The most common type is HbSS disease where there are two copies of the HbS gene inherited, one from each parent. Most or all of the individual's hemoglobin is sickle. This is the most severe type of sickle cell disease.

People with sickle cell disease often have episodes of severe pain. They also have a low number of red blood cells (anemia) because the red blood cells break apart easily, fatigue, and higher risk of infections. People with sickle cell disease are at risk for stroke, lung problems, spleen problems, kidney problems and eye problems, among other organ issues.  

Combination Syndromes

Combination syndromes are when a child has one sickle cell gene and another gene change that affects the hemoglobin protein.

Some examples are:

• Sickle cell-hemoglobin C disease: The mix of one hemoglobin C gene and one sickle cell gene. People with this type of sickle cell disease can have pain, anemia and other complications such as eye problems.
• Hemoglobin S-betaº-thalassemia: These kids have one sickle cell gene and one betaº-thalassemia gene. The combination results in red blood cells that can sickle and break apart easily leading to anemia. It causes symptoms similar to HbSS disease, ranging from mild to severe.
• Hemoglobin S-beta₊- thalassemia: These kids have one sickle cell gene and one beta₊- thalassemia gene. This combination results in red blood cells that can sickle and break apart easily leading to anemia. It causes milder symptoms.
• Sickle cell-hemoglobin E disease: One hemoglobin E gene and one sickle cell gene. This combination may cause mild anemia. They may have pain when dehydrated, fighting an infection or under extreme physical stress. 

If a child has one abnormal gene and one normal one, they have sickle cell trait. They can make normal hemoglobin and usually have no symptoms. But, the hemoglobin S may "sickle" when stressed or exhausted. 

Comprehensive care: The MACC Fund Center is here for you and your family through every step of your sickle cell disease journey. Our team includes experts in diagnosing and treating sickle cell disease and other blood disorders. We offer bone marrow transplantations and gene therapy.

Latest therapies: We are one of a few hospitals that can offer kids FDA-approved gene therapy, Casgevy.

Sickle Cell Disease Program: With more than 400 patients, we are the state's largest sickle cell disease care provider. We care for the whole child, from physical health to social and emotional well-being. Our wide array of experts help kids and their families navigate life with sickle cell disease.

S.T.A.R. program: The Sickle Cell Transition Adolescent Resource Program helps teens transition to adult sickle cell disease care. We empower teenagers with knowledge, skills and the tools to manage their disease confidently.

Clinical trials: Clinical trials drive the next generation in treatment options. Our patients get access to the latest treatments. Children's Wisconsin is in the ASH Research Collaborative Sickle Cell Disease Clinical Trials Network. As one of the selected clinical research sites, our patients can access the latest therapies. 

Sickle cell research program: We have a very strong research program that seeks to improve our understanding and treatment of sickle cell disease. Our research is funded by the National Institutes of Health and the Centers for Disease Control and Prevention. Current studies include learning about the causes of pain in sickle cell disease and how to better assess pain. We also conduct work establishing a data collection program for sickle cell disease in the state of Wisconsin.

Award-winning: U.S. News & World Report regularly names us one of the nation's best pediatric centers for cancer and blood disorders. We have the resources and expertise to provide the best possible care, from diagnosis to long-term care.

Complementary and integrative medicine: We offer creative arts therapies, access to integrative medicine and acupressure.

Sickle cell disease requires lifelong comprehensive care. At Children's Wisconsin, our sickle cell care begins at diagnosis. Families often learn their newborn has sickle cell disease from the Wisconsin Newborn Screening Program.

Children's Wisconsin has the state's largest comprehensive pediatric sickle cell disease program. We care for the whole child, from physical health to social and emotional well-being.

Our care team includes:

• Pediatric hematologists: A hematologist with expertise in sickle cell disease care is on call 24 hours a day, 7 days a week for children with sickle cell disease who need urgent medical attention.
• Nurse coordinators: Manage and schedule care for our patients.
• Nurse practitioner: Manages care for our patients.
• Neuropsychometrist: An expert in how sickle cell disease affects the brain. We test and screen patients and work with their schools.
• Psychologist: Supports our kids and families. They offer behavior management, psychological screening and provide ongoing counseling.
• Social worker: Helps our families access the resources and support they need.
• Clinical navigator: A certified Sickle Cell Disease Community Health Worker. They guide our families to resources in the community and work to improve the holistic health of the children cared for in our program.
• Genetic counselor: Helps families understand the genetics of sickle cell disease and what it means for their family. They also discuss what a diagnosis means for eventual family planning.
• Administrative coordinator: Supports our program, including scheduling and communicating with families.
• Researchers: Our physician researchers and clinical research coordinators work to learn more about sickle cell disease and its treatments.

At a patient's first visit, typically by 2 months of age, the child and family meet with our care team. They begin a treatment plan and learn about sickle cell disease and its care. Complications can come on suddenly and without warning. We make sure parents know when and how to seek care.

Patients have follow-up visits at six months and one year. After that, we see them annually or biannually for comprehensive visits.

We see kids on hydroxyurea every two to three months in our outpatient clinic. Kids who often need blood transfusions or other therapies come every month.

Clinic visits include:

• Meetings with the care team
• In-clinic tests
• Medicine refills
• Imaging
• Vaccines

We work closely with the Jane B. Pettit Pain and Headache Center to help manage kids' pain. We also work closely with the emergency department to ensure children with sickle cell disease receive the best and safest care. We work with the pulmonary team to ensure children with sickle cell disease and asthma or other lung complications receive coordinated care.

We manage sickle cell disease care until age 19. Our young adults then transition to seeing adult sickle cell disease experts. We refer local young adults to the Froedtert Hospital's Sickle Cell Disease Clinic. Our sickle cell disease team works with Froedtert to ensure a smooth transition.

Several factors predict the long-term outcomes of sickle cell disease.

They include:

• Type of sickle cell disease
• Severity
• How often and the type of complications that arise
• If your child can stick with their care plan

The life expectancy has increased over the past 30 years. Those with sickle cell disease now regularly live into their mid-40s and beyond. New treatments have improved the prognosis for these kids, but it is still a severe and chronic disease that requires careful and close management with a sickle cell disease expert.