CW: medical anomalies involving kids.  You know I never shy away, but it’s October, so the glove are coming off today!


On Easter Monday morning, in the village of Biddenden in Kent, tea, cheese and loves of bread are handed out to widows and old folks from the window of the Old Workhouse, along with Biddenden Cakes.  These simple flour and water biscuits are like bland gingerbread men, only they are girls, and in the shape of two girls tuck together, the Biddenden Maids. My name’s..

 

If you remember from last week’s episode on twins, identical twins come from a fertilized egg that splits.  If the zygote splits most of the way, but not all, it results in conjoined twins. Or if the zygotes collide and fuse, science isn’t really sure.  Thus conjoined twins are always identical, meaning the same gender. Why am I pointing that out? I met two moms of twins at the She PodcastsLive conference who regularly have people ask them if their identical twins are the same gender.  This is why we need sex ed in school. You’ll also notice I’m not using the term Siamese twins. That term comes from Chang & Eng Bunker, who were born in Siam, modern day Thailand, in 1811, connected by a band of tissue at the chest. It’s not offensive per e, but just doesn’t apply to anyone not born in Siam, so people have stopped using it.

 

Conjoined twins occur once every 2-500,000 live births, according to the University of Minnesota.

About 70% of conjoined twins are female, though I couldn’t find a reason or theory why.  40 to 60% of these births are delivered stillborn, with 35% surviving only one day. The overall survival rate is less than 1 in 4.  Often, one twin will have birth defects that are not conducive to life and can endanger the stronger twin.

 

Conjoined twins are physically connected to one another at some point on their bodies, and are referred to by that place of joining.  Brace yourself while I wallow in my medical Latin. The most common conjoinments are thoracopagus (heart, liver, intestine), omphalopagus (liver, biliary tree, intestine), pygopagus (spine, rectum, genitourinary tract), ischiopagus (pelvis, liver, intestine, genitourinary tract), and craniopagus (brain, meninges).  75% are joined at the chest or upper abdomen, 23% are joined at the hips, legs or genitalia, 2% are joined at the head.  

 

If the twins have separate organs, chances for separation surgery are markedly better than if they share the organs.  As a rule, conjoined twins that share a heart cannot be separated. Worldwide, only about 250 separation surgeries have been successful, meaning at least one twin survived over the long term, according to the American Pediatric Surgical Association. The surgical separation success rate has improved over the years, and about 75 percent of surgical separations result in at least one twin surviving.  The process begins long before the procedure, with tests and scans, as well as tissue expanders, balloons inserted under the skin and slowly filled with saline or air to stretch the skin, so there will be enough skin to cover the area where the other twin’s body used to be. It requires a whole hospital full of specialties to separate conjoined twins, from general surgeons, plastic and reconstructive surgeons, neurosurgeons, neonatologists, cardiologists, advanced practice nurses, and maternal-fetal medicine specialists, among others.  In fact, the longest surgery of all time was a conjoined twin separation. Separation surgeries often last an entire day; this one required 103 hours. If they started at 8am Monday, the team finished the surgery at 3pm Thursday. In 2001, a team of 20 doctors at Singapore General Hospital worked in shifts to separate Ganga and Jamuna Shrestha, 11-month-old twins conjoined at the head. Not only did the girls share a cranial cavity, their brains were partially fused. Each tiny brain had hundreds of bitty blood vessels, each of which had to be traced and identified as belonging to one or the other of the girls.  Their brains were not only connected, they were wrapped around each other like a helix. Plus, each twin’s skull needed to be reshaped and added to, using a blend of bone material and Gore-Tex fibers. Both babies survived the surgery. Sadly, Ganga died of meningitis at age 7, but Jamuna has gone on to live a healthy life and attend school.

 

We interrupt this podcast script for an exciting article.  Meaning I was almost done writing it, then I found something I had to go back and include.  There was another pair of conjoined twins named Ganga and Jamuna, this pair born in 1970 in West Bengal.  The pairing of the names makes sense when you learn that the Ganga and Jamuna are sacred rivers. The sisters are ischio-omphalopagus tripus, meaning joined at the abdomen and pelvis.  They have two hearts and four arms, but share a set of kidneys, a liver and a single reproductive tract. Between then they have three legs, the third being a nine-toed fusion of two legs, which was non-functional and they kept that one under their clothing.  They can stand, but they cannot walk and crawl on their hands and feet, earning them the show name “The Spider Girls”. Managed by their uncle while on the road with the Dreamland Circus, they exhibit themselves by lying on a charpoy bed, talking to the spectators who come to look at them.  They earned a good living, making about $6/hr, compared to the average wage in India of $.40.

