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Humerus Triptych: Fracturing & Fixing

22 Aug

I just can’t seem to help myself.  No sooner do I find out that I’d previously (and unknowingly) fractured a number of my ribs over a period of years, do I go and fracture my right humerus in the early evening sun of a peaceful July night.  It was, of course, shortly accompanied by the familiar wash of painkillers that helped numb the pain somewhat.  I’ve mentioned the humerus fracture a number of times in recent blog entries but I have not, until now, managed to obtain copies of the X-rays to highlight the break itself, and the subsequent surgical procedure that I underwent to fixate it.  With thanks to modern technology, I present to you below my right humerus in post-accident pre-surgery and post-accident post-surgery poses, if you will.  As White and Folkens (2005: 312) highlight fractures normally occur ‘as a result of abnormal forces of tension, compression, torsion, bending, or shear applied to the bone’, and they are often described by the features of the break itself (i.e. transverse, oblique, spiral etc).

I have long feared fracturing any of my bones in either upper arms (brachium), forearms (antebrachium) or hands (manus), even though I’ve had a somewhat turbulent history of pathologically fractured bones in my lower limbs.  Alongside this I have also undergone a fairly extensive list of elective surgery to fixate the femora and right tibia due to the effects of McCune Albright Syndrome (including improving the angle of the so-called shepherd’s crook deformity of the femoral neck).  Thus where a natural fracture or planned surgical procedure on the lower limbs may mean I cannot use my crutches for a few months, I can still use the wheelchair to maintain physical independence.  This is not so with a fractured upper limb, where healing will take many months.

To return to the common name usage, I rely on my arms not just for holding or grasping objects but for the locomotion of my manual wheelchair.  As such they are my legs for daily mobility.  I use them also to partially bear my weight when I use my crutches to walk, so a fractured upper limb bone would mean walking is out of the question as well.  I have fractured a humerus only once before, aged 13 at school.  An ill-advised arm wrestle resulted in my friend looking at my pale and quickly draining face in horror as I cradled my snapped right humerus in shock.  It is safe to say that my friend won that match, and I’ve been wary of competing in arm wrestles ever since!  The result of that match was a lengthy spell in plaster (or some variation thereof as, after few months, plaster gave way to support splint, and splint gave way to a laughable plastic guard).

right humerus fracture 2014 july

X-ray of my brachium (upper arm) with the transverse (possibly oblique) fracture of the right distal humerus in a cast before surgery (far left), the post-surgery fixation with a titanium plate and screws (centre), and finally a view of the brachium that highlights the plate and the depth of the screws (right), which help to keep the fixation and fracture site stable by equally distributing stress.  The tell-tale signs of the ‘ground glass’ appearance of polyostotic fibrous dysplasia (as a part of the McCune Albright Syndrome that I have) can also be seen in the X-rays, as can the evidence of a previous fracture and natural bowed shape of the humerus.

In truth the recent humeral fracture was the result of my impatience, gained as a result of quickly bouncing off a curb to catch a waiting taxi, and coming off worse for wear as the wheelchair tipped and I instinctively shot out my right arm to stop myself.  The pain from a fracture comes not from the bone breaking but from the damage to the soft tissues that surround the bone.  The periosteum, a tough connective tissue that nourishes and covers all outer external surfaces of the bones barring articular surfaces of the long bones, is home to nerves that the bones themselves are not (White & Folkens 2005: 42).  A fracture of the bone often damages the periosteum tissues (which causes pain) and leads to swelling of local tissues.  The periosteum, and associated endosteum membrane (located on the inner surface of bones), are also one of the origins where the precursor bone cells develop into chondroblasts and osteoblasts, which are essential for helping the bone fracture heal successfully (White & Folkens 2005: 43).

A small but significant benefit of having polyostotic Fibrous Dysplasia is the fact that the pathological fracture patterns tend to be transverse breaks due to the weak structure of the bone architecture, which tends to limit injury to both the nerves and the soft tissues surrounding the fracture area (Marsland & Kapoor 2008: 121).  However, due to the pathological bone porosity and the often high bone cell turnover rate as a part of the overall syndrome, there is the prospect of extensive bleeding during surgical procedures.  This can lead to extensive blood loss during major operations (such as during osteotomy procedures and/or internal and external fixations to help improve the bowing of a limb or to correct pathological fractures).  As such the patient’s blood is often cross matched beforehand with suitable blood groups, for infusion during major surgical procedures to combat excessive blood loss.

