areas of the lungs that have had consistent mucus plugging over the past few years. It also still shows bronchial wall thickening and bronchiectasis. If you watched this kid play baseball, you'd never believe his lungs looked like anything besides normal.

He will be on oral antibiotics for two weeks. They took a mucus sample as well has a swab from his nose to check for viruses. He ended up having a nose bleed from the swab, for some reason. If the culture shows Pseudomonas, we will be back on Tobi in addition to the oral antibiotics.

Update: The virus test came back as negative, still waiting on the results from the lung culture.

August 27, 2017

Endocrinology Appointment

Last year, we saw another endocrinologist and after her evaluation and blood tests, determined that he was just beginning puberty, and that because his nutrition was so good, that he would begin to grow in height soon. Friday, we saw the endocrinologist that works closely with the CF clinic. She noted that Azer is well into puberty, we have maintaned good nutrition, and yet he has not grown in height at all since his last CF appointment in April. Around eight years ago, his height was around the 25th percentile, which is likely genetically where he's supposed to be. But, for the past few years he's been platueing around the 10th percentile and it has not improved.  

She sent us down for a repeat of a bone age scan. Our last one was normal as was this one. 

When he was last tested for growth hormones, the levels were good. She said that they can fluctuate during the course of a day, so in a few weeks she wants to run another test. Azer will be fasting and will have an IV placed and over the course of about 4 hours, they will test his growth hormones and also inject him with growth hormones and see where his levels are at. If his hormones aren't where they should be, we might be able to start hormone replacement therapy. If they're fine, I'm not sure what else can be done.  

One concern of starting hormone therapy is it can cause CFRD (CF related diabetes). If someone is borderline with high blood sugars, it can push them over the edge and cause CFRD. Sometimes, when the therapy is stopped, their blood sugars go back down to normal, but sometimes, they end up with CFRD for life. Over the past few years, they've been watching Azer's blood sugar closely, as they've been high, but never seem to get super high from the OGTT. But this is a concern to keep in mind. 

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!