Cystic Fibrosis in Adulthood: Balancing Care and Quality of Life

Psychosocial adjustment to illness and disability has been one of the main challenges in rehabilitation practice and research in recent decades (Livneh & Parker, 2005). Cystic fibrosis (CF), a chronic and potentially fatal genetic condition, requires complex, intensive, and time-consuming daily care that includes chest physiotherapy, inhaled and nebulized medications, pancreatic enzymes, pancreatic enzymes, nutritional monitoring, and clinic visits. Advances in diagnosis and treatment have led to a substantial increase in survival rates and a growing percentage of patients reaching adulthood. In the wake of the increased longevity of CF patients, greater attention has been devoted to evaluating and improving long-term rehabilitation, social adjustment, and life satisfaction. These clinical goals are generally addressed by rehabilitation counsellors, an essential part of the rehabilitation of the multidisciplinary team whose goals are to help each patient adapt to the medical and psychosocial impact of the condition as well as to achieve personal, professional, and social goals. With the transition of CF from a terminal illness to a chronic condition, the goal became to support adult CF in their two simultaneous and often conflicting missions: maintaining lifelong adherence to a complex treatment protocol and, in light of their serious and demanding illness, achieving long-term personal, developmental, and social goals similar to those of their health peers. These goals include independence, employment, and the establishment and maintenance of social relationships.
To provide quality and optimal care to this population, it is imperative to fully understand the various factors that contribute to or hinder adaptation to this disease. The main objective of the current research was to examine the unique factors that contribute to psychosocial adjustment in young adults with CF. Additionally, this study aimed to examine factors contributing to adherence and the association between adherence and psychosocial adjustment. Applying this knowledge in practice will help develop appropriate interventions aimed at helping these individuals achieve this delicate and seemingly contradictory balance. For the purposes of this study, we adopted the conceptual model of Moos and Holahan (2007), who state that the intense confrontation with a serious physical illness, prolonged treatment, uncertainty and intense personal distress has a profound and lasting impact. Most individuals cope reasonably well with such a crisis and are able to recover and reach a new balance, but others are demoralized and suffer from lasting psychological problems. (p. 107)

The authors offer a multi-dimensional conceptual framework that views chronic illness and disability as a crisis of prolonged life and help identify the main determinants of individuals' psychosocial adjustment to chronic illness and disability. One of the main goals of Moos and Holahan's model is to examine factors that contribute to the psychosocial adjustment of people with physical disabilities or chronic illnesses. Their model was adapted for this study, as we sought to examine the psychosocial adjustment of young adults with CF through social adjustment and life satisfaction. The Moos and Holahan (2007) model suggests five main categories associated with adaptation:

1. Personal resources, which are evaluated in the current study as a sense of coherence (SDC) and personal characteristics (i.e. gender, age, education, etc.).
2. Health factors, represented here as medical conditions.
3. The social and physical context, examined in the present study by means of the perceived relationship with the professional team.
4. Subjective evaluation, examined in the present study through the patient's perception of the disease.
5. Coping tasks, which were assessed in the current study by the level of adherence to
treatment.

It is important to note that, for CF patients, adherence is an important goal in itself, as it is necessary for longevity (e.g., Sawicki et al., 2009). Therefore, in this study, we also examined personal and contextual factors that contribute to adherence in adults with CF. Similarities and differences between the contributors to adaptation and membership were also assessed.

Social adjustment and life satisfaction

The literature indicates conflicting conclusions regarding the effects of illness on social adjustment. The majority of studies indicate that the adjustment and functioning of CF patients are similar to those of the healthy population. These studies indicate that, despite the challenges they face, most adults with CF have significant interpersonal relationships and developmental tasks related to work, intimate relationships, and independence.

However, other studies indicate that the presence of the disease itself can interfere with normative development and that adults with CF tend to develop later than their healthy peers, which is manifested by a continued dependence on their parents and a difficulty in achieving independence in employment.

