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Logo of nihpaAbout Author manuscriptsSubmit a manuscriptNIH Public Access; Author Manuscript; Accepted for publication in peer reviewed journal;
J Cyst Fibros. Author manuscript; available in PMC Mar 1, 2009.
Published in final edited form as:
PMCID: PMC2490602
NIHMSID: NIHMS44615

SELF-REPORTED INVOLVEMENT OF FAMILY MEMBERS IN THE CARE OF ADULTS WITH CF

Abstract

Background

Few empirical studies have examined the role of family caregivers in the lives of adults with CF.

Methods

As part of the Project on Adult Care in CF (PAC-CF), an on-going prospective, longitudinal panel study of adults with CF, 119 family members and friends of adults with CF completed a mail survey in which they reported the frequency of help they provide for their family member with CF during routine care, hospitalization, and home IV treatment.

Results

The 119 caregivers were mainly spouses or unmarried partners (56%) and parents (29%). Fifteen percent of caregivers were children, friends, siblings or roommates. Family caregivers for adults with CF report assisting mainly with communication and social support during routine treatment, although one third provide some clinical care on a regular basis. Family caregivers report an increase in assistance during periods of acute illness, such as during a hospitalization and home IV treatment, especially with clinical care tasks.

Conclusions

The depth of commitment required of families of children with CF has been well documented for decades. Our results suggest that the responsibilities of family members diminish only moderately as those with CF reach adulthood.

INTRODUCTION

Improvements in life expectancy for cystic fibrosis in recent years have resulted in a substantial population of adults with CF. In 2004, over 41 percent of patients in the CF Foundation Registry were 18 years or older, as compared to 30 percent in 1990 [1]. Due to the recent growth in the adult population, there is a greater need for research and care focusing on adults [2].

At the same time as survival for those with CF has increased, there has been a gradual shift in the location of CF care away from the inpatient setting toward the home. Even for those with mild disease, the daily home regimen of preventive and maintenance care is complex and time consuming. Daily CF treatment regimens can include oral and/or nebulized antibiotics, chest physical therapy, nebulized mucolytic agents, pancreatic enzymes, vitamin supplements, exercise, and close monitoring of diet and nutrition [3]. The treatment regimen becomes more intense as the disease progresses [3, 4]. Even complicated and intensive therapies such as multiple intravenous antibiotics are now commonly administered at home. Patients experiencing pulmonary exacerbations (or patients having routine “clean-outs”) were previously hospitalized as a matter of course; current treatment practices commonly include either direct care at home or a combination of a shorter hospital stay, followed by home IV treatment [5].

The simultaneous rise in the complexity of the care regimen and the shift of care to the home means that the burden of care increasingly falls on patients and families; this requires that family caregivers provide direct and complex clinical care that was previously provided by professionals [6, 7]. Such a shift in care requires not only greater technical skill on the part of family caregivers, but also a substantial commitment of time and energy.

Parents of children with CF know the demands of the CF regimen well and the work of parents as family caregivers for children with CF is well documented. Research has examined the quality of life of parental caregivers and siblings of children with CF [4, 810] and the relationship between family function and the health of the child with CF [1114]. Patterson et al [14] found that the level of burden experienced by parents of children with CF is associated with decreased pulmonary function over time. Coyne et al [9] describe how time-consuming and complex daily care is for children with CF and point to the need to provide support for their parents.

Given the intensity of the clinical regimen, it would not be surprising if adults with CF continued to need substantial support from family caregivers, yet little attention in the medical literature has been paid to the involvement of family caregivers in the clinical care of adults with CF. To our knowledge, only one study has examined the role of family caregivers for adults with CF. [15]. As part of a qualitative interview study in the United Kingdom, thirty-one family caregivers were asked what types of care they provided to adults with CF. The caregivers reported picking up prescriptions, preparing and cleaning nebulizers, performing chest PT, and preparing and administering home IV therapies. Lowton found that parental caregivers provided more support overall to the adults, but spousal caregivers reported an increase in assistance to adults during periods of acute illness, such as during a hospitalization.

