Tatiana Neuser-Bostel

A happy and healthy Tatiana

People often ask me how I manage to stay so positive despite the challenges I have faced. I thank my father for that trait, as he always said “You must know you can count on yourself because when there’s no one else around, it’s only you who can get you through.”

When I was born the doctors suspected that I had a hole-in-the-heart.  At the age of three I was hospitalised and a femoral catheterization was performed to see into my heart. It was then I was diagnosed with Truncus Arteriosus with Ventricular Septal Defect or VSD.  This means instead of the normal two blood vessels from the right and left ventricles of the heart there was only one. Plus my heart also had an abnormal opening in the septum, which is the wall that separates the two ventricles, which thus allowed blood to pass between the right and left ventricles.

I remember being in hospital at that age and my memories are of a happy experience. I had my birthday in hospital. In fact, I spent quite some time in Princess Margaret Hospital over the years. I would often get chest infections or bronchitis, which meant weeks off school in winter-time, but I didn’t experience many of the complications other heart kids had.

At the age of 18 an elderly lady-friend of the family told me some news I had never been aware of. She asked how old I was and when I replied she naively said “That’s lovely dear, -- they didn’t expect you to live past 17, did they?” That comment stopped me in my tracks, and alerted me to something my parents had protected me from for all those years. Of course Mum felt terrible that I had found out that way, however, I took the news in my stride. 

My parents were told my condition, as a young child, was inoperable. I believe my dad had my medical files sent to doctors in the USA and (the former) USSR hoping someone knew of a technique that would help. They all sent them back saying treatment for this condition was unknown territory.

Mum and Dad never wrapped me in cotton wool. I was always allowed to try different activities, which I believe was a real tribute to them both. They trusted me to know when I had physically reached my limit of exertion. Usually after just a few minutes I was ready to take a rest-stop and catch my breath. As a child at school I was often the one sitting on a bench reading a book, while the other kids were running and playing around.
I believe not pushing myself beyond my limit developed my strength and will power, rather than continuing on just to fit in. In fact, sometimes other people appreciated this about me because it meant we enjoyed the journey and what was around us, instead of rushing through the experience just to be able to say we had done it.

I was born and grew up in Perth with my older sister Anna. My dad worked for many years with the forestry department and then went back to university studies in his forties, gained a degree in economics and worked at the Water Corporation as an Economist. My mum gave up her job when she had children and became a full-time Mum.

I was seen by my paediatrician up to the age of 20 before being referred to a cardiologist at RPH, Dr Krishna Somers.  He is a wonderful physician. He arranged another catheter investigation to see if there had been any changes, but results showed the condition had remained the same. He did an amazing job with me in managing my condition and my medication.  When I was in my early 30’s, he wrote to a colleague at a university hospital in the UK who was a specialist in adult congenital heart defects, seeking advice about my case.  She wrote back saying he was doing a marvellous job keeping me alive in the stable state of health I was presently enjoying, and that she couldn’t suggest any other treatment protocol.

I knew from a young age having a child wasn’t a viable option because of my heart condition.  My life would have been in danger during pregnancy. Thus, when doctors suggested for me to have a tubular ligation in my early twenties, I chose my future survival over trying to have children of my own. I wasn’t particularly saddened at the prospect of not having children, and I guess over the years I had reasoned with myself that having a family was going to be difficult just from an energy perspective. The idea of adoption had crossed my mind, however the same dilemmas arose about the energy needed to care for and keep up with a child. So, I have enjoyed other people’s children over the years.

I was lucky enough that my health allowed me to travel quite extensively throughout my 20’s and 30’s.  It was around the time I turned 40 that such opportunities became limited.  My mum fretted when I travelled around the United States by myself when I was 22 years old. Mum has always been, and still is, my best friend. She has always been there, whether it was on the sideline just in case I needed help when I was younger, when she stayed in the house with me when my health declined before my transplant, and now when she keeps me company at the numerous appointments at RPH.

I met my wonderful husband, Stephan, while he and his father visited Perth in 1992. We are actually distantly related, and share the same birthday – the 29th of July.  We became friends and wrote to each other, exchanging birthday wishes. Three years later when I travelled to Europe for a holiday I caught up with Stephan again and this was when our relationship blossomed.

Stephan visited me in Australia for a holiday later that year. It was during this visit he realised the full extent of my condition when I had a Transient Ischemic Attack or TIA, which is a mini or warning stroke. I ended up in hospital for a short stay for tests and subsequently commenced a new drug.  I was given the all-clear and continued on as usual.  I enjoyed the few weeks with Stephan, and after farewelling him at the airport, found myself back in hospital soon after with a raging fever and all the symptoms of a major infection.  It turned out to be Golden Staph, which I believe I had contracted via the IV drip whilst in hospital earlier on. I had intended on moving to Germany within the following four months, but with the obvious health drama I could not think of going anywhere.

