Rachel's Corner

One of the things that really stinks about PWS early on is that, as parents, we're always in the "waiting for the other shoe to drop" mode. (I wonder where that phrase came from. It's quite odd, isn't it?) All kinds of things are going on with any child that age in terms of growth and development and because, for the most part, they can't tell us all we need to know to help, it's frustrating on both sides. For the situation at hand, we have tried, as I know you all do, to have everybody eat at the same time. When that's not possible and Erin has asked for food, even the smallest bite--and I mean sometimes TINY--of whatever someone else is having has been enough to satisfy her. That says to me that it's more of a curiosity than a hunger issue. It's more like "I want to be sure I'm not missing anything" than "I'm truly hungry."One other thing we have done with Erin is a little visual trick. Whatever we give her, we break it into two pieces and say, "Here is one for each hand." The psychological effect of having two things and having something in both hands leads to more satisfaction (but not more food).One other problem with having an infant w/PWS is that they spoil their parents. They are so easy, aside from the initial health issues, that any time they start spreading their wings and asserting their upcoming quest for independence, it seems like things are coming loose at the seams. Jake is getting stronger, and, in a sense, waking up. He's got energy and preferences and maybe a growth spurt and maybe cutting teeth and who knows what else, but he's on the move and becoming his own boy. Seeing this new child emerge takes some getting used to and letting that passive, quiet baby stage fade takes some courage. :)Those are all things for today. In looking ahead, which all we PWS parents do more frequently and more intensely than anyone else, I'd focus on activities and therapies that calm his central nervous system. I've talked quite a lot about this and won't repeat it all again, but a focus on sensory integration and strengthening his central core are all-important at this age. Let's take a look at the Sensory Center up near you when I get home. How about that?I'd also be sure that all his meals and snacks have both carbs and protein. It's good to keep that blood sugar in mind early on and support his body in this way, especially considering the havoc that PWS can create on a person's hunger signalling. When you give him an apple, put a little peanut butter on those slices. Slap a little turkey on that cracker, or a little cheese. Introduce things like hummus and add some spice to up the sensory experience. Make sure those taste buds wake up and know that they have been fed.One more thing you might want to consider is a timer. When you tell Jake that his supper will be ready soon, it's vague and all he hears is "not now." We used a timer like the one on this link (http://www.flaghouse.com/itemdy00.asp?T1=36298) so Erin could SEE how much time was left, or at least that there was time left. We used it for lots of other things, too, but it was very helpful when she was younger in helping her deal with waiting. I hope that helps,,,You know when I get started on something like this, I can't shut up. I'm going to close myself down before you add me to your "blocked" list, but I just wanted you to know that this is neither the end nor the beginning...just another part, and one you can handle. As always, I'm right down the road if you need me!Rachel

Sometimes, it seems like learning to live with this diagnosis involves mastering abbreviations. PWS...FPWR...GH...PT...OT...IEP...Etc. When we talk among ourselves, we understand each other easily, but those outside of our circles would have to take a crash course in PWS or get an interpreter to find their way through our conversations. :)This morning, in a note from a John M., dad to Tommy,18 months, w/PWS, John included the abbreviation--NQTF. For the life of me, I couldn't think what that meant. I went over all the online ones like LOL...BRB...DH...IMHO...and still didn't come up with anything. Fortuately, John was kind enough to spell it out in the next part of the note.Never Quit The Fight! The timing for this note was so good. Some days, we all want to quit. We just want PWS to be over, GH shots to be done with, doctors and therapists to be history, and the fear of the power of food to be gone. We want our children to play freely, learn easily, grow steadily, and sleep peacefully. We want them to grow tall, strong, lean, and smart. We want them to love and be loved. We want them to find satisfaction from intimate relationships, meaningful work, and a stimulating enviroment. We want all the things that every other parent in the world wants for their children. We want the life that our children would have had without PWS. This is not too much to ask!Just like our children's developmental skills, what we want is going to take a little longer and demand a little bit more of us than is thought to be typical. We can look at what is required of us, throw up our hands, and just let nature take its course. We can lament about how unfair of a hand we were dealt. We can rage against the cruelty of life. We can do any or all of those things, but, at the end of the day, we still have a precious child waiting patiently for us to lay all that aside, pull ourselves together, and be the kind of parents that he or she deserves.Friends, whatever struggle you are dealing with right now--NQTF! Whatever curve has been thrown your way--NQTF! Whatever fear, whatever challenge, whatever hurdle is in your path--NQTF! FPWR believes that the power of parents to change PWS is limitless. Our mission--to eliminate the challenges of PWS through the advancement of research--is more than our intention. It is our calling! Whatever else life has brought to you, whatever gifts have come your way, we all know deep in our hearts that part of our purpose in this life is to change what it means to have PWS. Knowing that we are doing everything we can for our children, and for children yet to be born, should give us a sense of fulfillment unlike any other. Whatever it takes will be worth it!So, John, you're so right. NQTF! Thank you, my friend, and Happy Birthday! You're the best!!!RachelBy the way, in addition to the note of encouragement, John sent donations that were given to him in honor of his recent birthday. Catt gave him a surprise party and requested that donations be made to FPWR in lieu of gifts. What loving friends and family you all have!

