Description of a Standardized Nutrition Classification Plan and its

Description of a standardized nutrition classification plan and its
relation to nutritional outcomes in children with cystic fibrosis
Amanda Leonard,1 MPH, RD, CDE, Erin Davis,1 RD, CNSD, Beryl J. Rosenstein,2 MD,
Pamela L. Zeitlin,2 MD, PHD, Shruti M. Paranjape,2 MD, Donna Peeler,2 RN,
Cynthia Maynard,3 PHD, and Peter J. Mogayzel,2 JR, MD, PHD
1
Division of Pediatric Gastroenterology and Nutrition, 2Eudowood Division of Pediatric Respiratory Sciences,
The Johns Hopkins Medical Institutions and 3Department of Behavioral Psychology, Kennedy Krieger Institute
Objective Better nutrition enhances lung function and increases survival for children with cystic fibrosis
(CF). Therefore, we developed a standardized strategy to evaluate nutritional status and create individualized
treatment plans to ensure that all patients received the same high-quality care in a busy CF Center.
Methods A quality improvement approach was undertaken to develop a novel nutrition classification strategy
to identify and treat children with subtle manifestations of nutritional deficits in addition to those with
obvious nutritional issues. Results During the 15-month study period, the median body mass index (BMI)
percentile increased from 35.2 (0–95.9) to 42.0 (0–97.7), p < .005. Additionally, the number of children with
a BMI 50th percentile increased by 11.8%. Conclusions Adoption of a standardized approach to
nutritional assessment and treatment led to significant improvement in nutritional outcomes of CF patients,
demonstrating that systematic changes in clinical practice can improve clinical outcomes substantially over
a short period of time.
Key words
cystic fibrosis; nutrition; quality improvement.
A hallmark of cystic fibrosis (CF) is malnutrition leading to
poor weight gain. Many factors contribute to inadequate
weight gain and malnutrition in CF (Borowitz et al., 2005).
Pancreatic insufficiency, found in 85–90% of the CF
population, can be difficult to overcome, even with
pancreatic enzyme replacement therapy. A variety of gastrointestinal disorders, including gastroesophageal reflux,
constipation, bacterial overgrowth, and distal intestinal
obstruction syndrome (DIOS), can decrease caloric intake
and nutrient absorption (Borowitz et al., 2005). Worsening
lung disease increases caloric needs while also decreasing
appetite in some cases. Undiagnosed, or inadequately treated, CF-related diabetes (CFRD) can cause rapid weight
loss (Rolon et al., 2001). Behavioral barriers to optimizing
nutrition in CF patients have been well described (Stark &
Powers, 2005). Finally, socioeconomic and cultural barriers can also affect nutritional outcomes (Schechter,
Shelton, Margolis, & Fitzsimmons, 2001).
Nutrition plays a vital role in the health of individuals
with CF. In their classic article, Corey et al. (Corey,
McLaughlin, Williams, & Levison, 1988; Emerson et al.,
2002; Peterson et al., 2003; Steinkamp & Wiedemann,
2002) demonstrated a marked survival advantage in children with better growth parameters. This relationship has
been supported by several studies showing that better
nutritional status is associated with better lung function,
(Milla, 2007). Additionally, inadequate nutrition early in
life has been shown to affect lung function later in life
(Konstan et al., 2003). Using data from the US CF
Foundation Patient Registry, an expert panel convened
by the CF Foundation identified a significant correlation
between body mass index (BMI) and lung function
(Stallings, Stark, Robinson, Feranchak, & Quinton,
2008). These findings, which were widely disseminated
in the CF community prior to their publication, led to an
emphasis on maintaining individual BMI at or above the
All correspondence concerning this article should be addressed to Peter J. Mogayzel, Jr, MD, PhD, Cystic Fibrosis
Center, The Johns Hopkins Hospital, 200 North Wolfe Street, Baltimore, MD 21287-2533, USA.
E-mail: [email protected]
Journal of Pediatric Psychology 35(1) pp. 6–13, 2010
doi:10.1093/jpepsy/jsp029
Advance Access publication May 6, 2009
Journal of Pediatric Psychology vol. 35 no. 1 ß The Author 2009. Published by Oxford University Press on behalf of the Society of Pediatric Psychology.
