A Research in General Pathology
Submitted by:
F#5
Calibo, Jansen S.
DMD2AA
Submitted to:
Dr. Sales
3
CYSTIC FIBROSIS
Cystic fibrosis, also known as CF or mucoviscidosis, is a recessive genetic disease
affecting most criti...
4
PATHOGENESIS
I. Mutation in CFTR and their consequences
The CFTR gene encompasses approximately 180,000 base pairs on th...
5
partial CFTR ion-channel activity, a feature that probably explains a less severe pulmonary
phenotype.47 Other mutation ...
6
SIGNS AND SYMPTOMS
The signs and symptoms of cystic fibrosis (CF) vary from person to person and over
time. Sometimes yo...
7
Some people who have CF also develop nasal polyps (growths in the nose) that may
require surgery.
II. Digestive System S...
8
III. Reproductive System Signs and Symptoms
Men who have CF are infertile because they're born without a vas deferens. T...
9
CLINICAL LABORATORY PROCEDURES
Cystic fibrosis carrier detection
This test detects mutations in the cystic fibrosis tran...
10
The timing of laboratory tests may rely on the results or completion of other tests,
procedures, or treatments. Lab tes...
11
There is no preparation needed for this test.
C. Chorionic villus:
CVS is a procedure that requires your written consen...
12
B. Buccal cells:
Buccal cells are cells from the inner lining of the cheek or mouth. To collect a sample of
buccal cell...
13
needle will be withdrawn. All CVS procedures may need to be repeated to collect a sufficient
sample size.
IV. How will ...
14
A. Venous blood:
After a blood sample is collected from your vein, a bandage, cotton ball, or gauze may be
placed on th...
15
the puncture site. The person doing this test may need to perform it more than once. Talk to your
healthcare worker if ...
16
suggested below, this may not mean that you have a disease. Contact your healthcare worker if
you have any questions. T...
17
DIFFERENTIAL DIAGNOSIS AND TREATMENT
Doctors diagnose cystic fibrosis (CF) based on the results from various tests.
I. ...
18
A chest x ray. This test creates pictures of the structures in your chest, such as your
heart, lungs, and blood vessels...
19
CFTR genes on to their children. If you have a family history of CF or a partner who has CF (or
a family history of it)...
20
B. Treatment for Lung Problems
The main treatments for lung problems in people who have CF are chest physical therapy
(...
21
D. Exercise
Aerobic exercise that makes you breathe harder can help loosen the mucus in your
airways so you can cough i...
22
Bronchodilators help open the airways by relaxing the muscles around them. These
medicines are inhaled. They're often t...
23
Energy-conserving techniques
Breathing strategies
Psychological counseling and/or group support
PR has many benefits. I...
24
A feeding tube to give you more calories at night while you're sleeping. The tube may be
threaded through your nose and...
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Cystic Fibrosis

  1. 1. A Research in General Pathology Submitted by: F#5 Calibo, Jansen S. DMD2AA Submitted to: Dr. Sales
  2. 2. 3 CYSTIC FIBROSIS Cystic fibrosis, also known as CF or mucoviscidosis, is a recessive genetic disease affecting most critically the lungs, and also the pancreas, liver, sinuses, sex organs and intestine. It is characterized by abnormal transport of chloride and sodium across epithelium, leading to thick, viscous secretions. It is an inherited disease of your secretory glands, including the glands that make mucus and sweat. "Inherited" means that the disease is passed through the genes from parents to children. People who have cystic fibrosis inherit two faulty cystic fibrosis genes—one from each parent. The parents likely don't have the disease themselves. Mucus is a slippery substance that lubricates and protects the linings of the airways, digestive system, reproductive system, and other organs and tissues. In people with cystic fibrosis, the body produces mucus that is abnormally thick and sticky. This abnormal mucus can obstruct the airways, leading to severe problems with breathing and bacterial infections in the lungs. These infections cause chronic coughing, wheezing, and inflammation. Over time, mucus buildup and infections result in permanent lung damage, including the formation of scar tissue (fibrosis) and cysts in the lungs. CF is caused by a mutation in the gene for the protein cystic fibrosis transmembrane conductance regulator (CFTR). This gene is required to regulate the components of sweat, digestive juices, and mucus. Although most people without CF have two working copies of the CFTR gene, only one is needed to prevent cystic fibrosis. CF develops when neither gene works normally and therefore has autosomal recessive inheritance.
