Endocrine Appointment

Azer has been on antibiotics for about a week. A nurse popped her head in to ask him how he was feeling today. He feels better than he did last week. I wouldn't say he is back to sounding his normal best, as he is still coughing more than normal even though his culture came back with no Steno or Aspergillus, and just MSSA. His Pulmonologist thought it over and believes that since his new allergy shot, Dupixent, is helping to shrink his nasal polyps, that his sinuses are draining quite a bit and causing him to cough. She wants to treat his cough like a sinus infection, and continue the oral antibiotics, to make it a 21 day course.

It has been about a year since we've seen the Endocrinologist. She would like to see him every 4 months from now on. In the past year, he hasn't grown as much as she would expect. They ran some blood tests that will tell her if his growth hormone level is where it should be. She expects that it will need to be increased. She also ordered a bone age scan, to see how many years left he will probably grow. She mentioned he may have not grown that well this year based on how many times he has gotten sick and/or hospitalized.

He put on about a pound from last week. The Endocrinologist said today he weighed about the same as he did last year when she saw him.

We discussed the possibility of continuing growth hormones into adulthood for long term health and to also keep his muscle mass stable. She said when adults are deficient in growth hormones, they can have high cholesterol and other issues. We also discussed how high his glucose tests can be before it's time to start being concerned. His last OGGT was a bit higher than he normally is, but it isn't concerning at this point.

Azer also saw his PT today, who did sort of an out of the box test that is meant for athletes instead of CF patients. He did exceptionally well.

Azer was given tickets to one of the last Rangers game at the current stadium as a reward for helping out another CF kiddo at the hospital. He got to meet a former Rangers pitcher, Martin Perez, and watch his favorite Ranger, Josh Hamilton be inducted into the Rangers Hall of Fame. He also watched Nelson Cruz hit at batting practice.

CF Clinic - sick visit

On Sunday, Azer was by himself watching a movie when suddenly, he was coughing non-stop and was struggling to breathe. He did an albuterol breathing treatment to see if it would stop. Unfortunately, he continued to cough for two full hours. He was exhausted by the time it started to get better. He coughed up the most mucus I've ever seen at a time. At the end, he coughed up some brown mucus. He said his upper airways hurt after this happened. The on-call doctor said to take him to the ER if another attack happened. Luckily, it hasn't happened again.

He went to clinic yesterday to get a PFT and an X-ray to see if there were any significant changes. His lung function was down slightly, but within normal range, especially for it being allergy season. The doctor said his X-ray looked about the same from last time. The doctor wondered if the attack was a mucus plug coming up. Just to be safe, he will be on a course of oral antibiotics.

Azer lost even more weight from last month, and a total of 8 lbs so far. The dietitian came in to discuss what he's been eating, and what else we can do to change this trend. The appetite stimulant medication he is on seems to help, but with evenings being so busy, he isn't able to take it consistently. Even though his appetite has been pretty good, he hasn't been making meal times a priority. They would like to try another appetite stimulating medication and see if it helps him at all. This medication has the possibility of interacting with other medications. They had to do an EKG first before they could prescribe this medication. It took a while to get a good EKG result, because he wouldn't lay still! 😄

In addition to possibly starting the new medication, he has to start doing an extra can of formula. The doctor warned him that people with CF tend to lose lung function along with weight.

Discharge and the Plan

Azer using a resistance band the wrong way 😆

Yesterday, Azer ran around outside for a while. He said he felt like he shouldn't have gotten so out of breath so fast.

He had a repeat X-ray last night, which looked unchanged from two weeks ago.

Today, it was refreshing to see our main Pulmonologist. Azer told her how he felt, I told her my concerns. We have three options: 1. Stay in another week on the same antibiotics, 2. Go home with IV antibiotics, 3. Take out PICC line and see if his lung function goes down over the week.

Going home today sounds like what we all want regardless of what his PFTs look like today. Azer and I both wonder if there is something growing deep in his lungs that they aren't picking up on the culture. So, being off antibiotics for a week or so will give time to see if the bacteria in his lungs will come back. If in a week's time, his lung function has dropped, we know there is something there. If it has remained stable or gotten better, it is likely the air quality and allergens that are giving him problems.

Endocrinology

It looks like the Periactin is working! Azer gained about 4 lbs in only a few weeks just from an increased appetite. From his last Endo appointment, he grew about 1/3 of an inch. He told the doctor that he's had hip and knee pain lately, and he feels like he keeps banging his ankles into things frequently. These are signs that he is growing. Another sign we may see soon is he will need bigger shoes.

