20. MARST 2010
STUDY PROPOSAL
TITEL:
PATIENT-REPORTED OUTCOMES AND THE LIFE SITUATION OF ADULT SURVIVORS WITH SINGLE VENTRICLE PHYSIOLOGY
Coordination: Project leader Dorthe Overgaard, Ph.D., RH/UCSF.
Investigators: Researcher, Ph.D., RN, Dorthe Overgaard - MPH, senior lecturer, RN, Anne- Marie Schrader, University College Metropol - RN Catriona King RH - RN, MSc Karen H.
Lisby RH, and Ass. Professor. RN, Philip Moons, Centre for Health Services and Nursing Research, Katholieke Universiteit Leuven, Belgien.
Address for correspondence:
Dorthe Overgaard Ph.D., The Heart Centre, RH, Blegdamsvej 9. Opgang 3. - 14. sal.
2100 Copenhagen Ø, Denmark. Tlf: 35451489. Mail: dorthe.overgaard@rh.regionh.dk
Proposal
Research question:
What is the psychosocial profile and life situation of patients with single ventricle physiology - and how can these be improved?
The study is divided into three phases: study I, study II and study III.
Study I: A quantitative part with a survey Study II: A qualitative part with interviews
Study III: An evaluation of a Nurse-coordinated Intervention
Events
Study I: Descriptiveanalysis Study III: Nurse- coordinated Intervention Health Promotion
The study is divided into three phases
Study II: Qualitativeinterpretation Psychosocial counselling
Analysis of interviews
Palliative surgeries Survey-questionnaire Analysis of data In depth interviewsInter Exercise training
Proposal
The clinical setting for adult survivors with single ventricle physiology
Physical challenges
Children born with single ventricle physiology typically face three complex surgical procedures before the age of four. A small group of patients may only have the first two surgeries, while an even smaller group is not surgically corrected in any way. Therefore, adult survivors will not necessarily have had identical operations or treatment strategies.
The three surgical stages described here are the standard procedures recommended for patients with single ventricle physiology.
First stage is the Norwood procedure, which is often now carried out in the neonatal period.
Here, the aorta and pulmonary arteries are reconstructed and a Blalock-Taussig-shunt is placed in the right subclavian artery and connected to the right pulmonary artery (RPA). The mortality rate of the Norwood procedure is about 20 %, and the child is often in a serious condition afterwards (1). Very long hospitalizations of 1-2 months are common, and after discharge the child is a frequent visitor to the outpatient clinic.
The second stage of palliative surgery is called the Glenn procedure, and is carried out when the child is approximately six months old. Here, the Blalock-Taussig-shunt is removed and the superior vena cava is connected to the RPA. Surgical mortality is around 10 % (2).
After this procedure, the general condition of the child improves significantly. The child is often discharged after a few weeks, and can enjoy a relatively stabile period of normal physical development and increasing bodyweight. This progress is monitored closely in the outpatient clinic.
The third surgery is the Fontan procedure or the Total Cavo-Pulmonary Connex (TCPC). This surgery is undertaken when the child is around two years of age. Here, the inferior vena cava is connected to the RPA via an intracardial or extracardial tunnel. Thereby, all venous blood returns directly to the pulmonary arteries, bypassing the heart, and a normal arterial saturation is achieved. The survival rate after the final surgery is high at around 90 % (2), but long hospitalizations due to pericardial and pleural effusions are common (1).
After TCPC, most patients have a long period without serious health problems and live a close to normal life. After the age of fifteen, all patients are seen in the outpatient clinic (Grown-Up Congenital Heart) at least once a year.
In late adolescence or early adulthood, complications usually arise. Typical problems are tachyarrhythmia, pulmonary embolism, renal insufficiency and failure of the single ventricle.
A small group of patients (10 %) are afflicted with protein loosing enteropathy (PLE) – a serious and life-threatening condition (3). Some patients are on several medications to control symptoms or to prevent thromboembolism, and some patients with end-stage heart failure are referred for a heart- and lung transplant if possible.
Psychosocial challenges
Very little research has been done regarding self-reported QOL, health status or neurological and psychosocial outcomes after TCPC or Fontan surgery (4).
Many patients c al outcomes after TCPC or Fontan surgery (4).
Many patients struggle with chronic fatigue and are physically limited (5). Others are physically limited because of heart failure and cannot participate in rigorous exercise and their physical abilities are reduced. The average patient can only reach about 60-65 % of normal exercise capacity in comparison to healthy controls (6). Depression and anxiety is shown to be more common in patients with severe congenital heart defects, including single ventricle physiology (7).
Many patients have problems achieving independence from their parents. Socially, these patients can also be severely limited in terms of educational choices and career planning, family life, and the formation of meaningful romantic relationships. These problems arise before the transition period where their autonomy should be developed and is reflected at that time (8)(9). Socially, patients with single ventricle physiology can also be severely affected.
Educational and career choices may be limited due to the problems mentioned above,
specifically careers that are physically demanding or require travelling or working long hours.
