Conjoined twins

Parting Gift – Ruby and Rosie were more than identical twins. At birth they were conjoined, sharing vital organs and undergoing radical surgery. Audrey Ward reports on their extraordinary lives — together and apart. Portraits: Laura Pannack

3 November 2013

The Sunday Times Magazine

Sitting in her neat, orderly home in a quiet cul-de-sac in Bexleyheath, Kent, Angela Formosa proudly shows me a picture of her three daughters, taken last month on the sixth birthday of Lily, the eldest. Lily is flanked by her one-year-old twin sisters, Ruby and Rosie, who are dressed in matching black-and-white dresses — two little helpers ripping off the wrapping paper of her birthday presents. “Ruby is the quiet one, a thinker,” says the girls’ father, Daniel. “Sometimes it takes her ages to break a smile, but that’s changing all the time. Rosie is the chirpy one, but she has more of a temper.” Most of the time their parents can tell them apart — Ruby has a strawberry birthmark on her neck — but every now and again the girls can “switch personalities” and for a split second Daniel and Angela will get confused. So it can be with identical twins — but not all twins, and not all parents of twins have been through the trauma the Formosas have endured in the past year.

When Ruby and Rosie were born, on the morning of Thursday, July 26, 2012 — the day the rest of the world was getting ready to watch the opening ceremony of the London Olympic Games — they were conjoined at the abdomen.

Angela wells up as she casts her mind back to the crisp morning in January 2012 when she and Daniel arrived at the Queen Elizabeth Hospital in Woolwich for their initial scan, hoping for a first glimpse of their unborn child — a baby brother or sister for four-year-old Lily. Though they were giddy with excitement, they felt none of the first-timer’s anticipation of the unknown. They’d done this before. Just as had happened when she was pregnant with Lily, the sonographer ran an ultrasound probe over Angela’s abdomen. She studied the images, told the couple that she wanted to speak to the consultant, then disappeared from the room.

The consultant told them the good news. “Look, you’ve got twins,” she said, pointing to the screen. “They’re like two little baked beans.” Angela, a 33-year-old former beautician, burst into laughter. “Your mum was right, Dan,” she said. Their elation, however, was tempered minutes later when the consultant failed to find a dividing membrane, and remarked on how close together the babies were. She suspected the twins might be mono-amniotic, a condition affecting about 1% of twin pregnancies, in which an embryo fails to split until after the amniotic sac is formed. The potential problems were spelt out to them: the umbilical cords could get entangled, affecting the babies’ movement and development; one twin might receive the majority of the nutrients, causing the other to waste away; or one twin might compress the other’s umbilical cord, killing the other.

“I came home that night and was Googling mono-amniotic twins, and that was frightening. I was very stressed and very emotional,” says Angela. Daniel, a 36-year-old black-cab driver, refused to dwell on it.

“Mentally, I just put it away in a drawer and tried not to think about it,” he says.

The couple was thrust into a world of uncertainty, and the stress began to build. “On the eve of every scan, we’d just end up arguing about stupid things, like who had left a glass on the table or whose turn it was to change the loo roll. It would be normal things but we’d have an over-thetop argument,” Daniel remembers. SEPARATED Ruby Bowel Womb Finally, on February 28, 2012, a doctor at King’s confirmed that their babies were indeed sharing the amniotic sac and placenta — but he had far worse news: they were conjoined. Angela had gone with her mother to meet Professor Kypros Nicolaides, a fetal medicine specialist. He ran the probe over her For identical twins, the pregnancy. In rare cases, take place until after so the sac and placenta to conjoining: Ruby at the abdomen, swollen belly and broke the news to her as 20 medical students looked on.

As her mother began to cry, Angela broke down. She hadn’t for one minute expected this. “It’s so rare, you hear about it happening in the jungle, or in India — such a big country — but you never think it’s going to happen to you or anyone you know.” She knew only a little about conjoined twins, having seen pictures of them on the internet. “You see some terrible, terrible sights. When I found out, I didn’t feel normal.”

AFTER BIRTH Amniotic sac Rosie Upper intestine Until relatively recently they would have been called Siamese twins — a catchy misnomer coined after the birth of Eng and Chang Bunker, conjoined twins born in Thailand (then called Siam) in 1811. Eng and Chang were joined at the lower chest by a narrow band of flesh, which connected their livers. They were exhibited as exotic freaks in circus shows around the world. The word “Siamese” has since been displaced by the more prosaic, physiologically descriptive “conjoined” today. Eventually, Eng and Chang settled in America, where they married two sisters Shared lower intestine embryo separates during the separation fails to the amniotic sac forms, is shared. This can lead and Rosie were joined part of the intestine and had nearly two-dozen children. They worked as businessmen and ranchers in Wilkes County, North Carolina, where they lived until their deaths at the age of 63.

