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Management of Swallow Function in a Patient with an Orocutaneous Fistula

45 Management of Swallow Function in a Patient with an Orocutaneous Fistula

Amy Fullerton

45.1 Introduction

Speech and swallowing outcomes following treatment of largevolume oral cavity tumors can have varying eﬀects on speech and swallow function. Anticipation of these deficits and appropriate treatment planning can minimize loss of function due to disuse atrophy and facilitate safe resumption of oral diet. The literature also supports the role of preoperative counseling to reduce overall cost and length of stay as well as improve functional outcomes. Rehabilitative plans may need to be adjusted depending on the presence and duration of postoperative edema, flap dehiscence, and/or fistula formation and acute radiation toxicities, all of which contribute to complex rehabilitation.

45.2 Clinical History and Description

TL was a 65-year-old woman with pT4aN0M0 squamous cell carcinoma of the right alveolar ridge for which she underwent prophylactic gastrostomy tube placement and dental extractions in anticipation of adjuvant radiotherapy following composite mandibulectomy with fibular free flap and bilateral modified radical neck dissections. Five weeks following surgical resection, she received a total of 60 Gy at 2 Gy/fraction to the oral cavity and neck. Intensity-modulated radiotherapy (IMRT) was used instead of conventional radiotherapy to decrease the dose to normal tissues and, specifically, to decrease radiation exposure to the spinal cord, salivary glands, and brachial plexus.

45.3 Clinical Testing

Baseline evaluation of swallow function prior to oncologic therapy included the Functional Oral Intake Scale (FOIS), which is a 7-point ordinal scale that provides a measure of a patient’s diet. Scores less than 6 indicate varying levels of oral intake dysfunction. TL’s baseline score was 7, indicating she was on a total oral diet without restrictions. Measurements of oral aperture and tongue strength were obtained with the Iowa Oral Performance Instrument. Interdental oral aperture was 55 mm and linguapalatal pressure (an indirect measure of tongue strength) was 56 kPa. These measures were within the normal range for her age. The Performance Status Scale for Head and Neck (PSS-HN) cancer is a 0 to 100 scale with 10-point ordinal increments correlating to the ability to eat an oral diet. A score of 0 indicates nothing by mouth and a score of 100 indicates no diet restrictions. Videofluoroscopic evaluation of swallow function followed the protocol for the Dynamic Imaging Grade of Swallowing Toxicity (DIGEST) tool, which is a reliable and valid scale incorporating pharyngeal residue and penetration/aspiration scores that give an indication of level of swallowing impairment. DIGEST scores include both safety and eﬃciency sub-

scores (both of which were 0 at this time) as well as an overall score (which was also 0 at baseline). Preoperative scores were all within functional limits and no appreciable swallow deficits were identified. As expected, TL reported consuming a normal oral diet without restriction, denied appetite loss or dysgeusia, and reported her weight had been stable over the past several months. Preoperative evaluation of speech function included complete oral motor evaluation of cranial nerve integrity, diadochokinetics, and inventory of phonemes in isolated and connected speech. Baseline speech was also within functional limits without dysar thria or articulatory imprecision. TL denied changes to her speech and reported satisfaction with her current, baseline speech function.

45.4 Questions and Answers for the Reader

1. What is an appropriate time frame to schedule videofluoroscopic swallow study following free flap surgical reconstruction to an oropharyngeal structure(s) involved in speech production or deglutition?

a) Postoperative day (POD) 1. b) POD 5 to 14. c) POD 30. d) No swallow study is indicated.

Answer: b is correct. Orocutaneous fistula may present at this point and postoperative swallow function can be reliably assessed.

a is incorrect. Acute postoperative edema will preclude a reliable result and surgical drains and/or tracheostomy, both of which are typically left in place 3 to 5 days postoperatively, will obscure views, and confound func tion.

c is incorrect. Thirty days is too long for a patient to remain without an oral diet unnecessarily and will delay functional rehabilitation.

d is incorrect. A swallow study is necessary to guide appropriate return to oral diet and rehabilitation as well as assess postoperative anatomical and physiological changes to structures involved in speech/deglutition.

2. What is an appropriate contrast material for initial postope-

rative swallow study when a leak or fistula may be present? a) Gastrografin. b) Cystografin. c) Omnipaque. d) Bar ium sulfate, BaSo

Answer: c is correct . Omnipaque is appropriate for oral administration and, with a very low osmolarity of 520 mOsm/L, is nearly iso-osmolar. Although aspiration of this contrast is discouraged in copious quantities, it is better tolerated than hyperosmolar contrast materials.

169

Management of Swallow Function in a Patient with an Orocutaneous Fistula

a is incorrect. Gastrografin, a lemon-flavor Food and Drug Administration (FDA)-approved contrast material for oral ingestion has an incredibly high osmolarity of 1,900 mOsm/L or approximately six times that of extracellular fluid. If aspiration occurs, serious pulmonary complications including pulmonary edema, pneumonitis, or death through copious osmotic eﬀusion may occur. Gastrografin may also have iodine-mediated thyrotropic eﬀects, which would be contraindicated in a large proportion of head and neck cancer patients.

b is incorrect. Cystografin contrast material is not meant for oral ingestion.

d is incorrect. Although BaSo sumption and routinely used for videofluoroscopic swallow studies due to water insolubility, it is inappropriate for patients with free flap reconstructions or where fistula is suspected as it will maintain patency of any postoperative fistula.

