Adult "child" with CCHS (congenital central hypoventilation syndrome)

[CCHSMOM]

I am new to this site, and I'm not sure yet how to "post". My daughter has CCHS which has caused her to have seizures, mild hirschprungs, J-tube feeds, generalizes autonomic dysfunction, Asperger's/high-functioning autism, and a primary immune deficiency. She is also trached and 24/7 vent-dependent. She used to have moderate pulmonary hypertension and right-sided heart enlargement, but these are now mild after being put on full-time (instead of night-only) ventilation. Despite all this to deal with, she still graduated from Yale, and I am very proud of her!!!

I have read a number of member comments and have experienced many of the same issues: LMV alarms, no nursing care, no portable back-up vent, questions over which trach to use, etc!

I wanted to say "HI" and also ask if any of you have had to deal with the whole transition problem yet. We live in Ohio, and I have spent hundreds of hours trying to find care for her. ALL of her other doctors are still pediatric (ENT, GI, genetics, surgery, dentistry, neuro, hematology, cardiology, etc.), but I cannot find a pediatric pulmonologist in Ohio to follow her "because she is an adult" - even those peds pulmonary docs who see adult CF patients much older than she. However, none of the adult pulmonary docs here follow vent patients as outpatients (some do follow patients on vents in critical care/ICU).

I did find a peds pulmonary doctor out-of-state who has 15 CCHS patients (5 of whom are adults) and who is willing to follow her - if I agree to drive her to New York (10 hrs away) every six months. This will mean paying for his fees, gas, and hotel bills OOP, traveling with no help to care for her and no back-up vent, in a van with 160K miles on it, through a lot of countryside with no help available if something goes wrong. The only other option at this point is to continue having her pediatrician write the vent orders. Having her totally vent-dependent with no pulmonologist is really scary, but so is the idea of this trip (appt. on 3/31/10.)

Any suggestions? Do you think the trip is safe enough to try?

Thanks for any ideas!
CCHSMOM

[kadiera]

Welcome!

I think, if it was me, I'd start with her current pediatric pulmonologist, and tell him that if he is not willing to continue seeing her, he needs to find someone to hand her care off to - I don't think a doctor can just drop a patient like that, you know? It might be worth asking all
of her doctors if they have suggestions for who they'd like to hand her off to, or who other adult patients of theirs see now.

I'd think the trip might be ok, but it's a lot of driving to do back and forth....

[CCHSMOM]

I'm so glad your little guy is vent-free and walking! That is great!

Thanks for the welcome, too!

My daughter doesn't have any pulmonologist right now. Her pediatrician is writing her vent orders. She was supposed to see the peds pulmonary doc at Rainbow who took care of the trach/vent kids until 18 months ago. Right before she was supposed to see him, though, he moved to a different hospital.

The only docs who are willing to follow her are out of state (and not covered by Ohio Medicaid).

The one in New York is very experienced in CCHS, is really nice, and talked to me on the phone for over an hour before even seeing my daughter. He also said he'd follow her by phone and work with all her doctors here, if I take her to see him every 6 months. He seems like the best option we have right now - I'm just concerned about making that long trip.

Does anyone have any experience taking a ventilator kid on a very long car trip? Any suggestions for what to bring (other than the really obvious - like your kid and the vent!), what to tell the hotel, etc.?

Thanks!

[lynn]

welcome to the boards! I dont have any experience with transitioning over to adult care... but I have been successful in getting out of state care covered by our PA medicaid(which is HMO)..have you tried to have the doc who is willing to see her write a letter stating that he is an expert in the field, and that she will benefit from seeing him.. I have copies of letters I had my docs write if you would like me to scan and email them-It may be worth a try-PM and let me know

We took a road trip to NC last summer (11hrs drive) and also this past Nov. we went to the Columbus childrens hosp (4 Hrs).. just make sure to give yourself plenty of time for stopping and care if needed. Make sure you have plenty of extras of the supplies you use (including extra circuits) and I always make sure I have plenty of formula(special order stuff) because it is hard to come by(even in the hospitals)

Can you check in with the hospital and see if they have any sort of agreements with local motels for discounts while you are there for care? We had a free night covered because we were traveling such a distance and it was arranged thru the patient services dept of the hosp.

[jamie]

just a thought but can you see if there is a ronald mcdonald house in the area? they should be able to set you up for a night or two? if not them maybe a kiwanis? both places here in sacramento are 'medical' facilites so they understand the whole vent issue.

[JWorthington]

I know of parents that have taken their kids on long trips to see doctors, with vents, etc. It's not something we've done as Sam was never on a home vent. Just wanted to say hi and welcome and congratulations to your daughter on her graduation!:)

[ealouie]

Hi and welcome!!!!
I am a CCHS mom, too, however my daughter is only 3 and in preschool. She has a trach and is vented when she sleeps.

We have taken long road trips (11 hours) to Canada as well as commercial flights to California and we live in Nashville, however, her dad was with us. I would not want to venture alone on a long trip.

I was wondering if you have heard of Angel Flight? Apparently, there are pilots who volunteer their time and planes to people with medical needs free of charge. I have only read about them and have not used their service. Their website is www.angelflightne.org

Best of luck!
Liz

[trainmanj]

I guess I'm answering from the other end of the spectrum. I'm vent dependent due to a spinal cord injury when I was three. In the quarter century since then, I've learned with my parents quite a bit.

I don't get why a pulmonologist won't see your daughter. As someone using a vent due to health needs, it should be an easy need to fill.

For the past several years, my family and I have traveled about 12 hours from our home to a camp in Ohio I volunteer at. In traveling that far, I would recommend having a backup vent and assistance. Your vent supplier would very likely be able to give you an extra for a few days. Also, having a friend or relative come along could be helpful for driving and assistance if needed.