Seeing a smile spread across a toddler’s face is a comfort to most parents.
But for Kira Hnidey, it’s a reminder of the agony that her daughter Madeline is in every single day.
The three-year-old, from Texas, suffers from an incurable rare disease that causes her to appear constantly happy. But despite sounding pleasant, Angelman Syndrome – which affects just one in 20,000 people – affects the nervous system and causes severe physical and learning difficulties. Worst of all, the rare genetic condition – once dubbed happy puppet syndrome – can mask what’s really going on in her head.
Shock diagnosis
Madeline was diagnosed after suffering a seizure at just eight-months-old.
Kira, 38, said: “I was in shock when she was first diagnosed and I just wasn’t prepared. The first thing I wanted to know was whether she was going to die but luckily it doesn’t really affect life span but they can be more susceptible to pneumonia and have seizure accidents.”
She gave up her job in the air force to care for Madeline, who needs to be looked after 24/7.
“One of the hardest things is the lack of sleep. She usually sleeps for two to three hours per night and we can’t live like that.”
Daily struggles
Madeline also struggles to communicate, is tube fed and suffers from one seizure every minute where she loses control of her muscles.
The mum-of-three said: “Maddie can say ‘mum’ so far. The seizures are difficult to control because the quantity is dependant on the day but they have been extra bad lately.
“It’s loss of muscle and her eyes roll back for a few seconds but we’re up to one a minute now. She’s taking several medications so we can try to control them. They are difficult to quantify at the minute but they are very bad, we have times where they are barely noticeable and some days that are just bad.”
Always smiling
A symptom of the syndrome is frequent laughter, smiling and being easily excitable meaning that despite the tot’s suffering, she constantly has a smile on her face.
Kira said: “She’s always smiling, the only time she cries is when she’s in pain but even then it alternates between crying and smiles – it makes it more bearable. She has this innocence that kids usually grow out of. She’s so happy and loving and wants to pull you close.
“It’s part of the symptoms that she’s happy and has an excitable demeanour. I don’t know what goes on in her head but the way her expressions are say that she’s happy. When we’re in hospital and she’s having needles and bloods taken, the nurses can’t get enough of her because she’s so smiley and happy and isn’t that what every one wants to be?”
Sibling bond
Madeline, who lives with her mum and sisters, Emberlynn, 12, and Avalon, 10, goes to physical therapy, occupational therapy and speech therapy and Kira said she’s getting stronger by the day.
Kira said: “Her sisters absolutely love her, they think she’s special. It must be hard because she needs a lot of care and attention from me. Maddie absolutely loves them and she gets so excited when she sees them.
“I never thought I would have a child like this. It’s very difficult but when you love your child you’d do anything for them. I hope people accept her for who she is as she gets older.”
