PERCY
We first met Percy when he was 2 years old. Diagnosed at 7 weeks with Prader Willi Syndrome (PWS) the family has adapted to living life with Percy and are accommodating to his daily needs. He originally came along to the studio with his mum Susan and dad where they spoke about the worries for the future.
“Percy is a very sociable little boy who loves attention. His two brothers are so close to him and he loves to spend time playing with them. His bubbly personality and bright smiles really make sure he captures the hearts of anyone he meets.”
To be told that there is nothing we can do to stop it is the hardest part. Percy has Prader-Willi syndrome and when we were given his diagnosis, we were devastated but in some strange way a bit relieved as his diagnosis could have been so much worse. Prader Willi is a rare genetic condition that, amongst other issues, causes the person who has it to have a constant desire to eat. They are driven by a permanent and all-consuming feeling of hunger and can easily lead to dangerous weight gain. People with this syndrome also can have restricted growth and low muscle tone.”
Percy smiles into the camera at her photoshoot in 2016 - all photographs by Ceridwen Hughes
Today age 10 Percy is thriving with the love and support of his family. We spoke to mum Susan about how he is getting on today and Percy happily came along to the studio for a catch up. Like many 10-year-olds he is sports-mad and loves football, in particular Wrexham F.C. and Harry Kane, although he will watch any sports. At home having a lock on the kitchen is just a normal part of everyday life. It's no different to just locking the door as you leave home. “When we first put the lock on it took us getting used to it, but as time has gone on it gets locked all the time now. If it isn't locked, Percy does tell you and will remind us to make sure it's locked. There have been times in the night when Percy has got up to go to the kitchen for food. He doesn't feel happy about it and does feel upset. Having the kitchen locked he says makes him feel happy and less anxious.”
Currently, food control is working for Percy and with help from a dietician, he is at an ideal weight. “I don't monitor his calories as closely as I used to. At the moment he is on around 1000 Cals per day. We use a certain size plate and a certain size bowl every day so we can judge portion sizes more easily. We stick to the same foods for breakfast and lunch and then we eat similar meals in the evening just sort of alternating. We all eat the same. By sticking to the same breakfasts and lunches any changes in his weight I know whether it’s coming either evening meals or if he's been snacking, and it's just easier to control.”
During school, if something is happening such as a birthday where there will be cake, Mum sends in a treat such as a sugar-free jelly that he can enjoy. “I always have something in place. Just so that he's got something a little special. Sometimes I let him have the school treat, I don't want him to feel left out. The school inform me of the calories that are in it usually, and I can adapt his diet at home. He does notice if the other children have cake, but he is ok with it if he has a treat such as jelly as he doesn’t have them often. He also goes trick-or-treating on Halloween.”
“The sweets get put away, and he'll just get one every so often, a little treat. He is not completely removed from doing that.”
As he is growing up Percy does know he is different with food. “He's not started with being really hungry, I think that's probably going to come a bit further in the future. To be honest, we've been quite lucky that it hasn't sort of ramped up like that. He's definitely got more interest in food. So he’s got a keen eye on what you're doing in the kitchen or if I'm shopping. If he gets on YouTube, he loves watching videos about food.”
Percy looks into the camera during his 2024 photoshoot.
The family are very aware and considerate of Percy. It hasn’t changed since he was little. Nothing more has been needed to be put in place and food safety has become second nature to them in the home; his older brothers have adapted equally. “I mean, they've not got a problem with having food, there’s lots in the kitchen. We just make sure they don't leave food lying around, and that they don't take food to their room. It's not a big change that they've suddenly had to do because they've grown up with this.” From his diagnosis, at 7 weeks the family contacted the Prada-Willis Syndrome Association. It was invaluable having the support where questions could be answered. The family have raised funds for the charity and plans on doing more in the future. Alongside the charity, they found support from online groups such as Facebook forums and peer support groups which have been invaluable.
Holidays are still tricky at times and the family still do not use food as rewards or gifts such as Easter. There have been difficulties in the past where he has been gifted an Easter egg from a kind intentioned person and Susan has had to explain his condition. This is where awareness is so important. “When I do speak to people, they can be quite dismissive and say ‘Oh, yeah my son's always hungry.’ So, it is difficult to explain to people that it's not just hunger, they don't understand the effects it has. Especially with Percy looking so healthy.”
Percy attends numerous hospital appointments every few months. “He goes in to have his checkups about every four months. He gets to see the endocrinologist and we see the dietitian. We keep in contact with them and have regular phone calls just to make sure he's keeping on track. Scoliosis is very common in people with Prader-Willi, around 70% of people get it to some degree so he's checked regularly along with bone age and density as he could develop weaker bones due to lacking vitamins as his diet is restricted. His bloods are taken every 4 months to check his thyroid, cholesterol levels and to check for vitamin deficiency.”
“Currently, he has a growth hormone injection that helps with the muscles, which also means he can eat slightly more as well. He will be on these until he is 18 but they are looking at having people on it for longer. At the moment there are some trials ongoing for certain drugs to help with Prader-Willi but we will have to wait and see what that brings in the future.”
The future is still a worry for Susan and the family. “I still don't think he’d be able to live independently. He's going to have to have things in place like supported living. It makes me sad as he's very capable in all areas, it’s just that one thing of not having control of the hunger in the future that could hold him back. I still worry about what's to come. This hunger thing can start at different ages, so, it's waiting for it to happen. We have no idea how strong that feeling will be, it's always a worry.”
One thing is for sure, Percy inspires his family and the love they have for him is abundantly apparent. “My oldest son did sports at college. He is a sporting coach and personal trainer. That's all because of wanting to help Percy and keep him fit and healthy. That's what he chose to do afterwards. Percy loves to join him when he is doing exercise.”
“As a family, we live in hope that one day they find a way to control appetite and this constant hunger can be alleviated. We hope that by sharing our story through the Rare Project more people will be aware of conditions like Prader Willi and that they will not judge someone because they look or act differently.”
In this short video, Percy’s mother describes her experience of his rare disease.
FIND OUT MORE
PRADER WILLI SYNDROME (PWS)
Prader-Willi syndrome (PWS) is a rare, complex genetic disorder that affects both males and females from birth and throughout their lives. It causes low muscle tone with consequent motor developmental delays, a mild to moderate learning difficulty, incomplete sexual development, and emotional and social immaturity, which can lead to challenging behaviours. During childhood, an overwhelming and insatiable chronic appetite usually develops which, without rigorous food management and exercise regimes, leads to food seeking, stealing and life threatening obesity. PWS occurs randomly in about 1:22,000 births and it is estimated that there are about 2,000 living with PWS in the UK.
To find out more about this condition, click here.
PRADER WILLI SYNDROME ASSOCIATION UK
PWSA UK works with those living with PWS, their families and friends together with those supporting or working with people with PWS, to help them manage this complex syndrome. The services they provide include a telephone, email or Facebook hotline, an information hub on their website and a wide network of peer support groups.
To find out more about PWSA UK and how the can support you and your family, click here.
Source: https://www.pwsa.co.uk/services
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