Parents of children with cystic fibrosis talk of care they receive

A child is born with cystic fibrosis or mucoviscidose every three days in France and there are around 7,000 people living here with the condition.
Every September the national association Vaincre la Mucoviscidose organises an awareness day when events are held throughout the country to raise money for research. This year Virade de l’Espoir or Action for Hope day is on September 25.

Helen Mann lived in France until recently with her daughter Clara who has the condition and has described what it is like to live with a child born with cystic fibrosis in a book called Love Like Salt*, published this year.

The title is aptly chosen because of the nature of this genetically inherited disease. “Salt is unable to pass easily from one cell to the next, with the result that the body’s balance of salt and water is upset,” she said.

“This leads to a build-up of thick mucus and consequent infections in the body’s organs, particularly in the digestive system and lungs.

“It requires intensive treatment by physiotherapy and medication. Although until relatively recently it was unusual for ‘the child whose brow tastes salty when kissed’ to survive into adulthood, new treatments have improved the outlook considerably.”

There are about two million healthy carriers of the cystic fibrosis gene and when both parents carry the gene there is a one in four risk of their child having the disease.

It is an invisible disease because there are no outward signs. However people with cystic fibrosis need around two hours of treatment every day including up to 1.5 hours of physiotherapy, which can rise to six hours when needed, and they have to take around 20 pills every day.

The condition affects their lungs but also their digestive system and they need to take enzymes to help them when they eat. They are extremely vulnerable to infection and frequently have to take antibiotics.

The Mann family lived in a village in the Lot for seven years and found that the medical treatment they received was excellent. There are 45 specialist centres in France for patients and treatment is free as it is one of the illnesses for which there is 100% cover by the health service.

They did however have problems at primary school when Clara was in her last year before collège and was bullied and became unhappy.

Mrs Mann says she did not feel the school tackled the problem adequately and was afraid that the situation would become worse in collège where there is a lack of pastoral care in some French schools and children are encouraged to conform rather than allow their individuality to flourish. A child with cystic fibrosis cannot help but be different.

Eventually, despite having loved life in rural France the family decided it would be best to move back to the UK where they were lucky to get a scholarship in a Quaker private school where Clara is now flourishing. She also took the brave decision to take part in trials for a new drug called Orkambi which has made her much healthier.

Another English family who have stayed in France with their son who has cystic fibrosis and who was born in their first year here near Samatan in the Gers say, that though there were times when they were tempted to move back to be closer to family, they decided to stay. Jack is now 13 and at collège and his mother, Stephanie Johnson stays at home to look after him and receives a carer’s allowance to be able to do so.

“We made a choice to stay here. It has not been an easy ride but I can’t fault the medical treatment Jack has received. We have been lucky with schools as teachers at both the primary and secondary schools have been understanding and Jack has never complained of bullying, but if he did I am confident the teachers would deal with it sensibly.”

Life has been hard though: “It has been a struggle and it still is a hell of a struggle. People say to me that Jack looks well and he must be fine,” says Stephanie Johnson. “But it is difficult for them to realise that sometimes you are just working hard to keep him going until the next hospital visit. Every morning we wake at 6.30 and he has his first nebuliser and then 20 minutes of physiotherapy to clear his lungs of mucus.

“I then drive him to school because the bus leaves too early for us. I have to be on hand to go and fetch him at any moment when he may fall ill. In the evening he has to have another physiotherapy session which I carry out because it is easier to fit in than going to see a professional and I now know exactly what he needs from day to day.”

She says that despite it all Jack remains cheerful: “He is the most delightful child. I don’t know how he does it but he is always up beat and even when he is feeling ill he remains joyous.”

She feels that awareness is better than in Britain: “I have never met anyone who didn’t know what mucoviscidose is even though they may not understand all about it.”

There was huge publicity about the condition when a singer with CF who had won Star Academy, Grégory Lemarchal died at the age of 23 in 2007.

Vaincre la Mucoviscidose has 7,700 members. It was created in 1965 and is the biggest provider of finance for research and has enabled considerable advances.

Life expectancy used to be seven years for children born in 1965 and is now close to 50 years for those born today. Since 2014 new born babies in France are screened for the condition so it can be treated immediately if necessary. There is as yet no cure but lung transplants are an option which are becoming increasingly successful due to progress in surgical techniques and the association campaigns to attract donors.

There have been huge advances in drugs like Orkambi which Clara takes, though it is not suitable for all types of CF, and gene therapy may be able to provide a cure in the future.

Every year the Virades de l’Espoir raises valuable funds for medical research and patients care. More than 30,000 people take part and there are around 400 virades nationwide which vary from walks, marathons, bike rides and other non-sporting events. You can take part whether you are a member of the association or not. For details of events near you see virades.org or vaincrelamuco.org

• Love Like Salt is written by Helen Stevenson (her writer’s name) and printed by Virago.