crisis management

Sickle-cell anemia remains more common among people of African ancestry, but science has made strides in treating this blood-borne disease.

BY GLENN TOWNES

he sharp or stabbing pain can attack anyplace in the body where sickle-shaped cells pile up and block blood vessels. The painful blitzkrieg may strike suddenly and range from mild to severe and from acute to chronic. It’s triggered by dehydration, infection, fever, cold temperatures, drugs and alcohol and any kind of oxygen loss to body tissue. The feeling can last for just a few hours or as long as a week. Those who have sicklecell anemia know these symptoms well. The pain is called a “crisis.” It’s so severe, patients admit they’ve thought hard about suicide.

That’s what Albert Johnson considered when he was a child. “At one point, the sickle-cell pain was so bad I tried to kill myself with a steak knife,” he says. “I was only 12 years old, deeply depressed and very unhappy.”

Today, Johnson is better known as Prodigy, one half of rap duo Mobb Deep. When the hip-hop star isn’t busting rhymes in the recording studio or taking home Grammy Awards, he’s speaking to people as a national advocate for the Sickle Cell Disease Association of America. Johnson says he’s committed to spreading the word that it’s possible to enjoy a full and productive life despite living with sickle-cell anemia (SCA).

For Elijah Bobbitt, who grew up in Trenton, New Jersey, childhood sickle-cell crises were chronic. His body ached, his joints were sore, and his muscles throbbed with incessant and excruciating pain. No one, including his parents, knew why.

“At the time, my wife and I couldn’t understand why Elijah was crying so much,” recalls Bobbitt’s father,

Andrew. “We constantly took him to various doctors being treated. The reason? Trenton had no treatment and hospitals. No one could tell us what was wrong facilities for people with sickle cell. “The entire situation with our child.” put a financial and emotional strain on our family,” the

Finally, after a litany of tests, including lab work, X-rays elder Bobbitt shares. “There was very little awareness and a lot of poking and prodding, doctors diagnosed about sickle-cell anemia in Trenton. Believe it or not, a Bobbitt with SCA at age 4. “I didn’t know anything about lot of people knew little if anything about the disease.” sickle-cell anemia,” says Bobbitt, now 21. “All I knew was that I was always in a lot of pain and felt sick all the time.”

What Bobbitt didn’t know then was that he’d received sickle hemoglobin genes—a.k.a. sickle-cell trait genes— Even among some doctors today, ignorance of SCD remains. from each parent. (Hemoglobin is a protein molecule in “There is a significant need for public awareness about red blood cells that carries oxygen from the lungs to the sickle-cell anemia and for funding to support research body’s tissues and then returns carbon dioxide from the into treatment options and supportive care for those tissues to the lungs. It also helps red blood cells maintain affected by SCA,” says Ifeyinwa Osunkwo, MD, a pediatric their healthy round shape.) hematologist at Aflac Cancer Center of Children’s

Sickle hemoglobin genes can cause red blood cells to Healthcare of Atlanta. “The majority of America is become crescent-shaped and sticky. The abnormal shape unaware of what SCA is, how it is inherited and what and stickiness stop the cells from properly flowing complications it can cause.” through the blood vessels of limbs and organs. The For starters, because African Americans are more result? Pain, serious infections and organ damage. likely to get SCD, it’s often ignored that other races can

When both parents pass these abnormal genes to their also develop the condition. “Most Americans with baby, the child is very likely to develop sickle-cell anemia, SCA have African ancestry, but the disease also affects a specific and common type of sickle-cell disease (SCD). Hispanics and occasionally Caucasians,” says John Cunningham, MD, director of the sickle- cell disease program at La Rabida Children’s ONE OUT OF Hospital in Chicago. EVERY 500 “Worldwide, SCA affects persons from AFRICAN North, Central and South America, the AMERICANS Mediterranean region, DEVELOP India and Africa.” Many people also SICKLE-CELL don’t know that treatANEMIA. ment is key to avoiding complications stemLeft: Elijah Bobbitt stands with his father, Andrew. Right: Grammy Award–winning rap star Prodigy. ming from sickle-cell disease. Unfortunately, treatment is often longThis genetically inherited blood disorder affects about term, and issues of affordability and costs arise. In 100,000 Americans, according to the Centers for Bobbitt’s case, the bulk of his medical treatments were Disease Control and Prevention. What’s more, one out covered under his father’s health insurance. But for many of every 500 African Americans develop the potentially people living with sickle-cell disease, access to care is not fatal condition. so readily available. “Even though we have effective treat-

