Telford mum driven to help cystic fibrosis fight

Chester Hughes, who is just 10 months old, has already spent much of his short life in and out of hospital.

After learning about what life is like battling the illness, Emma has already raised almost £6,500 for the Cystic Fibrosis Trust and plans to keep doing as much as she can to make more people aware of the disease and raise even more vital funds in the hopes of one day finding a cure.

Chester's older brother Oakley, who just turned four, will be taking part in a mini-mud run this weekend to do his bit to raise money.

• Around one in 25 people carry the faulty gene behind cystic fibrosis.

• Currently there is no cure for cystic fibrosis, and sufferers have to manage their condition with physiotherapy, exercise, medication and nutrition.

• The Cystic Fibrosis Trust, a research charity, says that currently half of the cystic fibrosis population in the United Kingdom will live past 41, and improved care and treatments mean that a baby born today is expected to live even longer.[/breakout]

Mrs Hughes, 34, said: "I just want to spread the word and raise money so my baby may live a long and full life.

Chester was born and almost immediately taken to Birmingham Children's Hospital for surgery and stayed there for three months.

He was born with a condition called meconium ileus, a blocked bowel, which is often linked to cystic fibrosis.

At only a week old, Chester was diagnosed with the condition and has spent much of his first year in and out of hospital with bronchiolitis, requiring intravenous antibiotics for two weeks each time.

The illness is life-limiting, but progress in research means that children diagnosed with it today have far better prospects than those born a generation ago.

A new drug called Orkambi is offering new hope of a cure. Chester has a type of cystic fibrosis that means that he is unlikely to benefit from it, but all progress is welcome news to Mrs Hughes. She says it is vital that the Cystic Fibrosis Trust is given the funds its needs to push on with vital research.

She said: "Since Chester's diagnosis I've made it my mission to spread awareness and raise vital funds for the Cystic Fibrosis Trust.

"I've only once not seen him smiling. For him it is normal and because he has spent so much time in hospital with different doctors and nurses he is happy to go up and sit with anyone. Oakley looks after him and when he is in hospital he checks that all the nurses have done everything they need to."

Each day, Chester has to use a nebuliser and have half an hour of massage too, and takes about 10 pills up to 20 times a day to manage his condition.

His condition also makes it difficult for him to put on weight, so he still has three night feeds as well as three meals a day topped up with bottles

Mrs Hughes and her husband Lee, 38, serve in the army and only moved to their home in Arleston, Telford, from Aldershot a month before Chester was born.

It has been dubbed the 'wonder drug' that could transform the lives of thousands of cystic fibrosis patients.

Orkambi is a combination of two drugs that when combined tackle the cause of the genetic disease rather than just relieving its symptoms.

Cystic fibrosis, CF, causes the lungs and digestive system to become clogged with thick, sticky mucus. If a patient's lungs become extensively damaged then a transplant may be necessary. The average life expectancy of sufferers is just 41 years.

The new twice-a-day tablet, which is being considered for use in the NHS, works by thinning the mucus, preventing damage and allowing the lungs to heal.

Experts say that while Orkambi is not a cure for CF, the treatment could allow many patients to lead near-normal lives without the need for a transplant. Orkambi was licensed for use in the UK last November, and health watchdog the National Institute for Health and Care Excellence, NICE, is now considering the case for it to be made available on the NHS, with a decision expected later this year.

Respiratory consultant Professor Stuart Elborn, who trialled the new drug at Queen's University in Belfast, said: "These two new treatments are game-changers. We are now seeing CF becoming a chronic disease that you live with instead of one that takes your life."

Around 10,000 people in Britain have CF, and they form the single largest group needing a lung transplant. Orkambi works to correct a faulty gene, known as the cystic fibrosis transmembrane conductance regulator. The gene usually creates a protein that moves salt and water out of a cell, but when these proteins are missing or defective, a sticky mucus builds up in the lungs, causing infection, breathing difficulties and loss of lung function. Orkambi works by allowing the protein within the cells to rise to the surface to move salt in and out of the cell, keeping a healthy balance of salt and water in the lungs and other organs.

She started her fundraising in October by taking part in the Great Birmingham Run, which she described as a huge challenge as it was not long after Chester's birth.

Since then she has also held a Facebook auction, craft sales and a raffle.

Mr and Mrs Hughes will be taking on the Born Survivor obstacle course at Weston Park in July and then Mrs Hughes and Oakley will do the Spartan Race in October.

Cystic fibrosis is a genetic condition in which the lungs and digestive system become clogged with thick mucus. There is currently no cure for this condition, and every week two people die from the illness and five more babies are diagnosed.

Chester has a page on justgiving.com where people can donate to the fight to find a cure for cystic fibrosis.