To understand my sister Jennet’s organ donation story, you need to go back to the very beginning. She lived with a rare metabolic disorder, Erythropoietic Protoporphyria, which in simple terms meant that her body reacted to ultra-violet light, causing her to burn. However unlike regular sunburn, the reaction caused an excess of a chemical (protoporphyrin) in her bone marrow, plasma and red blood cells. This would cause severe pain, swelling and blistering internally as well as on her skin. If severe enough, it could cause issues with the gall bladder and in rare situations, liver failure.
She was diagnosed when she was two years old and as a girl living on a farm in country NSW in the 1970’s there was little information and knowledge about it, leaving my parents to do their best to ‘keep her out of the sun’, which is the only known effective treatment for this condition.
Despite this, my sister never let her condition stop her from doing things in life. In her short 41 years, she succeeded at school, entering ANU in Canberra where she completed a Bachelor of Science with Honours. Whilst there she had many adventures: skiing, travelling, camping, and making the most of her university days.
After this she applied and received a Rotary ambassadorial scholarship to America. She chose to attend University in Portland Oregon, as the climate was favourable. She had many wonderful experiences and met some lovely people that she had remained in contact with up until her death. Before returning to Australia she had an opportunity to visit Alaska. It was here that she met, Jimmy, on a bus trip into Denali National Park.
It is what storybooks are about: Girl meets Boy on Bus, spend a week together before she leaves for the other side of the world. Before Skype and Facetime, long distance phone calls and letters over the coming months led to Jimmy Jennet, beloved mum, wife, sister. Distance it seemed couldn’t keep the relationship from blossoming, with Jen returning to the USA and a proposal of marriage from Jimmy. The following year they were married in Australia’s outdoors and returned to America.
Both had a desire to study medicine, so their married life began at an international university on the island of Saba in the Caribbean! Where else would someone with an inability of being in the sun go?! For the next four years, they studied and had practical placements around different cities within America and a term in Oxford, UK. At graduation, she won the University Medal for her year.
Once qualified, they returned to the scene of their chance meeting, Alaska. It was here they both entered a family medicine training program. During her time in the program, Jennet had become known as a person with great compassion, care and excellence, gaining a position of mentorship in her final years.
After their training program was complete, they moved to the rural seaside tourist town of Seward in Alaska. It was here they expanded their family to include Sam and Will. Work and leisure was rich and full. Professionally Jennet became the director of the local hospital and earned a reputation of being an incredible doctor, teacher and friend. Socially they spent time exploring the Alaskan outdoors, camping in the summer and skiing or skating in the winter.
Jennet actually found the weather and climate in Alaska reasonably manageable. The summers were always hard, with nearly 24 hours of daylight, but given it was relatively short, she managed to work a lot and avoid the sunlight as much as possible. The winter light was kinder on her body and it was easier to cover up and not be so exposed.
However, Jennet missed home and wanted to return to Australia for her boys to grow up with the Australian lifestyle. It was a difficult decision and we had many conversations about how she would find the climate. In December 2010, she returned home to Australia and we spent our first Christmas together in a long time, and the first as mothers.
Immediately I was drawn back into her world of shade seeking. Watching her fiddling with her long sleeves and hats to cover any exposed skin took me back to our childhood, only now it was worse. She cried with uncertainty of how she was going to do it and be able to be there for her boys.
A month later, Jen came to Adelaide for a job interview. The dream of our families being together was coming true. She needed to return to Albury to pack up the boys who were staying with my mum. The night before she left she felt nauseous but put it down to nerves of the interview and not eating much. We went out to a movie, Black Swan, which was to be her last one. Many times, I’ve thought of this and felt angry that why wasn’t it a beautiful romance or wet your pants comedy. But the dark drama was nothing but an indication of the months to come.
What followed seems like a spiralling time warp of hospitals, tests, agony, bureaucracies and red tape, uncertainty, medicines, fear, waiting, lots of waiting. The nausea progressed, resulting in the horror show that was liver failure.
I rushed to her side in Albury Base Hospital where she was jaundiced and in pain. She needed to be transferred to Melbourne but due to the complexities within the health system, she was not critical enough and currently receiving care. Frustrated and confused, we fought for her to be transferred, which she eventually was.
