Discharged!

Azer came home last night! His lung function was holding steady from last week. His Tobramycin got delivered in time, and the clinic ordered a new nebulizer machine to hopefully help his treatment go a little faster. Adding Tobi to his morning and evening treatment will make his 30 minute treatments quite a bit longer. 

He got out just in time for his Ortho apparently. He should be getting is braces off by fall! 

Staying Through the Weekend

The inpatient doctors and the ID doctors were ready to send Azer home Thursday or Friday. The ID doctors had a few options other than to put in a port. They said they could send him home with the same oral antibiotic combined with inhaled Tobramycin, or with a different oral antibiotic, Minocycline. The problem with the Minocycline is it will take two weeks to find out of his type of staph is susceptible to it. 

Azer made the decision with his pulmonologist to stay through the weekend, and aim for discharge on Tuesday which will give him a full two weeks of IV treatment. He may need to stay a little bit longer past Tuesday, as they are working on insurance coverage of the Tobi. He would rather have the Tobi Podhaler instead, as it is much quicker, but it is unlikely to get the insurance to cover it. The regular inhaled Tobramycin takes 30-45 minutes via nebulizer, which he will have to do twice a day, but the added benefit is that he would be able to inhale it with a mask to target some of his sinuses where he is culturing abundent staph. 

The hospital staff and his pulmonologist noted that he seems much more like himself now that he's on the antidepressant. Azer has been enjoying Uno games with the nurses. Several will come in at a time and play. They have been keeping score on the whiteboard in the room through the two weeks, and the list is quite long now!

Update on Sick Visit

So today was mostly just a follow up for PFT's to see how his lungs are since he's been on steroids and antibiotics for a few days. His first few blows were exactly the same pre-albuterol last visit. They tried a different blowing technique which apparently works well for him when he's sick, and he blew just a bit lower than his post-albuterol test on Wednesday.

So his numbers look good, but numbers look good on paper, but don't always reflect the patient. Azer told the doctor that his lungs still do not feel good. He said they feel the same as they did before steroids. Usually, steroids make him feel a lot better quickly. The doctor did note that he's only been on this treatment for a few days, and it may take a little longer to start feeling the effects. She told him to have fun on our family vacation, and that if when he gets back, even if his PFT's are up to nearly normal, if he still feels like his lungs are sick, she can admit him to get his lungs back to normal before school starts. The doctor added that she's adding a test to his lung culture to test for non-TB mycrobacterium (we really hope it's not this, as it can take over 6 weeks for the culture to come back and the treatment is very intense and long. This bacteria grows very slowly, and treatment for it can take up to a year).

CF can be overwhelming and can really take over your day if you're not careful. Azer definitely felt those pressures when he was away for a week. Even at home, with not much else going on, it can be tough. I thought this picture really demonstrated him trying to fit everything in so he could go be a teenager. He was doing his inhaled Tobi podhaler and giving himself his growth hormone shot at the same time.

Sick Visit / CF Clinic

The Friday before Azer left with his youth group on a mission trip, he woke up that morning, and told me his tongue hurt and felt scratched up.

I was able to send pictures to the clinic that morning, and a few hours later they confirmed it was oral thrush, and sent the anti-fungal medication to the pharmacy.

Azer had a fantastic time and did as well as he could to keep up with his treatments during his mission trip. He told me mid-week that the schedule was not easy for a CF person to keep up with. He missed quite a few vest treatments due to how late at night they got in. By Thursday, he told me his lungs felt pretty sick, but he wanted to push through and finish the week. He didn't get enough time to do his Tobi or oral thrush medicine, but did most everything else and ate really well. He took the week off from his steroid nasal rinses, and ended up losing his sense of smell this week. He's hoping it comes back quickly now that he's back on it again.

When he got back home, Azer said his lungs felt pretty sick. When I listened to him, he didn't sound too bad, but had a few wheezes. Of course, perfect timing, his Vest ended up making horrible noises and needed to be replaced the day after he got home. Luckily, the Vest company was able to overnight us a new replacement machine!

Clinic: Azer had to get his annual Oral Glucose Tolerance Test today, so he was fasting since midnight. His height was the same as last time (he missed a week of growth hormones), and he lost about 3 pounds (not very concerning given how active he was last week. He was getting about 30,000 steps a day!) They drew his blood during vitals to test his fasting glucose level, which was normal.

