Disturbing Disorders: FOP (Stone Man Syndrome)

F1In a letter dated 14 April 1736, the surgeon John Freke (picture below) wrote to the Royal Society regarding a highly unusual case involving a patient at St Bartholomew’s Hospital in London.

A boy, who looked ‘about Fourteen Years old’, had come into the hospital to ask ‘what should be done to cure him of many large Swellings on his back’. These growths—some of which had started to develop three years prior—were now as large as a ‘penny loaf’. Freke continued with his description of this horrifying condition:

They arise from all the Vertebrae of the Neck, and reach down to the Os Sacrum; they likewise arise from every Rib of his Body, and joining together in all Parts of his Back, as the Ramifications of Coral do, they make as it were, a fixed bony Pair of Bodice.

Freke ended the letter by adding that the boy ‘had no other Symptom of Rickets on any Joint of his Limbs’. [1]

F4What Freke was describing is a rare condition now known as fibrodysplasia ossificans progressiva (FOP). A mutation of the body’s repair mechanism causes fibrous tissue—muscle, tendon and ligament—to ossify when damaged (pictured right: torso of man suffering from FOP). Although FOP is not fatal, most die young, starving to death after their jaws freeze shut or suffocating when new bone develops, making it impossible to breathe.

Freke was the first to describe the condition in detail. However, a French physician by the name of Gui Patin may have come across FOP in the 17th century when he wrote to a colleague that he ‘saw a woman today who finally became hard as wood all over’. [2]

F3Today, FOP affects approximately 3,300 people worldwide, or 1 in 2 million. A more recent example showing the effects of the disease can be found in the Mütter Museum in Philadelphia. In 1938, a 5-year-old boy named Harry Raymond Eastlack broke his leg while playing with his sister. Shortly afterwards, bone growths began to develop on the muscles of the boy’s thigh. Within years, the condition began to spread throughout Harry’s body so that by his mid-20s, his entire vertebrae had fused together. In 1973, Harry died of pneumonia, just four days before his 40th birthday. By that time, his body had completely ossified. Even his jaw locked up, leaving only his lips to move. Before he died, Harry had agreed to donate his body (pictured above, both alive and after death) to the museum for further scientific research, where it continues to be studied today.

Given the rarity of the condition, I was surprised to find an 18th-century skeleton showing the tell-tale signs of FOP in the Hunterian Collection at the Royal College of Surgeons in London. And yet there it was, catalogued simply as RCSH/P 804.


I wondered: whose bones were these? And how did they end up in the hands of the anatomist, John Hunter (below), for whom the collection is named after?

It turns out that when the surgeon, George Hawkins, died suddenly in 1783, he left behind a collection of anatomical specimens that were then auctioned off. Amongst them was the skeleton of an adult male with an excessive number of bony outgrowths on his rather twisted frame. Hunter—always on the lookout for rare and unusual specimens—purchased the skeleton for the extraordinary sum of 85 guineas. [3]

F6Records at the Royal College of Surgeons describe the skeleton as belonging to a 39-year-old man named Mr Jeffs. Hunter’s assistant, William Clift, later reported that he had been told that the skeleton had been buried for seven years before it was procured, presumably by Hawkins. According to Hunter,  this was ‘evident from the state of the softer parts of most of the bones’. [4]

Was this the body of the young boy whom had sought Freke’s help in 1736? If the boy was indeed 14-years-old, as Freke had guessed, that would put his death sometime around 1761. Add to that 7 years before his skeleton was recovered, and it is very possible the specimen now residing in the Hunterian Collection once belonged to the boy described in the letter.

F7Of course, like so many of the specimens residing in medical collections today, we will never be able to confirm the skeleton’s former identity. What I can say with some degree of certainty is that he must have suffered greatly in the 18th century. With no real way to manage his pain, everyday life would have been excruciating. It is also likely that he would have been unable to work at the end of his life. Depending on his financial circumstances, this could have been just as crippling as his debilitating condition.