According to these studies, it is often difficult for these individuals to develop and maintain social and intimate relationships based on trust and safety, especially with people who are not familiar with the implications or requirements of the condition. For some, social relationships are perceived as too threatening, so they choose to distance themselves from others. Contrary to common expectations that chronic conditions would have a negative impact on life satisfaction, research shows that adults with CF report similar (or even higher) levels of life satisfaction than their healthy peers. However, recent studies show that increasing disease severity, namely acuity translating into decreased lung capacity and overall functioning, leads to increased treatment demands and interference with normal routine, and therefore has more of an impact on life satisfaction than the presence of the disease itself.

Severity of the disease and subjective perception of the disease

While the objective severity of CF is medically assessed based on FEV1, number of hospitalizations, oxygen level, and BMI, the subjective severity of the condition is determined based on individual self-reports. Patients often rate the severity of their condition as being less than the severity reflected in their medical records. This may be the result of denial, anxiety, or the need for control. It is important to note that research indicates that assessing a patient's illness (severity and changes) is a better predictor of patient function and adjustment than medical indicators are. Thus, subjective perception is crucial for the adaptation of patients to the disease. Adult CF patients evolve according to the stage and severity of their condition and experience different clinical conditions for which diverse and unique medical and psychosocial interventions are required. Gender differences in mortality rates, adjustment, and quality of life for people with CF have been found; studies show that women report greater disease severity and lower adjustment and die at a younger age than men with CF. These studies show that men adapt better to the disease. Perceived illness severity and psychosocial adjustment vary from person to person due to personal characteristics that are also examined in current research as SDC.

Feeling of coherence

CDS is a personal characteristic that is the basis for an individual's adaptation to daily life and stressful events such as illness. Formed during childhood, it stabilizes in adulthood and remains unchanged regardless of circumstances. According to Antonovsky (1983, 1987, 1993), the SDC contains three fundamental elements: comprehensibility — the perception that internal and external environments are structured, predictable, consistent, and clear; manageability — the feeling that life can be managed and that resources are available to enable the individual to deal with the problems they encounter; and meaning — the perception that the demands of life are meaningful and worth investing in. energy, which allows the individual to seek meaning even in difficult situations. Although there is not enough data on the link between CDS and CF, studies looking at other chronic conditions like asthma or diabetes show positive correlations between CDS and the ability to cope with the condition. The results regarding the contribution of CDS to adherence are inconclusive, while some studies have found that higher CDS contributes to adherence for people with diabetes (e.g., Cohen, 2004), others report that adherence was only linked to adherence among those who perceived themselves to be healthy (e.g., SandeneRiksson, 2000); others found no association between the two. (e.g., Kamwendo, Hansson, and Hjerpa, 1998).

Relationship with the medical team

The multidisciplinary medical team is another central component that plays a crucial role in the lives of people with CF. This relationship between a patient and their medical team is often unique, ongoing, and characterized by intense intimate and emotional commitment. The better the relationship with the professional team, the better the understanding of the disease and therefore the better adherence to the treatment. It has been found that a good relationship with the medical team improves the effectiveness of coping resources for patients with chronic illnesses and is linked to better coping with the disease. Although the association between CDS and the ability to receive professional support has not been examined, Kobasa and Puccetti (1983) suggested that a person with high personal resources, such as self-control, makes better use of supportive relationships, which may lead to better
adaptation to the disease. CF requires continued adherence to a treatment protocol that is often a significant long-term challenge.

Adherence to treatments

Research has shown that adherence to treatments in patients with CF is associated with improved lung condition and increased longevity. In addition, non-compliance with treatments can cause significant problems, especially in adolescence, when responsibility for treatments and adherence is transferred from parents to the patient.

Recent research indicates that numerous factors contribute to adherence, including demographic factors (age, gender), interaction between the patient, family, and professional team, anxiety, and patients' desire to take control of their lives and coping styles. Although the association between adherence and the objective severity of the condition has not been examined, the perception of the condition (subjective severity) contributes to adherence; the more patients perceive their illness, the more they comply with treatments. Studies looking at treatment adherence in people with other conditions such as acute myocardial infarction have found that adherence was associated with low levels of depression and improved physical conditions.

