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Nachtengel
Sunday, May 23rd, 2010, 07:27 AM
Islanders suffer from rare genetic illness in far greater numbers than people in the rest of the world

A deadly genetic disease that is normally very rare is apparently hitting people on the Faroe Islands hard, according to new research.

The illness is called carnitine transporter deficiency and typically affects only around 1 in 50,000-100,000 people. In Denmark only two Danes have been diagnosed with the disease in the last 30 years.

But on the Faeroe Islands health authorities have already found nearly 100 people with the condition in just the past few months.

People with CTD are lacking carnitine, a natural substance used by cells to process fats. Without it the body is unable to convert the fats into energy, and this can cause the heart to pump too hard and suddenly stop.

Researchers had already been aware of the relatively high incidence of CTD on the Faeroes, but the seriousness of the situation really took hold early last year, when a 21-year-old man died of cardiac arrest at his dorm room. His sister had died a few years earlier, also of a heart attack.

While the family of the two pressured health authorities for answers, another young woman died of cardiac arrest this past January. This led to health authorities to initiate a free screening programme a few months ago for all 48,000 of the nation’s residents.

Those who have been diagnosed with the disease will now receive carnitine supplements to correct the illness’ detrimental effects.

But many Faeroese have been shaken by the breadth of the genetic disease among their population – one which often kills young adults.

Sissal Kampmann, who lives in Denmark but comes from the Faeroe Islands, has two brothers who were diagnosed with CTD through the screening programme.

‘You really panic with this type of illness where you can’t see anything and you have no clue as to whether you’re healthy or not,’ she told public broadcaster DR. ‘And then I began worrying that my daughter might also be ill. It’s been a shock.’

Health authorities estimate that 1 out of every 500 Faeroese probably carry the gene that causes CTD. It was only 15 years ago that the first cases were discovered, found in two children by a paediatrician.

Researchers at Rigshospitalet in Copenhagen are now racing to examine as many Faroese as possible for CTD.

‘Unfortunately we’ve seen people die from this disease and it can happen very suddenly, so it’s important we find the affected patients and get them treated as soon as possible,’ said Dr. Allan Meldgaard Lund.

Lund said CTD can jump generations in a family, but to contract the illness both parents must carry the gene.

Up to 10 percent of the Faroese population appear to be bearers of CTD, according to studies. And many marry other Faeroese, increasing the risk of their children developing the disease.

But CDT is not the only genetic defect plaguing the Faroese. Researchers say that between 8-10 other serious genetic illnesses are proving to be far more widespread in the Faeroes compared to the rest of the world – including cystic fibrosis, liver diseases and other physical and mental disabilities.

http://www.cphpost.dk/news/135-science/48995-faeroes-susceptible-to-deadly-illness.html

wittwer
Thursday, September 9th, 2010, 10:25 PM
Could this be possibly be due to close inbreeding on the island among various families giving rise to genetic abnormalities? Here in the States, we have seen a similiar phenomenon in isolated geographic locations causing physical and mental abnormalities which are believed to be genetic in origin. Such is the reason for legal strictures against marriage between first through third or fourth cousins.

Oski
Thursday, September 9th, 2010, 11:12 PM
Simple solution:

http://en.wikipedia.org/wiki/Lebensborn

wittwer
Thursday, September 9th, 2010, 11:32 PM
Yeah, Yeah, the Nazi's had a Eugenics program called Lebensborn, but not a lock on Eugenics knowledge nor it's medical implications... The Nazi model, been repudiated the World over.

Hamar Fox
Saturday, September 11th, 2010, 11:48 AM
Could this be possibly be due to close inbreeding on the island among various families giving rise to genetic abnormalities? Here in the States, we have seen a similiar phenomenon in isolated geographic locations causing physical and mental abnormalities which are believed to be genetic in origin. Such is the reason for legal strictures against marriage between first through third or fourth cousins.

Reproduction between third cousins has been shown to produce healthier offspring than between unrelated individuals, and as far as I know marriage between first cousins is legal, if not everywhere, then nearly everywhere, let alone between fourth cousins. It's also been the norm throughout human history.

The 'inbreeding is bad' myth definitely has its origins in the US, and is still held to more strongly and irrationally by Americans, even though it's sadly infecting the mindset of much of Europe. Its origins are twofold: A strong prejudice in the US against rural America, its perceived practices, and the perceived genetic quality of its population; and secondly, the mixed origins of the elitist urban Americans who created the myth.

Hellbound
Saturday, September 11th, 2010, 03:07 PM
I heard about that too. People fail to realise that we evolved in isolated conditions. Close family is bad but a large enough isolated community will produce the healthiest offspring.

Matamoros
Wednesday, September 22nd, 2010, 10:08 AM
Could this be possibly be due to close inbreeding on the island among various families giving rise to genetic abnormalities? Here in the States, we have seen a similiar phenomenon in isolated geographic locations causing physical and mental abnormalities which are believed to be genetic in origin. Such is the reason for legal strictures against marriage between first through third or fourth cousins.

This is a difficult subject area to understand, often having as much to do with statistics as with biology.

The relatively high level of genetic diseases could be due to any one (or a combination) of a number of causes, of which I believe the following are most likely:

1) Founder effect. The Faeroe Islands were originally settled by only a few thousand people. Even if only a few individuals in this group carried the allele concerned, the proportion of the population carrying it could be higher than the population in general, due to the small population of the island. It would then it would have spread as population of the island grew.

2) Disease protection. During the plague (black death) in the mid-fourteenth century the population fell as low as 2,000. It has been shown that some alleles can give partial or complete protection against infectious diseases, even when carrying two copies of the allele is undesirable. The most common example of this is sickle-cell anaemia - if one allele is carried it has little affect on the efficiency of the blood cells, but does provide some protection against malaria. A similar theory exists for the prevalence of cystic fibrosis in some populations, and another for the existence of a gene in some Europeans which prevents HIV infection. This is known as a heterozygote advantage.

It is unlikely to be caused by inbreeding, if this has ever occurred. Perhaps it would be more correct to say that the small size of the population, and limited opportunities for genetic flow have given rise to the prevalence of the disease.

It's also important to remember in these discussions that your fourth cousin is unlikely to be more genetically similar to you than a person picked at random from the population.

Matamoros
Friday, September 24th, 2010, 11:04 PM
After further consideration, I have come up with an analogy which may help people understand this a bit better.

Consider the gene pool being like a pool of water. The CTD allele is like a ripple in this pool of water, spreading outwards in all directions. However, for it to be expressed, there need to be two alleles, so the ripple must intersect another ripple before this disease is expressed. However, in the Faeroe Islands, the pool is much smaller than in other countries, so once the ripple reaches the wall of the pool, it reflects back inwards, where it will encounter other ripples also reflected from the other side of the pool.

I'm not sure how helpful that will be! Hopefully it helps with someone's understanding. :)