New Fund Launches to Provide Financial Assistance to People with Sickle Cell Disease
Copayment and Premium Assistance Now Available
(April 15, 2020 – Hanover, MD)  –   The Sickle Cell Disease Association of America is proud to announce its partnership with the HealthWell Foundation^®, an independent non-profit that provides a financial lifeline for inadequately insured Americans.  To support the sickle cell community, HealthWell has launched a new fund to provide copayment and premium assistance. Through the fund, HealthWell will provide up to $10,000 in financial assistance for a 12-month grant period to eligible patients who have annual household incomes up to 500 percent of the federal poverty level.
“We are excited that the HealthWell Foundation will provide much needed resources to individuals living with sickle cell disease during this difficult time. I am pleased that they are partnering with SCDAA to support the sickle cell community and reach as many individuals as possible,” says SCDAA President, Beverley Francis-Gibson.
“The HealthWell Foundation is proud to partner with the SCDAA to spread the word about this exciting new fund and to assist people living Sickle Cell Disease in accessing life-changing, sometimes lifesaving, medical treatments they otherwise would not be able to afford,” commented Krista Zodet, HealthWell Foundation President. “Thank you to our dedicated donors for recognizing this critical need and for helping us serve this patient community.”
To determine eligibility and apply for financial assistance, visit HealthWell’s Sickle Cell Disease Fund page. To learn how you can support this or other HealthWell programs, visit HealthWellFoundation.org
About the HealthWell Foundation
A nationally recognized, independent non-profit organization founded in 2003, the HealthWell Foundation has served as a safety net across over 70 disease areas for more than 500,000 underinsured patients. Since its inception, HealthWell has provided over $1.6 billion in grant support to access life-changing medical treatments patients otherwise would not be able to afford. HealthWell provides financial assistance to adults and children facing medical hardship resulting from gaps in their insurance that cause out-of-pocket medical expenses to escalate rapidly. HealthWell assists with the treatment-related cost-sharing obligations of these patients. HealthWell ranked 33rd on the 2019 Forbes list of the 100 Largest U.S. Charities and was recognized for its 100 percent fundraising efficiency. For more information, visit www.HealthWellFoundation.org.
 
About SCDAA
SCDAA’s mission is: To advocate for people affected by sickle cell conditions and empower community-based organizations to maximize quality of life and raise public consciousness while advancing the search for a universal cure. Visit www.sicklecelldisease.org.
About SCD
SCD, an inherited blood disease, causes red blood cells to have a sickle shape. Because of their stiffness and unusual form, blood flow is blocked to different tissues, ultimately damaging them. These sickle-shaped red blood cells contain an abnormal type of hemoglobin, hemoglobin S; normal red blood cells have hemoglobin A. Hemoglobin is important because it helps carry oxygen throughout the body. There is currently no universal cure for SCD.
 
CONTACT:
Jacqueline Burrell
Director of Communications
JBurrell@sicklecelldisease.org
 

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An Outline to Decrease Burden and Minimize Morbidity

This document will be updated as data and evidence emerge.
May 27, 2020 – Sickle cell disease (SCD) affects 100,000 individuals in the United States and millions globally. Individuals living with SCD suffer from both acute and chronic complications that require close contact with the medical system. These include acute sickle cell pain, fever, and the acute chest syndrome (ACS) which is the term used for a constellation of findings that includes chest pain, cough, fever, hypoxia and new lung infiltrates. There is a significant concern that the overlap of lung disease from COVID-19 with ACS may result in increased complications and amplification of healthcare utilization among individuals with SCD. Moreover, individuals with SCD, in general, experience high utilization of acute care services including emergency departments and hospitals and often present with fever, signs and symptoms of pneumonia or evolving ACS, as well as acute sickle cell pain requiring parenteral therapy. Thus, there may be specific diagnostic, treatment and logistical challenges in meeting the healthcare needs of this population during the COVID-19 pandemic.
Here, we provide suggested guidelines for the acute and chronic disease management of patients with SCD given the multidimensional and evolving changes and challenges in our healthcare operational landscape.

• If possible, convert all routine in-person appointments to virtual or telephonic Do not simply cancel appointments as patients need guidance and planning now more than ever.
• Educate patients and parents over the telephone about COVID-19 signs and symptoms and the importance of physical distancing to limit chances of exposure and infection. Encourage enhanced emotional connection through virtual or cellular-based modalities.
• Counsel patients and parents to continue to seek medical help for fever and other signs of infection. Counsel them to call first – their hospital, doctor, or nurse – for advice on where to go safely for evaluation.
• Make sure patients have a thermometer and know how to use it and clean it after each use.
• Make certain your patients have an ample supply of all prescribed medication at home (including analgesics) to manage both acute and chronic pain. If needed, reach out to your state medical board to institute a waiver on duration of opioid.
• Prioritize the use of pharmacies who deliver medications to patients.
• Counsel patients to adhere closely to use of hydroxyurea and other chronic medications such as L-glutamine, Voxelotor and Crizanlizumab as prescribed.
• Consider starting and/or optimizing existing therapies known to reduce sickle cell pain frequency (Hydroxyurea, L-glutamine, Crizanlizumab) as this is what most commonly brings older children and adults in direct contact with emergency departments and hospitals. The goal is to reduce this contact, if possible, to limit exposure to COVID-19.
• Halt all new subject enrollments for research requiring patient visits unless it is deemed in the patient’s best interest or involves COVID-19 clinical investigation or compassionate use protocols for very ill patients.
• Encourage patients without fever or signs of infection to manage pain at home with oral medications to reduce hospitalizations and visits to the emergency department.
• Consider prescribing naloxone for home use and educating patients and parents on when and how to use it.
• Call in or e-prescribe analgesic medications to the patient’s pharmacy and preferentially use pharmacies that deliver medications to patients’ homes.
• Call patient frequently to assess response to home-based treatment and offer in-person evaluation if this fails. Note that some patients with SCD present initially with acute sickle cell pain therefore close telephone contact should be employed with low threshold for in-person evaluation and COVID-19 testing.
• Urge patients to continue strict adherence to agents that reduce acute sickle cell pain frequency (e.g. Hydroxyurea, L-glutamine, Crizanlizumab) to reduce the likelihood of another pain episode.

