Sickle Cell Disease (SCD)


Sickle cell disease (SCD) comprises a group of inherited blood disorders in which the red blood cells of an affected individual become inflexible, sticky, and abnormally shaped like a sickle, a crescent-shaped farm tool. The life span of the sickle-shaped red blood cells is shorter than normal, resulting in anemia, a deficiency of red blood cells.1 

The abnormally shaped, inflexible red blood cells can also clog smaller blood vessels, blocking the flow of blood to tissues and organs. Obstructed flow may result in symptoms and serious complications such as chest pain, stroke, and an increased likelihood of infection.1 

Routine Appointments

To prevent serious complications, it is important for patients to schedule routine check-ups with their primary care physician as well as a hematologist, a physician who specializes in blood diseases like SCD.2 

It is recommended that babies with known SCD have follow-up visits with their doctor every 2 to 3 months from birth to 1 year of age. Children between the ages of 1 and 2 years with SCD should be seen by their doctor at a minimum of every 3 months. Children older than 2 years as well as adults should see their primary care doctor annually.2 

Healthful Habits

General dehydration may trigger painful episodes in which cellular dehydration promotes red blood cell sickling.3 Individuals with SCD should drink 8 to 10 glasses of water daily to stay hydrated and prevent cellular dehydration.2 

Other good habits include resting when fatigued, avoiding excessive exposure to the sun or cold temperatures to avoid overheating or overcooling, ensuring adequate oxygenation, and consuming a balanced, healthy diet.2,4

Tips for preventing oxygen loss include maintaining moderate activity levels to prevent overexertion and avoiding high altitudes, airplane flights in non-pressurized cabins, scuba diving, emotional stress, smoking, and respiratory infections.4

Individuals with SCD should refrain from using alcohol, recreational “street drugs,” and tobacco products because they elevate the risk for serious complications.4

Infection Prevention 

Because individuals with SCD are at increased risk for infection, hand washing with soap and water or a hand sanitizer containing alcohol frequently throughout the day is essential. Patients should wash their hands before preparing or eating food and after the following activities: using the restroom, shaking hands, sneezing, coughing, blowing one’s nose,  and coming into contact with frequently touched surfaces, persons who are ill, trash, animals, animal waste, and uncooked meat or eggs.5

Careful handling of food is crucial to preventing infection. This includes washing hands, cutting boards, counters, knives, and other utensils after they have been in contact with raw foods; washing vegetables and fruits before consumption; and cooking raw meat thoroughly.5 

It is recommended that patients not consume raw or undercooked eggs and unpasteurized milk or dairy products. Ingredients in dressings, desserts, unbaked cookie dough, frosting, and sauces, all of which may include raw eggs, should be carefully checked.5

Purposeful avoidance of reptiles such as snakes, turtles, and lizards is important because they may carry Salmonella, a strain of bacteria that can be harmful to children with SCD.5 

It is recommended that until the age of 5 years children with SCD receive daily doses of penicillin or another antibiotic as recommended by their physician for infection prophylaxis.5 

It is recommended that individuals with SCD who are considered to have functional asplenia follow a specialized schedule to keep their vaccination status up to date. Currently recommended vaccines of particular importance are those for Haemophilus influenzae infection, pneumonia, meningitis, and hepatitis B, as well as other flu vaccines.4,5 Vaccination recommendations may differ depending on the patient’s age.6 

Haemophilus influenzae type B Vaccine

It is recommended that individuals older than 19 years with SCD receive a single dose of the Haemophilus influenzae type B vaccine if they have not received it yet. If individuals with SCD undergo elective splenectomy, this vaccine should be administered at least 14 days prior to the splenectomy.6 

Pneumococcal Vaccines

For individuals with SCD older than 19 years, it is recommended that 1 dose of the pneumococcal conjugate vaccine (PCV13) be followed by 1 dose of the pneumococcal polysaccharide vaccine (PPSV23) at least 8 weeks later and subsequently a dose of PPSV23 every 5 years following the first PPSV23 dose. It is recommended that adults 65 years of age or older receive only 1 dose of PPSV23.6

Meningococcal Vaccines

Adults should receive the 2 doses of the meningococcal MenACWY vaccine series 8 weeks apart and then every 5 years thereafter. The 2 doses of the meningococcal MenB-4C series should be administered 1 month apart, or the 3 doses of the MenB-FHbp series should be administered 0, 1 to 2, and 6 months apart. These 2 MenB vaccines are not interchangeable. A 1-dose MenB booster shot should be administered 1 year following the first series and then every 2 to 3 years.6 

Hepatitis B Vaccines

Adults with SCD should receive 2 or 3 doses of a hepatitis B vaccine, depending on the vaccine.6 

Influenza Vaccines

It is important for persons past the age of 19 years with SCD to receive an annual flu shot.6

Support Network

Additional sources of information about SCD, patient advocacy, current research and clinical trials, and patient and caregiver support groups may be found on the websites of the Sickle Cell Association of America at http://www.sicklecelldisease.org, the Sickle Cell Information Center in Atlanta, Georgia, at http://www.scinfo.org, and the National Heart, Lung, and Blood Institute of the National Institutes of Health at https://www.nhlbi.nih.gov/health-topics/sickle-cell-disease 4 

Another source of information can be accessed at https://www.evelinalondon.nhs.uk/our-services/hospital/sickle-cell-and-thalassaemia/patient-leaflets.aspx. This link to Evelina London Children’s Hospital offers leaflets with information about bone marrow transplant, blood transfusions, and other health topics relevant to individuals with SCD.7

References

  1. What is sickle cell disease? Centers for Disease Control and Prevention. Reviewed December 14, 2020. Accessed November 11, 2021.
  2. Living well with sickle cell disease. Centers for Disease Control and Prevention. Reviewed December 16, 2020. Accessed November 11, 2021.
  3. Brugnara C. Sickle cell dehydration: pathophysiology and therapeutic applications. Clin Hemorheol Microcirc. 2018;68(2-3):187-204. doi:10.3233/CH-189007
  4. Patient education. Johns Hopkins Medicine. Department of Medicine. Accessed November 8, 2021.
  5. Infections and sickle cell disease. Centers for Disease Control and Prevention. Reviewed December 14, 2020. Accessed November 11, 2021.
  6. Adult immunization schedule by vaccine and age group. Centers for Disease Control and Prevention. Reviewed February 12, 2021. Accessed November 10, 2021.
  7. Sickle cell and thalassaemia patient information leaflets. Evelina London Children’s Hospital. Accessed November 8, 2021. 

Reviewed by Harshi Dhingra, MD, on 11/11/2021.

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