A backyard hockey game at our first ever T4T in 2007.
Today was long and frustrating at first, but nothing bad happened, so it turned out to be a good day.
We arrived at the MDU at 7:30am. Trey still had his runny nose from last week. We did his BP, temp, Emla, height, weight and had to leave it at that as Trey’s ERT doctors were not yet in to give the go ahead to order the drug and we had to get to our appointment with Dr. Reilly in orthopedics for 8:00am.
We got to orthopedics at 8:00am and were sent to x-ray for pictures of Trey’s upper cervical spine. At 8:30 we saw Dr. Reilly who told us that Trey’s x-rays are the same as last year. This is great as Hunter’s is progressive. Trey’s top two vertibrae are not fully formed, which we knew from last year’s appointment and is expected in children with Hunter’s. The concern with this and why we will continue to be followed by orthopedics until Trey stops growing is that if this gets worse, Trey can have instability in his head and neck. The good thing is, as long as Trey’s vertibra stay the same, Trey can live a full life doing whatever he wants. It is possible that these vertibrae will fill in with bone, but it is possible that they will not, and this is not a big deal. A great appointment. Good news is always nice to hear.
Then back to the MDU. Trey’s ERT doctor was again uncertain about giving Trey’s infusion because of his runny nose, so she asked Dr. Stockler. Dr. Stockler gave the go ahead providing Claritin and Ibuprofen were given one hour before the start of the infusion and providing the rate was slowed down. Instead of increasing every 15 minutes from 8mL/hr to 16, 24, 32, and finishing the infusion off at 40mL/hr, Trey started at 2mL/hr and went up every 15 minutes to 4, 8, 12, 16, 20, 24, 32 and 40. Needless to say, it made for a long day. We weren’t leaving the hospital until 6:00pm (thank you mom and dad for coming and staying!!). But again, no reactions and he got his enzyme, so I’m a happy mama. Next week, we’ll premed, but put the rate back to the usual rate and if everything is still okay after next week’s infusion, we’ll go back to the same old routine the following week.