Abstract
For children and adults with chronic disease, taking medication is often a critical component of daily life. An inability to swallow pills, particularly for children who are medically fragile, can add additional stress on the family and may reduce their quality of life. Limited ability to swallow pills may also restrict a person’s ability to participate in clinical trial research, or it may limit other options for managing disease symptoms. Families of young children will often resort to alternative means of presenting medications, such as crushing pills and hiding them in food; however, this method rarely works, and children may learn to be distrustful of food, which can lead to undesirable mealtime behaviors (Meltzer et al., 2006). Crushing pills can also lead to altered dosage, diluting the medication’s effectiveness.
Learning to swallow pills opens up more options for managing medical conditions and may reduce family stress (Meltzer et al., 2006). The purpose of this study was to evaluate the effectiveness of a brief, multifaceted pill-swallowing program that used evidence-based training methods for a specific population of people with Barth syndrome (BTHS).
Barth Syndrome
BTHS is a rare genetic disorder linked to the X chromosome that affects approximately 150–200 boys and men worldwide. It is caused by mutations in the TAZ gene that encodes for the protein tafazzin, an element that works to remodel cardiolipin. People with BTHS often experience medical complications such as skeletal myopathy, cardiomyopathy, and neutropenia (Clarke et al., 2013). Management of BTHS includes a variety of medications that may include β blockers, ACE (angiotensin-converting-enzyme) inhibitors, vasodilators, and antiarrhythmics (Barth Syndrome Foundation, 2005). Although some of these medications are available as an oral solution, liquid medications are often difficult to transport and store and may be rejected because of their unpleasant taste (Meltzer et al., 2006); moreover, some of these medications are only available in pill format.
New clinical trials are being conducted with the BTHS population; however, participation is often dependent on the ability to swallow large capsules. Unfortunately, some of the clinical features of BTHS make pill swallowing difficult. These features include generalized muscle weakness that can affect postural control, oral–motor weakness that can affect tongue movement and swallowing, and heightened oral sensitivity that leads to gagging and rejection of food (and pills) that are placed in the mouth (Clarke et al., 2013; Reynolds et al., 2012, 2015). The array of clinical features in BTHS suggests that a tailored and multifaceted approach to pill swallowing may be the most effective with this population. To date, however, no research has been done to evaluate the effectiveness of pill-swallowing training in this specific population.
Pill-Swallowing Training Techniques
Studies on pill-swallowing training across the age and diagnostic spectrum are limited; yet, those studies that do exist suggest that pill swallowing is a skill that can be taught (Patel et al., 2015). Because our focus was to develop a training program that could be used with children as young as age 5 yr, our literature review focused on techniques that have been used primarily with children but that could also be applied to adolescents and adults. The identified techniques can be roughly grouped into three categories: behavioral approaches, adaptive approaches, and positioning approaches.
Behavioral Approaches
Most published studies on pill swallowing in children have been conducted by researchers using techniques that fall within the domain of behavior-based approaches. The core component of these training protocols has been the use of behavioral shaping techniques in which children practice swallowing pieces of candy (or placebo pills) that are presented in increasingly larger sizes (Beck et al., 2005; Czyzewski et al., 2000; Garvie et al., 2007; Ghuman et al., 2004; Polaha et al., 2008). Within these studies, a trial is often considered successful when the child independently swallows a “pill” 2–3 times, and a successful intervention is when the child can swallow a “pill” the size of his or her regular medication. Other behavioral strategies are often used within this shaping paradigm; these strategies include verbal instruction, modeling, behavioral reinforcement, planned ignoring of negative behaviors, and relaxation strategies (Beck et al., 2005; Polaha et al., 2008). These approaches have yielded positive results in children with developmental conditions such as attention deficit hyperactivity disorder and autism spectrum disorder as well as children with chronic conditions such as HIV (for a review, see Patel et al., 2015).
Adaptive Approaches
Two adaptive tools have been identified in the literature for assisting children and adolescents who have difficulty swallowing pills: Pill Glide swallowing spray (FLAVORx, Columbia, MD) and adaptive cups. Pill Glide is a commercially available flavored spray that lubricates the mouth and tongue, facilitating the transmission of pills down the back of the throat. In a cohort study by Diamond and Lavallee (2010), Pill Glide was found effective for 7 of the 11 participants (ages 9–17 yr) who had past difficulty with swallowing medication. Other studies have yielded similar results, suggesting that children and adolescents who struggle to take medications in solid form may benefit from using Pill Glide sprays (Gaskell, 2016; Jagani et al., 2016).