All rights reserved. For permissions, please e-mail: [email protected]
Approach to Improving CF Nutrition
50th percentile for age rather than the previously accepted
standard of the 25th percentile (Borowitz, Baker, &
Stallings, 2002).
Achieving good nutritional outcomes is not always
easy. Review of clinical parameters, such as BMI, at CF
care centers within the US reveals wide variation in results
(Quinton & O’Connor, 2007). This disparity in outcomes
among CF care centers has led the US CF Foundation to
launch a far-reaching quality improvement (QI) initiative
(www.cff.org/LivingWithCF/QualityImprovement/)
that
advocates the use of CF Foundation guidelines, evidencebased medicine, and benchmarking of best care practices
to develop standardized treatment algorithms. The goal of
this program is to encourage the implementation of best
practices to improve clinical outcomes (Quinton &
O’Connor, 2007).
The Johns Hopkins CF Center pediatric care team
embarked on a QI initiative in 2005, which was designed
to improve the nutritional outcomes for children with CF.
At that time, the median BMI for the Johns Hopkins CF
Center was below the national average. Specifically, our
goals were to increase the median BMI percentile of
our patients and decrease the number of children with
suboptimal nutritional status, defined as a BMI <50th
percentile. We hypothesized that the systematic application
of a standardized evaluation and treatment algorithm would
improve nutritional outcomes for children in our clinic.
This project was championed by the physicians and dieticians within the CF Center and coincided with our adoption of the CF Foundation’s guideline that the BMI for
patients should be 50th percentile (Stallings et al., 2008).
Methods
This study was approved by the Institutional Review Board
at the Johns Hopkins Medical Institutions. This was a
prospective, observational study of a QI initiative within
the Johns Hopkins CF Center from September 1, 2005, to
November 30, 2006.
Subjects
During the 15 months of the study, all children aged 2–21
years who had not undergone lung transplantation were
evaluated. Children <2 years of age were not included
in the study because the initial visit for many of these
subjects was shortly after establishing the diagnosis of
CF. Therefore, improvements in nutritional status would
most likely result from initiating CF therapies, such as
pancreatic enzyme replacement, rather than the use of
our nutrition classification system and treatment
algorithm. All subjects were pediatric patients at The
Johns Hopkins CF Center.
Planning the Intervention
Although it is well accepted that optimizing nutrition is
important in CF care, this goal has proven difficult to
achieve at many CF Centers (Quinton & O’Connor,
2007). To date, only a few approaches for optimizing nutrition in CF patients have been studied in a systematic
fashion. We used these studies in conjunction with ‘‘best
practice’’ observations derived from CF Foundation benchmarking of care centers with excellent nutritional outcomes to develop a standardized approach to nutrition
classification and therapy. We began by developing
a novel classification system that focused attention on
children that had any evidence of nutritional problems.
In addition to assigning a nutrition risk category to each
patient, a standardized treatment algorithm was created to
ensure that all patients received the same high quality care
(see Supplementary Material). Treatment was geared
toward: (a) maximizing caloric intake; (b) optimizing
nutrient absorption; and (c) diagnosing and treating
CFRD. The primary goal of this classification and treatment
strategy was to identify affected children and establish a
therapeutic intervention as early as possible.
A core group from the multi-disciplinary staff attended
the Learning and Leadership Collaborative IV sponsored by
the CF Foundation to receive training in QI processes
(Quinton & O’Connor, 2007) and design an initial project
to improve nutritional outcomes. The entire multidisciplinary team attended weekly QI meetings that focused on
developing overall goals and specific interventions to
improve the nutritional status of children with CF.
The available literature including observations about
‘‘best practices’’ was reviewed. An intervention strategy
was developed that entailed systematically categorizing
each patient’s nutritional status, developing a treatment
algorithm, and implementing this revised practice pattern
in a busy CF clinic. The team agreed that in order to
achieve the goal of improved nutritional outcomes these
algorithms needed to be applied systematically and that
frequent follow-up for children with nutritional deficits
would be of critical importance.
