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1 W-F Professional Associates, Inc. 400 Lake Cook Rd., Suite 207 Deerfield, IL April 2003 Pharmacy Perspective: Cystic Fibrosis H01 MISSING A LESSON? IT S EASY TO GO TO OUR WEBSITE & DOWNLOAD WHAT YOU NEED. ( THIS MONTH Cystic Fibrosis LESSONS ARE USUALLY ON THE WEBSITE BY THE FIRST DAY OF EACH MONTH. ALWAYS KEEP A COPY OF YOUR QUIZZES. YOU MAY MAIL QUIZZES TO US, FAX THEM ( ), OR SEND US A CONVENTIONAL WITH YOUR ANSWERS. (info@wfprofessional.com) HAVE YOU RECENTLY MOVED? PLEASE NOTIFY US. Your comments and suggestions are valuable to us. Last month s lesson covered COPD. It is a natural progression of topics that we discuss another respiratory ailment, and that is Cystic Fibrosis. Our goal is to present viable treatment options. This lesson provides 1.25 hours (0.125 CEUs) of credit, and is intended for pharmacists in all practice settings. The program ID # for this lesson is H01. Pharmacists completing this lesson by April 30, 2006 may receive full credit. To obtain continuing education credit for this lesson, you must answer the questions on the quiz (70% correct required), and return the quiz. Should you score less than 70%, you will be asked to repeat the quiz. Computerized records are maintained for each participant. Complete List of 2003 Topics: See Page 10. If you have any comments, suggestions or questions, contact us at the above address, or call toll free (In Alaska and Hawaii phone ). Please write your ID Number (the number that is on the top of the mailing label) in the indicated space on the quiz page (for continuous participants only). The objectives of this lesson are such that upon completion the participant will be able to: 1. Discuss the epidemiology of CF. 2. Describe the pathophysiology associated with CF as it relates to a number of systems. 3. List the signs & symptoms of CF. 4. Comment upon & describe the commonly used diagnostic tests for determining presence of CF. 5. List & discuss therapeutic & treatment options that are utilized for CF. All opinions expressed by the author/authors are strictly their own and are not necessarily approved or endorsed by W-F Professional Associates, Inc. Consult full prescribing information on any drugs or devices discussed.

2 INTRODUCTION Cystic fibrosis (CF) is a genetically inherited disease that affects the body s mucus glands. It is a chronic progressive disease that affects the pulmonary, gastrointestinal, genitourinary, and hematologic organ systems. Patients can suffer from a wide variety of ailments such as frequent pulmonary infections, chronic obstructive pulmonary disease, malnutrition, sinusitis, nasal polyps, glucose intolerance, diabetes, sterility, and anemia. Demographic data indicate that the average lifespan of the CF patient is 30 years. EPIDEMIOLOGY The Cystic Fibrosis Foundation indicates that approximately 30,000 children and adults in the United States are afflicted with CF. 1 It is most common among Caucasians whose ancestors came from northern Europe. Although it affects all races and ethnic groups, it is less prevalent among African Americans, Native Americans, and Asian Americans. 2 As mentioned above, CF is a genetically inherited disease. The CF gene is a recessive trait that must be inherited from both parents for disease development to occur. 2 Data indicates that 1 in 28 Caucasians and 1 in 31 Americans are carriers of the CF recessive gene. When two carriers of the CF gene (heterozygous) conceive, they would have a 25% chance of having a child with the disease, a 50% chance of having a child who is a carrier, and a 25% chance of having a child being born normally without having the disease or being a carrier. 2 This concept is illustrated in the following diagram. Each year approximately 2,500 babies are born with CF in the U.S. 2 Figure 1: April 2003 Pharmacy Perspective: Cystic Fibrosis Volume 25 Number 4 Carrier Father (Cc) Carrier Mother (Cc) Normal (CC) Carrier (Cc) Carrier (Cc) Cystic fibrosis (cc) C = normal gene; c = recessive defective gene CE PRN (ISSN ) is owned and published by W-F Professional Associates, Inc. 400 Lake Cook Road, Suite 207, Deerfield, Illinois William J. Feinberg, President CE PRN is published eleven times per year, monthly, January through November. Subscription rate is $90.00 per year. Second-Class Postage paid at Deerfield, Illinois and at additional mailing offices by W-F Professional Associates, Inc. All rights reserved. None of the contents of this publication may be reproduced in any form without the written permission of the publisher. POSTMASTER: Send all address changes to W-F Professional Associates, Inc., 400 Lake Cook Road, Suite 207, Deerfield, IL April