Adaptive cups have not been robustly used in the literature; however, they are commercially available and have been used clinically with child and adult populations. Meltzer et al. (2006) used an adaptive cup manufactured by Promotions Unlimited (Baltimore, MD) as part of an intervention protocol that included 67 children (ages 6–11 yr). Scripted instructions were given to all children, and they first attempted to swallow a pill using an ordinary plastic cup; if they were unsuccessful, they were given the adapted cup. The results of this study showed that 70.0% of the children were able to swallow a pill using an ordinary cup, 13.5% were successful using the adaptive cup, and 16.5% were unable to learn within the one training session. These results suggest that for a small sample of children, adaptive cups may be useful for achieving pill-swallowing success, particularly when the amount of training time is limited.
Positioning Approaches
Research has suggested that head position can influence swallowing dynamics by changing the size of the cricopharyngeal opening and pressure of the esophageal sphincter (Kaplan et al., 2010). Kaplan et al. (2010) tested an intervention for teaching pill swallowing using five different head positions (center, up, down, left, and right). The study included a sample of children with chronic medical conditions and typically developing peers who all had a history of not being able to swallow pills. Participants in the study were educated about the esophagus, including its elasticity and ability to stretch to accommodate objects being swallowed. Participants were also introduced to the “ngunk” sound that occurs when swallowing liquids with the head turned to the side. They were then taught the five head positions for swallowing and were allowed to practice using water and candy pieces that ranged in size from cake decorations to Tic Tacs® (Ferrero, Alba, Piedmont, Italy). Participants were instructed to practice for 2 wk after the initial clinic visit. Of the initial 41 participants, 33 engaged in the practice sessions and were successful swallowing pills using different positions, although position preference varied among participants. Although these findings have not been replicated, they suggest that alternative head positions may ease the physical discomfort that may accompany pill swallowing and anxiety in people who have been unsuccessful with more traditional methods or positions.
Method
Study Design
This study was a retrospective review of data collected from the 2016 Barth Syndrome Scientific, Medical, and Family Conference that was held in Clearwater, Florida. During the conference, data were collected on 16 boys and men with BTHS who participated in a brief (approximately 40 min) pill-swallowing training session; participation included an initial screening for sensory and motor concerns followed by a 1:1 intervention with an occupational therapy practitioner. Participants were sent a follow-up email 6 mo after the conference and were asked to rate their current pill-swallowing ability. After data collection was completed, all forms were deidentified and names replaced with a subject number. Although data were initially collected for program evaluation purposes only, institutional review board approval was obtained retrospectively to analyze the data for research purposes.
Participants
Participants were 16 boys and men with a genetically confirmed diagnosis of BTHS. They ranged in age from 6 to 34 yr (mean [M] = 14.5 yr). All participants, or their parents, signed up for the pill-swallowing training on the basis of a current difficulty with swallowing pills or an anticipated difficulty with pill swallowing because of oral sensory sensitivities or motor weakness. All participants were able to communicate in English or had a translator present, and they were able to follow two- to three-step verbal directions (e.g., “open your mouth,” “stick out your tongue,” “swallow”). Although cognitive testing was not done, intellectual deficits are not part of the BTHS phenotype.
Procedure
Training sessions were 30–45 min and were conducted by two pediatric occupational therapy practitioners with advanced training in pediatric feeding and eating disorders. Each participant was seen by one practitioner only, and the practitioners rotated evenly within the training schedule.
All training materials were set out on the table for the participants to see and explore before beginning pill-swallowing training. These materials included candy that gradually increased in size in small bowls (Table 1), a bottle of water, small plastic cups, Pill Glide spray bottles (strawberry, grape, and peach flavors), and adaptive cups (Oralflo Technologies, Bridgewater, NJ). A picture chart with the five different head positions for swallowing was also positioned within view of the participants and was referred to as needed during the intervention.
