Tuesday, 14. May 2019
When parents learn that their child has cystic fibrosis (CF), it usually comes as a shock. Everything points to a short life expectancy, extensive treatments, and enormous suffering. We talked to parents of children with CF about their experiences: What was your path to getting the diagnosis? How did you handle the difficult diagnosis? What helped you to accept the CF? The conclusion: After an emotional low, hope, composure and the ability to enjoy life return.
Bennet was 21 months old when he was diagnosed with CF via a sweat test. He was tiny, much too small for his age, and had already had pneumonia once. Still, it took his mum, Christine Buchholz almost 2 years trekking from doctor to doctor before she finally knew what was wrong with her child. ‘The doctors didn’t take me seriously and always found different reasons for Bennet’s symptoms. They said when the mother is petite, so is the child. The pneumonia and breathing problems, they said, were because he had inhaled amniotic fluid during birth. But I always knew that something was not right with my son’, explains the mother of the now 12-year-old boy.
After worrying for so long, Christine finally had an answer. She says, ‘In the beginning I was shocked how short my child’s life might be because of the cystic fibrosis. But I actually was more relieved to finally know what was wrong with my son and to be able to help him. He quickly started feeling so much better after starting the therapy’, she recalls.
For the Krieger family, things were very different. ‘We were happy, and everything had gone perfectly up until then. Love at first sight, marriage, I got pregnant right away. Our son was a good size, happy and, according to our paediatrician, generally healthy. And then we were dealt a terrible blow’, says Mandy Krieger. At the age of 6 months, Leon was admitted to hospital for what was thought to be a stubborn gastrointestinal infection. A few weeks later we had a diagnosis: Leon had CF. ‘It was a shock. We were in a very dark place. We cried a lot and asked ourselves, “Why us? Will we survive this as a family and as partners? How do you arrange your life when you have a child who might only live for a few years?”’. Many families experience shock, despair and feel overwhelmed when they receive a diagnosis of CF.
‘The first few months were very difficult for us. The situation overwhelmed us all. My husband and also the kids’ grandparents were kind of paralysed’, reports Nadine Sachse. It was very hard on the family. After the younger of the two sons was born with an intestinal obstruction, and it soon became clear that he had CF, the older brother who had been thought to be healthy, was also tested for CF. The test was positive. ‘Within the space of only a few weeks, we learned that we had two critically ill children’, explains Nadine. She continues, ‘It was really hard. You only hear negative things about cystic fibrosis. Everything you hear makes it seem like it’s a serious, incurable disease that leads to an early death. The doctors overwhelm you with information on treatments, hygiene measures and the things that could potentially harm your child. All I could think was how can I possibly manage all of this? How am I supposed to fit all of the therapies into our daily routine?’.
‘We needed around 6 months to process both our children’s diagnosis’, recalls Nadine. ‘The CF clinic provided a psychologist who also came to see us at our house. We all sat together, my husband and I, and our parents. We talked and cried together, which helped us’.
Mandy Krieger had a similar experience, talking to others was crucial in the months after the diagnosis. She says, ‘It was unbelievably helpful to talk openly about the fact that our child had cystic fibrosis, and not to hide anything’. Also, talking to other affected families helps to ease suffering. Mentor families, parent meet-ups and sharing information on groups on social media can make CF and the anticipated overwhelming demands seem less scary. For example, Mandy explains, ‘You get a lot of practical information when you talk to other CF families. Over time you also see that everyone deals with the disease differently. Everyone has to find their own path and, in every situation think about what is the right thing to do for themselves. For example: Many parents believe children with cystic fibrosis should never use a hot tub because of the potential risk of exposure to germs. Others are more relaxed and want to make it possible for their kids to have a life that is as free and normal as possible despite their disease.”
After a while, CF is no longer scary. With many children one cannot even tell they have the disease. They live normal lives except for their treatments. Nadine reports, ‘Even if it seems unimaginable in the beginning, you get used to it. You adopt a more relaxed attitude toward dealing with the disease and find ways to integrate the cystic fibrosis into your life. This also includes nebuliser therapy for CF that has to be done two or three times a day and can take up to half an hour each time. In addition to inhaling saline solution and, in some cases, medications that dilate their airways, children with CF often need to inhale antibiotics. Parents can feel overwhelmed in the beginning by the labour-intensive therapy, which with the nebuliser therapy involves taking and calculating enzyme capsules and other pills. But parents learn to cope with this. ‘In the morning before school and nursery we perform the therapy using the eFlow [link to eFlow page] because it is faster. In the evening, we do a longer and more intensive session with the PARI JuniorBOY SX [link to PARI JuniorBOY]. It is important that the therapy becomes part of the daily routine and not a constant source of stress’, advises Nadine. Christine Buchholz notes, ‘While the therapy is intensive, you always have to remember that the kids feel better as soon as they are diagnosed and start the treatments. After a while, nebulisers and medications just become a regular part of daily life’.
The following were helpful to other parents when they learned their children had cystic fibrosis:
Note: The statements made in the report are the individual view of the person reporting. They do not necessarily reflect the PARI view or the general state of science.
An article written by the PARI BLOG editorial team.