Reflection During a Day of Skeletal Processing

I have a day off from my normal job and I find myself carefully wet sieving the cremated remains of a suspected Romano-British individual in the processing room at the local unit, but I’m not alone here.  Instead I’m surrounded by recently excavated Anglo-Saxon remains drying slowly on paper towels, organised in numerous plastic trays on various shelves to my side and up above me.  In each tray there is a plastic zip bag, the site code and context number inked on for identification purpose and later site reconstruction.  By taking the right femoral head and neck (upper thigh) as an identifier of the minimum number of individuals (MNI), I count at least six individuals represented in the new assemblage, although there are a few trays I cannot quite see and as I am not here to look at them I do not uncover them.  A quick look at the morphology (size and shape) of the individual skeletal elements is enough to see that, demographically speaking, adults and non-adults are represented in the assemblage.

Browsing the mandibles (lower jaw) that are present I can see a few without the 3rd molar fully erupted, one or two lying in crypts waiting to reach up for the shaft of light from the outside world that would never come.  Another mandible has the majority of the teeth present, including the 1st, 2nd and 3rd molars in each half, but it displays severe enamel wear of the crowns of the teeth (the occlusal or biting surface).  This is indicative of a rough diet and probable middle to advanced adult age.  The fact that most of the teeth are present suggest that the individual wasn’t too old though, as tooth loss is strongly correlated to increasing age for humans.

A day in the archive stores analysing non-adult skeletal remains from an archaeological site. Photograph by the author use a Pentax ME Super camera and Lomography Lady Grey film, if used elsewhere please inform the author and credit as appropriate.

I turn my attention back to the cremated remains.  These are something of a mystery having looked at the context sheets dating from the excavation itself.  There is evidence for cremated non-human remains, likely to be bovine (cow to you and me) as there are a few distinctive teeth included in the bags in an associated context found near the cremated remains that I’m now processing, which itself has been bulk sampled at 100%.  A proper look through the sieved cremated material, which has been processed in accordance with the British Association of Biological Anthropology and Osteoarchaeology guidelines, will have to wait though as they need to dry over the next few days, ideally for another few days after too.  Once dry I can go through each fraction sieved (10mm, 6mm, 2mm) and sort as human and non-human, before identifying specific osteological features and assigning the fragments to either skull, limb, or trunk sections of the skeleton.

As I think about this I remember that I must complete this human osteology report soon.

For many people the thought of touching or analyzing human remains is too much, that in many minds remains are parceled off to the medical realm or are hurried to the cemetery to be removed out of sight.  In reality though we are often surrounded by human remains, though we may not always know it and may not always want to know it.  In archaeology the skeletal remains of humans are often the only direct biological matter to survive of individuals and past populations.  They can encode and preserve a lot of information on biological matters and past cultural practices.  This has been steadily recognized within the past century as osteological methodologies are refined for accuracy and new technology is applied in novel approaches to the remains unearthed.  One of the prime concerns for any bioarchaeologist or human osteologist is that ethical codes and guidelines are adhered to, with the relevant legal permits acquired as appropriate.  As I glance upon the presumed Anglo-Saxon remains I remember that these too were unexpected finds by the construction workers, I briefly wonder how they felt and what they thought on seeing them for the first time.

Anyhow, back to processing the cremation and to thinking about writing the report.

It is pretty interesting as although I’ve part-processed cremations within urns before, with careful micro-excavation spit by spit, I’ve never fully processed a cremation to completion.  Whether these cremated remains represent human skeletal material, as the field notes state, remains a different matter though and it is one I am eager to solve…

Further Learning

• The British Association of Biological Anthropology and Osteoarchaeology (BABAO) promotes the study of understanding the ‘physical development of the human species from the past to the present’.  As an association they provide research grants for projects in which all members of BABAO are eligible, as well as offering prizes for presentations and posters in their annual conference, which is held in the United Kingdom.  I fully recommend attending and taking part if you are associated with any relevant field.