Discussion

Thanks to a better understanding of cystic fibrosis and medical advances in the management of the disease, the population of adults with cystic fibrosis is increasing. However, this population faces a difficult reality when the disease conflicts with their desire to lead a full and active life. This often results in intra- and interpersonal dissonances, as it is practically and emotionally difficult to lead a full life while meeting treatment requirements. Our study results indicate that CF adults reported high levels of life satisfaction and social adjustment. These findings support previous studies on the quality of life of CF adults that indicate that, despite challenges, most patients achieve their developmental tasks with respect to work, intimate relationships, and independence. The results also showed high levels of adherence to treatments, but it is interesting to note that there is no association between adherence and social adjustment or life satisfaction. Thus, the more patients perceive their illness, the more they adhere to treatments, but the less satisfied they are with their lives. In addition, in the study, those with more severe medical conditions adhered better to treatments but showed poorer social adjustment. Sawicki and colleagues (2009) found a similar relationship between perceived burden associated with adherence to treatments and increasing treatment demands, indicating that the greater the adherence to treatment, the greater the perceived burden on patients' lives.

Our results indicate that the two major challenges of adherence and psychosocial adjustment reflect different entities with relationship models that are dissimilar from the other variables examined. While internal resources contributed positively to both measures of psychosocial adjustment,
the subjective severity of the disease and the medical problems contributed positively to adherence on the one hand, and negatively to life satisfaction on the other hand. The results reveal that among patients with less serious medical problems, those who perceived their illness to be less serious were less faithful to treatment. This could indicate that those who are in a less threatening position have fewer treatment obligations and therefore lack the internal and external reminders that reinforce the importance of adherence.

Another factor that contributes to compliance is the patient's relationship with the medical team. The results show that, among people who have multiple medical conditions, the closer this relationship was perceived to be, the more the patient was on treatment. CF clinics in Israel are part of the public health services and are located in a number of hospitals in the country. In each health center specializing in CF, there are about 100 patients, a relatively small number. Ce
high counsellor-patient ratio makes it possible to establish a close personal relationship between patients and therapeutic staff and to make services more accessible and flexible.

CF is considered a chronic condition according to the basket of health services in Israel and as such, most treatments are covered by the Ministry of Health so patients are not required to pay for services. Team work
Medical treatment in the case of CF is characterized by unique elements including a high level of availability and a continued practical and emotional commitment over many years. Patients are treated by a team of professionals from birth and special attention is paid to the personal relationship between the patient and the multidisciplinary team. Personal and professional relationships contribute to patients' sense of safety, which is especially important when living with a condition marked by doubt and uncertainty. It is therefore important to note that adherence to treatment is essentially personal and is not linked to funding difficulties, team availability, or access to treatments. Therefore, this relationship becomes an important and even critical factor in the patient's life.

A significant correlation was found between the relationship with the medical team and the CDS, which suggests that CF patients with higher CDS are better able to take advantage of available support, as reported in the literature. As mentioned, various factors, including gender, contributed to adjustment: female patients reported better social adjustment and greater life satisfaction than male patients. The difference can be explained by the culture of Israeli society where men are expected to serve in the military; a prototypical male experience that contributes significantly to social status, personal development, and self-esteem. Most CF patients are unable to fulfill this national obligation and may therefore feel left out of mainstream society.

Another explanation proposed in the literature for the poorer adjustment in men relates to the possible impact of CF on reproductive and sexual functioning, the latter of which is more prevalent in men than in women. Moreover, contrary to the interaction observed for membership, the relationship with the professional team contributed to the social adjustment of all respondents. The various patterns of associations that emerged here highlight that adherence and social adaptation are two very different, and sometimes even conflicting, challenges for the adult patient.

Another important finding of the present study concerns the distinct and significant contribution of subjective illness severity to psychosocial adherence and adjustment. Similar findings are reported by Bucks et al (2009), where non-adherence was associated with the patient's perception of the need for a given treatment and their condition, as well as with their sense of effectiveness and self-control: the more patients doubted the need for treatment, the less they adhered to it. Our results indicate a positive correlation between subjective disease severity and social adjustment, especially in patients with high CDS. This suggests that, despite their serious illness, CF may demonstrate positive coping because the subjective perception of the condition and the personality resources of the SDC can contribute significantly to social adjustment.

