Dream Big Little One!

We all have high hopes and expectations for our little ones, as a parent its human nature to want to see them succeed.  So, when Chester was diagnosed with Cystic Fibrosis, I felt like all those hopes and expectations were on hold and out of reach, because his future was too uncertain.  I understand no one’s future is promised, but when facing the diagnosis classed as “Life limiting” I found it too difficult to look to the future, we just had to live in that moment, one day at a time, whilst trying to comprehend the situation and come to terms with the uncertainty.

Days old…First ambulance journey

It’s also normal to wonder what sort of person your child will be and what they will become, will they travel, live a nomadic life, study for a profession and become a Doctor or a Scientist?  The reality for any parent with children Chester’s age, is that it’s way too soon for all that and they have a lot of living to do before they are in any position to make such decisions, but the ability to ponder those questions was just something I couldn’t bring myself to do.

Another admission

For me, those are the questions that have always been tough, and I have to admit to actively having avoided thinking about Chester’s future.  With his health all over the place, endless admissions resulting in huge chunks of time out of school, I always had to consider the fact that there would be certain things that were clearly going to be unattainable for him……………. that was until now!!

Yesterday was the first anniversary since Chester started Kaftrio and what a year it has been!  Kaftrio has simply transformed our lives, none more so than for Chester.  It has quite literally been a life saver!

It has given us back the most precious of commodities, time!  Not just in terms of life expectancy, but all that time that would have otherwise been spent in hospital or doing treatments.  It’s given Chester the chance to live a relatively normal life, allowing him to get involved with his friends and to take part in activities that once seemed almost impossible.

It’s been so life changing that from day to day apart from the morning and evening treatments and his medication with food, we often don’t even think about it, life feels more “Normal” than it has ever been since his arrival.

Pre and post Kaftrio

This past year has seen Chester progress from being unable to walk 100 metres to school without stopping to catch his breath or rest his legs, where we had to use wheelchairs to help him get about, to a child that is bounding around sports pitches taking part in sporting activities that at one point he could only ever dream of doing. 

Being unable to take part in most physical activity, Chester turned to TV where he began to watch sport, inspired by sportsman Nathan Charles, the former Bath Hooker, who played professional rugby despite having CF, Chester became more and more interested in the game.  A sport that prior to Kaftrio would have been near on impossible for Chester. 

Nathan Charles – Chester’s hero!

A year later and Chester is now an active member in the local Amesbury kids Rugby Club where he trains, keeping up with his teammates and even scoring the odd try.

Defensive Line!

Now, hoping not to tempt fate, Chester has been admission free for 71 weeks, that’s 16.5 months or almost a year and a half!  For us, that is a cause for huge celebration, considering pre-Kaftrio we were averaging 4 weeks a year minimum for admissions into hospital for IV antibiotics.  His health has improved so much that we have even started discussions about having his port-a-cath removed, which will remove even more restrictions for him and will allow him to progress to full contact rugby, which is currently his ultimate goal.

I have always been honest, never holding back when admitting just how tough the early years were, when we had to take each day as it came, overcoming each set back as they were thrown at us, and I look back now at the weeks and months we spent in hospital, the endless nebulisers and physio treatments, the sleepless nights and stresses strains and anxieties that CF bought for all of us, and I am so grateful that we now find ourselves with this happier story.

This isn’t a cure and this isn’t the end of his CF, I know there will be the curve ball when we are least expecting it, but for now, we are going to embrace life, enjoy the freedom Kaftrio has given us and I make no apologies about being the “Shouty Proud Mama” on the touchlines screaming and cheering for my child, because I know the sheer grit, strength and determination he has had to show, to get him to this point.

Living his best life at “The Hooters”

It’s your time now Chester, no dream is too BIG!

Time flies! 6 months on Kaftrio.

It’s true what they say, time really does fly!  It’s amazing that it’s already been 6 months since we were fortunate enough to get our hands on Chester’s first prescription of Kaftrio and what an incredible 6 months it has been!

Day one of Kaftrio

To help you understand just how revolutionary Kaftrio is…..it is definitely worth me explaining (or at the very least trying to explain – bearing in mind I am not a scientist or a doctor) the science behind it…here goes….Cystic Fibrosis is caused by a mutation of the cystic fibrosis transmembrane conductance regulator gene (CFTR), which regulates salt and water transport in the body (measured as sweat chloride). The CFTR mutation allows too much salt and water into cells and results in a build-up of thick, sticky mucus in the body’s tubes and passageways. 