Patients that are not capable of working full-time may be forced into part-time employment or early retirement (2), which can cause financial problems and increased dependency on
parents and other family members. Finally, the heart failure may have a great influence on family life. Limited physical functioning, late maturity, dependency issues, poor social functioning, inability to tolerate pregnancy and uncertainty regarding long-term survival can cause family conflicts and impede the formation of meaningful romantic relationships (10)(11).
Study I
Introduction for study I
Single ventricle physiology refers to a group of cardiac defects that share the common feature that only one of the two ventricles is of adequate functional size. Anomalies that are described as single ventricle defects include tricuspid atresia, mitral atresia, double inlet left ventricle, atrioventricular septal defect with a dominant left or right ventricle, hypoplastic left heart syndrome, univentricular heart either or not in combination with transposition of the great arteries, and some variations of double outlet right ventricle. Single ventricle physiology is reported to occur in 1% to 2% of all congenital heart defects (1). Tricuspid atresia is the most common form. Most patients with a single ventricle physiology die in infancy or early childhood unless palliative surgery is performed. Biventricular repair is usually not possible.
Today, all surgical approaches are either staged or palliative in nature, each providing an atriopulmonary, atrioventricular or cavopulmonary connection. The overall aim of these procedures is to direct systemic venous blood flow directly into the pulmonary artery via the right atrium or via an intracardiac or extracardiac conduit.
Life expectancy after palliation for single ventricle physiology is considerably lower than that of other congenital heart defects. It is reported that the 10 year survival rates following Fontan-type repair is around 60%, although it is now increasing (12)(13)(14).
Survival is however dependent on the type of underlying defect, with a substantially better survival rate for patients with tricuspid atresia compared to patients with more complex congenital malformations (15).
However, the long-term concerns associated with these surgical procedures persist.
These include the physiological effects of persistent elevated right atrium pressure and elevated systemic venous pressure. Additional long term complications associated with the Fontan and its modifications include a chronic low cardiac output state, development of atrial arrhythmias, thromboembolism, Fontan pathway obstruction, and protein-losing enteropathy.
Despite the often chronic problems associated with Fontan procedures, adult survivors lead a relatively normal live, are able to work, and women are able to have successful pregnancies (16)(17). However, pregnancy in these patients is associated with substantial complications and morbidity (18). Altogether, these problems may significantly hamper patients‟ health status, functional outcome and quality of life, and the patients may feel limited in their daily lives.
The term „Patient - Reported Outcome‟ (PRO) (19) is a concept used to evaluate and communicate nursing. PRO is important to understand the impact of treatment on patient functioning and well-being. PRO is a broader term than health related quality of life with the patients perspective. Studies on patient-reported outcomes (PRO) in individuals with single
ventricle physiology are very scarce.
To our knowledge, only one study specifically investigated quality of life as part of a long-term outcomes study in adult patients after the Fontan operation (5).
This study assessed the quality of life in only 22 patients using SF-36. Information on perceived health status, functioning and quality of life in the long term is critical for a better understanding of relevant issues or concerns of the afflicted patients, in order to optimise clinical follow-up. Results showed that the mean follow-up period was 15 years and that the level of physical functioning, mental health and general health was significantly lower than in the normal population (5).
AIM/SUBAIMS for
Study I
Aim
To describe „Patient - Reported Outcome‟ among young survivors with single ventricle physiology
Subaims
- to describe the perceived health status, functional abilities, quality of life, anxiety and depression, sense of coherence, autonomy and health behaviour in young survivors with single ventricle physiology
- to explore determinants of quality of life in young survivors with single ventricle physiology - to explore determinants of autonomy in young survivors with single ventricle physiology - to compare patient-reported outcomes with healthy control subjects
Material and Methods
forStudy I
Study population
This study is a supplementary to a nationwide research project on long-term outcome in patients with single ventricle physiology in Denmark. Ph.D.-student, MD Lars Idorn from the Heart Centre has gathered a historical cohort study with retrospective data comprising all people born in Denmark with single ventricle physiology from the period 1977-2009 and is doing clinical assessment according to the underlying disease. This cohort will be further divided into four groups according to year of birth. Among these we have chosen the patients born from 1977-1994 for our study. Patients participating will be from 16 years of age and older.
In our study of patient-reported outcomes, patients can be included if they are currently still alive; if they are at least 16 years of age at 0104/2010; and if they are litterate and Danish speaking. They also need to have a diagnosis - code of functional single ventricle physiology, and they must attend the outpatient clinic either at The Heart Centre, Copenhagen University Hospital or at The Heart Centre, Aarhus University Hospital.
Approximately 90 patients will be eligible for inclusion. The Danish Statistical Society will create a control group matched 1:2 on age, gender and geography.
Study VARIABLES AND MEASUREMENT
Study I is a quantitatively oriented, descriptive survey where several validated scales and questionnaires will be used. The following instruments will be used to measure the variables of interest. The variables chosen represent important patient-reported outcomes.
