‘It could be worse’

Another year, another sick CF Christmas ‘break’ for my little lady. Thanks Santa for your second-year-in-a-row delivery of Parainfluenza and your ‘surprise’ gift of post-bronch pneumonia for a little extra, unexpected excitement. You arrived at exactly 3.30am on Christmas morning, bang on time for ‘Christmas fever’. If we are, in fact, on your nice list, I’d hate to see what the naughty nellies were given. Next year, please deliver to my house one extremely large DULL MOMENT. It is the only thing my family craves at YuleCrueltide.

For some reason, the cruelty of (any) sickness at Christmas is something that’s uniquely unquantifiable and intensely loathsome. It’s as if the baubles are laughing at your well-rounded stress and the fairy lights are flashing intermittent ‘fuck you’ signs in your miserable face.

As we emerge into the New Year – still coughing like it’s 2016 I might add – I find myself clinging to the phrase ‘It could be worse’ with every last sticky pad of my sanity. Yet it brings me little comfort as my mind appears to be embroiled in a fully-fledged war with demons that are possibly greater than the physical ones we are facing.

But in an attempt to convince my curled-up-in-a-ball-rocking mind that ‘it could be worse’, here are the actual reasons why it genuinely ‘could be worse’.

We went to A&E twice, but we got home again.
The previous two Christmases, we went to A&E and stayed for 2 weeks. This year, we went to A&E twice but came home again. This is a testament to the super strength of Little Miss. Sure, her sleeping sats kept dipping into the 80s. Sure, she’s coughing like someone who smokes 60 a day. But she’s still able to sit up and play ponies and convince the docs that she’s got this shit under control. (I need lessons from her.)

We’ve been dealing with low sats, but have managed to avoid being oxygenated.
Thank you four-hourly Ventolin nebs. This has never been enough for Little Miss in the past, but by the skin of our teeth and the steam of salbutamol, we’ve pulled it off. I may be suffering from PND (Parental Neb Depression – where you do nothing but administer and sterilise nebs for weeks on end, in a state of frenzied worry), but there is a success story in the blur of exhaustion and cough-till-you-puke-athons, should you choose to see it.

We’ve been treating with the wrong antibiotic, but there is a right one.
While the Parainfluenza has to just run its long, marathon course, the chest infection can be fought with antibiotics. Unfortunately we spent 12 days on one that this particular bacteria is unaffected by, but we’re finally on a different one and we’re hoping that we’ll see an improvement soon. The ‘it could be worse here’ is that many bacteria that CF sufferers culture are resistant to antibiotics so we’re lucky that the one we have is just resistant to penicillin. USE ANTIBIOTICS PROPERLY PEOPLE. People with CF’s lives depend on us not abusing them into oblivion. The phrase ‘post-antibiotic era’ is the scariest word combination on this earth to families like ours. Let’s protect the antibiotic sensitivities that are left.

Christmas is over.
So I won’t have to feel sorry for us at such biblical proportions hopefully for another 12 months. I’m not usually one for wallowing in self-pity, but Christmas unearths my depressive tendencies and ignites them with its humbug horrors. So long jingle bell-hells!

The coughing will stop sometime, right?
I can’t explain how every breath-stealing coughing fit causes a new break in a CF parent’s heart, but it does. To see a 4-year-old with lungs that can’t cope with a walk from the car to the hospital door is an injustice that my mind will never be able to reconcile. My little lady has been coughing since September, with a peak of RSV hell at the end of November and Parainfluenza purgatory from the end of December to…(please insert a coming soon date universe). It has to stop eventually. Doesn’t it?

So, in conclusion…
We’ve lost a little weight. We’ve lost a lot of sleep. And we’ve also lost a chunk of mental strength. But we’re here to tell the tale. So I guess it’s true that ‘it could be worse’.

Illustration courtesy of the talented Olivia Golden, @goldenwonder1

Orkambi and Vertex: the horror of hope

Your child is diagnosed with the life-shortening, life-limiting, chronic illness that is Cystic Fibrosis. You’re told that your child’s life expectancy is early 30s, the age you are when you receive this punch-in-the-gut news. The median age of survival in Ireland is only 27, YOUNGER than you are at the time. You want to vomit. But you’re also told that now is the best time for a child with CF to be born as we’re entering an unprecedented age of progress in treatments. And that is what gets you through – you cling to this glimmer of hope with gorilla glue fingers; it becomes everything to you.

