What precautions or lifestyle modifications are needed to help control sickle cell disease?

Sickle cell anemia (SCA), sometimes called sickle cell disease, is a blood disorder that causes your body to make an unusual form of hemoglobin called hemoglobin S. Hemoglobin carries oxygen and is found in red blood cells (RBCs).

While RBCs are usually round, hemoglobin S causes them to be C-shaped, making them look like a sickle. This shape makes them stiffer, preventing them from bending and flexing when moving through your blood vessels.

As a result, they can get stuck and block the flow of blood through blood vessels. This can cause a lot of pain and have lasting effects on your organs.

Hemoglobin S also breaks down faster and can’t carry as much oxygen as typical hemoglobin. This means that people with SCA have lower oxygen levels and fewer RBCs. Both of these can lead to a range of complications.

Sickle cell anemia is a genetic condition that people are born with, meaning there’s no way to “catch” it from someone else. Still, you don’t need to have SCA in order for your child to have it.

If you have SCA, this means that you inherited two sickle cell genes — one from your mother and one from your father. If you don’t have SCA but other people in your family do, you may have only inherited one sickle cell gene. This is known as sickle cell trait (SCT). People with SCT only carry one sickle cell gene.

While SCT doesn’t cause any symptoms or health problems, having it does increase the chances of your child having SCA. For example, if your partner has either SCA or SCT, your child could inherit two sickle cell genes, causing SCA.

But how do you know if you carry a sickle cell gene? And what about your partner’s genes? That’s where blood tests and a genetic counselor come in.

You can find out whether you carry the sickle cell gene through a simple blood test. A doctor will take a small amount of blood from a vein and analyze it in a laboratory. They’ll look for the presence of hemoglobin S, the unusual form of hemoglobin involved in SCA.

If hemoglobin S is present, it means you have either SCA or SCT. To confirm which one you have, the doctor will follow up with another blood test called hemoglobin electrophoresis. This test separates out the different types of hemoglobin from a small sample of your blood.

If they only see hemoglobin S, you have SCA. But if they see both hemoglobin S and typical hemoglobin, you have SCT.

If you have any kind of family history of SCA and plan to have children, this simple test can help you better understand your chances of passing on the gene. The sickle cell gene is also more common in certain populations.

According to the Centers for Disease Control, SCT is most common among African-Americans. It’s also found more often in people with ancestors from:

  • sub-Saharan Africa
  • South America
  • Central America
  • the Caribbean
  • Saudi Arabia
  • India
  • Mediterranean countries, such as Italy, Greece, and Turkey

If you’re not sure about your family history but think you might fall into one of these groups, consider doing a blood test just to be sure.

Genetics is a complex subject. Even if you and your partner are screened and found to both carry the gene, what does this actually mean for your future children? Is it still safe to have children? Should you consider other options, such as adoption?

A genetic counselor can help you navigate both your blood test results and the questions that come up afterward. Looking at test results from both you and your partner, they can give you more specific information about the chances of your child having either SCT or SCA.

Finding out that any future children with your partner could have SCA can also be difficult to process. Genetic counselors can help you navigate these emotions and consider all of the options available to you.

If you live in the United States or Canada, the National Society of Genetic Counselors has a tool to help you find a genetic counselor in your area.

SCA is an inherited condition, which makes it hard to prevent. But if you’re concerned about having a child with SCA, there are a few steps you can take to ensure that they won’t have SCA. Remember, children inherit genes from both partners, so make sure your partner takes these steps as well.

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

What precautions would you suggest to avoid sickle cell anemia?

Taking the following steps to stay healthy might help you avoid complications of sickle cell anemia:.
Take folic acid supplements daily and choose a healthy diet. ... .
Drink plenty of water. ... .
Avoid temperature extremes. ... .
Exercise regularly, but don't overdo it. ... .
Use nonprescription medications with caution. ... .
Don't smoke..

What activities lifestyle practices can you recommend to those with SCD to maintain a healthy respiratory and circulatory system?

Aim for about 2½ hours of moderate exercise a week, such as walking or biking. Talk with your doctor before starting a new exercise routine. Physical activity is key in staying healthy. At the same time, you don't want to overdo it.

Can sickle cell disease be prevented or treated with healthy lifestyle choices?

Lifestyle changes will not cure sickle cell disease. However, they may reduce the number and severity of sickle cell crises.

How can sickle cell infection be prevented?

Hand Washing. Washing your hands is one of the best ways to help prevent getting an infection. People with sickle cell disease, their family, and other caretakers should wash their hands with soap and clean water many times each day. If you don't have soap and water, you can use gel hand cleaners with alcohol in them.