Tag Archive | "cystic fibrosis patient"

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Self-Care for Cystic Fibrosis Patients


If you have a child diagnosed with cystic fibrosis one of the most important steps you can take is to learn all you can about the disease. The good news is with the Internet you are able to find multiple sites to give you the information you need. You can track recent developments in treating the disease and be instantly notified of any breaking news. Educate yourself about nutrition, medication, treatment and how to recognize infections early.

Having a child with cystic fibrosis is stressful, especially if you are the primary caregiver and need to give your child daily chest percussion. The child should have the treatment at least twice a day and for a thirty minute period of time. Learning how to perform chest percussion could potentially save your childs life. A doctor or respiratory therapist will help you learn the procedure.

Keeping your child healthy and caring for them includes keeping their immunizations up-to-date. Normal vaccines should be given on their appropriate schedule and you should consider having your child vaccinated for flu and pneumonia. Cystic fibrosis does not affect the immune system but when a child gets sick they may have more complications than a healthy child.

Your child should be encouraged to lead a normal life. They can take part in sports events and regular physical activity. Exercise will help loosen the mucus in the lungs and airways and improve your heart and lung functions. A child with cystic fibrosis may gain self-confidence and have a better self-esteem if they can take part in a team sport. If a cystic fibrosis patient starts exercising when they are young they are more apt to exercise when older. Simple exercise such as walking, riding a bike or swimming can help. Anything that gets you moving will help loosen the thick mucus build up.

Eating a healthy diet is important for all cystic fibrosis patients. Increasing your caloric intake, taking vitamin supplements that are fat-soluble, and taking pancreatic enzymes can help you stay healthier. Drinking plenty of liquids is another self-care tip you should know. The liquid will help thin the mucus and special care should be taken in the summer when the body lose fluids easily.

If you are a smoker, stop smoking! If you are unable to do that, dont smoke in your car, your home, and do not allow your child to be with people who smoke. Second hand smoke is bad for everyone but especially for those who have cystic fibrosis. Avoid places that will be smoky as much as possible.

Encourage good hygiene habits for everyone in your family. Teaching your family to wash their hands before eating, after they use the bathroom, or if they have been in a public place. Encouraging hand washing is the best way to avoid infections that may turn into severe complications for a cystic fibrosis patient. Especially encourage hand washing when a child comes home from school, or an adult comes home from work.

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Problems Associated With Cystic Fibrosis in Adults


Most patients with cystic fibrosis are diagnosed when they are infants or toddlers. Some adults may be diagnosed with the disease but this is normally rare. Modern medicine has found ways of giving a cystic fibrosis patient a better standard of living and a longer life span. With this longer life span come more medical problems caused by cystic fibrosis. Adults who recently have been diagnosed with cystic fibrosis may want to ask relevant questions of their doctor. Education is the key to controlling and treating any disease, especially cystic fibrosis. You should choose a medical team that specializes in treating the disease.

Drugs that are prescribed or found over-the-counter may be used to control the symptoms and allow the patient to have a better quality of life. Chest physiotherapy is recommended either manually or by a mechanical device that will loosen the mucus and help the body expel it. Some manual methods are controlled breathing exercises, and lying down while the chest and back area is manipulated and thumped to loosen the thick mucus and move it out of the air passages. This chest therapy should be done every day to avoid a massive build up of the thick mucus.

Cystic fibrosis patients may find they have digestive problems even though they are eating a healthy diet. They may be eating a balanced, high-caloric menu filled with all the essential vitamins and minerals your body needs and still having digestive problems. A doctor and nutrient specialist can help relieve excess gas; bloating, and accompanying pain felt when the body is having digestive problems. A patient may be asked to take supplements of Vitamins A, D, E, and K and a pancreatic enzyme to promote a healthier digestive tract.

An adult patient suffering from cystic fibrosis may develop liver and gallbladder problems. Diseases in the gallbladder and liver may be treated in various ways. A patient may have gallstones removed in a conventional surgery using small incisions to remove the gallbladder and stones. Some doctors today are using laparoscopic surgery and removing the diseased gallbladder through one small incision in the belly button area.

