Most readers are familiar with the "real Princess Diana." This book is about a courageous woman, Diane Cummings, who was the first person with the Tetralogy of Fallot (a congenital heart defect) to be operated upon by Dr. Willis J. Potts in 1946. He was the pioneer surgeon who developed an operation for this syndrome in infants who at the time were deemed too young to have the operation. Diane went on to lead a full life while subsequently undergoing four more open-heart operations done by leaders in the field For this to have happened, she survived four episodes of subacute bacterial endocarditis due to unusual bacteria. She was fortunate to be treated by a series of doctors who have been and still are the pioneers in the treatment of congenital heart disease. The doctors were: Dr. Willis J. Potts of Children's Hospital in Chicago, the originator of the Potts' operation; the father and son team of Drs. James and John Kirklin who were at the University of Alabama Heart Center; and Dr. Joseph Dearani of the Mayo Clinic. She was a true inspiration during the remaining forty-five years of her life. She died in January 2007, having lived a full life for 62 years. This made her one of the longest survivors on record of Potts' original operation. This is her story.
The Other Princess Diane
A Story of Valiant Perseverance Against Medical OddsBy Burton A. Waisbren, Sr.Trafford Publishing
Copyright © 2010 Burton A. Waisbren, Sr., M.D., FACP
All right reserved.ISBN: 978-1-4269-2504-7Contents
DEDICATION.........................................................................................................................................................vAUTHOR'S ACKNOWLEDGEMENT...........................................................................................................................................vPROLOGUE...........................................................................................................................................................ixINTRODUCTION.......................................................................................................................................................xiCHAPTER ONE - Dr. Potts' First Operation for the Tetralogy of Fallot, 1946.........................................................................................1CHAPTER TWO - First Bout of Staphylococcal Endocarditis Treated in Milwaukee, 1962.................................................................................7CHAPTER THREE - Second and Third Bouts of Bacterial Endocarditis - Streptococcal Viridians and Penicillin Resistant Staphylococcal.................................9CHAPTER FOUR - Fourth Bout of Endocarditis Due to a Gram Positive Bacilli that was Treated at an Air Base in Colorado, 1970........................................15CHAPTER FIVE - Second and Third Open Heart Operations Performed by Dr. John Kirklin at the Alabama Medical School Cardiac Center 1971 and 1975.....................17CHAPTER SIX - Fourth open heart operation performed by Dr. James Kirklin 1993 and a moment of truth................................................................33CHAPTER SEVEN -Events leading up to her fifth open heart procedure.................................................................................................41CHAPTER EIGHT - The Final Course...................................................................................................................................49CHAPTER NINE - Description of Diane written by Stephen Cummings, her husband.......................................................................................51INTRODUCTION TO APPENDIX...........................................................................................................................................53THE PLAYERS........................................................................................................................................................54CHRONOLOGICAL CORRESPONDENCE.......................................................................................................................................59CONTENTS OF THE APPENDIX...........................................................................................................................................60REFERENCES.........................................................................................................................................................87
Chapter One
Dr. Potts' First Operation for the Tetralogy of Fallot, 1946
Diane was born on December 10, 1943. It was apparent at birth that she had the congenital heart disease called the Tetralogy of Fallot (the blue baby syndrome) from the moment of her birth. Her parents lived in Waukesha, Wisconsin which is a small Wisconsin town about 30 miles west of Milwaukee. Her father was an energetic, loveable bear of a man who was involved in the brewery business in Waukesha. Her mother was a loving, warm woman who looked forward to being a "stay-at-home" mother. The parents were told from the beginning that at that time, blue babies rarely survived for more than a few years. Indeed that seemed to be the case since from the beginning, Diane failed to thrive. She could barely sit up and had periods of mild convulsions several times a day. Both parents were determined to do everything possible to help their tiny, nonthriving child but their efforts seemed to be in vain. However, Diane struggled on with constant loving attention from both parents.
When she was 18 months old, her mother heard at the bridge table that there was a doctor at Children's Memorial Hospital in Chicago who was trying to develop a surgical cure for blue babies. She told her husband about this and within that week, Diane, her mother, her father, and Dr. Martin Werra, her family doctor, went to see the doctor they had heard about. Dr. Potts was not at the Children's Hospital at the time but someone at the desk gave Dr. Potts' home address to her parents. The family then drove to the Potts' home where Dr. Potts was cutting his grass.