In the immediate aftermath of the fracture I was given heavy painkillers and taken to hospital where, after a light sleep overnight, my arm was put into a cast before I underwent surgery later in the week in a hospital nearer my hometown.  The decision was taken not to reduce the bone before the surgery and just to rest it.  On weight bearing bones (such as the tibia or femur) or load bearing bones (such as the mandible in adults), it is important that the bone is reduced quickly and properly to minimise complications and induce good healing (Marsland & Kapoor 2008: 120).  The humeral fracture was openly reduced and fixated under general anesthetic with a titanium locking plate, as can be seen in the above X-ray, and the surgeon achieved a good fix and stability of the distal humerus with the plate.

Curiously, even though the fracture was trauma induced, it was less painful than the fracture that had occurred when I was 13.  The arm still feels heavy and slightly cumbersome, but there is no doubt that the internal fixation is preferable to the months in the plaster cast.  It will still take many months for the bone to heal properly as it is still in the early stages of the primary bony callus, a process where woven bone bridges the initial fibrous connective tissue callus that responds to a fracture in the first few days.  This woven bone is, after a few months, later converted to lamellar bone and the fracture site will be further remodelled.  Eventually, if a fracture site is initially kept stable by immobilization or by fixation as in my case, the bone can remodel so completely as to eliminate any trace of the original fracture (White & Folkens 2005: 48).

Traumatic fractures are found in all periods of human and hominin history, and it is likely that you yourself have suffered a fractured bone of some description, perhaps even unknowingly (Marsland & Kapoor 2008: 121).  They can be devastating, requiring many years of surgery or physiotherapy to gain and improve movement as the sociologist Ann Oakley highlights in her 2007 book Fracture: Adventures of  Broken Body, a personal account detailing the social and professional impact of a fractured right humerus accident which had impinged on nerves, leading to reduced function and feeling.  Fracture treatment has been practiced for thousands of years and it has long been known that, with the reduction of the break and stabilization of the limb, good results can be achieved (Marsland & Kapoor 2008).  The study of fractures in populations can also highlight trends in the attention received as Meyers (2012) has highlighted in an entry on the differences of fracture treatment between Iron Age and Romano-British populations in Britain.

fractured right tibia digistied diseases 0365

The right tibia of an adult, courtesy of the free online resource Digitised Diseases. Notice the well healed mid-shaft oblique fracture in the (a) anterior view, (b) is the posterior view and (c) is the close up posterior view, where right is proximal and left distal. The callus is fully remodelled with smooth bone over the fracture site, where the end is displaced laterally and proximally. Image credit: Digitised Diseases 2014 (Master Record Number 0365).

Still this entry’s approach is focused on the personal, not at the population level.  Another part of my body has broken and it is once again held together by titanium, likely to be a permanent addition to my skeleton.  The movement at the glenohumeral joint (otherwise known as the shoulder) is normal while movement at the elbow joint (comprised of the humeroulnar, humeroradial and superior radioulnar joints) is almost back to normal.  There is still a lack of full extension of the joint, with noticed tension in the biceps brachii muscle as it acts as the antagonist to the triceps brachii muscle during forearm extension, although daily physiotherapy should help to regain full movement.  I am no stranger to the strength of the metal in my body and I remain impressed by its capability in the use of orthopaedic fixation.  The use of metallic implants to fixate fractures is nothing new as Lane (1895) and Uhthoff et al. (2006) attest.  Whilst the use of casts to set fractures continues, it is the increase in the use and versatility of technology and materials to give nature a helping hand that remains the next big step in treating bone fractures (Bali et al. 2013).

Metal plates have been in use for over a hundred years where early pioneers such as Lane (1895), Lambotte (1909) and Sherman (1912) first introduced plates to help stabilize fracture sites and help mobilize patients faster than plaster casts could allow (Uhthoff et al. 2006: 118).  Although these early plates suffered from corrosion problems it soon became apparent that internal plate fixation could provide a safe and efficient way for patients to heal, whilst also regaining some form of movement.  Various plate designs improved on earlier designs, allowing for micromotion at the fracture site and compensation for bone resorption during the healing process.

Uhthoff et al. (2006: 124) contend that there are still problems in the form of internal plates, where compression and stress shielding can still lead to bone necrosis and cortical porosis.  In their conclusion they argue that there still needs to be a fine balance attained between a plate design that managed to reduce stress shielding and allows adequate micromotion at the site of a fracture, both which they concur would help mimic biological healing.  There also drawbacks that can include plate palpability, risk of infection, temperature sensitivity and possible growth restriction with metallic implants (Bali et al. 2013: 167).   Ultimately however the body still has to heal the fracture itself over a matter of weeks and months (White & Folkens 2005: 48).

It is interesting to note that Sir William Lane himself, writing in the late 19th century and primarily focusing on lower limb fractures, indicates the marked differences between upper and lower limb fracture treatment.  He states that although the upper limb does not take the weight of the body:

… in the arm very considerable alterations may occasionally develop, and are more marked and depreciating to the value of the individual as a machine in proportion as changes have already taken place in the particular joint or joints from the prolonged pursuit of a laborious occupation.” (Lane 1895: 861).

