Cystic Fibrosis, my family and I

February 24, 2012 by
Family, Health   No Comments Yet

IN CONVERSATION WITH:  Jenny Agutter

What a privilege it is to be born healthy. And who amongst us is not a little blasé about it when we’re young?

BobbieLooking back, I know I was. It was the last thing on my mind. By 18, I had already had success playing Bobbie in The Railway Children and I was off to the National Theatre in London. It was an exhausting and yet exhilarating time. I simply took the future and my place in it for granted.

Compare my experience with that of my niece Rachel, who suffers from the incurable disease cystic fibrosis – the illness that Gordon and Sarah Brown’s baby son Fraser has just been diagnosed with. By 18, she was already accustomed to a life taking up to 30 tablets a day, twice daily-bouts of physiotherapy, and endless chest infections.

When I was her age, my life was unfolding in front of me – leaving home meant the chance to be irresponsible and just go with the flow. My niece, however, was facing up to a different reality. Even as she left home to go to university, Rachel had to shoulder the biggest responsibility of all – that, literally, of her own life. It is a credit to her and to her parents, my brother Jonathan and sister-in-law Caroline, that she has done so with aplomb.

Keep them safe, but self-disciplined

From watching the way their family works, I have learnt that there are two things every parent of a CF child should know, as the Browns will no doubt learn, too. They must keep their child safe and teach them self-discipline and sometimes be quite strict about it – but, when the time is right, they must also set them free.

The self-discipline is important. CF is a genetic disease that affects a number of organs in the body – particularly the pancreas and lungs – by clogging them with thick mucous. Symptoms include weight loss, coughs, chest infections and diarrhoea. Sufferers may have a slight salty smell to them as CF causes the body to produce too much salt. It is progressive and the average life span is currently around 31.

Children with CF have to learn very early on that daily physiotherapy is essential as it helps to clear the lungs of mucous. Too much mucous can cause chest infections which can permanently damage the lungs – and this is the biggest threat to sufferers. Rachel knows she mustn’t skip her exercises and that if she has an infection she must take care of it immediately.

Catching CF early is essential if the lungs are to be protected. Rachel wasn’t diagnosed until six months but Fraser Brown will have been picked up just after birth as all babies inScotlandare routinely tested for it. InEnglandandWales, more hospitals are checking young babies (blood is taken from the heel with a small needle), and I know that is on the agenda to be taken up nationally – although it can’t come soon enough.

Rachel’s CF was diagnosed after her parents realised she was having problems with her chest, and that she had a salty smell. It was instinct – they knew something wasn’t right. Since then they have devoted themselves to ensuring that Rachel knows she must take care of herself. Getting her to perform her daily physio but also getting on with life. That’s what the Browns will be doing too – they have said that Fraser is fit and healthy, and that the advice and treatments he will receive will keep him that way.

I imagine what the Browns will be looking to and hoping for is a cure – as we all are. The cystic fibrosis scientific community in theUKis a little unusual in that everyone is working together. There are teams in Oxford and Scotland, and led in London by the brilliant Prof Eric Alton. This means that there is no competition between teams. Instead mutual co-operation has led to the development of a gene therapy – which while not a cure – could mean CF patients enjoying a much longer, healthier life span.

The treatment, which will start human trials next year, will change the lining of the lungs. It could be a huge breakthrough. Parents such as the Browns have every reason to be optimistic as it could easily be available in the next decade.

Gordon Brown has always been supportive of the CF cause, before the disease affected him personally. We now know that neither he nor Sarah had any idea it ran in the family. But it is the most common inherited disease in this country, and more than 2.3million people carry the gene.

I didn’t know about CF until Rachel was diagnosed, so when I was pregnant I was tested and I am a carrier. But my husband Johan doesn’t carry the gene – so that meant my son Jonathan, who’s 15 now, couldn’t have the disease, although he can still be a carrier. At some point we will have to get him tested for that.

Every family affected by CF learns to take every day as it comes, and to make the most of each one. That’s why the Browns mustn’t let CF dominate theirs or little Fraser’s life. They must treat it seriously, but lightly. It was terribly hard for Rachel’s parents when she first went off travelling across the world without them. And even now I have to remember not to nag her about colds. She has her own life to live. We are proud of her independence.

Obviously, this Christmas my wish for Rachel would be to see a cure – for that cloud above all of those affected by CF to be lifted. But failing that, as she’s so outgoing and happy, I’d like to see her healthy enough to enjoy all the season’s parties. Life should be enjoyable and uncomplicated for CF families – I hope it will be for the Browns, too.

More information on Jenny Agutter here

[youtube]http://youtu.be/PEPvfZrXWPY[/youtube]

 

 

About the Author

Victoria Lambert has been a journalist for more than 20 years, and specialises in health and medical matters. She writes for the Telegraph, the Times, the Sunday Times, the Guardian, the Mail and the Mail on Sunday. She contributes to Saga, Geographical and First Eleven magazines – where she is the agony aunt.

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