If a medically fragile 12-year-old child is having difficulty swallowing, the decision about oral feeding is usually clear-cut: limit or restrict food or liquids until the child can consistently swallow safely, without risk of aspiration.
But what if the child has cancer, with very low chances of survival? She desperately wants to eat and drink, even if only in very small amounts, with her family and friends. Does her desire to be a part of the social routine outweigh the aspiration concerns?
This scenario is not unusual for pediatric speech-language pathologists who work with children who have feeding and swallowing difficulties accompanying degenerative and end-of-life conditions—neurologic issues, muscle diseases, cancer, and respiratory disorders. In these cases, the SLP's primary concerns shift from resolution of feeding and swallowing issues and rehabilitative care to implementation of compensatory strategies (Roe & Leslie, 2010). The ASHA Code of Ethics (ASHA, 2010) provides the guiding framework for SLPs, who must strive to use the best evidence available, clinical judgment, and the family's values and beliefs in decision-making (Arvedson & Lefton-Greif, 2007).
As in many clinical situations, there is no one "right" answer for how to proceed, as all situations are unique. Each child, family, culture, and support system is different. When questions arise about feeding, the pediatric SLP likely participates by providing information about the patient's feeding and swallowing function. But it is unlikely that decisions about oral feeding will be clear—or easy. At times, being able to maintain oral feeding, at least to a degree, is crucial to maintaining quality of life for patients and families.
Collaboration with other health professionals on the patient's care team facilitates compassionate, honest communication about feeding and swallowing skills and sets the stage for discussion of options. Team collaboration helps to facilitate discussion in the face of the complex medical and ethical issues surrounding care of pediatric hospice patients, who have a life expectancy defined in months. Ideally, our treatment should be based on the guiding principles of pediatric palliative care: enhancing quality of life and relieving suffering for the child, family members, and other significant people in the child's life.
Role of the Pediatric SLP
As members of the care team for infants and children in palliative or hospice care, SLPs may be consulted to provide assistance with communication (see The ASHA Leader, Dec. 15, 2009) and/or for feeding and swallowing assessment. SLPs' role in swallowing issues at end of life, however, is usually quite different from than the role they assume with other patients with dysphagia.
SLPs and other medical providers regularly are consulted on patients with conditions that are co-morbid with dysphagia. In these cases, a relationship can be established with the patient; providers assess function, discuss the findings with the patient, and often recommend intervention to improve function. In contrast, when an SLP is asked to provide input on swallowing and feeding for very ill or terminal patients, often some difficult decisions need to be made. These feeding management decisions should not be made based exclusively on the SLP's assessment; rather, decisions should be a collaborative effort among the patient, family, and care team (Roe & Leslie, 2010; Arvedson & Lefton-Greif, 2007).
In our clinical experience, the SLP's role is facilitative: The SLP works with the patient, family, and care providers to implement compensatory strategies if a decision is made to continue with some degree of oral intake. If the patient is allowed to continue oral intake for as long as possible, the SLP has an important role in the development of strategies to do so. The expected outcome of intervention is not improved ability; rather, the desired result is the patient's ability to enjoy eating and drinking to whatever degree possible. The ultimate decision about eating and drinking is made by the family and the patient. Our obligation is to provide information about swallowing function in an understandable, empathetic, and caring manner.
The stories of two patients illustrate the types of scenarios we experience in our clinical practice.
Case 1: Samantha
Samantha was 12 when she was diagnosed with a medulloblastoma, the most common type of malignant brain tumor. Prior to the diagnosis she was healthy and happy, participating in activities such as gymnastics and Girl Scouts. She began to experience intermittent headaches for a period of several months; these were first attributed to stress and thought to be migraines. When the headaches intensified, she was evaluated by her physician and sent for a head MRI. The startling diagnosis was medulloblastoma, and Samantha received the standard therapy—aggressive surgery followed by radiation with adjuvant chemotherapy.
Following treatment, she experienced severe balance deficits, dysarthria, and profound swallowing deficits. A swallowing study was performed, and she was placed on a strict "nothing per oral" status at the SLP's recommendation. This recommendation was devastating to Samantha, having a much greater effect than any of the other deficits on her quality of life. She simply wished to eat and drink again, even in small amounts, with her family and friends.
