Riley’s mum Jackie was prepared that her son might have EB as she has the condition, so, when Riley was born with no skin on his right leg from his knee to his toes she knew immediately what was wrong – but she wasn’t prepared for the severity, which was far worse than her own.
He had blisters under his finger nails, causing his nails to fall off (something she has experienced) and his first feed caused his tiny mouth to blister. Everyday more blisters materialised. He spent the first two weeks of his life in the special care baby unit being fed by a tube in his nose to minimise the trauma to his sensitive skin.
A blood test revealed that Riley had Recessive Dystrophic EB (RDEB) one of the most severe EB subtypes. It not only affects his skin but also the internal body linings of his throat and oesophagus. Riley is now five but his finger nails have never grown back and his fingers have started to fuse together where the skin has shed and healed so often, a common issue with RDEB. He is due to have a painful operation on one hand later in the year to separate his fingers, splints will then be used to try and prevent further fusing.
Riley is able to eat a very soft liquid diet but also has a gastrostomy and is fed through the night to ensure he gets enough calories to enable his body to heal and prevent weight loss. Jackie gave up work after a terrifying experience when paramedics were called because he started choking on the lining of his oesophagus. Jackie sometimes has to go into Riley’s school if he’s fallen over, to lance or dress a particularly large blister, or treat his gastrostomy if it weeps making it impossible for her to work. To try and prevent further choking episodes Riley has had his throat dilated on three separate occasions, to stretch it, where blisters have caused it to close. “Despite all the pain and medical procedures Riley is a cheerful and inquisitive little boy,” said Jackie. “He’s accepting of his EB but also has lots of questions, like: ‘Will my nails ever grow back?’ I try to be as honest as possible. Riley knows that he will probably never be free of EB and the pain.”
Jackie provides Riley’s EB care, she changes his dressings every other day - an excruciating and time consuming process, which starts with a cocktail of pain medication thirty minutes beforehand to give them time to work. Then begins the painful process of removing all of Riley’s bandages, after which he is rewarded with a long soak in the bath to sooth his skin (something he loves and looks forward to), his skin is treated and then the laborious process of cutting and reapplying his bandages starts, taking anything up to three hours. “I’m astounded at how well he withstands the pain, he rarely cries and I’m just so proud of him.” When asked how EB affects their life, Jackie responded: “I don’t know, Riley is my only child, so I don’t know what it would be like if he didn’t have EB. I don’t know any different. I just get on with it.”
Jackie is sometimes woken by Riley choking, the medication he takes to prevent him itching and to help him to sleep means he doesn’t always wake up himself and so she finds herself listening out for him in the night. Jackie was already aware of DEBRA as both her and her brother have EB and the charity has been able to help her complete the complicated Disability Living Allowance forms, to ensure they are able to access the benefits they are entitled to. “I don’t know where I would be without my DEBRA EB Community Support Manager, I wouldn’t know where to start completing the forms. She was also able to help with travel costs when Riley had his gastrostomy operation and for accommodation for my father, so he could support me, as it was a very worrying and stressful time for us all. Knowing DEBRA is there is so reassuring,” said Jackie. Jackie and her family have supported DEBRA in return by holding a fun day.
Photo of Riley courtesy of Claire Wood Photography: