Chapter 2 Consultation
Common criticisms of some psychological research, and indeed research in general, is that it is about the people rather than for them (Seymour & Davies, 2002). This study was initiated by the Cardiac Inherited Diseases Group (CIDG) in order to help them to better understand the adjustment processes of their patient group, evaluate the practices within their service from a pragmatic perspective and to provide feedback and results in such a way that positive change may be achieved, benefiting clinicians, individuals and families with LQTS. The following chapter outlines the process through which the research project developed, the consultation that occurred leading to the development of the specific research methodology and design, the relevant ethics involved, and finally, the rationale and aims of the research project are described.
The following section describes the group that requested the research and the youth and adult medical services they work through. Next, the process a LQTS patient typically goes through is illustrated and explained from both youth and adult perspectives.
The Cardiac Inherited Diseases Group (CIDG)
CIDG is a nationwide multidisciplinary group whose main aim is to “prevent sudden cardiac death due to inherited heart conditions in the young” (Cardiac Inherited Diseases Group, 2010). There are representatives from numerous professions including specialist Cardiologists, Electrophysiologists, Molecular and Clinical Geneticists, Genetic Counsellors, Pathologists and associated professional staff, from eight different regions around New Zealand. In Auckland, members of CIDG specialising in Pediatrics are based in the Department of Pediatric and Congenital Cardiac Services (Cardiac Services) at Starship Children’s Health and adult services are at the Greenlane Clinical Centre and in Auckland City Hospital. The Chairperson for CIDG and the Coordinator of the LQTS Registry, which was in the development phase when this research project began, are both based in the Department of Pediatric and Congenital Cardiac Services (Cardiac Services) at Starship Children’s Health, Auckland.
CIDG are interested in inherited cardiac conditions, including Long QT Syndrome, Brugada Syndrome, Dilated Cardiomyopathy, Hypertrophic Cardiomyopathy and Arrythmogenic Right Ventricular Cardiomyopathy. As a group, CIDG states its aims are to facilitate screening of at-risk individuals and their families, to provide education about inherited cardiac conditions to the public, patients and medical professionals alike, and to develop best practice guidelines to enhance professional practice in the field (Cardiac Inherited Diseases Group, 2010). CIDG aim for this screening and educating body to one day become a government funded national service and intend to make their case by demonstrating its utility in saving lives through clinical best practice and the clinical application of molecular genetics, better known as genetic testing (Cardiac Inherited Diseases Group, 2010).
Typical Process of CIDG Service Delivery
The Pediatric Cardiology department, in which the Pediatric Congenital Cardiac Service operates, is a tertiary referral centre for NZ, therefore they receive all complex case referrals and all cardiac inherited diseases go through CIDG (see Figure 7 below for example of typical referral process). The CIDG Chairperson is also the only Pediatric Electrophysiologist in NZ so he sees all the complex pediatric electrical arrhythmia cases including the congenital or inherited cardiac conditions such as LQTS. These inherited cardiac cases are allocated to the Pediatric Congenital Cardiac Service and the CIDG Chairperson is also the head of this service.
The process from referral to adult services is different to the pediatric cases only in terms of the initial presentation and referral. This is primarily because potential cardiac patients are typically seen as inpatients after having had a cardiac arrest. It is highly atypical for a patient to be referred as an outpatient. The process from referral onwards is the same as with Pediatrics (see Figure 8) except they are seen at Auckland City Hospital in the Cardiac Care Unit (CCU) (rather than at Starship Children’s Health).
Origin of the research with CIDG
The CIDG Chairperson and Consultant Pediatric Cardiologist from Cardiac Services first approached a faculty member of the Population Health Department at the University of Auckland to conduct research on the psychosocial impact of sudden death in families in 2004. A research proposal was developed and put forward to the department but was not pursued after encountering difficulties organising the research project’s practicalities with CIDG. Difficulties included the lack of a system for organising information relevant to the LQTS patients seen by the service and difficulties liaising with key administrative staff.
In 2005 CIDG referred a group of LQTS patients to the Consult Liaison Team (CLT), the mental health service available to patients at Starship Children’s hospital. CIDG clinicians reported some concern they may not be adequately identifying LQTS patients that could benefit by having mental health support and the two services together discussed the possibility of doing a piece of research aimed at identifying LQTS patients’ psychological needs and whether CIDG staff were currently meeting them. At the time, two clinicians from CLT held joint positions with the University of Auckland within the Psychology and Medical Health Sciences departments. At the end of 2005 a Doctorate of Clinical Psychology Candidate (the principle investigator and writer) was approached by one of these faculty members and asked to lead the project.
