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What Is Hereditary Spherocytosis?

In spherocytosis, a child's red blood cells have fragile membranes because of a genetic problem.

The red blood cells have a normal shape at first — flat discs, like a doughnut without the hole.

Over time, small bits of their membranes are removed when the cells pass through the spleen. This makes the cells become rounder, like spheres.

These cells are easily destroyed. They live a shorter life than normal cells, about 10 to 30 days instead of 100 to 120 days.

Red blood cells contain a protein called hemoglobin that carries oxygen around your child's body, dropping it off to cells that need it. In a child with spherocytosis, this process does not work well because so many of the red blood cells are destroyed.

The child develops hemolytic anemia - low levels of red blood cells because the cells are breaking down. So her tissues do not get enough oxygen.

Hereditary Spherocytosis in Children

Hereditary spherocytosis is passed down from parents to children. A parent with the disease has a 50% chance of having a child with the disease.

It is more common in people whose ancestors come from northern Europe.

Hereditary Spherocytosis at Seattle Children's

At Children's Hospital, we offer a full range of services to diagnose and treat this disease.

We follow children at least yearly to assess their anemia, their symptoms, the size of their spleen and their risk for gallstones. We can also counsel parents who have this disease and want to know more about their risk of passing it down to their children (called genetic counseling).

Read more about our experience and treatment of blood diseases through our Hematology Program.

Who Treats This at Seattle Children's?

Should your child see a doctor?

Find out by selecting your child’s symptom or health condition in the list below:

Summer 2014: Good Growing Newsletter

In This Issue

  • Understanding the Power and Influence of Role Models
  • Legal Marijuana Means Greater Poisoning Risks for Children
  • Why Choose Pediatric Emergency Care?

Download Summer 2014 (PDF)