For Angela, the massive emotional shock was compounded by the sense that perhaps she was to blame. “I did think, what have I done to deserve this? Was it something I ate? Was it some water I drank somewhere? Or perhaps I went for a drink before I found out I was pregnant. In my mind I knew it was impossible but it didn’t stop those thoughts.”

Professor Nicolaides could tell the babies were joined at the abdomen, but he couldn’t determine which organs in the pelvis they might share. He also intimated that the babies might not survive the pregnancy. “He said I should prepare myself every time I went for a scan, as the worst might have happened,” says Angela. She called Daniel from outside the hospital and told him the news. “How will we handle this sort of thing? That was running through my mind,” says Daniel, “but at the same time we had just bought a new house, so I was concentrating on moving us. I was trying to comfort Angela and look for the positive.”

They decided to confide in their families and just a few close friends. “I didn’t really tell too many people because I had to get my own head around it. It was really hard,” says Angela.

Two weeks after the diagnosis, the pair discovered the sex of the babies: Angela was carrying two girls. “At this point people were saying, ‘Are you sure you want to go ahead with it?’ But we were 100%,” says Daniel. “We just wouldn’t want to terminate a pregnancy. We both felt that we’d rather see what the outcome would be rather than having to intervene and put that on ourselves.”

On a blue-sky day in June, Angela had an MRI scan at University College Hospital (UCH). She was booked in for a Caesarian section to coincide with the 34th week of the pregnancy and was told that once the babies were born they would be transferred to London’s Great Ormond Street Hospital, which specialises in the management and separation of conjoined twins.

Although apprehensive about the birth, Angela never doubted her love for the twins. “I thought they might come out and be severely disabled, but I also knew they might not be. Whatever happened I would cross that bridge when I came to it. At the end of the day, they were my babies. I had a bond with them. I didn’t really think about much else.”

Meanwhile, Daniel was finding it increasingly difficult to concentrate on his job as a cabbie. “It’s not like a job where you can turn up and get away with shirking a bit. The number of times I did go to work and I was taking people to the wrong bridge or house. People were getting shirty with me because they thought I was trying to do them out of money. At one point I thought, ‘I SAW GIRLS ‘If you only knew.’ ” The day of the birth, three dedicated teams of doctors and nurses were on hand, one for Angela and one for each of the babies. “It wasn’t until I sat on the bed and they put the anaesthetic in my spine that I started worrying. I can remember I got the shakes. It was like I was in shock,” she says.

Throughout the operation Daniel sat with Angela, stroking her hair, trying to keep her calm.

They both had no idea what would happen after the birth. “The doctors couldn’t make any plans until the babies were out, as only then would they know exactly where the join was,” says Daniel.

At 9.11am Angela heard a guttural cry and then a minute later she heard an indignant wail. Rosie was born first and Ruby was next. “The doctors pulled the blanket down and let me see the girls. Then they took them next door to clean them up and make sure they were both breathing.”

Soon afterwards, a nurse brought the babies back to Angela for a cuddle. “I saw two perfect little girls wrapped in a blanket. I knew they had a join at the tummy, but I also knew they had their arms, their legs, their fingers and toes.”

Daniel’s initial feeling was one of exhilaration. “They were huddled together and they were both sleeping, but their hands kept stretching out of the blanket.” But reality soon hit. “To look at them, well, they looked beautiful, like there was nothing wrong with them. But then they were whisked away and I thought, ‘Where are we going to go from here — what’s next?’ ” What came next was the discovery by a paediatrician that the babies had a blockage in their shared intestine. They needed to go to Great Ormond Street straight away. There they would undergo ultrasounds and an x-ray called a contrast enema, which involves the injection of a liquid dye to show the structure of the large intestine.

Before the twins were taken away, they were blessed by a priest in the presence of their parents and grandparents. “I thought it would be nice if all the grandparents were there, if they had five minutes to see them. If the babies were going to survive or not, it would be nice for them to be part of that,” says Angela.

TWO PERFECT IN A Daniel kissed Angela goodbye and travelled in an ambulance with the babies, who were in incubators. On arrival, the twins were whisked away and Daniel was advised to take a pager and wait. “I sat and had a beer in the pub and I got something to eat. I spoke to one of my friends, but it felt like I had nobody to talk to. I felt quite alone.”

BLANKET’ When he returned to the hospital he was told that Ruby and Rosie would need to be operated on the following day. He called Angela to let her know. Dredging up memories of that night causes them both to unravel. “It was the worst night of my life,” says Angela. “They put me in a room with three other mums that had their babies with them. I could hear all the other mums moaning — ‘The baby’s crying, nurse, what can I do?’ ‘She’s not breast-feeding, what should I do?’ I think I held it together, but I was just getting annoyed. I wanted to tell them they had nothing to moan about.”