Azer told the doctor that the pharmacy recently changed needle sizes, and the longer needle is causing the injections to be a little more painful. She said the needles aren't a prescription, so the pharmacy themselves may be able to change this. He also mentioned that the most painful injection site is in his stomach. She told Azer he doesn't have to use the stomach if he doesn't want to. He does need to change injection sites each time, but scarring from injections happens more often with insulin, not so much with growth hormones.

The physical therapist came and looked at him for his scoliosis, since there is a risk of it worsening now that he's growing. She noticed that his left scapula area is higher than his left, and we were all concerned that his scoliosis got worse, so the doctor sent us to get an X-ray.

The results from the X-ray look good. His scoliosis hasn't worsened, which means that the changes in his posture are muscular from growing, and possibly from pitching. He has been working on pitching quite a bit lately. So, it looks like over the summer, he will be working with the physical therapist every other week again to correct his form. If we don't correct it now, it can eventually be permanent.

CF Clinic Follow Up

Today, Azer weighed about the same as last time. It's hard to tell on the growth chart, but we believe he grew some. Because his height is measured at Endocrinology, ENT, and the CF Clinic, and entered into the same growth chart, there are small differences between the measurements at the different clinics (as in, it appears he shrinks lol). But compared to his last CF clinic visit, he has grown a little. One noticeable difference (much to our PT's delight) is that he definitely putting on muscle because of the growth hormones. Another wonderful side effect of the growth hormones (and probably because of puberty as well) is that he has been truly hungry and eating full meals.

One concerning symptom he has had for the past few weeks is hip pain that seems to be worsening. The PT gave him a few stretches that might help, but when she looked and felt him over today, she was concerned the pain was not muscular, that it was in the joint. Hip pain is one side effect that we were told to watch for with growth hormones. We were sent to do a pelvic X-ray to see what is going on.

He also did a chest X-ray to see if that portion of lung that was plugged and collapsed has recovered.

His PFT was fantastic today, likely because he is on Qvar as well as Dulera. The doctor said it's fine short term, but long term being on two inhaled steroids can inhibit growth, and can also affect adrenal glands. So, when he feels bad, she said he can do Qvar for three days at a time.

The doctor also felt like when he had all that chest pain after the flu, that it might have been pleurisy, which she said has been very common with the flu this year. After recounting all the things to the doctor that happened after Azer got the flu has been remarkable.

He was put on an antibiotic that forced him to stop one of his GI motility medications.
This caused him to reflux and vomit, and made it harder to eat and stay hydrated.
They switched his antibiotic to one that would allow the GI medication.
He ended up with severe diarrhea, which forced us to do home-bound education.
He was put on a medication for intestinal overgrowth.
All the congestion in his lungs caused inflammation to the point of pain.
An area of his lungs ended up plugged and partially collapsing.
He was put on steroids, both inhaled and orally.
He coughed up bloody mucus, and had to stop an Hyper-Sal which made him feel congested.
A week later, he experienced chest pain either caused by inflammation of the cartilage of the chest wall, or the lining of the lungs.
A week of ibuprofen, and everything was back to normal!

The flu is something no one wants, and having it twice on top of having CF is potentially deadly. This is just a friendly reminder that if you are ill, please stay home. Get the flu shot if you can. You could save someone's life.

CF Clinic

This was basically a follow up appointment after Azer's hospital visit.

So, when Azer started growth hormones, I put him on 5 cans of formula a day instead of 4 to try to keep his nutrition up, since he hadn't gained any weight in quite a while (he also wasn't growing either). He gained two pounds since then, so the GI doctor said we can go back to 4 cans a day, since he's been having reflux and feelings of fullness in the morning from the extra formula.

The GI doctor said that in the event we have to use the antibiotic, Levoquin again, and are forced to stop Erythromycin, she said it might be worth while to temporarily give Azer a G-J tube instead of just a G-tube. This would help assure that he isn't refluxing formula into his lungs. Another alternative would be to temporarily start Reglan which worked wonders for him in the past, but does come with a black box warning. 

His PFT's went up, above where they were in October. The doctor was pleased with those results. She allowed us to look at his X-ray, and explained why the radiologist's report can sound completely devastating because they really don't see the full picture.

The doctor took her time and really explained to Azer the anatomy of his lungs, and what he was actually seeing on the X-ray.

