Recessive dystrophic epidermolysis bullosa (RDEB) intermediate

• Use non-stick dressings and gentle bandaging. Apply dressings to open wounds to encourage healing and to prevent wounds from sticking to clothing.
• It’s common for the skin to blister on bony bits of the body, such as elbows and knees. You can use non-stick dressings to pad and protect these areas to help prevent damage to the skin.
• IfYou can use a non-sting medical adhesive remover if dressings become stuck to the skin. This will help remove the dressing without causing pain or further damage.
• Keep the skin clean to prevent infection. Infected wounds can be treated with topical wound gels, creams, and ointments. However, if your child has a fever, you should contact the GP- they may need antibiotics to help treat the infection. Please ask the GP to take a wound swab before prescribing antibiotics.
• If you are concerned about your child’s wounds, please contact the EB nursing team to discuss the best treatment options.

Blisters and wounds can itch and be painful.

Over the counter medications such as paracetamol and ibuprofen can help. Please speak with your child’s EB team about how to manage your child’s pain if you have any concerns.

It can also help to moisturize the skin daily to reduce dryness.

It’s important to pop blisters to stop them from getting bigger and becoming painful or infected.

• Use a piece of soft gauze to gently compress the blister from the side to increase tension.
• Use a small hypodermic sterile needle (or small clean sharp scissors) to pierce the blister.
• Slide the needle through the blister to create an entry and exit point. If the blister is large, you may need to do this more the once.
• Withdraw the needle and gently press the blister with the gauze to remove the fluid.
• Dispose of the needle into a sharps bin.
• You don’t need to use a dressing if the top of the blister is still in place.
• Use a dressing is there is skin loss or if the area looks particularly sore.

If blisters are left this can result in further damage or wounds to the surrounding skin.

We recommend bathing or showering regularly to cleanse the skin. Commercial shower gels and washes are suitable for most children with RDEB Intermediate. If your child has recurrent skin infections, please contact your EB team who can recommend a mild antiseptic wash for the skin.

• Avoid using scented baby wipes if the nappy area is red or broken.
• Cleanse with 50/50 ointment or Emollin® emollient spray.
• Apply a layer of barrier cream, to any blistered areas or to any areas that the nappy may rub.

Constipation is a frequent problem with all types of EB. Blistering and soreness around the bottom can make it uncomfortable for your child to open their bowels. Other factors such as pain medicines and iron supplements can cause constipation too.

If worried about your child’s constipation, speak with the EB team who will be able to advise.

Children with RDEB Intermediate can sometimes develop blisters/small ulcers in the mouth. This can sometimes make cleaning the teeth painful.

To help we suggest:

• Brush teeth with a soft toothbrush.
• Try to avoid very hot or very cold food and drinks.
• Have regular check-ups with the dentist. They may also prescribe fluoride supplements.

Dry eyes caused by a reduced tear film can sometimes cause blistering on the surface of the eyes which can be painful.

An ophthalmologist (eye specialist) can prescribe eye drops or ointment to help keep the eyes moist and reduce the chance of blistering.

If the eye becomes scratched or blistered:

• Make an appointment to see the ophthalmologist.
• Use antibiotic eye drops rather than regular eye drops or ointment.
• Give pain-relief if needed.

Your child may find it more comfortable to keep their eye closed and avoid bright lights whilst the eye is healing.