When Garrett came to visit his mother in the hospital the day her gallbladder was removed, he probably never imagined he would end up staying. He was a robust college wrestler, and aside from a little headache and cough, he didn’t feel very sick. But his feet had been falling asleep, and he had a tingly feeling in his hands. He’d been dragging all month, and he felt so weak that he actually fell as he was leaving the hospital.
So the doctors admitted Garrett for observation. They found he couldn’t push or pull his arms with as much strength as a normal 18-year-old, and he could barely lift his legs. When they asked him to touch his nose, his fingers trembled, and when they tapped his tendons with a reflex hammer, his muscles barely responded.
After five days, Garrett hardly had enough strength to breathe. Doctors put a tube down his throat into his airway and connected him to a ventilator. Two days later, they transferred him from the community hospital to the pediatric intensive care unit at our advanced tertiary care hospital.
To solve the mystery of Garrett’s condition, doctors gave him special tests that stimulated his nerves with tiny electric jolts to see whether the nerves’ “messages” would be conveyed with normal speed and amplitude to his muscles. They weren’t. Garrett’s arm and leg weakness and the abnormal nerve studies pointed to Guillain-Barré syndrome.
The disorder affects about one or two in every 100,000 people worldwide each year, and except for head injuries and stroke, it is the chief cause of sudden paralysis in all age groups since the advent of polio vaccine. A half century ago, poliomyelitis affected 25 in every 100,000 people in the United States and left 21,000 patients a year paralyzed. What differentiates polio from Guillain-Barré syndrome is that polio patients, mostly children, tend to develop an asymmetrical paralysis with no sensory loss. In patients with Guillain-Barré syndrome, both paralysis and sensory changes appear, ascend symmetrically from the tips of the toes and fingers to the body’s core, and then largely disappear several weeks later. Another major difference was discovered by physicians Georges Guillain, Jean-Alexandre Barré, and Andre Strohl during World War I while treating two French soldiers. They found that the fluid cushioning the patients’ spinal cord showed an abnormal increase in protein—just as with polio—but no increase in infection-fighting white blood cells. And while researchers showed that a virus causes polio, they could not find an infectious cause for Guillain-Barré syndrome.
It took another 40 years to discover what does underlie the disorder. The answer came when experimenters succeeded in creating a model for the disease in rodents by inducing an allergy to nerve tissue. Further research confirmed that in Guillain-Barré syndrome the body mistakenly attacks some aspect of the nervous system—in essence, the body develops an allergy to a part of itself.
The most common target seems to be a protein called myelin. Myelin insulates the long projections of nerve cells. It multiplies the speed with which electrical signals travel up and down the long nerve-cell axons, instantly converting a thought into a movement and a pinprick into a sensation. When the immune system attacks and destroys myelin, the impulses that control movement and sensation slow down and sometimes stop. The longer the nerve-cell axons extend, such as to reach the feet or hands, the earlier and more severely the syndrome affects them.
Like Garrett, most patients with the disorder recall having symptoms such as sore throats, fatigue, or diarrhea in the weeks leading up to their weakness. Our tests showed that Garrett recently had a common respiratory virus. The theory is that while battling any number of infections, the body’s immune system occasionally produces an infection-fighting antibody protein that cross-reacts with and destroys myelin.
Fortunately, most cases of Guillain-Barré syndrome subside. The immune system attacks the myelin and then retreats, allowing the body to regenerate the protein. The vast majority of patients regain almost full mobility and sensation within a few months.
That was little comfort to Garrett now. He couldn’t eat or breathe on his own, and he was experiencing terrible back pain.
I had other worries. Up to 10 percent of those with the condition die of complications. Along with the nerves controlling Garrett’s muscles, the nerves regulating his vital functions like heart rate, blood pressure, and temperature also malfunctioned. Often, his heart sped up to 150 beats per minute—twice his baseline—and his blood pressure shot through the roof, putting him at risk for a stroke or heart attack. We had to treat him with cardiac medication and keep a careful watch on his vital signs. When he ran a fever, we cooled him with a bedside fan. And while lying in the intensive care unit with artificial tubes and catheters in his body, he was to bacteria as a sponge is to water. We used antibiotics to control a blood infection.
Ironically, the infection resulted from a line that had been inserted into an artery during a treatment aimed at speeding up Garrett’s recovery. In a procedure known as plasmapheresis, the line was used to remove his plasma, where the dangerous antibodies against myelin are thought to reside. Only one other treatment has been shown to speed recovery from Guillain-Barré syndrome—injection of doses of immune proteins, or immunoglobulins, by vein. Culled from the blood serum of many donors, immunoglobulins may counter Guillain-Barré syndrome by decreasing the production of new antibodies and neutralizing the existing antibodies against myelin. We tried both treatments—twice—and neither helped.
The weeks passed. Garrett’s infections cleared. We established better control over his blood pressure. And then, slowly, he began to make small movements of his arms and legs. Every morning, we detected a little more strength. He still required a mechanical ventilator to help him breathe. And although he was awake, he couldn’t talk. Bright, young, and frequently frightened, he wanted to express himself. I ordered a consultation with a speech therapist, who provided a special board with letters that Garrett could use to spell out words just by looking at the letters. The painstaking process required patience.
As the end of my month in the pediatric intensive care unit drew to a close, a bed for Garrett opened in a nearby rehabilitation facility. To reduce his discomfort, we gave him a tracheostomy—a minor surgical procedure that creates a small hole at the front of the neck so that a breathing machine can be attached there. As soon as Garrett regained enough strength to spend some time disconnected from the ventilator, he could plug the hole and talk.
After we both left the intensive care unit, I thought often of Garrett, wondering how he was feeling. One day I showed up at his room and found his entire family there. Garrett had just returned from physical therapy, where he had taken his first steps. Now he was sitting up in a wheelchair, exhausted but visibly pleased. The family told me that he still required the ventilator most of the time, but he could now breathe on his own for short periods. The respiratory therapist came in and turned off the breathing machine so that Garrett could speak.
What he had to say came as a surprise. I had expected him to express frustration at his time in the intensive care unit. Instead, in a voice gentler than I’d imagined, he offered thanks to me and the other hospital staff who had helped him through the most severe period of his illness and into his period of recovery.
Sheri Fink is a New York–based physician and writer. Her book, War Hospital: A True Story of Surgery and Survival, was published in August. The cases described in Vital Signs are true stories, but the authors have changed some details about the patients to protect their privacy.