Although Sue Kruk’s eldest son died when he was just 33, she feels lucky to have had him for so long.
Like all parents who have children born with the life-limiting condition cystic fibrosis, she and husband Zyg knew the time they had with their boys would almost certainly be cut short.
But rather than focus on the fact they were powerless to change, they were determined to live for every single day.
Although Sue insists they’ve never wanted cystic fibrosis to define any of their lives, it has been a constant presence.
And now she’s urging others to get behind an appeal that comes too late to help her oldest child, Ziggy, but could help extend the lives of others born with the disease.
The Cystic Fibrosis Trust is desperate to raise £6m by the end of this month to fund an innovative gene therapy trial (see panel).
For Sue – a long-time supporter of the CF Trust – it’s an opportunity not to be missed.
The trust has already raised over £30 million to fund the research but is now looking at a financial shortfall that could put the trial in jeopardy.
‘CF still comes as a bolt from the blue because carriers, one in 25 of us, are healthy and do not know beforehand,’ explains 68-year-old Sue.
‘Both parents must carry the gene before a CF child is born. It will be tragic if all the work in Portsmouth over the past
40 years goes to waste.
‘It will be tragic if future generations have to suffer for a relatively small shortfall in funding now.’
Sue found out that her sons, Ziggy and Xavi, had CF on the same day.
It was February 1970 and Xavi had just been born. Ziggy, who was coming up to his second birthday, had already been having tests to determine if he had CF and the results had just come through.
‘I’d given birth to Xavi and he was taken away for observation while my husband went off to talk to the consultant about the results,’ remembers Sue, from Hayling Island.
‘Ziggy had just been a bit on the slim side and I felt there was something not quite right. I was being treated as a fussy mother but I wanted to get to the bottom of it.
‘There had been a big poster for Cystic Fibrosis Week in Albert Road the year before. The little boy in the poster had dark eyes and pale skin that reminded me of Ziggy.’
A sweat test had been carried out on Ziggy because when you’ve got cystic fibrosis, you’ve got more salt in your sweat than is normal.
And in just a short space of time, Sue and Zyg discovered that both their newborn son and their toddler had the disease.
CF affects more than 8,500 children and young adults in the UK and five babies are born with the condition every week.
It affects the internal organs, especially the lungs and digestive system, causing them to become clogged with thick, sticky mucus.
CF is caused by a faulty gene that controls the movement of salt and water in and out of cells in the body. Sadly, there’s no cure, and those who have it find they can get recurrent chest infections and breathing problems.
Parents often have no idea that they are carriers of the CF gene until their child has been diagnosed.
‘We were in a state of shock,’ says Sue. ‘It’s a devastating piece of news. I’d got an out-of-date book from the library and read the worst.
‘The paediatrician was lovely. Lots of parents at that time were being told there was a chance their children would live until they were three or five.
‘He said the boys seemed to have it mildly and maybe they’d live until they were 11 or 12.’
Doctors knew a fair amount about CF in the 1970s but knowledge has come on leaps and bounds since then. Thanks to a better understanding of how physiotherapy and exercise can play a part, the life expectancy for those with CF has been rising over the years.
‘There’s a period of grieving and then you get on with it, you have to,’ says Sue. ‘I live for the day, we lived for the day.
‘We moved to London and they had pretty good childhoods. It was hard work as it took an hour in the morning and an hour in the evening to do the physiotherapy.
‘When they reached early teenage-hood they started to get more chest infections.’
She adds: ‘I have never wanted my life – their lives – to be defined by cystic fibrosis.
‘They started to have more hospital treatment but they still went to Cub and Scout camps – it’s just we would have to go and camp too and they’d sneak in to see us.’
Sadly Ziggy’s health did start to deteriorate as he got older and he began spending more time in hospital. He died in September 2001 after contracting MRSA. He’d been in hospital for three months before he passed away.
‘The thing with cystic fibrosis is you’ve got to do exercise to prevent yourself from getting ill,’ says Sue. ‘Because of physiotherapy and exercise Ziggy was always very agile. He would go off sailing at one stage and that would mean taking his nebuliser with him.’
She adds: ‘I had him for so much longer than I thought I was going to have him. When you read about people with cystic fibrosis they can achieve such remarkable things. He had a happy life.’
Xavi is now 41 and has to work hard at keeping well. He’s keen for the gene therapy trial to go ahead and hopes that it can help others diagnosed with the disease.
‘We started off supporting the Cystic Fibrosis Trust because we’ve got children with it but along the way you meet so many other families and people who support it for their own reasons,’ says Sue.
‘There are so many people who haven’t got cystic fibrosis in their family but who might have it in their family one day because it is something that is one of nature’s cruelest tricks.
‘One in 25 people are carriers of the faulty gene and if two carriers start a family, each baby has a one in four chance of having cystic fibrosis.
‘All the people involved with the trust know that they’re not just doing something good for themselves but for others as well, for future generations. We don’t want all that to go to waste.’
Sue Kruk is a member of the Portsmouth and Isle of Wight branch of the Cystic Fibrosis Trust and its members meet regularly.
When her sons were diagnosed, she says the support group gave her hope and support.
It was set up in the 1960s and although its members are spread out over a wide area, they still come together for events and monthly meetings at each other’s homes.
‘We meet up and take along newspaper cuttings and have a chat about local news,’ says Sue.
‘It’s very much on an informal basis. We meet up, have a chat and at our next meeting we’ll talk about how we can raise money.’
To get in contact, email Sue at email@example.com or contact the Cystic Fibrosis Trust on 020 8464 7211.
GENE TRIAL HOPE
While the Cystic Fibrosis Trust stops short of hailing its gene therapy trial as a potential cure, it insists that the work being done might give people a chance to enjoy a better quality of life.
Thousands of people are living with the genetic condition across the UK and it’s hoped that the trial might extend life expectancy and reduce the amount of time they’d need to spend in hospital.
The UK CF Gene Therapy Consortium – funded by the Cystic Fibrosis Trust – has been working for 10 years to find a way of inserting a healthy gene into a CF patient’s lungs to ensure that the damaging effects of the faulty CF gene are stopped in their tracks.
They are now ready to test the product on patients – but must raise £6 million to now fund the trial.
Matthew Reed, chief executive of the Cystic Fibrosis Trust says: ‘We have, to date, raised well over £30 million to develop this ground-breaking treatment which has the potential to lengthen a CF patient’s life by many, many, years.
‘We are hoping that the funding for this trial will be made available to us by October 31 so that we can give thousands of CF families throughout the world hope in achieving a better and longer life.’
If the money is raised, the trial will go ahead in spring 2012.
Donations to the appeal can be made at virginmoneygiving.com/CysticFibrosisGeneTherapy-1
For more information log onto the Cystic Fibrosis Trust’s website, cftrust.org.uk