Variables Measurement Self-perceived health
status
Linear Analogue Scale (1 item) (20)
Medical Outcome Study – Short-form 36 (11 items) (21) Functional abilities Ability Index (1 item) (22)
Physical activity Godin Leisure-Time Questionnaire (4 items) (23)(24) Quality of life Linear Analogue Scale (1 item) (25)
Satisfaction with Life Scale (5 items) (25)(26)(27)
Anxiety and depression Hospital Anxiety and Depression Scale (14 items) (28)(29)(30) Sense of coherence Sense of Coherence Scale (13 items) (31)(32)(33)
Autonomy Psychological Separation Inventory (26 items) (34)(35)(36) Health behavior: Health Behavior Scale-CHD (11 items) (37)
Except for the Health Behavior Scale-CHD, all instruments exist in Danish and have good validity and reliability. In addition to the established questionnaires, demographic variables (including age, gender, educational level and employment status) are collected, using a specific self-report form that will be developed for this study.
The following scales, appearing in random order, will be sent out to the patients. They will be asked about a number of background variables, including age, gender, educational level and employment status. In the following text all scales will be referred.
Perceived health status
Scale 1: Linear Analogue Scale (LAS)
Subjectively perceived health status will be measured using a Linear Analogue Scale (LAS). This is a vertical, graded, 10 centimetre line, ranging from the „worst imaginable health state‟ (=0) to the „best imaginable health state‟ (=100). This LAS is part of the EuroQol instrument, and is demonstrated to have good validity and reliability when used in adults with congenital heart disease (20).
Scale 2: Medical Outcome Study – Short-form 36 (SF-36)
Another instrument that will be used to measure perceived health status is the Medical Outcome Study – Short-form 36 (SF-36). The SF-36 is a widely used generic instrument to measure patients‟ subjective health status along eight dimensions (21). The SF-36 generates a score for each dimension, ranging from 0 to 100. Higher scores indicate a better health status.
The SF-36 has been used extensively and in different patient populations, allowing
comparison of SF-36 scores between single ventricle patients, healthy individuals and other patient populations with chronic diseases.
Functional abilities Scale 3: Ability Index
The Ability Index is created by Somerville (22), and measures the physical capability of patients with congenital heart disease. It is a figure with one question created as a colour code equal to the colours of the rainbow. The patients state how it is, at that moment, to live with a congenital heart disease. Red = ability index 1 (normal life), yellow = ability index 2 (able to work), green = ability index 3 (unable to work) and purple = ability index 4 (extreme
limitation).
Scale 4: The Godin Leisure – Time Questionnaire about physical activity
The Godin Leisure - Time Questionnaire reports how many times during the last week one exercises more than 15 minutes during free time. Is it a self- administered questionnaire which consists of 4 questions (23), it is commonly used in patients with heart failure and it is extensively validated (24). It is also easy to administer and provides detailed information on different activity levels as well as a single measure of the total amount of physical activity during the last week (23,23).
Quality of life (QOL)
Based on a thorough conceptualisation (25), quality of life is defined as “a sense of overall life satisfaction that is positively or negatively influenced by individuals‟ perception of life aspects, including health and non-health-related features, which are important to them” (26).
Consequently, quality of life can be measured in terms of life satisfaction.
Scale 5: The Linear Analogue Scale (LAS)
Overall quality of life will be measured using one question on a Linear Analogue Scale (LAS), ranging from the „worst imaginable quality of life‟ (=0) to the „best imaginable quality of life‟ (=100). The use of the LAS allows patients to give their own rating of their perceived quality of life. Experiences with LAS to measure quality of life in adults with congenital heart disease indicate that this measurement is valid and reliable (25).
Scale 6: The Satisfaction with Life Scale (SWLS)
Because quality of life is conceptualised in terms of life satisfaction, the Satisfaction with Life Scale will be used as a second indicator for quality of life (27,27). This instrument comprises five statements, and seven response categories, ranging from “strongly disagree” to
“strongly agree”. An aggregate score can be obtained by summing the individual item scores.
The minimum score of life satisfaction is 5 and the maximum score is 35. Validity and reliability have been assessed extensively, indicating good psychometric properties (25).
Anxiety and depressive symptoms
Scale 7: The HAD scale - Hospital Anxiety and Depression Scale (HAD)
Anxiety and depressive symptoms will be measured using the HAD scale. The Hospital Anxiety and Depression Scale is used to assess self- reported somatic problems (28)(29) and is one of the most frequently used measures of depressive symptoms in survey research. The HAD scale is used to assess the contribution of mood disorder, especially anxiety and depression, in order to understand the experience of suffering in the setting of medical
practice. The HAD scale comprises 14 questions about anxiety and depression during the last week. Of the seven depression items some reflect aspects of reduction in pleasure response such as: „I look forward with enjoyment to things” or “I feel as if I am slowed down”. The four categories for answering are: nearly all the time, very often, sometimes, not at all. Each item has to be answered by the patient on a four point (0 – 3) response category, so the possible scores range from 0 to 21 for anxiety and 0 to 21 for depression. The HAD scale has
been shown to have good psychometric and diagnostic properties when used in young people and also has potential with adolescents (30).