Because of our clinical suspicion that the involvement of family in the care of adults is substantial, and because of increasing use of outpatient therapies in the adult CF population, we sought to examine the self-reported role of family caregivers [16].

METHODS

The research reported here is part of a larger study of the care and quality of life of adults with CF in the United States called the Project on Adult Care in Cystic Fibrosis (PAC-CF). The design of the PAC-CF study and assembly of the study cohort have been described elsewhere in detail [17]. In brief, adults with CF 18 years of age or older were recruited from 10 CF Centers in the United States. Clinical characteristics provided anonymously by the CF Center were used to calculate each individual’s predicted probability of 5-year survival [18]. Because the primary focus of PAC-CF was to examine changes in quality of life as CF progressed, all adults with a predicted probability of 5-year survival [18]less than 0.975 and a randomly selected 25% of adults with a predicted probability of 0.975 or higher were asked by a study coordinator at their CF Center to participate in PAC-CF. Recruitment was initiated in 2004 and a total of 333 adults (58% of those approached) were enrolled. The study protocol was approved by the Institutional Review Boards at Education Development Center, Inc. and the ten hospitals in which the participating CF Centers are located.

For the caregiver component of the study, 289 of the PAC-CF participants were asked to provide the name of the family member or friend age 18 or older who was most involved in their care. Recruitment for the family caregiver study began about six months after data collection began with the adults with CF enrolled in the study. At that time, 16 participants had dropped out of the study due to death or receiving a transplant. An additional 28 adults enrolled in the study had not returned the first two surveys and were not asked to nominate a family member or friend. Of the 289 participants who were asked, 131 nominated a family member or friend (41% nomination rate) for recruitment. Of the 131 family caregivers nominated, 119 agreed to participate by completing a mail survey, for a 91% participation rate.

The family caregiver survey included demographic questions and a series of questions related to the type and frequency of assistance the family caregiver provides for their loved one with CF. Lists of the ways in which family caregivers may assist adults with CF were developed by a research team that included a pulmonologist who specializes in CF care and an adult with CF. The types of care included routine clinical care (e.g. “performing chest physical therapy,” “setting up nebulizer treatments,” “reminding to take routine pills”), obtaining and paying for medical care (e.g. “scheduling clinic visits,” “communicating with insurance companies”), care during hospitalizations (e.g. “bringing in outside food,” “meeting with a physician”), and care during home IV treatment (e.g. “taking care of home IV equipment,” “preparing meals”).

For routine care provided during the past four weeks and care provided during hospitalizations and home IV treatments, family caregivers were asked to rate the frequency of help they provide using six categories that included “not at all”, “a few times”, “about once a week”, “2–4 times a week”, “almost daily”, and “once a day or more”. Caregivers were asked to report how frequently they assisted their loved one with CF with activities related to obtaining and paying for routine care during the past 12 months using six categories that included “not at all”, “rarely”, “occasionally”, frequently, “almost always”, and “always”. As will be described in detail in conjunction with reporting of results, some categories were combined to facilitate summary of results.

Clinical data on PAC-CF participants, including maximum FEV1%, weight-for-age z-score, number of pulmonary exacerbations, diabetes, pancreatic sufficiency, and colonization with Burkholderia cepacia and Staphylococcus aureus, was provided by the participant’s CF Center. Patient age, gender, education, marital, and employment status were reported by the PAC-CF participant in the first PAC-CF mail survey.

Standard descriptive statistics were used to summarize the demographic characteristics of the caregivers and the demographic and clinical characteristics of the adults with CF who nominated the caregivers. T-tests and chi-square tests of independence were used to compare characteristics of adults with CF in PAC-CF who did and did not have a participating caregiver. One-way analysis of variance and chi-square tests of independence were used to compare the characteristics of adults with CF and their caregivers as well as the types of assistance provided by the caregivers by type of caregiver. The delineation of types of caregivers is explained below in the results section. Analyses were completed in SPSS version 14.0 (SPSS, Inc., Chicago, IL.).