There are lots of different mutations in the CFTR gene and each person with CF will have inherited two faulty genes, one from each parent, these aren’t always the same copies.  In Chester’s case he has one “Common” mutation and one that is more rare (DF508/F507Del).  Anyway I digress, in the past CF treatments have been mainly preventative, such as chest physio to prevent the build-up of mucus and the use of prophylactic antibiotics to prevent chance of infection, the ultimate aim to prevent irreparable lung damage.

Kaftrio is a triple combination of drugs that effectively fixes the faulty genes that cause CF, allowing the salt/water to move between cells as they should.

The anticipation of starting this new drug left us feeling overwhelmed, there were so many hopes being pinned on Kaftrio.  Chester didn’t qualify for the Orkambi and Symkevi rollouts and having suffered a significant decline in health post exacerbation in December 21, which hospitalised him and left him in quite a fragile state, it really was hard not to get over excited and we had to remain grounded.

Still smiling

On Mon 28 Feb 22, having had his height, weight and lung function recorded, our day finally arrived and Chester took his first dose of Kaftrio!  I was excited and nervous, this was what we had been holding out for, it was a huge deal, that said the worries that it wouldn’t make any significant difference were real!

His starting numbers were poor, lung function was sitting around 74% and his weight at 20.8kg was well below where we needed it, placing him on the lowest end of the percentile scale with a BMI of 13. We had reached a point where we were actively discussing interventions including surgical options with his team if there were no significant improvements on his weight. 

We watched him like a hawk for any signs of side effects, trying to remain positive that he wouldn’t suffer any of the severe side effects that had been reported and that would result in us having to stop the Kaftrio.  We were lucky, he remained tolerant which was the first big hurdle. 

The first noticeable change was well within 48 hours after he started, his skin was noticeably smooth and not clammy like normal and he was not as sweaty, but by far the most incredible change was that he didn’t taste particularly salty. 

For those that have read my previous blogs, you’ll know that as a CF Mum, I have been known to lick Chester’s arms and face to see if he is in need of any salt supplements and indeed in the very early days when we were waiting for the genetic testing to come back to confirm the CF diagnosis.

As the days passed by Chester became increasingly energetic, bounding around and full of beans, actively wanting to get involved in physical activity, a real change from the little boy who had previously struggled to walk 10 metres without needing to sit down and get his breath back.  There was a significant change in his confidence and it was amazing, watching him take deep breaths filling his little lungs with oxygen was simply incredible.  Chester would often say he felt weird, like his chest was empty, unable to fully understand the significance of what it meant. 

We were relatively lucky as far as side effects went, there were a few minor setbacks, he became very moody and did suffer with insomnia for a short period of time which left us all sleep deprived, but that came and went and were soon back on track.

Chester’s most recent weight was 23.8kg, whilst not a huge gain, it is a whopping increase for him, he is now out of age 6 clothes and is rapidly outgrowing his age 7 ones, his BMI is up and he’s filling out. 

Day one and six months in – what a transformation

His lung function test has hit late the 90s and he even managed to blow 110% on one test, although not sure how much of that might have been a fluke!  His numbers are all headed in the right direction which is the best news.

We recently asked our team to re-run Chester’s sweat chloride test which they a week ago, this was the test that the hospital used to assist in the diagnosis of CF when Chester was smaller.  His original reading was 115 mmol/L which fell into the category well above 60mmol/L indicating CF. 

Chester’s most recent result post Kaftrio was significantly lower at 43 mmol/L, which is literally just over the “CF is unlikely category”.  I have to admit I was initially a little disappointed with the result, especially knowing others have had their salt levels come back as low as 7 – 10mmol/L, but having taken time to reflect, I have to say that it really is still an amazing result.

The results to date have all been extremely positive, it is imperative though to understand that Kaftrio is not a cure but for now it’s the closest thing we have and the work to keep Chester well continues.  Chester’s treatments currently remain the same, with a routine that includes chest physiotherapy twice daily, nebulised medications to continue to thin the mucus, pancreatic enzymes to continue in assisting fat absorption but what is important is that his quality of life is massively improved, physically he looks better and more importantly for a boy of his age, he can keep up with his friends when playing and running around.  He recently ran the “Race for life” at school, managing to keep up and even overtake some of his class mates and has now signed up to the local Rugby Club where he has started training.

The next big test is to see how he fairs in the winter months, with the normal bugs and infections doing the rounds, but we remain hopeful and will continue to keep everything crossed for a winter with no exacerbation and no admissions.

I remain forever grateful to the CF community who we consider family, who tirelessly campaigned to make this drug available for our children. 

Kaftrio has given Chester a new lease of life, a new found confidence and the chance at a future unlimited by CF.

