Introduction
Cystic fibrosis (CF) is a chronic genetic disease that affects the quality of life for approximately 30,000 Americans (Hayek, 2006). About 1000 children are born each year with the disease (Cystic Fibrosis Research Directions: NIDDK, 2007). CF causes thickening of mucous that primarily affects the pulmonary and gastrointestinal systems. Thickening of the airway mucous blocks gas exchange and provides a growth medium for bacteria, which then leads to airway inflammation and infection, causing permanent lung damage, chronic sinus problems, or both. In the gastrointestinal system, the thickened mucous decreases pancreatic enzyme secretion into the intestines and absorption of nutrients, causing problems with malabsorption that can lead to malnutrition. The overall impact of CF on growth and development can be devastating throughout childhood and into adulthood.
Poor growth is a hallmark of children with CF. Among children with CF, an estimated 16.3% are below the fifth percentile for height and 15.7% are below the fifth percentile for weight (Cystic Fibrosis Foundation, 2004). Malnutrition often results not only from pancreatic insufficiency and malabsorption but also from anorexia and low energy levels (Peterson, Jacobs, & Milla, 2003). Inadequate nutrition causes an overall loss of muscle mass as well as a decrease in the contractility and endurance of muscles, including respiratory muscles (Pedreira et al 2005, Schechter 2003, Schöni and Casaulta-Aebischer 2000). This weakening in the respiratory muscles contributes to ineffective airway clearance and leads to increased risk for infection in persons with CF. The nutritional status of the patient with CF is crucial in the overall prognosis, especially in terms of pulmonary function. Chronic respiratory infections and progressive airway obstruction that lead to the destruction of lung tissue are responsible for most of the morbidity, mortality, and hospitalizations of patients with CF (Peterson et al 2003, Zemel et al 2000).
Background
The Cystic Fibrosis Foundation and the American Academy of Pediatrics (AAP) recommend that infants be breastfed up to the age of 12 months. However, as children grow and change, so do their nutritional needs. It is important for parents to remember that as activity level increases with increasing mobility, calorie requirements also increase. A balanced diet enhanced with high-fat snacks and supplements is necessary to increase caloric intake for children with CF. Children with mild pulmonary disease require only a 5% to 10% increase over the recommended daily allowance to achieve adequate growth, whereas those with more severe lung disease (forced expiratory volume in one second less than 40% of expected) require a 20% to 50% increase over the recommended daily allowance. Because high-fat foods are calorie dense, adding high-fat foods to the diet allows the child to consume higher caloric amounts without consuming great amounts of food.
The AAP (2004) also emphasizes the need for careful nutritional monitoring of patients with CF, recommending assessment of growth parameters every 3 to 4 months as well as the use of a careful dietary history or written dietary logs to evaluate nutritional intake. Such growth parameters include weight, height, weight for age, height for age, weight for height, ideal body weight percentile, body mass index (BMI) percentile, skin fold thickness, and mid upper arm circumference (Borowitz et al 2002, Wiedemann et al 2007). Many practitioners use ideal body weight percentile to examine the growth status of patients with CF, but BMI percentile has been gaining favor in the clinical field because of its ease of calculation and plotting.
Newborn testing assures that parents know early in life that their child has CF, allowing parents to support their child's health through early treatment and high-quality nutrition right from the beginning. Research has shown that nutritional status at age 3 years predicts lung function at age 6 years (Konstan et al., 2003); therefore, it is important to stress good dietary habits early in life. In a study by Powers, Patton, Henry, Heidemann, and Stark (2005), the eating habits of children with CF ages 7 months to 12 years (average age was 4.5 years) were examined. They found that on average, children skipped at least one meal or snack every few days and met their daily recommended caloric intake only one out of every four days. They also found that about half of the meals eaten were low in fat rather than containing the high-fat content as recommended. Children also failed to take their pancreatic enzymes as prescribed with meals and snacks 15% of the time. Clearly, children with CF are at high risk for malnutrition. Continued monitoring of growth patterns and reinforcement of nutritional goals in patients with CF cannot be overemphasized.