Deciding that fractures of upper limb need not be set directly in their original anatomical form, whereas lower limb bones should be set as close to as originally constituted due to their weight-bearing nature.  Furthering this view, in the same letter to the British Medical Journal in 1895, he highlights that:

One cannot but feel that the perpetuation of methods of treatment which have been in use up to the present time must depend on the fact that surgeons have not taken such trouble to inquire into the subsequent life-history of these patients as they have done in other departments of surgery.” (Lane 1895: 863).

There have been some distinct advances in using biodegradable plates in non-weight bearing locations, such as in the maxillofacial region, a position where many would like to avoid the intrusive nature of a temporary or permanent metal plate.  A study by Bali et al. (2013: 167) has highlighted the value of using biodegradable material to help fixate trauma-induced facial fractures, reporting that each individual in the small study cohort (N=10) of varying ages, reported good reduction of fracture and evidence for the total biodegradation of the plate after two years.

They also reported that no further surgical procedures were needed on their test cohort, a significant finding as metallic implants often either need removing if they are temporary or debriding if they become infected, both quite serious surgical procedures (Bali et al. 2013: 170).  Unfortunately the study highlights that biodegradable implants are unlikely to be currently safe to use in weight-bearing or load bearing bones.  Bali et al. (2013:171) conclude by stating that further studies are needed but biodegradable plates and screws can provide satisfactory, if expensive, stabilization as internal fixations for mid-face fractures.

Medical science and engineering has certainly come a long way since Lane first introduced the internal fixation plate, yet humans are as prone as ever to fracturing their bones.  As a person with McCune Albright Syndrome I may know the pain of breaking a bone, but I can be thankful that I live at a time and in a place where fractures can be confidently treated.

Further Information

  • I’ve written in more detail on polyostotic Fibrous Dysplasia and McCune Albright Syndrome here, which details the way in the which the disease has affected my skeleton.  Also, on that particular post, are a host of medical, palaeopathology and osteology related articles to do with McCune Albright Syndrome and Fibrous Dysplasia in general.  Alternatively search the blog for the keywords and numerous posts in which I’ve highlighted the syndrome and the bone disease will appear.
  • A previous post on 3D printing in orthopaedic surgery can be found here, and an entry giving a quick overview of some of the problems and approaches used in studying physical impairment and disability in archaeological contexts can be found here.

Bibliography

Bali, R. K., Sharma, P., Jindal, S. & Gaba, S. 2013. To Evaluate the Efficacy of Biodegradable Plating System for Fixation of Maxillofacial Fractures: A Prospective Study. National Journal of Maxillofacial Surgery4 (2): 167-172. (Open Access).

Digitised Diseases. 2014. Master Record Number 0365. Accessed 18/08/14. http://www.digitiseddiseases.org/viewer/viewer_overlay.php?MRN=0365#.

Lane, W. A. 1895.  Some Remarks on the Treatment of Fractures. British Medical Journal1 (1790): 861–863. (Open Access).

Marsland, D. & Kapoor, S. 2008. Rheumatology and Orthopaedics: Crash Course 2nd Edition. London: Mosby Elsevier.

Meyers, K. 2012. Break a Leg! Fracture Treatment in Iron Age and Roman Britain. Bones Don’t Lie. Accessed 11th August 2014. (Open Access).

Oakley, A. 2007. Fracture: Adventures Of A Broken Body. Bristol: Policy Press.

Uhthoff, H. K., Poitras, P. & Backmann, D. S. 2006. Internal Plate Fixation of Fractures: Short History and Recent Developments. Journal of Orthopaedic Science. 11 (2): 118-126.  (Open Access).

White, T. D. & Folkens, P. 2005. The Human Bone Manual. London: Elsevier Academic Press.

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Dental Delights and Disability in Archaeology

26 Mar

I’ve recently had the joy of a dealing with a dental abscess affecting the left hand side of my mandible, and whilst I’m thankful for modern medicine I can only imagine the pain and frustration for pre-modern populations suffering with such an infection, especially those who didn’t have access to antibiotics and strong painkillers.  As such I haven’t posted properly for a while, and it might be a bit longer before I do.  Having had surgery to relieve the effect of the swelling and to drain the infection and remove two pesky teeth (with added complications courtesy of Fibrous Dysplasia), I’m once again learning how to chew (farewell 1st and 3rd left mandibular molars!).  It has also given me the time to think about the role of disability in the archaeological record and how it is approached by modern-day researchers.  What follows below is a very quick and brief overview of the main points of how disability has been approached in the archaeological sector and the changes therein.  Articles of interest are noted in the bibliography.