Samantha received nutrition through a feeding tube, and she completed a period of rehabilitation. She gradually showed signs of improvement, and the tube was eventually removed. She continued with her oral feedings for a period of months, seemingly without difficulty, and then developed a severe pneumonia. The family aggressively sought advice and input, and brought her to us for a second opinion regarding her overall status, including a re-assessment of swallowing function.
As part of the overall re-assessment of her status, she was referred for a clinical oral motor/feeding assessment and videofluoroscopic swallowing study (VFSS) and a fiberoptic endoscopic evaluation of swallowing (FEES). Results of the clinical oral motor/feeding assessment revealed significant residual left-sided deficits, including facial paralysis, reduced velar elevation, and tongue deviation to the left with fasiculations. She was very motivated to accept small amounts of liquid and food during the exam, though she demonstrated fatigue and an increased wet, gurgly vocal quality as the examination progressed. These results raised concern about her ability to maintain airway protection during swallowing. We proceeded with instrumental examinations, including VFSS and FEES.
Before the VFSS, Samantha was agitated; she expressed concern that based on the test, the clinician was going to forbid Samantha from eating and drinking again. Based on their conversation, the clinician understood that for Samantha, eating and drinking with family and friends was extremely important.
In the VFSS, Samantha was able to demonstrate airway protection during the initial swallows of both fluid and thin puree boluses. However, as the study progressed, she began to display reduced ability to clear her pharynx with swallows, despite repeated efforts and attempts. This failure resulted in a significant buildup, with resultant penetration and later frank aspiration of material into the airway. She was not sensitive to the aspiration, and displayed no effort to cough and clear.
We introduced some compensatory strategies to assist with her swallow, including postural maneuvers, effortful swallow, and alternating solid and liquid boluses. Though the compensatory strategies helped to a degree, her "best" swallows were those that occurred early in the study. The FEES findings were similar to the VFSS results, indicating that her initial swallows were the best, followed by fatigue and an overall decline in swallowing function.
At the same time, other tests revealed that the cancer had returned, and Samantha's prognosis was poor. There was no question her time was limited. There also was no question that she still strongly wanted to eat and drink orally, if even in small amounts.
Were her skills safe for total oral intake? Clearly, the answer to that question was "no." But—were her skills such that continuing with very small amounts of food and drink for pleasure could be feasible, using study results to help guide how much intake was reasonable? The information was discussed and considered by the physician, care team, parents, and Samantha herself. The final decision by the family was to return to tube feedings to meet nutritional needs but also to continue with oral intake of very limited amounts of food and liquid for quality of life purposes.
Case 2: Jacob
Jacob, a 7-month-old in hospice care, was recently referred to our Interdisciplinary Feeding Team for input on the management of oral feedings. He was born at 35 weeks' gestation, and had been diagnosed with schizencephaly (a birth defect characterized by abnormal "slits" in the cerebral hemispheres). An MRI revealed an almost complete absence of brain tissue, resulting in massive cerebrospinal fluid spaces. His neurologic prognosis was considered to be extremely poor, and he was discharged from the neonatal intensive care unit to hospice care. Though Jacob had been discharged with a nasogastric (NG) tube for feeding, the family persevered with bottle feedings, and eventually transitioned him from NG to oral feedings. However, he was vomiting frequently and demonstrating poor weight gain. The hospice team (nurse, social worker, physician) sought the input of the feeding team for help in managing these feeding issues.
When his mother brought Jacob to the feeding team clinic, he was minimally responsive for most of the assessment. Jacob's mother demonstrated her usual arousal method prior to presenting his bottle feedings—gently wiping his face with a soft cloth and repositioning him. She offered his usual bottle, and he demonstrated a fairly functional ability to initiate a coordinated sucking pattern, though it took a prolonged period before he was successful in ingesting even a few ounces. He coughed and choked throughout the feeding. The dietician interviewed the mother regarding his usual oral intake, analyzed his growth, and found that his growth was inadequate. Throughout the assessment, the mother stated strongly that her wish was to orally feed him.