3 Scientists have identified over 700 gene mutations that are responsible for contributing to the development of CF. 3 These gene mutations have been identified on the long arm of chromosome 7. 3 The CF gene codes for a protein called the cystic fibrosis transmembrane regulator (CFTR) which regulates membrane transport of electrolytes and water. 3 The CFTR protein is expressed in epithelial cells of the airways, gastrointestinal tract, pancreas, sweat glands, and genitourinary systems. 3 Currently researchers are working with the CFTR protein using gene therapy. 1 Consult the references for more information. PATHOPHYSIOLOGY Normally, epithelial cells transport chloride out of the blood along with sodium and water. 4 Hormones, neurotransmitters, and cyclic adenosine monophosphate (camp) activate this process. When the CFTR protein attacks epithelial cells, it makes them unable to secrete chloride ions in response to camp signals. 3 The result is excessive amounts of sodium, chloride, and water being absorbed through these membranes. Because there is not enough water available on the airway surface to hydrate secretions, a CF patient s secretions become thick, viscous, and elastic-like, making them hard to clear by mucociliary mechanisms, reducing pulmonary function, and increasing the risk for bronchial infection. 3 These thick secretions can also block pancreatic, gastrointestinal and hepatobiliary organs. 4 Pulmonary system 3,4,5 Thick mucus secretions can accumulate in the small airways of the lungs, eventually leading to bronchitis. Airway obstruction from viscous mucus can lead to a chronic obstructive pulmonary disease-like syndrome. Since mucus is an excellent growth media for microorganisms, respiratory infections are common. The most frequently cultured bacteria from respiratory tract secretions in CF patients are Staphylococcus aureus, Pseudomonas aeruginosa and Haemophilus influenzae. With life expectancy increasing in CF patients, an organism called Burkholderia cepacia has emerged as a threat. This organism is resistant to aminoglycosides and polymixins, and when infecting a patient, can cause rapid deterioration in pulmonary function and death. Bacteria may also be responsible for causing inflammation and lung tissue destruction by means of stimulating the production of immune complexes and inflammatory mediators such as interleukins and tumor necrosis factor. With advanced disease, pneumothorax, right-sided heart failure, and pulmonary hypertension can result. Gastrointestinal 3,4 The gastrointestinal system exhibits the first sign of CF in a newborn, when the infant is unable to pass the viscous meconium. Excessive thick gastrointestinal secretions can lead to fecal impaction, peritonitis, rectal prolapse, and gastroesophageal reflux disease. Pancreas 4,5 As pancreatic ducts become filled with thickened mucus, they distend and cause lesions. These lesions impair pancreatic function by replacing ducts with fibrous and fatty tissue. As a result, pancreatic secretions are low in concentrations of pancreatic enzymes and bicarbonate. Inefficient pancreaticfunctioning may also cause an insulin deficiency and/or glucose intolerance in older CF patients. The islets of Langerhans located in the pancreas contain a normal amount of beta cells during the first decade of life; however, their function deteriorates during the second decade of life as fibrous tissue impairs pancreatic beta cell utility. Glucose intolerance and diabetes are estimated as afflicting up to 15% of the CF population. Intestinal tract 3 Mucus secretions engulf glands of the esophagus, duodenum, cecum, appendix, and rectum. 3