3. What speech deficits are anticipated following composite

mandibulectomy? a) Labiodentals, bilabials, and interdentals: /m/, /f/, /v/, /b/,

/p/, /∫/, /ʒ/. b) Alveolars and alveopalatals: /l/, /t/, /d/, /θ/. c) Palatals and velars: /k/, /g/, /ð/, /ŋ/, /j/. d) Glottals: /h/.

Answer: a is correct. Due to the dental extractions in anticipation of radiotherapy, these sounds will be aﬀected.

b is incorrect. Lingual range of motion is preserved. c is incorrect. Velar structure and lingual range of motion is

preserved.

d is incorrect. Glottal movement is preserved.

4. How is adjuvant radiotherapy anticipated to aﬀect speech outcomes?

a) It will improve speech outcomes. b) It will not aﬀect speech outcomes. c) It will hurt speech outcomes.

Answer: a is correct. Radiotherapy will further reduce flap bulk and improve conformity to native oral structures.

b is incorrect. See the above reasons. c is incorrect. Development of mucositis may impede speech

rehabilitation and slow progress temporarily however.

5. How is adjuvant radiotherapy anticipated to aﬀect swallow outcomes?

a) It will improve swallow outcomes. b) It will not aﬀect swallow outcomes. c) It will hurt swallow outcomes.

is appropriate for oral con-

Answer: c is correct. It is well known that radiotherapy has deleterious immediate and long-term eﬀec ts on swallow function, including late-onset, radiation-associated dysphagia.

a is incorrect. Radiotherapy and its concomitant sequelae such as mucositis, erythema, and possibly thrush may promote disuse atrophy through periods of reliance exclusively on enteral nutrition.

b is incorrect. Radiotherapy generally has a deleterious eﬀect on swallowing outcomes.

45.5 Description of Disorder and Recommended Treatment

Repeat swallow study on postoperative day 7 revealed oral stage def icits characterized by significant anterior loss of secretions as well as bolus materials. Floor of mouth (oral) bolus retention was also observed with poor anteroposterior propulsion. The pharyngeal phase was intact with functional airway protection. However, several submental lucencies were noted without an overt fistula (▶ Fig. 45.1) and it was recommended TL consume nothing by mouth. Three days later, TL developed a right orocutaneous fistula warranting packing (▶ Fig. 45.2). The packing was removed after 2 weeks and following two courses of antibiotics.

Postoperative oral motor evaluation revealed a nicely seated anterolateral t high flap in the floor of mouth and right mandible. Neck incisions were slightly erythematous, but dry. Packing was observed per right neck incision, presumably where Penrose drain was removed. The patient was edentulous with malocclusion and open-mouth post ure, resulting in the inability to achieve labial approximation, right less than left, due to f lap edema. Lingual strength/range of motion was preserved. Anterior loss of secretions was resolved with adequate secretion management observed during speech/swallow tasks.

Postoperative speech evaluation revealed intact diadochokinesis, and remarkably preserved intelligibility despite an inability to approximate the lips with anticipated deficits in production of labiodentals, bilabials, and interdentals. These deficits were attributed to acute postoperative edema and anticipated to resolve in the following few days; thus, rehabilitation of these sounds was not indicated. Overall intelligibility was 95% in known contexts and 85% in unknown contexts.

Pre-op POD 7 POD 25 1month post-IMRT 3months post-IMRT

PSS-HN 100 0 0 60 80

FOIS 7 1 1 5 6

DIGEST 0 1 2 1 0

Safety 0 0 1 1 0

Efficiency 0 2 2 1 0

Deficit phonemes None /m/, /f/, /v/, /b/, /p/,

/∫/, /ʒ/

/f/, /v/, /∫/, /ʒ//∫/, /ʒ//∫/, /ʒ/

170

Management of Swallow Function in a Patient with an Orocutaneous Fistula

Fig. 45.1 Lateral postoperative radiographic view of composite mandibulectomy with several submental opacities; no overt leak.

Postoperative fistula is an unanticipated and uncommon complication following surgical resection that warrants vigilance from the speech-language pathologist (SLP) for identification and management of speech/swallowing functions during the healing process. Although not easily predictable, several risk factors warrant monitoring, including anemia, cachexia, history of radiation, large tumor volume warranting free-flap reconstruction, and glottal primary tumors that require total laryngectomy. The presence of one or more of these risk factors is associated with a 13% to 35% chance of postoperative fistula development.

Management of orocutaneous fistula is the responsibility of the surgical team and a conservative approach is generally taken that includes wound packing, administration of antibiotics, and delayed return to oral diet. SLPs play an important role in early identification or verification of fistula, guide return to oral diet, and provide interval rehabilitation during wound healing. Therapeutic exercises are not contraindicated with an active fistula. However, an oral diet is contraindicated because it may prolong wound healing and yield complications such as flap dehiscence, loss of flap viability, and persistent infection.