Using a therapy to dissolve formations found in the liver can treat liver disease. Oral dissolution means taking an oral medication, normally a drug with ursodeoxycholic acid will dissolve any formations in the liver.

Infertility is a problem that affects men with cystic fibrosis. Almost 90 percent of men who have CF are infertile. Artificial insemination with donated sperm or adoption is the only options an infertile man has to father a child. A woman may become pregnant in the regular way but pregnancy may leave the woman with severe lung damage. This could be life threatening. If a cystic fibrosis patient cannot find any relief from these severe complications and are facing reduced lung efficiency and death a doctor may recommend a lung transplant. Fifty-five percent of patients who receive a lung transplant have a life expectancy of about three more years.

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Preventing Infections in Cystic Fibrosis Patients


There is no known cure for cystic fibrosis so the main purpose of treatment is to reduce the severity of the symptoms and give the patient the best possible quality of life. New antibiotics developed recently are more effective in fighting infections and killing the bacteria that causes them. Faster acting inhalers deliver the medications straight to the airways. Long-term users of antibiotics have the potential of developing a resistance to drug therapy.

The role of white blood cells in the body is to attack bacteria. When the DNA in the cells is released it makes the mucus thicker. There is a new aerosol drug that can be prescribed to breaks up the DNA into pieces and makes the mucus thinner. The new drug is called dornase alfa and it does have side effects. The drug can cause sore throat and increased irritation in the airways.

Medications including albuterol may help keep the air passages and bronchial tubes clearer. Cystic fibrosis patients must have a way to clear the mucus from their lungs and air passages. There are several methods used to clear mucus. Manually clapping the back and chest with a cupped hand will loosen the mucus. This is best done while the patients head is hanging over the edge of the bed. Gravity will help clear the thick mucus from the pulmonary area.

A mechanical device called a chest clapper is now available to cystic fibrosis patients. Also on the market is an inflatable vest that uses a high frequency vibration to loosen the mucus and allow the patient to cough it up. Patients with cystic fibrosis both adults and children need to have this treatment twice a day. Older patients may be able to clear their bronchial areas with these electrical devices but young children should have help from an adult.

A healthy diet can help a patient with cystic fibrosis combat infection. If the disease is affecting the digestive tract a patient can become malnourished because cystic fibrosis affects the pancreas and the enzymes needed to digest your food do not get to the stomach. A higher caloric intake will help with some deficiencies but a patient with this disease should take extra vitamins and enzymes to maintain weight. They may also consider nutritional drinks to supplement their diet.

The last resort for a cystic fibrosis patient is a lung transplant. Severe breathing problems may prompt your doctor to recommend this treatment. You should realize a lung transplant is not a cure for the disease. Your doctor will help you decide if you are a good candidate for a lung transplant. Some factors to consider will be your over-all health, your lifestyle and the availability of donors in your area. A lung transplant will mean replacing both sides of the lung because cystic fibrosis affects both lungs. Having a lung transplant is a major surgery and there are serious complications to consider before deciding to have the operation. There is a risk of postsurgical infections that could be risky.

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New Advances in Treating Cystic Fibrosis


Excitement is building among scientists and researchers who are looking for a better way of treating cystic fibrosis. There is no known cure for this inherited disease so the goal of treatment is to prevent suffering and prolong life. Improving the quality of life for a cystic fibrosis patient is one of the top goals for any doctor treating a CF patient.

In healthy individuals, a natural substance called antiproteases protects the lungs from the harmful effects of proteases we have in our body. In cystic fibrosis patients do not make enough of these antiprotease substances to protect their lungs. One specific area of research is in developing new antiprotease inhalers, which should help inflammation in the lungs and reduce damage done by natural proteases found in the body of a patient with CF.

