Sickle Cell

ATLANTA -- Medical history unfolded at Egleston Children's Hospital on Friday at noon with the world's first umbilical cord blood transplant for sickle cell anemia.As a syringe of dark red blood plunged into the intravenous line of Keone Penn, relatives and friends bowed their heads in prayer and talked of miracles, cures and gratitude.They also gently told the center of attention to wake up."Hey, Keone. God's working it out just like we said. Wake up and see your miracle happening," said Beverly Kennedy, a close friend of Leslie Penn, Keone's mother.

OFFICIALLY, HE was the Orioles' "video coaching assistant," the young man who recorded and logged videotapes of baseball games as a way of helping players with their hitting and pitching. But unofficially, Jeff Nelson was a lot more than that. In a short span -- the 1995 season through 1998 -- he became a clubhouse spirit, a friend of players and even a source of inspiration. His death the other day -- of complications after surgery -- shocked and saddened a whole lot of Orioles. Many of them will attend his funeral today in Randallstown.

She first heard the words in May 1953 at Johns Hopkins Hospital. The doctor pulled her into a small room, away from her 9-month-old son, John, who was squirming in a crib.Everyone thought he had jaundice. When the doctor told her the diagnosis, Vivian Paul looked up at him. The physician stared back.Slowly, stressing every syllable, she asked: "What in the name of heaven is 'sickle cell anemia?' "In the years to come, she would spit out the words "sickle cell" as if they were poison.Without knowing it, she and her husband would pass the devastating blood disease to their three youngest sons.

A major international study has shown that bone marrow transplants can cure sickle cell disease in some children, a finding that experts say could herald a new era in treating the blood disease.Researchers involved in the study said they had achieved the cure in 16 of 22 patients with advanced disease by replacing the bone marrow that produces the defective red blood cells characteristic of the disease. Two of the children died after undergoing transplants.Sickle cell disease is a family of inherited and previously incurable disorders that afflict several million people around the world.

Until this week, medical science held out little hope for victims of sickle cell anemia, an inherited blood disorder that causes organ damage, episodes of extreme pain and premature death. The announcement Monday that researchers at the Johns Hopkins University Medical School have developed the first effective treatment for severe forms of the illness means that sickle cell may one day become a manageable, chronic illness rather than the relentless killer it is today.Between 70,000 and 80,000 people in the United States suffer from the disease.

Oncor Inc., which two years ago launched a subsidiary to commercialize gene identification breakthroughs by Johns Hopkins University researchers, has formed a second subsidiary commercialize gene therapy research from Yale and Princeton universities.Stephen Turner, Oncor's chairman and chief executive officer, said yesterday that his company had formed OncorPharm Inc., which will search for ways to treat genetic diseases such as sickle cell anemia.OncorPharm, which has 10 employees in offices a few blocks away from Oncor's Gaithersburg headquarters, has been in development for about a year, Mr. Turner said.

Only Dr. Samuel Charache knows precisely where to place the needle. He can't put it in the arms of his sickle cell patients. Long ago, their veins collapsed, punctured too many times.To draw blood from John Paul, Dr. Charache deftly locates the place on the man's right index finger -- on the knuckle. To draw blood from William Thorn Jr., the doctor pierces the outside of his thumb.Dr. Charache, 65, knows these two patients almost as well as family. Out of this close relationship -- 28 years in Mr. Paul's case -- has grown a research effort that culminated last week in the announcement of an effective treatment for severe sickle cell anemia.

Dr. Edward J. Benz Jr., one of the nation's leading specialists in blood disorders, has been selected to become chief of medicine at the Johns Hopkins School of Medicine and physician in chief of the Johns Hopkins Hospital.His appointment is effective Sept. 1, although it still is subject to confirmation by faculty and physician boards of the medical school and hospital. Those approvals are considered routine.Dr. Benz, who is chief of medicine at the University of Pittsburgh, is a hematologist who has spent many years studying the molecular basis of inherited anemias such as sickle cell anemia and thalassemia.

One by one, in the stifling heat, they came to pay their respects. They used walkers, leaned on canes and hung onto each other.John Paul was dead. And no one could believe it.For while the Baltimore man, 42, had long endured the terrible pain of sickle cell anemia, he had deflected attention from his disabling medical problem. Earlier this year, he shared the triumph of the Johns Hopkins physicians who made a breakthrough discovery after years of using him as a research subject.In the midst of it all, he had managed to care for others.

EVEN though Baltimore resident John Paul was only 42 years old, far short of a full life span, his death last month was not premature for the average person who suffers from sickle cell anemia. But Mr. Paul was no average man.As a key subject in a long-running research study, his willingness to try experimental treatments for the painful disease that stunts the lives of some 150 of every 100,000 African-Americans helped lead to the announcement earlier this year by Hopkins physicians that the drug hydroxyurea can lessen the frequency and intensity of sickle cell attacks.

