An Aussie blogger pregnant with twins finds herself in a fight with an incredibly rare condition which threatens to kill her unborn twins.
Twins Mathilde (left and very red) and Emilie (right and very pale) – mum Stephanie first developed TTTS and then TAPS.
“Sometimes life throws something at you and you just have to deal with it. I sometimes asked myself how I remained sane,” Stephanie Ernst – blogger and mum of twins.
We all know that life can be random and just when you think things are running smoothly you are most likely to be thrown off course.
This certainly happened to Aussie Stephanie Ernst. First, she found out she was pregnant with twins – shock number one. Then then the twins developed an incredibly rare and life threatening disease whilst still in the womb, shock number 2.
This edition of the Here in Holland podcast features a story about the desire to have children and the fear of losing them.
Blogger’s battle with Twin to Twin Transfusion Syndrome
When the Aussie blogger heard she was having twins her reaction was typical: “You’ve got to be shitting me,” she told the nurse before going to ask if she could have a vodka.
But it certainly was for real. Terrible morning sickness followed: “I think I threw up in every bin between my house and the local hospital,” she says.
But apart from this, things were continuing smoothly. Until a routine scan at 23 weeks.
An anxious technician calls for the doctor and then the wheels of the rollercoaster are set in motion.
The doctor at the Flevo Hospital in Stephanie’s home town of Almere suspects TTTS – Twin to Twin Transfusion Syndrome – and she is sent to the LUMC, a specialist centre for this rare condition.
“It was terrible – it was like I’d been given a death sentence for the twins,” says Stephanie.
The diagnosis was confirmed in Leiden.
Twin to Twin Transfusion Syndrome occurs in some identical twins who share a placenta.
These twins are connected by veins cross the placenta and when the syndrome strikes one fetus transmits a lot of its blood to the other.
TTTS Donor and recipient
One baby is known as a donor and the other a recipient.
“I was told that baby A was stuck to the uterus wall and had very little fluid,” says Stephanie.
The recipient baby – Baby B in this case – then receives too much blood and as a result starts to pee too much.
This leads to a rapid build-up of amniotic fluid and call lead to a premature birth and death of the babies.
Signs of TTTS
One of the signs women pregnant with twins need to look out for is rapid growth over just one or two days. This can be due to a rapid rise in amniotic fluid resulting from TTTS.
Stephanie was given the choice of having an operation or adopting a wait and see approach as there was still a chance the fluid levels would stabilize.
She chose to wait – it was a dramatic time. Ultra-scans every three days at the LUMC and constant travelling and stress.
“Stable became our mantra – as long as everything was stable we were ok…”
Things did stay stable for a while but then another scan revealed more complications.
Twin Anemia Polycythemia Sequence
Another dash to Leiden from Almere follows and an even rarer disease is diagnosed – TAPS – Twin Anemia Polycythemia Sequence.
TAPS is very rare.
About 60 cases are recorded in the Netherlands each year, on average there are around 180,000 births each year in the Netherlands.
Two days of intense monitoring follow and then an emergency caesarian section.
The twins are born – Emilie and Mathilde. The fight to save their lives kicks into overdrive.
Emilie is pale and anemic whereas Mathilde is swollen and so red she’s almost purple. Mathilde is in the most danger her blood is also full of the blood from her sister and is so think there is a danger of clotting.
“One baby has blood which is like tomato sauce and the other has blood which is like rosé wine – both need immediate care in the neo-natal intensive care unit.”
Luckily the babies are in the right place as the LUMC is the leading centre of expertise on this rare disease.
LUMC expertise centre
The twins are constantly monitored and after a few days, and a few ups and downs, they can leave intensive care.
After several more weeks in hospital they eventual go home and normal family life can finally begin.
“I’m such a control freak normally but when something like this happens you realise there are some things you just can’t control. It just makes you feel so humble” says Stephanie.
For more resources on TTTS and TAPS you can go to the Twin to Twin Transfusion Syndrome Foundation is a good place to check online.
Leiden University Medical Centre discovered the disease TAPS and is world leader in research and treatment Dr Enrico Lopriore (link in Dutch) heads the Leiden team on this and is conducting pioneering research.
When asked if Stephanie’s babies would have survived if they had been in a different hospital he says:
“That’s impossible to answer – there are lot of what ifs – and I prefer not to go down that road. What I will say is that she was in a place with the right expertise and we were prepared to treat the babies and we knew what to do.”
“But we still need to do a lot of work, a lot of research, it is a work in progress. We all these books and all this knowledge but we still need to be humble – there is a lot more to learn.”