Today, Mr Jeffs ‘stands’ next to Charles Bryne, the famous Irish Giant (right). Visitors to the Hunterian are often mesmerised by Byrne’s 7’7’’ frame, and rarely cast more than a fleeting glance at the twisted skeletal remains next to him.

Hidden in the shadows of something much bigger than himself, Mr Jeffs is to visitors what he likely was to surgeons in his day: a passing curiosity. His story, however, warrants further attention, for FOP remains incurable. There is still much to be learned from Mr Jeffs and his skeletal remains.

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1. Philosophical Transactions of the Royal Society 41 (January, 1753): pp. 369 – 370.
2. Qtd in Thomas Maeder, ‘A Few Hundred People Turned to Stone,’ The Atlantic (Feb., 1998). I cannot track down the original source for this quote; although I do know that most people incorrectly date this letter to 1692. Gui Patin died in 1672, and his letters were posthumously published in 1692.
3. L. W. Proger & J. Dobson, comps., Descriptive Catalogue of the Pathological Series in the Hunterian Museum of The Royal College of Surgeons of England, vol. 2 (1972), pp. 68-70.
4. Ibid.

15 comments on “Disturbing Disorders: FOP (Stone Man Syndrome)

  1. Sauncea Marlow says:

    After a major car accident on July 4th 1983 I too have been diagnosed with … Stone man disease… It grew in my right hip and groin area but stopped… I have not had a lot of problems from it for years even after having 16 surgeries and kidney cancer in 2009… But as of resent my I have been having problems with breathing… The docs found a nodule in my left lung ( at least that’s what they think it is)… I found s lump in my breast … I have problems getting chocked from eating… This may be related to FOP or not… But let me tell anyone reading this that… God is good… All the time… And … All the time… God is good!!! I thank God for every day I have… What a gift!!! Praise God with me for His loving kindness… 🙏

  2. kittycatpajamas says:

    Thank you for the article on FOP. This may offer a clue to a mystery that interested me. I have some details of an American case of a “stone person” being stolen from their grave in a local cemetery, possibly for sale or exhibition. I have my notes/references tucked away and will gladly dig them out if anyone is interested.

  3. […] forum for a discussion of the real-world analogs to greyscale (including calcinosis, ichthyosis and “Stone Man Syndrome”). And don’t be surprised if we hear more about greyscale in the episodes to […]

  4. […] Disturbing Disorders: FOP (Stone Man Syndrome)  […]

  5. kirizar says:

    What a detailed look at an obscure condition. I wonder at the modern prevalence of such diseases. The life expectancy is likely greater because of what we can do to treat pain and circumvent some of the mechanical problems. But that we cannot address the cause, nor likely improve the quality of life strikes me as incredibly sad.

  6. […] The Chirugeon’s Apprentice: Disturbing Disorders: FOP (Stone Man Syndrome) […]

  7. […] Disturbing Disorders: FOP (Stone Man Syndrome) « The Chirurgeon's Apprentice […]

  8. sandvick says:

    Reblogged this on DailyHistory.org and commented:
    The Chirurgeon’s Apprentice has another article about a disturbing disorder. In this case it is fibrodysplasia ossificans progressiva (aka Stone Man Syndrome). FOP is a rare disease that causes a person’s body to ossify. The article posits that a skeleton in the Hunterian Collection at the Royal College of Surgeons in London is possibly a patient of John Freke whom he first treated in 1736. As the article points out, FOP is still incurable to this day.

  9. It’s true that Bryne’s remains command a larger space, but Jeffs’ corpus is sad and fascinating all at once. I’m glad I got a chance to see both last time in London.

  10. J.B. Lyle says:

    This explains to me at last a family story about a relative who gradually “turned to stone” and died at an early age. This would have been in the American South, probably Mississippi, in the first quarter of the 20th century. Ghastly suffering.

  11. Tragic, but very interesting.

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