WASHINGTON - -An analysis in 10 states of people hospitalized with the pandemic strain of H1N1 influenza shows that asthma is by far the most common underlying condition associated with severe cases of the disease. In children, other much rarer chronic conditions, such as sickle cell anemia, cerebral palsy and muscular dystrophy, are also predisposing patients to life-threatening bouts of the virus, federal health officials said. Epidemiologists at the Centers for Disease Control and Prevention studied the experience of about 1,400 people older than 18, and 500 children, who had been hospitalized in 10 states since the new influenza strain emerged in April.

With a James Bond party theme, it was no wonder the Hyatt Regency ballroom looked like a movie set. The room was swagged in white chiffon, with clusters of white couches and cube tables along the sides. Red velvet ropes cordoned off the back third of the room, which was elevated for the VIP section. "There's a bottle of vodka on every table here," said real estate developer Patrick Turner, noting the VIP bottle service. No set would be complete without its stars. And there were plenty, thanks to host Baltimore Ravens player Terrell Suggs.

At the post-show party for the 51st Annual Ebony Fashion Fair , held at Morgan State University's Murphy Fine Arts Center, it became apparent that it wasn't just the show that has a proud history. When the Baltimore Alumnae Chapter of the Delta Sigma Theta Sorority Inc. began hosting the touring fashion show more than 50 years ago, the chapter already had a 44-year jump on it. And for many of the party's guests, the history of both was a personal one. "It's awesome. I've been coming since I was in the fifth grade.

"I challenge you to find any one of my patients who has not been treated like a drug addict," says Dr. Sophie Lanzkron, director of the Sickle Cell Center for Adults at Johns Hopkins. "There are only so many times you can listen to those stories before you have to do something about it." So she did. In February, Hopkins opened a day hospital for sickle cell patients who need immediate pain medication when they feel overwhelming crises coming on. The staff knows their needs and treats them gently - often better than the suspicious staffs of emergency rooms.

AACC culinary team competes The Anne Arundel Community College student culinary team, Team Anne Arundel, placed fourth at the American Culinary Federation Northeast Regional Conference's student competition. The team competed March 2 and 3 at the conference in Pittsburgh, which attracted 700 chefs and cooks for workshops, exhibits and competitions. The student teams completed a cold food display, demonstrated their butchery and knife skills and prepared a four-course signature meal. The winner, a team from the State University of New York at Delhi, will represent the region at the ACF national conference in July in Florida.

Vernon Ingram, 82, the Massachusetts Institute of Technology professor whose landmark discovery on the cause of sickle cell anemia made him a pioneer in the field of molecular biology, died Aug. 17 in Boston after a fall. In 1957, he discovered that a single amino acid substitution is responsible for the molecular abnormality that leads to sickle cell anemia. In recent years, he had focused on neuroscience, especially Alzheimer's disease. Born in Breslau, Germany, he studied at Birkbeck College at the University of London, where he earned his bachelor's degree in chemistry in 1945 and doctorate in organic chemistry in 1949.

Mary Clayburn White, a pioneer of gospel radio who helped transform Baltimore's market into one of the country's largest, died of bone marrow cancer Wednesday at St. Agnes HealthCare. She was 71. With a warm, motherly voice, Mrs. White played spirituals, conducted interviews and served as an advocate for African-Americans on several AM and FM radio stations during a career that spanned three decades. "Back when she first started doing it, there were no 24-hour gospel stations," said Lee Michaels, who worked with her at "Heaven 600" WCAO-AM, one of two round-the-clock gospel stations in Baltimore.

A pharmaceutical maker called NitroMed is testing a drug it hopes to market as a treatment for heart failure in African-Americans. Pfizer, a drug industry giant, is holding a national series of health awareness meetings "to inform and educate African-American women about the diseases and conditions for which they're at risk." The Food and Drug Administration wants drug researchers to take pains to include racial minorities in their clinical trials. Although these might all be viewed as good examples of smart, practical medicine, biologists and anthropologists are increasingly warning the medical community against using race as a guide in diagnosis, treatment and research.

As scientists are delving ever more into mankind's genetic code, Phillip Hardt is trying to keep certain people out of his. Hardt, 44, suffers from Huntington's disease, a deadly brain disorder caused by a tiny typo in his genetic alphabet. Because the disease is hereditary, his daughter found it tough to get life insurance. If she tested negatively for the Huntington's gene, insurers said, they'd reconsider her application. "And if that isn't discrimination, I don't know what is," says Hardt, a retired AlliedSignal Corp.

PHILADELPHIA -- When the game is on the line, Temple's Mark Karcher knows the ball will be in his hands. He can drive to the basket, spot up for a three-pointer or try a pull-up jumper from the foul line. So many options, so much control. But there's another side to the former Baltimore high school basketball standout, a personal ordeal that has left Karcher feeling powerless. His 17-month-old daughter, Aria, has sickle cell anemia. It's an inherited, chronic disease in which the misshapen red blood cells function abnormally and lead to sudden, painful episodes affecting the bones of the back, chest and abdomen.