Lesley King | Today is World Sickle Cell Day

WORLD SICKLE Cell Day is celebrated around the world each year on June 19. It is a day to bring greater awareness about sickle cell disease (SCD) and the challenges faced by patients, families and caregivers.

Despite an estimated 500,000 babies being born worldwide each year with SCD, it is still considered a rare and neglected disease. Most babies are born in lower and middle-income countries (LMICs) like Sub-Saharan Africa (SSA), India and Jamaica. There are significant differences in the level of care and access to treatments in LMIC’s compared to high-income countries.

SCD is a genetic disease that affects the shape of red blood cells, causing them to be sickle-shaped, rigid and sticky. The sickle red blood cells block blood flow and the delivery of oxygen to the tissues. This can lead to many problems such as bone pain, stroke, kidney failure and chronic leg ulcers. Although we know that SCD originated hundreds of years ago in Africa, it was in 1910 that the first person, Walter Clement Noel, was reported with the disease.

AFFECTED PERSONS

Interestingly he was a dental student from Grenada studying in Chicago. One hundred and fifteen years later, much has been learnt about the disease and how to care for affected people. However, much more is needed to improve care, access to treatment and quality of life (QOL) especially for people living in LMICs.

People with the disease inherit two copies of abnormal genes, one from each parent. Persons with one copy of these abnormal genes have traits and usually have no problems. Public education and awareness are critical to enable persons to make informed reproductive choices. Everyone should know their status and that of their potential partners. This is an important step in efforts to decrease the number of babies born with SCD annually. In countries where custom and cultures allow, pre-marital and antenatal screening assist couples with family planning decisions.

In SCD, the first three years of life is an extremely vulnerable period, with high risk for death. Diagnosing SCD shortly after birth and enrolling babies immediately into a programme of care can make a difference. Disease education and implementation of simple, inexpensive interventions can lower complications and early death. Sadly, in areas such as SSA where most babies with SCD are born each year, this care is not available and 50-80 per cent will die before the age of five years. In Jamaica, almost all infants diagnosed at birth whose parents accept care, live long beyond five years.

UNPREDICTABLE COMPLICATIONS

Persons living with SCD (PLWSCD) are often affected by acute, unpredictable complications. The most common complication is bone pain crises which ranges from mild to severe and debilitating pain. For the PLWSCD, recurrent pain crises can consume their life; for the healthcare provider finding the balance between good pain control and the limiting side-effects of strong painkillers needed for treatment can be frustrating and challenging. A better understanding of sickle pain, development of safer painkillers and a holistic approach to pain management offers better outcomes.

We realise there are many unmet needs of PLWSCD. Support systems are needed at all levels – family, community, and health system. Without these, PLWSCD face challenges such as poor education, inability to find and keep jobs, and inability to have meaningful relationships. In addition, psychological problems such as loneliness, depression, and anxiety are common. Supporting, prioritising and allowing better access to these systems can have a significant impact on the QOL of PLWSCD.

Hydroxyurea, an effective treatment for SCD, is underutilised. Many people who could benefit from this treatment do not access it even though, in most people, it reduces complications and improves QOL. Curative therapies such as bone marrow transplantation (BMT), and gene therapy are now possible after years of research. The reality is that cure is expensive, costing US$100-US$250,000 for BMT and US$2.2–US$3.1 million for gene therapy. For the near future, these treatments will not be accessible for most PLWSCD.

In Jamaica, we will celebrate World Sickle Cell Day 2025 under the theme ‘Transforming Care and Strengthening Communities’. This theme highlights local initiatives which align with global recommendations, aimed at improving access and standard of care for PLWSCD. Jamaica has made significant strides in management of PLWSCD. This includes an SCD programme with governmental oversight, screening of all babies for SCD at birth, provision of care centers for SCD babies and vital treatments such as special vaccines and penicillin antibiotics free of charge. Hydroxyurea is available and drug costs are subsidised through the National Health Fund.

SCD PATIENT REGISTRY

Plans are afoot to create regional clinics where staff are trained in SCD care allowing all PLWSCD to access routine care, including Hydroxyurea therapy, closer to home.

The development of an SCD patient registry will give more accurate information on the number of persons living in Jamaica with SCD and where they live so that appropriate public health services can be provided where the need is greatest.

A patient passport has been developed which will enable better sharing of information especially for patients who visit multiple care sites. Enhancing public awareness campaigns can potentially contribute to better, more-informed reproductive choices and better outcomes for all affected.

A lot has been achieved over the past 115 years, but more must be done for a disease that continues to cause significant suffering for many. World Sickle Cell Day is here again to remind us of this.

Dr Lesley King is head, clinical services at the Caribbean Institute for Health Research, UWI. Email: lesley.king@uwi.edu. Send feedback to columns@gleanerjm.com