John Libbey Eurotext

Médecine thérapeutique / Pédiatrie

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Sickle cell disease, a chronic adult illness Volume 20, issue 4, Octobre-Novembre-Décembre 2017

Author
Hôpital Tenon, Centre de la drépanocytose, 4 rue de la Chine, 75020 Paris
* Tirés à part
  • Key words: sickle cell disease, adult, prevention, hydroxyurea
  • DOI : 10.1684/mtp.2018.0656
  • Page(s) : 249-53
  • Published in: 2017

Survival of sickle cell disease has improved dramatically. Sickle cell disease is now a chronic adult illness characterized by progressive multiorgan failure, particularly involving the eye, kidney and heart. It remains severe in its repercussions on everyday life, especially due to unpredictable, recurrent, and intense pain. Providing health care to these patients becomes a public health priority because of the increasing number and ageing of patients. Complications of sickle cell disease include acute manifestations such as vaso-oclusive crises, acute chest syndrome, priapism, infections, and chronic complications which lead to severe functional sequela such as renal, pulmonary, bone, retinal damage. The prevention of these complications becomes the prevailing therapeutic approach in sickle cell adult patients. The best treatment of severe acute complications that sometimes threaten life is the prevention. Hydroxyurea is the only treatment directed at the prevention of the complications in adult patients. Concerns about the effects on male fertility should not be a barrier for its prescription because its efficiency has been proven, even for survival.