Editorial

90 www.thelancet.com Vol 381 January 12, 2013

Boosting services for people with sickle-cell diseaseThe Igbo people of Nigeria called them Ogbanje—malevolent children destined to be lazy and die and be reborn repeatedly. Particular families were plagued by such children who usually died before their 10th birthday. Belief in Ogbanje is centuries old, but a little more than 100 years ago, a less ethereal explanation for the Ogbanje was found. Walter Clement Noel was of African descent and living in America. He had anaemia and chest pains, and in 1910 doctors Ernest Irons and James Herrick looked at his blood under a microscope. What they saw—sickle-shaped red blood cells—was published as the fi rst report of sickle-cell disease.

Sickle-cell disease is an autosomal recessive disorder. There is selection pressure for the persistence of the gene in African populations because heterozygotes, who are generally unaff ected by clinical symptoms, have protection from malaria. Homozygotes have sickle-cell anaemia and repeated crises, which eventually result in death if left untreated. When red blood cells are exposed to repeated low-oxygen conditions—which can be triggered

by infection, dehydration, or some drugs—aff ected haemoglobin polymerises. The resultant infl exible cells become trapped in the microvasculature and cause episodes of ischaemia. Such episodes cause excruciating pain. Other manifestations include haemolytic anaemia and red-cell sequestration. Without scientifi c justifi cation, evoking spirits seemed a reasonable explanation to Africans whose children had these recurring symptoms.

The only curative treatment is stem-cell transplantation, but other treatments, such as hydroxyurea, preventive measures such as treatment of infection, and supportive measures such as oxygen therapy and pain control, can improve life expectancy. Studies defi ning disease burden, as published and discussed in a Comment in this week’s issue, are important for distribution of these treatments.

It is essential that there is global investment in services for people with sickle-cell disease. Services should include aff ordable treatments, genetic testing, and diagnostic and genetic counselling, such that in the future the ghosts of the Ogbanje are exorcised for good. The Lancet

For Sickle-Cell Anaemia: An explanation for the ancient

myth of reincarnation in Nigeria see Lancet 1983;

322: 503–05

For more on sickle-cell disease see Lancet 2010; 376: 2018–31

For the UN commissioned report Preliminary Statistical Analysis of Documentation of Killings in Syria

see http://www.ohchr.org/EN/NewsEvents/Pages/Media.

aspx?IsMediaPage=true&LangID=E

For Syrian population statistics from the UN High

Commissioner for Refugees see http://data.unhcr.org/

syrianrefugees/regional.php

Reporting deaths in Syria: room for reviewOn Jan 2, the Offi ce of the UN High Commissioner for Human Rights issued a press release with the shocking fi gure of 59 648 individuals killed during the Syrian confl ict between March, 2011, and November, 2012. The UN commissioned report, Preliminary Statistical Analysis of Documentation of Killings in Syria, looked at 147 349 fully identifi able death records—rather than mortality estimates—and integrated data from seven human rights and Syrian Government datasets to arrive at the fi nal number. With more than 2 million people internally displaced, and 481 592 documented refugees in neighbouring countries requiring urgent humanitarian assistance, the plight of Syrians has long passed most defi nitions of an unacceptable threshold of death and suff ering.

The UN hopes that the new study will form a basis on which to build future investigations and accountability in Syria. However, doubts will likely be cast on the validity of the fi ndings. There is uncertainty around the causes of death, reported missing data, and duplication. 16·4% of records did not report sex. The

onus on the investigators was to try to reduce the risk of misunderstanding, because of the political nature of discussing death records in confl ict. Such fi gures inform the international response, so the presentation of methods and results in a transparent format is critical—publication in a peer-reviewed journal would have been the best forum for proper debate.

The Lancet’s experience of publishing studies of deaths in confl ict revealed a deeply controversial fi eld. Mortality estimation methods are complex and usually engender disagreement. Such studies are extremely diffi cult to undertake, because of the lack of accurate death records and the personal risk to researchers. But studies to count the dead deserve our attention—they are an important part of epidemiological research, are a historical testimony to victims, and can guide fi nancial and emergency aid for the living. On Jan 30, the UN will host a donor pledging conference for Syria. Unhindered access for medical and humanitarian assistance with the rigorous collection of peer-reviewed death data must be prioritised to deliver justice for the Syrian people. The Lancet

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See Comment page 98

See Articles page 142

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