*Breathe*

Brett's vest arrived this week! Click here to read my original post describing this device. It helps with airway clearance, which must be performed 2-3 times a day, every day, on someone who has CF. Until now, we've performed manual "Chest PT" several times every day. The way it works is that the hoses you see connect the jacket to the main machine, which sends air through to inflate the jacket. The jacket then vibrates and compresses at a high frequency and at various speeds and intervals to loosen the thick mucus from the airways.

Today was the first day we used the vest twice. He doesn't seem to mind it - and this afternoon he was relaxed enough to fall asleep on his daddy's lap while he got his airway clearance. I'm thinking of continuing the manual airway clearance at night because he usually goes to sleep and I put him straight in the bed. This vest will be a nice break from manual therapy every single time. It will also be nice to be able to count on the consistency of the quality of airway clearance when other people are responsible for the treatment. Also, when Brett is older, it will be very important to him because it means that he can be independent from us and still be able to get his treatments. It means he will be able to live away from home on his own and take care of himself without having to depend on another person to give him therapy.

Although it doesn't reduce the amount of time we spend on it (still 30 minutes for each session), it shakes all the lobes of the lungs at once for 30 minutes, versus us spending just 3-5 on each single lobe during manual PT. Therefore, it's very efficient and it's been proven to reduce hospitalizations for those who use it regularly. This device is going to ease the rigors of Brett's care quite a bit, and make our lives easier. We are very happy about that! Tomorrow he gets his first IVIG infusion.

*Breathe*

It's been a challenging few weeks! This past week was a lot better, but it was still a challenge as Mark started his first week of school and our new nanny, Erica, began her first 40 hour week. She worked quite a bit over the summer and she's doing great!

Here is Brett's most recent PICC line in his arm. For his daytime infusions, we'd hook up the syringe and pump and put it in his backpack so he could keep playing while he got the treatment. It worked out pretty well.

Last Sunday (August 15), I e-mailed Brett's doctor to ask if we could stop the IV antibiotics. It was getting really difficult for us not to get as much sleep since you have to stay up real late and now we have to get up at 6 or 6:30 all the time. He said that would be fine if I thought Brett was not coughing as much and he was "back to baseline". It had been 17 days of antibiotics already. I thought so, so we stopped. Since he still had the PICC line, we still had to flush it everyday, though, and the next Tuesday night I couldn't get it to flush. I called that night to let the doctor on call know, and we went to clinic the next day (last Wednesday, Aug 18). The good thing was that Brett's doc got to actually see him and agree with me that he seemed well enough to come off the antibiotics. Since we came to that course of action, they just removed the PICC there in clinic instead of trying to get it to work again.

This is the wrap we put over the PICC line for bathtime. 

The bad thing for us is that now that Brett has had pseudomonas, he will be treated as if he always has psudeomonas growing in his lungs. For right now that means going back on TOBI for the rest of the month. (TOBI is the inhaled version of the antibiotic Tobramycin. When Brett gets IV antibiotics, it's usually a combination of Tobramycin and Cefepime). It's basically one month off, one month on. We are having an extremely hard time with it. I don't know what to do to make it better. It's the time commitment of one hour in the morning (20-30 minutes chest PT then 30 minutes TOBI neb right after) in addition to all the other things we have going on in the mornings, and the screaming all the day through the 30 minute inhaling. We can do the other neb (Pulmozyme) in the afternoon if we find time for that, but if we don't and we have to tack it to the night time routine, he's got to do the Pulmozyme neb for 15 minutes, chest PT for 20-30 minutes, then the TOBI neb for 30. This also falls during the time we are trying to get the other 2 kids to bed as well. We don't really have any options as far as timing goes because the TOBI doses should be 12 hours apart, ideally. He's hardly even awake 12 hours, truthfully! I'm told that 10 hours is the very least the doses should be spread apart.