Day one of the rest of his life

Today is the day, his time has finally come.  We’ve been waiting for what feels like an eternity but today, it’s our turn, our delivery is finally here and holding this box of pills in our hands after all the campaigning, protesting and hard work by so many beautiful people in our CF family, I am so overwhelmed and beyond grateful, words cannot express how I feel right now.

The best delivery ever!

In the early days after Chester’s diagnosis I couldn’t bear to think about the future, it was too hard to look past the moment, because the future felt so uncertain and I couldn’t bear to think about existing in a world without Chester.  I spent much of my time grieving for a life and a future that we weren’t sure we would ever have. 

But this box, sat on my coffee table in front of me, contains so much more than just pills.  It’s filled with promises and hope, promises for more time and the chance of a normal life. 

I am excited for Chester to finally feel what it’s like to be able to breathe, actually inhale a deep breath that will fill his once logged lungs, for him to experience the ability to laugh without coughing or to walk up the stairs without stopping to take a seat whilst gasping for breath. 

This tolerant, kind hearted and beautiful little boy deserves this, he’s been through so much, he’s the strongest person I know.  

I can finally look to the future without fear, to Chester’s future and it looks so bright.

It’s your turn Chester, I’m so very excited for you.


And then he was 6!

And just like that he is 6….Where the heck did that time go?  I can’t quite believe I have been loving this little fella for 6 whole years!  As a CF parent, I go absolutely over the top on Chester’s Birthday and for obvious reasons, but I find as much as it is a time to celebrate another year of Chester absolutely smashing life and kicking the hell out of CF, it’s a very difficult time for me personally.  My social media accounts lovingly reminding me of the moments that my fellow CF Mum friends and I often refer to as the “Blissfully unaware” moments, which can be very difficult, the moments where I really had no idea what was about to happen and how much my life was about to change, where life was…. well, carefree and simple.

I find that as the years have gone by, there are certain things that still really get to me, the last photo of my bump before Chester was born, the Facebook post announcing that I had given birth to a Baby boy that say “Mum and baby doing well” and all the hundreds of pictures we took of that little salty bundle in the hours after his arrival as we marvelled over our new tiny human.  As well as the photos and the social media posts, there are a whole host of dates etched in my memory, that as of today I know are imminently inbound and I am approaching those as we move towards the dreaded diagnosis day later this month, so I am now mentally preparing myself for this emotional roller coaster ride of a month.  I find myself wishing I could just forget them, that I could push them out of my mind and carry on with life, but I have never quite managed to do this, I’m still learning to live with CF…..but as time goes by, who knows, maybe one year. 

Today, on his special day I am full of emotion over the little things.  I look back at all this little guy has accomplished, the fights and battles he has had with CF, the numerous conditions he has since been diagnosed with and I can’t help but look at him in absolute awe.  He takes everything as it comes, generally with a smile, his resilience and adaptability are second to none.  He has faced endless admissions, has been poked and prodded, scanned and jabbed and has been so debilitated he was unable to get out of bed without getting out of breath.  I have seen him so poorly that I honestly thought we would lose him, but every time he fights back with a strength that I have never seen in anyone else, nothing seems to faze him, he has started school, he has travelled and he has even climbed Snowdon which was no easy feat, he is doing things some kids without CF couldn’t even accomplish and that is the true testament of his strength, determination and courage. 

I look back at when Chester was small, when I prayed for the time when life would become a little easier, when he would be mobile and more active so he could help shift mucus on his chest alone by jumping, huffing and coughing, when he would be more physiotherapy compliant and help to do his own treatments, and for the times when he would be able to talk to tell me his belly was sore, or to tell me he was feeling short of breath.  The reality is that whilst some aspects are getting easier, we are faced with new and sometimes tougher challenges.  

Chester is becoming more and more aware that he is different to other children, he takes pills, he does physio and there are activities he isn’t allowed to do and this brings new challenges and frustrations.  As he becomes more aware I am growing ever conscious that I have to do what I can to protect him from the reality of CF until he is old enough to fully understand, this means as with any child to be honest, closely watching his online access and explaining as carefully and as truthfully as I can about CF without having to disclose the full facts.  It is difficult but we are managing it, I know that there will come a time when we have to have those difficult types of conversations, but for now I am not ready for that yet and with life expectancy ever increasing with new drugs and treatments, I pray that by the time those conversations reach us the news will be much more positive.

So in the meantime here’s to another Birthday, to lots of cake and especially lots of candles to blow out, but more importantly here’s to a weekend of constructing Lego sets.

Happy Birthday Chester, keep kicking butt!

Out into the big wide world they go….

