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Care of the adult patient with cystic fibrosis.

Cystic fibrosis (CF) is an inherited, autosomal-recessive disease that disrupts ion transport in epithelial-lined organs. The CF gene was first discovered in 1989 and mapped to a single locus on the long arm of chromosome seven. The protein product of the CF gene has been identified as the cystic fibrosis transmembrane conductance regulator (CFTR). CFTR is involved in the transport of chloride and sodium ions across the membranes of cells in the epithelial lining of the ducts of exocrine glands (sweat glands, pancreas, liver, and reproductive systems). When the CFTR malfunctions in the patient with CF, the membrane is unable to transport electrolytes normally, leading to impaired water secretion, salty sweat, and abnormally thick exocrine secretions (Orenstein, Rosenstein, & Stern, 2000). More than 1,000 mutations have been identified on the CFTR gene, creating the innumerable variations in the clinical progression seen in this disease. Probably the most common mutation cited in research is [DELTA] F508, which accounts for 66% of the CF mutated alleles worldwide (Yankaskas & Knowles, 1999). This classic CF clinical phenotype is associated with severe pancreatic insufficiency, obstructive azoospermia, and a variety of pulmonary manifestations (Davis, Drumm, & Konstan, 1996).

Once considered to be only a disease of childhood, CF is now also a disease of adults. Before 1950, the gastrointestinal, nutritional, and pulmonary complications of CF resulted in a median survival of 1 year or less (Yankaskas & Knowles, 1999). Due to a number of basic scientific discoveries and improvements in clinical care, the median survival of CF patients in 1999 rose to 29.1 years (Cystic Fibrosis Foundation Patient Registry, 1999). Adults over 18 years of age now account for more than 37% of patients with CF, and survival past the age of 76 years has been documented (Yankaskas, 2001). CF is one of the most common genetic diseases in Caucasians, with an incidence ranging from 1 in 1,900 to 1 in 3,700 in the United States (Hamosh et al., 1998; Welsh, Ramsey, Accurso, & Cutting, 2001). CF is less frequently found in Hispanic, Asian, and African-American populations (Gibson, Burns, & Ramsey, 2003).

CF is a multi-system genetic disease with a highly variable presentation and course that require comprehensive, multidisciplinary care. Some individuals with CF have severe pulmonary and/or gastrointestinal disease, while others have relatively mild disease that presents during adulthood. Outcomes range from early death as a result of pulmonary complications to mild atypical disease diagnosed in the 2nd and 3rd decades of life (National Institutes of Health [NIH], 1997).

Diagnosing Cystic Fibrosis

In the United States, the majority (71%) of patients with CF are diagnosed by 1 year of age; however, in 8% of the patients, the diagnosis is not made until 10 years of age (Rosenstein & Cutting, 1998). CF now is being diagnosed more frequently in adolescence and adulthood. The older patient tends to have fewer complications, live longer, and have fewer hospitalizations than the individual who was diagnosed as a child (Widerman, Millner, Sexauer, & Fiel, 2000). Confirming a diagnosis early in the course of the disease avoids unnecessary diagnostic procedures, provides an opportunity for genetic testing and counseling, and ensures access to specialized medical services (Rosenstein & Cutting, 1998).

The diagnosis of CF is suspected in a patient who has typical clinical features of the disease, including chronic sinopulmonary manifestations and exocrine pancreatic insufficiency (see Table 1). Most postpubertal males with CF also have obstructive azoospermia, which is sterility due to a blockage or incomplete formation of the vas deferens. The diagnosis of CF typically is confirmed by an elevated sweat chloride concentration (>60 mmol/L) on two separate occasions. In cases where sweat chloride tests are borderline or normal, or in the asymptomatic patient who is at increased risk (for example, an individual with an affected sibling or half-sibling), the diagnosis can be made by identification of CF mutations in each CFTR gene or the in vivo demonstration of abnormal ion transport across nasal epithelium (Rosenstein & Cutting, 1998). In this highly specialized diagnostic test, subcutaneous skin bridges are placed in exact locations within the nasal mucosa, enabling electrical activity to be measured with a high impedance voltmeter. Laboratories performing this test must ensure that equipment is rigorously validated and the protocol for measurement is standardized so the results are reproducible across facilities (Orenstein et al., 2000).

The only acceptable procedure for sweat testing is the quantitative pilocarpine iontophoresis sweat test (Rosenstein & Cutting, 1998). In this test, sweat production is increased by the use of a small electric current carrying the drug pilocarpine. The sweat is collected and analyzed to determine sodium and chloride content; higher concentrations (>60 mmol/L) are consistent with a CF diagnosis. The patient should be told that electrodes are placed on the forearm, thigh, and the back to induce sweat production. Although the procedure does not cause pain, a tingling sensation may be experienced under the electrodes (Daniels, 2003).

Nasal and respiratory epithelia regulate the composition of fluids that surround the respiratory tract by the transport of sodium and chloride ions. The active transport of these ions generates a transepithelial electrical potential difference (PD) which can be measured. The person with CF has a characteristic pattern of abnormal ion transport in the epithelia that may be used to diagnose the disease with relative certainty. As with sweat testing, a raised PD must be duplicated on more than one occasion to confirm the diagnosis (Rosenstein & Cutting, 1998).

Two known CF mutations must be identified in the CFTR gene for the diagnosis of CF to be confirmed by mutation analysis. The presence of mutations known to cause CF in each CFTR gene makes the diagnosis certain; due to the large number of CF alleles, however, an inability to detect mutations does not mean that the patient does not have the disease. Mutation analysis may also provide genetic information for the patient and family interested in making decisions about future pregnancies (Rosenstein & Cutting, 1998).

