In parents’ eyes, the faintest signs of hope blur the inevitable

Not long ago, a young family sat in my office, the baby securely cradled in the father’s arms. The parents looked very young, probably still in their teens, a baby now making them a family. They waited hopefully for the words that follow most well-baby checkups. “Your baby is growing well, strong and healthy” are words that can reassure even the most anxious parents.

Today there would be no encouraging words. The infant had been born with a condition called holoprosencephaly. In the first few weeks of embryonic development, something had gone awry, leaving her with a hollow shell of a brain, capable of only the most primitive functions: regulation of heartbeat, breathing and withdrawal from pain. Usually, these infants live only a few weeks, but with the aid of a surgically inserted feeding tube, she was now several months old.

Doesn’t she need immunizations yet? the parents asked me. When will she learn to roll over? Her cousin is the same age, they said, and can roll over and hold her own bottle.

Rather than reply directly, I asked what they had been told about her condition. I assumed they had been to many other doctors -- and that they were searching for someone to offer them the antidote for this rare and terrible condition.

The couple acknowledged that they had been told their baby wouldn’t live. But look at her, they said, she’s growing and doing well -- she even smiled once.

I saw nothing that resembled a smile, nor could I conceive of its possibility. However, with the aid of her feeding tube, she was growing and gaining weight. Without it, she would have died long ago, either from pneumonia or of starvation.

I reaffirmed what they had been told. I didn’t know how long their baby would live, but most infants with this condition rarely live more than a few months. They stared at me blankly, no emotion revealed on their faces. Did they understand what I had just said?

I tried a more blunt approach. I measured her head, showing them the growth chart that demonstrated how much smaller her head was than even the smallest newborn. I told them she needed more brain to roll over, to sit up, to smile, to eat.

Can she still be immunized? was the response. They seemed to hope that she would attend kindergarten someday and that they would need to provide proof that she, like all the other 5-year-olds, had been properly vaccinated.

Weighing the options, I agreed to vaccinate their daughter. All children deserve to be protected from measles, tetanus and whooping cough.

I saw the family several times over the next few weeks. The child neither improved nor deteriorated. The parents never wavered from the stance that she was a normal baby. Only one thing hinted at their awareness of her handicap: They covered her head in a bonnet that nearly disguised her deformity and hid her from the eyes of curious strangers.

Attempting to reassure them, I held her in my arms and told them that her condition was an accident of nature and no fault of theirs. They seemed more comforted by my gesture than by my words.

Over those weeks, I treated her coughs and her colds and her diaper rashes just as I would for other infants. I gave her the prescribed immunizations. The parents beamed proudly when I reported her weight gain. When they thought they saw her smiling or reaching for objects -- something I knew was medically impossible -- I didn’t argue.

She died peacefully in her ninth month of life. I was thankful she had passed away in her own home, with her family. If her parents had brought her to me in the last hours of her life, I doubt that I would have had the courage to refrain from using life-prolonging technologies.

Although initially frustrated and perplexed by the parents’ stubborn refusal to see the inevitable, I gradually came to see that their emotions were both necessary and natural. Necessary because it freed them to provide her with the best possible care during her short life. Natural because love blinds all parents to their children’s flaws, be they trivial or fatal.

Parents’ love can obscure logical thought and transcend medical fact. It is, clearly, an expression of our humanity. When I called the family to offer my condolences, I understood that they mourned her as fully as other parents would have. To them, she was simply their child.

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Carrie Knoll is a pediatrician in Pomona.