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by James L. Coyle, Caryn Easterling, Maureen Lefton-Greif, and Linda Mackay
Over the course of a career, the SLP's clinical expertise and judgment grow with experience and observation of outcomes. The clinician consumes external evidence in the form of clinical research to gain background knowledge and substantiate clinical decisions, judges the quality of the published works systematically, and estimates its clinical importance and value to the patient (Haynes, et al., 2006; Straus, et al., 2005). The key to clinical success, however, is the clinician and patient partnership. This relationship is critical for setting goals, planning and carrying out means to achieve these goals, and acceptance of and commitment to the proposed clinical procedures by both parties.
The three diverse cases that follow illustrate common and rare clinical dilemmas and demonstrate the common components of the process of evidence-based clinical problem-solving [PDF].
Case 1: General Medical Diagnosis
The first case is a 79-year-old female with a medical history of chronic obstructive pulmonary disease (COPD), congestive heart failure (CHF), and idiopathic pulmonary fibrosis (IPF), who woke in the night coughing and short of breath with nausea and vomiting. She fell and was admitted to the hospital with a hip fracture. She underwent chest x-ray, produced a sputum sample that demonstrated normal oral bacteria, and was also diagnosed with aspiration pneumonia. She underwent hip surgery and was endotracheally routinely intubated and extubated. The attending physician consulted the SLP two days later and requested videofluoroscopic evaluation because of right lower lobe infiltrate and admitting diagnosis of aspiration pneumonia.
Data Collection
Screening and testing revealed symmetrical sensory and motor functions of all structures innervated by cranial nerves V, VII, and IX through XII, intact cognitive and language functions for complex material, and normal motor speech production. The patient was edentulous. Throat-clearing was frequent, occurring approximately once per three-minute interval on average, both before and during clinical swallow trials.
Instrumental videofluoroscopic examination was performed, with each swallow scored using the Penetration Aspiration Scale (PAS) (Rosenbek, Robbins, Roecker, Coyle, & Wood, 1996), and an objective scale describing postprandial pharyngeal retention (Eisenhuber et al., 2002). In 16 trials, one deep laryngeal penetration event during one of three 3mL thin liquid trials (PAS score 4), and one minimal volume aspiration, with complete, spontaneous clearance of the airway (PAS score 6) during one of three large thin liquid trials, were observed. Postprandial retention occupied less than 25% of the height of the pyriform sinuses on all swallows. While the patient masticated a cookie, contrast flowed from the oral cavity to the valleculae, where it remained for two to three seconds before pharyngeal stage onset. Remaining physiology and biomechanics were within safe and functional limits.
Evidence Analysis
Does the Patient Have Dysphagia?
Probably not. Normal healthy elderly individuals may exhibit intermittent laryngeal penetration while swallowing (Robbins et al., 1999; Daggett, et al., 2006). Laryngeal penetration is seen in greater frequency in patients with COPD (Good-Fratturelli et al., 2000). Postprandial retention greater than 50% of the height of the pyriform sinuses is predictive of postprandial aspiration (Eisenhuber et al., 2002). Healthy normal individuals routinely "aggregate" material in the valleculae for several seconds while masticating solids (Hiiemae & Palmer, 1999). Prolonged endotracheal intubation can produce largely reversible, temporary swallowing dysfunction in the elderly (El Solh et al., 2003; deLarminat et al., 1995), but our patient was routinely extubated, had no history of dysphagia-producing conditions, and displayed normal oropharyngeal sensorimotor function for her age. There is little evidence suggesting intubation-related oropharyngeal impairments or risks of conditions caused by dysphagia exist. Conclusion: it is most likely that this patient's swallowing function is at or near pre-admission baseline.
Does the Patient Have Pneumonia?
Maybe. Pneumonia may be caused by oropharyngeal dysphagia or other causes, and it is sometimes diagnosed without sufficient evidence of infection and subsequent infiltrates within the alveoli (Marik, 2001). In this case, the onset of dyspnea co-occurred with an episode of emesis. Aspiration of stomach contents is a serious condition, indeed, but it is unrelated to oropharyngeal swallowing function. Aspiration pneumonitis is a more likely diagnosis, though secondary pneumonia following alveolar trauma is possible as a delayed outcome; in either scenario, dysphagia would not be suspected nor would intervention be indicated. The sputum culture containing normal oral bacteria seems suspicious, however; it was collected at the bedside in the emergency room and contaminated by the oral contents themselves, rendering the validity of the culture poor (Centers for Disease Control and Prevention, 2003). Other risk factors favoring pneumonia with or without dysphagia are absent (Langmore et al., 1998; El Solh et al., 2001).
