27th April 1997

Your Health

Crossing a Czech

by Dr. Sanjiva Wijesinha

My friend Tomas is a very fortunate man. His first stroke of good fortune was to be born in Prague during the Dubcek era.

Unfortunately, since Alexander Dubcek was a visionary born 25 years before his time, his era was shortlived - so in 1968, once the Soviet tanks rolled into Prague, Tomas's parents decided to migrate to Australia.

Here, Tomas had his second stroke of good fortune. He went to a good school, entered Melbourne university, and graduated as a doctor. Which is how I came to meet him, since we found ourselves working in the same hospital.

Tomas's third stroke of good fortune came when, in University, he met, fell in love with, and married a Sri Lankan medical student. By the time I met Tomas, he and Nelun had a beautiful six year old child - who seemed to have inherited her father's intellect, her mother's good looks - and the devil's own charm! Tomas used to proudly refer to her as "our crossed Czech".

Having told my readers all about Tomas's good fortune, I must mention that his family had had some bad luck going for it as well. Tomas's parents had lost three of their five children in early childhood - victims of an inherited disease called Mucoviscidosis or Cystic Fibrosis.

This disease (which I never saw even once when I was a medical student in Sri Lanka) is the result of the body's mucus and sweat glands not functioning normally. The sweat is highly concentrated and salty, while the mucus produced by the digestive system and lungs becomes abnormally thick and sticky. Consequently, children with Cystic Fibrosis cannot secrete digestive enzymes like pancreatic juice; they therefore cannot digest their food properly and so fail to thrive. Their airways also become clogged and so they suffer from persistent coughs and chest infections, and since there is no cure for the disease, until a few years ago most affected children died before they reached adulthood.

Cystic Fibrose, which is almost entirely confined to families of European origin, is inherited only if both parents have the abnormal Cystic Fibrosis (CF) gene.

Now all babies inherit 23 pairs of chromosomes from their parents, and these 46 chromosomes contain the genes which carry the characteristics which we inherit from our ancestors. The abnormal gene that is responsible for Cystic Fibrosis is carried on chromosome number 7 - but for a child to be affected, he must receive a CF gene from each of his parents. A person with only one CF gene, therefore, will not have the disease. He or she is termed a carrier (because they carry the abnormal gene) and can pass the gene on to their offspring.

If both parents carry the CF gene, their children have a one in four chance of being born with the disease - as happened to three of Tomas's unfortunate siblings. If however, a carrier (as Tomas very likely is) marries a non-carrier (as Nelun almost certainly is) then it is virtually impossible for their children be born with Cystic Fibrosis.

If Tomas had remained in Czechoslovakia where there are more carriers of the CF gene than there are in Sri Lanka, he would have had a much greater chance of producing a child affected by Cystic Fibrosis. By ensuring that his children acquire genes from Sri Lanka, Tomas has made certain that they will not suffer from the same terrible disease that killed his brothers and sisters. Which, Tomas says, is one (of many) good reasons for marrying a Sri Lankan girl rather than finding himself a Czech mate. After all, he adds, what better way of crossing a Czech?

Continue to Plus page 10 - Arun Dias Bandaranaike: The man behind the voice

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