Prognosis

Sickle cell disease is very variable in its outlook. In rural parts of Africa children rarely survive beyond the first year. On the other hand in developed Western countries many patients with sickle cell anaemia survive well into adult life. The commonest cause of death is infection.

Overall, people with sickle cell disease have a reduced life expectancy. The estimated life expectancy of people with sickle cell disease in the US is more than 20 years shorter than the average for the general population (1)

The most common cause of death in the first two years of life is infection, with or without splenic sequestration, and in adults the common causes of death are cerebrovascular accidents, sepsis, acute chest syndrome and pulmonary hypertension (2)

In Africa, 50% to 90% of children born with sickle cell disease die before they reach their fifth birthday. (3)

Factors which modify the condition are elevated levels of foetal haemoglobin and the co-existence of thalassaemia. For example, in India and parts of Eastern Saudi Arabia patients are often asymptomatic with sickle cell anaemia because they produce very high levels of foetal haemoglobin.

Note

Mortality and morbidity rates in sickle cell disease (SCD) have reduced considerably since the introduction of hydroxyurea (HU) in the 1990s (4)

Reference

1. Centers for Disease Control and Prevention (CDC). Data and statistics on sickle cell disease. May 2024 [internet publication].
2. Platt OS, Brambilla DJ, Rosse WF, et al. Mortality in sickle cell disease - life expectancy and risk factors for early death. N Engl J Med. 1994 Jun 9;330(23):1639-44.
3. Grosse SD, Odame I, Atrash HK, et al. Sickle cell disease in Africa: a neglected cause of early childhood mortality. Am J Prev Med. 2011 Dec;41(6 suppl 4):S398-405.
4. Rankine-Mullings AE, Nevitt SJ. Hydroxyurea (hydroxycarbamide) for sickle cell disease. Cochrane Database Syst Rev. 2022 Sep 1;9(9):CD002202.