26/02/2021

Living With Marfan Syndrome | Our Stories

Over the past 9 years I've spoken a lot about my personal experience of having Marfan Syndrome, a rare genetic connective tissue disorder, both on my blog and in the media. Raising awareness of the condition has always been a long term goal of mine, and whilst sharing my journey is definitely doing that, at the end of the day I am just one person with one experience. As I so often say, Marfan is a spectrum condition and even within families the manifestation of it can differ. So today, to mark Marfan awareness month I'm teaming up with Sarah, who also has Marfan, to show how differently the condition can present itself in two individuals. If you want to grasp a basic understanding of what Marfan Syndrome is first then head to my information page. 

My name is Sarah, I'm 31 and from Swansea, South Wales. I met Shona through social media a few years ago, and it's been fascinating (and somewhat reassuring) to follow her journey as a fellow creative young woman navigating life with Marfan Syndrome. Our experiences are similar in some ways, but very different in others.


DIAGNOSIS

Diagnosis for me came at a young age and I accredit my resilience and knowledge to being one of the lucky ones to be diagnosed early on. My mum was aware she had Marfan when she was pregnant with me and suspected that I too had the condition. Officially I wasn't diagnosed until I was a toddler, when I started attending cardiology appointments at Great Ormond Street Children's Hospital. My family history mixed with my own symptoms meant that being diagnosed was straightforward for me. My mum later went on to have a genetic test which only solidified our diagnosis further.

For some people they will be the first in their family to have Marfan, this is called a spontaneous mutation, but in my family the condition can be tracked back through a couple of generations. As well as my mum having the condition, both her dad and brother had it too. Sadly they both passed away from aortic dissection at a young age, but thankfully science has moved on a lot since then and those with Marfan are now heavily monitored to catch problems before they reach this stage. - Shona

Marfan Syndrome, like most other chronic conditions, presents itself very differently in every affected individual, even those from the same family. I too was diagnosed from birth. Marfan affects the paternal side of my family, including my father and three of his four siblings. I had no formal gene analysis until 2018, long after Marfan Syndrome had begun irrevocably shaping my life in numerous ways. - Sarah

SIGNS, SYMPTOMS AND SURGERIES 

For me it's been the spinal and skeletal conditions that have affected me the most. I've had spinal fusion surgery to correct Scoliosis, the curvature of the spine, as well as a second revision surgery due to a complication and a total right hip replacement. My left hip also needs replacing and I have a large Tarlov Cyst at the bottom of my spine, sometimes also called Dural Ectasia in someone with Marfan. All of this combined means I use a powerchair full time outside of my home, standing and walking is incredibly painful and when it comes to sitting up I'm also limited in how long I can manage before needing to lie down or stretch to relieve my pain. I live with chronic pain every day. It's only been this year that I've finally been told I now need major open heart surgery to repair my aortic aneurysm, up until now my heart had been very stable. I thought heart surgery would perhaps come in my 30s or 40s due to how unchanged my aorta had been, but I'd rather operate sooner rather than later. - Shona

I grew up in London and never questioned my frequent hospital trips to monitor my eye health and cardiovascular function. I assumed, as children often do, that my 'normal' was the same as everyone else's. When I was seven, my family moved to Wales and it was clear by this point that I struggled to see. In school, I'd need work from the blackboard to be written down on paper to copy, and I would hold that paper half an inch from my face. This was symptomatic of lens dislocation, a common problem among Marfan patients which leads to chronic near-sightedness. I was painfully embarrassed of my struggle and took to extreme lengths to hide it - often memorising lengthy passages of text and sight test letters to reassure those around me that I was coping. Eventually, I could not conceal my blindness any longer and in 2002 I had my first ever surgery to fully remove the lenses from both eyes. I would need very strong prescription glasses or contact lenses for the rest of my life, but the quality of my sight was improved to an incredible extent. For the first time, I could see stars in the night sky. A poignant moment, even now,

The following year, my family and I were informed that my aortic root had expanded to a significant measurement of 5cm and I required open heart surgery at Great Ormond Street Hospital to replace the enlarged section with a synthetic sleeve to reduce the risk of rupture. My aortic valve would also need replacing with a metal alternative, accompanied by a lifelong anticoagulation therapy. Fortunately, as a resilient, but typical teenager (with requisite FOMO) my first open heart surgery went very well, and I returned to school in just 10 days with no issue.