  3. 3. 4 PATHOGENESIS I. Mutation in CFTR and their consequences The CFTR gene encompasses approximately 180,000 base pairs on the long arm of chromosome 7. The protein contains 1480 amino acids (Figure 1). More than 1000 disease- associated mutations have been described in the coding sequence, messenger RNA splice signals, and other regions. These mutations can be classified on the basis of the mechanism by which they are believed to cause disease (Figure 2). The most common mutation, which is termed ΔF508 and is present in approximately 70 percent of defective CFTR alleles and in 90 percent of patients with cystic fibrosis in the United States, is categorized as a class II defect. CFTR with the ΔF508 mutation lacks a phenylalanine (F) residue at position 508. The defective protein retains substantial chloride-channel function in cell-free lipid membranes. When synthesized by the normal cellular machinery, however, the protein is rapidly recognized as misfolded and is degraded shortly after synthesis, before it can reach its crucial site of action at the cell surface. Like ΔF508, several other clinically important mutations — such as N1303K, G85E, and G91R — lead to misfolded CFTR protein that is prematurely degraded. About 5 to 10 percent of CFTR mutations are due to premature truncation or nonsense alleles (designated by “X,” such as G542X, a class I mutation). As a result of a genetic founder effect, prematurely truncated CFTR is particularly prevalent among persons of Ashkenazi Jewish descent. Other CFTR mutations encode properly processed, full-length CFTR protein that lacks normal ion-channel activity. For example, the G551D mutation (class III) is believed to possess little or no chloride-channel function in vivo because of abnormal function of a nucleotide- binding domain, resulting in disordered regulation. The A455E mutation (class IV) exhibits only
  4. 4. 5 partial CFTR ion-channel activity, a feature that probably explains a less severe pulmonary phenotype.47 Other mutation classes include reduced numbers of CFTR transcripts (class V) and defective CFTR stability at the cell surface (class VI). II. Mechanisms Underlying the Sweat Gland Abnormality There is widespread agreement that defects in ion transport, salt homeostasis, or both are intimately linked to organ damage in cystic fibrosis. The precise molecular basis for this connection, however, is unknown. Conversely, sweat glands in patients with cystic fibrosis, which usually do not become obstructed or show major pathologic abnormalities, have pronounced abnormalities in sodium chloride homeostasis that are well understood. In human sweat glands, primary secretion elaborated in the glandular coil is modified as it traverses the sweat duct, before emerging on the surface of the skin. Under normal conditions, sodium (followed by chloride counter-ion) is avidly reabsorbed from the ductular lumen, primarily through apical sodium channels and CFTR (Figure 3). In patients with cystic fibrosis, the absence of functioning CFTR restricts reabsorption of chloride, thereby limiting the amount of salt that can be reclaimed. Because there is no other pathway for effective chloride reabsorption in the duct, sodium is also poorly absorbed, and sweat emerging on the skin surface contains a high level of salt. By the same token, in cystic fibrosis, the transepithelial potential difference across the sweat duct (the lumen-negative transepithelial voltage) is two to three times the normal value. The increased lumen-negative surface charge is caused by an inability to reabsorb chloride despite the continued existence of pathways for sodium uptake.
  5. 5. 6 SIGNS AND SYMPTOMS The signs and symptoms of cystic fibrosis (CF) vary from person to person and over time. Sometimes you'll have few symptoms. Other times, your symptoms may become more severe. One of the first signs of CF that parents may notice is that their baby's skin tastes salty when kissed, or the baby doesn't pass stool when first born. Most of the other signs and symptoms of CF happen later. They're related to how CF affects the respiratory, digestive, or reproductive systems of the body (Figure 4). I. Respiratory System Signs and Symptoms People who have CF have thick, sticky mucus that builds up in their airways. This buildup of mucus makes it easier for bacteria to grow and cause infections. Infections can block the airways and cause frequent coughing that brings up thick sputum (spit) or mucus that's sometimes bloody. People who have CF tend to have lung infections caused by unusual germs that don't respond to standard antibiotics. For example, lung infections caused by bacteria called mucoid Pseudomonas are much more common in people who have CF than in those who don't. An infection caused by these bacteria may be a sign of CF. People who have CF have frequent bouts of sinusitis, an infection of the sinuses. The sinuses are hollow air spaces around the eyes, nose, and forehead. Frequent bouts of bronchitis and pneumonia also can occur. These infections can cause long-term lung damage. As CF gets worse, you may have more serious problems, such as pneumothorax or bronchiectasis.