Currently, sickle-cell screening of newborns is man- ment options for sickle-cell anemia, adults who do not dated in all 50 states. However, when Bobbitt was born have insurance cannot access this care,” Osunkwo says. in April 1990, there was no newborn screening test. One of the effective treatment options that excite Besides, his parents weren’t aware of their son’s height- Osunkwo is the controversial bone marrow transplant ened risk: “My wife and I didn’t even know we were that some doctors regard as a cure for SCD. If successful, carriers of the trait until after Elijah was diagnosed,” his bone marrow transplants can eliminate sickle cell, father explains. “That’s when we started intensively Osunkwo says. educating ourselves about the disease.” But other physicians, such as Carole Kenner, DNS,

After Bobbitt’s diagnosis, his parents entered counsel- PhD, dean of the School of Nursing and associate dean of ing and learned about available SCD medications and the Bouvé College of Health Sciences at Northeastern children’s treatment options. But each time Bobbitt University of Boston, say bone marrow transplants may required treatment, his father had to trek miles to either merely put sickle cell in remission and only cure the Philadelphia or New Brunswick, New Jersey, and spend disease in very rare instances. the night at a hotel near the hospital where his son was Nevertheless, apart from the controversy, doctors agree

that federal, state and local governments need to do more to offer SCD patients affordable treatment options. Although SCD bone marrow transplants are considered a standard care option and are fully covered by many insurance carriers, including Medicaid (if patients meet state-imposed eligibility requirements), they are extremely expensive. What’s more, the procedure is very high-risk for adults, and there are few available bone marrow donor matches for African Americans.

Perhaps this is why Kenner and other doctors stress the three traditional treatment management options for sickle-cell anemia: comprehensive care, the use of meds such as hydroxyurea and penicillin (prescribed to babies with sickle cell from 2 months to 5 years old), and red blood cell transfusions (of temporary benefit).

But lifestyle modifications also help treat and manage SCD symptoms and shouldn’t be overlooked, Osunkwo says. For example, after Prodigy agreed to his mother’s suggestion to go into counseling, at age 12, the therapy he received helped him change his lifestyle so he could better manage living with SCA. As he got older and educated himself even more about the condition, Prodigy learned the importance of healthy living as a way to control SCA.

“I drink water like a fish and just eat nutritious foods,” Prodigy says. “I notice that since I’ve been doing that for the past seven to eight years, I don’t get sick as much as I used to. And if I do get sick, it’s really because of something I’m doing wrong. I really know my body and how to control the disease.”

To regulate his SCA, Bobbitt normally visits the hospital once each week to monitor his blood cells. In addition, each day, he swallows several medications and supplements as part of a customized treatment cocktail.

Osunkwo says the medical

SPORTS BAR

Should having sickle-cell trait make student athletes ineligible for competition?

No, says the National Collegiate Athletic Association (NCAA). But the sports body did recommend member colleges and universities screen student-athletes—particularly football players—for sickle-cell trait.

Why? Because since 2000, studentathletes with sickle-cell trait have collapsed and died more often than their counterparts with other conditions, the NCAA says.

The reason this can happen is because during intense training, normal red blood cells can suddenly become sickle shaped and trigger a life-threatening medical condition called exertional rhabdomyolysis. When student-athletes with sickle-cell trait experience this condition, they could collapse and eventually die. And even though most studentathletes complete their careers without any major medical complications, to prevent untimely deaths, the NCAA wanted to ensure players received regular screenings and knew their health status.

Although there’s a system in place to inform people they have sickle-cell trait, some health professionals contend there’s a lack of adequate follow-up and medical treatment provided to those who have this potentially dangerous condition.

