Being Mummy and Nurse to a child with a CF.

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. 

Being a CF parent is hard. 

Because most of the time our children “look well”, on face value they look absolutely no different to any other child, it makes it all too easy for people to jump to the assumption that CF isn’t overly serious and easy to manage.  But in order for our children to look and be well, there is a huge amount both physically and emotionally that goes on behind the scenes.

Managing treatments and medications can be time consuming and exhausting, adjusting to becoming your child’s carer as well as their parent is something parents of children with chronic conditions have to get used to fairly early on post diagnosis, but it doesn’t mean it is by any means easy.

When entering parenthood I fully accepted the responsibilities that I thought would be expected of me as a parent,  what I didn’t consider was that I may find myself responsible for carrying out treatments and administering medicines that not only keep my child well in the present, but also have a huge impact on his future and life expectancy.


It’s not just the physical side of caring for a child with CF or any chronic condition for that matter that is hard work, there’s the emotional side as well.  Living with CF can be likened a little to riding a rollercoaster with the ups and downs, everything can be going so well, but in a very short space of time things can go from good to bad at a surprisingly quick pace.  That feeling of things swirling completely out of control is incredibly hard to cope with.

So many mixed emotions all the time, from being made up that your child has done a new physio treatment without issue to being completely gutted when a cough starts. The relief and exhilaration of a time consuming treatment ending and finding there is extra time to just be a parent and then the anger, dread and disappointment when another lengthy treatment is added to the routine. 


CF affects so many aspects of our lives and at times it can feel like it’s taking over even when we try our best not to let it.  Everything takes organisation and time to plan. Even something simple like a night out can take hours of planning just to make sure treatments have been done time.

I can honestly say if we didn’t have CF in our house there would be a lot more time to just have fun. I would be a little less nervous, CF has made me nervous about the silliest of things, things I completely took for granted before Chester came along.  Despite this nervousness there is the need to carry on and enable Chester to just be Chester. 

Without CF I would probably be a little less stressed and snappy.  With so much to think about at times my temper is short, on top of life in general there’s medicine and prescription collections, appointments for CF clinic, flu jabs, sleep studies, Physio training, port flushes, and not forgetting surgical appointments. 

As well as affecting my child, CF affects us as parents in so many different ways and living with CF is hard, but we are not after sympathy, we don’t need or want that, sometimes we might just need a hug, we need to know someone gets us and we need to know that we have people out there that understand some of our erratic behaviours are because of what we are dealing with behind closed doors.
 

 

When stepping out of my CF comfort zone results in an amazing adventure 🦇

Our recent holiday saw us head off on a spooky Halloween half term, Dracula themed city break to Cluj-Napoca, Transylvania. A strange destination some might say, lets face it Romania probably isn’t at the top of everyone’s must see destinations list, but as always it’s about doing something different and making it an adventure for the boys. It’s also important to me that the boys have a realistic view of the world, not everywhere looks like the Bahamas or Maldives and not everyone in the world lives life the way we do.

As with any trip the boys go on, especially as far as Chester is concerned the preparation starts a long time before, so by the time we arrived in Romania my research had been done. I looked at the local hospital and specialist centres “Just Incase” and read reports that they probably aren’t the best or cleanest hospitals around, but having identified there was a CF community I was hopeful if anything went wrong, someone, somewhere would know something about CF and in the absolute worst case, we were only there on a short break and could easily get a flight home quickly if needed.

It wasn’t until we arrived with his bag full of medical supplies and expensive equipment and I saw just how poor some areas looked that it really sunk in just how lucky we really are, and just how lucky Chester is to have been born in a country such as ours. 

As we were being transferred from the airport to collect our hire car the driver mentioned a visit to a “Salt mine” is a must while in Cluj. I must admit I didn’t think about it again, a Salt mine just didn’t seem interesting or appeal to me in any way and as soon as I hear mine or cave, I instantly think pseudomonas and that’s enough to put me off.

It wasn’t until we had been around the city looking at the beautiful buildings and monuments in the old town, but also looking on in sheer horror at the state of the concrete tower blocks that make up the majority of the City that we wondered what else we could do in event of bad weather. 

We had already decided that Corvinus Castle was a must for any boys on a Vampire hunt. A trip we were not disappointed by, especially Oakley who was amazed at finding some “blood” (Heinz tomato soup looking substance) on the stairs which only added to his fascination of vampires and bats and not forgetting the ‘Witches birds’(crows).


We knew rain was forecast so we decided we would think about the salt mine, I was anxious at the fact it was below ground, damp and dark and worried that it wasn’t a planned activity that I had done research on, if I’m honest I was out of my comfort zone as far as CF goes.  

