Bone Density Test and CF Clinic

Today Azer had a bone density test to test for osteoporosis that can affect people with CF. We'll find out the results later this week.

Azer has been working hard and taking a lot of shifts at work, sometimes working up to 40 hours a week. His pulmonologist is a little concerned about how many hours he's working as at times he's too tired to wake up on time and do his complete treatment. He is still working on trying to adjust his schedule and work out a routine to help remember his medications, and give himself enough time to do his breathing treatments.  

Azer has been been more vigilant about doing his overnight feeds, and gained several more pounds from last week. His lung function fell a little more from last time, but the doctor is going to continue him on Tobramycin instead of doing a month on and a month off. They did another virus test just in case, but it seems most of his coughing is coming from drainage. Two weeks ago, he did test positive for rhinovirus, and viruses are starting to pick up as more people are passing them around now. 

Since he's been working so much, he has been saving up money to pay for car insurance. He passed his driver's license test and is finally a licenced independent driver! He has been enjoying his freedom and has spent a lot of time fishing on his days off!

CF Clinic - Hospital Follow Up

The CF clinic has been calling previously made appointments to see if they can be cancelled. Azer had a mild virus at the beginning of the week when they called, and decided to keep his appointment, since his cough was much more frequent. It improved over the week, and today it was much better. It was hard to tell if any remaining lung irritation is from allergies, but given the weather, it's definitely possible. The clinic building was fairly empty, so it was easy to stay away from others.

Azer lost about 4lbs since he was discharged. His appetite hasn't been that great lately, but the doctor said she isn't worried, as he's still above 50% for his BMI.

The doctor was very pleased with how he sounded today. This is the first spring Azer has experienced with Dupixent, the allergy shot. Usually, he is feeling congested, wheezing, and close to needing antibiotics. The Trikafta really seems to be helping as well. He hasn't coughed up mucus during treatments since he was discharged from the hospital. He says it's weird to cough and not have mucus come up. He said sometimes he'll cough on purpose because it feels strange to not be coughing all the time. 

Azer's PFT today was one point lower than it was two years ago. He hasn't been able to blow that high in a long time, and this is also considering the computer is basing it off of his predicted size for his age (meaning the computer expects him to be nearly adult sized, based on his age). Azer was pleased to finally have a great clinic visit after nearly 6 months of being off and on sick, and not having a clear reason why. 

Azer has been staying busy playing Xbox and practicing hitting and pitching in the backyard. He has also been lifting weights more often now that he's feeling so well. If anyone wants to play Xbox with him, his username is SaltySP#6415

Week 6 and Clinic

Azer still felt good even through the switch to oral antibiotics. Going home without a PICC line was worth it, as the very next day he got discharged, it actually snowed, and he had a blast sledding and playing in the snow.

He hasn't been coughing as much, and isn't bringing up much mucus during treatments. He has gone back to doing hyper sal twice a day. He coughed up sort of bloody mucus one day and other days, dark mucus. His cough continued to be clear even after he finished his antibiotics. He hasn't been able to laugh without having a cough attack in a very long time, but this week, it has gotten so much better. Through out week 6, he got off and on pain in his chest, and he couldn't tell if heart pain or lung pain, but it was most likely a mucus plug moving out.

Insurance is requiring us to switch his Dupixant to another pharmacy or they won't pay it. We have been fighting to get it, and unfortunately he ran out of it and has been experiencing increased mucus and allergy symptoms, which proves how much it was actually helping. When he was admitted, they tested his IGE levels, and they were lower than they have been in 8 years. The Dupixant has helped immensely, so it is frustrating to not be able to have it right now. They are also requiring us to switch his Trikafta as well, and he has a little over a week left, so I am really hoping it gets transfered before he runs out. The doctor assured us that other people with CF that have struggled getting their Trikafta due to insurance have done pretty well being off of it for a week. We are also having a hard time getting his growth hormones covered, as he hasn't grown much at all in the past year, and according to the insurance, there is no reason for him to continue being on them. 

Azer has been continuing to do 6 cans of formula a day, and he has slowly been regaining the weight he lost. He feels like the Remeron is helping, as he actually has an appetite now, even when taking his ADHD medication. He has had some painful reflux since he started eating solid food though. The GI doctor is increasing his enzyme dosage and also temporarily doubling his Nexium dosage.

