Every year, 100,000 babies don't make it to their first birthday because of CHD.
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Zola Amelia Given is our Heart Warrior of the Week! Zola’s parents, Cortney and Jeff, were at their 18-week anatomy scan when they received the devastating news that their little girl was going to be born with Hypoplastic Left Heart Syndrome (HLHS).  This small but mighty Heart Warrior was born on April 19th, 2017 and her parents say that despite what she has been through as a result of her condition, she has been joyful, smiley, and content ever since.

Zola has undergone a total of five surgeries and procedures in her short five months of life, spending two and half months in the hospital during her battle with congenital heart disease:

  1. Norwood surgery at 36 hours old. This is the first of three open-heart surgeries that are used to treat those with HLHS.
  2. Diaphragm plication surgery to repair a paralyzed diaphragm muscle that was caused from a post-op complication from her first surgery.
  3. ECMO placement (heart & lung machine/life support). This was due to Zola going into cardiac arrest after complications from her diaphragm surgery.
  4. Gastrostomy tube (g-tube) surgery to assist in feedings and growth in preparation for her next open-heart surgery.
  5. Heart catheterization to asses Zola’s heart before her upcoming open-heart surgery. This was supposed to be a simple overnight stay but it turned into a week-long hospital visit due to heart complications and a blood clot.

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Zola is scheduled to receive her second open heart surgery (Glenn) at the end of October.  This will keep her stable until she is three years old when she will receive what will hopefully be her last surgery (Fontan) in the three-phase process to treat HLHS.  In addition to her multiple surgeries, Zola is also on nine different medications to keep her heart functioning: a shot given twice a day, blood thinners, heart-pumping medication, diuretics, blood pressure medication, pulmonary hypertension medication, acid reflux medicines and vitamin D.  Cortney and Jeff are hopeful that as she gets stronger and recovers from her upcoming surgery, that she will be able to be weaned off some of these over time.

We talked with Cortney who offered insight on what it’s like to be a family affected by CHD as they navigate the waters of balancing family life, school, and a daughter with a medically complex heart from across the pacific.

Save the Heartbeat (STHB): Are there any worries or challenges you have faced/continue to face being a CHD Family?

Cortney: The hardest challenge has been changing our plans.  When we found out about Zola’s heart condition we were students in college.  We moved from Hawaii to California to ensure Zola had the proper care to come into this world. We knew she’d be worth the fight but putting our lives on hold has been taxing. Recently, my husband and older daughter Grey (2-years old) moved back to Hawaii because school started up again. As we wait for Zola’s second surgery it’s hard to be apart from each other. We can’t wait to be a “normal” family, back home in Hawaii!

STHB: What inspires you about your child?

Cortney: Zola is the happiest baby I’ve ever met. She first smiled at one week old. I caught it on camera at two weeks old and she hasn’t stopped smiling since! She is a warrior and has fought some tremendous battles! She is happy 95% of the time and when she’s a little sad it’s usually because she needs her diaper changed (she does not like a wet bum)! Her joy gives me strength as we face the future.

STHB: Is there anything else you want to share about Zola? You know best!

Cortney: Zola’s story has spread all around the world and I’m so grateful we’ve been given the opportunity to spread awareness about congenital heart disease and HLHS. More than that, the community that has rallied behind her and our family during this journey is remarkable. We have seen first-hand that people are good! In today’s world – where there are a lot of awful things going on – it’s so good to be reminded by the examples of others that people are good-hearted and full of love. It’s a reminder that there is more good in this world than bad.

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About Hypoplastic Left Heart Syndrome

hypoplastic left heart syndrome, hlhs

The condition: In an HLHS heart, most of the structures of the left side of the heart are underdeveloped or missing altogether.  This includes the left atria, left ventricle, aorta and mitral valve. Basically, the child is born with half a heart.

Complications: Because the mitral valve is very small or not developed at all, this prevents blood from entering the left ventricle. As a result, blood is diverted to the right atrium through an atrial septal defect (hole in the heart). Because the left-sided structures of the heart are essentially missing, the right ventricle collects both oxygen-rich blood from the lungs and oxygen-poor blood from the body. The right ventricle is also responsible for pumping blood to both the lungs and the body. Since deoxygenated and oxygenated blood are mixing and being pumped to the body, HLHS babies are cyanotic (blue in color) and have low oxygen levels.

Symptoms: Rapid breathing or shortness of breath, rapid heartbeat or pounding heart, poor suckling and feeding, cold extremities, blue color of the skin, lips and nailbeds (cyanosis), overall body weakness.

Treatment: HLHS is treated with a three-part open-heart surgery: the Norwood, Glenn and Fontan procedures.  HLHS is sometimes treated with a heart transplant as well.

Prevalence: HLHS accounts for approximately 2-3% of all congenital heart defects. It occurs in 2-3 out of 10,000 live births worldwide.

Comments

Julie z.

Julie z. said:

Last September, we had our 20 week ultrasound on Friday afternoon. Our Little Z was active during our visit. I remember the tech sighing that she was having a tough time getting the images she wanted, but what we didn’t know is that a 4 chamber heart view was never obtained or communicated to us. We trusted that they did a thorough check during this routine fetal anatomy scan.

We heard shortly after he earned his angel wings that Our son, Nolan, had a congenital heart defect. We had to wait 60 days to know the details of his abnormalities, which included mitral and aortic valve atresia and no left ventricle. These defects are consistent with the diagnosis of Hypoplastic left heart syndrome. He also had unusual structures of his right ventricular, right atrium and other structures.

Statistics state that the chances of having a baby with a heart defect is 1 in 100. Baby’s born with Nolan’s defect, Hypoplastic left heart syndrome (HLHS), occur 2-3 in 10,000 live births. During my correspondence with a Doctor from Boston’s Children’s Hospital and Assistant Professor of Pediatrics, he shared that HLHS is diagnosed before birth ~60-70% nationally and ~80% in the Boston area.

Because of Nolan…we want to encourage our friends, families, co-workers, and community to be more aware of congenital heart disease and what to ask about the baby’s heart during a routine ultrasound!

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