Sickle Cell Disease

What is sickle cell disease (SCD)?

Sickle cell disease (SCD) is an inherited disease that changes the way hemoglobin – a protein in the red blood cells that carries oxygen – is made. Normal red blood cells are round and soft, but in a person who has sickle cell disease, the red blood cells become shaped like the letter C, or a sickle.

The red blood cells in SCD are also sticky and rigid and can block blood flow, leading to severe pain attacks, or crises, as well as crises in the chest and spleen. The blocked blood flow can also cause damage to organs, such as the kidney, brain, heart, and lungs.

Sickle cell disease is a serious, life-long disease that requires daily care. Improper care of SCD may cause serious health problems, long-term disability, or complications that can be life-threatening. The life expectancy for those with SCD has been steadily improving with proper care and treatment.

How do I know if my child has SCD?

When a child has SCD, early diagnosis can help prevent complications. In the United States, every newborn is tested for SCD as part of a regular newborn screening program. For the screening, a nurse draws blood from a heel prick of your newborn and places drops of the blood on special testing paper. The hemoglobin is then tested in the lab.

If your baby is found to have SCD, your doctor will follow up with you directly to let you know the results, and your baby will be retested.

Babies can also be diagnosed while still in the uterus, sometimes as early as 8 to 10 weeks into the pregnancy. This testing is done using the amniotic fluid or tissue taken from the placenta.

What is comprehensive care for SCD?

University of Iowa Stead Family Children’s Hospital is home to the only comprehensive pediatric care center for sickle cell disease in Iowa. The team includes:

• Dental hygienist
• Genetic counselors
• Hematologists
• Medical assistants
• Neuropsychologists
• Nurse practitioners
• Research coordinators
• Social workers

Comprehensive care focuses on developing meaningful partnerships with your family, your child’s primary care doctor, and other specialists.

What services are available?

Our specialists will work with you and your child to explain how to manage SCD and its complications, how to detect and prevent complications through screenings and diagnostic testing, and will coordinate your care among all specialties to make scheduling, and potential travel, easier on you and your family.

Our genetic counselors will meet with you and your child to discuss how SCD is passed on through families – something that may help make future family planning decisions with full knowledge of the risks.

We also offer evaluations for early recognition of impairments and cognitive or learning disabilities, as well as a psychosocial evaluation and counseling by a social worker to support families with mental health, financial, and social concerns.

There are opportunities to participate in clinical trials and cutting-edge research to further developments in the care of children with SCD. 

Resources

• www.sicklecelldisease.org
• www.nhlbi.nih.gov/health/health-topics/topics/sca