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Women's Health

Mercy Women's Care at St. Anne
3404 W. Sylvania Avenue
Toledo, OH 43623
419-407-1616

Mercy Women's Care at St. Charles
Navarre Medical Plaza
2702 Navarre Avenue
Suite 101
Oregon, OH 43616
696-7900

Mercy Women's Care at St. V's
2213 Cherry Street
Toledo, OH 43608
419-251-4340

Sickle Cell Disease

separator Sickle cell disease is an inherited blood disorder. When you have sickle cell disease, hemoglobin, which is a part of the red blood cells, doesn’t function well. We all rely on hemoglobin molecules in our blood to carry oxygen from our lungs to the body’s organs and tissues and to carry carbon dioxide back to the lungs.

Normal red blood cells are smooth and shaped like doughnuts. With sickle cell disease, hemoglobin molecules tend to cluster together and form long structures shaped like rods. This makes the red blood cells change their shape, from round and smooth to a sickle shape. When this happens, the cells can’t travel through the blood vessels to carry oxygen throughout the body. They stack up and cause blockages. Organs and tissue don’t get the oxygen they need to function well. This causes episodes of pain, and it can cause organ and tissue damage.

Another issue for people with sickle cell disease is that normal red blood cells last about 120 days in the bloodstream, but cells with the sickle shape last only about 10 to 20 days. The body can’t make replacement cells fast enough, so there’s a constant shortage. This causes anemia, which can make you feel constantly tired.

How do parents pass sickle cell disease to children?
Parents can pass the disease to their child if they are both carriers of sickle cell trait. About 1 in 12 African Americans has this trait. If both parents carry one sickle cell gene and one normal gene, then:
• There’s a 25 percent chance with each pregnancy that the child will inherit two sickle cell genes and consequently, will have sickle cell disease
• There’s a 25 percent chance that the child will have two normal genes and not have sickle cell disease or be a carrier
• There’s a 50 percent chance that the child will have one sickle cell gene and be a carrier, but have no symptoms

About 1 in every 500 African Americans has sickle cell disease.

Most common symptom: pain
When the blood vessels become blocked, pain and swelling are the result. In infants, pain and swelling in the hands and feet are often the first symptoms of sickle cell disease. Pain can occur in any joint or other part of the body. It’s unpredictable. Some people have very mild cases, and may only have episodes, also called “crises,” of pain once a year. Other people can have as many as 15 crises per year. When the crisis is severe, it might be necessary to go to the hospital to have fluids given through a vein and to receive painkillers.

Sickle cell disease can cause eye problems, yellowing of the skin and eyes, shortness in both children and adults, infections, stroke and what’s called “acute chest syndrome,” which is similar to pneumonia. But every individual who has sickle cell is unique, and it’s not possible to say who will have a mild case and who will have a severe one.

Frequent medical visits are crucial
If you or your child has sickle cell disease, the most important thing is to have a close relationship with your doctor so that you can address the issues in each crises as soon as they arise. Treatment for sickle cell consists primarily of easing the pain, having good nutrition, limiting stress as much as possible, being well rested, and preventing infections as much as possible. Working closely with your doctor is the best way to learn how to take care of yourself well.

If your child has sickle cell disease
It’s important for kids to lead their lives as normally as possible. But parents need to watch out for certain things in order to prevent sickle cell crises, such as:

To prevent tissue deoxygenation, avoid

  • Strenuous activity (especially if the spleen is enlarged)
  • Emotional stress
  • High altitudes (such as mountainous areas)
  • Anything that’s been known to cause infection

To prevent dehydration:

  • Recognize the signs of dehydration (dry mouth, little urine output, no production of tears, lethargy)
  • Avoid long exposure to the sun
  • Make sure fluids are easily available—at home, at school, at friends’ homes, etc.


To avoid infection

  • Make sure your child gets all vaccinations according to the schedule your doctor recommends
  • Make sure teachers and other caregivers know how to care for your sickle cell child
  • Consider having your child wear a MedicAlert bracelet

Families who have children with sickle cell often benefit from counseling to help them with the psychological, physical and economic issues involved with the disease. Get all the help you feel you and your family need. Your doctor and other members of your healthcare team can help connect you with support groups and counselors.

Source:
American Sickle Cell Anemia Association; National Institutes of Health.



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