Initial Assessment
Nutrition risk classification was completed either by the
physician with verification by the dietician or by the dietician alone during each clinic visit between September 1,
2005, and November 30, 2006 (see Supplementary
Material). The initial assessment and assignment of a nutrition classification category was completed at the first visit
7
8
Leonard et al.
during the study period. Both sick and routine quarterly
clinic visits were included in the study.
Classification
During the planning stage of this project, we created five
nutrition classification categories (Table I) that were based
on a modification of the CF Foundation pediatric nutrition
consensus statement (Borowitz et al., 2002). Children were
defined as: nutritional ‘‘failure’’ if the BMI was <10th
percentile for age, ‘‘at risk’’ if the BMI was 10–24th
percentile, ‘‘acceptable’’ if the BMI was 25–49th percentile, and ‘‘optimal’’ if the BMI was 50th percentile.
Minimal height and weight percentiles were also utilized
in the evaluation process (Table I). The unique aspect of
our classification schema was the use of a ‘‘concerning’’
category designed to identify and promote early intervention for those individuals with a BMI 25th percentile who
exhibited any weight loss since the previous clinic visit, no
weight gain, or a downward crossing of percentiles over the
previous three months. We continued to use the term
Table I. Nutrition Risk Classifications
Failure
Weight < 5th percentile for age
Or
Height < 5th percentile for age
Or
BMI < 10th percentile
At Risk
Weight 5–10th percentile
Or
BMI 10–24th percentile
Acceptable
All of the following
Weight > 10th percentile
Height > 5th percentile
BMI 25–49th percentile
Weight gain following growth channel
Optimal
All of the following
Weight > 10th percentile
Height > 5th percentile
BMI 50th percentile
Weight gain following growth channel
Concerning
Meet standards for acceptable or optimal
but
Had weight loss since the last clinic visit
Or
No weight gain in the last 3 months
Or
Downward crossing of weight or height
percentiles in the last 3 months
‘‘acceptable’’, rather than a new description such as ‘‘suboptimal’’, because a BMI 25th percentile had previously
been considered adequate in our clinical practice and by
the CF Foundation. The use of the term ‘‘acceptable’’ was
adopted to encourage patients and parents to aim for a
higher BMI percentile without diminishing their previous
efforts to maintain optimal nutrition. We did not want
patients to feel stigmatized by an unflattering label because
the bar had been raised for their nutrition goal. The use of
the term ‘‘optimal’’ was also new for our clinic.
Nutrition Intervention
A nutrition treatment algorithm was developed to standardize care once the need for intervention was identified,
with three primary areas of focus: caloric intake, nutrient
absorption, and CFRD (see Supplementary Material).
Caloric Intake
The caloric intake arm assessed frequency of meals and
snacks, use of high-calorie foods/additives, nutritional
supplement use, and identification of any barriers to
intake. The number of meals and snacks were evaluated
as well as the types of foods consumed. If the patient was
not already using a high-calorie oral supplement, one was
recommended. Barriers to intake were identified (e.g., food
availability, time for breakfast before school) and potential
solutions were discussed with the patient and family.
A referral to a pediatric psychologist was suggested for
behavioral intervention when deemed appropriate.
Nutrient Absorption
The absorption arm assessed the proper administration of
pancreatic enzyme supplements (e.g., appropriately mixed
if unable to swallow pills, no generic formulations, dosage,
and timing) and acid suppression to maximize pancreatic
enzyme efficacy.
A referral to a pediatric gastroenterologist was made
if caloric intake and absorption had been optimized and
weight gain continued to be inadequate. Referral was also
considered if the patient exhibited symptoms of severe
gastroesophageal reflux, severe constipation, bacterial overgrowth, or if placement of a gastrostomy feeding tube was
indicated.