Given the difficulties initially experienced careful consultation with CIDG was advised and undertaken and the cardiac clinic process and Cardiac Services structure was reviewed with an evaluative and pragmatic eye. This was in an effort to ensure the same difficulties would not be encountered again. At the time, a database was being developed that would hold all the data pertaining to the LQTS affected and at-risk patients. This database was projected by CIDG to be up and running before participant recruitment was expected to begin the following year. A good relationship was also established with key administrative staff and avenues of communication were specifically defined. This being in place, a research proposal was consequently developed and accepted by all relevant parties; including CIDG, the Department of Psychology at the University of Auckland, the Ministry of Health National Ethics Committee and Auckland District Health Board.
Consultation Process and Outcomes
In the following section the consultation process with CIDG is described, including information about who was consulted and the outcomes of this. Defining researcher roles is reviewed next and, finally, the ethical and cultural consultation process is described.
CIDG clinicians involved in the genetic testing for LQTS process were consulted at regular intervals throughout the research project and especially in the development phase. The author first attended meetings with the CIDG team as a whole in order to become familiar with the system, the clinicians and the clinical process involved for those experiencing LQTS and potentially undergoing genetic testing. The author then conducted a series of individual semi-structured interviews from key clinicians identified by CIDG. The purpose of these interviews was to ascertain common areas of research interest and establish the research aims. The following clinicians were identified and consequently interviewed for approximately 30-90 minutes:
- Consultant Pediatric Cardiologist & Electrophysiologist, Department of Pediatric & Congenital Cardiac Services, Starship Children’s Health & Auckland City Hospital
- Cardiac Inherited Disease Registry Co-ordinator & Senior Technologist, Department of Clinical Physiology, Starship Children’s Health & Auckland City Hospital who arranged and supported the cardiac clinics and follow ups
- Clinical Registrar and Fellow, Department of Pediatric & Congenital Cardiac Services, Starship Children’s Health & Auckland City Hospital whom assisted the Consultant Pediatric Cardiologist
- Child & Adolescent Psychiatrist, Consult Liaison Team, Starship Children’s Health & Senior Lecturer, Department of Psychological Medicine, University of Auckland who saw families referred by Cardiac Services with concerns about their mental health
- Geneticist & Associate Professor, Head of the Medical Genetics Group, School of Medical Sciences, University of Auckland and head of laboratory initially conducting LQTS genetic tests
The CIDG clinicians reported a primary interest in learning more about how LQTS patients psychologically adjust to, or cope with, having LQTS after they leave the hospital clinic following diagnosis. Specifically, they were interested in the impact of receiving genetic test results and how this may differ for those who experience symptoms, and therefore usually already have an initial LQTS diagnosis, and those who are Asymptomatic and may only have a tentative initial LQTS diagnosis, but have a family member who has been diagnosed for example.
CIDG clinicians were also seeking clarification around whether LQTS patients’ experience is that it’s better to know about their LQTS genetic standing or to remain unaware, as some patients appeared to prefer. Also, what was the impact on family relationships when family members received a positive or negative genetic test result? Specifically, they wondered whether guilt and blame affected parental, spousal and sibling dyads when looked at in the context of positive versus negative LQTS genetic results. For example, did parents with a positive genetic test result feel guilty for passing it on? Also, did siblings with a negative genetic test result feel guilty for not having the condition when another sibling received a positive test result and did anyone blame a genetically positive parent or spouse? Also of interest was identifying which aspects of resiliency and coping differentiated those that ultimately adjusted, coped and found benefits during the process.
The CIDG clinicians also wanted to know how well the service they currently provided matched the emotional needs of their LQTS patients, specifically in terms of the way the results were given, by whom and how, plus whether they were providing enough and the right supports to the children, adolescents and adults that made up the LQTS affected families.
The CIDG clinicians thought obtaining feedback and consequently learning about these factors could allow them to more adequately prepare and counsel LQTS patients about what they might expect during the genetic testing for LQTS process, make suggestions about what can help based on what they know has helped others in the past, and identify those families that may benefit from additional support.
Australasian Collaboration Initiated but Suspended
The primary researcher was alerted by CIDG to an Australian based project investigating the psychosocial impact of genetic testing for LQTS. Both NZ and Australian parties could see the potential benefits of having a larger data pool thereby adding statistical power to correlations, and enabling comparisons between the samples. A quantitative part of the present study was consequently designed to identify the psychological and demographic factors that distinguished those that struggled to psychologically adjust to their genetic test results from those who did not. This paralleled the study being conducted in Australia and, as consistency was required for direct comparisons, it was decided both would be longitudinal, over the course of the genetic testing process, and questionnaire based. Unfortunately, it was not possible to proceed with this collaboration or the quantitative portion of the research project, however, as data collection problems beyond the control of the primary researcher were encountered by the NZ study. These precluded continuing with the longitudinal project.
