Falls,Fear of Falling,and Preventive Measures in Huntington’s Disease: The Perspectives of Individuals with Huntington’s Disease and Caregivers in Long-Term Care

Abstract

Background:

Falls are common in Huntington’s disease (HD), which can have serious consequences and may therefore lead to fear of falling (FoF). There is little knowledge about falls or FoF in individuals with HD or about formal and informal caregivers’ fear about falls in individuals with HD.

Objective:

To explore prevalence of falls, FoF and fall preventive measures both those applied and those not included in managing falls in individuals with HD and their formal and informal caregivers, and to identify the relationship between FoF and, anxiety, awareness and cognitive functioning respectively.

Methods:

In a multi-center observational cross-sectional study, care-independent and -dependent individuals with HD and their formal and informal caregivers were recruited from six Dutch nursing homes specialized in HD. The participants were assessed by means of questionnaires enquiring about falls, FoF, awareness of fall risk, cognition, anxiety and fall preventive measures.

Results:

For all included 158 individuals with HD, the fall prevalence over the last 30 days was 28.8%. The prevalence of FoF in individuals with HD, formal caregivers and informal caregivers was 47.6%, 25.6%, and 63.5%, respectively, for care-independent individuals with HD and 46.9%, 26.3%, and 62.0%, respectively, for care-dependent individuals with HD. Anticipatory awareness of fall risks and gender are predictors of FoF in care-independent individuals with HD, though not in the care-dependent group. A combination of fall preventive measures is used in most individuals with HD.

Conclusion:

Fall prevalence is high and FoF is common in individuals with HD and their caregivers. Gender and anticipatory awareness are risk factors for FoF. In addition to the use of individual multifactorial fall prevention strategies, it is important to support both formal and informal caregivers in coping with falls

Keywords

Huntington’s disease falls fear cognition awareness caregivers

INTRODUCTION

Huntington’s disease (HD) is a rare, neurodegenerative disease, characterized by involuntary choreic movements, behavioral and psychiatric disturbances, and dementia. The mean age at onset is usually 30–50 years (2–85) with a mean duration of 17–20 years [1]. Progression of the disease leads to increased limitations in daily life and progressive care needs [1, 2].

Falls are common in HD and have a great impact on the quality of life [3, 4]. Annual rates of fall incidents in individuals with HD vary between 50%and 79.2%[3 , 5–7], frequently resulting in hospitalization often followed by nursing-home admission [3]. Even in nursing homes, individuals with HD have frequent falls [8]. A complex interaction of contributing intrinsic and extrinsic factors underlies falling in HD [3].

Intrinsic risk factors include gait impairment, which is characterized by chorea, bradykinesia, reduced velocity, and increased variability in spatiotemporal features [4]. In addition, there are various causes of body balance impairment, including impairments of anticipatory balance and reactive balance [4]. Besides gait and balance problems, disturbances in behavior and cognition, such as risk-taking behavior, lack of attention and lack of insight, contribute to falling [3]. In HD and other neurological diseases, also extrinsic risk factors, such as medication, the type of walking aid used and environmental factors play an important role [3 , 10].

Given the potential serious consequences, a fall may also lead to fear of falling (FoF), consequently resulting in activity restriction and loss of independence [11]. Hence, paradoxically, FoF is also a predictor of future falls [12]. However, in a retrospective study of falls in ambulant individuals with HD, only 15%of fallers were afraid of falling, comparable to the non-faller group [3]. Some studies in individuals with HD reported reduced fear responses [13, 14]. Experiencing less fear may influence the ability to act safely. A possible cause of experiencing less fear could be lack of awareness, defined as ‘the individual underestimates or does not notice symptoms or the impact, obvious to the objective observer’ [15]. The terms lack of awareness, self-awareness, unawareness, insight, anosognosia, and denial are often used interchangeably in literature for HD [15]. As a result of this lack of awareness, the person will take more risks [16]. In HD, lack of awareness can appear at any stage [17]. Furthermore, the level of fear that is experienced by individuals with HD can also be influenced by cognitive factors, such as the ability to evaluate possible consequences of behavior [3], the ability to comprehend information and being aware of what task demands involve [18].

