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Cystic Fibrosis is a genetic disease that effects mostly
people of Scandinavian, British and Irish ancestry. The
emigration, in recent centuries, of these peoples has led to
Cystic Fibrosis appearing in large numbers across North
America and Australasia.
Cystic Fibrosis is a recessive genetic disorder, which means
that both parents need to be carriers in order for a child to
be born with the disease. Many prospective parents are unaware
that they carry the Cystic Fibrosis gene until they have a
child with Cystic Fibrosis (CF). The reason for this is that
carrying the faulty gene does not appear to adversely effect
the health of the carrier, although there is some debate about
this as carriers tend to have higher rates of certain
disorders (chronic rhinitis for example) as compared to the
general population.
Both parents being carriers does not mean that all potential
offspring will have CF because carriers will have a pair of
genes one faulty and one healthy. This means that the parent
has a 50% chance of giving their child the faulty gene
resulting in the following odds: chances of a child being a
carrier 2 in 4 (50%), chances of child who is CF 1 in four
(25%), chances of child not having any faulty CF genes 1 in 4
(25%).
The
odds outlined above are for every birth, therefore it is
possible in a family of four children, that all or none of the
children have CF or are carriers.
The popular conception of CF is that it is a disease affecting
the lungs and although this is true, it may be better viewed
as a disease resulting in excessive mucous production which
causes damage to the lungs, pancreas, liver, sinuses, and
reproductive areas.
One of the major causes of death in the past was the damage to
pancreatic function which stopped enzyme production needed to
break down the fats in food, resulting in malnutrition and
weight loss. CF patients take pancreatic enzymes with their
food and some drinks (Coca Cola because of the vegetable fats
but not lemonade) in order to digest the fats.
Damage to the Pancreas can also result in the person with CF
developing type one diabetes which would necessitate insulin
injections along with the daily barrage of antibiotics used to
fight lung infections.
Reproductive areas are affected by the excessive mucous
including CF males. In the CF male, the Vas Deferens becomes
blocked and in the female the excess mucous makes conception
difficult.
The most damaging effect of CF is the damage to the lungs by
the mucous which itself creates the perfect environment for
bacterial infections that can eventually lead to irreversible
damage and eventual death. It is the area of lung function
that stem cell research offers the greatest hope. Stem cells
could be used in the future to rebuild the lungs of CF
sufferers using their own stem cells whilst replacing the
faulty gene with a normal one. |