Hailey, 6 years old

Our daughter, Hailey Marie, was born on March 9, 2000. The newborn nurse detected her heart murmur and tried to explain how common this was after birth. Excited about the arrival of our baby girl, we snuggled with her and were given some private time together. Later, the nurse was concerned about Hailey’s blue appearance and soon we were informed that she was in the Neonatal Intensive Care Unit.

In the NICU, Hailey was connected to many monitors and her medical studies were being sent to a children’s hospital. We were so frightened as we waited for their diagnosis. They soon determined that Hailey had Tetralogy of Fallot (a combination of four heart defects) with absent pulmonary valve syndrome and a small atrial septal defect.

We knew very little about the heart and were devastated with the news. We cried many tears, but knew we had to find out as much as we could before the pediatric cardiologist examined Hailey. Three weeks later, Hailey underwent open-heart surgery. Our research prepared us for what needed to be done to Hailey‘s heart, but nothing could prepare us for the devastation we felt during this time! Hailey was a strong little girl and recovered seven days in the Pediatric Intensive Care Unit and four days in the Moderate Care Unit.

Going home felt so good! We were reunited with Logan, our two-year-old son, and we felt such a sense of relief knowing Hailey was in a stable condition. Throughout the next year, Hailey started showing symptoms that her energy level was weakening. Once again, fear and devastation overcame us when the cardiologist reported that Hailey needed surgery to replace her homograft (human donor) heart valve. Hailey recovered quickly this time and was home on her second day after open-heart surgery!

Hailey continues to be full of energy and exuberance. We are thrilled to watch her grow and discover the world! She reminds us everyday that life is precious and we are thankful for all the people that have contributed to her life.

July 2006 Update

Hailey, 2-1/2 years old

Hailey has been doing very well since the last update. She had a great year in kindergarten with some respiratory illnesses and tons of sinus infections. The use of a nebulizer and inhaler “tamed” the respiratory issues and we hope Hailey’s surgery to remove her very large adenoids will prevent future sinus trouble!.

Her visits to the cardiologist have typically showed good heart function. The echocardiograms detected slight backflow from the homograft pulmonary valve and slight enlargement of the right ventricle. So you would think that we should be sailing along thinking that "things" are good. Right??

But that is never really something that we can do! Having a replaced heart valve means that there will be more future surgeries! And that is exactly where we stand today.

Hailey’s first cardiac MRI showed a significant difference in Hailey’s heart condition. Briefly, Hailey’s right ventricle has almost doubled in size, the leaflets of the homograft pulmonary valve/artery have totally degenerated (disappeared) and the pulmonary branch arteries are still severely dilated. The medical staff that follows Hailey recommends close monitoring. Soon she will be having a chest x-ray to check the right ventricle for further enlargement and an EKG to check for any rhythm changes.

So, a third pulmonary valve replacement is in the very close future! The good thing is that you would never know Hailey’s heart is in need of more repair. She continues to be energetic and full of spunky spirit! However, little changes are noticeable: the need for more sleep, becoming winded more easily, and a cough that is in need of increased treatments.

Our hope is that Hailey has a successful year in first grade and that her heart does not slow her down too much!

October 2007 Update

Hailey had a rocky year in first grade with several sinus and respiratory infections; many hospital and doctor visits; and a plethora of antibiotics and testing for asthma and allergies. Our frustrations increased!

We then sought second opinions from another institution. Diagnosis: enlarged right heart, moderate pulmonary regurgitation with moderate stenosis in the main pulmonary artery and severely dilated branch pulmonary arteries that were compressing Hailey's right and left main stem bronchi yet she continued to have good heart function. The near complete airway blockages had caused recurrent lung infections which resulted in a serious condition called bronchiectasis. (Hailey's congenital cardiac lesion, absent pulmonary valve syndrome, most likely caused the enlarged branch pulmonary arteries which had never been addressed surgically. Plus with an enlarging heart, space in Hailey's tiny body was getting cramped!)

The pulmonologist and cardiologist recommended immediate pulmonary artery reduction (plication) to correct the airway compression along with a pulmonary valve replacement. After 2.5 weeks of home IV antibiotics and doing chest physical therapy to clear Hailey's lungs of infection, her third open- heart surgery was performed. Hailey's homograft pulmonary valve was replaced with a porcine (pig) valve contained in a Dacron conduit and both her left and right branch pulmonary arteries were plicated (reduced) to half their original size which was 22 & 24 mm. Surgery and recovery went great with only one minor complication called clostridium difficile.

We are so thankful for obtaining second opinions! Currently, Hailey is 4 months post-surgical repair and doing quite well. This time we hope she has many healthy school years!!