Dettwyler famously wrote a paper entitled ‘Can paleopathology provide evidence for compassion‘ (1991: 375-384, PDF embedded) that rightly questioned the interpretations of archaeologists and osteologists on the inferred aspects of care and compassion that disabled individuals from the archaeological record may or may not have received during their lifetimes.  The author cautioned that archaeologists and researchers are not ‘justified in drawing conclusions either about quality of life for disabled individuals in the past or attitudes of the rest of the community from skeletal impairment of physical impairment’ (Dettwyler 1991: 375).  This was a much-needed wake up call, and rightly raised questions in the realms of archaeology and palaopathology regarding how we viewed individuals, and how we analysed them.

The majority of disability studies before the Dettwyler (1991) article focused on disabled individuals as case studies, reported in journals and rarely integrated or investigated as part of the society or cemetery population they may belonged to.  Mays (2012) rightly investigated the impact of the relative value of individual case studies compared to quantitative and problem orientated population studies, and found that although the publishing gap had lessened between the two types, singular case studies still predominated.  Mays (2012) main contention is that individual case studies do little to further the advance of palaeopathology, something which Larsen (1997) effectively demonstrates throughout his book and review (2002), in the consideration of how palaeopathology can indicate society or cultural wide rituals, actions or lifestyles.

Since the publication of the Dettwyler paper there has been a slew of articles, journals and books dedicated to researching disability as evidenced from the skeletal and archaeological record, both from a bioarchaeological perspective and from a theory perspective (Battles 2011, Brothwell 2010, Hawkey 1998, Kleinman 1972, Vilos 2011, Wood et al. 1992).  Indeed the study of disability and the implications for affected individuals, their communities and societies, has moved on considerably since the descriptive days of Calvin Wells, especially in the consideration of the theory of ‘compassion’ as an evolutionary force in the primate family (Hublin 2009, Stewart et al. 2012), or as evidenced in other mammals (Fashing & Nyuyen 2011).

This is in accordance with the rise and debate of disability theory and studies in numerous other disciplines.  This has had real life applications in many areas of modern-day life, where multi-agented approaches to understanding,  recognising and implementing programs that are designed to raise awareness or life quality for disabled individuals.  Two prominent examples from the UK are the 2005 Disability Discrimination Act and the 2010 Equality Law where disability itself is given a legal definition, and here we come to a prominent problem in the archaeological and palaeopathological record itself.

Disability, as we would recognise it today, can mean both a physical and/or a mental impairment that can be substantial and lifelong, and it is worth noting some problems inherent in the archaeological record.  Firstly, in the archaeological record, we can only recognise physical disability when it has affected the skeletal remains of individuals, normally at a late and severe stage in the disease progression (Aufderheide & Rodriquez 1998, Waldron 2009, Wood et al. 1992).  As such, a large number of individuals with diseases or traumatic injuries that only affected the flesh will go unknown, and as such are unstudied.  Secondly, there is no universal or standard definition of disability that archaeologists and researchers use, it is solely up to the person/persons to define clearly and openly which definition they are using at the outset of their research (and there are a lot of definitions and models depending on which source you base your definition on).  Thirdly, the usage of terminology itself, such as the very word disability, can have vastly different connotations or implications for different populations and cultures (Battles 2011).

There may have been distinct differences as to who was considered disabled or not in historic and prehistoric cultures, and we should, as researchers, always be aware of observer bias ourselves (Dettwyler 1991).  As such researchers should always be clear who they are addressing, and the possible differences highlighted, where evidence is available, as to how a disabled person was treated within their culture when archaeological or cultural evidence is available.

To complicate the matter further is the ‘osteological paradox‘, as highlighted by Larsen (1997), Woods et al. (1992) and Wright & Yoder (2003) amongst others, which heavily influences the health status of skeletal remains that survive and that are then studied.  Therefore it should always be understood that no skeletal sample is entirely representative of their population, that there are many caveats (Hahn 1995, Roberts 2000).

Battles (2011) highlighted the need to move towards a more holistic approach to disability, to take advantage of different fields (including physical anthropology, sociocultural anthropology, experimental studies and archaeology itself) to understand disability at archaeological sites and affected individuals, to a model that integrates the data and insight of the various fields.  In particular Battles (2011) makes the salient point of noting the individuals  (largely females and sub-adults) that historically have been under-studied in archaeological and population analyses.

An important methodological update has been the advancement of a ‘Bioarchaeology of Care‘, as espoused by Tilley & Oxenham (2011), where a four stage assessment of an individual produces an assessment of the care needed for the disabled individual found in a Neolithic Vietnam community.  The stages are: (1) describing,  diagnosing and documenting the individual and site, (2) identify the clinical/functional impacts of disease or trauma, and determine if care was needed, (3) produce a model of care, and finally (4) interpret the implications for the individual and society, as well as possible indications for the identity and nature of both (Tilly & Oxenham 2011: 36).  It could be argued that other researchers have espoused the same sentiments (Roberts & Manchester 2010, Vilos 2011), but it is the clear initiation of the applying the model to individuals who fit the criteria that will hopefully produce further studies and elicit meaningful result which highlight this recent study as one to watch.  The Tilley & Oxenham (2011) model is particularly useful for prehistoric cases where there are no written or documentary sources.