Conclusion: It is unlikely that the patient's current pulmonary condition is associated with oropharyngeal swallowing impairments, though pneumonia may indeed be present.
Other Information About the Underlying Disease
Recently, idiopathic pulmonary fibrosis (IPF) was found to be strongly associated with gastroesophageal reflux. Tobin et al. (1998) found that when patients with IPF were lying supine, they exhibited a significantly greater height of reflux of gastric contents into the proximal esophagus than in an age-matched group of healthy normals, suggesting that IPF may be caused by chronic aspiration of gastric contents in some patients.
Intervention
After consulting with the referring physician and providing evidence as listed above for consideration, the patient's oral intake resumed without alteration. Patient was later diagnosed with aspiration pneumonitis and was later referred for surgical evaluation to alleviate chronic aspiration of gastric contents contributing to idiopathic pulmonary fibrosis. A 24-hour esophageal pH study later confirmed the diagnosis.
The incidental "abnormal" swallowing findings were attributed to nearly resolved effects of intubation, normal aging, and the comorbidity of COPD. There was insufficient clinical and external evidence to warrant dysphagia intervention in this case and the patient was appropriately managed without adverse or undesirable outcomes.
Case 2: Neurology Referral for ALS
A 55-year-old man was referred because of gradual onset of hoarseness, occasional coughing on saliva, fatigue while chewing, feeling of "tongue thickness," increased difficulty speaking in the evening, and bilateral lower-extremity muscle-twitching with mild proximal muscle weakness in the right leg. ALS requires diagnostic confirmation by an experienced neurologist noting the presence and progression of upper (UMN) and lower motor neuron (LMN) findings. Therefore, after performing diagnostic electromyography, the diagnosis of amyotrophic lateral sclerosis (ALS) was confirmed.
Background
ALS, a progressive neurodegenerative disease, is the most common adult-onset motor neuron disease (Mitsumoto et al., 1998). Because of the progressive nature and the lack of a known cure for ALS, each patient is evaluated and managed by a multidisciplinary team (Miller et al., 1999). Members of the team include physical, occupational, and respiratory therapists; an SLP; and a neurologist, nurse practitioner, social worker, chaplain, dietician, and pulmonologist.
This approach is designed to improve the quality of life through prevention and relief of suffering; early identification of functional problems; assessment and treatment of pain and physical, psychosocial, and spiritual issues (Van den Berg et al., 2005). The team utilizes ALSFRSR, a 12-item functional inventory, to assess the patient's level of self-sufficiency in feeding, grooming, ambulation, and communication, while monitoring disease progression and predicting survival time (Kaufman et al., 2005).
Patients are evaluated for signs and symptoms of dysphagia, as all ALS patients experience dysphagia independent of the time of onset or duration of disease (Kawai et al., 2003). Signs and symptoms include tongue weakness, fasciculations, and dysarthria followed by pharyngeal weakness, and finally unilateral or bilateral facial weakness (Caroscio et al., 1987). Disorders of the oropharyngeal phase occur because of degeneration of cranial nerve XII motor nucleus as well as degeneration of cranial nerve IX and X motor nuclei, resulting in decreased bolus containment, increased transit times, decreased or ill-timed laryngeal elevation, soft-palate elevation, and decreased swallow pressures (Ertekin et al., 2000; Leder et al., 2004)
Data Collection
The multidisciplinary evaluation determined that the patient had mild right upper and bilateral lower extremity weakness and fasciculations. Pulmonary function testing revealed 90% vital capacity. During the cranial-nerve exam, right-tongue weakness and fasciculations were noted.
Speech was mildly dysarthric with increased articulatory imprecision in conversation related to fatigue. The patient coughed on his saliva while speaking. When given a cookie to chew, the patient complained of fatigue and requested liquid to help him clear the cookie from his mouth. The patient was scheduled for a videofluoroscopic evaluation to assess the physiologic swallow function and determine if compensatory strategies would improve oropharyngeal efficiency.