I went on to have a very average adolescence; I went to college, then university, partied hard and worked part time in retail from the age of 16 (albeit, with a few extra hospital trips, a loud ticking heartbeat and regular INR checks than my peers). However, in November 2011, I was unexpectedly, and very forcefully, reminded of my chronic condition and the catastrophic problems that can come of it.

On a Thursday evening I experienced sudden and excruciating back pain, and a panicked trip to A&E confirmed that my descending aorta had dissected, and the situation was life threatening. We medically managed things until 2013 when I underwent a second open heart surgery to control a subsequent aneurysm, I was in hospital for a month following complications. In 2016, a third aneurysm required surgery, I underwent a 12 hour frozen elephant trunk procedure and during this surgery I suffered a stroke and remained in an induced coma for several days. Fortunately there was no long term damage and I recovered a few months later. - Sarah


WORKING WITH MARFAN

My health went downhill when I was 16, after my first surgery, which meant I had to drop out of school a couple of months into my A Levels and I wasn't able to work. Thankfully, I had my blog to keep me busy and I feel incredibly lucky that it's led me to a career I can mostly do from home. Working traditional hours in a job outside my home just isn't possible for me, so being self employed gives me the control I need to only work when I'm physically able to. - Shona

I have worked since I was 16 and been lucky enough to have understanding employers and the ability to work from home on the more difficult days. Like Shona, I suffer from Dural Ectasia, which can sometimes cause debilitating pain and discomfort, leading to some days being easier than others, but generally I live a 'normal' life. In 2018 I began a new job, working in Government Communications. Five days in, I checked out a few strange symptoms including an increasing blind spot in my right eye and soon found out I required another retinal detachment surgery, 16 years after the first. The surgery was uncomfortable to say the least, but within a few weeks I was once again back at work. I can't pretend it's easy balancing full-time work with complicated health problems, but I enjoy flying the Marfan flag, educating others and proving a chronic diagnosis isn't necessarily the end of a person's chosen career. - Sarah

THE FUTURE

When you have a condition like Marfan I think you really do have to take things one day at a time, but like everyone else I have plans for the future. I'd love to be making enough money from my freelance writing and photography for it to give me a full time wage, and alongside that I want to continue using my platform to raise awareness of Marfan Syndrome and disability issues. I started speaking about my condition on my blog so other's wouldn't feel alone, and to raise awareness, and that's something I never want to stop doing. But, as proven to me this year, I never know what's around the corner when it comes my health, so it's one step at a time! - Shona

I currently have another aneurysm slowly growing in my abdomen, which is being regularly monitored by my brilliant medical team. I expect to have a forth major surgery in the next few years, but plan to achieve plenty of personal goals before and after. For me, Marfan has primarily affected my eyes, heart and blood vessels. My body has undergone major trauma and invasive surgery. I am so proud of it's strength and resilience. Thank you, Shona, for sharing your platform and allowing me to tell my story this Marfan awareness month! - Sarah

You can learn more about the signs of Marfan Syndrome on the Marfan Foundation website.

19/02/2021

Having A Stammer Is A Daily Struggle, But It Isn't A Weakness

This piece is part of a series of paid commissioned opinion pieces by Disabled writers. Disabled people are constantly asked to work for free, to give their opinion for free and to educate people for free, and so I have created this space to not only give Disabled writers the opportunity to write and speak, but to gain income as well. You can expect to hear from a wide variety of Disabled, Deaf and hard of hearing people, Neurodivergent people and those living with chronic and mental illness. This is entirely fundraised for, so if you can spare anything at all to help fund this then I would be so grateful if you donated to the PayPal Pool.

