Cystic Fibrosis (CF) is the most common fatal genetic disease affecting Canadian children and adolescents, with estimates of up to 100,000 people globally with the condition. Average life expectancy is about 35-40 years, but may be much lower for people in countries with poor or no developed healthcare. Currently, there is no cure for CF. Early diagnosis is key, as well as preventive treatment for a longer, and better quality of, life.
The Cochrane Cystic Fibrosis & Genetic Disorders Group aims to put together quality evidence from randomised controlled trials to help individual patients and their families, spanning interventions from medications to physical activity, and everything in-between.
This week’s blog is by Tess, a nine year old girl from the UK, who talks about what it’s like to have CF, and some of the challenges and successes of navigating her condition at school.
People think I’m a normal, healthy girl when they look at me. That’s wrong. I have Cystic Fibrosis (CF). It’s a little like having asthma because they’re both to do with your lungs, and with CF you’ve got it for life. On my first day of my new school, like most new kids are, I was scared and worried. You see, I had only just started year 4. My class didn’t have a clue that in their class was one of the 10,000 people with CF. They only found out that I had it when I did a presentation about it! After that, it was questions, after questions, after questions until the teacher hauled me to safety. Since that day, nobody teased or bullied me or my friends. I knew they knew that really, underneath the disease, I was just a normal fun loving child.
With Cystic Fibrosis comes responsibility, treatment, and medicine
There are several things I have to do at school to prevent illness. My first is anti-bacterial gel. It’s gloop-doopy stuff that (apparently) kills 99.9% of bacteria. Next, water. You’re thinking I might have to do a strange ritual, but no, all I have to do is drink gallons of it!!! Then, the school day goes on and it’s lunch time. Drink another gallon of water and juice from my lunchbox and go outside to play. Me and my friends play EXTREMELY energetic games and this is good for my health. You need to be fit if you have CF!
After lunch, I’ve got to head back inside for my physio. I don’t get pummelled on the back, no, I blow in a mysterious plastic machine called a “PEP”. Boring right? Well, imagine blowing into that 10 times, huffing and coughing and then repeat that sequence SIX times!!!!! I float into the land of boredom whenever I do it. Then, I get back to class and get changed for PE (Physical Education). As I said earlier, anything energetic = big thumbs up for CF. I put my best efforts into this. Then, back at the classroom, I get changed and learn about music or the terms topic. And that, my friends, is a complete day at school for a person who has Cystic Fibrosis! 🙂
There are lots of dangers for me at school
When I started school at The Batt, I went round the school with Mrs. Smart (the teaching assistant in my class) and pointed out hazardous zones. Some were drains or plants in the class. When I went round school, I did my very best to avoid these places. The teachers helped by moving the plant out of the class room and into 3P (the neighbouring class). Mr. Peacey was very kind and said he would look after it. I’ll let my Mum and Dad explain more about this:
“Each time Tess or Zac (who also has CF) move school or class we go in to meet with the class teacher and other adults involved in their care. We also invite the CF nurse to attend to give the school opportunity to ask questions and feel they have access to support if they are unsure of anything. Really it is just about keeping open communication between us, the school and Tess or Zac so that everyone is happy.
The main things we have to make them aware of are:
Hand hygiene – hands need to be washed after playing outside, touching shoes, seating on the floor, touching soil or animals or similar things. After going to the toilet and before eating anything. If they can be encouraged to use anti-bacterial hand gel as well all the better.
Drinking – it is important that they keep well hydrated as they sweat more than other kids and it helps with their bowels. This is always the hardest one to encourage.
Avoiding coughs and colds – it is also impossible to avoid catching coughs and colds from other kids as they are taught to share so well. We do ask the teachers to advise us though if there is a child in class with a particularly bad cough or cold then we can decide whether to keep Tess or Zac at home to avoid it. This would depend on how fit they were at the time and how many colds they had had recently. Often we spend all autumn and winter going from one antibiotic to another.
Medicine and Physio – there are medications each day that are taken at school administered usually by office staff or a teaching assistant. These are syrups, capsules or mixed into drinks. They also have some physio at school to alleviate pressure on time at home.