What follows is an article that Dr. Potts wrote in the Reader's Digest in November of 1964. This article will give the reader some idea regarding the type of man Dr. Potts was. It was entitled, We Gave Diane Back Her Life, by Willis J. Potts, M.D.
At first meeting, Andrew and Jane Schnell of Waukesha, Wisconsin, seemed unexceptional people. He was a businessman in his early 30's, prematurely gray; she, warm and outgoing, but with the subdued tension inevitable in a mother consulting one more in a long string of doctors, about a sick child.
And Diane, their daughter, was sick, mortally sick. She was twenty-one months old and weighed only 18 pounds: a frail, longish child, all bones, and her whole body blue as watered grape juice. She was unable to sit up without support. Any excitement or exertion - even laughing, or crying, or eating too fast - sent her off into unconsciousness because the heart deformity she was born with kept sufficient oxygen from reaching her brain.
Over the bridge table, of all places, her parents had heard that two doctors at Children's Memorial Hospital in Chicago were developing a new operation for the smallest and frailest of "blue babies". The Schnells had driven all the way from Waukesha on September 9, 1946, and I took Diane in without an appointment to see Dr. Stanley Gibson, our famed children's cardiologist. He brought them to the two experimenters: my colleague, Dr. Sidney Smith, and myself. And now Mrs. Schnell's eyes were pleading, Do you think you can do anything for our daughter?"
Even while Dr. Gibson and I were examining her, the little girl fainted dead away. We hurriedly ordered an emergency oxygen tent, but Mrs. Schnell quietly reassured us, "It's all right. She'll come out of it. This happens five or six times a day."
Diane did come around promptly, and Dr. Gibson and I breathed easier. It was the only occasion in my whole medical experience when the mother of a desperately sick child reassured me.
Mrs. Schnell came back to the point. "Now about this operation ..." I told them what I could do. "Yes, Dr. Smith and I were developing an operation for just such children as Diane. But so far we've done it only on dogs. We think it should work with a child, but we can't really be sure."
"Dogs? Just on dogs?" Mrs. Schnell asked. She hesitated, and for a long moment she and her husband merely looked at each other. Then she said, "The only other answer we've had from medical science is to take Diane home and make her life as happy as possible, because it's going to be a short one. We'd like to ask you, won't you try the operation on Diane?"
We admitted the child to the hospital for further examinations. Dr. Gibson, my co-worker and I told the Schnells that, "Yes, we thought the operation which up to that time had only been done on dogs would work, but if it was going to be done it had better be done quickly. Someday she was not going to come out of one of her spells."
I said I'd think it over. That night was for me a long and troubled one. True, the dogs had survived the operation - but they were healthy to begin with. Wasn't this child too frail to survive, to live? But then, she was already dying. Without the operation, she had no chance at all.
Next morning, I met with the Schnells again and explained the risks in some detail. I even drew them some sketches of the operation, which they studied silently. "It isn't perfect," I said. "It doesn't correct the deformities in the heart. It's designed to enable the patient to live, that's all. Do you still want to go ahead?"
They nodded. "Our minds are made up," Mrs. Schnell said to me. "Diane is a very poor surgical risk. So if you operate and she survives that will be proof that the operation will work."
"All right," I said, "tomorrow is Wednesday, we will operate in two days, on Friday morning."
The year before doctors Alfred Blalock and Helen B. Taussig at Johns Hopkins had taken a giant step toward solving the problem of the `blue baby'. By bypassing leaky heart valves and constricted lung arteries, they severed the subclavian artery which leads to the child's arm and connected it to a lung artery instead. This extra channel shunted more blood to the lungs. This relieved the lack of oxygen that causes the blue baby syndrome. Because at under two years of age, a child's subclavian artery is often too small to serve this purpose, this operation could not be done on babies of Diane's age.
I wondered if in these cases it might not be possible to connect a lung artery to the side of the aorta itself. The main artery or `river of life' that curves up out of the heart's left side. Medical experts agreed that such a connection would work, but they considered the feat impossible. Laboratory practice on dogs showed me that I needed at least 20 minutes to occlude, slit and sew together the two arteries. But clamping off a child's main artery and bloodstream for even less time than that will cause brain damage, paralysis or death.