I remember sitting on the couch on the evening of Eva’s Diagnosis Day, my heart on the floor; feeling empty, hopeless and so very lost. The Six One News was on in the background and a piece on a breakthrough CF drug from Vertex Pharmaceuticals came on. The drug was called ‘Kalydeco’. But the NCPE had advised against funding it. So had the HSE. And the CF Community was fighting for access. It was the first drug to treat the underlying cause of the condition for people with the G551D gene, the reporter said.

I decided the timing was a sign. A sign that my child would defy all the odds and stats because she was born in the year of Breakthrough A.D. She didn’t have the genetics that Kalydeco treated, but surely this drug was only the beginning. This ‘sign’ became my lifeline. I was convinced that she was diagnosed on the day this was all over the news for good reason. This was my reassurance and this was to be (and still is) my drive every single day of her life. A miracle was within reach – I could feel it, I could smell it, I could imagine it. I wouldn’t have to bury my child a quarter way through a lifetime – she would bury me as children are supposed to bury their parents. My husband would walk her down the aisle. She would have children of her own. CF was not a death sentence. Not any more. This news report was the only reason I could pretend to deal with her diagnosis. It was the only reason I could face the new reality we found ourselves in.

Kalydeco was finally approved when Minister James Reilly bravely overruled the funding recommendation and today, the people who are on it confirm it is utterly life-changing. His decision has improved and extended the lives of those patients and I can’t wait to see how many more years it adds to their lives. It’s the most inspiring medical breakthrough I have ever seen. (Of course I am slightly biased.)

Fast-forward almost four years and the CF Community is again fighting for access to a breakthrough drug, from Vertex once more. This one is called ‘Orkambi’. And a lot of history is repeating itself: once again my daughter doesn’t have the genetics it treats (this one is for ‘double deltas’, the most common genetic combination). Once again the NCPE and HSE have recommended against funding it (the final HSE ruling is due on December 12th I believe). And once again, the CF Community finds itself fighting for the lives of its family and friends.

So many things have changed for me personally though. I’ve lost my naivety and innocence. I’ve now seen the lows of CF first hand through my daughter’s exacerbations. I now know the people in the community who are fighting. I’ve met so many of them. I speak to a lot of them daily. I feel their pain on an extremely personal level. And this time, I’m fighting alongside them.

I feel sick when I see parents having to beg for medical breakthroughs for their children. I’m horrified at the idea that people who are on Orkambi from the trials aren’t guaranteed that they can stay on it for life. I’m disgusted at the way the HSE and Vertex have engaged in public mud-slinging of each other’s negotiation tactics, leaving patients drowning and terrified in the middle. Leaks to newspapers. Downplaying of the drug in interviews. Scaremongering that makes patients feel like they have to air every detail of their difficulties in public to make the case for why their lives matter. And of course I’m petrified that if this drug doesn’t get through, that I’ll never see a drug to treat my own precious little lady.

Is the HSE the big bad wolf for not paying for it? Is Vertex the big bad wolf for demanding a huge price tag? Are they both as bad as each other? Surely it’s not impossible to reach a fair, middle ground? We face a world where our knight in shining armour, Vertex, the company that could save our children’s lives, is also painted as greedy and immoral. This conflict is incomprehensible. We love them. We hate them. But boy do we need them. Regardless of how any of us feel or who we ultimately blame for this debacle, it doesn’t change the fact that patients are getting sicker while a drug that could change their lives sits on a shelf somewhere other than in their pharmacy.

My daughter is nearly 4 and is only starting to ask questions about her CF. But how is this circus affecting teens with CF? How is it affecting young adults? Facing the worst of their illness blasted all over the media. Finding their lives discussed in monetary terms of what they are ‘worth’. I can’t begin to estimate the psychological damage that this storm will leave behind. Every single one of us is being damaged by the cruel game we’re caught in the middle of.

I truly feel for the people waiting for this drug. And I truly feel for those who don’t even have a drug to fight for yet, ourselves included. Our groups may have different genetics and be facing different waits for new therapies, but one thing unites us all: we’re all clinging to hope. And it would seem that hope is perhaps the cruellest feeling of them all.

 

Image courtesy of the #YesOrkambi campaign, photographer: Simon Burch. The beautiful little girl featured is not my daughter – but she is one of the little ladies who deserves to try Orkambi.