The National Institute of Health has recently announced a new class of drugs that will be used to thin the mucus secretions in the cystic fibrosis patient. It is strange but true; the drug is being drawn from toxins. The same toxins that cause the red tides in Florida that kill fish and other plant life are being tested to use as a mucus-thinning drug. Red tide toxin contaminates shellfish, kill other fish, and create severe respiratory problems in humans who eat it. While trying to find a way to combat this contamination may have accidentally found a way to thin the mucus of a cystic fibrosis patient. Researchers have discovered by this new drug helps thin the thick mucus of the patient. Although scientists are not exactly sure why the new drug works they surmise it could be because it increases the movement of the cilia in the lungs. Cilia are the thin, hairlike projections that help move mucus across the surface of the lungs cells. The increased movement also helps keep the thick secretions from sticking to the lungs and air passages. More studies will need to be completed before scientists and researchers and discover the safety of this new medication. They do not know what the effect will be on cystic fibrosis patients because symptoms vary in severity from patient to patient.

More advances in gene technology have also caused much excitement in healthcare providers who specialize in diagnosing and treating cystic fibrosis. They are working on a way to insert healthy genes into the airways of CF patients that should replace the mutant genes that cause cystic fibrosis. Gene therapy may play a huge role in the future in treating this incurable disease.

Treatment for cystic fibrosis patients mainly consists of making the patient as comfortable and pain-free as possible. Since it is incurable, doctors and healthcare professionals try to give their patients a better quality of life and longer span. Since the mutant gene was discovered in 1988, the life span for cystic fibrosis has steadily risen. In ancient times, babies born with cystic fibrosis died in early infancy. In the middle 80s the lifespan was 16 years old. Today the average lifespan for someone who has cystic fibrosis is between 36 and 39 years old. There are still advances to be made, treatments to be discovered, and a cure may be found in the future.

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Medical Problems Caused by Cystic Fibrosis


Cystic fibrosis symptoms are caused by heavy, thick, mucus. They include frequent coughing that brings up thick discolored mucus and frequent bouts of chest congestion and frequent pneumonia. The skin may taste salty to when the child is kissed and dehydration. The stools of a cystic fibrosis patient are thick, greasy, and smell foul. The patient may be eating well and have a good appetite but fails to gain weight and thrive. Stomach pain, bloating and excessive gas is caused by too much gas in the intestines and is painful.

There are other medical problems caused by cystic fibrosis. Infertility in men is often caused by cystic fibrosis. Sinusitis is caused by the space behind your eyes, nose, and forehead being blocked by thick mucus. They become infected and cause sinusitis when the lining of the sinuses becomes blocked. Sinusitis is common in patients with cystic fibrosis. Nasal polyps may develop in the sinuses and require surgery to remove.

Cystic fibrosis affects the lungs and bronchial tubes. These are the large airways in your lungs. Bronchiectasis is a lung disease that causes these airways to become stretched and flabby. They produce pockets where mucus collects. These pockets are a breeding ground for bacteria and infection causes more damage to the bronchial tubes. This can lead to bronchial infections and serious illness including respiratory failure.

Your pancreas is another part of the body that cystic fibrosis affects. Severe inflammation of the pancreas causes extreme pain. Intestinal blockage is another problem a CF patient may face, especially a newborn baby.

If your lungs are not moving enough oxygen through the body clubbing can be a result. Clubbing is the widening and thickening of the toes and fingers. Clubbing is a definitive sign of cystic fibrosis.

Other serious illnesses and complications include liver disease, diabetes, gallstones, collapsed lungs, and rectal prolapse. Coughing and the body having problems passing stools cause rectal prolapse. This pushes the rectal tissue outside of the body.

Another serious illness you may have because of cystic fibrosis is low bone density. Your body does not get enough Vitamin D and can cause rickets. Your doctor may recommend Vitamin D supplements to prevent low bone density.

If you see any signs or symptoms of cystic fibrosis you should contact your doctor immediately. Frequent cough, repeated bouts of pneumonia and bronchitis, dehydration, diarrhea, stomach pain and excessive gas are symptoms of cystic fibrosis. If your child is eating normally but fails to strive, they should be checked for a diagnosis of cystic fibrosis. Kiss your child somewhere on his or her skin. If the kiss tastes salty that is another sign your child should see a medical professional.

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Lung TransplantsAre They An Option?


