Pain and quality of life in young people with Epidermolysis Bullosa

Short lay summary

Children and young people with EB experience acute and chronic pain due to skin blistering and secondary infections, joint pain because of deformities from scarring and osteoporosis, problems with their digestive tract, eyes and teeth, and procedures such as dressing changes. These affect the quality of life (QoL) of the child and their parents.

Pain can be avoided, reduced and treated in a number of ways. For example, there are many pharmacological treatments (medicines) for treating pain; some are given based on the intensity of pain in a step-wise fashion, others try to alleviate specific types of pain, such as nerve or bone pain. There are also special dressings, sprays and bath lotions that can help to reduce pain. Psychological interventions are also known to be effective for many types of acute and chronic pain. However, currently there seems to be limited use of psychological interventions to control pain in children with EB, despite success in children having acute or chronic pain from a variety of other medical conditions.

Researchers at Great Ormond Street Hospital and Birmingham Children’s Hospital asked 133 parent and child pairs with EB to take part in a study to evaluate how much pain they experience and how it impacts their QoL. This is important to know before any new medicines or pain management strategies can be tested to see if they are effective. They also wanted to see if families would like training in psychological interventions to control pain.

The parents and children were given questionnaires that have been established and widely used in medical research and also one devised specifically for this study.

Preliminary results show that parents’ levels of anxiety and depression were within normal levels, but QoL of young patients was impaired as reported by both parents and children. Young patients experienced weekly pain and were afraid of pain.

Parents and children were interested in the project and gave their time freely to complete survey materials. Whilst parents were keen to learn more about psychological methods of pain control, time constraints, logistics and distance proved an obstacle to deliver such interventions face to face.

Therefore, the group will be looking for ways to develop on-line materials to enable easier access to tools that can teach families about psychological methods of pain control to help improve QoL in patients with EB.

This project was partially funded by DEBRA UK. More on DEBRa UK's website.

 

Strategic relevance

“This research has allowed us to identify how prevalent pain is in young people with EB and the detrimental effect it has on their quality of life and that of their families.

“We are very excited about taking these important findings forward, and translating them into online resources that will make psychological interventions for pain management available to every family affected by EB.”