The following day, Daniel met Angela at UCH. Daniel breaks into a smile as he remembers pushing his wife around in her wheelchair and taking her in a taxi to Great Ormond Street. “It was like Lou and Andy [from Little Britain]. She had her bed socks on and was getting wheeled along and she was complaining about every bump.”

The Formosas met with their medical a

team and their permission was sought for the operation. “We signed the consent forms, which I found really hard to do,” says Daniel. “I got really emotional and I had to walk out of the intensive-care unit afterwards. Effectively, I was giving somebody permission to cut my children up. I didn’t know the operation would save their lives.” Professor Agostino Pierro, 59, is the paediatric surgeon who led the team that operated on Ruby and Rosie. An Italian, he trained in Toronto, and worked for four years in Liverpool, before moving to Great Ormond Street, where he remained for 19 years. Earlier this year he returned to Toronto where he is the division head of general and thoracic surgery at the Hospital for Sick Children.

He had hoped that the girls wouldn’t need to be operated on immediately after their birth, but it soon became clear that time was of the essence. “Normally we wait two to three months and then we plan the operation, but in this particular case we had to do the operation as an emergency because there was an obstruction,” he says in a sing-song Italian accent. “The intestine was joined and we didn’t know why it wasn’t working; we knew we had to operate.”

In the hours before the birth, Pierro began to visualise how the operation would go. “I think preparing yourself is the most important thing — preparing yourself not just physically but mentally. I usually relax and make a mental plan. I think things through. I think about possible complications occurring and how I could correct them and this is the most important thing when separating conjoined twins, the mental plan.”

On average, he operates on one set of conjoined twins a year. “This was not the most difficult separation,” he says, “but it’s not like removing an ingrown toenail. It requires expertise. You are confronting a situation in the anatomy that is unexpected and you have to make a decision coming from an angle which is not the usual one. You come from the side, not from the front, which is the normal route for a patient to be operated on.”

The day after their birth, the babies were ‘they laid side-on and Pierro cut across their abdomens at the point where they were joined. “When you separate conjoined twins there is always an uncertainty about what you’re going to find and in fact even in this case, not everything in the scan was shown. The anatomy was very disorganised.”

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didn’t Once he’d looked inside he could see that the babies were sharing the lower intestine. Ruby had her upper intestine only, whereas Rosie had both the upper and lower intestine. They were also linked by the bowel and a little bit of the bladder. “It was more or less what we expected but the exact point where the bowel was joined was not completely clear at the pre-op investigation,” he says.

He made a judgment over who would get what, then he started cutting and dividing tissue until they were separated. Afterwards one baby stayed put and the other was taken to another theatre. Then the babies were stitched up. The operation, which took about five hours and involved a medical team of 15, went smoothly.

The Formosas were hugely relieved to be reunited with the girls. “They were in an incubator so we couldn’t touch them or pick them up. Lily was ecstatic on meeting her new sisters. We let her put her hand through the hole in the incubator and touch them,” says Angela. But they knew the twins would need to fight on. “It was nice to see that the operation was a success and they were separated, but it wasn’t nice to see all the tubes coming out of them,” says Daniel.

After the operation the babies were put on a morphine drip and on a respirator. A catheter was inserted into their bladders and an intravenous line gave them their fluids. They spent five days in the intensive-care unit and then were moved to a ward where they remained for just over two weeks.

Angela recalls her trepidation in those first few days. “They were tiny little babies, they didn’t cry much at all. I was too scared to touch them. I didn’t feel like they were mine, but the nurses got me involved.” She had to be careful changing nappies because of their wounds. Initially they were tube-fed with Angela’s milk, but they started losing weight, so she switched to formula in the hope that they would quickly build up their strength.

Three weeks after the operation the girls were ready to go home. Daniel was frantically trying to finish off the work on their new house and get the twins’ bedroom ready. He arrived at the hospital in his black cab with Lily in tow. “I went through the whole thing of strapping them into the cab seats. I was frightened to go over a bump because I had these fragile goods on board.”

were they cry It was daunting for Angela to be away from the secure bubble of the hospital and she could see their new house still needed a lot of work. “I was really happy to have them home, but I was a bit scared too. The state of the house was quite depressing.”

One year on, the girls are thriving and their different personalities are shining through. In a few months the girls will need to undergo more surgery, but the Formosas aren’t worrying about that for now, they’re just focusing on the joy the girls bring. “I still can’t quite believe how far they’ve come,” says Angela. “I didn’t think I’d ever get them. I never thought I’d hold them in my arms.”

In the garden, on a neatly trimmed lawn, a climbing frame with a slide and swings takes centre stage — a playground, they hope, for many happy family days to come.