Genetic Considerations

In an autosomal genetic disorder, the gene associated with the disease is not sex linked; both males and females can have the disorder. In a recessive genetic disorder, the normal gene is dominant and the defective gene is recessive, which means that a heterozygous person (with both a defective gene and a normal gene) will not have the disorder but can be a carrier and pass it to his/her children. A homozygous person (with two defective genes passed from the parents) will have the disorder. If both parents are carriers, the risk of having a child with CF is 1 in 4 (25%), regardless of what the outcome of other pregnancies has been (Orenstein, 1997).

According to the NIH Consensus Conference Statement (1997), genetic testing should be offered to adults with a positive family history of CF and to partners of people with CF planning a pregnancy or seeking prenatal testing. The statement does not currently recommend CF genetic testing for the general population or universal screening of newborn infants, although this may become the practice of the future. Genetic counseling services and educational programs should help medical personnel and the public prevent discrimination and stigmatization against patients with CF and their offspring.

Pulmonary Characteristics of CF

Pulmonary disease is the cause of death in more than 95% of patients with CF (Yankaskas, 2001). Impaired airway mucus clearance is the primary cause of the chronic respiratory inflammation and infection seen in these patients. Once an infection is established, it is rarely eradicated; antimicrobial therapy is used to decrease bacterial load, bacterial virulence, and airway inflammation. Airway wall thickening, persistent neutrophilic infiltration, and plugging of the small and large airways with mucus, inflammatory cells, and bacteria lead to dilatation of the airways and enlarged bronchial arteries (Yankaskas & Knowles, 1999). Symptoms of lung disease progress slowly but tend to worsen during exacerbations of airway infection and inflammation. Pulmonary function, particularly forced expiratory volume (FE[V.sub.1]), or the volume of air that can be exhaled in 1 second, deteriorates as lung function declines; exercise tolerance decreases, and exacerbations occur with more frequency. Other late complications include pneumothorax and varying degrees of hemoptysis (Yankaskas, 2001).

Pulmonary Treatment

The treatment of respiratory infection should be based on the identification and susceptibility patterns of all significant lower respiratory tract pathogens. Because sequential cultures often isolate different bacteria, respiratory cultures should be obtained quarterly and during each exacerbation in patients with CF (Yankaskas, 2001). Sputum cultures should be marked as coming from a patient with CF and transported to the lab within 3 hours. If the patient is unable to produce sputum, oropharyngeal swabs or a bronchoalveolar lavage may be used to obtain a specimen (CF Foundation, 1994).

The most commonly isolated organisms in patients with CF are Staphylococcus aureus and Haemophilus influenzae. With advancing age, the mucoid variant of Pseudomonas aerugnosa is often dominant (Saiman & Siegel, 2003). Pseudomonas aeruginosa may be present in several different colonies in the sputum with different antibiotic sensitivity patterns. Other gram-negative rods that may be found in sputum of CF patients include Burkholderia cepacia, Alcaligenes xylosoxidans, Stenotrophomonas maltophilia, and methicillin-resistant Staphylococcus aureus (MRSA) (Beringer & Appleman, 2000).

Patients with cystic fibrosis are treated with antibiotics during an acute pulmonary exacerbation and often with chronic suppressive therapy during remissions. Cystic fibrosis alters the pharmacokinetics of many antibiotics, and patients often require higher doses of B-lactams, aminoglycosides, and sulfa drugs (Yankaskas, 2001). Oral drugs are commonly used for suppressive therapy; however, the emergence of bacterial resistance requires parenteral administration during respiratory exacerbations (see Table 2). Two antibiotics with different mechanisms of action that have efficacy against CF pathogens are used to reduce the risk of inducing resistance. Synergy testing offers a way to test the efficacy of combination antibiotics in patients who have multi-resistant organisms in their sputum (Yankaskas, Marshall, Sufian, Simon, & Rodman, 1999). Two-drug antibiotic synergy testing of multiple-resistant isolates may be obtained through a few reference laboratories in the United States.

A clinical response is often not seen for 4 to 7 days after antibiotics are started, and treatment is often needed for 10 to 14 days or longer (CF Foundation, 1994). Although oral drugs are sometimes used for suppressive therapy, the emergence of bacterial resistance requires parenteral administration during respiratory exacerbations. Multiple routes may be used at the same time to enhance antimicrobial eradication. For example, tobramycin (Tobi[R]) may be given by inhalation to achieve the desired concentration in large airways and intravenously to reach peripheral air ways that are blocked by secretions (Yankaskas, 2001). Aerosol administration of antibiotics is frequently used in CF because drugs can be directly delivered to the site of infection, enabling smaller doses to be given and thus reducing systemic toxicity (Yankaskas, 2001). Serum peak and trough levels are monitored carefully in patients with CF to ensure optimum serum concentrations of aminoglycosides and to help prevent toxicities.

Airway clearance is an essential component of treatment for all patients with CF. Airway secretions obstruct airflow and impair gas exchange, and contain a number of mediators that contribute to the inflammatory process. Chest percussion with postural drainage, percussion/vibration techniques, breathing exercises, and directed cough techniques have been the mainstay of treatments for decades (Yankaskas & Knowles, 1999). As adjuncts to standard therapies, the following are also used: the flutter valve, autogenic drainage (AD), forced expiration technique positive expiratory pressure mask (PEP), and high-frequency chest compression (vest) (see Table 3). These therapies are used most commonly in combination, depending on individual patient benefit and preference. The newer techniques do not require the assistance of another person, a distinct advantage for adult patients who wish to live alone or remain independent.