What are the next steps? The physician, occupational therapist, dietician, social worker, and SLP on the feeding team conferred before approaching Jacob's family. The team determined that the clinical presentation during feeding was strongly suggestive of possible swallowing issues, including aspiration, and ultimately implications for respiratory health. Providing the family with the best advice regarding future feeding would require an objective study of swallowing and formula changes to help increase caloric density. The VFSS would be explained to the family, and offered as an option.
But what if the results were abnormal? The team decided to introduce this possibility to the family to lay the groundwork for decision-making about oral feeding.
The team stressed to Jacob's family that decisions did not have to be made right away. We explained that if problems were detected during the VFSS, we would explore compensatory strategies for feeding that might be helpful in supporting safe and continued oral intake, at least to a degree. The family seemed receptive to the option of the VFSS, yet strongly in favor of continuing with oral feeding. However, they were willing to consider an alternative route of feeding if the study identified airway protection problems that could negatively affect Jacob's respiratory status. The outcome of this situation is unknown, though Jacob was recently admitted because of the onset of severe seizures, known to be associated with the diagnosis of schizencephaly.
Each case is different, but some general guidance can help in most situations.
Assess and Address Parent and Caregiver Needs
- Listen carefully to understand the perspective of the parent or caregiver. Wishes as well as concerns vary widely.
- Be familiar with additional resources that may be available for the patient's particular disease or condition and for parent support, and be prepared to share your knowledge about the disease or condition to answer questions related to speech-language pathology. Stay within your scope of practice. For example, request the input of a registered dietician if questions arise regarding calories and nutrition. If your interaction with the patient and family triggers significant emotional responses, pursue the input of the social worker or psychologist.
- If your work setting does not have palliative and hospice care services and resources, seek consultation directly with the child's physician, therapists, social worker, and other health professionals involved in the patient's treatment.
- Acknowledge, respect, and accept that the patient and family's values and beliefs are the "cornerstone" to the treatment plan (Roe & Leslie, 2010).
Address Patient Needs
The following strategies are examples of approaches that may be used in the treatment plan.
- Control rate of intake. Imposing breaks or pauses for respiration in the nutritive suck-swallow cycle during oral feedings may help to maintain airway protection during swallows. Ideally, the usefulness of this strategy is assessed during the instrumental study. Imposing pauses can be accomplished by briefly breaking suction on the nipple, or tilting the bottle downward to stop the flow of liquid, while maintaining contact of the nipple to the infant's lip.
- Modify bolus texture. For patients continuing with small amounts of solids, the use of easy-to-manage textures, such as non-chew, smooth, or very easily dissolvable consistencies is advisable. Avoid thick boluses or difficult-to-manage foods (e.g., mixed or dry, crumbly consistencies) that require more oral manipulation and effortful swallowing.
- Modify bolus viscosity and temperature. Altering liquid viscosity and temperature may be helpful. Thickening liquids may help modulate the flow of liquid intake and facilitate oral control, oral transfer, and the timing of airway protection with swallowing.
- Use flavor. Using strong, but pleasant flavors (citrus, sour, spicy) and variation in foods and liquids may heighten sensory input, and help facilitate swallowing onset and overall swallowing efficiency. Take care to establish taste preferences.
- Provide oral hygiene care. Daily oral hygiene care significantly affects a child's well-being, and is essential in minimizing the risk of infection. Poor oral hygiene has been associated with bacterial colonization of the oropharynx, and higher nosocomial infection rates. Provide a pediatric toothbrush and guidelines for use. Recommendations could include directions for care of teeth, gums, and tongue.
Consideration of patient and family wishes is primary in the development of a plan to manage issues related to feeding and swallowing in pediatric patients with chronic and life-limiting illnesses. The role of the pediatric SLP is supportive and facilitative as opposed to interventional and goal-driven. Difficult oral feeding decisions are best accomplished through the collaboration of medical professionals working together with the patient and family. This team approach fosters consideration of swallowing studies, clinical judgment, and patient and family wishes. Communication between the family and the team is key and allows family members to consider the information they are given and feel certain they are making the best choices.