Little is known about the effect of an HD patient falling, on the formal (e.g., nurse or physician) or informal (e.g., family members or friends) caregiver [19, 20]. Informal caregivers of individuals with Parkinson’s disease were concerned about falls and felt unprepared for their role [21, 22]. In people with dementia [23, 24], it is known that the fearful attitudes of (in)formal caregivers results in restriction of the care recipient’s activities. The caregiver’s fear may subsequently increase the patient’s anxiety and reduce their quality of life.

As there is little knowledge about falls or FoF in individuals with HD or about the (in)formal caregivers’ fear about falls in individuals with HD, we aim to explore the prevalence of falls and FoF, and the measures currently applied, as well as those not included in managing falls in individuals with HD and their caregivers. Studies show that motor dysfunction is the main contributor to institutionalization [2, 25]. In addition, reduced independence and functional capacity are related to falls in HD [5]. Therefore, we have chosen to focus on individuals with HD receiving care in nursing homes and thus for individuals with HD in moderate and advanced stages of HD.

The research questions of the study are, therefore:

What is the prevalence of falls in care-independent and -dependent individuals with HD?

What is the prevalence of FoF in care-independent and -dependent individuals with HD?

What is the association between FoF and lack of awareness, cognition and anxiety in care-independent and -dependent individuals with HD?

Which fall preventive measures are used and which are missed by care-independent and care-dependent individuals with HD?

What is the prevalence of formal and informal caregivers’ fear about falls in care-independent and -dependent individuals with HD?

Which fall preventive measures are used and which are missing, from the formal and informal caregivers’ perspectives?

This study is part of a larger study on FoF and fear of choking in individuals with HD [26].

METHODS

Design

This study is an observational cross-sectional study carried out in individuals with HD, receiving care in one of the Dutch nursing homes specialized in HD, and in their formal and informal caregivers. The extensive protocol of this study has been published elsewhere [26].

Participants

Individuals ≥18 years, with clinically and/or genetically confirmed HD were included. Written informed consent was obtained from all participants or their legal guardian. They were recruited from six Dutch HD specialized care organizations with long-term and day-care facilities, where we aimed to reach the maximum number of individuals with HD.

Individuals with HD with interfering neurological or serious psychiatric disorders, with balance disorders due to other causes, or who were suffering from a terminal or serious illness, or who had participated in intervention trials, were excluded. If individuals with HD were unable to answer all questions, only the information provided by their caregivers was used.

Informal and formal caregivers

One formal and one informal caregiver, closely related to each patient, were asked to participate to assess the prevalence of FoF and define the current and missing fall preventive measures. Because the individuals with HD were recruited in organizations for long-term care, the informal caregivers were generally not involved in the day-to-day care.

Assessments

Patient information was derived from the patient and their primary responsible nurse. The prevalence of falls was obtained, retrospectively, for a period of 30 days by a nurse on the basis of daily care reports. The prevalence represents the percentage of residents who have fallen at least once during the last 30 days. A fall was defined as an event when the resident accidentally ends up on a lower level or on the ground [27].

