Dactylitis and Sickle Cell Disease

Dactylitis, or hand-foot syndrome, is often the first symptom experienced by children with sickle cell disease (SCD). It is a condition associated with pain and swelling of the hands and feet. The swollen areas may also be red and have a high temperature.^1,2

Dactylitis happens when sickle cells block blood flow in the hands and feet. It is usually treated modestly because it only lasts for 1 to 2 weeks and rarely leads to permanent joint damage. Treatment involves pain medications, hydration, and other ways to ease discomfort.

What is dactylitis?

Dactylitis is the term for painful swelling of the hands and feet in SCD. It affects infants and young children from 6 months old to 6 years old. It is most common in the first 6 to 12 months of life. Most children who experience dactylitis have sickle cell anemia (HbSS), sickle cell hemoglobin C disease (HbSC), or sickle cell beta thalassemia.^3,4

Children with dactylitis have puffy, tender, and warm feet and hands. They may also have acute pain, fever, reduced movement, and an inability to hold weight. Episodes of dactylitis usually go away within a month. Dactylitis may return, but it usually will not cause any permanent joint damage.⁵

Why does sickle cell disease cause dactylitis?

Bones are the second-most affected organ in people with SCD, after the spleen. This is because red blood cells form in the bone marrow, and sickle cells build up in the small blood vessels in bones.⁶

The build up of sickle cells can block blood flow and prevent oxygen from reaching the bones in hands and feet. This often leads to pain and swelling in these small bones. About 45 percent of people with SCD experience dactylitis before they are 2 years old.^4,5

This rate goes down around 5 or 6 years old. This is because the bone marrow in the bones of hands and feet changes as we age. As this happens, the bones in the feet and hands become less affected by sickle cells.⁵

How is dactylitis treated?

The best treatment for dactylitis is usually a combination of pain medicines, hydration, and other ways to ease discomfort. Drugs typically used for moderate pain include:⁶

• Acetaminophen (Paracetamol)
• Nonsteroidal anti-inflammatory drugs (NSAIDs), such as aspirin and ibuprofen?
• Dipyrone

Treatment also usually involves bed rest, immobilization, and use of a heat pack to ease discomfort and swelling. People should avoid cold packs because severe cold can tighten blood vessels and reduce blood flow.⁷

Episodes of dactylitis may be triggered by:⁸

• Cold exposure
• Dehydration
• Infection
• Injury

During extreme pain with fever, fluids given by IV may be necessary.⁸

Antibiotics may treat or prevent infections that could trigger dactylitis. Children experiencing dactylitis should avoid exposure to extreme temperatures and conditions, especially cold.⁵

Some research has shown that hydroxyurea may prevent dactylitis and reduce pain in very young children. Hydroxyurea may reduce the need for hospital visits and blood transfusions. Every person responds to hydroxyurea differently, so its effects may be unpredictable.^5,8