45.6 Outcome

Although TL remained nil per os for several weeks postoperatively and experienced delayed initiation of radiation therapy, she ultimately resumed a liberal oral diet without restriction and achieved intelligible speech, both of which she was pleased with. Speaking with her several months following completion of IMRT, TL and her daughter stated that routine contact with SLP and education regarding reliable time frames for expected

Fig. 45.2 anterolateral postoperative radiographic view of composite mandibulectomy with barium contrast per pharyngoesophageal lumen with visible left-sided anterior orocutaneous extravasation of contrast material concerning the leak.

return to oral diet and return of speech function made recovery manageable even during times of unanticipated complication. She was adherent with dysphagia therapy throughout her cancer treatment. Despite the absence of penetration/aspiration during instrumental swallow evaluation, super-supraglottic swallow, Mendelsohn’s maneuver, and hard swallow were recommended 20 times a day to preserve function. Prophylactic intervention continued during IMRT, but her approach was altered to incorporate the Pharyngocise protocol, which consists of four sets of 10 of the following exercises:

●

Sustained falsetto phonation for 5 seconds.

●

Continuation of Mendelsohn’s maneuver with 5-second hold.

●

Tongue press with 5-second hold (instructions to patient: “Press your entire tongue against the roof of your mouth and hold it”).

●

Five-second manual jaw stretch (instructions to patient: “Open wide and pull down on your chin to increase the stretch”).

She continued these exercises for 1 month following completion of IMRT and maintained an oral diet, which she modified to soft solids and thin liquids only during peak radiation toxicity. She was followed by the SLP with evaluations 1 and 3

171

Management of Swallow Function in a Patient with an Orocutaneous Fistula

months after IMRT, at which point she reported satisfaction with her swallow and speech and was deemed to have achieved maximal rehabilitative potential.

45.7 Key Points

●

Identification of risk factors and early identification of fistula results in slightly prolonged return to oral diet.

●

The SLP plays an integral role in educating the patient preoperatively, regarding likelihood of surgical and radiotherapy risks, delineating timeline for return to oral diet, as well as informing patients of anticipated speech and swallow deficits with guided postoperative rehabilitation.

●

Guided SLP intervention is important in not only the postoperative period, but also pre-, peri-, and postradiation therapy.

Suggested Readings

[1] Clarke P, Radford K, Coﬀey M, Stewart M. Speech and swallow rehabilitation

in head and neck cancer: United Kingdom National Multidisciplinary Guidelines. J Laryngol Otol. 2016; 130 S2:S176–S180

[2] Hutcheson KA, Barrow MP, Barringer DA, et al. Dynamic Imaging Grade of

Swallowing Toxicity (DIGEST): scale development and validation. Cancer. 2017; 123(1):62–70

[3] van la Parra RF, Kon M, Schellekens PP, Braunius WW, Pameijer FA. The prog-

nostic value of abnormal findings on radiographic swallowing studies after total laryngectomy. Cancer Imagi. 2007; 7:119–125

172

Evidence-Based Intervention for Dysarthria in a Patient with ALS

46 Evidence-Based Intervention for Dysarthria in a Patient with Amyotrophic Lateral Sclerosis

Amy Lustig

46.1 Introduction

Nearly all individuals diagnosed with amyotrophic lateral sclerosis (ALS) will experience a decline in speech communication at some point during their illness. Management of dysarthria to maximize communicative competence is a key target for speech-language pathologists and must employ evidence-based practices to optimize eﬃcacy.

46.2 Clinical History and Description

DS was a 71-year-old, community-dwelling Caucasian man who was seen for speech pathology evaluation approximately 6 months after being diagnosed with ALS, following a long career as a civil engineer. His medical history included high blood pressure that was well controlled with diuretics, gastroesophageal reflux disease (GERD), including frequent heartburn symptoms (poorly managed with chewable antacids), and mild, untreated depression and anxiety. During the evaluation, DS reported increased diﬃculty speaking and swallowing. He also reported diﬃculty typing, which was particularly problematic as he was in the process of completing a book and also interfered with simple email communications. In addition, he reported diﬃculty ambulating and with self-care. He described his speech as “softer and quieter,” as well as requiring increased eﬀort to “get the words out.” These issues were particularly noticeable later in the day, on the telephone, and in group settings. He also regularly coughed and occasionally drooled while speaking. DS’s wife confirmed that his speech intelligibility was progressively worsening, and she occasionally had to ask him to repeat himself several times to increase intelligibility. They agreed that these speech changes had worsened over the past 9 to 12 months prior to receiving the ALS diagnosis (See Appendix).