His PFTs were lower than when he was discharged. This is most likely due to his allergy symptoms from not having his Dupixant shot. One thing that was different on the test was that normally the slope of his exhale shows lung restriction. This time, it was showing less lung obstruction. The doctor was so pleased with how he sounds, she said he can stop taking one of his inhaled steroids. There is a study starting soon that will show what other medications people with CF can stop taking. They are doing a virus panel to see if his dark/greenish mucus is from the rhinovirus still lingering. The doctor said the virus is presenting in a flu-like way this year, making even healthy people pretty sick. If his lung culture shows anything other than MSSA, she wants him to start I

inhaled antibiotics for a month. 

Azer had his annual PT evaluation, and his capacity on his test was lower than last year. He also has regressed on his posture so, we may need to start out patient PT again. Azer has had some tendon issues in his left wrist. It's not too severe, but he does have to hold off on some pitches for a while. The doctor said it's possible that the antibiotic he was on in the hospital caused this to happen. 

Azer mentioned to the doctor that he has had numb and tingly fingertips since starting Trikafta. The doctor figured out that it is aquagenic wrinkling, since the tingling is happening when his hands are sweaty. In some people with CF, aquagenic wrinkling can even be painful, which Azer has experienced when swimming. The doctors took note of all the Trikafta experiences, good and bad, as it helps Vertex see how it affects everyone differently!

Sick CF Clinic Visit

Everything had been going great, and we thought we had the summer to relax a little after doing IV antibiotics. He felt great and sounded clear.

Sunday morning, he suddenly sounded a little congested. Within hours, he spiked a fever and was suddenly coughing up brown/rust colored mucus. The CF doctor on call suggested perhaps it was transiet, but to bring him to the ER if his fever got any higher. He took some ibuprofen and the fever broke later in the evening. Yesteday, he was still coughing up some dark mucus, but seemed to feel better. Today, Azer said his legs don't hurt, but they feel uncomfortable, and he has a headache.

At clinic today they did a virus swab to see if he picked up a virus somewhere. He gained several pounds from last week. His lung function was down slightly. His lung culture from last week found that the Steno is now resistant to Levofloxin. In order to treat it, he will have to be on an inhaled antibiotic for a month. The doctor warned us that the nebulized antibiotic smells like cat pee (awesome). They put in an order for a CT scan for in a few weeks. The doctor said if they tried to do a CT scan right now, radiology wouldn't be able to tell if he has fungus spores or not because of the increased mucus.

It will take some time to receive the inhaled antibiotics since they have to come from a special pharmacy. Azer is currently laying in bed not feeling well...

Update: Azer's virus panel came back negative for all viruses, so his symptoms are from bacteria and/or fungus. He was also told not to use his IPV while he is coughing up brown or red mucus, just in case he is having a slow lung bleed.

Follow Up Admission CF Clinic

Azer has been working hard this week. To try to avoid his dressing coming off from sweating, he has been riding the exercise bike everyday instead of running. 

We did bend the rules a little bit. He actually rode his bike while hooked up to the medicine ball IV.

Medicine Infusion balls make IVs at home easier

Today marked one full week on home IV's and a complete 14 days on IV antibiotics. Azer ran this morning to help make his lungs as clear as possible. He was disappointed to see his numbers were lower than when he was discharged. He says his lungs feel completely clear. He sounds clearer than he has in a long time. The doctor said it was probably time for a good clear out of the two bacteria he normally cultures. She explained in a little more detail about the ABPA test and his high IGE. It is possible that he does have Aspergillus growing somewhere in his lungs. She said at his next CF appointment, if his PFT's continue to fall, we will start an antifungal medication. In the mean time, she will do some more research to see if an allergy shot specifically made to lower IGE levels will help him. 

The good news is he put on 5 more pounds since being discharged. He has been eating well lately. 

Both Azer and the doctor felt like he had gotten the full benefits from the two antibiotics he has gotten these past two weeks, so he was able to have his PICC line pulled today. He can stop the Reglan and go back to taking his EES and Probiotics! 

Summer can finally start for him now!