While clinically, he looks great, and he didn't culture anything serious, Azer told the doctor he felt like his lungs still feel sick and congested. She said that even though the antibiotics are really causing a lot of GI problems right now, that we should finish the course. She listened to Azer's lungs, and heard some wheeze and noises. She said it was a good thing that the noises moved around when he coughed or did treatments. She is adding a secondary inhaled steroid to the one he is using already. This is temporary until he starts feeling better.

Regarding, the continued bloody mucus Azer's been coughing up, the doctor said as long as it's mucus with blood in it, and not blood alone, or blood not mixed with mucus, this blood we are seeing is just from irritation, which is likely coming from the Hyper-sal, steriods, and the dry, winter air.

Azer hasn't been able to return to school yet, and the main problem is how many times he's been in the bathroom a day. We counted 8 watery diarrhea BM's yesterday, and the average is 6-8 times a day since he's gotten discharged. He is almost done with Flagyl, and the GI doctor said Flagyl doesn't always work immediately. They took a stool sample to check for any other bacterial overgrowth that might be causing him issues (when Azer came back from "getting the sample", he said, "I am not nurse material").

Based on this situation, we will be doing homebound school for the next few weeks, which gives more time for Azer to work on staying hydrated, and more time for extra breathing treatments (which, based on his PFT's, seems to be working!) I contacted this school this week, and the staff worked hard to get me the information needed. I was able to get all the paperwork and brought it today for the doctor's signature.

I brought up the PEP device we got at the hospital with a nurse and the Respiratory Therapist. They said they don't have them there at clinic, and really hadn't seen them used. While we were all talking, we got on the subject of the Vest. The RT was surprised that we had a SmartVest. She mentioned that most patients use the Hill-rom Vest, and was able to quickly call the company and find out if we could try one out on a trial basis. She said once we get our information settled with Hill-rom, we can be put on a waiting list for a new Vest called the Monarch. This new vest is portable, and is supposed to work more like hand CPT rather than just pressure and vibrations.

Side note - Wednesday the 17th, after he'd been on the Prednisone for about 5 days, his sense of smell started to return a little. Two days later, he can smell nearly everything. So, it's either the steroids reducing inflammation, or the antibiotics killing an infection in his sinuses.

First Day Home

We've been trying to do breathing treatments every 2-3 hours. Azer can only tolerate 2 minutes at a time on his chest. CPT is causing shooting pains going to his shoulders. Today, he says that it is getting harder to breathe that yesterday, and feels like his lungs are getting worse. His lungs definitely sound much noisier than yesterday, and he is producing more. I really feel like the largish spot on his X-ray may have been pneumonia instead of just simply mucus plugging.

After sending a semi-panic stricken email to the CF clinic, he will be starting steroids for the next week. Hopefully by the end of the course of steroids, the antibiotics will really start to kick in! 

Discharged

Azer had a very hard time doing a PFT test. He was refluxing and nearly vomited several times. Thankfully, the technician was very patient with him and let Azer blow when he was ready. He is, not surprisingly, about 10% lower than normal.

They switched him to a different antibiotic so that he can go back on the erythromycin. He was practically begging for it earlier.

His cough sounds pretty wet, and his x-ray showed some mucus plugging, which is probably why CPT hurts so much right now. Hopefully it will clear up soon, but we are very happy to be discharged!!

Day 3

Yesterday evening, Azer's IV really started to bother him. It flushed fine, but just running fluids through was causing pain. The doctor said as long as he drinks plenty of fluids, they could take it out and not replace it.

The most painful part of the removal is the tape. The nurse was very patient and let Azer remove it slowly himself a little at a time.

He hadn't drank much all day, so a resident doctor came in and essentially threatened Azer that if he didn't drink enough Gatorade, he wouldn't be discharged. It worked, and Azer managed to drink and hold it down.

He's had reflux issues since we've been here. It's difficult to keep track of what medications he's taking when they're given on a different schedule than we do at home. This morning I realized he hasn't been taking Erythromycin, which he takes for GI motility. Apparently, it reacts badly with the antibiotic he's taking right now. There is another antibiotic he could take, but he has experienced chronic vomiting with it. So, they are trying to figure out what antibiotic would work for the bacteria he cultures without causing an interaction with his other medications.