Pill-Swallowing Milestones
Intervention
The occupational therapy practitioner began the intervention session by explaining to the participants that they were there to learn about pill swallowing but would not be forced to swallow anything if they did not want to. The intervention progressed on the basis of the participant’s prior experience with pill swallowing, age, and comfort level. Participants who had never attempted pill swallowing before were asked whether they were interested in trying to swallow a very small cake decoration with water in an ordinary plastic cup. If the participant said “no” or appeared anxious (e.g., verbalized concern, was restless), the practitioner began by just having the participant hold the candy in his hand or touching it to his lips. For participants who wanted to proceed straight to pill swallowing, the practitioner modeled the desired behavior and then provided a candy for the participant to practice. After two consecutive successful trials, the next-size candy was introduced. The adaptive approaches (i.e., Pill Glide spray, adaptive cup) and positioning approaches were introduced when standard techniques proved unsuccessful or when the participant expressed discomfort.
Participants who had previous experience with pill swallowing were asked to choose the size of candy they wanted to practice with. They were also given the option to try swallowing with the ordinary cup first or to begin by trying out one of the adaptive tools or positioning techniques. The occupational therapy practitioner continued to use modeling and performance feedback to encourage success. After the participant was able to swallow a candy on two consecutive trials without discomfort, he was encouraged to proceed to the next larger size.
Verbal praise and gestures (e.g., high fives) were used throughout the sessions; however, no tangible reinforcements were given, and there were no negative consequences if a participant was unsuccessful or chose to stop. Breaks were given periodically if a participant appeared stressed or anxious; participants were encouraged to take deep breaths and relax. Mental rehearsal (i.e., imagining performing the task without physical movement) was used with 1 participant during a “break” session to master the motor sequence without the stress associated with performing the task.
Participants and their caregivers were encouraged to continue working on the pill-swallowing techniques at home, but no specific home programs or home exercises were provided. For the participants who benefited most from using an adaptive cup, one was provided for them to take home.
Measures
A pill-swallowing milestone scale was developed for the purpose of this study to measure participants' progress on functional aspects of the pill-swallowing process. Pill-swallowing skills were listed in order of difficulty and assigned a numerical value (see Table 1). A score of 0 indicated no experience with pills or pill swallowing. Scores in the 1–5 range indicated that the participant would engage with a pill (e.g., put a pill on his tongue) but would not swallow. Scores in the 6–12 range indicated that the participant would swallow a pill or candy ranging in size from a very small cake decoration to a very large Mike & Ike® (Just Born, Bethlehem, PA). Milestones were recorded at three time points. Pretraining scores were reported by the participant or his caregiver at baseline. The posttraining milestone was recorded by the treating occupational therapy practitioner immediately after the training session and reflected the highest milestone achieved during the session, which was often the largest candy that the participant could swallow at least two times without discomfort. The 6-mo milestone was reported by the participant or his caregiver in response to an email sent out by the study staff.
Data Analysis
Paired t tests were used to compare pretraining scores with posttraining scores, and posttraining scores with 6-mo follow-up scores. The α level was set at .05.
Results
The 16 participants included 7 children (ages 5–10 yr), 4 adolescents (ages 11–17 yr), and 5 adults (age 18 yr or older). In Table 2, we present participant scores at all three data collection points and the primary and secondary training strategies used for each participant. Before training, 5 children had no experience swallowing pills. Most of the adult and adolescent participants could swallow pills of some size but not large or extra-large pills that might be needed for participation in a clinical trial. One adult who participated in the study (Participant 13) was able to swallow extra-large pills but only with great discomfort; he enrolled in the training to learn strategies to reduce this discomfort.
Participant Information and Outcomes
Note. Specific ages are not included in the “Age Category” column because BTHS is a rare disorder, and older participants, in particular, may be identifiable by age. Ages are categorized as children ages 5–10 yr, adolescents ages 11–17 yr, and adults age 18 yr and older. N/A = not applicable.
On the basis of the pill-swallowing milestones established for this study (0–12 scale), 14 of the 16 participants showed an increase in their pill-swallowing ability after the initial training session. Paired t test analysis comparing pretraining milestone scores (M = 5.06, standard deviation [SD] = 3.99) with posttraining milestone scores (M = 9.56, SD = 2.80) indicated a statistically significant difference in pill-swallowing performance, t(15) = −6.53, p < .001.
Twelve of the 16 participants returned our 6-mo follow-up email indicating current status with pill swallowing. On the basis of these responses, there was no difference between milestone scores at posttraining (M = 10.25, SD = 2.45) and at the 6-mo follow-up (M = 10.67, SD = 1.67), t(11) = −1.45, p = .175. These results suggest that pill-swallowing abilities were either retained or improved over the 6-mo period for most participants.
