May is Prader Willi awareness month, even though I’ve worked with children with various additional needs I had never heard of this condition. That was until December when I had an enquiry for our Baby Milestones Plan for a gorgeous baby boy, Wolfe.
Of course I did the obvious and took to Google, Wolfe’s Mum and Gran had already told me a little about Wolfe; that he was on oxygen and had low muscle tone.
All of my sessions are baby led, so having a baby with additional needs is really not that different. With all babies it’s baby’s safety and comfort that are a priority, yes there are a few extra considerations to be made, but nothing that can’t be worked with by not being afraid to ask questions of the parents and doing a little research.
Wolfe was nearly 6 months old when he came into the studio, but having been born 13 weeks early and spending his first months in hospital this was the first opportunity for him to have “professional” photos and I was so pleased that his family chose me to document his baby milestones. Wolfe’s milestones may be a little different…but even more reason to document and celebrate them.
For Wolfe’s first session he was still on oxygen, but he had had his NG tube removed just a few days earlier. We were able to remove his pegs momentarily for each photograph, replacing them in between shots and while I positioned Wolfe. I had to be aware of his oxygen and pay a little closer attention to his breathing and circulation, but apart from that Wolfe was just like every other baby I have photographed….there were some positions he was comfortable in and some things that he wasn’t so happy about….he seemed quite determined that he wasn’t going to let me wrap him! I got there eventually….sort of…But he sure gave me a run for my money, much to our amusement.
Wolfe’s Mummy is trying to raise awareness of Prader Willi Syndrome and has been sharing something each day on her Facebook. When I read her posts it really opened my eyes to the many challenges that Wolfe and his lovely family have already overcome, it’s going to be a long road, but this boy is a determined wee boy and with the strength of his lovely family I’m confident he will go far!
I’m really looking forward to sharing some of those milestones with them.
I invited Emily to share their story so far, and I am so pleased she agreed.
Wolfe arrived early, 13 weeks early to be exact. At 1lb 15oz, he was born by emergency c-section into a clear plastic bag looking like a shake and bake chicken, made one beautiful cry and was whisked off to the NICU. We knew he had a tough road ahead, but we didn’t fully understand the rollercoaster we had just boarded. It would be 6 days before I held him to my chest, one month before we heard another wonderful squeak of a cry, and 153 days until he would be well enough to come home.
His initial weeks were full of ups and downs. His lungs were particularly bad for a baby born at 27 weeks, especially as I had been given steroids to help mature his lungs the week before he was born. He suffered several lung collapses requiring life saving emergency chest drains, he had several blood transfusions, his heart and kidneys failed, but somehow (in no small part due to the wonderful doctors and nurses at the Princess Royal Maternity Hospital) he just kept going.
Nearing his due date we were painfully aware that he was not like the other preemie babies. He hardly cried, he slept a lot, he barely moved and although he knew what to do, he just couldn’t get the hang of breast feeding. We had questions, and a day after his due date we got our answer. Wolfe has Prader Willi Syndrome.
PWS is a very rare and extremely complex genetic disorder caused by a missing or defective section of chromosome 15. It occurs in approximately one out of every 15,000 – 20,000 births. To put this in to perspective, Down Syndrome occurs in around 1 in 1,000 births, so it’s not surprising if you haven’t heard of it before.
PWS affects appetite, growth, metabolism, cognitive function and behavior. It is a spectrum disorder, meaning that not all people with PWS have all the symptoms, and not all to the same extent.
It is typically characterised by low muscle tone, short stature (when not treated with growth hormone), cognitive disabilities, behavioral problems, and the hallmark characteristics of chronic feelings of insatiable hunger and a slowed metabolism that can lead to excessive eating and life-threatening obesity.
Babies with PWS are hypotonic or “floppy”, with very low muscle tone, a weak cry and a poor suck reflex. They are usually unable to breastfeed and frequently require tube feeding. They may suffer from “failure to thrive” if feeding difficulties are not carefully managed. Wolfe required feeds through an NG tube until weaning, though managed to take the majority of his feed from a bottle once correctly assessed by a supportive Speech and Language therapist.
As babies with PWS grow older, strength and muscle tone generally improve. Motor milestones are achieved, but are often delayed due to earlier ‘floppiness’ and a different body composition from typical infants.
Wolfe is coming on great with building muscle tone – after months of nothing but face planting and tears during tummy time, he’s now confidently holding his head up, trying to prop himself up on his elbows and knees and learning to sit unassisted.
Toddlers typically enter a period where they may begin to rapidly gain weight, prior to having a heightened interest in food.
An unregulated appetite and easy weight gain characterise the later stages of PWS. People with PWS ALWAYS feel hungry and NEVER feel full.
In addition, the metabolic rate of people with PWS is much lower than normal, so their required calorie intake is far less (often as low as 60% of their peers) to sustain a healthy weight, meaning those with PWS must eat LESS than the average person, while always feeling hungry and never being satiated.
This combination of problems can lead to morbid obesity and many health complications.
In all honesty, Hyperphagia is what terrifies us most about PWS and the future for Wolfe.
While there have been lots of trials of things to try to stop the insatiable hunger, nothing so far has worked. There is NO cure for Hyperphagia, which is the biggest obstacle for people with PWS living independently and living life to the full.
Although in the past many people with PWS died in adolescence or young adulthood, prevention of obesity can enable those with the syndrome to live a normal lifespan. New medications, including psychotropic drugs and growth hormone, are already improving the quality of life for some people with PWS. Ongoing research offers the hope of new discoveries that will enable people affected by this condition to live more independent lives. This is why we need more funding for research – a cure is out there, let’s find it!
Wolfe is amazing, PWS will not define him.
He still requires oxygen 24/7, and has hospital appointments every week, but is getting bigger and stronger every day. This kid has already met so many obstacles head on and won! He makes us proud every day (even when he’s being a little bugger!)
You can find out more about Prada Willi Syndrome HERE & HERE
