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J Dev Phys Disabil (2008) 20:209–216 DOI 10.1007/s10882-008-9105-9 O R I G I N A L A RT I C L E

Learning in Post-coma Persons with Profound Multiple Disabilities: Two Case Evaluations Giulio E. Lancioni & Marta Olivetti Belardinelli & Claudia Chiapparino & Maria Teresa Angelillo & Fabrizio Stasolla & Nirbhay N. Singh & Mark F. O’Reilly & Jeff Sigafoos & Doretta Oliva

Published online: 15 April 2008 # Springer Science + Business Media, LLC 2008

Abstract Finding signs of learning in post-coma persons with profound multiple disabilities (i.e., in a vegetative state or minimally responsive) would underline (a) an awareness/consciousness of their responding and its links with environmental stimuli and (b) a positive development in their immediate situation with the possibility of treatment (rehabilitation) advances. This study was aimed at assessing signs of learning in two of these persons (i.e., two adults). The learning setup involved eye blinking as the persons’ responses and microswitch technology to detect such responses and to present stimuli. The technology consisted of an electronically regulated optic sensor mounted on an eyeglasses’ frame that the persons wore during the sessions and a control system connected to stimulus sources. The study involved an ABABCB sequence, in which A represented baseline periods, B intervention periods with stimuli contingent on the responses, and C a control condition with stimuli presented non-contingently. Data showed that the level of responding during the B phases was significantly higher than the levels G. E. Lancioni (*) : C. Chiapparino : M. T. Angelillo : F. Stasolla Department of Psychology, University of Bari, Via Quintino Sella 268, 70100 Bari, Italy e-mail: [email protected] M. O. Belardinelli University “La Sapienza” of Rome, Rome, Italy N. N. Singh ONE Research Institute, Midlothian, VA, USA M. F. O’Reilly University of Texas at Austin, Austin, TX, USA J. Sigafoos Victoria University of Wellington, Wellington, New Zealand D. Oliva Lega F. D’Oro Research Center, Osimo, Italy

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observed during the A phases as well as the C phase (i.e., indicating clear signs of learning by both persons). The implications of these data for assessment and rehabilitation of post-coma persons with profound multiple disabilities are discussed. Keywords Microswitch technology . Eye-blinking responses . Multiple disabilities . Vegetative state . Minimal responsiveness . Minimally conscious state Treatment strategies frequently employed for improving the condition of post-coma persons with profound multiple disabilities (i.e., in a vegetative state or minimally responsive) include systematic sensory stimulation and music therapy (Crews et al. 1997; Gerber 2005; Giacino 1996; Lombardi et al. 2002; Magee 2005; Oh and Seo 2003; Wales and Waite 2005; Wilson et al. 1991, 2002). In spite of the large emphasis on these forms of intervention, the literature available presents relatively limited amounts of usable (reliable) data and fairly heterogeneous outcomes (Aldridge et al. 1990; Avesani et al. 2006; Giacino and Trott 2004; Lombardi et al. 2002; Wales and Waite 2005). The heterogeneous outcomes seem to underline, among others, differences in the persons’ neurological conditions as well as difficulties in drawing diagnostic statements with predictive implications for intervention effects and rehabilitation results (Jennett 2002; Lomber and Eggermont 2006). At least two hypotheses may be formulated with regard to persons with profound multiple disabilities who seem to remain in a vegetative state or are minimally responsive (e.g., showing some smiles to specific environmental stimuli) after long periods of systematic stimulation and music therapy. First, one may hypothesize that their level of functioning is so low that it precludes any strong increase in responsiveness (Jennett 2002). Second, one might also hypothesize (a) that they remain passive because the stimulation presented in those programs is not related to any specific response that could then become actively linked to it and/or (b) that the motor repertoire is so limited that no obvious responsiveness can easily emerge (Avesani et al. 2006; Haggard 2005; Lancioni et al. 2005c; O’Brien et al. 1994; Whitnall et al. 2006). Assuming that the second hypothesis is correct, a learning setup involving a most basic response and the use of stimuli contingent on it through the help of microswitch technology might be reliably adopted as an assessment strategy as well as a treatment procedure to help the person progress in his or her condition (cf. Boyle and Greer 1983; Lancioni et al. 2005b, c; Naude and Hughes 2005; Ptak et al. 1998). If the response increases when contingent stimuli are used and declines in the absence of stimuli or when the stimuli are unrelated to the response, one may suggest that the person is showing an awareness of the response–stimuli link (i.e., signs of learning) (Lancioni et al. 2003, 2005c; O’Brien et al. 1994; Saunders et al. 2003). Observing signs of learning might help replace a rating of vegetative state with a rating of minimally conscious state (cf. Giacino 2004; Morin 2006) or may provide explicit support and justification for the use of the latter rating if this was already being adopted (cf. Giacino and Trott 2004; Haggard 2005). In both cases, the learning signs would assert the person’s new knowledge and call for an extension of the intervention process in use with the establishment of new responses (Giacino