Patient questionnaires were administered by means of a standardized (semi-)structured interview. In order to standardize the interviews, all questions and answer options were integrated into a standardized flowchart. FoF was quantified with a single question requiring a yes-no answer (‘Are you afraid of falling?’) [28]. In addition, the Short Falls Efficacy Scale-International (Short FES-I) was used, in which FoF is conceptualized as low-perceived, self-efficacy at avoiding a fall during basic activities of daily living [28] when carrying out seven physical and social activities [29, 30]. To examine awareness of personal fall risk, parts E and A of the Self-awareness of Fall Risk Questionnaires (SAFR) [31], developed for older persons, were used. In these subscales, using a calculation of discrepancy scores between patient-rated ability and informant-rated ability on parallel questionnaires, the Emergent awareness (part E) and Anticipatory awareness (part A) of fall risks are examined [31]. Emergent awareness is defined as the ability to identify a problem as it is occurring. Items evaluate aspects such as ‘ability to lift your feet from the floor’ [31]. Anticipatory awareness is defined as the individual’s ability to anticipate a problem occurring due to some deficit [31]. Items explore the patient’s ability to understand the level of assistance needed to perform activities (e.g., the amount of assistance needed to ‘getting dressed’) [31]. In addition to the patient’s version, an informant version was completed by the physiotherapist (part E) and the nurse (part A), regarding their specific skills in evaluating a walking task and knowledge of the assistance needed, respectively. For scale E (6 items; total range 6–30) a negative discrepancy score and for scale A (10 items; total range 7–70) a positive discrepancy score is interpreted as less awareness. Cognition was examined using the Montreal Cognitive Assessment [32], a brief cognitive screening tool. The Hospital Anxiety and Depression Scale (HADS) [33] was used to measure the level of anxiety experienced by the patient. The fall preventive measures currently used and those that were missed were asked about in an interview. The missing measures were defined as measures that are not applied but the patient would like to see applied. The answer could be selected from a variety of possible standard measures, but there was also room for alternatives.

In addition, the trained primarily responsible nurse completed an online questionnaire about general patient characteristics and mobility. One item of the Braden scale for predicting pressure sore risk was used to specify the level of physical activity [34]. One item of the Unified Huntington Disease Rating Scale For Advanced Patients specified the need for walking assistance or use of walking aids [35]. Functional capacity was examined with the Total Functional Capacity Scale, a 5-item clinician rating scale part of the Unified Huntington Disease Rating Scale [36]. The extent to which the patient is care-dependent was examined with the Care Dependency Scale [37], which consist of 16 items based on 15 basic needs and one subjective judgement of care dependency. A score of 45 or higher means that the patient was classified as care-independent, a score of 44 or lower as care-dependent.

Informal and formal caregivers

Subsequently, the prevalence of the formal and informal caregivers’ fear about falls in individuals with HD was assessed using a self-administered, parallel, caregivers’ version of the single direct question about FoF (‘Are you afraid the patient will fall?’) and the Short FES-I (‘How concerned are you the patient might fall?’). Hereafter, caregivers’ fear about falls in individuals with HD will be referred to as caregivers’ FoF. Concluding this topic were questions about fall preventive measures that are currently used and that are missing, in parallel with the version for individuals with HD’.

Ethical approval

The Medical Ethics Review Committee of VU University Medical Center (2017.445) declared that the Medical Research Involving Human Subjects Act (WMO) does not apply and hence official approval by the committee is not required [38]. Written consent was obtained from all participants.

Statistical analyses

Analyses were performed using the Statistical Package for the Social Sciences (SPSS) version 26. The results are shown for the patient group as a whole, and separately for care-independent and care-dependent individuals with HD based on the Care Dependency Scale score. Hereafter, these individuals with HD will be referred to as independent and dependent.

To evaluate the differences between the independent and dependent groups for demographic data and patient characteristics and FoF and short FES-I scores Generalized Linear Models were used. The outcome measures FoF and short FES-I was assessed by the patient themselves, and by their formal and informal caregivers. Because these assessments concern the same patient, they are statistically correlated. To account for this, we used Generalized Estimating Equations (GEE) with an exchangeable correlation structure, to evaluate the differences within the independent and dependent group. Finally, we evaluated the outcome measure FoF in a multivariable logistic regression analysis. Included in the regression model were Emergent and Anticipatory awareness of fall risks, cognition, anxiety, age, gender, falls, activity level and walking assistance. A p-value ≤0.05 was considered significant.

Multiple imputation for missing data was used. Ten imputations were computed. The imputation model for the individuals with HD’ characteristics and factors associated with FoF included Emergent and Anticipatory awareness of fall risks, cognition, anxiety, age, gender, falls, activity level, walking assistance, individuals with HD’ FoF, individuals with HD’ Short FES-I score, Care Dependency Scale score and Total Functional Capacity score. For FoF and Short FES-I scores, individuals with HD’ and (in)formal caregivers’ FoF, individuals with HD’ and formal and informal caregivers’ Short FES-I score, age, gender, activity level, walking assistance, falls, Care dependency Scale score and Total Functional Capacity score were included. Finally, in addition to the measures mentioned by individuals with HD and (in)formal caregivers, the model for current and missed measures included age, gender, falls, Care Dependency Scale score and Total Functional Capacity score.

