Jennifer Knight-Madden | Sickle cell disease – coming out of silos to advance care

Sickle cell disease (SCD) is a genetic disease that affects the blood. Persons inherit a gene from each parent that changes how haemoglobin is made. Haemoglobin is a protein in red cells that gives blood its red colour and delivers oxygen to the organs around the body.

The abnormal sickle haemoglobin doesn’t transport oxygen efficiently. It also causes the cells to become sickled shaped. These cells break down more quickly than normal red cells, leading to anaemia. The severity of anaemia and other complications is variable. Some people living with sickle cell disease (PLWSCD) die in infancy if not treated, while others live long lives with relatively few complications. Most PLWSCD are between the extremes, many having significant suffering from the illness. All organs may be affected by complications that can be acute (sudden) or chronic (long lasting).

Therefore, people living with PLWSCD need to have a medical facility that is their “home” that provides preventative care and education about their disease as well as access to different medical and surgical specialists. The most common acute complications are severe pain in the bones and acute chest syndrome (similar to a severe pneumonia). Patients with SCD often have to go to the Accident & Emergency Department and may be admitted to hospital. This illness can adversely impact the lives of patients and their families. They may need access to professionals such as social workers and psychologists and resources such as Programme of Advancement Through Health and Education (PATH).

In the past, SCD stakeholders in Jamaica worked in silos, each trying to improve the lives of people living with SCD (PLWSCD) in their own way. The Ministry of Health and Wellness developed and promoted policy, the Regional Health Authorities provided on-the-ground-care to PLWSCD who presented at their healthcare facilities as did clinicians at the Sickle Cell Unit (SCU) and doctors in their private practices. Academics at SCU, and elsewhere, undertook and published research and PLWSCD, their families, and NGOs advocated for improvements in care. Since 2014, the chief medical officer-mandated Sickle Cell Technical Working Group has brought all of these players to a common table where they have worked together to advance care for PLWSCD.

SUCCESSFUL

The joint approach, supported by the leadership of these entities, has been incredibly successful. As of 2015, screening of newborns in public hospitals for SCD became universal, building on a process that started in 1995. By 2020, all private hospitals had also joined the newborn screening programme. In recent years, more than 99 per cent of infants born in Jamaica have been screened.

The SCTWG is now working to get all infants who screen positive for SCD into care in a timely manner. As a part of the care provided, special vaccines that significantly decrease the chances of specific severe infections that PLWSCD are more prone to are now free of cost to the patients. Infants receive antibiotics from four months of age. Many deaths were prevented in these infants by teaching parents/guardians what to look for to avoid severe complications.

Also in 2015, SCD was added to the list of conditions for which medications are subsidised by the National Health Fund. This included the subsidy of hydroxyurea, the only oral medication available to decrease the severity of the disease, even decreasing strokes in children with SCD. Other medications such as antibiotics, pain killers, and metered dose inhalers (pumps) critical to the treatment of SCD are also covered. In 2021, additional items were covered, including those used in treating the dreaded chronic leg ulcers, which occur often, and kidney complications.

Patients attending public-health facilities get medications free of charge. Through the advocacy of the SCTWG, patients attending the SCU, the largest specialist centre for SCD in the hemisphere, now also access medications through Drug Serv Pharmacies with no user fee.

A major activity of the SCTWG is education of healthcare providers. Scores of training sessions have been undertaken, with recent emphasis on how to treat SCD pain, an area of concern highlighted by a needs assessment among adults with SCD and parents of children with SCD. Other areas covered have included newborn screening and early childhood care, use of hydroxyurea, care of the many complications of SCD and the importance of teachers and school nurses to children with SCD.

PUBLIC AWARENESS

Public awareness continues to be a focus. Annually, World Awareness SCD Day on June 19 and SCD Awareness Month in September have seen the stakeholders collaborate to be part of Church services, hold webinars, utilise opportunities in print and electronic media as ways to raise the profile of the disease. Education of PLWSCD to enhance their lives continues. Recently, videos have been produced and shared on social media which give an overview of SCD, tips on self-help, and guidance in accessing healthcare and other governmental resources.

A major aspect of the work of the SCTWG is the integration of the care of PLWSCD into existing public-health programmes, with the aim of providing the same standard of care across the island. Tools such as audits have been developed and are being implemented to review the quality of the care provided so that areas in need of more training and/or resources can be identified. A passport is being developed that will allow healthcare providers in different health facilities to be aware of the patients’ previous medical history across settings.

SCD stakeholders have shown that progress can be made when we are willing to work together for a greater good. We invite all Jamaicans to be part of improving life for PLWSCD.

Jennifer Knight-Madden is professor of paediatric pulmonology and clinical research, Sickle Cell Unit, Caribbean Institute for Health Research, The University of the West Indies. Send feedback to jennifer.knightmadden@uwimona.edu.jm.