When PAULA HUGHES and her husband Emmett found out that their two-year daughter had a RARE NEUROLOGICAL SYNDROME, their world was turned upside down …
Seven years ago, creative director and stylist, Paula Hughes’ lifestyle was a whirlwind of fashion shoots and long-distance travel, then she and her husband received the terrible news that their two-year daughter had a rare neurological syndrome. Here she describes the impact on their lives and how they were inspired to start a new business to help others in times of need …
Life was busy but fun. My work took me from desert islands in the Bahamas, sand dunes in Morocco and wildlife parks in South Africa, to the glamour and bustle of Miami, Paris and London. I loved the creative challenge of communicating my clients’ stories in an engaging and powerful way. And I loved being home in our oasis of calm and joy back in Bray. Kayla was such an easy baby, who slept well, babbled and smiled all day. From an early age she loved her food and would insist on feeding herself. She mastered crawling up and down steps early. Such an independent little madam. Your typical toddler, she was into everything, always getting up to mischief. She loved her pink pram, which she marched around the house. She loved her books and demanded to be read to, morning, noon and night. She’d hold the book and turn the pages, insisting we start back at the beginning as soon as we finished.
But a few months after she turned one, we began to notice she wasn’t progressing. Her gross motor skills were slowing down. Her grandad bought her a brightly coloured shape sorter and we sat on the ground with her, showing her where to put the little square, circle, triangle. But she couldn’t get it, no matter how often we played with it together. In fact, she seemed to be having problems even picking up the shapes. More and more often she began to stuff both hands in her mouth. She didn’t hold her books anymore. Despite the signs, we tried to convince ourselves that it wasn’t anything serious, thinking that we just needed to unlock something and all would be fine again. Gradually we noticed that she stopped managing the step out to the garden. She couldn’t crawl up and down the stairs anymore. Her giddy shouts of “Mama” and “Dada” began to disappear. Worryingly, she lost her sparkle, her infectious giggle, her mischievous smile, replaced with frustration, discomfort and pain. Now her nights, and ours, were broken by anguished growls of distress. We didn’t know what was happening to our beautiful little girl. Without a diagnosis we didn’t know what we were dealing with. It was sheer torture watching her in such pain – we didn’t know how to fix it.
We voiced our growing concerns with the doctors and the local public health nurse who confirmed our worries. Something was amiss. They put us in touch with the HSE Early Services who assessed Kayla, confirming she needed to see a paediatrician. We were given an appointment for six months’ time. Every bit of research said “catch them early.” Yet we had to wait six months just to sit in front of a paediatrician! They were six months of torture. After the appointment we were none the wiser, but hoped the tests would provide an answer. We naively still thought it would be something we could fix.
Then a month before her second birthday, our world came crashing down. The paediatrician informed us that Kayla had Rett Syndrome; a rare and debilitating neurodevelopmental disorder that affects only girls and robs them of their ability to use their hands, to talk. About 50 per cent lose their ability to walk. Incurable. Regressive. Cruel.
We walked out of her office shell-shocked. Numb.
All our hopes and dreams for Kayla, our plans for our and her future in smithereens. We were looking at a syndrome that would rob our little girl of the most basic abilities. She would need one-on-one care, for the rest of her life. She’d never be able to talk to us, communicate with us, or so we thought.
Immediately we went online and delved deeper into the world of Rett Syndrome, hoping to find some good news, some sliver of hope, but were stunned and in disbelief when we read about the horrific progression of the disorder. Rett often brings with it a wide range of serious medical complications including issues with chewing, swallowing, seizures, scoliosis, hand wringing, severe reflux, abnormal breathing, as well as gastrointestinal disorders. The list goes on. The motherlode of all syndromes. And these girls can live up to 40 years or more with these horrendous issues. So cruel.
At first we just got through the days in a daze. We worked on autopilot, going to work, minding Kayla, trying to make her as comfortable as possible but the terrible cries of pain and sleeplessness continued. There seemed to be nothing we could do. We now know she was going through developmental regression along with having severe sensory problems and horrendous pain from constipation.
My work took me out of the country, sometimes up to three weeks at a time, leaving the bulk of the caring to Emmett, my husband. Jamie, our second daughter, had arrived in 2011, and sleep deprivation was wreaking havoc on our already fraught lives. We felt there was no light at the end of the tunnel. Life and the future looked bleak. Emmett was exhausted almost to breaking point, trying to work and mind Kayla at the same time. Something had to give. I have chronic back pain from a car crash and found it impossible to physically handle Kayla while pregnant. So after Jamie was born, we decided that Emmett would give up his work for six months in order to mind Kayla full-time and give her the attention needed. But even with that it was such a struggle. At every corner we hit roadblocks. We had to fight for every service, battle for anything that would make Kayla’s life more bearable. And ours.
I must admit, those years are a blur. After three gruelling years of trying homeopathic and natural remedies, we finally gave in and succumbed to medicating Kayla to fall asleep. Clonadine was like a magic bullet. Kayla became a different child, now that she was sleeping more regularly, and we were able to deal with all the other issues one by one. She became more alert, smiling her gorgeous smile again. She simply felt better, and hence, so did we. She still had a lot of challenges but she was happy, happier than we had seen for a long time. We started to understand her needs and realised that she needed to be fed often and a lot.
