WHAT IS SICKLE CELL DISEASE?

Sickle Cell Disease is an inherited, but not contagious, blood disorder where normal round shaped red blood cells change to a crescent (sickledor “quarter-moon” shape.   Sickled shaped cells have difficulty passing through small blood vessels so they often jam up, blocking the flow of blood and oxygen to body parts and tissues causing extreme pain.  Lack of oxygen flow can also damage muscles, bones and internal organs and lead to other serious medical problems.  Today, approximately 1 in 500 African American children is born with a serious sickle cell disease, making it the most common long term illness identified in this population.  In Virginia, Approximately 1 in 325 babies are born with sickle cell disease, far higher than the national average.
Sickle Cell Disease is among the most prevalent of genetic diseases in the United States.  It is one of the most common chronic illnesses found in African Americans.  It is estimated that over 4,000 persons in the Commonwealth of Virginia, are potentially living with the disease.
The most common forms of Sickle Cell Disease are:
  • Sickle Cell Anemia (Hemoglobin SS)
  • Sickle C Disease (Hemoglobin SC)
  • Sickle Beta-Thalassemia

WHAT IS SICKLE CELL TRAIT?

The term Sickle Cell Trait is used for the condition characterized by the presence of normal adult hemoglobin (Hb A) and sickle hemoglobin (Hb S) in an individual’s hemoglobin make-up.  Sickle Cell Trait is the gene carrier state of the disease.  People with the trait are usually healthy and experience little or no discomfort due to the sickle cell hemoglobin.  However, those persons with trait should avoid high altitudes, unpressurized aircraft, deep sea diving, extreme fatigue or overly strenuous physical activity.  Since you have no symptoms, you may not know you have Sickle Cell Trait unless you take a hemoglobin electrophoresis blood test.  Every person of childbearing age should be tested to find out whether they have Sickle Cell Trait.  One in every twelve African Americans has Sickle Cell Trait.  

ARE SICKLE CELL ANEMIA AND SICKLE CELL TRAIT AND OTHER FORMS OF SICKLE CELL DISEASE ONLY FOUND IN BLACKS?  ABSOLUTELY NOT!  

Although Sickle Cell Disease occurs mainly in people of African American Heritage who inhabit Africa, North, South and Central America, Puerto Rico, the Caribbean and West Indies Islands, it can be found in the Mediterranean and Asian areas of Greece, Italy, India, Sicily, Turkey, Thailand, Korea, Iran, and Saudi Arabia.

SYMPTOMS OF SICKLE CELL DISEASE

THE PAINFUL EPISODE OR SICKLE CELL CRISIS is the most common symptom suffered by those born with a sickle cell disease.  The patient experiences severe pain in chest, abdomen, arms, legs or hips.  An attack can last for days or weeks; may occur several times a year.  

OTHER SYMPTOMS

Symptoms usually appear after six months of age, even though sickle cells are present at birth.  Some cases are mild and these symptoms may not always be apparent.
  • Anemia – less than the normal amount of hemoglobin in the blood.  Sickle cells have a shorter life than regular red blood cells.  The body cannot make new red blood cells fast enough to replace old ones.  Red blood cells in a typically normal person live for about 120 days.  In a person with sickle cell disease, the red blood cells live less than 50 days.  With fewer red blood cells, organs and tissues do not get the oxygen they need.
  • Jaundice – a yellowish tinge on white of eyes.
  • Shortness of breath – difficulty in breathing/labored breathing.
  • Fatigue – weariness from exertion.
  • Infections – bacterial contamination.
  • Leg Ulcers – sores around the ankles that don’t easily heal.
  • Slow Growth – small size and poor general health.
  • Painful Joints – arthritic-like aches and pains.
  • Hand-Foot Syndrome – hands and feet swell, become hot, red, and painful.
  • Complications During Pregnancy – Women with sickle cell disease can have a healthy baby.  However, risks are involved, and both mother and the baby should be closely monitored by a health-care provider.
  • Organ Damage – Lack of oxygen over many years can lead to tissue and organ damage including the lungs, heart, kidneys, liver and spleen causing numerous medical problems. 

OTHER PROBLEMS ASSOCIATED WITH SICKLE CELL DISEASE 

  • Stroke
  • Gallstones
  • Pneumonia
  • Blood in the urine
  • Chest pains and trouble breathing
  • May develop Pulmonary Hypertension 
  • Vision problems
  • Loss of hearing
  • Priapism (painful, persistent erections in men caused by sickling)

SATISTICS

  • Approximately 1 in 500 African American children is born with a serious Sickle Cell Disease while 1 in every 12 African Americans has Sickle Cell Trait.  One in every 36,000 Hispanics has Sickle Cell Disease.
  • In Virginia, approximately 1 in 325 babies is born with Sickle Cell Disease, far higher than the national average.
  • An estimated 100,000 people in the United States live with Sickle Cell Disease while over 1 million persons globally are affected with the disease.  Over 2 million people in the USA have Sickle Cell Trait.
  • Around 4,000 people in Virginia live with Sickle Cell Disease while around 155,000 people in Virginia have Sickle Cell Trait.
  • Approximately one out of 1,000 African-Americans has Sickle Beta-Thalassemia disease.
  • Approximately one out of 50 African-Americans has Beta-Thalassemia trait.  There is also an increased incidence of Beta-Thalassemia trait among Italians, Greeks and Asians.
  • The National Institutes of Health (NIH) estimates that almost one-third of adults with SCD develop pulmonary arterial hypertension, a life-threatening condition resulting in a 10-fold greater risk of death.
  • Due to this high disease burden, the Sickle Cell Disease Association of America (SCDAA) reports that Sickle Cell Disease results in an estimated 750,000 hospitalizations a year. The cost of these hospitalizations is estimated at $475 million annually.
People with Sickle Cell Disease have a much longer life expectancy now.  When rules of good health are followed, discomfort, pain and spells of illness or crisis may be less frequent.  There are some simple things, which will make life more comfortable for Sickle Cell Disease patients.

EVERYONE NEEDS TO:

  • Have a primary physician knowledgeable about Sickle Cell Disease
  • Have regular visits to your physician
  • Have regular eye exams
  • Have regular dental checkups
  • Maintain immunization schedules
  • Drink at least eight, 8-ounce glasses of water daily
  • Drink more if you have a fever or are experiencing a crisis
  • Get 8 hours of rest each night, more when in crisis
  • Eat complete and balanced meals; avoid junk foods and carbonated beverages
  • Avoid strenuous exercise.  Don’t push too hard to “keep up”.
  • Avoid exposure to extreme temperatures
  • Avoid stress, fatigue, colds and infections
  • Avoid low oxygen level situations such as flying in an un-pressurized aircraft, mountain climbing or scuba diving
  • Exercise moderately
  • Parents or guardians should continue to encourage independence in their children
  • Wear a medic alert bracelet or pendant
  • Carry a form that provides all information needed by hospital emergency workers, including your doctor’s recommended emergency room treatment with your doctor’s signature.