RESULTS

Participants

A total of 245 individuals with HD meeting the inclusion criteria were approached for participation. In total, 161 individuals with HD or their legal representatives, gave consent. Three individuals with HD died before the start of the assessments, resulting in 158 participants.

General patient characteristics and prevalence of falls at baseline are shown in Table 1. The dependent group was slightly but significantly older (p = 0.05), had lower functional capacity (p < 0.001), a higher average number of falls (<0.001), lower activity level (p < 0.001), a greater need for assistance with walking (p < 0.001), a lower level of cognitive functioning (p < 0.001) and less anticipatory awareness of fall risks (p = 0.010) compared to the independent group. There was no significant difference between the groups with regard to gender (p = 0.72), prevalence of falls (0.14), anxiety (p = 0.27) and emergent awareness of fall risks (p = 0.12).

Table 1

Demographic data and general patient characteristics (means and percentages) of the individuals with Huntington’s disease

	All Individuals with HD (n = 158)	Independent group (n = 83)	Dependent group (n = 75)	p
Age (SD)	58.8 (12.1)¹	57.1 (12.8)	60.7 (10.9)¹	0.05
Male (%)	46.8	48.2	45.3	0.72
UHDRS Total Functional Capacity (SD)	2.0 (2.5)	3.7 (2.4)	0.2 (0.4)	< 0.001
Care Dependency Scale (SD)	45.2 (17.9)	60.3 (7.4)	28.5 (9.2)	< 0.001
Prevalence of falls (%)	29.3²	24.1	34.0²	0.14
Average number of falls (SD)	0.8 (1.8)²	0.6 (1.5)	1.0 (2.0)²	< 0.001
Activity level (%)				< 0.001
–Bedridden	5.9	0.0	12.5
–Confined to a chair	17.6	1.2	35.7
–Walks occasionally	23.5	15.7	32.3
–Walks frequently	52.9²	83.1	19.5²
Walking assistance (%)				< 0.001
–Possible without help	45.1	63.9	24.4
–Possible with aid	24.1	28.9	19.2
–Possible with the help of a person or in a wheelchair, which patient can operate himself	8.5	4.8	12.3
–In a wheelchair, the patient must be helped to steer it	14.9	2.4	28.8
–Impossible (bedridden patient, unable to sit)	7.3²	0.0	15.1²
MoCA (SD)	11.9 (8.4)³	17.3 (5.8)⁷	5.9 (6.6)¹¹	< 0.001
HADS-anxiety (SD)	4.6 (5.4)⁴	4.5 (4.9)⁸	4.8 (5.8)¹²	0.79
SAFR-E (SD)	–5.5 6.2)⁵	–3.1 (4.7)⁹	–8.2 (6.5)¹³	0.12
SAFR-A (SD)	3.6 (15.5)⁶	–0.6 (9.7)¹⁰	8.2 (18.9)¹⁴	0.01

Scores are based on pooled imputed data. HD, Huntington’s disease; SD, standard deviation; UHDRS, Unified Huntington’s Disease Rating Scale; MoCA, Montreal Cognitive Assessment; HADS, Hospital Anxiety and Depression Scale; SAFR, Self Awareness of Fall Risks. Data were unavailable for the following number of individuals with HD: ¹ 1 individual; ²2 individuals; ³individuals; ⁴65 individuals; ⁵78 individuals; ⁶56 individuals; ⁷18 individuals; ⁸15 individuals; ⁹23 individuals; ¹⁰11 individuals; ¹¹51 individuals; ¹²50 individuals; ¹³55 individuals; ¹⁴45 individuals.

Falls

In the total group, 29.3%of the individuals with HD had experienced one or more falls in the previous 30 days. In the independent and dependent groups, the prevalence of one or more falls was 24.1%and 35.0%, respectively (p = 0,14).

