Sir Tommy Steele: how he overcame King’s disease

Lucky in ill health

At 82, Sir Tommy Steele has secured success as a singer, actor, author and sculptor – and has triumphed over King’s disease. “I’ve been lucky, mate,” Sir Steele told the Glasgow Times. “All my ill health happened before I was 11 and I was ill for four years.”

Steele revealed: “The war had been over three or four years, and I was trapped in a hospital bed. It was only when these red spots exploded on my ankles that the [doctors] realised I had the King’s disease.

“When I learned of the madness [that King George III suffered from] I panicked – and so did my mum.”

Kings’ disease

The English entertainer was diagnosed with porphyria (i.e. King’s disease), an incurable condition. The Mayo Clinic explained porphyria results from a buildup of natural chemicals that produce porphyrin in the body.

It’s believed porphyria is caused by a hereditary faulty gene, which can lead to skin sensitivity to sunlight.

The heme connection

The Mayo Clinic says: “All types of porphyria involve a problem in the production of heme. Heme is a component of haemoglobin, the protein in red blood cells that carries oxygen from your lungs to all parts of your body.

“Heme production, which occurs in the bone marrow and liver, involves eight different enzymes. A shortage (deficiency) of a specific enzyme determines the type of porphyria.”

Symptoms and treatment

When porphyrins build up in the skin, and that skin is exposed to sunlight, symptoms emerge. Treatment for cutaneous porphyria includes reducing exposure to triggers and medication.

Hydroxychloroquine or chloroquine can be taken, which absorbs excess porphyrins.

An alternative treatment method to help keep the condition under control is phlebotomy. Phlebotomy is when blood is periodically drawn to reduce the amount of iron in the body, which decreases porphyrins.

Supplementation with vitamin D all year round is also recommended as Sir Steele would most likely need to limit his exposure to direct sunlight.

Living with porphyria

The chronic condition, while incurable, is manageable with treatment and lifestyle adjustments. And it seems Sir Steele has lived up to the notion that people with the condition can go on to live a full and healthy life.

The Tommy Steele Story is showing on Saturday, March 25 at 1:10pm on BBC Two.

Credit: https://www.express.co.uk/life-style/health/1750370/sir-tommy-steele-health-porphyria-symptoms

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