Other Therapies

Bronchodilators, especially beta-adrenergic agonists and anti-cholinergics, are used to treat airway hyperactivity and to reverse bronchospasm. Aerosolized solutions such as normal saline, hypertonic saline (3%-12%), or oral mucolytic agents may be used to add liquid to the airways and hydrate secretions. DNA is present in large concentrations in the sputum of patients with CF due to persistent neutrophil influx into the airways as a component of the chronic inflammatory response. Recombinant human Dnase (rhDNase/Pulmozyme[R]) reduces viscosity of secretions and enhances airway clearance by lysing the extracellular DNA. Although this drug is usually most effective when administered once daily, some patients may require less or more frequent dosing (Orenstein et al., 2000). Hoarseness, voice alterations, and pharyngitis are the major adverse events reported with Dnase; however, these effects are usually self-limiting and do not generally require cessation of the drug (Yankaskas et al., 1999). Other aerosolized therapies are aimed at correcting the fluid and electrolyte imbalance on the mucosal surface of the airway.

The anti-inflammatory drug ibuprofen (Motrin[R]) can reduce the rate of deterioration in CF respiratory disease in younger patients with mild disease (Yankaskas, 2001). Routine use of corticosteroids in the CF population is not recommended because of unacceptable long-term and short-term side effects; however, they are often used in late-stage disease or during severe respiratory exacerbations to reduce inflammatory airway edema (Yankaskas et al., 1999). Chronic use of alternate-day systemic steroids appears to slow the decline in lung function over time but causes significant toxicity, especially growth retardation, hyperglycemia, and cataract formation (Gibson et al., 2003). Inhaled steroids have the potential to reduce inflammation without significant systemic adverse effects and are widely used in CF, although their efficacy has not been substantiated. CF patients with bronchial hyperresponsiveness may obtain some benefit from inhaled steroids (Gibson et al., 2003).

Infection Control

In 1999, a multidisciplinary committee with expertise in CF reviewed the relevant literature and developed evidence-based infection control recommendations for patients with CF (Saiman & Siegel, 2003). These guidelines incorporated previously published Healthcare Infection Control Practices Advisory Committee/ Centers for Disease Control guidelines for preventing health care-associated infections and include recommendations developed by the committee that are specific to patients with CE They are summarized below.

Standard precautions must be applied to all patients. In addition, transmission-based precautions (contact, droplet, or airborne) must be applied to all patients with documented or suspected infection with highly transmissible or epidiologically infectious bacteria (for example, B. cepacia complex, multidrug-resistant P aeruginosa, MRSA, or Mycobacteriuim tuberculosis). Bacterial pathogens in CF are spread by direct contact, indirect contact, or droplet infection, and health care workers should assume that all patients with CF could have transmissible pathogens in their respiratory secretions (Saiman & Seigel, 2003).

When hands are visibly dirty or soiled with blood, body fluids, or respiratory secretions, they should be washed with an antimicrobial soap and water. Alcohol-based antiseptic hand rubs are now recommended when hands are not visibly soiled with blood or body fluids (Saiman & Seigel, 2003). Hand hygiene should be performed after removing gloves, and before and after contact with any patient (CF or non-CF). Health care workers should wear gloves when caring for patients who require contact precautions and when handling respiratory secretions or objects contaminated with respiratory secretions. Gowns should be worn when caring for patients who require contact precautions or when soiling with respiratory secretions is anticipated (for example, during chest physiotherapy or suctioning). Mask and eye protection or a face shield should be worn when splashes or sprays of secretions, body fluids, blood, or excretions are anticipated. All patients with CF who are infected or colonized with B. cepacia complex, MRSA, or vancomycin-resistant enterococcus (VRE) should be placed in private rooms that do not share common facilities (such as bathroom or shower). Other patients with CF may share rooms with patients without CF who are at low risk for infection (Saiman & Siegel, 2003).

Sources of transmission that have been implicated in hospital outbreaks of CF-related pathogens include contaminated respiratory therapy equipment, multi-dose vials, home nebulizers, and tap water (Saiman, 2001). General guidelines to prevent transmission of these organisms include placing the patient with CF in a private room, rinsing respiratory therapy equipment in sterile water after disinfecting, assuring that all respiratory therapy occurs inside a patient's room, asking the patient to bring personal airway clearance devices from home, and using single-dose vials for aerosol medications.

Families, visitors, and patients should be taught to wash their hands correctly before leaving the patient's room. The patient should contain respiratory secretions in a tissue when coughing to prevent possible contamination of exposed surfaces. The patient with CF needs to know the possible risks of close contact with other CF patients in nonhospitalized settings. Behaviors that increase risk include physical intimacy, assisting with another CF patient's respiratory treatment, sharing personal items, being in a hot tub or whirlpool together, and sharing poorly ventilated areas (for example, bus or airplane). The patient with CF should also be warned to avoid areas where building construction or renovation is occurring due to high aspergillus exposure (Saiman, 2001).

