Cystic Fibrosis
Cystic fibrosis or cystic fibrosis is a genetic disease that causes mucus to thicken and stick to the body, blocking various channels, including the respiratory and digestive tracts.
Under normal circumstances, the mucus in the body is liquid, slippery and acts as a lubricant. While in patients with cystic fibrosis, there are abnormalities in genes that make mucus sticky and inhibit a number of channels, including those located in the lungs and pancreas. This condition causes respiratory and digestive disorders in people at an early age.
Cystic fibrosis can be diagnosed with a blood test in newborns. In the case of a diagnosis of cystic fibrosis, treatment usually consists of a combination of drugs and treatments. Operation options can also be made if the conditions are severe. The form of treatment will depend on the health problems and symptoms experienced by the patient.
Symptoms of cystic fibrosis
The symptoms of cystic fibrosis can vary depending on the severity of the disease. Symptoms can appear after birth or only when someone is an adult.
Blockage of the airways can occur in people with cystic fibrosis. This will cause symptoms like
In addition to the above symptoms, lung infections are also sensitive to people with cystic fibrosis because mucus is a breeding ground for bacterial reproduction.
The same condition can also occur in the digestive system, where the pancreatic duct can be blocked by sticky mucus. As a result, digestive enzymes produced by the pancreas cannot reach the small intestine, which facilitates digestion of food. This condition often causes symptoms in the form of:
In addition to the symptoms of the respiratory and digestive system, people with cystic fibrosis also tend to:
Cystic fibrosis is a hereditary disease or disorder that affects a person from both parents due to genetic mutations. Half of children whose parents have a genetic disorder are carriers, which can reduce this disorder in children. While a quarter becomes cystic fibrosis.
Genetic diseases modify proteins that regulate the entry and exit of salt into cells, thus forming sticky mucus in various body channels.
Diagnosis of cystic fibrosis
The diagnosis to determine the presence of cystic fibrosis or cystic fibrosis can be made from a newborn baby. The goal is that treatment can be done as soon as possible.
DNA testing is a type of routine examination. In this method, damage to the genes responsible for cystic fibrosis can be detected. Before conducting a DNA test, the doctor will usually recommend a blood test for signs of cystic fibrosis at the age of 8 days. In addition, if deemed necessary, genetic or DNA testing can be carried out. This is done by taking DNA samples from baby's saliva or blood.
Another type of test is the sweat sample test. The purpose of this review is to detect the presence of cystic fibrosis by measuring salt levels in sweat. In general, the salinity of cystic fibrosis sufferers is greater than normal size. Examination of sweat samples can usually be done when the baby is at least 2 weeks old.
The next test to determine the diagnosis of cystic fibrosis is the Nose Potential Difference Test. During this examination, electrodes are placed on the nose to observe the fluidity of salt in the nasal passages.
In addition, examination of organ function makes it possible to measure the level of health of the liver and pancreas. This method can also be used to detect symptoms of diabetes and is usually done periodically after the patient is ten years old.
If the results of the examination can be determined in children with cystic fibrosis, further tests are carried out to determine the condition of the disease. Here are some possible checks:
This test is also recommended for children or adults who do not have blood tests at birth, especially those who have recurrent infections, infertility (in men), pancreatitis or inflammation of the pancreas and polyps. nose.
Treatment of cystic fibrosis
Until now, cystic fibrosis could not be cured. Treatment is limited to relieving symptoms, preventing complications and infections, and helping patients carry out daily activities. Treatment can:
Drug delivery
Antibiotics are the most commonly used drugs to fight lung infections. The forms of antibiotic use vary, some are in the form of capsules, pills or syrups and others are used in infusions or injections. Tobramycin and ciprofloxacin are examples of antibiotic drugs for cystic fibrosis.
In addition to antibiotics, other drugs can also be used to reduce inflammation in the lungs, such as prednisone or fluticasone, which are included in the cortocosteroid group, with chromoline in the membrane stabilizer and ibuprofen groups, for the nonsteroidal anti-inflammatory drug group (NSAID).
