Asthma question

[Justinsmama]

Hi,

My son Justin (8) was diagnosed last year. We have had a relativly calm 6 months, other than a couple of respiratory viral infections that lasted about a month total. Starting last week, Justin developed a non-productive cough that gets worse with exercise. He would cough up to 30 times in 6 minutes. We put him back on Symbicort 160 and neb treatments every 4 hours last Thursday. He got a little better, but not completly. I took him to the ped's yesterday, and luckily he had a lot of cough when we were there (usually stops when we pull into the parking lot). His FVC was 108 and his FEV1 was 97 (both up from last visit with virus of high 80's) but his 25/75 numbers were only at 70 (up from the last visit with the virus of 60's). They gave him a neb treatment and then the 25/75 went up to 90 so the doctor (not our usual) said it is asthma. He is now on an oral steroid started yesterday. He is outside playing basketball and still coughing as much as before. This is the first time his asthma has ever been out of control other than when he has had pneumonia. We have only used the neb once or twice a year prior to last year when all of his problems started (after being severly PI and his BMI dropping below 3% and going on enzymes). My questions for those who have children with asthma too:

1. How long until the oral steroid works?
2. Is he at greater risk of an infection?
3. Are there other CF related things that could act like asthma?
4. should his cough go completely away?

Any experience with would be greatly appreciated.

 

[Aboveallislove]

our son is not yet 4 but they think there is an asthama component b/c he was coughing especially during exercise. too young now to do fev to confirm asthma, but as a baby when he did the infant tests the first showed a similar response and cf dr said likely an asthama component. sooo with that backdrop ie that we arent positive it is asthama...he seemed to respond same day to oral steroid, but instructions said it could take 1 - 2 weeks. re infections--he's never had any colds, flu, escerbations, etc., but cultures staph and sometime h-influenza, so not sure how the asthama affects lung helath in general. his cough did go almost completely away but he has horrible acid reflux and still had some coughs from that--im assuming from that b/c his bark cough was completely gone and almost completely gone for daytime cough and nighttime cough went away when we got him to sleep upright. good luck.

 

[Ratatosk]

DS doesn't have asthma; however, we do nebs (albuterol/atrovent) with every vest treatment to open up the airways. Been doing it since ds was first diagnosed as a newborn. We've never used oral steroids. His doctor was concerned with using them unless absolutely necessary as they have the potential to raise blood sugar.

When he was 4 or 5 he developed a dry cough that I thought was just a habit -- kind of a throat clearing cough, but a constant. Turned out he was culturing Steno. Maltophilia. Was put on an antibiotic -- septra and it went away. Have spoken with another parent whose child had similar issues, same thing.

 

[Justinsmama]

Hi Abovelallislove and Ratotosk,

Thank you both for responding. Ratatosk, could you tell me a little more about the Steno Malto? Did your son have more cough on exertion? I am starting to worry because Justin is still coughing 30 hours after the first dose of steroids. I hope and pray tomorrow is better but have a feeling that the cough will not go away. Did the infection affect his PFT's? The xopenex seems to bring his 25/75 back up to normal (90) BUT it does not stop the cough. Is this just "habit" cough or could there be an infection? The ped that we saw is very nice, but not like our "angel - Dr. Ryder" who was a CF doc and is AMAZING!!!! I see my little guy stretch his neck to get a breath after a cough and I want to cry. My "Mama feeling" is that there is something else going on. I hope I am wrong. Maybe just a normal case of asthma. . .

 

[Ratatosk]

His cough seemed like more of a nervous habit. This was before pfts, which he still really hasn't mastered. He didn't act sick, just kept clearing his throat. Have they run a culture on him? One time ds was on both keflex, tobi and zithromax and developed a terrible cough -- his baseline is no cough -- and turned out he needed a little extra help. So his doctor prescribed yet another antibiotic. If the doctors prescribe something that the bug isn't sensitive to or is resistant to, it's probably not going to help. Also, we never have had much luck with regular pediatricians and contact the CF clinic rather than the local ped department. I was once told "they cough, it's what they do" and it turned out ds had bronchitis. Other times I was told it "was just a virus and to let it run it's course"

 

[JustaCFmom]

Hi Abovelallislove and Ratotosk,

Thank you both for responding. Ratatosk, could you tell me a little more about the Steno Malto? Did your son have more cough on exertion? I am starting to worry because Justin is still coughing 30 hours after the first dose of steroids. I hope and pray tomorrow is better but have a feeling that the cough will not go away. Did the infection affect his PFT's? The xopenex seems to bring his 25/75 back up to normal (90) BUT it does not stop the cough. Is this just "habit" cough or could there be an infection? The ped that we saw is very nice, but not like our "angel - Dr. Ryder" who was a CF doc and is AMAZING!!!! I see my little guy stretch his neck to get a breath after a cough and I want to cry. My "Mama feeling" is that there is something else going on. I hope I am wrong. Maybe just a normal case of asthma. . .