Fear of falling

FoF did not differ between the independent (47.6 %) and dependent (46.9%) individuals with HD (p = 0.94). Nor was any difference found between the groups with the Short FES-I (Table 2). The caregivers FoF was much higher in the informal caregivers (62.8%) compared to the formal caregivers (25.9%), with no difference between the two subgroups of HD. The average Short FES-I scores for the formal and informal caregivers showed no significant difference.

Table 2

Percentages of Fear of falling and total scores of fall-related self-efficacy in individuals with Huntington’s disease and from their formal and informal caregivers’ perspective

	Fear of Falling (%)				Short FES-I (SD)
	All individuals with HD (n = 158)	Independent group (n = 83)	Dependent group (n = 75)	p	All individuals with HD (n = 158)	Independent group (n = 83)	Dependent group (n = 75)	p
Individuals with HD	47.2¹	47.6⁴	46.9⁷	0.94	13.6 (6.9)¹⁰	12.6 (7.9)¹³	14.6 (5.8)¹⁶	0.27
Formal caregivers	25.9²	25.6⁵	26.3⁸	0.93	12.9 (5.7)¹¹	12.9 (6.4)¹⁴	12.9 (5.1)¹⁷	0.99
Informal caregivers	62.8³	63.5⁶	62.0⁹	0.90	16.2 (6.9)¹²	15.0 (7.3)¹⁵	17.6 (6.3)¹⁸	0.07
p-value Individuals with HD vs formal caregivers	0.002	0.007	0.20		0.51	0.67	0.35
p-value Individuals with HD vs informal caregivers	0.025	0.042	0.06		0.014	0.004	0.14
p-value formal caregivers vs informal caregivers	< 0.001	< 0.001	< 0.001		< 0.001	0.012	0.001

Scores are based on pooled imputed data. HD, Huntington’s disease; SD, standard deviation; Short FES-I, Short Falls Efficacy Scale-International. Data were unavailable for the following number of participants ¹58 individuals with HD; ²14 formal caregivers; ³40 informal caregivers; ⁴12 individuals with HD; ⁵10 HD formal caregivers; ⁶20 informal caregivers; ⁷46 individuals with HD; ⁸44 formal caregivers; ⁹20 informal caregivers; ¹⁰54 individuals with HD; ¹¹13 formal caregivers; ¹²56 formal caregivers; ¹³11 individuals with HD; ¹⁴9 formal caregivers; ¹⁵26 informal caregivers; ¹⁶43 individuals with HD ¹⁷4 formal caregivers; ¹⁸30 formal caregivers.

Factors associated with fear of falling

SAFR-A (p = 0.01) and gender (p = 0.01) were found to be predictors of FoF in the independent group (Table 3). Controlling for emergent awareness, cognition, anxiety, age, falls, activity level and walking assistance, showed that both female individuals with HD and individuals with HD with more anticipatory awareness of fall risks are more likely to experience FoF. In the dependent group, no significant predictors of FoF were found.

Table 3

Factors associated with Fear of Falling in care independent and dependent individuals with Huntington’s disease

	Independent group (n = 83)			Dependent group (n = 75)
Variable	O.R.	95%C.I.	p	O.R.	95%C.I.	p
SAFR-E	0.97¹	0.83 –1.14	0.73	1.01 ⁵	0.81–1.25	0.95
SAFR-A	0.89²	0.81–0.97	0.01	0.93⁶	0.84–1.04	0.21
MoCA	1.03³	0.91–1.17	0.64	1.10⁷	0.84–1.44	0.49
HADS-anxiety	0.97⁴	0.84–1.11	0.61	1.05⁸	0.81–1.35	0.72
Age	1.0	0.96–1.05	0.86	0.97⁹	0.89–1.06	0.49
Gender	4.65	1.44–15.01	0.01	1.23	0.21–7.21	0.82
Falls	1.09	0.59–2.02	0.77	0.98¹⁰	0.63–1.52	0.92
Activity level	0.78	0.12–5.15	0.79	0.20¹⁰	0.02–1.68	0.14
Walking assistance	2.06	0.63–6.73	0.23	1.10¹⁰	0.23–5.16	0.90

