Finding the joy: how top stylist Paula Hughes and family came to terms with their daughter Kayla's Rett Syndrome
Photography by Kip Carroll
Paula Hughes and Emmett Harten's daughter Kayla was 13 months old, when they realised that something was amiss.
"She was a chubby little thing, loved her food, slept really well. Looking back, she was the perfect baby," reflects Paula, as we sit around the couple's kitchen table at their Wicklow home. Everything changed just after Kayla turned one, and she was failing to reach typical developmental milestones. With a lot of practice, Kayla might get the hang of a new skill, only to lose it again within a few weeks.
The public health nurse agreed that there was cause for concern. "That's when the alarm bells really got loud," says Emmett. Kayla's condition continued to worsen. She lost the use of her hands. "That was when we really knew we were in some sort of trouble," Emmett adds. "She would constantly ram her hands into her mouth and chew them. You go from, 'Oh she might have a slight learning difficulty' to, 'Well, maybe she has dyspraxia.' You try to come up with the least bad explanation."
Kayla's behaviour changed in other ways, too. Their daughter went from being a happy child, to a child who was clearly in huge distress at times. "She started having these horrible, horrible episodes," Emmett remembers. "It was like a snarling pit bull; an animal growl of distress. There was a wild look in her eye."
"It was pure discomfort. Pain," her mother recalls.
There would be nights when the small child would thrash and growl, beyond comfort or settling. "She would bite," says Emmett, describing how he and Paula had "necklaces" of bite marks on their necks, arms and hands.
Eventually a consultant in Temple Street diagnosed the problem - Rett syndrome. "That's when it really caves in on you. Because now you know what it is," says Emmett.
Rett syndrome is a rare genetic neurological and developmental disorder which affects the way the brain develops. It almost exclusively occurs in girls; in Ireland there are approximately 70 known cases. Symptoms include loss of movement and coordination, loss of communication abilities, breathing problems, agitation, periods of severe distress, cognitive disabilities, possible seizures, possible scoliosis, pain, difficulty chewing and swallowing.
"And there was nothing positive," Paula explains. "I mean, no matter what you read, there was nothing positive about the syndrome."
"You've got this huge range of outcomes, from awful to only not so awful," Emmett adds, wryly. "So far we've been very lucky that Kayla hasn't had chronic epilepsy, and she's not looking at acute scoliosis. That's the kind of thing we're left hoping for. Your whole sense of optimism is shifted. We're still quite optimistic people, but we have a different set of optimistic outcomes," smiles Emmet.
The impression of positivity, of a life enjoyed to the full despite its difficulties, that Paula and Emmett present is remarkable.
How do they stay optimistic? "I think the bottom line is that when you have a child like Kayla, it's not about you any more, it's about them," Paula explains. "Obviously, we have to take care of ourselves, to give Kayla a good life. But when you watch what she deals with every second of the day, we have no right to moan about stuff that we're going through. I can't even fathom how she copes. When I go there, and try and think about it, it's just... wow," she sighs in awe.
"She's the most amazing child," Paula continues. "That on its own will give you hope. When she was diagnosed, I remember thinking that what I want for her is to have a blast of a life. If it's short, it's short, but I want her to have the best she can possibly have. And that's all I can hope for. It's not about us. Your priority becomes your child and the crap that they deal with."
In the aftermath of the diagnosis, advice from other parents was the most helpful thing for the couple - in person with the Rett syndrome Association of Ireland, and online with the Rett Net.
Initially, reaching any sort of plateau from which they could formulate some sort of coping plan, or take stock, was impossible due to Kayla's sleep problems. "People would say, 'What's wrong with her?' and we'd go, 'Oh, it's this and that,' but to be really honest, we didn't know," Paula says of the fits of distress Kayla would suffer.
"Looking back on it now," she adds, "Kayla was exhausted because she couldn't fall asleep. And her bowels were causing her pain."
Kayla lacked the ability to self-soothe. Her parents tried melatonin capsules, but they would leave her in a daze the next day and eventually stopped working.
"I don't know how many years it went on," Emmett says now of the sleep deprivation. "I remember a statistic I would quote to anybody that would listen to me, that for any length of time, neither Kayla nor I would sleep for more than two hours at a stretch."
How did they cope? "We didn't really cope. It was like being a zombie. You're slowly falling apart," he recalls, as Paula nods her head in agreement.
"She sometimes went 36, 40 hours without sleep. I would take photographs and put them up on Facebook, just so someone could hear me scream in the forest," Emmett says, and they both laugh.
Eventually the public health carers intervened. Arriving at the house one day when Kayla was six, "they took a look at me and said, 'We're going to have to do something here,'" Emmett laughs at the memory. "They told me later that I was grey, and empty." Respite and in-home care were procured in the months that followed.
Their younger daughter, Jamie, now seven, was born three years after Kayla. "I think it was the best thing," Paula says of her arrival to the family, "because, you know, babies bring joy. And thankfully we had her, because there has to be joy and fun. And Jamie brings a lot of joy and fun for Kayla."
After much pushing, they were given the right medication to help Kayla go to sleep. "It was then we were able to start living. We could start tackling everything," Paula recalls. "That cloud hanging over the whole house lifted."
To this day, either Paula or Emmett must lie down with Kayla at bedtime. "If you don't, she'll have her hands in her mouth, will get soaked, develop a rash, and she'll be stressed and upset. You have to put your body weight on her, your limbs, so that she knows where she is," Paula explains. This can be a short process, or it can take hours. The night before we meet, Paula spent three-and-a-half hours with Kayla before she fell asleep.
Paula singing to her daughter can soothe her when she becomes agitated. "Even though I've such a terrible voice," Paula says, "when I sing to her, it's unbelievable how much she changes from a very distressed child to just so engaged with my voice, smiling and everything."
