October 8th

First of all happy Birthday to Wendy.
This week has brought lots of answers to our questions, but still there are unknowns.
PT is slow going as Irelyn still gets sleepy why trying to feed with the bottle. She has only taken 5cc at both sessions so far. We saw specialty care today and she increased her intake to 65cc every 3 hours. We are also going to increase by 5 cc every week for 4 weeks. We have seen good weight and length gains so far. Today she weighed 7lbs. 5 oz. was 20 1/2 long. She still is really sleepy most of the time but does have periods of staying awake. Tuesday she was awake for 3 hours straight.

Wednesday we went to KU (University of Kansas) to see Merlin Butler, he is one of the foremost experts in the world on Prader-Willi. He operates one of only 4 PWS specialty clinics in the US. The other 3 are at UCLA, Vanerbilt, and University of Florida. He sits on the board for PSW and is the editor of "the bible" of PWS. He is extremely knowledgeable about PWS and is getting us in contact with other families in the metro that have a PSW member. Our questions where asked and answered here they where.

How long will she have the g-tube?
It will depend on her as every case is different, but as a general rule about 9 months or so.
This really helped Wendy out as he reassured her that Irelyn would eat but at her pace.

Milestone at what pace?
Usually double what is normal for children.

Growth Hormones? When to start and do you recommend then?
Yes, he recommends themas they are standard of care now(even now as tx for PWS, insurance companies fight not to pay for them, it might be because they are around 40,000 a year for 18 years). As for when to start, he said we have time around 6 months to 1 year or so is what he recommends.

Should we get vaccinations?
Yes, there is no increased risk for PWS and EVERYONE in the house has to get both flu and h1n1 vaccinations.

We agreed to do further genetic testing on both mine and Wendy's part to see if Irelyn has UDP or misimprinting on my part as misimprinting can be passed on. We also agreed to do some testing of Irelyns urine to see if she has adrenal deficiency as this is a MAJOR cause of people with PWS to die prematurely. This is also needed before she is started on growth hormones.

Other important things we learned: Our congressman that represents our area had a child with PWS that died and he is a big supporter of the cause, there will be a fundraiser sometime in the spring for PWSUSA, Irelyn is doing as she should with someone with PWS, and the state rep for PWS lives in our city. We go back in a month to see Dr. Butler and thank god for him being here so close to us. He told us he sees families from all over the midwest as he has been doing this for 30 years.