With Chesters track record of growing Pseudomonas it seemed like the worst place we could take him. I emailed his consultant who said the trip would be ok, and then I started to look further into Pseudomonas and CF in general in Romania. I was horrified to read that 50 per cent of children diagnosed with CF don’t survive to make it into adulthood, it angered me that in this day and age with precision medicines and treatments that half the CF population in this country would never become adults. It made me genuinely grateful that we have such a good health service and that Chester has the ability to do what half their CF population doesn’t.

I was also unnerved by the fact there is a high Pseudomonas rate amongst their CF population. This set me off questioning myself about whether or not the Salt mine would be a good idea. I hated myself for questioning the Doctors, I was angry that CF was having such a huge impact on our plans and I was sad at what I had read, I couldn’t stop thinking about how lucky Chester really is and I couldn’t help but worry about this decision.  

The majority of decisions we face as CF parents impacts on our children’s health in later life, it makes me feel sick most of the time, but with most decisions I make, I have to weigh up the risk versus his quality of life and not forgetting Oakley and his need to experience different things. 

The more research I did into the Salt mine at Turda the more I read about it’s positive effects of those suffering with respiratory illness such as Asthma, COPD and CF, there is even a clinic where treatments can be booked. This was great news but then it set me off thinking about cross infection and the high Pseudomonas rate, I always seem to overthink, but mainly when Pseudomonas is the risk. 

After thinking about it non-stop for almost a day, and weighing up the pros and cons we decided that not only would it be an amazing experience, after all there was a Ferris wheel at the bottom of this mine as well as rowing boats, but we knew it was probably the only time we would visit this area.  


I know if he’s caught Pseudomonas again I’ll blame myself and feel like utter crap, but looking further into it, he’s more likely to have caught it from another child with CF than from the mine because the mine most certainly was too cold for a bug like Pseudomonas. 

I managed to keep Chester in a rowing boat without the need to shout man overboard and it was a fantastic experience that they both loved, that was until they were outside rubbing their eyes with salty hands. 

Feeling guilty for needing a break.

As I sit here in the sweltering heat of the Caribbean (not boasting……Well ok, maybe just a little) having just finished my book ‘A son for tomorrow’ by Alice Peterson based around the life and death of Alice Martineau, a singer who lost her battle with CF, a book that has taken me some time to pluck up the courage to read, I would be lying if I said I’m not thinking about Chester and his Cystic Fibrosis. 


Wondering where his story will lead him, will he have a wife? children? Will he lead a life that allows him the freedom to travel as we have done? What will his chosen career path be? Will he ever see a life without CF?

I wonder if he will resent being called brave or inspirational as Alice did? If his CF will cause him to be as driven and determined as Alice was. I already see so much fight in him I find it hard to imagine that he wouldn’t have her determination to succeed.  

Life for anyone is uncertain, we never truly know what is in store for each of us but for Chester, we know only too well what could potentially be in store for him. 

I feel guilty sat here on holiday without my boys, even more so that I felt I needed a break from being “Mummy” and a break from CF, especially when I know full well that is something that just isn’t on the cards for Chester, he doesn’t get a break from it; so I do feel incredibly selfish for needing this time away. 

I felt odd packing my suitcase, a ritual I am more than used to doing, but this time I found it odd that it took little more than half an hour and I was fully packed and ready to go. No need to consider fridge packs, face masks, hand luggage, meds, Physio equipment and not forgetting snacks and toys to aid in occupying kids on the journey. 

I watch kids playing in the turquoise waters, seemingly carefree, being careful not to assume all is perfectly well and “normal” as many watching us may do; oblivious to the fact our child is always fighting on the inside. I find myself not jealous but perhaps envious watching small children help themselves to ice-cream from the all you can eat buffet not having to worry about taking a handful of pills with it. 


I wonder what life would have been like if CF hadn’t entered it. How easy would it be? One thing is for sure, I wouldn’t have met the lovely ladies I now call my best friends, and I can’t imagine not having them in my life. I probably wouldn’t appreciate the beauty of life like I do now. I would probably be far less appreciative of time, knowing now that a lot more can be forged into one day than one would think. Most importantly I don’t think I would ever appreciate something as simple as the ability to take a breath and fill my lungs with fresh air like I do now. 

I often wonder if Chester would be the same without CF, would he still be as cheeky and mischievous, would he be as strong-willed and most importantly would he still be as brave? All pointless things to be thinking about, but something I think about all the same.

Sitting here I am missing my boys, always worrying in case something happens and I am not there with them, I find myself trying to occupy my wandering mind, being so used to being constantly on the go makes it difficult to sit and actually do nothing, and I find myself already planning the next family adventure, if there’s one thing that I can be sure of and have control over, it’s being sure to make the most of life and live for the now. I don’t want any room for regrets and I certainly don’t want to ever say CF held Chester back. 