4 Hepatic-biliary tract 3,4 Over abundance of mucus can block hepatic bile ducts and cause biliary cirrhosis. In addition, up to 30% of patients develop fatty infiltration of the liver. Although this can occur at any age, it is most common in older CF patients. This appears to be unrelated to a patient s nutritional status. Negative outcomes of liver involvement are portal hypertension, esophageal varices, and hypersplenism. Mucus material can also overwhelm the gallbladder and cause gallstones. Nasal sinuses 3,4 The nasal sinuses can become filled with excess mucosal secretions causing sinusitis. Nasal polyps may form as well from inflamed, edematous, nasal mucosa. The same microbes responsible for CF pulmonary infections are common to sinusitis infections. These include Pseudomonas aeruginosa, and Hemophilus influenzae, plus Streptococci. Sweat glands 3,4 The sweat glands of CF patients contain high levels of sodium and chloride, since the disease destroys the ability of the epithelial cells to excrete these electrolytes. Supplemental electrolytes are often needed to offset this process. Reproductive system 3,4 Up to 95% of male CF patients are sterile. The epididymis, vas deferens, and seminal vesicles become blocked with mucus material, preventing the formation of semen. Female patients develop abnormal cervical mucus that impairs fertility. Both male and female CF patients may experience late onset of puberty and maturation of reproductive organs. Hematology 3,4 Some CF patients develop iron deficiency anemia. It appears that impaired gastrointestinal absorption of iron, low erythropoetin levels, or increased blood loss are to blame. Laboratory indices will show a decreased hematocrit, serum ferritin, increased carboxyhemoglobin, and/or low to normal hemoglobin levels. Bones and joints 3,4 CF patients frequently develop arthritis. This disease may develop because of immune complex formations secondary to chronic pulmonary infections. CF patients also have a high frequency of osteopenia and osteoporosis. Malabsorption of fat-soluble vitamin D, poor exercise tolerance, and malnutrition are contributors. SIGNS AND SYMPTOMS Respiratory 4,5 One of the first symptoms of pulmonary involvement is a cough. The cough can initially be dry and hacking, but then becomes productive and loose. The patient will expectorate mucus that is usually purulent. Some CF patients will have a chronic cough that presents itself at birth. Others may be asymptomatic but have a history of frequent bronchial infections. As pulmonary disease progresses, pneumonia, bronchiolitis, shortness of breath, exercise intolerance, failure to thrive, and malnutrition predominate. On physical examination, patients may have a barrel chest that develops from using accessory chest muscles to breathe because of poor air exchange by the lungs. A flat diaphragm can also be observed. When listening to the lungs, coarse crackles and expiratory wheezes may be heard. Fingers may exhibit clubbing. Patient s pulmonary function will show decline. 4

5 Gastrointestinal 3,4 At birth, up to 20% of infants with CF have meconium ileus. This condition is characterized by an inability to pass the meconium along with abdominal distention, vomiting, and pain. Older CF patients can experience this too, when the ileus becomes obstructed with fecal material. Maldigestion of fats and protein are evident in up to 85% of CF patients. Symptoms of bulky greasy foulsmelling stools, flatulence, abdominal distention, decreased muscle mass, failure to grow, and late maturation is apparent. Other GI symptoms can include fecal impaction of the cecum, esophageal reflux, appendicitis, and rectal prolapse. Biliary system 3,4 Biliary cirrhosis, jaundice, ascites, esophageal varices and hypersplenism may affect 2-3% of CF patients. Pancreas 3,4 Glucose intolerance and diabetes are estimated as afflicting up to 15% of the CF population. Signs of diabetes such as polyuria, polydipsia, hyperglycemia, glucosuria, and weight loss may appear. Although ketoacidosis does not occur, long term complications such as diabetic retinopathy, renal and cardiovascular disease have been noted. Genitourinary system 3,4 In males, rates of inguinal hernia, hydrocele, and undescended testicle are higher than the general population. Adolescent female CF patients can experience amenorrhea and cervicitis. Pregnancy in women with CF who have good pulmonary function can be risky, but many have had healthy children. It is important to note that the incidence of CF in their offspring is 50%. Sweat glands 3,4 CF patients can develop hypochloremic alkalosis from excessive salt lost in their sweat, during a bout of