J and his father have inserted themselves deeply into the hearts of all who care for them. Their faces drift into consciousness even when we are not at the hospital: on the drive home, in line at the grocery store, on a morning walk. We all read and re-read J’s medical history searching to understand the anatomy of his exhausted body, to understand where this germ lurks unseen. We consult the sparse medical literature describing cases of refractory infections to guide us in constructing an effective treatment plan that keeps side effects to a minimum.
Despite the difficulty eradicating this cruel infection, J and his father are patient and courageous. Bad news—of which there has been plenty—is met with disappointment but remarkable equanimity. J attends “hospital school” every day, where he is a good student. He remains polite and respectful to all of his caregivers. He is such a brave little boy. He cries only in anticipation of the painful but necessary changes of his bandages. His beautiful smile, sense of humor, and kindness lighten our days and lift our ache. His father is a thoughtful advocate for his son’s care and his calmness settles like a blessing on our shoulders. We ask them, how is it possible to suffer so, to live with setback after setback, but still convey such extraordinary light and love to each other and to all of us who meet you? Their answer of course is that for them there is no other choice except to respond with grace—and one can only imagine how difficult this must be.
At our weekly clinical conference, one of our fellows, a trainee, spoke of J. She reported another major procedure on the immediate horizon to remove bone pieces from J’s forehead in the persistent battle to eradicate this infection. To conclude her presentation, our trainee said, “Their patience is a miracle.” And so it is. For the surgical and medical teams working alongside J and his dad, struggling to accomplish what we want for them, their grace and courage are truly miraculous and inspiring.
Postscript: It is now over a year since this essay was originally written and, much to our great joy, J is doing well. Although we still follow him for any setbacks, he has had none. He has not required additional surgery or new therapy in all that time, and it is our great hope that the grace and patience he and his father have shown will finally herald a cure.
Date of event: 1981
Unequal Twins
Edward J. Goldson, MD
Ah, little person, you are trying so hard! Sean was one of fraternal twins born at about thirty-two weeks gestation, two months premature. Although he was a little bigger than his twin sister, he struggled more medically. At one point he required help breathing with a mechanical breathing machine, had an ongoing dependence for additional oxygen, did not feed well, and had trouble maintaining his temperature. In contrast, his twin sister was a superstar, breathing room air, feeding normally, and in an open crib growing just as expected.
Sean’s parents and older sister were devoted to the twins, but Dad had a special relationship to Sean. Dad had always wanted a son and was very excited about the baby. He also knew that this would be his wife’s last pregnancy as she had been very ill carrying these babies.
Time passed and Sean’s twin sister was discharged home, now at the healthy weight expected for a full-term baby. She was healthy in every other way also. Sean, however, continued to have challenges with breathing, weight gain, and temperature control. It also became apparent to those of us caring for Sean that his father was also having difficulties with his son’s slow prog-ress. Dad began to visit Sean less frequently. This can mean many things in the context of a sickly baby, but those of us caring for Sean were concerned that his father was disengaging from the baby and wondered how he would accept the infant once he came home. Would Sean meet his father’s hopes and expectations?
To our collective relief, Sean finally began making progress. He was able to maintain his temperature, began eating better and gaining weight, seemed more alert, and was able to wean from his supplemental oxygen. At discharge time we gathered the family together. As part of the discharge protocol, I performed a complete neonatal assessment and physical examination of Sean with his family attentively observing. It was a wonderful time, but a scary time for the parents. Would the assessment find Sean truly ready to go home? The excitement was palpable as Sean was able to accomplish all of the tasks presented to him and he looked and acted like a healthy baby. It was hard to believe, considering how difficult the days and weeks before had been.
At the end of the assessment Sean was wrapped snugly in a warm blanket and presented to his father, a happy day for all. Afterward, we saw him only once or twice in follow-up as the family lived far from the hospital. Several years passed and the family was in Denver for a visit and happened to be in the lobby of the hospital just as I was. We had a warm reunion filled with hugs and greetings. As the kids’ mother wandered off in the lobby to corral the children, their father turned to me and asked if I remembered that “exam” I had performed the day of Sean’s discharge. Of course I did!
Father turned to me and said, “I don’t know if you realize it, but that day you give me my son.” My sense was, until that examination demonstrating Sean’s wonderful and hard-earned abilities, he feared he would be taking home a son who was not “normal”; a child who would not go fishing or play ball with his father or participate in all the father-son activities he looked forward to sharing with his son.
And then, as if on cue, Sean came running down the hall to his beaming dad, kicking a soccer ball, a perfectly normal miracle of a child. I was almost as proud as Sean’s dad, proud at how we had all worked together to help establish this healthy, loving family.
It’s often hard to know the exact ingredients that go into a dramatic medical turnaround like Sean’s, but I was grateful to have been there to see it.