Brief Updates: Archaeological Desks & Palaeoanthropology

The archaeologist Robert M Chapple has recently done something a bit special to celebrate his 100th post over at his blog.  In a thoughtful and entertaining entry Robert discusses the writing and thinking space of the humble desk, that much maligned friend of the archaeologist.  Indeed when a person thinks of an archaeologist the first thing that pops into a person’s head is the excitement of fieldwork in far-flung countries, a trowel perhaps, maybe some bones or Indiana Jones cracking his whip.  It is rarely the vital tool that is the desk, a space in which to hunker down, study site reports, books and process the archaeological record properly over a hot cup of tea, that pops into the minds of people asked to think about archaeology.

Yet the desk is where the action happens!  This is where the hard work of the amalgamation of knowledge happens, where the fieldwork is fleshed with the existing archive and the site is put within a larger context.  Interpretations are made and broken on the humble desk.  So Robert, recognising this vital space of thought and action, also saw it as a deeply personal space for the individual.  As such he asked a wide variety of his archaeological friends to send their own photographs of their desks for his 100th blog entry.  And it is a lovely entry, displaying both academic desks and personal spaces.  I was also asked to join in and you can see my little bedside table from which I am writing this now!  Although my work area is pretty bare compared to the desks (and fantastic 2 or 3 screen adapted computers) on show here, I got a serious longing for the university library where I carried out the majority of my dissertation research.

In other news I have produced a small article for the Teesside Archaeology Society TEESCAPES magazine.  I was kindly asked to write for them by my good friend Spencer Carter, who is the edited of the magazine and a specialist in studying and understanding the context of prehistoric microlithics.  Spencer is currently researching the Mesolithic period of northern England and his fantastic Microburin site, which documents his research and outreach work, can be found here.  My article, which was published in the 2014 Spring Edition of TEESSCAPES, focuses on the amazing palaeoanthropological highlights of 2013 and specifically mentions the Georgian site of the Homo erectus finds at Dmanisi (1), the Spanish site of Sima de la Huesos, and the Rising Star South African project.  It is an informal look back on year of research and excavations that bought much to the table in terms of our of knowledge of understanding human evolution.  (I may also have sneaked in an Alan Partridge joke).

A great Spring 2014 edition of TEESSCAPES by the Teesside Archaeological Society with articles on a variety of topics including, but not limited to, history and archaeology in the national curriculum, the Mesolithic forests of the coast of NE England, museum reviews, Streethouse before the Saxons and human evolution. There are also field notes and books reviews. Read more about the editor’s views, Spencer Carter, in his enlightening blog on post on publishing and editing archaeology journals and open access in archaeology over at Microburin here.

I’ve tried to frame the article within a basic introduction to palaeoanthropology, some of the major new techniques being used in the study of past populations and some of the problems in trying to understand the fossil record and of human evolution in general.  It is a short article but I have to say I am very impressed by the presentation of the article, so a big thank you Spence!  I hope to start producing articles for TAS as and when I can, but this aside I would urge any reader to check it out and to check out any local archaeology societies or companies near to you.  They really are a wealth of original research and really help you get to grips with what is going on in your region and further abroad.  My own article also includes a cheeky photography of me in a lab coat which is sadly, at the moment, a rare occasion.  If you are an archaeologist, a student archaeologist or someone who just manages to engage in their passion between sleep and work then I heartily recommend jumping in and writing for your local society!

Notes

(1).   The article is a review of the amazing palaeoanthropological finds and research of 2013 and as such is likely to become out of touch with the passing of years, as new research highlights new evidence or different perspectives are investigated, hypothesized and studied in-depth.  A good example of this is the fairly recent claim that the Dmanisi individuals, discussed in my article, could possibly (but unlikely) represent different lineages of hominin species (check out Jamie Kendrick’s site The Human Story for more information on this issue and for in-depth entries on human evolution in general).

