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The cure PSP care guide: a telephonic nursing intervention for individuals and families living with progressive supranuclear palsy.


Progressive supranuclear palsy (PSP) is a rare, progressive, and terminal neurodegenerative disease characterized by problems with ambulation, balance, mobility, vision, speech, swallowing, and behavior during the 7- to 10-year course of the illness. Substantial evidence in the nursing literature supports the benefits of patient education, self-management, chronic disease management, telehealth, and nurse navigation programs, which enhance patient and caregiver knowledge, improve day-to-day management by developing an awareness of resources, decrease dependence on services, and address caregiver needs. The Cure PSP Care Guide is a targeted telehealth nursing intervention aimed at providing knowledge, guidance, and resources to the vulnerable individuals and families living with PSP; identifying local resources; and building community. During the course of two telephone calls, individuals and their caregivers are assessed to develop a Cure PSP Care Guide designed to provide guidance along the trajectory. A knowledge assessment, self-efficacy scale, and Caregiver Strain Index are administered before and after the intervention to determine the program intervention effect. Caregiver knowledge assessments improved after the intervention, whereas strain scores were static. Qualitative data show the ability of the intervention to address caregiver needs for knowledge and support, daily management tips, and resource identification. The preliminary quantitative and qualitative data collected on this pilot project justify further exploration of the use of telehealth to remotely deliver nurse case management to the vulnerable individuals and families living with PSP.

Keywords: caregiver strain, nurse case management, progressive supranuclear palsy (PSP), telehealth


The Cure PSP Care Guide

Cure progressive supranuclear palsy (PSP) states that "five to six persons per 100,000 have PSP, a number similar to that of Lou Gehrig disease, but only about a third of these have received the correct diagnosis" (Cure PSP, 2012). About 4500 people in the United States are diagnosed with this disorder (Cure PSP, 2012). Many physicians have not heard of PSP, and neurologists often initially diagnose the disorder as Parkinson disease. PSP is considered a terminal disease. Individuals gradually lose the ability to move, speak, and swallow over the 7- to 10-year course of the illness, ultimately contributing to the need for total assistance. Consequently, this is a vulnerable population marginalized because of the prevalence of the disease, lack of provider knowledge, and limited resources. The Cure PSP Guide aims to address this disparity.

PSP is one of a group of illnesses called Parkinson plus syndromes or atypical parkinsonian disorders. Wenning, Litvan, and Tolosa (2011) clarify that atypical parkinsonian disorders refer to the following neurodegenerative illnesses: multiple system atrophy, dementia with Lewy bodies, PSP, and corticobasal degeneration. As neurologists differentiate between the unique presentations of each of these disorders, the diagnostic categories have been more clearly defined. The target population for the purpose of this project is people with PSP and their caregivers.

In their seminal work, Moore and Guttman (2011) found that knowledge challenges are the priority need identified by individuals with PSP and their families. They define knowledge challenges as a "lack of knowledge among physicians, community workers, patients, and families" (Moore & Guttman, 2011 [poster]). They identify three additional areas of need for support: "symptoms including falls, mobility, mood, thinking, speech, and swallowing; service access and interactions with physicians, community workers, and long term care staff; and a perceived lack of research and failure to communicate research findings" (Moore & Guttman, 2011 [poster]). Moore and Guttman also found that the following has helped families the most: knowledge, symptom support, therapy services, and awareness of research studies (Moore & Guttman, 2011 [poster]).

The Cure PSP Care Guide pilot project is a targeted community-focused program using telehealth, concentrating on individuals from Minnesota and the surrounding states. The specific aims of the project are threefold: to telephonically assist individuals with PSP and their families by providing guidance, knowledge, and resources; to identify a targeted group of individuals sharing this diagnosis and the professionals providing services to this PSP community; and to create a foundation from which the individuals living with PSP and the professionals offering resources might build community.

Review of Literature

There is a plethora of nursing literature about such topics as patient education, self-management, chronic disease management, telehealth, and nurse navigation. These publications provide ample evidence of successful nursing interventions. Nurses identified a need within a specific population and developed interventions to address the need. Coster and Norman (2009) conducted a Cochrane review of patient education and self-management interventions by analyzing 30 publications. They conclude that patient education and self-management programs have positive benefits to patient knowledge and day-to-day management and may decrease dependence on services (Coster & Norman, 2009, p. 526).

Patient education and self-management are terms that are used interchangeably in the literature. Patient education refers to the provision of disease-specific information and technical skills, whereas self-management instructs in problem-solving skills (Bodenheimer, Wagner, & Grumbach, 2002). Patient education and self-management are part of chronic disease management. In a literature review of 160 publications, Forbes and While (2009) conclude that nurses have made significant contributions to chronic disease management interventions at the patient and system levels. They attribute the success to the nursing process of assessing needs, creating interventions, and implementing interventions. Furthermore, they stress that patient-oriented models of care are far more efficient than disease-oriented approaches.

