When I think about the big things that have shaped my life, they have been things that have blessed me yet also greatly challenged me – giving me the opportunity to grow and realise my value, inner strength, and gifts. With age and wisdom, I acknowledge these blessings and I’m thankful for them.

When I was younger, I didn’t appreciate how life’s hurdles were necessary factors in my personal development journey. Life isn’t easy for many people and especially not for youth as they navigate their place in the world. But all of us, regardless of age, can choose to find meaning in tough times.

You may be surprised – but genetics is the big thing that’s shaped me… literary. Inheritance. My body, for all the disdain I’ve heaped upon it, has been my great teacher. From my dad, I inherited a chronic disease called Marfan syndrome which has taken me on the wildest ride of self-discovery!

My dad most likely inherited the disease from his father, which is fascinating to me, because all our lives we’ve wondered about this enigmatic man who so tragically died during World War Two. Ivan was in his forties when he was executed and my dad was seven. It’s said that dad looked like him.

We can’t be sure as no photos of my grandfather exist – they were destroyed by fire. Yet, he gifted both dad and me with his defective gene and distinct features. In this way, though both men have died, I feel intimately connected to them. (A theme I’ve explored in Where The Light Lives.)

*Please note – Spontaneous mutation accounts for at least 25% of Marfan syndrome cases.

 

 

February is Marfan Awareness Month

Hearing about how Marfan syndrome has impacted me may help you or someone you love or know. It’s a disease that can show signs at birth or during childhood or teenage years, or later. Sometimes, it doesn’t make itself known until it becomes life-threatening – in the case of aortic dissection. 

Interestingly, I wrote Where The Light Lives not knowing I had Marfan syndrome. I even say in it that I don’t have it (my dad had it). I’ve thought about updating the book to reflect my current diagnosis but then I like that it reflects a time in my life when I actually thought I was Marfan-free.

 

“Where The Light Lives is a beautifully written account of a young woman’s extraordinary visionary experiences. Diagnosed with a painful and humiliating disease of the spine, Linda Cull wanted to die. Without even nearly dying, she then had a series of encounters with a dazzling light-being that transformed her life. Similar to the classic near-death experience, Linda’s sense of being flooded with lessons from a transcendent source awakened her artistic imagination, which launched her on a career as a brilliant painter. Her book is a document of hope to all and should expand the horizons of human psychology.” 

-Michael Grosso

 

Marfan syndrome is a connective tissue disorder, affecting 1 in 5000 people. Connective tissue holds all the body’s cells, bones, muscles, tissues, and organs together. In a non-Marfanic body connective tissue acts like “super glue” (strong binder). In a Marfanic body connective tissue acts like “Clag glue” (weak binder).

Everyone has a fibrillin-1 protein, which makes elastic fibers in connective tissue. However, people with Marfan syndrome inherit a gene mutation in the fibrillin-1 gene on chromosome 15 that changes how the body uses fibrillin-1, leading to excess growth which causes:

• Tissues in blood vessels, the heart, ligaments, tendons, and cartilage to stretch more, making them weak
• Overgrowth of bones, making them longer than usual

 

 

Symptoms of Marfan syndrome (MFS) can be mild or severe:

• Tall, thin builds
• Long, narrow face
• Long arms, legs, fingers, and toes
• Curved spine
• Protruding or indented sternum
• Flexible joints
• Flat feet
• Stretch marks
• Etc.

MFS often causes pain and disfigurement. At worst, it affects the heart, aorta, lungs, and eyes – my dad, for instance, had multiple heart surgeries and an aortic dissection (which he survived!).

I haven’t had heart problems, but I’ve had spinal fusion along the whole length of my spine (titanium rods & screws). Where do I fit in the spectrum of MFS symptoms? Maybe somewhere in the middle. Since fourteen, I’ve had acute scoliosis which has progressively become more painful.

 

My Marfan story

When I was a child, in the 1980s, I didn’t notice any symptoms of Marfans aside from being very tall for my age and gender. I was always the tallest kid in my class and I hated it. It affected my self-esteem badly. I was very self-conscious about sticking out and all I wanted was to blend in.

