Ornithine Transcarbamylase Deficiency

The story of Brooke

Hi there!

I never know where too start when writing, so I guess I’ll just start with saying over 6 years ago I wrote one of these, and I didn’t love the reality of how I wrote it back when I was in year 11 and quite naive to my disease, I also was approached to re-write it! So here we go, in 2021 I’m going to re-write this after some encouragement from the people around me!

My name is Brooke, I’m a 23 year old female from Victoria, Australia.
& most people would call me your average ‘basic’ young women… I would also consider myself quite basic and am told I never stop smiling, which im working on haha!!
I love my dog Loki, musical theatre, spending money on clothes, aerobics, netball, greys anatomy, mamma Mia, açai bowls, driving, and making other people happy! Im a huge introvert and love nothing more than watching trashy TV (MAFS anyone?) in my oodie.

Im pretty normal as the eye would see – but beneath I have a rare disease that absolutely every person who doesn’t have it struggles to pronounce (and to be quite honest I get a good giggle out of!)
Even when I go to the emergency room, I am the one explaining what to do! (Which isn’t easy in a confused state!)

I have Ornithine Transcarbamalayse Deficiency (OTC Deficiency).
Looking at that extremely long word, you’d get overwhelmed… and that’s completely valid, even after 23 years of reading its never exactly sunk in that I’m not like every other 23 year old..

OTC Deficiency is a genetic Metabolic Disease that causes Ammonia to accumulate in the bloodstream. Ammonia is a toxin and when levels are increased it causes symptoms and effects to the nervous system.

Symptoms of OTCD (flares) include
episodic delirium, headaches, nausea/vomiting, lethargy, confusion and if left to long seizures, coma and death.

This gene runs in my family, but unfortunately I seem to be the most ‘severe’.
My mum had her first child and around 5 days after birth, he passed away from the effects of an out of control raised ammonia caused from undiagnosed OTC Deficiency.
My mum then fell pregnant with me a few years later and had specific testing to see whether I had OTC and un-suprisingly, I did.

5 years later, my little sister Georgia was born and she was completely healthy.
Although my mum has the gene, she doesn’t have OTCD. Meaning, she is also healthy.
So, Its just me!

As a child, I grew up not knowing any different. Hospital appointments, metabolic speciliasts, medications, diets & blood tests were all pretty routine for me, and as a kid I think I could back myself and say I became a bit of an expert in all these things. I got to a point where I didn’t flinch getting a needle poked in me! (If only I could say the same now!)

Whether it was getting a cold or a flu, breaking bones and needing surgery (happened a lot, I was a clumsy kid haha) or eating just the wrong thing – I would get driven to the emergency department of the Royal Childrens Hospital, and was poked, prodded and had tubes shoved in my nose – all for a good reason.

In my teenage years (13-17) I absolutely hated the fact I wasn’t ‘normal’.
This was peak time of when everyone around me was doing whatever they wanted, and I felt like I was stuck having to be home on time to take my medication or make sure whenever I went out I didn’t eat the ‘wrong foods’ and I honestly just wanted to be a typical rowdy teenager (even as the little introvert I am). I started hiding all of my medication in my drawers, down the sides of couches, and in my pockets. I also stopped caring what I ate and decided to eat whatever I wanted. Whether it was meat, dairy, nuts etc.

During year 9, I went and ate something incredibly high protein from McDonald’s on the way home from a hospital admission (cue stupid decision! But what made it worse is I was deprived from protein and calories for days during the hospital so my body was extra sensitive to proteins!)

The following day, I collapsed at school. An ambulance was called and I was placed in an induced coma in the intensive care unit due to the incredibly high ammonia levels attacking my nervous system, and causing everything to shut down.
I was dying. – My metabolic team told my mum to prepare for the worst.
& to everyone’s surprise, a week or so later of being in an induced coma, getting haemodialysis, and being pumped full of medication – I woke up.

After waking up, I lost almost all of my strength. I couldn’t stand up, I couldn’t walk to the bathroom, I couldn’t shower. I lost my independence that I wanted to gain so badly, so really this little stint put me in the opposite direction of where I wanted to be.
I was mortifyingly embarrassed during this admission, as in my mind… I had put myself there. I was the one who disobeyed my treatment plan, but at that age I had no idea the effects it would’ve caused, I just wanted to look ‘cool’.
I scared myself, I don’t think I’ve ever been so scared in my life & 14 year old me only wanted control over my health after that point on.
So, I stopped eating. – In my brain I had convinced myself, If I stop eating protein and I take my medication – I would be healthy.
But again, I was wrong. All this lead to was being kept in hospital for 6 months rehabilitating not only physically but mentally as well. – it caused not only emotional change on myself but everyone around me as well.

That hospital admission in 2012 changed the way I saw my health to this day.
From that day onwards I knew I had to be the person who took charge of their own condition.
This meant taking my 40+ tablets a day, not pushing myself too hard in my sports, work etc, and following my strict diet.
I reached out to people on the internet from all around the world who have the same disease as me to make the realisation I’m not as alone as I felt! I started asking for help when I needed it (even if I still do struggle to do that!), and I joined a chronic illness program where I met some of the most inspiring and amazing people I’ve ever come across.

Writing this on the 25/02/2021, I can confirm I am definitely no expert in my disease.
Yes, I can explain it word for word, and even say it in a way that makes non-medical professionals understand.
But,  It is a constant battle of doing things I love, but making sure I eat enough protein, enough calories & sugar. – Also not letting anything stop me, I don’t like to limit myself and give myself boundaries on what I can Do nor do I let other people give me boundaries, making my life as ‘normal’ as possible (I’m insanely stubborn and I’m so sorry to all the people who have to tell me to look after myself haha!)

I still have to have hospital admissions, take more medication than I’ve ever had to take, follow a STRICT diet (to everyone reading this who knows how bad I am with this, I’m trying okay haha)

I guess you could say even though I do have a rare disease, I consider myself pretty grateful for life, even if I have hard days. I can drive, dance, work, study and eat! Im grateful for the people I’ve surrounded myself with, who only bring out the best in me and push me to achieve amazing things.

If I had any advice for someone younger than me who has OTCD or any kind of metabolic disease it would be….
don’t let anything get in your way of doing what you want to do even if that means making adjustments, take your medication, follow your treatment plan, and take rests when you know you need it.
Advocate for yourself because nobody knows how you’re feeling, only you do. Be your best self and make sure you are being heard by medical professionals, family, friends and reach out for help when you need it.

Heres to the next 20 years with OTC and hopefully less hospital admissions.

all my love, Brooke x