Sepsis and Sickle Cell Anemia

Red blood cells are usually flexible and shaped like a disk. Sickle cell anemia causes the cells to become sticky, as well as curved. These get trapped or stuck in blood vessels, depriving body tissues of oxygen. 

The misshapen red blood cells also damage the body’s organs, including the spleen. As blood passes through the spleen, the sickle-shaped cells get stuck and die. The spleen is an integral part of your immune system and people with a damaged spleen (or no spleen) have an impaired immune system. 

Finally, red blood cells normally can live for up to four months, but the longest sickle-shaped cells can live is three weeks. This means fewer red blood cells are available to carry oxygen and nutrients throughout the body. This adds to the infection risk.

The only cure known for sickle cell anemia is a bone marrow transplant and it is only performed in children. However, the bone marrow must come from a full sibling (both siblings have the same two parents). This can limit a child’s options if they don’t have a full sibling or if there is a reason the sibling can’t donate. 

You can read about treatments to help reduce the chances of a sickle cell crisis or to treat a crisis here. 

People with sickle cell anemia are at high risk for severe COVID-19. They frequently develop complications like pneumonia, and the virus can also trigger acute chest syndrome (ACS). This syndrome causes chest pain, low oxygen, fever, cough, and rapid breathing. 

Infection prevention for people with sickle cell anemia is essential. There are standard infection prevention procedures, such as frequent and thorough hand washing, getting appropriate vaccines, and avoiding people who are sick. But in addition, people with sickle cell anemia should: 

Practice food safety 

Be aware of food poisoning. According to the CDC, people with sickle cell anemia are particularly affected by salmonella bacteria. The bacteria are spread by contaminated food and water. To reduce the risk of food poisoning from salmonella: 

• Wash hands, cutting boards, knives, utensils, and any surface touched by raw food (before and after).
• Wash uncooked vegetables and fruit before eating.
• Cook meat thoroughly.
• Avoid raw or undercooked eggs.
• Avoid unpasteurized dairy products.

Also, don’t handle reptiles as they can spread salmonella – including pets.

Get vaccinated 

 The CDC reported fewer deaths among children with sickle cell anemia – 42% fewer – when the pneumococcal vaccine became available in the U.S. The vaccine helps reduce infections like pneumococcal pneumonia and pneumococcal meningitis. Aside from routine childhood vaccines, others for children with sickle cell anemia include: 

• Haemophilus influenzae type b (Hib)
• Pneumococcal vaccines
• Meningococcal vaccines

Preventative antibiotics 

Children with sickle cell anemia often take preventative (prophylactic) penicillin every day up to the age of five. This is to prevent infections. If they can’t take penicillin, erythromycin is another option. 

Wound care 

Leg ulcers – painful open sores – are a common complication of the disease. Someone with sickle cell anemia who develops a leg ulcer should consult their doctor as soon as possible. Several treatments might be appropriate, depending on the patient and the ulcers. These can include medications, debridement (physically removing affected tissue from the wound), special dressing changes, compression bandages, and pain control. 

Monitor leg ulcers closely for any signs of infection. These include pus from the wound and/or any foul smell; the skin around the wound is darker or red, and warmer to touch than the rest of the body; and fever.