We were in a research project a couple of years back at the University of Louisville Sleep Center that studied children w/enlarged adenoids, like Erin's. They compared the results of six weeks of treatment w/Rhinocort (a prescription nasal spray) and placebo. What they found was that the adenoids of children on Rhinocort will shrink with its use. They can stay on the Rhinocort until their bodies essentially grow into their adenoids. Rhinocort may not suit everyone, but it was better than surgery for us. In the sleep study following the Rhinocort, Erin's apnea all but disappeared...just some tiny amount that would fall well within the normal range. Normal works! :)

Both girls are in camp this summer. The only problem we've had is the heat. We're just working around the hottest times of the day and enjoying all else. Erin is the Queen of the yoga class. Her counselors said that she was better than they are and she loves that! She has been helping the younger ones get and stay in position. She's such a hoot! This may be her calling. :)Here's the other interesting thing we've learned this summer. After all our winter woes with severe constipation, I came to the conclusion that she just wasn't getting enough food. We've kept her calories low her whole life and maybe by this age/size, she just needed more but how do you do that without weight gain? Of course, you increase your activity level. Well, I worked with a dietician and we came up with a plan that increased her calories from 800 to 1200 a day, a 50% increase. We also came up with an activity level during camp that we hoped would balance this out. Well, the first week of camp, with a 50% increase in food, she lost a pound. The second week, she broke even and since then she's maintained that. So now we know that she can handle this much food as long as she gets enough exercise.To do that, she was walking (with occasional jogs) a mile or more in the morning and then doing resistance training, mostly with bands. For safety, since they were working in groups, they didn't use weights, but we can use weights at home. In the afternoon, they have an hour of yoga and dance, then an hour in the pool. Inbetween, they walk some pretty steep hills to and from classes so that is added in. And bless her heart, she never complains. Some of the older ones were dropping like flies, but her counselors said that no matter what they asked of her, she kept trying. I told them in the beginning that they wouldn't have to push or motivate her. They'd be more likely to have to keep her from working too hard. She wants to meet or beat every standard and doesn't want the bar lowered for her even an inch!Her biggest struggle with sports comes with competitive group activities like basketball or soccer. She loves the games, but isn't aggressive or in any particular hurry. She will be the first to tell you that she's not "good" at these sports, but she still likes to play.Now, because of the heat, we're only doing the afternoon activities and we'll see what that does to the number of calories she can handle. I hate not to do those, but there's no shade at the track and it just gets too hot. I brought lots of fine motor things for us to work on so we do that in the down time.Now the challenge is to duplicate this program at home. We're going to be doing a combination program this year with her school that mixes some class time and some homeschool. I'll do all the core classes (literacy, math, science and social studies) at home and she'll go to school for the specialty classes (music, art, computer, library) as well as the extracurricular, field trips, class parties, school drama/music programs. I think it will be a good balance and am looking forward to this. It gives us a more flexible schedule so we can keep up this level of exercise and still maintain her therapy schedule, plus it keeps her involved with her school for, as she says, the "best" parts. :)We'll have the YMCA for bad weather and swimming, plus our treadmill and resistance equipment at home. We'll get her in a children's yoga class and see what else presents itself. We're in an intense program of craniosacral therapy and myofacial release at home and look forward to getting back to that. All in all, we should be able to do this and look forward at getting a chance to try. Having a diet that gives her a little more leeway and an exercise program that will match it is a great result for the summer. 

Friends,For your viewing pleasure, FPWR's new website is now officially launched. Many, many thanks to Jeff and Kari Porter, Carol Craig, Theresa Strong and everyone else who helped make this the excellent work that it is. As you know, a website is a work-in-progress. We have many other features to be added in time and changes to be made, but, for now, we're good to go. www.pwsresearch.org Enjoy!

Thanks, everybody, for all the good feedback about the website. I can speak for all of us involved by saying that it was a labor of love and we are proud to present it as the face of FPWR. So much of our communication takes place in cyberspace so it's very important that our web presence represents our work as clearly as possible. This new site does that and we're looking forward to all the ways that it's capabilities will make our work better and easier.I keep peeking at the new site, just to make sure it's still there. I'm as bad about this as I am about my children. Kristen is 15 and I still check at night to make sure she's breathing! It's probably a little late to be concerned about SIDS but old habits die hard! :)

Friends, Get out your calendars and sharpen your pencils! I've got a list of dates of upcoming FPWR fundraising events that will give you writer's cramp. :) No matter where you live, there will be an event not so far from you sometime during this year. Check out this list and make your plans to join with one or more of these fundraisers, all designed to support PWS research through FPWR. Thanks so much to all of you who are giving sacrificially of your time and energy to make these events happen. You're the best!

