When a girl I will call Rachel was born, everyone around her was full of expectation. She was active and full of life, but as soon as the midwife grasped her, blisters arose on her newborn skin right under the hands of the midwife. And when mom held her, more and more blisters grew at the points of contact. The doctor called to examine Rachel diagnosed her with “epidermolysis bullosa,” a very rare, devastating, and untreatable disease that causes the skin to necrotize and fall off by blistering just from soft contact.
The most natural and satisfying activity for a newborn is feeding, but when Rachel tried to suck, blisters appeared on her lips, and she cried inconsolably and refused to eat further.
Medically, we were challenged by many questions: How can we support her nutrition? How can Rachel be held without provoking further blistering? But the most dramatic question was: how can we manage her pain?
Discussions among the personnel, consults with other hospitals, and Internet searches of medical literature proved futile. This disease is very rare and can present differently. Some patients will die from infection, some will improve slowly over time, some will go through life with devastating lesions, but overall there were no suggestions regarding treatment.
Although all the people involved in her care wanted the best for Rachel, we could not find a treatment that could possibly satisfy our desire for Rachel’s good.
Should we let her die by dehydration and starvation? Should we choose aggressive management and perform surgery to insert a central venous line or a feeding tube to support her nutrition, not knowing whether the wound would ever heal, adding further suffering? Should we just give her a very high dose of morphine?
I work in one of the best intensive care units for newborns in the country, where heart surgery and heart transplants are almost routine procedures, where premature babies with birth weights of one pound have the highest chance for survival in the world. However, in front of Rachel and her illness, I very strongly sensed that all our knowledge, experience, and technology were not enough; we did not know what to do and desperately needed help.
I asked for help, inviting Dr. Daniel Sulmasy, Chair of the Department of Ethics in another institution in our city, to discuss Rachel’s care. Although he came to meet with the personnel directly involved in Rachel’s care, almost all healthcare providers in our unit attended.
Even more interesting is that the most-asked question at the meeting was not, “What should we do?” but, “How do we face this suffering?” Rachel’s case was so dramatic that people were obliged to look more deeply; suddenly, the problem was no longer finding “the correct thing to do”–as unfortunately too often happens in the medical field–rather, “what is the meaning of this baby and her illness and what is the meaning of the medical care that we provide?”
After listening to our questions, Dr. Sulmasy pointed out, “Our focus is to ask what is the right and good healing act for this baby in these circumstances. As such, we could never kill her. Killing involves bringing about some new lethal state with the specific intention that she should die by way of that act.” He also addressed the point that we are all finite–including our bodies and the art of medicine. He continued, “There is a point at which we might be doing more harm than good with medicine. We must acknowledge our limits and those of the patient. From this perspective, given the way her skin is reacting to everything else, it seems that inserting a feeding tube or a central line would cause much more suffering and only prolong her dying. Therefore, foregoing extraordinary treatment is not killing. We foresee that she might die from a pre-existing lethal state, but all we intend is to withhold or withdraw a treatment that is considered a burden that outweighs the possible benefit.”
After this meeting, we came to the conclusion that our plan was to “not have any plan.” Rather, we decided to follow reality and let Rachel tell us what she needed. Amazingly, this conclusion, reached after many days of discussion and consultation, was exactly what the family had asked us to do from the start.
We noticed that Rachel enjoyed her feeds as long as she had no blisters in her mouth, so we searched hard and finally found a bottle with a very soft nipple that does not require active sucking. So she started enjoying her feeds and putting on weight.
We noticed that, if her pain was adequately managed, she was more comfortable and less agitated, with less production of blisters. We gave her pain killers by a dropper. The doses were “approximate,” not exactly what a “perfect” doctor or nurse would like, but the result was great. At about one month of life, she was able to leave the hospital for her home. Her illness remains–the drama of Rachel and her family and our own drama remains–but we found a path to walk.
I learned many things from little Rachel. First of all, we need to be humble about our technology. “Aggressive” care, sometimes, can make things worse. Everyone “believed” she would die without a feeding tube or a central line, but it did not happen. In fact, everyone is now surprised and happy that she has not died. Being sincere with our desire and our intentions allowed something unforeseen to happen.
Lastly and perhaps more importantly, I realized that reality points out questions that we cannot answer, such as, why should a baby suffer? Yet, we cannot erase the question Rachel is here to remind us of. The only possibility is to stay with her and with her mom and dad, waiting for the mystery of her life to unfold.