Cystic Fibrosis: Part 1 The assignment requests that I describe how I went about locating the information needed to make ethical evidence based decisions about the nutritional care of a patient with Cystic Fibrosis (CF). In order to achieve this, I used Google (available through the internet) and EBSCO discovery (available through my LSU PAWS account) search engines to locate the scholarly developed articles associated with CF and the nutrition of those suffering from the disease. I conducted my search over a period of days from 1/27/2015 thru 2/1/2015. During my searches, I was careful to look at the search data returned in order to insure that the result was recent enough to be applicable to the current knowledge base on the subject, and that it was from a reputable source. These included such sources as peer reviewed scholarly journals, textbooks, or reputable organizations with special knowledge in the field, such as the Cystic Fibrosis Foundation (CFF). I also considered authors and sources outside of the U.S., as long as they were associated with these same reputable entities. While using the Google search engine, I used the following key terms and phrases in order to locate the most current and up to date information I could about CF and the nutritional aspects of patient care associated with CF: Cystic fibrosis pathophysiology Cystic fibrosis Cystic fibrosis and pediatrics Nutrition practices for cystic fibrosis Nutrition guidelines cystic fibrosis Nutrition and cystic fibrosis Cystic fibrosis nutrition Cystic fibrosis update on nutritional complications I then used Google to access my LSU PAWS account to access the LSU Library electronic databases. I used the EBSCO Discovery service, and the key terms and phrases shown above in order to locate the most current and up to date information I could about CF. Finally, I used the GOOGLE search engine to access my AND account in order to see if there was anything of value in the EAL section of the AND. Once there, I used the same key terms and phrases used with the previous two search attempts in order to locate the most current and up to date information I could about CF and the nutritional aspects of patient care associated with CF. Unfortunately, I was surprised to find that the EAL located on the AND website did not return any results associated with my query. That being said, I proceeded to make use of the resources I located during the Google and EBSCO searches. The following is a step-by step description of how I located my reference material for this assignment: 1. I Googled “cystic fibrosis pathophysiology “ 1.1. clicked on “Chapter 7 Cystic Fibrosis” and located the following:
Transcript
Cystic Fibrosis: Part 1
The assignment requests that I describe how I went about locating the information needed to make ethical evidence based decisions about the nutritional care of a patient with Cystic Fibrosis (CF). In order to achieve this, I used Google (available through the internet) and EBSCO discovery (available through my LSU PAWS account) search engines to locate the scholarly developed articles associated with CF and the nutrition of those suffering from the disease. I conducted my search over a period of days from 1/27/2015 thru 2/1/2015. During my searches, I was careful to look at the search data returned in order to insure that the result was recent enough to be applicable to the current knowledge base on the subject, and that it was from a reputable source. These included such sources as peer reviewed scholarly journals, textbooks, or reputable organizations with special knowledge in the field, such as the Cystic Fibrosis Foundation (CFF). I also considered authors and sources outside of the U.S., as long as they were associated with these same reputable entities.
While using the Google search engine, I used the following key terms and phrases in order to locate the most current and up to date information I could about CF and the nutritional aspects of patient care associated with CF:
Cystic fibrosis pathophysiology Cystic fibrosis Cystic fibrosis and pediatrics Nutrition practices for cystic fibrosis Nutrition guidelines cystic fibrosis Nutrition and cystic fibrosis Cystic fibrosis nutrition Cystic fibrosis update on nutritional complications
I then used Google to access my LSU PAWS account to access the LSU Library electronic databases. I used the EBSCO Discovery service, and the key terms and phrases shown above in order to locate the most current and up to date information I could about CF. Finally, I used the GOOGLE search engine to access my AND account in order to see if there was anything of value in the EAL section of the AND. Once there, I used the same key terms and phrases used with the previous two search attempts in order to locate the most current and up to date information I could about CF and the nutritional aspects of patient care associated with CF. Unfortunately, I was surprised to find that the EAL located on the AND website did not return any results associated with my query. That being said, I proceeded to make use of the resources I located during the Google and EBSCO searches.
