December 6, 2022 — The Sickle Cell Disease Association of America (SCDAA) Medical and Research Advisory Committee (MARAC) shares the following:

For individuals with sickle cell disease and their caregivers

What is influenza, and why should I worry about it?

Influenza (“flu”) is a contagious viral infection that can cause severe medical problems in anyone. It is worse than a common cold. The problems are potentially bigger for individuals with sickle cell disease. Influenza can trigger sickle cell vaso-occlusive pain or acute chest syndrome. This means that influenza has a high chance

of sending you to the hospital and possibly to the intensive care unit with severe breathing problems. If you already have lung problems from asthma or from repeated acute chest syndrome, then influenza is even more likely to cause you to be severely ill.

What can I do?

Prevention Tip #1: MARAC strongly encourages all individuals with sickle cell disease (and their families) to get their flu shot every year. Although the shot might cause symptoms for a day or two (sore injection site, muscle aches and mild fever), these problems are small compared to the potential problems of influenza infection. To find a flu shot near you, visit vaccines.gov.

Prevention Tip #2: Wash your hands. Stay away from people who are coughing or sneezing or wear a mask around them.

Prevention Tip #3: During flu season, it is very important for individuals with sickle cell disease to take all their medications as prescribed. Hydroxyurea can protect against sickle cell pain or acute chest syndrome. Penicillin can protect against bacterial infection when a person is already weakened by influenza. Asthma medications can keep lungs functioning better.

If you think you have the flu, see your health care team early. If you can be diagnosed with influenza in the first 48 hours of the illness, you will probably be eligible to start a prescription treatment for influenza called oseltamivir (Tamiflu). Oseltamivir can shorten the course of influenza and reduce the risk of severe problems.

For health care providers and policymakers

Sickle cell disease has a high risk for complications and high morbidity from influenza (references 1-6 below). Sickle cell disease has both altered immune response and vulnerability to lung inflammation, triggering hospitalizations for acute chest syndrome, pain or other sickle cell complications. Health care providers should monitor sickle cell patients with influenza for possible acute chest syndrome. Acute chest syndrome is greatly

feared because it is the leading cause of death in sickle cell disease in the United States. Much of the clinical experience regarding sickle cell and the flu was gained from the 2009 H1N1 influenza but some is more recent, including a CDC report from 2021 (ref 2).

SCDAA MARAC notes that there is a national shortage of the treatment for influenza called oseltamivir (Tamiflu) and stewardship of its use is important. Oseltamivir reduces complications in influenza (Lee 2020, Wiemken 2021). MARAC recommends that sickle cell disease should be included in the list of conditions to prioritize for oseltamivir. Sickle cell disease is a rare disease nationally and might be overlooked in national policy because of its rarity. However, the special needs of sickle cell disease should be acknowledged.

For references and highlight points, download the full statement.