Scores are based on pooled imputed data. O.R., odds ratios; C.I., confidence interval; SAFR-E, Self Awareness of Fall Risks part E; SAFRA, Self Awareness of Fall Risks- part A; MoCA, Montreal Cognitive Assessment; HADS-anxiety, Hospital Anxiety and Depression Scale part Anxiety. Data were unavailable for the following number of individuals with HD: ¹23 individuals; ²11 individuals; ³18 individuals; ⁴15 individuals; ⁵55 individuals; ⁶45 individuals; ⁷51 individuals; ⁸50 individuals; ⁹1 individual; ¹⁰2 individuals.

Fall preventive measures

The average number of fall preventive measures used, according to individuals with HD, was 4.1 in the independent group and 6.0 in the dependent group (p < 0.001). According to the formal caregivers, an average of 4.2 different measures were used in the independent group and 4.4 (p = 0.26) in the dependent group. For informal caregivers, these numbers were 4.2 and 4.8, respectively (p < 0.001).

Therapeutic exercises/training or physiotherapy was the most frequently used fall preventive measure in the independent group according to both individuals with HD and (in)formal caregivers (64.5–73.9%). In the dependent group, both individuals with HD and (in)formal caregivers mentioned that assistive devices were most frequently used, followed by exercises/training and physiotherapy (see Table 4). In addition to a list of possible standard measures, participants could also name other alternative measures currently applied. Individuals with HD reported in this section: getting used to a new living environment (0.6%), fall training (0.6%), stopping cycling (0.6%) and wearing compression stockings (0.6%). The involvement of an occupational therapist (1.9%) was mentioned by formal caregivers and providing distraction in order for the individual with HD to remain seated (0.6%) was quoted as an alternative currently used measure by the informal caregivers.

Table 4

Percentages of current and missing fall preventive measures from the perspective of individuals with Huntington’s disease and their formal and informal caregivers

Measures	Independent group (n = 83)						Dependent group (n = 75)
	Current measures in %			Missing measures in %			Current measures in %			Missing measures in %
	Individuals with HD¹	Formal Caregivers²	Informal Caregivers³	Individuals with HD¹	Formal Caregivers²	Informal Caregivers³	Individuals with HD⁴	Formal Caregivers⁵	Informal Caregivers⁶	Individuals with HD⁴	Formal Caregivers⁵	Informal Caregivers⁶
Evaluation of current medication	20.6	30.8	24.7	9.1	0	18.0	41.9	42.4	35.8	28.1	0	14.8
Therapeutic exercises/training or physiotherapy	73.9	68.5	64.5	9.0	11.6	22.1	56.6	45.3	49.4	29.4	4.4	22.9
Assisted walking	31.2	35.0	34.2	0	11.1	23.9	44.9	34.3	39.9	0	6.2	18.2
Evaluation of assistive devices	46.6	41.0	36.8	8.1	11.1	17.1	64.3	56.5	48.3	23.2	6.2	16.7
Alarm	39.4	24.3	30.0	0	0	15.4	51.8	30.5	41.3	0	0	14.8
Evaluation of eyesight/vision	22.3	13.5	17.0	0	5.1	14.4	41.1	12.8	18.4	0	4.5	8.0
Evaluation of footwear	35.0	45.8	39.8	7.1	12.3	21.1	36.1	31.2	32.1	30.0	1.8	21.8
Evaluation of day program/activities	12.9	29.2	30.4	0	6.6	16.6	29.8	9.5	30.0	0	2.6	23.2
Supervision	16.0	29.3	28.4	7.8	0	19.4	34.6	45.3	45.1	35.2	0	20.0
Patient education	43.4	36.3	28.6	7.8	8.0	13.6	46.1	30.3	34.0	33.5	3.3	12.5
Agree with resident and/or relatives about preventive interventions	22.3	22.7	22.9	0	8.2	19.0	35.3	35.3	32.6	0	2.1	19.6
Safety adaptation of environment	14.1	21.8	27.2	0	4.7	17.8	22.2	30.6	23.8	0	2.3	16.4
Use of restraints	8.1	7.5	12.9	0	0	12.8	29.4	21.0	22.6	0	0	18.2
Injury prevention	7.1	6.5	14.0	0	5.3	14.8	29.8	4.0	17.2	0	5.4	15.4
Other interventions	11.7	5.2	11.7	9.3	0	19.2	34.7	6.1	12.9	28.1	0	16.4
Patient refuses all fall interventions	9.0	0	14.8	n.a.	n.a.	n.a.	33.7	0	18.5	n.a.	n.a.	n.a.
No interventions	11.2	22.1	28.7	83.0	75.9	57.4	24.2	19.5	19.2	69.3	89.9	59.9