We recognize that almost all institutions have established COVID-19 task forces with specific protocols. We underscore that it is essential that every institution includes SCD patients as a high-risk category, thus we advise taking the following into consideration:

• Make every effort to interview the patient by telephone, text monitoring system, or video Temperature monitoring could be reported by phone or shown to a provider via video conferencing.
• For patients with COVID-19 symptoms (fever, cough, or shortness of breath):
• Schedule patient for an outpatient visit immediately. Avoid the emergency department (ED), if possible. If the ED must be used, call ahead to facilitate care and isolation.
• If it is possible at your center, test patient for COVID-19. If it is not possible, follow guidelines and collect appropriate sample and send to a testing facility.
• Follow standard of care for managing SCD and fever including culturing of blood and other specimen (as indicated), testing for typical viral infections, administration of empiric broad-spectrum antibiotics to cover encapsulated organisms (e.g. ceftriaxone), and assessing for signs of acute chest syndrome.
• If the patient is COVID-19 negative and close telephone contact is possible to assess routinely for progression of symptoms, consider management at home with oral
• If possible, give the patient an incentive spirometer to use at home.

 
This is a rapidly evolving area of medicine without fully established standard of care for any population of patients, thus we advise taking the following into consideration when treating SCD patients with COVID-19:

• Monitor closely for signs of ACS and treat aggressively.
• Be vigilant for signs of rapidly progressive ACS, especially in adults: thrombocytopenia, acute kidney injury, hepatic dysfunction, altered mental status, and multi-organ failure (Chaturvedi et al. Am J Hematol. 2016). Use standard treatment protocols for ACS.
• Standard of care for ACS includes empiric antibiotics and use of oseltamivir until influenza is ruled out, supplemental oxygen, incentive spirometry, and good pain control to reduce atelectasis.
• Transfusion for ACS – Transfusion should be performed in patients with worsening anemia, evidence of hypoxia and chest x-ray changes. Initiate simple transfusion if patient is symptomatic or there is significant anemia (hemoglobin < 9 g/dl or greater than a 2 g/dl fall in hemoglobin; modified from NIH Recommendations). Initiate exchange transfusion for progression of hypoxia or clinical deterioration.
• Be vigilant for signs of Fat Emboli Syndrome: worsening anemia and mental status, hemolysis, thrombocytopenia, hypoalbuminemia, respiratory distress, and petechial rash. Can progress rapidly and mortality can be >60% in 48hrs.
• SCD patients often have undiagnosed pulmonary hypertension (PH) which could affect management of COVID-19. This should be considered in those who are acutely ill as patients can develop increased pulmonary pressures and, at times, right sided heart failure during ACS (particularly in those with known PH) and if these are present, consultation with Cardiology or Pulmonary is warranted.
• Significant numbers of patients with SCD have co-morbid asthma which may be exacerbated by acute viral illnesses. Review your hospital policies regarding the use of nebulizers during the COVID-19 pandemic as many institutions have advised against the use of aerosol-based interventions. Under such circumstances, consider using metered-dose inhaler instead.
• Many SCD patients are chronically prescribed NSAIDs, angiotensin converting enzyme inhibitors, and angiotensin II receptor blockers. Data are emerging regarding possible negative effects of these classes of drugs on people being treated for COVID-19. We suggest regular review of emerging data to guide decision-making about these drugs on a case-by-case basis.
• Management of hypercoagulability:
• COVID is associated with elevated D-dimer, prolongation of the prothrombin time, thrombocytopenia, reduction fibrinogen and these have prognostic significance in infected individuals without sickle cell disease. Such findings are also common in sickle cell patients with severe acute chest and multiorgan failure. These should be measured and monitored in all patients admitted with COVID infection and a risk stratification algorithm has been developed by the International Society of Thrombosis and Hemostasis. https://onlinelibrary.wiley.com/doi/abs/10.1111/jth.14810. Also see ASH recommendations. https://hematology.org/covid-19/covid-19-and-coagulopathy.
• There also appears to be an increased risk of thrombosis with or without SCD. ISTH also recommends that prophylactic heparin be considered in all hospitalized patients that are not bleeding and who have platelet counts greater than 25,000. Anticoagulation and risk stratification recommendations for children and adults hospitalized with COVID are available from the American Society of Hematology https://hematology.org/covid-19/covid-19-and-vte-anticoagulation
  • Kawasaki-like syndrome.

• Recent reports highlight an increase in children of a Kawasaki–like disease, a systemic vasculitis, sometimes presenting in a very severe form. While the connection with COVID-19 has yet to be demonstrated, these data should be taken into careful consideration in the upcoming months. (See   https://www.thelancet.com/journals/lancet/article/PIIS0140-6736(20)31103-X/fulltext)

• Managing the COVID-19+ SCD patient following discharge from the hospital.

• Patients with SCD who are discharged from the hospital and known to be COVID-19 positive remain at risk for serious complications including the acute chest syndrome and secondary bacterial infection.
• Patients should only be discharged if close outpatient follow up can be instituted, preferably with daily phone calls to the patient by an outpatient provider (physician or nurse) to assess for symptoms suggesting progression of COVID-19 disease severity or the development of sickle cell-related complications.

In the setting of blood shortage, clinicians will need to prioritize transfusions according to clinical need. Highest priority indications for continued transfusion include stroke prevention, progressive or critical neurovascular disease, those with recurrent acute chest syndrome unresponsive to Hydroxyurea, and significant cardiac or respiratory co-morbidity. To date, data suggest that transfusions remain safe.

