Severe recessive dystrophic epidermolysis bullosa (RDEB)

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.

Apply dressings to open wounds to encourage healing and to prevent wounds from sticking to clothing. Your child’s EB nurse can recommend suitable dressings and show you how to apply them to your child’s wounds.

You can use a non-sting medical adhesive remover if dressings have become stuck to the skin. This will help remove the dressing without causing pain or further damage.

If you are concerned about your child’s wounds, please contact the EB nursing team to discuss the best treatment options.

Infected wounds can be treated with creams and ointments or specialised dressings. However, if your child has a high temperature, you should contact the GP as they may need antibiotics to help treat the infection.

Please ask the GP to take a wound swab before prescribing antibiotics. Wounds should be cleaned with saline or water (not antimicrobial washes) prior to taking a wound swab.

Blisters and wounds can be painful, and each child has different pain management needs.

Please speak with your child’s EB team about how to manage your child’s pain. As well as giving you advice they can refer your child to a specialist pain team if needed.

If your baby has a lot of very fragile skin, you can nurse them on a small soft pillow/ mattress. Your EB nurse can provide this.

To lift your baby from the mattress, apply a ‘roll and lift’ technique:

1. Roll your baby onto their side.
2. Place one hand behind their head and one hand under their bottom.
3. Allow them to roll back onto your flat hands.
4. Lift.

As you become confident in lifting your baby you may find you don’t need the pillow/ mattress.

To reduce the risk of skin blistering or tearing we also recommend:

• Try to avoid picking babies and toddlers up by their armpits.
• Pad areas of the body prone to knocks and friction with dressings when you baby becomes more mobile.
• Apply dressing to open wounds to help reduce pain and potential infection.

If your child has a lot of damaged skin, we recommend delaying bathing until healing has taken place. This is because bathing can be painful and it’s difficult to protect against further skin damage when all the dressings are removed at once.

Instead, use antimicrobial wipes or wound irrigation fluids to cleanse when the skin is sore. Your EB nurse can also recommend dressings or creams that can help cleanse the skin until a bath is possible.

Tips for bathing:

• Pre-cut replacement dressings before the bath so they are ready to put on.
• If needed, give prescribed painkillers before bathing.
• Add a mild antimicrobial wash to the water instead of commercial baby products. Your GP can prescribe this.
• Use a towel to line the bath to help prevent skin damage.
• If your baby has a lot of dressings, sometimes it’s easier to bathe them with their dressings on, then change them after the bath.
• Pat skin dry with a soft towel rather than rubbing.

Initially dress your baby in a soft cotton baby grow that fastens at the front. They can wear this inside out to stop the seams from rubbing and causing blisters. Watch out for fasteners that may rub.

Older children can wear ordinary clothes if they are easy to put on and take off and don’t rub. Some children prefer clothes which don’t have to go over their head and don’t have prominent seams.

GPs are sometimes able to prescribe specialist clothes such as Skinnies® or Tubifast® that can help keep dressings in place.

• Don’t use wipes if the nappy area is red or the skin is broken.
• Cleanse with 50/50 ointment or Emollin® emollient spray. Water can sting blisters and sores.
• Pop blisters (as above).
• Apply a layer of barrier cream such to any blistered areas or to any areas that the nappy may rub.
• Cover any open wounds with cooling type gel dressings. You may also need to line the edges of the nappy with a soft material to prevent rubbing.

Blisters in the mouth can make feeding sore and difficult.

Tips for breastfeeding:

• Protect your baby’s face with a layer of emollient/barrier ointment to reduce any friction from feeding.

Tips for bottle feeding:

• A special teat like a Medela special needs feeder can be helpful, as it has a longer, slimmer teat made of ultra-soft silicone.
• You can apply a teething gel to the bottle teat or directly to the mouth before feeding to help ease pain. Gelclair® (a specialist gel) can also be used around or inside the mouth to help reduces pain.

Tips for eating:

Your child’s EB dietitian can advise on appropriate soft foods and supplements to make sure your child can meet their nutritional needs.

They can also advise on how many calories your child needs to support their growth and health.

Some children may need to have regular blood tests to make sure they aren’t lacking in any vitamins or iron.

If your child’s mouth is sore, then you can use a teething gel or numbing spray before eating. Eat softer or mushy foods when the mouth is sore, drinking through a straw can also help.

If your child starts to have difficulty swallowing, this may be indicative of stricture. Please contact your EB team for further advice.

Constipation is a common problem with all types of EB. Blistering and soreness around the bottom can make it uncomfortable for your child to poo, and pain medicines and iron supplements can also cause constipation.

If you child is constipated, please speak with your child’s EB team who will be able to advise on medicine to help them poo or make pooing more comfortable.

Overtime your child’s mouth opening may become restricted, and their tongue movements may become limited. This can make brushing teeth difficult.

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 EB 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 the 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.