Original Article

American Journal of Hospice

& Palliative Medicine®
Advance Care Plan and Factors Related 2020, Vol. 37(1) 46-51
ª The Author(s) 2019
Article reuse guidelines:
to Disease Progression in Patients sagepub.com/journals-permissions
DOI: 10.1177/1049909119850797
With Spinocerebellar Ataxia Type 1: journals.sagepub.com/home/ajh

A Cross-Sectional Study in Thailand

Patama Gomutbutra, MD1 , and Megan Brandeland, MD2

Abstract
Background: Spinocerebellar ataxia type 1 (SCA1) is an autosomal dominant progressive neurodegenerative disease. Few
studies have been conducted regarding advance care planning in this population. Objective: This study explores advance care
planning preferences of patients with SCA1 and their association with disease progression and quality of life. Methods: The study
examined 12 Thai patients with SCA1 from 2 families living in Thailand. The advance care plan followed a Gold Standards
Framework. The 12 patients were interviewed and recorded in video. The research team evaluated neurocognitive functions
as measured by the following tests; Scale for the Assessment and Rating of Ataxia (SARA), Berg Balance Score, Mini-Mental Status
Examination, and Digit Span and Category Fluency. The quality of life was measured by a Short-Form Health Survey-36 (SF-36).
Results: Seven of 12 patients with SCA1 rated communication ability as most important for their quality of life. Patients identified
becoming a burden on their family members and ventilator dependence as the most undesirable situations. Half of the patients
preferred a hospital as their last place of care. Comparing patients prefer hospital to home has significantly high median SARA (23
vs 11.5; P ¼ .03) and low SF-36 (41.4 vs 72.4; P ¼ .02). Conclusions: Those patients preferring a hospital for end-of-life care
exhibited more physical disability and lower quality of life than those who preferred home care. Making assisted living health-care
services in the home more readily available and affordable may alleviate concerns of patients facing more severe physical
challenges.

Keywords
spinocerebellar ataxia, advance care planning, neurocognitive, quality of life

