Last week saw the end of our short series of articles on biofilms. This week we begin an entirely new topic, as we start to look at the relatively uncommon but extremely debilitating issue of sickle cell ulcers.

Sickle cell ulcers are a common manifestation of sickle cell disease, which is itself an inherited, genetic disorder of the oxygen carrying hemoglobin in red blood cells. Sickle cell disease is particularly common among people whose ancestors come from sub-Saharan Africa, India, Saudi Arabia and Mediterranean countries. Migration from these countries has raised the frequency of the gene in the American continent.(1)  A study of more than 3.3 million infant screenings in California between 1990 and 1996 showed a prevalence of sickle cell disease of 1 per 396 among black people; 1 per 36,000 among Hispanics; and 1 per 123,000 among white people.(2)

The most common manifestation of sickle cell disease is vaso-occulsive crisis, in which an individual experiences a sudden onset of pain which lasts for several hours to several days, and which can end as quickly as it began. A number of other debilitating symptoms are associated with sickle cell disease, but it is the leg ulcer which is of particular concern to wound care specialists. Sickle cell leg ulcers result from minor injury to the area around the malleoli. Because of relatively poor circulation, compounded by sickling and microinfarcts, healing is delayed and infection becomes established.(3) The prevalence of leg ulceration in sickle cell disease varies according to genotype and age of the patient, however the resulting pain in afflicted patients can be severe or even excruciating. In most patients, opioid analgesics are needed to achieve some pain relief.

Next week we will look at the underlying pathophysiology of sickle cell ulcers, and the following week will be devoted to strategies to manage the condition.

If you are interested in studying sickle cell ulcers and other unusual wound presentations in greater depth, you  may wish to consider pursuing some form of ongoing professional development. A certification in wound care provides the opportunity to study certain issues in more detail, while also improving your own career prospects. For further information, please visit www.woundeducators.com.

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References

1. World Health Organization, 59th World Health Assembly. Sickle-cell anaemia – report by the Secretariat. 24 April 2006 (Available from www.who.int/gb/ebwha/pdf_files/WHA59/A59_9-en.pdf).

2. Lorey FW, Arnopp J, Cunningham GC. Distribution of hemoglobinopathy variants by ethnicity in a multiethnic state. Genet Epidemiol. 1996; 13:510-512.

3. Arnold JL. Sickle Cell Anemia Clinical Presentation. Medscape. 2011 (Available from emedicine.medscape.com/article/205926-overview).