 

Do you find yourself or lose yourself on a career break?

 

One of the hardest decisions I’ve ever made during my 14 working years is the decision to take a career break. A break that I’m currently almost half way through.

Now I’ve done it for a very specific reason, in a very protected way and thankfully, with a very supportive employer. My little girl with CF has started playschool and I want to be there to support her through the health implications that mixing with other kids for the first time brings. So I’m on a type of leave known as Carer’s Leave, which allows me to take a break while protecting my position in the workplace.

But call the leave what you like, you still have to say these words out loud: ‘I want to take a break.’ That is a scary sentence to verbalise in front of the people that pay your salary. You worry that the second you say it, the working world will write you off as weak, uncommitted and unambitious. Even if they wouldn’t dare say so out loud.

In a time where women still earn less than men, where women still take charge of the bulk of domestic affairs, where women still feel that no matter how much they do it is never enough, it is terrifying to speak up and press that pause button. Because what if that pause button actually ends up rewinding your career?

But do you know what’s even more terrifying? Doing what you’re doing to please other people or because you’re afraid of what others will say, do or think of you as a result. Don’t ever let your pride get in the way of doing what’s right for you and your family. EVER. The bravest thing you can do is what’s right for yourself. And if anyone thinks less of you for that, it’s that person that deserves to be thought less of.

Now you may be reading this as another CF parent or you may just be reading it as a parent who is considering their options. Either way, the question of whether you find yourself or lose yourself on a career break is equally valid.

I’d love to say there was a straightforward answer to this, but of course (like everything else in life), it’s complicated. I would, however, like to offer you my experience on this journey of lost and found…

Things I’ve found:

  • My smile: I had myself under such pressure trying not to let anyone down, that I was letting myself down. By being honest and admitting I needed a time out, I found that my mouth could actually curve in a different direction. Who knew!
  • My yoga mat: Time to attend a class! This one hour for me is psychologically transformative. My body may be an inflexible mess but my mental health feels quite bendy.
  • Time for friends: I can’t quite believe how busy it still is when not holding down the day job, but I love that I have a little more time and energy to meet up with friends.
  • Time to write: Sure, I write for a living. But when you do it for a living, it can actually be incredibly hard to also do it for personal reasons. I have missed writing for myself more than I could ever express.
  • Domestic peace: Truthfully, I was worried about how I would cope spending so much time at home. I worried I would be less patient with the kids, rather than more so. But we’ve found our groove and while I’m far from a perfect parent, we’re all perfectly happy living in each other’s pockets. (We’ve even been known to find sunshine in them.)

Things I’ve lost:

  • Any tendency to over-react: Because I’m with my daughter every day, I’m better able to monitor and assess her coughing and health status. I also know that I can bring her to the GP or hospital at any time without a domino effect on work commitments. This allows me to be a lot more level-headed in my reading of her when I feel she’s not 100%.
  • A few wrinkles: Managing Cystic Fibrosis is a full time, grey-hair inducing job, no matter how much or little else is going on. But temporarily doing it without as much multi-tasking has smoothed out some forehead wrinkles (marginally, but still). I’m hoping the laughter lines will have caught up with the worry lines before my leave is up.
  • A little bit of myself: So with all the positives I’ve been experiencing, there are of course some negatives. I miss the feeling of accomplishment when we crack a business problem with a cracking idea. I miss the satisfaction that comes from a happy client reaction after you’ve presented a new campaign that nails it. I miss the camaraderie of the gang in the office (I might even miss the arguments a little! But I’ve always been a bit weird).
  • My bank balance!

So while my assessment comes to the woolly conclusion that a career break has left me both lost AND found, there are a few things that are for definite –

I will never regret hitting the pause button.

I will never regret putting my family first.

And after a few months of much-needed healing, I feel stronger than I’ve ever felt before.

Regrets? Je ne regrette rien.

 

10 CF phrases to help you understand young CF families

Perhaps you know us, but don’t like to ask too many questions. Perhaps you’re afraid of saying the wrong thing, or of upsetting us. Please don’t be. I think most families take comfort in you enquiring after the health of their child (or children) with CF, in our case our daughter. You might be paranoid about putting your foot in it, but we’re a forgiving bunch! Every conversation is education and it’s much worse to ignore the mucoid elephant in the room.

