When Chester was diagnosed and I became a CF mum, I became part of a much bigger family, a family of strangers forged together by a common enemy, Cystic Fibrosis.
We are a family that for the most will never meet and our children will never play together. We may be a relatively small community in the scheme of things, but this does not deter us, though we may be small we are proving to have a huge voice!
As a family we know the highs and lows, we live them too, we know the heartbreak and sadness that each of us has gone through and continue to go through daily. We know the pain, the torment and the guilt, we’ve probably all felt it at one time or another.
We are there for each other no matter what the time of day or night, we standby each other ready to pick up those who fall, with kind words, optimism and advice, always virtual counsellor ready at the press of a key. We are there for each other when our children are sick, to offer comfort, sometimes in the darkest of hours and we are there to rejoice in others good fortune, because that’s what this family does!
We have determination like no other, taking on challenge after challenge in our children’s names, finding strength each time to continue because we know our children have no choice than to get on with it. We dig deep to find the strength to take on the biggest of battles and we have the will, to want, to win and we do all this side by side, together every step of the way.
This community has been waiting for something special, for something to change CF, some have already waited too long already, so to be told that something is “Too expensive” and “Not cost effective” just isn’t going to work, you won’t be surprised to hear that we aren’t prepared to take that as an answer.
We have united in our fight for access to Orkambi, and we won’t rest until the negotiations start and a deal is struck, we are shouting louder than we ever have before and we are going to be heard!
Seeing this amazing team ethos and concerted effort by all those affected by CF and their friends and families has made me proud to be a CF mum and has restored my faith in humankind, that even when people are at their busiest in life, they can take two minutes out of their day to support our quest to get Orkambi for our children.
Like Chester there are many people in the CF community that possibly won’t benefit from Orkambi, but to us that doesn’t matter, we are family and we fight for all our children, one in all in!
It’s a fact that Orkambi is been proven to slow lung function decline and reduce exacerbations and is the first drug to actually treat the root cause of CF instead of treating the symptoms, this is a huge breakthrough and has bought much hope for people throughout this family. It’s approval also paves the way for future pipeline deals for other medications compatible for other mutations which essentially means one day Chester could get his cure.
All we want is for our children to get what they need, they didn’t ask for CF and fighting for them is all we can do. These beautiful children deserve to live their lives free from hospital admissions and from hours of physiotherapy and they sure as hell deserve to be heard and we will make sure they are!
Becoming a CF mum was never my choice, given the option I would never want to be a CF mum, but I am and for now I can’t change that.
One thing is for sure, that for a condition that can at times be isolating and lonely, I’ve never felt so surrounded by such wonderful people.
To me, diagnosis day and hospital admissions aside, this period has to be one of the most testing for me as the parent of a small child with CF, not only does he want to eat everything in sight except what I actually make for him (which I guess isn’t all bad) but I find he is now much more independent, headstrong and a lot more mobile, I am forever moving things higher out of reach, a change from my first child who must have just been incredibly lazy because he hardly moved.
I don’t doubt I am dealing with all the same problems any other parent would face, from the toddler tantrums, watching my once quiet and reserved baby boy become almost monster like, throwing himself around on the floor screaming in a rage, this coupled with the fact he has quite recently learned to say “No” mean I am hearing “No” pretty much all the time.
The typical food games toddlers like to play have started, refusing to eat anything I put in front of him, with a shake of his head he pushes every meal away. Now in an ideal world I would not be quite so pushy, but having lost a large amount of weight recently, I know how important it is to put some fat back on him. I find myself going back to the old tricks of adding cream, butter and cheese to everything I possibly can in the hope he eats something, even just a little. I even find myself buying him a cheeky McDonalds when his older brother isn’t around and desperately trying to fill him up on fast food snacks before going to clinic to be weighed just to be able to add a few grams on.
Despite refusing my culinary delights, I often find that while my back is turned he has raided the fridge and can often be found in the front room with the odd block of butter or a chocolate bar he has stolen. I know to most this would be of concern, however, I find myself looking to see just how much butter or chocolate he has eaten, feeling slight nauseous at the thought of him chewing on butter like he would a choc ice and a little worried about working out how much Creon I should give him.
Luckily this time I caught him before he ate it!
Talking about Creon, anyone know how many Creon you need for a worm and some additional soil? No, me neither! This is why Chester is not left unsupervised in the garden……ever!