The following is a step-by step description of how I located my reference material for this assignment:
1. I Googled “cystic fibrosis pathophysiology “
1.1. clicked on “Chapter 7 Cystic Fibrosis” and located the following:
D. Schraufnagel, Chapter 7, “Cystic fibrosis” in Breathing in America: Diseases, Progress, and Hope, The American Thoracic Society, 2010. http://thoracic.org/education/breathing-in-america/resources/chapter-7-cystic-fibrosis.pdf. Accessed January 27, 2015.
1.2. clicked on “Pathophysiology of cystic fibrosis and drugs used in associated digestive tract diseases” and located the following:
Haack A, Aragão G, Novaes M. Pathophysiology of cystic fibrosis and drugs used in associated digestive tract diseases. World Journal Of Gastroenterology: WJG [serial
online]. December 14, 2013;19(46):8552-8561. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3870500/pdf/WJG-19-8552.pdf. Accessed January 27, 2015.
2. I Googled “cystic fibrosis”
2.1. clicked “Cystic Fibrosis – the Merck Manuals” and located the following:
Cystic Fibrosis. Merck Manuals websitehttp://www.merckmanuals.com/professional/pediatrics/cystic_fibrosis_cf/cystic_fibrosis.html. Updated January 2014 by Beryl J. Rosenstein, MD. Accessed January 28, 2015
2.2. clicked “What Is Cystic Fibrosis? - NHLBI, NIH” and located the following:
What Is Cystic Fibrosis? - NHLBI, NIH. National Institutes of Health. National Heart, Lung, and Blood Institute website.http://www.nhlbi.nih.gov/health/health-topics/topics/cf . Updated December 26, 2013. Accessed January 28, 2015.
2.3. clicked “Cystic Fibrosis Foundation” and found the following:
2.3.1. http://www.cff.org/ where I clicked “treatments”, clicked “CF care guidelines”
2.3.1.1. http://www.cff.org/treatments/CFCareGuidelines/ then clicked on “Nutrition/GI” and found the following:
CF Care Guidelines - Nutrition/GI. Cystic Fibrosis Foundation website. http://www.cff.org/treatments/CFCareGuidelines/Nutrition/ . Updated 7/19/2012. Accessed January 28, 2015.
2.3.1.2. While in the Nutrition/GI section, I clicked the reference on “Evidence Based Care Guidelines” but could not access the document so I copied the title “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 the American Dietetic Association 2008;108(5):832-839” and put it in the Google search box.
2.3.1.3. I then clicked on the primeinc.org result and found the following:
Stallings V, Stark L, Robinson K, Feranchak A, Quinton H. Research: 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 The American Dietetic Association [serial online]. January 1, 2008;108:832-839. http://primeinc.org/downloads/cfnutrition08.pdf . Accessed January 28,2015
3. I Googled “Cystic fibrosis and pediatrics”
3.1. I clicked on “Consensus Report on Nutrition for Pediatric Patients With Cystic Fibrosis” and located the following:
Borowitz D, Baker R, Stallings V. Consensus report on nutrition for pediatric patients with cystic fibrosis. Journal Of Pediatric Gastroenterology And Nutrition [serial online]. September 2002;35(3):246-259. http://www.cff.org/UploadedFiles/treatments/CFCareGuidelines/Nutrition/
Consensus-Report-on-Nutrition-for-Pediatric-Patients-with-CF-JPGN-Sep-2002.pdf . Accessed January 30, 2015
4. I then searched Ebsco Discovery (accessed from my LSU PAWS account under library resources under databases) for “Nutrition practices for cystic fibrosis”
4.1. I located the article “A survey of nutrition practices for patients with cystic fibrosis”, but had to access the document through the “find at LSU” link on the side of the page. This eventually led me to the following:
O'Brien C, Harden H, Com G. A survey of nutrition practices for patients with cystic fibrosis. Nutrition In Clinical Practice: Official Publication Of The American Society For Parenteral And Enteral Nutrition [serial online]. April 2013;28(2):237-241. http://ncp.sagepub.com.libezp.lib.lsu.edu/content/28/2/237.full.pdf+html . Accessed January 30, 2015.