Hawkey’s (1998) study of musculoskeletal markers (MSM’s) of a disabled individual from a New Mexico Pueblo culture highlighted the worth of applying existing osteological techniques to disabled individuals in order to assess the quality of bodily movement.  The modelling of the movement capable for this individual suggested that bodily manipulation, feeding, and the cleaning of this person was likely carried out by members of his culture (possibly family relatives, although this is conjecture) due to the severity of his disability (Hawkey 1998: 330).  Craig & Craig (2011) make extensive use of modern medical imaging to diagnosis a specific disease (fibrous dysplasia) in the case of a sub-adult from an English Anglo-Saxon site.  The striking bone expansion in the mandible is discussed within the social sphere of the community that the individual belonged.  The implications, via the the inference of position of the body within the grave, grave goods and grave location, and studies into Anglo-Saxon culture and social stratification give rise to the theory that the individual was not treated any differently due to his disability, although it is unknown if the disease led to the early demise of the individual (Craig & Craig 2011: 3).

Craig & Craig’s (2011) case study, and the above studies, highlight the use of modern medical literature and imaging technology in establishing a likely disease diagnosis, yet Brothwell (2010) rightly highlights the dangers of the differential diagnosis of diseases in skeletal remains at a macroscopic level.  Waldron’s (2009) palaeopathology handbook presents an ideal source on how to identify diseases that can lead to disability, but highlights the value of the differential diagnosis when the osteologist cannot be exactly sure of the disease.

The battery of scientific techniques used in archaeological investigations, including aDNA analysis, trace chemical analysis, and isotopic analysis amongst others, have become significantly refined within the past two decades, and are now allowing for a more nuanced understanding of individual and population dynamics (Brown & Brown 2011).  This includes the ability to analysis the movement of a person in a landscape within their lifetime (Marstellar et al. 2011), and to understand the changes in diet and the effects of diet on the body (Larsen 1997, Roberts 2000, Roberts & Manchester 2010). It also includes the ability to indicate the likely exposure of populations to various chemicals and diseases (Barnes et al. 2011), and exploration of how social structure (Bentley et al. 2012), and hence the role of the population or of the individual, changed through time.

Perhaps what the above studies cannot show, especially in prehistoric societies, are the actions of the disabled individuals themselves.  It is most likely that we will never know if they took an active interest in their society, if they took part, or how they felt as disabled individuals, or even if they saw themselves as disabled (Battles 2011, Hahn 1995).  Compassion  itself cannot be excavated (Dettwyler 1991), but with careful examination of the available evidence results can be produced that suggest that severely disabled individuals did survive past natural limitations.

The progress continually being made in the hard sciences and in the humanities continues to advance our knowledge of past populations via their skeletal remains and their cultural context.  The understanding of disability within an archaeological and osteological context provides the opportunity to investigate of how individual’s survived, and whether care was a key component (Hawkey 1998, Kleinman 1978, Tilley & Oxenham 2011).  This is a burgeoning area of bioarchological research, and when combined with a multidisciplinary approach, it opens up a wide range of interesting and diverse approaches and avenues.

Case Studies, Theories and Further Information:

Full articles are linked where possible, although a number hide behind Journal pay walls.

Aufderheide, A. C. & Roderiquez-Martin, C. 1998. The Cambridge Encyclopedia of Human Palaeopathology. Cambridge: Cambridge University Press.

Barnes, I., Duda, A., Pybus, O. G. & Thomas, M. G. 2011. Ancient Urbanization Predicts Genetic Resistance to Tuberculosis. Evolution. 65 (3): 842-848.

Battles, H. 2011. Toward Engagement: Exploring the Prospects for an Integrated Anthropology of Disability. Explorations in Anthropology. 11 (1): 107-124.

Bentley, R. A., Bickle, P., Fibiger, L., Nowell, G. M., Dale C. W., Hedges, R. E. M., Hamiliton,. J., Wahl, J., Francken, M., Grupe, G., Lenneis, E., Teschler-Nicola, M., Arbogast, R-M., Hofmann, D. & Whittle, A. 2012. Community Differentiation and Kinship Among Europe’s First Farmers. Proceedings of the National Academy of Sciences Early Edition. 1-5. (Early View).

Brothwell, D. 2010. On problems of Differential Diagnosis in Palaeopathology, as Illustrated by a Case from Prehistoric Indiana. International Journal of Osteoarchaeology. 20: 621-622.