Date of event: 2002
Maybe Justin Will Be Different
Jeffrey S. Hyams, MD
I remember it was a Friday afternoon, ending a very long and difficult week. I was sleep-deprived, crabby, and just generally not in the mood to be cheerful. I am a gastroenterologist, a specialist in stomach and intestinal disorders, and when my beeper went off informing me of a consult in the pediatric intensive care unit (PICU) I knew it wasn’t going to be good news. Just a name and age appeared, with no clinical details: Justin, three years old. I went up to the third floor, swiped my badge, and entered the eighteen-bed PICU that usually presented me with sensory overload. I opened the chart to read about a young boy, totally well until a few days ago, who had developed abdominal pain and vomiting. He had initially been felt by his family to have a viral illness, especially after receiving phone reassurance from his doctor. But when his condition worsened, the family took Justin to the local emergency room. Justin couldn’t stop vomiting, the pain got more intense, and he became listless.
After a number of hours there, with his clinical condition deteriorating, he was transferred to our hospital where further testing suggested a bowel obstruction. He was brought to the operating room in the middle of the night and found to have a volvulus, with massive bowel damage caused by lack of blood flow to the bowel. A volvulus occurs when the intestine twists upon its nutrient blood supply and is deprived of oxygen. In young children, a con-
genital abnormality of the arrangement of intestinal loops, called malrotation, predisposes to volvulus. While some bowel loss is survivable, Justin had lost over 90 percent of his small intestine and was left with very little hope. He was unstable with low blood pressure, poor urine output, and fever. The immediate prognosis was guarded at best, and long-term it seemed unlikely he would ever be able to survive independent of intravenous (given into a vein) nutrition or without a bowel transplant.
After finishing my chart review I entered Justin’s darkened room, illuminated only by the green glow of the monitor. His little body rhythmically moved slightly with each breath entering his lungs from the ventilator (breathing machine). All of his muscles were paralyzed so he wouldn’t fight back agai
nst the breathing machine that was keeping sufficient oxygen flowing to his body. Multiple intravenous catheters (tubes in his veins) were giving him fluids, blood, antibiotics, and powerful medicines to sustain his blood pressure, which was dangerously low.
Huddled in the corner of the room were his parents, trying to understand why their normal toddler of several days ago was now desperately fighting for his life. My unenviable job was to tell them that even if he survived the present situation he was likely to require difficult, complicated, and expensive care for the rest of his life, and even then he had a good chance of dying. So I took a deep breath, introduced myself, and sat down beside them. I explained he had lost a large amount of his bowel, it was unlikely he would ever be able to sustain himself and grow normally eating food by mouth again, and he would be dependent upon intravenous nutrition. He would be prone to having serious infections with an intravenous catheter in his veins, and long-term intravenous nutrition (called hyperalimenation fluid) could cause his liver to fail. One day, if we were lucky, he would have a small bowel transplant and then would require anti-rejection medications the rest of his life. The five-year survival for small bowel transplantation at that time, 2002, was at best 50 percent. But, like I always did during these conversations, I never removed hope that maybe Justin would be different. I told them that even though I could not honestly be optimistic at that moment, surgery and medicine are powerful tools with which we’d try to make him better. I added that sometimes miracles happen and events turn out different than we expect.
When I returned to the hospital on Monday, I went to the ICU first and talked to the resident (physician-in-training) on call to hear about the weekend. She told me that Justin had done surprisingly well and was already being weaned off the ventilator. Over the next couple of weeks Justin had the usual setbacks of fevers, blood test abnormalities, and the like, but overall his progress was strong. As the acute surgical care was ending, the bulk of Justin’s intestinal “rehabilitation” now fell into my lap as the pediatric gastroenterologist. I have always considered short-gut syndrome, the condition Justin now had after most of his intestine had been removed, to be the epitome of intellectual challenge of my field. I have referred to it as the “champagne” of pediatric gastroenterology. Expertise in nutrition, gastrointestinal and liver physiology, kidney function, infectious diseases, and psychology are all required to optimize the care of these children.
So we started the process slowly by giving very small amounts of a specialized formula, which has already been predigested, through a tube inserted in the stomach directly through the abdominal wall (called a gastrostomy). This technique, known as trickle feeds, exposes the minimal remaining intestine to key nutrients and stimulates the growth of new intestinal cells. Our only hope was that over the ensuing years a process called intestinal adaptation would allow the intestine to grow in length (he was only three years old so there was still time for growth) and, more importantly, the remaining surface of the intestine would increase its ability to absorb nutrients.
Justin went home and I saw him weekly for the next several months. Our progress was quicker than I expected, and by six months he was receiving about 40 percent of his daily calories through the gastrostomy tube and the rest intravenously. Being a normal toddler, he wanted to eat but we had to be very careful because even small amounts of regular food caused him to get severe diarrhea. He didn’t have sufficient intestinal cells to digest and process regular food. At every visit Justin’s family asked if he would ever get off the intravenous nutrition and my reply was I wasn’t sure, but he was already doing better than I ever expected.