Further Information

• Learn about the Teesside Archaeology Society here.
• Current and past editions of TEESSCAPES can be found here.
• Robert M Chapple’s awesome blog can be found and read here.
• Spencer Carter’s fantastic Microburin site can be read here.

An Introduction to Fibrous Dysplasia & McCune-Albright Syndrome

Definition of Fibrous Dysplasia: ‘Fibrous dysplasia is a non-inherited metabolic bone disease in which abnormal differentiation of osteoblast maturation (which) leads to replacement of normal marrow and cancellous bone by immature bone and fibrous stroma’ (Fitzpatrick et al 2004: 1389).  Fibrous Dsyplasia (FD) can be described as either monostotic (one) or polyostotic (many), depending on how many bones are affected by the disease.  Fibrous Dysplasia lesions are often displayed as having a ‘ground glass‘ appearance on x-rays and are a distinctive radiographic feature of the disease, although it is not pathognomonic of it (Waldron 2009).  It is also noted that pathological fractures are a key defining feature of polyostotic Fibrous Dysplasia (Marsland & Kapoor 2008).  FD is described as a rare disease, with the monostotic form being more prevalent than the polyostotic form.

Definition of McCune-Albright Syndrome:  McCune-Albright Syndrome (MAS) was originally typically diagnosed and recognised when a person had any of the two of the triad of the following symptoms: polyostotic Fibrous Dysplasia, Cafe-au-lait marks and/or precocious puberty.  However it was later recognised that ‘endocrinopathies, including hyperthyroidism, growth hormone excess, renal phosphate wasting with or without rickets/osteomalacia, and Cushing Syndrome’  could be found in association with the original triad (Dumitrescu & Collins 2008: 1).  In all three systems (skin, skeletal & endocrine), the presentation and abnormality can be highly variable from person to person depending on the tissues involved and the extent of the involvement (OMIM-see below).  Estimated prevalence is 1/100,000 to 1/1,000,000, it is such a wide margin because no thorough prevalence study has been carried out in recent times (Dumitrescu & Collins 2008: 1).

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As a person who happens to have McCune Albright syndrome, to have known to have it from the first years of life, I have become somewhat forgetful of its origin: that somewhere in the early postzygotic  divisions of my life, the disease appeared and became a part of me.  Although I am aware each day of the ramifications that the mutation of the GNAS1 gene has caused I often consider myself lucky.  Lucky in the fact that in my case it has only led to broken bones and various surgeries rather than the full expression of the endocrinopathies that can occur.  I use a wheelchair for everyday mobility with limited use of crutches, mostly used for aiding inside mobility (and sometimes excavations!).

In my personal case, the disease has most affected the main weight-bearing bones of the lower limbs (fairly typical as they are the stress bearing bones, prone to fracture from weakened bone architecture).  Generally speaking,the long bones of the appendicular skeleton tend to be bowed naturally with a pathological weakness due to the lack of normal bone density and high bone cell turnover, with the aforementioned bone lesions occurring spontaneously which sometimes lead to fracture.  This includes the bilateral deformity of the femora with which I’ve had numerous pathological fractures (Five natural transverse fractures, five elective surgery initiated) on both the left and right sides, alongside a number of fractures of the right tibia and fibula (including both transverse and hairline fractures), two on the right humerus and the 5th metatarsal in the right foot.  The shepherd’s crook deformity is the common bowing deformity with varus angulation of the proximal femur (Fitzpatrick et al 2004: online).

As stated the primary bones affected by the MAS pathological fractures are typically located in the appendicular skeleton and include the following bones in order of prevalence first:  a) femur, b) tibia, c) fibula, d) humerus and e) the ribs.  It can also affect the craniofacial skeleton with distinct abnormalities in the amount of bone growth and deformity; however this tends to lessen with age after the primary and secondary growth periods (adolescence and sexual maturity), or ‘burn out’ as it is often called by medical specialists (Dumitrescu & Collins 2008: 8).

‘An example of the shepherds crook’ deformity of the femoral neck (coxa vara) with internal fixation.