While CF doesn’t define our family, it’s certainly an extra (uninvited) member of the household, and we have no choice but to learn to live with it. And for an ‘invisible’ illness, it certainly knows how to make its presence felt sometimes. To give you a little insight into what it’s like inside a CF family (or at least inside this one), I’ve been playing around with some phrases beginning with the letters ‘CF’. Probably a daft idea, but here goes…

  1. Constantly Famished

In our house, there just can’t be enough food! Why have one slice of lasagne when you can have two? Why leave any chicken on the carcass? Yes, I’d like fries with that! People with CF need to take in up to 50% more calories than their peers to allow both for malabsorption and for the extra energy they need to breathe. Even with enzymes to help absorb protein and fat, it’s not an exact science, so ‘more please’ is music to a CF parent’s ears. Even with all the extra food, some people with CF still struggle to gain sufficient weight. We are blessed that our daughter has a great appetite and is a really good weight. But I’m acutely aware that most parents deal with one of two extremes – ‘constantly famished’ or ‘can’t feed’. For some kids, the oral appetite just isn’t there (or weight gain is just not adequate) and peg feeds directly into the tummy are needed to bulk up intake.

  1. Crippling Fear

Of course you try and cover this one up, as you don’t want your child to feel different or frightened of the world. But often, while you’re acting normally on the outside, you’re screaming on the inside. The world is a scary place when your young child is susceptible to illness. If you allow yourself to think about it – other people, supermarket trolley handles, soil, hay, snotty noses, coughs, mould, dust – it feels like everything is a risk factor. Yet sometimes your own fear is the biggest risk factor of all when it gets in the way of living any kind of a normal life. I try to deep breathe and hand gel my way through the most anxiety-ridden days!

  1. Crazy Faeces

There ain’t no stool like a CF stool. Not for the faint-hearted, parents get over themselves pretty quickly when they have a baby with CF. From luminous green to orange oil, you see it all. What’s really important, though, is to pay close attention to that rainbow waste, as it gives you huge insight into the health of your child’s digestive system and how well their enzymes are working for them.

  1. Courageous Fighters

Our kids put up with interventions that most adults would struggle to deal with. Frequent cough swabs, IVs for weeks at a time, bloods, X-rays and hospital admissions. Some have ports. Some have feeding tubes. Most have a heavy load of daily medication and nebulisers. And yet all the kids with CF that I know are insanely agreeable, happy and inspiring. They could teach the rest of us a thing or two, that’s for certain.

  1. Can’t Function

While us parents aren’t the ones whose bodies are physically fighting CF on a daily basis, our hearts and minds fight it every second of every day. Sometimes we can’t sleep because we’re afraid our kids are breathing funny. When our children are coughing, our hearts are breaking. When our kids are poorly in hospital, our mental health goes under with them. And Dr. Google is a terrifying temptation. We’d give anything to trade places with our innocent children and take the pain away. I’d end my life today if I thought for one second it would guarantee my daughter a full one. We probably appear as people who flip-flop between ‘we’ve totally got this’ and total mess. Bear with us; our ability to function is generally linked to our child’s lung function.

  1. Cautious First

We will ask you if you’re sick before you come over for dinner. We’ll ask you if anyone in your house is sick before we call over to you. Please, please don’t be offended. We’ve seen two-week admissions come off the back of what’s a basic runny nose to someone else. We can’t control the rest of the world, but in our inner circle, we can at least ask sensible questions and avoid unnecessary risk. Remember that when you’re well, you are ALWAYS welcome. So visit us on the double then.

  1. Clean Freak

This one lessens over time as you learn that no matter how much disinfecting you do, there’s still bacteria practically everywhere. When our daughter was a baby though, all toys got disinfected every night and people practically got sprayed coming in the door! Now we apply more workable rules, like shoes off in our house and wash your hands when you come in. Clean hands are a bit easier to achieve than a clean world! But it takes time to learn to relax.

  1. Candid and Frank

The injection of perspective you get when your child is born with a health challenge is like nothing else I’ve ever experienced. You lose your ability to tolerate bullshit, time wasting and insincerity. So CF parents will tend to tell it like it is and be super-efficient with their time. We’re extremely compassionate people but also extremely straight-talking. Embrace the honesty – the world could use more of it.

  1. Cancelling Frequently

We make plans but sometimes, CF has other plans. We might show up to 10 things in a row and then back out of 5 in a row. Please don’t stop asking – we love you and want to see you (you do our mental health the world of good), but sometimes CF has other ideas. So let’s overdose on playdates, coffees and dinners when all is well, knowing that sometimes it won’t be.

