For some time, we have taken care of a little girl who was born without the use of any of her muscles. For some reason, her muscles simply failed to form properly during development. Born tiny, frail, and without any movement of her arms and legs, she immediately needed help to breathe in order to survive. Not knowing what caused her condition or how it might progress or improve, her parents and doctors made the logical decision to put in a breathing tube and to breathe for her. As time went on, she continued to have little spontaneous movement. More decisions about her care were made that would at once extend her life and compelled her family to make ever more difficult decisions along the way. She received a tracheostomy, a permanent breathing tube in her neck so the tube in her mouth could be removed. She required around-the-clock mechanical ventilation because of virtually nonexistent chest wall and diaphragm strength. A feeding tube was placed in her stomach because she was too weak to suck and would need long term artificial nutrition. Before long and almost inadvertently, she was, in layman’s terms, on full life support. This was before anyone could speak to the health of her brain, except that preliminary tests showed no significant problems.
Quite miraculously, she survived and spent months in the NICU. She was actually fairly stable by intensive care standards, and to their credit, the neonatalogists recognized that the ICU was no place to spend a childhood. Home was simply not an option. Her level of need coupled with parents’ lack of a safe home environment made going home at best a long-term goal.
She came to Ranken Jordan at about five months of age and could best be described as pathetic. Still small and frail with a normal sized head that made the rest of her body seem puny, she looked around, but more with a look of exhaustion and dismay than the inquisitiveness you’d expect from a baby her age. And she could barely keep anything on her stomach. She’d cough, gag, and vomit out almost as much food as we’d pump in. It was clear she would never grow without a fundoplication, an operation that keeps food in the stomach by wrapping part of it around the esophagus. When we consulted the surgeon to do the procedure, his response was, “What for?” as if to say, “Why bother?” He went on to say, “What kind of future does this child really have?” I was taken aback. Was he really saying that it is okay to bring all of the sophisticated resources of a NICU to bear on the survival of this infant for months, and then deprive her of the one procedure that would let her grow? What for?!
It took a face-to-face meeting to “plead her case” in which I pointed out that the tough ethical decisions had already been made for this child, consciously or not, and that it wasn’t our place to reverse course by depriving her of basic nutrition. He reluctantly consented and a week later she had her fundoplication.
Not surprisingly, she stopped vomiting and started to gain weight. That distressed look went away too. As she grew, we began to see small spontaneous movement, first in her face, then in her head, neck, and trunk. She began to really look at faces and respond to them, and she eventually began to smile.
Today, she is everyone’s favorite patient. She smiles broadly whenever she is approached. She gives kisses and scrunches her nose. She listens to Disney tunes all day and whether lying in bed or up in her wheelchair, she rocks her little body as if dancing to the music. When we put a speaking valve in her vent circuit, she has a lot to say, even though it is only baby babble. Except for her physical limitations and her dependence on technology, she is a normal little girl. She is unencumbered by her limitations and inspires us to forget our own. She has a big heart, a mind, and a soul. She is her parents’ pride and joy and as soon as they are able, she will be at home with them. That’s what for.
by Dr. Nick Holekamp, Chief Medical Officer