Yes, I said P.W.S. not P.M.S. Since May is ‘P.W.S. Awareness Month’, I decided to do the last blog of the month on it. Now some of you may be saying PMS – I know ALL about that! lol!! But look closely, no, I didn’t misspell it, it is P. W. S. or its formal name of Prader-Willi Syndrome.
I first encountered hearing about this 35 years ago when I was pregnant with my first child. As ‘coincidence’ would have it, I vaguely remember watching a talk show back in 1981 where they talked about children who had a rare condition called Prader-Willi Syndrome. These children couldn’t stop eating and had some developmental delays. Little did I know what I was to experience in my own life.
The year was 1981, August 18th at 8:18 in the morning – I was to give birth to my first daughter, who in my eyes was the most beautiful baby to be born! Little did I realize, at my young age of 20, I wasn’t fully aware of the concerns the doctor had about my precious baby. Jessica had been breech or ‘butt first’. After many, many hours of labor and realizing she wouldn’t turn, the doctors decided to do a Cesarean section delivery. No big deal right? We could handle this. Little did we know that the doctors were meeting and conferring on this new little baby and their concerns for her.
Shortly after she was born, the pediatrician said he had called in some specialists. The next day the geneticist from UConn came in to check our precious baby. He said, ‘there’s something wrong with your baby girl – we’re not sure what it is but her sucking is very poor and she’s like a rag-doll, she has no muscle tone’. He continued to say that she may never walk or talk and could have some degree of retardation. It would be years later I would see the report that said she might be severely retarded. The doctor left the room that day and I cried out to God, ‘how could there be something wrong with my precious child? Was it something I did?’
It would take 4 years before Jessica was confirmed with the diagnosis of Prader-Willi Syndrome. They highly suspected it with all her symptoms but it wasn’t until they did a chromosome test when she was 4 that it was confirmed, she had a deletion in her 15th chromosome. Prader-Willi syndrome is a genetic disorder that affects the part of the brain that tells these individuals they are full. Many of these children when born have poor muscle tone and poor sucking, thus causing some to be fed through a tube. Some of the other characteristics of P.W.S. include the children having no appetite at birth. Typically by the ages of 2 and 4 years old, they get an insatiable appetite where they literally feel hungry all the time. I recently heard a video which said, “imagine your hungriest day ever, feeling like that all the time, that’s what these kids experience daily”! Children on the spectrum may vary on the level of developmental delays. As the children get older, they may have small hands and feet & be shorter in stature (thus some have used growth hormone therapy). Most of these individuals only need half the calories of others their age.
In my personal journey, as I sat in my hospital room crying out to God, I opened my bible and got the scripture from John 9:1-3 regarding the blind man. In the story, a blind man is brought to Jesus for healing and the people asked him if he was blind because he sinned or, because of his parents’ sin? Jesus said, ‘neither’, and the man was given his sight, for the glory of God! I have held onto this verse from that day and I know one day Jessica will receive a full healing. I also understood that there were practical things we had to do every day until that time came.
Since Jessica was my 1st child, I tried nursing her but that proved impossible with her poor sucking skills. I would feed her by bottle but her sucking was so weak, it would take forever for her to drink an ounce! My sister-in-law recalls the time when Jess was 3 weeks old and she babysat for me for a whole day so I could go to a funeral out of state. By the time I got home, about 8 hours later, my sister-in-law was worried as Jessica barely drank a bottle!
Along with the poor sucking, Jessica was like a rag doll and had hypotonia or poor muscle tone. The doctors recommended a birth-to-three program for Jessica called ‘CREC’. Through the program, I was blessed with a nurse who came out to the house weekly to work with Jessica. She would teach me exercises to do with Jessie to try and build up her muscle tone. I never realized how much of the human body we take for granted until I had my sweet baby! Most of us walk and talked at normal stages and have seen our loved ones do the same. Unfortunately, that didn’t happen with my precious little girl. Every little thing was a milestone for her and we celebrated! We had to teach Jess how to crawl, one limb at a time; we would have to prop up one arm, then the next, then one knee, then the other only to start all over again because she couldn’t bare her weight. I remember her sitting in her walker and the first time she kicked off on her own, I cried. It was such a big step for her! When I would do exercises with Jess, she would cry and I would stop. I thought I was hurting her. What the nurse helped me see was that Jess was just playing me. She didn’t want to exercise and would cry to get out of it (sounds like some of us – hahaha!). As I sit and write this, the emotions of those days’ flood back and I remember wondering if the doctor just might be right – would Jessica ever walk or talk? She never did learn how to crawl like most toddlers instead, she learned how to roll! At a year old, she was the fastest roller in town and could get from one end of the room to the other rolling.
While I was pregnant with my son Rich, the CREC program changed funding and we were told Jess would no longer get services until she was three. Our only option at the time was to present a case to the board of education. The nurse and I worked together and gathered all kinds of statistics. We put together a report and presented it to the Board, trying to convince them of how beneficial it would be for them to continue providing services. Honestly, we would never have had to say a word because they fell in love with this beautiful little girl! She won their hearts and their funding! They agreed to continue her services.
My 1st son was born 17 months after Jessica in 1983. He had everything Jessica lacked and more! He was a football player at birth (9lbs. 5 ½ oz.) and had the strength and stamina of a healthy boy. He began to crawl at about 5 months old and would chase Jess! She would roll to get away or, roll to get to him! Ha-ha… Jessica wouldn’t walk until she was 26 months old but I didn’t care, I was just so excited she was walking, who cares how long it took!
