By Grace O.
A mother, who found out her unborn baby had Hypoplastic Left Heart Syndrome (HLHS), decided to carry him to full term. But she did not have him undergo the series of surgeries that might have saved his life. She said, “You must believe that the decisions you are making are the right ones for you, your baby, and your family; founded in love, they cannot be wrong.”
Although my choice was different from hers, I agree with her statement. When it comes to parental decisions, and there is no one-size-fits-all correct choice. It is all too easy to judge another’s choice or to think you “know” what you would do in a given situation. Unfortunately, it is not all black and white.
I was 17 weeks along in my second pregnancy in January 2000, when I learned, during a routine level II ultrasound, that my baby probably had HLHS. The perinatologist recommended that we do an amniocentesis to rule out any other problems. I asked him if the heart defect could indicate a chromosomal problem. He assured me that it did not, but said I should have the amnio just in case. I agreed.
Both the doctor and his nurse promised to contact me the minute the amnio results came back.
My immediate reaction was that we must do anything we could to give our baby every chance to live. I knew he would always be a heart invalid, but as long as he had any chance at growing into an independent person, I would do everything in my power to help him get there. I began researching on the Internet and at our local libraries. I learned everything I could about congenital heart defects, HLHS, and the Norwood series of surgeries that had been developed to save the lives of these children. The surgical series essentially re-plumbs the heart, and when it is successful, theoretically the child may survive to near adult size before needing a heart transplant. It is common for Norwood series survivors eventually need a heart transplant.
Less than week later we were at a major teaching hospital having a fetal echocardiogram. At that visit, we asked to know the sex of our baby because we felt the heart defect had been enough of a “surprise” to us. We felt that by knowing more, we could better pull for our baby’s health.
The technician said “I see a flagpole. It’s a boy!”
When the surgeon came in to view the fetal echo, she confirmed that our son had HLHS. I couldn’t hold back my tears. I had been hoping against hope that it would turn out to be something less severe than HLHS. I would have settled for anything with a higher probability of survival – or at the very least something that wouldn’t require open-heart surgery during the first few days of life.
Then the other shoe dropped. The surgeon told us that it wasn’t just HLHS: My baby had an additional heart defect; a large Atrioventricular Septal Defect (AVSD), also known as an endocardial cushion defect or AV canal defect. His was quite large.
When she told me that, the first thing I did was ask her if that could be an indicator of a chromosomal problem. She assured me that it absolutely was not.
I would later learn that she had just told me a bald-faced lie.
Back home again, we began planning for our son’s birth. We could no longer plan on having him locally. Our baby would need to be born at the teaching hospital where we’d had the echocardiogram—a two-hour drive from home. I called a cousin who lived closer to the hospital to ask if we could stay with her as the due date neared to make sure we’d get there on time. We talked about whom would care for our then two-year-old daughter.… and how she would handle the long separation from us as we stayed near the hospital for the baby. We talked about selling our house right away because we would no longer be able to afford it with all of the expenses associated with seeing our son through the surgeries. Nothing seemed to matter except how we would keep him alive.
We comforted ourselves that if he did die, we would donate whatever organs of his we could so that some other family might at least benefit from our tragedy.
I was so worried I no longer slept at night. One night I was up poring over a book about congenital heart defects when I stumbled upon a fact that hit me like a cast iron frying pan to the face: “AVSD is strongly associated with Down syndrome.”
I read that line over again and again in disbelief. Why had both of my doctors lied? To this day, I do not know. What I do know is that their breach of honesty played a role in my ultimate decision not to trust them with my baby’s care.
Early the next morning, 10 days after the amnio, I called the perinatolist’s office to find out if my amnio results had come back. The nurse said the doctor wasn’t in, and claimed “we haven’t had any amnio results come back this week.”
Desperate, I called my OB’s office next, hoping that perhaps they had the AFP results back by now. That might at least give me a clue as to what lay ahead. My OB’s nurse got on the phone and said “The perinatologist sent us your amnio results yesterday. I can’t believe they didn’t tell you. I am so sorry—your baby has trisomy 21 in every cell.”