• Monitor the availability of blood in your community closely as you may have to adjust your transfusion practices (e.g. apheresis vs manual/simple transfusion) to maintain current individual patient treatment goals.
• Consider transitioning to Hydroxyurea for patients eligible according to TWITCH (Ware et al Lancet 2016).
• If you match for CEK antigens, please continue.
• Indications where maintenance of current transfusion strategy is imperative:
• Children with history of stroke/abnormal TCD: maintain HbS < 30% or continue current strategy*.
• Adults with history of stroke or abnormal TCD as children: maintain HbS < 30% or continue current strategy*.
• Consider modification of transfusion strategy in order to conserve blood in the following:
• Patients receiving chronic transfusion for recurrent acute chest syndrome: continue current strategy*, individualize for maintenance of HbS < 30% vs < 50%, consider adding disease-modifying drug (Hydroxyurea).
• Patients on RBC exchange for end organ damage, priapism, or other non-neurologic indication: switch to simple transfusion or partial exchange for 3-6 months or until blood supply recovers, if baseline hematocrit allows (individualize, generally maintain hematocrit <33%).

*for patients who may be stable with a HbS goal that is > 30%, maintain current goal

• Encourage people to Donate, Donate, Donate.
  • Medical leaders should encourage local communities and political leadership to support local blood drives as blood shortages are

• During “shelter in place”, blood donation probably is considered an essential activity.
• We are not aware of any clinical trials in COVID-19 specifically for SCD. However, a non-research global registry collecting only de-identified data has been established as a voluntary effort to identify the impact of COVID-19 on people with SCD: https://covidsicklecell.org/
• People with SCD should not be excluded a priori from COVID-19 clinical trials.
• Modify other ongoing clinical trials for the safety of patients and staff.
• Halt all other new research enrollment requiring a patient visit, including gene therapy/bone marrow transplantation, unless it is deemed in the patient’s best interest or involves COVID-19 clinical investigation or compassionate use protocols for very ill patients.

 
Frequently Asked Questions
What should I do if a patient or a parent asks for a letter for work?

• Prepare a form letter that supports their request to work at home and can be easily customized for each patient. Highlight that SCD is considered to be a high-risk condition for severe COVID-19 infection. sicklecelldisease.org/template-letters-for-caregivers-2/

How can I get information for my patients about COVID-19?

• Please have them go to sicklecelldisease.org where they can get updated information that is specific for patients and their caregivers.

SCDAA Medical and Research Advisory Committee Members

Miguel R Abboud, MD
Professor of Pediatrics and Pediatric Hematology-Oncology
Chairman
Department of Pediatrics and Adolescent Medicine
American University of Beirut
Beirut, Lebanon
Biree Andemariam, MD
Chair, Medical and Research Advisory Committee, Sickle Cell Disease Association of America
Chief Medical Officer, Sickle Cell Disease Association of America
Director, New England Sickle Cell Institute Associate Professor of Medicine
University of Connecticut Health
Farmington, Connecticut
Shawn Bediako, PhD Associate Professor Department of Psychology
University of Maryland Baltimore County Baltimore, Maryland
Andrew Campbell, MD
Center for Cancer and Blood Disorders Children’s National Health System Associate Professor of Pediatrics
George Washington University School of Medicine and Health Sciences
Washington, District of Columbia
Raffaella Colombatti, MD, PhD
Physician Azienda Ospedaliera-Università di Padova
Department of Womens’ and Child Health Clinic of Pediatric Hematology Oncology Via Giustiniani 3
35129 Padova, Italy
Lori Crosby, PsyD
Co-Director, Innovations in Community Research, Division of Behavioral Medicine & Clinical Psychology
Co-Director, CCTST, Community Engagement Core
Psychologist, Research, Behavioral Medicine
& Clinical Psychologist
Cincinnati Children’s
Professor, UC Department of Pediatrics
Cincinnati, OH
Deepika Darbari, MD
Center for Cancer and Blood Disorders Children’s National Health System Associate Professor of Pediatrics
George Washington University School of Medicine and Health Sciences
Washington, DC
Payal Desai, MD Associate Professor
Director of Sickle Cell Research
The Ohio State University
JamesCare at Ohio State East Hospital
Columbus, Ohio
James Eckman, MD
Professor Emeritus, Hematology & Medical Oncology
Emory University School of Medicine Department of Hematology and Medical Oncology
Atlanta, Georgia
Mark Gladwin, MD Professor and Chair Department of Medicine
