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Lifestyle changes to adapt when you have Cystic Fibrosis

Cystic fibrosis or CF is a hereditary condition that affects your lungs and digestive system. This disease changes the way your body produces mucus, digestive juices, and sweat.

More about cystic fibrosis

In the case of a healthy person, the mucus is slippery and thin. However, in a person with CF, it tends to become gluey and thick. As a result, it starts blocking the ducts and tubes throughout your body (mainly the pancreas and lungs) that it is supposed to lubricate.

Over a period, this sticky mucus starts to accumulate and settle-down inside your air passages and leads to breathing problems. Also, germs and pathogens stick to the secretions and make room for infections.

Cystic fibrosis can also damage your lungs and form scar tissue (fibrosis) and fluid-filled sacs (cysts).

What are the symptoms and signs of cystic fibrosis?

This disease’s signs and symptoms vary from person to person, depending on the severity and duration of its presence. Moreover, these can also change (improve or worsen) over time in the same person.

In some cases, a person with CF may not experience any signs or symptoms until they are teenagers or adults. In cases where the doctors do not diagnose the disease until adulthood, the condition is likely to be moderate, and the symptoms are atypical (uncommon). It includes – recurring pneumonia, episodes of pancreatitis, and infertility.  Cystic fibrosis can lead to an increase in the salt content of sweat.

Usually , signs of the disease are mainly related to the digestive and respiratory system.

Respiratory symptoms

  • Wheezing.
  • Recurring lung infections.
  • Exercise intolerance.
  • Bouts of sinusitis.
  • Persistent coughing with thick sputum (mucus)
  • Nasal (nose) congestion.
  • Swelling in the nasal passages.

Digestive symptoms

When the thick mucus blocks the path of digestive enzymes on the way to your small intestine from the pancreas, your intestine cannot absorb nutrition from your food adequately. It results in the following –

  • Greasy stools.
  • Foul-smelling stools.
  • Chronic constipation.
  • Frequent or difficult bowel movements.
  • Intestinal blockage.

When is it time to seek medical attention?

As cystic fibrosis is a progressive genetic condition, regular screening is crucial at least once every three months. Get in touch with your doctor if –

  • You, your child, or any other family member has this condition.
  • You are experiencing any of the symptoms.
  • Your symptoms are worsening.
  • You have breathing problems, severe chest, or stomach pain.
  • You see blood while coughing.

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What are the risk factors of cystic fibrosis?

Cystic fibrosis is a genetic disorder that moves from one generation to the other in a family. Therefore, a family history of CF is the most significant risk factor.

What are the  causes of cystic fibrosis?

Cystic fibrosis is a genetic disorder that occurs due to a mutation in a gene. That gene is known as cystic fibrosis transmembrane conductance regulator (CFTR). This changes a protein which regulates movement of salt in and out of cells. The result is sticky, thick mucus in the digestive respiratory and reproductive systems, as well as increased salt in sweat.

As CF is a hereditary disease, you will get this condition from your parents only when you inherit (receive) a copy of your father and mother’s defective gene. If you get only one copy, you will not get this disease. However, you will act as a carrier of the defective gene and pass on to the next generation.

What are the complications of cystic fibrosis?

Respiratory complications –

  • Damaged bronchial tubes (airways)
  • Severe chronic infections
  • Nasal polyp
  • Blood in sputum
  • Pneumothorax (collapsed lungs)
  • Respiratory failure
  • Acute respiratory exacerbations

Digestive complications –

  • Diabetes
  • Liver diseases
  • Nutritional deficiencies
  • Intestinal blockage
  • Distal intestinal obstruction syndrome (DIOS)

Reproductive complications –

  • Reduced fertility (women)
  • Infertility (men)

What are the treatment options for cystic fibrosis?

Although cystic fibrosis is not curable, treatments aim to help you reduce the complications, relieve the symptoms, and improve the outlook of the disease. Also, regular screening and early medical intervention can slow down the progression of the same.

This health condition can be  complicated. Therefore, it is essential to get treatment at a healthcare facility with a team of medical professionals .

Your doctor will work on the following goals to treat you –

  • Preventing and managing lung infections
  • Providing sufficient nutrition
  • Treating and managing intestinal obstruction
  • Loosening and removing mucus from the lungs

The treatment options include the following –

Medications for gene mutation

Doctors may suggest CTFR (cystic fibrosis transmembrane conductance regulator) modulators for those with cystic fibrosis having certain gene mutations,. These newer medicines help improve the function of the faulty CFTR protein. The medicines may improve lung function and weight, and also reduce the amount of salt in sweat.

