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Avatar universal

Tracheomalacia still at 10 years old

My son Hunter was also dx with Tracheomalacia as an infant. We too were told he would "outgrow it" by 2 or 3. He is 10 and still has problems. He outgrew his stridor by about 3. However, since 3 he has had RSV, Pneumonia 6xs, Bronchitis 6xs, Bronchilitis and Croup 14xs. Remember he is only 10. So in 7 years he had all that.

He had his tonsils and adnoids removed in the spring in an effort to stop the Croup. Ten days later he had Croup. He had Croup this September for 4 1/2 weeks, it just wouldn't go away. Then he had a sinus infection (so they say) then Bronchitis again. During his bronchoscope they too said he had Innominate Artery Compression of 10-15%. He failed his PFT really bad. I think the numbers were 115 input and 72 out put. The doctor said there is an obstruction and we will operator in one of ofur ways after we get results from MRI --However, his follow-up MRI said there was no compression, no vascular rings nor any foriegn bodies. So the doctors say it's just Asthma now. So confused.

I feel crazy at times. I wish I knew how to keep him healthy. He has been tested for allergies, CF and his immune system all negative. He is on Advair and Xoepnex PRN. Two or Three times a year he is on steriods. I just want the best for him. He sees his primary all the time and his pulomary every three months. I want better answers for him. Do you have any suggestions?? Anyone??




This discussion is related to still having tracheomalacia "problems" at 23 months old.
23 Responses
90270 tn?1199338069
I feel terrible for the both of you...when they tested for CF, what did they do? I am just curious as I was diagnosed as an atypical CF'er (along with my other constellation of problems) in my 30's. I also had really bad "asthma" and frequent infections.
If they just did the sweat test, that really isn't enough. They need to do the full sequencing of the gene (there are 1500+ mutations now) not just the most common 28 or 40 something mutations.
If they already did all of that, I don't know what else to add except that I pray that you both get answers soon. This must be so nervewracking for you guys.
Prayers,
Sunny
Avatar universal
Thank you. Hunter had the sweat test only. I thought that was all there was. I will discuss this with his pulomary specialist soon. He also had his immune system tested. Is that what this test is?
--Colleen
mother to Dominick (12), Hunter (Tracheomalcia, Asthma and Failure to Thrive 10) and Jennifer (9)
90270 tn?1199338069
No, the immunity tests are different from the CF one. I can tell you that my sweat test was borderline at 48 and 50, but I do know of CF'ers who have sweat tests even lower than that who were diagnosed via the gene sequencing. Do you know what his sweat test level was? Some docs simply say negative (less than 60), but there are numbers. Less than 40 is considered negative, 40 to 60 is borderline and above 60 is positive. Mind you, most people with CF have a positive sweat test but there is a small percentage who have negative readings and have it.
For completeness sake, it wouldn't hurt to get the full testing through Ambry genetics.
Good luck,
Sunny
Avatar universal
Thank you for your information. What made you get tested for CF?? I guess what I am asking is what was your health history like?? Did you have a lot of Upper Respiratory Infections (URI)?? I have always wondered how he compares to a person with CF. I've also heard that people without CF can benefit from CF meds? What do you think??

I am on another forum/support group for Tracheomalcia it helps a lot.

Thank you
Colleen
mother to Dominick (12),
Hunter (Tracheomalcia, Asthma and Failure to Thrive 10)
and Jennifer (9)
Avatar universal
Hunter is home from school today. He threw up on th bus from all his coughing. The doctor said he has some kind of throat infection. But his strep test came back negative as it always does.

He was having breathing issues on Sunday, Monday and a bit on Tuesday but today he looks better. Go figure.

On Monday morning when he woke he looked like someone threw a ball at his face. He was a bit swollen and he had dark circles under both eyes. I have seen it before with him. We try never to keep him up late. He really needs his sleep.

Anyone else get like that??

Just trying to find answers,
Colleen
mother to Dominick (12),
Hunter (Tracheomalcia, Asthma and Failure to Thrive 10)
and Jennifer (9)
90270 tn?1199338069
Poor little man....All I can say is that maybe he has allergies to boot on top of all of his issues. Allergy testing wouldn't hurt if they haven't been done either to see what might be the culprit to his puffiness and black circles.
My doc tested me for CF after all of my respiratory problems but mostly when I started to lose a tremendous amount of weight quickly. I was overweight at one time, not now...I am considered pancreatic insufficient and need to take meds with each meal and snack to absorb my nutrients.
What also sent some red flags flying was the types of bacteria that were growing in my lungs. My cultures come back with some very strange bacteria, usually seen in those with CF, not the normal population. I grow out P. Aeruginosa (pseudomonas), staph and B. cepacia (that one really threw my docs off).
I have been on CF treatment for about 5 years now and require IV antibiotics to help with the flares that I get with success. I too take nebulizers, Xopenex like your son but also nebs that help break up my mucous called mucolytics. Pulmozyme and hypertonic saline both do a wonderful job thinning out my secretions so that they don't sit there in my lungs. I take antibiotics by mouth as well on a regular basis, mostly for inflammation so they say and vitamins to replete those that are lost with my digestive problems. I also use a device called the Vest, which is chest physiotherapy with high frequency. That has helped as well. I can tell you that when I first started with treatment, I was in rough shape and in the hospital every few months with whopping infections. These days, I am much, much more stable thanks to the meds that I take daily as well as my physio. I also keep as active as I can. I also have developed diabetes but it is manageable, I credit my primary doc for being as aggressive as she was to get me tested and treated for CF. My specialists at the time blew off CF as a diagnosis and said that I could not possibly have it as I was in my 30's. Boy were they wrong.
As for your son, it wouldn't hurt to take him to a pulmonologist who is very familiar with CF at a CF accredited center (look on CFF.org for a site near you). Sometimes another set of eyes and ears doesn't hurt.
I hope this has helped you:)
Sunny..
Avatar universal
Not sure if my last email posted or not.

