November 21, 2020

Surgery and ENT Appointments

This week Azer had an appointment with surgery and with ENT. The surgery appointment was basically just a formality to make sure his stiches had disolved and that things had healed well. He was given the all clear to increase the rate of his G-tube feeds, provided it's not causing pain. He has been gradually able to eat more food, but still has to eat smaller than his usual amount at a time. 

The ENT appointment was to see if anything in his sinuses is causing him to get sick again. The ENT doctor said everything looked good, and she didn't see any polyps. She recommended him to continue to use his sinus rinses at least once a day. 

He is still coughing quite a bit, but hasn't had fevers or chills this time. The CF clinic said that if his lungs continue to decline, they may start oral or inhaled antibiotics. 

November 10, 2020

CF Clinic

It's been about 4 weeks since Azer's surgery. He has been throwing up pretty much with each meal. The GI team said he should make sure everything he eats is basically moist or wet and that he should try to chew longer and take smaller bites.

He has lost 7 lbs since getting discharged from the hospital. His weight loss is due to him not being able to eat as much as he usually does. He also had a braces adjustment where they are correcting for a cross bite, and the elastics are causing a lot of pain, even weeks later. Azer is already off of one of his stomach motility medications. Today they want him to stop one of the antacids he's been taking for a few years. They said the goal is to stop taking most of his GI medications. 

About a week ago, Azer started having an increased cough and was coughing up darker mucus, pretty much all day. After increasing treatments, he started to feel a little better. His PFTs were pretty much at his baseline today, and a little higher than when he got discharged from the hospital. The doctor said his lungs sound pretty clear except for a few crackles. They wonder if most of it is draining from his sinuses and think a visit to see ENT might be helpful. In the meantime, they want him to use an anti-inflammation nasal spray before his nasal rinses. 

October 27, 2020


Surgery, GI and the Pulmonary teams all feel comfortable with Azer going home today. His PFTs were better than when he got admitted. He has a follow up with the CF clinic and the ENT doctor in a few weeks, as well as a follow up appointment with surgery. He needs to be on the mechanical soft diet for 6 weeks, and keep his G-tube feeds at a rate for the next 6 weeks as well. This one is tough, as his morning feed ends up lasting for 5 hours, so even though he is craving a lot of different foods, he is too full of formula to eat much at all. A friend of Azer's sent some Pho noodles which he should be able to eat!

Having the Nissen has been a learning experience for Azer. He learned he has to be mindful of how well he chews his food. He's found if he doesn't chew well enough, even soft foods kind of stay stuck and don't go down, and eventually come back up again.

GI took Azer off of one of his GI medications, as they felt like he didn't need it anymore, now that he can't physically reflux. He's been off of it for four days now, and he hasn't experienced any bloating, which is amazing. 

Overall, he is recovering very well and is so glad to be home!

October 23, 2020

Ready To Leave

Azer is very ready to go home, and has been cleared by the surgeon to go home. The GI doctor would like him to stay until Monday. Their reason is by Monday, his IV antibiotic course would be complete, and he would be able to do another PFT test. His PFT from yesterday was worse than when he came in, which is to be expected. They did an X-ray earlier in the week, which showed that he still had some gas in his abdomen, so it still hurts to take a deep breath.

They have been slowly increasing the rate of his G-tube feeds, and by Monday he should be at the rate they have been aiming for. They want to make sure that he is tolerating the speed and amount of formula. They are also taking away one of his GI medications now that Azer cannot reflux. 

Azer felt like he might have been refluxing, but he later realized that the burning feeling isn't going up to his esophogus, that it is staying lower. He said the new medication that coats his esophogus doesn't help the burning, but that tums does help. This is great news, as it shows that the Nissen is working. 

More good news is that Azer hasn't needed any pain medication for the last few days, even though he has been increasing his activity during PT. 

October 20, 2020

Continuing to Recover

Azer is recovering well. He is tolerating more of his G-tube feeds, and eating a small amount of food. He is now on all oral pain medications, and his mobility is a lot better. He played several rounds of Foosball and even went to PT.

They did an X-ray just to check on some shoulder pain he had, but it's just some air left in his abdomen from surgery. His lungs look pretty good.

Azer has had some burning in his stomach and reflux that has gotten into his throat. The GI doctor prescribed a new medication that should coat his esophagus so it doesn't burn, and that it may take time for the Nissen to completely prevent reflux. We all felt surprised that he is having reflux so soon after this surgery. 

October 17, 2020

Doing Better

Azer is doing ok today. He is very sore, especially where the incisions are. They are keeping his G-tube venting to relieve some pain in his stomach. They are trying to switch him over to oral pain medications from IV medication, but his pain levels are still high. He isn't using the vest yet during breathing treatments, but he was able to walk around a lot more than yesterday. They are restarting his Hypertonic saline. 

