Visit with the Rett Specialist

Last week the family packed up and took a trip to H-town (Houston) for Reagan's much anticipated appointment at Texas Children's Hospital Blue Bird Rett Clinic.  The Rett Clinic has a team of Doctors that specialize in Rett Syndrome.  There are several clinics around the country but I'm happy that one is only 2 hours away from us!

We visited with Dr. Glaze (neurology) and his nurse Judy, who were both wonderful and incredibly insightful of our new world of RS.  He talked to us about the possibility of seizures, cardiac problems, weight gain, stereotypical hand movements, and current RS clinical trials.  We were excited to hear that he thinks Reagan will continue to walk!  He gave us a prescription for Carnitor to increase her energy, focus, and improve muscle tone.

We also saw Dr. Motil who specializes in GI/Nutrition and this was the appointment that I was most anxious about, and it turns out for good reason.  I have read that continued weight gain can be an issue and was concerned since I have been having trouble with Reagan eating and keeping food down.  She gave me the grim facts, she must gain 3 pounds (increase from 26 to 29 lbs) by her next appointment in 6 months or we will then have to discuss possible feeding alternatives.  She is in the 50% percentile for weight in  her age group (among typical children) but her BMI is only 10% because she is so tall.
I was even more nervous about her gaining weight when I realized that she has only gained 8 ounces in the last 3 months.  How would we ever get her to gain 3 pounds in 6 months?!?
Dr. Motil gave us some good feedback on food ideas and said that any type of high calorie and/or high fat food she be given at every opportunity.  So, Wes went on a mission that  night at the grocery store and came home with some things that make me gain weight by just looking at them:  whipping cream, syrups, cream fruit dip, whole milk, Carnation instant breakfast, bologne, hot dogs, spray cheese spread, full fat yogurt and honey.  We also use Boost Breeze drink to add calories.  She is now getting about 1400 calories day (and 60 grams of fat!) and already feels more solid after just one week so I am very hopeful!

Reagan has an EKG today to monitor her heart for the long QT Interval (creates an irregular heart rhythm), which can be common in girls with Rett Syndrome and possibly explain sudden death.    We are hoping that it comes back normal today and then we will have it monitored every few years to make sure that there are no changes.