The Science and Sentiment of Division

 
 
 
 
 
 
 

From freaks of nature and medical conundrums to individuals, conjoined twins have come a long way since "Siamese twins" Chang and Eng. Esquire takes a closer look at the mathematical, emotional and ethical considerations of bisecting a human body and what life is like after separation via brothers Muaiman and Muaimin Azli, the most recent case of separated twins in Malaysia.

Esquire MalaysiA, April 2014 )

 
 
 

An Unexpected Birth

When Muaiman and Muaimin Azli were born by Caesarean section on April 14, 2011 at Sultanah Aminah Hospital, Johor Baru, they came as a complete surprise.

The twins were conjoined at the pelvis, with three lower limbs (one abnormal) and what looked to be one penis between them. Such was the angle at which they were fused together—facing across each other at 180 degrees—that their upper torsos formed the horizontal line of a “T”; and they were missing an anal opening.

Their parents, Azli Sabran and Nazilah Abdullah, had known that the boys were going to be twins, “but no one said anything about Siamese twins,” housewife Nazilah, 40, tells Esquire when we visit their family home in Kluang, Johor.

She didn’t see her babies immediately after they were delivered because she was still weak from the C-section—“I couldn’t even get out of bed, and I guess the doctor didn’t want me to be susah hati,” she says—but her husband did.

“I was immediately quiet. It was like my head was in a jam. I didn’t speak very much for a month after that. I guess I was in shock,” Azli, a 41-year-old rubber tapper, says. He recalls that during his wife’s pregnancy, he had had a premonition of sorts after a series of repeated events. “While driving, I collided with a chicken, a monkey, a cat, a snake... and people say that...” he trails off and hesitates. “I don’t know lah. I don’t even dare to say it. But they say the most potent one is a four-legged creature, like a frog.” He’s referring to a superstition, that these occurrences may somehow throw the forces of the universe off-kilter and affect the outcome of a pregnancy.

“When I think about it now, it was clearly a test,” he says. And what made this difficult moment worse was that he couldn’t share the weight of the burden. He had to keep it from his wife until the next day, just before an ambulance was dispatched to bring the twins to Kuala Lumpur Hospital (HKL), which had carried out several successful separations of conjoined twins, three hours away.

“When I saw my children for the first time, I was a little sad. I hadn’t expected that they would be conjoined... But what can you do? You just have to accept it,” Nazilah says. She couldn’t leave with them as she was still weak physically, and would soon have to begin her confinement. Azli would do double duty for them both, commuting back and forth between Johor and Kuala Lumpur until Nazilah was well enough to join him on the road. Of course, it’s affected his ability to earn a living. “I can’t work with other people anymore—and if I do, it’s only for a short while. I have to go to the hospital a lot, and if I work with people who don’t understand, they get angry. I don’t want to inconvenience them, so these days, I mostly work alone,” he says.

Luckily, besides the matter of their shared body parts, the twins were otherwise relatively healthy when they were born—with no breathing problems or other serious issues. The lack of an anal opening, though, had to be rectified immediately; and the surgeons at HKL made an incision in the twins’ abdomen to give them a single, temporary stoma for faecal diversion—basically, drawing out the end of the colon through an abdominal opening. Then it was a matter of waiting for Muaiman and Muaimin to grow up in order to obtain the most accurate diagnosis possible for their future before making any permanent changes to their anatomy.

Azli and Nazilah always knew that they wanted their sons to be separated. To them, it seemed like the boys’ best shot at having as productive and as ordinary a life as possible.

 
 
 

Then and Now: The Malaysian Context

When most people think of conjoined twins, Chang and Eng probably come to mind. Born in what was then Siam in 1811, which earned them the moniker “Siamese twins”, they were joined at the sternum and the liver. Back in the 19th century, human abnormality was still something to be gawked at, and when Chang and Eng turned 17, they were put on a ship bound for a world tour of America and Europe for public exhibition. Still, they managed to live individual, productive lives even after retiring from show business: in 1839, they settled on a farm in North Carolina and, a few years later, married two sisters and had 21 children between them. Given the voyeuristic nature of people’s interest in the sex lives of conjoined twins, it’s hard to imagine the same thing happening today.

Conjoined twins are a subset of identical twins, so they are always of the same sex. When a woman’s ovary releases two eggs and they’re fertilised by two sperm, she gets fraternal twins; when one egg is released, sometimes it divides after fertilisation, begetting identical twins. Conversely, conjoined twins are formed when the resulting cell mass of the fertilisation process takes longer than usual to divide, interrupting the process of the twins’ full separation, explains Dr TP Baskaran, Maternal Foetal Medicine Unit chief of HKL’s Department of Obstetrics and Gynaecology. It’s not clear what triggers the incidence of conjoined twins, but Dr Baskaran agrees that the risk is higher when you have a history of twins in the family, and that, for reasons nobody yet knows, “there are more female conjoined twins than male ones.”