I felt the floor drop out from under me. I thought of my toddler downstairs and grabbed a pillow to cover my face before I screamed “No!” and sobbed. Didn’t my baby have enough problems already?
Forty-five minutes later my friend showed up for a trip we had planned to take to the bookstore. She knew about the heart defects and was being very supportive. I was silent in the car, knowing I could not repeat the news my OB’s nurse had just given me without breaking down and alarming our toddlers tucked safely in their carseats. At the bookstore, I asked her to watch my daughter and sneaked over to the “special needs parenting” section. I found the book Your Child with Down syndrome and took it to the checkout.
Later, in the car, she asked me why I was so quiet. I could barely get the words out. I said “I got the amnio results” and pulled my new book out of its bag. She hugged me and we both cried.
That afternoon I felt—strange as it seems now—like the baby had played a trick on me. I resented him for not being normal. I was angry, having been through so much emotionally for him already. This was too much. I needed to clear my head, so that night I took a long soak in a deep bath. As the water dripped from the faucet, my anger slowly washed away. This little baby had not set out to trick me. It wasn’t his fault he had Down syndrome. He was still my little boy and he still needed my love and care. My heart softened and I decided at that point to continue the pregnancy.
I devoured my new book about Down syndrome, feeling overwhelmed by the challenges this baby would bring. Although several of the stories in that book talked about heart surgeries and heart defects so common to children with Down syndrome, none were nearly as severe as the heart defects our baby was afflicted with. When I read about HLHS, none of the tiny survivors were children with Down syndrome.
I didn’t know what to think anymore. I called our surgeon at the teaching hospital and asked her what she thought the prognosis was, given our baby’s additional diagnosis. She hemmed and hawed, and finally, sounding too much like a teenager trying to explain why she was holding a cigarette, made the nonsensical statement “Well .… that left ventricle could grow.”
Frankly, I was stunned. I’m no doctor, but I know enough about biology to know that symmetrical body parts do not grow asymmetrically—in utero or anywhere else. I realized this was the second time she had lied to me. I asked her why she had said the AVSD was “unrelated” to chromosomal defects when in fact is was a marker for them, and she claimed that she thought the HLHS somehow cancelled that out.
I asked her if he would ever be a candidate for a heart transplant, and she said no— they don’t offer them to children with Down syndrome. I asked her if we could have the baby and let him die peacefully without surgery (this is called comfort care), then donate his organs to help other babies in need. Again, she said no. They didn’t accept organs donations from babies with Down syndrome.
Furthermore, she added that after he was born, whether or not to do the surgeries would ultimately be up to the hospital, not us.
She could not promise me that he would be allowed to die peacefully. No family in need could benefit from our tragedy via organ donation. The surgeon’s words had completely drained me of my last, desperate hope.
I began to think about whose hands I would be putting our baby into, and why. I realized it wasn’t about my ability to be “strong.” The reality hit me that my baseless hope for a medical miracle would result in his pointless suffering.
I would not be the one undergoing risky surgeries. I would not be the one whose best hope in life would be as a mentally disabled heart invalid, one who – even if he somehow defied every odd against him and survived – would be denied a heart transplant (which HLHS patients need eventually as the small, “replumbed” heart cannot meet the demands of a growing body). I applied the golden rule and thought about what I would want my mother to do, were I the one in my baby’s position. I realized I would want to be spared all of that and allowed to go peacefully.
We chose a labor and delivery termination at 20 weeks. Our baby was born silently and knew not a moment’s suffering.
Today, I never second-guess the parental decisions of others facing poor prenatal diagnosis. I know first hand how it feels to decide to have the baby and pursue surgery; to decide to have the baby and opt for comfort care; and ultimately, to decide that ending the pregnancy is sometimes the most compassionate choice a mother can make under hopeless circumstances.
We still miss and love our baby, even knowing that our decision was the right one for him and our family.