Posted by The Children's Institute - Mar 28, 2017
It’s snowing outside The Children’s Institute of Pittsburgh but, a few hundred miles away on the East Coast, where Amy McTighe, PhD, is beaming a group
of school teachers a PowerPoint training deck on Prader-Willi syndrome (PWS), the temperature is 30 degrees warmer.
McTighe, though, isn’t concerned with the clouds. She has a job on her mind: getting teachers for a teenager with PWS, who was an inpatient at The Children’s Institute’s Center for Prader-Willi Syndrome, up to speed on treating the debilitating disorder and understanding how it dramatically impacts the young girl’s life.
“The hypothalamus, which PWS affects, is the area of the brain where you, as a typical, normal-functioning person feel full when you eat,” said McTighe, who directs the PWS program here in Pittsburgh. “The thing that you need to keep in mind with PWS is that it’s a life-threatening condition. People with PWS, among other things, can participate in what’s called a food binge and eat themselves to death, to the point where their stomachs rupture.”
Three times this week, McTighe will sit down in a fourth-floor conference room lined with photos of children with special needs and lead distance- or tele-training for interested parties in PWS. It’s part of her mission; training modules also are available for purchase in The Children’s Institute’s Knowledge Center. The brunt of the institute’s work these days with PWS, however, revolves around therapy and treatment. The CARF-accredited facility, established in 1902, cares for more than 300 inpatients and averages 70,000 outpatient visits per year, and its Day School serves nearly 200 students from 67 school districts.
PWS, the disorder of the body’s 15th chromosome, in addition to food-related behaviors, brings with it cognitive rigidity, oppositional or defiant behavior, anxiety and insecurity, and picking of the skin and body.
Gone are the tactics of preparing patients with PWS for independent living; their lives are lived supervised and, when it comes to food, under lock and key.
“This is a very different syndrome than ones you’re accustomed to in the school setting,” McTighe told the teachers, as a tele-training camera panned the room. “The ultimate goal for kids with PWS is maximum functioning, not independent functioning. We are not aware of any person with PWS who’s living independently in the community. They’re always going to need food security, they’re always going to need food supervision.”
At The Children’s Institute, she told the teachers, that means a special low-calorie diet we dub Red Yellow Green. The “green” is food that is low in calories, not unlimited but okay for seconds. The “yellow” food is high in calories, rich in nutrients and consists of the basic foods for a well-balanced diet. These foods are eaten in specified amounts.
The “red” food is high in calories and limited in nutrients and very limited in the daily meal plan. The red foods are items that are offered maybe once a month or on a special occasion or holiday.
Patients with PWS also have a poor sense of temperature and difficulty retaining information they read and learn. McTighe and her staff of therapists, clinicians and support personnel are ready for the challenge.
Life expectancy for PWS used to linger in the 20s, but times have changed, and The Children’s Institute of Pittsburgh has treated patients aged 5 to 65. And all patients receive one-to-one care.
But training is just one part of a larger picture.
“Training about PWS is essential for professionals and staff working in group homes, schools and any location where persons with PWS are being served. It is wonderful that we offer this quality training and support,” McTighe said. “The state of the art equipment that we have here at The Children’s Institute allows us to educate more people across the country and the world, rather than just locally. It makes a difference for so many involved in the care of a person with PWS. Most importantly, it makes a difference for that child or adult with PWS.”
The Children’s Institute recently expanded our care and service delivery through extensive telepresence infrastructure by hosting the PWS conferences, remote Individualized Education Program (IEP) conferences with school districts, and parent support groups. Lead donors contributed nearly $500,000 for our telepresence program.
To learn more and view training modules through The Children’s Institute’s Knowledge Center, visit http://amazingkidslearning.org/ today! Read More...
Posted by The Children's Institute - Feb 19, 2016
For people with PWS, just thinking about food causes high levels of anxiety. And because of their insatiability, they tend to think about food all the time. Insecurity about food causes overeating and medical problems resulting from obesity and poor nutrition. It can also lead to unwanted behaviors such as skin picking, obsessive/compulsive actions and emotional outbursts.
Achieving food security helps to address many of the challenges of PWS. By developing a nutrition plan that assures where
the next meal is coming from - and what foods will be served - both children and adults with PWS experience less anxiety, enabling them to fully engage in and enjoy other life activities.
Food security helps to:
• Prevent over eating and inhibit weight gain
• Improve nutrition
• Promote a healthier lifestyle
• Decrease anxiety and stress
• Decrease unwanted behaviors
A high level of commitment and dedication from families, other caregivers, support personnel and connected loved ones is vital to achieve food security, but it's not an impossible task. The multidisciplinary team at The Center for Prader-Willi Syndrome at The Children's Institute is ready to help every step along the way.
1. Prepare Menus in Advance - Start planning a day or two at a time, with a goal of creating a weekly menu. Include your loved one with PWS in a few preliminary decisions about meal choices and times. Once the plan is set, STICK TO IT. Avoid spontaneity related to food. Predictability decreases anxiety and eliminates disappointment related to false hopes for "treats" or other foods not included in the plan.
2. Post the Plan - Everyone involved should be able to see menus for mealtimes and snacks. It's essential that everyone participate 100% of the time for the plan to succeed.
