Need advice from more experienced parents

[humphrey711]

Hi All,
My daughter has been doing really well up until a couple of months ago. Her PFTs started dropping. She just finished a course of Augmentin as she was culturing an abundance of staph. PFTs stayed the same. Had a CT scan which shows loss of elasticity and some scarring in the lower lobes. Is going in next week for a PICC and IV antibiotics and intense chest therapy. She is in the Orkambi trial and, thankfully, I believe it has helped her maintain a healthy weight. She was in hospital two years ago at this time and she looked very sick. Today she is pink, active, has a healthy BMI, o2 at 98%.

My fear is that she is losing her lung capacity rapidly and she is only 8. Doctor says some of this may be reversible with the IV and PT. So my question to those of you that have dealt with this in the past is what can we try at home to make sure she doesn't lose any more lung capacity? We already do the vest for 30 mins daily but I don't think that really reaches the lower lobes that well. She also sees her PT once a week. She plays hockey but she is on a beginner team so she isn't skating that much. She also does gymnastics about one hour a week. I am thinking we need to increase the physical activity. Are some activities better than others?

Any suggestions are really appreciated. She is my only child and I am so scared of losing her too soon.

 

[Ratatosk]

I guess I would look at increasing the number of times she vests a day. Maybe look at the settings -- do you have the hz and pressure varied throughout the treatment? I know most people do 2 vest treatments a day. We have always done 3 vest (30 minute) treatments a day. Before school, after school and before bed. If DS has developed a cold we've increased treatments. You could also look at doing chest physiotherapy (CPT) in the areas where she's having trouble, focus on those trouble spots.

What is she doing for nebs? DS does a bronchodilator (albuterol and atrovent) to open up the airways. He also does pulmozyme once a day. Some people neb hypertonic saline to bring up mucus as well, some neb mucomyst.

 

[Rebjane]

Sounds like you are doing things right. Has your daughter's CF doc ever suggested doing her VEST or CPT twice a day? We actually do my daughter's VEST 3 times a day. Before school, after school, before bed, but 20 min session. We started doing this when she was 2 1/2 because she was having alot of respiratory issues. We also taught our daughter how to use a flutter valve.

Another thing we've done is huff cough, pause the VEST and have your daughter try to cough something up(there are youtube videos to learn to huff cough). You could speak to the RT at your clinic to teach her as well.

Another treatment that I feel has helped my daughter is Hypertonic saline. Is your daughter on that? I am just brainstorming.

What nebs does your daughter do?

In 5th grade my daughter did cross country(this was wonderful for her lungs) She also likes to sing.

Hopefully the PICC line and CPT will knock the staph down and may even get rid of it?

 

[humphrey711]

She does albuterol to open things up and then does hypertonic to thin the mucus. After that it is vest for 30 minutes.

I am thinking she might need to do more cardio exercise.

 

[Rebjane]

Does she do Pulmozyme?

 

[humphrey711]

She doesn't do it on a regular basis but I think we will start. We do have some so I think we need to add it in. If you do Pulmozyme how long do you wait after it finishes in the neb to start the vest. I was told to wait an hour but that seems like a long time.

 

[Rebjane]

I would speak to the CF doc to double check with him/her before initiating a new treatment. The reasoning is to make sure you are all on the same page. Also, as long as my daughter is stable we will add in one thing at a time to see if it makes a difference. My daughter does her pulmozyme last treatment of the nite after her vest. This was her CF docs recommendation. The reasoning is it sits in there at nite and gets banged out of her in the morning with her morning treatment.

 

[emilimo1]

Not a parent but a 35 yr old CFer. My parents enrolled me in ballet classes twice a week starting at age 3 and this was extremely beneficial for my lung function and posture. I continued thru high school. Once I reached junior high I also became more serious about sports. Exercise has been key to maintaining my health (as well as treatment compliance)

 

[Printer]

I vest for an hour at a time WITH 7% Sodium Chloride nebs

 

[Gammaw]

You never mentioned where her PFT's are - what are her numbers? It would help give us perspective. I can't imagine not vesting at least twice a day. Twice a day is our normal. When my little guy is ill, we vest every 4 hours with nebulized xopenex (a modified version of albuterol). He used to use a metered dose inhaler for his xopenex, but on one occasion when he was sick, we found the nebulized version worked so much better for him and we've never quit. I keep an inhaler of it handy for when we're out and need it, but at home we use the nebulized version. It's "wet" and seems to get deeper and work better for him. You might ask your pulmonologist what they think of that idea.

I'm also a little confused when you say she had "staph". Staph aureus? MRSA? And they have only tried Augmentin? Was this from a throat swab? Or was she symptomatic, like with strep throat? And how long did they have her on Augmentin? CFers need longer than 10 days or even the 14 days usually prescribed by pediatricians. The pulmonologists usually go for longer than that in my own experience. And is she still culturing "staph" after her antibiotic treatment or is it gone but her PFT's have remained lower and her chest x-ray shows issues?

