Children with immune deficiency must spend much of their time in isolation but a new project brings hope

Published on Sunday 1 November 2009 10:55

IMAGINE your child is ill. Very ill, but you can't kiss her better. In fact you can barely touch her until you've scrubbed your hands for three minutes and put on a gown because she's isolated in a 'bubble' and any contact you make lays her open to a potentially fatal infection. Even a kiss from a mother with a cold could be fatal.

This is the situation for parents of children without a proper immune system. These are youngsters who cannot be exposed to other people and have to live in a hi-tech sterile space while they are treated. Not only that, but they're subjected to a double dose of isolation since there are almost no wards dedicated solely to children with immune system illnesses. Instead, the babies and youngsters are dotted around various other wards depending on where there is space.

Kelso-based Sarah and Richard Thomson know how that feels as their two-year-old daughter Ellie has spent too much of her young life in a 'bubble', or sterilised 8ft by 8ft space, out of reach of family and friends while she fights the MHC class II deficiency that has ravaged her little body. As a result, her mother has also spent much of the last two years in hospital, either at the Edinburgh Royal Hospital for Sick Children or in Newcastle General. But there are no immunology departments, specialist nurses or beds at the Sick Kids or elsewhere in the country and children have to find spaces in cancer wards or other secluded areas. While the Bubble Foundation in Newcastle is leading the way in England, there is little support for parents battling these serious conditions in Scotland.

In a bid to rectify the situation, Thomson is launching the Children's Immunology Trust, to found an immunology base at Edinburgh's Royal Hospital for Sick Children and to support families. The initial aims are to raise enough money in 2010 to fund one or two nurses.

Now two and a half, Ellie was just two weeks old when her problems came to light. She was admitted to Edinburgh's Sick Kids' hospital with a twisted gut and went on to develop recurring chest problems and epilepsy. By the age of six months, she was on a ventilator and doctors had identified difficulties with her immune system, so she was admitted to one of the two specialist paediatric immunology centres in the UK, ward 23 in Newcastle General Hospital (known as the Bubble Unit) – the other is at London's Great Ormond Street.

In the Bubble Unit, Ellie was diagnosed with MHC class II deficiency, a condition that affects only about 120 children in the world. "It was a case of being in Newcastle for a year and coming home to Kelso at the weekend to look after her 11-year-old brother Will and 13-year-old sister Lucy.

"Her only chance of survival was a bone marrow transplant. Without it, children normally won't live beyond four years old. Fortunately, and unusually, her siblings were a perfect match."

Will and Lucy both volunteered to provide bone marrow for their little sister, and second time around the transplant was a success. A year on, she is able to live at home, although "still on a massive amount of medication" interspersed with regular trips to hospital. "Because there are so few with her condition, there's not much to go on," says Thomson.

"She has to be in a bubble environment where no-one can go near her. It's an 8ft by 8ft room with a red line that you can't cross unless you're a fully scrubbed-up parent or doctor. Any toys or clothes that go in have to be sterilised, and you can't get close as she picks up bugs so quickly. Our dilemma is do we want to have her in hospital where she's safe or at home where she's at risk but can live a normal life. It's a balance, and we have to accept that we may have to go in every few days or live in hospitals all the time."

The experience has led the mother of three, who juggles caring for Ellie with running a fashion business, to set up the trust, with the aim of supporting other parents and children in the same situation.

"You spend all day in the bubble with the children and meet other parents in the same situation. Some of the children are born without immune systems and some have leukaemia but, because there are no dedicated immunology beds, you get bounced around from ward to ward. Because Ellie has complex needs there's always someone who will take us but children can end up on wards where they don't know your background.

"Ellie was the first of five children in the last five years to be sent to Newcastle for a transplant so it's likely there will be more to come. We want to raise funds to give them a base in Edinburgh where any research on immune system diseases can be shared. We also want to raise funds for the parents and brothers and sisters to give them support. It's devastating for the whole family. The older children were old enough to understand what was going on and were very aware of the consequences of what would happen if it went wrong. My daughter doesn't like to focus on it and doesn't like to talk about it, but if Ellie goes into hospital, they both wobble, especially my son.

"We're hoping to set up a drop-in centre where parents can leave siblings and also maybe be able to treat the kids to some kind of outing. Lucy especially found it sore giving bone marrow and although we did give them little presents, we didn't know how to make them know how fantastic it was of them. It's difficult, because you don't want to leave the one that's in hospital, but you have to go out with the others too."

Flushed with the success of her first fundraising venture, a fashion show that raised 60,000, Thomson is now organising a charity ball in Kelso, with Kenny Logan volunteering some of his glittering Strictly Come Dancing shirts for the auction.

"Every mother has to juggle, and having a little child is hard even without the complications," says Thomson. "I'm just doing it from a mother's point of view and the staff at the Sick Kids have been so brilliant we want to give something back. I'm doing what any mother would."

The Children's Immunology Trust (01896 820111, www.bubblefoundation.org.uk)

This article was first published in the Scotland on Sunday on November 1, 2009