5. I proceeded then to Google “nutrition guidelines cystic fibrosis”
5.1. I clicked Dietary intakes in adult patients with cystic fibrosis–do they achieve guidelines?, then clicked on PDF file to the right of the webpage to find the following:
White H, Morton A, Peckham D, Conway S. Dietary intakes in adult patients with cystic fibrosis–do they achieve guidelines?. Journal Of Cystic Fibrosis [serial online]. January 1, 2004;3:1-7. http://www.cysticfibrosisjournal.com/article/S1569-1993(03)00122-X/pdf . Accessed January 31, 2015.
6. I then Googled “nutrition and cystic fibrosis”
6.1. clicked on “Nutrition in patients with cystic fibrosis: a European Consensus” where I found the following:
Sinaasappel M, Stern M, Döring G, et al. Nutrition in patients with cystic fibrosis: a European Consensus. Journal Of Cystic Fibrosis [serial online]. June 2002;1(2):51. http://www.elsevier.com/__data/promis_misc/2002.pdf. Accessed January 31, 2015.
7. I proceeded then to switch back to the Ebsco Discovery database and searched for “Cystic fibrosis nutrition”
7.1. I then clicked “Digestive system dysfunction in cystic fibrosis: Challenges for nutrition therapy” where I then selected the “find at LSU” link in order to locate the following:
Li L, Somerset S. Digestive system dysfunction in cystic fibrosis: Challenges for nutrition therapy. Digestive And Liver Disease [serial online]. n.d.;46(10):865-874. http://www.sciencedirect.com.libezp.lib.lsu.edu/science/article/pii/S1590865814004241. Accessed February 1, 2015
8. Finally, I Googled “Cystic fibrosis update on nutritional complications”
8.1. I then clicked on “Cystic fibrosis: An update for clinicians. Part 1: Nutrition and gastrointestinal complications” This led me to the abstract for the article, but I then clicked on the “Get PDF” link in order to access the following:
Haller W, Ledder O, Lewindon P, Couper R, Gaskin K, Oliver M. Cystic fibrosis: An update for clinicians. Part 1: Nutrition and gastrointestinal complications. Journal Of Gastroenterology And Hepatology [serial online]. n.d.;29(7):1344-1355. http://onlinelibrary.wiley.com/doi/10.1111/jgh.12546/pdf. Accessed February 1, 2015.
The following is an interpretation of the whole of the literature that was located in the above detailed search effort. The intent of this interpretation is to combine the findings from the scientific literature in order to present a coherent compilation that can be used as an evidence based practice reference to develop nutritional treatment plans for patients suffering from cystic fibrosis. What follows is an overview of the disease and its pathophysiology. Afterwards the paper will address the specific macronutrients and micronutrients of primary concern in patients suffering from Cystic Fibrosis. Additionally, I intend to address applicable medications to improve nutritional status. Finally, the paper will address the subject of enteral feedings and details on the administration of such.
Cystic fibrosis, or CF, is an inherited disease and is the “most common, life-shortening genetic disease in Caucasians”1. The common symptoms associated with CF are “chronic cough, chronic diarrhea, and malnutrition”2. The symptoms associated with CF are due to the effect the disease has on the exocrine glands which then manifests itself primarily in the lungs and the gastrointestinal tract3. Those with CF are afflicted with mucus that is thick and sticky, which causes it to build up in the lungs where it blocks airways and causes infections4. Additionally, the thick and sticky mucus blocks the ducts in the pancreas, preventing the release of enzymes into the small intestine, which results in malabsorption of nutrients4. Studies have shown that special attention paid to the proper nutrition of people with CF leads to a longer, higher quality of life5. The understanding of the impact of good nutrition on people with CF has led the Cystic Fibrosis Foundation (CFF) to spearhead the development of evidence based practice recommendations for nutrition of people with CF6.
