With the increasing survival rate of people with developmental disabilities into adulthood and later life, nutritional support and feeding of these individuals frequently becomes a critical problem which must be addressed by their caregivers and healthcare providers. Problems surrounding mealtimes include difficulty with the mechanisms of feeding as well as medical complications including aspiration and gastrointestinal dysmotility. No comprehensive guidelines exist to aid caregivers and healthcare providers regarding the issues in feeding and nutrition in this population. We offer an algorithmic approach to the nutrition-related problems of aspiration, laborious meals and mealtime refusal, choosing the best route for tube feeding, and when to return patients with developmental disabilities back to oral feeding.
People with intellectual and developmental disabilities (IDD) comprise 1.5-2.5% of the U.S. population (Morstad, 2012; Schalock et al., 2010), which amounts to 4.6 to 7.7 million people living in the United States (Larson, 2000; Morstad, 2012). These individuals with IDD often experience difficulties and complications with oral feeding. Studies have found that 15 to 30% of people with IDD have mealtime difficulties (Ball et al., 2012; Malone, Sharif, & Glennon-Slattery, 2015; Matson & Kuhn, 2001). Feeding difficulties are present at all levels of disability, and current figures may be underestimations (Gal, Hardal-Nasser, & Engel-Yeger, 2011). The prevalence of feeding difficulties may be as high as 80% in those with severe intellectual disability (Ahearn, Castine, Nault, & Green, 2001).
Feeding difficulties can be due to many causes that include behavioral, environmental, neural and physical etiologies. Ball et al. (2012) described multiple problems surrounding mealtimes, including impaired ability to suck, chew, and swallow, leading to undernutrition and lengthy feeding times, contributing to food refusal. Feeding difficulties also include gastroesophageal reflux, delayed stomach emptying, rumination, vomiting, lost appetite, and food selectivity in regard to feeder, temperature, texture, environment, etc. (Gravestock, 2003; Malone et al., 2015; Matson & Kuhn, 2001).
Nutritional support frequently becomes one of the most challenging medical problems facing these individuals, with the potential for greatly increased morbidity and mortality. Lauer (2013) described a relative risk of death from aspiration for individuals with developmental disabilities to be 21.1 compared to that of the average U.S. adult. The decisions of whether to place a nasogastric (NG) or nasal-jejeunal (NJ) tube, or to place and start gastrostomy (G) or jejunstomy (J) tube feedings, and when to allow a patient to return to oral feeds, can make a critical difference in patient outcomes. Providing nutrition via tube feeding can be life-saving when oral feeding is not well-tolerated. Scolapio (2007) described decreased aspiration risk in people with developmental disabilities and enteral feeding. There is strong evidence to support an immediate decrease in pneumonia risk after G tube placement (Gray & Kimmel, 2006), although there continues to be a risk of aspiration (Mizock, 2007). Pharmacological therapies have been shown to help reduce the risk of aspiration in these patients (Pareek et al. 2007). In some instances, however, patients with gastric tube feedings can continue to experience aspiration, leading to the decision to institute G-J tube feeding (Cogen & Weinryb, 1989).
These complex feeding issues often necessitate physicians, nurses or other medical staff to make decisions about the most ethical and safe methods of caring for people with IDD. Many individuals with developmental disabilities may not even be referred to a specialist for advice on feeding (Ball et al., 2012). A systematic, evidence-based model for diagnosis and management of feeding difficulties in adults with IDD is needed (Gravestock, 2003). More research needs to be done to determine the best method of managing feeding issues in people with IDD in order to provide the best care and decrease morbidity and mortality (Gantasala, Sullivan, & Thomas, 2013; Groce et al., 2014; Robertson, Chadwick, Baines, Emerson, & Hatton, 2017). In this paper, we provide algorithms based on experience in a large government-run facility in an effort to standardize the management of feeding difficulties in adults with IDD. Our goals included improving patient outcomes and facilitating future research in this area.
We developed these algorithms for a population of full-time residents of a state-operated facility for people with IDD. All of the 350 residents were initially evaluated and monitored for signs and symptoms of aspiration, laborious meals, meal-time refusals, and nutritional compromise. Based on the results of these evaluations and ongoing monitoring, we developed specialized dining plans and modified the means by which nutritional support was provided.
Residents requiring specialized dining plans had neurologically based dysphagia and/or gastrointestinal dysmotility of developmental origin. Although we did not include any patients with specific progressive neurological disorders in the study, the debilitating effects of aging and acute illness required monitoring for the progression of disability affecting swallowing, gastroesophageal reflux (GER), other gastrointestinal dysmotility, and state of nutrition. The facility's Physical and Nutritional Management Team (PNMT) developed individualized dining plans. This interdisciplinary team was comprised of speech-language therapists (swallowing specialists), occupational therapists, physical therapists, registered dietitian/nutritionists, registered nurses with certification in IDD, behavior analysts, a neurodevelopmental disabilities physician, the patient's primary care physician, and a consulting gastroenterologist. Members of the team conducted periodic inservice training for all direct care staff regarding the signs and symptoms of dysphagia/aspiration. In addition, they conducted person-specific periodic mealtime monitoring to assure adherence to dining plans. When a specialized dining plan could no longer meet the nutritional and safety needs of a patient, the PNMT considered enteral feeding. The PNMT also monitored all patients receiving enteral nutrition for the possibility of returning to oral feeding, the need for alterations in diet, timing of feeds, and the possible need for altering the site or type of enteral tube.