Medications that control the volume of mucus and the secretion of mucus in the lungs can also be prescribed for people with cystic fibrosis. Examples of this type of drug are hypertonic solutions to secrete mucus in the lungs, deoxyribonuclease (DNase), which functions to thin the mucus in the lungs, mucolytic (for example, acetylcysteine) to reduce lung viscosity. mucus in the intestine, and ivacaftor to reduce mucus levels in the body.
To expand the airways and relieve breathing, bronchodilators (such as albuterol and salmeterol) or anticholinergic drugs (such as ipratropium bromide) can be used. Both of these drugs also function to facilitate coughing out mucus. Meanwhile, to help absorb nutrients in food, pancreaticipase can be given as a substitute for pancreatic enzymes.
Other therapy
In addition to drug administration, symptoms of cystic fibrosis can also be treated with physiotherapy, including:
Mode of operation
Doctors recommend that doctors treat cystic fibrosis with surgical procedures if symptoms worsen and can no longer be treated with drugs or other methods. In cases of severe cystic fibrosis with a lung that cannot function anymore, lung transplantation is perhaps the most effective way to prolong a patient's life, even if this operation is considered to be very risky. Surgery is also needed if cystic fibrosis causes a blockage in the large intestine, to eliminate the blockage.
Complications of cystic fibrosis
Some of the complications that can occur due to cystic fibrosis are:
Under normal circumstances, the mucus in the body is liquid, slippery and acts as a lubricant. While in patients with cystic fibrosis, there are abnormalities in genes that make mucus sticky and inhibit a number of channels, including those located in the lungs and pancreas. This condition causes respiratory and digestive disorders in people at an early age.
Symptoms of cystic fibrosis
The symptoms of cystic fibrosis can vary depending on the severity of the disease. Symptoms can appear after birth or only when someone is an adult.
Blockage of the airways can occur in people with cystic fibrosis. This will cause symptoms like
- Prolonged cough
- Hard to breathe.
- Diarrhea
- vomit
- Shortness of breath or difficulty breathing
- Sighs
- Airways widen due to inflammation (bronchiectasis).
In addition to the above symptoms, lung infections are also sensitive to people with cystic fibrosis because mucus is a breeding ground for bacterial reproduction.
The same condition can also occur in the digestive system, where the pancreatic duct can be blocked by sticky mucus. As a result, digestive enzymes produced by the pancreas cannot reach the small intestine, which facilitates digestion of food. This condition often causes symptoms in the form of:
- Weight loss or even stunting due to undigested food can cause malnutrition or malnutrition.
- The stool texture is thick, oily and gives off a strong odor.
- Severe constipation.
- Disruption of the initial excretion process (meconium) on the first or second day after birth due to a blockage. This condition is called meconium ileum.
- Baby's skin color turns yellow (jaundice).
In addition to the symptoms of the respiratory and digestive system, people with cystic fibrosis also tend to:
- Nasal infections, such as nasal polyps and sinusitis.
- Pancreatic diabetes cannot produce enough insulin.
- Infertility in men is caused by obstruction of the channel that carries sperm and infertility in women due to disruption of the menstrual cycle and the thickness of the fluid in the lining of the uterus.
- Weakened and lean bone condition (osteoporosis).
- Urinary incontinence due to loss of automatic bladder control, allows urine to flow at any time.
- Liver disorders.
Cystic fibrosis is a hereditary disease or disorder that affects a person from both parents due to genetic mutations. Half of children whose parents have a genetic disorder are carriers, which can reduce this disorder in children. While a quarter becomes cystic fibrosis.
Genetic diseases modify proteins that regulate the entry and exit of salt into cells, thus forming sticky mucus in various body channels.
Diagnosis of cystic fibrosis
The diagnosis to determine the presence of cystic fibrosis or cystic fibrosis can be made from a newborn baby. The goal is that treatment can be done as soon as possible.
DNA testing is a type of routine examination. In this method, damage to the genes responsible for cystic fibrosis can be detected. Before conducting a DNA test, the doctor will usually recommend a blood test for signs of cystic fibrosis at the age of 8 days. In addition, if deemed necessary, genetic or DNA testing can be carried out. This is done by taking DNA samples from baby's saliva or blood.
Another type of test is the sweat sample test. The purpose of this review is to detect the presence of cystic fibrosis by measuring salt levels in sweat. In general, the salinity of cystic fibrosis sufferers is greater than normal size. Examination of sweat samples can usually be done when the baby is at least 2 weeks old.
