Breakthrough brings hope in CF battle
HOLLY Elwood's mother Paula is today clinging on to hope after the announcement of breakthrough in the treatment of Cystic Fibrosis. As organisers of the Ipswich and Suffolk Press Ball pledge to raise thousands to beat this life threatening and cruel disease, JAMES MARSTON reports.
HOLLY Elwood's mother Paula is today clinging on to hope after the announcement of breakthrough in the treatment of Cystic Fibrosis.
As organisers of the Ipswich and Suffolk Press Ball pledge to raise thousands to beat this life threatening and cruel disease, JAMES MARSTON reports.
PAULA is a busy mum.
With a house to keep, two lively and active young girls and her work as a freelance writer, life is a juggling act.
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The 39-year-old also lives with Cystic Fibrosis (CF) in her family as her six-year-old daughter Holly is a sufferer.
Living with the knowledge that her daughter may die before she does is something, Paula says, no parent can bear easily.
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But there is hope.
A recent breakthrough in gene therapy means Paula can be optimistic about the future in a way unimaginable just a few years ago.
She said: “Ever since they discovered the defective gene which causes Cystic Fibrosis back in the 1980s they have been trying to find a way to correct it.
“How to get the cure into the body has been the problem but it now looks as though they may have succeeded. It is fantastic news and this therapy would have the same impact on CF as insulin does on diabetes.
“It is not an outright cure but means the condition will be manageable.”
Trials for the therapy are now taking place with talk of a possible product for sufferers by 2012.
Paula is sensible enough to know not to hold her breath - CF is a dangerous condition and serious illness can strike at any time - but for the first time she has real hope for the future.
She said: “The CF community is quite sceptical about dates. This has been a long process but we are optimistic, this is a case of now if now but when.”
Of course research, medical trials, and developing medicines costs huge amounts of money and this is why organisers of the Ipswich and Suffolk press Ball have chosen the Cystic Fibroisis Trust as its beneficiary charity.
Paula said: “I went to the Press Ball last year and I'll be there again. It is a fantastic night and guests raise a huge amount that really makes a difference to families like mine.”
While Holly and sister Ruby, 3, run off to watch a DVD, Paula goes through the story of Holly's diagnosis.
She said: “When Holly was diagnosed the first thing I thought of was Cerebral Palsy. I'd heard of Cystic Fibrosis but didn't really know what it meant or what it was. I couldn't get my head around exactly what it was.
“Which is why events like the Press Ball are so helpful in raising awareness as well.”
Holly was born on December 15 and it was discovered she had a blockage in the bowel.
Paula said: “She didn't have the greatest start. She was born in Ipswich Hospital and she was rushed to Great Ormond Street for surgery.
“She stopped breathing on the operating table but they got her back and she was in intensive care for two days. Holly didn't come home until New Year's Eve.
“I had no idea about the implications of CF. The prognosis at the time was bleak and she had a life expectancy of about 28 years.”
Cystic Fibrosis is the UK's most common life-threatening inherited disease affecting 8000 children and adults. People who have CF produce a thick sticky mucus which clogs their lungs and digestive systems making it difficult to breathe and absorb food properly.
Holly starts her day with a cocktail of drugs, including a selection of enzymes to help digestion.
Paula said: “It is impossible to predict how the disease will progress. At the moment Holly is a robust six-year-old and she does the same things as her friends and her sister.
“Holly has physiotherapy every day. The best way I can describe it is like patting a tomato ketchup bottle. I pat her chest on both side and under her arms so that any gunk in her lungs is coughed up.
“We also do things like trampolining, blowing bubbles, space hoppers and other activities which are fun but also good for her.”
Holly is a regular visitor to Ipswich and London hospitals for X rays, lung function tests, check ups and other tests.
Holly, who walks in to the room asking Mum for a drink, said: “The best bit about having it is that it makes me special and different but the worst is the pills and blood tests.”
Paul added: “Ruby sometimes wants me to do physiotherapy on her as well and lots of teddy bears have it as well.”
The condition can be isolating, children with CF are not encouraged to meet and mix due to the dangers of cross infection.
A student at Combs Primary School, Holly takes with her a card to remind staff she has to have a high fat, high calorie diet.
Paul said: “A jam donut is a health food for Holly. She has to eat cakes and sweets.”
There are over 8,000 people in the UK with Cystic Fibrosis and each week five babies are born with Cystic Fibrosis and three young people die - 90per cent from lung damage.
Average life expectancy is just 31, although improvements in treatments mean a baby born today is expected to live longer.
Paula said one person in 25 carries the faulty CF gene and if two carriers have a baby, the child has a 1 in 4 chance of having Cystic Fibrosis.