  1. Completed Family

The first thing I need to say is that this is absolutely not the case for everyone who has a child with CF, but it is the case for us. Plenty of parents go on to have more children who may be born with or without CF. I admire their bravery so much but after several years of debating this issue with ourselves, we’ve decided that CF has put a full stop to the number of bodies in our household. (Perhaps we’ll add a four-legged friend!) Decisions like this are mostly about recognising your own personal, emotional limitations. You have to know where your strength begins and ends. Our children need functioning, loving parents, and that’s where our future energy will be channelled. Of course such a decision comes with a heavy heart, so be gentle when talking to us about your plans to procreate a football team 😉

That’s it – crash course in CF over. So to keep up with the theme, c’est fini!

 

Illustration courtesy of the talented @goldenwonder1

 

Learning to let her go…

I’m not the first (and certainly won’t be the last!) mother to write about how emotional they’re feeling packing their butter-wouldn’t-melt little beauty off to playschool.

But it’s virtually impossible to explain just how big a deal starting playschool is for the Mum of a kid with CF (or any other medical issue). Notice I said it’s a big deal for Mum! The issue is entirely mine. My daughter couldn’t be more ready to take this step – she spent the evening before her first day hugging me and telling me she was soooooo excited. And I’m absolutely thrilled for her. But while the excitement is all hers, the fear is all mine.

You see, as horrible as this might sound, when you have a child with vulnerable health, you see pretty much every thing and every body as a possible source of infection. CF Mums have bionic ears for coughs or sniffles for good reason, having seen their kids suffer the extreme consequences of the most ‘innocent’ of viruses. We also have super-sight for bacteria-friendly environments, knowing that antibiotic-resistant, opportunistic pathogens are ten-thousand-a-penny in this fabulously moist country.

You might read this and think I’m particularly over-anxious or over-thinking it, but the fact is, it’s my duty to make sure my little lady isn’t put in unnecessary danger. She will appear to ‘muck in’ with the rest, but everything she interacts with will have been thought about and talked about with her school. We make modifications in advance, so she doesn’t feel any different to the other kids when she’s there.

I’m sure you’re thinking what the hell kind of danger is there in a playschool of all places? You’d be surprised. With CF, the dangers lurk in the most seemingly innocent of places:

  • Towels – the most germ-infested objects in any home or school! Paper towels are a must for people with CF.
  • Soap – we need to make sure it’s liquid. No sharing soap bars.
  • Poor hand washing facilities – splashing your hands in cold water does not a hand wash make! Warm running water and soap is a must – sing happy birthday to yourself twice while bubbling up!
  • Compost – while making compost is seen as environmentally friendly, rotting veg grows B. Cepacia, a potentially lethal bacteria for people with CF.
  • Soil – grows pseudomonas aeruginosa. Many people with CF will still do gardening and that’s a totally personal choice. But caretakers need to know to follow strict hand hygiene rules if a child with CF is taking part in any planting activities (and check if their parents will allow them to in the first place).
  • Tyres – you see them all the time in playgrounds. Lovely big black tyres – great craic to jump into and over; great places to grow pseudomonas and lots of other baddies in the stagnant water that they collect inside their rims. These ‘playthings’ are actually horrifying ‘germthings’.
  • Rainwater buckets – more stagnant water, more problems! These are a no-no for us.
  • Other children – another child’s sniffles could be my child’s hospital stay. This one, of course, is not an easy fix as none of us can lock our children up! And children at this age catch viruses like spiders catch flies. But keeping a child with respiratory symptoms separated from my child is a must.

It probably all sounds a bit grim, but I guess like everything else in CF, prevention is protection. We certainly won’t be able to completely stop our wee woman from picking stuff up, but it’s important to limit unnecessary risk.

Of course, Little Miss is blissfully unaware of all the meetings and care plans, all the worried faces as I briefed the playschool team, all my tears and fears behind closed doors. The main thing is: she’s ready. My little magnetic, giggly, social butterfly is ready to get out there and make her mark on the world. I, on the other hand, am not a bit ready. And I never will be. But I’m getting much better at pretending.

Enjoy yourself little lady.

This Mama is doing her best to let you go and not hold you back any more.