With that news Stephan said ‘Well, if you can’t come to Germany, I will come to Australia!’ and he picked up his life and came out here.  We married on the 27th of March 1997 and lived in Perth until late in 1999, then moved to Germany where we resided till 2003. At that stage we believed the moist climate in Europe caused my health to take a downhill turn, so we returned to Australia at the end of 2003.

From wear and tear over the years, I developed Eisenmenger Syndrome. This syndrome was caused by the untreated truncus arteriosus and VSD and is a specific form of pulmonary hypertension. Put simply, living was causing more and more pressure on my lungs.

It was this wear and tear that, in 2004, led my Cardiologist Dr Somers to suggest the possibility of transplantation.  My immediate reaction was ‘NO’. I just didn’t want to enter into it. The idea of being opened up like a lemon really put me off. But when you realise your health is on the decline, days are numbered, and with Stephan by my side and so much to live for, it became clearer and clearer that transplantation was the only option.

Stephan has been a huge, enormous support with all of this. He knew what he was getting himself into when we married and I don’t know of too many people who would have gone down that path. To his credit he has been very brave, very generous, compassionate and offering complete selflessness. I would be lost without him that is for sure.

I was first listed for transplantation in July 2005. A few weeks earlier I began swelling up with an overload of fluid, which was an indication that cardiac failure was increasing its impact on my well-being. This was the first of many hospitalisations for progressive heart failure over the ensuing two years.  I was treated with a diuretic to flush the fluid from my system, however, in that process I ended up with gout. Doctors treated the gout, and then gave me another drug to prevent further incidents.  It was this drug I had a severe allergic reaction to, known as Stevens-Johnson Syndrome. (SJS is a life-threatening condition affecting the skin, in which due to cell death the epidermis separates from the dermis.)  Given the bad state I was in, I was temporarily taken off the list.  I was re-listed in November 2005 when I worked my way back to semi-health.

In December 2005 I received a call from RPH about a potential donor but the routine tests prior to any transplant operation revealed my kidneys weren’t functioning appropriately, so the transplant could not go ahead. I wasn’t in a particularly good way at that point because the SJS had knocked the wind out of my sails, and in some ways I am grateful I wasn’t operated on. Knowing now what I didn’t know then, I think the recovery process at that time would have just been too much.

After further tests doctors felt my kidneys, and possibly liver, weren’t up to scratch. At the end of 2005 RPH Cardiologist, Dr Laurie Dembo came to me with the bad news that they could be looking at a triple-organ transplant. Heart, lungs and kidney – I just thought, “No way!”

Because of the TIA in 1995, I was currently taking Warfarin.  It is a tricky drug for many reasons, but I needed it to thin my blood, which was thick, and gluggy due to a greater amount of red blood cells to compensate for low oxygen levels. However, for a transplant operation my blood had to be reverted to normal viscosity.  Unfortunately, tests showed this was proving to be a difficult process due to liver impairment.

From what I understand, the problems with my liver and kidneys placed me into the “too hard” category. The head of the Lung Transplant Unit at the time, Dr Eli Gabbay carried out numerous tests and looked at different perspectives, but in January 2006 it was decided by the various teams of medical professionals that my case was just too complicated, and of such a high level of risk, and I was again taken off the list. It appeared to me, this time, the decision was final.

Instead of falling into a heap, I spent 2006 working very hard on building and maintaining my health and fitness level. I thought then that I only really had myself to do all the right things. I felt it was largely up to me to move myself into a position where I might hopefully be reconsidered for a transplant, sometime in the future.         

Although I felt my fitness had improved, I was still experiencing recurrent episodes of cardiac failure, requiring regular hospitalisation for treatment.  During one of these stays in RPH, Cardiologist Dr Gerry O’Driscoll tried a different approach to thin my blood, by venesections (blood-letting) of considerably greater volume than during the previous 12 months. This proved to be a major breakthrough and resulted in my kidney function improving, and me being taken off Warfarin (thus allowing my blood to return to normal levels of viscosity).

Thanks to the new treatment regime, and my improved “fitness”, I was ecstatic to be re-listed in November 2006.  I would also like to think the various medical teams acknowledged how hard I had worked in 2006, physically, as well as keeping myself mentally focussed on the future.  I never gave up on life!!  Dr Gabbay did warn me at this stage that I could end up with kidney damage and may need dialysis for the rest of my life.  I was willing to take that chance. I felt in myself that my kidneys would come back in to order once the blood supply was regular.

In May 2007 I received THE call from RPH about a potential donor and it was then I became the second person to have a heart and lung transplant performed here in Western Australia.  Given my pre-existing heart condition was extremely rare, in an adult, and more complicated from a technical point of view, my transplant operation was photographed. To see some of these photos click here.