The recommendations in the aforementioned document reflect the understanding of the importance of nutritional intervention throughout a CF patient’s life. From infancy, at the first diagnosis of CF, through adulthood, a patient’s development and nutritional status must be monitored and managed in order to deliver the longest and highest quality of life7. Due to the effects of malabsorption, patients with CF require greater energy intake, especially from increased fat and protein content, and special monitoring of certain fat-soluble vitamins (i.e. A, D, E, & K)7 and certain minerals such as calcium, iron, zinc, and sodium7. Additionally, CF patients require special monitoring of bone health due to the disease’s effect on calcium and vitamin D uptake8. All these issues combine in order to present a picture that depicts the elevated risk that CF patients face for malnutrition and reduced clinical status9. Although there is no known cure for the disease, a great deal of work has been done to affect the life expectancy and life quality of CF patients. Nearly all of people who suffer from CF suffer from pancreatic insufficiency (PI)10. Due to this, PERT, or pancreatic enzyme replacement therapy, has become a well-established medication therapy used to improve the nutritional status of patients by improving the absorption of nutrients6, 11. For those who are still malnourished or at risk of nutritional failure, guidelines have been established for enteral feeding as well12.
From: “Evidence-Based Practice Recommendations for Nutrition-Related Management of Children and Adults with Cystic Fibrosis and Pancreatic Insufficiency: Results of a Systematic Review5.
From: Consensus Report on Nutrition for Pediatric Patients With Cystic Fibrosis7
As previously mentioned, the pathophysiology of CF that effects the need for additional energy intake requirements is from the malabsorption of nutrients resulting from severe reduction in pancreatic excretion because of duct pluggage by mucus. Additional energy requirements stem from recurring lung infections caused by the same mucus that occurs in the lungs as well. After synthesizing the information presented in all the documents reviewed in this effort, the majority of recommendations for energy intake landed on a range of 120%-150% of normal recommended intakes for age, sex, and BMI. This range falls within the values shown in the evidence based recommendation chart (figure 8) above6. It is worth noting that all the articles reviewed stressed that monitoring health and nutritional status at regular intervals is of utmost importance when determining the required energy intake. As weight and nutritional status fluctuates, altering the energy intake recommendation is also required. In terms of specific macronutrients, the specific recommendation for fat intake is 40% of diet content as opposed to <30% as is normally recommended. The additional fat serves a dual purpose. First of all, it is the most calorie dense nutrient, which helps achieve the additional energy intake requirements, and secondly it helps to supplement fat availability in the GI tract because of the reduced absorption caused by pancreatic insufficiency (PI). Additionally, elevated protein is recommended up to 200% of RDI for non-CF patients. The elevated protein is required to help preserve musculature, improve nutritional status, and establish normalized BMI. Elevated protein is required because PI leads to reduced protein absorption as well. Finally, with the recommendations followed as prescribed above, that would leave the balance of the calories to be made up of carbohydrate intake.
Because of pancreatic insufficiency (PI) and its effect on reducing the absorption of fat in the GI tract, the absorption of fat-soluble vitamins such as vitamin A, D, E, and K are effected as well. These vitamins are recommended for supplementation as per the chart in table 4 above7.
Additional micronutrients requiring supplementation (based on biochemical analysis) are calcium, iron, zinc, and sodium7. Calcium is recommended in response to low absorption of vitamin D and is called for to combat osteoporosis, and osteopenia7. Iron supplementation is recommended based on monitoring results because CF patients are at risk due to increased inflammation of tissues7. Zinc deficiencies are associated with reduced vitamin A absorption, and sodium supplementation is called for because one of the hallmark symptoms of CF is excess sodium loss through the skin7. PERT (pancreatic enzyme replacement therapy) is one nutritional status improvement medication that was universally referenced. PERT supplements work to increase the presence of the pancreatic enzymes in the GI tract that are reduced or eliminated in CF patients due to PI. Although the effectiveness of PERT supplementation is evidence based, he dosage levels are consensus based6. Be that as it may, the recommendations are 500-2500 units of lipase per kilogram of body weight per meal, or <10,000 units lipase per kilogram body weight per day, or <4,000 units lipase per gram of dietary fat per day6.
