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Brent was born in Spokane, a town nestled within the most eastern part of northeastern Washington, over in a corner, distant from most of Washington, as well as away from the main part of the United States. Spokane is larger than a town; it is a small city.
Gwyn Margaret McCann, Brent's mother, born August 18, 1946, was an attractive twenty-nine-year-old woman. She was not a native of Spokane. She was the second oldest of four beautiful sisters, preacher's kids, no brothers, born to a Canadian Protestant minister and his attractive Detroit wife. The family moved from Detroit to San Diego, California, where Gwyn's father served his church.
Brent's lovely mother became a college student at San Diego State College where her handsome professor turned her pretty head. They fell in love, which culminated in a trip to the altar. After they married, Gwyn and her husband moved to Spokane, where he accepted a faculty position in the Modern Language Department of Gonzaga College. Brent's oldest sister, Paula, was born in Spokane on Thanksgiving Day in 1969, before Brent's mother discovered that her husband no longer wished to be married to her. She and her first husband divorced, although they continued to remain amicable.
Gwyn married another man, a dynamic twenty-nine-year-old Spokanite: Leland Hubert Perry. With Lee, Brent's mom had a second child, Colette, who was born in 1971. His mom and dad and two sisters lived in a cute little one-story brick house at West 2905 32nd Street in Spokane. Not far away from the house, in the early morning hours, Brent came into this world at 10:24 am on October 4, 1975, in Sacred Heart Hospital, Spokane, Washington. His first view of the world outside his mother's womb was in the hospital where his life began. Like many of us, his life did not end in the same hospital, or the same city, or even in the same state where he was born.
Preceding Brent's birth, Gwyn's older sister, Louise McCann Carson, gave birth to an infant son who was born with hydrocephalus, a birth defect presenting with an abnormally large head. Hemophilia was not suspected at the time of his birth, and no tests were completed. However, it is possible that the cause for the child's large head was an undiagnosed bleed inside his skull. In 1962, he died at six months. A second son was born to Gwyn's sister, a normal child. Eight years before Brent's birth, a third child was born to the same sister, Louise, in 1967, a baby boy, Brent's cousin, Bill Carson, who was circumcised and almost bled to death a few days later. Tests completed in California, where the family lived, revealed that the baby boy had hemophilia. There had been no ancestors with bleeding tendencies in the family. He was the first case of the bleeding disorder in the McCann sisters' family. Everyone in the family was tested to identify carriers of hemophilia-who would be at risk of giving birth to another male infant-by obtaining blood samples that were sent to Johns Hopkins Medical Center for analysis. The 1967 test results were normal for the mother of the infant born with hemophilia, her three sisters, her mother, and her grandmother.
They were informed that they were not carriers of hemophilia. If they had sons, they were told, none would be affected with hemophilia. The family assumed the birth of an infant male with hemophilia to one of the sisters was an instance of a new mutation, and hemophilia was not inherited in the family. Gwyn thought she was off the hook if she were to have a son-he would not have hemophilia. Even though Gwyn was informed that she was not a carrier of hemophilia, she wasn't completely confident of the test results. When Gwyn and her husband moved away from her family in Santa Barbara, she did not know that she was a carrier of hemophilia. Gwyn became pregnant for the third time. Again, she was told she was not a carrier and not at risk for having a son with hemophilia, as her sister had before her. At six months of gestation, Johns Hopkins Hospital notified her, reporting that the tests previously completed were not reliable. A new method of testing for carriers of hemophilia, which was more accurate, had become available. However, she was already far along in her pregnancy.
Brent, the third child, her first son, was born headfirst. "And it was fast," his mother proclaimed. There were no abnormal signs of bleeding at birth, no scalp hematomas, and no bruises.
Birth is a rough trip. During birth, the infant's head is squished against the inside of the hard bones of the mother's pelvis. The arms and legs, shoulders, and hips are twisted and squeezed, and yet the slippery baby usually emerges unscathed, a miracle of nature.
Why don't all infant boys who have severe hemophilia bleed during their birth? Some mothers believe their blood protects their babies from bleeding, and some doctors let the mothers continue to harbor their maternal protective beneficial beliefs because it makes them feel better, even if the mother's impression is incorrect.
Brent did not bleed excessively after a heel stick to collect newborn blood samples. This test is accomplished by jabbing the point of a sharp blade into the soft skin of a newborn's heel, followed by squeezing the baby's foot until blood flows from the wound.
Before Brent went home from the hospital, the doctor examined him. He proclaimed that Brent was a healthy, normal little boy. Brent opened his eyes. He moved his arms, legs, fingers, and toes; he had a robust cry. His testicles were descended. Gwyn remembered that she had a nephew, her sister's son, Bill Carson, who was a bleeder. He had hemophilia. He was Brent's maternal cousin. She decided not to have her son circumcised.
