Living with Cystic Fibrosis: Fleur’s Journey and New Treatment Options

Rianne has bladder and bowel problems and uses a toilet pass: ‘If I have to, then I have to’ Read also

New drugs

Their daughter is now seven years old and Fleur still suffers from enormous stomach pains. “So much that she doesn’t want to talk about it. She thinks it’s kind of normal. It belongs to her, she says. Everyone thinks that cystic fibrosis mainly has to do with the lungs. But it has to do with all kinds of organs. Like the intestines, the pancreas, you can develop diabetes and it’s also a lot of coughing and phlegm. Summer is better than winter. Then she is much more sensitive to respiratory infections.”

Fleur with all her medication. Text continues below the photo.

Last year there was a new drug on the market that would make the disease chronic. That meant that Fleur’s life expectancy would increase and she would no longer be terminally ill. Fleur started it last year. But unfortunately she became so tired from the medication that she could no longer function. Going to school or playing with her friends was no longer an option.

“The quality of life for her decreased enormously. Then we had a conversation with Fleur. It was a choice between always a stomach ache or always tired with the result that she would hardly be able to go to school. She said, “Then do the stomach ache, because I already know that.” Then we stopped the meds and went back to the old. Then you choose quality of life. And eventually we can try again, but for now it is not an option to repeat this.”

Normal

Fleur also has a sister, Suze (2 years old), and unfortunately she is also ill, although she does not suffer from cystic fibrosis. “It is double bad luck, but luckily she is not terminally ill.” She has gastric emptying problems (a stomach disorder that causes various stomach complaints). Suze has a PEG probe for this, a tube between the skin of the abdomen and the stomach for food and medicines. “You can grow old with it,” says Marloes.

“Because her sister is also ill, Fleur thought it was normal for every child to have to take medication. Or she asked, “When do you have to go to the hospital.” Now she’s getting to an age where she realizes it’s not normal. That she is terminally ill and may not live to be older than forty or fifty I do not yet discuss with her. But I know the question will come. I don’t know what to say or do.”

The pick-up

Last week, Fleurs’ biggest dream was fulfilled by the Opkikker foundation: marrying mom. The whole day was all about Fleur. The family was allowed to play the drums with the music band, they were given a chocolate making course, there was a castle with a footman. The ceremony was with white chairs and balloons. There was a singer, there were rings, children’s champagne and so on. “Everything had been thought of, it was just like a real wedding. Fleur immediately started crying when she saw all the family and friends. And she never cries. She thought, ‘What’s happening to me now?’”

Marry mom. Text continues below the photo.

“On the outside you don’t see anything about her. But when it’s there, it’s there. Last winter she was in so much pain. Then she asked, “Mommy, am I going to die?” Then you are afraid that something will go wrong. But such a day from the Opkikker Foundation ensures that you can get back to it.

The way things are going with Fleur now, it’s going well. We can deal with this. The support of our friends and family also makes it a bit more bearable for us. In addition, developments in the medical field are moving very quickly. Hopefully by the time Fleur gets older there will be a drug that she can tolerate and that will extend her life. But even now I am happy and proud of our family. I see a bright future.”

Three sisters, one rare hereditary lung disease: ‘You are not advised to have children’Read also
2023-06-28 13:29:46
#Marloes #daughter #cystic #fibrosis #incurable