Background As with many progressive neurodegenerative diseases, there

The spinocerebellar ataxias (SCA) encompass a diverse group
of disorders characterized by hereditary progressive cerebellar
dysfunction. Researchers have identified a significant number
of subtypes of SCA; currently, more than 40 subtypes are
known.1 To distinguish between the subtypes, the nomencla-
ture uses a numbering system (SCA1, SCA2, etc), according to
the order in which the mutated genes were discovered. The
most common subtypes in Thailand, in order of frequency, are
SCA3, SCA1, SCA2, and SCA6, which corresponds to world-
wide epidemiological distributions.2 Among these, SCA1 is the
most rapidly progressive form of SCA with the poorest prog-
nosis. In one study, the 10-year survival rate for SCA1 was
shown to be 57%, compared to 87% for SCA6.3 The average
age of symptom onset of SCA1 is in the 40s but following
generations with more expanded CAG repeats would present
an earlier age of onset. Gait ataxia and spasticity are usually the
earliest symptoms, followed by brain stem manifestations such
as dysphagia, dysarthria, and cognitive deficits at a later stage.4
is no specific curative treatment available. The patient will
inevitably experience physical dependence and cognitive
impairment; therefore, patients must create an advance care
plan as early as possible. Evidence suggests that such advanced
care planning can both reduce cost of care and provide
improved quality of life for patients. In one study, patients with
the progressive neuromuscular disease amyotrophic lateral
1
Department of Family Medicine, Palliative Care Unit and The Northern
Neuroscience Center, Faculty of Medicine, Chiang Mai University, Chiang
Mai, Thailand
2
Department of Internal Medicine, The Global Health Pathway, The Minnesota
University Medical School, Minneapolis, MN, USA
Corresponding Author:
Patama Gomutbutra, MD, Department of Family Medicine, Palliative Care Unit
and The Northern Neuroscience Center, Faculty of Medicine, Chiang Mai
University, Chiang Mai 50200, Thailand.
Email: patthama.g@cmu.ac.th
Gomutbutra and Brandeland
sclerosis (ALS) received advanced care planning and were able
to make appropriate choices for home-based, long-term medi-
cal ventilator care, resulting in higher quality of life scores, and
reducing the costs associated with hospital stays by approxi-
mately one-third.5 Another study identified 3 main factors that
influenced care decision-making of patients with ALS as the
health-care service structure, the interactional experience of
patients with health-care providers, and patient personal values
and perceptions of control.6 A panel of ataxia specialist neu-
rologists and members of the patient support group Ataxia UK
recently produced health-care professional guidelines recom-
mending palliative care service for all patients with SCA.7
Studies of advance care planning in progressive ataxia are
still sorely lacking. The reason beside rarity of disease is pro-
gressive ataxia patient usually has barrier to get education
hence low socioeconomic status. The disparity research found
low socioeconomic status related to low rate of advance care
planning.8 In order to make more informed and better deci-
sions, health-care professionals, caretakers, and patients need
a deeper understanding of advanced care planning in a local
context, to include knowing how functional decline will influ-
ence decisions for prioritizing health-care services to meet
patients’ needs. Therefore, this study explored advance care
plan preferences and their association with neurological func-
tion from a group of SCA1 patients living in a rural Thai
community.
Methods
Study Design and Population
This was a cross-sectional study of 12 Thai patients with SCA
living in Dok Khamtai district, Phayao province, a rural area of
Northern Thailand. Baseline data were collected in April 2014
in patients’ homes and a community health center in Phayao
province, Northern Thailand. Patients ranged in age from 28 to
77 years and exhibited different degrees of ataxia (some were
able to walk unassisted, and others relied on a walker or wheel-
chair). Two index cases were found to have SCA1 by genetic
testing, while the remaining patients were diagnosed with SCA
based on clinical findings and family history. The study was
approved by the Chiang Mai University Research Ethics Com-
mittee (REC255702126). Written informed consent was
obtained from each patient prior to starting the study.
Advance Care Planning
Literature review regarding advance care planning suggests
that an approach focused on informal communication is more
acceptable for non-Western patients than an approach relying
on formal documentation.9 The Gold Standard Framework is a
simple conceptual framework used in one-third of primary care
settings in United Kingdom and validated in various patient
populations including those with dementia.10,11 We applied 3
interview dialogues from the Gold Standard Framework12 to
develop open-ended questions, modifying question 3 by asking
47
respondents to make a choice between home or hospital end-of-
life care. The questions were administered in Thai language,
with the English translation as follows.
1. What elements of care are important to you and what
would you like to happen in future?
2. What would you wish not to happen?
3. In which one of the following do you prefer to be taken
care in the last days of life—hospital or home?
4. Who would speak for you if you are in an uncommunic-
able status?
We recorded patients’ responses in video for reference,
combining similar responses into subgroupings for each
question.
Neurological Assessment
The Scale for the Assessment and Rating of Ataxia (SARA)
was used to evaluate the severity of disability in ataxia patients.
The SARA is well validated for this purpose and includes 8
items related to gait, stance, sitting, speech, finger-chase test,
nose-finger test, fast alternating movements, and heel–shin test.
The SARA scores range from 0 to 40. A score of 0 indicates no
ataxia while higher scores indicate more severe ataxia.13 The
Berg Balance Scale (BBS) is a functional balance assessment
consisting of 14 items, each of which is scored on a scale of 0 to
4. A score of 0 indicates inability to complete the task while a
score of 4 indicates ability to complete the task without assis-
tance or error. The maximum score of 56 points would indicate
no impairment. There is a study suggesting that the BBS is
acceptable property and feasible in people with cerebellar
ataxia.14 The Halstead Finger Tapping Test (FTT) is a more
general measure of neurologic impairment that assesses motor
speed and lateralized coordination. It has been shown to corre-
late with severity of disease and functional outcomes in a vari-
ety of neurologic diseases including stroke, Alzheimer disease,
and cerebellar ataxia. This test evaluates the number of times a
participant can tap their fingers as fast as possible for 10 sec-
onds. The test consists of 5 tapping trials (all trials must be
within 5 points of each other). The normal value in general
population varied by age and sex, with the average for normal
participants around 50 to 55 taps per 10 seconds.15 The Mini-
Mental Status Examination (MMSE) was primarily used to
assess memory function. The tests for executive functions
included the Digit Span Forward Test to evaluate working
memory. The ability to accurately repeat 5 to 7 digits is con-
sidered the “normal” range for those with unimpaired cognitive
ability. The Trail Making Test Form B was administered for
mental flexibility, while the Stroop Interference Test was used
as a measure for inhibitory control. Finally, a verbal fluency
test using the semantic category of “animals” was used to
assess word-generation ability. We chose this semantic cate-
gory over a phonemic test or other categories due to its simpli-
city, making it less affected by education levels or
socioeconomic factors. The test considers that a noncognitive
48 American Journal of Hospice & Palliative Medicine® 37(1)

Table 1. Advance Care Plan Preference.