  6. 6. 7 Some people who have CF also develop nasal polyps (growths in the nose) that may require surgery. II. Digestive System Signs and Symptoms In CF, mucus can block tubes, or ducts, in your pancreas (an organ in your abdomen). These blockages prevent enzymes from reaching your intestines. As a result, your intestines can't fully absorb fats and proteins. This can cause ongoing diarrhea or bulky, foul-smelling, greasy stools. Intestinal blockages also may occur, especially in newborns. Too much gas or severe constipation in the intestines may cause stomach pain and discomfort. A hallmark of CF in children is poor weight gain and growth. These children are unable to get enough nutrients from their food because of the lack of enzymes to help absorb fats and proteins. As CF gets worse, other problems may occur, such as: Pancreatitis. This is a condition in which the pancreas become inflamed, which causes pain. Rectal prolapse. Frequent coughing or problems passing stools may cause rectal tissue from inside you to move out of your rectum. Liver disease due to inflamed or blocked bile ducts. Diabetes. Gallstones.
  7. 7. 8 III. Reproductive System Signs and Symptoms Men who have CF are infertile because they're born without a vas deferens. The vas deferens is a tube that delivers sperm from the testes to the penis. Women who have CF may have a hard time getting pregnant because of mucus blocking the cervix or other CF complications. IV. Other Signs, Symptoms, and Complications Other signs and symptoms of CF are related to an upset of the balance of minerals in your blood. CF causes your sweat to become very salty. As a result, your body loses large amounts of salt when you sweat. This can cause dehydration (a lack of fluid in your body), increased heart rate, fatigue (tiredness), weakness, decreased blood pressure, heat stroke, and, rarely, death. CF also can cause clubbing and low bone density. Clubbing is the widening and rounding of the tips of your fingers and toes. This sign develops late in CF because your lungs aren't moving enough oxygen into your bloodstream. Low bone density also tends to occur late in CF. It can lead to a bone-thinning disorder called osteoporosis.
  8. 8. 9 CLINICAL LABORATORY PROCEDURES Cystic fibrosis carrier detection This test detects mutations in the cystic fibrosis transmembrane regulator (CFTR) gene. This genetic test is used to help screen for cystic fibrosis. A sample of blood, buccal cells, or chorionic villus may be collected for this test. I. What are other names for this test? Cystic fibrosis DNA detection Cystic fibrosis mutation analysis II. What are related tests? Serum immunoreactive trypsin measurement III. Why does patient need this test? Laboratory tests may be done for many reasons. Tests are performed for routine health screenings or if a disease or toxicity is suspected. Lab tests may be used to determine if a medical condition is improving or worsening. Lab tests may also be used to measure the success or failure of a medication or treatment plan. Lab tests may be ordered for professional or legal reasons. The following is a possible reason why this test may be done: A. Cystic fibrosis screening I. When and how often should I have this test? When and how often laboratory tests are done may depend on many factors:
  9. 9. 10 The timing of laboratory tests may rely on the results or completion of other tests, procedures, or treatments. Lab tests may be performed immediately in an emergency, or tests may be delayed as a condition is treated or monitored. A test may be suggested or become necessary when certain signs or symptoms appear. Due to changes in the way your body naturally functions through the course of a day, lab tests may need to be performed at a certain time of day. If you have prepared for a test by changing your food or fluid intake, lab tests may be timed in accordance with those changes. Timing of tests may be based on increased and decreased levels of medications, drugs or other substances in the body. The age or gender of the person being tested may affect when and how often a lab test is required. Chronic or progressive conditions may need ongoing monitoring through the use of lab tests. Conditions that worsen and improve may also need frequent monitoring. Certain tests may be repeated to obtain a series of results, or tests may need to be repeated to confirm or disprove results. Timing and frequency of lab tests may vary if they are performed for professional or legal reasons. II. How should I get ready for the test? A. Venous blood: Before having blood collected, tell the person drawing your blood if you are allergic to latex. Tell the healthcare worker if you have a medical condition or are using a medication or supplement that causes excessive bleeding. Also tell the healthcare worker if you have felt nauseated, lightheaded, or have fainted while having blood drawn in the past. B. Buccal cells:
