Severe 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.

In severe junctional EB, wounds look very wet and are often in places that are difficult to dress, like the neck, ears and the back of the head.

• Applying topical gentamycin to severe junctional EB wounds can help with the healing.
• Apply dressings to open wounds to encourage healing and to prevent wounds from sticking to clothing.
• 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.
• Keeping the skin clean will 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 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.

Severe junctional EB can be very painful and babies can have chronic, non-healing wounds in difficult to dress places.

From birth, babies with this type of EB are referred to the palliative care team. Together with the EB team they will work to relieve symptoms and ensure your baby is as comfortable as possible.

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 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 baby 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 Octenisan Wash Mitts to cleanse when the skin is sore. Use Octenilin irrigation fluid to help clean the skin. Your EB nurse can also recommend dressings or creams that can help cleanse or keep bacteria off the skin until a bath is possible.

Tips for bathing:

• Pre-cut replacement dressings before the bath so they’re ready to put on.
• Give prescribed pain relief 30 minutes before dressing change to allow the medication to work.
• Add a mild antiseptic such as Octenisan® or Dermol 500 to the water instead of commercial baby products. Your EB team can prescribe this.
• Milton or salt bathes can also be beneficial to help clean your baby’s skin. Please contact your EB team to discuss this before starting.
• 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.
• 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.

• Try to avoid commercial baby wipes as these may cause damage to the nappy area.
• Cleanse with 50/50 ointment or Emollin® emollient spray. Water can sting blisters and sores.
• Wipe poo/ wee away gently away.
• Pop blisters, leaving the top of the blister on.
• Apply a layer of barrier cream such as Proshield® Plus or Bepanthen, to any blistered areas and any areas that the nappy may rub.
• Cover any open wounds with a dressing such as Intrasite® Conformable. You may also need to line the edges of the nappy with a soft material such as Conti® SuperSoft (available on prescription) to prevent rubbing.

Blisters in the mouth and oesophagus can make feeding very painful and difficult.

Reflux is very common in babies with severe junctional EB. This means that your baby may vomit after feeding. Medications can be prescribed to help with this.

If your baby is struggling with feeding and weight gain, please speak to your EB team for further advice.

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 baby’s with severe 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 severe 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.