That might have been the end of Maranda’s story except for her tenacious parents. Terry Francisco checked on every lead she could find. As soon as she heard of anyone who was an expert in the field of seizures she made contact. When this person couldn’t help her, she would say, “Do you know anyone else? Anyone who might be of some help to us?” Someone finally suggested she contact Dr. John Freeman at Johns Hopkins because of his well-earned reputation in the area of seizures. By phone Terry Francisco described everything to the pediatric chief of neurology. When she finished, she heard the most encouraging words she had received in months. “Maranda sounds like she might be a good candidate for a hemispherectomy,” Dr. Freeman said.
“You mean it? You think—you think you can help?” she asked, afraid to use a word like cure after so many disappointments.
“I think there’s at least a good chance,” he said. “Send me her records, CT scans, and anything else you have.” John had been at Stanford University Hospital before hemispherectomy fell out of favor. Although he had not performed any himself, he knew of two successful hemispherectomies and was convinced that they were viable surgical options.
Hardly daring to hope, Maranda’s mother copied all the records she had and mailed them that same day. When John Freeman received the material, he studied everything carefully, then came to see me. “Ben,” he said, “I’d like you to take a look at this.” He handed me the records, gave me a chance to study them thoroughly, and then said, “There is a procedure for a hemispherectomy that I know you’ve never heard of —”
“I’ve heard of it,” I said, “but I’ve certainly never done one.” I had heard of it only recently when, in looking up some other material, I flipped through a medical text and saw the material about hemispherectomy and skimmed it. The information didn’t offer much optimism about such surgery. “I believe a hemispherectomy could save this child,” Dr. Freeman told me.
“You honestly have that much confidence in the procedure?”
“I do.” His eyes held mine. “Do you think you could do a hemispherectomy on this girl?” he asked. While I considered how to answer, John went on to explain the rationale behind his faith that such a surgical procedure could be done without terrible side effects.
“Sounds reasonable to me,” I answered, growing excited about having a challenge. However, I wasn’t going to jump into some new kind of surgery without more information—and John Freeman wouldn’t have wanted me to anyway. “Let me get hold of some of the literature and read up on it, and then I can give you a more informed answer.”
Beginning that day, I read articles and papers that detailed the problems causing the high complication rate and mortality. Then I did a lot of thinking about the procedure and examined Maranda’s CT scans and records. Finally I was able to say, “John, I’m not sure, but I think it’s possible. Let me consider it a little more.”
John and I talked and continued to study the records, and finally he phoned the Franciscos. Both of us talked with Mrs. Francisco and explained that we would consider doing a hemispherectomy. We made her no promises, and she understood that.
“You bring her for us to evaluate,” I said. “Only then can we give you a definite answer.”
I was eager to meet Maranda and happy when a few weeks later her parents brought her to Hopkins for further evaluation. I recall thinking how pretty she was and felt such a heaviness for the child. Maranda, then 4 years old, was from Denver, and she used to say, “I’m from Denverado.”
After extensive tests, a lot of conversation with John Freeman and a few others I consulted, I was finally ready to give them my decision. Maranda’s dad had flown back home to work, so I sat down with Terry Francisco. “I’m willing to attempt a hemispherectomy,” I told her. “But I want you to know that I’ve never done one before. It’s important that you understand —”
“Dr. Carson, anything—anything you can do. Everyone else has given up.”
“It’s a dangerous operation. Maranda may well die in the operating room.” I said the words easily enough, but I also sensed how terrible they must have sounded to that mother. Yet I felt it was important to give her every negative fact. “She might have significant limitations, including severe brain damage.” I kept my voice calm, not wanting to frighten her, but I also didn’t want to give her false hope.
Mrs. Francisco’s eyes met mine. “And if we don’t agree to the surgery, what happens to Maranda?”
“She’ll get worse and die.”
“Then it’s not much of a choice, is it? If there is a chance for her—even if a small chance — “ The earnestness of her face showed clearly the emotion she had gone through in arriving at her decision. “Oh, yes, please operate.”
