Twin to Twin Transfusion Syndrome Australia Inc.

A Member Group of the Australian Multiple Birth Association
 

Max and Oliver

Rachel, together with her husband Pete, are the proud parents of Charlotte, Max and Oliver.
At nineteen weeks gestation, Max and Oliver were diagnosed with severe twin to twin transfusion syndrome. They were born at 28 weeks gestation. Max, our recipient twin thrived. Oliver gave his all and died in our arms at six days of age cuddled up next to his brother.
This presentation was given at the Multiple Birth Conference held in Melbourne on February 15th and 16th, 2003.

Nature can be cruel. Severe twin to twin transfusion syndrome is nature at its cruellest. First there is one, then two, and then in our case only one again.

My name is Rachel. I am married to Pete and we are the proud parents of three beautiful children. Charlotte is three, Max will be one at the end of March and Oliver is our angel child in heaven. The following is our own personal experience.

January 23rd 2002 was the day my family’s life was turned upside down forever.

At 19 weeks gestation I was very big. Twin jibes had started early. At my sixteen week check up with my independent midwife I was slightly bigger for dates, but there was no second heartbeat. We talked about twins and Annie had a good feel of my stomach – it appeared to be one. Over the next three weeks, I ballooned out so that by the time I was checking in for my ultrasound at 19 weeks I was getting many questioning looks. I was in fact the size of a full term pregnant woman – and although I felt very uncomfortable and was trying to work out where the next 20 weeks of growth was going to go, I didn’t realise I was THAT big !

The signs were there that this was a twin pregnancy – extreme morning sickness, early movement at 14 weeks, big for dates……

When the ultrasonographer pointed out two babies within a few seconds of commencing the ultrasound my heart jumped but it only confirmed what I already knew. Overwhelming tears followed – how was I going to cope with a toddler and two babies? But something was not right and I sensed it almost immediately. Twin 1 was swimming in a huge pool of fluid. Twin 2 was smaller.

I had heard of TTTS – I had worked with the severely disabled survivors in two different cases in my profession as a paediatric physiotherapist. I offered this information to the sonographer – she didn’t say much only that all the babies’ structures were present and normal. A second opinion was sought immediately from the head of the practice and then we were asked to sit in a private room. Having worked in the health field I know that this is not a good sign. Bad news was pending. In an honest and frank way we were given the news. Severe twin-to-twin transfusion syndrome. The prognosis was presented – our twin boys were in significant danger of premature birth, death or disability.

Our plans and preparations for a homebirth with our independent midwife were thrown into disarray. As a proponent of minimal intervention in normal pregnancy and birth, I was distrusting of the obstetric profession. Suddenly this pregnancy was no longer normal and I needed an obstetrician. Someone who knew about TTTS, and importantly respected my opinions and wishes. We were given a name and after phone calls to Annie and my GP we fronted up to his private rooms unannounced, without a referral and in fairly desperate need of some quick attention. Within three hours we were sitting in his office and spent over an hour on that first day with him. It was important that we make some rather difficult decisions quickly as premature labour was predicted to be imminent.

We were given all the facts, with medical literature and websites to review. We were told that we had a 33% chance of bringing both boys home, and a 60% chance of getting one of the boys home. Firstly though I needed to have an amnioreduction. This allowed us a little more time to make the hard decision about ongoing management of the pregnancy. In our minds we had to give both of our boys every chance. Therefore, amnioreduction and laser surgery were the only two options that we were prepared to consider.

At the time no laser surgery had yet been performed in Australia. To us it was extremely risky, especially as it presented a maternal risk. Added to this complex and in our minds, experimental surgery was the fact that I had an anterior lying placenta. It was explained to us that this made the surgical procedure more technically difficult. I would also have to fly to Brisbane to be assessed for surgery. It was a very difficult decision for us to choose the more routine management of serial amnioreductions. By not trying to stop the placental blood flow problem we felt that we were sacrificing one or both our boys. We sat down with all the facts as they had been presented to us and drew up a list – pros and cons for the two options we were considering. In the end we felt we made an informed decision. A decision that we felt was right for ourselves, our two year old daughter and our unborn babies at that very moment in our lives.

The next nine weeks we lived from ultrasound to ultrasound, reduction to reduction. It was a roller coaster ride. I immediately quit work and tried to rest as best I could with a two year old. She had seven day a week care from family for the entire time until we brought her brother home from hospital 17 weeks after that first U/S. I found that the most crucial time that I needed to rest was after a reduction. I tended to have uterine contractions for several hours after each reduction and I found a combination of rest, listening to relaxation tapes and meditation assisted me in recovering from each reduction.

