June 13, 2016

Emily’s Story

Kia ora,

I’m Emily and I live with Kartagener’s Syndrome and Bronchiectasis. Kartagener’s syndrome is a rare genetic syndrome that consists of two conditions, Primary ciliary dyskinesia (PCD) and Situs Inversus.

  • The first condition, PCD, affects the cilia (tiny, hair-like structures that line the airways). Normally, cilia move together in wave-like motions to clear mucus out of the respiratory system. With PCD, cilia don’t work as they should so mucus stays in the airways and results in frequent infections of the lungs, sinuses, and ears.
  • The second condition, Situs Inversus, is where all of the internal organs are on the opposite side of the body to normal (an exact mirror image). This does not cause any complications… I just use it to play tricks on the medical professionals!

As a result of having recurrent chest infections due to Kartagener’s syndrome, I have also developed bronchiectasis. Bronchiectasis is where the breathing tubes (bronchi) have been damaged, enlarged and scarred. This causes mucus to build up in the extra space, and is a breeding ground for bugs like pseudomonas to colonise.

– Treatment –

To keep sinus, ear, and lung infections to a minimum I do daily nebulisers, chest physio, sinus washouts, exercise, and ensure I eat and sleep well. Due to stubborn chest infections, I also require regular IV antibiotics. See the photos below for more of an insight!

The medical breakthroughs I’d wish for are a cure for PCD, and antibiotics that could eradicate colonised bugs such as pseudomonas.

– Impact –

Day to day, my condition can best be compared to having a chronic cold (not that I know anything else!). While sinus headaches, blocked ears and a cough are the ‘norm’ for me, these symptoms can be energy zapping, and, at times, self-esteem and confidence zapping too!

The past year has seen me need IV antibiotics more frequently. Disliking any attention and/or sympathy when unwell, I am grateful for the option to self-manage the IVs at home as it allows me to carry on with things as ‘normal’.

While my condition’s unpredictable nature can be frustrating (e.g. getting pneumonia 2 nights before a trip to Australia that I had to cancel), it is what drives me to make the most of everyday. Even when unwell, I try not to let my condition stop me from doing anything – even marking exam papers from a hospital bed when working as a tutor!

– Living life to the full –

Travel 

The travel bug is one bug I’m happy to have caught! I am fortunate to have done several trips around South East Asia, and, most recently, Europe!

Exercise

Reaping the respiratory related benefits of increased exercise, I now take the opportunity to exercise at any opportunity. Some of my favourite activities are mountain biking, kayaking, and walking in the Port Hills. I also now walk and bike everywhere (including to meetings offsite), and recently sold my car as it was not being used!

Work & Volunteering

I work at the CDHB as a health promoter for the All Right? Campaign. Having a career that supports my passion for helping people, accompanied with the continuous opportunities to learn and develop a wide skill set, means I find my work incredibly rewarding!

Grateful for the support & opportunities I’m privileged to have, I’m dedicated to helping others. Recent volunteer engagements include Bounce.org.nz (Youth Peer Educator), Camp Quality (companion) and the Student Volunteer Army.

Friends and Family

Friends and family are hugely important to me. I am grateful to my parents for never letting my condition stop me from doing anything, and for their continual support (even with the little things!). I love having a good laugh with friends and family and doing random things out of spontaneity. Fortunately, my boyfriend and I are on par when it comes to living life to the full, and he has the ability to know exactly what I need when I’m not feeling 100%. I am passionate about supporting and encouraging others, and am grateful that I can draw on the support of a positive network when I need it.

Open and honest, and getting on with it!

The hidden nature of my condition, coupled with just toughing things out, means that people don’t always understand how I might really be feeling because I “don’t look sick”. However, I have recently learnt the value of being open and honest with others about my condition. This has made it easier to draw on support when I need it, however getting on with things remains the easiest coping mechanism for me (as the below images illustrate)!

While I wouldn’t wish my condition on anyone, it has made me who I am. Knowing how quickly things can change empowers me to live in the moment, take opportunities, and do everything I can to keep well!

– 750km Running Challenge –

Better Out Than In is currently fundraising for the Asthma and Respiratory Foundation. For my fundraiser, I’m running a total of at least 750km in 100 days!

It is fair to say running 750km will be no easy feat for me. However, I am motivated to do so to not only support my lung health, but to

  • Fund the much needed research into respiratory conditions – it’s not fair that people try their best but nothing can eradicate bugs
  • Raise awareness of respiratory conditions and the value of exercise to keeping lungs healthy
  • Inspire people to use their lungs
  • Promote Better Out Than In – helping to connect, support, and empower people affected by chronic respiratory conditions

Click here to sponsor me in the 100 Day Challenge!