There is no cure for epidermolysis bullosa (EB).
Treatment aims to:
- relieve symptoms
- prevent complications, such as infection
A team of medical specialists will help you decide what treatment is best for your child. They will offer advice about living with the condition.
Managing EB can include:
- applying protective dressings
- puncturing (lancing) blisters with a sterile needle
- avoiding things that make the condition worse
You can use medicines to treat infection or to reduce pain.
Your child may need surgery if:
- their food pipe narrows
- they have problems with their hands
Getting treatment at a specialist centre
After your child is diagnosed, you and your child will have follow-up appointments at a specialist centre. This is so a treatment plan can be drawn up.
When your child's symptoms improve or stabilise, it may be possible to get treatment closer to home. This means you'll only need to visit the specialist centre occasionally.
But with more severe types of EB, this may not always be possible.
Specialist care centres in Ireland
There are 2 specialist care centres in Ireland:
- for children's care - Children’s Health Ireland at Crumlin (CHI)
- for adult care - the dermatology department in St James’s Hospital
Get free transport to and from hospital appointments for children with EB - debra.ie
Care team
Children with EB often have complex needs, particularly if they have a severe form of the condition. They will have to be treated by a team of specialists (multidisciplinary team).
This team may include a:
- doctor who specialises in treating skin conditions (dermatologist)
- dentist
- dietitian
- physiotherapist
- play specialist, who uses playful activities to help improve a child's physical and psychological wellbeing
- specialist nurse, who usually acts as the contact between you and other members of the team
General advice
The treatment plan will include advice about preventing damage to your child's skin. This can help you reduce the risk of blisters.
The advice can vary depending on the type of EB and how serious your child's symptoms are.
It is important that your child takes part in all normal childhood activities. But they may need some extra support.
Do
-
use protective dressing and padding to minimise damage to your child's skin
-
keep your child as cool as possible in warm weather
-
wear clothes made from natural fabrics such as cotton (this will also help keep your child cool)
-
avoid clothes that fit tightly or rub against their skin
-
choose comfortable shoes that fit well and do not have lumpy seams inside
Skin care
Your care team will tell you how to care for your child's skin.
For example:
- when and how to puncture (lance) new blisters
- how to care for wounds left by blisters and prevent infection
- when to leave wounds uncovered or use dressings
- what dressings to use, how to put them on and remove them, and how often to change them
- when to use moisturising creams
You should normally puncture (lance) new blisters using a sterile needle. Your GP can give you a supply of sterile needles.
Lancing the blisters will stop them getting bigger. Large blisters can leave large, painful wounds that take longer to heal.
It's usually recommended to leave the skin on top of the blister to protect the lower layers of skin.
For an open wound, use a dressing that does not stick to the skin and is easy to remove.
You may be advised to use a sock, cotton bandage or tubular bandage to hold a non-stick dressing in place.
Avoid using regular sticking plasters.
Infections
Open wounds or raw patches of skin can often become infected and need to be treated.
Signs that an area of skin has become infected include:
- heat or redness around the area (this can be harder to see on brown and black skin)
- pus or a watery discharge
- a scab on the surface of the wound
- a wound not healing
- a red streak or line spreading away from a blister, or a collection of blisters
- a high temperature
Urgent advice: Contact your GP if:
- you think your child has a skin infection
If it is not treated, a skin infection can often quickly spread to other parts of the body, particularly with more severe forms of EB
Treatment for skin infections include:
- antiseptic creams or ointments
- antibiotic creams or lotions
- antibiotic tablets
- specially designed dressings to help skin heal
Pain relief
The blisters and wounds can be painful and make simple activities such as moving and walking difficult.
If your child has a milder type of EB, you may be able to use a painkiller you can buy without a prescription. For example, paracetamol or ibuprofen.
You may need stronger painkillers such as morphine for:
- severe types of EB
- long-term pain
Important
Do not give aspirin to children under the age of 16. There's a small risk it could trigger a serious condition called Reye's syndrome.
Dental care
Soreness caused by blisters inside your child's mouth can make cleaning their teeth difficult.
The following things can help:
- good dental hygiene
- a soft toothbrush
- a mouthwash that contains fluoride
- regular visits to a dentist
Nail care
Your child's fingernails and toenails may become thicker than normal. This can make them difficult to cut, especially if blisters form under the nail.
Your care team may recommend creams that soften nails and make them easier to cut.
Eye care
Children with severe types of EB can have blistering and irritation in and around their eyes. They will have this checked as part of their multidisciplinary care.
They may need eyedrops and ointments to keep their eyes moist.
Feeding and nutrition
If your baby has blisters in their mouth, it can cause problems with feeding. Your care team can give you advice about how to deal with feeding problems.
They may recommend:
- feeding your baby using a syringe or eyedropper
- adding liquid to mashed food to make it easier to swallow (when your child is old enough to eat solids)
- including lots of soft food in your child's diet
- not serving food too hot, as this can cause further blistering
If your child is older, your care team can also give you advice about their diet.
A healthy diet can help heal your child's skin wounds and avoid malnutrition.
Your child may also need supplements. For example, milk-based drinks or puddings that have high levels of protein and calories.
Blood tests will show if they need supplements of vitamins, iron or zinc. Your dietitian will be able to advise about this.
Constipation is a common problem for children with EB. This is because blistering around the bottom can make it painful to poo. It's also a side effect of some types of painkillers.
If your child regularly has constipation, they may benefit from having a fibre supplement or laxative.
Surgery
Your child may need surgery to treat complications from severe EB.
Foot and hand surgery
If your child's fingers and toes become fused together by scar tissue, they may need to have surgery to separate them.
Food pipe surgery
If your child's oesophagus (the tube connecting the mouth and stomach) has become narrowed by scarring, they may need food pipe surgery.
This is done by placing a balloon inside the oesophagus and inflating it to widen the narrowed area.
Tummy surgery
If your child is not growing properly because eating is so difficult, they may need tummy surgery.
This is where a feeding tube is inserted into their tummy.
Clinical trials
Clinical trials are research studies that help you access new treatments.
Ask your doctor or care team if they know of any clinical trials that you may be eligible to join. If a clinical trial is relevant for you, they will discuss this with you.
If you take part in a clinical trial, you may benefit from a new treatment.
But there's also a chance that the new treatment turns out to be no better than the standard treatment.