Admission Follow Up

Azer blew a few points higher on his PFT than he did the day he got discharged. He still felt like it was harder that it should have been to get there. The doctor felt like he could still get back to his base line if the weather and his allergies were optimal. The great news is that he feels like his lungs are very clear, and he has been running in the mornings and has not been out of breath. So we will try to make the most of the rest of summer before high school starts!!

Azer also felt that dermatology was wrong about his skin peeling. He said that as soon as they had switching him from Gentamicin to Tobramycin, the peeling stopped progressing. The doctor said that it was a possibility it was from the antibiotic, but that the reaction he had was just undocumented.

He ate very well while in the hospital. His appetite hasn't been great since getting home. He has had an upset stomach and diarrhea for the past week. The good news (sort of) is that I also ended up with these symptoms, so it may be a virus.

While the IPV did great for him in the hospital, and we are persuing getting one for home use, he felt like getting back home and using his vest helping clear out some mucus that wasn't moving otherwise. Of course, him getting back to running has helped him feel 100% back to normal!

CF Clinic

About 2 weeks ago, Azer suddenly started to sound congested. Oddly enough, it started during his week off of Periactin. The week off of Periactin, his seasonal (year round lol) allergies hit him hard. During Memorial Day Weekend, he began to sound so congested, we all wondered if it was time for an admission. He also began to cough up brown mucus. The on-call doctor prescribed some oral antibiotics to see if that would help. Since then, his cough has improved slightly, but he says his lungs have stayed full.

Not surprisingly, his PFT's were down today. The Pulmonologist said that his small airways were the same as they were last time, so whatever is going on with him is unlikely to be ABPA (our worst fear, as it would put a damper on summer plans). She said he should finish his course of antibiotics, and start Tobi Podhaler while we wait for his culture to come through. Even if it isn't Pseudomonas, the Tobi should also help out with the Staph he normally cultures. She heard some mild wheezing in his lungs. They also took a nose swab to see if he's culturing a virus.

He put on some weight, so the Liquigen is helping him out. His appetite is very good when he's on the Periactin, and he has been enjoying a healthy appetite, although it has come and gone since he's been sick. He is also growing!

Azer is going on a week-long trip with his youth group. This will be the first time he is fully responsible for all of his treatments and medications. The doctor went over things that were the most important (such as staying hydrated and ways to add sodium) and she went over a few ways to make his treatments easier, such has medications he doesn't have to take during that week.

Azer is still taking Dulera and Qvar. Well, apparently Qvar has stopped making the inhaler type, and is now coming out as an inhaled powder. Since it's already been ordered, and the doctor feels like he's old enough to be able to inhale it properly, he can try it out and see if it works for him. If not, we have to go back to Flovent.

- Update: Azer's viral panel came back positive with metapneumovirus. His lung culture only came back with MSSA. He is doing great with the new Qvar, and doesn't really notice a difference in how it feels, other than this Qvar is not taken with a spacer.

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.

CF Clinic - Annual Visit

Today was Azer's "Big Clinic Day", which includes a mental health screening, blood draws, two sputum cultures, a fasting glucose test, and a glucose test 2 hours after drinking liquid glucose.

Azer has been able to maintain his weight since the last clinic. The GI doctor was very happy with his weight, and he has grown some, but his height is still plateauing. The GI doctor said that at our next clinic, they will bring in the Endocrinologist and we will discuss growth hormones again. Azer told the GI doctor that he is still willing to start growth hormone shots.

About a week ago, Azer started oral antibiotics after some sinus congestion seemed to move into his chest. He cleared up very quickly after starting the medication, and today his PFT's were 95%, and the doctor said he sounded clear. The doctor said that if the chest congestion returns after he's off of the antibiotics, they may consider putting him on inhaled antibiotics until winter time. Azer told the doctor about how physically active he is, and both the doctor and respiratory therapist were impressed with him, and agree that exercise is so often downplayed when it's one of the most effective airway clearance treatments.

They noted that his blood sugars have been a little high in the past, and that if this one turns out to be borderline again, we may do a month of additional blood sugar testing to ensure it isn't getting dangerously high.

Trying to raise a successful teenager is challenging. Trying to raise one with a chronic health condition definitely is more difficult! Everyone was impressed that Azer has been getting more independent with his care, medications and exercise.

