My latest blog is a slight departure from my usual mumblings. The 5th-13th May is Cleft Lip and Palate Association awareness week. CLAPA, as it is known, is a charity close to our hearts and so I thought what better way to use my blog than to share our experience with my readers and hopefully beyond(!) to raise awareness.
One in seven hundred.
Bubbins first day…
He’d arrived…..I had always suspected I was carrying a boy and was spot on! One rather arduous labour later and our precious boy had arrived. His arrival was not all plain sailing. An Apgar score of three even after seven very long minutes – I cannot begin to describe the relief upon finally hearing a noise utter from his tiny body. The thought itself brings me to tears!…Having narrowly escaped the need for NICU we began to relax. He was just perfect. We shared the joyous news with family and then just cuddled and stared at his little face for hours, a tiny replica of his older brother….
As time passed, I was unable to get my head around Bubbins eager desire to feed, yet seeming disinterest and frustration at each attempt. I put it down to being a tired newborn and simply continued to offer whilst we cuddled. It was evident he was becoming distressed but I couldn’t clearly recall what ‘normal’ was, having been high as a kite when our first was born. Hours later when we were joined by a midwife, coming to complete the newborn ‘checks’, a special new chapter of our life began….
…Something was wrong but the midwife couldn’t say what as she truthfully didn’t know at that point. I won’t say what I thought the midwife had said, just imagine the most misconstrued words and you’d be about right. I heard everything incorrectly and entered a fog. To this day, despite their inaccuracy, the words still echo. I’d just had an incredibly hard labour, fairly scary birth and now I cradled my baby, tears falling on his tiny head, wondering what on earth was wrong with him and how this could be when he looked so perfect.
Skip a few hours and some very confused physicians. At 13 hours old Bubbins entered NICU afterall, due to hypoglycaemia and suspected stridor (a breathing problem). He had not been able to consume any milk, not mine, not formula, not at the breast, by spoon, or syringe. By this point our tiny baby simply didn’t wake, he just lay there jittering from lack of sugar. The lead NICU nurse barely took one look at him before immediately whizzing him away. It had been so worrying seeing his decline during the course of the day despite everyones best efforts and we felt such relief knowing something was finally going to be done to help him.
Finally in the safe, amazing hands of NICU and on our sons second day of life we were enlightened with a diagnosis. We were visited by one of a group of specialist cleft nurses, our saviour (no coincidence the team became our life crutch from that point on), who confirmed for us, what was actually ‘special’ about Bubbins. This was the point at which my husband became a mini scientist, something very far removed from his normal self, whilst I, the actual scientist, remained in a foggy emotional cloud. As ever the solid rock that he is!
Our son had been born with a cleft palate, a very wide, isolated, cleft of his hard and soft palate. Not sure what I mean?!….Then pop your tongue to the roof of your mouth and imagine nothing exists other than a hollow straight up through to your nose. In Bubbins case the cleft was linked to PRS, Pierre Robin Sequence (Robin pronounced Roe-ban), a condition where the chin is much smaller and set further back than normal (known as micronagthia) in turn pushing the tongue backwards. Both the cleft palate and the PRS contribute to feeding difficulties due to an inability to create suction and can result in breathing difficulties. A cleft palate requires surgical intervention to close the cleft, or in our sons case, create a palate! His surgeon said his cleft fell into the definition of ‘no’ palate as opposed to a ‘cleft’ (gap) in the palate. Longer term, most children require speech therapy in order to learn how to use the palate correctly (to make sounds, letters and words) and some require further surgeries to improve palate function. Hearing problems can also occur as a direct result of the cleft, requiring correction, to support development of speech and engagement with the world around them.
His lack of suction meant that Bubbins couldn’t feed at the breast. He required a nasogastric feeding tube. As, I would guess, do all PRS babies initially, with the hope and aim that they can later feed by specialist bottles. We were incredibly lucky…Bubbins was fed by NG tube for the first four days of his life and then by some greedy miracle, the second time he inadvertently pulled his tube out the nurses didn’t replace it and we found him one morning being fed from the special bottle (essentially milk is squeezed into the mouth). Most mothers get to cherish the first feed(s) with their baby but I reflect on this time differently. I was terrified to do any of the NG feeds, daddy was our hero and learnt the ropes. Then when it came to the bottle feeds, I made the cleft nurse do the first feed as I was even more terrified that I would somehow choke Bubbins by squeezing the milk too fast (which I subsequently found is possible(!) but not as frightening as I’d imagined and you do eventually find your childs own rhythm). I’ll certainly never lose these memories, they are clearly etched and precious in their own way.
We came to learn that Bubbins really was a little miracle in the cleft world. Many babies affected by PRS stay in hospital for weeks before coming home and even then they remain on NG tubes for months on end, unable to take enough milk orally to sustain growth. Many need breathing support too sadly, due to anatomically restricted airflow. We were so unbelievably fortunate on both fronts – other than extreme reflux, he fed well and his oxygen saturation was always good, so simple adjustments such as side sleeping (tricky but achievable) prevented any risks of suffocation. We will never take for granted how lucky Bubbins, and we as a family, have been.
The diagnosis had come as a complete shock. A cleft palate is rarely detected at scans, even in cases associated with a cleft lip (visable on scan) where they know what to look out for. In the case of a PRS cleft I suppose the only potential flag might be the smaller jaw/chin in those with a family history. So despite me noticing and saying to the sonographer “He has no chin, just like his brother at his scans!” it never occurred to anyone to even consider PRS or a cleft palate as we have no family history of either.