  10. 10. 11 There is no preparation needed for this test. C. Chorionic villus: CVS is a procedure that requires your written consent. Review the consent form with the healthcare worker and ask any questions that you have before signing the consent form. Tell the person doing the CVS if you have a history of pregnancy difficulties, such as premature (early) labor, incompetent cervix (a weak or failing cervix), placenta previa (a placenta that is abnormally low, near or over the cervix), abruption placentae (the placenta is separate from the uterine wall too early), and if you are Rh negative (Rh incompatibilities happen when a baby’s blood has a protein that the mother does not, thus causing an immune reaction). Tell the healthcare worker if you have a medical condition or are using a medication or supplement that causes excessive bleeding. You should also report if you have a history of allergic or other reactions to local anesthetics. Depending on method used to do the CVS, you may be asked to drink extra fluids and have a full bladder for the procedure. III. How is the test done? A sample of venous blood, buccal cells, or chorionic villus may be collected for this test. A. Venous blood: When a blood sample from a vein is needed, a vein in your arm is usually selected. A tourniquet (large rubber strap) may be secured above the vein. The skin over the vein will be cleaned, and a needle will be inserted. You will be asked to hold very still while your blood is collected. Blood will be collected into one or more tubes, and the tourniquet will be removed. When enough blood has been collected, the healthcare worker will take the needle out.
  11. 11. 12 B. Buccal cells: Buccal cells are cells from the inner lining of the cheek or mouth. To collect a sample of buccal cells, you will need to open your mouth wide. A special brush or swab will be rotated rapidly up and down on your inner cheeks for 30 seconds. Do not close your mouth when the sample is being collected. After the sample has been collected, the brush or swab will be taken out and tested. C. Chorionic villus: The chorionic villus is a part of the placenta (the organ that nourishes the baby during pregnancy). A sample of chorionic villus is collected by a procedure called chorionic villus sampling (CVS). Depending on the location of your placenta, CVS may be done either through your cervix (transcervically) or abdomen (transabdominally). Both methods will require you to lie down and will use ultrasound to assist the sample collection. For a transcervical CVS, you will be in a position similar to a Pap smear. A speculum will be used to gently spread apart your vagina. Your cervix or vagina will be cleaned with an antiseptic solution. A flexible catheter will be placed through your cervix and a small sample removed. For the transabdominal method, a needle will be used to go through the abdominal wall into the placenta. This will allow a syringe to draw out a small sample of placenta. If a transabdominal method is used, you will be asked to lie on your back. An area of skin on your abdomen will be cleaned with an antiseptic solution, and a sterile area prepared. You will be given anesthetic to numb your skin. When the area is numb, a needle will be placed through your skin and into the placenta. A small sample of the placenta will be collected and the
  12. 12. 13 needle will be withdrawn. All CVS procedures may need to be repeated to collect a sufficient sample size. IV. How will the test feel? The amount of discomfort you feel will depend on many factors, including your sensitivity to pain. Communicate how you are feeling with the person doing the test. Inform the person doing the test if you feel that you cannot continue with the test. A. Buccal cells: This test usually causes no discomfort. B. Venous blood: During a blood draw, you may feel mild discomfort at the location where the blood sample is being collected. C. Chorionic villus: During a transcervical CVS procedure, you may feel mild cramping in your abdomen or pelvic area. Before a transabdominal or transcervical CVS procedure, a local anesthetic is given to the procedure site to numb the area. You may feel mild discomfort or stinging when the numbing medicine is injected. As the procedure needle or catheter is inserted through the abdomen or cervix, you may feel some discomfort and pressure. You may feel mild cramping in your abdomen and pelvic area during the procedure. The procedure site may be sore for several days. V. What should I do after the test?
