‘Sickle Cell and Thalassemia Screening – What Parents Think’

Parent’s Stories’ is a new report that has been launched containing experiences from parents of African, Caribbean, Middle-Eastern, Asian and Mediterranean origin who have been through the United Kingdom’s National Health Service (NHS) Sickle Cell and Thalassemia Screening Program and who were at increased risk of having a baby with either sickle cell or Thalassemia, two serious genetically inherited blood conditions. The report stems from a successful collaborative project between the Screening Program and two patient representative organizations – the Sickle Cell Society (SCS) and United Kingdom Thalassemia Society (UKTS). The project aimed to determine any barriers affecting the timeliness of the offer of screening and prenatal diagnosis (PND) to couples and recommend ways of improving screening services.  As part of the project,  ‘at –risk’ couples who each carried a gene for sickle cell or Thalassemia who had been through antenatal screening within the last five years were interviewed and their shared stories made up the rich evidence contained in the ‘Parent Stories’.
Some Key Points that emerged:
  • Most women had told their doctor (GP) about their pregnancy early on and already knew their sickle cell or Thalassemia status before becoming pregnant.
  • Parents said some healthcare professionals (including some GPs) did not have much knowledge of the conditions or the screening pathway and did not recognize the need for prompt referral to counseling and PND. Parents wanted prompt referral or ‘self-referral’ to specialist nurses and midwives at the Sickle Cell and Thalassemia Centers as from their experience this speed things up.
  • Parents also wanted to meet individuals who were living successfully with sickle cell or Thalassemia and to be put in touch with patient organizations who could find someone in the community to support them.
What Happens Next?
These parent stories are an invaluable resource for people commissioning services and working in the Screening Program and have contributed to a report with recommendations for community organizations, the public and screening service providers.
The full ‘Parent Stories’ report is available at:
For hard copies please email: info@sicklecellsociety.org

Living Well with Sickle Cell Conference

Sickle Cell Conferences and Events

The Sickle Cell Disease (SCD) Program at Children’s National invites you to the 7th Annual Family Education Symposium on “Living Well with Sickle Cell”.
Saturday, October 29, 2016 11:30 AM – 4:30 PM
Sheikh Zayed Campus for Advanced Children’s Medicine Children’s National Health System 111 Michigan Ave NW, 2nd Floor, Auditorium
Washington, DC, 20010
This year’s symposium will focus on helping patients and their families manage sickle cell disease while living life to the fullest.

Improving Emergency Care: Free Guidebook SCD

Anemia word cloud concept
Anemia word cloud concept

NICHQ, the National Institute for Children’s Health Quality, today announces a new free guidebook: “Sickle Cell Pain in the Emergency Department: A Guide to Improving Care,”

NICHQ, the National Institute for Children’s Health Quality, today announces “Sickle Cell Pain in the Emergency Department: A Guide to Improving Care,”  a step-by-step manual for anyone on the front lines of acute care, from emergency room (ER) doctors and nurses, to hematology specialists. The guide, launched on World Sickle Cell Day, is the result of a five-year collaboration between 15 multidisciplinary health care provider teams nationwide. Throughout the process, teams traded lessons learned and best practices for improving treatment for adults and children with sickle cell disease (SCD) in the ER.
An inherited red blood disorder, SCD is a chronic illness characterized by anemia and unbearable bouts of pain, so providing relief quickly is crucial to treatment. This incurable disease can lead to declining health, poor quality of life and early mortality. NICHQ Strategic Project Director Dr. Suzette Oyeku, said, “Sickle cell disease patients wait in excruciating pain longer than they have to for treatment. Guidelines do exist, but they have not been consistently implemented, particularly in acute care settings. Our guide was designed to help providers bridge this gap.”
NICHQ’s SCD work was funded by The U.S. Health Resources and Services Administration (HRSA) which oversees the SCD Treatment Demonstration Program and the SCD Newborn Screening Program. As lead facilitator for the programs, NICHQ was responsible for organizing and reporting on meetings; collecting, analyzing and distributing best practice data; and serving as the liaison between HRSA and the teams. The two projects yielded many results in improving care for SCD patients in the ER. These included a reduction in the wait time for evaluation by 69 percent and a 29 percent improvement in the time between triage and receiving pain medication. One of the most significant outputs of the teams’ extensive work is “Sickle Cell Pain in the Emergency Department: A Guide to Improving Care.” The online publication offers best practice findings such as standardized order sets, a recommendation to consider using intranasal fentanyl and a color-coded, full body chart, reviewed by patients and doctors, as a standard pain assessment tool.
According to HRSA Project Officer Dr. E. Donnell Ivy, NICHQ and the Treatment Demonstration and Newborn Screening program grantees have pulled together an excellent resource. He said, “On World Sickle Cell Day and as Sickle Cell Awareness Month approaches, the timing for sharing this comprehensive guide could not be better. We congratulate NICHQ and all of our grantees on advancing the quality of care for these patients. As a result of their work, thousands of patients, from coast to coast, will receive better treatment in the ER.”

PhenX Toolkit Seeks Comments

The Toolkit provides standard measures related to complex diseases like sickle cell disease, phenotypic traits and environmental exposures. Use of PhenX measures facilitates combining data from a variety of studies, and makes it easy for investigators to expand a study design beyond the primary research focus. All Toolkit content is available to the public at no cost.

Information about the project is available at www.phenx.org The National Human Genome Research Institute (NHGRI) awarded RTI International a four-year cooperative agreement to expand and enhance the PhenX Toolkit at https://www.phenxtoolkit.org/

An important aim of this grant is to review the measures in the 21 research domains after receiving input from the scientific community.

Please take a few minutes to review the measures in these PhenX domains: Demographics, Environmental Exposures, and Social Environments. Your rating (number of stars) should reflect how useful this protocol is to your work and its relevance to the PhenX Toolkit. We tried to make it easy for you to rate the measures you are familiar with; you do not have to rate all of them. Please click Review PhenX Toolkit Measures to rate and comment on these measures.