As we all know, physical activities are fantastic for people with CF but I am beginning to wonder if all the activities he takes part in, such as bouncing on his trampoline, running games and “play fighting” with his brother have given him extra strength, I swear he’s almost superhuman, it never ceases to amaze me how physically strong this boy is. Which is fantastic until it comes to him becoming the toddler that doesn’t want to go where you are going.
The majority of outings end the same way, me carrying a screaming toddler under one arm. He has simply taken to lying down on the floor and refusing to move. Let’s face it, any CF parent’s worst nightmare right there, all I can think about are the germs on the floor, never quite knowing who or what has trodden that path before. But any parent of a toddler or older will know how difficult it is trying to pick up a child that has started lifting up their arms to make getting a grip on them impossible and with having mastered making himself extra heavy it just adds to the embarrassment of being a fully grown adult, physically incapable of lifting up the little person on the floor.
Just a few of our wonderful trips out!
Then with CF comes the pill taking, and refusal to do so. I find everything becomes this game of “How many times in one day can I say No?” and to be honest, I have lost count by the end of breakfast most days. Up until Christmas Chester couldn’t say a single word, Christmas day started with Mamma, which was music to my ears, but soon overshadowed by him saying No to everything.
Anyway I digress; Chester has around 30 tablets a day, vitamins, antibiotics, antacids and enzymes, all with their specific purpose, the majority he has to take with food. But being this power crazy, stubborn little being, on occasions he doesn’t want to, so I find myself using the good old trick of parent bribery, and this see’s Chester looking smug in the knowledge that he will get that milkybar dessert after his dinner because it is already on the table!
My mother in law often says she thinks Chester has been on this earth before because he just seems to know, and I am starting to think she is on to something. Needless to say, Chester takes his tablets and will have at least one high fat mouthful of food which is a win for me, even though the smug toddler thinks he has won!
So with toddlers comes potty training, what a delight! After “Poogate” last week which saw Chester trying to change his own dirty nappy, by smearing poo all over the floor, his teddy bears, his bed and in around 15 nappies which he had used to try and clean it up, I can’t say I am looking forward to this stage. We have still yet to get his movements to an acceptable level per day, and he currently goes to the toilet, a lot, and although he is showing signs of wanting to use the toilet. This area of parenting is the one I am dreading the most, especially with a beige carpet, let’s just say it won’t be little nuggets of poop we are cleaning up!
To me, CF is just a part of who Chester is, I wouldn’t say I have come to terms with it, because I don’t think I ever will, and whilst CF throws other factors into things we do, and sometimes shapes how we deal with things, I am keen to remember CF or not, he is a toddler first and I have to try and step back and let him learn for himself.
An average 0430 hr wake up call from Chester who has “tidied up”
As the year ends and the blank pages of the story book for this year opens, I find myself full of mixed emotions, for most it’s a fresh start, for me, it’s still the same CF. That said, I feel that glimmer of hope that this could be the year technological advances take us one step closer to finally being able to say our child used to have CF. Sadly I find it somewhat overshadowed by a deep sadness that has come from seeing far too many little ones pass away this past year, their fight cut short too soon and the feeling of helplessness at the apparent lack of progress to get them the precision medications they need.
I struggle when I think about the heartbreaking pain and the sadness of the parents who have lost their beautiful children and I cannot begin to comprehend the grief they face now. I only know the awful pain I feel in my dreams when CF takes over causing tragedy in my sleep, a reoccurring nightmare I have and even though only a dream it often feels too real, waking me up in tears and an awful sickly feeling.
I have spent the past few weeks struggling with a heavy mood often deep in thought about CF, something which hasn’t happened to me in a long time and something I find myself incredibly uncomfortable with. I found I was withdrawing from all CF related conversations because I felt I just couldn’t cope talking or hearing about it. I just tried to focus on the here and now, and get into the Christmas Spirit.
I began thinking about how CF affects so much, I sat watching a mother feeding her beautiful new baby without a thought for things like creon or having to rush home to fit in Physio and treatments, thinking about all the things my children miss out on because of CF. For the first time in a long time I couldn’t help but think about what my life would be like without CF in it.
I was becoming so negative in my own thoughts that I knew I had to snap out of it and I started to turn it around to try and think about some of the positive things I have found since having CF in my life.
Let’s face it, no one wants CF in their life, but we have it and there’s no changing that right now and when I actually snapped out of my mood I realised having CF in my life has taught me a great deal of positive things.