The next test to determine the diagnosis of cystic fibrosis is the Nose Potential Difference Test. During this examination, electrodes are placed on the nose to observe the fluidity of salt in the nasal passages.
In addition, examination of organ function makes it possible to measure the level of health of the liver and pancreas. This method can also be used to detect symptoms of diabetes and is usually done periodically after the patient is ten years old.
If the results of the examination can be determined in children with cystic fibrosis, further tests are carried out to determine the condition of the disease. Here are some possible checks:
- X-ray scanning to get images of the chest, including the heart and lungs.
- Computed tomography can detect serious disorders of the pancreas, lungs or other organs.
- Liver function tests to detect complications of cystic fibrosis.
- Examination of the throat or sputum to determine the bacteria that causes the infection.
- Examination of lung function and analysis of oxygen and carbon dioxide levels in the blood to assess lung performance.
- Stool analysis determines the body's ability to digest and absorb nutrients in food.
This test is also recommended for children or adults who do not have blood tests at birth, especially those who have recurrent infections, infertility (in men), pancreatitis or inflammation of the pancreas and polyps. nose.
Treatment of cystic fibrosis
Until now, cystic fibrosis could not be cured. Treatment is limited to relieving symptoms, preventing complications and infections, and helping patients carry out daily activities. Treatment can:
Drug delivery
Antibiotics are the most commonly used drugs to fight lung infections. The forms of antibiotic use vary, some are in the form of capsules, pills or syrups and others are used in infusions or injections. Tobramycin and ciprofloxacin are examples of antibiotic drugs for cystic fibrosis.
In addition to antibiotics, other drugs can also be used to reduce inflammation in the lungs, such as prednisone or fluticasone, which are included in the cortocosteroid group, with chromoline in the membrane stabilizer and ibuprofen groups, for the nonsteroidal anti-inflammatory drug group (NSAID).
Medications that control the volume of mucus and the secretion of mucus in the lungs can also be prescribed for people with cystic fibrosis. Examples of this type of drug are hypertonic solutions to secrete mucus in the lungs, deoxyribonuclease (DNase), which functions to thin the mucus in the lungs, mucolytic (for example, acetylcysteine) to reduce lung viscosity. mucus in the intestine, and ivacaftor to reduce mucus levels in the body.
To expand the airways and relieve breathing, bronchodilators (such as albuterol and salmeterol) or anticholinergic drugs (such as ipratropium bromide) can be used. Both of these drugs also function to facilitate coughing out mucus. Meanwhile, to help absorb nutrients in food, pancreaticipase can be given as a substitute for pancreatic enzymes.
Other therapy
In addition to drug administration, symptoms of cystic fibrosis can also be treated with physiotherapy, including:
- Therapy for removing thick mucus from the body by patting the chest or back, using breathing techniques or special tools.
- Pure oxygen therapy to overcome the decrease in oxygen levels in the blood and prevent pulmonary arterial hypertension.
- Sports and sports to improve fitness.
- Modified postural drainage, so that mucus is easily removed from the lungs by changing body position.
Mode of operation
Doctors recommend that doctors treat cystic fibrosis with surgical procedures if symptoms worsen and can no longer be treated with drugs or other methods. In cases of severe cystic fibrosis with a lung that cannot function anymore, lung transplantation is perhaps the most effective way to prolong a patient's life, even if this operation is considered to be very risky. Surgery is also needed if cystic fibrosis causes a blockage in the large intestine, to eliminate the blockage.
Complications of cystic fibrosis
Some of the complications that can occur due to cystic fibrosis are:
- Chronic infections, such as bronchitis, pneumonia and sinusitis.
- Pneumothorax, which is the accumulation of air in the pleural cavity (the cavity that separates the lungs and chest wall).
- Bronchiectasis, which is a lesion in the airways that makes it more difficult to expel phlegm.
- Acute exacerbation, symptoms that worsen suddenly which is marked by shortness of breath or cough for several days or weeks, requires hospitalization.
- Hemoptysis or blood spitting due to thinning of the walls of the airways.
- Respiratory insufficiency due to swelling of the lungs.
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