What to expect next 3 years (3-6)

[Aboveallislove]

We've got our first 3 years under the belt and how I wish I had a blurb at the beginning saying what treatments would be like/doctors visits/what to expect/watch for, etc. So I'm hoping folks can give me some insights on the next 3 year (ages 3 -6). I don't want every possibility i.e., everything to worry about, but the basic things to watch for, discuss with doctors, changes in treatments. As backdrop, DS has had 1 hospitalization for DIOS and has major GI issues. He cultures staph and has since 10 days old and had h-influenza on and off for a year but none the last 9 months. No antibiotic courses (other than one when 1 month old with cough which was acid reflux in retrospect) and hasn't had any illnesses, i.e., no ear/sinus infections, no colds or flu. We do Xopenex (2x) Hyper-sal (2x), Pulmozyme (1x), vest 30 minutes 2x, zantac and prilosac, vitamins, miralax, and milk-of-magenisia (as needed). Cough base line is a few throughout the day random ones. Sometimes a few when we run or with treatments that sound more productive, but othertimes he'll get running or do treatments and no coughs at all. Has nighttime coughs on and off which we're virtually positive are acid reflux b/c usually when eats more at dinner and none as soon as he is upright. So these are my thoughts/questions. Your thoughts are greatly appreciated.

1) He has yet to cough out mucus. Sometimes he does have clearing sounding coughs but even then I can't see that anything has come in his mouth. At what point will he cough mucus out? How do I help teach him to do that?
2) I think that they are suppose to stop vest and do coughing, but when can they start that?
3) When can they start doing pft (terminology)? We did 3 when he was little in the incubator. What do they need to be able to do to do those in check-ups? Does it make sense to get a home one to help teach him to do that?
4) When do sinus issues typically pop up? Can they see those during office visits? What do I look for?
5) When do the typically start doing pills without putting in puree? How do you teach them to do that?
6) I know that pseudmonas is a likely culture by age 6, and that obviously treatments will differ depending on what bacteria cultures. So I know that is a possibility in next 3 years, as well as other maybe not as common bacteria. (That one I don't want to think about though! ;-).

Thanks!

 

[Aboveallislove]

Meant to also say DS is a ddf508.

 

[Ratatosk]

DS is 9 and baseline is still no cough. We do 3 vest treatments a day for 30 minutes. He's on albuterol/atrovent nebs as well as pulmozyme. Takes a couple oral antibiotics, actigall to keep his liver happy, vitamin K, A & E as well as a Source CF chewable. He's always had sinus issues. His nose never runs due to the extra thick sticky mucus. We did get him to start doing sinus rinses at age 4 to help clear the crud, which helped a lot. About a year or so ago he couldn't breathe at all out of his left nostril and we thought it was polyps. Was inflammation. We now use a nasal steroid spray -- no more snoring or mouth breathing. I DO have friends whose children have had sinus issues from day one and have had surgeries.

DS cultured Pseudo at 3 months and started tobi. He hasn't cultured it for a few years. At age 2 months and age 4 he developed junky coughs (bronchitis). Othewise he's gotten a few colds. Stomach bug last Fall. Also cultured H flu as a baby, but it went away when he started azithromycin at age 2 1/2. Lately he's been culturing steno maltophilia. He had DIOS and needed surgery at age 6. Was caused by adhesions from his orginal surgery as a baby which caused a narrowing in his intestines and blockage.

He doesn't cough up anything unless it's postnasal drip. We have been pausing to do huff coughing for the past few years.

He started doing PFTs at age 6; however, hasn't mastered it. Has been a dismal failure. But his lungs have been clear. Eventually he'll get it figured out. We were told to practice with party favors, etc.

I mentioned DS always had sinus issues, however, when it got so bad that he was snoring, losing sleep and breathing from his mouth and affecting his speech. We made an appointment with an ENT.

DS began swallowing pills at about age 3 or 4.

When DS cultured Pseudo he was put on tobi -- nebbed twice a day for 28 days, then off for 28 days.. When he developed a cough, bronchtis Cipro was added to the mix.

I have friends whose kids have never cultured psuedo. Some cough, some don't. Some have had sinus surgeries, some don't have issues at all. All are dd508, so there are differences amongst them despite the same mutation.

 

[DaraR]

My oldest is about to turn 3 and I appreciate your questions as I often find myself wondering when the other foot is going to drop. My son has zero base line cough, gains fairly well, and only does vest 2Xday, enzymes, vitamins, lactulose, and prevacid. I know things are going to start to get worse, but I don't know when. I just try to live it day by day and tackle the new things as they come.
I can answer your fifth question. My son started swallowing pills a few months ago. I used empty enzyme capsule to try it out. I just handed him one and handing him his drink. I put the pill in his mouth and told him to drink it down. He didn't even have time to think about it. We didn't talk about it first, we did nothing to make him think that this was strange. He swallowed it no problem. I was so excited I danced around like an idiot and chased him around beaming. He thought it was so cool that he got that type of reaction from me for doing nothing, that we really never went back to the purees. I do have to occassionally warn him not to chew them, but to swallow them whole. And, I do have to sit and watch him take them as he sometimes gets distracted and it is 5 pills. Otherwise, it was a breeze. Now, I don't have to make such a big deal when he swallows them, but he still gets double high fives!!
Good luck,
Dara

 

[Mommafirst]

Well my daughter is 6. I so wish I could tell you that your next three years will be like ours were. . . but there are so many variables. But I'll answer what I can:

1. My daughter still can't cough mucus up and has a zero cough baseline.
2. We stop and do huff coughing....when he is old enough to learn the huff cough and do it...maybe 5 or 6.
3. We started PFts when she turned 4.5. I don't think you need to do them at home. The first year was more to get practice vs. meaning much of anything.
4. They look in her sinuses at each visit, but we haven't had sinus issues yet.
5. My daughter could swallow pills at age 2. We got a McDonald's straw (they are wider for shakes) and I would put a pill in and put in a tall drink. Put your finger on top of straw and pick up straw so the fluid stays in, the pill floats to the top....then put the other end in their mouth. They will suck the drink out, you move your finger and drink and pill go right down their gullet. After a week of this, my daughter was able to pick up a pill and take a drink on her own.
6. So far no PA for us. I've read 80% culture it by age 16, so I'm not sure that age 6 is nearly that high.

