Managing the skin condition, EB, can be hard, but worth it

When Tina Tully and husband John Clancy adopted daughter Kathleena in November 2018, nobody had ever heard of Wuhan, the city she’s from.

“Now everybody has heard of it,” says Tina, who’s glad Kathleena’s only three and not yet at school where she might hear “goodness knows what” about the place of her birth, now forever linked with the start of coronavirus.

“It all goes over her head,” says Tina, a development worker from near Virginia, Co Cavan.

But whatever about coronavirus, Kathleena’s starting to ask questions about EB (epidermolysis bullosa) — the painful genetic skin condition that causes skin layers and internal body linings to blister and wound at the slightest touch.

Kathleena has a milder form of EB, yet the effect on her and impact on her family can still be severe.

Tina and John knew that she had EB before adopting her.

“We’d seen TV programmes — it seemed to be the most excruciating, horrifying condition anyone could have,” says Tina.

They’d waited six years for their little girl from China and now, knowing she had EB but not yet sure how severe it was, their first thought was for their son, Ivan, now 9, also adopted and from Russia.

“A biological parent could give birth to a child with whatever medical needs, but we could make a choice,” says Tina.

Our primary concern was Ivan. If we adopted a child with severe EB, we’d be deliberately doing something that’d have a major effect on him.

Approved by Adoption Authority of Ireland to adopt a child with milder medical needs, and having got dermatologist confirmation that Kathleena’s files sent from China seemed to indicate milder EB, the family’s decision was helped by something one consultant said.

“We were discussing what EB would entail, and he said: ‘You have to think about the good this child will bring into your family’. It stuck in my mind,” says Tina.

“Whenever little worries crept in, his words reassured us: it wouldn’t be plain sailing, but there’d be lots of positives and joy.”

Meeting Kathleena for the first time in a municipal building in Wuhan, along with the children’s home director and one of Kathleena’s nannies, was very surreal, recalls Tina.

“Here was this little girl, almost two, and she had no notion who we were — that we were going to adopt her.

"I was looking at her as if she was someone else’s child — it was very hard to feel or think of myself as her mother.”

It was also scary, hearing about Kathleena’s skin condition and its care.

“They explained that any blisters would have to be punctured with a needle and drained — otherwise they’d grow — and bandages applied.

"That’d be alright if medical help was available nearby or an outreach nurse could come, but here we were, facing 10 days in China on our own with nobody to help.”

Once the director and nanny left, leaving Kathleena with her adoptive parents and brother, Tina “instantly became her mother”.

Kathleena had been one of a small group of children in the orphanage, cared for by just a few nannies, so she’d formed bonds.

She was lucky in that, but my heart just broke for her — she was in absolute bits [when they left]. Some children go into complete shock. She roared crying. It was very tough.

In their hotel, Kathleena fell asleep exhausted, and they wondered how she’d be when she woke.

“She sat up, gave a little whimper and went on from there. I’m not saying it’s been easy.

"She struggles still with letting me out of her sight,” says Tina.

Kathleena’s “strong-willed, funny, hugely empathetic, loves animals and is robust and clever”.

She needs it all to live with EB, which predominantly affects her left leg, and parts of her right.

Her condition is unpredictable — a year ago she was blister-free, but last summer saw her condition deteriorate.

“She has never fully recovered from that cycle of wounds. Her whole left leg has been in bandages since July.”

Bandaging and applying creams twice daily takes about 90 minutes.

“Sometimes she screams with particular blisters — if one needs puncturing, it could take three of us to hold her still. It’s very distressing.”

In December, Tina felt overwhelmed.

“I’m not a nurse. I’m an ordinary person looking at really bad sores and wounds. You’re always hoping her skin will go back to what it was.

"It might start healing — then you take off the bandages and you’re back to square one. It’s a constant drip-drip of trauma.”

Contacting support organisation Debra Ireland really helped.

Family support worker Deirdre arrived within days and Tina confided how she didn’t feel entitled to complain because her child has milder EB.

“But Deirdre said it’s still a horrific condition, and I shouldn’t underrate what we’re going through. It was so reassuring.”

On Deirdre’s suggestion, Kathleena and Tina have had acupuncture — a great practical/emotional support — and Tina has chatted with a mother of two children with milder EB, which has been “really helpful”.

Kathleena’s now becoming aware she has EB and other children don’t.

Seeing her process this can be heartbreaking. Her parents say she equates having EB with being a girl.

“Somebody gave her a doll and she was calling it ‘he’ – she mixes up ‘he’ and ‘she’.

John said: ‘the doll’s a girl’, and Kathleena straight away said: ‘I’ll rub her sore leg when it’s annoying her’ — because we rub her leg to prevent her scratching.

Kathleena had just started pre-school before the shutdown. She’s playing outside a lot — and coming in with more blisters.

“We had to deal with five in one afternoon. Sometimes I want to shout: ‘Don’t run!’ but I try to minimise that.

"As she gets older and becomes more aware, she may become more mindful.”

But what matters, says Tina, is that Kathleena lives her life despite EB – and she’s certainly doing that.

Covid-19 impact on EB

About 300 families in Ireland live with EB – equating to approximately one-in-every-18,000 babies born here.

Some 500,000 people worldwide are affected by it. The Covid-19 emergency has impacted in a number of ways.

“The big difference is the dramatic reduction in the interaction with key carers who monitor and manage the physical and psycho-social impact of EB.

"Multi-disciplinary clinics are now held over the phone rather than in person,” explains Jimmy Fearon, chief executive of support organisation Debra Ireland.

He adds that a number of families living with EB are vulnerable, and if Covid-19 enters their household it could have a direct impact on their vital daily care, for example changes to bandage requiring two people that take up to three to four hours.

As with other charities, the shutdown and restrictions have brought cancellation and postponement of Debra Ireland fundraising events, which could result in a major drop in income of about €300,000.

Debra Ireland hopes fundraising events will be held once restrictions are lifted but say there’s no guarantee this will happen.

“We’ll need to fundraise to maintain and continue our EB Community Care programme and our research, critical to finding new treatments and a cure,” says Fearon.

Anyone who’d like to support families living with EB should donate at debraireland.org/.