ABOUT SICKLE CELL ANEMIA

 

With sickle cell disease, an inherited group of disorders, red blood cells contort into a sickle shape. The cells die early, leaving a shortage of healthy red blood cells (sickle cell anemia), and can block blood flow, causing pain (sickle cell crisis).

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The most common type of sickle cell disease issues:

• Hand-foot syndrome                                                                               

• (dactylitis)

• Fatigue

• Pain, plus

• Eye problems

• The blockage of the blood vessels in the eye caused by sickle cells can severely damage the retina and may lead to blindness.

• Jaundice

• Yellowing of the skin and eyes, which is caused by the rapid breakdown of red blood cells.  This produces excessive amounts of bilirubin in the body.

• Infections

• Sickle cells can damage the kidneys and other body organs. When the kidneys become damaged, it is hard for the body to hold water. 

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Children and adults with sickle cell anemia are more vulnerable to infections and have harder time fighting infections once they start.

•  Vulnerability to infections is the result of damage to the spleen from the sickled red cells.  This prevents the spleen from destroying bacteria in the blood.

• Infants and young children are especially susceptible to bacterial infections.

  • Bacterial infections can kill in as little as 9 hours from the onset of fever.

• Pneumococcal infections were the principal cause of death in young children with sickle cell anemia.

• Deaths were reduced when physicians routinely gave penicillin (prophylactic penicillin) to infants identified at birth or in early infancy with sickle cell anemia.

  

Stroke

• Sickle cell causes damage to the walls of the blood vessels by sticking to them.

  • This action restricts the flow of blood to the brain, causing a stroke.

  • This type of stroke occurs primarily in children.

 

Acute chest syndrome

Acute chest syndrome is a life-threatening complication of sickle cell anemia, similar to pneumonia.

• This syndrome is caused by:

  •  infection, or

  • Sickled cells trapped in the lungs.

  • This syndrome is characterized by:

    • Chest pain

    •  fever, and

    • Abnormal chest x-rays.

 

What persons with sickle cell anemia should do?

It is important for those with sickle cell to stay hydrated. Drinking water promotes healthy blood flow and reduces the chance of our red blood cells sickling and sticking together. It keeps our blood cells supple, so they can move more freely within our blood vessels.

 

​Water is the best fluid, but also helpful is:

• Milk

• Soup

• Fruit

• Sports drinks, and

• Juice (that is more alkaline).

Avoid caffeine.

• Caffeine can cause the kidneys to release more water into the urine.

Important symptoms

Fever is usually an important indicator of infections.

•  Infections can present a life-threatening situation for individuals with sickle cell.

See a doctor or healthcare provider immediately — especially if a child with sickle cell has a fever of 101° or higher.

Signs of fever in babies include:

• Extreme crankiness

• Incessant crying

• Rapid breathing

• Screaming even when touched or held by family members

• Lack of energy

• Poor appetite

• A decrease in wet diapers (which indicates dehydration)

What is folate and why is it important?

Folate, often called folic acid, is one of the most important of all vitamins.

• Folic acid helps make red blood cells

• Folic acid helps renew all the cells in the body.

When the body does not have enough folate, many health problems can result.

• The body’s ability to take in important nutrients can decrease, and the production of new red blood cells can be impacted.