Brown, T. & Brown, K. 2011. Biomolecular Archaeology: An Introduction. Chichester: Blackwell Publishing.

Churchill, S. E., Franciscus. R. G., McKean-Peraza, H. A., Daniel, J, A. & Warren, B. R. 2009. Shanidar 3 Neandertal Rib Puncture Wound and Palaeolithic Weaponry. Journal of Human Evolution. 57: 163-178.

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. (Early View doi: 10.1002/oa.1288).

Dettwyler, K. A. 1991. Can Palaeopathology Provide Evidence for “Compassion”? American Journal of Physical Anthropology. 84: 375-384.

Fashing, P. J. & Nguyen, N. 2011. Behaviour Towards the Dying, Diseased, or Disabled Among Animal and its Relevance to Palaeopathology.  International Journal of Palaeopathology. 1: 128-129. 

Hahn, R. A. 1995. Sickness and Healing: An Anthropological Perspective. New Haven: Yale University.

Hawkey, D. E. 1998. Disability, Compassion and the Skeletal Record: Using Musculoskeletal Stress Markers (MSM) to Construct an Osteobiography from Early New Mexico. International Journal of Osteoarchaeology. 8: 326-340.

Hublin, J. J. 2009. The Prehistory of Compassion. Proceedings of the National Academy of Sciences. 106 (16): 6429-6430.

Kleinman A. 1978. Concepts and a Model for the Compassion of Medical Systems as Cultural Systems. Soc Sci Med. 12: 85-93.

Knusel, C. J. 1999.  Orthopaedic Disability: Some Hard Evidence. Archaeological Review Cambridge. 15: 31-53.

Larsen, C. 1997. Bioarchaeology: Interpreting Behaviour from the Human Skeleton. Cambridge: Cambridge University Press.

Larsen, C. S. 2002. Bioarchaeology: The Lives and Lifestyles of Past Peoples. Journal of Archaeological Research. 10 (2): 119-166.

Marstellar, S. J., Torres-Rouff, C. & Knudson, K. J. 2011. Pre-Columbian Andean Sickness Ideology and the Social Experience of Leishmaniasis: A Contextualised Analysis of Bioarchaeological and Palaeopathological Data from San Pedro de Atacama, Chile. International Journal of Palaeopathology. 1 (1): 23-34.

Mays, S. 2012. The Impact of Case Reports Relative to Other Types of Publication in Palaeopathology. International Journal of Osteoarchaeology. 22: 81-85.

Roberts, C. A. 2000. ‘Did They Take Sugar? The Use of Skeletal Evidence in the Study of Disability in Past Populations’. In Hubert, J. (ed) Madness, Disability and Social Exclusion: The Archaeology and Anthropology of Difference. London: Routledge. 46-59.

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

Stewart, F.A., Piel, A.K., O’Malley, R.C., 2012. Responses of Chimpanzees to a Recently Dead Community Member at Gombe National Park, Tanzania. American Journal of Primatology. 74: 1–7.

Tilley, L. & Oxenham, M. F. 2011. Survival Against the Odds: Modelling the Social Implications of Care Provision to the Seriously Disabled. International Journal of Palaeopathology. 1 (1): 35-42.

Vilos, J. D. 2011.  Bioarchaeology of Compassion: Exploring Extreme Cases of Pathology in a Bronze Age Skeletal Population from Tell Abraq, U. A. E. Master’s Dissertation. Las Vegas: University of Nevada.

Waldron, T. 2009. Palaeopathology. Cambridge: Cambridge University Press.

Wood, J. W., Milner, G.R., Harpending H. C., & Weiss, K. M. 1992.  The Osteological Paradox: Problems of Inferring Prehistoric Health from Skeletal SamplesCurrent Anthropology 33:  343-370.

Wright, L. E. & Yoder, C. J. 2003.  Recent Progress in Bioarchaeology: Approaches to the Osteological ParadoxJournal of Archaeological Research 11 (1): 43-70. (**An extensive bibliography of articles can be found in the bibliography of this article**).

Infectious Disease Part 2: Malaria and Associated Anaemic Conditions

5 Oct

This second post, and the first part, deal with biomolecular approaches and research studies in detecting  the presence of infectious diseases in human bone from archaeological material.  The recent coming of age of biomolecular techniques, as applied to archaeological material, has provided a rich and complex source of information in helping to uncover how infectious diseases spread in the historic and prehistoric past.  The second post, here, describes recent research focused on Malaria and associated anaemic conditions, including Sickle Cell Anaemia and Thalassaemia.  The first post can be found here.