Testament to the advances in imaging, surgical and anaesthetic techniques since the ’50s, not many twins are left conjoined in the world today. In Malaysia, the first successful case of separation—of two sisters joined at the buttocks—was carried out in 1981 by surgeons at the University Malaya Medical Centre, and since then, there have been 20 more cases of separations in the country. By virtue of the fact that most of these are successful and the subsequent celebration in the media every time one succeeds, one might be under the impression that carrying conjoined twins is no longer something to be too worried about, that it’s a manageable risk. But in fact, more conjoined twins overall die rather than live.

According to popularly cited global estimates, conjoined twins occur once in every 200,000 live births, and Malaysia’s numbers don’t fall far off. The Malaysian government doesn’t record statistics on conjoined twins, but “we have about 500,000 deliveries every year, of which we see about one or two pairs of conjoined twins,” says Dr Zakaria Zahari, head of HKL’s Department of Paediatric Surgery. However, not all of them survive—some live mere hours, weeks or months. Moreover, these numbers don’t take into account the number of conjoined twins who are stillborn, which the University of Maryland Medical Centre in the US estimates to be between 40 and 60 percent of the total number of conjoined twins; and in fact, the overall proportion of conjoined twins who survive is reported to be somewhere between only five and 25 percent.

Twins who share a heart are at the greatest risk. “They’ll likely die within a few hours if they are not supported on a ventilator, and maybe two or three weeks if supported,” Dr Zakaria says. Unfortunately, the most common type of con- joined twins are connected front-to-front, starting anywhere from the sternum to the navel; and of this category, the majority of them are joined at the heart—either two hearts that share a wall or a single abnormal one, usually with fewer than the four chambers a heart normally has to operate properly. “If the heart is fused at the level of the ventricle [whereby blood pumps out of the heart], you can’t separate the twins, because once you cut the heart muscle, it probably won’t contract anymore. If, however, it is fused at the atrium [whereby blood rushes into the heart], an attempt could be made to separate the twins; and there have been attempts, but as far as I’m aware, only two or three have survived in the world,” Dr Zakaria says. He has never attempted to separate twins conjoined at the heart or the brain—because the risks have been too great in both instances; and also because Malaysia has never had a case of twins fused at the brain.

How many pairs of twins have HKL had to turn away when their parents came hoping for a separation? Dr Zakaria says that between 1996 and 2011, seven pairs were deemed inseparable, because they all had a fused atrium and ventricle—and all of them died within three weeks of being born. Then came baby girls Nor Shazwanie and Nor Shazrinie Zullkifli, who had two hearts, but were effectively sharing one because one child's heart was deformed—and they lived for two years before dying from heart failure. Last year, two pairs of twins were born conjoined at the heart; they, too, were inseparable and soon died. “How many twins are actually separable? I think the figure is less than 15 percent,” Dr Zakaria says.

Since 1981, a total of 21 separations have been performed in Malaysia, 14 of them at HKL. The first separation performed by HKL surgeons was in 1988 and, since then, it’s built a reputation as the go-to place for such surgeries. According to Dr Zakaria, all separations have been referred to HKL since 1995 by both state and private hospitals, the last seven of which he presided over as lead surgeon. “Because I’m good,” he says, with a light laugh in a rare, joking instance of professional bluster, at odds with his unassuming head-down, get-to-work demeanour. “Once you do something successfully, people will tend to send cases to you. And HKL has all the facilities and support.” Still, the chances of survival for conjoined twins are, inherently, far from assured. Of the 14 pairs who have undergone separation at HKL, eight have died: some shortly after surgery, some after a few months. In most of these cases, separation was done as an emergency procedure due to complications that arose that had to be dealt with immediately—in which, Dr Zakaria says, “the chances of surviving will usually be 20 percent”—rather than one anticipated with months of planning, which has a survival rate of 80 percent.

Thankfully, Muaiman and Muaimin’s case, though complicated by the existence of a single, shared penis, was relatively low-risk. “Dr Zakaria had told me it would be fine, and so I wasn’t too scared before the operation,” Azli says. “In most cases, ischiopagus twins [joined at the pelvis] and pygopagus twins [joined at the buttocks] are not a problem. You can separate them and they will survive, but they may have mobility issues,” explains Dr Zakaria. The last ischiopagus case he had was in 2006, where “the twins had four limbs between them, and they were lucky enough to have two separate genitalia,” so sepa- ration was easier.