Once they had agreed to the surgery, Terry and Luis sat down with their daughter. Terry, using a doll, showed Maranda where I would be cutting into her head, and even drew lines across the doll. “You’ll also end up with a really short haircut.”
Maranda giggled. She liked that idea.
Certain her daughter understood as much as she was capable of at 4 years of age, Terry said, “Honey, if you want anything special after the operation, let me know.”
Maranda’s brown eyes stared at her mother’s face. “No more seizures.”
Tears shining in her eyes, Terry embraced her daughter. She held her as if she could never let her go. “That’s what we want too,” she said.
On the night before surgery I walked into the pediatric playroom. Mr. and Mrs. Francisco were sitting on the edge of the play pit, a special area that the children especially enjoy. A little giraffe on wheels stood across the room. Trucks and cars were scattered around the floor. Someone had lined stuffed animals against one wall. Mrs. Francisco greeted me calmly, cheerfully. I was amazed at her calmness and the brightness in her eyes. Her serenity encouraged me to know that she was at peace and ready to accept whatever happened. Maranda played with some toys nearby.
Although I had warned them of the possible complications of surgery at the time they consented, I wanted to make certain they heard everything again. I sat on the edge of the play pit with the couple and carefully, slowly, described every phase of the surgery.
“You’ve obviously had some information already about what we need to do,” I said, “because you talked to the pediatric neurologist. We expect the surgery to take about five hours. There’s a strong possibility that Maranda could bleed to death and die on the table. There’s a chance that she’ll be paralyzed and never speak again. A multitude of possibilities exist of bleeding and infection and other neurological complications. On the other hand, she might do very well and never have seizures again. We don’t have a crystal ball, and there’s no way to know.”
“Thank you for explaining,” Mrs. Francisco said. “I understand.”
“There is one more thing we do know,” I added. “I’d like you to understand that if we don’t do anything her condition will continue to worsen until you can’t keep her out of an institution. And then she will die.”
She nodded, too emotional to risk speaking, but I realized she had fully grasped what I said. “The risk for Maranda is compounded,” I went on. “The lesion is on the left side—her dominant half of the brain.” (In most right-handed people, the left hemisphere dominates speech, language, and movement on the right side of the body.) “I want to emphasize,” I said, and paused, wanting to make certain they fully understood, “the major long-term risk, even if she survives surgery, is that she’ll be unable to talk, or she might be permanently paralyzed on the right side. I want you to be clear about the risk you’re facing.”
“Dr. Carson, we know the risk,” Luis said. “Whatever is going to happen is going to happen. This is our only chance, Dr. Carson. Otherwise she might as well be dead now.”
As I stood to leave, I said to the parents, “And now I have a homework assignment for you. I give this to every patient and family member before surgery.”
“Anything,” Terry said.
“Say your prayers. I think that really does help.”
“Oh, yes, yes,” Terry said and smiled.
I always tell parents that because I believe it myself. I’ve not yet had anybody disagree with me. While I steer away from religious discussions with patients, I like to remind them of God’s loving presence. I think what little I say is enough.
I was a little anxious as I went home that night, thinking about the operation and the potential for disaster. I had talked about it with Dr. Long, who told me he had once performed a hemispherectomy. Step by step, I went over the procedure with him. Only later did I realize that I hadn’t asked him if his one surgery had been successful.
So many things could go wrong with Maranda, but I had come to the conclusion years earlier that the Lord would never get me into anything He couldn’t get me out of, so I wasn’t going to spend an excessive amount of time worrying. I’ve adopted the philosophy that if somebody is going to die if we don’t do something, we have nothing to lose by trying. We surely had nothing to lose with Maranda. If we didn’t proceed with the hemispherectomy, death was inevitable. We were at least giving this pretty little girl a chance to live.