Please find a CF center/doctor to check him out. They specialize in this complicated condition.

Be strong.

 

[Justinsmama]

Hi,

Thanks for all of the advice. We saw the CF clinic last Friday (new doctor). He was having bronchospasms. Apparently, the thick mucus is clogging his small airways along with inflamation. His 25/75 is the only thing that is low (still somewhat low on the 75 side after 1 week of oral steroids and still on higher dose of Symbicort). She thought it was time to add either Pulmizyme or 7% saline. I chose the saline to try first because it is not another drug. She also decided to keep Symbicort as a maintenance drug and not just when he is sick or flaired up. She explained that (not sure if it is for all CF kids or just some) but that small airway problems then lead to large airway problems. They tend to pull down the numbers. Our goal over the next 3 months is to try to thin the mucus in the small airways, get it out, and get rid of the inflamation. This should raise his 25/75 up. His numbers were running about 60% before the steroid (higher at the 25 and lower in the 75). His 75% was 55% and went up to 70% on the steroids. This should help with the bronchospasms.

As far as infection, the prelminary results only show staph. He has cultered this straight for quite a while. Very thankful that there is nothing else.

 

[CrisDopher]

I would not have a general peds be diagnosing or prescribing things for lung-related problems. I'd only have a CF pulmonologist do that.

Different steroids do different things in the long term. I certainly wouldn't wish for a kid to be on oral steroids at all, if it can be helped. Symbicort is a common long-term asthma treatment, with or without CF, (and I'm on it myself) so I think that's pretty safe. Just remember to always swish and gargle with water afterwards as you don't want that stuff hanging out in the mouth and throat (can lead to thrush).

Asthma is common in people w/ CF, though by no means universal. A CF pulmonologist should be able to differentiate between infections and asthma flareups and long-term degradation, which of course everybody is trying to prevent.

 

[Ratatosk]

I'm still not clear -- are you doing or have you increased chest physiotherapy (CPT) or vest treatments to help get that mucus out. I didn't see mention of that. Also saline I believe can cause bronchospasms. I believe one usually starts with a bronchodilator to open up the airways and it also helps with potential issues.

 

[Aboveallislove]

I'm glad the appointment went well and you are having some more guidance. Knowing what I know now, if it were me, this would be what I would push the doctor for "regular" daily morning treatments: 1) Bronchodilator, either Xopenex or Leverbuterol (generic) which are easier on the system than albuterol, 2) Hypertonic saline; 3) Pulmozyme, 4) Inhalled Steroid (QVar or similar) and 5) the 30 minutes of vest treatment. It is ideal to do vest after all, but you can do at same time and I wouldn't worry about doing at the same time. And then evening: 1) Xopenex/Leverbuterol; 2) Hypersal; 3) 30 minutes of vest. And then when sick/more of a cough, up the Xopenex/Vest to 3x-4x. Obviously you need to discuss with doctor. As Ratatosk says, you need to do a bronchodilator before the hyper-sal. zom

I understand the fear of using so many medications, but with CF we know what that means and the risk for these medicines definitely outweighs the risks for the medicines. My understanding is that hyper-sal does NOT thin the mucus, but it hydrates the air ways. Pulmozyme DOES thin the mucus though. In my view by 6 months all CFers should be on Pulmozyme and by 2 hyper-sal. Others may disagree, but my reasoning is simple: Studies show that there is lung damage long before it shows up with symptoms and that these medicines help delay lung damage. And there are new meds right around the corner that are so promising but can't fix the damage already done. Also, I know of one mom whose little one is on Kalydeco and it is such a change that his body no longer thinks it has CF, i.e., the sweat tests are "normal" now. The doctor is overseeing them decreasing daily treatments but the last to go and what they still do is pulmzyme because it actually thins the mucus and that is so important. Those are my thoughts, for what they are worth. Good luck.
Hugs and prayers,
Love

 

[Rebjane]

A component of CF is inflammation. It can act like reactive airway or asthma and or you can have asthma in addition to CF. My daughter with CF has a large inflammatory component to CF and she seems to have some reactive airway. When she gets an infection; the inflammation can brew increases mucus, irritation and exacerbate coughing as well as exacerbating her bacteria load.

