Monday, 24 March 2014

Food

Food is everywhere.

The day after we got the Lodger's diagnosis, we were walking into the hospital and all we could notice was Food!!  
Adverts for food, shops selling food, cafes, restaurants, a bus with an advert for a burger on it, people eating food... it went on and on! In the early days of learning about Prader Willi Syndrome our focus was almost entirely on trying to get our minds around hyperphagia.

Hyperphagia?


Defined as: 

abnormally increased appetite for consumption of food frequently associated with injury to the hypothalamus

In other words, a preoccupation with food that if left uncontrolled can lead to life threatening obesity. 
The part in the brain that tells us we are full, is broken in people with PWS. Even after eating, someone with PWS is hungry.

As parents, we both felt enormously guilty anytime we ate something. We worried and felt very anxious about how The Lodger was going to be able to live in a world that is filled with food. We ate a fairly healthy diet but with The Lodgers diagnosis, we decided it would only be fair to him if we adopted a much healthier diet.


I can't remember the last time we had a takeaway. At the weekend, we plan the meals for the week ahead. We don't eat, other than at meal times. We try not to eat infront of The Lodger if he isn't eating. We only eat in the kitchen. We allow ourselves one treat a week. These little changes have made a huge difference in our house.


I used to be a chocoholic! ! !


When does hyperphagia start?


We've been told, anywhere from 18months onwards. Sometimes earlier, sometimes later. 


Is the Lodger aware of food?


Yes he is. Though since his arrival  his meals have been scheduled. 


Breakfast, snack, lunch, snack, dinner.


He could just be genuinely hungry when mealtime arrives. It's so hard to know.





We've learned from other families who have kids with PWS, that behaviour around food is important in the long-term, that putting in rules now will help us in the future (as habits are so hard to change).


We both  have become FOOD POLICE! 


When we were having a meal on holidays, the waiter picked up a chip to give it to The Lodger and we both dived across the table. The waiter must have thought 'overprotective, crazed parents'. It was our first incident of a stranger giving him food- I'm sure it won't be the last! 


Family and friends know not to bring food if they are visiting. 


The Lodger is never  rewarded with food. If he's sad, he's never cheered up with food. It can be hard, as it's in human nature to spoil kids with treats.


The Lodger's grandparents are getting a new kitchen fitted and are making it Lodger friendly. A locked pantry will be added so The Lodger can feel comfortable and safe in their home. It's little things like this, that can make a big difference to someone with PWS, reducing their anxiety around food and lessening the urge of  food seeking behaviours.


We've been looking into alternative birthday cake designs... 




There are many other symptoms associated with PWS. Hyperphagia is just one.
We are only 20 months in. We are still learning and there's a lot to learn.

We just hope medical advances through research will change the future for all with PWS.


This gave us a giggle on a recent trip to Dublin Zoo- edit out 'these animals' and input The Lodger. Respect our decisions as his parents to protect him from food :) 









Wednesday, 19 March 2014

Milestones in The Lodgers First Year

 Milestones! A baby's first year is all about milestones.

For most people baby milestones mean: Rolling Over, Sitting, Crawling, Walking...

For us milestones are a little different.

I remember texting my parents from NICU when The Lodger was 5 days old to tell them he moved his arm today. It was litreally just that, he lifted his arm and it fell back down!! In our little bubble, this was a huge moment. This happened before the diagnosis of Prader-Willi Syndrome so we were still living with the hope that The Lodger was just taking a little bit longer to settle into the world than other babies.

The Lodger never required oxygen once he got home, which was a great relief to us. However due to a dislocated hip, he was in a harness for 8 weeks. I had completely forgotten about this until I had a look through his appointment diary. The dislocated hip meant a weekly visit into hospital to get the harness refitted. We used to love the day before the harness refit, as this was bath day and The Lodger loved the bath. He moved so much in the water!! This day was also harness free cuddle day!!

Week by week, The Lodger got a little stronger. I've it noted in the diary that he started to play at 16 weeks old. He was able to reach forward to move the toys hanging infront of him. At 5 months old there was a noticeable improvement in his head control. His neck didn't have the neck strength to hold his head up but could move his head side to side.



Physiotherapy plays a big part in The Lodgers day. It did when he was smaller and still does. Tummy Time was so important for him getting him stronger to lift up his head. 

We were on holidays when The Lodger surprised us with his next milestone. HEAD CONTROL!!



Just before his 1st birthday, The Lodger was able to sit!!! For his birthday, he spent most of the day sitting in a cardboard box- as it gave him great support! He also loved sitting in things, still does!



With PWS, you notice every little development that other parents might not see-
The day he started playing with his hands.
The day he started chewing his fingers.
The day he was able to lie on his back and lift his legs in the air.
The day he pulled my hair.
The day he was able to move each finger independently.
The day he was able to play with his toes.
The day he pulled his socks off.
The day he took his hat off.
The day he started laughing, smiling and finding his voice.
The day he rolled over.

Low muscle tone means alot of things are challenging for someone with PWS, as all their muscles are so weak.

There are days when The Lodger really doesn't want to do physio. For a little boy that couldn't cry, he'd have his own ways of showing us his determination for not wanting to do something. As a mother, it's hard to make your little baby work hard but seeing The Lodger reach milestones makes it all worth while.

Physio continues on a daily basis, it's always based around play and rewarded with doing his favourite activities- reading, music and lego!











Friday, 14 March 2014

The beginning

The Lodger was diagnosed with Prader-Willi syndrome when he was just two weeks old.

Two weeks old.

Two weeks old and your whole future changes in the blink of an eyelid because of one deletion from one chromosome.

Throughout the whole nine months of pregnancy, we thought everything was fine. We just had the usual first time parent nerves about becoming protectors of a tiny human.

Looking back, I don't think anything could have prepared us for the bombshell of hearing the diagnosis- Prader Willi Syndrome.

We had spent one blissful hour with The Lodger on his birthday. I will treasure this short time forever. One hour of no knowledge of medical terminology to describe your baby. For that one hour he was just The Lodger, perfect in every way. 

He was quickly whisked away to the neonatal intensive care ward where he would spend the next three weeks.

The Lodger had hypotonia. Hypotonia?We'd soon learn the medical terms for all the symptoms associated with PWS. 

Hypotonia = low muscle tone. 

The Lodger could move his eyes and nothing else. He couldn't move his arms or his legs. He couldn't cry. He didn't make a sound.

When you're in NICU, you live in a bubble and quickly learn the hospital routines and rotas, amidst learning how to be a parent of a baby who needs a little bit more care and attention than most. 

The Lodger required oxygen from the start, as his muscles were too weak so he struggled with breathing and keeping his oxygen saturation levels high.




Unlike most PWS babies, the Lodger took a bottle- although it took a lot of work and feeding techniques taught to us by the nurses caring for him. Feeding the Lodger was on a schedule from Day One, even before the diagnosis of PWS. 

Prader-Willi Syndrome is strange. Failure to thrive at the beginning where babies struggle to feed and gain weight and then anywhere from two years old onwards, an insatiable appetite and rapid weight gain. 

At 3 weeks old, The Lodger was finally able to come home- off oxygen but with a monitor, oxygen canisters and an oxygen machine at home.

This is where the journey continues...



The Lodger and Us

I was looking back through photographs and videos  of The Lodger today and I quickly realised, apart from the fact he is growing up very quickly, he has achieved alot of things in the short time he's been here.

The Lodger is 20 months old now. He was diagnosed with Prader-Willi Syndrome when he was just two weeks old. 

I hope to document the highs (and lows) of living with Prader-Willi Syndrome.

Welcome to Our Life!