  13. 13. 14 A. Venous blood: After a blood sample is collected from your vein, a bandage, cotton ball, or gauze may be placed on the area where the needle was inserted. You may be asked to apply pressure to the area. Avoid strenuous exercise immediately after your blood draw. Contact your healthcare worker if you feel pain or see redness, swelling, or discharge from the puncture site. B. Buccal cells: There are no special instructions to follow after this test. C. Chorionic villus: After all CVS procedures, ultrasound and fetal monitoring may be done immediately after the procedure. If a needle was used, pressure may be held to the site until the bleeding or drainage has stopped. A bandage will be placed over the site if a transabdominal method was used. After all CVS procedures, rest is necessary. Do not have sexual intercourse, douche, and avoid heavy lifting for at least 24 hours after all the procedures. Contact your healthcare worker if there is redness, swelling, pus, drainage, or pain at the procedure site if the transabdominal method was used. For all procedure methods, alert your healthcare worker immediately should you develop a fever; bleeding (heavier than light spotting), fluid leakage or discharge from your vagina; or severe abdominal cramping or pain. An ultrasound is usually done 2 to 4 days after the CVS to make sure that the fetus is doing well. VI. What are the risks? Blood: During a blood draw, a hematoma (blood-filled bump under the skin) or slight bleeding from the puncture site may occur. After a blood draw, a bruise or infection may occur at
  14. 14. 15 the puncture site. The person doing this test may need to perform it more than once. Talk to your healthcare worker if you have any concerns about the risks of this test. Buccal cells: A buccal cell collection is generally considered safe. Talk to your healthcare worker if you have questions or concerns about the risks of this test. Placental tissue (chorionic villus): A placental tissue sample is collected by a procedure called chorionic villus sampling (CVS). Depending on where your placenta is located, different methods may be used. CVS risks, depending on the method, include bleeding and infection at the site. If you have a medical condition, or are using a medication or supplement that causes excessive bleeding, you are at a higher risk of bleeding from the puncture site. It is possible that the needle or catheter that is used to collect the tissue will injure the baby. You may develop a fever, an abnormal vaginal discharge, abdominal pain or cramping, or go into labor. If there is a possibility that you and your baby are not Rh compatible, you may need additional treatment to avoid further complications. There is a risk that your baby will not survive the procedure, or may be adversely affected by this procedure. It is possible that the baby’s limbs, fingers, and toes may be affected by this procedure. The chances of these risks vary depending on your health status, how long you have been pregnant before the CVS procedure, and other factors. The person doing this test may need to perform it more than once. Talk with your healthcare worker if you have any concerns about the risks of having CVS. VII. What are normal results for this test? Laboratory test results may vary depending on your age, gender, health history, the method used for the test, and many other factors. If your results are different from the results
  15. 15. 16 suggested below, this may not mean that you have a disease. Contact your healthcare worker if you have any questions. The following is considered to be a normal result for this test: Negative VIII. What follow up should I do after this test? Ask your healthcare worker how you will be informed of the test results. You may be asked to call for results, schedule an appointment to discuss results, or notified of results by mail. Follow up care varies depending on many factors related to your test. Sometimes there is no follow up after you have been notified of test results. At other times follow up may be suggested or necessary. Some examples of follow up care include changes to medication or treatment plans, referral to a specialist, more or less frequent monitoring, and additional tests or procedures. Talk with your healthcare worker about any concerns or questions you have regarding follow up care or instructions. A. Chorionic villus: After a CVS procedure, it may take from 1 to 4 weeks to receive results. There is a possibility that you may need an amniocentesis if the CVS was not successful.
  16. 16. 17 DIFFERENTIAL DIAGNOSIS AND TREATMENT Doctors diagnose cystic fibrosis (CF) based on the results from various tests. I. DIAGNOSIS A. Newborn Screening All States screen newborns for CF using a genetic test or a blood test. The genetic test shows whether a newborn has faulty CFTR genes. The blood test shows whether a newborn's pancreas is working properly. B. Sweat Test If a genetic test or blood test suggests CF, a doctor will confirm the diagnosis using a sweat test. This test is the most useful test for diagnosing CF. A sweat test measures the amount of salt in sweat. For this test, the doctor triggers sweating on a small patch of skin on an arm or leg. He or she rubs the skin with a sweat-producing chemical and then uses an electrode to provide a mild electrical current. This may cause a tingling or warm feeling. Sweat is collected on a pad or paper and then analyzed. The sweat test usually is done twice. High salt levels confirm a diagnosis of CF. C. Other Tests If you or your child has CF, your doctor may recommend other tests, such as: Genetic tests to find out what type of CFTR defect is causing your CF.
