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Sickle Cell, Leukemia and Survivors Frequently Asked Questions


Q. What is sickle cell disease?

A. Sickle cell disease results from a single amino acid substitution in the beta globin chain of hemoglobin. This results in abnormal hemoglobin that is insoluble in conditions such as hypoxia (low oxygen levels) and dehydration creating sickled cells. These sickled cells are prone to hemolysis (breakdown of red blood cells) and vasocclusion (blockage of blood vessels). This leads to recurrent vasoocclusive pain episodes, hemolytic anemia and increased risk of infection due to damage to the spleen. There are estimated to be about 100,000 people affected with this disease in the United States.

Most Common Types of Sickle Cell Disease:

  • HbSS - The most severe form of the disease. These patients have two copies of the sickle hemoglobin gene).
  • HbSC- One sickle S and one hemoglobin C. Patients tend to have a milder form of the disease.

Sickle Thalassemia

  • HbSC - Inheritance of one sickle and one thalassemia gene that makes no beta globin (similar to HbSS)
  • HbSC - Inheritance of one sickle and one thalassemia gene that produces some beta globin. These patients tend to have a milder form of disease.

Q. What is leukemia?

A. Leukemia is a cancer of the bone marrow and blood and is the most common type of pediatric cancer. In leukemia, the bone marrow which is the factory that produces blood cells, makes “young” blood cells that don’t mature properly. These cells grow and crowd out the healthy cells that form the bone marrow. Leukemia is most common in children between two and six years of age but can occur from the newborn to the young adult. About 80% of pediatric leukemia is the acute lymphoblast variety (ALL) with 15-20% of the acute leukemia being acute myelogenous leukemia (AML). Chronic myeloid leukemia (CML) occurs in children but is much less common.

Q. What is the treatment of leukemia?

A. Leukemia is treated with chemotherapy (drugs that kill cells that grow rapidly) The chemotherapy is also given into the spinal fluid (the fluid surrounding the brain) to protect that area from developing leukemia. In some cases, radiation therapy to the brain is also used.

The therapy for acute lymphoblast variety (ALL) and acute myelogenous leukemia (AML) is different in intensity and duration.

Leukemia is a curable condition in the vast majority of children.

Q. Who is considered a survivor?

A. There are many ways to define a survivor. We define survivorship as anyone diagnosed with cancer or a similar illness at least five years ago that finished treatment at least 3 years ago and is now cancer free.

Q. Who can use the Center?

A. Cancer survivors treated at any hospital can come to the Center. We can help make lifetime follow-up plans and are available for single consultation or regularly scheduled visits.

Q. What happens on the first visit?

A. Before the first visit, we review all prior medical records which will allow us to create a summary of treatment from the day of diagnosis to the present. This information helps us determine the risk of long-term side effects, and develop personalized guidelines to help each patient maintain the best possible health. On the first visit patients meet with a pediatric oncologist for a complete physical exam and any required testing. At that time we will review any potential long-term effects from the cancer or treatment. Above all, we will spend as much time as needed to answer questions and help our patients develop a lifetime plan for wellness.

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