Patients with cystic fibrosis that have the lungs involved are the most vulnerable to infections and risk of death. The lungs are essential to life and when their capacity to breath is severely affected quality of life can be affected. Treatments for patients with cystic fibrosis in their lungs and air passages should have daily physiotherapy and postural chest therapy. Tapping on the back and chest manually is one form of treatment. It is performed using a cupped hand and is called chest percussion. Other methods are using a specially made vest that vibrates to loosen the mucus so it can be coughed up. Other breathing devices have been invented to exert pressure on the chest and lungs to loosen the mucus. These are called oscillatory positive expiratory pressure devices. By breathing out into one of these devices, vibrations are created that loosen and help remove the thick secretion build up.

Normally patients with cystic fibrosis affecting the lungs and air passageways should have thirty-minute treatments twice a day. As an adult, you may be able to perform the physiotherapy on yourself. A child will need the help of a parent, adult, guardian, or older sibling. This therapy is necessary but many times a teen will rebel and refuse to take part in treating their disease. If that should occur, you should contact your doctor for a referral to a psychologist or counselor. It is necessary for treatments to continue especially the chest physiotherapy and nutritional supplements.

In severe cases of cystic fibrosis with lung involvement a patient may need oxygen on a continuous basis. In the past a patient may have been restricted to his or her home because of the bulkiness of the oxygen tank. Today there are many more improved methods of receiving oxygen. A liquid oxygen machine run by electricity gives a constant supply of oxygen through a tube that has the capacity to run throughout the house. Oxygen concentrators are available that are small enough to be carried around and provide oxygen while the patient runs errands and attends social events. This allows the cystic fibrosis patient to have a better quality of life and aid in their breathing.

A lung transplant may be an option a cystic fibrosis patient may consider. Although a lung transplant will not cure the disease, it will allow them to have better breathing capacity. It will not stop the mucus from forming in the transplanted lungs. Only the most severe cases of cystic fibrosis will be considered for a lung transplant. They will be put on a waiting list and need to be considered as good candidates for a lung transplant. As with any major surgery, there are risks and complications involved. Patients that have cancer, AIDS, severe damage to other organs, overweight, or if they are diagnosed with a major psychiatric illness are not good candidates for lung transplants. This method of treatment should be considered a last resort and again, it will not cure cystic fibrosis.

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Lung Infections in Patients With CF


Lung infections are especially hard on patients with cystic fibrosis. Unfortunately, one of the symptoms of the disease is frequent respiratory, lung, and bronchial infections. When a patient has cystic fibrosis it is harder to treat the infection because the bacteria that causes them stick to the thick mucus found in the lungs and air passageways. While they are there they have a good breeding ground to multiply and grow. Mucus also prevents antibiotics from reaching the source of the infection, which makes it even harder to treat.

People with normal thin mucus can easily expel any mucus or sputum that builds up when they get cold or bacterial infections. A patient with cystic fibrosis has thick, sticky, heavy mucus that sticks to the walls of the passageways to their lungs and their gastrointestinal system. There are drugs available to thin mucus by breaking apart the genetic material found in the mucus. By breaking up the material it reduces the thickness of their mucus and helps them to cough it up and expel it easier.

Another tool for treating lung infections for the cystic fibrosis patient is an antibiotic. Lung infections are difficult to treat because the mucus keeps the antibiotics from reaching the area of the infection. Fortunately, there are now new methods of getting antibiotics into the body. They can be taken orally or even inhaled, which will allow the antibiotics to reach the infection easier and faster.

Antibiotics used to treat symptoms of cystic fibrosis are effective but there is a danger the body will build up immunity to the drugs. New strains or variations may also develop that regular antibiotics will not help. There are over 100 strains of CF and some easily become resistant to antibiotics.

Recent research has isolated the protein that is linked to the inability of the lung to rid itself of the mucus. New medications and treatments are waiting approval from the Federal Drug Administration that will be used to help thin mucus and keep it from clogging essential passages through the body. A new clinical study will soon be started that will include cystic fibrosis patients and healthy patients. They will be testing a new drug that may restore the role of the defective CFTR protein. This is the protein that is responsible for spreading water and salt through the body.

Enzymes are important for a cystic fibrosis patient. Enzymes normally produced by the pancreas gland have to pass through small ducts into the small intestine when food is present to aid in digestion. Those patients with cystic fibrosis that is concentrated in the digestive tract will have these small ducts blocked by the thick mucus. This keeps the proper enzymes from entering the small intestine and digesting the food that is there. Often CF patients are malnourished and have a slower growth rate than those who are healthy.