gastroenteritis, or warm weather. The first sign of this can be a parent reporting that the child s skin tastes salty when they kiss their child. DIAGNOSIS Sweat chloride testing 2,3 The most common test to diagnose CF is the sweat test. This measures the amount of sodium chloride from sweat obtained on an area of skin. The forearm is routinely used. In order to induce sweating, the drug pilocarpine and a mild electric current are applied to the skin. The pilocarpine stimulates sweat glands. A piece of gauze or filter paper is then placed over the area of skin, and the area is wrapped in plastic. After 30 to 40 minutes, the plastic and gauze filter paper are removed, and the concentration of sodium chloride in sweat is analyzed from the paper. Having sweat chloride levels of >/= 60meq/L from samples done on two separate days, as well as one or more of the following criteria: presence of respiratory, GI, or GU disease, positive family history of CF (i.e. a sibling), genetic identification of two CF mutations, positive newborn screening or nasal abnormalities, is diagnostic for CF. There are several non-cf health conditions that can produce false positive sweat chloride elevations such as untreated adrenal insufficiency, diabetes insipidus, glucose-6-phosphatase deficiency, hypothyroidism, hypoparathyroidism, and pancreatitis. Immunoreactive trypsinogen test (IRT) 2,3 Some patients may not produce enough sweat for testing, i.e. newborns. For these types of cases, the immunoreactive trypsinogen blood test is used. In this test, blood is drawn 2-3 days after birth and analyzed for the protein trypsinogen. Having a positive IRT, along with positive sweat tests and/or DNA genetic testing, indicates CF. 5

6 Pancreatic dysfunction 2 Evaluating pancreatic function by measuring the amount of undigested fat in stools collected over a 3-day period is sometimes used. However, this type of screening does not always produce definitive results and is viewed by many parents as invasive. Radiology 2 Chest x-rays showing lung abnormalities such as bronchial thickening, hyperinflation, bronchiectasis, or nodes may suggest CF, but are not diagnostic. Fetal ultrasounds showing an ileal obstruction do not predict meconium ileus at time of birth. Pulmonary 2 Pulmonary function screening for older children (5-6yrs) indicating obstructive airway disease with minimal relief from bronchodilator drug therapy points to a CF diagnosis. Culturing S. aureus, P. aureginosa, or B. cepacia from sputum is a strong indicator of CF. Genetics testing 2 Prenatal genetic testing via either amniocentesis or chorionic villus biopsy may be performed to see if CF genes are present. Testing is recommended for women who already have a child with CF to determine if the fetus inherited genes from both parents, or is a carrier for the CF gene. However, the high monetary cost, health risks to the pregnant woman, and inability to detect all 700 different CF gene mutations are drawbacks to these types of tests. TREATMENT The goals of therapy are to halt lung damage by controlling bacterial infections with antibiotics, removing thick viscous airway secretions, and providing proper nutrition for the CF patient. 5 Although a variety of drug therapies are used to treat disease-related complications of CF, the focus of this section will be pharmacological agents used to treat bacterial infections, improve respiratory function, and support nutrition. Consult the references for more information about treating other organ systems. Antibiotic therapy 1,3,4,5 Antibiotics are the mainstay treatments for this disease. They are used for both the prevention and treatment of respiratory infections. Since mucus is an excellent growth media for microorganisms, respiratory infections are common. The most frequently cultured bacteria from respiratory secretions in CF patients are Staphylococcus aureus, Pseudomonas aeruginosa and Haemophilus influenzae. During the first 2 years of life, S. aureus colonizes the respiratory tract in approximately 30% of CF patients. Later, during childhood and early adolescence, up to 80% of CF patients become chronically infected with P. aeruginosa. About 10-20% of CF patients become infected with methicillin resistant Staphylococcus aureus (MRSA). Burkholderia cepacia is another bacterium that has emerged since the 1970 