Moyamoya: Emily’s Story

Interviewed by Ben Harden

The unexpected happened in 2005– when Emily suddenly developed symptoms such as trouble forming words and loss of connection with her right hand.

Emily Jones or Emily Pip as she is also known had no previous health issues that could have warned her about an impending problem.

She was only 23 years old, living in Brisbane and after almost exactly one month of symptoms including minor TIAs, major strokes, and several brain bleeds, Emily would finally learn of her life-changing diagnosis after a month of uncertainty.

Do you remember the day you first started seeing symptoms?
“My first ever TIA or small stroke was on September 9th, 2005, I remember it clearly: I was in Coles Supermarket and I dropped my shopping,” Emily said.

“I tried to pick it back up again but couldn’t connect to be right arm or hand at all – it felt like it kept going “through” the items and eventually I used my left hand and got through the checkout.

“I then continued on my way,down to the park to meet a friend. I went to great him and had another episode: I couldn’t speak – my words came out as jumbled gibberish.

Emily recalls her first ever brain bleed as if it were only yesterday.

“A few days after those first episodes, I was asleep and woke up in the middle of the night with the most horrible, intense pain and pressure in my head,” she said.

“I remember lying there, holding my head, trying to keep everything in there together, thinking – and thoughts were very hard to formulate – that maybe if I cut all my hair off, the pressure would release and the pain would go away.

“I tried to text a friend to say I needed help but I couldn’t work out my phone.

“Over the next few weeks the symptoms increased in severity and frequency.

“Most of my symptoms were either right side body(weakness/numbness/paralysis) or speech difficulties, often accompanied by a ‘brain fog’ that would often last much longer than the other issues,” Emily said.

After more symptoms started to appear and continuous loss of feeling in parts of Emily’s body, she finally went and saw her GP in National Stroke Week.

However, with no clear solution, she decided after another severe episode at work that she would visit the Royal Melbourne Hospital Emergency Department.

What was it like when you were diagnosed with Moyamoya disease?
“I was in the RMH for 2 weeks, on a heparin infusion(anticoagulant), while they decided whether or not, and how much, to believe me,” Emily said.

“Some friends had to come and get my car which I’d left in a carpark across the road.

“I remember being present for MDT meetings where I was discussed, and the language: “claims” this… “denies” that… was incredibly offensive!

“Thankfully, a few doctors were able to witness my symptoms, in particular a dysphasia episode where I experienced extreme word-finding difficulties.

“I became available for an MRI scan and they thought they’d slot me in as a last-ditch effort.

“The MRI report wasn’t even done when a doctor literally ran up the stairs to my room to stop me from leaving.

“He said that the scan showed several strokes and multiple TIAs that had occurred within the last 2 weeks – which was about as good as the technology was back then,” Emily said.

Back on the heparin infusion and for more investigations, Emily remembers the first cerebral angiogram she had.

“This was, the most traumatic experience of my life (I’ve since had another one which wasn’t a big deal at all!),” she said.

“That first time, I had tubes and lines everywhere, they were struggling to get the images right and my head was strapped down to the table and it all took hours longer that they said it would.

“Moyamoya diagnosis was immediate, and surgery discussions commenced due to both of my Internal Carotid Arteries being almost completely occluded, and the obvious “puff of smoke” produced by the collateral vessel formation.

“Thank goodness, a very experienced Mr John Laidlaw, heard of my case and intervened, offering to do the bilateral direct bypasses I needed asap.

“I had the left side done on November 17, 2005 and the right side on April 13, 2006. “Each surgery was approximately 1 week in hospital and sometime after with a “No Pressure” bandage on my head.

“Mid-way through 2011, I had my second cerebral angiogram; I chose to fly to Melbourne for this. As previously mentioned, this second experience was not remotely traumatic.

“It was just me and the clinician and they setup the screens so I could watch and it was fascinating!” Emily said.

Emily says she has some ongoing issues with her left eye, which results in her losing vision intermittently due to a lack of blood flow resulting from my Moyamoya.

“Fludrocortisone, and a love of handstands, helps to manage this and symptoms are rare these days,” she said.

“I do believe that the amount of time I have spent upside down and flying around in the air has resulted in very strong blood vessels throughout my brain and attributed to my ability to adapt and succeed.

“These days I work full-time as a clinical nurse and feel that my cognition (finally) is just as sharp as it ever was,” Emily said.

Emily says the nurses and medical staff at the Royal Melbourne Hospital were wonderful and remembers that all 14 of them in there with me were female.

How has the Moyamoya disease affected you and your family?
“I can mostly only speak of how it has affected me; however, I think my words will be relevant for all of my immediate family,” she said.

“Obviously, initially, it was incredibly scary and unknown.

“But I have never sustained any true anger or resentment about this journey.

“I have learned so much,about life, and about my own resilience.

“It has made many things in life seem so very easy, many other things seem quite petty and it has showed me to value life, every single moment of it,” she said.

Emily says her Moyamoya diagnosis has showed her not put things off for the future and her passion for travel and adventure.

“I have trained and performed in the circus, I have travelled as far as Chile and Montreal to study with the best in the business,” she said.

“I have sailed across the Pacific Ocean as one of only 4 crew on a yacht, from the Galapagos Islands to French Polynesia.

“I’ve had many careers including being a Yoga Teacher and worked in Music Management.

“Within nursing I have specialised in Neurosurgery, Vascular Surgery, Diabetes and now Palliative Care (I have post graduate qualifications in the latter two).

“I have travelled to many places in the world and continue to so do, the next on the list is Samoa.

“In the past year I became a Paragliding Pilot and fly, solo, over mountains and coastlines with the view of a bird, (aerials in the circus obviously wasn’t quite high enough!).

“I continue to seek knowledge, to improve my physical skills and abilities and to plan new and exciting adventures.

“As a nurse, it has made me the best advocate for my patients, as I truly understand what it is like to be on that side.

“I will not stand for anything but the absolute best care, provided with respect and without judgement,” Emily said.

Why is it important to have more stories of your own out there for the public to understand more about the Moyamoya disease?
“Awareness is the key to diagnosis, and fortunately the incidence of this is improving, but Queensland still has a long way to go compared to other states,” Emily said.

“There are still no surgeons in QLD competent and confident in attending to a Direct bypass surgery- actually, one popped up late last year at the Royal Brisbane and Women’s Hospital, claimed to be trained in the UK, however, I haven’t heard anymore since,” she said.

“As well as the medical diagnosis, there needs to be more stories available about life “after”Moyamoya. Emily believes it is important to her that she is not labelled as a victim or as others say battling or fighting the Moyamoya disease and that she wouldn’t consider herself a Moyamoya warrior.

“That is definitely not a way that I consider my situation to be,” she said.

“As is obvious in my story and the continuing niggles I experience, there is no “after”: Moyamoya is progressive and doesn’t go away, but how much does it have to affect the everyday?

“Like any hardships or challenges, getting through it absolutely makes you stronger,” she said.