He had to wait a while to try out some soft foods. They had to clear it with the speech therapist first before he could have anything beyond ice chips. He isn't very hungry, even though a lot of things mentally sound good. His G-tube feeds seem to be causing pain, but he was actually able to eat some mac and cheese this morning. 

Overall, he's doing a lot better than yesterday, even though he's still in quite a bit of pain. 

October 15, 2020

Surgery Done

The ENT doctor finished his part quickly. He injected some gel in the area where the cleft is to temporarily swell it up. This gel will get absorbed in a few months. If the problem returns, Azer may need further surgery.

The Nissen surgery went well. He has 5 incisions but the surgeon didn't need to move his G-tube, which was Azer's biggest fear. He won't be able to eat solids for a while, but he can start on liquids and soft foods tomorrow. 

He is in recovery and doing well. 

Swallow Study and Surgery

Azer had fun with trick shots yesterday.

So the swallow study results came back. It showed that he isn't aspirating when he swallows, but an ENT doctor will be doing a temporary repair on it during the same surgery as the Nissen. If Azer starts to go downhill again, the ENT doctor can perform a more permanent repair. 

Azer was put NPO last night tentatively for surgery today. It looks like it will happen for sure very soon this morning. 

October 13, 2020

Surgery Not Done

So, in one of my previous posts, I said that the surgeon explained he would do a bronchoscopy before surgery to check for any birth defects. It turns out Azer does have a small birth defect, called an LCET, a Laryngeal cleft, that could cause what he is swallowing to go down into his lungs. He said the type he had was Type 1. The surgeon consulted an ENT doctor to confirm visually this is what he was seeing. The surgeon is holding off on doing the Nissen for now, as he isn't completely sure if Azer needs it.

Azer is waking up from the bronch now. The plan is to do a swallow study to see if the cleft is causing him to aspirate. If not, the surgeon do the Nissen. 

Here is a link explaining what Azer has.

Surgery Happening This Morning

It was very unpredictable when Azer's surgery would happen, as the surgeon also does emergency surgeries, so it wasn't even known what day they would be able to fit him in. They were really trying to push the surgery earlier than later because his lungs got bad so quickly. Luckily, the IV antibiotics worked fast, and his PFT yesterday looked pretty good, and the CF doctor felt confident that Azer's lungs were healthy enough for surgery. 

Azer is glad he has a PICC line in for this surgery, as last surgery he only had IVs, which can fail often. Unfortunately, the PICC team told him that his vein could only handle 2-3 more PICCS before he would need a port. Hopefully this surgery is the key to keeping him healthy for the rest of the year! 

October 10, 2020

Admission - Again

So originally, Azer's surgery was scheduled for the end of October. He had a telemed visit with his pulmonologist. Azer has been doing an inhaled antibiotic which still hasn't helped the chills, increased cough or the fatigue. She opted to push the surgery date to next week, and to admit today. She wanted to admit Azer several days before surgery to start IV antibiotics, to get his lungs as healthy as possible. He will likely have trouble coughing after surgery. 

It feels strange to be back in the hospital so soon after last time. Azer had his third Covid-19 test today. There isn't an exact date or time for surgery yet. The main worry Azer has is about the possibility of the surgeon moving his G-tube to a different location. Azer remembers that the surgery for his G-tube was pretty painful and he doesn't want to experience that again. 

October 1, 2020

Met With Surgeon

Today we met with the surgeon regarding Azer's reflux surgery, called a Nissen Fundoplication. Since medication has failed to control his reflux and aspiration, surgery is the only other option. The surgeon answered a lot of the questions we had about the operation, recovery and life after.  He said he will be doing a bronchoscopy at the time of the surgery to check for any rare birth defects in the esophagus. Apparently, there are two rare defects can happen, one around the voice box area, and another between the bronchial tubes and the esophagus, that could allow reflux to enter the lungs. There is a possibility of Azer getting a new G-tube stoma if the surgeon has a hard time operating through the existing hole. Azer may have a hard time eating and drinking for a few weeks after the surgery, but he will be able to get plenty of nourishment through his G-tube while he is recovering. He might have to be admitted before the surgery if the CF team feels like he needs IV antibiotics for his lungs. 

It's been just over a week since Azer got off IV antibiotics. He already has been coughing up darker mucus, has been experiencing fatigue and chills. He messaged the clinic who wants to start him on inhaled antibiotics to hold him over until the surgery. They said if the antibiotic are helping him feel better, he can stop taking the antifungal, since it's obvious that it isn't helping his lungs at this point. 

September 25, 2020

Kluber's Kid

Last njght, Azer got an awesome planned surprise. The Texas Rangers pitcher Corey Kluber has a foundation called the Kluber's family foundation. They made our family into DoppleRangers, brought us dinner from a restaurant of our choice, brought all kinds of Rangers goodies and even showed us a video recorded especially for Azer from Corey Kluber and his wife!