May 2, 2024

Startling New Research: Sickle Cell Disease Is 11 Times More Deadly Than Previously Thought

A brand-new analysis reveals that sickle cell illness is a leading cause of death throughout numerous age groups and is most prevalent in sub-Saharan Africa, where newborn screening and treatment access are urgently needed. Rising mortality rates since 2000 are mainly driven by population growth in South Asia and sub-Saharan Africa.
The hereditary disease impacts 8 million people, disproportionately impacting young grownups, adolescents, and children.
A recent research study shows that the death toll credited to sickle cell illness is likely 11 times higher than what mortality records alone recommend. Not only is the condition typically underdiagnosed, however it likewise increases the threat of different problems such as infections, strokes, cardiac issues, kidney dysfunction, and problems during pregnancy.
This indicates that a doctor dealing with a patient with sickle cell illness who dies due to a stroke might be unaware that the person had the condition, or might not acknowledge that sickle cell disease can cause stroke. As a result, the doctor may not list sickle cell illness as a cause of death for that person.
When other sources of information on frequency and birth occurrence were integrated with mortality data in epidemiological modeling, in 2021, the “overall mortality problem” of sickle cell illness was 373,000 deaths, compared to 34,600 sickle-cell-only deaths, or “cause-specific deaths.” The increase was especially noticable in South Asia and sub-Saharan Africa, where the fatality figures were 67 times greater and 9 times higher, respectively.