 

Ganga and Jamuna have two ration cards for subsidized grain, though they eat from the same plate.  They cast two votes, but were refused a joint bank account. They also share a husband, Gadadhar, a carnival worker who is twenty years their senior.  When asked which he loves more, Gadadhar replies, “I love both equally.” In 1993, the twins had a daughter via Caesarean section, but the baby only lived a few hours.  Though the sister would like to have children, doctors fear that pregnancy would endanger their lives. Doctors have offered them separation surgery, but they’re not interested.  They feel it would be against God’s will, be too great of a risk, and put them out of a job. “We are happy as we are. The family will starve if we are separated.”

 

In january 1950, a mother in Moscow labored for two days and two nights before being taken to the hospital.  Doctors performed a c-section and delivered a pair of girls. They had twenty fingers between them, but only ten toes.  Masha and Dasha Krivoshlyopova shared a set of legs and a pelvis, their torsos branching off from it at a 90deg angle. The doctors saw in the twins a unique opportunity.  When their mother recovered from anesthesia, she was told that the babies had been unable to breathe and died. Masha and Dasha were taken to the Academy of Medical Sciences Pediatric Institute begin years of experimentation, helmed by Soviet physiologist Pyotr Anokhin.  

 

The girls shared a blood system but had separate nervous systems – so were seen as ideal subjects for research.  Much like twin-obsessed Josef Mengele during WWII, the Soviet scientists used the girls to compare and contrast. Kept in a cot in a glass box next to a laboratory, scientists used the twins to determine the effects of extreme temperature change, sleep deprivation and hunger.  They were burned to see if the other twin felt pain, dunked in ice water to see how it affected the core temperature of the other twin’ torso, forced to stay awake, starved, and electrocuted to test their conditional reflexes. Despite their shared blood, one could get sick while the other would be fine.  At age 6, Dasha and Masha were transferred to the Central Scientific Research Institute of Traumatology and Orthopedics, where they would be kept for eight years before being moved to a boarding school for children with motor-impairment in southern Russia. Each twin controlled one leg, making walking difficult, even with turdy crutches to support each torso.  They did not understand what “conjoined” meant; they thought that they have been born separate and then fused together.

 

The twins were institutionalised, unknown to anyone but the staff, for much of their lives but their horrific story has now been revealed in full for the first time by journalist Juliet Butler.  Butler befriended Masha and Dasha and found some fascinating things about them that the doctors didn’t study. The girls seems largely unaware of what they had gone through, possibly from having repressed it.  They didn’t remember doctor burning them, but Dasha remembered a nurse bringing her a toy. Despite sharing the same genes, a pair of legs, and the same horrific childhood, the sisters had wildly different personalities.  Dasha was gentle and kind, while Masha was mean, controlling, and emotionally abusive to her sister. In my mind, to keep them straight, I think of them as Mean Masha and Dear Dasha.

 

Masha drank to excess, which meant Dasha also got drunk whether she wanted to or not.  Dasha met a boy and fell in love, and the boy loved her back, but Masha wouldn’t allow it.  Masha forced Dasha to dress and cut her hair in a masculine style, because that’s what Masha wanted.

 

‘I’ve no doubt at all that Masha was a psychopath – she ticked all the boxes,” Butler said in an interview.  “Dasha was in an emotionally abusive relationship – similar to the situation some people find themselves in with a partner.  But while those people have a chance to leave, Dasha physically couldn’t.”

 

Dasha wanted a normal life, as normal a life as they could have.  She got a job putting the squeeze bulbs on pipettes. While she worked, Dasha would smoke and read magazines.  The twins’ mother found out that her babies hadn’t died and she began to visit them. After a few years, Masha broke off that relationship, too.  With medical advances, many doctors offered over the years to separate the girls, but were declined – by Masha.  

 

“Masha denied Dasha everything she ever longed for – a chance of love, a relationship with their mother, a job and even what she wanted most: a separate body.” 