CF has taught me about strength, not only a strength in myself I never knew I had but also the huge strength in those around me, more often than not, small children. People with CF have this incredible ability to see the positive in everything, they are so incredibly resilient and I have come to realise that I have often been inspired by these amazing people that whilst fighting their own private battle are always there for each other sharing their highs and lows, picking each other up when they are down.
Having CF in my life has taught me to come to terms with the fact that it’s not possible to remain strong all the time and crying, getting angry and feeling down is perfectly normal and definitely allowed, that to be strong you will often get knocked back with difficult times, but with each time being able to reappear stronger and more knowledgeable than before.
If I didn’t have CF in my life I wouldn’t have the appreciation for time like I do now. Time is incredibly precious, CF or not, no one ever knows how long they have, so I have learned to make the most of what time we do have, doing all the things we want to do, enjoying every moment whilst making lifelong memories.
Without CF I would never know the kindness and humility I have seen from the wonderful CF community I have become part of, the people who drop everything for those in the same situation, each parent going through their own individual heartache but always there no matter what, with words of kindness and wisdom.
I have seen kindness and thoughtfulness from parents have lost their own children and even in their own immense grief, it doesn’t stop them from carrying out acts of kindness for others. I will never stop being amazed at the kindness and generosity of this wonderfully selfless community.
So whilst I hate CF and have moments where everything feels so miserable and heavy, I have to look at all the positives it has bought to my life, I have a beautiful little boy who is fighting hard and probably wouldn’t be the boy he is without CF and I can honestly say that without CF in my life I wouldn’t be the person you see today.
After weeks of waiting for a bed at Stoke Hospital for Chester to trial the new Nippy Clearway machine that should help him clear his airways we have finally got a bed, it’s been a frustrating wait, but I fully appreciate the need for beds for poorly babies. To be honest this has to have been the most stressful admission we have ever had and being in a different hospital than normal I find myself feeling a little “homesick” for our usual hospital, if that is even possible. We were lucky enough to have been booked in by a nurse we met before on another admission here but I still feel a little lost not being on our normal ward with familiar faces.
We were initially told his morning that there was no bed available, however, I won’t lie, that was a godsend because at the time I had literally just abandoned my car at my mother in laws after spending 40 minutes rocking back and forward in an attempt to get my car moving in the foot and a half of snow. That adamant was I that I wanted to make it to work, I found myself stuck literally seething that I faced abandoning my car on the middle of a roundabout.
After making my way home, with my children, with no car, I set about making a plan for the next day which included getting my car, getting kids to nursery and school and then getting to work. The phone rang and I recognised the hospital number, it was a call to say a bed had opened up and I was asked to bring Chester in this evening ready to start the Nippy trial first thing tomorrow.
Knowing we weren’t just going to be 5 minutes down the road like normal I literally packed for every eventuality which meant loading my car with way to many bags. I then realised that because of the snow I hadn’t been to get his antibiotics and I didn’t have enough to last. This meant once I had got my car dug out (with the help of friends), had dropped Oakley off with his Nanny I then had to take a trip to the pharmacy, which is on a hill, with a car park at the bottom, so like an a*^#hole driver I abandoned my car right outside the pharmacy, there was no way I was chancing the car park even with the shovel in my boot.
The drive to hospital was uneventful with cars abandoned all along to route, knowing what hospital food is like I made the decision to take Chester for a cheeky McDonalds on the way in, a decision which proved to be a great CF parenting win after they decided to weigh him on admission! All those fish fingers, nuggets and chips added a few grams, win win for me!
We came to the ward with all our bags and the nurses giggled at my pillows strapped on the outside of my bag, I know too well how awful the plastic pillows are and didn’t fancy waking up to my sweaty face stuck to one so opted as I always do for my own. Nothing wrong with some home comforts (I may have also bought my own mug so I don’t have to share, maybe I am just a snob).
Chester always settles in so quickly, helping me unpack his teddy’s and pyjamas before running himself a bath and proceeding to get in fully clothed, really helpful! He’s never phased and trotted off down the corridor with his nurse to be weighed, not even bothered I wasn’t with him. I know this is a good thing, as it means he won’t ever be worried being in hospital, which given his track record is a likely reoccurring event, but it always makes me so sad that it’s something he’s had to get used to. Some children will go their whole childhood never setting foot in a hospital and it’s no wonder it can be terrifying.
The new shiny all singing and dancing, expensive machine was already waiting in our room and Chester being Chester was straight in the box having a good look, I’m trying to remain hopeful that he will behave and accept this new treatment and not throw one of his terrible twos tantrums, but if I am honest with myself I know what’s coming!