Tube based enteral feedings are presented as evidence based recommendations for supplemental nutrition for CF patients over the age of 26. The implementation of enteral feedings is recommended for only the most malnourished of patients, or those who have already experienced nutritional failure. The formulas used should be calorie dense (1.5-2.0 kcal/cc) with the application usually preferred to be at night so as to promote normal daytime eating patterns7. A total of 30-50% of energy intake requirements should be the initial goal of enteral feedings, with the application of tube placement being determined by patient tolerance and the expertise of the facility7.
References
1. D. Schraufnagel, Chapter 7, “Cystic fibrosis” in Breathing in America: Diseases, Progress, and Hope, The American Thoracic Society, 2010. http://thoracic.org/education/breathing-in-america/resources/chapter-7-cystic-fibrosis.pdf. Accessed January 27, 2015.
2. Haack A, Aragão G, Novaes M. Pathophysiology of cystic fibrosis and drugs used in associated digestive tract diseases. World Journal Of Gastroenterology: WJG [serial online]. December 14, 2013;19(46):8552-8561. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3870500/pdf/WJG-19-8552.pdf. Accessed January 27, 2015.
3. Cystic Fibrosis. Merck Manuals websitehttp://www.merckmanuals.com/professional/pediatrics/cystic_fibrosis_cf/cystic_fibrosis.html. Updated January 2014 by Beryl J. Rosenstein, MD. Accessed January 28, 2015
4. What Is Cystic Fibrosis? - NHLBI, NIH. National Institutes of Health. National Heart, Lung, and Blood Institute website.http://www.nhlbi.nih.gov/health/health-topics/topics/cf . Updated December 26, 2013. Accessed January 28, 2015.
5. CF Care Guidelines - Nutrition/GI. Cystic Fibrosis Foundation website.http://www.cff.org/treatments/CFCareGuidelines/Nutrition/ . Updated 7/19/2012. Accessed January 28, 2015.
6. Stallings V, Stark L, Robinson K, Feranchak A, Quinton H. Research: 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 The American Dietetic Association [serial online]. January 1, 2008;108:832-839. http://primeinc.org/downloads/cfnutrition08.pdf . Accessed January 28, 2015
7. Borowitz D, Baker R, Stallings V. Consensus report on nutrition for pediatric patients with cystic fibrosis. Journal Of Pediatric Gastroenterology And Nutrition [serial online]. September 2002;35(3):246-259. http://www.cff.org/UploadedFiles/treatments/CFCareGuidelines/Nutrition/Consensus-Report-on-Nutrition-for-Pediatric-Patients-with-CF-JPGN-Sep-2002.pdf . Accessed January 30,2015
8. O'Brien C, Harden H, Com G. A survey of nutrition practices for patients with cystic fibrosis. Nutrition In Clinical Practice: Official Publication Of The American Society For Parenteral And Enteral Nutrition [serial online]. April 2013;28(2):237-241. http://ncp.sagepub.com.libezp.lib.lsu.edu/content/28/2/237.full.pdf+html . Accessed January 30, 2015.
9. White H, Morton A, Peckham D, Conway S. Dietary intakes in adult patients with cystic fibrosis–do they achieve guidelines?. Journal Of Cystic Fibrosis [serial online]. January 1, 2004;3:1-7. http://www.cysticfibrosisjournal.com/article/S1569-1993(03)00122-X/pdf . Accessed January 31, 2015.
10. Sinaasappel M, Stern M, Döring G, et al. Nutrition in patients with cystic fibrosis: a European Consensus. Journal Of Cystic Fibrosis [serial online]. June 2002;1(2):51. http://www.elsevier.com/__data/promis_misc/2002.pdf. Accessed January 31, 2015.