RESULTS

We compared the demographic and clinical characteristics of PAC-CF participants with and without a participating family caregiver to determine whether the 119 adults with CF with family caregivers differed systematically from the 214 adults without. The mean age, gender distribution, and clinical characteristics of these two groups did not differ significantly. However, adults with a participating caregiver are more likely to be married (61% vs. 30%, p=.0001), live with family members (82% vs. 53%, p=.0001) and be employed (62% vs. 50%, p=.03).

About two-thirds of the caregivers live with the adult with CF. Just over half of the caregivers are spouses or partners (56%, n=67), about 30% are parents (n=35), and the remainder include siblings (n=8), an adult child (n=1), friends (n=7), and a roommate (n=1). Some parents did not currently live with the adult with CF (n=24) and some parents did (n=11). Nearly all (65 of 67) of the spouses/partners were currently living with the adult with CF while most (15 of 17) of the other caregivers (siblings, adult child, friends, roommate) did not currently live with the adult with CF. Since living with the adult with CF and the relationship of the caregiver to the adult with CF could impact the type of assistance provided, four types of caregivers were delineated by a combination of these two characteristics: 1) Spouse/partner living with the adult with CF (n=65), 2) Parent living with the adult with CF (n=11), 3) Parent not living with the adult with CF (n=24), and 4) Friends/siblings/adult children,(n=15) not living with the adult with CF. The small number of unusual cases, two spouses/partners not currently living with the adult with CF and one roommate and one sibling who did currently live with the adult with CF are excluded from comparisons based on the type of caregiver but included in analyses of the entire sample of caregivers.

Demographics of the Caregivers

Table 1 reports the demographic characteristics of the caregivers by type of caregiver. The age and gender of the family caregivers vary by type of caregiver with parents being older than the other types of caregivers. Most caregivers are women, except for spouses/partners, where only 43% are female. In general, the majority of caregivers are employed (78%) and have a college degree (60%). Most caregivers are also married, though this characteristic varies some by type of caregiver.

Table 1
Demographic Characteristics of Caregivers by Type of Caregiver

Demographics of the adults with CF

Table 2 reports the demographic and clinical characteristics of the adults with CF by type of caregiver. There are significant differences in some demographic characteristics of the adults with CF by type of caregiver. The adults with CF who live with parental caregivers are the youngest (mean age=21.4) and the adults with CF with spouse/partner caregivers are the oldest (mean age=36.4). These age difference may contribute to the lower level of education (only 9% with a college degree), the smaller percentage who are married (0%), and the better clinical status (mean FEV1%=77.6 and mean number of exacerbations=.82) of the adults with CF who live with their parents. Similarly, the increased age of the adults with CF with spouse/partner caregivers may account for the higher level of education (77% with a college degree) and the poorer clinical status (mean FEV1%=59.0 and mean number of exacerbations=1.2) of these adults with CF.

Table 2
Demographic and Clinical Characteristics of Adults with CF by Type of Caregiver

Assistance Provided by Caregivers during Routine Care

Table 3 summarizes the assistance provided by caregivers with twelve tasks related to routine care during the past month. Of 12 types of assistance, only two, reminding to complete treatments and preparing special meals, differed significantly by type of caregiver. Two-thirds of caregivers who lived with the adult with CF reported that they reminded their loved one to complete treatments. Over half of parents who lived with the adult with CF but only one-quarter of spouses/partners reported reminding their loved one more than once a week. In contrast, less than half of caregivers who did not live with the loved one with CF reported reminding their loved one to complete treatments and very few reminded them more than once a week. With preparing special meals, 60% of parents who lived with the adult with CF prepared special meals more than once a week while 73% of siblings/friends never prepared special meals.