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It has long been realised that malaria can only be recognised in skeletal remains via indirect evidence of presentation of the following pathological lesions- porotic hyperostosis, cribra orbitalia and marrow hypertrophy- which are taken as evidence of the presence of anaemia, the main contributor of mortality in malarial victims (Roberts & Manchester 2010).  However there is no pathognomonic bone lesion for either Plasmodium vivax or P. falciparum, the main human species of malaria causing Plasmodium genus  (Gowland & Western 2012: 303, Roberts & Manchester 2010: 233), and the above skeletal lesions have varying aetiologies including anaemia, osteitis, parasitic infection, and other interrelated deficiency diseases which are still not clearly understood (Gowland & Western 2012: 302).  To securely diagnose malaria in skeletal material, DNA identification of the Plasmodium genus must take place, and even then current Polymerase Chain Reaction (PRC) tests ‘do not appear to be able to amplify routinely the DNA of malaria pathogens from ancient bones’ (Gowland & Western 2012: 302).

Recent immunological techniques to identify antigens have also been used to isolate and identify P. falciparum, although false positives can occur as a result of contamination or diagenetic factors(Gowland & Western 2012: 302).  Gowland & Western (2012) have recently proposed a spatial epidemiological model for malarial spread in Anglo-Saxon England, which highlights the re-surging interest in malaria in the modern context as well as one affecting a past population.  This holistic approach used GIS data with diagnosed porotic hyperostosis in skeletal remains, mosquito (Anopheles atroparvus) habitat information and historical data in presenting a locality data set for malaria infected individuals (Gowland & Western 2010: 304-305).  The modelling of palaeopathological, climatic, and historical data, provides new information on disease range, mechanism of transmission, and infection localities.  However, there are also complicating factors in assessing and diagnosing malaria from other diseases, as noted below (Roberts & Manchester 2010: 234).

Particularly important are two inherited haemolytic anaemia’s, thalassaemia and sickle-cell anaemia, who are characterised by abnormal haemoglobin and increased destruction of red blood cells (Jurmain et al. 2011: 312, Roberts & Manchester 2010: 232).  Thalassaemia is a genetically determined disorder which is caused by a ‘problem of haemoglobin synthesis’ (Roberts & Manchester 2010: 233).  This results in failure or depression of synthesis of the chain, this leads to pale cells with low hemoglobin content which are then rapidly destroyed once formed.  There are three grades of the disease, minor, intermediate and major, the last of which includes severe anemia and possible bone changes; the range of the disease is typically centered in the Mediterranean, Middle East and Far East (Roberts & Manchester 2010: 233).  The importance is that it is seen as an adaptive response to malaria infection through the development of this heritable disease; that the high red blood cell turnover stalls and negates any effect of malarial infection.  Archaeological evidences comes from Greek, Turkish and Cypriot populations deriving from marshy contexts, which are ideal breeding grounds for mosquitoes, the prime vector for malaria (Roberts & Manchester 2010: 233).

Sickle-cell anaemia occurs as a result of the deformation and destruction of red blood cells which leads to over enlargement of bony centres (centered on the skull, pelvis, vertebrae) and over-activity of marrow production as the body produces more red blood cells (Waldron 2009).  This inheritable disease range is mainly located in Central and Eastern African populations who have high rates of the disease, but also affects Indian, Middle Eastern, and Southern European populations (Roberts & Manchester 2010: 234).  Jurmain et al. (2011: 312) remark that the sickle-cell allele hasn’t always been effective in malarial negation in human populations, and primarily came to prominence during the advent of agriculture, and in particular during the last 2000 years in Africa.  The origin of the mutation of the allele responsible, HB5 in haemoglobin, has been dated to 2100 to 1250 years ago in African populations (Jurmain et al. 2011: 312).  Although malaria infection has only relatively recently affected human populations, it has become a powerful selective force that still affects large portions of the world’s population today.

In conclusion, biomolecular approaches to archaeological and osteological remains are vital in unraveling past populations and the natural world (Jurmain et al. 2011).  The interactions between wild and domesticated animals, humans, insects and the environment are a prerequisite for understanding the mode of transmission and virulence of infectious diseases (Barnes et al. 2011, Gowland & Western 2012, Jurmain et. al 2011).  Yet, we must take into consideration the difficulties in understanding infectious disease; examples of the osteological paradox are ever present, understanding the aetiology of bone changes, and the context of genetic differences between populations must be noted whilst PCR amplification, aDNA detection and genome explorations methods must be continually improved for clearer results (Li et al. 2011, Schurch et al. 2011, Spigelman et al. 2012, Tran et al. 2011); this approach must be multidisciplinary in understanding past and present populations (Jurmain et al. 2011, Roberts & Manchester 2010, Waldron 2009).

