Observation of Causes of Death from Sickle Cell Anemia in Niger

Case Report

Ann Hematol Oncol. 2021; 8(2): 1329.

Observation of Causes of Death from Sickle Cell Anemia in Niger

Djibrilla Almoustapha A1,3*, Maman Brah M1, Chefou M1, Ousseni M3, Ali B3, Lawali Issa H1, Mahamadou S3, Bade MR1, Issoufou F3, Brah S2, Malam-Abdou B1,3 and Adehossi EO2,3

¹Department of Onco-Hematology, The Niamey National Hospital, Niger

²Department of Internal medicine, The Niamey General Reference Hospital, Niger

³Niamey National Reference Center for Sickle Cell Disease, Niger

*Corresponding author: Djibrilla Almoustapha Amadou, Onco-Hematology Department of the Niamey National Hospital, Bp: 223, Niger

Received: January 18, 2021; Accepted: February 22, 2021; Published: March 01, 2021


Purpose: Improve the management of Major Sickle Cell Syndromes (SDM) by studying the circumstances in which patients affected by this disease die in Niger.

Methods: This was a retrospective study of descriptive and analytical type in sickle cell patients followed at the National Sickle Cell Reference Center (CNRD) in Niamey with a follow-up file, who died during our study period, which was 9 years (January 1, 2010 to December 31, 2018).

Results: During the study period, 6,465 sickle cell patients were followed up at the CNRD, 249 deaths were recorded, representing a frequency of 3.8%. There is a male predominance with a sex ratio of 1:3. The 0-15 age group was the most represented with 34.5% with an average age of 9.25 years. 34.1% of the patients came from a consanguineous marriage. Of the 249 deaths, 94% (n=234) were SS, 5.6% (n=14) were SC and 0.4% (n=1) was S/β. The majority of patients died at the time of registration, i.e. 58.25% (n=145 (SS=137. SC=8.)). 90.4% had died in a health-care facility. The main cause of death was anemia in 73.1% of cases.

Conclusion: The management of sickle cell disease requires adequate preventive action to reduce the rate of early mortality.

Keywords: Death- Major sickle cell syndrome; CNRD; Niamey; Niger


SDM: Major Sickel Cell Disease Syndrome; CNRD: National Reference Centre for Sickle Cell Disease; PRN: President of the Republique of Niger; ALDN: Association for the Fight Against Sickle Cell Disease in Niger; CVO: Vasooclusive Crisis; STA: Acute Thoracic Syndrome; AVP: Public Road Accident


Sickle cell disease is the most common genetic disease in the world, characterized by the substitution of glutamic acid by valine in position 6 on the beta chain of globin, which generates multiple clinical manifestations [1]. It is recognized as a public health problem and affects around 50 million people worldwide [2]. Its evolution is interspersed with acute and chronic complications. In recent years there has been an improvement in the care of sickle cell anemia, which considerably reduces the mortality and morbidity rate of this disease. Thus, the precise analysis of the causes and co-morbidities associated with death in sickle cell patients is important for each center in order to recognize risk situations and improve hospital care, hence the goal to carry out this study at the National Reference Center for Sickle Cell Disease (CNRD) in Niamey.

Purpose: Improve the management of sickle cell anemia by studying the circumstances surrounding the death by this disease in patients who’d been followed up at the National Reference Center for Sickle Cell Anemia (CNRD) in Niamey.


Study framework

The study took place at the National Reference Center for Sickle Cell Disease (CNRD) in Niamey. The CNRD was created by decision N°011/PRN of August 12, 2009, thanks to a partnership and a financing agreement 2007-2008 between the Republic of Niger through the Ministry of Public Health (MPH), the Principality of Monaco, AMADE Mondiale and the Association for the Fight against Sickle Cell Disease in Niger (ALDN). It is a Public Scientific and Technical establishment.

Type of study and sample

It was a retrospective descriptive and analytical study conducted at the National Reference Center for Sickle Cell Disease (CNRD) in Niamey, running from January 1, 2010 to December 31, 2018, i.e. a period of 9 years. The records of patients who died during the study period served as a source of data collection. The information collected was: age, sex, socio-demographic data, acute and chronic complications, the reasons and the conditions of death. After collecting the data on a pre-established survey form, they were analyzed by SPSS 20.0 software. A value was considered statistically significant if the P-value is less than 0.05.


Sociodemographic characteristics

Frequency: During the 9-year study period, two hundred and forty-nine (249) patients died out of six thousand four hundred sixty five (6,465) patients followed, representing a frequency of 3.8%. The majority of deaths occurred in 2015, i.e. 17% (n=43).

Age and gender: The CNRD are a center for the care of both children and adults with sickle cell anemia, so the majority of patients were under 15 years of age, or 38.5% (n=96). The average age was 9.25 years with the extremes of age ranging between 3 months and 49 years. Our patients were divided into 106 women or 42.6% and 143 men or 57.4%. A sex ratio of 1:3.

Level of education and consanguinity: Most of our patients were out of school, 33.3% of cases. 34.1% of the patients were the result of a consanguineous marriage.

Clinical characteristics (Table 1): The causes of admission before death were as follows, 58.2% (n=145) died when the file was opened, i.e. at the inclusion stage, followed by vaso-occlusive crisis in 32, 9% (n=82). Fever was identified in 2.4% (n=6) of the cases. Hepatic complications dominated chronic complications in 4.8% of cases, followed by cardiac complications and necrosis of the femoral heads in 3.2% of cases respectively.

Citation: Djibrilla Almoustapha A, Maman Brah M, Chefou M, Ousseni M, Ali B, Lawali Issa H, et al. Observation of Causes of Death from Sickle Cell Anemia in Niger. Ann Hematol Oncol. 2021; 8(2): 1329.