As our social media newsfeeds fill with ‘First day of School’ pictures of their children stood proudly by the front door in their new shinny school uniforms, mine included, I am filled with an abundance of emotions that feel somewhat overwhelming.

As a CF parent the path leading to this exact moment has been full of hurdles and difficulties that have had to be overcome, but more than anything it has taken a lot of behind the scenes work to make sure that we are ready to send him for his first day at big school knowing the school are content with his care and are therefore able to keep him safe. 

I am also relieved that Chester has been fortunate enough to be well and able to start and I can’t stop thinking about those of his friends with CF who are unable to start school as planned because of admissions for CF. CF really is crap!

 I am full of the usual parental nerves, apprehensive as to how he will settle, wondering will he behave, will he understand what is asked of him and most importantly will he enjoy it?

But today, the day before he starts, I find myself feeling overly apprehensive. We have done everything we can over the past four and a bit years to keep Chester well and most of all safe.  I am faced with handing over that responsibility to others, some of which will be complete strangers who may know very little about CF and it’s scary. 

Don’t get me wrong, I am incredibly appreciative of the school taking the time out to read up about CF and for meeting with me numerous times to discuss this next big step and I know they will do their absolute best, but as someone with a vested interest in making sure my little boy is kept away from harm I feel anxious that neither I, his Daddy nor his Nanny (who normally cares for him) will be able to be there for him all of the time and will have to rely on others to be there to watch out for him.

 I am fearful of him being rejected by his classmates, whilst he looks no different and, in most ways acts no different, there will be activities he won’t be able to take part in and there will be activities he may at times find hard and struggle with.  Plus popping pills with every meal will, I am sure be overwhelming for some of the children in his class and will most likely lead to questions.  My biggest fears are that without meaning to be, children can sometimes be cruel, and everyone knows someone that is a bit ‘different’ is often an easy target.  I just hope that the children he will be sharing these early years with are kind.

I worry that I can’t protect him from things he may here, this is especially worrying when he starts to hear about what CF is and what it means to have it….I’m not ready for that conversation just yet.

I am nervous that he may be inadvertently placed into situations that put his health at risk, whether that be being around other poorly children which I have literally no control over or sneaking off to play with mud kitchens or stagnant water because I know how difficult it can be to watch him all the time, and after all, he is a boy and this boy just wants to be like the other boys.

I worry that he will struggle to keep up, having to take time out for hospital admissions and treatment which will see him fall behind but like any parent I want to see him succeed, I want the very best for him and I don’t want CF to hold him back.

 More than anything I am bursting with pride. From day one he has amazed me with his strength and sheer determination. His ability to take anything thrown at him and carry on astounds me. He is simply incredible and I wish for incredible things for him. If I could give my boys the Earth I would. If I could trade with Chester, I would, in a heartbeat.

There were times we didn’t think we would see this day and I am forever grateful that he is still with us, I know that in the Cystic Fibrosis community alone, there are parents whose beautiful children have gained their angel wings and my heart breaks for them.

I know only too well the unpredictability of CF means we never truly know what’s waiting for us around the corner and the harsh reality for me is that whilst I am truly hopeful for the future of our CFers I also understand that we just don’t know how many First day of the school year pictures we will get to share. So despite the moaning and groaning from the few about the clogged up back to school posts in social media news feeds I’m going to stick my fingers in the air and carry on sharing my proud Mummy moments!

Dearest Chester, go spread your wings little man, for I know you are going to accomplish great things.

Now I’m off to sob about the fact my baby is going to school 😭

An open letter to Matthew Hancock the Secretary of State for Health and Social Care – Orkambi access

This is my son Chester, he is 3 years old. He’s funny, cheeky, kind, affectionate and is literally always on the go, rarely stopping for anyone or anything. If you were to see him at pre-school surrounded by other children his age, you would see he looks and behaves no differently to his peers.

Whilst he is no different to look at, Chester is in fact very different to his class mates, because Chester has the genetically inherited condition Cystic Fibrosis. Right now, his future mental health, the quality of his life and his life expectancy, are hanging in the balance.

For years the Cystic Fibrosis community has waited with baited breath for a much-needed cure, or at the very least have held out for a treatment that treats the underlying cause of Cystic Fibrosis as opposed to just treating the symptoms. Imagine the excitement when Orkambi came along! https://cysticfibrosisnewstoday.com/orkambi-lumacaftor-ivacaftor-vertex/

Yet we find ourselves in a predicament where despite a treatment being readily available in Countries across the world, we still do not have access in this Country!

