Sepsis and Sickle Cell Anemia

Sickle cell anemia is a common inherited blood disease that causes red blood cells to form into a curved or sickle shape. This makes it hard for the blood cells to flow throughout the body, carrying oxygen and nutrients to the body tissues. 

In the United States, sickle cell anemia affects mostly affects people of African descent, followed by Latinos. According to the Centers for Disease Control and Prevention (CDC), sickle cell anemia affects about 1 out of every 365 Black or African-American births and 1 out of every 16,300 Hispanic-American births. The disease affects about 100,000 people in the U.S. The average age for diagnosis is around 5 months.

Worldwide, sickle cell anemia also affects people of several other ancestries, mainly from Southern Europe, such as those from Portugal and Greece. People with the disease have a higher risk of contracting infections that, in turn, could cause sepsis. 

Sepsis is a life-threatening emergency that happens when your body’s response to an infection damages vital organs and, often, causes death. Like strokes or heart attacks, sepsis is a medical emergency that requires rapid diagnosis and treatment.

Suggested Citation:
Sepsis Alliance. Sepsis and Sickle Cell Anemia. 2024. https://www.sepsis.org/sepsisand/sickle-cell-anemia/

Updated June 18, 2024.

 

More About Sickle Cell Anemia

Effects

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.

Treatment

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. 

Infection Prevention

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.   

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I’ve lost my dear sister on August 28th, 2024, due to sepsis. She was 65 years old, had a small wound on her foot, was in septic shock. I understand now. She thought she suffered from the flu for 6 days, and collapsed in the toilet because of sudden blood pressure failure. She was admitted on the ICU, foot had to be amputated, but her whole leg was so bad they had to amputate that as well. She suffered from severe diabetes, heart failure and ICD for 5 years and she decided to choose for palliative care. (Sepsis and Diabetes) ... Read Full Story

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Allison B.

Ten months ago on a Tuesday, I developed what I thought were cold symptoms – a sore throat, low grade fever (100) and a swollen lymph node in my neck. Overall I felt okay, it wasn’t anything too unusual. By Thursday, my lymph node was VERY swollen. I called my doctor’s office, and they gave me some suggestions over the phone to help my throat, etc. Later that night, my fever went up to 103. On Friday, one of the doctors finally agreed I should be seen. He tested me for Covid and flu, but not strep because my throat ... Read Full Story

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Alyvia C.

Towards the beginning if this year, I came down with what was believed to be strep. My illness consisted of swollen lymph nodes, tonsils, and a sore throat. I was in bed for days. I began feeling better after I went to an urgent care in my town and started on antibiotics. Stupidly, I didn’t finish my round of antibiotics because I began to feel better. (Sepsis and Strep Throat) About a week later, I started experiencing extreme pain in my left lung. Thinking it was muscle spasms, I ignored it. After suffering from the pain for a week, I ... Read Full Story

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Rose Aumen

As a registered nurse, I knew when assessing patients in the Emergency Room the appearance/signs/symptoms of sepsis. I never thought I would become a sepsis patient myself. I was in pretty good health for a 65-year-old female. March of 2024 I had chills that morning, My right leg was hurting the day before and was still somewhat painful but tolerable. I took two Tylenol and went back to bed. After resting I felt pretty good, Later that evening I suddenly was unable to walk and had severe pain in my right hip. Bacteremia and sepsis took over my entire body. ... Read Full Story

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Krystal Anderson: A Life Cut Short from Maternal Sepsis, A Legacy of Strength and Resilience Krystal Anderson’s life was marked by vibrant dedication to her passions and profound love for her family. A mother, wife, daughter, sister, and friend, Krystal was a beacon of joy and inspiration to all who knew her. Her sudden passing at the age of 40 from maternal sepsis, mere days after the tragic stillbirth of her daughter Charlotte Willow, left a community in mourning and a family without its cornerstone. Krystal was a dynamic and impactful person to everyone she encountered. She thrived in dual ... Read Full Story

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Sickle Cell Anemia