I’m now here in he dark, writing my blog with the blinds that may as well be sheets of toilet paper blowing in the draft from the window, sat on my parents bed, which I have to add is definitely an upgrade from the paper and pencils I’ve become used to, listening to Chester snoring from his hospital bed. He literally doesn’t care where he is, he can sleep anywhere, except nursery because there are way too many toys to sleep! But once he gets that bottle of warm milk his eyes start to roll in his head and before long, he’s gone (yes health visitors out there, he does still have his bedtime milk in a bottle! Shoot me now!).
I can’t help but feel a little homesick still, we’ve become so used to being around the same faces, the same routines and rules that I find myself feeling a little bit lonely. Don’t get me wrong the staff are lovely and very helpful and it’s actually great to be left alone, I guess it’s just different to what we are used to.
I often think about the amount of time we have spent admitted to hospital, just under half of his life already has been spent in one hospital or another, then I wonder what the future will bring, will all these interventions and treatments pay off, or will I just have to accept this is just the path Chester has been destined to walk?
When Chester was diagnosed, I remember the Doctors explaining about CF, telling us what we would have to do to keep him well and being able to give a brief outline of things that could happen, with CF affecting each person so differently they couldn’t tell us exactly what to expect only the possibilities. Looking back now, all the chats we had were about what we should expect from Chester, we were never really told what we should expect as the parent. I am still on this constant cycle of learning about CF and there is always something new to learn, so I wanted to put together a short list of things that they don’t tell you about becoming a CF parent.
You will find you have a sudden fascination with all things poo related (well related to your child’s poo anyway) it will be entirely normal for your daily conversations to revolve around poo and you will regularly receive pictures of other people’s children’s poo which you will be required to review and compare and comment on, as much as it sounds weird and maybe quite disgusting, I have found this has become completely normal. You will also probably find yourself looking at other children’s poo wishing that your child’s offerings looked more like this child’s. poo envy is real and perfectly normal (I think).
Although Google is not always your friend when it comes to researching CF, it does have its uses and you may find yourself doing that much research you can basically be classed as a scientist, Vertex Pharmaceuticals may as well take you on staff right now, you have it all except the actual qualifications! Before you know it you’ll be talking chloride channels, gene therapy and genetic coding. Whilst you’ll be engaging with science buffs around the world it is worth remembering that it is more than likely that no one around you that isn’t affected by CF will have a clue what you are talking about.
If your child is anything like mine, you will almost certainly become a little bit of a pro at hospital admissions (not quite expert level yet but Chester is working on it!), you will get used to sleeping on a mattress that feels like you are laying on a piece of paper which has been placed on top of a row of pencils. You will wake with your body aching like you did 10 rounds in the ring with a pro boxer and you will feel like you have visibly aged after each admission. You will find it perfectly normal to pack your “Hospital clothes”, yes, I have two sets of clothes these days, ones for “normal everyday life” and then ones for hospital admissions. Making sure you have appropriate clothing for an environment that fluctuates from hot to cold in seconds is difficult but is much needed for any hospital stay.
You will find yourself getting used to your child making the most awful smells in public, whether it be filling their nappy at a really inappropriate time (praying it stays contained in the nappy) or just a bit of wind, the embarrassment will eventually cease as you gradually get used to the disapproving looks you get and you will eventually find it funny (on the inside) watching others choking on your sons vile smells as you queue to pay for your shopping. You’ll find it easy to clear the immediate vicinity of any enclosed space and you should be reminded that on an airplane there is nowhere to run.
You will know the fat content of every children’s snack going, in fact ignore that, you will know the fat content of every snack going, you won’t need a book or app to tell you which one is laden with fat, you will just be drawn to them from here on in, you will no longer be hunting for low fat, low salt, or low calorie treats, if it’s not loaded with fat it won’t be going in your cupboard, this does not help anyone who wishes to be successful in dieting!
Your house will go from a peaceful haven to being full of awful noisy toys, trumpets, saxophones, whistles, flutes and recorders, any parents nightmare! But you will learn to embrace the racket your out of tune, tone deaf child produces all in the name of physio, you may find yourself donning your ear defence or if not lucky enough to be the owner of such items sitting with your fingers, but deep down you will be pleased that racket is shifting stubborn mucus! If you live in a semi-detached or terrace house, your neighbours may actually end up hating you.