s. This bacterium originates in the natural environment and is resistant to cleaning disinfectants and antibiotics. Initially the organism is sensitive to trimethoprim-sulfamethoxazole and antipseudomonal beta-lactam antibiotics, but resistance occurs quickly, even to aminoglycosides and polymixins, making infectious disease therapy a challenge. For healthy people, this organism poses no threat; however, to CF patients with compromised respiratory function, it can cause rapid deterioration in pulmonary function and rapid death. Burkholderia cepacia can be transmitted to individuals by direct contact with infected sputum, saliva, or when shaking hands, or indirectly from touching contaminated surfaces. Because cross contamination of B. cepacia can easily occur between CF patients, the Centers for Disease Control established disinfecting procedures to reduce contamination when cleaning patients rooms and respiratory equipment. The best way to choose an antibiotic is to obtain a culture and sensitivity to identify the pathogen in the respiratory tract. However, sometimes this is not possible, and an empiric choice must be made. The antibiotic doses required for CF patients are usually higher than what is recommended for minor infections, since CF 6

7 patients have an increased renal clearance, leaner body mass, and because it is hard to reach effective antibiotic concentrations in respiratory tract secretions. The course of therapy for antibiotics is usually 2 weeks or longer at maximum doses. Long-term therapy with lower doses increases the development of bacterial resistance. Broad-spectrum oral antibiotics such as amoxicillin, cephalexin, clindamycin, amoxicillin-clavulanate, and trimethoprim-sulfamethoxazole are indicated for acute respiratory exacerbations. These agents are typically effective against S. aureus and H. influenzae although P. aeruginosa is usually resistant. The Cystic Fibrosis Foundation has been studying the antibiotic azithromycin as a treatment against P. aeruginosa. One clinical trial found azithromycin therapy to improve lung function, help patients gain body weight, and assist in reducing the number of days in the hospital for treating lung infections. More research is still needed, since it is unknown how this drug works in CF. Other antibiotics such as tetracycline are rarely used in children because of the risk of teeth discoloration. The quinolone ciprofloxacin is a broad-spectrum antibiotic that is effective against P. aeruginosa, but resistance develops quickly. For patients who require frequent treatments for P. aeruginosa, polymixins and colistin may be the only drug therapies that are susceptible. Intravenous antibiotics are indicated for patients with moderate to severe progressive infections that are unresponsive to oral antibiotics or other drug therapies. For P. aeruginosa treatment, two agents are usually required. Sometimes a third agent is recommended for adequate S. aureus coverage, such as intravenous nafcillin and vancomycin. Pharmacological agents suggested to eradicate P. aeruginosa are carbenicillin, ticarcillin, piperacillin, ticarcillin-clavulanate, imipenem-cilastin, ceftazidime, and aztreonam, as well as the aminoglycosides tobramycin and amikacin. Tobramycin and amikacin will have a short half-life in CF patients, and are generally recommended on being dosed on an every 8-hour frequency. Attaining a peak of 10mg/L and a trough of <2mg/L minimizes ototoxicity and nephrotoxicity. Although once daily dosing has gained popularity in many hospital settings, the safety and efficacy of this regimen has not been adequately studied in CF patients. Aerosolized chloramphenicol and oral trimethoprim-sulfamethoxazole are suggested as antimicrobial treatments for B. cepacia lung infections. Respiratory therapy 3,4,5,6 Liquefying and removing thick viscous airway secretions helps improve pulmonary function, as well as helping to prevent microbial lung infections, since mucus is an excellent growth media for microorganisms. Chest percussion physical therapy and postural drainage help clear mucus from the lungs. Nebulized 0.9% sodium chloride assists in liquefying pulmonary secretions, as does the mucolytic agent N-acetylcysteine. Yet because of its unpleasant taste and odor many patients prefer nebulized saline to N-acetylcysteine. Patients may also