CPT has been painful for him, especially the upper loves, and we've only been able to do a few minutes at a time. He's been trying out a different device called PEP which is different than the Aerobika in that instead of using positive and negative airway vibrations, it only uses positive pressure to open the airways. It has been helping bring up a lot of mucus. Unfortunately, last night, his mucus had some bloody streaking in it, so we are keeping an eye on that.

The doctor ordered an X-ray to see how his lungs look. She said if we did a PFT, it would be kind of pointless because we know it would be abnormal between his refluxing and congestion. After she looks at the X-ray and figures out his medications, we might be able to be discharged today!

Admission

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. 

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.

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.

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!

Clinic

Had another fairly good CF clinic appointment. He is now 100 lbs! Triple digits! The formula is working great, and he is absorbing most of it. He isn't eating a whole lot, but they aren't really concerned with that right now since our goal at the moment is to get him growing taller. Which isn't really happening. In a few months, he's only grown a tiny amount. We have not heard back from the endocrinologist since the last appointment, so our GI doctor is going to put in another referral. He is going to need an X-ray for bone age to rule out any osteopenia. While their at it, they will do an X-ray to make sure his scoliosis hasn't progressed. We are facing a real possibility of growth hormones, which can put him at an increased risk of CF related diabetes, so the endocrinologist will need to evaluate the risk versus the benefit of getting him to grow. The GI doctor said with the X-rays and all these months of aggressively gaining weight without growing will provide evidence to the endocrinologist that we have tried to get his body to grow on it's own. It is not clear why children with CF sometimes do not grow as they should, even with above optimum nutrition

Azer's Mic-key button has been bothering him a great deal lately. Apparently within a few months, he outgrew his last size, so an ostomy nurse came and put in a larger size. He said it felt so much better after. 

The erythromycin has been working very well to control his feelings of bloating and fullness, although it doesn't seem to be actually making the stomach empty faster. The CF doctor wants us to discontinue azithromycin, since they both are in the same class of drug, and he has not cultured pseudomonas since 2013. She mentioned that if he does culture Pseudo again, we can put him back on azithromycin at that time. Some studies have shown that azithromycin can prevent pseudomonas from growing a biofilm, which is like a shield that it can grow to prevent antibiotics from killing it.

During spring break, our GI doctor would like us to back the bethanechol to twice a day instead of with every meal. 

We all had a virus come through the household about a week ago, and as can often happen with Azer, the virus left, but the congestion in his lungs can stay and get worse in a short time. His cough was concerning to her, so she wants to put him on an oral antibiotic for a few weeks to help his lungs clear the junk out. His Fev1 was 86%, which both the doctor and I agreed wasn't bad, but we know he can blow higher.

Our CF clinic is beginning to test CF patients 12 years and older for depression and anxiety, which will be confidential between him and the doctors. It is good that they have started this early, so if he has issues later in life, he will hopefully feel confident enough to share any of those feelings with them before they become issues. 

Going Home!

Yesterday, Azer blew 94% FEV1 on his PFTs. After only a few days on IV antibiotics, his lungs have improved considerably, so the plan is to continue the IV antibiotics at home. They are also waiting to see if he cultured any Aspergillus antibodies. His IGE level was elevated but not considerably high for him. His CT scan on his sinuses came back. Apparently, they are quite blocked and there is scarring and thickening. He had no obstructing polyps. The plan is to do a special sinus nebulizer after we are finished with the IV antibiotics.

It seems like the reflux hasn't really been addressed yet. The increased Zantac hasn't improved things. The latest I heard was we are going to restart the Nexium and continue with the Zantac.

He is ready to GO! 

Clinic Day

When Azer was on the Voriconizole (antifungal medication) he had cracked and bleeding lips for nearly a month, and also his very first sunburn. The sensitivity to sunlight was so bad, that even sunscreen didn't help. The nurses were pleased to see his face and lips had healed completely. Where he had a sunburn on his cheeks, he now has freckles. He is on a pretty low dosage of steroids now, and his appetite is slowly going away too, unfortunately. Thankfully, he gained a few pounds.

We saw a different doctor today, but both Azer and I really enjoyed him. He explained a lot of things to Azer, and talked to him on his level. He also commented how smart Azer was when he asked him a few questions about his G-tube and what would he do if it ever fell out. 

His lung function was great, and he sounded pretty clear. His X-ray from October still showed some mucus in spots. During the time when the doctor was explaining the chest X-ray to Azer, he explained what bronchiectasis was to Azer in a way I had never heard it, and it was just a bit frightening. He brought up a CT scan that was done on Azer's lungs last year. He explained bronchiectasis is when the bronchi get damaged and floppy, and they also get holes in them. He showed us the tiny holes that you can apparently only see on the CT scan. 