Discussion
In this study, we tested the effectiveness of a multifaceted approach to enhance pill-swallowing ability in children and adults with BTHS. The findings indicate that a brief intervention, conducted by trained professionals, can considerably improve pill-swallowing skills in this population and that these skills can be maintained over a 6-mo period.
Although the intervention was uniquely tailored to each client, some interesting trends emerged from the collective results. First, it was noted that adults and adolescents in this study came in with more pill-swallowing experiences, many of which were negative. Many of the adult and adolescent participants expressed concern about the pill-swallowing process and associated it with discomfort or a sense of failure. These feelings often led the older participants to respond most effectively to either the adaptive or positioning approaches as opposed to the more traditional behavioral shaping or modeling techniques. However, 5 out of the 7 children in the study came into the training with no pill-swallowing experience, and they were often excited to try swallowing the candies laid out before them.
Second, all of the children in the study responded best to behavioral approaches as either their primary or secondary training technique and rarely needed adaptive approaches. This finding is similar to that of Garvie et al. (2007), who found that children ages 4–5 yr needed fewer training sessions than older children to learn how to swallow pills; they attributed this finding to the younger children having fewer negative pill-swallowing experiences. These results suggest that young children with BTHS should be screened for pill-swallowing readiness and exposed to this type of graded training at an early age. Parents and families should simultaneously be educated about how to progress children in the pill-swallowing sequence while avoiding situations in which negative associations with pills or pill swallowing may be developed.
It is also interesting that 2 participants in this study did not show improvement in their pill-swallowing abilities. The first participant was an adult who could already swallow extra-large pills, and therefore he was unable to demonstrate a change in scores on our pill-swallowing milestone scale. However, this participant was successful in using head positioning techniques to reduce the discomfort he had associated with pill swallowing.
The second participant who did not improve was also an adult who entered the training unable to swallow pills of any size. This participant presented with considerably limited tongue movements, reduced tongue strength, and a retracted jaw; he was unable to maintain a “pill” on the surface of his tongue or to propel the “pill” backward toward his throat. This participant also had difficulty keeping his head tilted backward while swallowing and would thrust his head forward interrupting the swallow. Behavioral, positioning, and adaptive approaches were all attempted with this participant without success. This finding suggests that clients with oral–motor strength and coordination that are impaired at a certain level may not be appropriate for this brief pill-swallowing intervention, and they may need more intensive therapy to address underlying oral–motor deficits. Although conclusions cannot be drawn on the basis of 1 participant, future studies may need to examine what baseline oral–motor skills are required for pill-swallowing training to be effective.
Limitations
We acknowledge the small sample size used in this study. However, BTHS is a rare disorder, and our sample represented approximately 10% of those people currently living with the condition. In future studies, researchers should consider testing this protocol with a larger sample of participants with chronic medical conditions, which would allow for statistical analyses to examine differences in participants’ responses to intervention on the basis of age, oral–motor abilities, sensory sensitivities, and other potentially confounding factors such as anxiety level. Finally, although candy pieces have been commonly used for pill-swallowing training purposes, future researchers may consider using research-grade placebo tablets that progress in equal size increments (e.g., 2 mm, 4 mm) to enhance the rigor of the research and options for statistical analysis.
Implications for Occupational Therapy Practice
The results of this study have the following implications for occupational therapy practice:
Pill swallowing is an important occupation for many people; working on pill-swallowing abilities may fall within the scope of occupational therapy practice.
Behavior-based approaches, including shaping and modeling of skills, may work best for children with minimal prior pill-swallowing experience. Older children and adults who have prior negative experience with pill swallowing may learn best using adaptive or positioning approaches.
Underlying issues such as poor motor planning or poor oral–motor control may need to be addressed before a pill-swallowing intervention program is implemented.
Conclusion
Pill swallowing is an important skill for many people, and it may be an essential daily routine for those people with chronic medical conditions. For people who have difficulty swallowing pills because of sensory or motor deficits, there are training techniques and adaptive approaches that may be effective. The results of this brief intervention are promising and provide a mechanism by which occupational therapy practitioners can help clients increase their independence with medication management and possibly increase their overall quality of life. A brief pill-swallowing training program may be effective in increasing the BTHS population’s capacity to participate in clinical trial research, opening up new possibilities for disease management.
Footnotes
Acknowledgments
This project was supported by a travel grant from the Barth Syndrome Foundation. We thank Shelley Bowen and Michaela Damin for their help in participant recruitment and scheduling.