Harold Freeman (2013) is credited with the development of the oncology nurse navigator model of care, which includes guidance by nurses from the point of diagnosis through to the end of life. Kazimierczak et al. suggest that "nurses need therefore to consider adopting flexible roles as supporters who facilitate patients' use and interpretation of health information, and remain highly sensitive to the specifics of individual patients' needs and preferences at any given time" (Kazimierczak et al., 2013, p. 9). Becze (2011) found that barriers to care are decreased with nurse navigators.

Nurses have used telehealth to provide telephone nursing practice to vulnerable persons and those with chronic disease. Telehealth has been defined as (a) the use of telecommunication technologies to facilitate health and wellness and (b) the provision of services with the use of telecommunication technologies to facilitate health and wellness (American Academy of Family Physicians, 2007). Telephone nursing practice refers to the delivery of nursing service over the telephone (American Academy of Ambulatory Care Nursing, 2007). The growing literature of the past 2 decades provides ample evidence of the efficacy of telephone nursing practice.

Greenberg (2009) developed the seminal theoretical model of telephone nursing practice. This model is composed of three phases: gathering information, cognitive processing, and output. Winters and Winters (2007) state, "perhaps the most important application of telehealth technology in nursing practice is management of chronic health problems" (p. 55). Pare, Jaana, and Sicotte (2007) conducted a systematic review of the outcomes resulting from home telemonitoring of four chronic diseases and conclude that this intervention "potentially improves medical conditions" (p. 269). Fincher, Ward, Dawkins, Magee, and Wilson (2009) developed and implemented a telehealth medication counseling intervention for veterans living with Parkinson disease. This intervention resulted in "improved patient problem identification, individualized problem solving, and facilitated action planning" (p. 23). Telephone nursing practice contributes to improved patient outcomes by providing access to nursing expertise.


The Cure PSP Care Guide intervention was reviewed and approved by the Towson University Institutional Review Board for the Protection of Human Subjects. Participants made the initial contact with the investigator primarily via email. After initial contact, a packet of material was mailed to the potential participants. The packet included a cover letter, basic information from Cure PSP, the Cure PSP Assessment Questionnaire (Table 1), preevaluation and postevaluation, the consent, Five Wishes (Aging With Dignity, 1997), and "Getting Help for Progressive Supranuclear Palsy: A Guide for Patients and Families" (Moore, 2013). During the course of two telephone calls, individuals and their caregivers were assessed to develop a Cure PSP Care Guide designed to provide guidance along the trajectory. The Cure PSP Assessment Questionnaire (Table 1) was utilized during the initial telephone call, after obtaining verbal informed consent.

Data Collection

Quantitative and qualitative information was collected before and after the intervention. Qualitative data included verbatim transcription of a final open-ended question. Quantitative data included the use of two validated instruments: the chronic disease self-efficacy scale (Lorig, Sobel, Ritter, Laurent, & Hobbs, 2001) and Caregiver Strain Index (CSI; Robinson, 1983). The self-efficacy scale was administered to those living with PSP. The CSI is a validated caregiver assessment tool in which respondents answer yes or no to 13 items, which contribute to stress. A positive answer is indicative of a need for nursing intervention. A score of 7 or higher signifies a high level of stress. A project-specific tool (Table 2, available as Supplemental Digital Content 1 at JNN/A58) was designed to assess knowledge, self-management skills, resource identification, and life planning in the participants.


The characteristics of the participants (Table 2) include those living with PSP (n = 11; seven men, four women) and caregivers (n = 11; four men, seven women). These dyads represent individuals who have been living with this diagnosis from 1 to 5 years. In all cases, the families shared a familiar story of having been diagnosed with Parkinson initially, and only after unsuccessful pharmacological management of the alleged Parkinson did the PSP diagnosis surface. Many went to multiple physicians before receiving the diagnosis of PSP at an academic center. Participants resided at home or in assisted living facilities. Most of the individuals with PSP were not able to participate.


The 11 dyads who participated in the Cure PSP Care project were recruited over a period of 5 months. The pre and post caregiver knowledge assessment scores' (Table 3) paired samples t test shows the intervention effect of improved knowledge (95% CI, -14.201 to -1.799). Because of the small sample of participants with PSP, it is difficult to draw conclusions from the pre and post self-efficacy and knowledge scores. The CSI scores (Table 3) represent a high level of strain with the average score being 10.18 before and after the intervention, on the 13-item scale. The pre- and post-CSI scores did not show any significant quantifiable change as represented by the paired samples t test (95% CI, -1.083 to 1.083).