Sense of coherence
Scale 8: Sense of Coherence Scale (SOC)
Sense of Coherence Scale (SOC) will be assessed using the 13-item Orientation to Life Questionnaire, which is an abbreviated version of Antonovsky‟s original instrument,
comprising 29 items. SOC-13 is a self-report questionnaire with three components:
meaningfulness; comprehensibility; and manageability. All 13 items are scored on a 7-point semantic differential scale, in which the response options range from1 to 7. The total score is calculated by adding the scores of each item, yielding a possible score of 13–91. Higher scores indicate a stronger SOC (31). SOC-13 has proven to be valid and reliable (32)(33).
Autonomy
We want to measure and assess in which way the young patient with single ventricle physiology achieve autonomy and identity by managing self care abilities in life. This
“Transition into Health Care” is conceptually defined as a process, not an event, and it occurs over time. Here, the separation process from the parents is the big challenge (34).
Scale 9: The Psychological Separation Inventory” (PSI)
Efforts to act independently may be reflected during adolescence as the ability to manage and direct one's practical and personal affairs without the help of the mother and father”. This definition is labelled ‟functional independence‟ and can be measured using the following scale: The Psychological Separation Inventory (PSI). The Psychological Separation Inventory (35) is a 138 - item instrument that assesses an individual's perceptions of
psychological separation from the parents. Items are rated on a 5-point Likert scale ranging from 1 (almost never or never true) to 5 (almost always or always true). The instrument contains four scales: Functional Independence, Emotional Independence, Conflictual Independence, and Attitudinal Independence. An example of a statement: “My mother's/father's wishes have influenced my selection of friends”.
In our questionnaire, we will only use 26 questions. These questions will concern functional independence in the relationship with both parents respectively (36).
Health behavior
In this study, the concept of health behaviour is based on an existing Health Survey Form that is developed and used in the Netherlands to survey the general population.
Scale 10: A Health Behavior Scale-CHD
A Health Behavior Scale-CHD is developed at the University of Leuven, Belgium.
This questionnaire includes items that are specifically relevant to alcohol use; smoking;
marijuana and illicit drug use; and dental health behaviors. This instrument only exists in Dutch/English, and will therefore be translated into Danish (37).
Procedure
The questionnaire will be sending out to the patients home address. After 3 weeks they will receive a reminder by post together with a new questionnaire. According to creating the control group matched 1:2 on age, gender and geography the same questionnaire will be send out to 450 young persons and after 1 week they will receive a reminder.
Statistical analysis for study I
For descriptive statistics, nominal variables will be expressed in absolute numbers and percentages. Means and standard deviations will be used for continuous data, if they are normally distributed. For non-normally distributed variables, medians and quartiles will be computed.
Group differences between patients and healthy controls will be investigated by
calculating standardized differences between patients and healthy control subjects. For each patient, the score of the corresponding control subject will be subtracted from the patient‟s score and this will be divided by the standard deviation of the control group, generating a standardized difference for that patient. Averaging this difference over all patients result in a mean standardized difference. A one sample t-test will be used to evaluate whether the mean standardized differences significantly differed from zero.
Multivariable linear regression analysis will be performed to investigate potential determinants of quality of life and autonomy, after compliance to the assumptions have been tested. If needed, data will be transformed. Two-tailed tests will be used, and the level of significance is set at p 0.05. SPSS-package v.18. will be used for statistical analysis.
Study II
Introduction for Study II
Since 1971, the survival of patients with single ventricle physiology has significantly
improved. Paradoxically, and despite the vastly improved survival rate, very few studies have been undertaken to explore the life and experiences of these patients. The studies that do exist are primarily conducted from a biomedical perspective, and only a small handful investigate the psychosocial aspects of living with a single ventricle.
The general field of congenital heart disease has been more widely explored. Small numbers of patients with single ventricle are contained in several studies of CHD, but are not
emphasized as a special group. Therefore, our current perception of patients with single ventricle is based on studies of a much broader and more diverse patient population.
In this study, we specifically wish to explore the life situation of young people and adults with single ventricle. To our knowledge, this will be the first study to use a varied approach in examining quality of life in these patients, which we wish to illuminate from many different perspectives.
Study II is the qualitative part of our study. The intention is to do an in-depth interview with 15 patients to describe what it is like to grow up and live with a single ventricle. As mentioned earlier, only one other study had assessed quality of life and it was done in only 22 patients (5).
Some studies indicate deficits in physical and psychological functioning in patients with single ventricle physiology (4)(5), whereas other studies showed no difference compared to the general population (16). Saliba found that a higher educational level predicted a better score for physical and mental health measures (16). Even a few studies found that the quality of life in adults with congenital heart disease was significantly better than in the general population (20).
The young person with a single ventricle has been taken care of during his entire
childhood by his parents, especially by the mother. For obvious reasons, it has been necessary for her to have a high degree of control over her child‟s life for many years. When growing into adolescence the perceived needs of the person with a single ventricle may begin to change. This journey or transition from an adolescent to an adult living with a chronic disease is a process where life skills, abilities and choices related to education and career are
considered (35).
Claesens et al. (11) examined what it means to patient to live with a congenital heart Disease. They interviewed 12 patients about their lived experiences as adult patients and found that „Feeling different‟ was the central theme and that the process of normalisation was
the most important issue. The reasons for feeling different vary according to age and coping strategies. Due to overprotection, parents made decisions for their children even when they became older, resulting in a lower degree of autonomy.
