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15 Surprising Facts About Cystic Fibrosis

Cystic Fibrosis, also known as CF, is a rare inherited disorder that affects the lungs, digestive system, and other vital organs of the body. These systems are usually severely destroyed by the disorder. The cells affected by CF are those that produce mucus, sweat, and digestive juices.

These secretions and fluids are normally slippery and thin and very important to the body. However, in people with CF, when there is a defective gene, the secretions become sticky and thick. This abnormality makes them stop acting as lubricants, and they plug up in the tubes, ducts, and passageways.

The lungs and pancreas are majorly affected by Cystic Fibrosis. After they clog, long-term issues like having difficulties in breathing and coughing up mucus come up. Patients with cystic fibrosis also present with sinus infections, stunted growth, finger clubbing, and fatty stool, which leads to infertility in males.

Cystic Fibrosis comes with different degrees and presentations of symptoms. This condition is progressive and requires daily care for patients affected. Patients with cystic fibrosis are able to carry out their daily activities as well as attend school.

Initially, people living with CF never had a quality life, unlike these days whereby they live better and quality life. Various treatments and screening have improved people living with CF who can live for more years. Here are the 15 Surprising Facts About Cystic Fibrosis;

1. CF is A Rare Genetic Disorder that Affects Different Parts of the Body

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Many people do not have the full idea of what Cystic Fibrosis is. This is a rare disorder that is inherited and can be passed down through generations.

Even though it is a rare disorder, CF can affect multiple body organs which makes it a severe and sometimes fatal condition. It majorly affects the lungs, but it can also affect other body organs like the pancreas, liver, and small intestines.

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2. Mutation in the Gene Cystic Fibrosis Transmembrane Regulator Causes CF

You might ask yourself how Cystic Fibrosis comes into existence or what causes it. Cystic Fibrosis is caused by a mutation in a special gene, the cystic fibrosis transmembrane conductance regulator (CFTR).

The commonest mutation which is ΔF508 leads to the deletion of three nucleotides. Deletion of these nucleotides leads to the loss of the amino acid phenylalanine at its 508th position on the protein.

What is more astonishing is that the deletion of these nucleotides does account for 70% of cases of CF worldwide. It also accounts for 90% of cases in the United States.

3. Cystic Fibrosis Occurs When Two Carriers have Children

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Statistically, one in 25 individuals who have their ancestry from Northern Europe are considered to be genetic carriers. This means that they have the genes that can lead to cystic fibrosis.

When two of these individuals who are carriers get married and have children, the disease appears. Each pregnancy between the two carriers has a 25% chance of getting a child who has CF.

4. CF is Not Common in Other Ethnicities Like in Whites

The test screens for different mutations depend on an individual’s ethnicity or whether CF runs in the family. One variant of the CF gene affects more than 10 million Americans, including one in every 25 white people.

Cystic Fibrosis is not common in other ethnicities but it is very frequent in Whites. A CF gene mutation is present in roughly one in 46 Hispanic Americans, one in 65 African Americans, and one in 90 Asian Americans.

5. The Disorder Can be Diagnosed Easily in New-borns

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In the United States of America and other developed countries, it is very easy to pick out and diagnose individuals with cystic fibrosis. This is done by screening for cystic fibrosis in newborn babies.

Immediately they are born and/or in the first days of life, blood tests for high levels of immunoreactive trypsinogen are carried out. When the baby is diagnosed with CF, they are started on treatment and proper management.

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6. Suspected New-borns Undergo Sweat Tests to Rule Out the Disorder

The early tests and screening help in getting to group the newborns as carriers or infected with the disease. For those who return positive tests and those suspected of having CF based on family history, several tests are done to confirm the existence of the condition.

They have to undergo a sweat test which is a definite test to confirm the diagnosis. Physicians use an electric current that drives pilocarpine into the skin which stimulates sweating.

The produced sweat is harvested for analysis of salt levels. If it turns out that there are unusually high levels of chloride in the sweat, it becomes suggestive CFTR is dysfunctional. This is when physicians concluded that the child has cystic fibrosis.

