Kindler epidermolysis bullosa (EB)

• 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 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.
• Keep 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, and each child has different pain management needs.

Over the counter medications such as paracetamol and ibuprofen can help a child with kindler EB. 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.

It is common for the skin to blister at parts of the body which are bony or rub. You can use non-stick dressings to pad and protect these areas.

• The edges of dressings can rub and cause blisters.
• Try to use as few dressings as possible once raw areas have healed to help prevent further rubbing and blisters.
• Wear soft, seamless, loose-fitting clothing.
• Choose footwear to avoid rubbing.
• Avoid knocking/rubbing and overheating.
• Treat hard and thickened skin on the soles of the feet with urea cream.

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 if there is skin loss or if the area looks particularly sore.

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 the dressings are removed at once.

When you and your child are ready, we recommend bathing or showering regularly to cleanse the skin and 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 towel lined 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 is safer to bathe them with their dressings on, then change them after the bath.
• Use salt baths when the feet are blistered and sore.
• Use an antimicrobial wash to help prevent skin infections. These should be rotated on a regular basis to prevent antimicrobial resistance.
• Give Milton baths 1-2 times a week.
• Pat the skin dry with a soft towel rather than rubbing.

Please speak with your EB nurse for further advice on salt and Milton baths.

People with kindler EB or photosensitive skin may experience blistering or burning from even a short time in the sun. They may also have pigment changes or scarring and increased fragility in sun exposed areas.

• Wear SPF 50+ sunscreen, wide brimmed hats, and protective clothing.
• Avoid direct sunlight where possible.
• Wear UV blocking sunglasses to protect the eyes.

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’re 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.

Clothing with SPF (sun protection factor) is specially designed to protect skin from harmful UV rays, which is especially important for people with photosensitive conditions like kindler EB.

Your child’s GP may be able to prescribe specialist garments that can help keep dressings in place.

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.

• Avoid using scented baby wipes if the nappy area is red or the skin is broken.
• 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.

It is important to look after the nails- they can become thickened, discoloured, or even fall off in people with kindler EB.

Tips for managing nails:

• Keep nails short and smooth. Trim nails regularly with gentle, rounded scissors or a nail file.
• File rough edges carefully to avoid scratching nearby skin.
• Soften nails before trimming by soaking in warm salt water. Your EB team can recommend a urea cream to help soften the nails.
• Prevent trauma- avoid tight shoes or socks that rub the nails.
• Use protective dressings around the fingers/toes if they frequently blister.
• Watch for signs of infection- look for redness, swelling, pus, or increased pain around the nail.
• Moisturise regularly- use gentle moisturisers around the nail folds and cuticles.

For thickened skin on the soles of the feet or very painful nails which are difficult to manage at home your EB Team can refer you to an EB Podiatrist who can help and show you what to do.

Footwear needs to be chosen carefully to prevent friction, reduce pressure, and protect fragile skin.

Tips for choosing shoes:

Try to pick shoes with:

• Soft, seamless insides. Avoid rough seams or stitching that could rub or cause blisters. Some brands offer seamless socks/shoes specifically for sensitive skin.
• Extra depth and width. This allows room for dressings and bandages without pressure. Avoid shoes that are too tight.
• Cushioned insoles. Soft, padded insoles help absorb shock and reduce impact on the feet.
• Adjustable closures. Velcro or soft laces are best for adjusting fit without causing pressure points. Avoid zips or buckles near sensitive areas.
• Breathable materials. Leather or mesh helps reduce heat and sweating of the feet, both of which can increase the risk of blistering to the feet.
• Flexible soles. A soft, flexible sole moves with the foot and reduces friction while walking.

Blisters in the mouth can make feeding sore and difficult.

Tips for breastfeeding:

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

Tips for bottle feeding:

• A special feeder bottle which is easier for the baby to suck 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:

It is important to monitor swallowing issues or digestive problems.

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 development.

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

In some cases, surgical treatment is needed for tightness (strictures) in the throat.

Constipation is a frequent 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.

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. A dietitian will be able to offer advice on how diet can improve this.

Children with kindler EB often develop gum inflammation and dental issues. This can sometimes make cleaning the teeth painful and lead to dental problems.

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.