Friday, 15 January 2016

PHighter Friday: Tegan

First international trip to Japan 2010
My PH story starts when I was six, sometime in 1995. I suddenly started having fainting episodes and was quickly diagnosed with Epilepsy, having uncontrolled Tonic-Clonic seizures. 

Within a year I developed a chest infection/cough, that wasn't going away with antibiotics. The local doctor sent me and mum down the road to have a chest x-ray. I don't remember specifics but I know that it landed me pretty quickly in the Royal Children's Hospital Melbourne on the Cardiac ward. Throwing my family in to chaos with my two younger sister being looked after by my grand-parent, while mum looked after me.

Being diagnosed with "Primary Pulmonary Hypertension" Functional class 4 (now known as Idiopathic Pulmonary Arterial Hypertension) was life changing to say the least. This diagnosis didn't just effect me emotionally and physically, but my entire family, my circle of friends, my school life, after school activities and general living. I was initally started on "Diltizem", and given a 5 year life expectancy. As a 6 year old I was blissfully unaware of this.

I managed to go to school full time, partake in sedate after school activities and play with my 2 sisters. If I forgot to take my medication, or refused (as i did in the beginning), I could feel the difference, and would often have a seizure as a result.

Around the 5 year mark I got really sick. I was unable to start high school, I was in and out of hospital trying to manage the symptoms. The addition of a few medications and eventually oxygen.

Hosptial stay 2001/02, recently started
 Epoprostenol and looking much better already
I tried "Beraprost" for a short time, but this made me feel sick three times a day for about thirty-sixty minutes after each dose. It made me feel isolated from my friends as I had to go to the school sick bay to have it during class, and rest until the side effects wore off. It also didn't make me feel much better. 

This period is very vague to me and everything sort of blends together. I know I had multiple seizures at school, ambulances were called on multiple occasion, late night trips into the city to get admitted quickly and always extra drugs and bloods and tests. Mum and I spent countless hours stuck in traffic on long stretches of road on the way to the hospital, we played many cars games, but often I was vomiting or simply too lethargic to play. 

Around this time I was placed on the transplant list waiting for a heart and lungs. I was too ill to go to school at this time, nothing was working. I made it through the year using a wheelchair, oxygen and many many medications. I was in heart failure and the rest of my organs were soon to follow, fluid retention, vomiting and weight loss among the symptoms. I was palliated, something i wasn't really aware of. New teams of medical professionals were seeing me all the time to help with one symptom or another, there was a constant stream of consults, reviews and medical rounds. I had privileges from hospital, and small windows at home.

Early 2001 I was given the green light to start this magical, mystical drug from the US (Epoprostenol) as well as oral "Bosentan" (plus my cocktail of "normal" meds). Conditions applied, a fair few. Mum had to be trained in making the medications, and had to commit to making it up from powder to liquid every single day. We had to be self caring with a PICC line (a semi-permeant line directly into the heart.) I had to do any test they requested (6 minute walks, ECHO's, ECH/EKG, bloods and Cardiac catheters). I was so unwell I was not yet able to have the permeant line, so I had a temporary one inserted with light sedation. 

Kakadu National Park 2014
The temporary line proved to last quite a while. By the time the 2nd one had packed it in, I was well enough to have a bit more sedation and get my permanent line. This was a pretty good indicator that this drug was working. I made leaps and bounds from here. I came off the transplant list, I was no long made palliative and I slowly started going to school. I had missed a few months of school and had to be slowly reintroduced. Luckily with a supportive school, and some great friends I got back into it.

The combination of drugs has changed a bit over the years,. I'm no longer in any organ failure, and I take a portion of the drugs I was taking. I take 2 medications for PH, Epoprostenol and Sildenafil. However, I do take a few a preventions of to make life a little bit easier with symptoms.

It took me a very long time to come to terms with my illness and accept it as part of my identity, but not something that will define me. I can thank the use of this drug for the life I lead now.

I work full time as a paediatric cancer nurse, I ride my bike 7kms each day to work and I have the travel bug. I have it in a big way. 

I still have to undertake the same tests, just much less frequently. I can't swim in the ocean/pool, I can never be too far from a hospital and I have to carry around a pump everywhere I go 24 hours, 7 days a week. BUT I will always try to do this with a smile on my face and a positive attitude!

I've been to over 20 different countries and can never wait to plan my next one. Of course I have limitations, but I'll take what I can get.

Repackaging 3 months worth of medication
for my upcoming holiday
My next trip is the biggest and the scariest and the absolute most exciting thing that I have been able to achieve since diagnosis. I have spent the last 6 months planning everything I might need for a 3 month holiday in Sweden and Europe. There is about 40 kg of supplies and 4 kgs of clothes.

This is a recent development as I switched to a more stable version of my drug. It means more freedom and less volume of medications (Thanks Veletri!). 

It has been a lot of preparation, research and challenges at every corner. I started to document my issues with travelling with a serious chronic illness and intend to continue while I travel.

I know I've had a hard life but I know its also been full of interesting moments, filled to the brim with love and most of all its been surprising. When you exceed the doctors expectations by 15+ years you learn to live life to the full.

There are so many people who help me with my challenges. I feel this is the key to my success.

Stonehenge England 2013

My blog can be found at https://www.tumblr.com/blog/tegamegadrive and my instagram at https://www.instagram.com/tegamegadrive/

7 comments:

  1. My sister was diagnosed with PPH in 2000. She was on Flolan and all the same drugs you are on. She underwent 2 double lung transplants . One in 2005 and the second one in 2009. Unfortunately, we lost her in 2011. She was a fighter. Watching her live with this disease was horrific, but, SHE NEVER COMPLAINED. She is and will always be my hero. I also lost my cousin to PPH in June 2015 and her younger sister was diagnosed this past November with PPH. God Bless and keep living:)

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    1. I am sorry to hear about your mother and cousins. It sounds like your mom was an incredible fighter. Sorry again about your loss. xo

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    1. Hi Samantha- if you contact Tegan on Tumblr or Instagram I bet she would be happy to answer any questions you may have. Are you a young adult with PH? If so, there is a Facebook group called "Generation Hope" full of young adults (I think adults from 18-40) who would be able to tell you more about traveling with a line. I hope that you'll be feeling better in no time and that you'll be going on exciting adventures soon.

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    2. Hi Serena, I do have PH. I was diagnosed in November. Thanks for letting me know about the Facebook group.đŸ˜„

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  3. Thank you so much for sharing your story. I am in hospital right now and have just had my line put in. There is a lot to think about with all the changes that have happened over the months since my diagnosis but i did worry a lot about traveling. It has been such a huge and important part of my life. I'm so glad to see that with some extra work you can make it happen. So grateful for you sharing your experience!

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    1. Hi Samantha,
      I've had my IV for nearly three years. Didn't get off to a great start and I'm on my fourth line already - the first three within 12 months. This had nothing to do with how I was looking after the site, but more down to the positioning of it in theatre and rejection. But I'm over all that now and things are going really well. I love that I can breath and do so much more than I could. It immediately alleviated the pains and discomfort I had in my chest and I no longer felt like 'my head was screwed on too tight!'
      I'd been on nebulised iloprost for 13 years, along with sildenafil. I knew I was deteriating and have always known that there would come a time when I would have to have a Hickman line fitted, so I took control and chose when to have it. I almost felt that I'd got the upper hand. I felt very lucky to be in that position, because normally patients don't have that luxury.
      I think I'm a little too old to join the 'Generation Hope' FB page, but I'd like to drop by and take a look. Maybe it's something we could do an article about in emphasis, the U.K. associations magazine.
      Good Luck with your line xx

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