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Cystic Fibrosis Disease

Cystic fibrosis can be described as an inherited disease, which causes thick and sticky mucus to accumulate within the digestive tract as well as the lungs. Cystic fibrosis disease is a fairly common chronic health condition, which can affect both adults and children. It is quite a serious problem, which can also result in death. Studies on cystic fibrosis facts indicate that 1 out of every 3,200 Caucasian children born in the United States has this disease.

Cystic fibrosis can be a result of a genetic or a birth defect.
There are more than 1,400 types of cystic fibrosis mutation that lead to this medical condition. Around 70% of all the people who suffer from this condition have inherited the gene from one or both parents. Therefore, those parents who suspect that their children may be at a high risk of this disease could opt for genetic testing, even before the child is born.

Cystic Fibrosis Life Expectancy

Studies indicate that cystic fibrosis life expectancy has been increasing in the last 40 years or so. Around 35 years ago, the average life expectancy of a person suffering from this condition was 14 years, which later increased to 18. Today however, the average cystic fibrosis life expectancy is 35 years or so.

Earlier, babies born with cystic fibrosis were not expected to live beyond 4 years, but that has now increased significantly. Presently, the cystic fibrosis life expectancy of a newborn is 32 years or so. It is believed that kids who follow a healthy cystic fibrosis diet and take the required medication can improve their overall health and well being and can live longer.

Cystic Fibrosis Causes, Risk Factors

People suffering from cystic fibrosis have a defective gene, which alters the main protein, regulating the usual movement of salt in and out of the cells. As a result of this, thick and sticky secretions are built up in the digestive tract as well as the respiratory system. The reproductive system can also be affected by this condition. It is possible for a child to be born with this disease, in case one of the parents happens to be a cystic fibrosis gene carrier. In order to get the disease, a child needs to inherit two copies of the recessive gene, one from each parent. If a child inherits only one copy of the gene, he or she will not develop the condition, but will be a cystic fibrosis gene carrier and can pass it on to their child.

There are several babies and children who do not show any of the symptoms of this condition, until they are much older. Several people are only diagnosed with cystic fibrosis when they are in their 20s or 30s. This does not mean that they get the disease at a later age; all people are born with the defective gene that causes this condition. However, since cystic fibrosis is a progressive condition, it worsens with time and the older the patient gets, the more marked the disease is likely to be.  

There are several people who check with their doctors if there is any connection between smoking and cystic fibrosis, i.e. if pregnant women are could be increasing the risks of this condition, by smoking. A woman can expose her unborn baby to several health problems and birth defects by smoking during pregnancy, but cystic fibrosis is not one of them. This medical problem only occurs because of a particular defective gene. Nevertheless, a study carried out by Canadian Researchers claims that some of the components of cigarette smoke can destroy defenses against cystic fibrosis. There is no further clinical evidence to prove this claim though.

There are several complications that can arise because of cystic fibrosis. Some of the effects that this condition can have on the body include:
  • Respiratory system complications, such as bronchiectasis, chronic infections, collapsed lung, nasal polyps and respiratory failure
  • Digestive system complications, which include intussusception, blocked bile duct, nutritional deficiencies, rectal prolapse and diabetes
  • Reproductive system complications, like infertility, mainly in men
  • Other health related complications, comprising electrolyte imbalances and osteoporosis
While there is a lot of information on cystic fibrosis causes and effects easily available through various resources, it is best to consult a doctor for accurate details.

What is the genetic cause of cystic fibrosis?

The genetic cause of cystic fibrosis is a mutation in the Cystic Fibrosis Trans-membrane conductance Regulator (CFTR) gene. This CFTR gene provides the required instructions to make a channel, for transporting the chloride ions, in and out of the cells. Chloride ions are negatively charged particles, which help in controlling the movement of water in the tissues, which is essential for the production of mucus. Therefore, the gene is instrumental in the important function of creating mucus, digestive juices as well as sweat.

At mutation in the CFTR gene cause a disruption in the functioning of the chloride channels, which in turn interferes with the flow of chloride ions as well as water across the cell membranes. Because of this, the cells that usually line the passageways of some of the organs, such as the pancreas, liver, lungs, and so on, begin to produce mucus, which is a lot thicker and stickier than normal. This thick mucus clogs the glands and the airways and consequently triggers off the signs and symptoms of cystic fibrosis. While defects in other genes cannot lead to cystic fibrosis, they can aggravate the condition. This is why some people are more severely affected by this disease, as compared to others.

Cystic Fibrosis Treatment

Unfortunately, there is no cure for cystic fibrosis, but by going through treatment in the earlier stages of the disease, the patient’s life expectancy and quality of life can improve a great deal. It is important to understand that the treatment of the condition is a lifelong commitment and requires regular follow-up and monitoring, in addition to a lot of patience and determination. Most of the cystic fibrosis treatment guidelines are aimed at:
  • Preventing lung infections and controlling them
  • Reducing the chances of and treating any blockages in the intestine
  • Decreasing the risks of dehydration
  • Loosening the thick and sticky mucus and getting rid of it from the lungs
  • Providing the body with adequate nutrition
Cystic fibrosis treatment options could require a patient to go through treatment for lung problems and bowel problems. Some of the most common cystic fibrosis treatment options include:
  • Medication like mucus-thinning drugs, anti-inflammatory medicines, antibiotics and bronchodilators to prevent lung related problems
  • Vitamin supplements, oral pancreatic enzymes and a special diet to improve the health of the digestive tract
  • Nutritional therapy for increasing strength and levels of physical activity
  • Lung transplant to deal with severe lung disease (However, this is an option only in a few severe cases and therefore, it is important to consult a doctor for the feasibility of lung transplant and cystic fibrosis)
Cystic fibrosis treatment for children may require hospitalization, depending on the severity of the condition. Most kids with this disease are advised to visit their doctors at least once a quarter. Cystic fibrosis treatment guidelines in children may include pulmonary therapy as well as nutritional therapy.

Cystic fibrosis treatment costs may vary considerably, depending upon the severity of the disease, as well as the drugs or therapies that are required. Several insurance companies in the United States also cover cystic fibrosis treatment costs in children, if the mother had taken a comprehensive medical insurance plan before getting pregnant.

Cystic Fibrosis Symptoms

There are several signs and symptoms that are seen in people suffering from cystic fibrosis, though they may not be evident for several years. Moreover, the cystic fibrosis symptoms in children are different from the signs seen in adults. Given below are some of the common cystic fibrosis signs in toddlers and newborn babies:
  • Delayed growth
  • Being underweight
  • Productive cough, which lasts for a long time
  • Recurrent instances of sinus or lung infections
  • Excessive fatty or odorous stools
One of the most important cystic fibrosis signs in babies is the absence of bowel movements within the first 48 hours of being born. Another sign of this condition in children is a salty taste to the skin, which is evident even when a parent kisses the child.

The cystic fibrosis symptoms in adults could also be quite varied, depending upon the severity of the condition. There are separate respiratory signs and digestive signs that are seen, which include:
  • Recurrent lung infections
  • Persistent cough
  • Repeated instances of sinus infections
  • Wheezing problems
  • Nasal congestion because of polyps
  • Severe constipation, which causes stomachache
  • Bloating and excess flatulence
  • Loss of appetite, accompanied by nausea and consequently weight loss
  • Pale colored stools that have mucus or a foul smell
  • Fatigue
As soon as any of these symptoms are evident, it is essential to consult a doctor.
Submitted on January 16, 2014