The Gold Standard Framework Open Number of Responders Who Rated This the
Questions The 3 Most Common Prioritized Issues Highest Priority (From 12 Responders)

What elements of care are important to you and  Able to communicate and maintain 7
what would you like to happen in future? social interaction as long as possible
 Able to walk as long as possible 2
 Live with their family as long as possible 2
 Able to eat by mouth 1
What would you wish not to happen? Is there  Being a burden on their family caregiver 9
anything that you worry about in the future?  Being dependent on a ventilator, 3
including having a tracheostomy
Where do you prefer to be taken care in the last  Hospital 6
days of life—hospital or home?  Home 6
Who would speak for you if you are in an  The family member shares the end-of- 12
uncommunicable status life care decision with health-care
providers

impaired person should be able to name more than 10 animals
within 1 minute.16 These neurocognitive tests were conducted
in patient’s homes by the principle investigator with assistance
from hospital staff.
Quality of Life
Quality of life was assessed by local community health workers
using the Thai version of the Short-Form Health Survey-36
(SF-36). The SF-36 consists of 36 items measuring 8 dimen-
sions of functioning. As each question carries equal weight, all
scores are transferred to a common scale in order to make
comparisons possible. The scaled scores, which are the
weighted sum of all questions concerning that health concept,
are consolidated into each of the 8 dimensions.17 The scale for
each dimension measures between 0 and 100, with 100 repre-
senting the highest level of functioning possible.
Statistical Analysis
The descriptive statistics were summarized as means and stan-
dard deviation or median and range for continuous variables,
and as percentages for categorical variables. A t test was used
to assess for significant differences among continuous vari-
ables for the 2 groups described (patients who survived through
the follow-up period vs patients who died). The Mann-Whitney
U test (ranked-sum test) was used for variables with significant
deviation from a normal distribution, while the w2 test was used
to analyze categorical variables. A P value of less than .05 was
accepted as statistically significant for the univariate analysis.
Due to low sample size (<20), we were not able to conduct
multivariate logistic regression. Data analysis was performed
using STATA version 11 (STATA Inc, Redmond, California).
Results
Patient Characteristics
Of the 12 patients in this study, 9 were women and 3 were men.
Patients’ average age was 49, with a standard deviation of 12.8,
and the range from 28 to 77 years-old. The majority of patients
had no higher than primary school education.
The Advance Care Plan Preference
Responding to the question of what were considered to be the
most important factors for their quality of life, 7 of 12 SCA1
patients rated communication ability at the top. Other factors
such as mobility, living with family, and eating ability rated
highly as most important to life quality. Patients identified
becoming a burden on their family members and ventilator
dependence as the most undesirable situations. Half of the
patients preferred a hospital as their last place of care. The
detailed response results are displayed in Table 1.
Neurological Test
We used SARA, BBS to measure severity of ataxia, and FTT to
measure fine motor ability in all 12 patients. The mean score
was 16.3 standard deviation (SD) 7.85, the median score was
18.5 (range: 2-25) of a maximum score of 40. In patients who
preferred for end of life to occur at the hospital, the median
score was 23. For patients who preferred for end of life to occur
at home, the median score was 11.5. Given the nonstandard
distribution of the results, we compared these 2 groups using
the Mann-Whitney U test and found the hospital group had
worse ataxia (P ¼ .03). The median BBS was 22 (range: 0-
53) of a maximum score of 56. Berg Balance Score in who
preferred for end of life to occur at the hospital the median
score was 6 while who preferred for end of life to occur at home
was 44 (P ¼ .01). The median of FTT score was 15.7, FTT in
who preferred for end of life to occur at the hospital the median
score was 13.8 while who preferred for end of life to occur at
home was 22.4 (P ¼ .01).
Mini-Mental Status Examination, digit span, and categorical
verbal fluency were used to evaluate cognitive function in all
12 patients. The median MMSE was 22.5 (range: 14-30) of
maximum score of 30. The median digit span was 6 (range:
5-7). The median categorical fluency was 15 (range: 8-27).
Gomutbutra and Brandeland 49

Table 2. Comparison of Characteristics for Patients With SCA1 Who Preferred Hospital as the Last Care Setting Compared to Patients Who
Preferred Home as the Last Care Setting.

Patient Characteristics Hospital (N ¼ 6) Home (N ¼ 6) P Value

Age, mean (95% CI) 41.8 (31.6-52.0) 54.3 (25.3-62.7) .08

Sex: Female (n %) 4 (67%) 5 (83%) .75
Years of education, median (IQR) 3.5 (0-8) 5 (0-7) .80a
SF-36 quality of life, mean (95% CI) 41.4 (24.0-58.9) 72.4 (51.1-93.71) .02
Physical function
SARA (all items), median (IQR) 23 (21-25) 11.5 (2-16) .03a
SARA (speech), median (IQR) 3 (1-5) 1 (0-2) .02a
BBS, median (IQR) 6 (0-22) 6 (22-53) .02a
FTT, median (IQR) 13.8 (12.2-15.5) 22.4 (13.5-31.4) .01
Cognitive function
MMSE, median (IQR) 20 (14-25) 20 (14-25) .29a
Digit span, median (IQR) 11.5 (11-15) 12.0 (11-18) .50a
Category fluency, median (IQR) 11.5 (8-19) 17.5 (11-27) .07a
Abbreviations: BBS, Berg Balance Score; CI, confidence interval; FTT, Finger Tapping Test; IQR, interquartile range; MMSE, Mini-Mental Status Examination; SARA,
Scale for the Assessment and Rating of Ataxia; SCA1, Spinocerebellar ataxia type 1; SF-36, Short-Form Health Survey-36.
a
Nonparametric statistic using Mann-Whitney rank sum test.