When Jessica was diagnosed in 1985, she was one of only 600 known cases. Now that we had a name for what we were dealing with, I knew I needed to find out all I could. It just so happened that in June of that year, there was going to be a Prader-Willi Syndrome conference in CT. I made arrangements to go with my then husband but, we had been having some marital issues and when it came time for the conference, I ended up having to go alone. I have to say, it was one of the toughest seasons in my life. At this conference, I would see adults who were severely obese and hear about how they ate everything in sight. They are not only food but whatever they could get their hands on. The horror stories just about did me in. There were some who would drink whole bottles of cough medicine, almost killing themselves; there were some who would eat things like cigarettes or non-food items, you name it… thus causing more severe health issues. There were stories of having to put locks on cabinets and refrigerators; stories of growth hormones to try to bring height to these shorter individuals, just to name a few of the sessions. All I could do was try to stay strong during the sessions but go back to my hotel room at night and cry – ‘Lord, is this what I had to look forward to?’ The fact was, my marriage was falling apart, I had 2 babies at home and I was a little overwhelmed. But, God reminded me of the promise, He would take care of me and my babies… and He has; the LORD was my peace in the storm!
It has been an interesting journey with P.W.S. and I could write a book on all the adventures we’ve faced but I’ll highlight just a few:
– In the early days, I would keep the promise of God before me and treat Jessica as normal as I could, considering the challenges she faced.
– there was the time when Jessica was 4 and she ate the whole blueberry coffeecake that had been in a covered casserole dish. She put the cover back on so I wouldn’t know and she was promptly corrected, as my other children would have been.
– there was the time in kindergarten when I went to visit the school and her teacher realized she could talk (all this time she had been faking them out and was silent) lol!
– there were the many times she would get caught sneaking food when we suspected it but didn’t know the source
– there was the decision to attempt private school for a season, with a tutor in 4th grade
– there was the opportunity to play the flute. The day of the concert, Jess got her pinky caught in the car door but insisted on playing in the concert that night. She was determined to still play and did so with such grace and victory!
– there were middle school days of playing unified sports including volleyball and basketball
– oh, and did I mention that she was the best and quickest puzzle maker around?
– she is also an amazing card maker
– this little girl did learn to walk and talk! She also learned to read and write and learned she could write notes to help communicate what she needs
– now that she is an adult, she works at a Spring Making shop assembling parts & packages
– these are just some of the adventures with our miracle girl!
There are many families who deal with P.W.S. and have had to lock food up in cabinets or refrigerators. We were very fortunate in the early years, as we did not have to do that. It seemed every time Jess snuck food, she got caught. When Jessica got older, we did go through a stage where we had to break down and use locks. For whatever reason, she went through a time where she was sneaking at home, at work and wherever we were. You could always see her wheels turning and conniving how she could manipulate getting food. You may have long forgotten about that thing left behind on the counter like that banana or cookie, but she hadn’t. It got to the point where Jessica would sleep walk and get into the cabinets and fridge. She was putting weight on and we had to help her, however we could, as a family. Fortunately, where she worked, they were familiar with these types of things and did their part. At home, we got a cabinet with a lock that we put the food in so we could lock it. The fridge on the other hand, well that was a funny story – one of those things you need on this journey sometimes because laughter is good medicine! Ha-ha.
We bought a lock for our fridge and my husband decided to drill a hole in the fridge for the screws. The 1st hole went in o.k.. As my husband drilled the 2nd hole, we heard this whrrrshing sound and saw a cloud-like stream coming from the fridge! He had hit the Freon…. ha-ha-ha… all we could do was laugh! I needed a new fridge anyway, right?? Wow! That was quite some way to get a new fridge but we did get it. The one we picked out had handles so that we could put one of those chained locks through it. Thankfully, we only needed to use the locks for a short time and then it seemed like the phase had passed.
Recently we had to revisit a modified version of the locks; Jess began to take things again. It’s been a process that we have had to walk through and try to educate others on. When Jessica was younger, she was so cute that everybody just wanted to feed her, not quite understanding that she only needed half the calories of most individuals her age. Now that she’s older, it still can be a struggle. People don’t realize the problem it is for her and just leave food out openly. It’s neither their fault or hers, in the sense that she can’t help that she’s insatiably hungry all the time. With a lot of patience and educating, we try to work together to modify any issues that come up, as well as to give Jess consequences for the behaviors when they are out of hand.
In all of this, I think the important thing to remember in all of life is to that each person is an individual, created in the image of their Creator God. We all have things we are amazing at and areas that we are weak at. Just because a person has a genetic disorder with differences does not make them any lesser of a person. We have always treated Jessica as normal as we can, with modifications for her areas of weakness but her strengths are championed! She is one of the most compassionate young ladies I know. When I’m feeling low, she’s one of the first ones to pick up on it and her hugs are the bomb – there’s none like them! Her laughter lights up a room and her cards bring a smile to those who get them. I’ve always been saddened when she was ignored or looked down on by her peers but, God’s love has always prevailed! Like the time she wanted to take a knitting class at church, one of the older women in the ladies knitting group took her under her wing and taught her. Or, the fact that she loves little ones so much and took a childcare class to learn how to care for them. She passed as a helper and now helps with the little ones once a month, working alongside an adult so she can do what she enjoys. We still are waiting on her complete healing but are so thankful for the amazing growth she has had. Prader-Willi Syndrome is just a piece of who these individuals are, it’s not the whole of them! Believe for the impossible and you might be surprised! Love ya & thanks for sticking with us to the end!
P.S. – if you’re interested in more information on PWS you can go to: http://www.pwsausa.org/