Everything seemed fine until this weekend, when Saturday Brett seemed just a tiny bit under the weather. By Sunday (yesterday, August 22), he was sleepy and lethargic all day long. We do have an appointment tomorrow, but based on the way he felt I thought it was not wise to wait that long to see the doctor. Again I called the on-call doc and she made us an appointment for this morning around 10:30. Today he's actually acting a bit better. He's so complicated! The good thing is that they all realize how complicated and tricky Brett is too! I'll update when I can!

*Breathe*

Oh, TOBI. We now have a love/hate relationship! I love it that it appears to be working! I hate it that it takes so long and we have to do it twice a day. Also, the ideal time between doses is 12 hours. Therefore, we have to do it as soon as he gets up and right before he goes to bed. The TOBI is an inhaled antibiotic designed to work against pseudomonas. Using the compressor we have, he has to breathe the medicine for 30 minutes. The TOBI must be done after airway clearance (chest PT), so we're pretty much making him sit in the chair for an hour morning and night.This is in addition to Albuterol in a metered-dose inhaler, once-a-day Pulmozyme treatment which lasts 15 minutes, and Flovent also in a MDI.  Sometimes it takes 15 minutes just to prepare the medicines, not to mention all the washing and sanitizing of the equipment and syringes.  At 15 months old, and he's got his parents sticking stuff on his face all the time. He'd rather be doing something else. If not for the g-tube, he'd have us forcing all his oral meds down, too. To some others who have CF or other CF families, this regiment might seem fairly "easy." But for us it's quite an adjustment.

We took Brett in to clinic Friday afternoon because he developed a fever after his shots, which is normal, but his breathing rate was 80-90 times a minute and his sats started getting pretty low (87-89). After an Albuterol treatment and 20 minutes of airway clearance, the sats improved to 92-94. He still felt fine during all this - we were really just being cautious. Brett is tricky, and you never really know what is going on with his body. It's like playing detective. He got a chest x-ray which looked improved from 2 weeks ago! That was reassuring that the TOBI and Cipro appear to be effective working against the psuedomonas. Hopefully that is really the case! He also had a nasal swab for viruses and a throat culture. These are easy tests that are pretty much routine and can give us good information at times. Otherwise, we're just hanging out, doing the meds for 3 weeks, then off for 3 weeks, then go back for another bronchoscopy. Here are the meds he's on now.

Enzymes - 18 capsules/day

Prisolec - 2x/day

MCT oil - 2x/day

Vitamins - 1x/day

Probiotics - 1x/day

Actigall - 2x/day

Baclofen - 3x/day

Zinc - 1x/day

Cipro (oral antibiotic) 2x/day

Potatoes in g-tube 3x/day

Chest PT 2-3x/day (20-30 min each time)

Pulmozyme 1x/day (15 min)

Albuterol 2-3x/day

Flovent 2x/day

TOBI 2x day (30 min each time)

Table Salt

20 hour Tube feed - still wears the backpack most of the day

Total Time spent managing CF:  4 hours

*Breathe*

I feel like I need to breathe! It's like my tennis instructor gets on to me for holding my breath during the drills. He yells, "Breathe! You're not breathing!" We've had a really crazy two weeks.

Grandpa playing Star Wars with Mark and Gantt

Brett went into the hospital 2 weeks ago, then got out, then scheduled to go back the next Monday (7/12), then postponed a day (7/13), then having the bronch but not having to stay after all, then going back the next day (7/14) for another appointment. That meant packing, unpacking, packing, unpacking, etc. The next day (7/15), Rob had an eye surgery. It was to revise his Lasik he had done last February. Thankfully, in addition to my helpers at home, Grandpa came up for that to take Rob there and back and to help me take care of the kids. 