In the three and a half years since my son was diagnosed I have witnessed too many parents having to bury their children and as a small and extremely close family like community the death of a child with Cystic Fibrosis affects our whole family. We mourn and our hearts break for those families, knowing all too well that as things stand that could one day be us!

Having watched the House of Commons Health and Social Care Committee inquiry on the availability of Orkambi on the NHS, I was left feeling incredibly deflated and let down. Somehow the welfare of my son and his future have become lost in a battle over finances. I think it’s time that the focus was shifted back to what this medicine can do for those up and down the country who are living and fighting this cruel condition on a daily basis.

Cystic Fibrosis has a huge impact on Chester, but also on our family. There isn’t one aspect of our life that Cystic Fibrosis doesn’t affect or impact on, from holidays, days out, activities or a normal daily routine Cystic Fibrosis always finds a way to interfere and wreak havoc. As much as I dislike the word, Cystic Fibrosis is a ‘burden’ on our family and the impact is felt by all of us involved in Chester’s life. From his brother having to take a backseat whilst he has treatments, to having to take time off work because Chester has appointments or is admitted to hospital because he is unwell.

Chester is still too young to understand the implications of his condition, and I dread the day I will have to tell him, because right now I find it incredibly difficult to find the words to explain the enormity of his condition in a way that he will understand. I worry about his health, I worry about how rapid any decline may be and on top of the physical implications I worry about his mental health, if I am struggling to deal with his diagnosis, how will he deal with something this big?

Having already spent approximately 25 weeks in hospital for bowel obstructions, exacerbations, pseudomonas growths, port surgery and bronchoscopies, much of his early life has been lost to sickness and admissions. That time doesn’t account for the additional courses of 14-day IV antibiotics we have been lucky enough to manage at home, or the monthly clinic appointments, the port flushes, the cough swabs or the additional check-ups when a cough is developing. It also doesn’t account for the hours of physiotherapy and nebulisers he has to do every day without fail as part of his daily routine just to keep him well. Given how unwell Chester was over his first years and the amount of treatment and admissions he required, I would anticipate his NHS bill to date to already be well above five figures.

Whilst Chester is front and centre in all this, I cannot dismiss the impact this condition has on the family, it is also huge, whilst I am extremely fortunate that my employer is incredibly supportive, and I have family around me to help and to enable me to continue with my chosen career, some haven’t been so lucky, having to give up everything they worked towards to become their child’s carer, always without a second thought. This can lead to a financial burden within the family and hardship when two wages are dropped to one.

Cystic Fibrosis is mentally and emotionally draining for all involved, there is always something to worry about, most of the worries are completely outside of my control, sometimes it doesn’t matter what I do, my child will be at risk and I have to live with the guilt of putting him in that situation every day.

Imagine having to sit up at night, tending to a breathless, restless child, or a child who can’t stop coughing and is exhausted just from sitting up and just breathing, imagine having to put your child through pain and suffering because in the long run it’s better for them. Imagine knowing as a mother I should be able to comfort and protect my child but can’t, it is painfully unbearable, this is my reality.

I wouldn’t wish this condition on my worst enemy, its cruel and it’s heart-breaking. I should be excited to watch my child grown up, to sit and wonder what he will be and what he will achieve , and I should be able to enjoy celebrating his birthdays’, but I live in fear that I could lose him before he reaches adulthood, the fear he may never get to experience life as it should be. No parent should live in fear of having to bury their own child!

The argument for Orkambi and other precision medicines has gone on so long, leaving the Cystic Fibrosis community emotionally drained, this shouldn’t even be a fight! Just when we think we are getting closer to a deal, our hopes are shattered and we end up back at the start. Time is passing far too quickly and for those that need Orkambi now, time is running out, and sadly for many it is already too late.

Whilst I am fully appreciative of the argument of clinical effectiveness versus cost effectiveness, especially when our NHS is on it’s knees financially, finding that we are being priced out of a drug is unacceptable and having no one to fight that battle is even worse. My child’s life is worth more than this!

A resolution is needed now!

No one in this negotiation can seem to come to an agreement, and no one seems to want to take charge. We need someone to get the parties involved around the table, take ownership and responsibility to ensure this deadlock is overcome.

My son didn’t ask for this condition, and I would give all I have to take it away, but we are where we are, nevertheless, he should be able to live with this condition with the same kind of normality as any other child, and where there is a drug that could help, that is widely available, there is no doubt in my mind that access to it should be a given.

I am appalled that in a time when mental health and emotional well-being is a hot topic and in some cases deemed to be a high priority, the mental health and well-being of Cystic Fibrosis sufferers and their families is being completely overlooked and ignored all in the name of money.

My son deserves more!