 

In 1988, the twins went on a TV talk show to make a public appeal to be allowed to leave captivity.  Their pleas were successful and they moved to a Home for Veterans of Labour where living conditions were great improved and they were able to buy themselves luxuries like a television set, an Atari, a cassette player and a computer, thanks to charitable donations.

 

As the years progress and surgical procedures improved, doctors continued to offer Masha and Dasha separation surgery.  Masha always declined for both of them. In April, 2003, at age 53, Masha died of a heart attack. Doctors offered Dasha separation, but this time she refused of her own volition.  17 hours later, due to blood poisoning from the decomposition toxins being circulated into her body from Masha’s, Dasha passed away.

 

Masha and Dasha were both fairly developed, almost fully formed, but what would happen if one twin was not?  Such is the parasitic twin. This can occur when one of the fetuses is partially absorbed by the other in early pregnancy.  The partially absorbed fetus stops developing and becomes parasitic. The other twin continues to develop normally and the autosite, or the thing that supports the parasite.  Many cases of people and animals with extra limbs are examples of parasitic twins. One of the most interesting recent cases was that of Lakshmi Tatma of Araria, India. Lakshmi was born with four arms and four legs, a set of each growing from a torso attached to her torso.   Her namesake, the goddess of wealth Lakshmi, has four arms and baby Lakshmi was born during a festival to celebrate goddess Lakshmi in 2005.

 

Word of the many-limbed girl spread across India by the tie Lakshmi was 2.  People would pimagrage to worship her. But attention is a double-edged sword.  People kept coming and coming. A man from a circus asked to buy her. Lakshmi’s parent, poor laborer, had to move the family into hiding.  The parasitic twin was also taking a toll on Lakshmi’s health. The “girls” were fused at the pelvis, the bones forming a circle. Lakshmi was too little to move two bodies and didn’t walk because of a club foot, so the parasitic twin was prone to pressure sores, which would get infected and sicken Lakshmi.  (I can’t quite bring myself to call the incomplete twin “the parasite,” even though that’s the term used in the surgical report. Maybe because Lakshmi was soo cute).  

 

There was no way Lakshmi’s parents could afford even a fraction of her separation surgery, which was estimated to cost $625,000.  Thankfully, the full amount was raised by the charitable arm of the Sparsh Hospital in Bangalore, where teams of doctors worked in shifts for 27 hours.  Lakshmi and the parasitic twin each had one functional kidney, so parasite’s kidney was transplanted into Lakshmi, as were the bladder and uterus. Lakshmi would need more surgeries to bring her legs closer together and build her pelvic floor muscles; she also got her clubfoot fixed.  Lakshmi needed to wear a large brace to correct scoliosis, an incorrect curvature of the spine, and help bring her legs into the right position, but her parents wouldn’t enforce her wearing it because it hurt her, and that’s the last I could find of her case. Soc med CTA if you find anything.

 

Not all parasitic twins are as obvious as a torso with arms and legs.  The condition is called fetus in fetu, a parasitic twin developing or having been absorbed by the autosite twin.  It’s extremely rare, occurring only once in every 500,000 births and twice as likely to happen in a male. The question of how a parasitic twin might develop is one that currently has no answer.  To say the fetuses in question are only partially developed is still overstating thing. They are usually little more than a ball of tissues with perhaps one or two recognizable body parts. One school of thought holds that fetus in fetu is a complete misnomer.  Adherents contend that the alien tissue is not in fact a fetus at all, but a form of tumor, a teratoma, specifically. A teratoma, also known as a dermoid cyst, is a sort of highly advanced tumor that can develop human skin, sweat glands, hair, and even teeth. Some believe that, left long enough, a teratoma could become advanced enough to develop primitive organs.

 

There have only been about 90 verified cases in the medical record.  One reason fetus in fetu is rare is that the condition is antithetical to full-term development.  Usually, both twins die in utero from the strain of sharing a placenta. Take 7 year old Alamjan Nematilaev of Kazakstan, who reported to his family abdominal pain and a feeling that something was moving inside him.  His doctors thought he had a large cyst that needed to be removed. Once they got in there, though, doctors discovered one of the most developed cases of fetus in fetu ever seen. Alamjan’s fetus had a head, four limbs, hands, fingernails, hair and a human if badly misshapen face. 