This began the process of finding out what was causing the failure and where to next. Scans, blood tests, doctors and more doctors who had never seen anyone with this condition came. It felt like a circus. What was making life difficult was trying to get the staff to understand that due to her EPP, she was reacting to the fluorescent lighting in the hospital rooms, and she needed to be in darkness.
The test results showed severe liver failure with the only option being a transplant. But they had some time as her bloods were stabilising. Transfer from one hospital to another occurred along with new doctors and nurses who had no understanding of this condition. Due to her sensitivity to light she needed special filters for the theatre lights for her procedures/ operations, which became a big stumbling block. They needed to come from France and despite efforts made they never actually arrived.
In all of the craziness of the ward routines and medical procedures, drugs, pain, and waiting, lots of waiting, was her husband and children, my parents, my own family in Adelaide, my brother who lived overseas, all trying to get on with day to day life. It was hard. Hard to see her own sons too scared to give her a hug because of the scary drips and machines, hard to see Jen sitting in her room waiting and wondering how her boys were and getting to say goodnight on the phone at the end of the day instead of being there to kiss them goodnight. Her boys were her world. Many times she said she was going through with this for them. They were only four and six at the time; too young to lose their mum.
Jennet’s condition worsened with her sensitivity to the light so severe her room was blacked out, lights off, even the IV pump machine light and TV screen was causing reactions. One way to reduce the pressure on the liver was to ‘clean’ her blood of the toxins. Her plasma was black. This was unchartered territory for the medical staff. All the time her other liver function tests were okay. The transplant doctor kept reassuring her that she had time and believed a liver would become available. However, what the doctors did not realise were the toxins had started attacking Jennet’s nervous system. I recall distinctly the day Jen realised things were critical.
She had been experiencing the odd tingling sensation in her legs or arms, but this particular moment she said to me: “Look Lizzie, I can’t puff out my cheeks.”
As a doctor she knew this meant there was damage to the nerves controlling her face. What happened beyond this point was a nightmare. The following day there was weakness to her hands and she said: “I need to write to my boys today because I don’t think I will be able to tomorrow.”
So we set to work writing what was going to be the most important letters ever written. As you can imagine she was fighting a losing battle, but her spirit was never broken. I have some very precious memories of this time and many more that still haunt me.
The final days Jennet was ventilated in ICU and still receiving treatment until the doctors performed tests on her nervous system. She had developed irreversible nerve damage to her whole body and there was nothing more that they could do for her. Without the ventilator keeping her alive, she would die.
It was then the doctors discussed the possibility of her becoming an organ donor. It wasn’t something that I thought would be possible given how sick she was. Also Jen’s situation was somewhat different because she wasn’t brain dead. She was fully aware of her condition and ultimate fate. She clearly consented to organ donation after cardiac death. For our family, we had to shift our thoughts from being a recipient to donor. Not an easy switch. And so the process began.
We were able to have one last magical moment in all this ugliness. We took her outside to look at the stars, one last time. Bundled up, machines and staff in tow on a beautiful crisp winter evening, we went and sat with her to feel the breeze again. It had been months since she had been outside. Two days later, the lives of three other families changed as well. An older lady received her lungs and two others received her kidneys and I believe are doing well today. We have never heard from the families but through the DonateLife co-ordinators, we have been given updates at our request. Unfortunately, the lady who received the lungs passed away but not as a result of the transplant. We believe she felt so grateful as she returned home and spent time with her family and grandchildren. Although you wish everyone’s story would be picture perfect, we felt Jennet would be so pleased helping another family be together, even if just a little longer.
Our experience of waiting for transplant and then becoming a donor, I think, is unique. It provides me with a greater awareness of the need for organ donors and yet the tragedy that comes with that gift. For this reason, I have volunteered with DonateLife in South Australia for the past five years. More recently I have become a member of the DFA family as well as the SA representative role. My experience has given me an appreciation for the need to support families post organ donation and see DFA being a part of this.
Everyone has a remarkable story to tell; this has been mine. Lizzie Mazur