Just keeping it real ~ it matters to me because people have it so twisted and Sickle Cell Disease is so much more than a pain crisis!

---Nita T.

Sickle Cell Disease:

Sickle cell disease is a condition which affects red blood cells.

It is a "tropical disease" where the red blood cells mutated to protect the body from malaria.  Those individuals have the Sickle Cell Trait. 

When two people with the Sickle Cell Trait have a child, there is a one (1) in four (4) chance that each child they have will be diagnosed with Sickle Cell Disease. 

People diagnosed with Sickle Cell Disease usually can't fight off malaria and usually die from it.

Sickle Cell Disease (SCD) affects more people in the world than any other genetic disease, was the first genetic blood disease to be understood and has served as a model in research to assist with the identification and treatment of other conditions.

It affects about 72,000 people in the US and affects millions of people worldwide!  It kills an estimated half-million people worldwide annually. Globally, almost one-half million babies die from SCD each year.

Within the US SCD affects primarily people of African descent. Throughout the world it affects people of various racial and ethnic backgrounds.  The facts are being updated world wide because SCD is being found in large numbers among Hispanics, Native Americans and Caucasians. SCD affects a large percentage of people in Mexcio, Central America as well as significant numbers in the Carribean and in South American countries such as Brazil. In the Mediterranean countries, SCD is found in people of Portuguese and Spanish descent, the French Corsicans, Sardinians, Sicilians, mainland Italians, Greeks, Turks and Cypriots. It is increasingly seen in the new births of European countries like France, Germany, and the UK,  also because of the migration from African countries. Sickle cell disease also affects large numbers of people in Middle Eastern countries of Pakistan, India, Saudi Arabia, and Asia. Although approximately 70 percent of the global number of SCD cases are in Africa SCD is the most frequently occurring genetic disease on earth.

Sickle Cell Trait:

Cell Component     Alteration                      Global Distribution

Membrane             Duffy antigen null          Africa

                            Melanesian

                            Elliptocytosis                *Melanesia

Hemoglobin          Hemoglobin S                Africa, Middle East, India

                           Hemoglobin C                Africa

                           Hemoglobin E                 S.E. Asia

                           ß-thalassemia                Africa,Mediterranean,

                                                                India, S.E. Asia, Melanesia

                           a-thalassemia                Africa, India, S.E. Asia

Red cell enzymes  G-6-PD deficiency          Africa, Mediterranean,       

                                                                India, S.E. Asia

*Melanesia (from Greek: μέλας black, νῆσος island) means "islands of the black-skinned people".  It’s one of the three main divisions of Oceania, in the SW Pacific Ocean, NE of Australia and S of the equator; it includes the Solomon Islands, Vanuatu, New Caledonia, Tuvalu, the Bismarck Archipelago, the Admiralty Islands, and Fiji.

By Shirley Hawkins | OW Staff Writer | 04.JUN.09

The silent disease

They are brave survivors who valiantly battle an illness that impacts the lives of thousands of Americans in the United States—and its name is sickle cell disease (SCD).

While diseases such as diabetes and breast cancer are well known among the general population, sickle cell disease—an inherited blood disorder that causes the red blood cells to become crescent or sickled-shaped, cluster together and cause blockages in the blood stream, is still relatively unknown by many in society.

Although the disease affects one in 400 African Americans, the disorder is also seen among Asians, Latinos and others with Mediterranean or African ancestry.

Approximately 130 babies are born in California with sickle cell disease every year. The disease is passed to the child when both parents have the sickle cell trait. According to Sickle Cell Disease Association of America, Inc. “sickle cell trait is an inherited condition, (but it) is not a type of sickle cell disease. People with sickle cell trait are generally healthy.

“Sickle cell conditions are inherited from parents in much the same way as blood type, hair color and texture, eye color and other physical traits. The types of hemoglobin a person makes in the red blood cells depend upon what hemoglobin genes the person inherits from his or her parents. Like most genes, hemoglobin genes are inherited in two sets…one from each parent.” When this happens with SCD, that both parents have the trait and each passes on one set, there is a 25% chance that their child will have the disease.

When the sickle shaped cell is unable to pass through tiny blood vessels, this can result in tissue and organ damage. Symptoms can be fatigue and strokes—even as young as age three, and include chronic pain, shortness of breath, pain in organs or joints, infections, pain and swelling in hands and feet, chest pain, fever, and the need for regular blood transfusions for severe anemia.

A sickle cell crisis can last anywhere from hours to days. Some people with sickle cell disease have only one crisis every few years, while others have many crises in a year.

Blood transfusions play a major role in treating the illness. The transfusions increase the number of normal blood cells in the body and help correct anemia. Blood transfusions are best tolerated when the donor blood closely matches the patient’s.