Paula is one of the most respected creative directors and stylists in Ireland, with a career that spans several decades. In her work as a creative fashion consultant, she has worked with clients from Brown Thomas to Marks & Spencer. She famously shot Cara Delevingne for A-Wear, shortly before the model became a worldwide star.
Paula and Emmett met 20 years ago, working on a fashion show. She was styling, he was the lighting designer. Now, alongside Paula's work, the couple run their business, Kayla's Kitchen. It's a frozen-food delivery service, providing healthy food, with a particular focus on food for people with health issues.
After Kayla's diagnosis, and in order to give their daughter the best possible care, the couple decided that Emmett would become a full-time carer, while Paula went back to work.
Part of the reason for that was that a serious crash in 2001, in a Winnebago returning from a job, had left Paula with chronic back problems. "The photographer and the PA died. I was working down the back. They left the long part of the sofa for me to lie on. They lay on the table, asleep both of them. I was just thrown on to the sofa. I always think it's because of their generosity and kindness I'm still here and they're not," Paula says.
Further issues were created when she suffered with SPD (Symphysis pubis dysfunction) during her pregnancy with Kayla, which compounded the damage from her injuries. It all meant that being Kayla's main carer was physically unfeasible for Paula much of the time. Her work as a producer, stylist and art director on editorial fashion shoots, advertising, and other creative projects, involves a huge amount of travel, in Ireland, and abroad.
"I didn't have a choice, but it was hard going, to travel to the extent I did, leaving them," Paula says. "I suppose you just get into it, and you focus. It's only now that I look back and think, 'How did I do that?'
Emmett working as a full-time carer also meant he was able to put in a crucial amount of focus on Kayla's communication skills.
A few months after the Rett diagnosis, Paula had managed, through contacts, to secure an appointment with the leading neurologist for the syndrome in New York. The doctors she met with had told her never to underestimate her daughter's ability, and will, to communicate. Even a cry was a form of communication.
"For somebody to have all of the experience and to then inform me, 'never underestimate, it's a locked-in syndrome, she's locked-in, inside', that stood to me," Paula explains now. "She has difficulty processing stuff, but she's still going on in there, and she's still a very bright child."
At home, Emmett put work into teaching his daughter how to read words and symbols. Her parents worked with their daughter on eye movement as a form of signing. Now, she is able to use her Tobii Eyegaze device, an infrared communication computer, which tracks the movements of Kayla's eyes, and which she is able to use in school.
"Today she told us, 'I want banana'," says Emmett. "All the learning was about food. She would be able to say, 'I want toast now. I want a drink, please'."
"Food is everything to her," Paula smiles. "She enjoys chewing. She has an incredible palate for such a young child. She doesn't enjoy processed food."
By the time Kayla was six, it transpired that her weight was at the very bottom of the scale for her body mass index. "She was nothing but skin and bone," Emmett recalls. "That's where I got serious about nutrition, when we realised that she was really not getting the nutrition from the food. Girls with Rett syndrome are prone to malnutrition."
Getting the right foods into Kayla was both key to promoting the best possible physical state for her, and a way of giving their daughter pleasure. Emmett had noticed early on that if he accentuated the flavours of a dish, he would get a particularly enthusiastic response from his daughter. "We'd start to get this 'nom nom nom nom'," he smiles. "And if she didn't like the food, you weren't going to get that."
Emmett now runs Kayla's Kitchen full-time. He creates healthy recipes aimed at people whose health is suffering and who may not be able to cook for themselves, but for whom good nutrition is of particular importance. It's also just delicious food, so it's also suitable for anyone short of time, for whatever reason, who wants a truly healthy frozen option. Kayla's Kitchen home delivers nationwide.
"To this day," Emmet says, "Kayla enjoying a meal is the seal of approval; we don't put make food for the site that she hasn't tasted and liked."
Originally, Kayla's Kitchen started as batch-cooking in their own Wicklow kitchen for their family of four, and then for Paula's brother Karl, and her parents. Karl had been diagnosed with motor neuron disease, and Paula's parents, in their 80s, found their mobility becoming challenged. So Emmett began cooking for everyone, buying six freezers he put in the back garden under a makeshift shelter.
"Karl started to take notice of when Emmett was doing a cook," Paula says with a smile, recalling how her brother, who has since passed away, would arrive at the house around meal time. And so Kayla's Kitchen was born, originally as a means for providing good, nourishing food for their family.
"The principles are banging flavours, and then as clean and healthy as I can make a thing," Emmett says.
I know your pain
"The idea is the best kind of home-cooked food, from our kitchen to yours, for people unable to provide that for themselves," Emmett explains. "They could be recovering from cancer or going through chemo. They could have motor neuron disease and be experiencing difficulty swallowing, maintaining their appetite, struggling to keep their weight on. We recently learnt that there are 140,000 people in this country who are suffering from malnutrition due to an illness. That's a massive number of people who need help."
It is a remarkable thing these people have done, to make something valuable, so helpful, from circumstances so hard. Emmett spends a significant amount of time talking to new customers on the phone, supporting, helping, advising. You have to find the joy, he tells me.
"The road to here means I can relate to all the situations," he says. "You're going, 'Jesus, I know your pain, I know your trouble'."
Kayla's Kitchen is food made from scratch, using the highest quality fresh ingredients, portions for adults and children, delivered nationwide.
Kayla's Kitchen has just launched its new Breakthrough range, a selection of high-protein, high-calorie meals, specifically intended for those dealing with cancer and its aftermath. These meals are based on recipes created by dieticians, researchers and oncologists working with Breakthrough Cancer Research. Tel: (01) 404-5210, or (087) 906-7766, or see kaylaskitchen.ie