Ten Things Every Cystic Fibrosis Parent Has Heard

Having a shock diagnosis of cystic fibrosis thrust upon parents is never going to be easy. While people mean well, I have found that in the beginning I just didn’t know what to say to people. When they enquired about my new baby’s health I felt awkward and struggled to find the words. Did they know? Had they heard? Most of the time, even now when people ask, I just say she’s doing great and very little else. In the same vein other people also feel awkward. I try to imagine what I would say to someone if they told me their baby had CF. I’d like to think I would try to say something fairly supportive but who knows unless you’re placed in that situation.

I am very lucky to have a great support network online from other CF mams and we have all recently discussed this awkwardness in terms of dealing with other people’s comments with regards to cystic fibrosis. So without further ado I thought I would share with you all the top ten comments made to us CF parents – all real. Some good, some bad and some downright hilarious! Enjoy!

1. ‘But they look so well!’

Ah yes, as if we have just made the whole thing up. I wish. Cystic Fibrosis is what is known as an invisible illness. Patients will always look well on the outside despite lots of internal problems, affecting many organs in the body.

 

2. ‘Will he/she be able to walk?’

Yep, again CF affects internal organs only. No physical disability at all.

3. ‘My brother’s friend’s, cousin’s, next door neighbour had CF. They died when they were twelve.’

Lovely. Just lovely. And just what you want to hear when you have a baby with CF. Hopefully, whoever said this was talking about something that happened a very long time ago when medicines and treatments weren’t as good as they are now.

4. ‘Has he/she got to have that EVERY time they eat?’

People with cystic fibrosis are pancreatic insufficient and before they eat something, if it has fat in it, they have to take medication called Creon. This helps to absorb everything in their food. It’s a right pain, especially when they are too small to swallow capsules and the contents have to be poured onto a spoonful of apple purée. Trying to work out how many pills they will need for different food items is also a pain. So comments like this will no doubt grate on any CF parent!


5. ‘So they have to have all this every day?’

Linked to the above in relation to the syringes full of different medicines babies have to have each day, not just once in the morning, but at night too.

6. ‘But they will grow out of it though?’

Sadly, no. Cystic fibrosis is a genetic condition which progresses as a child grows.


7. ‘They can just have a lung transplant then they will be fine.’

Ah yes, wonderful. A major operation with huge risks. Lung transplants are amazing but new lungs do not grow on trees. Despite a recent major push in the UK, lots of people are still not signed up to the organ donation register. I also cannot even envisage this for my child.

8. ‘I don’t know how you deal with it all, you’re all so strong, you’re inspirational.’

These are really lovely things to say to people but what most CF parents will say is that they are just doing what anyone else would do in the same situation. There is no choice; you have to deal with it and try to stay positive every day. CF children do look so well because of the many hours of medicines, care and treatments that parents do behind the scenes.

9. ‘But they won’t know any different as they’ve been on all the medication since being tiny babies.’

Great! Yes, they won’t know any different but we all wish things were different for them.


 

10. ‘How severe is it going to be?’

We all wish we knew the answer to that. CF affects everyone differently. I have read about twins who obviously have the same mutations and one is still going strong while sadly the other passed away at a young age. Who knows? But what I do know is that nobody is guaranteed a ‘tomorrow’ so enjoy every second!

If you want to find out more about cystic fibrosis and ways you could help click here.

Cystic Fibrosis FAQ’s

May is cystic fibrosis awareness month so I thought I would answer some of the questions we have been asked recently. They do range from fairly sensible to completely ridiculous but to be fair we knew very little about the condition before our daughter’s diagnosis so any question is a valid one!  I am still learning every day so apologies to any CF experts; please correct me where needed.

The questions are a mixture of what we have asked our CF team and what others have asked us. Some will be directly related to Harriet and the rest applicable to the cystic fibrosis community in general.


What is cystic fibrosis?

