Tonya Wilkinson and her family were recent subjects of a ABC’s “20/20″ piece. Tonya’s 14-year-old daughter Hannah was born with a rare condition called Prader-Willi syndrome, caused by a chromosomal flaw. Prader-Willi syndrome, which strikes only one in 15,000 people, can cause learning issues, muscles weakness and a slow metabolism. Below, Tonya reflects on a day in Hannah’s life and what it’s like to raise a child with Prader-Willi syndrome.
A typical day in a Prader-Willi Syndrome household is nothing like that of a family with “normal” teenagers.
Sure, most teens will say they have an insatiable appetite, but for Hannah, food is literally on her mind all the time and that genetically driven obsession is something we confront all day long.
Hannah usually gets out of bed at 6 a.m. (sometimes earlier) to wake me up to tell me she is starving and that she needs breakfast. If it’s too early, I encourage her to watch TV or play on her iPad, because I know if she eats breakfast too early that the day will be more stressful for her between meals. Even if Hannah can wait a little bit in the morning, the next 30 minutes or so will be a barrage of, “Mom, can I eat yet?” “Mom, I’m hungry!” and “Mom, is it time yet?” That will go on about every five minutes until I fix her breakfast.
The breakfast process starts with the unlocking of the pantry and fridge so I can prepare Hannah a low calorie breakfast. Typical breakfast options are turkey sausage and one egg with six ounces of milk, or maybe one cup of cereal, or a half cup of low fat yogurt with a small amount of fruit. We target 200-240 calories for breakfast. Breakfast prepared, the pantry and refrigerator are locked again.
Inevitably, 10 minutes or so after breakfast Hannah will say, “Mom, I am hungry – how long till I can have a snack?” I tell her it is only 7 a.m. and she doesn’t get a snack for three hours. I know it’s going to be a long morning and I know I will hear “How much longer till snack,” “What will I get for snack today,” and “What are we having for dinner?” frequently for the rest of the morning. All she thinks about is food – it consumes her life. Even on her iPad is an entire folder of food games. She always wants to know what’s for breakfast, for lunch, for dinner, when can she eat, and how much can she eat. It can be frustrating to answer her, but I try to remember how she must feel and how much stress and anxiety these food questions will cause her all day.
At snack time Hannah might get 16 low fat wheat thins or 10 grapes for her snack. This is never enough and she gets upset and will cry because she’s still hungry. I have to distract her and have her go play in her room and do crafts or she will look up animal rescue facilities in Arizona so she can see what dogs are available. (Hannah and “Siri” have a great relationship – at least until Siri doesn’t look up what Hannah wants. Whether it’s a speech misunderstanding or Hannah’s query is too complex for Siri, it gets her frustrated and she could have a meltdown or a “non-epileptic event” that can last up to hours at a time. And the questions never stop. I still hear “Mom, when is lunch,” “Mom, what’s for lunch,” “Mom, what’s for dinner,” and “Mom, what’s for dinner tomorrow?”
For lunch, I try to keep her lunch very low calorie. If it’s a school day I pack her lunch for her – she’s not allowed to eat at school. I have to unlock the pantry and refrigerator and relock it all back after her lunch is made. Lunch is typically a half turkey sandwich on low fat, low calorie bread with a little mustard, a 10-calorie jello, 100-calorie bag of chips or crackers, and a 5-calorie juice.
After lunch, the questions about her next opportunity to eat will start again, and the questions won’t stop until dinner is on the table. I try keeping her busy during the afternoon as it helps keep her mind off the food a little. Hannah likes to swim if we are able to, play with her baby dolls, do crafts or make her Chihuahua go through some “therapy” (the same kids of instruction she’ll do in her OT or PT sessions typically). The busier we can keep her, the better as I might only get questions every 20 minutes instead of every 10 minutes.
If we need to go to the grocery store this afternoon, I have to watch Hannah closely as she’s apt to steal food off the shelf and take it into the bathroom to eat it – she needs to be watched 24/7.
Dinner is similar to her other meals: healthy and low calorie. And we all eat the same as Hannah, maybe some grilled chicken breast, salad, a vegetable and a bottle of water. Even though she has had half or less of what a normal 14-year-old would eat she has probably has had around 1,000 calories today which means she will probably not lose any weight. After dinner is exercise: We might go for a one mile bike ride, a one mile walk or we might try and do some aerobics in the house if it’s too hot outside. We try to get her to keep moving for at least 30 minutes each day.
Now the day is almost over. I have to help her after her shower with personal care, because of her weight she has a lot of skin break down and I need to make sure it gets dried and has medicine on. I need to help her dry off and brush her hair. And the questions are still coming: “What’s for breakfast tomorrow,” “I am still hungry, can I have a popsicle,” “do we have stuff ready for lunch for tomorrow,” “What are we doing tomorrow,” and “What time are we going to the store?”
Hannah will get a little bit of TV, iPad or game time before bed. Just before bed, I tell her and show her everything is locked up so she doesn’t have stress and anxiety all night that she can get to any food. Usually though, I hear her yell from her room asking again about what will be in her meals tomorrow. I am exhausted and need to get some sleep because it all starts again at 6 a.m..
This is a weekend for us. During the week we have to also integrate work, school and therapies into our schedule.
Prader-Willi syndrome is a very hard and complex syndrome to have and live with. It’s very hard on those who have it and the family members who care for them. Hannah is 14-years-old and, but has the understanding of an 8-year-old.
She is going into the ninth grade because of her age, but will be doing second grade level school work. At 340 pounds, she’s not able to do the things she would like to do, such as baseball or cheerleading.
Our children don’t want to have Prader-Willi syndrome and it doesn’t make them who they are. Hannah is my whole world and has taught me so much in these past 14 years. She has taught me to love deeper, forgive easier, and that tomorrow is another day. It can be frustrating at times, but she will inevitably come up or say something really funny and makes me smile. She’s my baby, she’s an amazing girl, and I just want to do whatever I can to help her. I love this kid more than anything.