Sickle Cell News | July 2015

Sickle Cell News for July 2015 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,” at http://nichq.org/sitecore/content/sickle-cell-disease/sickle-cell-disease/resources/scd-acute-care-guide  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.” New Video Resource from Duke University - http://sickleemergency.duke.edu/ Several different types of resources are included.  These resources are designed to be beneficial for healthcare professionals (e.g. physicians, nurses, nurse practitioners, physician assistants) who are not sickle cell experts and practice in a variety of settings (e.g. hospitals, primary care clinics, and emergency departments).  ·         “SCD Conferences” include select presentations from a two day, yearly SCD conference held at Duke. ·         “Short presentations for online learning” were specifically developed for adaptation to hospital-based, online learning systems. Each presentation includes 10 slides of content, and two post test questions. Short videos are included in many of these slide sets. ·         “Videos” include links to professional videos that serve as additional learning opportunities. Some of the videos from the short presentations are also listed. Sickle Cell Disease modules at http://www.bioethicsinstitute.org/research/projects-2/hope/module-directory Bipartisan Stem Cell Therapeutic and Research Reauthorization Bill Heading to Floor Action - Congress must approve before Sept. 30 deadlineVital legislation to reauthorize America's bone marrow and cord blood donor registry cleared a major hurdle today as the full Energy and Commerce Committee unanimously approved the measure just before adjourning for the August district work period. Sponsored by Rep. Chris Smith (R-NJ) with lead co-sponsor Rep. Doris Matsui (D-CA), the Stem Cell Therapeutic and Research Reauthorization Act of 2015, H.R. 2820, ensures that two collaborative programs that support treatment and therapies derived from adult stem cell lines will not expire at the end of the federal fiscal year. First passed in 2005, the original legislation established a nationwide integrated bone marrow and cord blood stem cell transplantation program. Stem cells derived from cord blood and bone marrow have been used successfully to treat thousands of patients with such diseases as leukemia and sickle cell anemia, and genetic disorders. The enactment of H.R. 2820 will enable more people to receive similar treatments and go on with their lives. Sickle cell patients in Angola to receive important treatment through partnership programhttps://www.bcm.edu/news/hematology/angola-sickle-cell-patient-treatment Sickle cell disease patients who receive care through the public health service in Cabinda, Angola, Africa, will now have access to an important treatment for the disease, announced leaders of the Angola Sickle Cell Initiative (ASCI), a public-private partnership of the Angola Ministry of Health, the Baylor College of Medicine International Pediatric AIDS Initiative (BIPAI), Texas Children’s Hospital and Chevron Africa Latin America Exploration and Production Co.  The medicine, called hydroxyurea, was donated by the global biopharmaceutical company Bristol-Myers Squibb.  “Hydroxyurea reduces the frequency of pain events and need for blood transfusions in patients with sickle cell disease; however, most patients in resource-poor Angola have not had access to the treatment,” said Dr. Gladstone Airewele, associate professor of pediatrics at Baylor College of Medicine, head of the Global Hematology Program at Texas Children’s Hospital and leader of the Angola Sickle Cell Initiative.  Sickle cell disease is an inherited disorder in which red blood cells become irregularly shaped. These sickle-shaped cells can get stuck in small blood vessels, which can slow or block blood flow and oxygen to parts of the body, resulting in painful episodes, serious infections, chronic anemia and damage to body organs. Higher hemoglobin concentration reduces the chance that the red blood cells will become sickle-shaped. Blood transfusions are a common treatment for sickle cell disease – also called sickle cell anemia – but there is an absence of reliable blood supply in Angola.  “This makes the need for hydroxyurea urgent because it raises the hemoglobin concentration, reducing the need for transfusions,” said Dr. Maria de la Paz Noli, a physician with BIPAI’s Global Health Corps in Cabinda, Angola. "A child’s place of birth should not determine whether he or she survives a treatable disease. Similar to children with sickle cell anemia in the U.S., those with the disease in Angola should also be able to look forward to lives as productive adults.  Angola has one of the world's highest rates of the genetic blood condition with up to 10,000 babies born with the disease each year, contributing greatly to the country's high death rate of children under 5 years of age. “For the children in Angola who are born with sickle cell anemia each year, life is often painful and painfully short,” said John Damonti, president of the Bristol-Myers Squibb Foundation. “Hydroxyurea is an effective treatment for sickle cell anemia that avoids costly blood transfusions but this medicine is not available in Angola, which has one of the highest rates of sickle cell anemia in the world. Our donation to Baylor College of Medicine/Texas Children’s Hospital’s demonstration project on humanitarian grounds will help the Angolan government understand available options to treat this deadly disease.” According to Bristol-Myers Squibb, hydroxyurea is indicated to reduce the frequency of painful crises and need for blood transfusions in adult patients with sickle cell anemia with recurrent moderate to severe painful crises (generally at least 3 during the preceding 12 months). Hydroxyurea should be administered under the supervision of a physician experienced in the use of this medication for the treatment of sickle cell anemia since treatment of patients with hydroxyurea may be complicated by severe, sometimes life-threatening, adverse effects. Texas Children’s Cancer and Hematology Centers have provided key support in the success of the Angola Sickle Cell Initiative. Since 2011, the Angola Sickle Cell Initiative has screened more than 115,000 newborns for sickle cell disease and provided high-quality care and treatment to affected children. Chevron has provided $9 million in funding over nine years to the Angola Sickle Cell Initiative.