I finally said, “God, if Maranda dies, she dies, but we’ll know that we’ve done the best we could for her.” With that thought I had peace and went to sleep.
* The procedure known as hemispherectomy was tried as long as 50 years ago by Dr. Walter Dandy, one of the first neurosurgeons at Johns Hopkins. The three biggest names in neurosurgical history are Harvey Cushing, Walter Dandy, and A. Earl Walker, who were, consecutively, the three people in charge of neurosurgery at Hopkins dating back to the late 1800s.
Dandy tried a hemispherectomy on a patient with a tumor, and the patient died. In the 1930s and 1940s a number of people started doing the hemi- spherectomy. However, the side effects and mortality associated with the surgery were so great that hemispherectomy quickly fell out of favor as a viable surgical option. In the late 1950s the hemispherectomy reemerged as a possible solution for infantile hemiplegia associated with seizures. Skilled neurosurgeons started doing the operation again because they now had the sophisticated help of EEGs, and it seemed in a lot of patients that all the abnormal electrical activity was coming from one part of the brain. Although the results of previous hemispherectomies had been poor, surgeons felt they could now do a better job with fewer side effects. So they tried and did at least 300 of the surgeries. But again, the morbidity and mortality turned out to be high. Many patients hemorrhaged to death in the operating room. Others developed hydrocephalus or were left with severe neurological damage and either died or were rendered physically nonfunctional.
In the 1940s, however, a Montreal doctor, Theodore Rasmussen, discovered something new about the rare disease that affected Maranda. He recognized that the disease was confined to one side of the brain, affecting primarily the opposite side of the body (since the left side of the body is controlled mainly by the right side of the brain, and vice versa). It still baffles doctors why the inflammation remains in one hemisphere of the brain and doesn’t spread to the other side. Rasmussen, who had long believed that the hemispherectomy was a good procedure, continued to do them when virtually everyone else had stopped.
In 1985 when I first got interested in hemispherectomy, Dr Rasmussen was doing a diminishing number and recorded quite a few problems. I suggest two reasons for the high failure rate. First, the surgeons selected many inappropriate patients for the operation who, consequently, did not do well afterward. Second, the surgeons lacked competence or effective skills. Again the hemispherectomy fell out of favor. Experts concluded that the operation was probably worse than the disease, so it was wiser and more humane to leave such procedures alone.
Even today no one knows the cause of this disease process, and experts have suggested possible causes: the result of a stroke, a congenital abnormality, a low-grade tumor, or the more common concept, a virus. Dr. John M. Freeman, the director of pediatric neurology at Hopkins, has said, “We’re not even sure whether it’s caused by a virus, although it leaves footprints like a virus.”
CHAPTER 15
Heartbreak
In one sense, I was moving into groundbreaking surgical procedure — if I succeeded. Surgeons had recorded so few cases of full functional recovery that most doctors wouldn’t consider a hemispherectomy as viable.
I was going to do my best. And I went into the surgery with two things clear. First, if I didn’t operate, Maranda Francisco would worsen and die. Second, I had done everything to prepare myself for this surgery, and now I could leave the results in God’s hands.
To assist me I asked Dr. Neville Knuckey, one of our chief residents, whom I had met during my year in Australia. Neville had come to Hopkins to do a fellowship, and I considered him extremely capable.
Right from the beginning of the surgery we had problems, so that instead of the expected five hours we stayed at the operating table exactly twice that long. We had to keep calling for more blood. Maranda’s brain was very inflamed, and no matter where an instrument touched, she started to bleed. It was not only a lengthy operation but one of the most difficult I’d ever done.
The dramatic surgery began simply, with an incision drawn down the scalp. The assisting surgeon suctioned away blood with a hand-held tube while I cauterized small vessels. One by one, steel clips were placed on the edge of the incision to keep it open. The small operating room was cool and quiet.
Then I cut deeper through a second layer of scalp. Again small vessels were sealed shut, and a suction tube whisked away blood.