Today, we were introduced to a new product called Relizorb for G-tube feeds. It is basically a cartridge of enzyme beads (Lipase only) that screws onto the bag of formula and gives continuous dosages of enzymes through the feed. Since this product only helps with the absorption of fat, they recommend still taking regular enzymes at the beginning of his night feed. They have already had some great results from other people, so we are looking forward to trying it out! 

CF Clinic

Last week, Azer seemed to have come down with a virus that only seemed to last for a few days. The virus brought nasal drainage, so that on top of any allergies he has right now has caused all that mucus to end up in his chest. On Friday, he really seemed to be having trouble breathing through it all. We increased breathing treatments, and did manual CPT about 5 times a day.

His PFT's were 86% FEV1, which is lower than his usual baseline. We were expecting it to be lower the way he sounded. When the doctor listened to him, he had some wheezing that moved around, and some noisy spots on his lungs. We all agreed that a course of oral antibiotics would help a lot right now. Luckily he only cultured sensitive staph again.

We had a new member of our CF team come visit us. Our clinic now has a Physical Therapist. She said she loves working with the CF team, and thought it was kind of interesting that most of what she sees in the CF clinic is sports injuries. Although that's not necessarily a good thing, it means that the majority of our CF kiddos are very active and athletic.

Even though Azer is already very active, she did measurements on him in various places. She noted that his posture is incorrect, and is likely due to all the years of coughing and also having a G-tube. She came back a few times during our clinic visit. She had him do some movements after his PFT's, and then later on, she took him out into the hallway to try out some stretches. There was a lot more to it than I ever thought there could be. She hopes that in three months, his form will get better. After his form has improved, she hopes to have him start weight bearing exercises. She expects that this will help his pitching form, his Osgood-Schlatter's knee, and his scoliosis. There is also evidence that doing the proper PT could reverse low bone density.

Azer lost a few pounds, which put him down closer to 50th percentile for weight, which is a bit more normal. He also grew nearly an inch since his last clinic visit. The doctor is hopeful that his body will catch up in height now that his nutrition is more than sufficient though puberty.

After clinic we had chest X-rays just to see how everything looks. Overall, everyone thought he looked and sounded great, and is very happy with all the work we put in at home. 

CF Clinic

Our appointment today started out with a visit from the psychiatrist's assistant. She had several surveys for us as parents to fill out, and several similar surveys for Azer to fill out. She also did a verbal survey with Azer over the how he felt about his treatments, and how much annoyance they caused in his life. The surveys we took had to do with our family dynamic, how we function as a family, and how we cope with problems together. They are using these for research purposes. Just a good mental note to make, that not all CF research has to do with pills or medications!

Today, Azer's weight is stable. His height is still on the 10th percentile, so the doctor said she would talk to the endocrinologist again and see if growth hormones would help since he has already started puberty.

A few months ago, Azer had his teenage well visit with his pediatrician. She was very concerned with how much weight he had gained over the year, and put in an order to have his cholesterol tested. I mentioned this to the CF doctor today, who essentially said that if the pediatrician had even read the notes in the system, she would realize he is being followed closely by a whole team of doctors regarding his growth and weight. She said it would be very unlikely that any person with CF would have high cholesterol due to diet. She reminded Azer that the general goal is for his weight to be 50% percentile or better as research has shown this helps lung health. She also said that if as a doctor, if she only focused on the numbers, and treated a patient by what the numbers said (pft's, weight, etc.) the quality of the life of the patient might not be so great.

His lungs sounded completely clear, with no crackles and his PFT's were at 93%. He still has nasal drainage and has been complaining of a bad odor he smells when he breathes out. This hopefully will be resolved when the ENT doctor removes the splints stitched in his nose.

Since his sense of smell has returned (yay!), Azer's appetite has been improved. He has been showing a lot more interest in food, and has been enjoying the smells of foods being cooked. So, I believe the polyps removal and the reconstruction of his septum has been a big improvement for his quality of life.

Azer recently competed in cross-country with his school and did fantastic. Although he wasn't first, I reminded him he outran a whole bunch of boys that don't have Cystic Fibrosis! 

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.

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.