All the things I wish for you….

My bed has been invaded, it’s a king, but still I find myself pushed right to the very edge to make way for my poorly boy who has snuck into my bed, literally going back to sleep the minute his sleepy little head hit the pillow. I can feel the warmth of his breath on the side my face and I can feel the rattling of his chest each time he breathes in. He’s hot and clammy, yet his little pig-sticks (the word for feet in our house) which are nestled into my side are freezing. I know he’s not well and I feel completely powerless, as I often do when CF throws us these curve balls.

Now, I don’t often allow either of my children to co-sleep, but after a night of broken sleep and with another full day of work tomorrow, I’ll honestly hold my hands up to doing whatever I need to do to get some sleep tonight.

Every time he coughs I cringe, I can’t help but picture the irreversible damage this cough is doing inside, the scarring any infection might leave behind. The invisible damage we work so hard everyday to prevent or at the very least slow down.

We have been incredibly lucky this winter, with no real cough or cold and no requirement for him to go onto his back up antibiotics, this has been (touch wood) his best winter yet! But now I find myself nervously and impatiently waiting for the results of his recent cough swab, my mind now running off to thoughts of bacteria growths and IV antibiotics. Wondering if there is something sinister down there growing, lurking……just waiting. The worry that a cough swab is negative just because it didn’t pick up the growing bacteria, is a thought always at the back of my mind.

Having made the decision to start him on a 21 day course of oral antibiotics to head off any bacterial infection, I am also drawn to thinking about antibiotic resistance, knowing Chester has already had prolonged periods of time on antibiotics it is a worry that we will start to run out of antibiotics to rid future infections.

I also find myself retracing my steps day by day over the past week, since his symptoms first started. Where has he been, what has he been doing? What and who has he had contact with, what has he touched. It’s clearly impossible to tell where the infection came from or what caused it, but it doesn’t stop me wondering if I missed something or did something wrong. Perhaps I didn’t do his physio hard enough or for long enough, perhaps I should have been more insistent that he do more.

I think about how shitty this boy has it, the things he has to go through and how I would do or give all I have to take it away. I think about all the things I want for him, the things many of us would normally take as a given and I wonder what great things he will go on to accomplish.

Having spent the day walking around like a sleep deprived zombie, I am hoping that he will settle and I will manage to get some sleep. I find myself hugging and holding him so tight, not wanting to let go, thinking of all the things I wish for him.

A brick to the face.

With several hospital admissions under our belts, we sadly seem to have become experts in the packing of hospital bags, remembering the “Comfy items” needed for a comfortable stay is a priority!

Topping my list is a decent jar of coffee, however, nothing beats us having our own pillows, cups and of course our slippers.  But as expert as we have become, no matter how many times I pack those bags, when I know the admission involves a trip to theatre it still fills me with nerves.

The purpose of this admission was to take samples from the lobes within the lungs to check for any growths, Chester’s history of growing pseudomonas has been problematic and having stopped antibiotic nebulisers at annual review in September on a trial basis, a check-up was required to ensure nothing sinister was lurking that hadn’t been captured on his cough swabs.

Following his first bronchoscopy, Chester was diagnosed with severe tracheomalacia and bronchomalacia, essentially floppy windpipe and airways, in some locations, up to 90% obstructed, which as you can imagine on top of CF is not the ideal combination, thick sticky mucus already clogging the airways, on top of severe obstruction makes an ideal bug breeding ground, and has potentially been the reason he has suffered from so many infections.

The hustle and bustle of medical professionals coming in and out prepping him for theatre helped to keep me preoccupied.  The care assistant weighing him, the nurse accessing his port and the anaesthetist doing their bit, followed by the Consultant explaining what they intend to do, how long it should take and detailing all the risks before asking for me to sign on the dotted line.

Having not needed a pre-med this time, Chester, being the independent, ‘I can do it’ little person he is, when given the choice to ride on his bed or walk to theatre, he obviously decided he wanted to walk.  Watching him trundle along in his new slippers and PJ’s behind his bed and a team of porters and nurses without a worry in the world made me feel immensely proud, yet incredibly sad.  Proud that nothing seems to faze him, but sad that this situation, whilst terrifying for most is something that he appears to be completely at ease with, like it is perfectly normal.

Sitting in the brightly lit, sterile smelling theatre with Chester cradled on my lap watching the IPad, the theatre staff bustling away behind me, checking their equipment, my nerves kicked in and my leg started to shake, I could see the anaesthetist pushing the white liquid anaesthetic into his port line and he gave me the warning that Chester would go under quite quickly.  I could feel his little body starting to slump as he started going limp, his eyes rolling, the physiotherapist who came in to watch the bronchoscopy took hold of the IPad for me before it dropped to the floor, and that was it, he was gone, fast asleep snoring.  As I laid him down on the bed fighting back tears, I gave him a kiss on his cheek and left the room, leaving him yet again in the hands of complete strangers.