Azer has been eating very well with the Periactin and as such has had changes to his stool and hasn't been absorbing his food as well as he could, so the dietitian came in to go over enzyme dosaging.

His PFT's were considerably lower than his baseline and lower than last month. The doctor listened to him and wanted him to do an albuterol treatment and then re-do PFT's and see if there was any difference. His FEV1 did go up about 7%. Azer told the doctor his cough never completely went away after the virus last month, but that his lungs felt much worse on his mission trip, but since he's gotten back home, they've gotten a little better.

The best course of action would be an admission to the hospital at this time, but we have already planned to be on vacation next week. The doctor felt like a quick admission for a PICC line would be pointless if he had to go back in to the hospital when we got back home. So, we are doing the next best thing: Hospital at Home: breathing treatments every 4 hours, antibiotics and steroids. The doctor warned Azer he needs to "get his butt in gear" (her actual words). We will redo PFT's before we leave, and if he hasn't improved when we get back, he will probably be spending a few weeks in the hospital on IV's.

The doctor told Azer to not feel like he didn't do a good job over his week on his own. She said the work he was doing combined with the weather and the environment he was in could be contributing factors to how his lungs are right now. They are running another virus panel, as well as a lung culture to see if he's growing anything other than MSSA. I will update as I find out the results.

Clinic Day

"Well, I never thought I would say this" said our GI doctor, "but we need to back off of the formula a little...." Azer weighed in at 108 lbs today. She even said he could stand to lose a few pounds without harm. And while his weight continues to go straight up on the chart, his height is still not climbing back to his genetic potential. In a few weeks, we will see an Endocrinologist to see if he has a growth problem, and a need for growth hormones. The GI doctor said that if that's the path we chose, it will mean daily shot injections at home. While there is no direct health benefit for him to grow taller, it does mean better quality of life.

His stoma was a bit irritated, so an Ostomy nurse looked at it. His G-button is still fitting well, but she said if he rotates it throughout the day, and use some gauze under it, it should heal fine.

His Fev1 was 84%. His chest X-ray from today looked cloudier than the last time. His cough has been more frequent and junkier, but as anyone around here right now knows, the allergens in the air are very high. The Pulmonologist joked, "sorry dude, you're just allergic to like, everything, outside". So, that doesn't help his lung function. We agreed to see what he is culturing, and if he isn't culturing anything that needs attention, in two months if his PFT's are still low, we will go back on Tobi for 6 month cycles, just in case there is still some Pseudomonas lerking somewhere in his lungs.

Also, May is CF Awareness month! The clinic put these up in the rooms, and encouraged us to take a picture to put on social media.

While we were waiting for X-rays before clinic, Azer noticed the mom of a blog I follow, whose son also has CF. They waved at each other from a distance, and smiled under their masks, lifted their shirts to show each other their G-tubes. Such a small gesture, but very impacting for Azer to see another that does many of the same things he does. He asked a lot of questions later on in the day about what the boy does that is the same as him, and what he has had to deal with that is different. I hope that someday, there doesn't have such a great distance between those who share the same burdens of this disease.

Vomiting

The antibiotic called Bactrim, that Azer has been on has been making him vomit, usually once a day. Last week, he vomited so hard, his nose started to bleed. Last night, we started the antibiotic Cipro. The Bactrim had to be given three times a day, the Cipro twice a day, so he ended up having to take them at the same time this morning. On our way to his baseball tournament, he threw up everything, formula, antacids, antibiotics... I knew this wasn't going to work.

So, I called the on-call doctor, and they are taking him off the Bactrim and Cipro, and putting him on Levaquin. The doctor said it treats all the of bacteria he is culturing including the Pseudomonas, which is awesome. I am glad he only needs to be on one oral antibiotic.

Azer did say that the inhaled Tobi is making him feel a little icky, like the beginning of a cold feeling. Hopefully that feeling won't last through the whole month.

So, basically, the Tobi Podhaler has these four capsules. He has to inhale 4 of them twice a day.