North Carolina Children's Hospital

We spent most of the day Tuesday in Chapel Hill preparing for, doing, and recovering from the bronch. Wednesday we had a 9:30 AM appointment with Brett's gastroenterologist, and that lasted about 2 hours. We made just a few adjustments to his GI meds and did some tests to find out a little more information about what is going on with his nutrition (malnutrition/malabsorption) and see what kind of bacteria might be present. She decided that the pH probe and night in the hospital would be better postponed until he completes his treatment for the pseudomonas and staph in his lungs. His lungs are growing 2 types of pseudomonas, the smooth and the mucoid type, as well as a staph. The staph IS susceptible to antibiotics.

While we were there, we also got to see his pulmonologist, nutritionist/dietician, care coordinator, and even ran into the CF social worker. And of course the wonderful nurses in the clinic. While we were there, we got to see UNC's speech pathologist, who is also a leader in the field of feeding therapy. She squeezed us in her schedule, so we had an hour and a half to kill, but I thought it was worth it for her to take a look at Brett. Brett and I had lunch together then walked around the campus just to get some sunshine. The speech pathologist was impressed with how challenging Brett is to feed, and suggested an official 2 hour feeding evaluation, so we scheduled that for August 6th.

On the way back I got to stop in and visit with the great folks at MidCarolina Homecare Specialists, who even gave me a demonstration on how to use the new feeding bags I'll be getting. They provide the best service and they're always on call, 24/7, for questions or emergencies regarding equipment. Thanks, guys!

With the additional medications he is now taking and the additional inhaled meds, it has been a struggle to figure out the best way to do it. We are having a difficult time adjusting to the new routine and getting everything else done that needs to get done in our family life. I'll probably explain more next week. But the plan for now is that he will take this TOBI, which is an inhaled form of Tobramycin, for 3 weeks then have 3 weeks off, then go back in for another bronchoscopy to check to see if the pseudomonas has been eradicated.

*Breathe*

Brett is enjoying his newly-earned mobility! He's been working hard in physical therapy every week learning to strengthen those muscles and use them to lift himself and get around. It's quite a challenge, though with him still being on a 20-hour tube feed. He has to "carry his own load" by wearing his little backpack (thanks, Annie!) because the IV pole would just fall down if he pulled that around.

We all marvel at his "flexibility." Mostly it's because his muscles are weak. Here is Mark trying to figure out how Brett's legs fold that way!

Originally, Brett was going in only to get the pH probe test done.We have new information from the throat cultures taken last Tuesday - the cultures grew a couple of types of bacteria, so our plans have changed, and he needs to have a bronchoscopy done. This is when they go down his trachea with a camera and look at the state of the insides of his lungs. They also wash a few areas with saline and get a few cultures from inside the lungs. This will be Brett's 4th bronch. While he is under anesthesia they will go ahead and place the pH probe so he won't have to "bear" that while awake. We will stay one night at the least, but we will know more tomorrow.

We've increased the chest PT from 3 times a day to 4 times a day since we know there is bacteria in the lungs growing into an infection. This helps to actually physically knock the mucus loose from the lining of the lungs so he can cough it up. He usually doesn't, but it at least keeps the mucus moving around more so it has less of a chance to sit there and collect more bacteria in one place.

Here's our regimen this week.

Enzymes - 18 pills a day
Prisolec - 2x/day
MCT oil - 2x/day
Vitamins - 1x/day
Probiotics - 1x/day
Actigall - 2x/day
Baclofen - 3x/day
Augmentin (oral antibiotic) 3x/day
Potatoes in g-tube 3x/day

Chest PT 4x/day
Pulmozyme 1x/day
Albuterol 2-3x/day
Flovent 2x/day

Table Salt
20 hour Tube feed†

Some of these medications we have to mix and prepare. We are preparing 25 oral syringes per day to administer through his g-tube. I am so thankful for the g-tube because without it we would have to somehow get all these meds into his mouth every day. I hate to even think about that!

Total Time spent managing CF: About 3 hours

*Breathe*

"Breathe" is the title of my *new* weekly post for what is going on with Brett medically and his life with Cystic Fibrosis. I have decided to post something either about Brett's health or about something CF related every week.

