PWS Awareness Month- Day 1

Hi Everyone,

Welcome back to Prader-Willi Awareness Month!  We want to thank everyone ahead of time for taking the time to read our blog, ask questions and just learn about Kemett.

It has been a great year so far with a lot of progress being made.  I cannot wait to share it all with you.

First I wanted to give you all a quick refresher on PWS.  Here is a great picture that describes the genetics.

PWS Genetics

Handout from PWSA

________________________________________________________________________________

What are the symptoms of Prader-Willi syndrome?

The symptoms of Prader-Willi syndrome are likely due to dysfunction of a portion of the brain called the hypothalamus. The hypothalamus is a small endocrine organ at the base of the brain that plays a crucial role in many bodily functions, including regulating hunger and satiety, body temperature, pain, sleep-wake balance, fluid balance, emotions, and fertility. Although hypothalamic dysfunction is believed to lead to the symptoms of PWS, it is not yet clear how the genetic abnormality causes hypothalamic dysfunction.

The symptoms of PWS change over time in individuals with PWS, and a detailed understanding of the nutritional stages of PWS has been published.  Overall, there are two general stages of the symptoms associated with PWS:

Early Life

Infants with PWS are hypotonic or “floppy”, with very low muscle tone. A weak cry and a poor suck reflex are typical. Babies with PWS usually are unable to breastfeed and frequently require tube feeding. These infants may suffer from “failure to thrive” if feeding difficulties are not carefully monitored and treated. As these babies grow older, strength and muscle tone generally improve.  Motor milestones are achieved, but are usually delayed.  Toddlers typically enter a period where they may begin to gain weight easily, prior to having a heightened interest in food.

Childhood and Beyond

An unregulated appetite and easy weight gain characterize the later stages of PWS. These features most commonly begin between ages 3 and 8 years old, but are variable in onset and intensity.  Individuals with PWS lack normal hunger and satiety cues. They usually are not able to control their food intake and will overeat if not closely monitored. Food seeking behaviors are very common. In addition, the metabolic rate of persons with PWS is lower than normal. Left untreated, this combination of problems leads to morbid obesity and its many complications.

In addition to obesity, a variety of other symptoms can be associated with Prader-Willi syndrome. Individuals usually exhibit cognitive challenges, with measured IQs ranging from low normal to moderate intellectual disability. Those with normal IQs usually exhibit learning disabilities. Other issues may include growth hormone deficiency/short stature, small hands and feet, scoliosis, sleep disturbances with excessive daytime sleepiness, high pain threshold, speech apraxia/dyspraxia, and infertility. Behavioral difficulties may include obsessive-compulsive symptoms, skin picking, and difficulty controlling emotions. Adults with PWS are at increased risk for mental illness. PWS is a spectrum disorder and symptoms vary in severity and occurrence among individuals.

Information taken from FPWR.org.

Let us know if you have any questions during this month.  We are very open and honest about our lives with Kemett!

See you tomorrow!

Love,

The Demands

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PWS Awareness Month- Day 1

Hi Everyone,

Welcome back to Prader-Willi Awareness Month!  We want to thank everyone ahead of time for taking the time to read our blog, ask questions and just learn about Kemett.

It has been a great year so far with a lot of progress being made.  I cannot wait to share it all with you.

First I wanted to give you all a quick refresher on PWS.  Here is a great picture that describes the genetics.

PWS Genetics

Handout from PWSA

________________________________________________________________________________

What are the symptoms of Prader-Willi syndrome?

The symptoms of Prader-Willi syndrome are likely due to dysfunction of a portion of the brain called the hypothalamus. The hypothalamus is a small endocrine organ at the base of the brain that plays a crucial role in many bodily functions, including regulating hunger and satiety, body temperature, pain, sleep-wake balance, fluid balance, emotions, and fertility. Although hypothalamic dysfunction is believed to lead to the symptoms of PWS, it is not yet clear how the genetic abnormality causes hypothalamic dysfunction.