The little device in his hand pierces the capsule and then he has to inhale the powder (weird right) and hold it for 5 seconds. He says the urge to cough it out immediately makes it hard to hold his breath, but he manages.

He did great pitching today, and pitched into the 4th inning!

Pseudomonas

Well, the antibiotic Azer has been on has been causing some vomiting, but nothing too terrible. He initially said the Bactrim was helping his lungs, but a few days into it, he said his lungs felt tight. Unfortunately, the reason for this is that his culture came back, and it was positive for Pseudomonas. He hasn't cultured this since 2013.

Pseudomonas Aeruginosa (or PA), can be a very aggressive bacteria in CF. It can cause further lung damage and can be difficult to eradicate. PA is one of the reasons for boiling the nebulizer cups, as it can survive normal washing. They said he has 2 colonies of it right now. Apparently, we can think of the colonies of pseudomonas like little apartments of bacteria, each one living in a different complex. How they can figure that out from a sputum culture, I have no idea.

We will treat the Pseudo using the Tobi Podhaler again. This worked out very well last time. The normal treatment for pseudomonas is inhaled Tobramycin, or Tobi, which is an antibiotic which is inhaled. This is inhaled though the nebulizer and usually takes about 45 minutes. With the Tobi Podhaler, which only takes about 6 minutes to inhale the powdered antibiotic.

On top of the Tobi, he is going to have to do Cipro in addition to finishing his course of Bactrim. The PA has to be treated aggressively. I hope we can control or eradicate it as quickly as we did last time!

No Psuedo!

So, the sputum culture from our last clinic came back clear of Pseudomonas! Yay! I called the clinic last week to ask them if they wanted us to do another round of Tobi, but they said nope! They will test for Pseudomonas again when we have clinic in a few months. But, thank God we cleared it out!

Clinic Day

Well, it was a long day, but it turned out well. Early in the morning, Azer got the CT scan off his sinuses to check for nasal polyps. The machine he used was brand-new, so he was the very first person to try it out!

The CT scan revealed there are no visible polyps, and just typical CF mucus thickening. So, that's a relief!

He managed to gain 3 lbs in a month. The Tobi isn't supposed to make you hungrier, but oddly he has had a bit more of an appetite lately. His FEV1 was 92%, which is around his normal baseline. His lungs sounded clear, and overall, the doctor was quite pleased. We haven't received the results of his culture yet, but the doctor said that the colonies of pseudomonas aren't resistant to antibiotics, so there is hope for possible eradication of it.

I wanted to share a little information I was reading recently on another CF mom's page.

This article in the CFWA RED Magazine, summer edition, lists several studies done and have found the Vest to be much less effective than Positive Airway clearance methods.

Here is a segment of the article.

"Another study in Thorax by Osman et al 2010 compared the Vest to the usual airway clearance techniques (such as Flutter, PEP, active cycle of breathing technique, autogenic drainage) performed by the participants. This was a short term study, and looked at sputum production during the physio session and for 24 hours after using either the Vest, or the participant's usual technique. When participants used the Vest they produced less sputum in both the physio session and the w24 hours afterwards. The majority (55%) of participants also said they preferred their usual technique over the Vest. in fact, the only time the Vest has shown promise in CF studies is when it is compared to percussion and Postural Drainage - a technique that is now outdated and no longer used (Bradley 2010)

There may be a couple of reasons why the Vest is not as effective as other physiotherapy techniques for CF. One is that it provides no positive airways pressure, which helps to splint floppy airways open to allow sputum to travel from the small airways up into the larger airways, where it can be coughed up. If you have damaged, floppy airways, and they are not being splinted open by positive pressure during chest physio, they can close down and trap sputum deep in the lungs.
Positive airways Pressure also helps to get air behind trapped sputum, forcing it out of the small airways. Positive Airways Pressure techniques include PEP (including Bubble PEP), Flutter, and Acapella - but can be produced whenever you blow out against resistance (blowing out under water, blowing up balloons, blowing through pursed lips). The Vest may also not be as effective because it is a passive technique. Whenever you have to blow out against resistance, or use techniques like ACBT or autogenic drainage to control your breathing, you engage and strengthen your muscles of breathing - it's a workout for the muscles that help your lungs work! you can't get this with the Vest."