Claesens et al. claimed that only four studies investigated lived experiences of patients with CHD using qualitative research methods. These four studies were authored by L. Gantt, E.M.
Tong and R. McMurray, respectively. Gantt investigated in 1992 (38) and again in 2002 (39) this group of patients and the relationship between mother and daughter. She found that the core concept was “growing up heartsick”, which included feeling different from others, parental overprotectiveness and fear of death (38).
In her recent research, about the experience of growing up heartsick and the mother - daughter relationship, Gantt also found (39) that the overriding theme was „normalization‟
and that two subthemes were named: “It is not a big deal” and “Sometimes, it is a very big deal”. She described patients‟ struggles due to the ongoing health problems, and the
normalization as a strategy for living with CHD. Some mothers were overprotective and the heart disease had affected the mother - daughter relationship, but she found that the mother - daughter dyade was not problematic.
Tong interviewed adolescents and young adults with CHD and revealed the dilemmas of normality and management of illness (40). She claimed that although the clinicans
recognize that these survivors have specific and unique health care needs, there are still many unknowns about their experiences (41).
Tong described that: Transition is a multifaceted process which needs to be understood from four perspectives: the patient – the parents – the pediatric cardiologist and the adult cardiologist. Transition is divided into three stages: early (11-14 years), middle (15-18 years) and late (19-21 years). In our study, we will investigate patients in middle and late
adolescence. „Middle adolescence‟ is characterized by a rapid growth in cognitive skills, whereas „late adolescence‟ is the time when self develops and sexual identity continues to unfold. Tong found that both parents and adolescents often use similar coping strategies to gain control over the disease (41). Murray also found (10) that the psychosocial needs of adolescents is imperfectly understood. Five themes emerged in his research: difficulty coping, physical limitation, social exclusion, discrimination and hopes for life improvement.
What is the best way to assume responsibility for oneself, including one‟s own health?
Patterson (34) examined this question and conducted three focus group interviews. Results suggest that a mentor might be helpful. She puts emphasis on the different barriers. One
solution is to begin the transition-proces early at the age of 14 or 15 years of age (34), which was the same solution that Claessens and Moons came to (11)(42).
AIM/SUBAIMS
for Study II
Aim
To investigate the life situation of young survivors with single ventricle physiology Subaims
- to illuminate the lived experiences for young survivors - to illuminate how it feels to live with a single ventricle
- to illuminate how the transition period from adolescent to young survivor is experienced
Material and Methods for Study II
The selection of patients and our considerations regarding the chosen qualitative methods for study II are described.
Participants
Patients in the middle and late stage of transition will be chosen. From the database created by Idorn, patients born from 1977 - 1994 are included in our study; i.e. the patients will be from 16 years of age and older. These patients are considered “Key Informants”(43) with unique experiences (44).
The inclusion criteria
Patients that are currently still alive; if they are at least 16 years of age at 0104/2010; if they are literate and Danish speaking. Additionally, we are looking for a varied selection of patients according to age, gender, underlying disease in order to reveal diverse experiences.
Methods
Our study combines quantitative with qualitative research. This is called "Sequential Triangulation", where the results from the quantitative survey in study I are the basis of planning for the methods in study II (45)(46)(47). Triangulation is chosen in order to answer the research question from a broad perspective and to obtain different but complementary data on the same topic (45). This research will reveal the experience of adolescence and growing up with a single ventricle (48)(49). In-depth interviews with 15 patients were chosen as a research method because of the fact that meaning can be constructed through a creation of verbal viewpoints (50)(51)(52). A wide interpretation of the interviewees' statements is
sought, and Kvale's interpretation levels will be used. The number of interviewed is expected to provide enough data (52).
Procedure
The patients will be contacted by letter asking for participation. We draw up a semi- structured interview guide for focus areas. The interview conducted by two people,
distribution of tasks during the interview is the one will be the primary interviewer and the other secondary.
The interviews will take place in patients' homes or they will be offered to proceed at the hospital. The interviews will be tape recorded and transcribed soon after the verbatim and converted to text.
Data analysis
A wide interpretation of the interviewees' statements is sought, and Kvale's three levels of interpretation will be used. Kvale was chosen because his three questions types and three levels of interpretation are operational, just as his many questions regarding interview transcripts leave room for a wide interpretation. The first type of Kvale's questions is distributed on three levels of interpretation: The level of self-understanding, the level of common sense and the theoretical level, where within the scope of each level various questions are asked to the text.
The first level is the participant's self-understanding. Here the researcher tries to give a concentrated opinion of the particular person's own opinion of the statements. The point is to understand the participant's own statements and then afterwards to summarize their meaning.
At Kvale's second level common sense knowledge is involved as a universally agreed knowledge of the contents of the statement. Common sense goes beyond the interviewee's opinion and comprises a wider frame of understanding. At the third level a theoretical interpretation is applied, or the theoretical frame which goes beyond both the self- understanding and common sense level is involved (52).
