United to Conquer Sickle Cell Disease

The State of Sickle Cell Disease

The State of Sickle Cell Disease: 2018 Report Card was created by sickle cell disease partners as a follow up to the previous report card which was released two years ago, in 2016. The 2018 Report Card is based on the feedback of more than 100 stakeholders in the SCD community and showcases a snapshot of the state of four major areas: access to care in the United States., training and professional education, research and clinical trials, and global issues. The report card indicates that initiatives like nationwide training programs for providers and recent legislation to enhance access to quality care have moved the needle in the right direction. While progress is being made, the report card also serves as a reminder that more can be done across many priority areas for individuals with SCD.

The following organizations have endorsed the report card:

  • American Academy of Emergency Medicine
  • American College of Emergency Physicians
  • American Society for Apheresis
  • American Society for Clinical Pathology
  • American Society of Gene & Cell Therapy
  • American Society of Hematology
  • American Society of Pediatric Hematology/Oncology
  • ApoPharma
  • bluebird bio
  • Cerus Corporation
  • Functional Fluidics
  • Global Blood Therapeutics, Inc.
  • International Association of Sickle Cell Nurses and Professional Associates
  • Ironwood Pharmaceuticals, Inc
  • Medunik USA
  • NMDP/Be The Match
  • Novartis Pharmaceuticals Corporation
  • Sickle Cell Aid Foundation (SCAF)
  • Sickle Cell Community Consortium
  • Sickle Cell Disease Association of America, Inc.
  • Sickle Cell Disease Association of Canada/ Association d’Anémie Falciforme du Canada  
  • Sickle Cell Outcome Research (SCORE) program, the Netherlands
  • Sickle Cell Warriors
  • STAR - Sickle Transplant Alliance for Research
  • Terumo BCT
  • Vanguard Therapeutics, Inc.

To further understand the state of sickle cell disease in 2018, those who were polled were asked to include any comments they felt may be relevant regarding their thoughts about access to care in the U.S., research and clinical trials, education and training, and global issues. The following are direct quotes from the SCD stakeholder community.

Access to Care in the U.S.

When there are millions of dollars and programmatic structures in place for every person with sickle cell disease in the US to have access to quality care, why are we struggling to implement care guidelines across the nation? Research grants like the NHLBI $36M and PCORI $18M are beginning to look at what is happening at the patient level in transition, but how much of this will be replicated for use across the country once these projects end?

Access is probably best in urban pediatric centers. Rural areas are highly underserved. There is a great shortage of health care providers interested in and experienced in caring for adults with SCD.

Access to knowledgeable care for adults with sickle cell disease is poor, at best, with very little if any improvements. The fact sheet addresses patients; however, access to GOOD care also includes ensuring that providers are well equipped to care for these individuals.

Training and Education

The American Society of Hematology and American Academy of Pediatrics initiatives are reaching mostly the people who are already interested in SCD. More progress is expected through the Emergency Medicine, Emergency Nursing, and Hospitalist societies.

There needs to be training directed towards primary care providers in internal and family medicine, and pediatricians outside of urban areas with comprehensive care centers.

Education among medical professionals has not been implemented nationwide. There are sporadic providers in each state that are knowledgeable when it comes to Sickle Cell Disease and treatment. The majority of providers depend on the knowledge and information from the few which places an undue burden on the few knowledgeable providers. I hope that upon publishing of the new treatment guidelines in 2019 and nationwide training will follow.

Research and Clinical Trials

The burgeoning of clinical trials is great but informing patients about them is difficult.

While there is a lot of attention right now to clinical trials, there is very little attention paid to the sickle cell patient aside from entering them into registries and helping them understand clinical trials for the sole purpose of getting them to participate. More should be done to support persons with sickle cell disease and their families, so that they are in a state of wellness and stability so that they can participate.

 It is encouraging that there is a current surge of interest and investment by pharmaceutical companies, and in gene therapy, gene editing, and alternative donor transplants. 