Cystic Fibrosis-Related Diabetes

A frequent complication of CF is glucose intolerance and cystic fibrosis-related diabetes (CFRD), which occurs in approximately 20% to 30% of patients over the age of 20. CFRD is rarely found in children, but occurs more frequently with advancing age 0Rodman, 2001). The diagnosis of CFRD is associated with worse pulmonary disease, earlier death, and poorer nutritional status (CF Foundation, 1999a). CFRD shares some characteristics of both Type 1 and Type 2 diabetes, but it has recently been recognized as a separate disease because of features that are unique to the CF population. Although the primary cause of CFRD is insulin deficiency, glucose metabolism is also impaired 0Rodman, 2001).

Four different glucose tolerance categories are currently recognized in CF (see Table 4) (CF Foundation, 1999a). In CFRD with hyperglycemia, both the fasting glucose and the postprandial blood sugar are high. CFRD with hyperglycemia is treated aggressively with insulin and nutritional therapy, although the principles of treatment differ from those of either Type 1 or Type 2 diabetes (Yankaskas et al., 1999). In CFRD without hyperglycemia, only the postprandial blood sugar is elevated; this form of diabetes is considered a mild disease and generally not treated unless symptoms are present. The patient with CFRD without hyperglycemia should he told insulin is needed during periods of illness or treatment with corticosteroids, and in the future if fasting hyperglycemia develops (Hardin, Brunzell, Schissel, Schindler, & Moran, 1999).

Cystic fibrosis-related diabetes has an insidious onset and a characteristic pattern of intermittent hyperglycemia between periods of normoglycemia. Although ketoacidosis is rarely seen in CFRD, the microvascular complications of Type 1 and Type 2 diabetes are frequently seen, and screening is similar to that of Type 1 and Type 2 diabetes (CF Foundation, 1999a). Interestingly, the macrovascular complications of Type 1 and Type 2 diabetes have not been found in patients with CFRD. Symptoms often occur late in the disease process and include polydipsia, polyuria, weight loss, an inability to gain weight despite aggressive nutritional intervention, poor growth, delayed puberty, and unexplained pulmonary decline (CF Foundation, 1999a).

Therapy goals in CFRD are similar to those in Type 1 and in Type 2 diabetes with some important exceptions (Yankaskas et al., 1999). Oral agents are generally ineffective in CFRD, and most patients require insulin to manage their diabetes. Adult CF patients with diabetes require sufficient calories to ensure normal weight and near-normalization of blood glucose levels for optimal nutritional and metabolic status. A high-fat, high-protein, high-calorie diet is recommended. Because postprandial hyperglycemia is often a prominent feature of CFRD, patients are taught to count carbohydrate units and to cover the counted carbohydrates with a very short-acting insulin prior to each meal and snack. A typical regimen also includes a small amount of long-acting insulin at night or in the morning. Carbohydrate counting allows patients with CF more flexibility to eat all favorite foods, which can be included in the meal plan by determining the grams of carbohydrate per serving (Yankaskas et al., 1999). As with any intensive insulin therapy, this approach requires frequent self-monitoring of blood glucose levels.

Nutritional Recommendations

Salt is never restricted in the meal plan of CF patients; instead, patients should be reminded to increase salt and fluid intake during hot, humid weather and during exercise. Due to the increased work of breathing and shortness of breath that occur during exacerbations, the patient may complain of anorexia, nausea, or early satiety. Meeting nutritional needs at this time becomes an important goal; generally the patient is told to eat whatever he or she likes at any time, and to add nutritional supplements whenever possible. The patient who is unable to meet nutritional needs despite aggressive interventions may need short-term or long-term supplemental enteral or parenteral nutrition. It is the authors' experience that the patient with long-term needs often decides to have a percutaneous gastrostomy tube placed and to have continuous, cyclic tube feedings during the night.

Gastrointestinal Problems

Over 90% of patients with CF have pancreatic insufficiency, primarily due to pancreatic fluid abnormalities and a deficiency in bicarbonate secretion (Yankaskas et al., 1999). Clinical manifestations of pancreatic insufficiency include multiple bowel movements each day of bulky, greasy-appearing stools that float. The patient also has increased flatulence and an inability to gain or maintain weight despite adequate caloric consumption. Treatment consists of taking micro-encapsulated pancreatic enzyme with food to aid absorption. The patient should be reminded to take capsules at the beginning of or during meals (not after the meal is done) and with all snacks. Side effects of pancreatic enzyme supplementation are rare, although constipation may occur. If this becomes a problem, the patient should be encouraged to increase fiber and fluid intake but not to decrease medication dosage or change the dosing schedule. Cost may become a factor because pancreatic enzymes are costly (about $1.25/capsule) (Simon, 2001).

The patient with pancreatic insufficiency will have difficulty absorbing fat-soluble vitamins and should receive daily supplementation of vitamins A, D, E, and K as well as a daily multivitamin (Orenstein et al., 2000). The patient with CF is also at higher risk of developing pancreatitis; nearly 10% of patients with pancreatic insufficiency are affected (Simon, 2001). Acute symptoms include severe epigastric or upper abdominal pain, nausea, and vomiting, and signs such as tachycardia, fever, and abdominal distention or guarding. Diagnosis and treatment of pancreatitis does not differ for the patient with CF (Simon, 2001).

Gastroesophageal reflux disease (GERD) occurs more frequently in patients with CF (Yankaskas et al., 1999). The head-down position during respiratory treatments, the effects of certain respiratory medications (theophylline and alphaadrenergic agents), and the coughing or forceful expirations that occur after chest physiotherapy may facilitate reflux and worsen GERD. General lifestyle and dietary recommendations to manage GERD include maintaining an upright position for 30 minutes after meals, scheduling postural drainage and other airway clearance techniques before meals, eliminating foods and fluids 2 hours before bedtime, elevating the head of the bed on 8-to-12 inch blocks, and avoiding large meals and tight clothing (DeVault & Castell, 1999).