I concluded I would have to pinch off a bit of the side wall of the aorta without impeding the flow in the bloodstream. But how? A baby's aorta, besides throbbing with every heartbeat, is as thin and slippery as a tube of wet macaroni. Every clamp I fashioned to do the job slipped off when we tried it on animals.
It was Dr. Smith who finally came up with an effective clamp that encircled the slippery aorta, cradling it, so that a workable fold of tissue could be pinched off. Using this clamp, Dr. Smith and I had performed thirty successful operations on dogs. But we were still refining our techniques when the Schnells appeared with Diane.
Now it was 9:00 a.m., September 13, 1946. The girl's thin, little body lies before me on the operating table, breathing softly under a light anesthetic. I could feel the tension myself and sense it in the nurses and doctors gathered in the operating room. Dr. Smith broke it with, "Chief, remember you've done this operation thirty times already." As I began the incision under the left armpit, my anxiety vanished with the need to concentrate.
The incision complete, I lifted the aorta and saw trouble. A startling network of at least a dozen small arteries branched off the aorta at the very spot where our clamp was to go. These were nature's own effort to compensate for Diane's inadequate heart. Each one would have to be ligated and cut before we could progress. Instead of a 30-minute operation, it would be a long one now.
Two hours later we had finished with those small arteries. Thanks to the efforts of expert anesthetist, Dr. William McQuiston, Diane was still breathing lightly and steadily. And now events seemed to turn our way. We tied off the pulmonary artery and applied our clamps to the aorta. It held perfectly. In each of the two vessels we made a precise lengthwise cut, one sixth of an inch, then joined the two slits together with fine silk thread.
After the suturing, we untied the pulmonary artery and slowly released the clamp on the side of the aorta. I had my fingers on the connection and felt it throb now with the new flow of blood into the pulmonary artery. The results became apparent as slowly miraculously, Diane's color began to turn from blue to a rosy hue. Dr. Smith and I looked at each other, we didn't try to tell each other how we felt - there were no words adequate.
Later Dr. Werra, the Schnells' family doctor who had been present in the operating room, took me aside to report the instructions Mrs. Schnell had given him that morning: "If Diane died on the operating table, I was to tell you to complete the operation to give you the experience that might help another child. I can only offer a silent salute to the deep love and perception of parents like that.
At noon, Diane was wheeled out of the operating room. I went downstairs with her. Mr. and Mrs. Schnell jumped from their chairs and stared at their baby. In unison they cried, "Look, she's pink!"
We placed Diane in an oxygen tent as a precautionary measure. She improved each day. By the nineteenth day, she was almost like a normal child, and the Schnells took her home. There she skipped the crawling stage, got up on her feet and started to learn to walk.
Our prayers had been answered. Today at nineteen, Diane is a lively, bright young lady with laughing brown eyes. She swims, dances, bicycles and leads a normal life.
Every year on September 16, our anniversary, I telephone her and we have a chat. Sometimes I use the nickname her classmates gave her which was "Dynamite".
When one reads Dr. Potts' writings, his humanity and love for children stand out. His brilliance is evident when one reads the account of Diane's operation, which was just presented.
After the operation described by Dr Potts in Readers Digest Diane became for all intents and purposes, a normal child. She was very popular and as her sister put it, "beloved", as she kept up with the boisterous family activities with the brother and sister. Dr. Potts, true to his work, called Diane and her parents annually on the anniversary of her surgery which was done September 16, 1946, to see how she was getting along and to convey his good wishes. Diane and her parents reciprocated by accepting Dr. Potts' invitation to come to New York some years later to address a group of hospital administrators and doctors who were starting a pediatric surgical department. She told those at the meeting what the operation had meant to her.
Chapter Two
First Bout of Staphylococcal Endocarditis Treated in Milwaukee, 1962
Shortly after Diane received her annual call from Dr. Potts in September of 1962, she began to run a daily fever. Dr. Werra, the family doctor who had originally gone with the Schnells to see Dr. Potts and who had observed the original operation, hospitalized Diane at Waukesha Memorial Hospital in Waukesha, Wisconsin. Waukesha is a town that then had a population of about 40,000, and is located thirty miles from Milwaukee. Diane became acutely ill and had several blood cultures that were positive for a coagulase positive staphylococcus which was resistant to the Penicillin. To complicate matters, Diane had developed a generalized rash from either Penicillin or Erythromycin that had been her initial therapy. She became increasingly ill, febrile, and hypertensive. I do not remember if Dr. Werra had been a student of mine who had heard me lecture to the students at the Marquette School of Medicine or whether he had had me consult on other of his patients. At any rate, he told the Schnells that he was stumped and wanted me to see Diane in consultation. He called me with the information that Diane was running a high fever, had a low blood pressure and seemed to be lapsing into a coma. I was making teaching rounds at the Milwaukee County Hospital at this time. I ended the rounds and drove to Waukesha Memorial Hospital, and indeed Diane was acutely ill. She had a high fever, a very low blood pressure, and a generalized rash that was, in all probability, a drug reaction to her previous antibiotics.
