Sickle-cell disease natural history, complications, and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. ; Associate Editor(s)-in-Chief: Aarti Narayan, M.B.B.S , Shyam Patel 
The natural history of sickle cell disease is characterized by various vascular phenomenon that begin at variable ages, and these vascular complications include, but are not limited to, the extremities and the brain. The complications of sickle cell disease involve various tissues and organs, including the chest, bones, and GI tract. The prognosis of sickle cell disease is variable, with a median survival of age 50 years. Of note, the prognosis of patients with malaria who have sickle cell disease is better than patients without sickle cell, since there a protective advantage.
The natural history of sickle cell disease involves manifestations that begin shortly after birth. If left untreated, the following is a general timeline of the effects of sickle-cell disease:
- By 2 months of life, infants can be affected by dactylitis (vaso-occlusive episodes in the hands and feet), or other complications. Dactylitis episodes typically resolve after 5-7 days with conservative measures. The reason is that bone marrow is present in the small extremity bones during the early weeks of life.
- By 3 months of life, splenic sequestration can occur. This is the age at which routine spleen size examination is important. If a person has 2 episodes of splenic sequestration, splenectomy should be considered. Symptoms include tachycardia, tachypnea, abdominal pain, and abdominal fullness, which are reflective of trapping of sickled red blood cells in splenic sinuses.
- By 6-12 months of life, death has been observed but can generally occur at any age after 1 year. One of the causes of death is acute chest syndrome. Other reasons for death in sickle cell anemia include sepsis and aplastic crises. Splenic dysfunction can occur by the first year of life.
- By 24 months of life, stroke can occur from vaso-occlusive episodes in the brain. Within 3 years of the first stroke, recurrent strokes are known to occur, which can pose significant morbidity.
- By 5 years of age, dactylitis usually does not occur, since the bone marrow is no longer present in small bones of the extremities by age 5.
- By late childhood, bone pain crises and avascular necrosis of the femoral head can occur. If sickled red blood cells become lodged in the penis, priapism can occur. Chronic leg ulceration is also a common problem in late adolescence.
- By adult age, patients can develop subarachnoid hemorrhage, berry aneurysms, and direct intracerebral bleeding.
Sickle-cell anemia can lead to various complications, including:
- Vaso-occlusive crisis
- Acute chest syndrome
- Overwhelming post-(auto)splenectomy infection
- Cholelithiasis and cholecystitis (gallstones)
- Decreased immune reactions due to hyposplenism (malfunctioning of the spleen)
- Priapism and infarction of the penis
- Opioid tolerance
- Acute papillary necrosis in the kidneys
- Leg ulcers
- Orbital infarct
- Central retinal artery occlusion
- Vitreous hemorrhage
- Retinal detachment
- During pregnancy:
- Pulmonary hypertension
- Left-sided heart disease
- Neurocognitive impairment (unrelated to vaso-occlusion)
The prognosis of patients varies based on degree of sickling and vaso-occlusive crises in vital organs. Sickle-cell heterozygosity has a protective advantage in infection with Plasmodium falciparum, one of the causative agents of malaria.
The median survival of patients with sickle cell disease in the USA is 45-55 years. The prognosis of sickle cell disease is better in geographic areas where there is a lower burden of infections, such as malaria or other blood-borne pathogens.
- ↑ 1.0 1.1 1.2 1.3 Serjeant GR (2013). "The natural history of sickle cell disease". Cold Spring Harb Perspect Med. 3 (10): a011783. doi:10.1101/cshperspect.a011783. PMC 3784812. PMID 23813607.
- ↑ 2.0 2.1 2.2 2.3 Ballas SK, Kesen MR, Goldberg MF, Lutty GA, Dampier C, Osunkwo I; et al. (2012). "Beyond the definitions of the phenotypic complications of sickle cell disease: an update on management". ScientificWorldJournal. 2012: 949535. doi:10.1100/2012/949535. PMC 3415156. PMID 22924029.
- ↑ 3.0 3.1 3.2 Kato GJ, Hebbel RP, Steinberg MH, Gladwin MT (2009). "Vasculopathy in sickle cell disease: Biology, pathophysiology, genetics, translational medicine, and new research directions". Am J Hematol. 84 (9): 618–25. doi:10.1002/ajh.21475. PMC 3209715. PMID 19610078.
- ↑ Kato GJ (2012). "Priapism in sickle-cell disease: a hematologist's perspective". J Sex Med. 9 (1): 70–8. doi:10.1111/j.1743-6109.2011.02287.x. PMC 3253142. PMID 21554552.
- ↑ Hasan SP, Hashmi S, Alhassen M, Lawson W, Castro O (2003). "Depression in sickle cell disease". J Natl Med Assoc. 95 (7): 533–7. PMC 2594635. PMID 12911250.
- ↑ Colah RB, Mukherjee MB, Martin S, Ghosh K (2015). "Sickle cell disease in tribal populations in India". Indian J Med Res. 141 (5): 509–15. PMC 4510747. PMID 26139766.