Table 3
Involvement of Caregivers during Routine Care in the Past Month

The assistance provided with most routine tasks did not differ by type of caregiver. In general, more than half of caregivers did tasks such as reminding the adult with CF to exercise or take pills, though less than one-quarter did these activities more than once a week. Less than one-third of caregivers were involved in more clinical aspects of care such as cleaning nebulizer equipment, performing chest physical therapy, or maintaining oxygen supplies. Very few caregivers reported doing these more clinical activities more than once a week.

Assistance with Obtaining and Paying for Care

Caregivers were asked to report how frequently in the past 12 months they helped the adult with activities related to obtaining and paying for medical care. Table 4 reports the percentage who reported frequently, almost always, or always (versus those who reported not at all, rarely, or occasionally) by type of caregiver. One-third and one-quarter of caregivers reported, respectively, going to clinic visits and communicating with clinic staff during visits though less than 3% of caregivers reported communicating with clinic staff between clinic visits. On these tasks, there were not significant differences between types of caregivers. However, spouse/partners and parents living with adult are significantly more likely to order or pick up prescriptions (p=.006). Parents living with adults are significantly more likely than other types of caregiver to schedule clinic visits (p=.0001), take notes at clinic visits (p=.042), communicate with hospital billing offices (p=.0001), communicate with insurance companies (p=.0001), and complete insurance forms (p=.0001).

Table 4
Percent Reporting Assistance with Obtaining and Paying for Medical Care in the Past Year by Type of Caregiver a

Assistance during hospitalization

Table 5 summarizes the assistance provided during the most recent hospitalization by the 81 caregivers who reported that their loved one with CF had been hospitalized in the past 12 months. The assistance provided during hospitalization did not vary by type of caregiver, so the percentage of all caregivers who provided each type of assistance is reported. Caregivers spent considerable time with the adult while hospitalized, with 80% reporting that they spent all day in the hospital at least once, and 34% report spending all night at the hospital at least once. Almost all reported bringing in outside food for the adult with CF. Nearly three-quarters reported meeting with a physician and/or a nurse. Nearly half met with a social worker and accompanied their loved one to testing during the hospitalization. The assistance provided by the caregivers came at the expense of lost time at work. About two-thirds of caregivers reported missing at least one day of work during a recent hospitalization and one-fifth reported missing 3 or more days of work.

Table 5
Percent of Caregivers Providing Assistance during the Most Recent Hospitalization

Assistance during home IV treatment

Caregivers provide considerable assistance to adults with CF during home IV treatment. As reported in Table 6, routine household maintenance is provided by the overwhelming majority of caregivers during home IV therapy, even by those who do not live with the adults. Caregivers also report assistance with complex clinical tasks directly related to the home IV treatment: 62% take care of the IV equipment, 54% prepare antibiotic doses, and 42% maintain or change dressings on IV lines. Caregivers also report playing a role in managing the professionals who enter the home during IV therapy, as 81% of spouses/partners, 100% of parents living with adults, 39% of sibling/adult child/friends, and even 57% of parents not living with the adult communicate with home nursing staff during home IV treatment.

Table 6
Percent of Caregivers Providing Assistance during the Most Recent Home IV Treatment by Type of Caregiver

DISCUSSION

Family caregivers report substantial roles in both the routine and intermittent care of adults with CF. The depth of commitment required of the families of children with CF has been a recurrent theme in the literature on CF for decades; our study strongly suggests that the responsibilities of family members diminishes only moderately as those with CF reach adulthood. These findings should not be surprising, given the burden of self-management which is characteristic of CF; yet we suspect that the depth of involvement of family members reported here will be surprising to some because the care provided outside the walls of medical facilities is often invisible to clinicians. The increasing shift of care away from the institutional setting has it benefits, such as greater autonomy, but these results suggest that moving care into the home has burdens as well. Family members are essential partners in the care of adults with CF, not only as psychosocial support, but as providers of direct clinical care. The 91% response rate among caregivers in this study may be evidence of their commitment to their loved one with CF as well as their eagerness to have their role recognized and understood.