46.3 Clinical Testing

Structured testing of DS’s speech, language, and cognitive status was conducted. Each test has been standardized for diagnostic purposes except for the Montreal Cognitive Assessment (MoCA), which provides a cognitive screening score, and is not a comprehensive test of cognitive status. On the Frenchay Dysarthria Assessment (FDA-2), erately severe and mixed upper- and lower-motor neuron dysarthria. Lip function was better than laryngeal and tongue function, with a severely impaired cough reflex, visible lingual fasciculations, and variable palatal function, which were somewhat better during swallowing than speech. A mildly slumped posture and slow speech rate were identified as factors potentially influencing testing results, and the constant presence of oral secretions interfered with speech production and intelligi-

DS was classified as having a mod-

bility. On a perceptual dysarthria profile utilizing items from the Robertson Dysarthria Profile (Revised), sification of moderately severe mixed spastic–flaccid dysarthria characterized by slow speech rate, equal and excess/reduced stress and pitch, hypernasality, strained-strangled vocal qualit y, and distorted consonant and vowel production, particularly for voiced/voiceless cognates, fricatives, and complex consonant blends. Overall speech intelligibility was 75% in conversation and 85% for single words and short phrases. Vocal intensity ranged between 59 and 64 dB during conversation (typical conversational range =72–77 dB).

On the MoCA, mal range for his age; however, deficits in visuoconstruction skills, delayed word recall, and language fluency were observed. On the Boston Naming Test (BNT), normal range for his age. DS and his wife denied diﬃculties with word finding, language comprehension, reading, or writing, other than those associated with the physical mechanics of these activities (e.g., holding a pen, etc.).

DS obtained a score of 27/30 within the nor-

DS obtained a clas-

DS scored 14/15, within the

46.4 Questions and Answers for the Reader

1. Are eﬀortful exercises targeting articulatory or respiratory

strength and range of motion appropriate for DS?

a) Yes, because they will help increase muscle strength and

control.

b) No, because they will not improve muscle strength or

control and will fatigue DS.

c) Yes, because that is traditionally how speech pathologists

treat dysarthria of speech.

d) No, because respiratory strength does not aﬀect the

clarity of speech articulation.

Answer: b is correct. Because the pathophysiology of ALS is characterized by progressive and permanent decline in the numbers of f unctional motor neurons, the benefit of eﬀortful exercise in ALS is controversial. No randomized trials of eﬀortful exercise targeting speech articulation in ALS have been published, and a small handful of case studies report detrimental eﬀects of intensive exercise on speech intelligibility and vocal

5,6

quality.

a is incorrect. A 2013 Cochrane review of randomized and quasi-randomized studies involving resistance and/or aerobic exercise training for individuals with ALS or other motor neuron disease found no studies regarding aerobic exercise in this population and little evidence of the benefits of resistance training.

c is incorrect. Intervention goals and targets should be tailored to specific needs and circumstances, account for the nature of the underlying impairment, and be based on the best available clinical evidence for predicted treatment choices and eﬀects. Dysarthria interventions targeting muscle strength are

173

Evidence-Based Intervention for Dysarthria in a Patient with ALS

appropriate when the underlying impairment can accommodate the core target of the intervention (i.e., when motor neurons are available to facilitate increased muscle contractile strength). When the underlying condition is inconsistent with traditional interventions, such as in ALS, which is associated with progressive decline in the number of available motor neurons, alternative approaches to maximizing muscle strength for dysarthria management must be utilized.

d is incorrect. Strong and positive correspondences between respiratory muscle strength and intelligible speech are well recognized.

2. Would it be appropriate at this point in DS’s illness trajectory

to introduce the use of augmentative and alternative communication (AAC) strategies? a) No, because his speech is still understandable to others

who know him well.

b) No, because he would prefer to speak rather than employ

a compensatory strategy.

c) Yes, because he should stop speaking now to spare his

strength.

d) Yes, because it can provide him with communication

options in the future.

Answer: d is correct. Individuals with ALS should be encouraged to maximize intelligibility through managing fatigue, conserving speech and voice output, and the use of augmentative/ compensatory communication strategies.

a is incorrect. Even though DS may be reasonably well understood by familiar individuals, his speech intelligibility will continue to decline. Additionally, it would be useful for some of his closest relatives and friends to become familiar with the alternative communication strategies he may choose to employ, to improve his comfort with using them, and provide assistance.

b is incorrect. Virtually everyone would prefer to speak rather than utilize an alternative approach to communication with the critical caveat that if DS were to assert that he would rather rely solely on verbal communication for the duration of his illness, and was fully disinterested in exploring alternatives, it would be necessary to respect his wishes, assuming that he was fully aware of the consequences of this choice.

c is incorrect. Although alternative communication strategies can spare eﬀort and conserve energy, DS would likely have little motivation to stop speaking if his speech was still reasonably intelligible. There is insuﬃcient evidence to support substantially avoiding motor speech production in the interest of preserving energy while speech is still reasonably intelligible. However, it would be appropriate at this point to introduce the advantages of incorporating adjuncts to speech communication that DS might consider implementing for even short periods of time. For example, using a voice amplifier during telephone calls or in other challenging communication situations could increase intelligibility and communicative eﬀectiveness.

3. What would you expect to happen with DS’s speech intelligi-

bility over the course of his illness? a) It will get progressively worse. b) It will remain at the current level indefinitely. c) There is a reasonable chance for improvement in speech

intelligibility.

d) It will change from day to day, from very intelligible to

poor intelligibility.

