The Elephant Man

Auteur: Gepubliceerd op: 
Medisch

 

At birth, Joseph Carey Merrick (he was named after his father) seemed just an ordinary little boy. Nothing went wrong during childbirth, and the days after he also seemed fine. But it was at the age of only 2 years old, that he started to develop swellings on his lips and a bony lump started to grow on his forehead. Since it was only the 19th century, no one could explain what was happening to poor Joseph, and no one knew how his condition was going to progress. His mother blamed his state on the fact that she was knocked over and frightened by an elephant when she was pregnant with Joseph. In the 19th century, it was a common belief that emotional distress during pregnancy could have disastrous consequences for the baby. 

As Joseph grew, his right arm began to grow bigger than his left and also his feet were greatly enlarged, with patches of loose, greyish skin appearing all over his body. As one could imagine, he had an increasingly complicated life. Being that special education was not only commonly available until the late 20th century, Joseph was sent to a regular school. The only people he could really talk to were his teachers and his mother.

Joseph never had a good relationship with his father, and when his mother died due to bronchopneumonia in 1873, he was forced to work on the streets, selling his father’s products. Because of the tumours, now also appearing in his head, growing in size, he was unable to speak and walk properly. Joseph often frightened people that met him on the streets up till the point where people only stared and made fun of him and was, therefore, unable to do his job. When he got home, he was beaten by his father who would say that he didn’t earn enough money. His father got remarried, and when his stepmother had also started taunting him, he decided to leave home for good.

After moving in with his uncle at age 17, he had to make a living working in a workhouse. He found his work there unbearable and therefore decided to quit in 1884. It was at this point that there was no other option than for him to try and make good use of his condition working for a circus. From here on out, he was referred to as “the Elephant Man, half man, half elephant,” this because of his greyish, elephant-like, skin colour. He had a tough time here as well, as he was constantly harassed by mobs of people, which was such a big problem that he had to wear a cape and veil to conceal his deformities. But there was a different kind of audience he attracted as well: medical doctors and students also went to visit him to research his remarkable condition. Amongst these people was a surgeon named Frederick Treves. After he first saw Joseph, he wrote the following about him: "[he was] the most disgusting specimen of humanity that I had ever seen ... at no time had I met with such a degraded or perverted version of a human being as this lone figure displayed." And, other than doing research, he wanted to have nothing to do with him. Joseph, however, did not want any research to be performed on him as he felt like “an animal in a cattle market.”

When the circus and especially the so-called ‘freak show’ Joseph was in, started attracting violent crowds of people and was, therefore, beginning to raise concern amongst the British people, the circus was forced to move to Belgium. In Belgium however, Joseph was robbed of his savings by the circus’ new owner, and he was left fending for himself. With no one to turn to, Joseph managed to return to Britain but was there also because he was now unable to verbally communicate. After his arrival, he was quickly the centre of attention once again and was arrested by the police. The police brought him back to Frederick Teres since he was the last person known to have been in contact with Joseph. Even though Treves swore he wanted nothing to do with Joseph, the two developed a close relationship, and Treves provided human connection and a place to stay for him in the hospital.

Other than his incurable condition, Joseph was healthy, and therefore the hospital saw no reason to let him stay any longer. It looked like Joseph was going to be back on the streets, but after the chairman of the London Hospital, Carr Gromm, posted a letter in The Times, Josephs condition and ask for help were made public and, out of empathy, enough financial donations were made to provide him with a home and constant care. He was also aided by the British upper class, including the Prince and Princess of Wales. He spent his time writing literature and talking to Treves, who could still understand him. He was also able to go to the theatre every once in a while and was finally able to have regular social interactions again and spend the remainder of his life in reasonable comfort. 

Despite newfound efforts, Joseph’s condition began to deteriorate. On April 11, 1890, Joseph Carey Merrick was found dead in his apartment, lying in his bed with his head on his knees. His head was beginning to grow so big and bulky that he slept sitting up, and thus it was presumed he died due to asphyxiation because of a crushed trachea. But later it was found out that he actually died because of a severed spinal cord that might have happened when his head fell back while sleeping.

Up until this date, it is unknown which disease the elephant man really had, but after studying pictures and the bones of Joseph Merrick, two possible diagnoses were given: neurofibromatosis and Proteus syndrome, or maybe even a combination of the two.

Neurofibromatosis is a condition in which benign tumours appear in nervous tissue, depending on which type of cell develops into the cyst, three types of neurofibromatosis can be classified. The cause of this condition is believed to be genetic. A defect of the NF1 tumour suppressor gene causes the uncontrolled proliferation and differentiation of the aforementioned nervous cells. Unfortunately, there is no known cure for neurofibromatosis and treatment involves ‘regular’ cancer treatment: chemotherapy and surgical removal of the tumours.

The second and more interesting condition Joseph might have had, is the so-called Proteus syndrome. The syndrome is named after the Greek god Proteus, who could change shape. Proteus syndrome is recognised by an overgrowth of bone, muscle, fatty tissues and blood and lymph vessels. The condition is progressive, meaning that, at birth, a child may seem fine, but as time moves on, the patient will develop all kinds of possible symptoms. The most common symptoms are tumours of skin and bone, and they lead to problems relating to speech, mobility and breathing. Since the symptoms are usually severe, patients often die early due to deep vein thrombosis or pulmonary embolism. The causes of Proteus syndrome are thought to be genetic, and multiple genes are believed to be involved, although no real scientific evidence has been found.

Having discussed both conditions, one can see the similarities in the case of Joseph Merrick. It was thought, that both his mother and his sister also had physical disabilities, although they were less severe. This may point to a genetic cause of Joseph’s problems. Being there is such little information about Joseph Merrick, we might never know what was really wrong with him, and maybe that’s why the case of ‘the Elephant Man’ is such a mysterious and interesting one.