12-year old Adam from Kingaroy is facing a challenge unlike any other child his age. Never without a smile, it’s easy to forget Adam is living in pain due to a large tumour compressing his spinal cord.
Adam was diagnosed with neurofibromatosis type 1 in 2009 after being misdiagnosed at 6 weeks old. Since his diagnosis Adam has developed the symptoms characteristic of NF1 – lisch nodules, cafe-au-lait spots, and neurofibromas all over his body, however his biggest challenge has been a large plexiform neurofibroma that spans across his shoulder, chest, neck and face, including his left ear, and extends to a mass on the back of his neck that surrounds and compresses his spinal cord.
The large growth has caused Adam to experience changes in the sensations typically felt in his arms and legs due to the immense pressure that has been applied on his spinal cord. The impact of the growth has also created a curve in Adam’s spine and has caused his neck to develop into an S-shape. Adam’s spine has become so sensitive that sudden jolts to his spinal cord received through normal childhood falls often resulted in temporary paralysis episodes.
Although Adam is considered to be at the most severe end of the spectrum for NF1 cases, he has surprised doctors with the limited amount of pain he has reported experiencing in association with his myriad of medical complications. Doctors assumed Adam had learnt to deal with any associated pain as the tumour grew with him, however things changed in December 2014. Pain suddenly became a more frequent and prominent part of Adam’s NF battle. Movement and actions that appeared seemingly simple, such as walking from his bed to his bedroom door or sitting at the kitchen table for longer than a few minutes, became increasingly difficult and painful for Adam. His parents decided to make an emergency trip to Lady Cilento hospital in Brisbane over the Christmas period, although x-ray scans failed to reveal signs of any further abnormalities.
By mid-January 2015 doctors deemed it too much of a risk to send Adam home and decided to conduct emergency surgery to ease the pain. Adam was fitted with a halo brace for traction to help relieve the pressure on his spinal cord and reduce the pain he was experiencing. Adam underwent further surgery to insert tissue expanders which are injected with a saline solution on a regular basis in the hope that this will stretch Adam’s skin and encourage new skin to grow. The procedure requires Adam and his family to travel a total of seven hours every two weeks for 4-5 months. Doctors hope this will provide them with a way to remove part of the tumour in Adam’s neck and fuse and stabilise his spine, but the surgery is not without risks given the tendency for plexiform neurofibromas to bleed, heal poorly, and deteriorate the quality of the bone they surround.
This is just the beginning of Adam’s biggest challenge, but he’s determined to come out stronger.
“He shows resilience, strength, radiates positivity, is caring and gentle, and loved by so many,” says Adam’s mum, Kim.
“All he wants is for people to learn more about his condition so that they are more aware.”
Adam has chosen to share his story to help raise awareness about NF and “so that other people know that they are not alone in their battle.” His bravery is an inspiration to us, to his family, and to everyone he meets.