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What is cystic fibrosis? -

Cystic fibrosis (CF) is a progressive, genetic disease. The condition causes thick, sticky mucus to build up in the lungs and digestive system.

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Risk of lung infection -

CF is serious disease, with potentially life-threatening consequences. It can cause lung infections and problems with digesting food.

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Digestive disorders -

Besides interfering with the function of the lungs and digestive system, CF can also result in a host of fatal complications.

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Problems with the pancreas -

Build up of mucus can clog the pancreas—the organ that assists with digestion. This effectively stops enzymes reaching food in the gut and helping with the digestive process.

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Liver disease -

Cystic fibrosis can lead to liver disease by causing mucus to build up and block bile ducts in the liver. When this happens, bile is prevented from exiting the liver, which causes inflammation and produces scarring (fibrosis). In time, the liver ceases to function properly.

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Kidney complications -

Patients with cystic fibrosis are also prone to kidney disease. Ironically, increased longevity of those stricken with the disease are surviving long enough to develop kidney complications, concludes a study published by the National Center for Biotechnology Information.

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Newborn screening -

With newborn screening, cystic fibrosis can be diagnosed within the first month of life, before symptoms develop. Indeed, most cases of CF are picked up at birth using the newborn screening heel pinprick test. 

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Early onset -

It remains an unfortunate fact, however, that most symptoms of CF usually start in early childhood and vary from child to child.

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Historical perspective -

Some 60 years ago, CF was considered exclusively a childhood disease, with many infants succumbing to the disease before reaching elementary school age.

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Advances in treatment -

In the 1940s and 1950s, effective treatment of CF was still in its infancy.  Children simply didn't live long enough to develop, but instead died from malnutrition due to failure of the pancreas. Advances in medication to help combat CF in the 1960s helped, but the drugs needed were prohibitively expensive. This photograph shows a young CF sufferer in 1963 with the pills she needs to take to stay alive, according to the original picture caption. It adds that medication and equipment of cystic fibrosis patients cost up to US$200 a month. Adjusted for inflation, $200 in 1963 is equal to a staggering $1,700 in 2021!

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No cure -

Pictured in 1970 is a young boy receiving treatment for CF. The machine on the right is a nebulizer that delivers moist, medicated air into the plastic tent. The mist keeps his lungs and airways from clogging up, as does the face mask he needs to use several times a day. Advances in treatment mean that people today with CF often live into their thirties, forties, and beyond. Sadly, there is currently no cure for cystic fibrosis, and the Cystic Fibrosis Foundation estimates that there are 30,000 children and adults in the United States alone that have CF, with around 1,000 new cases diagnosed each year.

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Sweat test -

Besides the aforementioned heel pinprick test, CF in newborns can be detected with a sweat test. In the early days of CF research, it was ascertained that children with the disorder had abnormally high concentrations of salt, which led to a 'sweat test' being developed and first applied in 1948. It's still used today to provide a CF diagnosis by measuring the concentration of salt in a person's sweat.  

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Signs and symptoms -

Cystic fibrosis signs and symptoms vary, depending on the severity of the disease. While CF is often diagnosed early on in life, some people may not experience symptoms until their teenage years or adulthood.

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Chest infection -

Tell-tale CF symptoms are many and include recurring chest infections and wheezing, coughing, and shortness of breath. In time, the airways can become damaged and abnormally widened, a condition known as bronchiectasis.

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Jaundice -

Jaundice—the yellowing of the skin and whites of the eyes—is a sign that you may be suffering from CF symptoms.  Pictured is the hand of a healthy patient against one who has jaundice. 

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Respiratory disorders -

Inflamed nasal passages, a stuffy nose, or recurring sinusitis are respiratory disorders associated with the onset of CF.

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Diarrhea -

Diarrhea, constipation, and defecating foul-smelling, greasy stools are also potential symptoms of CF.

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Poor growth -

People with cystic fibrosis can experience poor growth and difficulty putting on weight.

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How do you get cystic fibrosis? -

CF is a genetic disease. To develop CF, you must inherit two copies of the cystic fibrosis transmembrane conductance regulator (CFTR) gene mutation from each parent.

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Adult carriers -

When two people who are carriers have a child, there is a 25% chance of giving birth to a baby with CF. When one parent has CF and one parent is a carrier, those odds increase to 50%.

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Carrier (or genetic) testing -

Carrier (or genetic) testing plays a key role in the diagnosis of cystic fibrosis. It can indicate whether or not a person carries one of the altered genes that causes CF.

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Treatment for cystic fibrosis -

Despite there being no cure for CF, a range of treatments can help control the symptoms and prevent or reduce complications. Crucially, it is vital for people with CF to get rid of mucus from their lungs to allow clear breathing and minimize lung infections. With the assistance of a therapist (pictured), airway clearance techniques can be administered to help sufferers achieve this.

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Inhaled medicine -

People with CF will most likely need to take different medicines to treat and prevent lung problems. Inhaled medication using a nebulizer (pictured) is effective at reaching the airways and is commonly used.

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Oral medication -

Medication to control CF may also be taken orally. Medicines for lung conditions include antibiotics and those that reduce levels of mucus in the lungs and make it thinner and easier to cough up.

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Keep up to date with routine jabs -

It's important that people with CF minimize the risk of any lung infection. They should keep up to date with all routine vaccinations, including getting a flu shot every year.

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Quit smoking -

Smoking is absolutely out of the question for anyone with CF. It's also imperative to avoid secondhand smoke.

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Vitamins and supplements -

As CF can play havoc with the digestive system, people with the disease should discuss their diet with their doctor. Good nutrition is vital in helping to control CF and ward off lung infections. Extra vitamins and supplements will likely be required to help balance the absorption of nutrients.

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Get physical -

Physical activity is a great way of clearing mucus from the lungs and improving overall health and vitality. Swimming, running, or soccer are excellent options. But it's a good idea to supervise or partner up anybody with CF who undertakes exercise, especially youngsters.

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Living with CF -

Remember, effectively managing cystic fibrosis is key to making the condition easier to live with, and treatments are available to reduce the problems caused by the debilitating impact of the disorder.

Sources: (Frontiers in Medicine) (Cystic Fibrosis News Today) (Stanford Children's Health) (Medical News Today) (NHS) (American Lung Association) (Cystic Fibrosis Australia) (Mayo Clinic) (Cystic Fibrosis Foundation)

See also: Heartburn? Here's how to tame the flame

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