I found myself trying to leave the theatre as quickly as possible as I had begun welling up, I stupidly felt embarrassed at feeling tearful and upset because he was only in for a bronchoscopy, but to me, no matter how many times I hand him over, it doesn’t get any easier.  I dashed off to get a coffee and some toast, not only because I was starving from not having had anything while Chester was nil by mouth but also to keep myself occupied, I then went back to his empty room to wait, wanting to be there when he came back.

Although the waiting felt like an eternity, it wasn’t long before he was back, fast asleep, snoring and hooked up to a SATs monitor which continuously bleeped, his face covered by an oxygen mask, the nervous wait to watch his oxygen levels rise to where they should be so the oxygen could be slowly turned down. Sat watching the numbers hovering around the 93% mark willing them to hit 95%.

I patiently waited for the Consultant to come and let me know what had been found.  The news he came with wasn’t great, he explained that despite their hope that the airways would have strengthened as he had grown, the obstruction was still there, and still just as severe, on top of that there were vast amounts of secretions in his lungs.

I felt my heart breaking all over, like I had failed him, that despite all our efforts in religiously carrying out his treatments and physio and going all out to ensure he was active, it all felt like it had been for no reason, that nothing we are doing seemed to be working.  I could see that the Consultant was disappointed that he wasn’t able to bring better news.  He advised the next step would be to discuss with the physio and our Consultant and try to devise a plan.  He left, leaving me feeling completely deflated.

I tell myself all the time not to get too complacent, because I know too well that when I have done in the past, something has come along and knocked me off my feet, but it seems I had forgotten my own rule again.  When things are going well it’s all too easy to think that we are doing well, especially when Chester has shown no symptoms of cold, flu or infection (touch wood) and he has had no bug growths, so to hear this, was completely unexpected and it served as a reminder that CF is unpredictable, that no matter how well someone can look, we never really know what is going on inside. My friend, a fellow CF mum explained that news like this, out of the blue feels like being hit in the face with a brick, and it’s so true, that is an excellent way to describe how it feels.

Despite feeling completely down in the dumps, like a complete failure, terrified again for Chester’s future, I am forced to remind myself to look at how far he has come, through the various obstacles placed in our path, each sent to remind us that CF is an unpredictable beast.  The hardest thing will be picking myself up again, but I know that I must be positive and stand by Chester ready to take on whatever we need to do to ensure that we do the best for this little guy who just keeps on fighting.

The power of positivity.

As the parent of a child with a chronic condition, I have faced my own emotional turmoil since Chester’s diagnosis.  I have often described it as a rollercoaster ride, and I don’t think I am far wrong.  I can literally go from high to low in a matter of minutes when faced with a difficult or unplanned situation.

In the early days, post diagnosis, I struggled to cope with my own negativity, I became so focused on the negatives of his condition that I couldn’t help but think of all the things that could go wrong and all the things he might not be able to do, my mind was full of worry, his life expectancy being my biggest fear.  There were even times when Chester was small I found myself afraid to go into his room to check on him through fear of what I might find and I painfully found myself thinking about life without Chester in it.  I worried so much that I felt like I was under a dark cloud that would not shift.  My negative mindset left me struggling to focus and I look back now, angry at myself that I let that black cloud take over, so many of my early memories of Chester tarnished and time lost that I won’t ever get back.

It is all too easy to fall into the cycle of worrying and asking myself “What if” and I am conscious that once I start falling into that black hole there is a long and difficult climb back up to get out.  They often say that time heals and I agree in a sense that it does, I don’t think I can ever be over the fact Chester has CF, but I think time has allowed me to accept that this is how things will be, there is nothing I can do to change it, but what I can do is make the most of what we have.  I know only too well that a negative atmosphere can breed negativity and I would not want my negativity to rub off on Chester, or on Oakley and it would be unfair of me to be the cause of that.

I am conscious that it isn’t about me at all, this is about Chester, this is something that clearly affects me but it is important to remember that in all this, he is the one living with CF and he will be the one battling with both the physical and emotional strains of living with a chronic condition.  Therefore, his perception of his condition is important and as a parent it is and will continue be my job to ensure that he remains positive and fights whatever CF throws at him.