The research study examined worldwide health data from 2000 to 2021 and was recently released in The Lancet Haematology journal. The research belongs to the Global Burden of Disease 2021 study coordinated by the Institute for Health Metrics and Evaluation (IHME) at the University of Washingtons School of Medicine.
” Our research exposes the plain reality that sickle cell disease is far deadlier than its textbook description,” states senior author Dr. Nicholas Kassebaum, Adjunct Associate Professor at IHME. “The number of infants born with sickle cell disease is increasing, which implies an extremely hard early youth. Clients are more vulnerable to infections and other extreme conditions, so early detection is key for treatment.”
In 2021, half a million children were born with sickle cell disease, and more than three-quarters of these births were in sub-Saharan Africa. Under the analysis of overall mortality concern (including secondary conditions), sickle cell illness was the 12th leading cause of death globally for kids under the age of 5 years. However, total sickle cell illness mortality burden was among the leading three causes of death in Portugal, Jamaica, Libya, Oman, and San Marino.
” Improved data collection is critical to tracking development on sickle cell disease. In order to overcome this data restriction, rather of utilizing mortality information alone to approximate overall sickle cell illness deaths, we used a mathematical algorithm that likewise takes input information from birth incidence, survival gradually, and frequency, and makes sure these measures are internally constant,” discusses Azalea Thomson, very first author and IHME researcher on the Neonatal and Child Health Team. “By using all readily available data, we were able to enhance our understanding of the true problem of sickle cell disease and better contextualize it along with other leading causes of death. In 2021, in kids under 5 years in sub-Saharan Africa, total sickle cell disease deaths went beyond those from malnutrition, measles, or syphilis.”
The research also highlights the requirement for policymakers and public health advocates to deal with the mainly underrecognized burden of sickle cell illness. Universal newborn screening, case monitoring through public registries, and early intervention treatment can ease suffering for some 8 million people coping with sickle cell illness.
” Universal newborn screening is essential for early diagnosis and management of sickle cell illness,” states Dr. Theresa McHugh, clinical writer at IHME who focuses on neonatal and child health. Increased worldwide awareness and adoption of health policies that expand neonatal screening and make treatment more available will go a long method in enhancing health results.”
Recommendation: “Global, local, and national frequency and mortality burden of sickle cell illness, 2000– 2021: a systematic analysis from the Global Burden of Disease Study 2021″ by Azalea M Thomson, Theresa A McHugh, Assaf P Oron, Corey Teply, Nikhil Lonberg, Victor Vilchis Tella, Lauren B Wilner, Kia Fuller, Hailey Hagins, Richard Gyan Aboagye, Melka Biratu Aboye, Eman Abu-Gharbieh, Ahmed Abu-Zaid, Isaac Yeboah Addo, Bright Opoku Ahinkorah, Aqeel Ahmad, Saif Aldeen S AlRyalat, Hubert Amu, Aleksandr Y Aravkin, Judie Arulappan, Maha Moh d Wahbi Atout, Ashish D Badiye, Sara Bagherieh, Maciej Banach, Morteza Banakar, Mainak Bardhan, Amadou Barrow, Deriba Abera Bedane, Isabela M Bensenor, Akshaya Srikanth Bhagavathula, Pankaj Bhardwaj, Prarthna V Bhardwaj, Ajay Nagesh Bhat, Zulfiqar A Bhutta, Mariah Malak Bilalaga, Jessica Devin Bishai, Saeid Bitaraf, Archith Boloor, Muhammad Hammad Butt, Vijay Kumar Chattu, Dinh-Toi Chu, Omid Dadras, Xiaochen Dai, Bardia Danaei, Anh Kim Dang, Fitsum Wolde Demisse, Meghnath Dhimal, Daniel Diaz, Shirin Djalalinia, Deepa Dongarwar, Muhammed Elhadi, Mohamed A Elmonem, Christopher Imokhuede Esezobor, Farshid Etaee, Oghenowede Eyawo, Adeniyi Francis Fagbamigbe, Ali Fatehizadeh, Lisa M Force, William M Gardner, Kazem Ghaffari, Paramjit Singh Gill, Mahaveer Golechha, Pouya Goleij, Vivek Kumar Gupta, Hamidreza Hasani, Treska S Hassan, Mohammed Bheser Hassen, Segun Emmanuel Ibitoye, Adalia I Ikiroma, Chidozie C D Iwu, Peter Bai James, Shubha Jayaram, Rime Jebai, Ravi Prakash Jha, Nitin Joseph, Farnaz Kalantar, Himal Kandel, Ibraheem M Karaye, Woldeteklehaymanot Dagne Kassahun, Imteyaz A Khan, Shaghayegh Khanmohammadi, Adnan Kisa, Farzad Kompani, Kewal Krishan, Iván Landires, Stephen S Lim, Preetam Bhalchandra Mahajan, Soleiman Mahjoub, Azeem Majeed, Bishnu P Marasini, Haftu Asmerom Meresa, Tomislav Mestrovic, Sonica Minhas, Awoke Misganaw, Ali H Mokdad, Lorenzo Monasta, Ghulam Mustafa, Tapas Sadasivan Nair, Sreenivas Narasimha Swamy, Hasan Nassereldine, Zuhair S Natto, Muhammad Naveed, Biswa Prakash Nayak, Jean Jacques Noubiap, Taylor Noyes, Chisom Adaobi Nri-ezedi, Vincent Ebuka Nwatah, Chimezie Igwegbe Nzoputam, Ogochukwu Janet Nzoputam, Osaretin Christabel Okonji, Adeyinka Omoniyi Onikan, Mayowa O Owolabi, Jay Patel, Siddhartha Pati, Shrikant Pawar, Ionela-Roxana Petcu, Frédéric B Piel, Ibrahim Qattea, Mehran Rahimi, Mosiur Rahman, Salman Rawaf, Elrashdy Moustafa Mohamed Redwan, Nazila Rezaei, Basema Saddik, Umar Saeed, Fatemeh Saheb Sharif-Askari, Abdallah M Samy, Austin E Schumacher, Elaheh Shaker, Adithi Shetty, Migbar Mekonnen Sibhat, Jasvinder A Singh, Muhammad Suleman, Dev Ram Sunuwar, Mindy D Szeto, Jacques JL Lukenze Tamuzi, Nathan Y Tat, Birhan Tsegaw Taye, Mohamad-Hani Temsah, Muhammad Umair, Sahel Valadan Tahbaz, Cong Wang, Nuwan Darshana Wickramasinghe, Arzu Yigit, Vahit Yiğit, Ismaeel Yunusa, Burhan Abdullah Zaman, Moein Zangiabadian, Peng Zheng, Simon I Hay, Mohsen Naghavi, Christopher J L Murray and Nicholas J Kassebaum, 15 June 2023, The Lancet Haematology.DOI: 10.1016/ S2352-3026( 23 )00118-7.
The research study was moneyed by the Bill & & Melinda Gates Foundation. The study team included researchers from IHME and GBD 2021 partners from worldwide.

Under the analysis of total death problem (including secondary conditions), sickle cell disease was the 12th leading cause of death internationally for children under the age of 5 years. Total sickle cell disease death burden was among the leading three causes of death in Portugal, Jamaica, Libya, Oman, and San Marino.
In order to overcome this data constraint, rather of using death information alone to estimate total sickle cell disease deaths, we used a mathematical algorithm that likewise takes input data from birth incidence, survival over time, and occurrence, and guarantees these procedures are internally constant,” describes Azalea Thomson, first author and IHME researcher on the Neonatal and Child Health Team. “By making use of all readily available information, we were able to reinforce our understanding of the true concern of sickle cell illness and better contextualize it along with other leading causes of death. In 2021, in kids under 5 years in sub-Saharan Africa, total sickle cell disease deaths exceeded those from malnutrition, measles, or syphilis.”