banner

 

homebut     gatebut   TS lobby  contact funny

 

 ts lester   ts b&h   ts hand   ts elmer   tscramond  ts bentham

 

TS Merrick  TA GAGE   TS AOKI  ts dan  TS eastlack

  

 

Harry Eastlack: Man of Stone

 

'Man of Stone': sounds rather exciting doesn't it? It implies thrilling, enviable qualities, and super powers, the ability to

fight crime among them. But Harry Eastlack had no fantastic powers. In fact, what he did have was more fantastical (adj

– ludicrously odd).

Harry Raymond Eastlack Junior was born in 1933. Though it isn't recorded, it's highly likely that he was born with

deformed toes – a classic predictor of the disease in question.

 

 

toes

 

When he was five years old, Harry broke his left leg in a rough and tumble play fight with his sister. Doctors followed the

accepted procedure, setting the bone and putting the limb in plaster to support it while it healed. However, it refused to

heal. Harry's hip and knee joint lost mobility and bony growths began developing in his thigh muscles.

 

Harry was exhibiting the first signs of an extremely rare genetic disorder, sometimes referred to as, 'Stone Man

Syndrome'. We'll examine its scientific name and meaning next but it helps to be aware of the barely believable rarity of

this affliction. Unverified medical reports suggest that globally, 2,500 people have been suspected of suffering this

ailment. Some 700 cases have been confirmed.

 

Three Letter Sentence

 

Medical science gives this disease the acronym F.O.P., which is easy to recall and far less esoteric than 'Stone Man

Syndrome'. However, when we take a look at what 'F.O.P.' stands for, we can find an explanation of what the

disease is all about.

 

'F' is for 'fibrodysplasia' (pronounce it fie – bro – diss – play – ze - a), a term meaning abnormal development of

fibrous connective tissue. So, muscles, tendons and ligaments are the structures in the body that are involved.

 

'O' is for 'ossificans' (oss – iffey – cans), a word stemming from 'ossification', which means the formation or

depositing of bone in soft tissue.

 

'P' is for 'progressiva', a term that needs no guidance in pronunciation and little explanation of meaning. This

simply means happening progressively or in stages.

 

Fibrodysplasia Ossificans Progressiva therefore translates to 'The ongoing, abnormal appearance of bone in soft

tissue.' This makes 'Stone man syndrome' at least adequate as a descriptive phrase. Read 'bone' for 'stone' and it

becomes a more accurate descriptor.

 

Typical Development

The hallmark of F.O.P. in an infant is the deformation of the big toes, These may have noticeable lumps or the

joint may be absent. But this is just a harbinger, a warning of future problems.

 

fop girlWhen a foetus, human beings carry a gene that

activates the release of a protein that helps to form

bone as part of the normal immune response. This

protein should stop working once the foetus's bones

are formed, some time before birth. However, in

F.O.P. sufferers, it keeps on working, in places

  where growth is continuing and in places where  injury

has occurred.

 

  

 

fop manThe incorrectly formed bony tissue isn't part of the skeleton,

although it can fuse together with the normal skeletal

structure. The first 'attack' of F.O.P. generally happens before

the individual reaches the age of ten. The continuing formation

of bony tissue typically follows a pattern. Extra bone will

develop at the neck first, before spreading to the shoulders,

arms and chest. The lower extremities are the last to be

affected.

Similarly, the sequence of tissue formation starts around the

head, spine and central portions of the skeleton, moving

onward to the extremities.

It's also important to be aware that injury can also cause

additional bony tissue to develop, at the site of the damage. This clearly happened as a consequence of Harry's leg injury

in 1938. Such is the rarity of F.O.P. that its symptoms may be misdiagnosed as fibrosis (the formation of excess scar

tissue), or even as cancer. In the latter case, tumour-like lumps can be mistaken for growths.

 

Harry's Progress

 

Harry Eastlack's broken leg never knitted properly but the advancement of the F.O.P. didn't end there. As he

aged, Harry's joints began to solidify and his muscles, tendons and ligaments turned into unyielding bony material.

By the time Harry was in his mid-twenties, the 24 articulated vertebrae in his spine had locked solid. The spinal

column lost all its flexibility and the bones were fused together. As the disease progressed, Harry steadily lost

more and more of his mobility.