I have put my own ability to manage and cope with how I feel down to remaining positive and I strive to show both my boys that despite everything there is so much hope, I now have the ‘He may not be able to this, but look at what he can do’ attitude and I strongly believe it is extremely important that I surround my boys with that positivity, after all the saying goes “Positivity breeds success” and I whole heartedly agree.

I am under no illusion that it can be difficult to be positive when times are hard, and whilst one person may be fortunate another may not, but the least I can do is hide the hard times from them, there is a time for me to feel down and sad, but it is not in front of them.  I have stopped myself from ‘Google research’ and I have unfollowed negative social media pages. I have also strived to find positive role models with incredible stories that I share with both boys, and in doing so have stumbled across some truly amazing humans with stories I regularly share with my boys.

The positivity I have from seeing people living with CF, doing the impossible, breaking world records and completely changing perceptions of people with CF is the much-needed boost I needed as a parent.  Seeing people like Ben Mudge and Sophie Grace Holmes sharing their fitness journeys and inspiring young people to do their treatments are just the types of role models that newly diagnosed parents should be pushed towards.

As for the absolutely incredible achievements of Josh Llewellyn-Jones attempting and smashing a world record attempt by lifting 1 million Kilos in 24 hours are just simply beyond words, far beyond what I ever expected a person with CF to be able to do and I am truly grateful that he continues to share his own CF journey with us.  If my son has half the determination Josh has displayed I will be one proud Mumma, and I will know I got it right!

I only wish I had stumbled across Sophie, Ben and Josh far sooner post diagnosis! Perhaps it would have saved me from hours of trawling through depressing out of date statistics.

They have individually helped me to be at peace with the fact that despite his condition, his fate isn’t sealed and on top of that I think it is extremely important for both Chester and Oakley when they able understand the extent of CF, to see that his condition doesn’t have to mould his future, that despite CF he has the ability to do and be whoever and whatever he wants, and that with hard work, grit and determination, success is well within his grasp.

A letter to my child without CF.

To my biggest boy,

You held my hand as my world fell apart with Chester’s diagnosis and despite trying my best to hide things from you, you have seen me at my worst. You have sat by my side through my darkest of times and without even knowing you have helped me to find the courage to face the world and see that everything will be ok.

You have sat on my lap, snuggling under an itchy blanket next to Chester’s hospital cot, you have spent nights sharing a cramped foldaway bed being woken periodically by the alarms on Chester’s monitor or the noisy nurse clattering around in the dark and you have you have waited patiently for Chester to fall asleep to be able to escape his room and go to the playroom.

You have had broken sleep from Chester’s coughing when he has been ill, you have spent far too many long and boring days sat in hospital rooms with limited access to the playrooms, making do with a hospital table and repetitive movies and activities.  You have been disappointed at family holidays and days out being cancelled due to hospital admissions and at times you have been pushed from pillar to post to allow me time to care for Chester when he has been extremely poorly.

You have coped with all life has thrown at you, you have your moments and your meltdowns but I understand that you need to let off steam, we all need to at some time or another and this is just your way.  Sometimes we are thrown into the most difficult of situations, and for someone so young you have an amazing ability to just take it in your stride.

I have shouted at you to be quiet when I’ve been trying to listen to the Doctors talking to me about Chester, I’ve told you off for trying to push buttons on his machines and I’ve yelled at you for not being careful around Chester always telling you to ‘watch his port’.  I’ve been cross with you when you have forgotten to cover your coughs or when you haven’t washed your hands and I’ve been frustrated with you when you have been impatient waiting for Chester’s treatments to be over.

For someone so small I have asked a lot of you, I have asked you to be patient and to be quiet, to be calm and to wait.  I have wished for you to grow up quicker than I should have, and I have expected you to be more mature than you should be.

Since becoming a big brother you have had to learn to share, not just toys and a seat next to me, but you have had to learn to share my time and I know at times it doesn’t fall in your favour or that at times I am distracted, but know I do my best to make sure that the time we do get to share is time you will remember.

I have always tried to shield you from the darker side of CF whether that be sending you on an ‘errand’ upstairs when I need to discuss something about it, desperately doing my best to change the subject to avoid answering your difficult questions or sometimes having to tell you little white lies to keep me from having to tell you the truth.

Witnessing the love, you have for your brother, in between the daily sibling fighting, fills my heart and I am full of pride.  I know that you will love and guide Chester and you will be there for him when things are hard for him.

What the future holds is unknown, but I know that one day your world too may be darkened by the reality of CF, you will probably have all kinds of feelings that you will struggle to deal with, just remember no matter what we have been through and no matter what we still must face, we will do it, together, holding hands.

Love you to the moon and beyond

Mummy xx 😘