 

Illustration courtesy of the lovely @goldenwonder1

 

Humanity blossoms on 65 Roses Day

There’s not a lot to love about a chronic illness like Cystic Fibrosis. It arrives uninvited into your home and into your heart and attempts to take over, with its constant demands for medication, nebulisers and inhalers, and its constant rumbling of your fears and emotions. You see your child go through things no child should ever have to go through. You see them build a resilience that’s far beyond their years. You see them cope with things that would have most adults running for the hills. But you keep the chin up and try not to let it consume you; you reframe everything in your mind in an effort to keep yourself sane – the entire family builds a thick skin in an effort to get on with it and keep things as normal as possible.

But while there’s nothing to love about Cystic Fibrosis, there’s a lot to love about the support that’s out there amongst the warm-hearted people of Ireland. Last week was Cystic Fibrosis Awareness Week – the first one that I’ve properly involved myself in since Eva was born (it takes a while to accept the condition and then turn that acceptance into action). So while I’ve done a fair bit of fundraising over the last 3 years, this was my first time to immerse myself in the ‘official’ world of CF.

But boy, am I glad I did. From the kind words in Blanchardstown Shopping Centre to the even kinder donations, my faith in humanity was more than a little restored on what is CF Ireland’s biggest fundraising day of the year. Truth be told, I was actually nervous about being a volunteer. Sounds ridiculous, but I worried that people would shimmy past us, trying to avoid eye contact because of charity fatigue. But instead people actively sought us out and did volunteering of their own – volunteering of thoughtful words, personal CF stories and resounding support on the issue of Orkambi.

Throw in a few Strawfies for good measure…

And as if we weren’t all busy enough fundraising on the streets, myself and a few other CF Mammies also decided to support the Straw Breathing Challenge online to see if we could bump the figures a little more. What this involves is a challenge to pinch your nose and breathe through a straw for 60 seconds to get an insight into what it feels like to struggle to breathe. You then take a selfie, post it on social media, make a text donation and nominate your friends to take on the challenge next. (See the breaking news article from today to see just how successful it was – there are a few famous faces in here like Nadia Forde, Simon Zebo, Conor Murray, Keith Earls and Paul Warwick!)

When ‘Breathe with me – the Straw Breathing Challenge’ contacted me about kicking this off in Ireland, I was willing as ever to give something a go, but I was a little sceptical as to whether anyone would bother. I have a love/hate relationship with social gimmicks, but once again was surprised (and more than a little emotional) at the amount of people who were willing to get behind us and give it a go. Your willingness to support us so publicly gave both a fundraising boost to CFI and a morale boost to a group of Mammies who live their lives on an emotional rollercoaster, never knowing what’s coming next. (By the way, if you haven’t done one yet, it’s not too late – just take a ‘Strawfie’ and text ’65Roses’ to 50300 to donate €2 to Cystic Fibrosis Ireland).

strawfie collage 2

Not your average week…

So what a week it was! And coming hot off the heels of our first trip to the annual CF Ireland Conference the weekend before, CF Awareness Week was a time of heightened feelings in every possible way. Because while I wish every single day that we weren’t on a CF journey at all, I feel lucky that we’re at least sharing it with the most wonderful of people.

Speaking of wonderful people – to the families of those who have lost their fight to CF, who spend so much time fighting alongside those who are still in it to win it, words can’t express the admiration I have for you. Your strength and relentless efforts are mind-blowing and if I’ve even absorbed even a percentage of your determination to beat CF, our family is in a good place.

Always say thank you…

That’s it – my gush rush is over! But just to say thanks to my lovely Mammy for stepping out with me on 65 Roses Day. Also thanks to fellow volunteers Lisa, Chris, Phil and Lesley for the lovely chats on the day. Thanks to all you crazy Facebookers and Twitterati for your ‘Strawfies’ and text donations. Thanks to everyone who supported 65 Roses Day and reminded us that humanity in Ireland is anything but wilting; in fact, it’s in full bloom.

Most of all, thanks to my beautiful Eva Rose for being my perfect little flower, every single day.

 

 

 

Why Ireland needs to approve Orkambi and every other CF drug that Vertex can dream up

oxygen dreams HR

So 2016 has started pretty much the same way as 2015. Watching our beautiful little girl struggling for breath, with nose-diving oxygen sats and a wheeze that would fill every cell of your body with dread. It started with a fever, turned into a runny nose and 72 hours after the first cough, she ended up in hospital with pneumonia. From first cough to pneumonia in 72 hours, just contemplate that for a second. That’s what living with CF is like. It’s that cruel. It’s that unpredictable. It’s that heartbreaking. Our daughter has only just turned 3 and has just spent another 10 nights in hospital on oxygen – what kind of a life is this?

