Improving Influenza Vaccine Compliance Through Patient Education for Patients With Cystic Fibrosis
Article Outline
- Pathophysiology of CF
- Influenza
- Influenza vaccine
- Previous efforts to promote immunization for high risk populations
- A Pilot strategy with CF families
- Conclusions
- References
- Biography
- Copyright
Mary Margaret Gottesman, PhD, RN, CPNP
Ohio State University College of Nursing
Columbus, Ohio
Cystic fibrosis (CF) is an autosomal recessive disorder that affects about 30,000 children and young adults in the United States. More than 1000 new cases are diagnosed each year, with only 10% of patients with newly diagnosed disease being 18 years of age or older. A sweat test performed at a CF center is the standard method for diagnosis; however, genetic testing confirms the diagnosis. The median age of survival for patients with CF has risen to 36.8 years, which is an increase of 5 years in the last 4 years (Beall, 2006).
Complications from chronic lung disease are the leading cause of mortality in the CF population. Standardization of care and additional CF therapies are factors in the improvement of the length and quality of life for patients with CF. According to the Cystic Fibrosis Foundation Clinical Practice Guidelines (1997), all patients with CF older than 6 months of age should receive the influenza vaccine annually. Thus, encouraging flu vaccinations through patient education and monitoring of vaccination status is an important way to prevent deterioration of lung function in patients with CF.
Pathophysiology of CF
Patients who have CF inherit a mutation causing the abnormal expression of the protein CF transmembrane regulator (CFTR). The mutations can be homozygous or heterozygous. The most common mutation is delta 508. CFTR is involved in the transport of electrolytes across the cell membrane. CFTR acts as a chloride channel present on the surface of many types of exocrine tissue, including those lining the airways, bile ducts, pancreas, sweat ducts, and vas deferens (Rowe, Miller, & Sorscher, 2005).
Lung damage is progressive and causes more than 95% of deaths from CF. The typical features of CF lung disease are mucus plugging, chronic inflammation, and infection. Mucus plugging in CF results from both the increased production of mucus and altered physicochemical properties of the mucus. There are an increased number and size of mucus-secreting airway cells coupled with alterations in CF mucus, which is dehydrated and viscous because of abnormal chloride secretion and sodium absorption (Behrman, Kliegman, & Jenson, 2003).
Inflammation is another typical feature of CF. Abnormal cytokine profiles, such as deficient interleukin-10, promote chronic inflammation and contribute to long-term lung damage. The airways of individuals with CF also have increased numbers of neutrophils. The byproducts of neutrophil metabolism cause direct damage to structural proteins, such as elastin, as well as inducing airway cells to produce interleukin-8, which attracts more neutrophils and worsens the vicious cycle of inflammation and the destruction of respiratory tissue elasticity. Neutrophils also cleave immunoglobulin G and other immune components important for the phagocytosis of pathogens and directly stimulate more mucus secretion (McCance & Huether, 2002).
Pathogens commonly causing lung infections in patients with CF include Staphylococcus aureus, Pseudomonas aeruginosa, Burkholderia cepacia, Mycobacterium avium-intracellulare, and occasionally nontypeable Haemophilus influenzae, Moraxella catarrhalis, Xanthomonas maltophilia, Alcaligenes xylosoxidans, Enterobacteriaceae, the fungus Aspergillus (Steele, 2006), and Stenotrophomonas maltophilia (Lang et al., 2004).
Children and young adults with CF are at an increased risk of pulmonary complications if they contract influenza. They have an increased incidence of endobronchial infections for reasons that are poorly understood, because there is no systemic immune defect (McCance & Huether, 2002). The most likely cause is the CF airway environment, which favors bacterial colonization within viscous airway secretions because of the individual’s inability to remove secretions adequately from the airways (Rowe et al., 2005). Conversely, infection with the influenza A virus may lead to patients’ lungs becoming chronically colonized with P. aeruginosa bacteria (Bhalla, Tan, & Smyth, 2006). Infections with viruses, including the influenza virus, lead to 20% of episodes of increased bronchial infections in patients with CF.
Influenza
Influenza viruses spread from person to person via respiratorydroplets. The incubation period is typically 1 to 4 days, with an average of 2 days. For the majority of the population, symptoms last 3 to 7 days, although a cough and symptoms of fatigue may last as long as a week. Influenza is potentially serious in the very young because of a lack of immunity, in the elderly because of decreased resistance to infection and a poorly operating immune system, and in those with underlying medical conditions such as CF, which render them at greater risk for infections (Harper, Fukada, Uyeki, Cox, & Bridges, 2005). The primary way to reduce the incidence of influenza in the United States is to vaccinate those people most at risk, their household contacts, and health workers with either inactivated or live attenuated influenza vaccine. Ideally, this is done by administering the vaccine to people at their place of work, during hospitalizations, or during routine health care office visits before the start of the flu season. The Advisory Committee of Immunization Practices recommends strong efforts to increase vaccination rates, including reminder/recall systems and standing order programs (Harper et al.).