Increased movement and activity also help keep the mucus thinner and easier to expel from the body. Much excitement has been spurred in health care circles because of a new technique, which inserts healthy genes into the airways of the cystic fibrosis patient. These are meant to replace the mutant gene that carries the disease.

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Introduction to Cystic Fibrosis


Cystic fibrosis was a mystery disease until the mid 1940s. Then it was identified with the inability to clear mucus from the lungs and air passages. Some scientists and researchers believe it developed because of preventive measures taken for cholera. If a child was diagnosed with cystic fibrosis in the 40s they rarely survived childhood. In the 1970s the survival rate of a child with cystic fibrosis was sixteen! Treatment was supportive in nature and did little more than improve their quality of life. Today there is still no known cure for cystic fibrosis but there are treatments and medications available that prolong life and give the patient a better quality of life. In 2006 the survival age of cystic fibrosis patients rose to the late 30s.

In 1989 scientists discovered the cystic fibrosis gene was a mutant gene that is passed on from parent to child. Children cannot inherit cystic fibrosis if only one parent carries the mutant gene! It takes both parents to have the gene for a child to develop cystic fibrosis. For parents who each carry the mutant gene there is a 25 percent chance for having a child with cystic fibrosis and 25 percent the child will not inherit the gene. The remaining 50 percent could be a carrier but not show any symptoms of the disease.

Cystic fibrosis affects many parts of the body including the respiratory organs, reproductive organs, and the digestive system. The normal mucus that lines the passageways of bronchial tubes, lungs, and digestive organs is not think and watery. Instead, it is thick and sticky and adheres to the passageways that lead to the respiratory, digestive, and reproductive organs. Those born with the defective gene makes this heavy mucus amasses and cause problems. If the mucus builds up in the respiratory passages and lungs, the patient will find it harder to breathe. If it builds up in the digestive tract it will not allow essential nutrients to go through the digestive system. A man who is diagnosed with cystic fibrosis is normally sterile.

This disease is detected in children before the age of three but there are occasions when a preteen is found to have cystic fibrosis. One of the symptoms of the disease is a delay in the onset of puberty. There are five common tests for cystic fibrosis. One of the earliest signs an infant or child may have cystic fibrosis is a salty taste to their skin when you kiss them. A high salt level often signals the presence of the cystic fibrosis in the body. Confirmation of a cystic fibrosis diagnosis may be done in four different ways. A trypsin test evaluates the function of the pancreatic gland by detecting amounts of trypsin in the stools of the patient. A patient may go through a pulmonary function test to look at their breathing ability and lung function. Chest x-rays may be used to discover any mucus deposits that have collected in the lungs. Finally, genetic tests can detect the mutant gene that causes cystic fibrosis. These tests can be done before, during, or after a pregnancy. If you know there is cystic fibrosis in your family, you may want to have genetic testing to find out if you and your spouse are carriers of the mutant gene.

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General Symptoms of Cystic Fibrosis Throughout The Body


People often think of cystic fibrosis as only affecting the respiratory organs of the body. This the place cystic fibrosis patients experience the most severe symptoms of cystic fibrosis. The common signs of cystic fibrosis in the pulmonary and respiratory system are commonly known. Coughing, wheezing, frequent bouts of bronchitis and pneumonia, sinusitis and fleshy growths in the nose. Thick, sticky mucus that is discolored when it is brought up is a sure sign of cystic fibrosis.

Cystic fibrosis often will show up in other parts of the body and will not be as easily recognized. This disease will often show up in the digestive organs. A common sign of digestive problems is stomach cramping, pain, and excess gas. Other common signs of a possible diagnosis of cystic fibrosis are diarrhea, stools that look greasy and smell foul. In worse cases a patient may experience a bowel blockage. A patient with cystic fibrosis may have severe vomiting.

The reproduction organs may also be affected. If a child has a delayed onset of puberty and have other signs of the disease this could be a sign of cystic fibrosis. Most males with cystic fibrosis will be sterile. Women with this disease may have difficulty becoming pregnant and be aware of the dangers of passing on the mutant gene that causes cystic fibrosis.