Distal intestinal obstruction syndrome (DIOS) is a chronic intestinal problem seen in 3.5% of patients with CF (Yankaskas et al., 1999). DIOS, which occurs in patients with pancreatic insufficiency, is related to the abnormal intestinal water and electrolyte transport, prolonged gastrointestinal transit time, and abnormal intestinal mucins seen in CF (CF Foundation, 1991). The patient typically has intermittent occurrences with varying degrees of small bowel obstruction, and may complain of crampy, right lower quadrant pain, distention, anorexia, weight loss, and flatulence. DIOS may occur with a normal stooling pattern, or the patient may report recent constipation. While the occurrence begins as a partial obstruction, it can progress to complete obstruction.

A partial obstruction is treated by giving a balanced polyethylene glycol-electrolyte solution (GoLytely[R]) by mouth or by nasogastric tube. Treatment is successful if there is evidence of the passage of stool, resolution of symptoms, or the disappearance of a previously palpated right lower quadrant mass. Plain abdominal radiographs may also confirm the resolution of DIOS (CF Foundation, 1991). Although this treatment is generally safe, electrolyte or fluid imbalances may occur; at particular risk is the patient who is undernourished, very ill, or has significant co-morbidities. Treatment of complete obstruction generally requires surgical intervention (Yankaskas et al., 1999).

Abnormal CFTR is present in the biliary tree of all patients with CF; however, most patients do not develop clinically significant liver disease (CF Foundation, 1999b). Identifying fibrotic liver disease in CF is difficult because symptoms are minimal during the time that fibrotic liver disease is silently developing. Altered bile composition (increased viscosity and concentration) and decreased bile flow may lead to biliary obstruction, periportal fibrosis, and eventual cirrhosis. One of the most common early presentations of liver disease is found in the asymptomatic adult during annual physical exams and routine screening, when hepatomegaly or splenomegaly is detected or when elevated serum liver enzymes are found. Liver biopsy is helpful in determining if steatosis or focal biliary cirrhosis is the primary abnormality, and it can also determine the extent of portal fibrosis or cirrhosis. Cholestasis, focal biliary cirrhosis, and multilobular cirrhosis are the most common types of CF-related liver disease and part of a sequential progression that occurs over time. Management typically includes medical therapy with ursodeoxycholic, nutritional therapy (replacement of fat-soluble vitamins), and treatment of complications (portal hypertension and liver failure). All patients with CF-related liver disease should also receive a complete immunization series for both hepatitis A and hepatitis B (Yankaskas et al., 1999).

Reproductive Issues

Because of the dramatic increase in the survival of patients with CE reproductive issues have taken on new importance (Tullis, 2001). The nurse shares responsibility with other care providers to ensure that the patient has the knowledge to make informed reproductive decisions. Counseling for both genders should begin early, ideally before puberty. Both men and women need genetic counseling before considering pregnancy to assess their partner's CF genetic status.

Although nearly all men with CF are infertile because of azoospermia, contraceptive use should be suggested until a semen analysis is complete (Tullis, 2001). Sexual functioning and sexual development, although often delayed, are normal in most men with CE Fertility options for men include microscopic epididymal sperm aspirations, in vitro fertilization, and intracytoplasmic sperm injection (Tullis, 2001). Pregnancy rates of 30% to 40% have been reported with these newer techniques (McCallum et al., 2000).

Most women with CF have decreased fertility due to thick, tenacious cervical mucus, which obstructs sperm migration. Menarche may be delayed, and there is an increased incidence of amenorrhea (Tullis, 2001). As more affected women reach childbearing age, the reported number of pregnancies has also increased. The patient who is considering pregnancy needs to know that careful assessment and planning are key, especially with regard to optimizing pulmonary and nutritional status before conception and during pregnancy. Maternal and fetal outcomes depend on maternal health; the absence of B. cepacia (an important CF pathogen that signifies a poorer prognosis), pancreatic sufficiency, and pre-pregnancy FE[V.sub.1] greater than 50% were associated with better maternal and fetal survival rates in one study (Gilljam et al., 2000).

End-of-Life Issues

With increased longevity and improved treatment options, the patient and family are faced with numerous choices and dilemmas regarding end-of-life issues. Factors that complicate their decisions include the scarcity of donor/cadaveric lungs, ethical issues surrounding living donor transplantation, and the real need for the adult patient to make informed decisions regarding advanced directives and DNR status, especially during end-stage disease admissions. The traditional end-of-life model of palliative care that is seen in other diseases such as cancer is often not used in the patient who is terminally ill with CF (Robinson, Ravilly, Berde, & Wohl, 1997). For example, the ill patient may elect to continue intravenous antibiotics or take daily vitamins while receiving an opiate for pain and dyspnea at the end of life. This may be because the short-term outcome of respiratory failure in CF is often unpredictable, and there is a reluctance to stop a potentially effective therapy, even if the long-term outcome is known. The chest pain and dyspnea of death from respiratory failure in CF can usually be effectively managed with small doses of aerosolized or intravenous opiates (Robinson et al., 1997).