I drilled six holes, each the size of a shirt button, in Maranda’s skull. The holes formed a semicircle, beginning in front of her left ear and curving up across her temple, above and down behind the ear. Each hole was filled with purified beeswax to cushion the saw. Then with an air-powered saw I connected the holes into an incision and lifted back the left side of Maranda’s skull to expose the outer covering of her brain.
Her brain was swollen and abnormally hard, making the surgery more difficult. The anesthesiologist injected a drug into her IV line to reduce the swelling. Then Neville passed a thin catheter through her brain to the center of her head where it would drain off excess fluid.
Slowly, carefully, for eight tedious hours I inched away the inflamed left hemisphere of Maranda’s brain. The small surgical instruments moved carefully, a millimeter at a time, coaxing tissue away from the vital blood vessels, trying not to touch or damage the other fragile parts of her brain. The large veins along the base of her brain bled profusely as I searched for the plane, the delicate line separating brain and vessels. It was not easy to manipulate the brain, to ease it away from the veins that circulated life through her small body.
Maranda lost nearly nine pints of blood during the surgery. We replaced almost twice her normal blood volume. Throughout the long hours, nurses kept Maranda’s parents up-to-date on what was happening. I thought of their waiting and wondering. When my thoughts turned to God, I thanked Him for wisdom, for helping to guide my hands.
Finally we were finished. Maranda’s skull was carefully sewed back in place with strong sutures. At last Neville and I stood back. The OR technician took the last instrument from my hand. I allowed myself the luxury of flexing my shoulders, rotating my head. Neville and I and the rest of our team knew we had successfully removed the left hemisphere of Maranda’s brain. The “impossible” had been accomplished. But what happens now? I wondered.
We didn’t know if the seizures would stop. We didn’t know if Maranda would ever walk or talk again. We could only do one thing—wait and see. Neville and I stepped back as the nurses lifted off the sterile sheet and the anesthesiologist unhooked and unplugged the various instruments that had recorded Maranda’s vital signs. She was taken off the ventilator and began breathing on her own.
I watched her closely, searching for any purposeful movement. There
was none. She moved a little when she awakened in the OR but did not respond when the nurse called her name. She did not open her eyes. It’s early, I thought as I glanced toward Neville. She’ll wake up before long. But would she? We had no way of knowing for certain.
The Franciscos had spent more than 10 hours in the waiting room designed for the families of surgical patients. They had resisted the suggestions to go out for a drink or to take a short walk but had stayed there praying and hoping. The rooms are cozy, decorated in soft colors, as comfortable as a waiting room can be. Magazines, books, even jigsaw puzzles, are scattered about to help pass the time. But, as one of the nurses told me later, when the morning hours stretched into afternoon, the Franciscos grew very quiet. The worry lines in their faces said it all.
I followed Maranda’s gurney out of surgery. She looked small and vulnerable under the pale green sheet as the orderly wheeled her down the hall toward the pediatric intensive care unit. An IV bottle hung from a pole on the gurney. Her eyes were swollen from being under anesthesia for 10 hours. Major fluid shifts in her body had altered the working of her lymph system, causing swelling. Having the respirator tube down her throat for 10 hours had puffed her lips badly, and her face looked grotesque.
The Franciscos, alert to every sound, heard the gurney creaking down the hallway and ran to meet us. “Wait!” Terry called softly. Her eyes were red-rimmed, her face pale. She went to the gurney, bent down, and kissed her daughter.
Maranda’s eyes fluttered open for a second. “I love you, Mommy and Daddy,” she said.
Terry burst into joyful tears, and Luis brushed his hand across his eyes.
“She talked!” a nurse squealed. “She talked!”
I just stood there, amazed and excited, as I silently shared in that incredible moment.
We had hoped for recovery. But none of us had considered that she could be so alert so quickly. Silently I thanked God for restoring life to this beautiful little girl. Suddenly I caught my breath in amazement, as the significance of their conversation reached my brain.
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