BRAVE GIRL WHOSE HOME IS HOSPITAL; Syndrome victim Rhiannon fights continual pain.

Byline: Greg Tindle

THIS is the family forced to live in hospital as their youngest battles a devastating disease.

Itwas onlymeant to be a three-day stay for Rhiannon Brawn - but four months later she remains in hospital with a crippling illness so rare experts have recorded less than 200 cases worldwide.

The 12-year-old's sister Angharad and her mum Debbie now live at the University Hospital of Wales in Cardiff indefinitely, keeping a daily vigil at Rhiannon's bedside as she undergoes treatment while in constant pain.

She has one of the world's rarest conditions - Schwartz-Jampel syndrome - that causes bones to crumble and muscles to waste.

But despite its extreme rarity, it has hit the family twice. Angharad, 14, also has the inherited syndrome.

Their mum said: "To watch your child growing up in this condition is not easy and I can'twave amagicwand to giveme a cure."

The double dose of misfortune happened because Debbie and her former partner - two healthy carriers of a rare rogue gene - had a 25% chance of having a child with the syndrome. Initially Debbie had no idea there was anything wrongwith her daughters,whodeveloped normally up to the age of five.

She told the Echo: "Rhiannon then started to complain about a pain in her hipsandlegsandanMRIscanandX-rays discovered a deterioration in the bones andthe socket ofher hip.Theboneswere crumbling.

"This painwent from bad toworse and became intense. The condition not only affected her hips and legs but also started to affect her mouth, tongue, hands and gradually over 18 months she became housebound.

"Angharad didn't have so many problemsandwas stillable togoto school.But I couldn't cope and had to give upmy job as a teacher and, as a single parent, had little or no help or support to deal with the care - 24 hours a day and then constant appointments with physio and other therapists and others specialists."

Rhiannon was admitted to UHW on July 28 for treatment.

Hermumand sister nowface spending Christmas in the hospital's parents accommodation.

Debbie said: "She was admitted to the children's wards for botox operations, which she needed to cope with the pain, and we are still here and will definitely be here this Christmas."

She and Angharad, a wheelchair user, live at the hospital and Debbie is at Rhiannon's bedside from 7.30am until her daughter's bedtime at 10.30pm. Meanwhile, Angharad is picked up from the hospital and taken to school at Blackwood Comprehensive every day. There teachers help the teenager to catch up when she is not well and has to miss classes.

Debbie, 43, said: "I'm here in hospital all the time and very fortunate to be allowed to stay and I thank God for the NHS. Without its help, I don't know where we'd be.

"For most of the time Rhiannon is in pain and her eyes tell me what sort of pain she's in. She can be whimpering or in screaming pain. It's horrible but I've accustomed myself and have to remain positive. It's the bones and the nerves around them that are causing the problem and she's onmorphine patches to try to control the discomfort.

"I live in the hospital because there is no way a nurse could give the one-to-one care that she needs. I monitor her, know when she needs medication and I'm there to feed her and cut up her food because she's limited to a soft diet.

"I try to keep her entertained and the play specialist is brilliant; she has games and DVDs and PlayStations.

"But what we would really like is a laptop so she can keep in touch with her friends; that would be brilliant for her.

"Rhiannon is very intelligent: she loves scrabble and chess and has a wicked sense of humour and I'm here to make sure she has the best that life can offer.

"I'm lucky that I'm a devout Christian and still keep my faith and know that so many others from my church are supporting me with their prayers."

To give Debbie and Rhiannon a break fromthe hospital setting they will shortly be taking a five-day break at Ty Hafan Hospice in Sully.

On Tuesday theWelsh Blood Service laid on a special bedside visit to Rhiannon as a thank-you for entering its Children's Christmas Card competition which received 200 entries from children in wards across South Wales.

Although Rhiannon did not win, her effort touched organisers so much she received a surprise visit from cartoon characters Alice in Wonderland and the Mad Hatter.

Health special report: See Pages 8-9

CAPTION(S):

Rhiannon Brawn and mum Debbie; right, Rhiannon's sister Angharad Rhiannon Brawn, 12, in the Children's Hospital for Wales at UHW, Cardiff, with, from left, director of the Welsh Blood Service PICTURE: Darren Britton