Having a stammer is a daily struggle, but it isn't a weakness

I know it's there. Of course I do, it's my name - who doesn't know their name? Me; I literally, literally look like I don't know my name. "What's your name?" does not have to be a complex question , but I know that it won't come out. The response is stubbornly lodged somewhere in my throat. 'K' has always been a problematic letter for my stammer. 

In theory, I am not alone in stammering, a condition which affects over 70 million people worldwide. It comes in the form of blockages, repeating words and prolonging sounds. To counter this, I rush words. I avoid words. I dread introductions. It is difficult to shake off the lingering shame of being unable to say a word that defines your identity. 

After 12 years of having a stammer, I have heard lots about awareness and techniques, but little about the isolation that accompanies having a stammer; it isolates you from a crowd because you don't know how to introduce yourself; it isolates you from others because it is a sign of difference; the isolation of not knowing anybody who struggles with the same thing.

I have become a hyper-speed thesaurus. Almost every type of interaction is rehearsed endlessly beforehand. I am acutely aware of holding up the other person when I stammer, which in turns makes me feel like a barrier as they choose to finish my sentence for me. While some may appreciate somebody stepping in to help, finishing somebody's sentences because you don't want to have your time wasted is a failure of empathy. Nobody should have to think of themselves as an obstacle or irritation for a condition they cannot help.

Stammering is often situational, amplified by pre-existing fears and the reactions of listeners. There is an unspoken societal need to be 'fluent' - it's in interviews, framed as being able to communicate clearly and fluently. I can see why a stammer crushes ambitions, with it being a condition widely attributed to weakness. Stammering is still a heavily misunderstood and somewhat stigmatised condition, often used in films to evoke cruel laughs.

Seeing Joe Biden as President fills me with pride to see somebody who can both understand and emphasise with the difficulties of communicating in a position of power. Nevertheless, I still find it difficult to see him congratulated for "finding his voice", as though he didn't already have one. As a person of colour with a stammer, it was somewhat empowering to see Amanda Gorman, who struggled with a speech impediment, not a stammer, recite her poem. Knowing nobody else who stammers, especially a person of colour, always felt particularly isolating.

A stammer is continually framed as a "defect", something we "suffer" from when in reality, we "suffer" more from the reactions and attitudes of others on a daily basis than the stammer itself. It is difficult for people to place themselves in the position of someone who has spent years trying to talk, to be listened to, only to have sentences cut short and people snigger as you order a coffee. I could copyright the amount of times I've been told to "take a deep breath... and think about what you're going to say, before you say it". Trust me, I know exactly what I want to say. I find myself somewhat irritated by the number of people who are under the impression that watching the King's Speech or Educating Yorkshire makes them a fully qualified speech therapist. It doesn't. 

The uncomfortable reality is that many stammers don't align with the traditional narrative of 'suffer' stammer in childhood, have therapy, which is often very expensive, and overcome stammer. There is no shame in not overcoming a stammer. There is strength in dealing with it every day. 

I am tired of being told that it is something I can 'overcome', which implies it is something akin to an illness, something that can be beaten. It is something I have learned to manage, every single day. Some days are better than others. Times where it is easier, times where I've either slept a little more or am generally calmer. 

There are still triggers. Telephone conversations. Zoom. Fatigue. Stress. Pandemic life, it would seem. Previously, I relied on eye contact during conversations to maintain some fluency - seeing people helps - but now there is nobody to see. There is Zoom, but looking at myself stammering does not do wonders for both my stammer and my self-esteem. 

When what's normal for you is seen as a weakness, it takes courage to be who you are. I am slowly learning to cultivate an attitude of indifference, to find respite through writing. Having a stammer is a daily struggle, but it isn't a weakness.

Kimi Chaddah (she/her) is a politics, philosophy and economics student at Durham university and a freelance writer. She frequently writes on politics, mental health, education and identity. Follow Kimi on Twitter. 

16/02/2021

My Curly Hair Journey

As a child my ginger curls gained me the nickname 'Annie' but at some point during my life the curls just disappeared. Or so I thought. Turns out, I just had no idea how to manage my curly hair and I'd been brushing it furiously in an attempt to tame the frizz and thickness, which had only made matters worse. It took the curly girl method, a set of steps to achieve your best curls, going viral on TikTok for me to wonder, could I get back to those ginger ringlets? 