The first successful heart-lung transplant surgery was performed in 1983; by 1998, nearly 400 patients with CF had received some form of thoracic transplantation (Yankaskas & Mallory, 1998). The current post-transplant survival rate for end-stage CF patients is nearly 50% (Marshall, 2001). The procedure of choice today is the bilateral lung transplant. As increasing number of patients are being listed for transplantation, the waiting time has lengthened due to a scarcity of donor lungs. A newer surgical approach to transplantation in the CF population is the bilateral sequential transplant of a lower lobe from each of two living donors, which can be related or unrelated. Short-term and intermediate outcomes after living donor procedures appear to be similar to the standard cadaveric approach (Yankaskas & Mallory, 1998).

Conclusion

The growth of the adult CF population is predicted to continue into the near future. Even without future improvements in survival, the number of adults with CF in the United States in the year 2005 will likely exceed 10,000 and represent more than 40% of the total CF population (Yankaskas et al., 1999). Adult patients with CF and their families have significant physical, psychosocial, and educational needs that require comprehensive multidisciplinary care. Ideally, this care is provided in a CF center specializing in the care of adults. As key members of the health care team, adult-health nurses must be aware of the most current thinking regarding the diagnosis and optimum evidence-based treatment of CE As direct care providers and coordinators of care, they are in the position to provide holistic, individualized nursing care that can significantly improve quality of life for patients with CF.
Table 1.

Phenotypic Features Consistent with a Diagnosis of CF

Chronic sinopulmonary disease characterized by:

* Persistent colonization/infection with typical CF pathogens (S.
  aureus, nontypeable H. influenzae, mucoid and nonmucoid P. aeruginosa
  and B. cepacia)

* Chronic cough and sputum production

* Persistent chest radiograph abnormalities (bronchiectasis,
  atelectasis, infiltrates, hyperinflation)

* Airway obstruction manifested by wheezing and air trapping

* Nasal polyps; X-ray or CT abnormalities of the paranasal sinuses

* Digital clubbing

Gastrointestinal and nutritional abnormalities

* Intestinal: meconium ileus, distal intestinal obstruction syndrome,
  rectal prolapse

* Pancreatic: pancreatic insufficiency, recurrent pancreatitis

* Hepatic: chronic hepatic disease including focal biliary cirrhosis or
  multilobular cirrhosis

* Nutritional: failure to thrive, hypoproteinemia and edema, fat-
  soluble vitamin deficiency

Salt loss syndromes: acute salt depletion, chronic metabolic alkalosis

Male urogenital abnormalities resulting in obstructive azoospermia

Adapted from Rosenstein & Cutting (1998).

Table 2.
Typical Parenteral Antibiotic for Respiratory Exacerbations]

Bacteria in Sputum                  Drug                    Dose (mg)

S. aureus and H.      Mehicillin                          200
influenzae            and
                      Tobramycin (Nebcin [R])             10 mg/kg/day

S. aeureus            Cefazolin (Ancef [R])               1,000

S. aeureus            Vancomycin (Vancocin [R])           1,000
(MRSA)

S. aureus and P.      Tobramycin (Nebcin [R])             10 mg/kg/day
aeruginosa            and
                      Ceftazidime (Fortaz [R]) or         2,000
                      Ticarcillin-
                      clavulanate (Timentin [R]) or       3,000
                      Imipenem-
                      cislastatin (Primaxin [R]) or       500-1,000
                      Peperacillin (Zosyn [R]) or         4,000
                      Azetreonam (Azactam [R])            2,000

P. aeruginosa         Tobramycin                          As above
                      and B-lactam (above)                As above
                      or
                      Ciprofloxacin (Cipro [R])           400

B. cepacia            TMP/SMX (Septra [R]) and            5mg/kg
                      Chloramphenicol                     (TMP)
                      (Chloromycetin [R])                 15 mg/kg

         Drug                          Dose
                                     Interval/
                                       Hours

Mehicillin                           6
and
Tobramycin (Nebcin [R])              8 or 12

Cefazolin (Ancef [R])                12

Vancomycin (Vancocin [R])            12

Tobramycin (Nebcin [R])              8 or 12
and
Ceftazidime (Fortaz [R]) or          8
Ticarcillin-
clavulanate (Timentin [R]) or        6
Imipenem-
cislastatin (Primaxin [R]) or        6
Peperacillin (Zosyn [R]) or          6
Azetreonam (Azactam [R])             8

Tobramycin                           As above
and B-lactam (above)                 As above
or
Ciprofloxacin (Cipro [R])            12

TMP/SMX (Septra [R]) and             6
Chloramphenicol
(Chloromycetin [R])                  6

                                     Guidelines for
              Drug                   Administration/Toxicity

Mehicillin                           Dilute to < 20 mg/ml for infusion
and
Tobramycin (Nebcin [R])              Titrate to peak serum 10-12 mEq/ml
                                     Potential for ototoxicity/
                                     nephrotoxicity
Cefazolin (Ancef [R])

Vancomycin (Vancocin [R])            Infuse over 90 minutes to minimize
                                     "red-man" reaction
                                     Potential for nephrotoxicity/
                                     ototoxicity

Tobramycin (Nebcin [R])              Once daily dosing may increase
and                                  toxicity
Ceftazidime (Fortaz [R]) or          Infuse over 5 minutes
Ticarcillin-
clavulanate (Timentin [R]) or        Platelet dysfunction possible
Imipenem-
cislastatin (Primaxin [R]) or        Doses > 4 gm cause nausea/vomiting
Peperacillin (Zosyn [R]) or
Azetreonam (Azactam [R])             Major indication in penicillin
                                     allergy

Tobramycin                           As above
and B-lactam (above)                 As above
or
Ciprofloxacin (Cipro [R])            Interferes with theophylline
                                     metabolism

TMP/SMX (Septra [R]) and             Potential for nephrotoxicity/
Chloramphenicol                      hepatotoxicity
(Chloromycetin [R])                  Neutropenia, photosensitivity
                                     Monitor serum levels, can cause
                                     leukopenia

Table 3.
Newer Airway Clearance Techniques

Positive expiratory             Series of breaths through a fixed
pressure (PEP)                  orifice to provide backpressures which
                                help maintain open airways and promote
                                collateral ventilation; this exercise
                                is commonly followed by huffing and
                                coughing.

