MANAGEMENT OF THE PATIENT WITH ADVANCED AMYOTROPHIC LATERAL SCLEROSIS: THE CAREGIVER’S OPINION

Objectives. The possibility of prolonging and augmenting the quality of life of respiratory patients in end-stage disease and in particular neuromuscular disease patients depends on an increased level of care. Amyotrophic lateral sclerosis (ALS) is a neurological disease characterized by a fast progressive impairment of respiratory function which leads to mechanical ventilation and high burden of care. A specifi cally designed questionnaire was administered to caregivers to analyze the level of care provided to these patients in our districts. Patients and methods. The study population consisted of 70 patients (44 males and 26 females). Of the 70 patients, 46 were being treated with non-invasive ventilation and 24 with invasive mechanical ventilation. The questionnaire was divided in two parts: questions 1 to 13 investigated life conditions and 14 to 22 the quality of care provided. Results. The patients studied present a heavy burden of care and this is in relation to the quantity of respiratory aids prescribed. The symptom which created statistically signifi cant problems for care was breathlessness, as is often reported in the literature. The presence of homecare tended to create conditions of greater serenity for the caregivers. Conclusions. This study shows that the burden of care in end-stage ALS patients is very onerous, and it can help to point out problems not suffi ciently contemplated in healthcare planning.


INTRODUCTION
Amyotrophic lateral sclerosis (ALS) is a neurological disorder characterized by a progressive impairment of motoneuron function due to degeneration of upper and lower motor neurons (1).
The improvement of medical care techniques is increasing the number of end-stage respiratory patients dependent on the continuous administration of mechanical ventilation and mechanical cough assistor. This situation is contrasted with a modifi ed social and family organization which is not always ready and prepared to sustain this burden of care (1,2). Symptoms of depression, lifestyle changes and a reduction in working hours are constant fi nd-ings among caregivers of family members with severe respiratory problems (2,3). The literature also reports of the signifi cant economic burden and provision of care which the family must sustain in a chronic setting such as the end-stage respiratory patient (2)(3)(4). In addition, the increasing need for care and surveillance of these patients is also well known (2)(3)(4)(5). This has prompted numerous studies which have evaluated either the psychosocial condition of the caregivers (2,3,4) or the quality of care seen from the perspective of the patient (5,6).
The aim of our study is the evaluation of: • the perception of the caregiver in terms of the state of health of their loved one; • their perception of the degree and quality of care provided; • the socio-economic situation of the caregiver of patients affected by a severe and • progressive respiratory condition subject to home mechanical ventilation.

MATERIALS AND METHODS
We enrolled 96 caregivers of patients affected by end-stage respiratory failure due to ALS. Eightyone of them accepted to participate to the study, fi fteen refused.
A specially designed questionnaire was administered to each caregiver. The questionnaire was proposed directly to the caregiver during a routine check-up of the patient. The aims of the study were clearly illustrated by a healthcare worker who was not involved in the care of the patient and the material was returned anonymously to a non-healthcare worker at each centre. The results of the questionnaire were processed independently by an operator external to the study.
The questionnaire has been reproduced in Appendix 1 and can be divided into two main parts. Questions 1 to 13 investigate the life conditions of the patient and caregiver, while questions 14 to 22 seek the views of the caregiver in terms of the care received from the national healthcare system and from the physician who discharged the patient.
The fi rst part of the questionnaire is further divided into two sub-groups. The fi rst analyses the lifestyle changes the caregiver had made (e.g. possible changes in the type of work, economic problems and the possible support received from other persons such as family or friends). The second subgroup of questions investigates the impact and psychological burden derived from caring for a person with a progressive and invalidating disease. The second part of the questionnaire, which as stated sought the perception of the quality of care received, was aimed at quantifying the relative level of satisfaction with the care provided by the centre where the patient was treated, from the home nursing service and lastly from the patient's general practitioner, with particular reference to possible problems regarding the use of respiratory support systems at home and the real availability of the various healthcare workers. The questionnaire was drawn up by a group of pulmonologists, physiotherapists and nurses operating in both centers. The questions of the questionnaire were processed and agreed upon by the physicians, physiotherapists and nurses involved in the study.