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2004; Watson et al. 1999). This in turn would increase the person’s overall level of activity and provide basic choice opportunities with the possibility of further progress (Davis and Gimenez 2003; Giacino 2004; Giacino and Kalmar 2005; Lancioni et al. 2005c). This study was aimed at applying the learning setup outlined above with two post-coma adults who had profound multiple disabilities. The response adopted was eye blinking (i.e., a single blink or a sequence of two blinks within a 2-s interval, based on the characteristics of the participants). The microswitch technology consisted of an electronically regulated optic sensor mounted on an eyeglasses’ frame that the participants wore during the sessions and a control system linked to stimulus sources (cf. Lancioni et al. 2005a, 2006). Method Participants The participants (Luke and Hilda) were 29 and 49 years old, respectively. Both participants had a normal life until they were involved (about 5 and 4 years prior to the beginning of this study, respectively) in road accidents with severe brain injury. Following the injury, they spent 2 and 7 weeks, respectively, in a coma from which they emerged in a condition that was rated as vegetative state combined with pervasive motor disabilities (spastic tetraplegia with minimal motor behavior) and lack of speech. Hilda also presented with general drowsiness and epilepsy. Both participants were fitted with a gastrostomy tube for nutrition, although Luke could be partially fed via the mouth as well. Their condition required them to spend their time in a wheelchair or in bed but they could orient their eyes to various stimuli. Luke also showed smiles in relation to some environmental stimuli (e.g., joyful laughing). This behavior had led to re-labeling his condition as minimal responsiveness (or minimally conscious state; see Giacino 2004; Giacino and Trott 2004). They lived at home with both parents and a sister (Luke) or the mother and a sister (Hilda) and received rehabilitation programs involving essentially physiotherapy and stimulation sessions. Their families had provided informed consent for their participation in this study. Position, Response, Microswitch Technology, and Stimuli During the study, both participants sat in their wheelchair. The response recorded for Luke (who tended to have low rates of eye blinking) consisted of a single eye blink (Tota et al. 2006). The response recorded for Hilda (who tended to have relatively high rates of eye blinking) consisted of a sequence of two blinks occurring within a 2-s interval (Lancioni et al. 2005a). The technology consisted of an electronically regulated optic sensor mounted on an eyeglasses’ frame that the participants wore during the sessions and a control system linked to stimulus sources (see Lancioni et al. 2005a; Tota et al. 2006). The optic sensor involved an infrared light-emitting diode and a mini infrared light-detection unit (positioned in front and to the external side of the participants’ left or right eye) (see Tota et al. 2006). When a response was

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detected, a signal was transmitted to the control system. This system in turn activated one or more stimuli (see below) for 4 or 6 s for the two participants, respectively, during the intervention phases. The stimuli used for the two participants were selected following interviews with their families and brief stimulus preference screening (Crawford and Schuster 1993). Screening involved four to ten nonconsecutive presentations of each of the stimuli suggested by the families as presumably pleasant. A stimulus was selected for the study if the participant seemed to alert to it during more than half of the presentations. The stimuli selected included familiar voices and stories, songs and musical items. Experimental Conditions The study involved an ABABCB sequence in which A represented baseline, B intervention phases with stimuli contingent on the response, and C a non-contingent stimulation phase (Barlow et al. 2006). Sessions lasted 10 min for Luke and 5 min for Hilda (on medical and caregivers’ advice) and typically occurred three to six times a day, based on participants’ availability. During the first baseline and first intervention phase, the participants were prompted to respond (i.e., a light air-puff at the corner of the eye) at the start of the sessions and after 30 s of nonresponding during the sessions. Prompting during the first intervention phase was to increase the frequency of response–stimulation pairings and possibly speed up response strengthening. Prompting during baseline served as a basic control for its use/impact during the first intervention phase. Responses were recorded automatically through a counter connected to the control system. A response would be ignored by the system if it occurred during a stimulus presentation interval (i.e., within the B and C phases) or an equivalent time interval (i.e., within the A phases) (Lancioni et al. 2005a). Baseline (A) Phases The two baseline (A) phases included six and 19 sessions for Luke and six and eight sessions for Hilda. The microswitch technology was available, but responses did not produce any stimuli. Intervention (B) Phases The three intervention (B) phases included 57, 87 and 28 sessions for Luke and 62, 33 and 21 sessions for Hilda. Procedural conditions were as during baseline except that responses produced the occurrence of stimuli. Non-contingent Stimulation (C) Phase This phase included 14 and 22 sessions for Luke and Hilda, respectively. Stimuli such as those used in the B phases were presented independent of responses, at a mean frequency matching that of the last 20 sessions of the second B phase.

