September is Sickle Cell Awareness Month

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Sickle Cell

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The Sickle Cell Program at Cook Children's Sickle Cell Center serves more than 400 children annually across northern Texas and adjoining states.

Our program offers testing, diagnosis and treatment for the many types of sickle cell disease. Research is also an important part of our program as we seek new ways to treat and prevent the many complications that can occur as a result of this disease.

Sickle cell disease is a group of inherited red blood disorders affecting an important protein in the red blood cell. This protein is called hemoglobin. Hemoglobin helps the red blood cell to carry oxygen to all the different parts of the body. In sickle cell disease, the body produces one or more abnormal types of hemoglobin. This means that the ability of the red blood cell to carry oxygen to all parts of the body is changed. Sickle Cell Disease is a lifelong illness that can result in serious health problems.

There are different forms of Sickle cell disease which are named depending on whether a person inherits a sickle cell trait from each parent or if the person inherits sickle cell trait from one parent and a different abnormal hemoglobin trait from the other parent. Some common types of sickle cell disease include SS disease also known as Sickle Cell Anemia, Sickle Hemoglobin C disease, and Sickle Beta Thalassemia.

Sickle cell disease affects about 70,000-80,000 people nationwide. People with this disorder have atypical hemoglobin molecules including hemoglobin S, which can distort red blood cells into a sickle shape, thus the name "sickle cell disease." Normal blood cells are smooth, round, flexible and shaped like the letter O so they flow easily through the veins. However, if your child has sickle cell disease, his blood cells can become stiff, sticky and shaped like the letter C, causing a variety of problems. The sickle cells can block veins, causing damage to organs, and can also cause mild to severe and sometimes excruciating pain for the patient. Sickle cells also have a shorter lifecycle, which results in anemia because the body cannot regenerate new cells fast enough.

Sickle cell disease is a genetic or "inherited" disease, meaning it is passed down from parent to child. In the U.S., approximately 3 million people carry the sickle cell trait, meaning they don't have sickle cell disease, but they are carriers for it. When each parent carries one of the gene traits, it is possible for their child to be born with sickle cell disease.

The sickle cell gene is most common among people whose ancestry is

African
Arabian
Asian Indian
Mediterranean
Hispanics from Caribbean, Central and South America

Fortunately for patients today, advanced testing, early diagnostics, research and state-of-the-art treatments help many patients to live longer, healthier lives.

graphic-sicklecellgene

Depending on the type of sickle cell disease, your child may not have any symptoms or the symptoms could range from mild to severe. Some of the most common are:

Jaundice (yellowing of the eyes and/or skin)
Anemia
Pain
Delayed growth
Facial bone deformities
Stillbirth
Enlarged spleen
Gallbladder disease
Damage to the kidneys, lungs, and other vital organs
Stroke
Bacterial infections

Newborn screening – All children born in the United States are screened for sickle cell disease. As a result, most children are diagnosed early in life.
Genetic testing and specific types of blood tests enable the specialists at Cook Children's to determine the type of sickle cell disease the patient has and helps to determine the best course of long- and short-term treatment.
Complete blood count (CBC) identifies anemia
Hemoglobin electrophoresis identifies an abnormal form of hemoglobin

Stroke is a major complication of SCD. Children with SCD are much more likely to have a stroke than are children in general. Children with severe types of sickle cell disease receive routine monitoring with a test called a Transcranial Doppler (TCD) to determine which children are at highest risk for stroke. A program of regular red blood cell transfusions has been shown to reduce stroke risk in people with SCD who are found to be at highest risk. Learn more about the Cook Children's stroke center here.

The types of treatments and therapies your child receives will depend upon his or her diagnosis. Treatments and therapies are designed to control sickle cell disease and to alleviate the complications that result from the disease and include:

Antibiotics
Pain management
Medications to improve red blood cells
Blood transfusions
Surgery

One of the greatest advances in the management of sickle cell disease is stem cell transplantation. A stem cell transplant replaces unhealthy red blood cells with red blood cells from a healthy donor and in most cases, can restore a patient to a sickle cell disease free life. A patient's eligibility for stem cell transplant requires a donor match. The people most likely to provide a match are full siblings. It is also sometimes possible to find a match from outside the family.

Cook Children's Hematology and Oncology Center is actively involved in sickle cell disease research, including stem cell transplantation. Our recognized team of researchers works with leading organizations across the U.S. and around the globe to find solutions to treating, preventing and curing the disease. As a research facility, we also have a number of clinical trials available and we are able to match our patients to trials at other facilities. To learn more about the ongoing research at Cook Children's, click here.

photo-podcast-clarissajohnson

Advanced Treatments and Research for Sickle Cell Disease

Dr. Clarissa Johnson, director of the Sickle Cell Program at Cook Childrens, talks about advanced treatments and therapies as well as promising new clinical research on the horizon for sickle cell disease. Her extensive knowledge and passion for treating patients with sickle cell disease is the driving force behind bringing relief to hundreds of patients at Cook Children’s.

Listen to podcast

We are here to help.

If your child has been diagnosed, you probably have lots of questions. We can help. If you would like to schedule an appointment, refer a patient or speak to our staff, please call our offices at 682-885-6152.

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September is Sickle Cell Awareness Month

Sickle Cell

What is sickle cell disease?

Who gets sickle cell disease?

Sickle cell symptoms and complications

Testing and diagnosis

Treatment

Research

Advanced Treatments and Research for Sickle Cell Disease

We are here to help.

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