When 33-year-old design manager Dan Bodio was diagnosed with cystic fibrosis at two months old, doctors told his parents his prospects were gloomy. Three decades on, Dan is continuing his bid for a life unlimited, as he explains to DENISE BARKLEY

DESIGN manager Dan Bodio has Cystic Fibrosis, but he doesn’t let it hold him back.

“Cystic fibrosis works around me, I don’t work around it,” said Dan, 33, from Swindon.

And that is clear to see - he has a Masters in mechanical engineering, a challenging career, and has been married to Alexa for six years. They are buying their home, enjoy foreign travel and love walks with their chocolate Labrador Daisy.

Cystic Fibrosis (CF) is one of the UK's most common life-threatening inherited diseases and is caused by a single defective gene. As a result, the internal organs, especially the lungs and digestive system, become clogged with thick sticky mucus resulting in chronic infections and inflammation in the lungs and difficulty digesting food. For many it involves a rigorous daily treatment regime including physiotherapy, oral, nebulised and occasionally intravenous (IV) antibiotics, and taking enzyme tablets with food.

Life is a constant battle for Dan, but one he is used to and accepts – and it’s given a boost when he gets a good result from treatment. He has just spent two weeks in hospital where a persistent lung infection has been treated with three different intravenous antibiotics, and the good news is that it has worked well and his lung function has increased by 22 per cent, up from 42 to 64 per cent.

“My doctor said the antibiotics had done a great job, and that’s a massive morale booster,” grinned Dan, who follows a strict regime of diet, exercise, physio and medication in an effort to stay healthy and fend off infection.

“From word go Cystic Fibrosis has never defined me,” he continued. “I was diagnosed as a two month-old baby in the early 1980s and mum and dad were given a leaflet which was just all doom and gloom.

“It said I wouldn’t see my sixteenth birthday and mum burst into tears, but the doctor grabbed the leaflet and told them it was ridiculously out-of-date and the prognosis was improving all the time.

“My parents decided that whatever we did as a family, my condition would be treated as normal, part of the daily routine – and that is how we have continued.”

Born in York, his family moved to Devizes when he was aged six. His parents now live in Wootton Bassett and his grandparents at Fairford.

Dan was fortunate that he was diagnosed as a baby, otherwise irreversible lung damage could have resulted.

A rigorous programme of ‘chest clapping’ was put in place, where a cupped hand is used to clap the chest firmly and rhythmically to help loosen secretions. Regular exercise is important in CF sufferers to help them stay fit and healthy enough to fight off infections and, aged four, Dan learned to swim and went on to represent Devizes at county level. He also played football and rugby.

Dan explained that usually CF primarily affects either the lungs or the digestive system, but with him “it’s a bit of both”. As a child he had to take 25 tablets a day and physio, exercise and school had to be fitted in as well.

“It sounds a lot but I don’t know any different, it’s just what I do,” he said. “As I got older nebulisers were introduced – they used to be big, heavy air compressors but now they are portable.”

A challenging time for young people with CF is when they leave home. Away from the supervision of parents “cracking the whip”, as Dan put it, routines can slip with catastrophic effects. But he has always been “quite regimental” in what he does.

“I am strict and hard on myself,” Dan revealed. “I don’t miss my treatments, but I do still get infections and I accept that and get it sorted, which means going into hospital for intravenous antibiotics.”

And he has had a tough 14 months, with five lots of IV.

“Despite my regime, I have a particular bug in my chest which has been proving difficult to get under control,” Dan said. So the news that his recent in-patient treatment has been successful is a big boost. He was treated at University Hospital, Southampton, the regional centre for patients living in Wiltshire.

But the medication he would really like to be prescribed is a drug called Orkambi. It is designed to treat the root cause of CF, not just the severity of symptoms, and is having positive results with patients in the USA and Canada. It has been shown to increase lung function and significantly reduce infection and hospital stays for those carrying two copies of the most common mutation of the CF gene, F508del. Dan has two copies of this gene.

However, in the UK, the National Institute for Health and Care Excellence (NICE), while acknowledging the clinical benefits of the drug, will not recommend Orkambi to NHS England based on its high cost and uncertainty of its long-term impact. The Cystic Fibrosis Trust has proposed a solution whereby Orkambi could be provided to all who need it while further evidence is collected on its long-term effects using the UK Cystic Fibrosis Data Registry, which records health data on CF sufferers.