CF-related Diabetes
The CFRD arm assessed premorbid symptoms of diabetes
(e.g., polydipsia, polyuria, unexplained weight loss or
unexplained decline in lung function). Additionally, a diagnostic evaluation for CFRD was conducted if the patient
had a history of casual blood glucose values that were
abnormal or if caloric intake and nutrient absorption
Approach to Improving CF Nutrition
had been maximized and weight gain remained suboptimal. Individuals with possible CFRD underwent an oral
glucose tolerance test and a glucometer was prescribed
for home use. Blood glucose values (fasting and 2 hr postprandial) were collected daily for 2 weeks, after which a
decision was made regarding referral to a pediatric
endocrinologist.
Behavioral Psychology Intervention
If behavioral concerns were identified by the care team,
referral was made to a pediatric psychologist who evaluated
the family and identified specific barriers (e.g., family and
psychosocial stressors, family functioning, parent–child
interactions, structure of meals, child behaviors, etc.)
that could negatively impact the child’s adherence to
behavioral and nutritional interventions. Recommendations were provided to parents that focused on behavioral
modifications, with an emphasis on differential attention,
contingent reinforcement, time-out procedures, stickers,
token economies, and direct commands. Parents were
also encouraged to refrain from participating in behaviors
which included feeding, coaxing, bartering, and physical
prompting. Parents were educated on ways to promote
structure during meal times and were encouraged to set
strict rules and expectations at meals, to limit meals to no
longer than 20 min, and to implement contingencies for
eating and non-eating.
Nutrition Education
Patients and families received education on the importance
of nutrition for the health of CF Patients. Education was
individualized to address the needs of the each patient and
family. Examples of topics covered include tips for adding
calories, suggestions for meal planning, assistance with
enzyme dosing and administration, and trouble shooting
for barriers to nutrition intervention identified during the
visit. At each visit, patients received a trend of their weight,
height and BMI percentiles over the preceding 1–2 years as
well as written goals.
Implementation
The team realized that the introduction of this QI project
would require several changes in clinic. For example, the
dietician needed to evaluate the trend of weight gain for
every patient that was seen in clinic, not just those that lost
weight or had obvious poor weight gain. Additionally,
more frequent follow-up would be required for many
children, thereby increasing the number of patients being
seen in each clinic session.
To pilot this new nutritional intervention program, we
first introduced it in one of the 3 weekly CF clinic sessions
utilizing a single dietician. This allowed several, short plan,
study, do, act (PDSA) cycles to be performed to refine the
program before it was introduced into all the clinic sessions. These analyses caused us to change the template of
appointment times and make adjustments to clinic flow to
better utilize the dieticians’ and other providers’ time.
Clinic paperwork was simplified and a white board was
installed to better track the location of patients and providers. Additionally, shorter ‘‘nutrition’’ follow-up visits
were scheduled at the beginning and end of each clinic
session.
Another change to our previous clinic practice
included the use of an algorithm to provide structure to
the treatment interventions (see Supplementary Material).
Finally, the use of the ‘‘concerning’’ category focused our
attention on children with an otherwise ‘‘acceptable’’ or
‘‘optimal’’ BMI who had subtle growth issues. These
changes in practice were agreed upon by all the physicians
and dieticians seeing children with CF.
Measuring the Response to Nutrition Interventions
Response to nutrition intervention was evaluated by
assessing change in BMI percentile for individual patients
as well as for the entire clinic population using run charts.
Additionally, the number of children in each nutrition risk
category was assessed. Run charts were utilized to track the
changes in the median BMI of the clinic and the number of
patients in each nutrition risk category over time. Median
BMI percentile for those patients with at least two clinic
visits during the study period was compared before and
after the intervention using the Wilcoxon Signed-Rank test.
Results
Subjects
There were 247 children evaluated, of whom 213 had had
at least two clinic visits during the study period. The
median age (range) for all subjects was 12.4 (2.1–21.5)
years, with equal distribution by gender of 123 females
(49.8%) and 124 males (50.2%). Twenty-three (9.3%) of
the patients were pancreatic sufficient.
