- Life Span
- What Is It?
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:
Latest Health and Living News
Daily Health News
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.
Health Solutions From Our Sponsors
Top Cystic Fibrosis Life Span Related Articles
What Is Bronchiectasis?Bronchiectasis has three types: cylindrical bronchiectasis, saccular or varicose bronchiectasis, and cystic bronchiectasis. Causes of bronchiectasis include infection, environmental exposure, drug or alcohol abuse, and alpha-1 antitrypsin (congenital). Symptoms of bronchiectasis include shortness of breath, fatigue, chronic cough, bloody sputum, and wheezing. Treatment for bronchiectasis includes antibiotics and possibly surgery.
Bronchitol (mannitol)Bronchitol is a prescription medicine that is used along with other therapies to improve lung function in people 18 years of age and older with cystic fibrosis (CF) who have passed the Bronchitol Tolerance Test (BTT). Serious side effects of Bronchitol include sudden breathing problems and coughing up of blood (hemoptysis).
Can You Get Cystic Fibrosis at Any Age?Cystic fibrosis is classically a disease of childhood that progresses into adulthood. If you have a genetic defect related to cystic fibrosis (CF), you are more likely to be born with cystic fibrosis. The symptoms might appear later, and hence, the age at diagnosis varies widely.
Can You Live a Long Life With Cystic Fibrosis?How long can you live with cystic fibrosis? Learn the signs of cystic fibrosis and what to do if someone you know has cystic fibrosis.
Cystic FibrosisCystic fibrosis is a disease of the mucus and sweat glands. Cystic fibrosis is an inherited disease. The outcome of the disease leaves the body malnourished, with bulky and fouls smelling stools, vitamin insufficiency, gas, painful or swollen abdomen, infertility, susceptible to heat emergencies, and respiratory failure. There is no cure for cystic fibrosis, treatment of symptoms is used to manage the disease.
How Is Meconium Ileus Treated?Meconium ileus, a serious condition in newborn babies in which the intestines are blocked by the infant’s first stool, often needs surgical intervention and usually indicates the child will struggle with the congenital disease cystic fibrosis throughout their lives.
How Long Can You Live With Cystic Fibrosis?The life expectancy of patients with cystic fibrosis has improved over the last 50 years. The improved chances of survival are due to advances in early diagnosis, supportive care, nutritional care, and infection control.
What Is Idiopathic Pulmonary Fibrosis (IPF)?Idiopathic pulmonary fibrosis or IPF is a progressive lung disease. There is no known cause of IPF. Symptoms include shortness of breath, cough, fatigue, mild fevers, muscle pain, clubbing fingers, and ankle swelling. Treatment of idiopathic pulmonary fibrosis is directed at managing symptoms. The life expectancy is about 2 to 5 years.
Kalydeco (ivacaftor)Kalydeco (ivacaftor) is a prescription medicine used for the treatment of cystic fibrosis (CF) in patients age 6 months and older who have at least one mutation in their CF gene that is responsive to Kalydeco. Kalydeco can cause serious side effects, including high liver enzymes in the blood.
What Is the Life Expectancy of Someone Born With Cystic Fibrosis?Cystic fibrosis (CF) is a genetic disease that causes progressive damage to the organs, especially the lungs and digestive system. People with cystic fibrosis often complete school, including college, have fulfilling jobs, find love, and have children.
Why Is Meconium Ileus Diagnostic for Cystic Fibrosis?Meconium ileus is an obstruction in the small intestine caused by thick, congealed meconium, the first stool passed by a newborn baby. Cystic fibrosis, a genetic disorder, is a common cause of this problem.
What Are the Benefits and Risks of Assisted Ventilation of the Newborn?To provide a baby assisted ventilation, a mechanical ventilator pumps oxygen to the lungs at pressure until the baby’s respiratory system works normally. This can help kickstart the baby’s breathing reflex if it’s compromised by underdevelopment or some congenital condition, but it may also lead to lung trauma.
Skin Problems: A Visual Guide to CystsThese small sacs filled with fluid, tissue, hair, or dead skin can form almost anywhere on your body. Find out more about why cysts form and what you can do about them.
Symdeko (tezacaftor/ivacaftor)Symdeko (tezacaftor/ivacaftor) is a prescription medicine used for the treatment of cystic fibrosis (CF) in patients age 6 years and older who have two copies of the F508del mutation, or who have at least one mutation in the CF gene that is responsive to treatment with Symdeko. Serious side effects of Symdeko include high liver enzymes in the blood, and abnormality of the eye lens (cataract) in some children and adolescents.
Trikafta (elexacaftor/tezacaftor/ivacaftor)Trikafta is a prescription medicine used for the treatment of cystic fibrosis (CF) in people aged 12 years and older who have at least one copy of the F508del mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Trikafta can cause serious side effects, including high liver enzymes in the blood.