Normally, most of the nutrients we eat are absorbed through the walls of the small intestines. When this process is impaired, whether by infection, certain diseases such as cystic fibrosis (CF), chronic pancreatitis, celiac disease, and other GI diseases, or surgical resection of the pancreas or small bowel, the result is typically malabsorption of nutrients.
Failure to absorb nutrients, calories (from fat, protein, and carbohydrates) and vitamins and minerals, leads to a host of symptoms including:
- weight loss or slow growth in children
- bloating and abdominal discomfort
- foul-smelling stools that float or stick to the toilet bowl
Malabsorption also leads to a variety of complications related to specific nutritional deficiencies including:
- weakened immune system
- fat-soluble vitamin (A, E, D, K) deficiencies
- essential fatty acid deficiencies
When patients are not able to absorb sufficient calories and nutrients from food, they struggle to gain or maintain weight. In children, this can result in growth faltering where patients are too thin and do not reach their genetic potential for height. It can even delay pubertal development.
Persistent malabsorption in adults leads to chronic malnutrition. It can also accelerate the progression of various diseases and contribute to frailty in older patients. This weight loss and GI symptoms (diarrhea, abdominal discomfort) also decrease the quality of life for patients and their families.
This article explains how clinicians can incorporate medical nutrition to improve weight, height, and body mass index (BMI) in patients with cystic fibrosis who, if not treated, will experience the ill effects of chronic malabsorption. By helping patients enhance dietary fat absorption and ingest a healthy source of fat calories, clinicians can guide patients to meet and maintain nutritional and weight goals.
Malabsorption in cystic fibrosis
CF is a progressive, genetic disease characterized by abnormalities in the transport of chloride and the flux of water across cell membranes. This leads to the mucous in various organs to become thick and sticky.
Although many think of CF as a pulmonary disease, it actually affects many other regions of the body, including the pancreas. The pancreas is the source of digestive enzymes that help break down food in the intestines. Malfunction of the pancreas in patients with CF leads to pancreatic insufficiency (PI).
About 85% of patients with CF have PI.1 It is caused by a build-up of mucus and fibrosis in the pancreas that halts the production and release of digestive enzymes and the needed bicarbonate fluid. These enzymes are essential for the body to digest and absorb food calories and nutrients, including dietary fatty acids and fat-soluble vitamins, that are essential for human health.
Malabsorption: an ongoing challenge in cystic fibrosis
Malabsorption is an ongoing challenge for patients with CF. Low weight, lack of height accretion, and unintentional weight loss cause numerous health concerns in these individuals. This is particularly consequential for children who are still growing, those patients preparing for a lung transplant, and pregnant women.
Patients who are able to achieve a higher BMI may have better outcomes and a slower rate of progression of their disease. In fact, a nutritional therapy plan that includes a higher energy and fat intake compared to generally healthy people of similar age and sex supports better pulmonary function and survival in these patients.2
It is vital that clinicians address the weight status in adults and weight and height patterns in children as a component of treatment. A healthier weight helps patients more successfully manage their CF.3 Therefore, it is important that clinicians caring for them consider how to effectively optimize nutritional status and support the quality of life for patients and families.
Current treatment for pancreatic insufficiency
Treatment of chronic malabsorption syndromes due to PI focuses on helping patients get the most out of the calories they consume.
Current approaches for nutrition care of patients with CF and PI include:
- lifelong pancreatic enzyme replacement therapy (PERT)
- increased food intake and calorie-dense/high-fat diet over a lifetime
- nutritional supplements by mouth or feeding tube when indicated
Each of these treatments has its limitations.
Pancreatic enzyme replacement therapy
PERT is the foundation of malabsorption treatment in CF and PI. It involves the use of oral medications that contain enzymes to replace what the pancreas is no longer able to produce or release. The enzymes replaced in PERT include:
- proteases that will digest protein
- amylases that digest carbohydrates
- lipases to digest fat
More complete digestion of these essential food components helps improve their absorption and prevents the symptoms of malabsorption.
PERT is key to malabsorption treatment in CF and PI. It is effective in helping patients absorb about 85% of the dietary fat found in their food.4
That said, these patients are still not able to utilize about 10% to 15% of dietary fat, which results in a constant loss of calories, essential fatty acids, and fat-soluble vitamins.
Increased intake of high fat, calorie-dense foods
The increased food intake required to reach an adequate number of nutrient-rich calories for patients with PI is substantial. Clinicians reommend that patients with CF eat more food. However, it is actually quite difficult to overeat on a regular basis in order to compensate for malabsorption.
To eat beyond your appetite is harder than you may think. This is particularly true for children who are just learning about hunger and satiety.
Increased calorie and fat intake can be achieved through the use of general nutritional supplements such as shakes, drinks, and snack bars. These products provide more calories but do not address the problem of reduced nutrient absorption.
The other consideration with this approach is that it takes patients away from their regular eating routines of enjoying usual foods and family meals. The same is true when supplementation takes the form of bolus or overnight tube feeding regimens.
These options limit the patient’s control, flexibility, and enjoyment of eating.
Currently, about 40% of patients with CF across the U.S. are utilizing some form of oral supplementation. Another 10% require a gastrostomy for supplemental tube feeding.5
New medical nutrition therapy is now available
A new advanced nutrition therapy, Encala (from Envara Health) is a medical food composed of a structured lipid matrix and active ingredients of lysophosphatidylcholine (LPC), monoglycerides, and essential fatty acids. It is a taste-neutral mixable powder that provides highly absorbable fat calories and essential fatty acids. It helps the body absorb fat and fat-soluble vitamins.