Initial Assessment
The initial median BMI percentile of all 247 patients in this
study was 37.8 (0–95.3). A nutrition risk category was
assigned to each patient during their initial clinic visit
(Fig. 1). Seventy-nine (31.9%) of the patients were
classified as ‘‘optimal’’, 53 (21.5%) of the patients as
‘‘acceptable’’, 46 (18.6%) of the patients as nutritionally
‘‘at risk’’, and 52 (21.1%) of the patients in nutritional
‘‘failure.’’ There were 17 (6.9%) patients classified as
9
Leonard et al.
‘‘concerning’’ at the initial visit during the study period.
The most common cause of weight loss or poor weight gain
in this group was poor appetite, which occurred in
7 (41.2%) of the patients. Other reasons for the ‘‘concerning’’ classification included: weight loss associated
with acute illness occurred in 6 (35.2%), skipping meals
in 2 (11.8%), malabsorption in 1 (5.9%), and increased
activity in 1 (5.9%).
Age Distribution
There was an age-related trend in the distribution of
nutrition classifications (Fig. 1). The 2- to 5-year-old
group had the highest percentage of ‘‘optimal’’ and
‘‘acceptable’’ patients (66.7%). The percentage of subjects
within these categories decreased in the older cohorts so
that only 18% of the 16-year-old group was considered
‘‘optimal’’. These results confirm previous findings that
nutritional status varies with age (Lai, Cheng, & Farrell,
2005).
100%
90%
80%
70%
Patients (%)
60%
50%
Optimal
Acceptable
Concerning
At Risk
Failure
40%
30%
20%
10%
0%
2-5 (n = 45)
6-10 (n = 61)
11-15 (n = 75)
≥16 (n = 65)
Age (years)
Figure 1. Distribution of nutrition classifications by age for the
247 patients at the initial clinic visit of this study period.
Nutritional Intervention
Patients classified as ‘‘acceptable’’, ‘‘concerning’’, ‘‘at
risk’’, or in nutritional ‘‘failure’’ received an intervention
according to a treatment algorithm that included three
arms as detailed above. Interventions varied based on
patient needs and individual treatment plans were developed for each patient, which included frequent follow up
in clinic and by phone.
The response to our nutritional intervention over time
was monitored using run charts. Figure 2 demonstrates the
change in median BMI percentile over time for all patients
seen in the clinic and for the cohort of 213 patients
who were seen more than once during the study period.
The median BMI for the patients who had at least two
clinic visits during the 15-month study period increased
19.3% from 35.2 (0–95.9) to 42.0 (0–97.7), p < .005.
A run chart demonstrating the nutrition classification
at each clinic visit is shown in Figure 3. During the first
2 months of the study, 10–11% of the patients were not
evaluated. With refinement of the clinic flow we were able
to assess every patient by the 5th month of the study.
During the course of the study there were 120 visits
when a child was classified as ‘‘concerning.’’ At the subsequent visit, 37 (30.8%) of the children were classified as
‘‘optimal’’, 40 (33.3%) were ‘‘acceptable’’, 27 (22.5%)
remained ‘‘concerning’’, 8 (6.7%) were ‘‘at risk’’, and
8 (6.7%) were in ‘‘failure.’’
Figure 4 shows nutrition classification at the beginning and the end of the study period for the cohort
213 patients with at least two clinic visits during the
study. The number of patients in the ‘‘optimal’’ and
‘‘acceptable’’ ranges increased while the number of
patients in both the ‘‘at risk’’ and ‘‘failure’’ categories
decreased. The greatest improvement was observed in
50
45
BMI Percentile
10
EntireClinic
40
35
Cohort
30
Sept-Nov 05
Dec 05-Feb 06
Mar-May 06
Jun-Aug 06
Sept-Nov 06
Figure 2. Run chart of the median BMI percentile for age during each quarter of the study. Open squares represent all the patients seen in clinic
and the closed triangles are members a cohort of 213 CF patients who had at least two clinic visits during the study period. If patients were seen
more than once during the quarter, the last measurement during that quarter was utilized for the analysis.
Approach to Improving CF Nutrition
Percentage of Visits
60%
50%
Optimal &
Acceptable
40%
30%
Failure
20%
At Risk
10%
Concerning
0%
Not
Assessed
Month
Figure 3. Run chart of the percentage of children in each nutritional status at each clinic visit across the 15 months of the study. Children may be
represented more than once in any given month.