Most people believe that CF is all to do with the lungs and a build up of thick, sticky mucus. This is true but is only one part of the condition as it affects the digestive system and other parts of the body too.


How do people get cystic fibrosis?

Cystic fibrosis is an inherited genetic condition. One in twenty five people are unaware that they are carriers of the faulty gene. My husband and I both carry the most common type which affects around eighty percent of CF patients. Harriet has two copies of Delta F508, and there are thousands of other gene types for cystic fibrosis and the discovery of this came not so long ago. Because of this, our families now have the option of having their own genetics tested if they want to find out their carrier status.

If we have another baby what are the chances of passing on cystic fibrosis?

There is a one in four chance. One in four that the child will have CF, two in four that the child would be a carrier like my husband and I, then a one in four chance the child would be free of any faulty gene. Our eldest daughter will have the option when she is older to have her carrier status checked. We have been offered IVF should we decide to have another baby but we are still unsure. If I could skip the pregnancy part I would have a house full!


Are there different types of cystic fibrosis?

There are different gene types but CF is a very individual disease. The picture below shows how the cells do or don’t work for the various mutations. As you can see, Harriet has a class two mutation. Class one being the worst through to five being the least severe. A sufferer has to inherit two copies of a CF gene but they can come in any combination. The majority of people with CF have the same mutation as Harriet but even if they have the same type their symptoms can wildly differ. I have often asked if there are any indicators symptom wise to tell us how Harriet will be affected, but apparently there is not. For example, Harriet was born with meconium ileus so I asked whether she will now be prone to bowel issues but seemingly no. Our CF team explained that they have seen babies have bowel surgery then go on to no future issues. In contrast they have had children not born with a blocked bowel and go on to have digestive problems so it is all very unknown.


How is cystic fibrosis treated?

At the moment treatment is mostly preventative. Patients are seen regularly at clinics where cough swabs and bloods are taken to make sure there is no signs of infection. Harriet is on daily medication for life which includes antibiotics, sometimes a few different types, vitamins, pancreatic enzymes, salt replacement, medicines for her liver and stomach function. Harriet also has daily percussion physiotherapy to help move the mucus clogging her lungs. She has two twenty minute sessions a day at present.

What is the current life expectancy for a person with cystic fibrosis?

Currently around forty years old although I read a different figure almost on a daily basis. The way we look at it is that over the last twenty years life expectancy has gone up dramatically and it’s only going to continue on that upward trend.

What does the future hold in terms of a cure for cystic fibrosis?

There are so many exciting developments at the moment and we are very positive for Harriet’s future. There is a wonder drug available to patients with a class three mutation and the company who produce this are launching a similar drug for Harriet’s mutation soon. It hasn’t proved to be as effective but they will continue to make improvements. There have been other breakthroughs in terms of potential cures but funding is needed to further the research.

Does Harriet need any further operations?

No. Hopefully her bowel issues are all over now and she will not require any further surgery. Only one in ten babies with cystic fibrosis are born with meconium ileus so most patients will not need any operations. Sometimes as adults a cystic fibrosis sufferer may require a lung transplant but that is something I can’t think about at this stage of our journey even though there are some amazing positive stories out there.

What can/can’t Harriet do?

There are very little restrictions – Harriet is just like any other child. There are precautions we can take e.g. not letting her come into close contact with people who have bad colds and she shouldn’t be around stagnant water but other than that she is fine. Exercise and keeping fit will be massively important for her to keep her healthy.

Will she be able to walk?

A valid question we were asked and the answer is yes. Cystic fibrosis affects a persons internal organs and there are no outward signs of illness.

And finally, my personal favourite…

Can you have a real Christmas tree?!

This one was asked by me to our consultant as I had read online that we shouldn’t get one because of spores the tree would give off. Needless to say it got a laugh and we did get a real tree!

If you have any questions about cystic fibrosis please ask and I will do my best to answer them for you!