I wanted to feel pretty, girlie, and likable and I never really did. To make matters worse, my mother had my hair cut short to thicken it, which made me feel even uglier! Sometimes children even asked me if my parents fed me enough because I was very thin – if only they knew! I was fed galore!!

High school elevated me to an extreme level of awkwardness. I suffered a lot of embarrassment – I was 6 feet tall by fourteen and very gangly. For my first three years there I was bullied daily, not by my peers but by older students – by girls calling out Anna (for anorexia) and Snap as I walked by.

Even though I was diagnosed with scoliosis at thirteen-ish by my GP during a health visit (when I had a virus), I didn’t notice my disfigurement until sixteen – I was devastated. For two years, lots of doctors looked at me and touched me as they assessed me for Marfan syndrome and scoliosis.

 

 

At this time, I obsessed over my height and regularly measured myself with dad’s industrial tape measure. At sixteen, I stopped growing – I was 6 foot 2 inches & a half. The half mattered to me very much! I felt freakish. Strangers stared at me and stores never stocked long-girl jeans!

The doctors told me I didn’t have MFS (there wasn’t genetic testing for it then). Apart from my crooked and twisted spine, I was healthy. There wasn’t anything they could do to help my scoliosis. By upper school, I was in severe mental pain and contemplating suicide yet still made A grades.

My life was in ruins. On other days, life was OK…I carried on. I rode the waves of moods – up and down I went for years. The best thing to come out of an idiopathic scoliosis diagnosis was I got out of sports. I hated sports. Instead, I spent the extra time doing art, which was a great release.

In Year 12, I painted a big, depressing painting for my final art project and it kind of saved my life. I cried a lot. I prayed a lot. I wrote sad stuff in my diary. All of this was in my private time. No one knew how difficult life felt. That’s why it’s so important to be with our teenagers and to know them.

 

 

Fast forward to my forties… I’d graduated from university, travelled the world, and snagged the best guy on the planet. We got married and had beautiful babies together – it’s all in my book! With pregnancies and age, my scoliosis worsened and I began to lose height so I considered surgery…

Our eldest son grew very quickly. When he was ten, my dad’s health declined, and his heart surgeon urged my parents to inform me to get DNA tested for Marfan’s syndrome (which became available in the 1990s – after my high school stint). I wasn’t concerned, but I wondered about my son.

As strange as it now seems, I didn’t understand the link between my dad’s health issues and the disease because it had long been explained to me, by my parents, that these were due to the hardships he’d endured as a child, living in war. Marfan syndrome – the term, wasn’t discussed.

I began to look into Marfan syndrome online. I couldn’t help but notice that we all shared Marfanic features. My concern grew for my son. I then discovered he couldn’t have the condition if I didn’t have it and DNA testing was the best way to find out. We were referred to Genetics Services of WA.

 

 

Dad was DNA tested first and the FBN1 gene mutation was discovered in him. From that, the geneticists more easily found the mutation in me. There and then my life changed in a very big way. Fear set in, not for myself but for my children. There was a fifty percent chance they could have it.

Talk about anxiety! Amazing how a person can live forty years of her life and be unconcerned by Marfans and as soon as she learns what it could mean for her children, she goes into full-on catastrophizing. Fortunately, my children don’t have Marfan syndrome. My sister doesn’t either.

Then, my own fear set in and I couldn’t sleep for months thinking my aorta might split at any moment – talk about stress. Eventually, I rationalised that we could all drop dead any moment now. All 8 billion of us that are on this planet. So it’s time to get on with living. And here I am.

I’m looking toward the future with happy thoughts. Big thoughts. Creative thoughts. I feel I have so much yet to give in this Marfanic body which I love and I’m deeply grateful for. Yes, I love my body! (Thanks, Claire) that birthed two beautiful children and enables me to experience life, every day.

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Awareness of Marfan syndrome leads to early diagnosis, treatment, and an extended lifespan. People with Marfan syndrome are usually tall and thin (but not always) so if you know someone with a lanky build, please talk to them about the disease, or if they’re young talk to their parents.

PS. For more info visit The Marfan Foundation

PPS. Happy Marfan Awareness Month!