Sickle Cell Disease

Why choose us for sickle cell disease care?

Sickle cell disease is an inherited red blood cell disorder that disrupts the flow of oxygen to tissue and organs. Experts at Mays Cancer Center, home to UT Health San Antonio MD Anderson Cancer Center, deliver comprehensive care that helps provide a good quality of life for adults and children.

Our team includes fellowship-trained pediatric and adult blood disorder experts (hematologists) who deliver trusted therapies. We use leading care practices, including newly approved medications, to prevent and treat complications.

Sickle cell disease is one of the many blood disorders we treat. Read more about our blood disorder program.

What you need to know about sickle cell disease

• Your red blood cells contain hemoglobin, a protein that transports oxygen throughout the body. With sickle cell disease, genetic changes make the sickle hemoglobin molecules fragile. Red blood cells with sickle hemoglobin take on the curved shape of a sickle rather than staying round and flexible.
• Sickle-shaped cells break apart easily and may block the normal flow of blood through vessels. When sickled red blood cells cause a sudden blockage (vaso-occlusion), blood can’t flow normally.
• When blood does not flow, you may experience complications, including sudden pain attacks (crises) and serious breathing problems. These problems sometimes require immediate care in the hospital.
• You can receive inpatient services at University Hospital and clinic-based (outpatient) services at Mays Cancer Center. Coming to the cancer center and seeing a hematologist oncologist does not mean that you have cancer.

How to talk to your doctor about sickle cell disease

Living with sickle cell disease requires ongoing treatment. You’ll do some of your treatment at home. We stay in regular contact with you, developing and tailoring care plans to help you stay as healthy as possible. We encourage you to ask questions at any time.

Feel free to ask us about:

• Preventive services, including evaluations from lung doctors (pulmonologists) and immunizations that lower your risk of complications
• Genetic testing to determine the type of sickle cell disease or understand the risk of passing it on to an unborn child
• Tips for staying healthy, which may include resting when necessary and avoiding extreme (hot or cold) temperatures
• How to get help when your symptoms feel out of control, such as deciding whether to call our team or go to the hospital
• Support for families from social workers who help navigate family, school and financial issues

We invite you to bring family members or trusted friends with you to care for appointments for extra support.

Types

All types of sickle cell disease involve hemoglobin gene mutations (changes). The gene mutations a person inherits from each parent determine the type of sickle cell disease.

Types of sickle cell disease include:

• Hemoglobin SS: Each parent passes one sickle cell gene (S) to their child. Hemoglobin SS is the most common and severe form of the disease, sickle cell anemia.
• Hemoglobin SC: One parent passes on a sickle cell gene. The other passes on a C gene, a different type of abnormal hemoglobin.
• Hemoglobin S beta thalassemia: One parent passes on an S gene and the other a beta thalassemia gene. Beta thalassemia decreases the amount of hemoglobin your body produces.
• Hemoglobin SD, SE, SO or Arab: One parent passes on an S gene. The other passes on a D, E or O or Arab gene. Any of these combinations result in rare forms of sickle cell disease.

Symptoms

Children born with sickle cell disease may start having symptoms as early as 4 to 6 months of age. Signs of sickle cell disease may include:

• Jaundice, a yellowing of the eyes or skin
• Swelling of the hands and feet
• Swelling of the spleen
• Unusual tiredness

As a person gets older, they are more likely to experience complications, such as:

• Joint and bone damage
• Pain crises and ongoing pain
• Pulmonary hypertension, high blood pressure in the blood vessels of the lungs
• Skin ulcers
• Stroke
• Vision problems

Diagnosis

In most cases, doctors identify sickle cell disease at birth through state newborn screening programs. Doctors may perform additional blood tests in infants to confirm a sickle cell disease diagnosis and determine the type.

A child can have sickle cell disease even if neither parent has it. If both parents have a sickle cell trait or are carriers of at least one sickle gene, there’s a one in four chance the child will receive the sickle mutation from both parents and have sickle cell disease. Genetic testing uncovers whether each parent carries the gene and the likelihood of passing it on to a child.

Treatment and prevention

The right therapies for you depend on the type of sickle cell disease and symptoms you are experiencing. Your personalized care plan may include:

• Antibiotics and immunizations to prevent infections and serious illnesses
• Stroke screening with ultrasound to identify children at higher risk of a blockage in the arteries that supply the brain
• Blood transfusions to restore levels of healthy hemoglobin using blood from a healthy donor
• Medications to control pain and other symptoms

Some people, especially older children and adults, may need a bone marrow transplant. This option replaces the abnormal hemoglobin-producing cells inside your bones with ones from a healthy donor — usually a sibling. We work with University Hospital to coordinate this treatment, so you can focus on healing.

Research and clinical trials

Our physician-scientists take part in research that’s advancing blood disorder care. Participating in these efforts through clinical trials, if eligible, may give you additional care options.

Find out more about:

• Cancer clinical trials and research for adults
• Pediatric clinical trials and research