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:
- Imbalance of electrolytes
- Clubbed fingers
- Pancreatitis (inflammation of the pancreas)
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:
- Liver disease
- Intestinal obstruction
- Other complications include:
- Infertility in men and women
- Osteoporosis (thinning of the bone)
- Depression, fear, and anxiety
- Electrolyte imbalances
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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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Top Cystic Fibrosis Life Span Related Articles
acetylcysteineAcetylcysteine, a mucolytic agent, is used to treat pulmonary diseases like chronic emphysema, asthmatic bronchitis, bronchiectasis, and acute pneumonia. Acetylcysteine breaks up mucus and makes it easier to cough up and clear the lungs. Common side effects include bronchospasm, odor, drowsiness, fever, coughing up blood (hemoptysis), increased volume of bronchial secretions, irritation of tracheal or bronchial tract, nausea, and others. Do not use if you have acute asthma. Consult your doctor if you have a history of bronchospasm, or if you are pregnant or breastfeeding.
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.
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.
chloramphenicolChloramphenicol is a synthetic broad-spectrum antibiotic used to treat severe systemic infections, including bacterial meningitis, bacterial blood infection (bacteremia), typhoid fever, Rickettsial infections, and exacerbation of cystic fibrosis. Common side effects of chloramphenicol include bone marrow depression, blood disorders (including anemia), headache, confusion, delirium, depression, nausea, vomiting, diarrhea, and others. Avoid use in nursing mothers; discontinue either the drug or nursing. Consult your doctor if pregnant.
Common Medical Abbreviations & Terms
Doctors, pharmacists, and other health-care professionals use abbreviations, acronyms, and other terminology for instructions and information in regard to a patient's health condition, prescription drugs they are to take, or medical procedures that have been ordered. There is no approved this list of common medical abbreviations, acronyms, and terminology used by doctors and other health- care professionals. You can use this list of medical abbreviations and acronyms written by our doctors the next time you can't understand what is on your prescription package, blood test results, or medical procedure orders. Examples include:
- ANED: Alive no evidence of disease. The patient arrived in the ER alive with no evidence of disease.
- ARF: Acute renal (kidney) failure
- cap: Capsule.
- CPAP: Continuous positive airway pressure. A treatment for sleep apnea.
- DJD: Degenerative joint disease. Another term for osteoarthritis.
- DM: Diabetes mellitus. Type 1 and type 2 diabetes
- HA: Headache
- IBD: Inflammatory bowel disease. A name for two disorders of the gastrointestinal (BI) tract, Crohn's disease and ulcerative colitis
- JT: Joint
- N/V: Nausea or vomiting.
- p.o.: By mouth. From the Latin terminology per os.
- q.i.d.: Four times daily. As in taking a medicine four times daily.
- RA: Rheumatoid arthritis
- SOB: Shortness of breath.
- T: Temperature. Temperature is recorded as part of the physical examination. It is one of the "vital signs."
digestive enzymesDigestive enzymes are natural substances that help the digestive system break down food. Taken as a supplement, digestive enzymes may be used to treat adults and children with pancreatic insufficiency caused by cystic fibrosis, chronic pancreatitis (inflammation of the pancreas), pancreatectomy (removal of all or part of the pancreas), or pancreatic cancer. Common side effects of digestive enzymes include headache, neck pain, dizziness, abdominal cramping, nausea, diarrhea, gas (flatulence), indigestion, abnormal stools, and others. Do not take if you have acute pancreatitis.
Digestive Enzymes OralOral digestive enzymes are found in the digestive tract, are naturally produced by the body, and help breakdown the foods we eat. The pancreas makes and secretes digestive enzymes. Prescription and OTC digestive enzymes are available in PEPs or pancreatic enzyme products. PEPs are prescribed for people with cystic fibrosis, chronic pancreatitis, pancreatic cancer or tumors, Shwachman-Diamond syndrome, or removal of part or all of the pancreas. Side effects include life-threatening allergic reactions, difficulty breathing or talking, swelling of the mouth/face/lips/tongue/throat, chest tightness, rash or hives, itching, red, swollen, blistered, or peeling skin; wheezing.
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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.
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.
nitric oxide gasNitric oxide gas is used for treating hypoxic respiratory failure in newborns caused by impaired lung function, which leads to low oxygen levels in the tissues (hypoxia). It may also be used for cystic fibrosis, acute respiratory distress syndrome (ARDS), pulmonary arterial hypertension (PAH), sickle cell crises, diagnosis of sarcoidosis, and others. Common side effects of nitric oxide gas include low blood pressure (hypotension), withdrawal, complete or partial collapse of a lung’s lobe or an entire lung (atelectasis), blood in urine (hematuria), high blood glucose levels (hyperglycemia), whistling sound while breathing due to obstructed airway (stridor), infection, cellulitis, and sepsis.
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.