almost four years ago. My intention was to raise awareness of Cystic Fibrosis, because it is so often misunderstood, I even had a GP once ask me; so when did he ‘get’ CF?! Also, to raise awareness of organ donation; one in three seriously ill people die waiting for transplants, and this is entirely avoidable, and that just feels nonsensical to me. My final reason was to update friends and family on Isaac’s health, so everyone could come here to see how he was, without us having to repeat the same story many many times; that is not to say that we don’t like people asking how he is, we do, but for more detail, and more regular updates when he is unwell, this is a great tool for sharing our news.
Anyway, I jumped right into blogging, and it’s been fun. I love to write, share, I’m a bit of an open book as my friends will know, and it has helped us to raise more funds for the CF Trust (we have raised over £55,000 since Isaac was diagnosed) and I hope we have urged people to sign up to organ donation, as well as tell their loved ones of their wishes (that conversation is vital, as signed up or not, it is their choice after you die), more about that
here. But diving into blogging, I never really introduced us, and shared our CF story..... so here goes (some facts are a little hazy, smudged with age, worry, and perhaps a little gin, but I’ll do my best):
Isaac was born on a cold and snowy day, with perfect pink and healthy lungs. We had hoped for a home birth, but after three days of labour, I wanted, like, ALL THE DRUGS, and so we had a hospital birth, and although it was long, everything was fine. We couldn’t believe how perfect he was. Despite vowing that I would have him sleep in a cot next to us, and never in our bed, on that first night in hospital, it felt so wrong. I pulled him into bed with me, and promised that I would love him forever and would always keep him safe. Less than 24 hours later we were home, tired, but excited about our new life. Family visited, and he was happy and content, and for that first day, I think we kinda thought, hey, how hard can this be? All you need is love, right? And we had that by the bucket full.... But that night he cried, and cried, and cried. All night. A screaming cry, with hints of pain and exhaustion. We had no idea what was wrong, and although we felt like something must be amiss, we convinced ourselves that we were being neurotic young new parents. We had no idea what normal was. We were just pretend parents, nothing felt real. We called the midwives as soon as dawn came, and Mary Poppins arrived by 9am. Just before she did, Isaac had his first dirty nappy. We’d been told that the first would be black, like tar, but his meconium (first poop) came out like a golf ball and rolled out of his nappy. No wonder the screaming. Mary Poppins reassured us, and by now he seemed content again.
For the next few days we had our only experience of a ‘*normal’ family life that we would ever know.
But then he started to lose weight. A lot of weight. I was breastfeeding, and soon the midwives and health visitors were coming daily to weigh him. He was having a dozen or more dirty nappies a day, so I was clearly producing enough milk, and he fed for hours, typically for an hour, every other hour. We were exhausted, so much so I found it hard to string together a sentence (that is no exaggeration, I clearly remember not being able to remember simple words like ‘much’ or ‘meal’). I could never have got through it without Dan. We playfully called Isaac chicken legs, as the skin started to hang off his little limbs. We were worried, but there was talk of food intolerances, a tummy bug perhaps? We sent off stool samples for tests and assumed that everything would be OK. On day 5 he had the heel prick test as all babies do. On day 10 this was repeated. They told us that he probably had a false positive test, and that this sometimes happened, and that they just needed to repeat the test to be sure... I asked ‘Repeat the test for what?’ And they replied ‘Cystic Fibrosis, but you don’t have any family history do you? So it’s very unlikely’.
And I just knew. It felt like our darling, sweet, innocent baby had a life sentence. The words, WHY HIM, AND NOT ME??? ran on repeat through my mind.
And they were wrong anyway, the vast majority of families in our position are the same, CF seems to strike out of the blue much more often than it does run in families knowingly; CF is genetic, both parents need to be carriers of the mutation, and in the U.K.1 in 25 of the population are carriers, when two carriers have a child, that child will have a 1 in 4 chance of being affected, and so generations of fortunate carriers may never have a child with CF, and many others may never have realised; how many of us have a great grandparent or relative that died many years ago of ‘pneumonia’ (CF related?) as a baby?