use a hand-held device called the flutter to help dislodge excess mucus from the lungs. The way this device works is the patient exhales through the flutter pipe and a special valve causes air pressure fluctuation that causes vibrations in the airway walls. This promotes the dislodging of mucus from the lungs. The advantage of this device is that CF patients can achieve airway mucus clearance on their own without the assistance of other family members. A pharmacological agent called dornase alfa is a DNA enzyme produced by recombinant gene technology that breaks apart DNA released from white blood cells that die when fighting bacterial infections. The DNA released is normally sticky, which further exacerbates mucus viscosity and thickness, worsening pulmonary function and serving as bacterial growth media. By helping reduce mucus stickiness, dornase alfa assists in improving lung function and lessening the chance for pulmonary infections. Dornase alfa is administered by nebulization. It should not be diluted or mixed with any other medications, since they may inactivate the drug. This product is nebulized once or twice daily and stored in the refrigerator. Patients who experience bronchospasms may benefit from inhaled bronchodilators like albuterol at doses equivalent to what is used to treat asthma. Cromolyn sodium and ipratropium are sometimes used as alternates. Systemic bronchodilators such as theophylline are considered second-line therapy because of the narrow therapeutic index, side effect profile, drug interactions, and pharmacokinetic monitoring. 7

8 Oral corticosteroids given on an alternate day regimen were historically thought to be an effective treatment for reactive airway disease of CF by reducing lung inflammation with little patient risk. However, one, long-term, 4- year study proved otherwise, reporting only minimal improvements in pulmonary function along with systemic steroid-induced side effects such as growth retardation, cataracts, and glucose intolerance. Inhaled corticosteroids may be a viable option, but long-term research is lacking. CF patients should receive a yearly influenza vaccination to avoid the complications of getting H. influenzae. Nutritional therapy 3,4,6 When pancreatic ducts become filled with thickened mucus, pancreatic function becomes impaired. As a result, pancreatic secretions are low in concentrations of pancreatic enzymes and bicarbonate. Lack of digestive enzymes leads to maldigestion of fats, protein, and starch. Examples of deficient enzymes are trypsin, chymotrypsin, carboxypeptidase, amylase, and lipase. Having low levels of the fat digesting enzyme lipase causes bulky stools and steatorrhea. Supplementing pancreatic enzymes with each meal and snack assists in protein, fat, and starch digestion. Microencapsulated products prevent the enzymes from being destroyed by stomach acid. Products are dosed based on their lipase content. The usual dose is 1000 lipase units per kg of bodyweight per meal. When ingesting snacks, one-half of this dose is administered. These enzymes are to be taken before or with meals. Patients who have trouble swallowing capsules can sprinkle the capsule contents on applesauce, or any other non-alkaline food. They should be swallowed whole and not chewed. Side effects reported with high dosages are diarrhea, hyperuricemia, nausea, cramps, and constipation. Replacing the fat-soluble vitamins A, D, E, K and the nutrients B12 and zinc are recommended. CONCLUSION Cystic fibrosis is an inherited fatal disease that affects many organ systems. Using pharmacological therapies to treat and prevent respiratory infections, thinning and liquefying excess mucus secretions, enhancing pulmonary function, and effectively supporting digestion may all help reduce the morbidity and mortality of this disease. Patients and caregivers should consult the Cystic Fibrosis Foundation, for current research on gene therapy, clinical trials, and innovative drug therapies. REFERENCES 1. Hill, A., Gompertz, S., and Stockley, R. Thorax, 55:970 (2000) 2. Ferguson, G., Chest, 117:23S (2000) 3. Singh, J.M., Palda, V.A., Stanbrook, M.B., and Chapman, K.R., Arch Intern Med., 162:2527 (2002) 4. Hurd, S., Chest, 117:1S (2000) 5. McKenry, L.M., and Salerno, E., Mosby s Pharmacology in Nursing, 19 th Ed., Mosby, St. Louis, 737 (1995). 6. Lehne, R.A., Pharmacology for Nursing Care, 2 nd Ed., W.B.Saunders, Philadelphia, 819 (1994). Jan: Pharmacy Law Update 2003 Mar: COPD May: Lipid Management Jul: Hepatitis Sep: Psoriasis TOPICS FOR 2003 Feb: Osteoporosis Apr: Cystic Fibrosis Jun: Acne Aug: Ulcer Management Oct: Essential Hypertension Nov/Dec: Anxiety MISSING ANY LESSONS? GO TO OUR WEBSITE ( & DOWNLOAD THEM. WE RE EXPLORING TOPICS FOR NEXT YEAR. PLEASE SEND US YOUR INPUT VIA (info@wfprofessional.com), FAX ( ), OR CONVENTIONAL MAIL: W-F PROFESSIONAL ASSOCIATES, INC. / 400 LAKE COOK ROAD, SUITE 207 / DEERFIELD, IL