The interviews will be tape recorded and transcribed verbatim. Three of the researchers will interpret the interviews together in order to reveal core themes. The interviews will be administered by a semi-structured guide for interviewing constructed for the purpose (52).
In this semi-structured interviewguide, we ask about the life of the young survivor with single ventricle physiology. The questions will be:
How do you assess your quality of life?
Which restrictions do you experience?
How do you manage to cope with your daily life?
How do you perceive your level of physical exertion?
How is your work - situation?
How is your lifestyle?
Are you satisfied with your life and why?
Do you feel anxious or depressed?
How do you think about the transition to adulthood?
What do you see as the biggest challenge for you in the transition period?
How would you define your relationship with the closest persons in your life?
How will your life be in the future?
Study III - An evaluation of the Nurse - coordinated Intervention Introduction for Study III
In the outpatient clinic at The Heart Centre, Copenhagen University Hospital, there is a specific nursing programme directed at young patients from 15 - 18 years of age with
congenital heart disease. This programme has been developed in 2004 to help young patients transition into adulthood. The aim of the programme is to teach the patients about their heart condition and counsel them on a variety of issues related to growing up and living a normal life with a chronic heart condition: i.e. awareness of cardiac symptoms, sports and physical activity, pregnancy and prevention of infective endocarditis and choice of education and work. This programme is used with all young patients, and the content is not disease-specific.
It is based on the recommendations (22)(42) about teenagers and young adults with CHD, and their need for support in the transitional process towards adulthood.
It is our aim to create, from the results of study I and II, an evidence-based, targeted, multi- level nursing intervention for patients with single ventricle physiology in the outpatient clinic.
AIM
for Study III
Aim
- To test the evaluation of a multilevel nurse – coordinated intervention
We will design a multilevel nurse - coordinated intervention and describe a frame for how it might be possible to implement the nurse – coordinated intervention for single ventricle patients.
The Nurse – coordinated intervention might consist of the following:
a) Nursing program - b) Health Promotion Programme - c) REHAB including a program for improving physical activity individually.
The nurse coordinated programme will take place in the outpatient clinic at The Heart Centre, Copenhagen University Hospital. Part of the intervention will be in group sessions, and several different professions will participate in training in order to teach the young patient how to cope with being chronically ill. These professions will include nurses, physicians, physiotherapists, dieticians and social workers. The role of the nurses will primarily be in the outpatient clinic doing individual counselling. This will most likely be necessary, as these patients are a non-homogeneous group.
Living with a congenital, chronic disease necessitates aquiring health promotional skills at an individual pace. A systematic intervention may lead to revelation of the individuals risk behaviour and improve the appropriate health behaviour (53). According to the concept of
“health literacy”, which is defined as a capacity to adapt and transfer knowledge regarding health, the young person needs to engage actively in order to take responsibility for their own lives (54).
When designing a three-months REHAB-programme for exercise training, the
physiotherapist will start with a group at Copenhagen University Hospital and use a pre-post test for exercising. The GUCH (Grown-Up Congenital Heart) consultant physician will determine at which level it might be feasible for each patient to exercise. The aim is to develop a suitable form of exercise precisely for this patient group. After having had the first team the exercise will be recorded on DVD and distributed to patients across the country.
Some patients will be offered the intervention programme, whereas others might not have the possibility. As mentioned above, the intervention will be based at Rigshospitalet, Copenhagen University Hospital and will be tailored to fit individual patients. By using a pre - post test design with a non equivalent comparison group we are confident that we will be able to reveal the effect of the intervention.
Towards the end of the study, the patients going through the intervention will be asked to evaluate it. This will be accomplished by the patients re-answering all the questions from the survey used in study I.
Ethical considerations
The Danish Data Protection Agency has approved the study. The questionnaires will be anonymised and the data kept according to recommendations from The Data Protection Supervisor. The Journal number is: (j.nr. 2007-58-0015).
References
(1) Wright C. Cardiac surgery 2002: staged repair of hypoplastic left heart syndrome.
Crit.Care Nurs.Q. 2002 Nov;25(3):72-78.
(2) Keld Sørensen. Medfødt Hjertefejl - om hjertesygdomme hos børn. ; 2006.
(3) Ghanayem NS, Berger S, Tweddell JS. Medical management of the failing Fontan.
Pediatr.Cardiol. 2007 Nov-Dec;28(6):465-471.
(4) Pike AN, Evangelista LS, Doering LV, Koniak-Griffin D, Lewis AB, Child JS. Health- related quality of life: A closer look at related research in patients who have undergone the Fontan operation over the last decade. Heart & lung : the journal of critical care 2006;36(1):3- 15.
(5) van den Bosch AE, Roos-Hesselink JW, Van Domburg R, Bogers AJ, Simoons ML, Meijboom FJ. Long-term outcome and quality of life in adult patients after the Fontan operation. Am.J.Cardiol. 2004 May 1;93(9):1141-1145.
(6) Paridon SM, Mitchell PD, Colan SD, Williams RV, Blaufox A, Li JS, et al. A cross- sectional study of exercise performance during the first 2 decades of life after the Fontan operation. J.Am.Coll.Cardiol. 2008 Jul 8;52(2):99-107.
