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Cystic Fibrosis

What is Cystic Fibrosis

Cystic Fibrosis or CF is a hereditary disease that primarily affects the respiratory system, the digestive system and the reproductive system.  This is also known as mucoviscidosis, a common disease that is life threatening genetic disorder that causes disability and most of the time early death.

What Causes Cystic Fibrosis

Cystic Fibrosis is a genetic disease which means that this is cause by a defective gene.  The defective gene on the other hand is inherited from the child’s parents which is a recessive gene.  This means further that a person found to have cystic fibrosis should inherit two copies of the gene that should come one from each parent.  In cases that only one parent has recessive gene passed on to the child, that child will be free from cystic fibrosis but will be a carrier to pass on the gene to the next generation.

This defective gene alters a protein that regulates the normal movement of sodium chloride which further results in thick and sticky secretions in the respiratory, digestive and reproductive systems.  The amount of salt in the body secretion such as sweat increases under this condition.

Cystic Fibrosis Symptoms

In most places in Australia and United States, Cystic fibrosis can be determined even right after the child birth.  A screening is being done through a collection of blood sample.  If the blood test resulted to very high levels of Immunoreactive Trypsin or IRT, the DNA in the blood will be further analyzed.  However, there are also various symptoms which can be associated with the disease.  The signs and symptoms may vary from different persons and different life stages.  Some symptoms manifest at birth, while there are some may begin experiencing the symptoms until they grow older.  The symptoms may also depend on how severe the disease is as these are worsening or improving as time goes on.

Some of the symptoms that can be observed include:

  • Excessive salty taste to the skin. This can be determined when parents kiss their child and taste the salt on their baby’s skin.  Person affected by cystic fibrosis have higher salt body secretion that comes out during sweating.
  • As cystic fibrosis develops thick and sticky mucus, the air passages are being clogged which further manifested with persistent cough, wheezing sound, recurring lung infections, and some may have difficulty in breathing.
  • Cystic fibrosis also affects the digestive system thus frequent visit to the toilet may also be a sign of having this disease.  Relatively, the build up of mucus can block the tubes that carry digestive enzymes.  These enzymes coming from the pancreas will not reach the small intestine and further disable the intestines to absorb nutrients from the food we eat. Problems in the digestive system also come out with foul smelling and greasy stools as well as intestinal blockage especially in newborn babies.

Parents should be aware and need to seek medical attention at once if the above symptoms are already visible on their child.

Cystic Fibrosis Facts

Although cystic fibrosis may not be the most common disease in the whole world, this is very common to Caucasian or Ashkenazi Jewish origin.  There is only one for every three thousand two hundred babies in the world that can possibly have cystic fibrosis.  In Australia, a baby is born with cystic fibrosis every four days.  This is the most common genetically-inherited chronic illness in Australia nowadays.  One in every twenty five people is carrier of the cystic fibrosis gene.

In United States, there are approximately thirty thousand people who were infected by cystic fibrosis.  Every year there is around one thousand new cases and one child for every three thousand five hundred babies is born with cystic fibrosis.

In United Kingdom, on the other hand, there is one chance for every two thousand four hundred births is being diagnosed with cystic fibrosis and four per cent of the population are carriers of cystic fibrosis gene.

Cystic Fibrosis Pictures

Picture 1 : Manifestations of cystic fibrosis

Image source :  wikimedia.org

Picture 2 : Inheritance of Cystic fibrosis

Image source : diabetesmonitor.com

Picture 3 : Health problems with cystic fibrosis

Image source : hpathy.com

Cystic Fibrosis Treatment

There is no real treatment that cures cystic fibrosis.  However there are several treatments to slow the progress and manage the disease properly.  It is also essential to be very cautious in the manifestation and progression of different complications of cystic fibrosis in order to have proper treatment and save one’s life.

