Medical nomenclature is almost exclusively couched in terms of "disease". The term "phenomenon" is much less frequently used; probably the commonest condition using this appellation is Raynaud's phenomenon.
Maurice Raynaud was a Paris physician who in 1862 first described 25 cases of "impediment of the arterio-capillary circulation". Raynaud's phenomenon is a temporary shutting down of the peripheral circulation in the limbs in response to cold and other stimuli.
Raynaud's originally described three distinct phases to the process: the digits first turned white, then blue, followed by a red colour. The white and blue discolouration occurs in response to exposure to cold or vibration, whereas redness represents subsequent rewarming.
The whiteness of the fingers or toes occurs when the peripheral blood vessels contract sharply. Blueness or cyanosis is caused by the deoxygenated blood which is now "trapped" in the digits. As the limb is rewarmed, the bright red colour represents a rapid flush of fresh blood back into the limbs.
In about 50 per cent of cases, there is no underlying cause found for Raynaud's phenomenon. Whether the primary reason is a transient narrowing of the vessels or whether there is an underlying abnormality in the nerves supplying the arteries remains to be established. The other 50 per cent of sufferers will have Raynaud's as a secondary phenomenon.
Conditions such as scleroderma, systemic lupus erythematosus, rheumatoid arthritis and various neurological conditions can lead to Raynaud's. Occupational causes are an interesting minority; the classic case involves vibration injury seen in construction workers who operate power tools used in road-breaking. Raynaud's can even be brought on by piano-playing and typing.
Robert Greacen, the award-winning poet and critic, developed Raynaud's about five years ago. He first noticed the problem during the cold and chilly days of winter. The blueness was associated with some pain in his feet and hands. In fact, the very first symptom he remembers was a "burning feeling in my feet". Although he has never suffered with chillblains, he recalls his mother having to wear mittens in an attempt to ward off these mildly gangrenous changes in her fingers. Robert Greacen works with a typewriter rather than a word processor, which in itself, may be a factor in the worsening he has noticed in the last few years.
Before a doctor can assume that Raynaud's in a patient is of the primary type, he will perform some blood tests. These will include tests for rheumatoid factor and abnormal proteins which may point to a more serious underlying cause.
Scleroderma is a multisystem disorder of unknown cause. Raynaud's phenomenon is one of the features of this complex disease and often precedes some of the other manifestations by years. Skin thickening of the fingers and toes is accompanied by similar changes to the face and body. Depending on the extent and subtype of the disease, other organs such as the kidneys and lungs may be affected.
Several environmental factors have been associated with the development of scleroderma. In 1981, in Spain, a multisystem disease resembling scleroderma occurred following the ingestion of adulterated cooking (rape seed) oil. Over 20,000 people were affected by skin thickening, arthritis, lung inflammation and Raynaud's phenomenon.
Maura Murphy works as the administrator of a relocation company. She was first diagnosed with Raynaud's in her 30s. She describes the classical colour changes in her fingers in response to cold. When on holidays in a warmer climate, she has noticed that entering an air-conditioned building can also trigger the condition. Five years after being told she had Raynaud's, she developed scleroderma: "I felt a tightening of the skin in my fingers and a reduced mobility of the finger joints," she recalls. Murphy also has problems with swallowing, which indicates that the scleroderma has affected her oesophagus (gullet). She takes the drug Losec for this, as well as Dilzem, which acts as a dilator of blood vessels, for the Raynaud's phenomenon.
Scleroderma requires specialist treatment, initially by a connective tissue disease expert, but also by other specialists depending on which of the body systems are affected. Raynaud's phenomenon, where there is no underlying cause, does not require drug treatment in the majority of patients. Calcium channel-blocking drugs, such as those used in the treatment of angina, will help with severe Raynaud's symptoms.
The Irish Raynaud's and Scleroderma Society (01-2350900) is holding a day-long conference for the general public on Saturday April 15th at Jury's Hotel in Dublin. The day starts at 10 a.m. and includes lectures on lung and dental problems associated with scleroderma, as well as practical information on Raynaud's phenomenon
Contact Dr Houston at mhouston@irish-times.ie or leave messages at 01-6707711 ext 8511