The primary researcher in the NZ study was a representative from the University of Auckland and as such was a separate party otherwise uninvolved with CIDG and the cardiac clinic process. While this was seen as an advantage in terms of providing an objective point of view for the research it was potentially also a disadvantage. Access to potential participants could be more difficult, as might accessing participant information in a timely manner. In an attempt to address these potential problems two members of the cardiac team agreed to be co-researchers with one specifically appointed as the Liaison for the study. The role of the Liaison was defined and primarily consisted of facilitating access to information about the clinical process of genetic testing for participants, assisting in accessing potential participants and advising the primary researcher immediately after participants received their genetic test results so further questionnaires could be sent out. The other co-researcher was the Chairperson of CIDG and a Consultant Pediatric Cardiologist and Electrophysiologist on the cardiac team at Starship Children’s Hospital. His primary role was to help the project maintain a high profile among the Cardiologists at both Starship Children Hospital and Auckland City Hospital so that they would give potential participants research invitations.
At the time of initially designing the study the laboratory conducting the genetic testing for LQTS in New Zealand was a University of Auckland research facility, but plans were in place for the testing to shortly be taken over by a hospital based laboratory.
Ethics and Cultural Safety
The study received ethics approval from the Health and Disability Ethics Committees, Northern X Regional Ethics Committee, Ministry of Health (NTX/06/12/158) and ADHB Human Participants’ Research Committee (3653). To ensure cultural appropriateness a review was provided by a Maori staff member at the University of Auckland, Department of Psychology familiar with conducting research with Maori participants, and the ADHB Maori ethics committee (3653). The Maori Clinical Advisor, Werry Centre, Child & Adolescent Workforce Development also agreed to being a co-investigator with his role being to support the cultural safety of Maori participants. That is, he could attend and facilitate introductions and co-facilitate the explanation of the research project and the potential use, storage and collection of the information when necessary. If preferred by the participant he could co-facilitate the interviews or collect the quantitative data verbally.
As identified in the literature and CIDG consultation reported above, there was a strong rationale for investigating the psychosocial experiences of those affected by, and undergoing the genetic testing process for, LQTS. The literature published to date illustrates the medical LQTS body of knowledge is developing quickly and it is therefore important the psychosocial factors, especially those relating to patient care, are also investigated and disseminated with equal pace. It is expected this information will be immediately useful to CIDG clinicians and the services in which they work. Hopefully it will also be useful for other services like it and contribute to the international body of knowledge from which the aims also arose. These aims were developed in consultation with CIDG and the research co-investigators.
The Aims of the Study
To describe the medical and psychosocial factors impacting adjustment to LQTS in New Zealand patients and families
To compare the experiences of (i) clinicians and patients, (ii) asymptomatic and symptomatic patients, (iii) diagnosed and at-risk patients and (iv) of adolescent and adult patients.
CHAPTER 1: LITERATURE REVIEW The Epidemiology and Etiology of Long QT Syndrome Prevalence
Distribution of LQTS
Role of Sympathetic Nervous System in LQTS
The QT Interval
Genes, Inheritance and the Etiology of Long QT Syndrome
Combined Electrophysiological and Genetic Etiology of LQTS
Medical Presentation and Care of Long QT Syndrome
Quality of Life
Effect on the Family
Social Support Groups
Psychosocial Factors Impacting Long QT Syndrome
Impact of LQTS on Psychological Distress
Impact of Genetic Testing on Clinicians
Psychosocial Impact of Genetic Testing for LQTS
Contributing Factors Leading up to Gene Based Diagnosis
Impact of the Initial Acute Phase after Gene Based Diagnosis
CHAPTER 2: CONSULTATION
The Cardiac Inherited Diseases Group (CIDG)
Typical Process of CIDG Service Delivery
Origin of the Research with CIDG
Consultation Process and Outcomes
Ethics and Cultural Safety
Aims of the Study
CHAPTER 3: METHODOLOGY 58 Qualitative Design of Present Study
Patient Interview Outline
Clinician Interview Outline
Thematic Plan of Analysis
66 Data Familiarisation
Generating Initial Codes
CHAPTER 4: RESULTS 72 The Themes
Clinician Themesimilarities & Differences
Summary of Similarities and Differences in Adjusting to LQTS
CHAPTER 5: DISCUSSION 122 The Aims of the Research
Discussion of Major Findings in Relation to Previous Findings
Psychosocial Impact of LQTS
Impact of Genetic Testing
Recommendations to CIDG
Final Conclusion and Reflections
GET THE COMPLETE PROJECT
“Am I going to die today?”