Some of the treatments may include:

  • Proper medications such as antibiotics to treat and prevent lung infections.  These can be through oral intake, through intravenous or through a nebulizer.  Mucus thinning drugs may also be prescribed to improve lung function as well as bronchodilators to open the airways and relax the muscles around the bronchial tubes.
  • Regular exercise like swimming and other related exercises that strengthen the lungs.
  • Physical therapy involving patting or manually clapping with cupped hands on the front and back of the chest to remove the mucus from the lungs.  Some mechanical devices may also be used like the chest clapper, inflatable vest and other breathing devices.
  • Intake of supplementary vitamins and salt supplement as well as enzyme replacement capsules.
  • Well balanced diet that is high in protein, fat and kilojoules.
  • Surgical and other procedures that may include but not limited to:

Threading of feeding tube through a patient’s nose to stomach temporarily or can be surgically implanted.  This will do the function of delivering the nutrition directly to the intestine as cystic fibrosis interferes with digestion system.

For severe breathing problems and life threatening pulmonary complications, a lung transplant may be applicable.  However this is a very critical procedure and considered as major operation.   Oftentimes, both lungs are affected by cystic fibrosis therefore both lungs should be replaced.   One has to be very vigilant in this procedure as well as with the manifestation of infections after the surgical procedure.

Some patients with cystic fibrosis may develop a blockage in bowel, thus an emergency surgery to remove it will be applied.

Cystic Fibrosis Diet

Since cystic fibrosis hardly affects the digestive system, it should be highly regarded the proper diet that a person should take.

A person diagnosed with cystic fibrosis should not eat large quantities of food that increase the production of mucus or can cause allergies such as:

  • dairy – milk, cheese and ice cream
  • eggs
  • peanuts
  • fruits like orange and bananas
  • food with too much sugar and saturated fats
  • wheat, barley, oats, rye
  • too much meat
  • too much salt

On the other hand, a cystic fibrosis patient should eat more quantities of food that reduce mucus build up and inflammation like:

  • garlic
  • onions
  • watercress
  • horseradish
  • mustard
  • parsley
  • celery
  • pickles
  • lemons
  • anti-inflammatory oils such as nuts, seeds, cold-water fish

A high-calorie diet and various vitamins supplements should also be taken into consideration.

Cystic Fibrosis Life Expectancy

A person with cystic fibrosis has a reduced life expectancy.  Fortunately, due to continuous research and studies on different treatments to manage the disease, the life expectancy of a person with cystic fibrosis is continuously increasing.  It initially started at around eighteen years, but now it reached up to more than fifty years although in rare cases.  The median life expectancy which was initially recorded at four years has now reached to thirty two years.  Studies show that there are over ninety percent of the affected newborns or infants are now surviving even beyond one year.

Cystic Fibrosis Gene and Carrier

Cystic fibrosis is a genetic disease. To understand better, every person has twenty three pairs of chromosomes. Each chromosome comes in pair with the same genes although not necessarily the same gene code.  Cystic fibrosis gene occurs when one of these genes is defective.  This is caused by a mutation of the cystic fibrosis trans-membrane regulator.  A person will most likely have cystic fibrosis disease if both parents are carrier of this gene.  If two people with this gene will have a child chances are:

  • one in every four chance will have cystic fibrosis
  • two in every four chance may not have cystic fibrosis but will carry the gene and can pass it on to the next generation
  • one in every four chance that the child will not have cystic fibrosis and at the same time will not carry the cystic fibrosis gene

Cystic Fibrosis Foundation

Cystic fibrosis foundation can be found all over the place like Australia, Canada and United States.  One of which is the Cystic Fibrosis Foundation or commonly known as CFF which was established in the United States during the year 1955.  CFF is a non-profit organization that aims to research about cure and other means to control cystic fibrosis.  The organization supports and finances various researches to assist people with the dreaded disease.  The foundation also serves as an avenue of information and data relative to cystic fibrosis to make people aware of things to expect, things to do in case a family member get the disease and convey everything to the world.  There are also various activities being sponsored by the organization to improve the quality of life of those people affected by this genetic disorder and their families.

References

http://www.mayoclinic.com/health/cystic-fibrosis/DS00287/DSECTION=symptoms



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