Another area of concern was Azer's growth. I hadn't really paid attention to his height as much as his weight, but that is usually how the information is presented to me during clinic. The doctor pointed out to Azer that he used to be around 25th percentile in height, but now he is below 10th percentile and really tried to make a point to tell him that he has got to eat in order to grow. 

To know that his lungs have tiny holes and that he has scarring that damage that will never go away is a let down, but I know I have to trust God in this and work harder than ever at keeping his lungs clear. 

Scoliosis and lower PFT's

Well, today was our annual big CF checkup. Once a year, they do basically a head-to-toe exam on him. This includes yearly blood work and X-rays.

He gained a pound since his last appointment in May. I really don't know how since his appetite has been next to nothing lately. He grew a whole inch, which makes his BMI just a little lower, but overall, they are not too concerned with his weight.

His PFT (pulmonary function test) showed that some of the smaller airways are constricted. His FEV1 was only 74%. That's down from 92% in June. He definitely has some infection going on. He has been coughing up nearly green mucus. Not quite Nickelodeon slime green, but about that thick! He has been on oral antibiotics about once every 3-4 weeks. They are going to try another course of oral antibiotics to try to clear this up. Lets pray it works. If not, it's IV antibiotics. Yikes.

We had a few concerns with trying to work out keeping him hydrated at school as well as snacks and restroom time. Whatever he needs was written in a letter signed by the doctor himself. It's so nice to have a team backing me up and fighting for what he needs.

We waited about an hour to get his labs taken. He had been fasting all morning so they could get a glucose level. They took quite a bit of blood this time. He said he felt weak afterwards...

So, at his recent pediatric appointment, she noticed possible scoliosis. The doctor today took a look at it, and did see some minor curvature, and said we would keep an eye on it with periodic X-rays. Orthopedic X-rays were done on a different floor than we're used to. I couldn't help but feel just a bit sad, even though it is minor and quite common. I didn't know this before, but apparently it's quite common among people with CF.

After 5 long hours there, and fasting since this morning, we went out for our lunch date afterwards. He definitely deserved it!

Er Visit

So Thursday, I got a call from the school. The school nurse said he was doubled over in pain. A few minutes later, I got a call from the doctor's office. The results of his ultrasound were complete. She said he has gallstones. After I told her how much pain he was in, they wanted to admit him.

By the time I picked him up from school, he was still in a lot of pain. I had given him ibuprofen and picked up  medication from the pharmacy that is supposed to dissolve gallstones. The pain hadn't subsided at all, so the CF nurse suggested taking him to the ER. Yay, our favorite place, right?

After an entire evening at the ER, and one X-ray later, they found out that he was incredibly constipated. About 30 minutes after they gave him an enema, he was 100% back to normal. So, apparently, keeping the intestines lubricated is extremely important, even though he was having normal BM's.

So, it seems like the gallstones aren't causing any pain, but he will be on the medication to dissolve them for a while, to prevent any complications.

I am very happy that it was a simple fix, and nothing scary like organ damage.

Sort of Better?

Tuesday morning, I rushed Azer to the pediatrician because I was nearly in a blind panic because his cough just wasn't getting better. I hate it when I hear that deep, mucusy cough, and I just get a terrified feeling when I hear it. After being stuck in a tiny room for three hours, and doing another X-ray, I was basically told to just continue doing what I'm doing.

His X-ray showed some improvement. The CF doctors never mentioned this term before, but the pediatrician said there was "atelectasis". The doctor said not to think that it was about lung collapse, but that it was more like mucus plugs in the lower left lobe. So, I've been trying to focus CPT in that area to try to clear that out. Otherwise, his X-ray is improvement since Saturday. I am continuing the Clindomycin. His diarrhea has improved since I changed the type of probiotics he's taking. He is coughing and spitting up mucus during CPT. I don't notice him coughing up mucus during the Vest.


This morning, he threw up again. Later on this afternoon, he was bringing groceries and he ended up getting something that was like an asthma attack, and he had a terrible coughing fit, and his face started looking slightly gray. So, a quick nebulized Albuterol and a 15 minute Vest treatment ended it quickly.

So, sometimes it is obvious he's getting better, and other times I just don't know. But, we'll keep pounding away at him. :)