Answer: a is correct. It is well recognized that speech intelligibility progressively worsens for the vast majority of ALS patients, with more rapid decline in bulbar cases.

b is incorrect. Diﬀerent individuals decline at diﬀerent rates, but over time, virtually all will show a decrement in speech articulation and clarity.

c is incorrect. No cases of improved speech intelligibility in patients with ALS have been reported.

d is incorrect. Although speech intelligibility can change to some extent from day to day due to factors including fatigue, stress, and medication eﬀects, overall speech intelligibility is recognized to progressively worsen over time.

4. In addition to DS’s articulatory distortions, what other

factors could influence his intelligibility?

a) Background noise, such as music, television, or

conversation.

b) A reluctance to speak in public due to feeling self-con-

scious about poor speech. c) Pressure to keep up with a rapidly paced discussion. d) All of the above.

Answer: d is correct. Many factors contribute to intelligibility. Background noise or other distractions can draw our attention away from the speaker or pose an auditory challenge that makes decoding spoken language more diﬃcult. Many individuals with ALS with dysarthria and other morbidity associated with the disease struggle with these changes and suﬀer from negative eﬀects on self-confidence and personal identity. Even with close friends or family, communication and cognitive issues co-occurring with ALS can result in diﬃculty participating in group conversations that are fast paced, have complex or

uent topic changes, or are competitive.

freq

46.5 Description of Disorder and Recommended Treatment

ALS is a fatal, neurodegenerative disorder aﬀecting upper and lower motor neurons of the spinal cord, motor cortex, and brainstem yielding progressive weakness and wasting of limb, bulbar, and respiratory musculature. Most individuals diagnosed with ALS experience progressive impairment of speech and swallowing function, typically associated with bulbar decline. Speech dysarthria in ALS is most often of the mixed flaccid/spastic type, given both upper and lower motor neuron involvement. Decreased speech intelligibility is due mainly to reduced movement of oral musculature and phonation changes resulting in slowed, slurred, and imprecise speech with harsh, hypointense, hypernasal, and monotonic vocal resonance.

DS was seen regularly for speech therapy visits for 8 months; therapy was scheduled weekly for 1 month, then biweekly for 2 months, once every 3 weeks for 2 months, and then again biweekly. DS was acutely aware of the decline in his speech intelligibility and voice quality. He initially requested exercises to maximize articulatory and vocal competence, but reluctantly acquiesced when provided with the rationale for avoiding them. A compromise was reached whereby he was provided with lists of challenging words and phrases on the condition that he agreed to practice them with another person for intelligibility feedback only, and not in a repetitive or fatiguing manner. Four

174

Evidence-Based Intervention for Dysarthria in a Patient with ALS

lists were provided over the course of 10 weeks, and these were periodically reviewed during speech therapy visits. DS was provided a portable voice amplifier and lightweight headset microphone to increase loudness and phonemic contrasts while with minimal eﬀort. DS employed this device in communication situations he considered particularly challenging, such as during mealtimes, automobile trips, and telephone conversations.

DS also participated in two trials (one during the first month of therapy and one at the end of the third month) of an AAC device capable of generating digital speech and a variety of interface modalities, including direct keyboard selection, head mouse, scan/switch interface, and eye-gaze technology. DS’s speech intelligibility declined substantially over 8 months of therapy, from 75% to 45% in conversation. Though he was initially reluctant to consider using a speech-generating communication device, DS eventually recognized that he might eventually lose intelligible speech and use of his upper extremities. AAC approaches are typically successful with individuals with ALS often up to within a few weeks of death and are acknowledged as a central component of speech pathology in-

tervention for this population.

AAC solutions range from “lowtech” approaches such as voice amplifiers, letter boards accessed via laser pointer, head mouse, scan/switch interface, or caregiver assistance wit h pointing or shared eye-gaze strategies to “high-tech” approaches such as speech-generating computerized systems accessed via sophisticated eye-tracking technology. The likelihood of upper and lower extremity impairment must be considered.

Additionally, it was suggested that DS contact his medical provider to discuss his reflux symptoms and the possibility of pharmacological management. Reducing oral secretions during speech as well as coughing/choking events during swallowing may be reduced by improved reflux management.

46.6 Outcome

A summary of treatment targets and outcomes is provided in

▶ Table 46.1. DS was generally willing to utilize the voice ampli-

fier in a private setting, such as in his apartment, which his wife (who had mild hearing loss) found very helpful to improve his speech intelligibility, nearly eliminating her requests for him to repeat himself. He initiated use of the device himself only infrequently, but was generally receptive when it was suggested that he utilize it, and eventually self-initiated use of the voice amplifier on approximately 85% of appropriate opportunities. Initially, DS was particularly reluctant to utilize the device in public settings such as the dining room at the residential community where he lived. He had the device for approximately 2 months before he used it in public, at a meal with friends, about which he reported a benefit of having much better success at maintaining his presence during conversation, though he found the microphone interfered with the mechanics of eating when it was too close to his mouth. At the end of 8 months, DS and his wife both confirmed that he utilized the voice amplifier in public settings in approximately 65% of opportunities, with 75% success for conversation participation; limitations were associated with keeping up with the speed of group conversations, fatigue, and some distraction when the device was placed on the table rather than worn around the waist.

