Living with Pfeiffer Syndrome
Hannah Lindfield, 22, was born with Pfeiffer Syndrome, a rare genetic disorder that causes the bones of the skull to fuse together so it cannot grow. This leads to pressure on the brain and can trigger a whole range of neurological problems, as well as hearing and visual impairment.
Hannah has one of the most severe forms of Pfeiffer Syndrome, which gave her the characteristic cloverleaf shaped head and bulging eyes at birth. As she has matured, she has gradually lost much of her hearing and eyesight. Because her skull cannot expand as her brain grows, Hannah has experienced episodes of severe pain, which sometimes leave her hospitalised for months.
At 22, Hannah is one of the oldest people in the world with Pfeiffer Syndrome. “This means that a lot of the problems I have haven’t been seen before, so the treatments are largely experimental,” she explains. “Some of them work and some of them don’t. When I was 18, I became seriously ill with nausea and vomiting. I spent months in hospital but no-one was able to find the cause of my digestive problems or offer an effective way of managing them. Gradually they subsided enough to allow me to get on with my life, but I missed a lot of college as a result. It is difficult then because I got behind and people had already formed their social groups and I was not part of them.”
More than 100 operations
Hannah has lost count of the number of surgical procedures she’s had “I’ve had well over 100 lots of general anaesthetic,” she says.
“I have spent a lot of my life in hospital.”
Hannah was born with narrow airways, which meant that she had to breathe through a tube. The tracheotomy that helped her breathe remained in place until she was six, so she was unable to speak until then.
At 13, she underwent major surgery at Great Ormond Street Hospital, which entailed having her skull pinned inside a large metal frame. Over the course of few weeks, the screws on the frame were gradually turned to bring the front of her skull forwards. With characteristic humour, Hannah says: “I was dangerous inside that thing. If I turned suddenly I could knock someone’s head off! It was worth it, though, as it enlarged my skull. They also gave me new eye sockets so my eyes didn’t protrude.”
Cup half full
It is her sense of humour and a determination to remain positive that has got Hannah through some of the most challenging times of her life.
“I’ve always been a cup half full person. Generally, I’m really positive and I love to make people laugh.
“I do have lows, like anyone” she adds. “I was bullied when I was at school by someone who was supposed to be a friend and that knocked my confidence a lot. I think she was jealous of all the attention I was getting because of my condition. In hindsight, I think I could have handled the bullying better and asked her why she felt like that instead of asking the teachers to intervene. I did try texting her after it happened but she never replied.”
An adventurous streak
Determined not to let her condition rule her life, Hannah has always had an adventurous streak. She is a keen sailor and loves rock climbing. When she is well enough, Hannah is out on the water most weekends with her older brother, Joshua, 25. “I got my first boat, Ray of Sunshine, at the age of 15,” says Hannah. “Now, I look after the sails and Joshua steers.” Just a year after having major surgery, Hannah went on a school skiing trip to Colorado, aged 14. “I was with my friends and I was able to travel and ski with a guide. It was great for my confidence.”
Art as an expression of feelings
Hannah loves to paint and uses art as an expression of her feelings and emotions.
“All of my life, I have used painting and drawing to express myself. When I was in hospital I would draw pictures to show where the pain was. I would paint to distract myself from the discomfort of coming off morphine and when I was unable to do the things I love, like sailing. Since I have started to lose my eyesight, I use a lot of texture and colour in my paintings. Rough textures and the colour red express pain, while smooth textures convey happiness.”
Hannah’s painting is of such a good standard that in 2010 she was accepted onto an art degree course at Plymouth College of Art. She has ambitions to train as an art therapist. “I love to help people. I’ve had so much help throughout my life, it would be great to give something back.” With amazing candour she adds: “Also, if I lose my sight completely, art therapy is a job I could still do if I’m blind.”
When her close friend Sacha, who also had Pfeiffer Syndrome, died at the age of 14, Hannah used painting as an outlet for her grief. “We were often in hospital together. I felt as though she was the one person who really understood my pain. It made me feel so alone when she died and I found the painting brought some relief.”
A loving family
During the most difficult times of her life, Hannah has relied on the support of her loving family – mum, Millie, dad, Mark and her brothers Joshua and Oliver, and sister Lamorna. At a young age, she also became an integral part of the Hewlett family. Phil and Wendy Hewlett stepped in to provide respite care for Hannah’s family from the age of three. Their daughter, Beth, is now like a sister to Hannah. “They have been a big part of my life. Wendy and Phil gave my mum and dad a break from caring for me and they gave me someone to talk to who wasn’t immediate family. I feel as though I slipped into their family as if I’d always known them.”
A guide dog named Bella
By the age of 16, Hannah’s eyesight was starting to fail and she was given a guide dog named Bella. “It made me a lot more independent, I didn’t have to rely on other people any more. And, it was lovely to have a friend. Bella’s really good at keeping secrets.” During a particularly difficult spell in hospital, when Hannah was blindfolded to prevent the light from hurting her eyes, her mum brought Bella to visit her to cheer her up. “She put my hand onto Bella’s back. It was such a wonderful surprise.” This was to prove the turning point in that particular episode of care.
Life is too short to sit in a chair
Living with a condition like Pfeiffer Syndrome has given Hannah a determination to really live her life.
“I want to live life to the full rather than just surviving. Life is too short to sit in a chair. I started doing sport when I was 12. I used to get frustrated that I was ill all the time and it was a good outlet for my anger. And, I love to make people laugh – it is such a powerful medicine.”
Her advice to anyone facing major challenges in their life? “Don’t think too far ahead. Take each day as it comes. Be there for your friends. And, above all, get on with living your life.”
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