What important health teaching should be included in the care of patients with anemia?

Sickle cell disease is an inherited blood disorder affecting red blood cells. Normal red blood cells contain hemoglobin A. People with sickle cell disease have red blood cells containing mostly hemoglobin S, an abnormal type of hemoglobin. These red blood cells become sickle-shaped (crescent-shaped), and have difficulty passing through small blood vessels. There are several different types of sickle cell disease; the most common types are homozygous sickle cell disease (SS disease), and sickle-cell beta thalassemia (Sß+ or Sß0 disease).

Why would I want a primary care physician? How common is sickle cell disease?
Who gets sickle cell disease?
What are the symptoms of sickle cell disease?
How do people get sickle cell disease?
Important facts about sickle cell disease
How can I prevent complications of sickle cell disease?
Where can I find additional resources about sickle cell disease?
If you live in Maryland and are affected by sickle cell disease

How common is sickle cell disease?

Sickle cell disease is one of the most common diseases in the world, with approximately 300,000 babies being born each year with some form of the disease. Sickle cell disease is the most common disease detected by newborn screening efforts in the United States, as between 1 out of every 2500 and 1 out of every 2000 babies born in the U.S. has some form of the disease. Overall, it is estimated that approximately 100,000 persons in the U.S.are living with sickle cell disease.

Who gets sickle cell disease?

Sickle cell disease can affect persons of any racial or ethnic background. In the U.S., African-Americans are most likely to have the disease, though it is found among many different racial and ethnic groups, including whites, Hispanics, Native Americans, and Southeast Asians.

What are the symptoms of sickle cell disease?

Sickle cell disease causes a number of health complications for the affected individual. Persons with sickle cell disease are more susceptible to infections and strokes. Sickle cell disease can cause progressive organ damage throughout the body, including the lungs, kidneys, and joints. Some people with sickle cell disease may develop neurocognitive deficits. The hallmark symptoms of the disease, however, are the episodes of severe acute pain, called vaso-occlusive crises (or sickle cell crises), that the individual can experience. These pain episodes can affect any part of the body. These episodes can be as short as a few hours in length, or they may last for days on end. In addition to this severe, acute pain, the disease is the source of chronic pain as well (in the hips, back, or other joints, for example). Sickle cell disease can cause early mortality, and even though people with the disease are living longer, it is estimated that persons with the most severe form of the disease (sickle cell anemia) have a median life expectancy approaching 50 years.

How do people get sickle cell disease?

• Sickle cell disease is not spread like a cold and cannot be caught from another person.
• It is an inherited condition (one that you are born with).
• Sickle cell trait is a carrier condition for sickle cell disease.
• Sickle cell trait originated many years ago in areas of the world where malaria was present.
• People with sickle cell trait inherit one gene for normal hemoglobin A and one gene for defective hemoglobin S.
• People with sickle cell disease inherit a hemoglobin S gene from one parent and another abnormal hemoglobin from the other parent (i.e., Hemoglobin S., Hemoglobin C, or Beta Thalassemia).
• If both parents have hemoglobin S trait, there is a one-in-four chance with each pregnancy the child will have SS disease.
• If one parent has hemoglobin S trait and one parent has hemoglobin C trait, there is a one-in-four chance with each pregnancy that the child will have SC disease.
• If one parent has hemoglobin S trait and one parent has beta thalassemia trait, there is a one-in-four chance with each pregnancy that the child will have Sß+ or Sß0 disease.

Important facts about sickle cell disease

• Diagnosis of sickle cell disease can only be determined by a special blood test. This has been part of newborn screening for all children in the United States since July 1, 1985.
• Comprehensive care includes early diagnosis, preentive measures, treatment of complications, and ongoing patient education.
• Many people with sickle cell disease live long and productive lives.
• Individuals with sickle cell disease can pursue a variety of vocations and professions.
• Many adolescents with sickle cell disease experience delayed puberty (the average delay is about two years).
• Yellowing of the eyes is common and should not be confused with hepatitis.
• The use of alcohol, "street drugs," and tobacco can greatly increase the risk of developing serious complications.

How can I prevent some of the complications of sickle cell disease?

With the necessary support, people with sickle cell disease should be able to lead normal lives.

Preventing Infections

• People with sickle cell anemia need to keep their immunizations up to date, including Haemophilus influenza, pneumococcal, meningococcal, hepatitis B, and influenza.
• Some patients may receive antibiotics to prevent infections.

Preventing Crises

There is no way to always prevent sickling and sickle cell crises. Here are some gneeral ideas that may help:

• Get enough oxygen
• Drink plenty of fluids
• Avoid getting over-heated or getting very cold
• Avoid getting infections, and quickly treat infections when you do get them
• See a primary care doctor

More specific recommendations to reduce the number of sickle cell crises an affected person may experience include taking the following precautions:

• To prevent oxygen loss, avoid:
  • Demanding physical activity (especially if the spleen is enlarged)
  • Emotional stress (or learn how to better cope with it)
  • Environments with low oxygen (high altitudes, nonpressurized airplane flights)
  • Smoking
  • Known sources of infection
• To make sure you're getting enough fluids:
  • Avoid too much exposure to the sun
  • Have fluids on hand, both at home and away
  • Recognize signs of dehydration
• To avoid infection:
  • You or your child should be vaccinated as recommended by the health care provider
  • Share the above information with teachers and other caretakers when necessary
  • Seek emergency medical attention for any temperature of 101.50 F or higher
  • Practice good hand hygiene, especially washing hands frequently when coughing or sneezing or caring for others who have colds.

Where can I find additional resources and information about sickle cell disease?

The Sickle Cell Association of America advocates for the needs of persons with sickle cell disease. They have member organizations all across the U.S. Please see their website for more information: http://www.sicklecelldisease.org

The Sickle Cell Information Center based in Atlanta, Georgia provides a wealth of news, information, and links to resources about sickle cell disease. Please see their website at: http://www.scinfo.org

The National Heart, Lung, and Blood Institute of the National Institutes of Health is one of the federal organizations that oversees research related to sickle cell disease. See their website for news, education, and other information about the disease: http://www.nhlbi.nih.gov/new/sicklecell.htm

If you live in the State of Maryland and are affected by sickle cell disease:

A number of community-based organizations exist in Maryland that seek to provide education, information, and support for persons and families affected by sickle cell idsease. See the below links for more information:

• The William E. Proudford Sickle Cell Fund
• The Maryland Sickle Cell Disease Association

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