“But he looks so well”

“But he looks so well”, something every CF parent will have heard numerous times when telling people their child has CF. It’s one of the most frustrating things I hear, because children with this invisible condition don’t look any different, but as I have said before looks can be very deceiving.  Yesterday during Chester’s annual review clinic, for the first time I uttered very similar words “But he’s been so well”.

I have said it many times before that one of the hardest things about CF is not being able to see what is going on inside and not being able to see what damage that is being done.

So yesterday when I was told that despite sticking rigidly to Chester’s treatment plan with physio, meds and exercise, his recent bronchoscopy showed an extremely large amount of secretions in his lungs. I honestly felt like I had been kicked in the stomach, like they would be questioning whether or not I actually do his treatments and like everything we had been doing was not enough and that I should have been doing more. I know deep down I haven’t, but I honestly do feel like I have let Chester down in some way, I am the one trusted with his care and yet here we are with this news. 

When Chester had his first Bronchoscopy last year post pseudomonas eradication treatment, in true Chester style, he was further diagnosed with another condition called “tracheomalacia” which is more commonly known as a “floppy windpipe”, this is where the windpipe collapses, and in Chester’s case it is severe, collapsing by around 80% which is why his breathing can appear noisy, a little like a 40 a day smoker.  It is beilieved that this condition has most likely been the culprit in the pseudomonas growths. With his windpipe the way it is, despite chest percussion physio Chester is not physically able to clear the secretions which in turn allows bugs to settle and grow and for secretions to build up clogging is lungs. 

The team have come up with a plan to trial a clearway ventilator machine which will blow air into Chester’s airways to open them up and allow him to move the secretions easier. This machine is incredibly expensive so will be on loan to trial it first before purchasing to ensure it works for him. They have made the decision to keep him on nebulised antibiotics which I was a little gutted about purely for selfish reasons that it adds another hour or so into our already packed schedule. 

They have also noticed that when asleep, Chester’s oxygen saturation drops significantly and they believe this also to be caused by the floppy windpipe; this may be when Chester has been more susceptible to growing pseudomonas. He will be admitted to undergo a sleep analysis and dependant on the results he may need to be ventilated on a machine overnight to increase his oxygen levels.

In comparison to Chester’s first year where we spent so much time in hospital with various chest infections, this last year was remarkably better with no admissions because he was “unwell” only admissions to treat pseudomonas. In the past 12 months Chester has only required the use of back up antibiotics where we increase his prophylactic antibiotic and add another antibiotic to help beat infection once. This was an achievement for us and I took this to be a good sign, things were starting to get easier, less meds and equipment to take with us on our travels, and now I feel like we may actually never leave the house with all the added kit and meds we are now on.


I was left a little shell shocked by yesterday’s review and angry that CF is doing this to Chester, I hate that I feel completely out of control and that I am powerless to stop it. I should be able to help him, but I can’t, and it’s the most awful feeling.  I can’t help but think that he is too young to have so many complications and I have found myself thinking about what the future holds for him.  I found myself thinking the absolute worst, I started to think about all the younger CF children I have seen lose their battle just in the short space I have been a CF parent and it is terrifying.

Last night I sat watching both boys playing together on the floor with their train set, both oblivious to the news I had been given, I had word with myself about feeling so down and realised I had to put my heartbreak to one side partly because I would hate for the boys to see me so broken, but also because I am determined that if Chester can pick himself up, smile and get on with it despite everything he goes through, then I have to man up and carry on as well. 


Yesterday was a blow and it doesn’t matter how hard it gets, I have to stay positive, I need to show the boys nothing has changed and I need to carry on helping Chester fight the fight.

This boy has had so much thrown at him and CF is much becoming the gift that keeps on giving, it doesn’t matter to Chester though, to him this is normal life, he knows nothing different and no matter how difficult the circumstances he always manages to smile and more often than not blow a kiss. 

 
 

The waiting is the hardest.

Usually I would be jumping at the chance to sit and have a child free coffee, to be able to drink it while it’s warm without one of my children kicking off because they’ve had to sit still for longer than five minutes or knocking my drink over whilst playing with their toy cars and trains. I often long to be able to just sit and enjoy the peace and quiet whilst taking in the local scenery, or what most would call people watching. 

But yesterday as I sat in the coffee shop next to an empty high chair staring at my cappuccino, I have never wanted my boys to be with me so much. Feeling a little lost that there were no sticky hands or faces to wipe and feeling a little guilty that I wish so often I could have just 5 minutes to myself to enjoy my coffee. 