Table 46.1 Dysarthria intervention targets and outcomes

Intervention Target % Com-

Voice amplifier (private setting)

Voice amplifier (public setting)

PPI use Secretion

AAC use (eye gaze)

AAC use (head mouse)

AAC, augmentative and alternative communication; PPI, proton pump inhibitor.

Speech intelligibility 80 90

Speech intelligibility 65 75

management

Generated speech for communication; rapid assistance; manuscript writing/editing; book reading

pliance

100 100

90 70

90 85

% Success

DS was prescribed a proton pump inhibitor (PPI) to manage his GERD symptoms and possibly reduce oral secretions. Within 2 weeks of starting the medication, DS reported he stopped coughing and choking while eating and that his GERD symptoms resolved. Coughing and drooling during speech was also eliminated.

Two therapy sessions consisted of trials of various AAC options; DS selected a dedicated speech-generating device that oﬀered a variety of interface modalities, including eye-gaze, head mouse, and scan/switch approaches. This device was delivered during the fourth month of therapy. DS preferred the eye-gaze technology, which he used with approximately 70% success in approximately 90% of opportunities, and considered to be only a moderately successful outcome. Challenges in device use were associated with several factors, including fatigue, seating and positional issues, and eye-gaze calibration for accessing targets at the edges and corners of the display screen versus those in the more central screen. Modifications were made to the visual presentation of preferred programs to minimize the need to access targets at display edges and corners. DS’s wife and children were also trained in device use, simple programming, and troubleshooting.

During the seventh month of therapy, DS’s eyelids began to droop slightly and his ocular secretions became more viscous, two developments that further interfered with successful use of the eye-gaze interface. At that point, DS transitioned from eye-gaze to head mouse use, which he accomplished with somewhat better success (85%), again in approximately 90% of relevant opportunities. Diﬃculties with head mouse use were primarily associated with maintaining a comfortable upright head position and coordinating the timing of head movement to the desired target as well as persistent and rapid fatigue. For both eye-gaze and head mouse interface modalities, training was provided to generate spontaneous spoken words and phrases, to create a database of organized words and phrases that could be quickly accessed to communicate daily needs, and to ensure access to applications such as digitized books and

175

Evidence-Based Intervention for Dysarthria in a Patient with ALS

quick select help options to meet DS’s expressed needs and interests. DS also utilized the keyboard to continue work on his book; he completed the manuscript. DS’s wife and children were essential partners to keep the device readily available to him through reminders, direct assistance, and solving problems as well as positive reinforcement.

46.7 Key Points

●

ALS is a fatal disease, with progressive decline throughout disease trajectory, though the rate and magnitude of functional decline varies across individuals.

●

AAC in patients with ALS is preferred over eﬀortful exercise for maximizing speech communication and minimizing fatigue.

●

People with ALS may be receptive to diﬀerent types of AAC interventions at diﬀerent points in time (e.g., voice amplifier vs. speech-generating device) and may require modifications to AAC strategies over time. It is critical to maintain contact over the course of the disease to ascertain shifting needs and priorities and to link the goals of device use with personally meaningful activities.

●

Engaging family members to assist and encourage AAC use can make a substantial diﬀerence in patient access and success.

●

It is important to identify and address symptoms, such as drooling or acid reflux, through medical management to maximize the benefit of functional speech therapy goals and objectives.

Suggested Reading

[1] Hanson EK, Yorkston KM, Britton D. Dysarthria in amyotrophic lateral sclero-

sis: a systematic review of characteristics, speech treatment and augmentative and alternative communication options. J Med Speech-Lang Pathol. 2011; 19(3):12–30

References

[1] Enderby PM, Palmer R. Frenchay Dysarthria Assessment. 2nd ed. Austin, TX:

Pro-Ed; 2008

[2] Robertson SJ. Dysarthria Profile (Revised). Chesterfield, UK: Winslow Press;

1982

[3] Nasreddine ZS, Phillips NA, Bédirian V, et al. The Mont real Cognitive Assess-

ment, MoCA: a brief screening tool for mild cognitive impairment. J Am Geriatr Soc. 2005; 53(4):695–699

[4] Kaplan E, Goodglass H, Weintraub S. The Boston Naming Test. 2nd ed. Phila-

delphia, PA: Lea & Febiger; 1983

[5] Dworkin JP, Hartman DE. Progressive speech deterioration and dysphagia in

amyotrophic lateral sclerosis: case report. Arch Phys Med Rehabil. 1979; 60 (9):423–425

[6] Watts CR, Vanryckeghem M. Laryngeal dysfunction in amyotrophic lateral

sclerosis: a review and case report. BMC Ear Nose Throat Disord. 2001; 1(1):1

[7] Dal Bello-Haas V, Florence JM. Therapeutic exercise for people with amyotro-

phic lateral sclerosis or motor neuron disease. Cochrane Database Syst Rev. 2008:CD005229

[8] Beukelman D, Fager S, Nordness A. Communication support for people with

ALS. Neurol Res Int. 2011:714693

Appendix: Sound Clips Collected Approximately 10 Months following DS Acquiring the Diagnosis of Amyotrophic Lateral Sclerosis