I followed throught with Hunter's primary for the test results for the sweat test. They were not sent to his primary doctor. In frustration I wrote to the dr, hospital and his pulmonary to get the results. Today his primary called me. Said it needed to be re-done asap.

His one arm sweat test was 40 the other arm never showed any sweat. 41 and over is the # for a positive result for CF.

Hunter is scheduled to go on Monday at 10:15am. Prayers will help. I thank you for sending this email without your email I wouldn't have known any of this.

Thank you so much
Colleen
mother to Dominick (12) Hunter (TM 10) and Jennifer (9)
90270 tn?1199338069
I will definitely keep Hunter in my thoughts and prayers. Sweat tests in the 40-s to 60-s range definitely warrants a better look into the CF possibility. When you go, ask for the full panel for genetic testing...not just 80 or so mutations that some tests offer. There are 1500 plus mutations out there, the full Ambry or Quest panel will check for all of them. Those results take at least 3 weeks, up to 4 or so even.
Please let me know how things go...
Sunny
90270 tn?1199338069
One more thing, his primary can even order the genetic testing, mine did to get the ball rolling...Just have him or her contact Ambry or Quest (depending on what type of insurance you have).  If you can go with Ambry, please do it.....they will send a kit to the doctor with all of the appropriate information.
I am very glad that his sweat tests are being repeated...is this at a CF accredited center? They have strict protocols regarding sweat testing ,they also do it frequently so less chance of errors in the results.
Again...keeping you guys in my prayers. If you have any questions, just holler.
Sunny
Avatar universal
We are going to Children's National Medical Center in DC for the Sweat Test today. I am not sure if they are an accredited center or not. How do I find out?

Not sure if I am understanding this correct; The genetic test; is that done at the primary doctor's office once they receive a kit? Or do we go to a Lab Center? What is Ambry or Quest? So do I just contact the primary and they order the kit, he does it and sends it out?

Plus you are saying that the test results can go past 41 without them saying it is CF. From my understanding anything above 41 was CF anything below 40 was negative. Hunter had a 40, but it was one arm.

Thank you so much
Colleen
mother to Dominick (12) Hunter (TM 10) and Jennifer (9)


90270 tn?1199338069
Hi Colleen.
Ambry and Quest are laboratories that perform the extended genetic test for CF. If your son's pedi decides to run the test before he sees a CF center, he can either send the specimen to Quest labs or Ambry depending on your insurance. If he choses Ambry, they have a kit that can be ordered. Go to the Ambry website, you will be able to see the tests they can run. They have an 800 number you can call if you have any questions, they are very helpful. Give this info to his pedi or tell him to look into it.
As far as the sweat tests go, they tend to run like this. If it is below 40, it is considered negative. If it is between 40 and 60, then it is borderline, if it is above 60, it is positive.
Like I said, usually CF'ers are positive, or above 60, but there are some that run in the borderline range (like myself) or even negative. That is why genetic testing is so important for those who are in these gray areas.
Children's national medical is an accredited CF center (I looked on the CFF.org site, here is the link: http://www.cff.org/LivingWithCF/CareCenterNetwork/CFFoundation-accreditedCareCenters/index.cfm?state=DC) so that is good as far as the sweat testing goes. You should get the sweat test results quickly, usually within a few days. When my center did mine, it took a few hours.
Because of all of your son's problems, it wouldn't hurt to have him see a CF pulmonologist at this center, even before he gets a diagnosis. With borderline sweat tests that he has and his history, it should be enough for them to see him. In that case, they would probably run the genetic tests (they have everything necessary to send it to Ambry or Quest) and know which tests to ask for.
I hope this helps, please ask any questions if you have them...this must be so overwhelming for you.
Keeping you both in my thoughts,
Sunny
Avatar universal
Very overwhelming...But I do understand it...I read read read...We had the Sweat Test done yesterday. I requested the Genetic Blood Test while we were there too. Without your insistance I don't know that I would have pushed for it so thank you.

Hunter's primary doctor called around 6pm last night with the results. They were an 18 on each arm!!! Life is good.

What a year it's been. When he had his tonsils and adnoid removed in the spring they said he had a Innominate Artery Compression that impinged his lung 10-15%. But the MRI said nothing was there.

So back then I freaked out so I tried to stay as calm as can be with re-doing this test. But sometimes knowing what you are looking at is scary. My husband does not get as worried. But he is not really aware of CF. I love him but if someone asked Hutner history he would have to refer to me. I forgot everything under the sun but I know Hunter's history like it was written on my hand in ink.

We will await the blood test but I feel a huge sense of relief. I will re-do his allergy tests too. I will turn over every stone to find an answer so I can help him.

Thank you thank thank you

Colleen
mother to
Dominick (12) Hunter (TM10) and Jennifer (9)
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