And yet whether or not Ireland will approve the new drug from Vertex Pharmaceuticals called Orkambi, the first drug to treat the underlying cause of Cystic Fibrosis for patients with two copies of the mutation Delta F508, is actually up for debate!

To be clear from the outset, my daughter unfortunately won’t benefit from Orkambi because she only has one copy of the Delta F508 mutation. Her other mutation is a rare one called R560T. But as I reflect on another arduous stay in Temple Street, with its several failed IV access attempts, the panic on our daughter’s face as she coughed till she vomited, the nose cannulas pumping oxygen into her lungs, the 9 nebulisers a day, and the emergency Doctor and Physio callout in the middle of the night due to tumbling sats not picking up with increased oxygen, I can’t help but think of the 600+ people in Ireland that would benefit from it. And it makes me sick to think that they have to fight for the right to this medicine. They fight for life every single day as it is. And now they have to fight to prove that their lives are worth the price tag that this life-changing medication comes with.

I understand that the price needs to be looked at. I agree that it’s expensive and the price needs to be negotiated. I take no issue with the fact that it has to go through an assessment and approval process. But I do take issue with the way the CF Community has been treated in advance of these assessments taking place – articles in the media stating that the HSE will not fund the drug ignited fear and panic in a community that’s already under stress and duress most of the time. Why prejudice the decision in advance of assessment? Why make people feel that their lives come with a price tag? Why make it seem like a decision has been made before a decision has been made?

People with Cystic Fibrosis often describe the illness as feeling like they’re drowning on the inside, as they struggle to deal with the excess mucus in their lungs. Meanwhile parents of children with CF frequently feel like they’re drowning in their own fears and feelings – feelings of being constantly overwhelmed, forever worried and scared for their children, while trying to put a brave face on it and truck on, wishing they could just take it away.

In the online CF Mammies support group I’m part of, the 16 days of this year alone have already seen several children in hospital for lengthy stays, some in ICU. These 16 days have seen children on oxygen, children on IV antibiotics, children on inhaled nebulised antibiotics that are so strong they come with the risk of hearing and sight loss, children screaming with the pain of bowel blockages, children losing weight despite eating constantly, children who need operations for PEG feeding tubes who are being given appointments that are over a year away. Then outside of our group, there’s an 11-year-old boy from Cork getting home after a lung transplant and another 11-year-old who just a few days ago lost their fight with CF. THIS IS REALITY.

And yet this is also reality – here are a few key facts about Orkambi from Cystic Fibrosis Ireland:

  • It is the first drug to impact on the underlying cause of CF for 60% of the CF population in Ireland.
  • The drug has shown up to a 40% reduction in worsening of CF requiring hospital admissions.
  • It has shown a sustained increase in lung function.
  • It has shown a sustained increase in weight, an important issue in CF care (nutritional status is shown to have a direct effect on lung function).

In Orkambi we have a drug that has the potential to lengthen lives, improve quality of life and delay the need for lung transplantation. So in 2016, let’s change our attitude to healthcare. Let’s try and decrease the number of CF infections, instead of just trying to treat them in hospital. Let’s move with the times. Let’s embrace the amazing science that’s out there; the science that’s hunting CF down. Because what good is this science without access to its fruits?

As CF parents, all we want to do is rewrite our children’s futures. We want them to dream big, to dream tall, to dream past the early expiration dates that have been put on their lives. Orkambi is the start of this dream becoming a reality. We shouldn’t have to fight for our children’s lives, but believe us if you make us, that fight will be relentless.

And just wait until a drug for Eva comes along…

 

If you haven’t yet signed the petition for access to Orkambi, please do so here:

https://www.change.org/p/leo-varadkar-irish-minister-for-health-approve-life-saving-medication-for-cystic-fibrosis-patients-before-its-to-late?recruiter=91550275&utm_source=share_petition&utm_medium=facebook&utm_campaign=autopublish&utm_term=des-lg-share_petition-reason_msg&fb_ref=Default

Illustration with thanks to Olivia Golden, @goldenwonder1
https://oliviagolden.wordpress.com/tag/olivia-golden/