3. Control Accessibility to Food - At home, lock the refrigerator, pantry, cupboards (or even the entire kitchen) to remove access to food. Supervise and minimize exposure to food whenever possible. At school, plan routes to classes without passing the cafeteria. When considering vocational training, avoid jobs that include food service.
4. Develop Contingency Plans - People with PWS and their caregivers should enter food situations with a plan that can help to manage the circumstances. Prepare ahead how to participate in social events, especially special occasions like birthday or holiday parties. Access restaurant menus in advance to discuss what will be ordered. Stress to others that non-compliance by anyone involved, no matter how well-meaning, is harmful.
5. Ask for Help - Life happens. Children grow, and circumstances change, so plans that work for a few months or years may not be sustainable long-term. Ask the treatment team at The Children's Institute for help in evaluating needs and implementing changes that address evolving challenges.
Food security builds trust and diminishes the brain's hyperawareness of food. The demands of achieving food security are great, but the benefits to overall health, wellness and quality of life are significant.
Posted by The Children's Institute - Dec 12, 2014
Holidays can be a stressful time of year for many people for a multitude of reasons. They are particularly stressful for individuals with Prader-Willi syndrome and their families. This time of year, there are many holiday parties and gatherings with family, at school, at work, and in the community. Most of these holiday festivities include food, are usually unstructured in nature and involve many people. All of these factors lead to increased anxiety for individuals with PWS, which may manifest itself in difficult behavior . The following are some behavioral and environmental guidelines to assist you with navigating through the holiday season.
• Make plans. If attending a holiday party with family, at school or in the community, always plan ahead with your child. Let your child know when the party will be, what activities your child will be participating in, and if your child will be eating prior to/after, or at the party. Identify exactly what your child will be eating.
• Review the plan with your child starting a couple of days before the party. Bring a copy of the plan to the party.
• Go over the “rules” for the party. Be specific about expected behaviors. Inform your child of who will be supervising them during the party.
• Host the party. You have much more control over food, structure, and managing the environment in your own home. You can be a model for the rest of your friends and family on how to throw a wonderful holiday party, without having the focus be on food.
• If going somewhere else for a holiday party, know the menu. Talk to the host and plan what food your child can have and what you will need to bring to the party for your child.
• Avoid family style meals. Plan to have the food prepared and served in the kitchen, separate from the dining area. Prepare the plate for your child with exactly what was planned on your child’s menu.
• You and your child may want a “special” place to eat, away from the majority of people who are eating heaping plates of holiday food.
• Once the meal is complete, engage your child in activities away from the dining area. (work from the plan you created and reviewed with your child prior to the party.)
• All food should be put away when the meal is complete so that it does not provide an additional source of temptation or anxiety for your child.
• If eating a meal during a holiday party is just too difficult for your child, you may want to join the party after the meal has been served and leftovers have been put away.
• The important aspect of the holiday season is to focus on the activities at the party and socialization with friends and family. Take the focus off of food! Read More...
Posted by The Children's Institute - Oct 29, 2014
Halloween is an exciting time of year for kids. Costumes, pumpkins, parties, parades… and of course, CANDY. But what do you do when the oodles of candy can be dangerous for your child? How does the parent of a child with Prader-Willi syndrome tackle this candy-crazed holiday? Read More...
Posted by The Children's Institute - Oct 07, 2014
Sunday, September 14th was a special day for the Prader-Willi syndrome community in Pittsburgh, because we were able to meet up with our favorite baseball team: the Pittsburgh Pirates! Pirates Charities hosted kids and families from the PA chapter of the Prader-Willi Syndrome Association for a fun filled day at PNC Park. The Children’s Institute’s patients with Prader-Willi Syndrome were lucky enough to be personally invited to spend the day with Pirates’ manager Clint Hurdle and assistant coaches. Read More...
Posted by The Children's Institute - Aug 28, 2014
It's back to school time, which brings new teachers, new friends, and sometimes new challenges. Our inpatient teacher, Amy McTighe, PhD, MEd, answered some questions that she frequently hears from parents of children with Prader-Willi syndrome. Read on, and make it a great school year!
Posted by The Children's Institute - Jul 25, 2014
It must be Christmas in July! One of our patients recently wrote a letter to Santa Claus and received a response with the help of our amazing nurses. Read More...
Posted by The Children's Institute - Jun 26, 2014
This month, we sat down with Dr. Roxann Diez Gross, PhD, CCC-SLP, Director of Research at The Children’s Institute, and Ronit Gisser, M. Sc., CCC-SLP, Research Administrative Assistant, to get the scoop on their most recent PWS research project. They are finalizing results of a study that has the potential to improve the clinical treatment of patients with Prader-Willi syndrome.
What was the focus of the study?
The study looked at swallowing and respiration of patients as they ate and drank. That’s important because, too often, patients with PWS choke and aspirate. This is the first time that swallowing function has been investigated in the PWS population. Read More...
Posted by The Children's Institute - May 31, 2014
May is Prader-Willi Syndrome Awareness Month. What month could be more appropriate to launch our new Center for Prader-Willi Syndrome Blog? Several times a year, our expert team members will share insights based on their experiences working with individuals with Prader-Willi syndrome. Read More...