December 28, 2017

Influenza

For a few days last week, Azer complained of a dry, raw feeling throat. We figured he might have caught a virus. On Christmas day, I was hit hard by what felt like the flu, and Azer started to feel awful. After going to the doctor and discovering I had the flu, I called the CF clinic to ask for guidance. We got Azer swabbed for a confirmation that he did indeed have the flu as well. He has been started on Tamiflu and increased treatments. He is feeling a lot better, but his lungs are full. We are all working hard to try to keep them clear. If anything, this has made me grateful for the years that the flu shot does work!

December 21, 2017

ENT

It's been about 6 months since Azer's last appointment with ENT. He has lost most of his sense of smell, and can only smell strong smells. It's also been nearly exactly a year since his surgery to remove polpys.

Whenever Azer does his nasal rinses with the Netipot squirt bottle, he says it is almost painful, as it feels like the water stays up in his sinuses instead of draining out.

The ENT doctor saw some polyps and edema. It was very swollen and irritated looking as well. The doctor said it was up to us to make the decision about the polyps surgery. He reminded us that it will be a regular, periodic thing. Azer felt like at this point, he wasn't ready to have surgery again, and said that being able to smell wasn't that important at the moment. Unfortunately, by waiting, it will only increase the number of polyps and may become painful at some point. 



The doctor said to wait 6 weeks and see how he feels about surgery then. In the mean time, Azer has to be vigilant with his nasal rinses and also needs to restart Pulmicort drops in each nostril. Hopefully this will cut down on the inflammation. 

December 19, 2017

Discharged from Physical Therapy

After being successfully treated for several injuries and painful areas, we have been discharged from seeing the Physical therapist every few weeks. We now will only see her at the CF clinic, and she will continue to monitor Azer's form and strength. 






As it was his last day for her to work with him, she worked him extra hard and gave us several more exercises that we can do at home. We now have a large arsenal of exercises to chose from everyday. 




December 15, 2017

Endocrinology Visit

We've had this appointment scheduled for a while, so there wasn't much that the doctor could do or tell us after only being on Growth Hormones for a few days.



While we were waiting to see the doctor, our Physical Therapist brought in some exercise mats and weights so Azer could do exercises while we waited (she didn't want him to get bored, haha!)


He was thrilled....  not really. The good news is his scapulars are starting to come together again, and his form is improving (again). We really have to stay on top of this now that he's on GH.

While we didn't go over much with the doctor, she did answer some questions both Azer and I had. He has to have his Scoliosis checked every 4 months, to make sure it isn't progressing. The doctor mentioned this is especially important given that he has CF. Scoliosis can impair the ability to breath in some cases.

If, after he is done growing, he is tested and still shows to be GH deficient, he might need to be on a low dosage of GH for the rest of his life, as studies have shown GH to be necessary for cardiovascular health, among other things.

I asked about the results of his MRI, showing that his Pituitary Gland was small. The doctor said other than having a traumatic head injury (he hasn't that I know of) the only other reason why it might be like this was he was born that way. She doesn't know why he's been GH sufficient up until puberty started. GH are most effective when started as early as possible, as when puberty starts, the window starts closing. But, she mentioned he should have a few years left to grow.

Azer asked her about the possibility of developing CFRD (Cystic Fibrosis Related Diabetes). She said that even in people without CF who are borderline diabetics, when taking GH, it often does push them into having full blown diabetes. She said that if he does develop CFRD, it's our choice to continue with GH along with insulin or not. Sometimes, when stopping GH, diabetes can go away, or sometimes it's there for life. Even without GH, he has a 60% chance of developing CFRD in his lifetime. The doctor said we would keep a closer eye on his blood sugar than before. So, we shall see!



December 12, 2017

Growth Hormone Class

The Growth Hormone class was about two hours long. A nurse went over all the side effects, what to expect, and when and where to inject the hormone. She also mentioned some insurance companies don't cover growth hormones! (Something to keep an eye on in the future).

She allowed Azer and I to practice putting the needle on the pen, injecting some "fake skin", and get a feel for how the injection pen works. Azer did very well, and was soon ready to try his first injection. He did great, and did it all himself. The needle is pretty small and he said it hardly hurt at all.





Of course, anything that is added to his CF life causes change. This time, it includes less fridge space, and a sharps container. We should watch for side effects, the most common would be a bad headache. Apparently, sometimes the pituitary gland can swell and cause pain when growth hormones are first started, or the dosage has increased. In this case, we would have to stop the injects, and then at a later time, start them in small dosages. We also have to keep track of where the injections are each day, what days they are given!


We are hoping for great results and little to no side effects!

November 29, 2017

Physical Therapy

Azer is coming to an end of his active treatment phase with the Physical Therapist. She has taught him how to do manage different pains in his body with exercises and stretches.

Over the past few months, we have allowed Azer to be fully independent with his stretches and exercises. As can be expected, he is not 100% compliant with them. The PT took his measurements again yesterday, and found that his form has regressed. She stressed to him that if he continues down this path, he will develop what's called "Barrel Chest". This causes the rib cage to be partially expanded all the time, and he won't be able to exhale completely. This will be permanent, and she said when she treats older patients with CF, there is nothing she can do for this. This is a crucial time for him to get his form back, especially now that he will begin growth hormones soon.





Because we feel this is so important right now in his life, he will be doing his stretches and exercises in front of us every night.  The Physical Therapist added a few more exercises that he can do daily that will improve his core strength as well as improve his Scapular form.


The Physical Therapist also shared that she has been advocating for CF patients to the insurance companies, Medicaid, and also to parents. She has been trying very hard to teach people that PT is as important to CF patients as is regular doctor appointments. If kids with CF's form and posture is not corrected at a young age, it is possible for them to lose lung function and require a lung transplant sooner than later. Doing the right exercises in the right way can also build bone density and can possibly reverse bone density loss. 

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

Discharged

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

Update

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...