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 is 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 can not 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:
The most common forms of Sickle Cell Disease are:
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.
When one parent has Sickle Cell Anemia (SS) and the other parent has
normal hemoglobin (AA), all their children will have Sickle Cell Trait
(AS).
| Remember, every person has two copies of hemoglobin genes |
Parent with Sickle Cell Anemia (SS) |
|
| Parent with Normal Hemoglobin (AA) |
S GENE |
S GENE |
| A GENE |
AS |
AS |
| A GENE |
AS |
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)
| Remember, every person has two copies of hemoglobin genes |
Parent with Sickle Cell Anemia (AS) |
|
| Parent with Normal Hemoglobin (SS) |
A GENE |
S GENE |
| S GENE |
AS |
SS |
| S GENE |
AS |
SS |
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