 

[littlemisscoughsalot]

DD is 5. Diagnosed last year. She had a false negative and was missed by newborn screening. I can tell you 100% that the treatments that you all do are definately making a difference. Having missed the opportunity for proper treatment from birth to age 4, She is definately worse off then most 5 year olds i read about on here. She has moderate bronchiectasis, has had collapsed lungs in the past, cultures 4 strains of PA (2 developing resistance to tobra) and just about always has a productive cough.

One of the major changes I've seen over the past year is her self realization that she is different due to her CF. Her attitude has changed from "i don't like my treatments" to "it's not fair that I have to and no one else does" At first when we would go inpatient, she sort of enjoyed it... lots of time with mom, toys to play with, lots of nurses and doctors paying attention to her. Now she is begining to dread even going in for a clinic visit, especially if she has been coughing a lot.

She hasn't started feeling self consious about things yet. I am concerned for that later on in her life. I know how awkward the teenage years are for anyone.

I wish you the best with your little one. Hopeful for few issues and hospitalizations. Just keep doing what you are doing and i'm sure you and your DS will be alright.

 

[Aboveallislove]

Thanks all! A few follow-ups:
1) How do you teach "huffy?"
2) How do you know if there are sinus issues? Can they see that that is an issue starting to brew in regular CF check-ups?
3) Also, at what point do you start having them use the tube thingey as oppose to a mask? (I like the mask b/c it gets it into his sinus' more I'd think. But is there a benefit to the tube thingey?)
4) And when have you started letting them do treatments by themselves? I can leave DS for a couple minutes doing the treatment if I have to run to sign for a delivery or something. But just wondering when it is typical to be able to leave holding (with me near-by) and then later to start on his own?? (DS already can tell you most of his medicine names--although I'm likely the only one who could understand) and what he needs to do and has told me a couple times when I forgot to do Pulmozyme after moving to morning from PM and ditto on enzymes when they're on table and I forgot to do--and one time when DH did and then he had a loooonnng potty break and needed to give him more he was insistent, "no, daddy already gave me my enzymes.")
Thanks again and hugs and prayers to all you other moms.

 

[Ratatosk]

With sinus issues, ds always snored, sounded as if he was gasping for breath when he slept as a baby and his nose never ran. We always had to get after him with the dreaded booger sucker and his nasal mucus was really thick. The first time I saw his nose run was when he was 2 1/2 and started pulmozyme. I was amazed as I'd never ever seen his nose run before. At the clinic they always ask if he snores. They look up his nose. Have him hold one nostril and breathe or try to breathe out of the other one. A friend of mines daughter couldn't breathe at all thru her nose and was always sooo very tired during the day because of polyps blocking he nose.

We still use the mask for the most part. Hands free usage during xbox while vesting. He did start using the mouthpiece at about 3-4, but with tobi we had to use the nose plug at first or remind him to breathe in thru his mouth. We rarely leave him alone to do his vest. One of us keeps him company while he plays video games, plays on the ipad or watches tv. Sometimes I'm in the kitchen in the next room, but I check on him frequently. Being that he's tied to that darned vest for 2 hours each day, no sense in him feeling isolated, too.

 

[Rebjane]

My daughter was found to have a nasal polyp at her last visit(she's 9 1/2)..Her CF doc looks up her nose every time he sees her. I had noticed her voice sounded different; more nasaly, sometimes she would seem like she was coming down with a cold; but she felt fine. She was started on Nasonex and it's really hard to tell if it's helping; maybe slightly?

She still does all her nebs via face mask. When she does them with the mouthpiece; she chews on them; drives me crazy. She mostly does that cause she is always multitasking and needs her hands; thus must chew on the mouthpiece; whatever.

My daughter kind of does her nebs/VEst alone. We have another child or should I say teenager(yikes). We have a common area she does her"stuff". Someone is always drifting in/out of the room; I don't think feels alone at all. She reads ALOT, does homework watches TV eats, pretty much can do lots of things while nebbing and VESTing. However, this summer again she is 9 ; I taught her how to do all her treatments(nebs) and VEST. She does her own enzymes. Knows most her pills. I am confident if she HAD to; she could do it all herself. Now, she does not LIKE to do it all her herself but knowing she could gives her such confidence. Now that school started it's harder and I do the nebs and VEST but on the weekend she should do it herself(We have to work on it

Teaching the huff cough. I actually had looked it up on You tube, watched a video of how to huff cough then taught my daughter; can't remember what age; maybe 2 years ago? She started PFT's at 5 years old. We would have her blow bubbles; blow up balloons to practice plus it's good for their lungs.

My daughter is fairly mature for her age BUT she is still a kid. She NEEDS to have fun; we try to fit that in as much as possible.

 

[Aboveallislove]

Thanks. DS's nose never ran until about 5 months after he started hyper-sal. Now he blows it easily. He had snored alot as an infant but not in 2 years that I can remembe. thanks that is very very helpful.

And totally agree re leaving along--I actually love all the cuddle time! I was thinking more from the helping him "own his CF" and be responsible.