1. SMR (sequential motion rate) – pa-pa-pa.

2. SMR (sequential motion rate) – ta-ta-ta.

3. SMR (sequential motion rate) – ka-ka-ka.

4. AMR (alternate motion rate) – pa-ta-ka.

5. Counting 1 to 5.

6. Counting back ward 5 to 1.

7. Pitch glide up.

8. Pitch glide down.

9. Prolonged /ah/.

10. One-syllable words: pet – thumb – dish – neck – safe – zero – juice.

11. Two-syllable words: behind – weather – rubbish – message – kitchen – power – finger.

12. Three-syllable words: yesterday – passenger – beautiful – visitor – tobacco – direction – charity.

13. Four-syllable words: population – development – majority – fundamental – generation – humanity – liberation.

14. Short phrases: You have to pay. – Go to bed. – She looks sad. – Where’s my coat? – Do what you like. – Is that a joke? – I need my hat.

15. Longer phrases: My daughter is a nurse. – What do you think? – She gave me a coin. – Can you go to the shop? – Put it in a dish.

16. Spontaneous speech sample: Byberly has … has a greenhouse that my wife is very much involved with. Uh … she mainly has orchids there. And sometimes she has to repot them and she shows other ladies how to repot them. Uh … A couple days ago, a man put a plant in there that they think And she got an expert in there and they are attempting what disease they may have. Um … there is a great interest in orchids around here because several of the ladies have got them.

has a disease and would spread to the other plants.

Structured word and phrase targets were obtained from the Frenchay Dysarthria Assessment evaluation materials.

176

Rehabilitation of Right Hemisphere Disorder in the Chronic Phase of Recovery

47 Rehabilitation of Right Hemisphere Disorder in the Chronic Phase of Recovery

Jamila Minga

47.1 Introduction

High-quality studies to direct treatment for cognitive-communication deficits associated with right hemisphere deficits are limited, likely related to the broad heterogeneity of this patient population. Theories and treatment approaches from other neurogenic populations can be employed, however, to guide rehabilitation.

47.2 Clinical History and Description

CP was a 48-year-old woman with a history of diabetes mellitus and hypertension who presented with left-sided weakness and facial droop after decreased responsiveness at the dinner table. Computed tomography (CT) of the brain revealed an infarct of the right middle cerebral artery (MCA) distribution. CP reported that she “lost her filter” and the ability to “organize her life.” Close relatives and friends reported that CP frequently made inappropriate comments and did not allow them to participate in conversations. CP lived independently with her two daughters, aged 13 and 9 years. She had a graduate degree and worked as an administ rator for a health care insurance company where she supervised eight employees. CP sought treatment for residual executive functioning deficits, and expressed a desire to reengage in community and occupational activities.

47.3 Clinical Testing

An initial cognitive profile was conducted approximately 10 months following CP’s stroke. The Cognitive-Linguistic Quick Test was administered. mild for attention and visuospatial skills and moderate for executive functions (EF). Memory and language were within normal limits (WNL; ▶ Table 47.1). Empathy was measured as below average; she scored a 45 on the Toronto Empathy Question-

naire.

Table 47.1 Pretreatment cognitive domain severity scores

Cognitive domain

Attention 149 215–180 179–125 124–50

Memory 177 185–155 154–141 140–110

Executive functions

Language 33 37–29 28–25 24–21

Visuospatial skills

Score Ranges of severity

17 40–24 23–20 19–16

60 105–82 81–52 51–42

Cognitive domain severity ratings were

Within normal limits

Mild Moderate

Visuospatial neglect was also observed; CP eliminated numbers 6, 7, and 8 from her clock drawing and eliminated the leftmost aspect of the page during maze and design generation tasks. Neglect was assessed further using the Apples Cancellation

Both egocentric and allocentric left neglects were

Test. observed. The total number of complete apples selected was 40/ 50 and the total number of false positives, selection of apples open on the left side, was 6/50. False positives were isolated to the lower left quadrant of the page.

47.4 Questions and Answers for the Reader

1. Knowledge of brain–behavior relationships can guid e assessment and treatment approaches. CP had damage to the right MCA distribution. What cerebral lobes are most likely involved when EF and visuospatial skills are impaired?

a) Right frontal lobe and temporal lobe. b) Right frontal lobe and parietal lobe. c) Left frontal lobe and temporal lobe. d) Left temporal lobe and right occipital lobe.

Answer: b is correct . The MCA supplies most of the lateral surface of the cerebral hemispheres, the thalamus, and basal ganglia. Since the right MCA was involved, damage to the right frontal lobe (EF) and the right parietal lobe (visuospatial skills) is likely.

a is incorrect. The temporal lobe is not associated with

impaired EF or visuospatial skills.

c is incorrect. The cerebrovascular accident (CVA) occurred in

the right MCA, which does not supply the left hemisphere.

d is incorrect. The CVA occurred in the right MCA, which does not supply the left hemisphere. The occipital lobe is associated with visual perception.