Active cycle of breathing       Uses low and high-volume huffs inter-
techniques (ACTS)               spersed with breathing control. May be
                                done in postural drainage positions.

Autogenic drainage (AD)         Method of controlled breathing in which
                                volume is varied to mobilize secretions
                                from all generations of airways.
                                May also be done passively by varying
                                manually applied extrathoracic
                                pressure.

Oscillating PEP                 PEP pressures oscillate at high
                                frequency to promote vibration of
                                airway walls, which loosens secretions;
                                examples include flutter valve,
                                acapella valve.

High frequency chest            Applied by a vest through which
compression (HFCC)              oscillating airflow creates vibration
                                of the chest wall at varying
                                frequencies and intensities; for
                                example, Therivest [R].

Intrapulmonary percussive       Creates crusts of airflow via a
ventilator (IPV)                mouthpiece at high frequencies to
                                provide a percussive effect within the
                                airways.

Adapted from Lapin & Lapin (20031.

Table 4.
Oral Glucose Tolerance Categories in Cystic Fibrosis

                                                         2-Hour
                                     Fasting          Postprandial
Category                              mg/dL              mg/dL

Normal glucose tolerance              <126                140
(NGT)

Impaired glucose tolerance            <126              140-199
(IGT)

CFRD without fasting                  <126                -200
hyperglycemia

CFRD with fasting                 [> or =] 126        Oral glucose
hyperglycemia                                        tolerance test
                                                     not necessary

Adapted from Cystic Fibrosis Foundation (1999a).


References

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Cystic Fibrosis Foundation Patient Registry. (1999). Annual report (September. 2000). Bethesda, MD: Author.

Cystic Fibrosis Foundation. (1991). Consensus conference: Gastrointestinal problems in CF. In Primer for adult-trained physicians and caregivers to develop special expertise in CE Symposium conducted at the Fifteenth Annual North American Cystic Fibrosis Conference, Orlando, FL.

Cystic Fibrosis Foundation. (1994). Microbiology and infectious disease in cystic fibrosis: Consensus conference. In Primer for adult-trained physicians and caregiver to develop special expertise in CF. Symposium conducted at the Fifteenth Annual North American Cystic Fibrosis Conference, Orlando, FL.

Cystic Fibrosis Foundation. (1999a). Consensus document: Diagnosis, screening and management of cystic fibrosis related diabetes mellitus. In Primer for adult-trained physicians and caregiver to develop special expertise in CF. Symposium conducted at the Fifteenth Annual North American Cystic Fibrosis Conference, Orlando, FL.

Cystic Fibrosis Foundation. (1999b). Consensus document: Recommendations for management of liver and biliary tract disease in cystic fibrosis. In Primer for adult-trained physicians and caregiver to Develop special expertise in CF. Symposium conducted at the Fifteenth Annual North American Cystic Fibrosis Conference, Orlando, FL.

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Case Study

Anita is a 38-year-old woman with end-stage CF. She was admitted to a respiratory unit that specializes in the care of adult CF patients with a 1-day history of shortness of breath and stated that she was "unable to catch her breath." She had a worsening cough that was productive of green-colored sputum. Anita was last discharged only 3 days previously after treatment for an episode of acute-over-chronic respiratory failure, hypercarbia, hypoxemia, and P. aeruginosa multi-resistant bronchiectasis. Anita has a history of cystic fibrosis-related diabetes (CFRD) and pancreatic insufficiency. She has been on the national donor transplant list for 2 years awaiting cadaveric donor lungs.

On admission, Anita was alert and oriented, but acutely short of breath and anxious. She had coarse anterior and bibasilar crackles upon lung auscultation, but no expiratory wheezing. Anita was placed on a 100% [O.sub.2] nonrebreather mask in the emergency room because of arterial blood gases that demonstrated severe respiratory acidosis, hypercapnia, and hypoxemia (on 100% [O.sup.2]: pH=7.25, PaO2= 125, PCO2>100, HCO3=55, O2 sat: 96%). Upon her arrival to the unit, vital signs were: BP=166/68, P= 130, RR= 26, temp= 99.8. She had trace pedal edema bilaterally and +4 digital clubbing, although there were no signs of hepatosplenomegaly. Laboratory results from the emergency room showed that all electrolytes were within normal range except the blood sugar, which was elevated (280 mg/dL). Anita's white blood count was also elevated at 15,500/[mm.sup.3].

A standard physician's order sheet for CF patients is used on the unit to prompt physicians and other caregivers to request the appropriate referrals, treatments, and medications that are included in the CF standards of care. Standard admission labs include CBC with differential; chem 7; sedimentation rate; urinalysis; vitamin A, D, E, and K levels; and nutritional labs. Supplemental oxygen is ordered if patient is hypoxic. If stable, all patients are scheduled for a chest x-ray and pulmonary function tests upon admission. A sputum specimen is sent for gram stain, culture and sensitivities, and a fungal culture to rule out aspergillus. A thorough physical assessment occurs on admission, with special attention to the respiratory and gastrointestinal systems. An admission weight is obtained and recorded, then daily weights are done on all CF patients while hospitalized. A dietician who is knowledgeable about the nutritional needs of CF patients moniters each patient closely. During the admission process, nurses are careful to ask each patient about respiratory, gastrointestinal, and nutrition changes since the last admission. All patients with CF are placed in private rooms, and guidelines as described by the CDC/HICPHC and the CF Foundation are implemented.