One way to find out if Brent would manifest signs of abnormal bleeding, to discover if he had hemophilia similar to his maternal cousin, would be a simple test: snip off the foreskin of his little penis and see if he bled afterward, the test of circumcision. But such a test is not without hazard. There is risk of uncontrollable bleeding afterward. Circumcision is not an accurate procedure to establish the diagnosis of hemophilia. Nearly one-third of the infant males who have hemophilia and have been circumcised, their unsuspecting parents unaware of hemophilia at their son's birth, have not bled following circumcision. A preferable test to establish whether Brent would develop signs of abnormal bleeding from hemophilia would be to complete a test after obtaining a sample of blood from one of his veins. However, obtaining a blood sample from the tiny vein of a newborn is not an easy task. To diagnose hemophilia, by measuring the clotting activity of blood to determine the amount of antihemophilia factor (AHF), special care must be exercised when obtaining the sample. A free-flowing blood sample must be collected without contamination from tissue juices. The collected blood sample must be promptly processed and sent to a laboratory that is experienced in testing for AHF. The type of hemophilia that affected Brent's cousin was type A, the result of a deficiency of AHF VIII in his blood. To determine if Brent had hemophilia type A, the laboratory must assay the factor VIII in his blood. Most hospital laboratories are not set up for AHF testing. A blood sample is usually sent to a reference laboratory for factor VIII assay when hemophilia is suspected. The most practical method for sampling a newborn's blood for testing is by catching the blood from the umbilical cord after it has been severed from the infant. The umbilical blood sample is free-flowing, and the source of blood is from the infant, not the mother.
Gwyn had informed the doctor and the nurse before her son was born that her nephew was affected with hemophilia. She instructed them that if her expected baby was a male, he should not be circumcised because there was a chance that her son might have hemophilia even if there were no signs of bleeding at his birth. She also informed both the doctor and the nurse that if her infant was a male, a blood sample must be collected from the umbilical cord after it was severed from the baby for testing for hemophilia. Neither the doctor nor the nurse honored her request. No sample was collected from the umbilical cord of her newborn son. After her son was born, Gwyn did not know if he was affected with hemophilia or if he was free of the bleeding disorder that affected his older cousin.
The explanation for not following the mother's request, for collecting a sample of umbilical cord blood at birth to be tested for hemophilia, remains unanswered. It is unlikely that the doctor and the nurse intentionally ignored the mother's request. That would be unethical. More likely, the explanation had a logistical basis. For example, when a test for hemophilia is to be performed on a blood sample, a special test tube-a prothrombin tube-is necessary. The collected blood sample must be kept cold by placing it on ice and promptly processed for analysis. Conditions may not have been arranged prior to delivery since babies are not born at scheduled times.
"Isn't he cute?" exclaimed Pierrette, a visitor friend of the mother, who came to the hospital to rejoice over the birth of the new baby. "Doesn't he look just like a little Buddha?" The newly born little boy had a red round face and a serious countenance-if newborns can be serious. He was named Brent McCann Perry. Brent died at age seventeen in 1993, the result of an infectious disease.
Brent was fortunate to have been born when modern medicine facilitated treatment of his uncommon medical disorder, which predictably should have allowed him to live many years. Advances in medicine in Brent's era were remarkable; however, the gains were polluted with havoc. How did this happen? Was this a mystery? Was this the way life was meant to be for some persons? The doctors who treat hemophilia were aware that pooling plasma from hundreds, sometimes thousands, of donors was risky. One contaminated donor would contaminate the entire large lot made from the pooled plasma. Yet they accepted medicine made from the pooled lot, reasoning that it was a trade-off, with the benefits outweighing the risks. Was it the impact of the doctor's treatment that caused Brent's death? Is the doctor guilty?
Discovery of Hemophilia in Spokane
On the second day of his life, Gwyn carefully carried her newly born son, swaddled in a blue baby blanket cozily tucked into an infant carrier basket, from the hospital where he first opened his eyes and made his first cry, to the little house on West 32nd Street. His father, Lee, and his two sisters-Paula, age six, and Colette, age four-were anxious to help take care of him.
The family did not know if Brent had hemophilia or if he did not. The opportunity to test for hemophilia with a sample of cord blood had been missed. Because of the difficulty of obtaining a free-flowing blood sample to test for hemophilia from his tiny veins, the doctor recommended waiting to see if signs of unusual bleeding appeared. If he was a few months older, the doctor reasoned, obtaining a blood sample for a test would be easier. Six months later, on the morning of April 2, 1976, Gwyn lifted her infant son from his crib. He was wide-awake. She kissed the top of his fuzzy head and then laid him down on the changing table. "Oh, you are a good baby," she said to him as she unsnapped his flannel pajamas and pulled them off over his head.