Ethical consideration
The study was conducted after obtaining the confi rmation of Local Ethics Committees. Every subject involved in the study has given the informed consent.

Statistical analysis
A descriptive analysis of the case series was performed with the Snedcor-Fisher F-test to evaluate the statistical differences between the group of patients with a greater number of prescribed respiratory aids (invasive mechanical ventilation group A) and the group of patients with lesser aids prescribed (non invasive ventilation group B) as well as a non parametric stratifi ed analysis with the Pearson chisquared test. The two groups were compared to investigate the difference of factors associated with burn-out of the caregiver (Table 2), diffi culty of the caregiver (Table 3) or factors possibly involved in producing or upsetting the serenity of the caregiver (Table 4). Findings were considered signifi cant for p<0.05.

RESULTS
Seventy caregivers fully responded to the questionnaire (86.4% of interviewees). The responses showed that in most cases patients are cared for by a close relative (usually the partner). Caregivers had to frequently modify their work choices or lifestyle as well as suffer the burden of increased expenses due mainly to the cost of transporting the patient. The responses also revealed that the care provided to the patient, both by the various healthcare facilities and the patient's general practitioner, was considered satisfactory for nearly all of the respondents.
Tables 1-4 summarize the main fi ndings. The two groups were not different in infl uencing the sense of burnout, of excessive burden of care, or in contrast in creating conditions of serenity for the caregiver (Table 1). Table 2 shows that none of the factors associated with burn-out of the caregiver reached statistical signifi cance. Burn-out was present in more than a third of the respondents, although this does not seem to be infl uenced by the patient's symptoms, the burden of care or the care provided by the healthcare services. In contrast some factors are signifi cantly present, thus dividing the caregivers into those with or without diffi culty in providing care (Table 3). When present, breathlessness proved to be a management problem in the group of care-givers with diffi culty, whereas the presence of homecare was more commonly associated with caregivers who perceived no diffi culties in providing care. Table 4 shows that the presence of a greater burden of care is signifi cantly present in the caregivers with diffi culties in relation to which external factors such as medical care or the support of other people, do not seem to help.

DISCUSSION
Respiratory failure is the most common cause of death for individuals with ALS, but the lives of most of these patients can be signifi cantly prolonged by support (6,7).The introduction of mechanical ventilation (both non-invasive and invasive) increased the burden of care for patients with neuromuscular diseases (7)(8)(9).
Our study shows that the burden of care for severe respiratory ALS patients is extremely onerous, with a signifi cant impact for the caregiver. These fi ndings confi rm other studies which have emphasized that the main problems encountered by caregivers are lifestyle changes, reduction in work activity and onset of symptoms of depression (2,3,4), with a degree of severity comparable to that experienced by caregivers of patients with Alzheimer's disease (4). It is also known that the quality of life of patients with respiratory symptoms is worse than in patients suffering from chronic heart failure or lung cancer (4). In addition, it should be noted that patients with respiratory complications usually receive less homecare and palliative treatment than patients with lung cancer (10,11). Despite the full participation of the caregivers in fi lling out the questionnaire, this study has a number of limitations.
The study was conducted in only two respiratory centers of North Italy and therefore it provided a limited picture of the ALS situation.
Another limitation is the fact that the clinicians who participated in the study dedicate most of their activity to the care of chronic respiratory patients and this could in some way produce a more positive result than in other less dedicated respiratory settings. In previous studies non-Italian authors have attempted to describe the level of care of patients with advanced chronic respiratory failure (12), but only in rare cases have attempts been made to evaluate the impact of the disease on their life conditions (2,3).

CONCLUSIONS
This study shows that the burden of care in severe respiratory patients as ALS patients is very onerous, with a signifi cant impact for the caregiver. It may contribute to highlight a problem which is still not suffi ciently recognized by the general public and healthcare planners not only in our country but worldwide by providing suggestions for the formulation of increasingly effective healthcare responses.

Acknowledgments
We thankfully acknowledge dr. Nicola R. Pizio and dr. Fabrizio Gallo for the valuable cooperation in this study.