My experiences of living with McCune Albright syndrome has included numerous hospitalizations due to fractured bones and planned corrective surgeries.  This has also included large amounts of time stuck in my old friend the Thomas Splint in bed bound traction, alongside enduring a host of various corrective surgical procedures to improve the angulation of both femoral necks.  Although the initial idea following a number of fractures was to treat the femoral deformities with an Ilizarov apparatus by manipulating the bone growth every day, it was quickly decided that an intramedullary rod (nicknamed the Sheffield Rod), carried out in conjunction with osteotomies to correct the femoral neck angle during surgery, would be a much safer and further reaching goal in stabilising both femoral necks in the long term.  (A rather wonderful digital video of a rod being inserted/hammered in can be viewed here).  Five major elective intramedullary rod surgeries later (3 for the left femur and 2 for the right femur!), and it seems as if they have thus far stabilised each femoral shaft/neck enough for them not to fracture again.  However this is also due to using the wheelchair much more extensively than before!

I also have had surgery to stabilise the right tibia and fibula.  This was decided after having undergone three accidental fractures of the right tibia and fibula with a space of 5 years (when the tibia breaks the fibula often follows because of their connection via the interosseous membrane), with each fracture requiring many months in plaster in order for the bone to heal.  Again this surgery included osteotomies of the tibia and fibula to improve the angle of the bone (and thus improve the bio-mechanical loading of the lower leg) and included the fixation of the tibia by means of a titanium plate.  It was hoped that an intramedullary rod could be inserted into the tibia after the tibial osteotomy but the risk of massive blood loss (an outcome of the porous bone and increased heartbeat/blood flow) and the presence of porous cortical bone meant that the tibia was probably unlikely to be able to ‘hold’ the rod in place.

I have also fractured the right humerus twice, with the second transverse fracture resulting in the fixation of the humerus with a permanent titanium plate and associated screws.  This is similar to my right tibia which has a permanent titanium plate and screws to fixate the bone and alleviate some of the pressure of walking.

I have undertaken treatment using biphosphonphates (in my case the drug pamidronate) to increase the bone density itself over a number of years in the past when I was a teenager, but the resultant bone density scans (taken at intervals before, during and after the treatment) showed little improvement and treatment was subsequently stopped.  Upon further reading into this it seems there are possible problems for long term users of biphosphonates.   This can include the higher risk of fracture after long term use due to the bodies inability to metabolize the drug and the natural effect of the biphosphonate inhibition on the bone cell turnover rate (Ott 2005: 31897).  There are many cases though where drug treatment has proved beneficial; however each case should be merited individually and each person monitored as appropriate.  I will stress here that there are many different types of biphosphonates available and that McCune Albright Syndrome varies in its intensity.

X ray of my left femur and hip with a locking intramedullary rod and screws.  Although please note that two of the femoral neck screws have now been taken out.

Although this is just a short post on the introduction to the disease that is sharing life with me it can also be found in the archaeological record.  Waldron (2009: 214) points out that Fibrous Dysplasia is often best diagnosed in an archaeological skeleton by the noting of either a shepherd’s crook deformity, healed fractures and findings of expansile swellings on one or more bones.  Subjecting the suspected sample to X-ray should show ‘lucent areas with endosteal scalloping and sometimes a thick sclerotic border’  (Waldron 2009: 215).  Unlike today’s vast array of modern medical treatment and surgical procedures, people in the past largely had to make do and mend.

As Roberts & Manchester (2010) discuss in their book, fracture treatment in the medieval age and before was fairly adept at helping in supporting and stabilising the fracture site.  However with repeated breaks in the main weight supporting bones, it is doubtful whether one could have led a normal life if the fractures were not reduced properly or repeatedly after continual breaks (Oakley 2007).  It also should be noted here that due to the nature of McCune Albright Syndrome it is unlikely to be described in the archaeology record as human skin rarely preserves.  It is far more likely that Fibrous Dysplasia is diagnosed based on the skeletal remains.