I have always felt that the Vest hasn't been as effective for Azer has Chest Percussion Therapy. While I am not considering eliminating the Vest from our treatments, I will be throwing in more CPT sessions along with using the Acapella. After a few days of using the Acapella in addition to his normal breathing treatment routine, I have heard a difference in his cough, and he says his lungs feel clearer. Just another weapon in the arsenal!

Tobi Podhaler

After waiting a few weeks to receive it, the Podhaler finally arrived.

It is quite odd sounding, I think, to inhale a powdered capsule, but that's what it is.

 It is not used with the nebulizer. It is inhaled with this plastic thingy.

 It took a few minutes to figure it out, with help from a video sent by our CF clinic, but it isn't too complicated.

Amazing that this little wonder just got approved by the FDA this year. It used to take about 45 minutes to inhale the traditional Tobi by nebulizer. Tack that onto a 30 minute Vest treatment and two other nebulized medications, do all this 2-3x a day and we don't have much "day" left to do other things. I'm grateful that we have this, since fall baseball is still in full swing, and we have tournaments coming up.

Cultured Pseudomonas

Well, the first time Az cultured pseudomonas was back in 2010. At his last CF clinic, they did their usual sputum culture, and on Wednesday the nurse called to tell me that he cultured pseudomonas again. Pseudomonas Aeruginosa (or PA), can be a very aggressive bacteria in CF. It can cause further lung damage and can be difficult to eradicate. PA is one of the reasons for boiling the nebulizer cups, as it can survive normal washing. They said he has 3 colonies of it right now. Apparently, we can think of the colonies of pseudomonas like little apartments of bacteria, each one living in a different complex. How they can figure that out from a sputum culture, I have no idea. What I do know about PA, is that it can form a bio-film to protect itself against antibiotics. The good news is, they have found his PA isn't resistant against any antibiotics at this time, so that is really good news!

The normal treatment for pseudomonas is inhaled Tobramycin, or Tobi, which is an antibiotic which is inhaled. This is inhaled though the nebulizer and usually takes about 45 minutes. What a drag, right? Well, the good news in all this is that he will be able to take his inhaled Tobi with the Tobi Podhaler, which only takes about 6 minutes to inhale the powdered antibiotic. The last time Azer did Tobi, he experienced ear ringing so bad it frightened him. With this method of taking it, it decreases the risk of ear damage.

On top of the Tobi, he is going to have to do Cipro, another antibiotic on top of the one he is already taking for the Staph in his sinuses. He has already been spending a lot of time in the bathroom because of the antibiotics, so needless to say, this isn't going to help that situation much. However, the PA has to be treated aggressively. I hope we can control or eradicate it as quickly as we did last time.

Continue with Tobi

Azer's culture came back clear of Psuedomonas, but his doctor wants Azer to continue with the Tobi. Azer's cough has been improving slowly. It has been very difficult waking up in the middle of the night to flush his tube, and to give enzymes at the end of the feed. Then, we have to wake up extra early because the Tobi takes FOR-EV-ER to finish.

So, to make the most of the day, we wake up early on the weekends too. We went to the lake last week end, swam in the lake, rode bikes, and had a picnic. It was a great escape from the "home hospital". Lately Azer looks a lot taller than his sister. Thank you Lord and G-tube.

Cough... Tobi Again

So, remember how I said that at clinic on Tuesday, Azer had a bit of a wet cough? Well, yesterday, it scared me a bit because it just sounded terrible. Really junky sounding. I asked the school nurse to listen to him, and she said it sounded like he had good air exchange. I called the doctor, and I thought maybe they would bring him in to look at him, but the he realized that Azer had just started the oral antibiotics on Tuesday, and that it might take more time for them to take effect. His culture still hasn't come back yet as of yesterday. His doctor wanted Azer to start back on the Tobi again. The last time we tried Tobi, Azer had ringing in his ears, but that may have been from the IV tobramycin.

So, we did a Tobi last night, and no problems. Azer said the Tobi makes his mouth taste bad. I just hate it because it takes about 30 minutes to run it, which adds more time onto the treatment time that seems to just run forever sometimes.