Nutrition education with specific nutritional goals for patients and their families is one way to encourage adequate intake. A study evaluating the use of verbal and written nutritional counseling during office visits for patients with CF found that the counseling not only improved nutritional status as measured by anthropometric data but also resulted in better compliance with prescribed enzyme therapy and the use of nutritional supplements, especially among younger patients (Adde et al., 2004). The counseling consisted of a 15-minute discussion and a booklet describing of the importance of good nutrition and the connection between nutrition and lung disease. In this study, patient education played an invaluable role in maintaining adequate nutrition for this population.
Current data imply that the use of nutrition education in patients with CF and their families has a positive impact on energy intake, the use of pancreatic enzymes and nutritional supplements, and overall nutritional status of the patient. Age-appropriate patient education handouts regarding nutrition in CF already have been developed by the Cystic Fibrosis Foundation and are available for use at CF centers. The partnering of a team of practitioners and patients in the development of a nutrition care plan has proved to be an effective way to improve patient outcomes (Lowes, 1998). Family-centered care allows the patient with CF and/or family to be an active participant in goal setting and nutrition planning and may be an excellent way to increase patient adherence to the nutrition treatment plan. A patient goal sheet that allows the patient to be an active participant in his or her nutritional therapy is a way to improve the nutritional status of patients with CF.
Patient Education Handout and Goal Sheet
To motivate patients to make healthy food choices as well as increase caloric intake, a goal sheet was created to be given to patients at each clinic visit. The goal sheet includes the patient's current weight, height, BMI, and corresponding percentiles as well as a goal weight and BMI to be achieved at future visits (Figure). For patients below the 50th percentile for BMI, the goal weight and BMI will correspond to the patient's age at the next visit, sex, and expected height, and will include a body mass index at the 50th percentile as well as the corresponding weight for that BMI. Patients who are already at or above the 50th percentile for BMI will have goal weights and BMIs that reflect maintenance of the current BMI percentile. Another important aspect of the goal sheet is that patients are given the opportunity to agree on three ideas they will try before the next visit to either achieve or maintain their goal weight and BMI. This aspect ensures that the patient is actively thinking about the goal and that the three ideas are a reasonable fit into his or her lifestyle.
Figure.
The goal sheet was developed to be given to the patient at each clinic visit, with a copy kept in the patient's medical record for future reference. At future visits, the health care provider will review the previous goal sheet with the patient, and together, the health care provider and patient can determine whether the goal was met and decide how to adjust the goals to meet the new goal. Having a personalized goal sheet at each visit will remind the patient that the provider cares about his or her nutrition and wants the patient to achieve the healthiest weight possible.
Field Testing
To determine the effectiveness of the goal sheet, it was field tested at a CF Center. One physician, two advanced practice nurses, two dieticians, one registered nurse, six patients with CF (ages ranging from 6-24 years), and seven parents of children with CF (ages 3-11 years) critiqued the handout. The professionals were asked about the accuracy of the information and the likelihood that they would use the goal sheet. The parents and patients were asked to consider ease of use and helpfulness of the goal sheet. All were asked to specify whether anything was difficult to understand or if any valuable information was missing.
The overall response was positive from all individuals surveyed. In general, the professionals believed that the goal sheet was a tool that would benefit patients in various nutritional states, not only those who are malnourished. The CF Center currently utilizes a similar nutrition goal sheet for patients who are below the 50th percentile for BMI, but the practitioners agreed that this new handout would be easy to complete at each visit and contains beneficial patient information that is lacking on the current goal sheet.
The patients and parents also gave the goal sheet an encouraging review. They agreed that it would be helpful to receive a serial report at each visit that they could compare with previous reports. This group also found the goal sheet to be easy to read and understand, and many parents supplied personal "tricks" or ideas to increase nutrients and calories. These ideas were incorporated into the handouts where appropriate. Adult patients believed that the goal sheets would have been more useful when they were younger and that maintenance of weight is more of a concern at this point in their lives.
Conclusion
It was concluded that there might be inevitable limitations to a goal sheet that is used at each visit. For instance, it would not be as useful for adult patients with CF or for those who consistently meet or maintain their goals. These limitations can be remedied easily by changing the frequency of its use or by limiting its use to growing children. This is another part of the care plan that the patient and provider can establish together. It is expected that use of these sheets will assist health care providers in early identification of and intervention with patients who are either malnourished or at risk of malnourishment, thus allowing patients with CF to reach their full growth and development potential.
Source : http://www.medscape.com/viewarticle/572930
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