an update

[Guest guest]

Sandi and Dale, thanks for the welcome back!

Things here in Australia are as busy as always. I dont know what not

feeling tired is anymore.

Since s diagnosis of FG Syndrome and the following year

starting pancreatic enzymes for pancreatic insufficiency the puzzle

that was seemed to be solved. The enzymes have made a huge

impact on his abdominal pain and we are working with the dietics team

to reduce supplement feeds and increase oral intake.

Neither children are on IVIG. I wish I hadnt been so hesitant on

starting it when was nearly 3yo, but we were dealing then

with the testing for brain tumor and I found myself questioning the

immuno on his deciion, which was a huge mistake. There is only one

childens hospital here and our immuno was the head of the immuno dept

there. He didnt like my asking so many questions and had a horrible

attitude and pulled the IVIG a few days b4 was booked for

his first infusion. It seemed personal, and we tried switching

immunos afterwards but they all answer to this particular doctor and

it became very frustrating. Knowing would be straight on

IVIG if we moved home to the US makes things even harder. So we stay

away from high infection risk areas, it has become second nature to

check environments for any signs of infection- play dates,

classrooms, shopping centres, etc etc, use Aquium hand gel when out

and about, and I could go on and on. Prophylactic abx helps to some

extent, but the break through infections happen regardless,

pneumonias etc, and still battles with her sinus infections

and cellulitis.

School has been hard this year, with s new teacher (school

year here runs Feb- Dec) deciding in the second week she could not

handle all of s medical conditions and asked for him to be

removed from her class. His previous teacher had him for 2 years

running and had no difficulty at all, so this was totally unexpected

and a huge shock. With s autistic characterisitcs, having to

move into another new environment was in no way easy, but his new

teacher is wonderful " if he wants to run, he can run. If he wants to

fly, he can fly " .

After s abdominal xray Monday showing obstruction we have been

hoping to clean him out at home without needing to go into hospital.

We started him on Pico Prep Monday, with the second dose Tuesday, and

I was hopeful it was going to do the trick because he did spend some

time on the toilet as a result. However he has not gone at all today

and is again tonight complaining his tummy is hurting. He has a

renal ultrasound booked for Friday and I was hoping to ask them to

give me some idea how his bowel looked while they were there, but

thinking on it perhaps the best thing to do is just arrange with

s dr to order another xray that we can have done while we are

there.

is less croupy tonight so the dexamethasone seems to have

helped. I had to take her in to the drs thisafternoon, having

suspected lastnight she may also have a sinus infection brewing, the

clincher was being told thisafternoon she could smell blood. She

looked dreadful and was running a temp (not a common thing for her to

be able to do) So now she is on abx and hopefully will respond.

I have a query on the pneumovax vaccine, and how often it is

suggested our pid pumpkins are given it on the assumption they are

able to mount a response. Is it every 5 years?

Bright wishes to all,

, mum to and and wondering how it got to be

midnight so quickly.

(10yo) Absolute IgA deficiency, low complement, asthma, chronic

constipation, FGS carrier and bossy big sister.

(8yo) Functional antibody deficiency, absolute IgA

deficiency, FG Syndrome, CPH, bilateral PUJ obstruction, pancreatic

insufficiency, asthma, gerd, hiatus hernia, chronic constipation and

a wicked sense of humour.