The role of family caregivers in maintaining a treatment regimen should not be underestimated. Family caregivers for adults with CF report assisting mainly with communication and social support during routine treatment, although one third provide some clinical care on a routine basis. One of the most frequently reported forms of assistance provided by family caregivers is to serve as reminders to take pills, complete treatments, and exercise. This simple assistance may be especially helpful to adults, since research on self-management for CF patients suggests that forgetfulness is the most common reason given for omitting treatment [3, 19]. Another type of frequently reported assistance provided by family caregivers is to pick up prescriptions. This is consistent with Lowton’s [15] study of family caregivers for adults with CF.

All types of family caregivers reported more direct involvement in providing assistance with care during periods of acute illness. Family caregiver involvement during hospitalization is substantial and led to missed work for two-thirds of the family caregivers for adults with CF. Adults with CF are likely to experience the most disruption of daily life during a pulmonary exacerbation, and family caregivers play an important role in assisting with day-to-day household tasks and providing support such as spending time with them while hospitalized. It is striking that even those family caregivers who do not live with the adults provide assistance such as preparing meals and housecleaning while adults are undergoing home IV treatment.

Parental caregivers living with the CF adult report the highest frequency of assistance with routine care and obtaining and paying for medical care, especially with tasks related to scheduling visits and communicating with CF center staff and insurance companies. This may be because adults still living with parents are younger, may still be included on their parent’s health insurance, and are undergoing the transition from pediatric to adult care. Even with the transition to adult clinical models of care, with the standard that adults be seen as responsible for their own care [2], young adults with CF continue to rely on parents for many aspects of routine care, and the dependence on parents persists even after they marry and no longer live under their parents’ roof.

Aside from the depth of involvement of family members, several aspects of the character of family care giving were notable. Although it was anticipated that most of the family caregivers nominated for participation would be spouses or parents, it was interesting to find that 42% of CF adults with parental caregivers not living with them were married. This suggests that adults with CF who are married may consider their parents, and not their spouses, as primary caregivers with regard to CF. This pattern is supported by Lowton’s finding that partners have less involvement in the care of adults than parents, except when the adult experiences an exacerbation. This finding may point to a different type of transition in care for adults than is typically described. Much of the literature on adults with CF has described the transition that CF patients make as they move from pediatric to adult care and take on greater responsibility for their own care. After transition to adult care, adults may experience a second transition involving a shift in the family caregiver from parents to spouse or partner. The mean age of adults in the sample with parental caregivers not living with them is 28. These adults may be experiencing a second transition involving a shift from parental to spousal/partner caregiver, approximately 7 to 10 years after the initial transition from pediatric to adult care.

Given their depth of involvement in the care of adults with CF, family caregivers may also experience similar burdens as has been reported for family caregivers in other illness [2022]. However, because of the much higher functional status of most adults with CF, comparison of family caregiver burden to adult children caring for elderly parents may not be informative. A second stage of our research to measure family caregiver burden is underway. Support for family caregivers of adults with CF is a necessary part of the activities of adult CF care. Even though adults with CF should be seen as autonomous individuals, our research suggests that family caregivers are deeply involved in achieving and maintaining the gains in life expectancy seen over the past decades in CF.

ACKNOWLEDGEMENTS

Supported by a grant from the National Heart, Lung, Blood Institute (R01 HL72938). The authors would like to acknowledge the contributions of Ian Huntington and Diana Metrick as well as the adults with CF, their caregivers, and the ten CF Centers participating in the study to the work presented in this paper.

Footnotes

Publisher's Disclaimer: This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.

Gregory Sawicki has no conflicts to disclose.

Deborah Sellers has no conflicts to disclose.

Kimberly McGuffie has no conflicts to disclose

Walter Robinson has no conflicts to disclose.

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