Qualitative results from the caregivers as summarized in the verbatim transcripts below indicate the ability of the intervention to enhance patient and caregiver knowledge, improve day-to-day management by developing an awareness of resources, decrease dependence on services, and address caregiver needs. Five themes emerged from the qualitative inquiry:

Enhance patient and caregiver knowledge
   I am learning things that I wish I would have
   known when we got the diagnosis.

   It was informative since it made us feel that we
   could relate to the literature and deal with what
   we have in front of us. I had not thought about
   the cognitive and mental issues.

Improve day-to-day management
   The literature helped immensely. It helped our neurologist help us,
   he saw onabotulinumtoxin A (Botox) mentioned in the book and said
   yes it would be a good idea to try.

   Mom was able to get signed up with speech therapy, occupational
   therapy, and physical therapy, when we saw the neurologist. This
   happened because of your encouragement.

Develop an awareness of resources
   You provided the idea of palliative care which
   really helped.

Decrease dependence on resources

We're having difficulty getting anyone involved including the neurologist. No one suggested occupational therapy (OT) to us. No one has suggestions. The OT assessment was not helpful since she didn't know about PSP. We've had two trips to the emergency room and I don't want more. One trip occurred after he went down the stairs head first. Thanks to you, we'll revisit the OT with this knowledge.

Address caregiver needs
   Before not knowing felt overwhelming, now
   knowing is comforting.

   This was definitely helpful. We have this terrible
   thing in front of us. We have had so many
   blessings in our lives. This is one of them.


The quantitative and qualitative caregiver data collected justify further exploration of the use of telehealth to remotely deliver case management services to vulnerable individuals and families living with PSP. The caregivers gained knowledge and support during this telehealth intervention, as evidenced by the difference between pre and post knowledge assessment scores and the qualitative statements. The remote delivery of services improves access to expertise and is an effective way to provide guidance, information, and resources to people who have a rare disorder and their families. Unfortunately, there are limited pharmacological options to effectively treat PSP, and medical management is focused on prevention and alleviating comorbidities, such as depression.

Nursing interventions provided during the implementation of this intervention were individualized to address the needs of each unique person with PSP and their family members. The interventions included nurse-delivered education in the following areas: PSP disease process; hospice and palliative care; the prevention of falls, decubiti, aspiration, and contractures; the development of support systems and community; the recognition of depression; the importance of home safety given the impulsivity present in some individuals with PSP; and education about research being done in PSP. Additional interventions included the identification of assistive devices that promote self-management such as home environment modifications, a Hoyer lift, adaptive utensils, walkers, and table top cycles for exercise. Many respondents were not familiar with the Five Wishes, a document that serves as an advanced directive decision-making guide. The provision of this document facilitated recognition of the necessity of end-of-life planning. Numerous participants had not been referred to appropriate allied team members such as speech, occupational, and physical therapy. Nursing encouragement of self-advocacy for these valuable services resulted in referrals and improved function. Caregiver support is vital in the setting of an illness such as PSP. Caregivers were referred to local caregiver groups, area agency on aging services, and pastoral counseling.

A key goal of the project included community building. Most individuals value knowing and interacting with others in similar life circumstances. Because of the project, a resource directory was developed for the region, and project participants were referred to support groups and encouraged to connect with groups serving individuals with similar illnesses. Several participants were introduced in the hopes that they will develop a support network.

There were some limitations to this pilot project, which include the small sample, the condensed implementation time line, lack of participation of the individuals living with PSP, and the necessity of providing information before the initial pretest. The CSI intervention effect may have been adversely affected by the abbreviated timeline between the intervention and the assessment of the effect. The knowledge intervention effect pretest scores were influenced by the fact that individuals had an opportunity to review the Cure PSP literature before the pretest.


The Cure PSP Care Guide intervention delivered via telehealth deserves further research. The experience gleaned from this pilot project can be used to develop future projects for patients but, more importantly, their caregiving family members. The responses of the caregivers indicate that telehealth is an effective way to provide services and support to those caring for loved ones with rare neurodegenerative illnesses. The true benefit of this intervention may be concluded from the qualitative results and not the inconclusive CSI data. These disorders have limited resources for diagnosis and management on account of a paucity of physician specialists. Specialists focus on the needs of the patients during clinical visits, which results in little to no time to address the needs of caregivers. Consequently, those living with these disorders are vulnerable, which contributes to care disparities. Specialty-trained neuroscience registered nurses are capable of addressing the needs of these patients and families. Remote delivery of nursing services with telehealth is one method of bridging the resource gap. The Cure PSP Care Guide telephonic nursing intervention improved caregiver knowledge and support, promoted self-care management, and assisted families to effectively navigate the healthcare system. It also promoted access to a healthcare professional with clinical expertise in PSP, ultimately improving the care of those with the disease.