Global Issues

SCD care has improved in recent years especially with hydroxyurea, still a lot needs to be done for early diagnosis and newborn screening in developing countries.

Globally we are becoming more aware of the issues pertaining to sickle cell, now, where is the funding to do something about it?

There is slow but steady progress. Clearly the problem being addressed is complex and is hard to tackle. I attended the 3rd Global Congress Meeting in India and was impressed with the increasing awareness and discovering a number of dedicated individuals trying to make a difference.

2016 

Over the past century, great advances have been made in the understanding and treatment of sickle cell disease (SCD). However, many basic scientific processes are still not fully understood, too few treatments have been developed, and most of the people who have SCD do not have access to the treatments that could improve the duration and quality of their lives.

The State of Sickle Cell Disease: 2016 Report has been created by the American Society of Hematology (ASH) based on the feedback of more than 100 thought leaders and representatives from other organizations to look at the state of SCD and identify the greatest opportunities for improvement. This report is a summary of the current state of research, access to care, and global issues, and it provides an outline of opportunities to change the status quo for people with this disease. ASH is inviting partner organizations to endorse the report and take on projects that will advance the outlined goals.

The following organizations have endorsed the report:

  • American Academy of Emergency Medicine
  • American Academy of Pediatrics
  • American College of Emergency Physicians
  • American Public Health Association
  • ApoPharma
  • American Society for Clinical Pathology
  • American Society of Pediatric Hematology/Oncology
  • Association of Public Health Laboratories
  • Emergency Department Sickle Cell Care Collaborative*
  • Emergency Nurses Association
  • Emmaus Life Sciences
  • Foundation for Sickle Cell Disease Research
  • Global Blood Therapeutics
  • Global Sickle Cell Disease Network
  • GlycoMimetics, Inc
  • International Association of Sickle Cell Disease Nurses and Physician Assistants
  • Mast Therapeutics, Inc.
  • National Marrow Donor Program
  • National Medical Association
  • Pfizer Inc.
  • Prolong Pharmaceuticals
  • Sickle Cell Adult Provider Network
  • Sickle Cell Disease Association of America
  • Sickle Cell Warriors

*The Emergency Department Sickle Cell Care Collaborative (EDSC3) endorses the State of Sickle Cell Disease: 2016 Report. The endorsement by EDSC3 does not represent or obligate in any way any of the other organizations that are represented by EDSC3 members.

Report Addendum: Priorities for Sickle Cell Disease

This is the full list of priorities for SCD that was developed from a 2015 Sickle Cell Disease Summit to provide a roadmap to move the needle forward on this disease. These domains and the long- and short-term priorities were suggested by SCD stakeholders: individuals with SCD and their families, care-givers, researchers, experts, and others.

Report Card

ASH has polled individuals with SCD, health care providers, and global health leaders to rank each area contributing to the overall state of SCD on a scale of 1 to 10. Though advances have been made, these scores show us that we have much to do to improve the state of care for those living with SCD.


State of Sickle Cell Disease 2016 Report is published by The American Society of Hematology. Copyright © 2016. No part of this publication may be reproduced without the prior consent of the publisher. All rights reserved.

ASH does not assume any responsibility for any injury and/or damage to persons or property arising from or related to any use of the material contained in this publication.

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The status quo is unacceptable, and we are setting out to change it

People with sickle cell disease (SCD) are afflicted on two fronts — one by having a serious, chronic condition that inflicts pain and other complications — the other by a fragmented system of care.

Today, there are opportunities to transform this disease and the way we care for people with SCD. We are launching an international call to action on SCD by bringing together researchers, clinicians, individuals with sickle cell disease and their families, policymakers, and the private sector to focus our collective efforts and change the state of SCD around the world.

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The status quo is unacceptable & we are setting out to change it. Join us to #ConquerSCD! www.scdcoalition.org

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