re Janet

[Guest guest]

Hi,

JANET, ooooooooh my.... How DO people like the doc who diagnosed your

children manage to stay in practice??? Ken and I may have differences of

opinion with our pulm on treatment matters sometimes, but you've just

reminded me how lucky we are to have a bright, professional, knowledgable

pulm who cares about her little patients, and always shares all the

information she has with the parents (she even sends us copies of the

reports she writes for our ped). The story of the first pulm you dealt with

absolutely takes the cake. It's sad that so many people have stories like

that to share.

LORI D., our pulm had Meagan do TheraPEP (PEP valve) when we first found out

she had CF, so I know she uses that method as an alternative to CPT

sometimes. The problem with that one (or the Flutter) is that the child has

to be older and VERY cooperative, for us that leaves CPT as the only option.

She said she doesn't prescribe the vest usually unless a child absolutley

won't do anything else, or a young adult is going off to college or moving

out on their own and doesn't have anyone to do CPT. Meagan is right up

there with Scout in HATING CPT lately, and Ken and I aren't really too fond

of the whole process either, though we do a thorough job of it 2x a day

because it really does help her. My wrists and shoulders get SO sore while

doing CPT, and have you ever noticed that if you're really tired when you do

it you get sort of neauseous and dizzy by the end-- maybe it's just me, and

it's a sign of how out of shape I am.... Anyway, I've got a serious case of

vest envy lately :-( It would be so nice for Meagan if she could sit

upright and watch a video during lung clearance, and even do nebs at the

same time-- boy, that would free up some time. Oh well, not going to

happen. Our HMO wouldn't go for it anyway, grrrrr! (I'm experiencing HMO

hatred today on a non-CF issue).

BECKY, congrats on the great news! Was it the oral steroids playing with

the blood sugar levels? We had that problem with M. once, but the numbers

weren't nearly as out-of-whack as Beckah's were. I just finished your

diagnosis story-- for once I'm at a loss for words. You're incredible. I

was so moved by your story. Hugs.

AGNES, believe me, it's absolutely possible to have CF with a negative sweat

test. Meagan isn't the only proof of that on the list (pseudomonas, staph,

klebsiella, sinus disease, two identified mutations, and all). Carol's kids

had similar experiences, Jennie's Mallory was sweat negative till she was

almost 2, and Katy has two children with R117H (my daughter's gene) who both

have CF with negative sweat tests, and the baby is so pancreatic

insufficient he had a G-tube really early on and had been pretty sick last

time I heard from her, so negative sweats don't always mean " mild " CF or

pancreatic sufficiency. I've also read about one severe mutation that's

also associated with negative sweats sometimes. Our pulm insisted that there

was absolutely no way to have CF with a negative sweat-- when she called to

give me Meagan's official CF diagnosis after the NPD test and talking to one

of the R117H experts at 's Hopkins, she said " Disregard everything I've

said before. " You can ask for a copy of the Diagnostic Criteria from the

CFF-- in there it states that 1% of people with CF are pancreatic sufficient

with negative sweat tests (the hardest group to diagnose, pancreatic

insufficiency makes it easier to spot CF). All I can say is that it sounds

like the NPD test is the best answer for you (they do it at UNC, U. of

Iowa-Iowa City, and several other centers). Hang in there. (Oh, also,

many, many people get negative sweat results if the test is done

incorrectly-- it should always be done at a CFF center!!!)

bye,

, mom of Meggers and Kalers monsters