She said: “People don't know if they are carriers. We didn't. There are thousands of people tip toeing through this genetic minefield. there's no history in our family of CF but before the 1960s it is unlikely it would have been recognised as CF.”
For Paula, a keen fundraiser for the Cystic Fibrosis Trust, the Ipswich and Suffolk Press Ball, and events like it, can have a direct impact on those living with the condition.
She said: “There are three main areas where people with CF need help. The first is to offer hope through gene therapy. At the moment researchers are positive that the life threatening aspect of the disease can be removed. I can't put into words what it would mean to me and my family if that happened.
“Until that day happens people like me need to be able to function on a day to day basis and manage the condition.
“There also needs to be consistent CF treatment across the country, we need to make sure there is best care and best practice and that is where the CF Trust comes into its own.”
To sum up Paula reminds Press Ball guests of the value of the money the event raises.
She said: “If you look at Holly she seems fine and full of life and loving life as long as there is breath in my body she will do so as long as she can.
“But at the moment Cystic Fibrosis has a 100 percent fatality rate, if you have CF then at some point it will kill you.”
Further information can be found about Cystic Fibrosis on the website www.cftrust.org.uk. Help and advice for those affected by Cystic Fibrosis is available through the Cystic Fibrosis Helpline on 0845 859 1000.
Have you been helped by the Cystic Fibrosis Trust? Are you a regular guest at the Ipswich and Suffolk Press Ball? What do you think? Write to Your Letters Evening Star 30 Lower Brook Street Ipswich IP4 1AN or send an e mail to firstname.lastname@example.org
Around 1 in 10 children with cystic fibrosis are diagnosed at or shortly after birth due to a condition called meconium ileus where the gut becomes blocked with meconium - a thick, dark, sticky substance which is made in all babies' intestines before being born. Urgent surgery may be needed to relieve the blockage.
Some children born earlier than 2007 who were not screened at birth will be diagnosed later after they have become unwell and developed symptoms, often involving many dirty nappies each day, chestiness and failure to thrive.
Symptoms usually first develop within the first year of life, but in a minority of cases may not appear until later in childhood. The severity of symptoms can vary and not all children with CF will have all the symptoms.
In those with Cystic Fibrosis, the lungs make thicker sputum (mucus) than normal. This can trap bacteria in the small airways and lead to infection. Symptoms which typically develop include, persistent cough, wheezing, shortness of breath and breathing difficulties, repeated chest infections
In people with Cystic Fibrosis, thickened secretions block the normal flow of the digestive juices from the pancreas. This results in food not being digested or absorbed properly, in particular, fatty foods and fat-soluble vitamins (vitamins A, D, E and K).
The Cystic Fibrosis Trust is the UK's only national charity dealing with all aspects of Cystic Fibrosis. It funds research to treat and cure CF and aims to ensure appropriate clinical care and support for people with Cystic Fibrosis.
The Cystic Fibrosis Trust was founded in 1964.
The trust's objectives are:
- To fund medical and scientific research to develop a cure and provide effective treatments for Cystic Fibrosis
- To ensure appropriate clinical care for those with Cystic Fibrosis
- To provide information, advice, support and, where appropriate, financial assistance to anyone affected by Cystic Fibrosis.
The Cystic Fibrosis Trust invests around �4 million each year in a programme of research into gene therapy to make it a clinical reality in the foreseeable future.
Gene therapy aims to add a healthy copy of the faulty CF gene to the lung.
The Cystic Fibrosis Trust has brought together 80 of the UK's leading CF scientists and clinicians to drive this research forward through the UK Cystic Fibrosis Gene Therapy Consortium.
The scientists have developed a product, which they plan to put into a one year multi-dose clinical trial beginning in 2010.
It is estimated the product for the trial will cost at least �6 � million. A more precise figure will be known following a single dose trial in 2008.
Since the inaugural ball in 1997 the Press Ball has raised thousands for charity and this year the Press Ball organising committee has chosen the Cystic Fibrosis Trust as the 2009 beneficiaries.
The event will be held at the Hotel Elizabeth Copdock on Friday June 19 with music dancing fine food and wines followed by an auction and tombola with prizes donated by businesses and organisations.
As announced in The Evening Star, headline acts will include rock legend and soul sensation Ray Lewis of The Drifters fame who will perform at the Soul Ball themed night of nights.
Regularly and strongly supported by the county's business community the Press Ball is once again sponsored by Ipswich firm Call Connection.
Press Ball bosses are today offering the chance for the business community to offer their help to raise cash for the event's chosen charities
If your business or organisation would like to donate a raffle or auction prize to the event please contact Bridget York on 07961 430599