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Sickle Cell Disease

What is Sickle Cell Disease?

Sickle Cell Disease is an inherited disease that affects our hemoglobin.  Inherited means it is passed from mothers and fathers to their children. Hemoglobin is a special protein substance inside the red blood cells that is responsible for carrying oxygen from the lungs and takes it to every part of the body.  Every person has two copies of the hemoglobin gene.  A change in the hemoglobin from the normal hemoglobin type may cause the symptoms of Sickle Cell Disease.  
 

Hemoglobin also gives blood its rich red color. Sickle Cell Disease can be a chronic illness.  Chronic illnesses are an
extremely expensive and highly stressful health-care crisis.

Chronic illness is a challenge for many patients and their family members.  
 

Chronic illness is defined as a condition that last over one year, limits the persons activities and requires on-going treatment and health care maintenance.  Sickle Cell Disease has been placed on the back burner and many people are not aware of this condition.  Education, testing and counseling is vitally important for persons affected by a chronic illness.

Sickle Cell Disease is a group of related disorders that affect the red blood cells.  Sickle Cell Disease occurs when a person inherits two sickle cell genes or a combination of one sickle cell gene plus any one of several other abnormal hemoglobin gene that affect the red blood cells.

Most people have normal/adult hemoglobin genes symbolized by the letter "AA". Normal red blood cells are soft, smooth and round. With Sickle Cell Disease, the red blood cells become hard, sticky, and their shape is like a banana or sickle.  Sickle red blood cells have trouble moving through small blood vessels.  Sometimes they clog up the blood vessels so that blood cannot bring oxygen to the tissues. This clogging causes severe pain and other problems. Sickle Cell Disease can cause many complications.  

The most common problems are:

  • Pain

  • Infections

  • Anemia (low blood)

  • Damage to the body organs

The most common forms of Sickle Cell Disease are:

  • Sickle Cell Anemia (SS)

  • Sickle Cell Hemoglobin C Disease (SC)

  • Sickle Cell Harlem Disease

Sickle Beta Thalassemia is a less common form of Sickle Cell Disease. The effects of Sickle Cell Disease vary greatly from one person to the next. It is important for everyone to get tested so they will know their hemoglobin type and the possibilities of passing sickle genes to their children.

There are several possibilities for children whose parent(s) have Sickle Cell Trait or Sickle Cell Disease.  Genes are passed from parents to their children.

 

pic of sickle cells.d.jpg
Normal red blood cell
Sickled red blood cell

When one parent has Sickle Cell Anemia (SS) and the other parent hasnormal hemoglobin (AA), all their children will have Sickle Cell Trait(AS).

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When one parents have Sickle Cell Trait (AS) and the other parent as Sickle Cell Anemia (SS), the chances for each pregnancy are:

1 in 2 (50%) that the baby will have Sickle Cell Trait (AS)
1 in 2 (50%) that the baby will have Sickle Cell Anemia (SS)

SC chart 1_edited.jpg

If both parents have Sickle Cell Anemia (SS), all their children will have Sickle Cell Anemia (SS).

Health Problems Related to Sickle Cell Disease

The effects vary greatly from one person to the next person.

  • Infections

  • Anemia

  • Organ Damage (heart, lungs, kidney, brain, abdomen, spleen, eyes and liver)

  • Pain

  • Complications during pregnancy

  • Hand and foot syndrome

  • Leg Ulcers

  • Jaundice

  • Chest pains and trouble breathing

  • Strokes (i.e. heart and brain)

  • Gallstones

  • Blood in the urine

  • Pneumonia

  • Painful erection in men

  • Exhaustion and tiring

  • Delayed onsets of puberty and growth retardation in both sexes

  • Nosebleeds

  • Frequency of urination

  • Kidney infections

  • Destruction of parts of the bones

  • Disturbance in vision

  • Enlargement of spleen or heart

  • Heart murmurs

  • Poor appetite

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