Hospital time – we see specialists for various parts of their care pretty regularly. There are Physiotherapists, Dieticians, Gastro teams, Psychologists and the Specialist nurses and doctors. This can take quite a bit of time away from school, more if they are admitted, but we ask the school for flexibility in school work and work to take home if necessary.
Avoiding environmental bacteria and moulds – people with CF are susceptible to bacteria and moulds that others may not even be affected by. Pseudomonas is a big one. It’s so common as its found in all damp places and drains. We encourage the school to pour Milton (a sterilising fluid) down sinks regularly and not have taps that direct water straight into the plug hole as this can aerosol any bugs down there. We also encourage the schools to use disposable cloths rather than have damp ones lying around the classroom. No standing water anywhere on site, such as tyres in the playground, water butts, stagnant ponds but even water tubs for the kids to play in need changing every day. No fish tanks or any animals in the class at all. Moulds are also a hazard, so damp leaves, old buildings with damp walls can be a problem, hay is a definite no go area so farm trips we need to supervise.”
When my brother Zac got pseudomonas (he was 3 then) I wasn’t allowed in the same room, or eat with cutlery he used. I wasn’t allowed to hug, kiss or touch him in any way for a year. It was really scary when we found out, but I guess you want to know how it happened…
At Zac’s nursery, there was a water butt with a pump and the helpers there let the kids make mud pies with it. As Zac was only 3 then it was an option he couldn’t resist. Mum says “the CF nurse visited the nursery and advised us to pull Zac out as there were too many high risk hazards for them to change. We learnt a lot more as a family about the various risks we needed to avoid in all types of settings. Moving around though we have realised that some CF teams prioritise this knowledge more than others. I think they are afraid of scaring families when they first receive the news that their child has CF.”
My wish list for school
• that I don’t have to repeat myself over and over again
• that they understand that some hazards aren’t as bad as they think
• that no one thinks I’m strange and have a mental problem
• that it’s not a reason to be teased
• that visitors helping out in my class are told of my condition
Our awesome CF Fundraising event
While at school some children were really thoughtful and wanted to raise money for my charity (CF Trust). Alright, yes, I started the thoughts, but every fire has to start with a spark!
A group of girls went and asked our teacher about it and she was delighted! I phoned the CF Trust and the next day they sent me a package. It was full of banners, balloons, money collectors, stickers, AND 2 different types of leaflets! I brought the package to school and showed Mrs. Mason (my teacher). We had to think of the way to raise money as well, so we stuck a piece of paper and pen to the wall outside our classroom and wrote, “Ideas for CF Fundraiser” on top. By the end of 2 weeks we had millions of ideas to choose from. “Cake stall” was an instant winner, but we knew, to raise loads of money we had to have a lot of stalls. Art and crafts, penalty shoot out, agility course and pinata also got a contract. Me, Archie and Erin set up an info stall. All leaflets, remaining balloons and stickers were given a home on our table. If you gave a donation you got a stickers and the little kids got balloons too!
When the pinata had broken, the winner couldn’t eat the sweets inside so he sold them to passersby and he raised £5. Now, I want to tell you how much we raised, so, take that £5 and multiply by 100. Give or take a few pence and that is £500. AMAZING and even better, I felt special. Like all of that was for me. All that money, for people like me.
About Tess Griffin:
Hi, my names Tess, I’m nine and I go to The Batt CofE Primary school in Witney. I have Cystic Fibrosis (CF) and so does my little brither Zac (6) Erin (my little sister who’s 8) is lucky in that respect. Mum and Dad are really encouraging and get me into loads of energetic hobbies. I like these the most: dancing (I don’t go to a special dance school), Kickboxing (at Windrush Kickboxing Club), football (in the Witney Vikings football Club). I also like some non-energy hobbies such as: chess (I love my Dad’s board), drawing ( I adore Manga), writing ( and don’t forget reading too!).
Original source: Evidently Cochrane at http://www.evidentlycochrane.net/
- Self management education for cystic fibrosis
- Physical training to improve exercise capacity in people with cystic fibrosis
- Interventions for promoting physical activity in people with cystic fibrosis
- Strategies to promote participation in physical activity for people with cystic fibrosis
- Cochrane Cystic Fibrosis and Genetic Disorders Group