Living with CF

We were over the moon when our second daughter was born in 2009 but within two days of the birth we were sent to the hospital. Looking back, we had prepared ourselves for a diagnosis of Cystic Fibrosis as we’d dealt with meconium ileus throughout pregnancy, but we had no family history of the disease and the little knowledge we did have came from television shows.

I was really unwell for more than a year after the diagnosis and I mourned for my daughter; the diagnosis felt like a ticket for a lifetime of hurdles. We were filled with anxiety and the disease only seemed to progress which made it feel like she would have fewer possibilities and more restrictions put on her life compared to the kid next door. Despite these hurdles, we’re very lucky to live in a great welfare country like the Netherlands. We have a great cf-team and access to all the registered therapies that we need. In the Netherlands there are 1500 patients who receive care in 7 different CF centers that are spread across the country.

The first few years with a baby/toddler with CF were hard; they were mostly dominated by severe and prolonged eating difficulties as well as recurrent lung infections. After one such lung infection, our daughter refused to eat and drink anything for more than a year. We kept offering her different foods and drinks, but she would only throw it away, close her mouth or spit it out. It drove us crazy. Some months later she was fitted with an NG-tube and a mic-key button. Changing the NG-tubes was really traumatic for us all; even seeing stories and pictures about tubes nowadays can directly put us back to that awful time.

As the eating problems didn’t seem to be resolved we decided to start with our last hope; an intensive eating program.This would last for nine months and take place five days a week – all this when she was only two years old. After this programme, she had to eat a thick liquid diet of food of drinks that we had to feed her with a spoon; but the good news was that she wasn’t refusing food anymore. We started her on the drug Orkambi at nine years old and now she eats and drinks by herself. We can actually enjoy meals together without any stress.
The other hard thing that we had to get used to was the breathing treatments; here in the Netherlands we don’t use the vest, but instead nebulise with hypertonic saline and pulmozyme. This can be hard for babies and toddlers, but she got used to it and we are very compliant. I have great respect for her doing this 365 days a year, twice a day. I’m not looking forward to adolescence so much!

As she has grown up, our girl has shown great character; she’s willing, spontaneous, has a great sense of humor, loves playing athletics and hockey, and has many friends at school. She is more than just a little girl living with CF.
Over the years as a family, we’ve adjusted to living with a chronic disease; we deal with misfortunes and feel satisfied when things don’t turn out as bad as we’d expected. We also enjoy spending time fundraising which brings us more opportunities to connect with other CF parents. In recent years since CFTR modulators and a greater variety of antibiotics have reached the market, we’re hoping for a better life for our daughter and as a family.

In 2008 our daughter has started with a modulator and in 2022 the next generation modulator. It was worth the waiting; since then the symptoms decline and she is living a better life. We are thankfull for the medical progression and we suffer to know that there are still patients who won’t benefit, don’t require or the medicals are not available in their country yet. But overall the future is more bright for the whole CF community.

Silvia van Engelen, the Netherlands

Life with CF in France.

My name is Audrey, I’m 32 years old and I live in France.

I was diagnosed with Cystic Fibrosis when I was born and I’m lucky to have had access to most of the therapies and clinical care for free all of my life. I have also been fortunate to have a team of specialized doctors and nurses in a CF center.

In the next few years, at least 75% of the patients living with Cystic Fibrosis in France will reach adulthood and it feels like the landscape will change dramatically for people with CF. I can spend time with my friends with CF in France as long as we are all wearing masks and we ensure that we have disinfected our hands.

On the whole, my health is in a relatively good place and it has been made better for being able to access Orkambi and now Kaftrio. My BMI is 20.5 and my FEV1 is 84%. I’m pleased to say that I had only one course of IV antibiotics since 2016 when I used to have them every 6 months before CFTR modulators. If I need a lung transplant one day, it probably won’t be too difficult to arrange thanks to the organisation of care in France.

I am thankful that I am able to work full time as a scientist for the National Centre for Scientific Research after obtaining 2 degrees in genetics and microbiology. I also volunteer for the French CF patient organisation and CF Europe as an ‘expert patient.’

I am very conscious that I have the life that many European CF patients dream about. Even though I live with CF, it doesn’t control my life. I am free to work, travel, I can date and spend time with my family and friends. It is maybe not what some people would call a ‘normal’ life – but what is normal anyway?

My motivation for helping patient organisations is the idea that one day, every CF patient will be able to have a ‘normal’ life outside of their treatments.

Unfortunately it’s not as simple as this.

Despite the many good sides of our care system, one of the main problems in France is having access to private insurance. If we are denied private insurance then we are unable to apply for a mortgage to buy a house, we are denied travel insurance, and sometimes we can be denied car insurance too if we have diabetes.

When we have to fill out the questionnaires to apply for insurance, the first question we are asked is: ‘Do you have a chronic disease?’ I can either tell the truth, knowing that I will not be covered, or lie, which would be a big risk if anything happens. Either way, it’s not an optimal situation.

As for living with CF, my condition is stable thanks to my medication – I work and contribute to society as anyone else would. Despite this, I am not allowed to enjoy everything that healthy people are allowed to. This is why we still need to work towards an actual cure for CF; I won’t be satisfied with a pill that stabilises my condition, even if I am grateful for it.

I want to be able to discover the smile of my CF friends without a mask on.

Audrey, France