For Muaiman and Muaimin, however, the math wasn’t so obvious. Who would get what?

 

The Ethical and Legal Dimensions

Because of their physical abnormalities, their dependence on each other, and the confusion sometimes over whether they should be treated as one or two beings before separation, conjoined twins can give rise to some ethical and legal dilemmas. What if the mother wishes to terminate her pregnancy because she doesn’t want to bring a child with physical difficulties into the world? What if both twins can’t be saved—would it be possible to try to save one, even at the expense of the other? Everyone will likely have their own views on how morally defensible such actions would be, informed by their own educational, cultural and religious prejudices, but in Malaysia, medical considerations take precedence. The law is silent on conjoined twins: there’s no statute specifically relating to them as a group, and no conflict involving them has ever come before a court.

Regarding lawful abortions, there’s at least some guidance in the Penal Code. Section 132 states that the termination of a pregnancy is illegal, except for when a registered medical practitioner does so if he or she “is of the opinion, formed in good faith, that the continuance of the pregnancy would involve risk to the life of the pregnant woman, or injury to the mental or physical health of the pregnant woman, greater than if the pregnancy were terminated.” Both Dr Baskaran and PS Ranjan, a partner at medical law specialists PS Ranjan & Co, agree that it is the mother’s interests that come first in these cases, and the unborn baby’s second. “When the baby is in the uterus, it has no legal rights,” Dr Baskaran says.

However, it can’t be the mother’s decision alone to terminate a pregnancy. “Malaysian law has never permitted and doesn’t permit termination of a pregnancy merely upon the request of the mother,” Ranjan says. It is, in the end, the doctor’s decision, although she must also consent to it, unless any delay would endanger her life. The law also doesn’t make any provisions on how many doctors, or what kind, must certify the decision; so presumably, as Dr Baskaran tells Esquire, “The law only requires one medical practitioner, and not necessarily a specialist, to certify in good faith that the pregnancy is detrimental to the mother’s health”—the term “good faith”, which in legalese means a sincere belief, in itself a deference to medical judgement. “But at HKL, we make it a rule that two doctors should agree before going ahead,” he adds, so there is less risk of any oversight—and indeed, Ranjan says, this has developed into a practice within the medical profession, though it’s not a legal requirement.

After a pair of conjoined twins is born, however, and the viability of separation has to be assessed, a paediatric surgeon will step in. At this point, Dr Zakaria runs a few questions through his mind: “First, is it feasible or not to separate the twins? In most cases where they’re conjoined at the heart, it’ll be impossible. So, if it’s impossible to separate them, then the second question is: is it feasible to save one of them?” Even if it means one might die in the process to give the other a fighting chance of survival, and even if the odds of the saved twin living aren’t guaranteed. As Ranjan says, “In medicine, there’s no undertaking by the doctor to provide a desired result.” Luckily, Dr Zakaria says, he has only ever had to make this difficult decision once in his career.

In 2007, a pair of conjoined twins from Kota Kinabalu was brought to his door. “One was relatively normal and the other had all kinds of abnormalities and was smaller than the other,” Dr Zakaria recalls. “We know that sometimes one becomes more dominant, so the other becomes like a parasite. This twin had a major blood supply from the other twin’s heart; so literally, the normal twin was pumping blood to help out the other twin. Also, this twin had a very abnormal face, a maldeveloped brain and severe scoliosis, and one of its upper limbs was abnormal. Their hearts were not joined, they each had a heart—but the abnormal twin’s had holes and abnormal valves. When we saw that, we thought that if we were to separate these twins, both would die soon after the operation.”

“So we counselled the parents that the best option would be to try to save the [stronger] twin. In this case, they agreed,” Dr Zakaria says. “We were also able to use the skin and tissues of the [sacrificed] twin to cover up the defects in the other twin after separation.” Unfortunately, the twin who was “saved” also had a heart problem and suffered with breathing difficulties that were difficult to manage. “The twin was ventilated for a long time, and then the parents decided that they wanted to go back to Sabah. He died at home soon after.”

Malaysian law doesn’t provide for such cases, but as in lawful abortion cases, the practice is that doctors, with their expertise, ultimately make the decision—as long as the parents agree. No official panel deliberates to reach a decision at HKL, Dr Zakaria says. “Basically, I’ll have to make the decision. There are other paediatric surgeons outside HKL, in UM and UKM, so sometimes, I will discuss the case with them.” And it seems to work out for the best, as long as the parents and the doctors think along the same lines. But what if the parents disagree with the doctor’s diagnosis? What happens then? No conflict in such cases has ever been reported in Malaysia before, but Ranjan suggests that, if a case were to come up, English law would be the most persuasive in Malaysian courts.

