Cold hands, warm heart, and a rare disorder

Raynaud’s Phenomenon, which affects 5 per cent of the population, is uncomfortable but not dangerous

THE ADAGE “Cold hands, warm heart” might describe me accurately if it also included “cold feet.” Every autumn, even before the leaves begin to fall from their airy perch, I begin an annual search for better ways to keep my hands and feet from freezing during the coming winter.

My investment in mittens and boots could stock a store and includes what is touted as the warmest of warm, but so far no product has been sufficiently protective. The popular advice, “Move to a warmer climate,” doesn’t mesh with my life’s interests, and so the search continues.

I may or may not have a version of Raynaud’s phenomenon, but I can surely empathise with those who do. First described in 1862 by a French medical student named Maurice Raynaud, it is characterised by highly localised cold-induced spasms of small blood vessels that disrupt blood flow to the extremities, most often the fingers and toes and sometimes also the tips of the ears and nose. Affected areas typically turn white when vessels collapse and cut off blood flow, then blue for lack of oxygen-rich blood, then red as blood flow is gradually restored when the areas rewarm.

While it is normal for blood to shift toward the body’s core to restrict heat loss when a person is exposed to cold, this reaction is exaggerated in the extremities of people with Raynaud’s phenomenon, also often called Raynaud’s syndrome or disease. Small vessels that supply blood to the skin of the fingers, toes, ears and nose overreact to cold, developing spasms that greatly reduce or completely shut down blood flow to these areas. The weather need not be cold to trigger an attack of Raynaud’s. Moving into an air-conditioned room to escape summer’s heat will do it as well.

The same reaction can occur when a person with Raynaud’s is under stress and the body undergoes the kind of fight-orflight response that protects animals in danger by shunting blood to tissues needed to aid survival.

In a recent review of the disorder in The New England Journal of Medicine, two professors at Johns Hopkins University School of Medicine, Dr Fredrick M Wigley and Nicholas A Flavahan reported that Raynaud’s affects between 3 per cent and 5 per cent of the general population. It occurs in two forms: primary, which is most common and has no known underlying cause; and secondary, in which it is associated with another disorder, usually a connective tissue or autoimmune disease like scleroderma, rheumatoid arthritis or Sjogren’s syndrome. People who work with certain chemicals, like vinyl chloride, or vibrating tools like a jackhammer are also susceptible to secondary Raynaud’s.

Primary Raynaud’s typically begins relatively early in life, between the ages of 15 and 30, and up to half of these individuals have a first-degree relative — parent, sibling or child — who also has the disorder. Women are more often affected than men.

As uncomfortable as it can be, primary Raynaud’s is not dangerous and is not as severe as secondary Raynaud’s, which has a later onset, usually starting after age 35 or 40. In rare severe cases in which blood flow is chronically diminished, secondary Raynaud’s can result in skin ulcers and even gangrene that require surgery.

When people with Raynaud’s are exposed to cold or are under stress, normal nervous system-induced constriction of the arterioles in these anastomoses is enhanced and may temporarily cut off blood flow to the affected parts, causing them to turn white and feel cold and numb. When the areas are rewarmed and the spasm resolves, blood flow resumes, often causing tingling or throbbing.

There is no cure for Raynaud’s, and remedies supported by solid scientific evidence are few. But there are well-established measures that can minimise its effects. Most important of these is to stay warm. For me that means wearing multiple insulating layers, especially on my arms and legs, so that any extra heat generated can transfer to my hands and feet. It’s a trick I learned ice-skating and skiing and that I’ve applied to cycling and hiking in New York winters. Wearing a hat and neck gaiter and a wind-resistant outer garment is also very helpful.

If you smoke, don’t. Nicotine causes a drop in skin temperature that adds to the problem. Certain medications that constrict blood vessels can also make matters worse. They include beta-blockers; many cold remedies, especially those that contain pseudoephedrine; some migraine remedies; and some blood pressure medications.

If undue stress triggers a Raynaud’s attack, try to avoid stressful situations and routinely practice stress-reducing techniques like meditation, progressive muscle relaxation or the relaxation response.

Regular physical exercise, while good for everyone’s well-being, is said to be especially helpful for those with primary Raynaud’s. Any activity that increases heart rate fosters circulation of warm blood to the extremities.

Although there are no drugs approved to treat Raynaud’s, calcium channel blockers, commonly used to treat high blood pressure, can be helpful. They dilate small blood vessels by relaxing the smooth muscles around them. NYT

Illustration: Manali Ghosh