Los Angeles resident Morgan Todd, 28, has lived with sickle cell his entire life.

After pain entered his arms one evening, Todd traveled to the clinic at Midway Hospital Medical Center in the Fairfax district to see his doctor. Like many sickle cell patients who are diagnosed with the disease at a young age, clinics and hospitals become like a second home for sickle cell sufferers.

“Having sickle cell brings undeniable pain,” nodded Todd, who talked to Our Weekly via cell phone while waiting in his doctor’s office to receive an intravenous dose of saline fluids and Dilaudid, a pain medicine.

Like most sickle cell recipients, Todd must grapple with the day-to-day physical trials of living with the disease. The result is that living a normal life is a challenge.

As a child, Todd dreamed of joining the ranks of the National Basketball Association as the first player to be officially drafted into the league with sickle cell. But the strain of living with the disease dashed his hopes of joining a professional team. “I could just be having a regular day, and then suddenly my body starts to hurt,” said Todd, who said that the excruciating pain from sickle cell can occur at any time. “Sometimes the pain strikes the whole body, and sometimes it’s in the arm, leg or chest. Imagine the pain of breaking an arm. The pain of sickle cell is 10 times worse-and then imagine if you’ve endured that pain for 28 years.”

Todd’s family has been especially supportive. Brandon Norwood, 24, Todd’s younger brother, said that the family tries to keep Todd’s spirits up. “We take him back and forth to the emergency room when he needs treatment,” he said.

“You don’t hear news about people with sickle cell,” Norwood pointed out. “A lot of people in the Black community are not aware of the disease. There needs to be more awareness.”

Yolanda Allen, who serves on the board of directors of the Sickle Cell Disease Foundation and is the mother of 17-year-old sickle cell patient Jackie Allen, said that raising a child with sickle cell poses special challenges. “It’s a hard road,” admitted Allen. “The biggest challenge is that the disease is so spontaneous, you never know when the symptoms are going to occur. Jackie can be fine one minute and go into crisis the next—and it can happen in the middle of dinner or a conversation.”

Allen said that children with sickle cell often have to forego normal activities. “Jackie wants to go out and do things, but as a parent, you have to put your foot down. I tell her, you have to get your rest today because she can’t get overtired or dehydrated.”

Allen is also aware that sickle cell patients are often misunderstood by the medical community.

“There is not enough being done for the parents, the patients or the families grappling with sickle cell,” maintains Allen. “For example, when you go to the emergency room, many times the nurses don’t know enough about how to treat a patient with sickle cell disease. They’ll have us sitting there for hours when my daughter or other sickle cell patients are in dire need of hydration or pain medication.”

Pausing, Allen added, “The most serious complication for sickle cell patients is acute chest syndrome which is one of the leading causes of death in sickle cell patients. It can cause death within two to three hours.”

Like Norwood, Allen said that society is still not aware that sickle cell is affecting people and they have yet to find a cure (for everyone). “People say, ‘I thought sickle cell was cured,’ or ‘We don’t hear anything about it,’ but people don’t know that sickle cell is the most prevalent genetic disorder in the country.”

Roger Brown, director of development for the Los Angeles based-Sickle Cell Disease Foundation of California, the oldest sickle cell disease organization in the country, has witnessed firsthand the devastation caused by the disease.

Brown said it is not unusual for sickle cell patients to experience strokes, heart attacks, joint degeneration, and infection. “There is also organ and joint degeneration, sepsis or infection, leg ulcers, and a large occurrence of strokes. Currently, there is no cure for sickle cell, with the exception of bone marrow transplants.”

Brown said that the agency services 750 families throughout the state and offers counseling and other supportive wraparound services. He maintains that it is difficult for sickle cell patients to live a normal life. “Sickle cell disease can hit the carrier any time of the day or night, and it doesn’t affect any two people the same way.”

Where once sickle cell disease patients were faced with a short life expectancy, advanced medicines are allowing sickle cell patients to live longer lives. “Because of the advances in medical care, people are living longer,” he pointed out. “Quite frankly, many of the hospitals and medical care facilities are not equipped to deal with adult patients with sickle cell. They didn’t think these people were going to live long.”

The excruciating pain that sickle cell patients endure means that they often need pain reducing medication such as morphine and Demerol. “When they go into the emergency room to get these drugs, doctors and nurses often think the patients were drug users,” said Brown. “These doctors and nurses have to apologize later when they realize how wrong they were.”

The director said that maintaining a job or finishing school are often challenging for sickle cell patients. “In many cases, kids do not graduate from high school on time,” reported Brown. “Sufferers have a difficult time completing college or maintaining a job. The stress of living with sickle cell means their ability to compete in society is compromised. It affects them on a physical, social and emotional level.”