Founder, Pittsburgh Heart, Lung, and Blood Vascular Medicine Institute
University of Pittsburgh E1240 BST
Pittsburgh, Pennsylvania
Jo Howard, MB Bchir, MRCP, FRCPath
Head of Red Cell/Sickle Cell Service Guy’s and St Thomas’
NHS Foundation Trust Great Maze Pond
London, United Kingdom
Lewis Hsu, MD, PhD
Co-Chair, Medical and Research Advisory Committee, Sickle Cell Disease Association of America
Vice Chief Medical Officer, Sickle Cell Disease Association of America
Director of Pediatric Sickle Cell
Professor of Pediatric Hematology- Oncology
University of Illinois at Chicago Chicago, Illinois
Professor Baba Inusa
Lead Consultant Paediatric Sickle Cell and Thalassaemia
Evelina London Children’s Hospital
Guy’s and St Thomas NHS Trust
Women and Children’s Health
Faculty of Life Sciences & Medicine
King’s College London
Lambeth Palace Road, London SE1 7EH
Elizabeth S. Klings, MD
Associate Professor of Medicine
Director, Center for Excellence in Sickle Cell Disease
Director, Pulmonary Hypertension Center
Boston University School of Medicine
Boston, Massachusetts
Lakshmanan Krishnamurti, MD Professor of Pediatrics
Director of Bone Marrow Transplant
Joseph Kuechenmeister Aflac Field Force Chair, Aflac Cancer and Blood Disorders Center Children’s Healthcare of Atlanta/Emory University
Atlanta, Georgia
Sophie Lanzkron, MD, MHS
Director, Sickle Cell Center for Adults The Johns Hopkins Hospital
Baltimore, Maryland
Julie Makani, FRCP, PhD
Associate Professor
Department of Haematology and Blood Transfusion
Muhimbili University of Health and Allied Sciences
Dar es Salaam, Tanzania
Caterina Minniti, MD Director, Sickle Cell Center Montefiore Health System
Professor of Medicine and Pediatrics Albert Einstein College of Medicine
Bronx, New York
Genice T. Nelson, DNP, APRN, ANP-BC Program Director
New England Sickle Cell Institute & Connecticut Bleeding Disorders Programs UConn Health
Farmington, Connecticut
Board Member, Sickle Cell Disease Association of America
Isaac Odame, MB ChB, MRCP(UK), FRCPath, FRCPCH, FRCPC
Professor, Department of Paediatrics University of Toronto
The Hospital for Sick Children Division of Haematology/Oncology
Toronto, Ontario
Kwaku Ohene-Frempong, MD
Director Emeritus, Comprehensive Sickle Cell Center
Emeritus Professor of Pediatrics, University of Pennsylvania
President, Sickle Cell Foundation of Ghana
Emeritus Board Member, Sickle Cell Disease Association of America
Gwendolyn Poles, D.O. Honorary Medical Staff Member
Former Medical Director, Kline Health Center
Faculty, Internal Medicine Program UPMC Pinnacle
Harrisburg, Pennsylvania
Board Member, Sickle Cell Disease Association of America
John Roberts, MD
Yale Adult Sickle Cell Program
Smilow Cancer Hospital at Yale New Haven
New Haven, Connecticut
Wally Smith, MD
Professor
Scientific Director, VCU Center on Health Disparities
Director, VCU Adult Sickle Cell Program Department of Internal Medicine
Division of General Internal Medicine
Richmond, Virginia
Crawford J. Strunk, MD
Director, Sickle Cell Disease and Hemoglobinopathy Clinic
Pediatric Hematology/Oncology
Debbie Brass Cancer Center
ProMedica Russell J. Ebeid Children’s Hospital
2142 North Cove Blvd, Ren 4 West
Toledo, OH 43606
Immacolata Tartaglione, MD PhD
Department of Woman, Child and General and Specialist Surgery
Università degli Studi della Campania “Luigi Vanvitelli”
Naples, Italy
Marsha Treadwell, PhD
Director, Sickle Cell Care Coordination Initiative
Regional Director, Pacific Sickle Cell Regional Collaborative
Professor of Psychiatry and Pediatrics
University of California San Francisco Benioff Children’s Hospital Oakland
Oakland, California
Winfred C. Wang, MD
Emeritus, St. Jude Faculty
Member, Department of Hematology
St. Jude Children’s Research Hospital
Memphis, Tennessee
Russell E. Ware, MD, PhD
Director, Division of Hematology
Institute Co-Director, Cancer and Blood Diseases Institute
Director, Global Health Center
Marjory J. Johnson Chair of Hematology Translational Research
Cincinnati Children’s
Professor, UC Department of Pediatrics
Cincinnati, Ohio
Julie Kanter Washko, MD
Associate Professor
Division of Hematology Oncology
University of Alabama at Birmingham
Birmingham, Alabama
Kim Smith-Whitley, MD
Professor of Pediatrics
Director Comprehensive Sickle Cell Center Division of Hematology
The Children’s Hospital of Philadelphia
Philadelphia, Pennsylvania
Board Member, Sickle Cell Disease Association of America
Wanda Whitten-Shurney, MD
CEO & Medical Director
Sickle Cell Disease Association, Michigan Chapter Inc.
Board Member, Sickle Cell Disease Association of America
Detroit, Michigan
Ahmar U. Zaidi, MD
Assistant Professor of Pediatrics Comprehensive Sickle Cell Center Children’s Hospital of Michigan Director of Physician Network Development, University Pediatricians
Wayne State University/Central Michigan University School of Medicine
Detroit, Michigan  

