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6

Rheumatic fever

For the first eleven years of her life my mother was a normal healthy child, a non-identical twin with her sister Margaretta. Then it happened. At 12.35 p.m. on Friday, 16 October 1931 the shining white-hulled 14,000-ton Royal Mail Ship Corfu eased away from the passenger boarding berth at London’s Tilbury dock. It was the Peninsular & Orient liner’s maiden voyage, off on the London to Hong Kong run via the Suez Canal, calling at Southampton, Port Said, Aden, Bombay, Colombo, Penang and Singapore. On board were 170 first-class and 211 second-class passengers; among them were the twins, Margaretta and Helen, with their eight-year-old sister, Priscilla, and their parents, bound for Port Said.

It was the girls’ first experience of foreign travel, off to Cairo and on up the Nile to Aswan, where their father was to take up his post as a consultant engineer. During the passage through the Med, Helen developed an angry sore throat. Two weeks later, as they disembarked to travel upriver, she was running a high temperature. Glandular fever, the English ship’s doctor had declared. He was wrong. So began the problems that would direct the course of her whole life.

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Rheumatic fever has nothing to do with rheumatism. It derived its misnomer because some symptoms mirrored rheumatism. Commonplace in Britain during the world wars, it remains widespread in developing countries, especially those without ready recourse to antibiotics. It almost always affects children between the ages of eight and fourteen, eleven being the median norm, girls suffering more than boys. Caused by a bacterial Streptococcus pyogenes infection of the throat, nowadays it is easily treated with penicillin. If allowed to persist, it triggers an auto-immune response, which dramatically shifts the consequences of the disease from a fever to a far more devastating chronic condition. Early diagnosis and treatment are vital if the infection is to be prevented from sliding into full-blown rheumatic heart disease.

The infection causes the body’s immune system to produce antibodies whose function is to attack the Streptococcus bacteria. The sore throat goes away, but in the process those same antibodies are corrupted, unable to detect the difference between the bacterial infection they are supposed to be attacking and other important parts of the body, such as the lining of arteries, heart valves and the heart muscle itself. It is known as ‘antibody cross-reactivity’. Untreated, what appears to be just a nasty sore throat becomes a life-long, life-threatening illness.

The mitral valve is the most commonly affected, developing a thickening of the flaps, known as mitral stenosis, eventually failing altogether. The mitral and the aortic valves sit side by side. If the mitral valve doesn’t close properly the blood flow to the aorta, the main artery to transport blood round the body, reduces, affecting the rhythm of the heartbeat and slowing the whole circulatory system down. But the auto-immune attack doesn’t rest with the mitral valve, it spreads to the aortic valve, causing it to falter too. In severe cases the other two valves, the tricuspid and the pulmonary, also become damaged.

Even that isn’t the end of the story. Patients experience shortage of breath and energy and develop a heart murmur – the Carey Coombs murmur – caused by the blood regurgitating back and forwards between leaking valves. The heart is forced to work harder, dilating its muscle tissue, causing an overall increase in the size of the whole organ. The risk of severe strokes from clots passing to the lungs greatly increases.

Rheumatic heart disease is not just a horror that afflicts young children. The corrupted antibodies go on gnawing away at the heart valves and muscles for decades, gradually affecting its victims more and more, taking between ten and twenty years to reveal itself as a serious, chronic and ultimately life-threatening disability.

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Helen was taken to hospital in Cairo too late. The chance to kill off the infection had been missed. Antibody cross-reactivity had set in and she was irrevocably doomed to a life profoundly complicated by heart disease. Yet, true to form, those complications would not properly emerge for a further ten years, by which time she would be an attractive young woman just married to a tall and dashing Englishman.

Initially at a French convent in Zamalek, the affluent district of Cairo, genteel and cultured nuns had schooled her and her sisters. She was a bright, cheerful child, always smiling with a ready, rippling laugh and an irrepressible desire to please, secure and happy in their lush Gezira island-in-the-Nile home among flowering jacaranda, banana and golden mohr trees, and scented datura, whose white bell flowers swayed in the hot afternoon winds. But there was another side to her education.