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Hi Ursula,

He recommended the PCP prophylaxis (of course) and checking her antibody

response to her immunizations. The antibody test must be drawn 4 weeks after

her immunizations were given. If she has no response (I guess it's a 50/50

chance kind of thing) then she will have a central line placed (I'm guessing a

port since the PICC lines have not been such a wonderful thing for Meg) and then

start on IVIG infusons. He also wants to give her some protection from RSV

(especially since I have two older kids). But, the respigam infusion that will

protect her from RSV will mess up the test for her antibody response. Also it

is a lot of volume, which is very hard for her to deal with from a heart

stand-point. So, I guess for this 4 week period, we are going to try the

synagis shot and just not expect much from it. When I take her for the blood

test for the antibody response test, they will also draw the test for adenosine

deaminase deficiency and nucleotide phosphoryase deficiency. If she is positive

for either of those, then she will definitely be placed in the SCID category and

considered for a bone marrow transplant.

I was concerned about the 4 week lapse between now and really finding out any

meaningful information. We have been instructed to follow pretty strict

anti-infective protocol. I can already tell I'm going to be exhausted by the

end of these next few weeks.

Her breathing is not so great today. It's hard to check her sats when she's

awake, so I will check again when she falls asleep. I've been crossing my

fingers all day that we are not going in the direction of pneumonia.

Does all of this make sense? I'm still trying to process it all. If there is

anything that doesn't sound right, let me know and I will look into it. I know

that parents are the experts when it comes to chronic kids.

Take Care

mom to (T cell deficiency)

Re: a brief update about

- it sounds like you had a very informed conversation with the doctor

this week. What type of management of her

immune problem did he recommend? How is her breathing today?

Ursula Holleman

Macey's mom (6 yr. old with CVID, asthma, sinus disease, GERD, kidney reflux,

Sensory Integration Disorder, Diabetes

Insipidus)

http://maceyh.home.att.net

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