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This document will be updated as more information becomes available.
May 27, 2020 – COVID-19, the coronavirus disease of 2019 – also known as Coronavirus-2 (also called SARS-CoV-2) – and the illness it causes is on everybody’s mind. If you or your family member has sickle cell disease (SCD), you may be worried about what this new disease may mean to you.
The more you learn about COVID-19, the better you can understand what to look for, how to protect yourself or your loved one, and what to do IF you feel sick.
SCDAA and its Medical and Research Advisory Committee want to help you understand COVID-19, how it may affect a person with SCD, and what you can do to help.
The potential health risk posed by COVID-19 for people with SCD is a real concern. The knowledge we have about how COVID-19 will affect those living with SCD is evolving constantly. In light of this, the risks to our community may change in the coming days, weeks and months. It is critical that you stay regularly informed.
Members of MARAC have been speaking daily with other experts around the world to get new information that may be useful to you.
What You Need to Know About the Coronavirus (COVID-19)
The coronavirus pandemic is real; it is not just a scare tactic, and it is not fake news. People who have SCD may have a more difficult time IF they get COVID-19; it is better to protect yourself from getting the infection.

Frequently Asked Questions

What can I do to make sure that I do not get COVID-19?
Stay home as much as possible.
• Do not leave home unless absolutely necessary.
• If you MUST go out, remember to do these when you get to where you are going and as soon as you return home:
o Wash your hands with soap and water for 20 seconds (that is, don’t stop until you finish singing the “Alphabet song” once or “Happy Birthday” twice) after you touch anyone or anything outside, as soon as you can; or,
o Use hand sanitizer with at least 60% alcohol to rub your hands.
What to do if you feel sick:
• CALL your doctor, nurse, healthcare team, or hospital immediately.
• Do not just rush to the hospital. CALL first, if possible.
• Tell them how you feel.
• Remember to tell them you have SCD.
• Please consider going to the hospital if you continue to feel sick and are unable to reach anyone for advice.
• Be careful when you meet other people. Try to protect yourself and them, as well.
o Do not get too close to anyone, especially a person who is coughing, or sneezing.
o Stay farther than you can touch each other by stretching out your arm.
o Greet one another by waving from a distance (no hugs or handshakes).
Reduce the spread of germs in your house or place of work:
• Use disinfectants: Use a disinfectant to clean surfaces (like counter tops, tables, and arms of chairs) or things that were touched by others because a strong disinfectant can kill the virus; and,
• Keep surfaces clean: Clean surfaces frequently with a disinfectant if you or other people use or touch the same surfaces or things often. The virus can live on surfaces for many days. Surfaces include phones, remote controls, counters, tabletops, doorknobs, bathroom fixtures, toilets, keyboards, tablets, and bedside tables.
• Do not share items, if possible:
o Avoid sharing personal household items such as dishes, drinking glasses, cups, eating utensils, towels, or bedding with other people in your home.
• Cover your mouth and nose: Remember to cover your mouth and nose with a tissue when you cough or sneeze, then throw away tissue in trash, and immediately wash your hands.
o If you do not have tissue, cough or sneeze into your clothes. Do not cough or sneeze into your bare hands or skin.
• Protect parts of your face: Do not touch your eyes, nose, mouth, or face; the virus can get into your body through those body parts.
• Stay away from anyone in your home that is sick: Those positive for COVID-19 or think they may have it should follow the advice at the CDC website (https://www.cdc.gov/coronavirus/2019-ncov/if-you-are-sick/index.html)
• Masks: They are not enough to protect you completely from the COVID-19 infection:
o The CDC recommends you wear a mask when out to protect others and yourself.
o Remember, wearing a mask does not protect you enough and you should continue to do all the other things to prevent the spread of germs.
o When you are using a mask please follow these guidelines:
 They can be reused but should be replaced when visibly soiled or damaged.
 Cloth masks should be washed and dried regularly.
 They should be stored in a clean paper bag between uses.
 When storing, fold the mask so that the inner surface is held against itself to reduce contact with the outer surface.
How can I get myself and my family prepared?
Use this checklist if it helps you to prepare.
____ Refills: Please check to see if you have refilled all your medications so that you do not run out.
____ Extra medications: Contact your doctor, nurse or hospital to ask about getting extra medications to have on hand in case there is an outbreak of COVID-19 in your community and you need to stay home for a long time. Try and get a 90-day supply. Some pharmacies are offering home delivery.
____ Over the counter medicines and supplies: Be sure you have over-the-counter medicines and medical supplies (e.g. tissues).
____ Thermometer: Make sure you have a thermometer to take your temperature and clean it after each use.
____ Take your prescribed medications for SCD: hydroxyurea, glutamine, penicillin, folic acid, Voxelotor, Crizanlizumab, Deferasirox, and any others. These medications will help keep your body in the best possible condition to fight off infection.
____ Pain medications: Make sure you have enough of your pain medications and use them when you have regular sickle cell pain.
In addition, plan for any of the following that apply:
____ Household items and groceries: Have enough household items and groceries on hand so that you will be prepared to stay at home for a period of time that could be many weeks.
____ Ways to stay in touch: Stay in touch with others by phone, text or email.
You may need to ask for help from friends, family, neighbors, etc. if you become ill.
____ Ways to keep children occupied: Keep children occupied with home school activities, arts and crafts.
____ Caretakers for loved ones and pets: Think ahead about who will watch your children, other loved ones, or pets if you become too sick.
____ Working from home: Find out if working from home is an option. You may need to ask your physician for a letter for your employer to support this option. Discuss with your doctor ways to stay safe at your workplace if you are unable to work from home.
How do I know if I have COVID-19?
The only way to know for sure that you have coronavirus is to get tested. However, it is not easy to get tested yet as many places have limited access to testing kits. We hope that this will change soon. In low resource countries, there are even fewer places to be tested. Your sample may need to be sent to a lab far from where you are. Your health care team will arrange for your test to be done.