Today was the sixth time we’ve handed Chester over in an anaesthetic room to a bunch of complete strangers who we find ourselves trusting with one of our very most treasured possessions. It really doesn’t get any easier, the only thing that made yesterday slightly less traumatic for me was the fact Chester was already out cold from his pre- Med so there was no physical fight to get him to sleep. As far as Chester is concerned he fell asleep in one place and woke up in another. 


Knowing both the procedures would take some time, we made the decision to wait a couple of hours in the cafe, I find waiting in an empty room with a space where Chester and his bed should be too upsetting so we choose to spend our time waiting in the cafe or the parents room or walking the corridors, anything to avoid waiting in his room. 

It is only a few hours of waiting but it’s agony, I cannot imagine what it must be like for parents waiting for children who are having timely procedures. For Chester both procedures are fairly straightforward but when the Surgeon starts to mention collapsed lungs, chest drains and perforated lungs just before the surgery whilst asking for consent, the hours of waiting feel like a lifetime and all those dreaded “what ifs” start to float round my head.

We have always been allowed to go to recovery to see Chester and yesterday was no different, it’s always a worry as we never know how the anaesthetic will have affected him. As we walked towards the cubicles I could hear his breathing straight away, and I was instantly relieved that I could hear him, his breathing was rattling and noisy but I didn’t care, he was still here and still fighting, that’s all that mattered to me.

Chester being the head strong stubborn little chap he is, was trying his hardest to sit up, despite being drowsy still and when he heard us he tried so hard to get to us, that the Doctor told me to get in the bed with him to keep him calm. There I am fully clothed in bed being pushed down the hospital corridors, snuggling with my baby and it was the best feeling in the world and right then nothing else mattered. I could smell the anaesthetic on him and I could see that he had a dressing on his left shoulder, I just wasn’t brave enough to look any closer at that time.


Back in his room we tried physio to release what we could from his lungs, the bronchoscopy had kicked up the mucus in his lungs and his cough was resembling a 90 year old, 50 a day smoker. He managed to cough up a lot and clear his lungs and airways a little which made him more comfortable. Then the regular poking and prodding, Oxygen levels, temperature and respiratory rate, putting on new probes and generally stressing Chester out part started. 

All the time he was ratty and whinging all I could think about is the fact he made it and all went to plan and that was enough to allow me to listen to the whinging and whining noises he was making and be incredibly grateful.

This was a pretty tough decision for me as a parent, to put my child through something like this, to change him forever, to give him something that straight away makes him look “different”, but having just had one day of painless and stress free IVs I know I have made the right choice for Chester.

This little one has bounced back time after time, the resilience he shows amazes me, no matter what CF throws at him he stands up to show CF who’s boss, he’s by far the strongest person I know. I can’t help but think about the path ahead for this little guy, he’s already been through so much, I really hope CF gives him the break he deserves, but even if CF doesn’t I know Chester will kick arse and give CF a run for it’s money because he’s alr day showing he’s more than capable! 

The night before port surgery. 

No one ever said parenting would be easy, but I never imagined it would be quite this hard, all the angst and worry, the endless doubt and worst of all, the millions of decisions you are expected to make as a parent. Always wondering and hoping the choices we make are the right ones for our babies. 

I have made the decision to put my son through what will most likely be a pretty traumatic ordeal tomorrow, something that I know will be painful and upsetting for him and something which he is too young to understand at the moment and I am wracked with guilt over it. I keep asking myself over and over, is this the right decision? 

I know that he needs this surgery and once the port is in, longterm it will mean his admissions are far less traumatic with no need for cannula changes every day, but even knowing this and hearing others success stories hasn’t made the decision any easier and hasn’t taken that guilt away.  

Don’t get me wrong, I’ve been so grateful to the other CF parents that have shared their stories and shown me pictures of their children’s ports and I feel fortunate to have my virtual family around me providing advice and allowing me to ask questions and I thank them for that.

As I sit here this evening having packed our hospital bags ready for the morning I find myself looking over the list of 35 odd complications the Surgeon gave me, complications ranging from Port rejection all the way through to heart problems like tamponades and endocarditis, I wonder to myself if the risk outweighs the end result. You always hear surgeons say “With every surgery comes risk” but until you are given papers with them listed it doesn’t seem so real and I never realised there could be so many for one procedure. 


I’ve spent the majority of my evening staring at Chester’s topless torso, wondering how different it will look after tomorrow’s surgery, wondering where it will be placed and how prominent it will be. Then wondering how he will feel about adding another scar to his already patchwork body. I guess only time will tell and I am hopeful that one day he will be proud of his war wounds and I will have to tell him off for showing them off to the girls. 


For now I wait and hope that his bed is still available so I don’t have to spend another evening worrying and stressing about putting my baby through another surgery.