The landmark case of conjoined twins Jody and Mary, for example, could be illuminative. Fused at the lower ends of their spines and a bladder, they also shared a common aorta. Aside from having a poorly developed brain and abnormal neurological responses, Mary was smaller and weaker than Jodie, and her heart and lungs didn’t function, so her supply of blood was pumped by Jodie’s heart. Their doctors agreed that, if the twins weren’t separated, Jodie’s heart would eventually fail from the pressure of pumping for two, and both twins would die within six months to two years; or, Mary might die, in which case doctors would then have to perform emergency separation—which itself holds inherent risks—to save Jodie. If, however, the twins were separated, Mary would surely die, but Jodie would survive and have a relatively normal life, albeit with some level of disability. So, the dilemma: should doctors separate the twins to attempt to save Jodie’s life, despite that by doing so Mary would die? Or should doctors do nothing, and as a consequence leave both twins to die?

In the end, the doctors decided to try to save Jodie. But their parents, who were devout Roman Catholics, opposed separation, preferring to leave their fate in God’s hands. The hospital then took the case to the English courts to seek a declaration for a lawful separation. It was granted, and the doctors went ahead with the separation. As was anticipated, Mary died in the operating room; Jodie lived. She’s still alive today.

In the end, it’s the fine distinction of a doctor’s state of mind that will decide if his actions are legal or not. “Nobody, not even the mother and a doctor acting in good faith, can agree to ‘sacrifice’ one conjoined twin for the purpose of saving the other, if by doing so there forms the requisite intention or knowledge that makes up part of the criminal offences of murder or culpable homicide,” Ranjan says. However, he clarifies, “If, by the operation, it is not intended, or known, to cause the death of one, or both, then the operation would be lawful.” This might seem like legalistic acrobatics to some, but in law, it’s the intention that matters, and perhaps, it’s a way in which we, as mere humans, can make difficult decisions. So if you intend to save one twin, but the other dies in the process, that may be lawful; but not if you intend to cause the death of one twin in order to let the other live.

Barring these exceptional and high-risk circumstances, though, separation seems to be the norm and the desired outcome wherever possible. “I think in this day and age, the probability of a successful separation is high, so I doubt that if you’re able to separate them that you’d leave them alone,” Dr Zakaria says. “The problem with Muaiman and Muaimin is, if you didn’t separate them, they would never be able to walk,” because their two good legs aren’t enough to support the weight of their horizontal bodies. In our modern world, which prides individuality and every person’s sovereignty over their own body, two is always better than one.

 

 
 
 

The Separation

It started at 7:40am on July 14, 2012 with a team of 60 medical personnel on standby—19 surgeons and anaesthetists, 20 nurses, 20 medical officers.

Dr Zakaria was expecting the next projected 24 hours to proceed mostly as planned. He had spent the previous three months charting out every minute detail and sequence of Muaiman and Muaimin’s separation. “It’s like a rehearsal,” he says. “You decide on the day and the time, the teams that need to be involved at which stage, where they should all be standing in the theatre at any one time, the position of the twins, the complications that may arise and what your backup plans are. It’s like a chess game.”

At the time of separation, Muaiman and Muaimin were a little over a year old—far along enough for their bodies to have developed enough for the operation and for them to have reserves in case of infection. Dr Zakaria had mapped out the internal anatomies of the boys and determined exactly which parts were definitely not joined (the liver and everything above it, spine, kidneys, one leg each), definitely joined (pelvic bones, hindgut bladder, two scrotums, penis, third leg, hindgut with absent anus), and possibly joined (urethra, bladder) using ultrasound studies, CT scans and MRI scans.

One thing, though, pleasantly surprised him during surgery. “Initially, we thought there was only one urethra—the opening where urine comes out. It was during surgery that we saw that there were two urethras. So, in fact, it was like two penises fused into one, rather than one penis,” which made things easier, Dr Za- karia says. “So we literally cut between the two urethras, and ended up with a smaller penis for both boys.”

As for which twin the extra leg should go to, it was a case of deciding whom it belonged to more. Before separation, Azli and Nazilah had asked Dr Zakaria if the leg could somehow be divided so that both twins could have two legs, “but he said that wouldn’t work for either of them,” Azli says, laughing. It had to be one or the other. “We initially planned to give Muaimin the extra lower limb, but in the end, Muaiman got it. You have to look at the anatomy and see, when you separate them, is the limb going to be more functional for this child or the other child?” Dr Zakaria explains. But before getting around to that, he had actually thought about doing away with the third leg, because “arguably, if you have a single good limb [rather than a normal one and an abnormal one], rehabilitation will be easier. Also, you can use the skin and tissues from that limb to cover up the defect where the twins are separated” instead of relying on tissue expanders to stretch their existing skin and tissue.