Dan said he was getting behind the Cystic Fibrosis Trust’s campaign to get Orkambi approved: “When you consider I have been in hospital and treated with expensive antibiotics five times this year, surely the cost of that and prescribing Orkambi must be offsetting each other. And what price can you put on prolonging someone’s life?”

The holy grail for CF patients is gene therapy, with the goal of replacing the faulty CFTR gene with a working one. Researchers have reported limited success.

Dan commented: “It’s what we call a life unlimited, but there is nothing on the horizon for people of my age. If they were successful it would have the effect of stopping the clock, meaning we could live to 60 or 70, not dying as young as 25 or 30.”

But Dan doesn’t dwell on the negatives and he and Alexa, 34, live life to the full.

“Alexa takes a lot of positivity from me and my attitude,” he explained. “And she keeps me on the straight and narrow, making sure I get on my treadmill, lift my weights and do my physio, and walk the dog twice a day.

“In the evening after work she will prepare dinner while I go upstairs to my multigym and do half an hour in there – she’s taken over where mum and dad left off!”

They both work for Bosch Rexroth in South Cerney. Design manager Dan has been there for ten years and it is where he met Alexa, who is an internal sales co-ordinator. They have known each other for nine years.

“On our first date we went to Lulworth Cove in Dorset – she told me she had asthma and I said I had Cystic Fibrosis, it was all quite natural from the start,” said Dan.

They love holidaying abroad and have travelled widely, getting engaged in Tenerife and going to Mexico on honeymoon. This year they are looking forward to a holiday in Florida. But travelling is challenging.

He revealed: “As I have Cystic Fibrosis I can’t get any travel insurance, so I travel without it, and I always carry a letter from my consultant explaining why my hand luggage contains numerous hundreds of tablets and nebulisers.”

He admitted that when he got repeated infections, like in the last year, it was hard not to get down and “think the worst”, but his wife helped to keep him positive, and the success of his recent hospital treatment gave him something positive to focus on.

“When I got the all-clear from the doctor I practically skipped out of hospital. Alexa and I didn’t wait for Valentine’s Day, we cracked open a bottle of Champagne and celebrated!” he grinned.

“Increasing my lung capacity to 64 per cent was more than even my doctor expected. It’s made such a difference too, I feel loads better.”

And with that in mind, it is onwards and upwards for Dan.

“I am quite a competitive person, and I’m not going to be beaten – I’m going to kick CF into touch!”

For more information visit the website for the Cystic Fibrosis Trust: www.cysticfibrosis.org.uk

FACT FILE

• Cystic Fibrosis is one of the UK's most common life-threatening inherited diseases and is caused by a single defective gene.

• Around 10,500 people in the UK have CF, that's one in every 2,500 babies born. It affects around 100,000 people worldwide.

• One person in 25 carries the faulty CF gene usually without knowing - over two million people in the UK. If two carriers have a baby, the child has a one-in-four chance of having cystic fibrosis.

• All babies in the UK are now screened for CF shortly after birth using the heel prick blood test.

• In people with CF the lungs make thicker mucus than normal, which can trap bacteria in the small airways and lead to infection. Symptoms that typically develop include persistent cough, wheezing, shortness of breath and breathing difficulties and repeated chest infections.

• CF is as common in both sexes and is more common in the Caucasian population.

• One of the most striking features of CF is cross-infection - the risk that sufferers pose to each other. Two people with the condition should never meet face-to-face, as their lungs will harbour specific bugs/infections that could cause a serious infection in someone else with the condition. This means no support groups, and requires strict segregation to be in place at CF clinics.

• It is misleading to suggest that there is an 'average life expectancy' that can be applied to everyone with CF. Life expectancy is likely to be different for people of different ages; and this is before taking into account the fact that CF can also affect people differently. The median predicted survival currently stands at 41 years-old.

• CF affects everyone differently, but for many it involves a rigorous daily treatment regime including physiotherapy, oral, nebulised and occasionally intravenous antibiotics, and taking enzyme tablets with food. Some people with CF will have a feeding tube overnight. For those with very poor lung function, daily life can be a struggle as basic tasks can leave them breathless. Some patients use a wheelchair to get around, and use oxygen to help them breathe.

• Great steps forward in CF treatment means that sufferers are leading longer and healthier lives, but many will reach a point where they require a lung transplant to prolong their life. Usually someone would be referred for transplant assessment when their lung function falls to about 30 per cent.

• For more information visit the website for the Cystic Fibrosis Trust: www.cysticfibrosis.org.uk