The shock, along with the hormones, pain and overall emotion of having given birth seemed to make me shut down. I didn’t feel able to bring myself to speak to anyone other than my husband. I bizarrely shut out our families for days, the people who would have given me the most comfort and support. It was incredibly hard for them, not least because my side of the family all live hundreds of miles away and couldn’t visit immediately, so just wanted to hear my voice and know we were all ok. They’d had limited information fed back by hubby when he could find a moment to call between looking after the three of us. I know it was a very worrying time for them. I cannot really explain what made me shut myself off, perhaps self preservation….I know my mum instinctively understood and I felt immense relief to have her by my side for support when the fog lifted.
Every parent will question ‘how and why?’ ‘What is PRS and cleft palate and why does it occur?’…. There is not really a simple answer. I hadn’t had any alcohol for about six months prior to conception, I didn’t even have a sip during pregnancy (despite unhelpful people saying I was overcautious). I’ve never done drugs. I didn’t eat foods on the ‘banned’ list. I had nine months worth of folic acid prior to conception and throughout. I didn’t have any illness or virus during pregnancy. I did take a drug used to help maintain and protect the pregnancy from the effects of polycystic ovarian syndrome but have had multiple reassurances that this would not have caused the cleft. We had genetic testing which took over a year to complete and again, no obvious cause. We’ve accepted that Bubbins cleft was just a random occurrence. I carry no guilt whatsoever but what can be upsetting is stumbling across ignorance in the world, where people with no scientific substance whatsoever speculate and lay blame on certain causal associations, when in reality, more often than not, it is just ‘one of those things’.
The early days and his first year….
His first year was by no means a breeze. I express fed for roughly five weeks before concluding that my breast milk fuelled his worsening reflux and the combination of expressing, feeds taking upwards of 45 minutes and reflux meant there was barely time to have a wee, let alone consider the impact this lack of attention was having on his big brother. Bubba quite frankly saw his brother as an obstacle between him and his mummy, which I’ve eluded to in other blog posts. There was also the prospect of Bubbins first operation, that ended up being done at 11 months old. I eventually required support from the cleft psychologist, another fantastic support service provided by the team – I’d managed to convince myself that something dreadful was going to happen during the surgery and suffered panic attacks and insomnia as a result. I fondly recall the kindness shown by the psychologist who came to see us as I cradled a very brave baby post-op. Offering a shoulder for support, she said “see, he came back from surgery, you made it through it!”
An intense period of recovery followed the operation to build Bubbins palate. This was incredibly challenging for us as parents and required a lot of support from a combination of family, the cleft nurse team, the CLAPA charity and the online cleft community. It was harder than we’d imagined and unbelievably disheartening to watch the repair and stitches come apart before our very eyes, meaning that at least one subsequent operation would be necessary. We made it to the light at the end of the post operative tunnel though, just in time for Bubbins to enjoy his first birthday in style, his first post operative solid food, four weeks on, was his birthday cake. The look of pleasure on his face is a memory I will cherish.
Of everything that surrounded the PRS diagnosis, an associated syndrome, known as Sticklers, was another of my main worries, besides the operation. Sticklers is a disorder that can affect the eyes, ears and joints and occurs in 50-80% of all PRS affected children. The syndrome can lead to loss of sight. We were warned about this the day after Bubbins was born and I remember thinking the palate is easy, that can be fixed but sight cannot and I shed many a tear about this, it felt like the toss of a coin. It was particularly hard knowing that tests could not be done until after the age of one. We just had to sit tight and ‘wait and see’. Yet again though we were blessed, Bubbins proved to be our little trooper and after a few months of tests he was discharged from the ophthalmic department with the all clear, we will be eternally thankful for this.
Beyond the operations and tests…..
I made a conscious vow to myself after Bubbins first birthday that I would stop with all the worrying and simply enjoy! I will always feel like I tainted his first year with stress and worry and didn’t enjoy it as much as I should have. I now compartmentalise everything to cleft review milestones, happy in the knowledge that the prospect of the next operation doesn’t even need to cross my mind until he is at least three years old. For now, we focus on the every day task of learning to speak – requiring a fair bit of patience, sign language to help fill the gaps and regular diagnostic speech therapy. Bubbins will get there eventually, we’ll hear the word ‘daddy’ as opposed to ‘a-ee’ and along the way the specialist team will support him (and us) with his future cleft needs.
There will never be a day goes by where we don’t thank our lucky stars for our healthy children – touch wood! We feel so incredibly fortunate that our son only has a cleft palate (again, touch wood). If there is one thing we’ve learnt as we’ve grown as parents, it is that there are so many worse things that can and do affect babies and children and you don’t have to look far within family and friendship circles to see those who are living with such heartbreak and worry. We never ever fail to feel immense emotion on our regular trips to the children’s hospital, where we are humbled by the strength of other families, yet deeply saddened for what their children have to endure, despite them smiling through it all.
Writing this blog has been a bit of a ‘process’ for me. I’ve always written Bubbins chronicles since his birth to help manage my emotions and give him some understanding of his early days as he grows older but these notes have always been for my eyes only. This ‘blog’ is a bit different – baring personal feelings and experiences and sharing them with the big wide world to raise awareness, whilst trying my best to be mindful that there are far worse things than a cleft! I just hope I’ve hit the right note…
People often say to me “Oh, you’d never know he had a cleft!” – if I had money for every time I heard this I’d be fairly wealthy! I probably would have said something similar myself many moons ago and from my perspective it demonstrates the broader lack of awareness of isolated cleft palate as a condition and a great reason for this blog.
I do wish I had known more about what is included in the newborn checks, perhaps even stumbled across something in the mummy magazines that I read religiously during pregnancy. Had I known more then maybe Bubbins cleft would have been less of a surprise. So in writing this blog I hope it goes some way to sharing a small element of what we now know and in reading this blog you are enlightened somewhat to our, very individual, experience of having our darling 1 in 700! One of the 1200 babies born extra special in 2015!