DebRA. A guide to caring for the hands for people with Epidermolysis Bullosa. for professionals, parents and their carers

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1 PR21924 Debra EB Care for Hands 18/12/07 11:00 Page 1 This booklet is designed to inform people with Epidermolysis Bullosa, parents, carers and professionals about how the condition affects the hands, and how best to care for them. for professionals, parents and their carers DebRA DebRA House 13 Wellington Business Park Dukes Ride Crowthorne Berks RG45 6LS A guide to caring for the hands for people with Epidermolysis Bullosa debra@debra.org.uk telephone: Registered Charity Number: Published by DebRA ' Nicola Jessop Occupational Therapist Great Ormond Street Hospital September 2007

2 PR21924 Debra EB Care for Hands 18/12/07 11:00 Page 3 What is Epidermolysis Bullosa? Epidermolysis Bullosa is a group of rare blistering skin disorders, characterised by extreme fragility of the skin. There are three main types: 1. Dystrophic 2. Simplex 3. Junctional The information in this booklet only refers to those people who have the severe, generalised dystrophic form, which is usually recessively inherited. Recessive Dystrophic Epidermolysis Bullosa (RDEB) is characterised by areas of blistered skin, which produce joint and skin contractures. Movement is limited by both pain and contractures. This affects the ability to undertake ordinary daily activities independently. How does the hand deformity develop? Deformity of the hands occurs as one of many secondary complications of RDEB. Flexion contractures of the fingers and fusion of the web spaces between the digits develops from infancy onwards. The severity of this varies from person to person. Children with RDEB are born with the possibility of good hand movement. Blisters occur as a result of minor bumps and knocks consistent with normal everyday activity. As the skin heals it tightens and does not allow the joints to move through the full range of motion. Gradually contractures develop, limiting movement and reducing function. Typically, contractures start to develop around the age of 2 years, with the little and ring fingers often first to be affected. The deformity follows the habitual position the hand adopts for grip and rest. At this mild stage, hand function remains reasonable. Later, more digits may become involved. The fingers fuse together, flex down onto the palm and the thumb web space becomes tight. Hand function is more limited, with the loss of fine manipulation and the ability to hold large objects. Reach may be affected by contracture development around the shoulders. Supination (the ability to turn the palm of the hand upwards) may also be limited. Pincer grip is usually retained, so that writing and handling small objects is possible. 2

3 PR21924 Debra EB Care for Hands 18/12/07 11:00 Page 4 What can be done? Treatment to try to delay and minimise these deforming processes aims to: - Protect the skin - Provide good skin care - Passively stretch the joints of the hands 1. Protection Protecting the skin from daily trauma minimises the development of blisters, which lead to contractures. There are numerous methods and dressings used to protect the skin of the hands. The EB Nurse Specialist will advise on what dressings to use and how to use them. During toddler years this is especially important as young children often fall and injure their hands. - One frequently used method is to pad the palm and the back of the hand with a suitable dressing and secure it with a retention bandage such as tubifast. This leaves the digits free for function. - Another method is to wrap each digit and the palm with a soft, thin cohesive bandage so that all of the hand is protected while digital movement is preserved. The EB Nurse Specialist or a Hand Therapist can teach carers this technique. - Sometimes lycra gloves are used to protects the hands. These should be close fitting, made to measure and have open fingertips so that they do not impede hand function. 3

4 PR21924 Debra EB Care for Hands 18/12/07 11:00 Page 5 2. Skin Care Good skin care reduces contracture development, which results from dry skin, blisters, wounds and infections: - The skin needs to be moisturised regularly using an absorbent moisturiser. This helps prevent the skin becoming dry and cracked and resistant to stretching. - Skin loss through trauma (degloving injuries) needs appropriate dressings to enhance healing. - Skin infections are treated as advised by your EB Team, in order to heal the wounds as quickly as possible. 3. Passive Stretches Stretching the hand joints in an opposite direction to the resting position of the hand is an important method of maintaining joint range of motion and delaying contracture development. This can be done in a number of ways: - During the day, when time permits, hold the fingers out straight into extension and the thumb out wide as if to pick up a large object. This is important before digital contractures have developed or are very mild. - Using dressings to separate the web spaces during the day helps delay the development of web space contracture. This can be done using the wrapping technique (described previously) or by using an appropriate dressing between the fingers. - Using soft, putty splints to separate the web spaces and straighten the digits is an effective way of achieving a prolonged passive stretch. These are worn at night and are secured with a bandage. Putty splints need to be moulded directly on the hand and changed regularly due to growth and/or change in the level of contractures. - Thermoplastic splints with putty inserts are used as an alternative to putty splints. These straighten and separate the digits and also support the wrist in a good position. They are made of a hard plastic and provide a strong corrective force. They are unsuitable for some people who use them to scratch itchy skin at night, as they can cause injury. They need to be moulded directly on the hand and changed regularly due to growth and/or change in the level of contractures. 4

5 PR21924 Debra EB Care for Hands 18/12/07 11:01 Page 6 What about hand surgery? The preventative measures described are effective in delaying the onset and may minimise the severity of the contractures and the impact this has on hand function. Unfortunately, hand contractures are an inevitable part of the condition and surgery may be considered when there is sufficient deformity present to impede function. It can be undertaken from 2 years old through to adulthood. However, it needs careful consideration for many reasons: - Hand surgery cannot permanently correct deformities, since the underlying skin condition remains. The effects of surgery last on average between 2 and 5 years with meticulous use of night splints and daytime wrapping or web space separation. Without splints and dressings contractures rapidly return. - The person must be generally well, and relatively free from skin infections to undergo surgery. Skin infections must be treated with antibiotics prior to surgery, and frequent bathing encourages good skin hygiene. - There must be sufficient donor skin for the skin grafts. This is usually taken from the thigh area. - The operated hand will be out of action on average for four to six weeks, and there will be a number of dressing changes required, some of which will require a general anaesthetic (GA). Pain relief will be needed immediately post surgery, and for dressing changes which do not require a GA. - Hand surgery for EB is only undertaken at specialist centres, and this may involve a considerable amount of travelling, over a number of weeks. 5

6 PR21924 Debra EB Care for Hands 18/12/07 11:01 Page 7 What does hand surgery usually involve? - Releasing the web spaces contractures. - Releasing flexion contractures with skin grafts used to cover the area. If the flexion contractures are severe, a thin wire may be inserted through the fingers to maintain the new position of extension. These are kept in for approximately one to two weeks and removed under GA. - Releasing the thumb contracture. This may require a skin graft, but not always. - A dressing and plaster cast is then applied to the hand. A dressing is applied to the donor site of the skin graft. - A dressing change under GA is undertaken at two weeks post surgery. - The dressings are subsequently changed every week, until the hand and donor site are healed. As the skin heals a GA is not needed. - A splint is made when the skin is healed. It is used every night to position the hand so that the fingers are separated and the digits are straight. 6

7 PR21924 Debra EB Care for Hands 18/12/07 11:01 Page 8 What does the hand look like after surgery? The effects of hand surgery can be dramatic and improve both the function and the appearance of the hand. BEFORE HAND SURGERY AFTER HAND SURGERY 7