Aging With Dignity. (1997). The five wishes. Retrieved from

American Academy of Ambulatory Care Nursing. (2007). Telehealth nursing practice administration and practice standards (4th ed.). Pitman, NJ: Author. Retrieved from online/en/home/aboutus/specialty/rural/telemed/telehealth.html

American Academy of Family Physicians. (2007). Telehealth discussion paper. Retrieved from home/aboutus/specialty/rural/telemed/telehealth.html

Becze, E. (2011). Patient and nursing aspects of oncology navigation. ONS Connect, 26(10), 14-15. doi: 10.1188/11.ONSC. 14-15

Bodenheimer, T., Wagner, E. H., & Grumbach, K. (2002). Improving primary care for patients with chronic illness: The chronic care model, part 2. Journal of the American Medical Association, 288(15), 1909-1914. doi: 10.1001/jama,288.14.1775

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Questions or comments about this article may be directed to Susan Rebecca Dunlop, RN MS, at She is the Associate Director, Johns Hopkins Parkinson's Disease and Movement Disorders Center, Baltimore, MD.

Vicky P. Kent, RN PhD, is an Associate Professor of Nursing, Towson University, Towson, MD.

Mary Lashley, RN PhD, is a Professor of Nursing, Towson University, Towson, MD.

Trish Caruana, MSW, is an Executive Vice President of Education and Support, Cure PSP, Timonium, MD.

This pilot project was completed as part of graduate study requirements by Susan Rebecca Dunlop. Ms. Dunlop gratefully acknowledges the guidance provided by Vicky P. Kent, Mary Lashley, and Trish Caruana. Trish Caruana currently serves as the Vice President of Programs and Education at Cure PSP. Ms. Dunlop appreciates and acknowledges the following colleagues who generously shared their knowledge and assisted with recruitment efforts: Teresa Moore, Lisette Bunting-Perry, Chantel Stokes, Shirali Nigam, Rosemary Wichmann, Joan Hlas, Christel Patnaude, and Dina Drubach.

The other authors have no disclosures or conflicts of interest.

Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal's Web site (

DOI: 10.1097/JNN.0000000000000194
TABLE 1. Initial Assessment Questionnaire

Cure PSP Assessment Questionnaire

1. Who made the diagnosis of PSP? How long have you (or your loved
one) been diagnosed? How often do you (or your loved one) see that
specialist? Has the specialist discussed a management plan?

2. What are your information needs? What questions do you have?

3. Have you (or your loved one) seen any allied health team members
such as a physical therapist, occupational therapist, speech
therapist, dietician, or nurse?

4. Do you (or your loved one) have difficulty swallowing, writing,
speaking, cutting food, turning in bed, walking, bathing, and/or

5. Do you (or your loved one) have difficulties driving, working,
managing your household, or participating in your hobbies and
leisure activities?

6. How is your (or your loved one's) mood?

7. How is your (or your loved one's) thinking?

8. Have you (or your loved one) made any life plans such as a
living will or advanced directive or plans for care if you are not
able to care for yourself?

TABLE 2. Characteristics of the Study

Months since diagnosis
  6-12                                     3
  13-48                                    7
  49-60                                    1
Source of diagnosis
  Diagnosis by academic center             9
  Diagnosis by other                       2
Living situation
  Living at home                           8
  Living in assisted living facility       3
  Living in skilled care facility          0
  Attending medical adult day services     1
Gender of participants
  Participant with PSP, male               7
  Participant with PSP, female             4
  Caregiver, male                          4
  Caregiver, female                        7
Employment of caregivers
  Employed                                 3
  Retired                                  8

TABLE 3. Caregiver Intervention Effect Scores

                              Caregiver Strain Index

               Preintervention   Postintervention
Subject No.          CSI               CSI          Change

001                  11                 12            1
003                   6                 4             -2
004                  11                 9             -2
007                   6                 9             3
009                  11                 11            0
010                  11                 12            1
011                  11                 11            0
012                  11                 12            1
013                  12                 13            1
015                  12                 10            -2
016                  10                 9             -1
Mean                10.18             10.18           0

                              Knowledge Assessment

               Preintervention   Postintervention
                  Knowledge         Knowledge
Subject No.      Assessment         Assessment      Change

001                  83                100            17
003                  100               100            0
004                  100               100            0
007                  83                 83            0
009                  83                100            17
010                  80                 80            0
011                  80                100            20
012                  100               100            0
013                  83                100            17
015                  83                100            17
016                  83                100            17
Mean                88.64             96.64          8.00

Note. A positive response on the 13-item Caregiver Strain
Index indicates a need for intervention in that area. A
score of 7 or higher indicates a high level of stress
(Robinson, 1983).
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Author:Dunlop, Susan Rebecca; Kent, Vicky P.; Lashley, Mary; Caruana, Trish
Publication:Journal of Neuroscience Nursing
Article Type:Report
Date:Apr 1, 2016
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