Funny things aside there is a serious side to this as well, sadly one of them is that you will lose friends. It’s inevitable, I’ve lost count of the friends who have un-friended me, it’s made my Christmas card list shorter that’s for sure! Although it used to bother me, I don’t let it anymore, I’ve put it down to the fact some people just don’t know how to cope with CF or they don’t know what to say, but in reality people lose touch all the time when their paths go different directions, so as sad as it may make you feel the most important thing to remember is that the people you can rely and depend on and that actually matter will stick around, they will stand by you and you’ll see they are the only ones that really matter, I am fortunate to be surrounded by some of the most amazing people.
On top of this the CF community is one of the most amazing communities, I’ve lost friends, but gained so much more, I gained a whole family, you will never again be alone and you will certainly not make the CF journey on your own. There will always be someone there that knows how you feel, knows what it means to be a CF parent and completely gets it.
As I sit here watching the gentle rise and fall of my sons chest as he sleeps, I can’t help but think that despite everything, I am unbelievably blessed, I have made not only one, but two beautiful tiny humans.
Whilst I fully understand that some women do not wish to have children, and I completely respect their right to decide, there are some women who will spend their lifetime wanting what I have right here, OK they may not want the Cystic Fibrosis aspect, who does? But to be able to create their very own little beings and to be a Mother will be something some women long for but may never experience and honestly I cannot even begin to understand how that must feel and it makes me even more grateful to be sat next to this little guy right now. CF or not I am a mother to two perfectly beautiful boys.
When I decided I wanted children I knew that it would mean caring selflessly for them, both physically and emotionally, I knew that I would no longer be able to think about just me and what I wanted in life, I would be the least important person and I knew over time the job description would change and I would also become the cook, cleaner, washer woman and taxi driver amongst all the other jobs a parent is expected to do.
Along came my children and suddenly I found myself with an added job, not one which I seemed to recall being in the job description and one I certainly hadn’t planned on my ever doing, that job was to be my child’s nurse.
Of course I knew I would be expected to put a plaster on a cut knee or elbow and sit next to a hospital bed and wait for X-Ray results to show broken bones but becoming a full time nurse was not in the description.
So here I am now, a Mother nursing a child with a difficult, heartbreaking chronic condition and although it’s been a steep learning curve, I’m confident in my ability and am content that most of the time I know what I am doing and why I am doing it but I have to be honest there are often days when I really struggle with doing everything that is expected of me.
As with many other CF parents I often find myself faced with incredibly difficult decisions relating to his care, decisions that if I was a professional with only a professional interest I would find far easier. But as a person with a vested interest in his care, a lot of my decisions are clouded by my personal feelings and this makes things so much harder. I see now why patients admitted to hospital are not allowed to be treated by family members; it makes it far too complicated.
The decisions I am faced with generally have huge implications and not only affect his quality of life and how we do things now, they can also have serious implications in his future.
Making the decisions like consenting to operations and procedures is difficult. Each surgery poses a different risk and each treatment has potential side effects or long-term complications and sometimes it’s hard to know what to do for the best.
As a parent I am supposed to keep him out of harm’s way, and in a roundabout way I feel like I’ve slightly failed on that part. He should be able to come to me for safety and comfort. Yet there have been numerous times when I’ve found myself assisting the Doctors to restrain him for painful procedures, seeing his sad eyes look to me to make him safe and then realisation that I’m not stopping them is just heart breaking. Right now he’s too young to understand that I’m not doing something I want to do, rather doing something that will help him, and I am thankful that he doesn’t yet hold a grudge.
I am blessed to have recently been trained to do my child’s IVs, port access has given us more independence and a little more control over his care, but it is also a huge responsibility, with the line directly accessing his heart extreme care has to be taken not to introduce infection or air bubbles. It has to be 100% sterile and there is no room for error.
Juggling both roles is hard, I know he needs to do certain treatments, and as his nurse I have to make sure that he is doing them and doing them correctly, I have to make sure that he is getting the best start including medically, and at times that means being stubborn and pushy when in all honesty I want to just sit down and play cars, have cuddles and just be mum!
I know I am far from alone, my social media feeds are full of amazing CF Mums and Dads who are also taking on both roles. I often sit down and think about the sheer gravity of what we do, the drugs, IVs, night feeds, nebulisers, physio and ventilators and it’s terrifying, but despite this I wouldn’t have it any other way, I jump at every chance to take back some control, even if that means being jack of all trades, including my child’s nurse.