(7) Bromberg JI, Beasley PJ, D'Angelo EJ, Landzberg M, DeMaso DR. Depression and anxiety in adults with congenital heart disease: a pilot study. Heart Lung 2003 Mar- Apr;32(2):105-110.
(8) Garris TM. Investigation of Self-reported Transitional health Care needs of the Adolescents with Congenital heart Disease. june 2006.
(9) Carson AR, Hieber KV. Adult pediatric patients. Am.J.Nurs. 2001 Mar;101(3):46-54;
quiz 54-5.
(10) McMurray r, Kendall L, Parsons JM, Quirk J, Veldtman GR, Lewin RJP, et al. A life less ordinary: growing up and coping with congenital heart disease. Cor Health Care 2001;5(1):51-57.
(11) Claessens P, Moons P, de Casterle BD, Cannaerts N, Budts W, Gewillig M. What does it mean to live with a congenital heart disease? A qualitative study on the lived experiences of adult patients. Eur.J.Cardiovasc.Nurs. 2005 Mar;4(1):3-10.
(12) Fontan F, Fernandez G, Costa F, Naftel DC, Tritto F, Blackstone EH, et al. The size of the pulmonary arteries and the results of the Fontan operation. J.Thorac.Cardiovasc.Surg.
1989 Nov;98(5 Pt 1):711-9; discussion 719-24.
(13) Driscoll DJ, Offord KP, Feldt RH, Schaff HV, Puga FJ, Danielson GK. Five- to fifteen- year follow-up after Fontan operation. Circulation 1992 Feb;85(2):469-496.
(14) Lee JR, Choi JS, Kang CH, Bae EJ, Kim YJ, Rho JR. Surgical results of patients with a functional single ventricle. Eur.J.Cardiothorac.Surg. 2003 Nov;24(5):716-722.
(15) Weipert J, Noebauer C, Schreiber C, Kostolny M, Zrenner B, Wacker A, et al.
Occurrence and management of atrial arrhythmia after long-term Fontan circulation.
J.Thorac.Cardiovasc.Surg. 2004 Feb;127(2):457-464.
(16) Saliba Z, Butera G, Bonnet D, Bonhoeffer P, Villain E, Kachaner J, et al. Quality of life and perceived health status in surviving adults with univentricular heart. Heart 2001
Jul;86(1):69-73.
(17) Canobbio MM, Mair DD, van der Velde M, Koos BJ. Pregnancy outcomes after the Fontan repair. J.Am.Coll.Cardiol. 1996 Sep;28(3):763-767.
(18) Drenthen W, Pieper PG, Roos-Hesselink JW, van Lottum WA, Voors AA, Mulder BJ, et al. Pregnancy and delivery in women after Fontan palliation. Heart 2006 Sep;92(9):1290- 1294.
(19) Acquadro C, Berzon R, Dubois D, Leidy NK, Marquis P, Revicki D, et al. Incorporating the patient's perspective into drug development and communication: an ad hoc task force report of the Patient-Reported Outcomes (PRO) Harmonization Group meeting at the Food and Drug Administration, February 16, 2001. Value Health. 2003 Sep-Oct;6(5):522-531.
(20) Moons P, Van Deyk K, De Bleser L, Marquet K, Raes E, De Geest S, et al. Quality of life and health status in adults with congenital heart disease: a direct comparison with healthy counterparts. Eur.J.Cardiovasc.Prev.Rehabil. 2006 Jun;13(3):407-413.
(21) McHorney CA, Ware JE,Jr, Raczek AE. The MOS 36-Item Short-Form Health Survey (SF-36): II. Psychometric and clinical tests of validity in measuring physical and mental health constructs. Med.Care 1993 Mar;31(3):247-263.
(22) Somerville J. Congenital heart disease in adults and adolescents. Br.Heart J. 1986 Nov;56(5):395-397.
(23) Godin G, Shephard RJ. A simple method to assess exercise behavior in the community.
Can.J.Appl.Sport Sci. 1985 Sep;10(3):141-146.
(24) Jacobs DR,Jr, Ainsworth BE, Hartman TJ, Leon AS. A simultaneous evaluation of 10 commonly used physical activity questionnaires. Med.Sci.Sports Exerc. 1993 Jan;25(1):81- 91.
(25) Moons P, Budts W, De Geest S. Critique on the conceptualisation of quality of life: a review and evaluation of different conceptual approaches. Int.J.Nurs.Stud. 2006
Sep;43(7):891-901.
(26) Moons P, Marquet K, Budts W, De Geest S. Validity, reliability and responsiveness of the "Schedule for the Evaluation of Individual Quality of Life-Direct Weighting" (SEIQoL- DW) in congenital heart disease. Health.Qual.Life.Outcomes 2004 May 28;2:27.
(27) Diener E, Emmons RA, Larsen RJ, Griffin S. The Satisfaction With Life Scale.
J.Pers.Assess. 1985 Feb;49(1):71-75.
