A Project Celebrating Differences and Triumphs
Henley is Beautifully Different
Meet sweet Henley! Henley is an incredible two year old girl with a rare genetic disorder, Prader-Willi syndrome. Prader-Willi Syndrome (PWS) is a complex genetic disorder caused by a loss of genetic material on the paternal copy of chromosome 15. PWS affects approximately 1 in 15,000 people and occurs randomly, affecting males and females and people of all races and ethnicities equally. Nearly every system in the body is affected by PWS: hormones, body composition, muscle tone, behavior, temperature regulation, cognition and learning, pain tolerance, sleep patterns, but the hallmark symptom is hyperphagia, or extreme, constant hunger. .
Every aspect of Henley’s life if affected by PWS. Her low muscle tone means she has to work twice as hard as her peers to complete the same activities. Her growth is also affected. People with PWS are deficient in growth hormone. She has to have a Human Growth Hormone shot every night. Individuals who have PWS also have trouble controlling their emotions. Henley seems to have extreme emotions no matter the situation (happy, sad, loving, etc.).
The biggest challenge is her diet. Although she has not yet reached the extreme hunger phase, individuals with PWS have a slower metabolism as well, only 60% of a typical persons metabolism. This means we must follow a strict diet. Henley does not get any candy, treats, juice, anything with added sugar, refined grain. She is on a diet that is low in carbohydrates and high in fat. She eats a lot of whole foods, nothing processed, no sugar or sugar substitutes, only complex carbs. Henley also has to adhere to a strict schedule. Eventually, when she exhibits extreme hunger, her parents will not be able to allow her snacks or meals outside of designated times.
Henley is a warrior. She is inspiring. She is determined.
Henley has never known any other life than the one she’s been dealt. Her demeanor, her persistence and her drive is uncanny. She lives with so much intention. For all of the hurdles in her path, she has overcome one after another. She works extremely hard at her goals. She loves her life and loves hard. She’s happy, smart, and defying all expectations. Although the road ahead of her may be difficult bumpy, her family is confident that Henley will manage all that she encounters with grace and grit.
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