Influenza vaccine
The 2005-2006 trivalent vaccine virus strains were A/California/72004(H3N2)-like, A/NewCaledonia/20/99(H1N1)-like, and B/Shanghai/361/2202-like antigens. Available influenza vaccine gave approximately 70% to 80% protection and reduced the likelihood of severe infection. Most vaccinated children had high titers that were protective against the strains in the vaccine, but the antibody response among high-risk children was usually somewhat lower. Vaccines are repeated each year because the principal surface antigen hemagglutinin of influenza viruses A and B undergoes changes by point mutation (antigenic drift) or by exchange of whole DNA segments encoding for this protein (antigenic shift) (Bhalla et al., 2006).
There are a few adverse reactions to and contraindications for the influenza vaccine reported by the National Vaccine Information Center. The most common adverse reactions begin within 12 hours after vaccination and include fever, fatigue, painful joints, and a headache. These symptoms may last for several days. A more serious reaction associated with the vaccine is Guillain-Barré syndrome, an immune-mediated nerve disorder characterized by several months of muscle weakness, unsteady gait, numbness, tingling, pain, and sometimes paralysis. Fewer than 5% of cases end in death. Contraindications to the flu vaccine include anyone who is sick with a fever or who has an egg allergy, a mercury allergy, or a history of Guillain-Barré syndrome. The flu vaccine is recommended only for pregnant women who are past their 14th week of pregnancy. In compliance with the recommendation in 1999 by the Environmental Protection Agency and the Food and Drug Administration to take mercury out of all childhood vaccines, a preservative-free vaccine in a prefilled syringe is available for children ages 6 to 35 months, with only a trace amount of thimerosal (National Vaccine Information Center, 2006).
Previous efforts to promote immunization for high risk populations
The Intermountain Cystic Fibrosis Center in Utah used retrospective chart review, a mailed survey, and telephone interviews to determine the vaccination coverage level for the 1997-1998 influenza season and the factors associated with nonadherence. The data for 362 patients at the center were reviewed from the Salt Lake City parent center and satellite clinics in Idaho. A total of 256 (70.7%) patients received the vaccine, including 181 of 229 (79%) children less than 18 years of age and 75 of 107 (70.1%) adult patients. Vaccination rates between the parent clinic and the satellite clinics were similar. More patients who had received care at the clinics had vaccines (80.8%) versus those who had not received care (59.5%). Patients who had visited any clinic during the last 3 months of the year had a vaccination rate of 87.1%. Those patients who were not vaccinated had fewer overall clinic visits, had fewer hospitalizations, and lived farther away than those who were vaccinated. The patients who did not receive the vaccine cited major reasons as “forgot” or being “too healthy,” “too busy,” “worried about the side effects,” or “too sick at the time” (Marshall et al., 2002).
A Cochrane Review done in February 2006 looked at all randomized and quasi-randomized studies on the effects of influenza vaccine on CF. There are currently no studies that compare a placebo with a vaccine given to people with CF and no studies comparing a whole virus vaccine with a split virus vaccine (Bhalla et al., 2006). The review outlined studies that showed whether influenza vaccine reduced morbidity in the 12 months after vaccination; changed the rate of deterioration in lung function, nutritional status, the frequency of intravenous antibiotic use, P. aeruginosa carriage, death, types of adverse effects or had variable effectiveness when compared with different types of vaccines.
Only four studies met the inclusion criteria. Two studies compared an intranasally applied live influenza A vaccine with an intramuscular injection of inactivated trivalent influenza vaccine. Two other studies compared a split virus influenza vaccine with a subunit influenza vaccine and a trivalent virosome influenza vaccine with a subunit influenza vaccine (Schaad et al., 2000). The main outcomes reported by the studies were adverse effects and the immune response generated by the vaccine measured by serologic antibodies. All vaccinations had self-reported adverse effects from 24% for the intranasal live vaccine to 43% for the split virus vaccine and 27% for a trivalent inactivated vaccine. None of the effects reported were serious. After allowing for the low power of the studies, there was no significant difference among the types of vaccines. All types of vaccines generated a satisfactory immunologic response. None of the studies answered the question of vaccine impact on important clinical measures for CF patients such as the rate of pseudomonal infection, changes in lung function, need for hospitalization, and length of hospital stay or effects on nutritional status (Bhalla et al., 2006).