100%
Patients (%)
80%
Optimal
60%
Acceptable
At Risk
40%
Failure
20%
0%
INITIAL
FINAL
Figure 4. Traditional nutritional classification of CF patients at the initial
and final visits for the cohort of 213 patients who had at least two clinic
visits during the study period.
children moving from ‘‘at risk’’ to ‘‘optimal’’ or ‘‘acceptable’’ groups. The number of children in the ‘‘nutritional
failure’’ group remained relatively stable, which was not an
unexpected finding because their heights, which were
often <5th percentile, were unlikely to change during
the study period. The percentage of children considered
either ‘‘optimal’’ or ‘‘acceptable’’ increased from 61.5% to
70.4% during the study period. The number of children
with a BMI 50th percentile increased from 76 to 85,
an increase of 11.8%. Patients were not classified as
‘‘concerning’’ for this analysis.
Discussion
Maintaining optimal nutrition is a challenge for both CF
patients and caregivers. We have shown that a systematic
approach to classifying and treating nutrition in a busy
CF clinic can improve outcomes in a short period of
time. Our goal of creating a standardized approach to
both nutritional assessment and treatment of CF patients
improved the median BMI percentile in our clinic by 19%
in just 15 months.
The rationale for developing our classification system
was to identify not only those children with obvious
nutritional issues, but also to recognize those children
who had subtle manifestations of nutritional deficits
regardless of their nutritional category. Any child with a
BMI <50th percentile was thought to need more aggressive intervention. Additionally, those children classified as
‘‘concerning’’ may have had an otherwise ‘‘optimal’’ BMI,
but had additional problems such as weight loss or slower
than anticipated weight gain. We believe that the increased
attention given to these children allowed us to alter weight
trends before these children developed significant nutritional deficiencies, which would be more difficult to treat
and reverse. However, the improvements in nutrition were
not limited to those children with subtle problems since
the number of children in the ‘‘at risk’’ and ‘‘failure’’
groups also decreased during the study.
Suboptimal nutrition in CF patients is often multifactorial. Therefore, we chose to develop treatment plans that
encompassed three aspects known to affect nutritional
outcomes in CF: caloric intake, nutrient absorption, and
CFRD. The interventions were coupled with education
about nutrition and its importance in improving CF patient
outcomes.
Increasing caloric intake can be a challenge in CF
patients. Since CF patients have a higher energy expenditure compared to unaffected individuals, they require
increased caloric intake to maintain adequate growth.
11
12
Leonard et al.
Additionally, these requirements are increased in children
with more advanced lung disease (Moudiou, GalliTsinopoulou, Vamvakoudis, & Nousia-Arvanitakis,
2007). Clearly, modifying behavior is critical to the success
of nutritional interventions (Stark, 2003). Several studies
have demonstrated that behavioral interventions can
improve caloric intake in children with CF (Stark et al.,
1996, 2000). However, barriers to increasing caloric intake
may be difficult to overcome. For example, many families
find it difficult to alter their typical routines and set aside
time for additional snacks during the day. The availability
of Behavioral Psychology services was of great benefit in
identifying and overcoming behavioral barriers.
Nutritional supplements, either oral or delivered by
gastrostomy tube, are frequently prescribed to enhance
caloric intake. However, these supplements have not
been shown to consistently improve nutritional outcomes,
especially when compared to behavioral interventions
(Koretz, Avenell, Lipman, Braunschweig, & Milne, 2007;
Smyth & Walters, 2007). Additionally, there are no
randomized control trials to support the use of enteral
feeding tubes in CF patients (Conway, Morton, & Wolfe,
2008). Appetite stimulants are also widely prescribed, but
there are no large trials confirming their usefulness.
Gastrointestinal problems, including gastroesophageal
reflux, constipation, bacterial overgrowth, and DIOS can
affect appetite and the ability to gain weight (Borowitz
et al., 2005). Caloric needs of CF patients may be further
increased due to acute illness, making weight gain even
more difficult. Enhancing absorption of calories by
appropriate use of pancreatic enzyme supplements is
vitally important. Drugs that block gastric acid production
are frequently used to enhance the function of these
enzyme preparations, but there are no large studies to support this practice. CFRD has been associated with worse
lung function and survival, especially for females with CF
(Marshall et al., 2005; Milla, Billings, & Moran, 2005).
