6 year old with mild CF (?)

[Mom_EC]

Hi All, Glad to find an active community for support. Jeez has this been a tough couple of weeks.
My son has had sinusitis for 2 years. Antibiotics were tried multiple times. Pediatrician said it was a bad cold or recurrent virus since he started school (and there were known recurrent viruses in the classroom). We saw an ENT who wanted to clear it up and then take a look. It didn't clear up and then I moved... saw an ENT in NJ who immediately wanted to take out adenoids and tonsils.

Luckily I got a 2nd opinion from an ENT in the city (NYC)-- who said tonsils looked fine, adenoids were enlarged but that could be a result of the infection, and tons of thick green snot. We did an aggressive, bacteria specific course of antibiotics for 21 days- nada, no change in the snot. ENT said the adenoids were normal size but he could see an obstruction in the nose, something that should be removed. We went for a CAT scan, so he could do cat assisted nasal surgery. That is when I got a call that he believed my son has CF. The ENT said it is clearly a variant because he is 6 and exhibits no GI symptoms (is in 90%th percentile) but we should sweat test him.

We did sweat and genetic. They found 2 variants, one is known to cause CF, and he got a 48 on his sweat.
We went back for a 2nd sweat. Then did a PFT (lung function), chest cat scan, deep throat culture and took his father and my genetic material to see if/how we are carrying the variant genes.
Director of the CF clinic said he is 90% sure my son has CF.
He wanted us to start preventative therapies, but agreed we could wait for our genetic tests to come back.

I am just looking for a support network as I wrap my head around the preventative care and how people organize their lives around it...

 

[Rebjane]

Hi there, It sounds like the doctors are taking your son's symptoms seriously; which is wonderful, they are validating your concerns. The fortunate thing is preventative therapy will keep your child healthy. The sooner you have a diagnosis; you can start airway clearance(chest PT) or VEST therapy; things like Hypertonic saline 7% saline(either inhaled as a nebulizer or used as a sinus wash) does wonders to clear the gunk out of sinuses and lungs of people with CF.

My daughter is almost 15 years old with CF(geez I can not believe it). She was diagnosed when I was pregnant with her. She goes to a public high school, is an excellent student and a high achiever. She does ALOT to maintain her health..She does a total of 8 nebulizers a day and 3 VEST treatments everyday to stay healthy among a slew of other things. She even has started riding an excersize bike a half hour a day after school(Exersize is medicine) Anyway; she has a FULL life, lots of friends. I would have so much more to add but I have to go to work, Please keep your head up and ask questions...There is hope .

 

[rlondon]

This sounds a lot like my son, 5 yrs old. Sinusitis for the past 3 years, rounds and rounds of antibiotics and nose sprays. Adenoidectomy at 3 yrs old. Finally switched doctors and he immediately had us do a CT scan and sleep study. Mild sleep apnea and blockages in his sinuses led him to recommend sinus surgery (and removal of adenoids and tonsils). It wasn't until post surgery that our doctor said hey, the amount of nasal polyps up there is very abnormal, I want you to get a sweat test to check for CF. It's been over 2 weeks and I don't have the results yet. Called today and the office has them but they wouldn't discuss until the doctor is available to give us a call, so I'm just really hoping to hear from the doctor before the long weekend. Anyway, I just have a gut feeling that we're going to get a diagnosis of some sort of atypical CF. The "permabooger" as we affectionately call it - thick, yellow, and coming out his nose and down his throat - is just not normal. I'm grateful this board exists and I'm with you on looking for support and information.