Part I - Feb 8th , A Long Day in the Hospital

Hi Everyone!

We just got in from a long day of tests and consults at the University of Minnestota Medical Center, and we're still not quite finished. I'll be dropping off one more urine specimen this eve for some additional tests. Then it'll be martini time. :) Gracie was soooo good today. She's a tough broad! I think Hurler and the transplant will be no match for her.

If you want to know how complex and entirely consuming this disease is, we'll have to post the schedule and the list of different folks Gracie is meeting. I'll give these folks a lot of credit for coordination thus far - at least for the first two days. Everyone has been extremely accommodating. Impressive stuff.

Oh, and anyone interested in their systems infrastructure - they use Allscripts in the outpatient clinics and Navicare in the OR. They have PACS but I didn't catch a glimpse of it yet, and also no real interaction with the inpatient systems yet. :)

Her first appt was in audiology. She failed the audiology test. :( (Her first and hopefully only "F") Next we went to see the ENT. Her tonsils and adenoids seem normal, so no planned intervention there. She has fluid in her ears so she will get tubes inserted on Tuesday. The ENT doc was an interesting character. I liked him, but he kind of reminds me of some goofy cartoon character - cant pin it down right now though. Seems like a really sharp guy, but just socially awkward enough to seem embarrassed and/ or bored and/ or uncomfortable each time he starts to speak. Rarely makes eye contact, etc. We couldn't help but chuckle when he left. Regardless, he's helping our Gracie, so he's all good in our eyes...

Next we went to anesthesiology. They needed more info than we had before confidently proceeding with anesthesia. Until they get MRI results, xray results, and echocardiogram, etc. Until then, they assume the worst to be safe. They'll get those results soon though. It was still good to meet them. It's clear they understand the unique issues and challenges from Hurler, including the immature cervical vertebrae issues I mentioned previously.

Next was the echocardiogram - it's like a sonogram for the heart. It was cool to watch on the screen. Gracie was very helpful through part of it. She wanted to hold the sonogrammer (?) and tried to eat it too, of course. Then she decided it was time to scream. That lasted for the last 3 or 4 minutes of the scan.

I'm trying to keep these posts somewhat bite-size and digestible, so they don't overwhelm me or you, and so you can read them without too much time investment. That said, I'll post separately to discuss our last meeting with the geneticist, Dr Whitley. He was part of the first bone marrow transplant for Hurler's at Univ of MN, and has been a leader in Hurler's treatment for many years. The meeting lasted at least 3 hours, and was really informative and started a host of questions for us and for the doctors to consider when making a treatment decision. Stay tuned.