December 23, 2021 – The Sickle Cell Disease Association of America’s Medical and Research Advisory Committee reminds the sickle cell community that the COVID-19 pandemic is having another increase in infections. New variants like omicron are emerging, as are common for RNA viruses, and vaccination rates need to consequently improve. Vaccinations can protect against severe illness. A new medication might also help as early treatment for infected individuals.

MARAC Recommendations

• No general recommendation for all individuals with SCD to stay home nor for all to return to in-person activities. Patients and families need to make individualized assessments of the risks and trade-offs of returning to work or school in person. Factors to consider include vaccination status, the rate of COVID-19 and variants in your community, family socioeconomic situation, protective measures in the building (proper ventilation, physical distancing, etc.), mental health needs and educational needs.
• Rare vaccine-related side effects, such as pain crises, have been reported after receiving a COVID-19 vaccine. However, they are not more likely to occur as a result of the COVID-19 vaccine compared to other common vaccines. No serious adverse events have been reported to date, and the risk/benefit ratio is in favor of vaccination for all individuals with SCD over age 5.
• Continue to recommend vaccination against COVID-19.
• Booster shots: All Americans are eligible, except children under 5. We recommend that individuals with SCD should receive a booster dose of the COVID-19 vaccine (Pfizer or Moderna).
• Continue to recommend general precautions like wearing masks, maintaining physical distancing, promoting good ventilation and washing hands. These are public health measures.
• The FDA has granted emergency use authorization of early treatments for people who test positive for coronavirus and who are at high risk for severe COVID-19, including hospitalization or death.
  • FDA announced on Dec. 22 and 23 that two new antiviral pills can be available to treat people with symptomatic COVID-19: Paxlovid (ritonavir plus nirmatrelvir, Pfizer) and Molnupiravir (Merck). Paxlovid is for patients ages 12 and up who weigh at least 88 pounds. It is available by prescription only and would be taken as soon as possible after diagnosis and within 5 days of the start of symptoms. Molnupiravir (Merck) is for adults, also byprescription only, also as soon as possible after diagnosis. MARAC is still learning more about these treatments.
  • Neutralizing monoclonal antibodies (bamlanivimab plus etesevimab (Lilly); casirivimab plus imdevimab (Regeneron); Sotrovimab (Glaxo Smith Kline) have been effective as early treatment for mild, symptomatic COVID-19 in some individuals with SCD [<10d after the test, age 12y+, wt 88 lb+ (40kg+), not hospitalized, not newly on oxygen]. However, these neutralizing antibodies might no longer be effective against the omicron variant of coronavirus. MARAC is monitoring the situation.

1. Please remember that you can take precautions to stop COVID-19. The coronavirus spreads in the tiny droplets breathed out by an infected person. The person might not know that he or she is infected. COVID-19 spreads quickly because many people breathe these contagious tiny droplets in the air.
   • Please stay aware of local conditions that impact the risk of making contact with someone who is infected. Keep track of the rate of new cases of COVID-19, the rate of vaccination against COVID-19 and whether new variants of the coronavirus are appearing near you.
   • Where vaccination rates are low, the coronavirus spreads quickly. Continue to take precautions so that you can reduce your risk of COVID-19 infection: wear masks, maintain physical distance from others, and avoid people who are coughing or otherwise contagious as well as indoor spaces and crowds.
   • Wearing a mask is not officially required in some situations but MARAC strongly advises that individuals with SCD should still wear masks in many situations.
   • Wear a mask if:
     • you must be in a crowded indoor space.
     • you are surrounded by strangers or friends who might have been exposed to people who are sick.
       You can remove the mask when you and all those around you have been vaccinated and you are certain that nobody around you is sick.
2. MARAC continues to advise individuals with sickle cell disease to get vaccinated against COVID-19.
   • Any of the three types of vaccines available in the U.S.A. are safe for individuals with SCD.
   • Vaccinations against coronavirus have now been shown to be safe for millions of people, with a known pattern of temporary aches that go away in about two days. It is rare for people to get severe side effects after receiving the vaccine.
   • The vaccines against coronavirus protect very well against serious symptoms of COVID-19 and reduce the chances of hospitalization and death. Break-through infections after vaccination have resulted in mild cases of COVID-19, not severe.
   • People who are not vaccinated may be barred from some travel and entertainment opportunities.
   • Protect the ones you love. Most senior citizen centers will not allow entry by people who are not fully vaccinated. Young children under 5 are not yet eligible for vaccination and depend on adults around them to be protected from infection.
3. How much riskier is COVID-19 for individuals with SCD? Research indicates that the risk of death from COVID-19 could be 1.5 to 2 times greater for individuals with SCD compared to the general population (based on the Brandow & Panepinto SECURESCD database, U.S. single-institution reports, UK and French databases). While still significant, the risk is not as high as MARAC feared at the beginning of the pandemic.
   • Risk of complications for COVID-19 in SCD is probably higher than in the general population. The rate of COVID-19 hospitalizations is higher because pain or fever from COVID-19 could result in SCD hospitalization.
   • Death and moderate to severe complications are higher for younger SCD patients. Organ damage risk factors such as kidney damage are also higher compared to the general population.
   • There are probably excess deaths during the pandemic because of deferred care for other problems (e.g., poor care in pregnancy, delayed diagnosis of cancer).
   • Individuals with SCD and kidney or lung problems are more likely to have severe COVID-19 infections.
   • People with SCD-SC and SCD-Sbeta+thalassemia can have moderate or severe COVID-19 cases.
   • Over half of the reports of COVID-19 in SCD were associated with intense vaso-occlusive pain or sickle acute chest syndrome.
   • Individuals with SCD can have mild cases of COVID-19.
   • COVID-19 remains dangerous for everyone, especially minority groups.