She went to a local special school and loved the company and the stimulation. This gave Emmett some time to get on top of things. Emmett’s six months at home became six years and Kayla thrived with the care.
Kayla always LOVED her food. She often has hunger pangs but we are very strict about her feeding times. On the dot of six she will bash the table demanding dinner. At 4pm she will start agitating for her banana. Food was the one way we knew how to make her happy. Sensitive to different flavours and textures, she always seemed to know what good food was and what was fake or processed. So Emmett started cooking up a storm in the kitchen. He picked only the best ingredients; local, free-range and fresh, making sure she always had a well-balanced diet. He really began to enjoy it and cooked delicious, nutritionally-rich meals for Kayla and the family.
The same month as Kayla was diagnosed with Rett, my brother was diagnosed with motor neurone disease. Emmett started to cook for my brother too, to ease the burden on him and my family. One less thing to worry about, with a freezer full of fantastic-tasting food. He’d batch-cook the dinners, perfecting the process of making, freezing and storing them, without any loss of flavour or nutrition. He experimented with containers, with ingredients, with home-made sauces, until he was finally happy with what he was producing. Kayla loved them and seemed to thrive. My brother loved them, keeping him independent and at home, as was his wish. Everything tasted wonderful. Soon my elderly parents took to dropping in when the big-batch cooking was happening. And going home with a week’s supply! The list of people availing of Emmett’s food service began to grow. And so Kayla’s Kitchen was born.
Emmett made numerous appointments with nutritionists and dieticians at The National Children’s Hospital in Tallaght, to learn the nutritional and functional benefits of a whole variety of foods. And he checked with Kayla every step of the way. A dish is only made if it gets a thumbs-up from Kayla. She has a very sophisticated palate. Suddenly our kitchen seemed to be shrinking, as more and more freezers filled every corner. So last January, Kayla’s Kitchen got a new space; a proper commercial kitchen that would comply with the food safety rules, and grow the business.
Our two girls get along so well, though it is hard for Jamie, now six, to understand why her sister can’t talk or do the things other nine–year-old girls do. But she is patient and kind with her and Kayla adores the attention. You can hear her giggle as Jamie whirls around the room singing and dancing.
One thing we noticed with Kayla as she began to blossom on her new nutritionally-rich diet was that she was more alert, more inquisitive. Eventually we taught her to sign with her eyes. Look to the right for yes, to the left for no. Emmett chanced asking her one day “Can you read?” She smirked a “yes.” Can you spell? No. Her smile dropped. It was like a lightbulb moment. No one had ever asked. So Emmett devised a test and videoed it. He held up a card which said broccoli, then a picture of broccoli on the left, a picture of a flamingo on the right. Eyes straight to the left. We were stunned. Time after time, Emmett tested her word recognition and she got it right every single time. Our hopes and suspicions were confirmed. All this time and she had been taking in everything around her.
Armed with this new information we decided to push for her to get an eye gaze device. Trying to convince the school of her potential and ability was again another struggle. The video helped. Kayla was delighted when we raised the €15k for a Tobii eye gaze computer. Once she started to learn how to use it she could make herself understood. She could reach out and communicate, learn like any other child. She blew us away with her appetite for learning. When we turn her computer on, the first thing she wants to go to is her virtual kitchen. Like her dad, she loves being in the kitchen, cooking up some tasty treat. It’s incredible how technology can unlock the world for our little girl. But it’s tiring and requires a huge amount of effort on her behalf. And naturally, like any nine-year-old she doesn’t want to be “working” all the time. She loves to watch television with her sister Jamie, listen to music or just hang out in the garden looking at the light and the shadows, the wind rippling across the grass.
Our aim and motivation is to keep Kayla out of State care. With this in mind we set up a trust fund to provide for her, if something happens to us. A percentage of Kayla’s Kitchen will go to the trust, to ensure her future care.
There was a time when we saw no hope, the future bleak. But building this business for Kayla and with Kayla is so exciting. It means so much to be doing something worthwhile. We eat Kayla’s Kitchen every day in our house (except for Platform Pizza night in Bray!). We know what it feels like to be desperate, at the end of our tether, hardly able to put one foot in front of the other, let alone put a dinner on the table. We want to help other people who are in this position. We want to lessen the load. We want to make a difference.
And we see it helping in a variety of situations, not just for the likes of Kayla. It’s for young mums, the elderly, the infirm, anyone under time pressure, anyone who can’t get out to the shops, can’t stand too long at a cooker. We can deliver a week or a month of dinners that will last for up to a year in the freezer with no loss of taste or nutrition. What peace of mind that would bring.
Kayla’s Kitchen has given Emmett a sense of purpose and drive and enabled me to develop as a stylist and a mum. We cannot tell what the future holds for Kayla, or for us. It’s the big unknown. But at least we know she’ll be well fed. And for now she is happy and that means everything to us.
As told to Justine Carbery.
For more information go to: www.kaylaskitchen.ie, www.rettsyndrome.org. Meet Kayla and her family at a charity book fair in aid of Rett; 47 Frascati Park, Blackrock, Co Dublin on September 16, between 11am and 2pm.
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