It will usually be posted on Monday. If it's not on Monday, it will be on Tuesday that week. They'll be labeled so you'll be able to find them easily if you miss one or two. And as usual, if you have a question, please comment on the post and I will answer it!

Brett in the pool with Daddy

I was having a hard time knowing how much or how often to talk about Brett's health and CF, because it's important and you want to know, but I also didn't want it to be the focus of this blog, the same way it is not the focus of our family. Rob suggested I update you on Brett once a week, and if there isn't much to tell I'll do a feature on something CF related. That will give you a Monday update, a Wordless Wednesday pic, and another couple of family-related posts throughout the week. Hope you enjoy it - here goes.

Although I mentioned a few changes we made to Brett's regimen during his last doctor's appointment, I wanted to update you on his status in general. Since Brett is now free of his Broviac-Hickman catheter (or any other sort of central catheter), he can now have regular baths and go swimming in a pool. We are so relieved!! He does not like bathtime, but it sure is easier for us to clean his body. The pool, as you can see in the photo, he does like!

Brett getting his Pulmozyme treatment

Current Medications and Supplements

Here is a photo of all the medications and supplements that Brett requires right now. YES, it feels like our kitchen is a pharmacy. Especially with Mark's meds too. Mark actually uses the exact same medication with the exact same inhaler and same size mask- so it's important to be organized and keep everything separate so that Brett doesn't get any of Mark's lung bacteria. And YES, the folks at Wake Forest Drug love us as much as we love them! We feel as though Scott and Debbie are a crucial part of Brett's care team. And since last April, they've been getting a LOT more business from us LOL!

Below is the laminated schedule I keep on the front of the refrigerator to keep us all on track with what he needs throughout the day. Whoever administers the meds or the treatments just marks it off with a dry-erase pen. At the end of the day I wipe it clean and start all over the next day. Whenever his regimen changes, I just change the document and print out/laminate a new one. This is also helpful to take whenever he is admitted to the hospital. The nurses are very good about keeping to it so that he doesn't get off schedule during his stay.

Daily Medication Regimen

In addition to this daily routine, Brett has a physical therapist come to the house once a week, a feeding therapist once a week, and a developmental therapist either once a week or every other week. That's 2-3 hour long appointments each week. Whenever he has a central line, the nurse also comes out once a week. Thank goodness they come to the house and I don't have to get him ready and go out. He gets these therapies from companies who work with the Raleigh CDSA. He's been getting physical therapy since first got home from the hospital in August 2009. He has come a long way - he's actually crawling now! More on that next week.

Current Meds:

Creon (digestive enzyme)

Albuterol (bronchidialator)

Flovent (inhaled steroid)

Baclofen (muscle relaxer)

Pumozyme (mucus thinner)

Ursodiol (helps control bilirubin level & liver enzymes)

Prilosec (prevents reflux & helps enzymes work better)

Current Dietary Supplements:

CF Vitamin Complex (A, D, E & K)

Jarro-Dophilus powder (probiotic)

Instant Mashed Potatoes (Stool Thickener)

Zinc Sulfate (to increase enzyme performance, for growth & fighting off infections)

MCT Oil (Fat, fat, fat!!!)

Table Salt (to make up for excessive salt loss)

Total hours spent daily managing CF:

about 3 hours

This is probably the least amount of hours it will ever be, so it's good right now! Relative to how much more intense the regimen has been at other times and what it could be in the future, it's not too bad. I choose the schedule for the medications and the times aren't as strict as IV antibiotic times, so we don't have to break ourselves to stay on the schedule to the half hour and don't have to get up in the middle of the night several times to give meds. With that said, it is still of utmost importance that all this gets done and gets done throughout the day. We can't do it all in the morning and have it all done for the day, and we can't save it all until evening, either. And we *never* skip the treatments. CF never takes a day off, so we can't either!!