As a result of the process described above and in consideration of the inherent risks and complications associated with tube feeding, our team recognized the need for a systematic approach to medical decision-making in this arena. We developed these algorithms and standardized approaches to suspected aspiration and dysphagia and refined them over time through the clinical processes of morbidity and clinical outcomes review, case study, expert panel review, and consensus.
Assessment and Monitoring
Many factors must be taken into consideration when choosing the safest and most effective method of feeding. This complex process of data gathering and decision-making must follow a multidisciplinary approach. Although feedings in institutional settings and specialized diets are ordered by physicians, their implementation and the non-medical data needed in order to make decisions regarding feeding and nutrition come, by necessity, from a variety of sources. This must include the person(s) who feed(s) the individual with IDD and provide the observations of what occurs during feeding, whether they are employees or family members. Decisions regarding feeding tube placement must be made with the involvement of family members and surrogate medical decision makers, and must address ethical considerations including the burden placed on the patient and the caregiver (Irazábal et al., 2012).
As described above, the clinical approach to assuring safe and effective nutritional support for people with significant dysphagia and/or gastrointestinal dysmotility presents the healthcare team with a complex array of factors that must be taken into consideration. With the goal of promoting a more standardized approach to this clinical problem we offer the following algorithms.
Each algorithm references similar building blocks of assessment and monitoring as described in the accompanying tables. The algorithms are organized based on the clinical presentations that may be encountered indicating the need for clinical assessment and alteration in mealtime management/dining plan, or indications for enteral feeding. Algorithms for deciding on the type of tube feeding that is indicated and consideration for return to oral intake after tube placement are also offered.
The algorithm guiding a clinical approach to aspiration is seen in Figure 1. Aspiration is a problem that complicates the management of dysphagia and/or significant gastrointestinal dysmotility. The signs and symptoms of aspiration range from overt, including coughing and gagging, to more insidious, such as reddening of the face or head and neck posturing. Clinical presentations of aspiration include coughing, gagging, wet vocal quality, gurgle, spillage of food/fluid, facial flushing, sweating, tearing, wheezing, bronchitis, respiratory infections/fever (silent aspirator), irregular breathing, and head extension patterns. When aspiration is suspected, its presence must be documented through imaging, such as a modified barium swallow (MBS), or fluorescent endoscopic evaluation of swallowing (FEES). A clinical mealtime assessment should include detailed examination of the patient as well as the surroundings and actions of the caretaker aiding in the feedings; this will ensure that other non-surgical solutions will not be overlooked. Table 1 details the many components of this assessment, ranging from observation of swallowing mechanics to mealtime environment. Medical/nursing/behavioral reviews (Table 2) should evaluate for conditions that may also contribute to aspiration. Options for intervention such as mealtime modifications stem directly from the problems revealed during the clinical mealtime assessment and may significantly improve morbidity as well as mortality in terms of repeat episodes of aspiration and undernutrition (Table 3). It is especially important to monitor patients who have a history of aspiration and to be mindful of caregiver compliance with mealtime modifications as proper training on an individual basis for each patient is crucial (Table 3). Ultimately, enteral tube feeding may be pursued for those who continue to have aspiration after non-surgical modifications are exhausted. It should be noted that when emergency indications for non-oral feeding arise, tube placement should not be postponed (Figure 1).
Emergent indications for non-oral feeding include
documentation by MBS of frank aspiration (one tsp) on the safest texture and presentation available;
a life-threatening episode of aspiration illness where mealtime/oral motor assessment or MBS indicates unacceptable risks for continuing oral intake at the given time;
severe malnutrition where current mealtime assessments demonstrate an inability to consume adequate calories for nutritional repletion and where temporary NG feeding cannot be tolerated;
and finally, when there is an inability to maintain hydration where current mealtime assessments demonstrate an inability to consume adequate fluids to maintain hydration and where temporary NG feeding cannot be tolerated and IV fluid maintenance is unacceptable.
