Jenny Agutter: Actress on the threatening condition she carried the mutated gene for

Call the Midwife: Jenny Agutter discusses clothes on the show

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When the beloved BBC drama Call the Midwife, in which Agutter plays Sister Julienne, featured a storyline about a baby born with cystic fibrosis, the actress spoke out about her poignant family connection. In the heart-rending episode, which aired back in 2019, a mother was distraught when her child refused to drink, was losing weight and becoming sick. Seeing familiarities within her own family, Agutter shared that she carries one of the fault genes which causes CF and suspected that both of her parents did too after they tragically lost a baby boy and a baby girl – Agutter’s siblings – in infancy.

CF 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. And thanks to this test, Agutter’s niece Rachel discovered that she carried the mutated gene shortly after her birth.

“The first I knew about cystic fibrosis was when my brother rang having discovered that his daughter, his first child, had CF,” she said in a past interview.

“When she was born he was absolutely delighted, they were both over the moon. And then
they went through three or four months of seeing her struggle to survive.”

When inherited, CF causes sticky mucus to build up in the lungs and digestive system. This causes lung infections and problems with digesting food.

Symptoms usually start in early childhood but vary from child to child. However, the condition is guaranteed to get slowly worse over time, with the lungs and digestive system becoming increasingly damaged.

“When Rachel was born, the median age for survival was about eight, and only a few people got into their teenage years,” Agutter added, explaining the toll it had on the family at the time.

“But in the past 40 years we have seen so many changes in medication, therapy and ­treatments for CF.”

Against all odds, Rachel recently celebrated her 40th birthday, a milestone her family once thought was impossible.

Agutter continued to say: “She has worked hard to remain healthy and kept moving along with the improvements in treatment and care.”

However, even with improvements in care for CF, Agutter has had to witness her niece’s anguish at trying to keep the condition at bay. She added: “My niece has been in and out of hospital but I think what the most scary time is when suddenly a CF sufferer does actually grow up and is suddenly in charge of their own life.

“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. 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 percent, which would be like trying to breathe through a straw.”

In addition to recurring chest infections, other symptoms of CF can include the following:

  • Wheezing, coughing, shortness of breath and damage to the airways (bronchiectasis)
  • Difficulty putting on weight and growing
  • Yellowing of the skin and the whites of the eyes (jaundice)
  • Diarrhoea, constipation, or large, smelly poo
  • A bowel obstruction in newborn babies (meconium ileus) – surgery may be needed.

Agutter herself decided to have herself tested for CF when she became pregnant with her son 20 years ago. The test revealed that, like her brother, she was also a carrier of a faulty version of the CF transmembrane conductance regulator gene (CFTR), which causes the condition.

Luckily her husband did not carry the same mutated gene, meaning that their children were not at risk as it takes two people with the one single gene to create this problem with CF. However, this did not mean that the run of CF in her family would stop as after Rachel’s diagnosis, Agutter’s great-nephew, Albie was also diagnosed.

Due to this Agutter became actively involved in her role as a patron and trustee of the Cystic Fibrosis Trust and remains optimistic for a better future for both sufferers and their families. She commented: “The Cystic Fibrosis Trust’s work is vital, providing life changing world class research, day to day care and support as well as campaigning for life saving drugs for all.

“The very exciting thing right now is that they are looking at changing the make up of the lung with gene therapy.

“Most people who have CF die with lung disease – about 90 percent. So if you can keep the lung healthy you can basically create an ordinary life for the CF sufferer.”

Although there is currently no cure, treatments for CF as described by the NHS include medication to help lung problems, airways clearance techniques, dietary and nutritional advice and lung transplants.

The Cystic Fibrosis Trust also notes that exercise is particularly important for managing CF. This is because it can help clear mucus from the lungs and help to improve overall health. Maintaining flexibility, particularly around the ribs and shoulders, is also important. Older children and adults may need to do stretching exercises to preserve full movement in the joints and muscles around these areas.

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