Trish Peterson, a pediatric nurse practitioner in the hematology program at Children’s Hospital Los Angeles (CHLA), said that mandatory screening for the disease in newborns began in 1990. Peterson said that early diagnosis is critical in order to identify or prevent SCD’s myriad symptoms and complications.

Peterson said that CHLA, home to one of the nation’s premiere centers for treating sickle cell disease, also offers a battery of wraparound services.

A tour of the facility reveals bedridden children who have been receiving treatment at Children’s Hospital since birth. Most are there to receive intravenous transfusions of blood or fluids. “We have a whole team approach that looks at how sickle cell is affecting their school performances and family,” said Peterson, adding that sickle cell advocates frequently visit schools to educate school nurses about the disease.

Blood drives are especially important in combating the disease. “SCD patients who receive frequent transfusions require specially matched blood, which further increases the need for donors. CHLA said that it needs 400 donors a month for patients. “We have been trying to reach out to the African American community,” said Peterson,” who said that CHLA regularly holds blood drives to solicit blood. “There are patients who need blood every two to three weeks, which are our chronic transfusion children. Then we have others who have complications from the disease such as when they have a stroke or acute chest syndrome and they need fresh supplies of blood.”

Charles Taylor, 45, was diagnosed with sickle cell at birth.

Although he maintains a positive outlook, the disease has taken a toll on Taylor’s employment. “When I was young, I wanted to be a police officer, but because I had the disease, it hindered me from passing the physical. I was eventually hired by the Los Angeles County Office of Education (LACOE) as a behavioral management instructional assistant, and I told the interviewers I had sickle cell disease,” said Taylor. “They understood that this was a particular challenge that I had. Despite having the disease, I was hired.”

Taylor, who has worked with LACOE for 18 years, said that he frequently has had to miss days each week from his job. “In every paycheck, I’ve had a deduction due to the fact that I had an episode with sickle cell. It took 15 years before I could even have a vacation because I had used up all my sick time for sickle cell.”

Noting the unpredictable nature of crises, Taylor added that, “One time I was driving down the freeway and I had to swerve away from another car to avoid an accident,” recalls Taylor. “My entire body went into panic mode and I experienced one big cramp from head to toe. That happened a number of times. I would have to drive myself to the hospital for treatment.”

Todd, who regularly visits Central Juvenile Hall to talk to youths about being tested for sickle cell, feels it is vitally important to educate the community about sickle cell disease. “Many Black people aren’t aware that this disease exists. If you ask them about leukemia, lupus, or cancer, everybody knows about those diseases,” he pointed out. “But when it comes to sickle cell, Black folks don’t know anything about it. It saddens me.”

Currently, bone marrow transplants have proven to be the only known cure for sickle cell disease, although complications from a transplant can be life threatening. As reported by the Sickle Cell Information Center at the Grady Health System in Georgia, “bone marrow transplants have been used to treat sickle cell disease for 20 years. . .The treatments—high doses of chemicals that knock out the patient’s own marrow before the transplant—are so toxic that adults with sickle cell-induced organ damage would be unlikely to survive them.” According to a Nov. 14, 2008 article in U.S. News and World Report, “bone marrow transplants rely on heavy doses of chemotherapy prior to a transplant in order to destroy a recipient’s bone marrow so it won’t reject the donated marrow. Finding a perfect bone marrow transplant is another obstacle, since the bone marrow must be an exact match. Family members are often tested for matches, especially siblings and parents of the patient.

When it comes to combating the disease, doctors recommend that people with sickle cell take good care of themselves and see a doctor regularly.

They also recommend that sickle cell patients maintain a healthy diet, get enough sleep and rest, avoid overexertion, stress, extremes of hot and cold, drink adequate amounts of water every day, get a flu shot and other vaccinations to prevent infections, and see a dentist regularly to prevent loss of teeth.

Allen said that the Sickle Cell Disease Foundation is pushing for changes in legislation to fund programs and services for sickle cell disease on the state and national level. “The Sickle Cell Disease Treatment Act of 2003 was supposed to be signed into law by President George W. Bush, which would have brought more resources for sickle cell patients, but it was never signed,” said Allen. “We are trying to get our state legislators to support our federal legislators to reauthorize $10 million in funding for that act, which would help to fund 10 comprehensive treatment centers nationwide. It would also support 40 demonstration projects to improve the prevention and treatment of sickle cell disease.”

Todd’s advice to other sickle cell patients is never give in to the disease. “Keep fighting,” he said. “Dealing with this disease is really hard, but I thank God that I’m alive and still battling this illness.”

To contact the Sickle Cell Disease Foundation of California, call (310) 693-0247.