Addie Kozak: Four Years Old and Battling PH
My daughter Adeline (Addie) was born with hypoplastic left heart syndrome (HLHS). HLHS is a complex and rare heart defect present at birth (congenital). In this condition, the left side of the heart is critically underdeveloped. In HLHS the left side of the heart can’t properly supply blood to the body because the lower left chamber (left ventricle) is too small, or in some cases, doesn’t exist. In addition, the valves on the left side of the heart (aortic valve and mitral valve) don’t work properly, and the main artery leaving the heart (aorta) is smaller than normal.
Addie has a very rare and unique case of HLHS which includes restrictive atrial septum and pulmonary hypertension. Typically, kids with HLHS need three surgeries: one within the first week of life, another between four and six months old, and a third between two and four years of age. Addie had a cardiac catheterization the minute she was born to balloon her septum. Four days later she had her first open heart procedure called the hybird. She recovered well, however, she got sick the day before we were discharged, so we stayed until she recovered. She was six weeks old.
At five months old, Addie had her third open heart surgery called the glenn. She recovered quickly after a 10-day stay. In March 2020, she had her fourth surgery, which was to open up her pulmonary veins as we discovered she also had pulmonary vein stenosis. After this, we were able to live life more normally. In May of 2022, Addie had the fontan procedure. Two weeks later she got an infection and had to go back to the operating room, resulting in her sixth surgery.
We spent more than 40 days in the hospital. In September and December of 2022, she was been admitted for “fontan tune ups.” We were told the fontan physiology was not working for Addie. In December 2022, we received a transplant evaluation. During that time, her care team found out that there was another surgical procedure for Addie, and we did not need to go down the transplant route.
In January 2023, Addie had a fontan revision which resulted in her seventh surgery. In this surgery, they put a PD catheter in her belly to drain fluid. We had hoped that this was going to be temporary. In June 2023, Adeline had another cardiac catheterization, and we saw that the fontan pressures just aren’t going down, and she will eventually need a transplant.
We have been living life this past year and Addie has enjoyed ballet, gymnastics, singing, music, and dancing. She has also been in school and truly loved it. Addie is currently obsessed with Disney princesses and we hope to take her to Disney World one day! Currently, we are in-patient in a hospital as she waits for her new heart!
-Lara Kozak, Addie’s mother
Team PH’s Unmet Needs Fund helped Addie’s family purchase groceries and secure safe transportation to and from Addie’s medical appointments. Make a donation to support pediatric patients like Louisa and their families at teamph.org/give.