I just heard of a new nebulizer called the Trio that can run Tobi in 2 minutes. Maybe I can ask the doctor about it at next visit. It looks like we have to wake up at 5:30 in the morning to get Azer's treatment done in time for school. Fun, fun, fun.

Ring ... Ring...

So, yesterday morning was the second time we did the inhaled Tobi. The night before, we did his first dose of Tobi, which seemed to run fine, and we had no problems.

Well, at 5:00 the morning I go in and start his Albuterol and I run his vest. About halfway through his treatment, he says one of his ears is ringing. I think nothing of it, and start the Tobi after his Vest is done. A few minutes into the Tobi treatment, he starts crying and saying his left ear is ringing very loudly. I stop the treatment and email the Respiratory Therapist right away. The ringing apparently stopped, and we went on with our day, and I took the kids to school. A few hours later, the therapist calls and says the doctor wants us to stop using the Tobi immediately and to schedule a hearing test ASAP with the audiology department. We manage to get an appointment in the afternoon that day. We take Azer out of school and drive all the way to the hospital (I'm telling you, we need to start paying rent there, if we are going to be there once a week). They tested his inner ear, middle ear, and outer ear, and everything came back perfectly normal. There were no signs of any hearing damage.


So, today we are waiting to hear back from the doctor to find out if they want us to continue the Tobi, or if they want to put us on a different medication to control the Pseudomonas. There is a medication that just finished it's trial runs, and it's being approved by the FDA right now. Kind of cool that it's being approved right around the time when we might need it.

Well, I'll let ya'll know what happens!

A Welcome Surprise

As I was putting a belt on Azer this morning (his pants are always falling down, that dude has no butt whatsoever) I discovered that I couldn't tighten it to it's usual hole. In fact, the belt fit snugly about 3 belt holes down! And this was before eating breakfast, so there was no stomach bloating involved. I was amazed. I hope to see weight gain at his next CF appointment. :D

I moved Azer to the bottom bunk of the bunk bed yesterday in preparation for the Tobi. Since we can't run Tobi during his vest, treatments are going to be a bunch longer. This is difficult in the morning. So, we are trying to do treatments in the morning before he has to get up. I'm hoping he can fall back asleep while it runs. We're giving it a trial run and see if it works.



I hope I will get the Tobi and Scandi-shakes tomorrow. I'm apprehensive about the Tobi. The respiratory technichan told me he may appear to be getting a cough during Tobi treatments, but that this was normal. Other CF people online told me they feel "ill" in a sort of way when they are on Tobi. It seems to defeat the purpose, in a weird way I guess.

Azer had T-ball practice last Friday and Saturday. He did very well for being only a few days out of the hospital. The coach was impressed with the accuracy and distance of his throws, so Azer may be put on 1st base, or short stop. I heard some dads behind me making comments about his throwing skills when it was Azer's turn to throw a ball to the coach like, "Huh ... looks a short stop right there." Yeah ... I'm a boasting, proud mama.

Tweaking his treatments

So, because Azer cultured Pseudomonas, he will be on inhaled Tobi for the first time. He will be on it for 30 days, and off for 30 days. They do this to prevent the bacteria from developing a resistance. I was surprised that they wouldn't treat the MRSA unless it was causing symptoms.

I am a bit worried about the hearing loss risk, so if anyone has advice about that, I would really appreciate it. We are going to need to start waking up earlier to do treatments before school, because you're not supposed to do Tobi during the vest treatment. We want the medication to stay in his lungs, not get shaken out. I looked up the price of Tobi, and wow. It is even more expensive than the Pulmozyme (dornase alfa). CF kids are expensive!

Azer had a great day at school. Everyone gave him a hug when he got in his classroom.

In the hospital, Azer was drinking milkshakes everyday. I think that was partly due to the milkshakes he was drinking during every meal. So, we are doing the same thing at home. He doesn't really care for the milkshakes with Carnation Instant Breakfast mixed in. That stuff does taste like liquid vitamins, but I thought he wouldn't be able to taste it through the chocolate ice cream. Oh well. He is drinking a milkshake at school for a snack instead of slowly chewing through like 5 potato chips for an hour. Everyone at school is very supportive of everything. The school nurse is like a champ in getting him to drink his milkshake!

We all will continue to work hard .... phew...