Author’s Note
Heptonclough
Heptonclough was inspired by, but not based upon, the village of Heptonstall (from the old English hep – wild rose, and tunstall – farmstead) on the Yorkshire Pennines, not far from the border with Lancashire. Like its fictional counterpart, Heptonstall owed its early wealth to the wool trade and today boasts two churches (one old, one very old), the White Lion pub, the old grammar school and numerous cobbled streets lined with tall stone houses. Visitors should not look for Wite Lane, the Abbot’s House or the Fletcher family’s shiny new home, but teenage boys can definitely be seen riding their bikes around the old church walls. I’ve watched them do it.
Congenital Hypothyroidism
‘I see a head of unusual form and size, a squat
and bloated figure, a stupid look, bleared hollow and heavy eyes,
thick projecting eyelids, and a flat nose. His face is of a leaden
hue, his skin dirty, flabby, covered with tetters and his thick
tongue hangs down over his moist livid lips. His mouth, always open
and full of saliva, shows teeth going to decay. His chest is
narrow, his back curved, his breath asthmatic, his limbs short,
misshapen, without power. The knees are thick and inclined inward,
the feet flat. The large head drops listlessly
on the breast; the abdomen is like a bag.’
Beaupre, Dissertation sur les cretins, c. 1850
Congenital hypothyroidism, which can be genetic, sporadic or endemic, is a condition of severely stunted physical and mental development, caused by a deficiency of the hormone thyroxine. In the UK around one in every 3,500 to 4,000 children are born with congenital hypothyroidism. Similar rates are reported in the USA and continental Europe. It is more common in girls than in boys, but the reason for this is currently unknown.
Without treatment, adult stature is below average, ranging from 1 to 1.6 metres; bone maturation and puberty are severely delayed and infertility is common. Neurological impairment, of varying degrees of severity, is to be expected. Cognitive development, thought and reflexes are slower. Other signs of the condition may include thickened skin, enlarged tongue or a protruding abdomen.
Fortunately, genetic and sporadic congenital hypothyroidism, caused by abnormal development of the thyroid gland before birth, has been almost completely eliminated in developed countries by newborn-screening schemes and lifelong treatment.
The endemic condition arises from a diet deficient in iodine: the essential trace element that the body needs to produce thyroid hormones. The soils of many inland areas on all continents are iodine deficient and food produced there is correspondingly deficient. Iodine deficiency causes gradual enlargement of the thyroid gland, and the resulting growth is referred to as a goitre. The endemic form of the condition continues to be a major public-health problem in many undeveloped countries.
‘Cretin’, from an Alpine-French dialect spoken in a region where sufferers were especially common, became a medical term in the eighteenth century. It saw considerable medical use in the nineteenth and early twentieth centuries, and then spread more widely in popular English as a derogatory term for someone who behaves stupidly. Because of its pejorative connotations in popular speech, health-care workers have mostly now ceased use of the term.