Also, what hasn’t been confirmed, says Ifeyinwa Osunkwo, MD, a pediatric hematologist at Aflac Cancer Center of Children’s Healthcare of Atlanta (CHOA), is what roll sickle-cell trait played in the deaths of student-athletes with the gene. “The medical community has not reached a consensus on the risks associated with having sickle-cell trait, and having the trait is not considered a medical diagnosis,” Osunkwo observes.

But do student-athletes with sicklecell trait automatically have an increased risk of death during rigorous practice or actual gameplay? The issue continues to be hotly debated by the medical and sports communities.

Osunkwo’s opinion? “Over-exhaustion from extreme physical training is not a good idea for any athlete whether or not they have the sickle-cell trait.” —GT community must do more to improve health outcomes for SCA patients. For starters, this includes: increased SCA education in medical schools and other paraprofessional fields; ongoing education to emergency room providers and hospital care personnel about how to manage SCA patients with acute complications; better ways to transfer medical information to keep patient care consistent; and more research into pain management and other related sickle-cell complications.

To optimize health outcomes, Osunkwo says, everyone must understand that SCA therapy needs a comprehensive approach: disease management, self-efficacy and quality-of-life improvements, not just a pill to make pain go away.

But even though there’s more to be done, science has made great strides. Now, the childhood mortality rate for those with SCA is almost zero. Today, people with sickle-cell disease live well into their 40s and beyond. Compare this with the sickle-cell prognosis in 1973. That’s when doctors estimated the median survival age for kids with the condition was about 14.

The good news is, more adults are living with the condition. But that’s created other problems. “Currently, there’s a serious deficit in providers to care for these aging patients,” Osunkwo explains.

Ultimately, doctors hope to stop SCD by changing or replacing the abnormal gene that causes it. “Research is looking into gene therapy as an alternative way to cure the disease,” Osunkwo explains. “But we are still several years away from administering a clinical application of this treatment.”

In the meantime, people like Prodigy and Elijah Bobbitt refuse to let living with SCD get them down. Prodigy launched a “No Pain” campaign earlier this year to raise awareness about the disease. Bobbitt focuses on day-to-day optimism. “Maybe one day there will be an [easy, effective and affordable] cure for it,” he says.

Until then, Bobbitt suggests SCD patients stay mentally positive. His advice? “Never lose hope.” ■

ome sisters view their wigs, weaves and extensions as a godsend. These hairstyle methods can instantly change a woman’s appearance, give her damaged hair a rest from chemical processing, and help her transition from chemically treated tresses to her hair’s natural texture. What’s more, these options may even aid hair growth by protecting tresses from heat styling tools and harsh weather.

If you’re considering going from real to faux real, here’s a look at the most popular styling options available and tips that will help keep your natural, God-given hair its healthiest while you try out a man-made mane or other enhanced add-ons.

But before we start, what’s key, says hair loss specialist Toni Love, author of The World of Wigs, Weaves and Extensions, is that you select an experienced professional to apply any type of commercial hair. And have the hairstylist examine your hair and scalp to ensure both are ready for the procedure you’ve chosen. If your hair is relaxed, experts suggest indulging your tresses in several deep conditioning treatments before the service. And if hair is in its natural state, professionals recommend one or two of these fortifying treatments.

WIGS The best thing about these hairpieces is their versatility. What’s more, wigs are a great option for women who don’t want to remain slaves to chemical treatments or who want to give their hair a break from daily styling, says New York City –based hairstylist Shedelle Holmes. “For those who are transitioning [from chemically treated styles], as your hair begins to grow, you can trim the straightened ends until you’re comfortable enough to do the big chop and remove all the relaxed hair.”

But wearing a wig doesn’t mean you can skip taking care of your hair and

scalp. “Make sure to wash and condition the hair weekly with a sulfate-free shampoo, and also use a detangling, leave-in conditioner,” suggests Peggy Fuller, MD, of the Esthetics Center for Dermatology in Charlotte, North Carolina. Fuller also recommends wearing low-maintenance styles such as loose plaits and braids, Nubian knots or loose pin curls under the wig to allow the scalp to breathe.

Currently, many women choose to wear popular lace-front or full-lace wigs—hairpieces constructed so that hair looks as if it’s growing out of its lace base. But under these wigs—and hairpieces of all types—women should “avoid tight elastic stockings or other protective head coverings that may cause friction and tension on the hairline and nape of the neck and exacerbate hair loss,” Fuller warns.

