The global burden of sickle cell disease: The quality of treatment shouldn’t depend upon where you live

The difference in the quality of life and longevity for sickle cell disease patients between high- and low- to middle-income countries is stark. Much like the differences in pediatric cancer survival rates, where you live is most important.

In high-income countries like the United States, sickle cell disease patients can expect to live well into their 40s or longer with proper treatment.

But 50-90% of children born with sickle cell disease in sub-Saharan Africa who don’t receive that care die before they turn 5.

St. Jude Children’s Research Hospital is uniquely positioned to help alleviate this burden by assisting to improve weak health infrastructure, promoting access to healthcare, and improving the quality of education for patients, parents and health care professionals. They already do this for pediatric cancer.

Sickle cell disease: Why I am here

Growing up in Southwest Nigeria, I became very familiar with the disease personally and professionally. Some of my friends with sickle cell disease were called derogatory names by many who had a lack of understanding of sickle cell disease. I mourned close friends who succumbed to complications from the disease. Later on, as a physician, I managed pain episodes, acute chest syndromes and myriad infections in sickle cell disease patients. In 2010, more than 300,000 babies worldwide were born with sickle cell disease. Of them, more than half were from Nigeria, DR Congo and India. And in Africa, it accounts for up to 15% of mortality in children younger than 5.

In the five years I’ve been in the U.S., I see the difference in the level of care children receive here. If we can provide that same level of care to sickle cell disease patients in developing countries, we can improve their health outcomes and quality of life. Because an overwhelming majority of sickle cell cases occur in the developing world, addressing this disparity can have a huge effect on the global burden of sickle cell disease. I joined St. Jude because I believe we can help solve this problem.

The global burden: scaling therapies and treatments

The World Health Organization (WHO) and the United Nations (UN) designate sickle cell disease as a global health problem. Both organizations have plans to address limited health resources in developing countries, specifically newborn screening programs. These are critical to improving survival, but also very challenging to implement in low-income, high-burden settings like countries in Africa.

The U.S. has a mandatory newborn screening program for sickle cell disease, which allows for prompt diagnosis and referrals to the appropriate providers. This comprehensive care was instituted several years ago in the U.S. and has dramatically improved health outcomes.

Why can’t we do this in other countries? In many parts of Africa, there isn’t newborn screening for sickle cell disease. In fact, no country in sub-Saharan Africa has implemented a universal screening program for any disease — there also aren’t preconception screening programs for adults who wish to have children.

There are five simple things we can work to implement that could potentially have an immediate, significant effect on health outcomes of children with sickle cell disease.

1. Newborn screening
2. Infection prevention using penicillin
3. Early use of hydroxyurea therapy
4. Routine screening for end-organ damage
5. Standardized treatment plans for acute complications

St. Jude is already improving global health outcomes for pediatric cancers through St. Jude Global and its partnerships. If we focus on improving global health outcomes for children, we also reach sickle cell disease and other childhood diseases that often disproportionately affect the most vulnerable communities.

Sustainable development goals to reduce global burden

Improving care for children with sickle cell disease ultimately improves care for all children. In 2015, the United Nations General Assembly set 17 interlinked objectives, called Sustainable Development Goals, to achieve a more viable and sustainable future for all. The third objective is good health and well-being, measurable through reductions in mortality and access to health care.

Through a combination of health care advocacy and clinical research, I hope to raise awareness among the scientific community to the disparities in care for children with sickle cell disease. I also hope to collaborate with colleagues who have devoted their lives to improving health outcomes for all children with sickle cell disease both in the U.S. and developing countries.

I want to find ways to implement culturally acceptable, affordable and accessible comprehensive care for children with sickle cell disease anywhere in the world.