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Anyway, I felt like the floor had just disappeared beneath me. I dreamt of black holes, and felt like I was grieving for the life Isaac would never have. We spent the next 11 days waiting for confirmation, where I fed, slept, and researched CF on a loop. The waiting was the worst, the more I read about CF, the more certain it seemed; his nappies were green, and often oily, as if he was not digesting the milk at all, and he continued to lose weight. But we also clung desperately to hope, and this just made it worse. On Easter Sunday I was home alone with Isaac when the health visitor called to say they wouldn’t be coming to weigh Isaac that day, as they were coming round on Tuesday with a CF specialist, I screamed down the phone; was that how the diagnosis was confirmed, over the fucking phone? (I probably didn’t swear really, I am way to polite in real life, and I’m adding it here for dramatic affect). She tried to back-track, as if a specialist nurse would be coming if the results were negative?! Worse, I knew now, but we still had to wait to see a doctor and start treatment, while he continued to lose more weight. My reaction must have caused a stir, as an hour later, the CF specialist, Joan, arrived on our doorstep. We asked a million questions. She answered them honestly, without giving us false hope, nor no hope at all. Compared to what I had read online, the prognosis seemed less bleak. By the Tuesday he was on treatment; daily vitamins (which his body was unable to absorb from his diet), antibiotics, to protect his lungs from infection, and most notably Creon; replacement enzymes which enable him to digest fats and proteins, which his body cannot, due to a blocked duct from his pancreas to his guts. He remains on these to this day (he takes around 30-50 capsules of these a day, depending on the fat and protein content of what he is eating) and most likely, always will. He gained a pound in the first week, and started to sleep for more than one hour at a time. He was no longer constantly hungry and in pain from malabsorption. The hospital were great, but gave us no written information (nowadays parents get a pack of information from the CF Trust) and so the CF Trusts parents forum online became my family, fraught with sadness at times, but also the only people who really understood.
From then on, Isaac went from strength to strength. We went to CF clinic every single week for many months, to learn how to do chest percussion for his physio to keep his lungs clear, and for the many tests he would have, and will always need. All went well, until he was 8 weeks old, when he caught bronchiolitis, which was just bad luck. He became very ill very quickly, and was still at this stage very underweight. He were admitted immediately, and it was here that he had his first two week course of IV antibiotics, and given oxygen to help his breathing. My absolute favourite memory of Dan (husband) is texting him in the night, asking for him to bring something in for me when he was to come the next day, and adding at the end how little sleep I’d had. Half an hour later, seeing a tall, bandy legged guy walking through the door at 4am made my heart weep with gratitude. Sleep deprivation is one of the oldest forms of torture.
Little Isaac:
But he got over bronchiolitis, and he got over it again at 8 months old, although we were in hospital then for a lot longer. He had his first broncoscopy during that admission, where they look at his lungs with a camera, and flush them out with saline at the same time to collect mucous for the lab. Only he was quite poorly going in to this, and he gave them all a scare when they tried to wake him from anaesthesia. Being told that afterwards will forever haunt me.
Isaac continued to have problems catching up with his weight for a few years, he tracked on the 0.8th centile on the charts, and we had to supplement his milk with sticky maxijul powder that we’d mix up into an icky paste and squirt into his mouth after feeds, pure glucose! Later he had prescribed milkshakes, and we would add cream, butter and sugar to everything he ate. Age two he was still small in height too, and I asked about his predicted height, as if he stayed on the centile he was on in the charts his adult height would be very small. His Dad is 6ft5, so it seemed likely that his lack of height was a result of his CF and his being so underweight in those early years. We were told he probably would always be very small, but he is now above average and needs to avoid extra calories rather than add them. Take that CF!
Over the next few years he was only admitted about once a year. He even had a couple of years without any IV antibiotics, his best stretch yet. Things started to feel more *normal, whatever that is. We relaxed a little, and just enjoyed him. He was a happy, active little soul, with dark eyes and blond curls. He had regular cough swabs taken, and these showed that his beautiful pink lungs were beginning to be infected with bugs, and so by the age of 18 months, he was taking daily nebulised antibiotics, which in those days was a big noisy machine, and Thomas the Tank Engine on full volume was our saviour. There are typical ‘CF bugs’ which love the sticky mucous of a CF lung, and Isaac mostly grew Klebsiela for his first few years, but by the age of 3 (I think) this was driven out by a big CF baddie, Pseudomonas Auriginosa, which still inhabit his lungs today. Bastards.
Otherwise, these years were mostly taken up with extensive house renovations. We bought a small Victorian cottage with a large garden, and not a single wall, floor or ceiling was left unturned. We knocked low ceilings up into the attic space, extended (twice) and lovingly restored all the character that we could. Dan is a builder, the only way we could have ever afforded all of this. I filled the house with art, books and music, and the kids filled it with shite, sorry, I mean toys.