Results The participants’ data are summarized in the two graphs of Fig. 1. Luke’s mean frequency of responses was about 20 per session during the first A phase, increased to 43 per session during the first B phase, and declined and increased again (to levels

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Fig. 1 The upper graph shows Luke’s data, the lower graph Hilda’s data. Each data point represents the mean frequency of responses over a block of two sessions. Data points with a single session can occur at the end of the single phases of the study

similar to those just reported) during the next A and B phases, respectively. A new decline in response levels (with a mean frequency of 22 responses per session) was observed during the C phase. This decline was followed by a new response increase (with a mean frequency of about 50 responses per session) during the final B phase. Hilda’s data differed from those of Luke in terms of overall frequencies (partially due to shorter sessions and a double blinking response), but presented a similar trend. That is, the mean frequencies were 24 to 27 for the B phases, 14 and 13 for the A phases, and 15 for the C phase. The differences between the A and B phases as well as between the B phases and the C condition were statistically significant (p< 0.01) for both participants on the Kolmogorov–Smirnov test (Siegel and Castellan 1988).

Discussion These data indicate that the learning setup using eye-blinking responses and microswitch technology with positive stimuli produced a fairly clear outcome with both participants. They showed response increases during the intervention periods when stimuli were contingent on their responding and response declines during noncontingent stimulation (i.e., indicating an awareness of the link between responding and consequences). These findings, which are noteworthy for both participants, appear even more remarkable for Hilda given her more severe condition including a tendency to drowsiness (cf. Lippert-Gruner et al. 2003).

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The implications of these findings are multiple. For example, the learning setup seems to add to the diagnostic strategies available for these persons and, specifically, to the neurological evidence of learning. This basically relies on some form of perceptual adjustment (relating to stimulus habituation and mismatch) rather than on active association (Kotchoubey et al. 2006; Morin 2006). The appearance of the aforementioned awareness (i.e., learning) could prompt a change of diagnostic label from vegetative state to minimally conscious state for Hilda and add strong, clarifying support to the rating of minimal responsiveness (minimally conscious state) available for Luke (Davis and Gimenez 2003; Giacino 2004; Giacino and Kalmar 2005). The learning evidence could raise expectations of cognitive and behavioral progress and encourage an extension of the intervention with the inclusion of additional responses (Avesani et al. 2006; Bekinschtein et al. 2005; Boyle and Greer 1983; Giacino and Trott 2004; Lomber and Eggermont 2006; Pape et al. 2005a, b). The responses could be used with microswitches allowing direct access to stimuli (as in the present study) or with Voice Output Communication Aids (VOCAs), that is, devices that the participant can activate to ask for contact with or specific help by the caregiver (cf. Schlosser 2003; Schlosser and Sigafoos 2006). The latter form of devices could extend the participant’s activation opportunities and also allow the assessment of his or her communication abilities, interests, and social– emotional conditions. Finally, extending the range of responses would provide the participant personal enrichment, in terms of output as well as environmental input, with positive consequences for his or her overall status, dignity, and quality of life (Schalock et al. 2005). New research could (a) assess the possibilities of a program extension (as discussed above) with both participants of this study and (b) pursue the replication of the present study with new persons with profound multiple disabilities (i.e., in a vegetative state or minimally responsive) to ascertain the generality of the reported findings and the strength and practicality of the intervention setup used here (Barlow et al. 2006; Kennedy 2005). New research could also examine possible correlations of the data obtained with a learning setup, such as that used in this study, with other types of data obtained from different behavioral and neurological assessments (Bekinschtein et al. 2005; Haggard and Eimer 1999; Kotchoubey et al. 2006; Pape et al. 2005a, b). In conclusion, the present study has suggested that a learning setup with suitable technology and apparently positive stimuli may be profitably used to enhance the assessment and treatment of post-coma persons with pervasive disabilities. Definite statements about such an approach would need to wait for additional evidence, given the limited amount of data available to date. The acquisition of new data in line with those obtained in this study could be highly encouraging as to the practically valuable function of the approach and provide fresh impetus for testing it further. References Aldridge, D., Gustorff, D., & Hannich, H. J. (1990). Where am I? Music therapy applied to coma patients. Journal of the Royal Society of Medicine, 83, 345–346. Avesani, R., Gambini, M. G., & Albertini, G. (2006). Case study. The vegetative state: A report of two cases with a long-term follow-up. Brain Injury, 20, 333–338.

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