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Can you live a long life with cystic fibrosis?
Cystic fibrosis (CF) is a progressive disease that needs daily care. Though a patient needs to take medications lifelong, most people with cystic fibrosis are usually able to attend school and work. The improvement in screening and treatments mean that people with CF now may live into their mid- to late 30s or 40s, and some are living into their 50s.
A majority of the children with CF stay in good health and partake in most activities, along with attending the school.
Life expectancy has been improving steadily every year. However, it must be remembered that if you suffer from cystic fibrosis, you are prone to lung infections and have a risk of developing symptoms of damage for your other organs.
What is cystic fibrosis?
Cystic fibrosis is a chronic, progressive, and hereditary disorder that leads to the production of thick and sticky mucus in the body. This mucus, instead of being a lubricant, clogs the various organs of the body and causes problems. The mucus in the lungs can lead to infection whereas mucus in the pancreas causes difficulty in digesting food.
What are the symptoms of cystic fibrosis?
Symptoms of CF differ in individuals, depending on the severity of the disease.
Symptoms of cystic fibrosis affecting lungs in adults include:
- A persistent cough
- Lung infections
- Reduced exercise intolerance
- Repeated lung infections
- Inflamed nasal passages or a stuffy nose
- Sinus pain or pressure caused by infection
Symptoms of CF affecting the pancreas include:
- Foul-smelling, greasy stools
- Increased gas or bloating
- Nausea and loss of appetite
- Intestinal obstruction
- Chronic or severe constipation
Symptoms that occur later in life include:
What are the complications of cystic fibrosis?
Complications of cystic fibrosis can affect the respiratory, digestive, and reproductive systems, as well as other organs.
Respiratory system complications include:
- Pneumothorax: Rupture of lung tissue and trapping of air between the lung and chest wall
- Sinusitis: Inflammation of nasal sinuses
- Nasal polyps: Fleshy growth inside the nose
- Hemoptysis: Coughing blood
- Cor pulmonale: Enlargement of the right side of the heart
- Bronchiectasis: Abnormal widening and scarring of lungs
Digestive system complications include:
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What causes cystic fibrosis?
Cystic fibrosis is mainly caused due to a mutation in a gene, the cystic fibrosis transmembrane conductance regulator (CFTR) gene. This results in the production of thick and sticky mucus in the respiratory, digestive, and reproductive systems. The CFTR gene is present naturally in most of the individuals, but they do not exhibit any symptoms. A person with CF inherits two defective genes, one from each parent.
How is cystic fibrosis treated?
Daily treatments for CF include:
- Mucus-thinning drugs, such as hypertonic saline, which help you thin and cough up the mucus. This can improve lung function.
- Antibiotics to treat and prevent lung infections as and when needed.
- Anti-inflammatory medications to reduce airway swelling.
- Inhaled medications called bronchodilators for keeping airways open by relaxing the muscles around your bronchial tubes.
Individuals with digestive problems need to take pancreatic enzymes before meals to help them digest their food.
The FDA has approved these medications for treating CF in people with one or more mutations in the CFTR gene:
- The newest combination medication containing elexacaftor, ivacaftor, and tezacaftor (Trikafta) is approved for people aged 12 years and older. It is considered as a breakthrough.
- The combination medication containing tezacaftor and ivacaftor (Symdeko) is approved for children aged 6 years and older.
- The combination medication containing lumacaftor and ivacaftor (Orkambi) is approved for children aged 2 years and older.
- Ivacaftor (Kalydeco) has been approved for children aged 6 months and older.
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