 

Fetus in fetu, when it is discovered, is usually found in children, but one man lived 36 years, carrying his fetal twin in his abdomen.  Sanju Bhagat lived his whole life with a bulging stomach, constantly ridiculed by people in his village for looking nine months pregnant.  Little did they know, eh? Fetus in fetu is usually discovered after the parasitic twin grows so large that it causes discomfort to the host.  In Bhagat’s case, he began having trouble breathing because the mass was pushing against his diaphragm. In June of 1999, Bhagat was rushed to Tata Memorial Hospital in Mumbai, India for emergency surgery.  According to Dr. Ajay Mehta, “Basically, the tumor was so big that it was pressing on his diaphragm and that’s why he was very breathless. Because of the sheer size of the tumor, it makes it difficult [to operate]. We anticipated a lot of problems.”

 

While operating on Bhagat, Mehta saw something he had never encountered.  The squeamish may wish to jump30 and think about kittens, though if you’ve made it this far, you’re cut from strong cloth.  As the doctor cut deeper into Bhagat’s stomach, gallons of fluid spilled out. “To my surprise and horror, I could shake hands with somebody inside,” he said. “It was a bit shocking for me.”

 

One unnamed doctor interviewed in the ABC News story described what she saw that day in the operating room:  “[The surgeon] just put his hand inside and he said there are a lot of bones inside,” she said. “First, one limb came out, then another limb came out. Then some part of genitalia, then some part of hair, some limbs, jaws, limbs, hair.”  There was no placenta inside Bhagat — the enveloped parasitic twin had connected directly to Bhagat’s blood supply. Right after the surgery, Bhagat’s pain and inability to breathe disappeared and he recovered immediately. Upon recovery from the surgery, in which his twin was removed, Bhagat immediately felt better. But he says that villagers still tease him about it.

 

Okay, I’m back at my desk after four days and two 8 hour drives that each lasted 10 hours.  I love you, Atlanta, but your traffic can go [expletive deleted].

 

There is a rather famous case of a parasitic twin, even more famous than the four-legged girl Myrtle Corbin. …  You don’t know about Myrtle Corbin? What about the conjoined twins who billed themselves as a single person, Millie Chritine?  Well, don’t fret they’re the subject of the bonus mini episode next week on patreon.com/ybof etc your two and fews help

 

The story I was referring to was made into a plot point on AHS:FS, the tale of Edward Mordrake, the man with two faces.  In 1895, The Boston Post published an article titled “The Wonders of Modern Science” that presented astonished readers with reports from the Royal Scientific Society documenting the existence of “marvels and monsters” hitherto believed imaginary.

 

Edward Mordrake was a handsome, intelligent English nobleman with a talent for music and a peerage to inherit.  But there was a catch. With all his blessings came a terrible curse. Opposite his handsome was, was a grotesque face on the back of his head.  Edward Mordrake was constantly plagued by his “devil twin,” which kept him up all night whispering “such things as they only speak of in hell.” He begged his doctors to remove the face, but they didn’t dare try.  He asked them to simply bash the evil face in, anything to silence it. It was never heard by anyone else, but it whispered to Edward all night, a dark passenger that could never be satisfied. At age 23, after living in seclusion for years, Edward Mordrake committed suicide, leaving behind a note ordering the evil face be destroyed after his death, “lest it continues its dreadful whispering in my grave.”

 

This macabre story …is just that, a story, a regular old work of fiction.  “But, but, I’ve seen a photograph of him.” Sadly, no. You’ve seen a photo of a wax model of the legendary head, Madame Toussad style.  Don’t feel bad that you were convinced. The description of the cursed nobleman was so widely accepted that his condition appeared in an 1896 medical encyclopedia, co-authored by two respected physicians.  Since they recounted the original newspaper story in full without any additional details, gave an added air of authority to Mordrake’s tale.