One of the photos shows a surgeon with my heart in her hands. Usually a heart will fit in one hand however, because my heart had to work so hard it had grown to more than twice the normal size. The surgeon said my only artery (instead of the normal two) was 5cm in diameter and the heart was very heavy and muscular.

From what I understand, I am one of only a handful (of adults) in the world with this specific congenital heart defect who has gone straight into transplant, without ever having previous corrective surgery of any kind.

I consider myself extremely fortunate in many ways.  The generous decision of the donor family to make organs of their loved one available was paramount.  Only combined with the world-class skill and confidence of the surgical team at RPH, headed by Rob Larbalestier, - who accepted my case and conducted the operation with absolute perfection -, and the competent and passionate care by all teams involved, before and after surgery, makes such a positive and successful outcome like mine, possible.
 
I have taken medication of some sort or another for most of my life so the drug regime post-transplant is not something that fazes me. I take a handful of medication each morning and night. All transplant patients manage a drug regime for the rest of their lives. I take about 16 tablets a day, which has reduced since the initial period post-transplant, and that also includes vitamins. It’s not all bad.

I have always been careful to watch the state of my health. Before my transplant Stephan described me as a finely tuned race car. Prior to the op I had to be absolutely fine tuned otherwise I would slip off the edge. I used to suffer from asthma and there was a fine line between medications for both of my health conditions. Both the Cardiology and Advanced Lung Disease medical teams did an outstanding job to manage my medications without causing unnecessary complications.
 
Post transplant I am still a finely tuned machine. I listen to my body as I have done all my life, I exercise regularly, eat well and the medications are simply a change of pace. I still frequent the hospital, sometimes three out of five days a week with appointments, the gym, this and that. But ultimately how can I complain when I receive such good service. RPH is wonderful. There is not an area they don’t look in to. I just had my 12month anniversary and the number of tests that I was lined up for was amazing. They go through everything again.

There is a post box around the corner from where we live. Before my transplant it was easier to jump in my car and drive around the block instead of walking. When I walked, it would take me at least 15minutes and by the time I got half way back I was too tired to keep going. Now I can stroll there and back in about four minutes.

I enjoyed an active couple of days cycling around Rottnest Island recently, and Stephan and I also spent a week down south in Pemberton, doing plenty of bush-walking. One of the hikes we did was 12kms, which in the past I never would have dreamed of. Previously I would have gone a few metres then had to send Stephan on ahead of me, telling him to enjoy himself, take lots of photos and come back and share them with me. It was so lovely to see a steep hill in the bush and be able to walk up like a ‘normal’ person. It was a huge achievement.  I was really thrilled, and so proud of myself.

Most people take activity like this for granted but it is the simple everyday things in life I enjoy the most, and cherish being able to do. From walking and swimming at the beach to climbing up a flight of stairs, it sounds so basic. For all my life when ever I have taken a set of stairs I would always count the stairs. I found counting them took my mind off having to climb them. I was still counting the steps after the op and it wasn’t till about October (5 months after surgery) when I walked a flight of stairs and turned around to Stephan and said “Guess what? I didn’t count them.” After 40 something years, I didn’t have to prepare myself for it and stop half way.

It’s like starting a new life in a way. I have the energy to cook now, so I love being in the kitchen and trying all sorts of new recipes, which tends to lead to more eating too. But it’s all good.  I also have gone back into the workforce for a couple of days per week with an accounting firm doing secretarial and admin work. Working with the firm was my very first job actually. I always kept in touch with the owner and he said “if ever you want a job, please let me know”.  At the beginning of 2008 I felt as if I needed to tease my brain with something. The past three years my main concern has been my health, what appointments I have, getting up in the morning to get to the hospital and do the routine I had to maintain.

There has not been a day in my life that I have felt bored, even when I have not been doing anything in particular. I have a pretty positive outlook no matter what cards I’m dealt. I’m a strong-minded person, and have been referred to as “fiercely independent”. I think it is this aspect of my personality that has enabled me to make it through the many tough times.  I suppose it could also be a mental strength that I inherited from my Dad.

Paul Barratt, the first heart & lung transplant patient operated on in WA, said to me once that despite sometimes despairing circumstances I always manage to smile. I don’t know why or how I do it, it just happens. I have a happy outlook on life, which is precious.

We are looking forward to more travel now that my health is good. I am keen to resume my life from where it started to wain, four years ago.  I remember in 2006 after a hospital short-stay treatment for cardiac failure, and saying to Dr Dembo how great and normal I felt and he replied with a smile “Tatiana, I want you to feel MY normal.” That’s the difference, I can pick up my life now from how it was, but in actual fact it is a different standard of life. I am still doing the same things but I am able to do them now with ease.