Anya, October 2003

Anya was born at home after a perfect, uncomplicated pregnancy. Although we had an ultrasound at 19 weeks gestation, her complex defects were not detected. The technician was unable to get a clear picture of Anya’s heart because she was so active during the ultrasound, and the radiologist who examined the results was not concerned because Anya was so active and her heart rate was normal. We were told we could choose to have another ultrasound in my third trimester if we wanted to, but it was not medically necessary. We opted not to have another ultrasound because we felt that if the radiologist wasn’t worried, we shouldn’t be either.

Anya tolerated the birth beautifully and seemed perfectly healthy when she was born. She latched on immediately and nursed vigorously for 20 minutes before falling asleep in my arms. Neither our midwives nor our family doctor, who saw Anya when she was one day old, heard a murmur or noticed anything out of the ordinary. She even gained 5 ounces above her birth weight by her two-week check up, where she was declared healthy and thriving. When she was four weeks old, we realized that she had started to lose weight, but we assumed it was a problem with breastfeeding. Although she latched on well, she never nursed for more than 10 minutes at a time and soon fell asleep, so we thought she simply wasn’t getting enough milk and took steps to encourage her to nurse more frequently and for longer. At 5 weeks she had gained another 5 ounces, so we thought our corrective measures were working and that we had resolved her weight gain problems.

Fortunately, our doctor had scheduled Anya for a six-week check-up rather than a 2-month check because the clinic was recommending earlier vaccinations due to a whooping cough epidemic in our area. Our family had had a cold that week, and Anya developed a terrible wet cough the day before her scheduled check up, so I assumed she had caught the same cold her sister and I had, and decided to wait to have her doctor check it at her appointment the next day. At the check-up, I was immediately alarmed because Anya had failed to gain weight again that week. When our doctor examined Anya, she was concerned about her poor color and her wet cough and decided to check her oxygen saturation, which was only 76%. She told me she was admitting Anya to our local hospital right away, and we left the doctor’s office by ambulance. When we reached the hospital, Anya was sent to the NICU. At that point they thought she had RSV or pneumonia, but started to do a battery of tests to figure out what was wrong with her. The first test they performed was an echocardiogram, so they discovered very quickly that she had some serious anomalies in her heart, and decided to transport her to Oregon Health & Science University’s Doernbecher Children’s Hospital in Portland.

My husband and I followed the ambulance to Portland in tears. We had no idea what was wrong with Anya’s heart, whether it could be corrected or not, or whether she would survive. We were really in a state of shock, because we had believed Anya to be healthy and normal in every way. When we arrived at OHSU, the wonderful cardiologists had already diagnosed Anya’s condition and could explain not only what was wrong with her, but told us exactly what surgeries she would need immediately and in the future, and described her prognosis. She has a complex set of defects that don’t fit any one syndrome or condition, including a hypoplastic right ventricle, transposition of the great arteries, aortic stenosis, tricuspid valve stenosis, atrial septal defect and ventricular septal defect. She would need one surgery very soon, the Damus-Kaye-Stansel (DKS) procedure, and two additional surgeries later on, the Glenn at 4 -6 months and the Fontan at 2-4 years old. By the time we discovered her condition, her heart was enlarged and she was in congestive heart failure, and was now obviously very ill. I couldn’t believe how quickly she had become so seriously sick, when I had thought she just had a cold earlier that day. I kept going over and over the past six weeks trying to remember any sign of her condition, other than poor weight gain, but there had been nothing that indicated such a severe problem.

Anya had her first surgery, the Damus-Kaye-Stansel procedure, five days after her admission to OHSU. She tolerated the surgery extraordinarily well and recovered beautifully. Everyone said that she must have read the book on how to handle the surgery, and was impressed by her rapid recovery. She stayed in the hospital for two weeks after surgery, and was 9 weeks old when we left the hospital. I have nothing but praise and gratitude for the exceptional doctors and staff at OHSU, including all the pediatric cardiologists and surgeons as well as the nurses in both the PICU and the DNCC (Doernbecher Neonatal Care Center). Anya received excellent care and the entire staff was attentive to our needs and always available to answer questions and discuss decisions regarding Anya’s care.

Anya has been at home for four weeks now, and continues to thrive. She no longer requires her NG tube and takes all her feedings by bottle, although feedings are still a challenge for us as she fatigues easily and must be coaxed to finish her bottles. Her left vocal cord is paralyzed, probably as a result of the heart enlargement, and she has reflux, so spitting up is a big issue for us. However, she is gaining weight steadily and is beginning to catch up developmentally, although she still has very little neck strength and head control. She is truly an angel baby; she sleeps well and often, and when she is awake she is alert and bright-eyed, engaging everyone around her with her smiles and coos. Despite all she’s been through, she is still a happy, good- natured little girl. Though we know we will face many challenges in the months and years ahead, including two more surgeries (the Glenn in March and the Fontan in a couple of years), we are so grateful to have her in our lives. She is truly our miracle baby.