 

“No, there’s a picture of his mummified head on a stand.”  I hate to puncture your dreams, but that’s papier mache. It looks great, but the artist who made it has gone on record stating it was created entirely for entertainment purposes.  If you were to look at that newspaper account of Mordrake, it would fall apart immediately. “One of the weirdest as well as most melancholy stories of human deformity is that of Edward Mordake, said to have been heir to one of the noblest peerages in England. He never claimed the title, however, and committed suicide in his twenty-third year. He lived in complete seclusion, refusing the visits even of the members of his own family. He was a young man of fine attainments, a profound scholar, and a musician of rare ability. His figure was remarkable for its grace, and his face – that is to say, his natural face – was that of Antinous. But upon the back of his head was another face, that of a beautiful girl, ‘lovely as a dream, hideous as a devil.’”  What did we say at the top? Conjoined twins are identical, meaning among other things, the same gender.

 

But more than one face on one head *can happen though it is exceedingly rare, with fewer than fifty cases documented since 1864.  The condition is called craniofacial duplication or diprosopus, Greek for two-faced. The degree of the condition can vary from a split nose with wide-set eyes to a second complete face.  Continuing in the theme, the cause of this deformity aren’t fully understood, although many researchers believe it to be another rare form of conjoined twinning. Fetuses with diprosopus often also lack brains or, if the brain is formed, it may have duplicate structures so most infants with diprosopus are stillborn.  Most, but not all. There are cases like Hope and Faith Howie of Australia who lived for 19 days and Lali Singh of India, who lived for two months. The people in Lali’s village believes she is the return of the Hindu goddess of valor, Durga, a fiery deity traditionally depicted with three eyes and many arms. Except for her ears, of which she had two and her cheeks, of which she has three, all of Lali’s facial features are doubled.  Hundreds of people travelled to their village east of New Delhi to touch Lali’s feet and leave offerings. A temple has been built in her honor with the donations.

 

Then there’s Tres Johnson of Missouri.  He could be described as having two partial faces, as if his body were just beginning to split from the top down, but had only gotten as far as his mouth.  Doctors didn’t hold out much hope for newborn Tres, who cleft pallet extended all the way to his sinus, which was open to the outside, but his parents were determined to fight for him.  It took a dozen incorrect diagnoses before the doctors realized he had diprosopus. At every stage of his development, Tres’ parents were told he would not live much longer. But Tres kept right on defiantly living, even after multiple surgeries on his skull and with epilepsy that caused him to have dozens of seizures a day.  Those were made manageable by medical marrijuana and Tres is now 15 years old.

 

And that’s where we…  but I need to finish telling you about the Biddenden Maids.  Mary and Eliza Chulkhurst were born to an affluent family in the year 1100, joined at the hips and shoulders.  They were naturally good friends, but prone to quarrels that could break out in fisticuffs. At age 34, Mary took ill and died suddenly.  Doctors proposed to separate Eliza from her sister’s corpse, but she refused, saying, “As we came together we will also go together”, and passed later that day.  In their joint will, the Biddenden Maids left 20 acres of land to the church, the rent from which was to provide for the poor in the village. Remember, sources…Thanks…

 

Sources:

https://www.dailymail.co.uk/health/article-4779058/Kind-sweet-twin-conjoined-life-psychopath-sister.html

https://www.mirror.co.uk/news/real-life-stories/one-smoked-drank-longed-husband-10964102

https://www.chop.edu/conditions-diseases/conjoined-twins

https://www.popularmechanics.com/science/health/a20571/longest-surgery-of-all-time/

https://www.rnfa.org/february-2016-conjoined-twins-separation-surgery/

https://en.wikipedia.org/wiki/Lakshmi_Tatma

https://www.twin-pregnancy-and-beyond.com/fetus-in-fetu.html

https://www.theguardian.com/science/2003/jul/17/thisweekssciencequestions3

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3996905/

https://scienceline.org/2006/09/blog-klein-fetus/

https://abcnews.go.com/Health/man-twin-living-inside-medical-mystery-classic/story?id=2346476

https://www.snopes.com/fact-check/edward-mordrake/

https://allthatsinteresting.com/edward-mordrake

https://www.documentingreality.com/forum/f149/human-spider-14921/

https://www.express.co.uk/news/world/117686/Circus-twins-refuse-to-be-parted

http://www.nbcnews.com/id/24012024/ns/health-childrens_health/t/baby-faces-born-north-india/#.XaKJZUZKjIV

https://embryo.asu.edu/pages/diprosopus-craniofacial-duplication

https://www.thesun.co.uk/living/3734564/boy-born-with-two-faces-celebrates-13th-birthday/