As a plant-based, gluten, and dairy-free source of fat calories, Encala helps patients meet and maintain nutritional and weight goals. Further, it allows patients to fit into their family’s preferred dietary patterns by mixing Encala into their favorite foods and beverages throughout the day. This approach offers an improvement for most patients over the available conventional therapies described above.
What research tells us about Encala
A recently published, NIH-sponsored study examined 66 children with CF and persistent fat malabsorption.6 It found that Encala was a safe, well-tolerated, and effective treatment. It improved dietary fat absorption, weight gain, and fatty acid status as compared to a placebo-treated group in a 3-month double-blind, randomized, placebo-controlled trial (a gold standard for intervention research).7 This report was based on the full scale of study (110 children, over 12-month treatment). 8
Here is a summary of the findings:
- Encala fat was well absorbed in the study subjects. All had CF and PI in which pancreatic-derived lipase (the enzyme needed to digest fat) and liver-derived bile acids (needed for fat absorption) are missing or greatly reduced. Improved fat absorption resulted in better clinical outcomes including improvements in
- weight and height
- nutritional laboratory measures
- overall dietary fat absorption
- The children and young adults, aged 5 to 19 years old, were able to mix Encala powder with their favorite foods and drinks across the day. They and their families embraced the treatment because it allowed flexibility and choice in the diet.
- Encala treatment may be considered before the initiation of tube feeding in patients with malnutrition who need intervention. About 10% of the patients in the study already had feeding tubes, a treatment that is not undertaken lightly. Encala proved successful and well-accepted when it was incorporated into the tube feeding regime. Absorption was related to better clinical outcomes including improvements in weight, height, BMI, and nutritional lab values. With this experience and published results, Encala may now be routinely considered to help patients with oral supplementation and feeding tubes achieve their weight goals sooner. This will likely support the successful transition from tube feeding back to regular foods, thus decreasing the duration, burden, and costs of tube feeding therapy in patients with CF and PI.
What about other patients with malabsorption due to pancreatic insufficiency?
Even if you do not treat patients with CF and PI, this research demonstrates how effective Encala is in addressing the fat malabsorption problem in one of the most clinically challenging populations. While it is encouraging to see the success of Encala in patients with CF, there are potential benefits for other patient populations with conditions related to reduced pancreatic function, fat malabsorption, and poor nutritional status, including those with the following conditions:
- chronic pancreatitis and celiac disease, common non-CF diseases with a high risk for fat malabsorption
- surgical pancreas removal for cancer and non-cancer conditions
- rare conditions including Shwachman-Diamond, Johanson-Blizzard, Pearson marrow-pancreas syndromes
- other GI-related conditions with inflammation or chronic infection
In addition, Encala is calorically dense and well absorbed. It may be considered for the nutritional support of patients with the following conditions:
- inadequate food/calorie intake including the frail elderly who often do not feel like eating enough food
- unintentional, disease-related weight loss, including those in treatment for
- heart failure
- renal failure
This new calorie-dense treatment is something that can be offered to maximize the benefits of the food our patients are ingesting.
The bottom line on medical nutrition therapy for malabsorption
Encala is a new medical nutrition therapy that serves both as a source of healthy, high-calorie fat as well as a facilitator of absorption of foods and snacks that are consumed with the product. The use of the intervention is simple, effective, and minimally disruptive to typical, preferred dietary patterns.
Encala can potentially have a positive, broad impact on the treatment of a range of conditions. It is important to stay informed about what is available so your patients with poor growth or unintentional weight loss can thrive again.
- Singh, VK, and Schwarzenberg, SJ. 2017. Pancreatic Insufficiency in Cystic Fibrosis. Journal of Cystic Fibrosis, Vol. 6, Suppl. 2, Nov., accessed via https://www.cysticfibrosisjournal.com/article/S1569-1993(17)30813-5/fulltext
- Physicians Committee for Responsible Medicine’s Guide to Clinicians. Accessed via https://nutritionguide.pcrm.org/nutritionguide/view/Nutrition_Guide_for_Clinicians/1342064/all/Cystic_Fibrosis
- Cystic Fibrosis Foundation. Healthy High-Calorie Eating. Accessed via https://www.cff.org/Life-With-CF/Daily-Life/Fitness-and-Nutrition/Nutrition/Getting-Your-Nutrients/Healthy-High-Calorie-Eating/
- Calvo-Lerma, J, Martínez-Barona, S, et al. 2017. Revista Española de Enfermedades Digestivas, versión impresa ISSN 1130-0108, Vol.109, No.10, Madrid, Oct., accessed via http://scielo.isciii.es/scielo.php?script=sci_arttext&pid=S1130-01082017001000003
- Cystic Fibrosis Foundation. 2019 Patient Registry Annual Data Report. Accessed via https://www.cff.org/Research/Researcher-Resources/Patient-Registry/2019-Patient-Registry-Annual-Data-Report.pdf
- Stallings, VA, Tindall, AM, et al. 2020. Improved residual fat malabsorption and growth in children with cystic fibrosis treated with a novel oral structured lipid supplement: A randomized controlled trial. PLOS ONE 15(9): e0239642. Accessed via https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0232685
- Misra, S. 2012. Randomized double-blind placebo control studies, the “Gold Standard” in intervention-based studies. Indian J Sex Transm Dis AIDS. Jul-Dec; 33(2): 131–134, accessed via https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3505292/
- Stallings VA, Schall JI, et al. Effect of Oral Lipid Matrix Supplement on Fat Absorption in Cystic Fibrosis: A Randomized Placebo-Controlled Trial. J Pediatric Gastroenterology and Nutrition. 2016; 63: 676-680.
Financial disclosure: Dr. Stallings is the founder and a medical consultant to Envara Health, the company that makes Encala. She was the principal investigator on the NIH cystic fibrosis research study described in the article.