Each time I've posted about my curly hair journey I've received so many questions from others who are interested in trying to revive their curls too so I wanted to share my experience so far for those who want to get started as well. 

My hair pre-CGM | After the 1st wash | After 2 weeks | After 3 months

The curly girl method is a set of guidelines for how to manage curly hair including things like using products without any silicones or sulphates, only brushing your hair when it's wet and washing less and conditioning more. It seems like a lot to get your head around at first but I'm almost 4 months in now and it's already second nature. I haven't followed the method to the letter, more using it as a guide than a bible, and there's certain elements that I want to try in the future, but I'm taking things slow. For example, I would like to try and stop using shampoo eventually, trialling a co-wash instead, to see if this improves my curl definition.

For years I complained about my thick, untameable hair, but I quickly realised just a few weeks into doing the curly girl method that in fact my hair was only acting that way because I was constantly brushing it when it was dry. I was brushing out my curls everyday creating frizz and leaving me with only two options: tie it up or spend an hour straightening it. In fact, I used to frequently spend an hour straightening and then curling my hair to create the curls that I now know I can achieve naturally. Sigh. 

My hair as a child

One of the first things I did was switch out my shampoo and conditioner for CGM approved ones, and immediately I noticed a difference. I truly had no idea how dry my hair was until I switched. Right now I'm using the OGX Damage Remedy+ Coconut Oil Miracle shampoo and conditioner, but as I said previously I would like to transition to a co-wash and leave the days of shampoo behind. For now though I find these give my hair the moisture it needs whilst keeping my curls soft and smooth. 

When washing my hair I'll brush through it when I have the conditioner in with a wide toothed comb. When my hair is dry I'll run my fingers through it to break up any knots, but I never brush my hair dry now, it's really not a good look if I do! 

Once I'm out the shower I'll scrunch my hair to remove any excess water with a t-shirt, rather than a towel, to avoid frizz and then apply the Umberto Giannini Creme de Curl Control Cream, which again I find helps with the frizz. I'll then 'plop' my hair, a technique where you use a t-shirt to wrap your hair up to keep your curls defined, whilst I do my skincare. After that it's time for the hair gel!

Right now I'm using the Eco Style Olive Oil Hair Gel and I'm loving it! I section off my hair and use a Denman brush to get any last tangles out and then apply the gel, scrunching as I go. Now, this next step really depends on my energy and pain levels but sometimes I'll give my curls a little help by using the Denman brush to twirl them into ringlets. Most of the time however, just using the gel is enough to help them hold their shape. My hair goes back into the t-shirt after this for as long as I can tolerate it, often the weight on top of my head gets too much for my neck to handle after a while. 

I did buy a diffuser for my hair dryer at the start of the curly hair journey, but I rarely use it if I'm honest! It can take a long time to dry my whole head of hair and I really struggle with how long you need to hold the hair dryer in one position for, so I tend to just let my hair air dry. I get more frizz this way but it saves me a lot of pain and energy! To combat that frizz and refresh my curls in the days after I've washed my hair I use the Cantu Coconut Oil Shine & Hold Mist which gives me moisture and shine. 

I only wash my hair once or twice a week right now, often wearing my curls down for around 2/3 days after washing and then I'll wear it in a ponytail until I wash it again. At night I sleep with it in a really high ponytail, I should probably invest in a silk pillow case to combat frizz but right now this is working for me. I also try and use a hair mask once a week, right now I'm loving the Garnier Ultimate Blends Hair Food in coconut, it was recommended by so many people and I can strongly recommend it too!

At first the curly girl method seemed like a lot of extra work, but my hair is so much easier to manage now. I am so glad that the days of needing to straighten my hair for an hour to wear it down are gone. I can wake up, refresh my curls in a couple of minutes and go. I can't remember the last time in my life when wearing my hair down took such little time. I feel like I'm finally falling in love with my hair now.

I'm only 4 months into this process so I know I still have a long way to go, but I'm excited to see how much more curl definition I can get! 

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