 

ageprog2

Harry, photographed at various ages, showing how his deformity increasd

 

Harry's life lasted just under forty years. When he died, six days before his 40th birthday, the ossification of his

skeleton had become complete. His lips were the only mobile part of him, since his lower jaw had been rendered

immobile. The cause of death was pneumonia.

harry bef aftSo sad a case didn't go unremembered, especially as

Harry Eastlack was clearly public-spirited. Mindful of

the speed at which the inexorable disease was claiming

his body, Harry spoke to his doctor, bequeathing his

body for the advancement of medical science. The

doctor donated Harry's skeleton to the Mütter Museum,

the museum of The College of Physicians of Philadelphia.

In accordance with Harry's wishes, his rare skeletal

remains are displayed in a glass case. They have been

invaluable for forty years, to scientists and physicians

studying F.O.P. They are likely to remain available for future

generations to study.

 

Solid Evidence

 

When required for display as an anatomical specimen, a human skeleton has to be 're-articulated'. Without its

connective tissues, the skeleton is simply a collection of 206 or so separate bones. Reassembled for display, the

skeleton's missing tissues are replaced with wire and glue.

 

      full skel     back        bowed

 

 

Harry Eastlack's skeleton is a rare specimen because practically all of it has been merged together by F.O.P. The

skeleton stands in a bowed posture displaying the physical burden it carried. Harry himself necessarily stood in

the same way.

 

Close examination shows the extent to which new bony material established itself over Harry's lifetime. Webs of

bone link the normally mobile parts of the skeletal structure. Bony cords tie the spine to the shoulders, and run

from the shoulders to the arms. The skull is secured to the neck and the jaw is attached to the skull. Spicules of

bone head upwards from the pelvis to lock the spine and rib cage together, while the sternum (breastbone) and

ribs are amalgamated similarly.

 

neck   

 

shoulder

 

Studying Harry's skeleton brings home the actuality of F.O.P. far more starkly than any clinical diagram or scan.

The specimen is a harsh illustration of the totality a case of F.O.P. can achieve.

 

Harry's Lesson

 

What has Harry Eastlack's skeleton taught science? From it usual home and at The Second and Third

International FOP Symposia, to which the museum kindly lent it, this specimen has been examined by scientists

and doctors from all over the world.

 

Starting from the earliest problem of his fractured leg, doctors detected that operating on a patient with F.O.P.

can be beset with major problems. Whilst on the operating table, patients could be extremely difficult to intubate.

Breathing could be further complicated by the onset of restrictive pulmonary disease and variations in the

electrical conductivity of the heart muscle could occur. However, smooth muscle tends to remain unaffected. A

heart of stone therefore belongs in the realms of fantasy.

 

Results have verified that post-operative plain sailing is also a myth. Where doctors have mistaken an F.O.P.

growth for a tumor and requested a biopsy, still more bone growth results. Similarly an impact from a fall would

cause bruising in a normal individual. In an F.O.P. patient the bruise will probably turn to bone. So, the surgical

removal of F.O.P. material simply causes it to be replaced as the body attempts to heal itself. Since something as

minor as a vaccination can trigger bone growth, F.O.P. sufferers must be meticulously careful.

 

back

 

 

Future Hopes

 

Just before the turn of this century, scientists made what seemed to be a groundbreaking discovery. An

antiangiogenic substance can be used to interfere with or even destroy the fine blood vessels a tumor needs to

grow. Such a substance might be used in the treatment of cancer.

 

Squalamine, a compound found in Squalas acanthias (the dogfish shark) has this property, particularly in

cartilaginous tissue. Simply put, it prevents bone growth in sharks. Trials of this compound ran between 2002 and

2007 but as of 2010, no further clinical trials concerning F.O.P. have been performed.

 

The bottom line is that there is no cure for F.O.P. but research continues. In the meantime, some palliative

treatments exist to make life easier for F.O.P. patients.

 

Final Analysis

 

Far from just being a medical anomaly, Harry Eastlack – and his remains – are in fact famous. Though this may

have been of little consolation to the Harry in life, somewhere, somehow, his spirit might appreciate the point.

 

                  

 

                                                                                Copyright Gatekeeper 2014