Percentages are based on pooled imputed data. HD, Huntington’s disease. Data were unavailable for the following number of participants: ¹12 Individuals with HD; ²10 formal caregivers; ³21 informal caregivers; ⁴47 Individuals with HD; ⁵4 formal caregivers; ⁶21 informal caregivers.

Then participants were asked what measure was not applied currently, but they would like to see applied According to the individuals with HD in the independent group, the ‘evaluation of current medication’ (9.1%) was most frequently not applied in order to prevent falls. Formal caregivers most often would like to see applied ‘Evaluation of footwear’ (12.3%) and informal caregivers ‘Assisted walking’ (23.9%). Individuals with HD in the dependent group most frequently quoted ‘Supervision’ (35.2%) as the measure they would like to see applied, while the formal caregivers reported ‘Assisted walking’ and ‘Evaluation of assistive devices’ (both 6.2%) and the informal caregivers ‘Evaluation of day program’ (23.2%). In addition to the standard measures, alternative measures which the individuals with HD would like to see applied were music therapy and an anti-slip doormat. Informal caregivers recommended advice from an occupational therapist, the prevention of stress, more frequent and prolonged physiotherapy and offering more activities tailored to individuals with HD.

DISCUSSION

The results of this study confirm that the fall prevalence in individuals with HD is high and that FoF is a common problem, not only in individuals with HD but also among their (in)formal caregivers.

The fall prevalence over a 30-day period of almost 30%, found in this study is in line with the prevalence of 30.4%in a study we conducted earlier [39]. However, the present study contains data from six different care organizations, whereas the earlier study was carried out in one center. Although there is no significant difference between the independent and dependent group, the trend indicates a greater fall prevalence in the dependent group. A study in which fallers were less independent [5] supports this trend. Other studies which also show that falls are a serious concern in HD, are difficult to compare because the time frames in which the falls were reported differ [7, 8].

FoF was reported by approximately 50 percent of individuals with HD. This prevalence is much higher than earlier studies in early and mid-stage individuals with HD in which the prevalence was approximately 15%[3, 39]. The Short FES-I indicates moderate to high rates of concern about falling in individuals with HD according to cut-off points established in a study in community-dwelling elderly [40]. The Short FES-I has not previously been used in individuals with HD. Although the fall prevalence in this study was similar to our previous study of individuals with HD in a nursing home [39], the extent to which FoF is reported in this study is higher. The most likely explanation is that the previous study used the nursing staff’s interpretation to determine the patient’s FoF. Studies of expression of emotions in HD showed impaired expression of emotions on the motoric level, interrupting non-verbal communications [41] and possibly causing an underestimation of emotions. The percentage of individuals with HD who indicated FoF was also lower in another study in early and mid-stage individuals with HD [3]. The stage of the disease may thus also play a role in the amount of FoF experienced.