An advocacy program at British Columbia’s Children’s Hospital (Bjornson et al., 2000) targeted patients in its subspecialty clinics with diabetes, asthma, and bronchopulmonary dysplasia to increase their use of influenza vaccine and prevent influenza complications. Information packages addressing the child’s disease in relation to influenza infection and vaccination were developed and sent to parents and health care providers. They were mailed in September 1998 with a letter from their clinic director recommending vaccination. Reply cards and telephone interviews conducted in December or later evaluated the response. Of the patients with diabetes who received the information and responded, there was a 19.4% increase in vaccinations over the previous season and the children with asthma or bronchopulmonary dysplasia showed an increase of 20.6% and 38%, respectively. In both the diabetes and asthma groups, patients over the age of 15 had a lower vaccination rate than during the previous season. The conclusion was that educational efforts addressing the impact of influenza on individuals with a specific disease endorsed by a familiar specialist are effective in increasing immunization rates. A challenge to the program was to determine how best to communicate the importance of the flu vaccine to their adolescents.
A Pilot strategy with CF families
Influenza vaccine has proved to be a safe and effective vaccine recommended by the Centers for Disease Control and Prevention and Cystic Fibrosis Foundation Clinical Practice Guidelines (1997) for the prevention of influenza. The literature supports the effectiveness of programs to improve adherence in high-risk populations. The challenge is to determine how best to monitor compliance and to communicate to patients and families about the need for the vaccine.
The Cystic Fibrosis Center at a major children’s hospital believed its compliance rate was high but had a difficult time identifying how many patients actually received the vaccine. The first step was to identify those who received the vaccine and then either vaccinate those who had not received the vaccine or encourage them to become vaccinated.
Phone calls were made to all patients who did not have clinical documentation of having received the flu vaccine. There were 388 patients on the original list of patients. Of these, 27 were determined to be inactive patients, who had moved out of the area or were being treated elsewhere. Two patients were ineligible to receive the flu vaccine because they were allergic to eggs. Of the 359 people remaining on the list, 299 were determined to have had the vaccine (83.3%). Thirteen patients did not receive flu shots. One patient was a 16-year-old girl who was seen at the CF clinic but refused the shot. Another patient did not receive the shot because her local physician decided not to give it as a result of her poor health at the time. The other 11 patients were encouraged by phone to get the shot but “never got around to it.” All but one of these patients lived in the immediate area of the CF Center but were not seen in the clinic during the flu season.
Conclusions
The flu immunization compliance rate of those patients with CF who were able to be contacted was very high (95.8%). Those who were not able to be contacted or those who had not been seen in the clinic lowered the compliance to 83.3%. CF morbidity and mortality has improved tremendously over the past two decades, and encouraging all aspects of preventive health management in the high-risk CF population will only continue to improve quality of life and life expectancy. Calling dozens of patients was very time consuming. Another option of sending an educational flyer to the patients with CF before the flu season to increase awareness may be an effective alternative (Figure 1).
FIGURE 1.
Educational flyer for patients with CF. This figure is in color online at www.jpedhc.org.
It appears from this project that patients who receive preventive care are more likely to get their flu vaccine than those who do not receive preventive care. The flyer is also a reminder to receive ongoing care. It was interesting to find through this project that patients with CF get their flu vaccine at many different locations, but there is no communication among providers. In an effort to share vaccination information with the entire health care team, a section at the bottom of the flyer was designated for documentation of the flu vaccine. It is hoped that use of the flyer for documentation will result in better communication among the health care providers.
It is important for all health professionals involved in the care of patients with CF, as well as many other chronic conditions, to encourage patients and family members to become immunized to prevent or minimize infectious diseases. Whether they get the flu vaccine in the primary care setting, the specialty clinic, the health department, or other sites, if everyone works together through education and monitoring we can help preserve lung function for the patient with CF for as long as possible.
References
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- Beall, R. (2006). Cystic Fibrosis Foundation. Posted May 24, 2006. Retrieved August 12, 2006, from www.cff.org
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- In: Clinical Practice Guidelines for Cystic Fibrosis. Bethesda, MD: Cystic Fibrosis Foundation; 1997;p. 8
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- National Vaccine Information Center. (2006). The flu and the flu vaccine. Retrieved August 12, 2006, from http:www.nvic.org/Diseases/influenza_facts.htm
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- Comparison of immunogenicity and safety of a virosome influenza vaccine with those of a subunit influenza vaccine in pediatric patients with cystic fibrosis. Antimicrobial Agents Chemotherapy. 2000;44:1280–1284
- Steele, R. W. (2006). Vaccinating children with cystic fibrosis. Ask the experts about vaccines. Retrieved August 9, 2006, from Medscape Pediatrics: http://www.medscape.com/viewarticl/523024
Catherine Tran is School Nurse, Columbus Public Schools, Columbus, Ohio.
Judy Pitts is Pediatric Nurse Practitioner, Pulmonary Clinic, Columbus, Children’s Hospital and Clinical Instructor, Pediatric Nurse Practitioner Program, The Ohio State Unversity, College of Nursing, Columbus, Ohio.
PII: S0891-5245(06)00558-X
doi:10.1016/j.pedhc.2006.08.014
© 2007 National Association of Pediatric Nurse Practitioners. Published by Elsevier Inc. All rights reserved.
