Pain and itch relief for EB patients

There is currently no cure for epidermolysis bullosa (EB) and at DEBRA UK we are working hard to change this. However, there are treatments that can help with EB pain relief and itch management, as well as other symptoms. Your EB specialist healthcare team will be able to advise which treatments are suitable in individual cases, but below is a general overview of the causes and treatments for these two common symptoms.

If you are not under an EB specialist healthcare team, then please ask your GP to refer you. You can find out more about accessing specialist healthcare, including our template referral letter you can use, on our managing medical care page.

This page also includes information on our financial support grants which cover a wide range of items, including things like fans and specialist products to help alleviate some of the symptoms of EB.

1. Causes of pain 
2. Dressing changes 
3. Infection
4. Stress and sleep 
5. EB pain relief 
6. Itch management for EB
7. DEBRA UK’s support grants
8. Extra resources

Causes of pain

There are many complex reasons why people with EB experience pain. Identifying the cause of pain is important so that the right advice to reduce the pain can be offered. If you’re experiencing pain and need support, your EB healthcare team will be able to advise and help you with pain management.

For emotional support and practical help, such as financial support to fund items which may help with pain and itch, please get in touch with our EB Community Support Team.

Some of the reasons for pain from EB are: 

Blister sites and blister healing.
Areas of skin loss.
Open wounds.
Lesions on mucous membranes (tissue which secretes mucus and lines cavities and organs, e.g. the mouth, eyelids, stomach, etc.), the cornea (front part of the eye), and from non-cutaneous (unrelated to the skin) complications. 
Infections.

Application of incorrect dressings or topical treatments.
When dressings are changed.
Internal blistering.
Trauma to the skin like a rub or bang.
Overheating.
Sensitivity to products such as laundry detergents and deodorants.
Clothing materials.
Unknown causes.

Once you know why you are experiencing pain (even if there are unknown causes), you can work with your EB healthcare team towards a plan for EB pain relief.

The following sections offer some general tips on minimising pain for people living with EB. However, what works for one person may not work for another, so you should always seek advice from your healthcare team about your individual situation and EB type.

EB dressing changes

The dressing changes required to manage EB can be extremely distressing and painful, yet are a lifelong and important part of regular (sometimes daily) skin care, wound and blister management.

Blisters should be lanced as soon as possible to prevent them causing further pain and damage; bigger blisters = bigger wounds. The formation of blisters and breakdown of skin can cause significant pain, although some people living with EB also report pain when they don’t have blisters. Once a blister has been lanced, it will need to be dressed to protect the wound while the skin tries to heal. 

You can find more information about caring for blisters on our skin and wound care page, and a link to the DEBRA International patient guide in the resources section at the bottom of this page.

“The most difficult thing about EB is seeing your child in pain, knowing that the care you are giving is causing so much distress.”  Katie, mum to Jamie who has severe EB

The time taken to complete dressing changes can vary greatly, so for the best possible pain reduction it’s advisable to complete dressing changes in the least time possible. There are many different dressings available, and selection of the most appropriate dressing is important. For instance, severely affected newborns need minimal handling and may require dressings that can remain in place for several days. Also, non-adhesive dressings are crucial to reduce further damage and pain. If an adhesive dressing is applied in error, there are adhesive removal products available which may limit damage to the skin. 

The EB specialist healthcare teams have extensive expertise in this area and will be able to advise you on the right treatment plan in individual cases. You can contact your healthcare specialist, or if you don’t have access to an EB specialist, please get in touch with our EB Community Support Team who will be happy to help you with referrals. 

You can also find out more about home delivery dressing services you could use, such as Bullen Healthcare, on our managing medical care page.

Infection

Open wounds or raw skin can become infected and may need urgent treatment to prevent further pain and damage. A streptococcal (bacterial) infection can be particularly painful.  One of the most common causes of infections is poor hand hygiene, so thorough hand washing and clean equipment are essential when lancing blisters and changing dressings. 

Signs that an area of skin has become infected include:

Redness and heat around the area.
The area leaking pus or a watery discharge.
Crusting 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 (fever) of 38C (100.4F) or above.
An unusual odour.

Contact your GP or healthcare provider at the first sign of infection. Treatment may include antiseptic creams, antibiotics, gels, specialist dressings and for longer term support you can boost immunity via nutrition and dietary supplements. Nutrition is especially important as nutrients can be lost through wound fluid. Talk to a specialist to discuss the best nutritional plan for you as nutritional advice for people with EB may differ to that of people without EB.