Anita, like many patients with advanced respiratory disease, developed bronchiectasis and hypoxemic, hypercapnic respiratory failure late in the disease process. Anita also had several chronic pulmonary phenotype features that are consistent with the disease. These included persistent colonization with a typical CF pathogen (multi-drug resistant P. aeruginosa), a chronic cough with sputum production, and digital clubbing. Her thoracic CT showed bronchiectasis, a mucus plug in the right upper lobe, complete collapse in right upper and right middle lobes, and pretracheal and subcarinal adenopathy; these findings were consistent with end-stage CE. On admission she was acutely short of breath, hypoxic, and tachypneic, and had an increase in her characteristic cough and sputum patterns typical of a respiratory exacerbation. Her admitting pulmonary function tests were also representative of an acute respiratory exacerbation and pending respiratory failure. Her FE[V.sub.1] of 0.37 liters of oxygen was only 15% of what is predicted for a healthy woman of the same age, height, and weight as Anita.

She was placed on nasal bilevel positive pressure (BiPAP) 15/5 (4 liters of [O.sub.2]) (15 : inspiratory positive airway pressure [IPAP]; 5 = expiratory positive airway pressure [EPAP]) for 16-18 hours of the day, and a decision was made to keep her in the hospital until transplant preparations could be finalized. If her respiratory status continued to decline, she would be converted to a full-face BiPAP, leaving intubation and mechanical ventilation as a last resort.

Anita's sputum culture indicated that two strains of multi-drug-resistant P. aeruginosa were present. Her respiratory status slowly improved with a complex respiratory treatment plan that included two antibiotics, bronchodilators, mucolytics, an oral steroid, bilevel positive pressure ventilation, oxygen, and the use of a flutter valve and Therivest[R] 3 times a day. Anita received her cefepime (Maxipime[R]) and tobramycin (Nebcin[R]) through an implanted port in her right chest. Strict aseptic technique was maintained by staff who manipulated her IV lines and port, and routine flushing protocols were followed to prevent catheter occlusion. Staff ensured that she received her antibiotics within 4 hours of admission, and intermittent doses were scheduled to allow Anita at least 6 hours of uninterrupted sleep at night. A blood gas on the 8th day of admission indicated some improvement in her hypercapnia and hypoxia. Her serum creatinine and BUN were carefully monitored to rule out renal insufficiency from chronic aminoglycoside use. As part of the standards of care, Anita was screened earlier in the year for hearing loss. At that time, she also had her yearly bone density testing.

During this admission, Anita was seen by many members of the health care team, including a social worker, dietitian, physical therapist, infectious disease physician, pulmonary therapy rehabilitation specialist, stress management services, and primary pulmonary physicians. A nurse case manager and the inpatient CF coordinator, who evaluate each patient, began discharge planning early to ensure access to needed home services such as respiratory therapy and intravenous medication administration. Adjunct therapies such as Reiki, massage therapy, and guided imagery were also offered. A hospital chaplain was available if the patient or family wanted assistance with spiritual needs.

Anita was placed on regularly scheduled doses of NPH and Humalog[R] insulin before meals with sliding scale doses of Humalog between meals. Her blood glucose was checked before meals and at bedtime each day. Her fasting blood sugar demonstrated gradual normalization (from 289 mg/dL on admission) over the course of the admission. Anita was very knowledgeable about her diabetes regimen, counting carbohydrate units at home during meals and snacks, and adjusting her insulin to achieve optimum glucose control. Although Anita did not gain weight during the admission and remained at 85% of her ideal body weight (IBW), she did not lose any additional weight and usually are about 75% of her meals. Her meals were supplemented with high-protein, high-calorie snacks such as yogurt shakes, and her family often brought favorite items from home to boost calorie consumption.

Although Anita had pancreatic insufficiency and required supplemental pancreatic enzymes, she did not appear to be developing any other gastrointestinal complications of CF. She had no evidence of spleen or liver enlargement on physical exam, and her liver enzymes were within normal range. The respiratory therapist on the unit developed a treatment plan that allowed for all of Anita's airway clearance activities to occur before meals to prevent regurgitation and reflux. Staff on the unit encouraged Anita to keep her own supply of enzymes at the bedside and take them as needed. This fostered her sense of independence and allowed for more effective dosing with unplanned meals and snacks. Anita's medication regimen included a daily multivitamin, as well as vitamins A, D, E, and K. Because of past deficiencies, she also received zinc and calcium supplements.

Fortunately, this admission turned out to be the last for Anita. She was transferred to a regional transplant center within a couple of weeks, where she successfully received a double-lung transplantation from two living, related donors. She is being monitored closely at both the transplant center and also the CF clinic, and is showing no signs of rejection.

Barbara S. Aronson, PhD(c), APRN, CS, is an Assistant Professor, Southern Connecticut State University, New Haven, CT.

Melissa Marquis, RN, is a Staff Nurse, Cystic Fibrosis Inpatient Coordinator, Hartford Hospital, Hartford, CT.
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Date:Jun 1, 2004
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