  17. 17. 18 A chest x ray. This test creates pictures of the structures in your chest, such as your heart, lungs, and blood vessels. A chest x ray can show whether your lungs are inflamed or scarred, or whether they trap air. A sinus x ray. This test may show signs of sinusitis, a complication of CF. Lung function tests. These tests measure how much air you can breathe in and out, how fast you can breathe air out, and how well your lungs deliver oxygen to your blood. sputum culture. For this test, your doctor will take a sample of your sputum (spit) to see whether bacteria are growing in it. If you have bacteria called mucoid Pseudomonas, you may have more advanced CF that needs aggressive treatment. D. Prenatal Screening If you're pregnant, prenatal genetic tests can show whether your fetus has CF. These tests include amniocentesis and chorionic villus sampling (CVS). In amniocentesis, your doctor inserts a hollow needle through your abdominal wall into your uterus. He or she removes a small amount of fluid from the sac around the baby. The fluid is tested to see whether both of the baby's CFTR genes are normal. In CVS, your doctor threads a thin tube through the vagina and cervix to the placenta. The doctor removes a tissue sample from the placenta using gentle suction. The sample is tested to see whether the baby has CF. E. Cystic Fibrosis Carrier Testing People who have one normal CFTR gene and one faulty CFTR gene are CF carriers. CF carriers usually have no symptoms of CF and live normal lives. However, carriers can pass faulty
  18. 18. 19 CFTR genes on to their children. If you have a family history of CF or a partner who has CF (or a family history of it) and you're planning a pregnancy, you may want to find out whether you're a CF carrier. A genetics counselor can test a blood or saliva sample to find out whether you have a faulty CF gene. This type of testing can detect faulty CF genes in 9 out of 10 cases. II. TREATMENT Cystic fibrosis (CF) has no cure. However, treatments have greatly improved in recent years. The goals of CF treatment include: Preventing and controlling lung infections Loosening and removing thick, sticky mucus from the lungs Preventing or treating blockages in the intestines Providing enough nutrition Preventing dehydration (a lack of fluid in the body) Depending on the severity of CF, you or your child may be treated in a hospital. A. Specialists Involved If you or your child has CF, you may be treated by a CF specialist. This is a doctor who is familiar with the complex nature of CF. Often, a CF specialist works with a medical team of nurses, physical therapists, dietitians, and social workers. CF specialists often are located at major medical centers. The United States also has more than 100 CF Care Centers. These centers have teams of doctors, nurses, dietitians, respiratory therapists, physical therapists, and social workers who have special training related to CF care. Most CF Care Centers have pediatric and adult programs or clinics.
  19. 19. 20 B. Treatment for Lung Problems The main treatments for lung problems in people who have CF are chest physical therapy (CPT), exercise, and medicines. Your doctor also may recommend a pulmonary rehabilitation (PR) program. C. Chest Physical Therapy CPT also is called chest clapping or percussion. It involves pounding your chest and back over and over with your hands or a device to loosen the mucus from your lungs so that you can cough it up. You might sit down or lie on your stomach with your head down while you do CPT. Gravity and force help drain the mucus from your lungs. Some people find CPT hard or uncomfortable to do. Several devices have been developed that may help with CPT, such as: An electric chest clapper, known as a mechanical percussor. An inflatable therapy vest that uses high-frequency airwaves to force the mucus that's deep in your lungs toward your upper airways so you can cough it up. small, handheld device that you exhale through. The device causes vibrations that dislodge the mucus. A mask that creates vibrations that help break the mucus loose from your airway walls. Breathing techniques also may help dislodge mucus so you can cough it up. These techniques include forcing out a couple of short breaths or deeper breaths and then doing relaxed breathing. This may help loosen the mucus in your lungs and open your airways.
