Daily Mirror (Northern Ireland)
The cystic fibrosis storyline rang so true for my family
Call The Midwife’s Jenny Agutter speaks to JANE SYMONS about how cystic fibrosis has caused heartbreak for her family.
“One of the wonderful things about Call The Midwife is that we are seeing our future being shaped,” says its bestknown star, Jenny Agutter.
“Historically, it is very accurate and the scripts are wonderful at identifying the changes in healthcare which were good, and the problems which are still relevant today.”
But for Jenny, 66, who plays Sister Julienne in the hit BBC drama, one storyline was particularly poignant – a newborn baby with cystic fibrosis (CF).
In the heart-rending episode, a mother was distraught when her child refused to drink, was losing weight and becoming sick. Seeing the baby’s plight, the staff were at a loss as to what was wrong, until Sister Monica Joan kissed the baby and realised he tasted of salt – a side effect of cystic fibrosis.
Set in 1959, at the time the disease was only just beginning to be recognised. It’s a story Jenny knows only too well as she carries one of the faulty genes which causes CF.
And she suspects both her parents were unknowingly carriers, too, as they tragically lost a baby boy and a baby girl – Jenny’s siblings – in infancy. However, it wasn’t until Jenny’s niece Rachel was diagnosed with the disease years later that the family heard about CF.
Cystic fibrosis is one of nine inherited conditions which can be picked up in the routine heel-prick test babies now have in their first few days of life.
But in an echo of the Call The Midwife storyline, it was some months before Rachel was diagnosed because she was not putting on weight.
There is no cure for CF, and until recently, treatment has focused on preventing infection and minimising damage to the airways and other organs. Now there are drugs, such as Orkambi, which can prevent life-limiting lung damage by tackling the faulty genes.
But alarmingly, for sufferers and their families, the Government and Vertex, the company which makes Orkambi, cannot agree on a price.
“It is so hard knowing there is a better medication, which is being funded everywhere else, but that is not available here,” says Jenny.
“When Rachel was born, the median age for survival was about eight, and only a few people got into their teenage years. But in the past 40 years we and kept moving along with the improvements in treatment and care,” says Jenny.
However, she knows all too well what the future might hold. “There are so many different problems that come with CF, but the primary one, and the one which is most threatening, is damage to the lungs.”
An infection could lead to spending months in hospital.
“Every time there is an infection and you can’t move the mucus, there is more damage to your lungs. Eventually, when your lungs are no longer functioning well enough, you go on the waiting list for a transplant. But your lung function has to be down to something like 25%, which would be like trying to breathe through a straw.”
Jenny discovered she carried the CF gene when she was 37 and pregnant with her son Jonathan in 1990. “I really should have been tested before I got pregnant, because I knew I had a 50-50 chance of being a carrier. But marriage and babies came rather on top of one another, and I wasn’t even sure I could get pregnant.”
Fortunately, her husband, Swedish hotelier Johan Tham, does not carry any of the known gene faults, but she admits, “I worried all the way through the pregnancy, and then he was born early, on Christmas Day.”
Jenny, who says she draws on her experiences for her role in Call The Midwife, is about to start work on the ninth season of the popular show.
“We are in the mid-60s, and my memories of that period are so strong – going up to the King’s Road in Chelsea,” she smiles.
And for people with CF, an extraordinary story of scientific breakthroughs and survival against the odds continues – although some of the plot twists are still being played out.