In the archaeological record Fibrous Dysplasia remains a rare and elusive disease to diagnose, whilst is has actively been described and documented in more recent human remains (Nerlich et al. 1991).  The following two case studies highlight individual cases of where Fibrous Dysplasia has been documented in archaeological material.

A recent case study presented by Craig & Craig (2011) discusses a juvenile skeleton with evidence of polyostotic Fibrous Dysplasia.  The skeletal remains of a child aged 7 years presents with Fibrous Dysplasia with evidence of involvement most noticeable with large bone expansion on the left mandible alongside involvement of the temporal, maxilla, parietal and frontal craniofacial bones.  A review of the burial context of the skeleton and of the Anglo-Saxon cemetery population that the child comes from shows no differentiation between this and other burials, indicating no differentiation in the disposition of this child’s body or associated grave goods.  Craig & Craig (2011) also cite further Ango-Saxon literature to suggest that it is highly unlikely that the child was stigmatized due to his disability, although we can never know for sure.

Recent evidence in a 120,000 year old Neandertal individual from the Upper Pleistocene site of Krapina in present day Croatia highlights the likely evidence for Fibrous Dysplasia presence in a small rib fragment (Monge et al. 2013).  This is extremely rare to find a bone lesion or tumour  in skeletal material from such a period and it is extremely exciting.  The rib was allocated original as a faunal remain when the site was initially excavated, but the rib was recognised for being of Neandertal origin by sharp eyed human osteology legend Tim D. White (Monge et al. 2013).

X ray of the transverse fracture of my right tibia and fibula in the summer of 2009.  This was the first of three transverse fractures of the right tibia and fibula that followed in quick succession over a short number of years, and resulted in the fixation of the tibia with a permanent titanium plate.

Below are some medical and non-medical sources of information on the various aspects of both Fibrous Dysplasia & McCune Albright Syndrome (FD and MAS). This includes a few recent palaeopathology articles that are freely available, medical articles discussing both FD and MAS, core palaeopathology textbooks and support groups in the US and UK for sufferers of the bone disease.  Although the disease is not headline grabbing news, the lack of research into the socio-economic aspects of the disease is distinctly lacking, as is the number of foundations or adult support services for sufferers with the disease.

I am thankful for the support of my friends, family & my consultant in the treatment of this syndrome and for continued support given.

N.B. The origin of the Ilizarov frame is particularly interesting.  It was first used in the 1950s in the USSR, with Dr Gavril Ilizarov originally using bicycle wheel spokes to fixate, support and lengthen badly fractured bones.  It was only introduced to the West in the 1980’s as a direct result of Ilizarov’s corrective surgery on a patient in Italy when all other options had failed in healing the patient’s fractures.  So far I have managed to avoid having the frame but it is still a standard procedure for badly fragmented fractures, in particular it is often used after motorbike accidents or reconstructing limb angulation/length.

Bibliography and Further Sources:

Fibrous Dysplasia:

• Fibrous Dysplasia Support Society UK.
• Fibrous Dsyplasia Foundation.
• Magic Foundation: MAS division
• Mayo Clinic Overview of Fibrous Dysplasia.

Medical Articles:

• Burke, A. B., Collins, M. T., & Boyce, A. M. 2016. Fibrous Dysplasia of Bone: Craniofacial and Dental Implications. Oral Diseases. doi: 10.1111/odi.12563.

• Feller et al. 2009. The Nature of Fibrous Dysplasia. Head & Face.  5 (22): 1-5.

• Fitzpatrick, K. A. et al. 2004. Image Findings of Fibrous Dysplasia with Histopathologic and Intraoperative Correlation. American Journal of Roentgenology. 182 (6): 1389-1398.

• Lee, J. S. FItzgibbon, E. J., Chen, Y. R., Kim, H. J., Lustig, L. R., Akintoye, S. O., Collins, M. T. & Kaban, L. B. 2012. Clinical Guidelines for the Management of Craniofacial Fibrous Dysplasia. Orphanet Journal of Rare Disease. 7 (1): 1-19..