In addition, I'm a big proponent of exercise. My kiddo was doing pretty well all along, usually at an Fev1 of anywhere from 84% to low or mid 90s (except when he was sick). He too was in gymnastics and loved it, but it was only once or twice a week. It really helped build up his upper body which I think is extremely beneficial. But then he decided to join the basketball team over a year ago, and his coach made them practice twice a week for 2 hours each time - running up and down the court constantly the whole time with a few minutes out here and there for a drink. And then of course they had games at least once a week which kept him moving for another hour. That was 5 hours of serious exercise each week in addition to gymnastics. I discovered his PFT's started to climb, just a bit, but consistently. Then he wanted to swim constantly (well chlorinated pools only) and play soccer, and baseball, and play the trombone. I relaxed my only-one-outside-activity-after-school rule because I thought we were on to something. His last Fev1 (just yesterday) was 108%. He's never been that high before. And his small airways were at 100% as well. Eureka! Now, I do understand that Fev1 is not everything, although frankly it is extremely significant. And the chest xrays can sometimes tell a whole different story although I'm still exploring and trying to educate myself on that as well. But, as I said, increased exercise - not incredibly excessive which can cause the opposite effect - but regular strenuous exercise as tolerated - really seems to do wonderful things for CFers (as well I dare say as the rest of us). There are studies to that effect as well.

Some of that same benefit found in exercise is likely to be found in activities that are similar to huffing - anything to move air around the mucous and cut it loose. Like the suggestion for mastering the huff cough, which would be great. And frankly, playing a woodwind instrument like the trombone or trumpet. If you can add some "traditional" exercise - try another sport that she might enjoy - and do it often, several times a week if possible. Tennis? Basketball? Track and field? Running club? Gymnastics is much more strenuous for boys than girls, but if her routines will work up a sustained sweat, then maybe she can even just increase her gymnastics to several times a week, or join a gymnastics team. You will find committed weight lifters on this forum because of the perceived benefits of increasing upper body strength which increases lung capacity as well.

Do let us know what they find, how they treat it and what effect it has on her. Obviously, there is some kind of infection or issue that is causing the scarring and the drop in PFTs. So that needs treatment first. But I think the utility of exercise, huff cough or other techniques to clear her lungs is something that will give her a great long term benefit by clearing out that sticky mucous before bacteria can breed there and cause issues, keeping her lungs healthier, longer.

Blessings. . . .

 

[fourdeans]

Just in case you're tempted to think that YOU are doing something wrong, please hear this truth: you are not! We can always be puzzling and thinking and asking advice but please do not blame yourself!

My son's "wellness" routine is much like this:
Morning - 2 puffs albuterol, saline neb, 15-30 minutes vest, 2 puffs advair ( he also has several other meds he takes)

After School - 3x per week (minimum) physical activity of his choice (dog walking is mandatory and is not included which he prefers to do on his bike) right now he is in club Baseball x 2 per week, running club x1, and then we downhill ski as a family one or both weekend days.

Evenings - 2 puffs albuterol, Pulmozyme neb, 30 minutes of vest, 2 puffs advair

He also has significant asthma component which manifests during lung infections. When he is sick he uses a combi-neb of ipratropium bromide and albuterol, about every four hours. He also does his vest as close to every four hours as possible. When he is well, we go about life as usual, eating healthy whole foods based diet and living a healthy active lifestyle as a family.

In addition to his CF team prescribed meds, a naturopathic pulmonologist (and CF parent) has "prescribed" high dose/quality Fish Oil supplement, BCQ (bromelaine, curcumin and quercetin) supplement, and a higher than average Vitamin D. These are all known anti-inflammatories which are beneficial especially during lung exacerbation. Definitely not confused with a "cure" but we have noticed a difference in length and depth of illness, if that makes sense.

 

[ethan508]

Just in case you're tempted to think that YOU are doing something wrong, please hear this truth: you are not! ...please do not blame yourself!

Totally agree with this^. Sometimes despite our best efforts things will still go south. Not the most comforting thought I know, but sometimes we get so focused on beating the disease that we forget to also live and enjoy life along the way.

Second thought, some of your treatment routines seem excessively time consuming. Maybe I'm doing it wrong, but I've been doing my neb medicines (albuterol, hyper-sal, pulmozyme) while I do my vest. Now I'm aware that neb medicines shouldn't be mixed. But I've never heard that you can't/shouldn't do nebs concurrent with airway clearance. By doing nebs back to back (with a different neb cup for each med) while on the Vest your treatment time would be shorter for each session. By making each session shorter, you might find it easier to fit in an extra session when you child feels extra congested.

 

[kenna2]

I agree on the extra vest, it's never going to hurt to get an extra session in. Also, does she like to swim? I swam on a team for 8 years. It's great because it's a full body and low impact workout. It kept my lungs so strong and built them up, from having to hold my breath and what not. She is old enough to be on a team. It's a great way for her to make new friends and get those lungs healthy at the same time.

 

[Imogene]

fyi
We are hoping to work with a special set of caregivers for a project involving AI....more info to come...