Undiagnosed CFRD may contribute to poor weight gain,
and once treated, may still have a negative impact on overall nutritional status (Rolon et al., 2001). The targeted
interventions outlined in this study allowed us to identify
the reasons for weight loss, or slower than expected gains,
and determine an action plan with the family to improve
nutritional status.
Since preventing malnutrition can be difficult in CF
patients, care teams must strive to develop the best possible approach to this problem. This study demonstrates that
QI techniques can be used to bring about improved
nutrition in a CF center. Involvement of the entire care
team in identifying an important clinical problem ensured
the success of this project. A core group of care providers
received training in QI techniques and championed this
project. However, the entire care team was involved in its
planning and implantation. One of the biggest challenges
to successful implementation of this project was to make
more efficient use of the dietician’s time. At the beginning
of the study, 10–11% of the children were not assessed,
however, we were able to assess every patient by the 5th
month of the study. This was accomplished by streamlining the clinic paperwork and tracking the location of
patients and care providers. Additionally, we strove to
decrease the length of time that patients spent in clinic
by decreasing nonvalue added time when patients were
in rooms without being seen by a member of the team.
Achieving this goal allowed us to see more patients in each
clinic session without sacrificing patient care or the
amount of time that any individual care provider spent
with a patient.
Clearly, there are limitations to this study since it is
not a randomized trial but rather a reflection of current
clinical practice. Although this study was carried out at a
single center, we believe that its results can be duplicated
at other centers, not necessarily by adopting our exact
strategy, but by identifying and systematically addressing
an important clinical problem. We have shown that
QI techniques can be used to develop a standardized
approach to classification and treatment of CF patients
with suboptimal nutrition, thereby leading to substantial
improvement in a short period of time.
Supplementary Data
Supplementary data can be found at: http://www.jpepsy.
oxfordjournals.org/.
Conflicts of interest: None declared.
Received July 22, 2008; revisions received and accepted
March 20, 2009
References
Borowitz, D., Baker, R. D., & Stallings, V. (2002).
Consensus report on nutrition for pediatric patients
with cystic fibrosis. Journal of Pediatric Gastroenterology
and Nutrition, 35(3), 246–259.
Borowitz, D., Durie, P. R., Clarke, L. L., Werlin, S. L.,
Taylor, C. J., Semler, J., et al. (2005). Gastrointestinal
outcomes and confounders in cystic fibrosis. Journal of
Pediatric Gastroenterology and Nutrition, 41(3),
273–285.
Approach to Improving CF Nutrition
Conway, S., Morton, A., & Wolfe, S. (2008). Enteral tube
feeding for cystic fibrosis. Cochrane Database System
Review, 2008(2), Art. No.: CD001198, doi:10.1002/
14651858.CD001198.pub2.
Corey, M., McLaughlin, F. J., Williams, M., & Levison, H.
(1988). A comparison of survival, growth, and
pulmonary function in patients with cystic fibrosis in
Boston and Toronto. Journal of Clinical Epidemiology,
41(6), 583–591.
Emerson, J., Rosenfeld, M., McNamara, S., Ramsey, B.,
& Gibson, R. L. (2002). Pseudomonas aeruginosa and
other predictors of mortality and morbidity in young
children with cystic fibrosis. Pediatric Pulmonology,
34(2), 91–100.
Konstan, M. W., Butler, S. M., Wohl, M. E., Stoddard, M.,
Matousek, R., Wagener, J. S., et al. (2003). Growth
and nutritional indexes in early life predict pulmonary
function in cystic fibrosis. Journal of Pediatrics, 142(6),
624–630.
Koretz, R. L., Avenell, A., Lipman, T. O., Braunschweig, C.
L., & Milne, A. C. (2007). Does enteral nutrition affect
clinical outcome? A systematic review of the
randomized trials. American Journal of
Gastroenterology, 102(2), 412–429; quiz 468.