The modern world has changed, and the boundaries that once protected various human populations has changed dramatically with cheap air travel and vast population movement; this is unprecedented in both history and prehistory, and in population density and scale, but also at the genetic level in human genetic variation (Hawks et al. 2007, Jurmain et al. 2011: 311).  The eradication of smallpox, the Bill and Melinda Gates foundation in fighting malaria, and the ongoing WHO (World Health Organisation) case against polio (Branswell 2012: 50) are strong examples of what can be achieved worldwide.  By building a past population profile of the effects of infectious disease, we are better prepared for the fight tomorrow.

Bibliography:

Barnes, I., Duda, A., Pybus, O. G. & Thomas, M. G. 2011. Ancient Urbanization Predicts Genetic Resistance to Tuberculosis. Evolution. 65 (3): 842-848.

Branswell, H. 2012. Polio’s Last Act. Scientific American. 306 (4): 50-55.

Gowland, R. L., & Western, A. G. 2012. Morbidity in the Marshes: Using Spatial Epidemiology to Investigate Skeletal Evidence for Malaria in Anglo-Saxon England (AD 410- 1050). American Journal of Physical Anthropology. 147: 301-311.

Hawks, J., Wang, E. T., Cochran, G. M., Harpending, H. C. & Moyzis, R. K. 2007. Recent Acceleration of Human Adaptive Evolution. Proceedings of the National Academy of Sciences. 104 (52): 20753-20758.

Jurmain, R., Kilgore, L. & Trevathan, W. 2011. The Essentials of Physical Anthropology, International Edition. Belmont: Wadsworth.

Li, Y., Carroll, D. S., Gardner, S. N., Walsh, M. C., Vitalis, E. A. & Damon, I. K. 2007. On the Origin of Smallpox: Correlating Variola Phylogenics with Historical Smallpox Record. Proceedings of the National Academy of Science. 104 (40): 15787-15792.

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

Schurch, A. C., Kremer, K., Kiers, A., Daviena, O., Boeree, M. J., Siezen, R. J., Smith, N. H., & Soolingen, D. V. 2010. The Tempo and Mode of Molecular Evolution of Mycobacterium Tuberculosis at Patient-to-Patient Scale. Infection, Genetics and Evolution. 10 (1): 108-114.

Spigelman, M., Shin, D. H., & Gal, G. K. B. 2012. The Promise, the Problems and the Future of DNA Analysis in Palaeopathology Studies. In Grauer, A. L. (ed). A Companion to Palaeopathology. Chichester: Blackwell Publishing Ltd.  pp.133-151.

Tran, T., Aboudharam, G., Raoult, D., & Drancourt, M. 2011. Beyond Ancient Microbial DNA: Nonnucleotidic Biomolecules for Palaeomicrobiology. BioTechniques. 50: 370-380.

Waldron, T. 2009. Palaeopathology. Cambridge: Cambridge University Press.

An Introduction to Fibrous Dysplasia & McCune-Albright Syndrome

28 Oct

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:

Medical Articles:

  • 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.

McCune-Albright Syndrome:

Medical Articles:

Palaeopathology:
  • Aufderheide, A. C. & Rodríguez-Martín. C. 1998. Cambridge: Cambridge University Press. (pg.420-421).
  • Roberts, C. & Manchester, K. 2010. The Archaeology of Disease Third Edition. Stroud: The History Press.
  • Waldron, T. 2009. Palaeopathology: Cambridge Manuals in Archaeology. Cambridge: Cambridge University Press.
General Medical
  • Pub Med, a US National Library of Medicine website.

Sheffield Human Osteology Short Course

10 Jul

I recently attended the week long Human Osteology short course at the University of Sheffield, at the back end of June.  This course was offered within the Manor Lodge excavation project, and was ran by the archaeology department.  It was particularly great to see so many interested (and interesting!) young people into human osteo and willing to learn.  The course covered all the basics of aging & sexing skeletons found in archaeological contexts, as well as recognizing human bone elements.  We also had the chance to examine both adult and juvenile skeletons. alongside a good look at the different pathological diseases that can be present on skeletal remains.

The course was delivered by lectures and hands on work, with the opportunity to lay out the skeleton each day of the course.  Although this course was part of the Manor Lodge excavations, it is held every year at the University of Sheffield Archaeology department, and as such I’d highly recommend it.  There is also another day coming up in September where people in professional jobs which includes looking at or moving/using human remains in the heritage sector can have an intensive day study of the human skeleton.

On a personal note, it was great for me to experience what it will be like to start in September, on a day to day basis.  The short course helped to strengthen my resolution in the feeling that I have picked the right University, in pursuing this course.  In the future I aim to include upcoming human osteology short courses when I know they are available, for future reference is anybody is interested.

Broken Bones

26 May

My right tibia and fibula, with a transverse fracture gained in the summer of 2009.  A result of polyostotic fibrous dysplasia.  Needless to say, I spent the summer reading in the sun.