Justin grew, gained weight, and became an otherwise normal child. At age nine, defying any sane prediction of his eventual course, we were able to remove the intravenous catheter through which he had been receiving nutrition and sustain normal growth on the gastrostomy tube feedings into his stomach. We were even able to allow him small amounts of regular food by mouth. The removal of the intravenous catheter was a momentous event, a cause for celebration, as he now had the opportunity to go into the ocean with his family when they traveled to the beach each summer. To Justin, the ability to swim in the ocean was more important than this newfound intestinal freedom.
He did well for a number of years, but at age thirteen his growth rate started to slow and he was not keeping up with his peers. It appeared as if the amount of nutrition he could absorb was not enough to foster a growth spurt associated with puberty. After long negotiation we finally were able to convince Justin, his family, and ourselves that an intravenous catheter needed to be reinserted for extra calories. It was, and things went well for about two months before he developed a clot in the vein in which the catheter was placed and the catheter had to be removed. The clot was large enough that his face swelled and we had to give him powerful medicine to help dissolve the clot. But he got through it fine and we finally were able to adjust his feedings to get him to slowly gain weight again.
So now we are a dozen years following that eventful day when I first met Justin and his family. As a fifteen-year-old now, he has not only defied all of my initial predictions but thrown them far, far away. Justin has survived, and even thrived, a bright light at the end of what has been a very long tunnel for him and his family.
I have taken care of other children in similar circumstances, used the same passion and knowledge on their cases, and yet none have had this degree of medical success. Indeed, Justin did turn out different than what we expected. I still don’t know why he has been different than the rest. It wasn’t anything I did differently or more brilliantly.
As I told the family those many years ago, sometimes miracles happen.
Date of event: 1985
My First Patient
Robert J. Buys, MD
At last I had finished all my education. Four years of medical school, a year of internship, three years of residency in ophthalmology (the specialty providing medical and surgical care of the eyes), and two years of subspecialty fellowship training in diseases and surgery of the retina and vitreous had prepared me for that first real day of my professional career. Or so I thought.
I will never forget my first patient.
Kim was twenty-four and blind when we first met. She had given birth to a healthy baby boy a year before and never seen his face. The pregnancy had caused her diabetes to go on a rampage and resulted in a sudden loss of vision. Diabetes can have catastrophic and often permanent consequences to the eye. It does this by destruction of the blood supply to the retina, the lining in the back of the eye required for vision. As the blood vessels die away, the eye will try making new vessels to replace them. Unfortunately, these new blood vessels do not restore the lost blood supply and can break, causing bleeding into the jelly of the eye (called the vitreous).
Or worse, the blood vessels can scar and pull on the underlying retina, causing detachment, which was the case with Kim. One eye had this so-called tractional retina detachment, and such significant loss of blood supply I considered it hopeless. The other eye was not far behind; diabetic retinopathy is nothing if not democratic, affecting both eyes equally given enough time. In this race against time I was losing—badly.
I tried to operate on the worst of Kim’s two eyes and the result was what I’d feared. Even though I was able to get anatomical success, reattaching the retina to the back of the eye, the retina had no blood supply and the eye quickly became painful and unable to see light. Reluctantly, I had no choice but to remove the eye.
That still left her with one eye, even though she could barely detect light. We had to go back to the OR and try to salvage it. My expectation was that with or without surgery the chance of success was grim. The night before the surgery she called me, hysterical—how could she go ahead with surgery when the other eye had done so badly? I rushed to the hospital and sat by her bedside. I held her hand and did my best to reassure her this time would be different. Bu
t who was I fooling? I knew her only chance for any vision at all was surgery, but in my heart I agreed with her—it was undoubtedly futile.
The morning of surgery arrived and we quietly went together to the OR. I held her hand again and whispered reassurances to her as the anesthesia took its effect. I took a moment before the surgery began to compose myself and prepare for the test ahead.
By end of the surgery I felt I had done as well as I could, but my assistant, who was also a retinal surgeon, was convinced we had lost the eye. I didn’t know it then but he had gone out to Kim’s parents in the waiting room and informed them of the expected bad result.
The memory of the next morning when I made rounds will be with me forever. I can still see her freckled face and strawberry blond hair, feel her sweet demeanor and the warmth of her personality all these years later. She was rubbing the edge of a coffee cup, turned to me and asked: “Does this mean I will be blind for the rest of my life?”
I could not bring myself to tell her what I feared. “I am not giving up, let’s wait and see what happens.” It was as honest a response as I could come up with.
The next few weeks were the hardest. She would come to the office disheveled, depressed. Her vision remained limited to barely seeing hand motions, but the front of the eye remained free of new blood vessels—a good sign—and the pressure inside her eye remained normal, also a good sign. She asked if it would be okay for her to call me at home if problems arose, and I quickly agreed. And she did call—whenever she was afraid, or just needed to cry and release her emotions. I did not want to be anywhere else but at the other end of the phone in those dark and difficult times. To my mind, being there for the hard times with your patients is what being a doctor is all about. When she hung up after those calls I cried, too.
Miracles We Have Seen Page 36
