Leg ulcers in sickle cell patients: management challenges

Abstract

Sickle cell disease is an autosomal recessive hemoglobinopathy caused by an amino acid substitution from glutamic acid to valine in the beta hemoglobin chain. One of the common symptoms occurring in sickle cell patients are leg ulcers, which are notoriously painful, difficult to treat, and frequently recurrent. These ulcers pose a therapeutic challenge with multiple modalities proposed for treatment, but with scarce evidence of efficacy of any single modality. Ulcer prevention, rigorous wound care, pain control, and surgery are the current mainstays of sickle cell leg ulcer treatment.

Estimated costs in treating sickle cell disease leg ulcer

“…It is important to maintain levels between 8.0 g/dl and 10 g/ dl to promote healing, with the latter being ideal (15) . Blood transfusion seems to benefit some patients with ulcers who are resistant to more conservative measures based on the premise that higher hemoglobin levels and subsequent better oxygenation provide better healing (7) .…”

“…Patients may be debilitated and incapacitated due to the presence of an ulcer, which has the characteristic of being painful, difficult to heal, with an evolution lasting for months and even years (7) . Although 75% to 80% of patients can heal, ulcers can persist for more than 20 years in some patients and/or never heal.…”

“…They are resistant to therapy, tend to be recurrent and cause physical, psychological and social disability. The pain may be severe, excruciating, penetrating, acute and burning (7) . Health care costs generated in treating skin lesions are directly related to the dressing type used, the usage period, the exchange pattern, the wound healing time, the use of resources and adjuvant therapies, and the type of professional responsible for the treatment (9) .…”

Enhancing the care of patients with sickle cell disease

“…The genetic mutation that causes sickle cell disease is the substitution of one amino acid, glutamic acid, for another, valine, in the beta haemoglobin chain (El Khatib and Hayek 2016). This change causes the production of sickle haemoglobin (haemoglobin S or HbS), which can aggregate in the blood vessels.…”

“…Leg ulcers are common in patients with sickle cell disease, and can be extremely painful, challenging to treat and often recurrent (El Khatib and Hayek 2016). Management of sickle cell leg ulcers includes wound care, debridement and skin grafts (El Khatib and Hayek 2016). Sickle Cell Society (2018) guidance recommends preventing leg ulcers through a well-balanced diet, avoiding injury and using moisturisers.…”