Another part of the body that can be affected by cystic fibrosis is the blood system. Anemia may occur. Anemia is a decrease in the number or make-up of the blood cells. Bleeding disorders such as inability to clot are also associated with cystic fibrosis.

Bone and joint problems is a little known sign of cystic fibrosis. We normally think of respiratory and digestive problems when a person is diagnosed with this disease. It might be surprising to learn that CF may affect arthritis, stunt the growth of a cystic fibrosis patient, severe pain in the joints and bones, and cases of osteoporosis may be attributed to this disease.

Another unusual sign of cystic fibrosis is an enlargement of the fingertips and toes. The digits swell, this symptom is called clubbing. This symptom is not rare but is often overlooked when searching for causes of respiratory, digestive, and reproduction problems. Other miscellaneous signs of cystic fibrosis is the inability to gain weight while still having an appetite and eating a normal diet. Salty skin and sweat, and liver problems are signs that may be overlooked in a diagnosis of cystic fibrosis. This disease could be mistaken for other problems with similar symptoms. Genetic testing needs to be done to confirm the diagnosis of cystic fibrosis. Knowing your family history may help your doctor decide what is causing your distress.

If you are experiencing any problems with breathing, frequent bouts of pneumonia or upper respiratory infections, or have polyps in your sinuses, you should contact your doctor. If you are diagnosed with cystic fibrosis it is helpful to have a medical team that specializes in this disease. Educate yourself also so you will be able to contribute to your own treatment.

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Eating for Health–Foods to Enjoy and Foods to Avoid


Cystic fibrosis patients often have their digestive system attacked by the disease. Obviously a healthy diet is important for everyone but especially for those who are suffering from cystic fibrosis that is affecting their digestive system. The thick secretions caused by the mutant cystic fibrosis gene often block the small ducts that take enzymes from the pancreas to digest the foods in the stomach. When food is not digested properly, the body fails to receive the essential nutrients to grow, keep their weight, and stay healthy.

A person with cystic fibrosis should be able to eat normally but there are some foods they should avoid to prevent complications in the digestive tract. Foods that should not be eaten in large quantities include foods that increase mucus production or can cause allergies. If you are a person with CF and are lactose intolerant, you should not consume large amounts of dairy products. This includes eggs, milk, cheese, and ice-cream. Other foods you should eat only in small quantities are peanuts, bananas, and oranges. Cystic fibrosis patients should avoid saturated fats, wheat, barley, oats, and rye. They should also avoid eating red meat, and of course do not use much salt!

Foods a patient should eat in large quantities are foods that reduce inflammation and mucus. Some foods are widely known to help with health problems such as cold-water fish, nuts, lemons, garlic and onions. These foods can be taken in supplements or they may be added with more consumption of the foods that contain them. Other less widely recognized foods that will help reduce inflammation and mucus include, watercress, horseradish, mustard, umeboshi plums, rose pits tea, pickles, parsley, celery and any foods that include anti-inflammatory oils.

Adult cystic fibrosis patients know their body. They know what helps them and foods that makes them worse. A child is different and they need help from a parent, guardian or caregiver so they will eat right and avoid foods that will hurt them.

It is often difficult for cystic fibrosis patients to receive the vitamins, minerals, and enzymes the body needs. It is recommended that valuable nutrient supplements be added to diet of a cystic fibrosis patient.

Pancreatic enzymes should be taken before each meal or snack. This will help food to be properly digested by replacing the enzymes that are not coming through blocked passageways.
Antioxidants are also important. These fight against the toxins that we take into our body by breathing the air around us. Selenium, vitamin E, vitamin C. and beta-carotene should be taken as a supplement to a healthy diet.
Z-acetyl cysteine should be part of your supplement routine because it helps to thin mucus.
Zinc taken daily will help support the immune system
Quercetin is another supplement that can help reduce inflammation.

Eat lean protein, fresh vegetables and fruits that are allowed and stay away from fats and processed sugar. Eating healthy should be a habit that everyone should practice. It is especially important for a patient with cystic fibrosis.

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