  20. 20. 21 D. Exercise Aerobic exercise that makes you breathe harder can help loosen the mucus in your airways so you can cough it up. Exercise also helps improve your overall physical condition. However, CF causes your sweat to become very salty. As a result, your body loses large amounts of salt when you sweat. Thus, your doctor may recommend a high-salt diet or salt supplements to maintain the balance of minerals in your blood. If you exercise regularly, you may be able to cut back on your CPT. However, you should check with your doctor first. E. Medicines If you have CF, you doctor may prescribe antibiotics, anti-inflammatory medicines, bronchodilators, or mucus-thinning medicines. These medicines help treat or prevent lung infections, reduce swelling, open up the airways, and thin mucus. Antibiotics are the main treatment to prevent or treat lung infections. Your doctor may prescribe oral, inhaled, or intravenous (IV) antibiotics. Oral antibiotics often are used to treat mild lung infections. Inhaled antibiotics may be used to prevent or control infections caused by the bacteria mucoid Pseudomonas. For severe or hard-to-treat infections, you may be given antibiotics through an IV tube (a tube inserted into a vein). This type of treatment may require you to stay in a hospital. Anti-inflammatory medicines can help reduce swelling in your airways due to ongoing infections. These medicines may be inhaled or oral.
  21. 21. 22 Bronchodilators help open the airways by relaxing the muscles around them. These medicines are inhaled. They're often taken just before CPT to help clear mucus out of your airways. You also may take bronchodilators before inhaling other medicines into your lungs. Your doctor may prescribe mucus-thinning medicines to reduce the stickiness of your mucus and loosen it up. These medicines can help clear out mucus, improve lung function, and prevent worsening lung symptoms. F. Treatments for Advanced Lung Disease If you have advanced lung disease, you may need oxygen therapy. Oxygen usually is given through nasal prongs or a mask. If other treatments haven't worked, a lung transplant may be an option if you have severe lung disease. A lung transplant is surgery to remove a person's diseased lung and replace it with a healthy lung from a deceased donor. G. Pulmonary Rehabilitation Your doctor may recommend PR as part of your treatment plan. PR is a broad program that helps improve the well-being of people who have chronic (ongoing) breathing problems. PR doesn't replace medical therapy. Instead, it's used with medical therapy and may include: Exercise training Nutritional counseling Education on your lung disease or condition and how to manage it
  22. 22. 23 Energy-conserving techniques Breathing strategies Psychological counseling and/or group support PR has many benefits. It can improve your ability to function and your quality of life. The program also may help relieve your breathing problems. Even if you have advanced lung disease, you can still benefit from PR. H. Treatment for Digestive Problems CF can cause many digestive problems, such as bulky stools, intestinal gas, a swollen belly, severe constipation, and pain or discomfort. Digestive problems also can lead to poor growth and development in children. Nutritional therapy can improve your strength and ability to stay active. It also can improve growth and development in children. Nutritional therapy also may make you strong enough to resist some lung infections. A nutritionist can help you create a nutritional plan that meets your needs. In addition to having a well-balanced diet that's rich in calories, fat, and protein, your nutritional therapy may include: Oral pancreatic enzymes to help you digest fats and proteins and absorb more vitamins. Supplements of vitamins A, D, E, and K to replace the fat-soluble vitamins that your intestines can't absorb. High-calorie shakes to provide you with extra nutrients. A high-salt diet or salt supplements that you take before exercising.
  23. 23. 24 A feeding tube to give you more calories at night while you're sleeping. The tube may be threaded through your nose and throat and into your stomach. Or, the tube may be placed directly into your stomach through a surgically made hole. Before you go to bed each night, you'll attach a bag with a nutritional solution to the entrance of the tube. It will feed you while you sleep. Other treatments for digestive problems may include enemas and mucus-thinning medicines to treat intestinal blockages. Sometimes surgery is needed to remove an intestinal blockage. Your doctor also may prescribe medicines to reduce your stomach acid and help oral pancreatic enzymes work better. I. Treatments for Cystic Fibrosis Complications A common complication of CF is diabetes. The type of diabetes associated with CF often requires different treatment than other types of diabetes. Another common CF complication is the bone-thinning disorder osteoporosis. Your doctor may prescribe medicines that prevent your bones from losing their density.

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