Friends, Please read the following post from researchers at UNC working with Dr. Mel Levine. This study is funded by FPWR and we support this work proudly. Enjoy!

Friends,As I said in an earlier email this week, I am posting a mock-up of the new website w/the logo that the Integer Group has developed for FPWR. I think most of us really liked the 'puzzle piece' theme, but as I mentioned, since the puzzle theme is now so closely tied to the (much larger) autism awareness movement, the board felt it would be detrimental to continue to use it.We have tried to use this opportunity to bring the mission of FPWR into sharper focus, and we are very excited about this "new face" of FPWR.

Friends,Every day there seems to be more to be thankful for in terms of hopefor our children. Theresa's posts about new possibilities alwaysthrill me. Knowing that people who have expertise in variousspecialties are uncovering new information that could lead to newtreatment options for our kids really comforts me. Even those whoare not looking specifically at PWS, like the prostate cancerresearch she posted about earlier this week, have the potential tobenefit the world of PWS. All of this works together to increasethe chances of our children living healthy, fulfilled lives. That'sreally what we want, isn't it? We want our children to live thelives they should have had without the diagnosis of PWS. We wantthem to be free to make choices, even mistakes, as both are part ofwhat it means to live as a truly independent human being.Even now, with Erin being only 10, I can't help but look ahead andwonder what life holds for her. These first ten years have beenincredible, days full of challenge and a decade full of overcomingchallenges. How different things would have been, though, if we hadjust passively waited for PWS to do its thing. If we listened tothe doctors when they said that there was nothing we could do, theprobable outcome is terrifying. Looking at her now, after 10 years,I can say that all the therapy, all the doctors, all theintervention, even all the worry, has been worth it. I'd chooseexhaustion over despair any day. :)I can't imagine how differently Erin's future might look if we hadalso listened to those who said that there was nothing we could doto influence the quality and quantity of research being done onPWS. If we had just remained passive, hoping that someone somewherewould do something, then the 12 studies that we have sponsored wouldyet be undone. The top medical journals that have published ourwork would have published someone else's findings about somethingelse. Thousands of people who have participated in our collectiveevents would still be unaware of PWS and the danger it presents toour children. All this, just because we had chosen to do nothing.But we didn't, did we? Those of us in FPWR are the kind of peoplewho need to be personally involved, who need to get our hands dirtyworking toward the answers we need. We need to know that we aredoing everything we can to make our children's lives better NOW.Our children don't have time for us to wait patiently for someonesomewhere to act. They don't have time for us to hope that otherstake the lead. And they don't have time for us to get stuck inworry when we should be out there working. As much as I wish itwere different, as much as I wish there were an easier way, I justdon't see it.We know what is possible when we have the money to supportresearch. Scientists COME TO US with ideas, possibilities to createa new future for our children. A NEW FUTURE! None of us have anyguarantees when it comes to our children, especially when you factorin PWS. The only guarantee comes when we take the path of leastresistance, throw our hands up in the air, and let nature take itscourse. This is one of the cases where nature, potentiallybeautiful and awe-inspiring, has fallen down on the job and left ourchildren holding the bag. Fortunately, in God's infinite wisdom, weare all designed for wholeness and we will keep looking for the keysto that wholeness until we find what we need.Health, happiness and the freedom to make choices are the primecomponents of an independent future and that is our ultimate goalfor our children--independence. As history has shown, gainingindependence often involves struggle and ours is no exception.Let's commit ourselves anew to doing everything we can to clear thepath for our children's independence. Nothing is more importantthan this. When we succeed, we will have made life better for ourchildren w/PWS, their siblings, and ourselves. We will have changedhistory--all part of a day's work for those of us in FPWR. :)We know we can make a difference. In the beginning, we hoped wecould, but now we KNOW we can. We just have to keep on doing thethings that we have been doing so well, and add to that the type offundraising techniques that can take us to the next level. So, ourjob now is to keep thinking of ways to reach out to our business andsocial contacts, to our friends and family, to anyone who is in aposition to help us. We look forward to the day when we no longerhave to ask for help, to the day when we can wish a fond farewell toconcerns stemming from PWS, to the day when our children's futuresare secure--to nothing less than our children's independence.Keep your mind sharp, your head up high, and your hands busy.Together we are doing great things for our children and for familieseverywhere dealing with PWS!Rachel