 
 
 

Muaiman and Muaimin: The Future

It’s a normal, lively household at Azli Sabran’s home in Kluang when we visit: his four young children tumble around on the carpet as they play with each other and alternate between screaming, laughing and whimpering. Three-year-olds Muaiman and Muaimin can get from here to there at speed, relying on their arms and their one good leg to propel themselves forwards. Learning how to move has been intuitive for them, Azli says. However, they can’t really talk yet—they mostly just make sounds—but their mental development isn’t delayed in any way that Dr Zakaria is aware of. “I don’t think it has to do with the fact that they were conjoined. My other kids were slow to talk as well,” Azli adds.

At some point, Muaiman tries to stand by leaning part of his weight on the sofa, eager to show the small audience gathered in his living room that he can. After a few tries, he succeeds, and proudly draws himself up tall. Seeing this, Muaimin edges out of his father’s arms and slides over to his older brother—Azli and Nazilah refer to Muaiman as “abang” because he was taken out of the womb first, and he was the first to be able to hold up his head—and tries to do the same. “My brother can stand, I can too!” Azli says, mimicking what he imagines must be the thought running through Muaimin’s head. But the boy’s going about it the wrong way—he has his back facing the sofa, and he’s trying to pick himself up so that he can lean on it the same way Muaiman is, but the upper part of his body keeps tilting him forward. He keeps at it, over and over and over, until finally, he falls forward and hits his head on the floor. When Nazilah asks him if he wants to try again, he sobs in a rare outburst—“No!” In other situations, too, the twins often seem to take their cues from each other—mostly Muaimin from Muaiman. When one does something, the other wants to follow suit. “They don’t mind if they don’t have the same clothes, but they must have the same toys. Otherwise, they’ll fight,” Nazilah says.

“I think they know that they’re different from other people,” Azli says. “When they see other people stand or walk, they want to walk too. And they really try. Even when we open up their diapers to check on their stomata, they get embarrassed and try to wriggle away.” But their siblings completely accept them, even the normal kids they sometimes play with. “In fact, some of them look at Muaiman and Muaimin and say, ‘Eh, handsome! Why did they become like this? Later, I’m going to scold God! Why did he do this to them when they're so handsome?’” Azli recounts, laughing.

With four kids in the family, the parents make an effort to ensure each child gets their share of individual attention. Muaiman and Muaimin seem more attached to their father, bursting immediately into tears whenever Azli stepped out of the doctor’s room, even just for a moment—during their recent visit to Dr Zakaria to check on the twins’ progress— and were inconsolable even as Nazilah tried to calm them down. It’s because Azli was the one who pretty much took care of them in the early days, commuting between Johor and Kuala Lumpur during their first year and two months at HKL before their separation, Dr Zakaria says. Their mother had to stay home and take care of their two sisters, aged four and two, who still require close attention.

What does the future bode for the twins? It’s not clear yet if they’ll be able to walk properly. Muaiman’s abnormal limb is a confused fusion of two legs, angled such that when he’s standing upright on his strong leg, the other hovers 45 degrees off the ground to his side. The sole of the foot is also curled like a cupped palm, and aside from five toes, he has an extra one that’s longer and separate from the other five, so the foot resembles a claw. In the upcoming months, doctors will look at the possibility of restructuring the leg to increase its functionality.

As for their bodily functions, the boys can now urinate normally, but they still suffer from faecal incontinence. Dr Zakaria says he hasn’t decided whether the boys should be given an anal opening, because if their incontinence continues into adulthood, “it’ll be easier to manage in the abdominal wall. At least, you can put a bag over it and just clean it; otherwise, you’d have to wear diapers.” He adds, “It’s also too early to tell whether they’ll be functional males. That may have to wait until they reach puberty.”

Muaiman and Muaimin are one of the luckier ones. When Nazilah hears the news that heart-conjoined twins Nor Shazwanie and Nor Shazrinie died last year, she’s quiet and bewildered for a moment. “Their mother was on the bed next to mine when I was at HKL. I knew her,” she said. Last the women had spoken, the sisters were still alive; but the mothers haven’t been in contact since Nazilah lost her mobile phone. At least her sons are healthy. She’s grateful for that.

As for the future: how close to normal will their lives be? It’s too soon to tell.