9 Families Live with Williams Boren

Julian Dufresne-Lammy met nine families with Williams-Buren Syndrome to write Blessed, an original and bright book about these unique, almost ungodly children that does not lose sight of the hardships of everyday life.

Thomas barely speaks but reproduces twenty-five animal sounds. Enzo never sleeps, and wakes up to thirty times a night. Axel rarely goes out without noise protection. There are also Mary, Arthur, Marius, Roman, Svetlana and Malina. unique objects. some creatures “The genies”. subordinate “Sprites”. subordinate “congrats”. This is the title of the eighth novel by Julian Dufresne Lamy (editions color). A poignant and radiant literary description of Williams-Burain Syndrome, a rare genetic disease affecting nearly 3,000 French people and manifested in particular by psychomotor and intellectual retardation, heart defects, or even physical and behavioral characteristics such as hypersociality. Julian Dufresne-Lammy lifts the veil on this pathology “spiritual” He reveals behind the scenes this immersive two-year literary experiment, the longest of his career.

Handicap.fr: Blessed be, why this address? In your opinion, living with a rare and debilitating illness is certainly not incompatible with happiness?
: I collected a whole bunch of words I imagined appearing in the title: Sleepless babies, Kindergarten… But the main feature of these young people is their constant happiness. However, I didn’t want to refer to the idea of ​​”blessed” a little too simply, which is why I started the book with this definition: “A radioactive being pollutes the other with its joy.” And that’s exactly what these kids do. I could have come up with a story, a journey and made it a model (note: a model) of joy, a symbol, but I preferred telling their life story, a model, a good lesson about what we should learn from disability and difference.

H.fr: In short, what are the characteristics of this neurodevelopmental disease?
This syndrome was first described in 1961 by Dr. Williams, a New Zealand cardiologist, and then in 1964 by Dr. Buren, a German cardiologist. The first genetic tests were done in 1993. Of course, symptoms vary from child to child. But this syndrome often begins with premature birth with a relatively light weight and poor silhouette. Most are born with heart disease, which sometimes requires open-heart surgery, hernia, or hypercalcemia (high levels of calcium in the body) leading to vomiting and hyperactivity episodes. These children are often diagnosed by the fact that they do not sleep and eat very little. They cannot stand the presence of hard tissue in their mouths, many eat compote only because the rest disgust them. For them, a pasta dish is equivalent to a dish of insects. It can also be a real way to sleep on the cross, like 5-year-old Enzo; His mother had to sleep in a wheelchair next to him. They also have intellectual disabilities and sometimes absolute tone associated with hyperacusis. Some hear a train arriving at the station 5 kilometers before, while Thomas, Arthur and Enzo are able to recognize the car’s sign by the sound of its engine. But this excessive sensitivity to sound remains a burden because the slightest splash of noise makes them panic, almost imploding. Clapping and fireworks, for example, are deafening and force them to wear noise-canceling headphones.

H.fr: This is your first book on disability. why did you choose this topic? Have you been criticized for an idealistic vision, especially in the choice of title?
: During the 2022 literary season, out of 500 books, only two spoke about disability. The idea is not to idealize the world of disability, and I am not ignoring the difficulties faced by families and particularly the administrative strictness. It’s all not cute and cute, there’s a certain heft but it feels a little bit uplifting because these kids need each other and that we ease contact with them. The first time I was told I was told: “You’ll see, these are love bubbles.”. They all have something rare, but to find out, you have to meet them.

H.: What is the purpose of this book?
: I don’t think the book has any purpose… My first project was to deal with the intimacy of people with disabilities, but this can quickly become “confusing” if you’re not completely thorough. I often write about “sensitive” topics, which are not very present in the literature (AIDS, prostitution, polygamy…). I’ve always wanted to write about disability but I’m often told it’s not a topic “Fashion”. I often felt an a priori and this fear of a subject that was very painful or miserable. It took two years of sprouting before I made up my mind. Thus, the goal, if there is one, is to show these children as they are, without imagining them. Therefore, I had to be as close as possible to families.

H.fr: says: “In every rare disease there is a whisper in the novel, a trembling story.” Why this particular?
: Actually, I made sure to generalize my point, it might be a book for any child, any syndrome. But this one is full of mystery and sensitivity. These people’s illusion of the world ceases in their teens. Small, very beautiful, solar, attractive, often stops on the street. But with age, features of the syndrome begin to appear and the appearance of others changes.

H.fr: How did the adventure begin?
JDL : You called me for the first time
Ann Laurie Thomas, president of the Autour des Williams Association, to lead a business conference, and she told me about her son, Marius. Then she asked me to write a Christmas story to raise money for the research. This mom inspired me. I established an association at the age of 28, and was stunned by the fact that no one talks about this disease or, like doctors, in primitive and archaic terms: “Your child has this syndrome but no one has to know.” What also intrigued me was writing about these caregivers and paying tribute to those mothers who often hold the vault of heaven a little, sacrificing their careers, their money, their social lives…

H.V.: Imagination, a semi-magical world, seems to be very present in this novel. Is it a way to escape this tolerant and inadequately conditioned little reality?
: That’s what we remember from the back cover but I don’t make it a high point in this story. I mention it because this fiction surrounds the syndrome in a rather vague way, but only the chapter “Like a Tale” dwells in it.

H.fr: You are comparing these kids to “Elves beings”. What are similar?
: Relationship to nature, to others, to music, to do it yourself … This is not an invention on my part, they inspired, according to the scientific literature, the legend of elves and elves. They have physical similarities in particular: very small, with a pixie face, starry iris, large mouths with widely spaced teeth…Moreover, it’s amazing because almost all of them have the same face. They seem to be siblings.

H.fr: Immersed in nine families, what has surprised you the most?
: I’ve been amazed by every family, every confidence, and insistence, especially the role of mothers who have been kind enough to make room for me in their ministerial agenda, as Gayle, Axel’s mother, 11 years old. I was only able to meet her when she was waiting for her daughter during her medical appointments. Consulting a dozen practitioners (psychiatrist, dentist, psychiatrist…) per week, everything is settled like clockwork. There is no room for the unexpected. For these parents, time management is a real hurdle. Stolen time, meant for a disability, will never come back… Then a semi-philosophical question arises: When do you accept your child’s disability? Some took ten years.

H.Far: What is the importance of associations to support these families?
: Associations such as the Autour des Williams are a crutch, a second family, where members hear from each other. This association organizes a big weekend every year that allows you to create a bond, unload, breathe and find some form of solidarity.

H.F.R: Another book on disability in the works?
: This is not planned at the moment. Writing a book means taking a journey, to discover a new country, a new continent. Today, I want to take a different ride. My next novel will tell two true stories of identities on the fringes in South Korea: K-pop schools (a music style very popular in this country) and a woman who has been locked in her apartment for fifteen years. I will be traveling to the “Land of Morning Calm” in the spring of 2023. In the meantime, I’ve been learning the language for two years. 곧 뵙겠 (See you soon, in French)!

“All rights of reproduction and representation reserved. © Handicap.fr. This article was written by Cassandri Rogeret, Handicap.fr journalist”

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