Dan (and no, he did not catch that tuna, only in his dreams):
The other major thing that preoccupied these years was choosing to have more children. We always thought we would have 3 or 4, but now we faced the chance that our next child would have CF too. For us, this was not an option. No one would ever choose to have this disease, and to knowingly pass it on, now we knew we were carriers? That didn’t feel like our choice to make; it was about that child that we would be bringing into the world, not us, and our desire for another baby. Besides, having a sibling with CF could negatively potentially affect Isaac’s health as people with CF are encouraged never to meet, as the bugs that love their lungs are easily transmissible between CF patients. Naturally siblings can’t avoid this risk, so would likely share bugs throughout their lives. This is a hugely emotive subject, and one debated on CF forums regularly, and I do not sit in judgement. Many families choose to risk those odds, or conceive and then test, but for us, IVF became a possibility and we chose that route.
The hardest thing about the IVF was the waiting to have it; Although we were not having IVF for fertility issues, I can identify with that desperate longing for a child, and that feeling of it being out of your hands. For us, the year or so waiting was mostly because we needed to secure funding. As well as IVF, which meant stimulating my egg production, taking the eggs when ready, and injecting the sperm into the egg to make an embryo (ICSI) when the embryo was five days old and around 8 cells big (a blastocyst) one of those cells was removed and tested to identify the CF mutation, to ascertain whether that embryo was affected by CF. This is called pre-implantation genetic diagnosis (PGD), and a single cycle costs around £7000 (probably more now). We were told we had about a 1 in 5 chance of success.
Things didn’t go quite to plan, I overreacted to the IVF drugs and developed ovarian hyper-stimulation syndrome (OHSS) which left me looking 6 months pregnant overnight, in hospital for 10 days, and needing to inject myself daily with blood thinning drugs for 13 months..... but it was worth every second. Anouk was THE most beautiful baby, with dark hair and the sweetest temperament, and Isaac adored her. After the blur that was Isaac’s first few months, Anouk’s was peaceful and happy (full post about our PGD story can be found here.).
Baby Anouk:
We had our challenges; at one point we had no kitchen wall, a shell of an extension built, a chicken wondering into the house, baby Anouk, and community nurses coming round whilst Isaac was on home IVs. At times chaotic, but always happy. Three years later, knowing we had three more healthy embryos in a freezer at Guys hospital in London, we thought we would have a final go at IVF, this time self funded, but without the expensive PGD part as the tests were already complete, we had our little frosty implanted, knowing we had just a one in four chance of success, and feeling like we’d pretty much already won that lottery.
Our feisty, funny Rosa was born four years after both Anouk and her were conceived, our twins born at different times. We said ha! Our family is complete, but a few years later that yearning came again, but this time we added Obie to our family, the worlds most handsome and loving dog.
We have amazing science and the NHS to thank for our family. What words could cover that?
Baby Rosa:
I’m not sure quite when, but probably around the age of 8 Isaac started to suffer with his digestive system more often. He was diagnosed with episodes of Distal Intestinal Obstructive Syndrome (DIOS) which saw him admitted to hospital many times. For maybe 2 or 3 years, he had pretty much continuous problems with nausea, vomiting and DIOS, and developed a deep hatred of the many medicines he needed to take to prevent this; Ironic when the medicine you need to help your digestive system is the same that also makes you sick. It wasn’t always awful, but came in waves, but he started to miss more school as a result, often not being able to make it in first thing as he’d been sick. He was referred to a gastroenterologist, and had many tests, including eating radioactive egg on toast and being popped through the CT scanner, and dozens of X-rays. Thankfully during this time, chest wise, he was stable. But seeing him eat and then throw up most days, I think we started to resign ourselves to this being his norm, after so long. Until one day, it just wasn’t. He does still have problems with nausea, and takes anti-sickness drugs for this most days, and CF digestion is never normal (Creon dosing is an art!) but he hasn’t been admitted with DIOS for over a year now, which is fantastic. I hope this is welcome news to other families affected by regular DIOS, things can get better!
Frustratingly, as things were looking better tummy wise, he started to have more exacerbations (flare up of the bugs living in his lungs, causing more symptoms), and in the last few years, he has had many more admission for IV antibiotics to treat these chest infections, ultimately leading to him being very unwell last year, spending time in intensive care, and him finally accepting that he needed a partacath fitted as his venous access was so pants. Anyway, you can read all about within this blog.
So that’s us really, in a happy, shiny nutshell. I am probably forgetting about a million things inbetween. The biggest being that we have amazing friends and family who help, love and keep us sane. And also that CF can be very much in the background of our lives when it’s behaving. We laugh, we dance, we have fun. And even when it is not so great, and we are stuck in hospital, we are mostly there to keep him well, so it’s worth it.
Everyone with CF deserves the chance of a longer, healthier life. Please support our skydiving fundraising by clicking here. x
