Intermediate junctional epidermolysis bullosa (EB)

It’s important to pop blisters to stop them from getting bigger.

• 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.

• Use non-stick dressings and gentle bandaging. Apply dressings to open wounds to encourage healing and to prevent wounds from sticking to clothing.
• You can also use padded, non-stick dressings on areas prone to knocks such as elbows, hands and knees. This can help prevent damage to the skin likely to be exposed to damage,.
• Use a non-sting medical adhesive spray to remove dressings if they become stuck to the skin. This will help lift any dressings without causing pain or further damage.
• Keep the skin clean to help prevent infections.
• Infected wounds can be treated with topical wound gels, creams, and ointments. However, if your child has a fever, you should contact your GP- they may need antibiotics to help treat an infection. Please ask your 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, and each child has different pain management needs.

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

· Use prescribed creams or oral medications as needed.

· Moisturise the skin daily to reduce dryness.

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

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 skin tears 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 your 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 at birth, 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 dressings are removed at once.

When you and your child are ready, we recommend bathing or showering to regularly cleanse the skin and to help prevent infections.

Tips for skin cleansing:

• Pre-cut replacement dressings before the bath so they’re ready to put on.
• If needed, give prescribed painkillers before bathing.
• Use a towelling baby seat rather than a plastic one if it’s difficult to hold your baby in the bath.
• 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.
• Use an antimicrobial wash to help prevent skin infections. These should be rotated on a regular basis to prevent antimicrobial resistance.
• Milton baths, one to two times weekly can also be beneficial to help clean your baby’s skin.
• 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.

Try to dress your baby in softer, seamless clothes made of materials like cotton or bamboo.

Your child’s GP can also prescribe specialist clothes that can help keep dressings in place.

• Avoid using scented baby wipes if the nappy area is red or broken down.
• Cleanse with 50/50 ointment or Emollin emollient spray.
• Pop blisters (as above).
• Apply a layer of barrier cream, to any blistered areas or to any areas that the nappy may rub.
• Cover any open wounds with a dressing. You may also need to line the edges of the nappy with a soft material to prevent rubbing.

Tips for breastfeeding:

• Protect your baby’s face with a layer of emollient to reduce any friction from feeding.
• You can also apply emollient to the area around your nipple for further protection.

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.

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

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.

Missing and thickened nails is very common in babies with intermediate junctional EB.

• Keep nails short and smooth by trimming nails regularly with gentle rounded scissors or baby nail clippers.
• You can also file your baby’s nails to prevent them from scratching and causing blisters on other areas of skin.
• Wearing soft seamless mittens can help prevent further damage on the body from sharp nails.
• Thickened nails and inflamed nail beds can cause infection. Look out for signs of redness, swelling, pus or increased pain around the nail.

It is common in intermediate junctional EB for nails to fall off. If this happens keep the area clean. Cover the area with a small dressing or a wound gel and a mitten if you can.

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.

Please speak to your child’s EB team if you think your child has dry or sore eyes so they can advise you further.