Limits

There are some limitations in this study and these should be taken into consideration before arriving at practical implications. First, the research tools were self-reported and based solely on patient reports. Future studies should include observational methods and data obtained from other relevant sources, such as professionals or family members. Second, the sample included patients who were receiving treatment at different CF centers in Israel and as a result, some of the factors identified could have been related to the local hospital environment and professional staff. Thirdly, no patients with other chronic conditions were included for comparison and, therefore, no generalizations to people with other chronic conditions can be drawn. Fourth, the variables were measured at a given point in time. As CF is a very dynamic disease that progresses and changes over time, studying individuals at a specific time leaves many of the dynamic and changing factors unexplored. Given the unique nature of CF, future research may adopt a longitudinal model to examine the association between adherence and psychosocial adjustment over time. Fifth, other dimensions of adjustment such as professional adjustment, quality of life, well-being, or family adjustment should also be examined in future studies. Finally, it cannot be ignored that specific cultural factors can have an impact on results. Subsequent studies may further examine psychosocial adjustment and patient adherence
with CF in the context of other cultures in order to obtain intercultural validation.

Implications for practice and conclusions

Despite its limitations, this study highlights various factors that contribute to life satisfaction and social adjustment in CF adults. This study confirms previous reports identifying the importance of subjective perception of disease severity in relation to objective impairment as it greatly affects compliance and life satisfaction. However, it is also crucial to add to the dialogue the importance of evaluating objective data and not relying solely on subjective experience as there is often a discrepancy between the two, as our findings indicate. The results suggest that, given the unique nature of CF, coping is not necessarily associated with, and may even conflict with, adherence. These findings challenge the common hypothesis that adherence to treatment leads to improved health status and, therefore, automatically improves coping. As the goal of social workers is to help people with CF cope with their developmental challenges, it is important that they consider the various elements of coping. The primary goal of social workers is to improve the quality of life of individuals, which is reflected in areas such as personal well-being, employment, social and family relationships, and recreation. Our findings highlight the importance of patients' relationships with their multidisciplinary professional team as well as the sense of coherence that play an important role in psychosocial adjustment. This is a unique challenge for CF adults as they must maintain a delicate balance between medical treatment protocol and quality of life.

Contrary to the pressures of CF and the demands for treatment, there are critical human needs such as independence, spontaneity, enjoyment, and personal development that reflect quality of life and lead to adjustment. This dissonance often leads to conflict between different areas of life, resulting in a lack of collaboration with health professionals and a reluctance to adhere to the treatment regimen.

There is a great personal cost to non-adherence. Medical needs are overlooked, but that's not to mention that individuals often do so in secret, without consulting or informing the medical team, family, or friends. This secrecy and non-adherence often leads to feelings of guilt, anxiety, and depression.

The role of the social worker is therefore to help adults bridge these two contrasting dimensions of their lives. In addition, they can serve as mediators between the individual and the main medical team concerned with the consequences of non-adherence. It is important to note that while those who are in better health are less cooperative and adapt better in the short term, lack of adherence to treatment can cause damage to patients' health and long-term adjustments. It is therefore very important for rehabilitation counsellors to be vigilant and to bring them to a compromise in which they will strictly adhere to the critical parts of the treatment in order to prevent a deterioration in their health in the future.

Finally, it is important to consider care and plan unique intervention strategies for men with CF who, according to research findings, have less life satisfaction and less satisfying social lives. This multi-dimensional approach values not only the rigorous treatment requirements, but also the interests, ambitions, and developmental tasks of the person with a disease, as well as the respect and consideration of these interests, ambitions, and tasks. This vision allows for the creation of more entrenched and better adapted treatment protocols that can promote and support greater cooperation and consistent adherence between adults with CF, which is an essential tool for quality of life and personal development.

Prof. Liora Findler
Department
Social Sciences from Bar-Ilan University
Tel Aviv, Israel

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