We utilize alot of meds as others have listed; Albuterol; Hypersal, Pulmicort, and Pulmozyme.

They have been added over time. I do think it can be wise to add one treatment at a time so you know what your child's response is to that med and if it is actually working. If your child is doing poorly and you have to throw a bunch of meds in the mix; you do what you have to do.

Each CF kid is different. We have changed the way we do her nebs, as a learning process. ALWAYS we start with a bronchodilator: Albuterol. THen comes hypersal. We do her VEST, then Pulmicort (inhaled steroid) After her vest is done.

Her Pulmozyme we do last treatment of the day at night after her vest.

Hope that helps. We recently had to increase her Pulmicort dose since she has been having more inflammatory issues lately, with wheezing and tightness. Still her PFT's were good at the time, go figure.

 

[Aboveallislove]

Rebjane makes great points re slowly adding new treatments. . . I guess at the end of the day those are the meds I think it would be best to have kids on, but not necessarily doing a dump at once. And also great point re the pulmozyme. . . I'd heard morning is better b/c then day movement can clear out thinned mucus, but there are different views on that and in the end you have to do what works. . . we use to do pulmozyme end of day but it just worked better earlier. And everyone is different for what routine works best. Also, fyi: I'm not suggesting inhalled steroids for everyone, but if there is an asthama "twitchy" component, it is suppose to help with inflamation, so I don't think that is "standard of care" for everyone.

 

[Rebjane]

We have gotten differing views of Pulmozyme from different CF docs, so it can be confusing. My daughter started Pulmozyme when she was about 2 1/2 years old. We first had done it in the morning after her albuterol then immediately did her VEST. We did it like that for a while; then when she was 4 we added in Hypersal. We had done Albuterol the Hypersal then Pulmozyme then VEST.....She was having issues and I thought perhaps the Hypersal was inactivating the Pulmozyme; so we moved her Pulmozyme to the afternoon away from Hypersal. Finally her Cf doc suggested doing the pulmozyme to nite time after her VEST to let the Pulmozyme sit over nite and then bang it out in the am......That's how we ended up in that regime......Trial and error.... CF always keeps you on your toes.

 

[Justinsmama]

Thank you again and sorry for the confulsion. Yes, Justin has been on the vest for over a year now. The problem was that he could feel the mucus but not clear it. We are now doing the same schedule Aboveall mentioned, without the Pulmozyme and with Symbicort instead of QVar. We do it in that order too. We were doing it minus the hypotonic saline, but got the package two days ago. Rebhane, what you mentioned about the inflammation acting like asthma is what the CF doctor explained to us. If he does not show improvment by our next appointment, then I will ask to add the Pulmozyme. I did not know that it does not thin the mucus or I would not have gone with that option. He has only used it twice at home (we did not get the mask delivered yet) so it is hard to tell how well it will work expecially because he is not used to using a mouthpiece and I don't think he is getting the full treatment.

As far as allowing the general ped to make decisions, I was not clear on that either. Our CF doc knows of our ped (he is known throughout our state as an "asthma specialist" and worked in a CF clinic for years. He (and everone in the practice) calls the CF clinic before anything is done for Justin, even if it is not CF related. They did this when they put him on an antibiotic for Lymes. The CF clinic oked the oral steroid because of the spasms he was having in the office and the low 25/75 to get him to baseline without the inflammation.

Thank you again for your input. It is so helpful to know what has worked for others and what questions to ask when at the Clinic.

 

[Ratatosk]

You should ask one of the respiratory therapists or the pulmnologists to show him how to do huff coughing. Also, we started off using a nose plug with ds when he was able to use the mouthpiece for his tobi. He was about 4 and thought it was funny. A lot of times he still uses a mask so he can have his hands free to play video games during his vest/nebulizer treatments.

 

[Aboveallislove]

hyper-sal should help him clear since it induces coughes. good luck!