"Oh my God!" Gwyn shrieked when she discovered extensive purple blotches discoloring her baby's chest along the lower ribs, extending from the front to the sides of his chest. She rolled him over and noted that the marks were also on his back. She inspected them closely and thought they resembled bruises. He was not crying as if in pain. "Come here quickly!" Gwyn screamed at her husband. "Look at your son. He's all black and blue!" Terrified, the parents wrapped their infant in a blanket and rushed to the doctor's office in Spokane. On that April day, two days before Brent was six months of age, they recounted to the doctor that the day before their infant son had been pushing with his small stockinged feet while sitting in a jump-up swing that was suspended in the kitchen doorway, laughing while bouncing up and down.
After undressing and examining the infant, the doctor quietly declared that the purple discoloration of Brent's little ribs in his chest did not resemble the nature of bleeding he expected to see if the baby had hemophilia. He told Gwyn that other possible causes may have produced the bruises. For example, he said, leukemia, a cancer of the blood, often first appears as bruises of the skin in infants. He also mentioned "battered child syndrome"; however, the doctor did not infer that Gwyn or her husband had beaten their infant son. This unfortunate condition was newly recognized in pediatric circles and was on the tip of most pediatricians' tongues.
The doctor successfully obtained a sample of blood from Brent's little arm vein, which was processed and sent to a special laboratory to be tested for factor VIII, the clotting factor deficient in hemophilia type A. While the needle was still in place in the vein, after the blood sample had been withdrawn, the doctor infused Brent with concentrate, even though hemophilia had not been diagnosed. The unusual bleeding into his chest was suspicious enough to warrant the infusion, knowing that hemophilia was present in the infant's male cousin.
Several days later, when the doctor received the report of the results of the blood test, he immediately called Gwyn to his office to notify her of the test results. He said he was surprised and dismayed when he discovered that he must report to Gwyn that her son had severe hemophilia, the same disorder that affected his eight-year-old cousin. The factor VIII in Brent's blood was less than 1 percent of the normal amount, which established the diagnosis of severe hemophilia type A. Brent was given his first infusion of concentrate in 1976, in Spokane, to arrest bleeding in his chest.
From that moment on, for the rest of his life, although a normal child, Brent's life would be different from those of other little boys. Gwyn was pleased that she did not know that her son was affected with hemophilia until he was six months of age. Some people refer to a person who has hemophilia as a hemophiliac, a word Gwyn detested. She did not want her child labeled. Her infant son was not a hemophiliac-he was Brent. The six months of not knowing he had hemophilia gave her a chance to bond with him. Not fearing for him, she had cared for him as a normal baby boy during those happy six months. Nature seems to feel that way too. Even infants who are born with severe hemophilia often do not bleed after birth. Sometimes no signs of bleeding appear for several months. In some instances, infant males born with hemophilia do not reveal signs of abnormal bleeding until they begin to crawl and walk, as late as one year of age.
Signs and Symptoms of Hemophilia
The signs and symptoms of hemophilia are the result of a deficiency of the blood to clot normally (Genetics Home Reference 2009). A person's blood repeatedly circulates over and over again, confined to the inside of the blood vessels. The route of the circulating blood is a constant, endless, very long trip, night and day, year after year. And yet the blood does not leak from the tubes that allow it passage. If blood escapes from the tubes, that is called a hemorrhage. A common sign of a localized hemorrhage is a bruise, such as a black eye from a damaged blood vessel. After an injury or a ruptured blood vessel, blood escapes from the tubes into the tissues, where it decomposes and becomes discolored. Hemophilia families refer to hemorrhages as bleeds.
Why don't bleeds occur more often in people who do not have bleeding disorders? If a person were to grasp a sharp knife and slash across the back of his hand, he usually would not bleed to death. His body is protected by three processes to prevent blood from leaking outside the blood vessels, a process referred to as hemostasis. After a blood vessel is severed or broken, the first thing that happens in hemostasis is the release of adrenalin by the tissue surrounding the blood vessel. Adrenalin produces an immediate contraction, vasoconstriction, shrinkage of the blood vessel. The second thing that occurs within a few seconds is the formation of a seal at the site of injury by platelets, which plug the rent in the blood vessel wall. Platelets circulate in the blood and are attracted to an injured blood vessel wall by the exposure of the tissues in the blood vessel wall. Third, and more slowly, fibrin is laid down into the platelet plug. Fibrin acts as cement to hold the platelet plug in place while healing occurs. However, if significant amounts of fibrin were constantly present in circulating blood, blood clots would form, and a stroke or heart attack would result from excessive clotting. To prevent unwanted clot formation, fibrin must be activated from its precursor condition before it can be laid down.
One of the precursors of fibrin formation is antihemophilia factor, or AHF. AHF is necessary to form an effective blood clot at the site of a blood vessel injury. A person who has hemophilia has a deficiency or a defective AHF. His platelet plug does not cement in place; it does not retract. It is soft and mushy rather than firm. After the platelet plug forms at the site of a damaged blood vessel in a person who has hemophilia, oozing slowly begins around the soft plug and continues at the injured site.
Excerpted from Doctor Guilt? by Everett Winslow Lovrien Copyright © 2010 by Everett Winslow Lovrien, M.D.. Excerpted by permission.
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