Stress and sleep

Stress and lack of sleep can be an issue when dealing with painful wounds and can negatively impact EB wound healing and the ability to deal with pain. These symptoms can be improved through stress management techniques, nutritional supplements, medication, meditation, mindfulness and other wellbeing interventions.

Your healthcare team will be able to support you to find the right treatment for you and there are some links in the resources section below for additional support, such as Mind’s advice on coping with sleep problems.

Our support grants can also provide items that can help with sleep, such as fans to help keep you cool, particularly during the warm weather, and sheepskin bed toppers to reduce friction on skin.

EB pain relief

There are many different options to help with EB pain relief depending on the type and severity of pain, including creams, gels, oral medication and psychological interventions. 

In some instances, with mild EB, over-the-counter pain medication such as paracetamol may provide relief, but often stronger medication is required and is available through healthcare providers. Morphine may be prescribed, especially prior to dressing changes, and oral sucrose solution has shown to be beneficial prior to and during procedures in newborn babies.

Please note, the NHS advises that children under 16 should never be given aspirin as there is a small risk it could trigger a serious condition called Reye’s Syndrome. 

Reducing the time taken to do dressing changes and selecting the appropriate dressings and materials can help to reduce pain. You can also use templates to cut dressings in advance, therefore reducing the time that the individual experiences distress.

An adult and a child sit at a table assembling a stuffed animal, during the DEBRA UK Members' Weekend event.

Some people living with EB find that doing things they enjoy, such as listening to music, spending time with others, getting outside, playing games or watching TV provide a useful distraction. Using mindfulness and breathing techniques amongst other wellbeing interventions can also be helpful.

The teams at the EB specialist healthcare centres can support you with pain management techniques or a crisis plan for pain management. They have extensive experience in this area and however mild or severe your pain is, they should be able to help. The resources section below also contains links to organisations that offer support on pain management techniques.

Our research strategy strives to achieve cures and provide effective drug treatments for all types of EB, so we can improve quality of life for the whole EB community. Please visit our research page for more information about our current projects.

A photo of Maya, who has recessive dystrophic EB and is a DEBRA UK Ambassador.

Itch management for EB

“One of the most frustrating things for me is the itching. It’s like my whole body is on fire and I have ants crawling all over me. It’s so overwhelming and no one without EB can understand how bad the itch actually is.” Maya, who has recessive dystrophic EB and is a DEBRA UK Ambassador

Itching (pruritus) is defined as “an unpleasant sensation that provokes scratching.” 

With EB, itching can be very painful. Scratching, which can be difficult to resist, can cause new skin trauma and lead to the breakdown of wounds that are nearly healed. Scratching can also lead to an inflammatory reaction, which further strengthens the itch sensation.

Some causes of itching are:  

Healing blisters.
Dry skin. 
Overheating.
Inflammation.
Persistent skin damage due to re-occurrence of blisters in the same area.
Anaemia.

Some opiates/opioids may increase itch. 
Sensitivity to products such as laundry detergent, deodorants and other products which come into contact with the skin.
Stress can increase itch – please see the extra resources section below for support in dealing with stress.
Other unknown reasons or a combination of factors.

Good skin care is important in reducing itch. You can find some general advice on our skin and wound care page. Although the urge to scratch can be difficult to resist, some people find patting the area gently with a cool damp cloth provides some relief or taking a cool bath. If severity requires, there are medications available such as topical creams and ointments.

Other helpful tips for itch relief in EB include ensuring you’re hydrated enough, avoiding overheating and being mindful of the products that come into contact with your skin. Relaxation, breathing and mindfulness techniques can provide relief, often in combination with medical and other behavioural treatments. Different solutions work for different people so it’s best to discuss your individual needs with your healthcare team.

DEBRA UK’s support grants

We offer funding for a variety of grants to support our members’ physical and mental wellbeing during times of financial hardship or in recognition of the additional costs of living with EB. Members can apply for grants covering a wide range of items, including travel and accommodation to help attend vital EB specialist healthcare appointments, contributions towards a stay in one of our holiday homes, and specialist products to help alleviate some of the symptoms of EB and live more independently.

Please see our support grant overview to find out more, including a summary of the grants we offer with examples and our eligibility criteria.