Most people who have COVID-19 have the following symptoms:
• Fever
• Cough
• Shortness of breath
NOTE: These together can be a sign of Acute Chest Syndrome of SCD, as well as
the serious pneumonia seen in COVID-19. This would be the major concern of COVID-19 in a person with SCD.
**Some people who have COVID-19 have diarrhea and/or a change or loss of smell or taste.
If you have any of the above symptoms:
Call your doctor, nurse or hospital right away to discuss what you should do next.
In addition, you or someone should call for emergency help, (911 in the US), if you have:
• Difficulty breathing
• Pain or pressure in the chest different from your usual sickle cell pain
• New confusion or inability to wake up easily
• Darker lips or face than usual
What should I do if I have a fever?
• Call your sickle cell doctor, primary care physician, nurse, or hospital to report your illness and arrange to be checked.
• Do NOT immediately rush to the emergency department.
• It is not a good idea to stay at home to “wait and see” and just take medications to force down your high body temperature.
• Be aware that lots of different things can cause fever, such as infections and sickle cell tissue damage. Fever does not mean you have coronavirus!
• If you need to go out or to the emergency room or clinic, make sure to wear a mask so you do not infect others or catch an infection.
How do I get or make a mask?
• There are many ways to make a mask at home and this may be your best option since there are shortages of masks in most communities.
• The CDC has guides on ways to make masks here: https://www.cdc.gov/coronavirus/2019-ncov/prevent-getting-sick/diy-cloth-face-coverings.html
• The CDC wants you to make sure of the following when using a mask:
o Mask should:
 Fit snugly but comfortably against the side of the face.
 Be secured with ties or ear loops.
 Include multiple layers of fabric.
 Allow for breathing without restriction.
 Be able to be laundered and machine dried without damage or change to shape.
What are some good ways to stay as healthy as possible?
• Take your medications as prescribed.
• Drink plenty of fluids, as usual.
• Try to rest and not do too much physical activity.
Should I continue getting my chronic transfusions?
• Regular transfusions are given often to prevent serious complications like stroke.
• Continue your regular transfusions unless your healthcare team tells you to stop.
• Blood supply may be short so your doctor may need to change the transfusion plan.
• Blood transfusion is still safe; COVID-19 has not been passed through transfusion.
• Talk to your healthcare team if you have concerns about blood and COVID-19.
What if I don’t have a doctor?
• Many hospitals are setting up ways for patients to have a visit with a healthcare provider over the telephone. These are called “e-visits” or “telemedicine”.
• Many communities have hotlines available for people to call for help and advice.
• If you are unsure, you can always call your local SCDAA chapter for advice on resources in your community.
Should I go to the emergency department if I am ill?
• If you have a doctor, nurse, or health care team, it is recommended that you call for advice, if you can, before going to the emergency department.
• Emergency departments are very full of sick people right now and it is likely that there will be long waits.
• Also, it is very likely that people with COVID-19 infection will be there.
• If you have no other option, then going to the emergency department may be the only option.
• Try and call ahead to see if they have recommendations beforehand.
I think I am having sickle cell pain. What should I do?
• If your pain feels different or is not responding to your usual home treatment, or you also have fever, cough or trouble breathing, call your healthcare team for advice and arrange to be checked.
• Otherwise, try to manage your sickle cell pain at home in order to avoid a busy emergency department or medical center that may have people with COVID-19 seeking care.
Is it safe to travel?
• It is best to avoid all travel at this time unless there is some emergency.
• If you must travel, talk to your healthcare team or visit the website of the Centers for Disease Control and Prevention (CDC) for travel guidance (www.cdc.gov/covid19) to stay up to date.
What if I cannot work from home?
• If you are unable to work from home, ask your employer to implement the CDC recommendations:
o social distancing (keeping everyone at least 6 feet apart);
o all employees wearing some type of mask or face covering; and,
o increased access to hand washing/hand sanitizer.
• Help your employer understand that it will benefit the company if everyone stays safe.
• Provide your employer or human resources department a copy of this advisory if you think it may help.
• Give your doctor a printed copy of a sample letter for your employer. It can be found on the SCDAA website here: www.sicklecelldisease.org/template-letters-for-caregivers-2
What do I do if I am on a research study?
• It is important that you get in touch with your research team right away to check if there are any changes. And, thank you for being on a clinical trial.
I feel fine so far. Is there anything I can do to help others?
• If you know others living with SCD, contact them by phone, text or social media. Make sure they are doing “ok” and see if they need help or reassurance. It is a stressful time for a lot of us. If you know people who are willing to donate blood, encourage them.
Will there be a shortage of blood soon?
• This is very possible, but you can help! If there are people in your family or community that are willing to donate blood, please encourage them to call the local blood bank right away. During times like these, there can be a lot of blood shortages and we know that many people with SCD (as well as other conditions) need blood. See if you can get some people to donate. People with sickle cell trait are still able to donate so please encourage them to do so.
How do I stay informed?
(1) Go to www.OneSCDVoice.com, SCDAA’s online information superhighway where we will post updates regularly that are specific to SCD. It is free to join.
(2) Go to the website of your local SCDAA organization. You can find the one closest to you at www.sicklecelldisease.org. There may be some useful information that applies directly to your community.
(3) Go to the CDC’s website (www.cdc.gov/covid19) for regular updates on the COVID-19. Information is updated routinely and will keep you abreast of the latest guidelines and recommendations. There is information on how you can start to prepare in your homes and community.
(4) Download template letters for caregivers. www.sicklecelldisease.org/templateletters- for-caregivers-2
For More Information, contact info@sicklecelldisease.org
 