9 Fill in the information below, answer questions and return Quiz Only for certification of participation to: CE PRN, 400 Lake Cook Road, Suite 207, Deerfield, IL NAME (1st line on label) ADDRESS CITY STATE ZIP CHECK IF NEW ADDRESS COMPANY WHERE EMPLOYED LESSON EVALUATION Please fill-out this section as a means of evaluating this lesson. The information will aid us in improving future efforts. Please rate each of the following from 1 to 7. Circle your choices. (1 is the lowest rating; 7 is the highest). Poor Average Excellent 1. Relevance of topic to practice Author s ability to communicate Author s knowledge of topic Appropriateness of topic Do you have any further comments about this lesson? Please Select the Most Correct Answer 1. What is the percent chance of having a 6.Which of these antimicrobial agents could child with CF for 2 parents who are both be used to treat P. aeruginosa lung infection? carriers of the CF gene, but do not A. Nafcillin have CF? B. Tobramycin + ceftazidime A. 25% C. Amoxicillin - clavulanate B. 50% D. Vancomycin C. 75% 7.Which of the following microbes do 80% D. 100% of CF patients become chronically 2. Which of the following tests is ill with? diagnostic for CF? A. Staphylococcus aereus A. Chest X-ray of lungs B. Hemophilus influenza B. Stool analysis C. Burkholderia cepacia C. Sweat chloride test >/=60meq/L D. Pseudomonas aeruginosa D. Fetal ultrasound 8.Which bacterium is not commonly 3. Which statement is true? associated with CF? A. Dornase alfa breaks apart DNA A. Staphylococcus aereus released from white blood cells B. Hemophilus influenza B. Dornase alfa may be diluted or mixed C. Pseudomonas aeruginosa with other aerosolized medications D. Streptococcus pneumoniae C. Dornase alfa should be stored at 9.What dosage (approx) of pancreatic enzymes room temperature based on lipase units should a 28 pound child D. Dornase alfa is a bronchodilator be taking with meals? 4. CF is most prevalent among African A. 8,000 lipase units Americans, Native Americans, and B. 9,000 lipase units Asian Americans. C. 12,000 lipase units A. True B. False D. 28,000 lipase units 5. Which statement is true? 10.Approximately how many children A. CF is a genetically inherited are born each year with CF in dominant gene the United States? B. The average lifespan of a CF A. 2,500 patient is 20 years of age B. 5,000 C. Up to 95% of males with CF are sterile C. 7,500 D. Burkholderia cepacia is sensitive D. 10,000 to aminoglycoside therapy E. None of these 9

10 Contributing Author Alison Lapinski, RPh, PharmDNaturally Speaking SeminarsPharmaceutical Educational ConsultantChicago, IL Executive Editor William J. Feinberg, BS Pharm, MBA CE PRN is a publication of W-F Professional Associates, Inc. This program is in printed format. W-F Professional Associates, Inc. is approved by the American Council on Pharmaceutical Education (ACPE) as a provider of continuing pharmaceutical education. Providers who are approved by ACPE are recognized by the following states: Alaska, Arizona, Arkansas, California, Connecticut, Delaware, District of Columbia, Florida, Georgia, Hawaii, Idaho, Illinois, Indiana, Iowa, Kansas, Kentucky, Louisiana, Maine, Maryland, Massachusetts, Michigan, Minnesota, Mississippi, Missouri, Montana, Nebraska, Nevada, New Hampshire, New Jersey, New Mexico, New York, North Carolina, North Dakota, Ohio, Oregon, Pennsylvania, Puerto Rico, Rhode Island, South Carolina, South Dakota, Tennessee, Texas, Utah, Vermont, Virginia, Washington, West Virginia, Wisconsin and Wyoming. Pharmacists completing this course by April 30, 2006 may receive full credit. This program has been approved by the State Boards of Pharmacy in Alabama and Oklahoma. This lesson furnishes 1.25 hours (0.125 CEUs) of credit. Program ID # H01.