7. CF Manifests Early in Life

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Parents tend to spot this condition in their children early in life because CF manifests early in life. The newborns and infants who suffer from CF usually have frequent, large, greasy stools and are often underweight compared to their ages.

A large percentage, 15-20% of these babies get their intestines blocked by meconium which requires surgery to correct. Some newborns present with neonatal jaundice because of the blockage of the bile ducts. Parents tend to see crystallized salt on the skin of their children because of excessive loss of salt.

8. Cystic Fibrosis Leads to Blockage of Narrow Passages of Affected Organs

Vital body organs normally produce secretions that are used to regulate various body processes. However, when affected by CF, there is blockage of the narrow passages of affected organs. This comes up from the thickening of the secretions.

The blockage caused by the thick secretions leads to remodeling and infection in the lung. There is usually an accumulation of damaged digestive enzymes in the pancreas and blockage of the intestines by thick feces.

9. CF Can Lead to Chronic Infections in Affected Lungs

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People with cystic fibrosis develop bacterial colonization and infection in their lungs at a young age. These bacteria grow in the altered mucus that builds up in the tiny airways of the lungs and is frequently contagious among CF patients.

This mucus promotes the development of biofilms, which are tough for immune cells and drugs to penetrate and are bacterial microenvironments. By gradually redesigning the airways, viscous secretions, and recurrent respiratory infections continuously harm the lungs, making it much more challenging to treat the infection.

10. Patients with CF are Isolated to Prevent Cross Infection Unlike in the Past

Chronic Infections can be spread easily from one patient with CF to the other. Previously, CF patients used to participate in summer CF camps and several other recreational gatherings. This was a major risk that led to the cross-infection of chronic infections as non-sterilized nebulizers were shared.

However, this gradually changed when scientists made the discovery of cross-infection. In recent days, patients with CF are isolated to prevent the menace of cross-infection. Health providers are also advised on wearing gowns and gloves when examining them to prevent contracting virulent infections.

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11. CF has No Cure

Cystic Fibrosis is another major disorder that does not have a known cure. CF does not have a cure because it is a genetic condition that affects the genes.

However, early diagnosis of cystic fibrosis is encouraged to start palliative management in the affected individuals. With the advancement of technology and health, there is hope that a permanent solution will be achieved.

12. Individuals with CF Can Live Fuller Lives with Less Encumbered by the Disorder

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Infants now are likely to live well into adulthood, but infants born with it 70 years ago would not have survived through their first year. Cystic fibrosis patients can now live more fully and with less restriction due to their condition thanks to recent advancements in therapy. The prevention of airway infection, promotion of a healthy diet, and promotion of an active lifestyle are the cornerstones of management.

13. Pulmonary Rehabilitation & Occupational Therapy are the Standard Management of CF

Without having cure, many might think that there are no ways of managing cystic fibrosis. Pulmonary rehabilitation and occupational therapy are the major ways of managing cystic fibrosis.

Pulmonary rehabilitation usually aims at maximizing organ function and improving quality of life. Some occupational therapy techniques like the use of energy conservation techniques also help in the process of managing CF patients.

Another method of managing cystic fibrosis is administering intravenous antibiotics. Giving the patients probiotics also help in boosting the immunity of the affected.

14. CF is One of the Life-Shortening Conditions that Causes Infertility in Males

There are several genetic conditions in the world other than cystic fibrosis. These infections are usually life-shortening.

Cystic Fibrosis has been ranked as one of the life-shortening conditions in the world. The worst part is that it can lead to infertility in males.

15. The Prognosis of Cystic Fibrosis Has Improved Over the Years

Earlier on, cystic fibrosis was a life-threatening condition to those who were infected. The prognosis of it was so poor and many people lost their lives.

However, as technology advances, the prognosis of cystic fibrosis has improved over the years. Quality of life has been improved after early diagnosis and screening which usually follows better management.

Cystic Fibrosis can be a killer condition if it is not diagnosed earlier in the life of an individual. People living with CF have to be accepted by society for them to live a better life.

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