SCDAA Medical and Research Advisory Committee Members

Miguel R Abboud, MD
Professor of Pediatrics and Pediatric Hematology-Oncology
Chairman
Department of Pediatrics and Adolescent Medicine
American University of Beirut, Lebanon
Biree Andemariam, MD
Chair, Medical and Research Advisory Committee, Sickle Cell Disease Association of America
Chief Medical Officer, Sickle Cell Disease Association of America
Director, New England Sickle Cell Institute
Associate Professor of Medicine
University of Connecticut Health
Farmington, CT 06030
Shawn Bediako, PhD
Associate Professor
Department of Psychology
University of Maryland Baltimore County
Baltimore, Maryland
Andrew Campbell, MD
Center for Cancer and Blood Disorders
Children’s National Health System
Associate Professor of Pediatrics
George Washington University School of Medicine and Health Sciences
Washington, DC
Raffaella Colombatti, MD, PhD
Physician Azienda Ospedaliera-Università di Padova
Department of Womens’ and Child Health
Clinic of Pediatric Hematology Oncology
Via Giustiniani 3
35129 Padova Italy
Lori Crosby, PsyD
Co-Director, Innovations in Community Research, Division of Behavioral Medicine & Clinical Psychology
Co-Director, CCTST, Community Engagement Core
Psychologist, Research, Behavioral Medicine & Clinical Psychologist
Cincinnati Children’s
Professor, UC Department of Pediatrics
Cincinnati, OH
Deepika Darbari, MD
Center for Cancer and Blood Disorders
Children’s National Health System
Associate Professor of Pediatrics
George Washington University School of Medicine and Health Sciences
Washington, DC
Payal Desai, MD
Associate Professor
Director of Sickle Cell Research
The Ohio State University
James Care at Ohio State East Hospital
181 Taylor Avenue, Columbus, Ohio 43203
James Eckman, MD
Professor Emeritus, Hematology & Medical Oncology
Emory University School of Medicine
Department of Hematology and Medical Oncology
49 Jesse Hill Jr. Drive SE
Atlanta, GA 30303
Mark Gladwin, MD
Professor and Chair
Department of Medicine
Founder, Pittsburgh Heart, Lung, and Blood Vascular Medicine Institute
University of Pittsburgh
E1240 BST
200 Lothrop Street
Pittsburgh, PA 15261
Jo Howard, MB Bchir, MRCP, FRCPath
Head of Red Cell/Sickle Cell Service
Guy’s and St Thomas’
NHS Foundation Trust
Great Maze Pond
London, United Kingdom
Lewis Hsu, MD, PhD
Co-Chair, Medical and Research Advisory Committee, Sickle Cell Disease Association of America
Vice Chief Medical Officer, Sickle Cell Disease Association of America
Director of Pediatric Sickle Cell
Professor of Pediatric Hematology-Oncology
University of Illinois at Chicago
Chicago, Illinois
Dr Baba Inusa
Honorary Reader in Paediatric Haematology, King’s College London
Lead Consultant Sickle Cell and Thalassaemia Service
Evelina London Children’s Hospital
St Thomas’ Hospital
Westminster Bridge Road, London SE1 7EH
Elizabeth Klings, MD
Associate Professor of Medicine, Boston University School of Medicine
Program Director, Center of Excellence in Sickle Cell Disease
Director, Pulmonary Hypertension Inpatient and Education Program
Medical Director, Pulmonary Rehabilitation Program, Boston Medical Center
72 East Concord Street, R-304
Boston, MA 02118
Lakshmanan Krishnamurti, MD
Professor of Pediatrics, Director of BMT
Joseph Kuechenmeister Aflac Field Force Chair
Aflac Cancer and Blood Disorders Center
Children’s Healthcare of Atlanta/Emory University
Atlanta, GA
Sophie Lanzkron, MD, MHS
Director, Sickle Cell Center for Adults
The Johns Hopkins Hospital
1800 Orleans St
Baltimore, MD 21287
Caterina P. Minniti, MD
Director, Sickle Cell Center
Montefiore Health System
Professor of Medicine and Pediatrics
Albert Einstein College of Medicine
Bronx, NY
Genice T. Nelson, DNP, APRN, ANP-BC
Program Director
New England Sickle Cell Institute & Connecticut Bleeding Disorders Programs
UConn Health
Farmington, CT 06030
Board Member, Sickle Cell Disease Association of America
Isaac Odame, MB ChB, MRCP(UK), FRCPath, FRCPCH, FRCPC
Professor, Department of Paediatrics
University of Toronto
The Hospital for Sick Children
Division of Haematology/Oncology
555 University Avenue
Toronto, Ontario M5G 1X8
Gwendolyn Poles, D.O.
Honorary Medical Staff Member
Former Medical Director, Kline Health Center
Faculty, Internal Medicine Program
UPMC Pinnacle
Harrisburg, PA
Board Member, Sickle Cell Disease Association of America
John Roberts, MD
Yale Adult Sickle Cell Program
Smilow Cancer Hospital at Yale New Haven
35 Park Street, Ste 7th floor, Multispecialty
New Haven, CT 06511
Wally Smith, MD
Professor
Scientific Director, VCU Center on Health Disparities
Director, VCU Adult Sickle Cell Program
Department of Internal Medicine
Division of General Internal Medicine
West Hospital W10W-403
P.O. Box 980306
Richmond, VA 23298
Crawford J Strunk MD
Pediatric Hematology/Oncology
Pediatric Hematology/Oncology Program at Toledo Children’s Hospital
2142 N. Cove Blvd.
Toledo, Ohio 43606
Immacolata Tartaglione, MD PhD
Department of Woman, Child and General and Specialist Surgery
Università degli Studi della Campania “Luigi Vanvitelli”
Naples, Italy
Marsha Treadwell, PhD
Director, Northern California, Network of Care for Sickle Cell Disease
Co-Principal Investigator and Regional Director, Pacific Sickle Cell Regional Collaborative
Director, Hematology Behavioral Services
Comprehensive Center for Sickle Cell Disease
Main Hospital
747 52nd Street
Oakland, CA 94609
Winfred C. Wang, MD
Member, Department of Hematology
St. Jude Children’s Research Hospital
262 Danny Thomas Place, MS 800
Memphis, TN  38105
Russell E. Ware, MD, PhD
Director, Division of Hematology
Institute Co-Director, Cancer and Blood Diseases Institute
Director, Global Health Center
Marjory J. Johnson Chair of Hematology Translational Research
Cincinnati Children’s
Professor, UC Department of Pediatrics
Cincinnati, OH
Julie Kanter Washko, MD
Associate Professor
Division of Hematology Oncology
University of Alabama at Birmingham
1720 2nd Avenue South, NP 2540
Birmingham, AL 35294-3300
Kim Smith-Whitley, MD
Professor of Pediatrics
Director Comprehensive Sickle Cell Center
Division of Hematology
11th Floor Colket Building
The Children’s Hospital of Philadelphia
34th & Civic Center Blvd.
Philadelphia, PA 19104
Board Member, Sickle Cell Disease Association of America
Wanda Whitten-Shurney, MD
CEO & Medical Director
Sickle Cell Disease Association, Michigan Chapter Inc.
Board Member, Sickle Cell Disease Association of America
Detroit, MI
Ahmar U. Zaidi, MD
Assistant Professor of Pediatrics
Comprehensive Sickle Cell Center, Children’s Hospital of Michigan, Wayne State University/Central Michigan University School of Medicine
Detroit, MD  