The Schnells readily agreed to my suggestion that she be immediately transferred to St. Joseph's Hospital in Milwaukee. There and at Milwaukee County General Hospital, I had been studying combinations of the newer antibiotics in endocarditis by the tube dilution method. Based on this experience, I started Diane on the combination of Lincomycin and Gentamycin. The tube dilation studies confirmed that this was the combination of antibiotics that might be of help. She made a rapid clinical response to 4 grams of Lincomycin and 500 mg of Gentamycin given in combination intravenously. These were continued for 4 weeks and blood cultures became negative and she became afebrile. During this time, I got to know Diane as an optimistic teenager who went along cheerfully with the difficulties of prolonged intravenous antibiotic therapy that were present in 1962. I also got to know her parents who were the quintessential examples of dedicated parents. They stood by Diane for the rest of their lives.
Chapter Three
Second and Third Bouts of Bacterial Endocarditis - Streptococcal Viridians and Penicillin Resistant Staphylococcal
Diane returned from Milwaukee to Waukesha in December of 1962 and resumed a normal life. Diane's father's brewery business was sold in Waukesha and in 1964 he moved his family to Woodbury, New York to run a bakery that he and his partners had purchased.
Several months after they arrived, Diane began to run a fever and gallstones were found. Because of this, her gall bladder was removed on August 24, 1964 at Syosset Hospital in Stoneybrook, New York. She became feverish after the operation. Blood cultures revealed streptococcal viridians, the most common type of bacteria that causes bacterial endocarditis. The resident physician did not consider subacute bacterial endocarditis as the cause of her fever and treated her empirically with Novobiocyn and Tetracycline. Her fever abated and she was discharged. This information I obtained from her hospital record, which was provided by the Syosset Hospital. Several weeks after discharge, she developed a high fever and was admitted to the New York Medical School Hospital in Manhattan. There by coincidence she came under the care of a Dr. Anderson who happened to be the wife of Dr. Harold W. Anderson, the surgeon who had taken out Diane's gall bladder at Syosset Hospital.
The doctors Anderson are still active. He is a surgeon with business interests and she is a practicing internist in the City of New York. I contacted both of them in the fall of 2008. Dr. Anderson vaguely remembered taking out Diane's gall bladder and that she had turned up several weeks later under the care of his wife who was a senior medical resident at New York Medical School University Hospital. Dr. Anderson's wife remembered Diane as a lovely, cooperative young woman. She remembered that she had been admitted to her service at the New York University Hospital and was found to have acute penicillin resistant staphylococcal bacterial endocarditis. She also remembered that Diane had not been responding to treatment with any available antibiotics and that one of the consulting infectious disease doctors knew of a new antibiotic that might be of help. I assume that the antibiotic was Lincomycin, the drug I had treated her with during her first bout of endocarditis. The consultant called the medical director of UpJohn, the pharmaceutical company that made Lincomycin and he agreed to ship a supply to New York by air. Diane's father met the plane when it landed and hand delivered the Lincomycin to Dr. Anderson. There was almost an immediate response and after a prolonged course of intravenous therapy, Diane was discharged from the hospital with negative blood cultures. Dr. Anderson did not remember any details of the treatment and a prolonged search by the record department of the hospital failed to turn up the record. A long search by a helpful employee at UpJohn also failed to yield a record of the incident that Dr. Anderson mentioned. A possibility exists that Ristocetin from the Abbott Laboratories was the antibiotic involved but Abbot's pharmacology archive department also could not find any record of what has been described.
(Continues...)
Excerpted from The Other Princess Dianeby Burton A. Waisbren, Sr. Copyright © 2010 by Burton A. Waisbren, Sr., M.D., FACP. Excerpted by permission.
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