Sickle Cell – a rare cause of leg ulceration

What are Sickle Cell ulcers

Leg ulcers can occur in people already diagnosed with Sickle Cell Disease (SCD). These can be nasty ulcers and add to the pain and debilitation that having SCD brings.

What causes Sickle Cell related leg ulcers?

The cause is complex but is thought to be related to the sickled red cells causing obstruction to the small blood vessels, reducing oxygen to the skin and venous incompetence. Pain in sickle crisis also reduces mobility and may cause stiffening of the ankle, thereby reducing venous function further. The combination of all these factors can create the environment for an ulcer to develop followed by infection and inflammation.

How do Sickle Cell ulcers present?

Sickle Cell ulceration may be more common in men or those with chronic anaemia due to a severe rate of haemolysis.This condition may present in teenage teenage years but more commonly in their 20’s to 30’s. This can create a major disruption to their employment.

Sickle Cell ulcers usually present around and behind the malleolus although they can also present around the base of their toes in more extreme cases. They can be single or multiple or unfortunately bilateral. The ulceration is usually painful and adds to the general debilitation for people with Sickle Cell disease.

Sickle Cell ulcers often deteriorate during a sickle crisis due to the general reduction in oxygen to the skin. Tolerance of treatment may be difficult at these times, especially the use of compression therapy. If admitted, compression therapy may even be stopped. Neuropathic pain and hypersensitivity around the ulcer may also be extreme.

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Treatment of Sickle Cell ulcers

Treatment can be complex with the need to work alongside the other side effects of SCD that you are trying to manage. The patient will have a concoction of general medication and will be trying to minimise the impact of ulceration on their employment. Attending appointments will be difficult.

Treatment requires the following important interventions:

Medical management

Medical management by a Haematologist to their SCD is managed as well as it can be.

Management of pain

You may need to address neuropathic pain – note the sharp or burning pain will not be managed with standard pain killers.

Compression therapy

Compression therapy can be used that may be bandages, socks or wraps. Compression is an essential part of the treatment for any ulcer on the legs or feet and can get forgotten. For those with SCD, their legs are not generally swollen; they are skinny and often long. The compression bandage or garment is required to reduce venous hypertension and reduce wound exudate. You may want to start with mild compression, but if the ulcer is large do not shy away from strong compression. This will also work to reduce the local inflammation and is critical part of the treatment for this nasty condition. In severe cases of lower leg ulceration, elevation and even bed rest initially may be essential.

Review of gait and biomechanics

Seek a review of their gait and biomechanics if walking is becoming a problem. During crises, mobility will reduce and ankle may stiffen; unmanaged pain often forces people to walk on their toes thus impacting on their Achilles tendon. Exercises and/or orthotics to keep ankle function are essential. Refer to podiatry or physio if concerned; there is a high risk of foot and ankle deformity associated with ulceration and must be addressed early to prevent this.

What can reduce healing?

The site of the ulcer can make standard compression therapy less effective. If the ulcer is behind the malleolus, the compression therapy chosen will not generally apply the level or dosage of compression to the wound bed that it requires to heal – it ‘hammocks’ between the malleolus and heel. If the ulcer is failing to heal with standard and consistent compression, seek help from your local Tissue Viability Nurse. Ask about the application of special padding to the ulcer behind the ankle or the use of compression strapping.

If the patient is tall with a long leg, standard compression may not deliver the dosage of compression required to heal – extra strong compression may be required. Again, seek help from your Tissue Viability Nurse.

Further reading

Sick Cell Anaemia News (Website)

Article by Accelerate (pdf)

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