Evidence Analysis
Does the Patient Have Dysphagia?
The patient's videofluoroscopic findings revealed: prolonged but functional oral phase with slow bolus manipulation for all consistencies and volumes and slow mastication; piecemeal bolus movement into the pharynx with uncontrolled bolus movement over the tongue base with thin liquid; inconsistent predeglutitive aspiration of thin liquids secondary to delayed pharyngeal swallow; spontaneous cough resulting in bolus clearance from the laryngeal vestibule and trachea of thin liquid aspirant; and mild to moderate postdeglutitive residue on the tongue base and in the valleculae.
The following were compensatory strategies attempted during the videofluoroscopic evaluation:
- Chin tuck effectively eliminated predeglutitive aspiration of thin liquids. The chin tuck posture effects the pharyngeal swallow by improving airway protection through narrowing of the airway entrance, positioning the tongue base and epiglottis toward the posterior pharyngeal wall, widening the vallecular space, decreasing distance between the larynx and hyoid and mandible, and reducing the potential depth of penetration and aspiration of material (Welch et al., 1994; Bulow et al., 2001).
- Alternating liquid and solid boluses effectively eliminated post-deglutitive tongue base residue.
- Effortful swallow was effective in decreasing the post-deglutitive residue; however, the maneuver was fatiguing for the patient.
Impressions: Pre-deglutitive aspiration secondary to bolus movement into the pharynx prior to superior and anterior hyolaryngeal excursion. Patient sensed aspiration and spontaneously coughed to successfully clear aspirant.
Does the Patient Require Rehabilitative or Compensatory Treatment?
Compensatory strategies were recommended because exercise is contraindicated for patients with motor-neuron disease. Recommended strategies:
- Chin tuck with thin liquids to prevent pre-deglutitive aspiration
- Alternate liquids and solids to clear post-deglutitive residue
- Soft mechanical diet for energy conservation
- Registered dietician consultation to determine optimal caloric and nutritional intake
- Monitor pulmonary function to assure gastronomy-tube (GT) placement prior to vital capacity reaching <50% (Miller et al., 1999).
Does the Patient Require Re-Evaluation?
Re-evaluation is recommended in 6–8 weeks to monitor functional change (Miller et al., 1999).
Case 3: Pediatric Referral for Pierre Robin
A 10-year-old female with a diagnosis of Pierre Robin asked whether she would ever be able to eat and drink like her friends. Her question evoked issues about prognosis. Sackett et al. (1997) provided general guidelines for conducting evidence-based reviews of the literature for determining prognosis. Specific to this case, it would be important to ascertain whether previous investigations: compared their subjects at a comparable time during the course of the condition, followed patients long enough to address questions of interest, and defined subgroups of the population and made adjustments for relevant prognostic indicators.
Background
Pierre Robin (PR) is the name given to co-occurring congenital defects that include micrognathia, glossoptosis, and usually a cleft of the palate. Since first described in the 1920s, this triad of defects has been referred to as a syndrome, sequence, anomaly, and complex. These differences in names reflect efforts to define a condition that may be caused by different etiologies. The specific cause of PR remains unknown; however, it may be caused by a sequence or part of a genetic syndrome. A sequence occurs when a single developmental defect causes a cascade of events. For example, in PR, mandibular hypoplasia may cause glossoptosis, which in turn may cause a cleft palate. In contrast, syndrome refers to a group of symptoms that are caused by a single underlying genetic condition. Stickler syndrome, fetal alcohol syndrome and Treacher-Collins syndrome are common syndromes associated with PR. When PR is part of a syndrome, there is an increased risk for development of other problems associated with that syndrome. Hence, etiology is critical to prognostic determinants.
Anticipation and treatment of respiratory compromise and nutrition needs are the primary management concerns for infants and young children with PR (Taylor, 2001). Common feeding/swallowing problems include excessively long meals, abnormal sucking or oral phase skills, incoordination between oral and pharyngeal phase transitions, and pharyngeal phase deficits (Monasterio et al., 2004; Baudon et al., 2002; Cruz et al., 1999; Renault et al., 2000; Baujat et al., 2001). Despite the paucity of data detailing long-term outcomes for feeding/swallowing development, current evidence suggests that children with PR caused by an isolated sequence have the best prognosis for oral feeding, and the success of oral feeding is related directly to the degree of airway issues (Smith & Senders, 2006; Cruz et al., 1999).
