Our first few days in Minnesota

This is our first journal post about Lyla and we have a lot to update everyone on already!  We've had a very busy three days so far in Minnesota, kicking off the process for Lyla's stem cell transplant.

Lyla has been so good and has managed to charm every single doctor that has seen her. They are all amazed by how excited she gets when they exam her and she's known as the happy, smiling baby.  She's in her element with so many people around :)

We are very impressed with the hospital and doctors and confident Lyla is going to be in good hands for her upcoming operations later this week and stem cell transplant, which is tentatively planned for mid-July.  Finding a donor and hopefully cord blood is key. If not cord blood than it could be months for a bone marrow donor. We are in a race against time because the disease has not effected Lyla's brain yet but it will start to do so anytime in the next 6 months or so. 

 Some of the key things we've done and found out so far this week are:

- Lyla had a lot of blood taken for DNA sequencing to find a donor match. With cord blood, an acceptable level is 6, 5, or 4 out of 6 with 6/6 being a perfect match. They also consider cell count in blood so a lower match might be accepted if it's a high cell count. They are pretty confident there will be a match. We will have prelim findings for cord blood matches in about a week.

- Besides the mitral valve prolapse, the cardiologist was happy with the condition of Lyla's heart. Her valve is pumping well and the medicine also seems to be helping. The hope is that the Enzyme infusions will prevent any further heart disease but it likely won't significantly improve her heart so there is no reason for her to stay on enzyme longer than the planned 8 weeks. 

- The biggest concern at the moment is her hemoglobin levels are lower than they would like due to her having thalassemia minor (8.4 and the ideal is above 12.0).  This is causing her heart to work harder than normal and an increased risk for operations. She needs to be on heart medicine (mulrinone) starting one day before her operation. It will help with the stress of operations so her heart doesn't have to work so hard. She will need a blood transfusion before operation to help with hemoglobin levels as well. 
-we need to decide if she gets a Hickman catheter (comes out of skin with multiple IV lines) now instead of port (under skin with one IV line that still has to be accessed by needle and used for enzyme infusions). She will need a Hickman before transplant but a port is alot less maintenance and less risk of infection while at home in the few months before transplant
- Hearing tests didn't work because of fluid in her ears. Lyla will likely need tubes do reduce risk of infection during transplant when her immune system will be wiped out. They are deciding if that will be done this week. We are lucky she hasn't had more infections so far
- The anesthesiologist is worried about her cold/runny nose and won't allow operation on Thursday if it doesn't get better.  We are going to postpone the operation until Friday hoping her cold clears up. The Surgeon is not as worried but it's up to anesthesiologist. If her cold doesn't get better than we either stay here until next week or come back later this month. 

-overall Lyla's symptoms seem to be less than some other babies. We are very lucky it's been diagnosed so early.

- we learned that her IQ levels will decline significantly every month starting sometime in the next 6 months. Mental retardation will occur by age 2-3 and can't be corrected. We also learned that IQ can still drop in the few months after transplant but it's usually only between 5-10 points and will stay there the rest of her life. The hope is that Lyla has an above average IQ now and that transplant is done before disease effects her brain. 

-Although her IQ level can't be tested since she's so young, they did perform an extensive developmental test on her. Overall, the results were really positive and Lyla is pretty on track for her age. They feel like she's slightly behind in oral and gross motor skills but lots of babies can develop those skills at different ages.  And, maybe she just didn't feel like rolling over lots while the test was being taken :) 
- We are still feeling very optimistic about Lyla's future. In speaking to Lyla's head nurse, she has seen several MPS patients and the successful transplants have had great results. She's knows of at least a few that have gone to college (which is amazing since transplants have improved significantly in the last 10 years). She said a lot are doing really well, they are in school and play sports and look pretty normal. She did warn us that transplant does not fix all cardiovascular symptoms and children often have knee, hip carpel tunnel and other joint problems that require surgery and cause problems their whole life. The yearly checkups will try to catch those things early so they can intervene before too bad. She did say that growth is also still effected. It's not nearly as bad as 10 years ago though because the hormones used to be severely damaged by the chemo but they have better ways of administering now. But, Lyla will likely be of short stature. 
 
That's all for now...
Love to all,
Helen, Steve and Lyla