By Filipa and Will Hinton, parents to three-year-old Arthur, and his twin brother, Finlay

Discovering our child had EB

We hadn’t heard of epidermolysis bullosa (EB) before, not many people have, as we’ve since found out.

When our son, Arthur, was born, he had a spontaneous mutation in his genes, which caused epidermolysis bullosa simplex or EBS, the most common type of EB.

Within several days after his birth, we were referred to the specialist EB healthcare team at Great Ormond Street Hospital (GOSH) and through them, we found DEBRA UK.

Up until that point, outside of the brilliant EB healthcare provided by the team at GOSH, we had been trying to manage Arthur’s EBS as best as we could, and on our own. We were aware that there could be financial support available for us, but it just felt so hard to reach. That was until Amelia from the DEBRA UK Community Support Team came into our lives.

 

The support we have received

Having someone like Amelia by our side who understood what we were going through made such a difference. She provided us with useful information and advice, she was positive, always patient, and she walked us through the process of applying for Disability Living Allowance (DLA), step by step. She saved us so much time and without her support we would have walked away, as it just felt too overwhelming to complete the forms with everything else we had to contend with. 

Amelia also advocated on our behalf so that the people making the decisions about the DLA could understand what this unknown condition is really like, and what it means for our family every day; an hour spent changing dressings in the morning, 2 hours at night, and consequently, 2-3 hours of lost sleep, every night. This is the reality of our lives now, and most likely a very similar reality of many families all over the country affected by EB. 

Having DLA has made a real difference to us. Arthur’s DLA funding includes covering expenses to the many medical appointments he attends, including a 5 to 6 hour round trip to GOSH, and all his local weekly appointments such as with the podiatrist. The DLA also helps fund specialist items such as flat seam clothing and shoes, tools to deal with his blisters and remove scabs/flaky skin (such as scissors and tweezers) that are not available by prescription, a car seat without shoulder straps, and satin coverings for all the surfaces he sits or lies down on. Due to his wounds and treatment on a daily basis, he needs several changes of clothes per day, as well as changes of bedding and towels to minimise risk of infection. In addition, the DLA helps cover some of the extra cost of the electricity and water bills we incur every month for his baths and the additional lighting during wound care sessions.

Through Amelia, DEBRA UK also provided us with financial grants which enabled us to buy specialist items that have enabled us to better manage Arthur’s EBS symptoms including sheepskin bedding toppers, which has helped reduce his friction wounds during sleep, a fan to help keep Arthur cool in the warmer summer months, and specialist silk-based clothing when he was first born, which allowed his skin to breathe and helped control temperature and humidity – reducing his itch.

 

Daily challenges of living with EB

Because EB is so rare, very few people know about it or really understand the impact it has on Arthur and on us as a family. In our experience, awareness is also low amongst healthcare professionals. We are so grateful to the EB team at GOSH, they have made a huge difference to our lives, but outside of this specialist team we still need to educate others about EB. For example, when Arthur needs blood tests, we must remind the nurses that they cannot use a plaster to cover the wound, otherwise it will damage his skin by causing it to blister or tear. They do an amazing job, but unfortunately this is the reality of living with a rare condition like EB, every day is an ‘educating about EB’ day.

Arthur’s EB affects the outer layer of skin (epidermis) of his whole body. One of the biggest problems we must contend with is regularly fighting the skin infections that he is at high risk of developing due to his daily wounds. It is an ongoing battle, and to reduce infection, Arthur is often being treated with antibiotics.

The blisters on Arthur’s feet have had a negative impact on his development. It wasn’t until he was two and a half years of age that he started to walk by himself. However, he can only walk for short periods before he reverts back to going on his knees to move around the house due to the pain in his feet. We understand that a wheelchair may be inevitable at some stage.

 

No longer feeling alone

As challenging as life has been living with EBS, we have found immense comfort and take great satisfaction from seeing Arthur overcome some of the challenges that many children his age tackle with ease. He is a determined little boy who doesn’t take “no” for an answer! He is also very happy and positive, despite the constant pain and discomfort he endures.

The challenge of securing the support for Arthur’s needs has been an emotional rollercoaster, from the fear of finding a nursery willing to take on the additional responsibilities of caring for a child with EB, to the elation of Arthur’s first day, and of seeing the pleasure he takes from doing the simple things that three-year-olds get to do every day at nursery.

Amelia and the Community Support Team have also really helped us by getting the necessary understanding and allowances from Arthur’s nursery, including recognition that his physical condition can impact his rate of learning (due to pain and discomfort, hospital appointments, etc.), and the need for all his classrooms to be on one level. Knowing that the nursery understands Arthur’s needs and will make these necessary allowances reassures us and takes some of the stress out of Arthur attending nursery.

Due to the brilliant support we receive from the EB team at GOSH and the Community Support Team at DEBRA UK, we no longer feel alone with EB. We now have people by our side to support us on this journey, and we have met others who are facing similar challenges to those we face every day. This makes a huge difference. Just being able to talk, to share experiences and ideas, has changed our lives for the better, and we now feel part of a community that supports each other.

 

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The DEBRA UK Membership Team run community events throughout the year, including virtual ‘Members Connect’ calls and in-person events, where members can share their knowledge and experiences of living with EB, and connect with others. DEBRA UK have also recently launched a page on EB Connect, an online social collaboration platform for the global EB community.

The DEBRA UK Community Support Team provide support and care for the EB community, including helping to secure financial support through government schemes and providing grants to fund specialist equipment to help make life more comfortable.

 

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The EB stories blog on the DEBRA UK website is a place for members of the EB community to share their lived experiences of EB. Whether they have EB themselves, care for someone living with EB, or work within a healthcare or research capacity related to EB. 

The views and experiences of the EB community expressed and shared through their EB stories blog posts are their own and do not necessarily represent the views of DEBRA UK.  DEBRA UK is not accountable for the opinions shared within the EB stories blog, and those opinions are of the individual member.