Defying Goldenhar Syndrome
Goldenhar Syndrome, which is also known as oculo-auriculo-vertebral (OAV) syndrome, is a rare congenital defect characterized by incomplete development of the ear, nose, soft palate, lip and jaws. It is an ‘umbrella’ term for a wide range of bone abnormalities affecting the face and sometimes the vertebrae. In about 60 per cent of cases, those facial abnormalities occur on one side of the body, but in approximately 10 to 33 per cent of cases, a patient has malformations on both sides of the face, with one side typically more affected than the other. The severity of the disease can vary greatly.
The cause of Goldenhar Syndrome has yet been identified. A baby’s face develops during the 8th to 12th week of pregnancy where several types of tissues grow together and form facial features. The tissues that become the face and jaw start separate from the upper part of the face. Goldenhar Syndrome occurs at this phase. However, the cause is still unknown. However, parents should not worry as children with Goldenhar Syndrome have normal intelligence and live normal life spans.
Kerry Holmes could not hold back her tears when she dropped off her son to school on his first day. Morgan, who was born with Goldenhar syndrome, has keen intelligence and strong optimism. Although he was born with one eye and ear, he has proven to be a survivor.
“The doctors can’t believe he’s alive. Never mind attending a mainstream primary school,”says Kerry. “I’m not surprised, though. I know Morgan will do whatever he wants in life.”
When she was six months pregnant, the fluid on her unborn baby’s brain had increased to an extent that doctors told her that her child would have major brain damage. It was at this point she was ushered into a room with a counsellor who told her that she had the option of aborting the baby. However, Kerry chose to keep her baby after going through an MRI scan. The scan showed there was brain activity which meant the baby was not brain dead. But still, she was not prepared for the physical abnormality.
“As soon as I’d given birth, my baby was immediately whipped away from me and all I could hear was the father saying, “What’s wrong with his ear? Why is his ear so little?’’, she recalls. In fact, baby Morgan’s ear was not little, it was missing.” says Kerry.
‘I demanded they give me my baby, so they put him on my chest. I looked down at his little face and saw he had only one eye. I really don’t know what I was feeling. I was high on gas and air, and I was so tired. I can remember looking at him and thinking, “What is going on?” ‘
For four days, Kerry only allowed her immediate family and the medical staff to look at Morgan.
“I needed to get used to the way he looked before I started letting other people see him. I didn’t want others judging him,” she says.
A few weeks after being allowed home, Morgan was rushed back to hospital after contracting a lung infection and turning blue. Soon afterwards, he suddenly stopped breathing and went into cardiac arrest. It was tough for Kerry to watch the little one suffering. There was blood everywhere because the medical staff had to shove tubes down his throat. Then, he was sent to Oxford’s John Radcliffe, where he had an emergency tracheotomy operation. The doctors explained his airways were twisted and that a tracheotomy was his only chance of survival. She was devastated when she was told that Morgan only had five percent chance of surviving.
Morgan was taken off his life-support machine a few weeks later. Kerry was taught how to change her son’s tracheotomy apparatus and Morgan was allowed home. Morgan, miraculously, became stronger and more able to fight infection. He became strong enough to go through two major operations. One on his jaw and the other on his eye.
He was well enough to undergo two major operations – one on his jaw, the other on his eye. Another concern that Kerry has is the reactions that Morgan receives when he mixes around with other kids.
“People’s reactions to Morgan vary. Some parents act embarrassed when their kids point or stare, and tell them to be quiet. I actually prefer that they come up to me and ask me about Morgan. I’m not ashamed of my son and Morgan isn’t ashamed of who he is. I’d rather answer questions than be pointed at,” says Kerry.
However, Kerry found the joy witnessing Morgan’s mental and emotional development over the years. He is a bright boy, despite he is physically challenged. Given his intelligence, Kerry said no to many schools she visited until she found one she felt was best suited to helping him develop, Westlea Primary in Swindon.
“On the first day, I thought he would ask me to stay with him. But as soon as he got into the classroom he started to play with some cars. He then turned to me and said goodbye. When I picked him up, he told me he had fun and I gave him a big cuddle,” says Kerry.
Morgan’s achievements have astounded doctors and defied all their expectations. It is more important that Morgan has proven that Goldenhar Syndrome did not deter him from achieving his potential and Kerry has shown us the importance of believing in him.
Leong Kim Weng is a writer who writes about parenthood's articles. He uses this platform to reach out to the young parents. Writing for www.parentsdojo.com has given him the opportunity to learn and share interesting perspectives with others.