Clinic Day

About two weeks ago, Azer was stilling having reflux issues, so the GI doctor decided to switch him from bethanechol to reglan which can be used to treat gastroparesis. While it hasn't made the reflux/vomiting completely go away, it has helped significantly. Currently, he has only been having food come up if he is running or playing aggressively. The GI doctor said there is little they can do about that, because most of what is in his stomach is usually just formula, and it is easier for liquid to come up than solid food.

So, yesterday, both the GI and Pulmonary doctors were very pleased. He is now 95 lbs, which puts him around 60th percentile for weight. The ideal weight for a CF person is 50%, so we are doing fantastic. On the growth chart, it looks like a straight line up.  His height is finally starting to creep higher above the 10th percentile. It is moving slowly in the right direction. When the pulmonary doctor came in, she said that the GI doctor was grinning out in the hallway. His weight is high enough now to have to move up to a higher strength of enzymes and he hasn't seemed to have as much constipation since we started the Reglan.

His Fev1 was only 83%. However, the doctor said they now have a new computer system they use for PFT's. She showed us based on the volume of air he blew out, that his total volume is increased compared to last time. She believes his Fev1 was actually in the 90's because of this. I was grateful for her taking the time to explain and compare the test. She listened to his lungs and she said he had great airflow and no wheezing at all. She mentioned that the pushing technique I do after CPT helps clear the mucus out of the smaller airways, so on we must continue!

CF Clinic and Annual Blood Work

So, today was one of those longer clinic days. He had to be fasting starting at midnight for his glucose test today. They drew blood for a number of tests when we first got there. He then had to drink a few cups of glucose solution and wait two hours.

He weighed in at 86.8 lbs today! He gained almost 8 lbs in just two months. It appears the higher calorie formula is working! On the down side, he just didn't grow much at all, and hasn't, even since increasing his caloric intake. We will see an Endocrinologist in a few months who may have some ideas as to how to handle this issue. We hope his body will kick it in gear and start growing now that it has some extra nutrition.

About two weeks ago, we had a week of vomiting only in the morning. After switching his morning medications to afternoon, it seems to have cleared up, but he is still having reflux frequently. Because of this, the GI doctor wants him to have a gastic emptying scan to see if his stomach is holding onto food for too long. She also wants to do an endoscopy to check for any other issues that could cause problems.

He got an 83% Fev1 on his PFT. It's down about 10 points from last time. Because it seemed like the Symbicort worked well last month, we are going to start it back up to see if the decline is related to his asthmatic/allergic components.

After the two hours passed, they pricked his finger to test his glucose again, which came back normal. No CFRD! (CF related diabetes). Later on this week, we are scheduled for the gastric emptying study.

After fasting and a long clinic visit, we decided tacos sounded great.

PICC Line is out!

Yesterday was Azer's follow up appointment with our CF doctor. We have been working hard to get his lungs clear by running in the mornings again (until he sprained his ankle Tuesday lol), and manual CPT. The GI doctor was able to get his reflux under control to the point where he has had almost no trouble with it at all, so coughing all the junk out has been much easier. Doing IV's at home is always a bit challenging, but in the end, it is always worth it for him as he gets to play with his friends and ride bikes. Of course, there were moments of longingly looking at the swimming pool. 

We have been very vigilant about keeping up with the bolus feeds, and saw the rewards of our labor. He is nearly 77 lbs, and for the first time in his life, he is finally over the 75 lbs! He also grew about a quarter of an inch in a month. The doctor still wants to see his weight closer to the 50th percentile, and hopes if we get his weight higher, that his height will inch back up towards the 25th percentile. 

He got a 92% on his PFT's. I was surprised it was lower than when he was in the hospital. The doctor took time to explain that because he got taller, the calculations for lung volume can change slightly. I feel like no mater how many times they explain what all the other numbers on the PFT's mean, it still never makes complete sense! 

The doctor felt like Azer had spent enough time on IV antibiotics to have his PICC line removed yesterday. 

Because Azer has been playing a lot outdoors, he has been sweating too. This can cause the sticky dressing to come lose, and expose the wound to infection, so we've had to use extra dressing to keep it on.  The removal of the actual catheter isn't painful at all, but removing the dressing (or any dressing changes) can be very painful. It is much worse than removing a band-aid, as the special dressing they use is meant to stick as well as stitches. Cleaning the area stings as well, because removing the bandages does cause some skin irritation.