(28) Zigmond AS, Snaith RP. The hospital anxiety and depression scale. Acta Psychiatr.Scand. 1983 Jun;67(6):361-370.
(29) Bjelland I, Dahl AA, Haug TT, Neckelmann D. The validity of the Hospital Anxiety and Depression Scale. An updated literature review. J.Psychosom.Res. 2002 Feb;52(2):69-77.
(30) Puhan MA, Frey M, Buchi S, Schunemann HJ. The minimal important difference of the hospital anxiety and depression scale in patients with chronic obstructive pulmonary disease.
Health.Qual.Life.Outcomes 2008 Jul 2;6:46.
(31) Antonovsky A. Unraveling the mystery of health: how people manage stress and stay well. San Francisco, Calif.: Jossey-Bass; 1987.
(32) Eriksson M, Lindstrom B. Validity of Antonovsky's sense of coherence scale: a systematic review. J.Epidemiol.Community Health 2005 Jun;59(6):460-466.
(33) Feldt T, Lintula H, Suominen S, Koskenvuo M, Vahtera J, Kivimaki M. Structural validity and temporal stability of the 13-item sense of coherence scale: prospective evidence from the population-based HeSSup study. Qual.Life Res. 2007 Apr;16(3):483-493.
(34) Paterson D, Lanier C. Adolescents Health Transition: Focus Group Study of teens and Young Adults with special health care needs. Family Community Health 1999;22(2):42-58.
(35) Hoffman J. Psychological separation of late adolescents from their parents. J Couns Psychol ;31(2):170-178.
(36) Lopez FG, Campbell VL, Watkins CE. Family structure, psychological separtion, and college adjustment: A canonical analysis and cross-validation. J Couns Psychol
1988;35(4):402-409.
(37) K. Vanheusden. Mental health problems and barriers to service use in Dutch young adults. Rotterdam: Erasmus University Rotterdam; 2008.
(38) Gantt LT. Growing up heartsick: the experiences of young women with congenital heart disease. Health Care Women Int. 1992 Jul-Sep;13(3):241-248.
(39) Gantt L. As normal a life as possible: mothers and their daughters with congenital heart disease. Health Care Women Int. 2002 Jul-Aug;23(5):481-491.
(40) Tong EM, Sparacino PS, Messias DK, Foote D, Chesla CA, Gilliss CL. Growing up with congenital heart disease: the dilemmas of adolescents and young adults [see comment.
Cardiol.Young 1998 Jul;8(3):303-309.
(41) Tong EM, Kools S. Health care transitions for adolescents with congenital heart disease:
patient and family perspectives. Nurs.Clin.North Am. 2004 Dec;39(4):727-740.
(42) Moons P, Pinxten S, Dedroog D, Van Deyk K, Gewillig M, Hilderson D, et al.
Expectations and experiences of adolescents with congenital heart disease on being transferred from pediatric cardiology to an adult congenital heart disease program.
J.Adolesc.Health 2009 Apr;44(4):316-322.
(43) Gilchrist VJ, Williams RL. Key Informant Interviews. In: Crabtree BF, Miller W, editors. Doing Qualitative Research. 2. edn ed. Thousand Oaks: Sage Publications; 1999. p.
71-88.
(44) Malterud K. Kvalitativa metoder i medicinsk forskning: en introduktion. Lund:
Studentlitteratur; 2009.
(45) Morse JM. Approaches to qualitative-quantitative methodological triangulation.
Nurs.Res. 1991 Mar-Apr;40(2):120-123.
(46) Creswell JW, Clark P, Gutmann M, Hanson W. Advanced mixed methods research designs. In: Tashakakkori A, Teddlie C, editors. Handbook of mixed methods in social an behavioral research Thousand Oaks: Sage Publications; 2003. p. 58-88.
(47) Hunger BP, Polit DF. Nursing Research: Principles and Methods. ; 1995.
(48) Lorensen M. Spørsmålet bestemmer metoden: forskningsmetoder i sykepleie og andre helsefag. Oslo: Universitetsforlaget; 1998.
(49) Lorensen M, Hounsgaard L, Østergaard-Nielsen G, Brødsgaard A. Forskning i klinisk sygepleje. [Bind] 2 : Metoder og vidensudvikling. [Kbh.]: Akademisk; 2003.
(50) Miller W, Crabtree BF. Depth Interviewing. In: Crabtree BF, Miller WL, editors. Doing Qualitative research. 2. edn ed. Thousands Oaks: Sage Publications; 1999. p. 89-108.
(51) Kruuse E. Kvalitative forskningsmetoder i psykologi og beslægtede fag. 5. udgave ed.
Virum: Dansk psykologisk Forlag; 2003.
(52) Kvale S, Brinkmann S. Interview: introduktion til et håndværk. 2. udgave ed. Kbh.: Hans Reitzel; 2009.
(53) Tønnesen H. Terminologi: forebyggelse, sundhedsfremme og folkesundhed. Version 1 ed. Kbh.: Sundhedsstyrelsen; 2005.
(54) Sundhedsstyrelsen. Sundhedsdokumentation. Health Literacy - begrebet, konsekvenser og mulige interventioner. ; 2009.