Data Collection
In addition to PR, our patient had diagnoses of arthrogryposis multiplex congenita (congenital contractures of multiple joints associated with developmental defects of the nervous system), hypotonia, and oropharyngeal dysphagia. During her first year of life, a GT was placed and her cleft palate was repaired.
This child presented as bright and communicative. Speech intelligibility was poor and characterized by articulation problems and velopharyngeal insufficiency. Most of her nutrition and hydration was delivered by a GT. She continued to experience intermittent respiratory problems, including two recent bouts of pneumonia, and fatigue during meals and other activities of daily living. She complained of choking episodes and remained fearful of choking. She said that she did best with very small bites of pureed foods, when drinking from a straw, and when eating and drinking slowly.
After taking several discrete sips of milk from a straw, she began clearing her throat. The frequency of throat clearing increased as she continued to drink. She appeared to work hard to clear her mouth. A videofluoroscopic swallow study revealed: prolonged oral phase with slow bolus formation and transfer for very small volumes of all consistencies, and greater inefficiency with purees than liquids; delayed onset of swallowing; decreased hyoid excursion and limited base of tongue movement; incomplete palatal and pharyngeal wall approximation in the lateral plane; and post-deglutitive residue in the valleculae and pyriform sinuses, resulting in intermittent supraglottic penetration without aspiration, with all consistencies. Neither dry nor water swallows cleared residue from the pharynx.
Assessment and Evidence
Analysis for Prognosis
Given the presence of other diagnostic conditions and persistent difficulties, this child had a PR phenotype that was more consistent with a syndrome than an isolated sequence. She exhibited profound dysphagia that required GT feedings for nutrition support. Previous investigations focused on children younger than this patient and did not adequately describe subgroups for making relevant comparisons. Although limited evidence regarding prognosis was available, this child's prognosis was considered to be "guarded" given her existing comorbidities, persistence of respiratory problems, and extent of dysphagia. Recommendations focused on encouraging her to continue to eat and drink as "she did best," and to return to clinic when she wanted more information or thought she could change how she was eating or drinking. This case illustrates that although phenotype may dictate some treatment decisions, etiology drives prognosis.
Summary of Case Studies
As these cases illustrate, evidence-based case management in speech-language pathology involves careful consideration of the medical record and patient examination data, sound clinical judgment, and the integration of the patient's expectations and values. Our first case illustrated the dilemmas faced by clinicians managing some of the most frequently encountered questions that arise for SLPs in acute-care settings, and underscored the importance of a sufficiently broad knowledge base by the SLP for use in collaboration with physicians in the differential diagnosis of various types and sources of pneumonia. It also highlighted the role of the SLP in the prevention of adverse health outcomes such as pneumonia and malnutrition.
Our second case highlighted the significance of a clear understanding by the SLP of the natural history of the underlying diseases causing dysphagia, and the critical importance of counseling and patient values on the likelihood of satisfactory clinical outcomes, even in cases of terminal illness. Our final case underscored the importance of combining precise, objective prognostic indicators with patient values and expectations, even in children, when a clinical "happy ending" may be out of reach.
All three cases demonstrated how an evidence-based approach is used when evaluating and treating patients with dysphagia. Evidence-based practice is what the SLP should apply on a daily basis.
Jim Coyle, an instructor in the Department of Communication Science and Disorders at the University of Pittsburgh, can be reached at jcoyle@pitt.edu. Caryn Easterling, an assistant professor at the University of Wisconsin in Milwaukee, can be reached at cseasterling@aol.com. Maureen A. Lefton-Greif, an associate professor in the Department of Pediatrics at the Johns Hopkins University School of Medicine, can be reached at mlefton@jhmi.edu. Linda Mackay, managing director at Saint Francis Hospital and Medical Center in Hartford, Connecticut, and associate professor of surgery at the University of Connecticut School of Medicine, can be reached at lmackay@stfranciscare.org.
The authors are board-recognized specialists in swallowing and swallowing disorders. This article is based upon a presentation at the 2006 ASHA Convention.
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