Sickle-cell anaemia - Sickle-cell disease (Paediatrics)

Medium urgency
-Very severe

Alteration in human blood that causes the red blood cell to deform and acquire a crescent-shaped appearance, which hinders blood circulation and causes microinfarcts, haemolysis and anaemia in the patient.

It is a disease of genetic origin.

Symptoms usually do not occur until after the age of 4 months. Almost all people with sickle cell disease have painful episodes, called crises, which can last from hours to days. These crises can cause pain in the lower back, legs, joints and chest. As the picture becomes more severe, symptoms may include fatigue, abdominal pain, pallor, rapid heart rate, shortness of breath, yellowing of the eyes and skin (jaundice), painful and prolonged erection (priapism), poor vision or blindness, impaired thinking or confusion caused by small strokes, and leg ulcers.

Diagnosis is made through medical history, physical examination and complete blood work.

The goal of treatment is to manage and control the symptoms and reduce the number of attacks. Treatment for a sickle cell crisis includes blood transfusions (these may be given regularly to prevent strokes), painkillers and plenty of fluids. Invasive treatments will be required if complications arise.

Bibliographic references
  1. Elliott P Vichinsky, MD. Overview of the clinical manifestations of sickle cell disease. Uptodate. Dec 14, 2016.
  2. Treadwell M, Johnson S, Sisler I, et al. Self-efficacy and readiness for transition from pediatric to adult care in sickle cell disease. Int J Adolesc Med Health 2016; 28:381.
  3. Treadwell M, Telfair J, Gibson RW, et al. Transition from pediatric to adult care in sickle cell disease: establishing evidence-based practice and directions for research. Am J Hematol 2011; 86:116.
  4. Rees DC, Williams TN, Gladwin MT. Sickle-cell disease. Lancet 2010; 376:2018.
  5. Edward J. Benz, Jr. Hemoglobinopatías. Harrison. Principios de Medicina Interna. Volumen 2. 19º Edición. 631:636.
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Symptoms

    Tiredness / fatigue


    Very severe pallor


    Bone pain


    Neurological symptoms: impaired speech, physical strength or feeling


    Sharp stabbing chest pain

Pre-hospital care recommendations

Maintain hydration of 2 litres per day.
Consume over-the-counter pain relievers or anti-inflammatories.