2. CP scored below average on the Toronto Empathy Question-

naire. If she plans to retur n to the workforce in her previous position, which of the following areas may be impacted by

diminished empathy? a) Communication. b) Organization. c) Supervision. d) Both a and c.

Answer: d is correct. Empathy can be thought of as the ability to take another’s point of view with respect to desires, needs, and emotional state. Empathy plays a role in communication in that the manner in which a person decides how and when to communicate includes the perspective of communication partners. Supervision requires the ability to communicate eﬀectively and to demonstrate empathy for employees.

b is incorrect. Empathy does not influence organizational

capabilities. Organization can be subsumed under EF.

177

Rehabilitation of Right Hemisphere Disorder in the Chronic Phase of Recovery

3. If a clinician seeks to employ the client’s concerns as the basis for prioritizing treatment, which area should be prioritized? a) EF. b) Left neglect. c) Pragmatic communication.

Answer: a is correct. Most of CP’s limitations can be captured under EF. She reported diﬃculty organizing her life and filtering communication. Furthermore, her prior occupation necessitated intact executive functioning. Since evidence-based treatment exists for deficits of executive functioning in the literature of acquired disorders (e.g., traumatic brain injury), therapy should target executive functioning first.

b is incorrec t. Neglect was not identified by CP as one of the primary motives for seeking treatment. In addition, her neglect was isolated to one visual field quadrant and she seems to be functioning with the neglect. Additional testing may be warranted to further assess visual capabilities, and specifically to determining whether the observed deficit is truly neglect or an anopsia.

c is incorrect. Cognitive processes are thought to contribute to deficits in pragmatic communication, with respect to language production, in adults with right hemisphere brain damage (RHD). Pragmatic communication may be observed concurrently with improvement in cognitive domains, especially if the cognitive treatment includes the use of communicativetasks.

47.5 Description of Disorder and Recommended Treatment

approach in the chronic phase of recovery. This approach, however, requires close interaction with a neuro-ophthalmologist. In the absence of such collaboration, standard therapy for neglect is visual scanning training. Career counseling was also recommended. CP supervised eight employees, and at the onset of therapy, it was unclear whether she could return to work in this capacity.

47.6 Outcomes

CP participated in individual treatment once per week for 10 weeks and then transitioned to ongoing group treatment. Individual treatment focused on executive functioning with embedded communicative activities and visuospatial skills. Group treatment continued with a frequency of once a month. Group treatment focused on EF skills, planning, problem solving, and organization.

A cognitive profile was gathered after 12 weeks of treatment. Improvement was noted for the domains of attention, visuospatial skills, and EF (▶ Table 47.2). Anecdotally, CP reported that communication with close friends and loved ones improved. She reported that she was more conscious of what and how she communicates and that she asked more questions in conversation. CP returned to work part time as an administrative assistant.

Given that CP was in the chronic stage of recovery, it was likely that the gains were related to treatment and not spontaneous recovery. This concept, however, was not formally assessed. An inability to determine whether such improvements would have occurred simply with time precludes such a conclusion.

RHD can result in cognitive-linguistic def icits that impact quality of life. The representation of these deficits can vary greatly. Adults with RHD typically express themselves with appropriate grammar, morphology, and syntax (i.e., preserved basic language abilities). However, as noted in the current case, pragmatic aspects of language may be impaired. Underlying cognitive deficits have been postulated to contribute to abnormal pragmatic communication. nication is not widely examined in the RHD literature, literature can direct the treatment of cognitive deficits. tive deficits may result in improved communication.

CP had deficits of attention, executive functioning, and pragmatic communication in the chronic phase of recovery. A functional approach to rehabilitation utilizes patient motivation; considering CP’s goal to return to work and her perceived inability to organize, skilled treatment for EF and visuospatial neglect was recommended. EF therapy was consistent with the EF/frontal lobe dysfunction model. Deficits of EF can impact all aspects of communication; impaired social interactions may be related to an inability to organize thoughts, shift attention, and plan. Within this model, treatment foci included organization, planning, and problem solving. Moreover, it was recommended that communicative activities and tasks be embedded in EF treatment. CP was motivated to return to work and desired improved organization at home.

An approach to the treatment of chronic neglect is prism adaptation intervention. Growing evidence supports this

2,4

Although pragmatic commu-

Targeting cogni-

47.7 Key Points

●

RHD can result in a number of cognitive-linguistic deficits that can negatively impact quality of life. There is an increasing need to understand the utility of improved cognitive process on pragmatic communication.

●

EF is one cognitive deficit that may contribute to aberrant communication behaviors in RHD.

●

EF can be targeted in structured context using evidence-based treatments. Targeting EF may result in improvements in pragmatic communication, particularly if communicative act ivities are used in treatment (e.g., organizing a speech or problem solving what goes wrong in a conversation).

Table 47.2 Posttreatment cognitive domain severity scores

Cognitive domain

Attention 189 215–180 179–125 124–50

Memory 172 185–155 154–141 140–110

Executive functions

Language 34 37–29 28–25 24–21

Visuospatial skills

Score Ranges of severity

Within normal limits

22 40–24 23–20 19–16

62 105–82 81–52 51–42

Mild Moderate

178

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