Disclaimer

The Foundation for Sickle Cell Disease Research’s medical practice, located at 1685 South State Road 7, Unit 4 Hollywood, Florida 33023, remains open for regular business hours. We are instilling COVID-19, otherwise known as Coronavirus, precautions. In order to protect our patient population that is at risk, due to having sickle cell disease, a chronic illness, we ask that you DO NO VISIT THE FACILITY if you have:

• Traveled internationally or on a cruise ship in the past 14 days
• Had contact with someone with or under investigation for COVID-19 (Coronavirus)
• Have the following “active”, or otherwise, unusual symptoms for yourself:
  • Fever
  • Cough
  • Sore Throat
  • Difficulty Breathing

In order to keep our facility free of COVID-19, we will begin screening ALL visitors prior to entering past our lobby. Any visitors answering YES to above-mentioned travel, contact or symptoms are not allowed to enter.

We appreciate your understanding during this time. If you have any questions or concerns, please do not hesitate to call our facility at 954-397-3251.

Recommendations for Management of Pediatric and Adult Patients with Sickle Cell Disease and COVID-19, Adapted from the Boston University Center of Excellence in Sickle Cell Disease 

Background 

Patients with sickle cell disease are at increased risk of serious complications with COVID- 19 and should be tested for the virus. Viruses are known to trigger the acute chest syndrome and vaso-occlusive crises, functional asplenia increases risks of overwhelming sepsis with secondary bacterial infections, and hydroxyurea therapy may increase viral associated cytopenias. Although no cases of COVID-19 are reported in sickle cell disease yet, we are concerned based upon the H1N1 experience, that if infected, severe Acute Chest Syndrome in sickle cell patients may require ICU admission and intubation for acute respiratory failure. 

Recommendations for Emergency Department & ACS Management 

1. Follow guidelines in patients’ individualized care plans 
2. In patients with cough, fever, fatigue, or other symptoms suggestive of an acute respiratory illness, test for COVID-19 along with other respiratory viral pathogens in all sickle cell disease patients at presentation, or who develop new respiratory symptoms if in the hospital 
3. If suspicion for COVID-19 is high or test is positive, page Hematology and Pulmonary immediately. (Specific directions should be provided for each center) 
4. Obtain a Chest X-Ray for all sickle cell disease patients requiring admission for a vaso- occlusive crisis and monitor oximetry. During the H1N1 pandemic, patients who developed severe respiratory infections most commonly were admitted for vaso-occlusive crises. 
5. If COVID-19 is present or infiltrates are present on Chest X-Ray suggestive of ACS, admit to the MICU for adults and strongly consider PICU admission for Pediatrics 
6. Management of Acute Chest Syndrome in SCD Patients Infected with COVID-19: 

• Early Exchange Transfusion 
• Broad spectrum antibiotics for MRSA, atypical mycobacteria, pneumococcus 
• Echocardiograms – RV dysfunction/elevated PA pressures 
• Inhaled Epoprostenol may be helpful 
• Plasmapheresis may have benefit 
• Consider high dose steroids (although not clearly helpful in COVID-19) 
• Consult Pediatric or Adult Pulmonary and Hematology 

Additional Information: 

1. The Massachusetts Department of Public Health Authorized Testing for COVID-19 in Patients with Sickle Cell Disease under CATEGORY 8: 

Other symptomatic individuals for whom knowledge of COVID-19 infection is medically indicated including older individuals and those with co-morbidities 

2. Extensive Guidance on Diagnosis and Management is available on the CDC website under Coronavirus 2019 (COVID-19) under the Healthcare Professionals tab: 

https://www.cdc.gov/coronavirus/2019-ncov/hcp/clinical-guidance-management- patients.html 

3. The COVID-19 Pandemic and Haemoglobin Disorders, a Contribution of the 

Thalassemia International Foundation (to follow) 

4. Of the recent new drug approvals, only L-glutamine is associated with reduced frequency of acute chest syndrome. It has not been tested in a pandemic of severe acute respiratory syndrome. (NEJM 2018 379: 226-35. DOI: 10.1056/NEJMoa1715971)

ACS Guidelines provided by Dr. Elizabeth Klings, Director, Center for Excellence in Sickle Cell Disease, in collaboration with Drs. Gregory Kato, Claudia Morris, Roberto Machado, Elliott Vichinsky, and Mark Gladwin ED Guidelines provided by Drs. Elizabeth Klings, Patricia Kavanaugh, Amy Sobota