The symptoms of PWS change over time in individuals with PWS, and a detailed understanding of the nutritional stages of PWS has been published.  Overall, there are two general stages of the symptoms associated with PWS:

Early Life

Infants with PWS are hypotonic or “floppy”, with very low muscle tone. A weak cry and a poor suck reflex are typical. Babies with PWS usually are unable to breastfeed and frequently require tube feeding. These infants may suffer from “failure to thrive” if feeding difficulties are not carefully monitored and treated. As these babies grow older, strength and muscle tone generally improve.  Motor milestones are achieved, but are usually delayed.  Toddlers typically enter a period where they may begin to gain weight easily, prior to having a heightened interest in food.

Childhood and Beyond

An unregulated appetite and easy weight gain characterize the later stages of PWS. These features most commonly begin between ages 3 and 8 years old, but are variable in onset and intensity.  Individuals with PWS lack normal hunger and satiety cues. They usually are not able to control their food intake and will overeat if not closely monitored. Food seeking behaviors are very common. In addition, the metabolic rate of persons with PWS is lower than normal. Left untreated, this combination of problems leads to morbid obesity and its many complications.

In addition to obesity, a variety of other symptoms can be associated with Prader-Willi syndrome. Individuals usually exhibit cognitive challenges, with measured IQs ranging from low normal to moderate intellectual disability. Those with normal IQs usually exhibit learning disabilities. Other issues may include growth hormone deficiency/short stature, small hands and feet, scoliosis, sleep disturbances with excessive daytime sleepiness, high pain threshold, speech apraxia/dyspraxia, and infertility. Behavioral difficulties may include obsessive-compulsive symptoms, skin picking, and difficulty controlling emotions. Adults with PWS are at increased risk for mental illness. PWS is a spectrum disorder and symptoms vary in severity and occurrence among individuals.

Information taken from FPWR.org.

Let us know if you have any questions during this month.  We are very open and honest about our lives with Kemett!  This week we will be giving updates on Kemett’s different doctors and medications he takes.

See you tomorrow!

Love,

The Demands

Kemett is 8 Months!

Happy May everyone!

This month is Prader-Willi Awareness Month!  Will you participate in a month long challenge with us? Pledge to eat well for the month, or exercise daily. By sacrificing the sweets or promising to sweat, your joining our friends with PWS in things that they have to do every single day. Andrew and I have changed our diets over the past couple of months for Kemett- removing processed foods, added sugars, and hormones in meat. So, I pledge to exercise daily with and for Kemett! Let us know what you will be doing or share it on your facebook page!I hope to update you a little more often on the blog about PWS and Kemett!

Kemett has had a pretty good month.  Here are some highlights:

  • Kemett has 4 teeth!
  • He graduated from the helmet last Wednesday.
  • He is using a straw cup now!
  • We changed Kemett’s schedule, so he eats three meals a day in his high chair and has two snacks.  He now goes to bed at 7:45 each night!
  • We think he has some food allergies, so we have removed Dairy and Soy from his diet and mine as well
  • We worked with a PWS nutritionist from California this month and learned so much about Kemett’s nutrition!
  • Kemett took a road trip to Atlanta, GA driving through Texas, Louisiana, Mississippi, Alabama and Georgia.
  • He is 16 lbs 3 oz and 27 inches long!

Kemett also had a sad month.  We experienced three deaths- Andrew’s grandfather, Lyman Demand, my dad’s brother, Gerald Fredrick and one of Kemett’s special friends with PWS, Violet Hasenmyer.  We love all of these individuals and our thoughts and prayers are with their families.

Below are some pictures from the past month.

Love,
The Demands

Kemett is 8 Months!

Kemett is 8 Months!

Kemett is 8 Months!

Kemett is 8 Months!

Kemett is 8 Months!

Kemett is 8 Months!

Loves his high chair!

Loves his high chair!

Headed out on the town!

Headed out on the town!

Tummy Time!

Tummy Time!

Sipping from a real straw cup!

Sipping from a real straw cup!

Happy Easter!

Happy Easter!

My diploma! No more helmet!

My diploma! No more helmet!