11. Li L, Somerset S. Digestive system dysfunction in cystic fibrosis: Challenges for nutrition therapy. Digestive And Liver Disease [serial online]. n.d.;46(10):865-874. http://www.sciencedirect.com.libezp.lib.lsu.edu/science/article/pii/S1590865814004241. Accessed February 1, 2015
12. Haller W, Ledder O, Lewindon P, Couper R, Gaskin K, Oliver M. Cystic fibrosis: An update for clinicians. Part 1: Nutrition and gastrointestinal complications. Journal Of Gastroenterology And Hepatology [serial online]. n.d.;29(7):1344-1355. http://onlinelibrary.wiley.com/doi/10.1111/jgh.12546/pdf. Accessed February 1, 2015.
4. Nutrition Focused Physical Findingsa. Notes of recent hair lossb. Difficulty seeing at nightc. Greasy stools
5. Client Historya. Increased coughingb. Chronic pulmonary infectionsc. Diagnosed with Cystic Fibrosis and Osteopenia
Diagnosis
Narrative:
The patient presents herself not only with a known diagnosis of CF, but also with all the unfortunate classic symptoms. The diagnosis of osteopenia is no doubt the result of the pathophysiology associated with decreased absorption of fat-soluble vitamins (particularly vitamin D). Although she was not presented with a diagnosis of PI, its likelihood is evident. The increased coughing and history of chronic pulmonary infections are also supported by the pathophysiology of the disease and the way the mucus blocks airways and leads to infections. The decrease in appetite is common among people with CF, and is of utmost importance to this assessment. CF patients are normally underweight due to the disease’s effect on the GI tract, and additional reduction in energy intake is of great concern. The malodorous, greasy stools are a common symptom of the disease as well due to the increased fat content and undigested nutrient material that is consistent with CF. Finally, the recent manifestations of hair loss points to deficiencies in vitamins D and biotin, as well as iron, zinc, fat, and protein; while the reduction in night vision is indicative of vitamin A deficiency. Both of these symptoms are consistent with the underlying pathophysiology associated with CF.
In terms of the patients lab results, they are also consistent with the classic markers of CF. Below normal Serum vitamin A, and zinc are indicators of deficiencies in both which is common among people with the disease. Additionally, the less than normal hemoglobin and hematocrit results indicate anemia caused by iron deficiency, which is another common ailment among CF sufferers. On the bright side, however, her fasting glucose level was normal at 80mg/dL. This indicates that she is not suffering from diabetes, which unfortunately can be common in patients with CF.
PES Statements:
* Inadequate energy intake related to pathological and physiological causes that result in increased energy requirements as evidenced by failure to maintain appropriate weight and estimated energy intake from diet being less than the needed amount estimated or measured metabolic rate.
* Inadequate vitamin (A,E,D,K) intake related to physiological causes increasing nutrient needs (disease state and malabsorption) as evidenced by low levels of Serum A and D (Osteopenia), and presence of CF and accompanying malabsoprtion of fat soluble vitamins.
Interventions
Narrative continued:
According to the anthropometric data provided, and use of the CDC BMI percentile calculator (http://nccd.cdc.gov/dnpabmi/Calculator.aspx), the patient presents herself with a BMI of 14.6 and a BMI percentile of 16 among children of the same demographic. With these measures, she is considered at risk of nutritional failure7. Evidence based guidelines for children age 2-20 with CF recommend the use of BMI percentile as an indicator of health among the group6. Additionally, it is recommended that nutritional intervention be implemented in order to achieve a BMI percentile of >/= 50th percentile6. Based on the evidence based guidelines and protocols developed in conjunction with the Cystic Fibrosis Foundation, it is recommended to pursue the following6:
Increase their energy intake to be between 110-200% of the standard for health people of the same population
Implement a combined behavioral and nutritional intervention for weight gain
Nutritional supplementation (oral and enteral) Achieve optimal weight-for-age, and stature-for-age Apply proprietary pancreatic enzyme preparations for PI Maintain growth status by using a target of BMI>/=50th percentile
Her current energy intake is approximately 1200 kcal/day, which is far below the 1600 kcal/day that is the normal recommendation for non-CF people. Due to her raw BMI
and her BMI percentile, I recommend that her energy intake requirement be increased to 150% of normal recommendations or 2400 calories. This increase in intake is to be coupled with a requirement that 40% of the total energy intake be from fat, her protein requirement be more than doubled from the normal recommendations of 34 grams per day (per the CDC recommendations) to a level equating to 15% of total energy intake. The balance of her diet (45%) is to be made up of carbohydrates6,7.