The FDA-approved medications include –

  • The latest combination of three medications comprises – Tezacaftor, Elexacaftor, and Ivacaftor. This combination is known as Trikafta. It is safe for people who are 12 years old or more.
  • The combination of Tezacaftor and Ivacaftor, also known as Symdeko, is allowed for those aged  6 years old or more.
  • The combination of Lumacaftor and Ivacaftor, known as Orkamni, is for individuals who are 2 years old or more.
  • Ivacaftor, also known as Kalydeco, is effective on 6-months olds or more.

Other medications include –

  • Antibiotics for preventing and treating infections in the lungs
  • Mucus-thinning medicines
  • Anti-inflammatory drugs for treating swollen airways
  • Inhaled medicines for opening up your airways
  • Pancreatic enzymes for better absorption of nutrients
  • Stool-softening for constipation
  • Disease-specific medications, if you have diabetes.
  • Acid-reducing pill for aiding digestive enzymes to work properly

Airway cleaning treatments

Airway clearance is also known as chest physical therapy or CPT. This therapy will help you in loosening and getting rid of mucus. There are different types of CPT. It includes –

  • Clapping on the back and front part of your chest with cupped hands
  • Applying certain mucus-loosening coughing and breathing techniques
  • Using oscillating devices to ease coughing while loosening the mucus.

Pulmonary rehabilitation

Depending on your health condition, your doctor may also suggest going for long-term pulmonary rehabilitation programs. Such programs often include the following  –

  1. Physical exercise that can improve your condition
  2. Breathing techniques that may improve breathing and help loosen mucus
  3. Nutritional counseling
  4. Counseling and support
  5. Education about your condition

Surgery and other medical procedures

Other treatment procedures include –

  • Oxygen therapy
  • Nasal and sinus surgery
  • Non-invasive ventilation
  • Bowel surgery
  • Feeding tube
  • Lung transplant
  • Liver transplant

What lifestyle changes should you consider making when you have cystic fibrosis?

Some simple lifestyle changes can make it easier to manage this condition better. These include:

Paying attention to your diet and fluid consumption

Cystic fibrosis can lead to malnourishment. The reason is because it  limits the digestive enzymes  from reaching  the small intestine, resulting in malabsorption of food. Therefore, the calorie intake of people with this condition should be more than people without CF. Maintain a healthy diet and make sure to drink lots of water and other fluids. If you are not sure what to eat and what to avoid, you can get in touch with a dietician.

Your doctor is may recommend the following –

  • Anti-acid medications
  • Pancreatic enzymes with every meal
  • Fat-soluble vitamins
  • High-calorie nutrition
  • Extra fiber
  • Additional salt intake, mainly before working out or during hot weather

Get vaccinations on time.

Besides other regular vaccines for children, your doctor will recommend getting an annual flu vaccine if your child has CF. Although cystic fibrosis does not hamper your immunity, kids with this condition are likely to experience various health complications when they fall ill.

Workout

Do any exercise that you find comfortable, and that gets you going, such as biking, walking, jogging, etc. Exercising will keep you fit and also help you with mucus loosening.

Quit smoking

If you smoke, quit. Make sure no one smokes when you or your kids are around. Passive smoking can be dangerous, especially when you have cystic fibrosis.

What preventive measure can you take?

Here are some preventive measures to consider –

  • If you or your partner have CF or this condition is there in your family, make sure to go for genetic testing before conceiving.
  • Also, take all medicines on time and do not miss your appointments.

Medical attention is crucial!

You or anyone in your family who has CF may experience emotions like anger, fear, depression, or anxiety. Make sure to keep your calm and handle the situation carefully or, if needed, seek help.

Frequently Asked Questions (FAQs)

  1. What methods does a doctor use to diagnose CF?

Your doctor will conduct a physical examination and some tests, including blood tests, to determine if you have CF.  For new borns, a blood sample is checked for higher than normal levels of a chemical called immunoreactive trypsinogen (IRT), which is released by the pancreas. A sweat test may be also be done once the infant is at least 2 weeks old. Doctors may also recommend genetic tests for specific defects on the gene responsible for cystic fibrosis. In older people with suepcted signs of CF , genetic and sweat tests for CF will be done .

  1. Is cystic fibrosis life-threatening?

Despite being a progressive condition that needs daily care, people suffering from CF can lead a regular life, including – going to school or office. Advancement in medical science and screening techniques has changed this disease’s outlook a lot compared to the previous decades. It means a person with CF can now expect a longer life than before.

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Verified By Dr Battu Chaithanya

Consultant Interventional and Transplant Pulmonologist,

Apollo Hospitals, Jubilee Hills, Hyderabad

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