In the current study, most FoF was among informal caregivers, and less among formal caregivers. Although there are no studies describing the prevalence of formal and informal caregivers’ fear about individuals with HD falling, there are qualitative studies in community-dwelling individuals with Parkinson’s disease and frail elderly persons [21, 42]. A large impact is seen on informal caregivers, at the physical, psychological, and social level. Possible explanations for the highest rates of FoF related to individuals with HD’ falls in informal caregivers are described in a qualitative study on FoF among caregivers of individuals with Parkinson’s disease and of those who are frail and elderly [21, 42]. Informal caregivers are worried about these individuals hurting themselves and the consequences of a fall on the individual’s quality of life [21]. Informal caregivers feel unprepared for their role and need support from health professionals about managing falls [21], support which may not yet be sufficient. In addition, individuals with HD in this study refuse to comply with or misunderstand measures. A possible reason why formal caregivers experience less FoF is that they are more continuously present in the care environment of the individuals with HD and can therefore better monitor the risks and intervene if necessary.

Since the focus of this study was mainly on FoF, we examined the risk factors for FoF. Anticipatory awareness and gender were predictors of FoF in independent individuals with HD. The results in our study demonstrate the importance of recognizing the different levels of awareness, in order to ensure optimal patient treatment [17]. In HD female individuals and individuals with a higher level of anticipatory awareness of fall risks are more likely to have FoF. Fear has a preventive effect, because it increases the ability to act safely [43], but fear can also increase the risk of falling [12]. An intervention to reduce FoF in older community dwelling people, ‘A matter of balance’, which focuses on both physical and environmental safety and reducing anxiety [44], addresses this discrepancy. Perhaps an intervention like this could also be applied to individuals with HD to cope with FoF. In individuals with HD with a lower level of anticipatory awareness of fall risks and individuals where a lack of fear impedes acting safely, safety adaptation of the environment and supervision by formal and informal caregivers seems to be more relevant.

‘Therapeutic exercises/training or physiotherapy’ and ‘Evaluation of assistive devices’ were the most frequently used fall preventive measures in this study. For most individuals with HD, however, a combination of measures was used. Studies on exercise interventions, rehabilitation [4] and assistive devices [9] in individuals with HD, showed a positive effect on gait and balance deficits. However, because these studies included individuals from HD centers in hospitals and not individuals in the advanced stages of the disease, more research is needed. A meta-analysis of fall prevention programs in nursing homes showed that multifactorial interventions (two or more intervention components customized to each resident’s fall risk) reduced the number of recurrent fallers [45].

The strength of this study is the fact that so many participants with this rare disorder [46] could be included. By choosing individuals with HD with moderate and advanced stages of HD we encountered a number of challenges. As cognitive performance gradually worsens during the disease [47]. we realized that not all individuals with HD could fill in the questionnaires themselves. However, although participation of cognitively impaired individuals with HD is commonly avoided [48], we aimed to reach the maximum number of individuals with HD. Therefore, we used standardized interview-based questionnaires instead of self-completion questionnaires [48]. Using this strategy we expected a large number of missing values, particularly for care dependent individuals with HD, making the results of this group less reliable. Therefore, the results offered more a rough indication of the prevalence of FoF than a concrete hard outcome. In addition, while many care-dependent individuals with HD could not answer questions themselves, data provided by the (in) formal caregivers of these individuals with HD helped to add information on FoF in HD. Thus, although care-dependent individuals with HD’ data on FoF should be interpreted with caution, this study provides initial insight into an important area of concern for this often excluded population from a variety of perspectives.

CONCLUSIONS AND IMPLICATIONS

Fall prevalence is high in HD and FoF is common in individuals with HD and their caregivers. Gender and anticipatory awareness are risk factors for FoF in independent individuals with HD. A combination of fall preventive measures is used in most individuals with HD. In addition to the use of individual multifactorial fall prevention strategies, it is important also to support caregivers in coping with falls. Our findings emphasize the importance of recognizing lack of awareness. Other possible risk factors and interventions may be subjects for future research.

Footnotes

ACKNOWLEDGMENTS

The authors gratefully acknowledge M. Ekkel of Amsterdam UMC and all involved staff, residents, and informal caregivers of Atlant, Topaz, Amstelring, Land van Horne, Archipel and Mijzo care organizations for their help in this study.

This research did not receive any funding from agencies in the public, commercial or not-for-profit sectors.

CONFLICT OF INTEREST

The authors have no conflict of interest to report.

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