Sickle cell and leg ulcers

Overview – what are sickle cell leg ulcers?

Leg ulcers can occur in people already diagnosed with sickle cell disease. These can be nasty ulcers and add to the pain and debilitation that having sickle cell disease brings.

Causes – 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 within sickle crisis can also reduce 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.

Why can sickle cell disease cause leg ulcers?

Sickle cell related ulcers may be more common in men or those with chronic anaemia due to a severe rate of haemolysis. This condition may present in 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 show around and behind the ankle, they can also present around the base of the toes in more extreme cases. They can be single ulcers or multiple and can occur in both legs at once. 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. Neuropathic pain and hypersensitivity around the ulcer may also be extreme.

Treatment – how to treat and manage sickle cell leg ulcers

Treatment can be trickier simply because you will also have the other side effects of sickle cell disease that you are trying to manage. It’s likely you will also have a concoction of general medication and you may also be trying to minimise the impact of this ulceration on your employment.

Please talk to your healthcare professional about these complexities so that they can be taken account of within the management plan.

Treatment requires the following important interventions:

Medical management of sickle cell leg ulcers

Medical management with a haematologist to ensure your sickle cell disease is managed as well as it can be.

Pain management for sickle cell leg ulcers

You may need medication for nerve related pain (neuropathic pain); this can result in a sharp or burning pain and may not be managed with standard pain killers. Make sure you describe your pain to your doctor.

Compression therapy of sickle cell leg ulcers

Compression therapy may be bandages, socks or wraps. Compression is an essential part of the treatment if you have ulcers on your legs or feet and can get forgotten. Medical management may not be enough on its own to control this condition. Even if your leg is not very swollen the compression bandage or sock will reduce the level of fluid leaking from the ulcer(s). You may want to start with mild compression, but if you have a large wet ulcer, try to ensure you get a stronger level of compression. This will also work to reduce the local inflammation and is critical part of the treatment for this condition. In severe cases of lower leg ulceration, elevation and even bed rest initially may be essential.

Review of your walking and footwear

Seek a review of your walking and footwear if you believe this is becoming a problem. During crises your mobility will reduce and your ankle may stiffen. It is essential to keep stretching your ankles and that you try not to ‘walk on your toes’. There is a high risk of foot and ankle deformity associated with sickle cell ulceration; this must be addressed early to prevent this. You may need special exercises and orthotics for your shoe.

What can reduce healing of sickle cell and leg ulcers?

The site of the ulcer can make the standard compression therapy less effective. If your ulcer is behind the ankle, the compression bandage or sock will not generally apply the level or dosage of compression to the wound bed that it requires to heal. If your ulcer is failing to heal with 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 you’re quite tall and you have a long leg, then you may also need extra strong compression to heal. Again, seek help from your local Tissue Viability Nurse.

Harm – the risk factors

Sickle cell ulceration on the legs needs compression therapy alongside the medical management. The powerful anti-inflammatory effects of compression therapy can get missed whilst the doctors focus on establishing the correct medication and pain relief. While sickle cell ulcers are not caused by venous hypertension, it is clear that the ankle mobility is reduced thus impacting on venous return; any wound on the leg heals faster with compression therapy.

Resources

Sick Cell Anaemia News (Website)