By the eighth reduction I was a pro, but I wasn’t sure I would make it past the first reduction when as the needle was passed into my swollen uterus, amniotic fluid spurted forth like a geyser. I soon was able to predict when I would need a reduction – generally discomfort in my back, a hard stomach and difficulty taking a deep breath were good indicators, but I also became quite in tune with the boys movements. Once Max slowed down and I could not palpate his limbs, a reduction was pending.

I also became a bit of a pro at how my boys looked on U/S – I was probably the radiologists worst nightmare as I was asking questions one step ahead of the picture on the screen. Each time the image flicked up on the screen I would immediately look for two heartbeats. Then we would go through the checklist – brains, hearts (Max our recipient, had a VSD and cardiomyopathy), tummies – no excess fluid around any of the organs, Oliver’s (our donor) kidneys and bladder. Umbilical blood flow. Estimation of excess amniotic fluid around Max. Need for a reduction or not. It was so reassuring to see each of the boys moving – they always had their little heads orientated the same way – Oliver squashed down on the left and Max swimming around on the right. The first six weeks I needed a lot of fluid removed – I became a pin cushion - this was always stressful, but at 25 weeks after 2 litres came off in one reduction the process seemed to stop and I didn’t require another reduction. Those last three weeks were a little more reassuring as we felt we were being blessed with extra time.

During those nine weeks I did a lot of research and planning. As a type A personality this helped to ease some of my stress. I went on a high protein diet –with daily banana and egg smoothies and red meat or fish or both every day. I wrote and emailed regular updates to my family who are scattered across the globe so that all our family had the latest news. I kept a diary. We met with the neonatologist that would look after the boys in the NICU to discuss likely outcomes when the boys were born. We made heart-wrenching decisions about when we would ask for life support to be given and when we would ask for it to be withheld. I drew up birth plans for pre 25 weeks gestation and then once we made it past 25 weeks I drew up a new birth plan and plans for how we wished our boys to be cared for in the NICU. It was important to us that if we were not with our boys that the staff looking after them knew how we felt about certain aspects of their care. I researched the effect of prematurity on family and siblings so that we could best prepare ourselves for the road ahead.

It was very important to me that my midwife continue to be part of my birth team, as I needed her emotional and psychological support for the rest of the pregnancy and at the birth. We spoke on the phone regularly and I visited her office a couple of times for a cuppa and a reassuring chat. It was important that once the boys were born, Pete would go with them to the NICU, but I did not want to be left alone in theatre and recovery with strangers. My babies were fighting for their lives and I needed a friend with me. My midwife was “with woman” not in her traditional sense but still with me nonetheless and her role in my birth team was vital.

All of my wishes in respect to my birth team, birth plans and the boys care plans were respected by the health professionals we came into contact with. This respect was so very important to my sense of being in some semblance of control in a situation that was totally out of my control. I felt that Pete and I were part of the team that was caring for this high-risk pregnancy and the very fragile little individuals that were to be born.

Nine weeks later, eight reductions, more then 13 litres of amniotic fluid removed, over 12 ultrasounds, weekly reviews with the obstetrician and meetings with a neonatologist and other hospital staff… nine weeks later, Oliver’s heart started to fail – it was time for the boys to be born. Max, twin 1, the recipient twin and Oliver, twin 2, the donor twin, were born at 28 weeks and one day gestation. Our initial goal had been to get them to between 28 and 32 weeks – we scraped in. I had a second dose of steroids the night before the boys were born, having had a dose at 25 weeks when we thought their birth was imminent earlier.

Max breathed at birth and went on to thrive. He did so very well in those early days. Oliver struggled for life from the moment he was born. After six days, his little lungs and heart were finding the struggle too hard. We agreed to turn off life support and he died in my arms cuddled up to his big brother and with his daddy kissing him. It was the hardest decision we have ever had to make.

In the last 12 months we have ridden the biggest roller coaster ride of our lives. We have faced the devastating blow that a diagnosis such as TTTS brings, we have had the uncertain stress of imminent premature labour, experienced uncomfortable procedures, we have witnessed the premature birth of our babies and watched helplessly as they have fought for life, our hearts have broken as we watched our son die in our arms and our hearts have rejoiced as his brother has gone from strength to strength. We continue to live with the uncertainty of the possible long term effects on Max of the TTTS and his premature birth. We continue to mourn the loss of Oliver, our son, Charlotte’s brother and Max’s twin. There are so many “what ifs”. Despite knowing in my head that where we are today is where we are meant to be, my heart agonises over the events and decisions that we made.

We would not have made it through the last year without the love and support of our family, friends and community and without the professional knowledge and expertise of the health professionals we entrusted our care in. We have been humbled by the overwhelming response from those around us that has come from such devastation.

First there was one, then there were two and now we have one here with us on earth and one above us in heaven.

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(c) 2004 TTTS Australia Inc.