Lai, H. J., Cheng, Y., & Farrell, P. M. (2005). The survival
advantage of patients with cystic fibrosis diagnosed
through neonatal screening: Evidence from the United
States Cystic Fibrosis Foundation registry data.
Journal of Pediatrics, 147(3 Suppl), S57–S63.
Marshall, B. C., Butler, S. M., Stoddard, M., Moran, A. M.,
Liou, T. G., & Morgan, W. J. (2005). Epidemiology of
cystic fibrosis-related diabetes. Journal of Pediatrics,
146(5), 681–687.
Milla, C. E. (2007). Nutrition and lung disease in cystic
fibrosis. Clinics in Chest Medicine, 28(2), 319–330.
Milla, C. E., Billings, J., & Moran, A. (2005). Diabetes is
associated with dramatically decreased survival in
female but not male subjects with cystic fibrosis.
Diabetes Care, 28(9), 2141–2144.
Moudiou, T., Galli-Tsinopoulou, A., Vamvakoudis, E.,
& Nousia-Arvanitakis, S. (2007). Resting energy
expenditure in cystic fibrosis as an indicator of disease
severity. Journal of Cystic Fibrosis, 6(2), 131–136.
Peterson, M. L., Jacobs, D. R., Jr., & Milla, C. E. (2003).
Longitudinal changes in growth parameters are
correlated with changes in pulmonary function in
children with cystic fibrosis. Pediatrics, 112(3),
588–592.
Quinton, H. B., & O’Connor, G. T. (2007). Current issues
in quality improvement in cystic fibrosis. Clinics in
Chest Medicine, 28(2), 459–472.
Rolon, M. A., Benali, K., Munck, A., Navarro, J.,
Clement, A., Tubiana-Rufi, N., et al. (2001). Cystic
fibrosis-related diabetes mellitus: Clinical impact of
prediabetes and effects of insulin therapy. Acta
Paediatrics, 90(8), 860–867.
Schechter, M. S., Shelton, B. J., Margolis, P. A.,
& Fitzsimmons, S. C. (2001). The association of
socioeconomic status with outcomes in cystic fibrosis
patients in the United States. American Journal of
Respiratory and Critical Care Medicine, 163(6),
1331–1337.
Smyth, R., & Walters, S. (2007). Oral calorie supplements
for cystic fibrosis. Cochrane Database System Review,
2007(1), Art. No.: CD000406, DOI:10.1002/
14651858.CD000406.pub2.
Stallings, V. A., Stark, L. J., Robinson, K. A., Feranchak, A.
P., & Quinton, H. (2008). Evidence-based practice
recommendations for nutrition-related management of
children and adults with cystic fibrosis and pancreatic
insufficiency: Results of a systematic review. Journal of
American Diet Association, 108(5), 832–839.
Stark, L. J. (2003). Can nutrition counselling be more
behavioural? Lessons learned from dietary
management of cystic fibrosis. Proceedings of
Nutrition Society, 62(4), 793–799.
Stark, L. J., Jelalian, E., Powers, S. W., Mulvihill, M. M.,
Opipari, L. C., Bowen, A., et al. (2000). Parent and
child mealtime behavior in families of children with
cystic fibrosis. Journal of Pediatrics, 136(2), 195–200.
Stark, L. J., Mulvihill, M. M., Powers, S. W., Jelalian, E.,
Keating, K., Creveling, S., et al. (1996). Behavioral
intervention to improve calorie intake of children with
cystic fibrosis: Treatment versus wait list control.
Journal of Pediatric Gastroenterology and Nutrition,
22(3), 240–253.
Stark, L. J., & Powers, S. W. (2005). Behavioral aspects of
nutrition in children with cystic fibrosis. Current
Opinion on Pulmonary Medicine, 11(6), 539–542.
Steinkamp, G., & Wiedemann, B. (2002). Relationship
between nutritional status and lung function in cystic
fibrosis: Cross sectional and longitudinal analyses
from the German CF quality assurance (CFQA)
project. Thorax, 57(7), 596–601.
13