It's Been Too Long........

  It has been months since I have updated this blog again.  The good news is that the reason why is because the girls have been so busy with all of their activities!  Having 2 kids involved in sports, and extracurricular activities sure keep mommy and daddy on their toes!
  Lily finished up a fantastic school year this year.  She really enjoyed 1st grade, and did incredibly well, excelling in Reading and Math.  We couldn't be more proud of all of her accomplishments.  She played Softball for the first time this year, and really enjoyed it, and she was able to dance in her 4th dance recital.
  In June, Lily made her first visit to the Bone Health Clinic at CHOP.  We were referred after her DEXA scan showed low bone density and a high PTH.  At the clinic she was seen by endocrinology, nutrition, and physical therapy.  I expressed some of my concerns to the PT regarding Lily's physical abilities, and an assessment showed that she has some general weakness in her right leg, as well as a shorter leg length on that side.  The PT felt she would benefit from 6 weeks of Physical Therapy over the summer to build muscle tone on that side.  So, this month we began once weekly sessions which daddy is taking her to.  The therapist is already impressed with Lily's work ethic and determination, and we know she will excel at whatever challenge she faces.  She is our rock star!
  We have lots of small trip and mini camps planned for the summer including art camp, Girl Scout camp, trips to NY and Ohio to see family, and Ocean City, NJ for some beach fun.  Before we know it the new school year will be upon us and we will have a 2nd grader!  I am posting a few recent pictures for you all to enjoy.  We hope you have a safe, happy, and healthy summer!!!

Heart Hugs,
Julia                                                                2013 Softball

                                                                             Dance Recital

                                                                           Dance Recital

                                                               Last day of School 2013
                                                                         

 

CHD Awareness Week 2013

This week February 7-14, 2013 is Congenital Heart Defect Awareness Week. CHDs are the most common birth defect in the U.S. 1 in 100 babies born each year will have some type of CHD. 1.8 million Americans alive today have CHDs. CHDs are the leading cause of all infant deaths in the U.S., yet CHD research is grossly underfunded relative to the disease prevalence.
Educate yourself!!! We like to think that something like CHD would never impact out family. That is how I felt before having my daughter 6 years ago. I know different now.

Heart Moms Unite!!

Last night I had dinner with some of the most amazing moms around. Not only are they amazing, but they all have children with HLHS. It was so wonderful to network with a group of ladies who share the same journey that I do. We had many great conversations, and lots of laughs!! Hope to see you all again VERY soon!

My Heart is FULL!

Today was the annual Philadelphia Heart Walk. We once again had the honor and privilege to walk as team Lily's Heart Heroes. It was a beautiful sunny day, with over 40 of our friends and family who turned out to support Lily and our family. Our biggest team yet! This year was especially significant because our team included 3 children who are all heart warriors. Lily-HLHS, Danny-HLHS, and Molly-Kawasaki Disease. To say that these three kids are an inspiration is an understatement!! Their families are amazing, and have become such a wonderful support system for me :) Being able to share our journey with families who are just beginning theirs is something that I consider to be a great honor!! So....thank you to all of you for always reminding me how grateful I am for the amazing people in our lives. My cup is full today because of all of you :)

PTH and Calcium

We received a call from Endocrinology at CHOP today. Lily's PTH levels are high, and her Calcium levels are low. They want to start her on 500mg of calcium carbonate daily and retest in 2 weeks. If her Calcium levels don't come up, then they may have to order more testing to find out why? Have any other HLHS families had experience with low calcium or high PTH?

Today's CHOP visit

Well, we survived!! Today was Lily's first visit to the Single Ventricle Survivorship Program at CHOP. I can't say enough how lucky our heart kids are to have a program like this at our hospital. What other programs don't realize is that single ventricle defect kids can have lasting effects on other systems in their bodies due to the type of circulation they have. These effects can be very damaging if not evaluated and treated early in their progression. Because of this, the children in CHOP's SVSP program are evaluated by a team of physicians in various specialty areas including Immunology, Gastroenterology, Endicrinology, and Cardiology. They work together as a team to treat each patient and work with the families as the kids grow into young adulthood and eventually transition to an adult congenital heart program.
Lily was first evaluated by Immunology. They follow the kids for any protein losing conditions as well as any other immune system or allergy disorders. Dr. Heimal feet that Lit looked great, no indication of PLE or any other immune deficiencies.
Next we saw Dr. Iyer for Lily's routine pacemaker check. She has about 3 years left on the battery. Not sure whether it will be replaced then, of just removed. We will reevaluate her status once we get closer. We also saw Dr. Ravishankar who will be Lily's new primary cardiologist since our Dr. Donner retired. She was so excited to see Lily as she hasn't treated her since she was an infant in the CICU. She is just as loving and compassionate as I always remembered her to be, and extremely knowledgeable in the treatment of HLHS kids. We are very lucky to have agree to take Lily on as her patient. We will see her again in 6 months for a regular cardiology followup.
We then saw Dr. Mancilla with Endocrinology. They follow the growth of these children. Lily's DEXA scan showed lower than normal bone density and a low PTH. She sent us for repeat blood work, and a repeat urine. If the levels come back low again, she will start Lily on a Calcium supplement daily to hopefully boost them up.
Finally we saw Dr. Rand with Gastroenterology. Their main focus is the liver. Children with single ventricle Fontan circulation tend to have blood that pools in the liver. This can lead to scarring and eventually cirrhosis. They would like to do a liver biopsy on Limy sometime in 2019 to rule out cirrhosis. The good news is that now her liver looks great. It is normal size, soft, and liver studies are normal! All great news!!
The doctors mentioned that Lily looks like a "perfect Fontan patient." They were excited to hear that she is active....dances, does gymnastics, and participates in gym class with the other kids. They switched her liquid Enalapril to a 5mg tablet twice daily, and also added a twice daily aspirin regimen. This will be new for us, but something that has been discussed in the paste a
All and all, it was a very positive visit and we don't have to come back to SVSP clinic for another 2-3 years. Just regular cardiology followups. Below are some pictures from our day.