• Marsland, D. & Kapoor, S. 2008. Rheumatology and Orthopaedics. London: Mosby Elsevier.

• Ott, S. M. 2005. Long-Term Safety of Biphosphonates. The Journal of Clinical Endocrinology & Metabolism. 90: 31897-31899.

McCune-Albright Syndrome:

• Brief overview of MAS symptoms.
• OMIM entry on history and presentation of MAS.
• Detailed general and genetic overview of MAS from the Online Medelian Inheritance In Man project.

Medical Articles:

• Charpulat, R. D. 2008. Fibrous Dysplasia of Bone and McCune-Albright Syndrome.  Best Practice & Research Clinical Rheumatology. 22 (1): 55-69.

• Dumitrescu, C. E. & Collins, M. T.  2008.  Overview: McCune-Albright Syndrome. Orphanet Journal of Rare Disease. 3 (12): 1-12.

• Hannon, T. S. et al. 2003. Is McCune-Albright Syndrome Overlooked in Subjects with Fibrous Dysplasia of Bone?  Journal of Pediatrics. 142 (5): 532-538.

• Laven, J. S. E, Lumbroso, S., Sultan, C. & Fauser, B. C. J. M. 2001. Dynamics of Ovarian Function in an Adult Woman with McCune-Albright Syndrome. Journal of Clinical Endocrinology & Metabolism. 86 (6): 2625-2631.

• Parisi, M. S. & Oliveri, B. 2009. Long-Term Pamidronate Treatment of Polyostotic Fibrous Dysplasia of Bone: A Case Series in Young Adults. Current Therapeutic Research. 70 (2): 161-172.  
  

Palaeopathology:

• Aufderheide, A. C. & Rodríguez-Martín. C. 1998. Cambridge: Cambridge University Press. (pg.420-421).

• Craig, E. & Craig, G. 2011. The Diagnosis and Context of a Facial Deformity from an Anglo-Saxon Cemetery at Spofforth, North Yorkshire. International Journal of Osteoarchaeology. doi: 10.1002/oa.1288.

• Milella, M. Knüssel, C. J., & Haddow, S. D. 2016. A Neolithic Case of Fibrous Dysplasia at Ҫatalhöyük (Turkey). International Journal of Palaeopathology. 15: 10-18.

• Monge J, Kricun M, Radovčić J, Radovčić D, Mann A, et al. 2013. Fibrous Dysplasia in a 120,000+ Year Old Neandertal from Krapina, Croatia. PLoS ONE. 8(6): e64539. doi:10.1371/journal.pone.0064539 (free open access article).

• Nerlich, A., Peschel, O., Lohrs, U., Parsche, F., Betz, P. 1991. Juvenile Gigantism plus Polyostotic Fibrous Dysplasia in the Tegernsee Giant (Letter). Lancet. 338: 886-887. (please email if you pay walled and would like a copy of this article for personal use).

• Roberts, C. & Manchester, K. 2010. The Archaeology of Disease Third Edition. Stroud: The History Press.

• Traversari, M., Serrangeli, M. C., Catalano, G., Petrella, E., Piciucchi, S., Feletti, F., Oxilia, G., Cristiani, E., Vazzana, A., Sorrentino, R., De Fanti, S., Luiselli, D., Calcagnile, L., Saragoni, L., Feeney, R. N. M., Gruppioni, G., Cilli, E., & Benazzi, S. 2019. Multi-analytic Study of a Probably Case of Fibrous Dysplasia (FD) from Certosa Monumental Cemetery (Bologna, Italy). International Journal of Palaeopathology. 25: 1-8.

• Waldron, T. 2009. Palaeopathology: Cambridge Manuals in Archaeology. Cambridge: Cambridge University Press.

General Medical

• Medline Plus, a US medical site.

• Pub Med, a US National Library of Medicine website.