The enteral feedings will require parental/caregiver intervention for application. The exact type of tube feeding apparatus will depend on the patient’s tolerance and the expertise of the center applying the method7. The initial recommendation calls for 30-50% of estimated energy requirements to be applied overnight by this method. Standard energy rich formulations (1.5-2.0 kcal/cc) are recommended for this purpose7.
Additionally, parents and caregivers will need to be involved in addressing PI through the implementation of PERT. PERT (pancreatic enzyme replacement therapy) supplements work to increase the presence of the pancreatic enzymes in the GI tract that are reduced or eliminated in CF patients due to PI. Although the effectiveness of PERT supplementation is evidence based, he dosage levels are consensus based6. Be that as it may, the recommendations are 500-2500 units of lipase per kilogram of body weight per meal, or <10,000 units lipase per kilogram body weight per day, or <4,000 units lipase per gram of dietary fat per day6.
Vitamin supplementation is to be as per evidence based recommendations shown in the chart below. The supplement levels shown here are requirements over and above the administration of an age appropriate multivitamin7. Additional supplementation of Iron, Calcium, and Zinc is to be applied as per established guidelines with an age appropriate mineral supplement7.
Age Vitamin A (IU) Vitamin E (IU) Vitamin D (IU) Vitamin K (IU)
>8 10,000 200-400 400-800 0.3-0.5
Interventions for PES statements
*Inadequate energy intake
-Recommend energy modified diet (ND 1.22) and schedule of food (ND 1.3) modification: 2400 kcal with 3 meals and 3 snacks per day to promote weight gain.
-Recommend feeding assistance with adaptive tube feeding device (ND 4.1): enteral tube feedings will require caregiver assistance to ensure that the proper amounts of nutrients are being consumed and that they are in the process of restoring their ability to eat independently.
-(E 1.4) Implement initial nutrition education on nutrition relationship to Cystic Fibrosis and further preventions of complications. Patient education handouts were used when discussing this topic with caregivers and were provided for them to take home at the end of the session.
-(C 2. 2) Engage in goal setting with client and caregivers to discuss plans of future weight gain and assist in any knowledge deficit that may be encountered when deciding on a path forward.
-(RC 1.4) Collaborate with other providers if necessary to assess the implementation of PERT (pancreatic enzyme replacement therapy) and any GI tract issues that may arise due to that. Patient provided with providers information and asked to update on nutritional status every 4 weeks.
*Inadequate vitamin intake
-Recommend vitamin modified diet (ND 1.29) to increase the consumption of vitamins A,E,D, and K meeting the needed requirements: A- 10,000; E- 200 to 400; D- 400 to 800; K-0.3 to 0.5.
-(E 1. 6) Recommend patient and caregivers follow up with RD at the facility in 4 weeks for evaluation and monitoring, revision of diet changes if need be, and to continue educating and counseling on the need for modifications in the diet to maintain a healthy lifestyle.
-(C 2.3) Provide the need for and instructions for self-monitoring a vitamin modified diet. Review self-monitoring logs to identify patterns that may be contributing to either an increase/decrease in vitamin intake and assist with any problems that may occur.
Monitoring/Evaulation
In terms of monitoring and evaluation, the Cystic Fibrosis Foundation recommends evaluating nutritional status every 3 months for girls with CF between the ages of 9 and 167.
Indicators Methods Criteria
Anthropmetrics Pt actual weight Weigh pt Acceptable wt for age of pt with CF
Intake Current energy and vitamin intake
Food Diary & 24 Hr recalls*
Goal: weight gain and vitamin intake recommended
Physical Findings Greasy stools Pt reports of persistent signs and symptoms
Healthy bowel movement
Biochemcial Defiecient levels of Serum Vitamin A/Zinc, and Hemoglobin/ Hematocrit
