Reesa Visits a Special Doctor in Pittsburgh

When we first found out that Reesa was diagnosed with Krabbe Leukodystrophy, we researched the disease and connected with other families in our situation. Almost all of the Krabbe families we spoke to recommended that we visit Dr. Maria Escolar at her Neurodevelopment of Rare Diseases clinic in Pittsburgh. Dr. Escolar has spent the last 13 years studying Krabbe among other rare diseases. In 13 years she has seen over 70 Krabbe patients which is more than anyone in the world (this shows how rare this disease is). Although there is no cure, Dr. Escolar helps maximize the patient's quality of life through medication and educates families about the disease, its progression and the types of equipment and therapy needed to keep the child comfortable. We knew that visiting Dr. Escolar was very important for Reesa, so we booked our trip immediately. We were extremely nervous as the trip was getting closer because the flight from Phoenix to Pittsburgh (with a 2 hour layover in Cincinnati) was an all day event. Not only was this Reesa's first time on a plane but she was also extremely irritable. Reesa would cry for hours unless you bounced her legs up and down so we were prepared to bounce her on both flights. We packed up all of our gear and headed to the airport and to our surprise we couldn't believe how perfect Reesa was on the plane. She slept most of the flights and when she was awake she was as happy as could be. It was a very long day.We left Phoenix at 11am and arrived at our hotel in Pittsburgh at 10pm. We were exhausted and Reesa wasn't ready for bed because she slept all day plus she was off schedule because of the 3 hour time difference. We had a very early appointment in the morning so we needed to get some sleep. After rocking Reesa for about an hour we were laying her down in her pack-n-play and the next thing we new her G-tube came completely out of her stomach! We had to rush Reesa to the hospital and knew that we only had 30 minutes to get the tube back in or the hole would close up and Reesa would need surgery again. Long story short....after being at the hospital for 5 hours, they were able to re-insert the tube, and we got back to our hotel at 4am got about an hour of sleep and then our appointments with Dr. Escolar and her team began.
  The next 6 days consisted of tests and therapies ranging from spinal tap, MRI, hearing tests, vison test, and occupational, physical, and speech therapies and Reesa was a such a trooper through all of them.  
                     
Dr. Escolar also started Reesa on new medications (Baclofen for spasticity and Neurontin for nerve pain) and the first day and we noticed a change immediately. Reesa started laughing, babbling and was full of smiles. We hadn't seen our baby girl like this in over a month. What was planned to be a two-day trip was extended to 6 days so that Dr. Escolar could adjust Reesa's medications each day and get her to a point where the meds were doing the best job of keeping her limber and comfortable. We will see Dr. Escolar again in three months so that she can evaluate how Ressa's motor skills are and to evaluate if she is regressing and what pace. Dr. Escolar and her staff were so professional and knowledgeable. She was able to restore peace and comfort to Reesa's life (and ours).
While in Pittsburgh, we had a little bit of down time, so we took Reesa to the botanical gardens and had a ride on the merry-go-round!

Girl’s Weekend

Daddy and Grammy went out to New York for our cousins Greg and Laura's wedding so mommy decided it was time for a girl’s weekend.    Mommy's two best friends from college flew out to be with us; Miss Abby came all the way from New  Jersey and Miss Kelly came from Washington. Bre, Melissa, Zim and Somer also came over to play, we had so much fun.  We pretty much stayed in our pj's all weekend but still managed to have a blast. We had cupcakes, dance parties, lots of bouncing; my favorite (it’s when you take my legs and bounce them up and down, I think it’s so much fun) we also had lots and lots of laughs and some tears too.  I also spent some time with Sherry and my Uncle Houston they bounced me too; my Grandpa Jim came over and we watched some basketball and then he rocked me to sleep. We had such a busy weekend with so many visitors, it’s a weekend I will remember forever.

 

Reesa Gets a G-Tube

Happy That This NG Tube is Coming Out!

We recently had a  planned, 5 night stay at Phoenix Children's Hospital. The main purpose was to have a g-tube placed in Reesa's stomach, along with a couple of other secondary procedures.  Krabbe disease has diminished Reesa's ability to suck and swallow which puts her at risk of aspirating on liquids, food, mucus, etc. This development is frustrating because, since the day she was born, Reesa has been a terrific eater and has always loved sucking on her pacifier. Over the last month Reesa has been fed via NG feeding tube which goes in her nose to her stomach.  The new g-tube replaces the NG tube and is a more convenient, comfortable and permanent solution for feeding. It is basically a quarter-sized hole through her belly into her stomach where we attach a feeding machine. We also administer medication and even burp her through this hole. For the first few weeks Reesa will be fed continuously via the g-tube at about an ounce/hour for 24 hours. It makes moving around pretty tricky since Reesa is always trailed by a tube, feeding machine/bag and an IV pole. It's amazing how we have gotten used to this crazy routine. You just adapt. Although no hospital visit is fun, Phoenix Children's Hospital is an unbelievable place with wonderful doctors and nurses.  They've created an environment that makes hospital visits comfortable for the patients and their families.  As usual Baby Reesa was a trooper. No matter how many times she was poked, prodded, awakened, examined...she always had a smile for mommy and daddy at the end of the day!

Getting Ready for Surgery

Recovery...Puffy from the IV Fluids

 

Daddy & Reesa

 



Auntie Heather & Chris


 



Reesa Loves Uncle Houston