For 10-year-old Phoebe Bolton, keeping active every night is extremely important. It is never a chore as she knows it is vital to keep on top of her condition. Her mum, Sharron, speaks to NATALIE WALKER about how her daughter copes with cystic fibrosis.
Sharron Bolton, of Walton-le-dale, near Preston, had a 25 per cent chance of giving birth to a child with cystic fibrosis.
Having had her first child, Benjamin, now 15, without any complications, she had a test to determine whether her second-born would have cystic fibrosis.
As tests came back positive, Sharron began dedicating her life to ensuring she was able to support her and give her the best care at home.
She says: “There is a one in 25 chance of someone carrying the faulty gene, but you don’t know as you are never tested for it.
“My ex husband - Phoebe’s dad - has a nephew with cystic fibrosis and so he was screened, along with myself and it turns out we both have the faulty gene - DF508.
“I knew that every pregnancy I had, I would have a one in four chance of having a child with cystic fibrosis.
“I opted to have the amniocentesis test during both my pregnancies.”
Phoebe was placed under the care of Manchester Children’s Hospital, where she attends three monthly clinics and extra visits for chest X-rays, blood tests, ultrasounds and physio fitness tests.
Sharron says: “Phoebe’s life is affected in many ways.
“Her morning routine is to have a shower as she secretes a lot of salt and so sweats during the night.
“Then she has to have 20 minutes of physiotherapy on her lungs and she has to take a nebuliser.
“She has a high fat breakfast and she has to have eight different medications just for that one morning meal.
“Her condition means she doesn’t absorb fat in the diet, meaning she can become vitamin deficient.
“Her child’s body burns the equivalent of an adult’s intake, so she needs more fat and protein.
“She has to take Creon tablets (pancreatic enzyme replacement) for every four grams of fat. For example, if she has a chocolate bar, we look at the fat content. If it has 12g of fat, she has to take three Creon tablets in the hope it allows fat to be absorbed so she can gain weight.
“She also has to have special medicine because it can cause problems with her liver.
“Mucus forms on her lungs and she is prone to chest infections.
“Phoebe gets very tired because we have to keep her active. She has to do a minimum of one hour of exercise a day to go with her physiotherapy.
“She goes to Nuffield Health in Preston with me, which delivers an exercise programme for young people who have cystic fibrosis.
“She also does dancing, drama, singing lessons and plays hockey.
“So she has a very busy life, on top of her homework.
We don’t have the mindset where we dwell on Phoebe’s condition. We see it as it is.
“If the medical team at Manchester tell us to do something, we do it. Phoebe knows that if she doesn’t do it, she will get poorly.
“She knows nothing else. She is so disciplined and she has learnt to accept it.
“She knows she will be tired and there is no cure.
“She has been poorly, but at the moment she is going through a good patch where she is not ill.
“Until recently Phoebe had an outreach nurse from the Lancashire Teaching Hospital to flush her Port-A-Cath, a device under her skin which fed into her heart to allow us to administer medications.
“She has had three of these devices - her first at just nine months of age. Until two months ago, she had always had a device, but it has been removed because Phoebe is currently so well that it is not needed. Should her health deteriorate then a similar device will be put back.
“But we would be foolish to think we can take the foot off the gas. It’s our way of life.
“All cystic fibrosis suffers will at some point find themselves on a transplant list.
“This condition does not improve. It is a daily degenerative disease, so it is about continuing on an aggressive and overactive lifestyle to stay healthy that gives them a fighting chance to get to adulthood and try to live a life that others take for granted.”
Determined not to let her condition hold her back, Phoebe has cycled the length of the Guild Wheel to raise funds for Cystic Fibrosis Trust. Sharron, 43, adds: “I work at Lancashire Teaching Hospitals as a professional theatre nurse in the oral and maxillofacial surgery (MAXFAX) theatre.
“One of my colleagues lost her niece, Donna Lloyd, last year, while waiting for a lung transplant. She was aged 35.
“We said we would raise funds for the Cystic Fibrosis Trust and walk the length of the Guild Wheel. Phoebe and I decided to do a practice run and she cycled it.
“It was a challenge for Phoebe but if a child with cystic fibrosis can do it, we hoped it would motivate others to do it.
“She was elated after she did it.
“The official fund-raising 21-mile walk raised more than £2,100, with 24 people taking part.”
St Patrick’s Roman Catholic Primary School, Walton-le-Dale, has also supported Phoebe by holding a Wear It Yellow Day and a cake sale during cystic fibrosis week.
Pupils raised £175 for the charity.
Sharron says: “Everyone has been fantastic in raising money for such an important cause to us.
“Research and treatments are vital in helping to save lives.
“For example, a colleague’s friend has recently had a baby whose cystic fibrosis was picked up at birth from the heel prick screening. Years ago, he would have been undiagnosed and suffered until symptoms such as poor weight gain and recurrent chest infections would get a GP to refer for testing. This could take a few years for a referral and many courses of antibiotics, because the GP would not immediately think of cystic fibrosis, but a child picking up coughs and colds.
“That family had no idea they carried any faulty genes.”
To support Phoebe donate to http://www.justgiving.com/fundraising/sharron-bolton.