We are going to do another week of oral antibiotics just to make sure that he stays clear for a good while. 

Luckily, his sprained ankle is healing quickly, and he was able to get some batting practice in for the first time in two weeks!  

Reflux, blech!

So, our GI doctor has been out of town, which was part of the problem of treating his reflux in the hospital. Yesterday, his reflux was terrible, and he couldn't hold it down about an hour after his afternoon bolus. It was a little scary, with the poor kid retching and crying in pain. I can't deal with vomit, so he was trying to shoo me out of the bathroom. He was so bloated after, even though his stomach was pretty much empty.

I called the CF Clinic, and was very grateful to have someone answer the phone (do to some awesome people :-) and she was able to get a hold of the GI doctor quickly, and we made a few small changes to make it through the night. We went to clinic this morning. We didn't do another PFT since we had one just a few days ago in the hospital. He somehow managed to gain a pound and grew a bit.

We increased dosages of a few medications (bethanechol and zantac), added some new medications, some to help the stomach empty, some to help the stomach hold food better, and some to try to coat the irritation (Carafate). I learned today that when he has a lung flare up, it can also cause inflammation in other parts of his body, and can cause his GI system to work even slower than it normally does. So not fair for someone who needs to eat more when he's sick! The doctor mentioned a new type of Proton Pump Inhibitor that has helped a few other patients. It might be tricky to get it, but we're hoping.

We had a dressing change on his PICC line today. We plan to do one next Thursday, or possibly have it taken out! We might get an endoscopy done when the IV's are over to make sure there aren't any other issues causing his reflux and tummy troubles.

CF Clinic

After the last sick visit, he was prescribed some pretty tough antibiotics along with a brief time on steroids. His cough improved temporarily but his cough came back again. So this was a follow-up and sick visit.

He gained a few pounds this time (despite his poor appetite) which is fantastic. They are still pushing for more calories because his growth is just so slow lately, and his height percentile is tapering from where it used to be. We will be switching to a higher calorie formula, which should push a few more calories per ounce.

He has been having a lot of issues with reflux, but our GI doctor decided a few weeks ago to try a different type, and it seems to be working. We may have to try a course of Flagyl soon if he still continues to have issues. We are also increasing his Bethanechol which helps his stomach move food along faster.

His salt was a little low, and I will be adding salt to some of his G-tube feeds as it is a struggle to get him to drink one (1) bottle of Gatorade a day.

His Fev1 was 79%. I know it can be much higher. So, we had a choice. Do another long course of oral antibiotics (the last course just cleared all the bacteria out of his colon and his very hard on his stomach, which means lots of time in the bathroom), or do a course of IV antibiotics to clear him out. Doing IV antibiotics seems to be the best choice at this time. It's been a few years since his last tune up, so it is needed. The good news is he has only been culturing bacteria that responds well to broad spectrum antibiotics. The plan is to wait until school is out, go in for the PICC line and some X-rays, and then do the rest of the IV antibiotics at home. Hopefully this will clear all the junk out of his lungs so he can have a summer free from antibiotics, steroids and IV's!

We are still participating in the CF walk this Saturday. It will be rain or shine, so I hope to see everyone there!!!

Sign up or donate here! http://fightcf.cff.org/goto/joggersforazer

Clinic Day

At Azer's clinic visit three months ago, the doctor noticed his growth was starting to plateau out. Since then, he has been doing 5 cans of formula a day through his G-tube instead of 4. Three at night, one in the morning, and one when he gets home from school. He gained 4 lbs in 3 months, and grew almost a whole inch. I believe it's working unless his long hair is adding to the weight!

He got a few colds over the last few months, and the last one just seemed to stick in his lungs. His Fev1 was down to 81%. He has been junky for weeks. The doctor is putting him on oral Bactrim for 2 weeks unless he has cultured something that would need different treatment. They also tested his IGE level again to see if it's up. I'm really hoping not. I feel like the last ABPA took so long to treat, and he was on Prednisone for months! Hopefully the oral antibiotics will get his lungs feeling better again. He commented recently how much relief he gets from manual CPT and running, so we have been doing both without fail!