Cord Blood Stem Cell Transplant for Sickle Cell Anemia

Cord blood stem cell transplant is a potential cure for sickle cell anemia, a blood disease passed on to children through genetic traits.

What is sickle cell anemia?

Sickle cell anemia is a serious inherited disorder where the body makes sickle-shaped, crescent-like red blood cells. Normal red blood cells are disc-shaped and contain an iron-rich protein called hemoglobin. They move easily through the blood vessels and carry oxygen from the lungs to the rest of the body. Sickle cells contain abnormal hemoglobin called sickle hemoglobin or hemoglobin S. Sickle cells are stiff and sticky. They tend to block blood flow in the blood vessels of the limbs and organs. Blocked blood flow can cause pain and organ damage. It can also raise the risk for infection.

What causes sickle cell anemia?

Sickle cell anemia is caused by a mutation (an abnormal change) in the gene that instructs the body to produce hemoglobin. A person is affected by sickle cell anemia only if he/she inherits the defective genes from both parents. If the gene is inherited from one parent, that’s known as the sickle cell trait, which means the person will not be affected by the disease. Instead, will be a carrier of sickle cell anemia and may pass the abnormal gene to their children.

What are the symptoms of sickle cell anemia?

Major symptoms at all ages include:

Fatigue
Pain crisis
Arthritis
Swelling and inflammation of the hands and feet
Liver congestion
Leg ulcers
Bone infarcts
Eye damage
Bacterial infection

Symptoms in babies and children include:

Fever
Abdominal pain
Bacterial infection
Painful swelling of the hands and feet
Sudden pooling of blood in the spleen

What are the complications of sickle cell anemia?

The symptoms of sickle cell anemia can have a significant impact on a person’s quality of life. If complications develop, these can be very serious.

Possible complications include:

stroke
vulnerability to infections
acute chest syndrome (the lungs suddenly lose their ability to breathe in oxygen)
pulmonary hypertension (the blood pressure inside the blood vessels that connect the heart to the lungs becomes dangerously high)

However, following improvements in preventative treatment, many complications associated with sickle cell anemia can be avoided.

When to seek urgent medical advice?

Because of the risk of potentially life-threatening complications, it’s important to look out for any signs or symptoms that the health of a person with sickle cell anemia has suddenly declined.

These include:

a high temperature (fever) of 38C (100.4F) or above
breathing difficulties
severe pain that can’t be controlled using over-the-counter painkillers

How does cord blood stem cell transplant cure sickle cell anemia?

Bone marrow is the substance in the center of the bones that produces red blood cells. A person with sickle cell disease has bone marrow that produces red blood cells with defective hemoglobin S. But if that bone marrow is replaced with healthy bone marrow through stem cells transplantation, a person’s body may start to produce normal hemoglobin.

Before transplantation, the person who has sickle cell anemia is treated with drugs that destroy his/her bone marrow cells. Then the stem cells are injected into the vein. After the process is complete, the stem cells begin to reform into new bone marrow cells. These new cells restore the immune system and make normal red blood cells.

Though bone marrow stem cells from a donor can be used in the transplant, cord blood stem cells that were banked at birth offer a more readily available, painless source of stem cells for transplantation. Another advantage is the cord blood of a baby can be a positive match to his/her siblings as well. Hence, cord blood banking is an investment towards a family’s healthy future.

References:
http://www.medicinenet.com/sickle_cell/article.htm
http://www.stlouischildrens.org/about-us/meet-our-patients/gabbys-story-cord-blood-transplant-cures-sickle-cell-disease
http://www.stemcellresearchfacts.org/sickle-cell-anemia