Laborious meals are addressed in Figure 2. Laborious meals can present clinically as fatigue at the end of a meal, long pauses between bites increasing the length of mealtime, needing to take frequent breaks, and a high percentage of the individual's day devoted to intake in order to maintain their nutritional status. When laborious meals threaten nutritional security, it is an indication for further assessment and possible changes to a patient's nutritional strategy. While mealtime assessment and corresponding modifications can be made similar to aspiration, there are many medical conditions that particularly lend themselves to mealtime fatigue, such as depression, anemia, and cardiac disease. Patients with IDD must be screened for medical factors before mealtime problems are attributed to their neurological deficits. A systematic medical, nursing, and behavioral review must therefore be carried out prior to proceeding with modifications or enteral tube placement (Table 2). If aspiration or behavioral factors are identified and addressed, ongoing monitoring for continued problems is indicated (Table 4). Surveillance for emergent indication for non-oral feeding is also required (as discussed above).
Meal refusal is addressed in Figure 3. In patients with limited ability to communicate, a pattern of refusing meals is an important sign that they may be having difficulty with their nutritional support. An initial assessment to determine the root cause of a patient's mealtime refusal of feeds can result in dining plan, medical, or behavioral modifications to address these causes (Tables 1, 2, & 3). The medical and nursing review of conditions that may predispose to feeding difficulties, such as gastroesophageal reflux disease (GERD), medication effects, and seizure disorders, is paramount with this presentation (Table 3). Consultation for a thorough dental examination may also be helpful as oral/dental pathology can be painful. Patients who cannot communicate or otherwise indicate that they are experiencing pain with eating may avoid food in order to minimize their pain. After modifications are instituted, it is necessary to monitor for the need for reassessment (Table 4) and the possibility of the emergent need for non-oral feeding (as discussed above).
Enteral Tube Placement
When dining plan modifications fail, or there are emergent indications for non-oral feeding, the patient is referred for enteral tube placement (Figure 4). Decisions must be made regarding the site and type of tube feeding that is indicated. In emergent situations, nasogastric or intravenous nutrition can support nutrition for a short time. Radionuclide gastric emptying testing can help determine whether gastric tube or jejunal tube placement will be better tolerated. Gastric emptying times and esophageal manometry can provide data to guide decision making regarding the need for anti-reflux surgery and/or a gastric outlet procedure. The results of these tests may prevent a failed G-tube trial. There are situations, however, where enteral tube feeding is not tolerated and/or symptoms persist (Figure 4). Severe, persistent gastroesophageal reflux disease (GERD) with gastrointestinal (GI) bleeding or recurrent aspiration, gastroparesis with high tube feeding residuals or increasing discomfort for the patient, and dumping syndrome are some of the conditions for which further surgical options may need to be pursued, such as anti-reflux surgery, gastric stimulators, or G-J/J tubes (Table 5). Keeping in mind that aspiration can still occur after G and/or J-tube placement, caregivers should be trained to be vigilant for its signs and symptoms despite tube placement.
Return to Oral Feeding
Figure 5 describes the process of evaluating a patient's readiness to return to oral feeding. Instances when it may be in the best interest of the patient to return to oral feedings after a period of enteral tube feeds are often overlooked. After an acute illness or restoration of nutritional balance, some patients may be capable of returning to oral feeding. This is an important aspect of patient care, as enteral tube feeding carries its own risks. In these situations, a mealtime assessment (Table 1) should be performed to develop an individualized oral habilitation and dining plan. During this transition, careful monitoring is required for patient safety and to determine whether or not the patient is able meet adequate nutritional goals once entirely transitioned back to oral feeds. In instances where a complete return to oral feeding does not meet nutritional goals, supplementation by enteral tube feeds for additional caloric intake may be required. For those individuals for whom mealtime modifications are unsuccessful, therapeutic oral tasting with or without an oral habilitation plan may be enjoyable for the individual and may also lead to reassessment for return to oral feeding at a later date.
Assuring safe and adequate nutrition for individuals with IDD who have dysphagia and/or significant gastrointestinal dysmotility presents a medically complex clinical picture that requires input from many sources when deciding on a proper course of action. A reasonable approach begins with symptom presentation and proceeds through a detailed and thorough assessment, leading to an individualized treatment plan and ongoing monitoring. We have attempted to provide the clinician with a systematic, algorithmic approach to this process that aids decision making and standardizes treatment.
We recognize that our algorithms may have limitations. These algorithms were largely based on the best course of action found in a large government-run facility with 24-hour care and easily accessible healthcare professionals, laboratory testing, and imaging. In recent years, the average daily population of large state residential settings for individuals with intellectual or developmental disability has declined to just over 30,000 people (Larson et al., 2012). Further research is needed to determine the generalizability to other types of settings for people with IDD where healthcare access may differ, as the majority of these individuals live either in smaller settings or with their family.
Further research examining patient outcomes based on these algorithms will provide validation and recommendations for refinement. Improving outcomes for people with IDD experiencing nutritional compromise and/or physical debilitation from aspiration and gastrointestinal dysmotility will contribute greatly to decreasing the cost of care and improving quality of life and longevity for this population.
The authors would like to thank the Arlington Developmental Center Physical and Nutritional Management Team, and Catherine McBride at the University of Louisville for help with manuscript preparation.
This study was covered by an Institutional Board Review from the University of Tennessee.