Joint FDA/ASH Led Initiative Highlights Importance of Using Patient Reported Outcomes and Biomarkers in Clinical Trials to Advance SCD Therapies
(WASHINGTON, DC, Dec. 6, 2019) — The American Society of Hematology (ASH) today released the most comprehensive set of recommendations to date aimed at establishing uniformity and global standards for clinical trial endpoints used to evaluate new therapies for sickle cell disease (SCD). The new recommendations – published in two companion papers in the current issue of Blood Advances – are the result of seven expert and patient led panels convened by ASH and the U.S. Food and Drug Administration (FDA) to improve the design of clinical trials for new SCD therapies, including promoting broader use of patient reported outcomes and biomarkers as clinical endpoints.
Sickle cell disease is the most common inherited red blood cell disorder in the United States, and it affects millions of people worldwide. In people living with SCD, the red blood cells, which are normally round, become crescent or sickle-shaped which contributes to the vaso-occlusive crises these patients experience. People with SCD suffer from an array of physical complications, including acute pain crises, joint and organ damage, impaired cognitive function, and a reduced life expectancy. In addition to the immense physical burden they must endure, people with SCD are often stigmatized due to a poor understanding among healthcare professionals and the general public of the life-limiting effects of the disease. Supporting SCD research and access to care efforts for all people living with the disease is a chief priority for ASH.
While the molecular basis of SCD has been well understood for decades, there are currently only four FDA approved treatments for this debilitating condition. Bone marrow transplant is a cure for some individuals with SCD, but it is not an option for everyone. Although two new treatments for SCD have recently been approved, and many others – including potentially curative gene therapies – are in development, medical experts and FDA officials agree there is a need for uniformity around clinical trial endpoints to ensure these new therapies deliver a meaningful benefit from the patient’s perspective. “There are a number of investigational drugs in development that target different manifestations of SCD,” said Julie Panepinto, MD, MSPH, FAAP, Professor of pediatric hematology, Medical College of Wisconsin/Children’s Wisconsin, co-chair of ASH’s Guideline Oversight Committee, and co-chair of the workshop. “However, there are no clear standardized endpoints for evaluating the effect of therapies on clinical outcomes and patient well-being.”
Dr. Panepinto said that amid the burgeoning effort to develop curative SCD therapies, clinical research should incorporate endpoints that are not only measurable, but also relevant and directly beneficial to the patient based on their preferences and experience.
“What’s happening in SCD is really exciting and many of us feel we are on the cusp of identifying multiple disease-modifying therapies,” Dr. Panepinto said. “The field is exploding, so we want to be sure we are measuring relevant endpoints for researchers, clinicians, and patients because that helps us advance the field and get new therapies approved.”
Ann Farrell, MD, Director of the FDA’s Division of Hematology Products and co-chair of the workshop, points out that the scientific understanding and the treatment of SCD have evolved to a point that the endpoints used to evaluate earlier SCD treatments are now inadequate.
“These changes have greatly impacted the discussion the Agency is having with the pharmaceutical industry as new clinical trials are designed,” said Dr. Farrell. “We need endpoints that better reflect the patient experience of their disease in the current healthcare system.”
To address the global burden of SCD, ASH and the FDA convened seven panels of clinicians, investigators, and people with SCD in a two-day workshop to bring uniformity and standards to existing endpoints, identify gaps, and propose development of new endpoints as a focus for future research. Led by Drs. Panepinto and Farrell, the panels conducted extensive literature reviews, assessed available evidence, and used expert judgement to identify which existing endpoints can be incorporated into SCD clinical trials and what additional data are needed. The panels focused on the following areas:
· Patient reported outcomes (PROs) – the panel suggested that future trials should measure the impact in three key domains: crisis and non-crisis pain; affect (including emotional impact, sleep quality and fatigue), and function (social, physical and cognitive),
· Pain (non-PROs) – this panel recommended measuring healthcare utilization, analgesic use, and physical function as a complementary measure to PROs on pain,
· The brain – the panel reviewed and identified diagnostic modalities to assess neurological risk, document stroke, and measure cognition and educational attainment,
· End-organ considerations – the panel stressed the need to use biomarkers and endpoints that capture the progression of renal and cardiopulmonary disease,
· Biomarkers – this panel overlapped with other panels addressing various disease manifestations, highlighting the importance of developing and validating a broader array of biomarkers that can measure response to therapy,
· Measurements of cure – given this is a relatively new area of research, the panel identified the need to develop appropriate biomarkers to evaluate the effect of curative therapies, and to capture and share these data in a central repository to help advance the scientific understanding and broader use of these treatments,
· Care in low-resource settings – this panel noted an opportunity to accelerate clinical trials in regions with a high prevalence of SCD, such as sub-Saharan Africa. They also recommended capturing data on early childhood, peri-operative and pregnancy-related mortality, as well as PROs from children and their caregivers relating to growth and development.
The published recommendations represent the most comprehensive review of science to date in the treatment of SCD and will be a valuable reference for academic researchers seeking funding for scientific studies and pharmaceutical companies looking to identify endpoints for specific clinical trials.
“The papers document the wide-ranging discussions held by researchers, patients, caregivers, international experts, pharmaceutical industry and government to understand where we are in terms of defining clinical trial endpoints for current use and those for future development that will serve patients best,” said Dr. Farrell. She also noted the initiative identified several opportunities to incorporate the patient voice in clinical practice to help better understand how effective current medical treatments are.
The workshop was modeled after an earlier ASH/FDA initiative that focused on leukemia and myeloma, and was generously supported solely by the philanthropic support from numerous individual donors who contributed to the ASH Foundation’s Sickle Cell Disease Initiative Fund and by the Doris Duke Charitable Foundation.
In 2016, ASH launched a multifaceted initiative to address the burden of disease both in the United States and globally. For this initiative, ASH has developed clinical guidelines for SCD management and care, expanded education and training efforts, advocated with policymakers to enhance and expand federal SCD programs, and founded the Sickle Cell Disease Coalition. In addition to these efforts, the ASH Research Collaborative (ASH RC) SCD Clinical Trials Network was developed with the mission to improve outcomes for individuals with SCD by expediting SCD therapy development and facilitating innovation in clinical trial research. It provides the infrastructure for identifying patient cohorts for trials, matching trial sponsors with sites, facilitating recruitment of eligible patients, and ensuring optimally designed trials and an efficient, coordinated approach. Through patient engagement and optimized clinical trial execution, the Clinical Trials Network is helping to bring new and more effective therapies to individuals with SCD.
Papers in Blood Advances:
· End points for sickle cell disease clinical trials: patient-reported outcomes, pain, and the brain
· End points for sickle cell disease clinical trials: renal and cardiopulmonary, cure, and low-resource setting
The American Society of Hematology (ASH) (www.hematology.org) is the world’s largest professional society of hematologists dedicated to furthering the understanding, diagnosis, treatment, and prevention of disorders affecting the blood. For more than 60 years, the Society has led the development of hematology as a discipline by promoting research, patient care, education, training, and advocacy in hematology. ASH publishes Blood (www.bloodjournal.org), the most cited peer-reviewed publication in the field, and Blood Advances (www.bloodadvances.org), an online, peer-reviewed open-access journal.