Holmes also cautions lace wig aficionados to be careful. “The excessive use of adhesives, or other bonding agents used to attach these hairpieces to the head, can pull the hair and eventually cause traction alopecia [a type of balding],” Holmes says.

EXTENSIONS Peruse online forums about braided hair, and you’ll read numerous stories about severe hair damage women experienced from putting in extensions—a quantity of hair added to one’s own for length and volume or to get a new look. One woman revealed what happened when a technician braided too much hair onto her own fine strands: The added hair was too heavy. When she removed her braids, she also lost a handful of her own hair—from the roots.

The big problem with extensions is the hair’s weight and the pull it exerts on women’s natural tresses. “When added hair is applied or sewn too tight on women’s heads, this—along with the weight and volume of the commercial hair—can do great damage to the natural hair, especially if it’s thin or fine,” Love says. “Once traction alopecia sets in and damages the follicles and scalp, it’s hard for hair to bounce back.”

But despite these possible problems, when properly used, braided extensions are a great protective style. While wearing these add-ons, wash hair once a week to remove product buildup. And always dry hair thoroughly to prevent bacteria buildup that can cause rot and mildew. Also, if possible use human hair extensions because they’re washable. (Synthetic hair isn’t waterfriendly so it can matte and cause your own strands to break when you try to take out the braids.) And when removing braids from relaxed hair, be careful not to stress tresses at the demarcation point where the difference in texture weakens the hair. Give relaxed and natural hair a rest for at least two weeks before rebraiding. Opt for a very low-maintenance style, or consider wearing a flattering wig.

WEAVES A weave is the process of adding a full head of hair extensions to one’s natural tresses by sew-in or glue-in methods. And though dermatologists have knocked poorly applied weaves because of balding risks, this popular styling option can also give hair a rest from chemical treatments or help women transition from relaxed to natural hair.

While dermatologist Brooke Jackson, MD, founder of the Skin Wellness Center of Chicago, finds nothing wrong with wearing weaves and braids, she suggests women don’t perm their hair before putting in a weave or braids. What’s more, if your hair is already traumatized from using relaxers, Jackson cautions, don’t damage tresses even more by putting in a weave.

And if your hair is healthy enough to support a weave, don’t leave it in for more than four to eight weeks. In addition, have it professionally cared for by a stylist. “It’s always good to have your stylist deep condition any visible hair on a weekly basis,” Holmes recommends. “Also, give your hair a break in between weaves, and remember to trim the ends.”

And when it’s time to remove a weave, preferably have it done by a professional who follows the proper removal procedures for sewn- or glued-in hair.

“I’ve had all of these styles,” Jackson admits. “My weave was too tight and made me feel like I was going to become my own patient. No one should need to take Tylenol after a hair appointment!”

Needless to say, when Jackson got her weave removed, she followed her own advice: A professional did it. ■

Hair Rules

How much do you know about protecting your hairline from damage?

1. Brushing your hairline will help the hair grow. a. True b. False

2. You should avoid gluing baby hairs onto your weave. a. True b. False

3. Which method can damage the hairline? a. Micro braids b. Fusion weaves c. Box braids d. Any styling method that’s done improperly

4. Which of the following conditions can cause a receding hairline? a. Tight braids b. Ponytails c. Buns d. All of the above

5. What type of wig cap is the least likely to cause hair damage? a. Cotton caps b. Nylon caps c. Gel wig liners

6. You should remove bonding glue by… a. Saturating the area with braid spray b. Following the manufacturer’s instructions c. Having a professional remove it

Answers: 1) b. False. To protect the delicate hairs along the hairline, you should avoid excessive brushing; instead, smooth edges with your fingertips and apply a nutrient-rich hair product. 2) a. True. These hairs can easily be ripped out when you remove the weaves. 3) d. 4) d. 5) c. Gel liners such as Comfy Grip Wig Liners have a therapeutic headband filled with medical-grade gel. 6) b & c. Removed improperly, bonding glue can rip hair out from the roots. Though you should remove bonding glue according to manufacturer’s directions, you should have weaves professionally taken out.

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