There is no significant difference of these cognitive function
between who preferred for end of life to occur at the hospital
and who preferred for end of life to occur at home.
Quality of Life
The mean quality of life as assessed by the SF-36 was 50.07
(range: 20.72-92.73, SD: 24.04) of a maximum score of 100.
The average of each dimension on the SF-36, from low to high,
was physical functioning 30.45, role-physical 34.09, general
health perceptions 37.42, role-emotion 54.54, vitality 64.09,
mental health 73.09, social functioning 75, and bodily pain
78.06. Those preferring hospital care had a lower overall SF-
36 quality of life rating of 41.4 versus a score of 72.4 (P ¼ .02)
for those preferring home end-of-life care.
Table 2 compares characteristics between patients who pre-
fer hospital versus home as the last place of care.
Discussion
The objective of this study was to explore advance care plan
and factors influencing the end-of-life care decisions in patients
with chronic progressive ataxia. We found the Gold Standard
Framework advance care plan discussion with 3 simple ques-
tions was practical to use with our patients who had low edu-
cation and mild cognitive impairment. The majority of
participants identified communication ability as having the
highest importance for their quality of life. Patients were most
concerned about becoming dependent and posing a burden on
their family. Half of patients who exhibited more severe phys-
ical function impairments chose end-of-life care in a hospital.
All of them preferred shared decision-making between physi-
cians and their family after they lost decision-making capacity.
Western culture patients of progressive ataxia also placed a
high value on social engagement.17 Although data are limited,
this may indicate that social engagement is a cross-cultural
need. From a neuroscience perspective, this identified need
might be explained by the fact that executive functions, such
as social cognition, are the least impaired functions in SCA1.18
Our own results from Digit Span and Categorical Fluency tests
showed that executive cognition functions were pretty well
preserved in our participants. We found it surprising that pro-
gressive ataxia patients with increased disease progression
tended to choose end-of-life care at a hospital instead of spend-
ing their time with family at home. The decision of end-of-life
care seemed to be a push and pull between what they wished (to
be engaged with others) and what they feared (becoming
dependent and a burden to family caregivers). With higher
decline in physical functioning and lower quality of life, the
“fear” factor appeared to outweigh the “wish” factor. There are
2 hypothetical potential mechanisms to explain this
phenomenon.
1. Depressive mood: Unfortunately, we did not perform
any standard test specific to depression. However, we
might make inferences from quality of life scores on the
SF-36, especially physical function and social function
subscale scores. Previous studies have shown a close
association of these SF-36 subscale scores to scores
on tests for depression.19 The lower SF-36 scores asso-
ciated with our patients who chose hospital care may
thus indicate that they struggle with depression.
2. Increased attachment to formal health-care service:
When patients have more disease progression, they visit
the hospital for either outpatient or inpatient services
more frequently. More frequent contact increases famil-
iarity, influencing the patient’s attachment to a hospital
care. Moreover, the Thai health-care system provides
free medical care in government hospitals for handi-
capped patients, which may strengthen patients’ resolve
to choose hospitalized end-of-life care. Meanwhile,
home health-care services in Thailand are overloaded
50
with work such as noncommunicable disease health
promotion and with cases of palliative care for patients
with cancer. Despite very attentive attitudes of local
home health-care teams, limited resources make it
extremely difficult to regularly visit and meet the needs
of patients, especially in rural areas where meeting is
particularly arduous. Without experiencing dedicated,
frequent home health-care service, patients may not
have the opportunity to become familiar with and
develop a preference for it. An ALS study identified
the importance of patient–health-care provider interac-
tion in influencing decision-making,6 so fewer oppor-
tunities for patients to interact with providers in the
home may negatively affect decisions for home versus
hospital care. In addition, evidence suggests that hospi-
tal visits are reduced when community-based palliative
care is practiced, where caregivers provide companion-
ship to the patient especially in duration longer than
30 days.20
This study had a number of limitations. First, not all of the
patients in this study were confirmed by genetic testing to have
SCA. Given that SCA1 is an autosomal dominant condition
with unique features and that genetic testing is expensive and
not easily accessible in rural Thailand, we decided that a clin-
ical diagnosis in the setting of a strong family history of the
disease should be acceptable. Second, the data were collected
only once and therefore we did not have information about the
clinical trajectory of these patients over time. Most impor-
tantly, this study was conducted on a small sample size of
SCA1 patients living in the same rural community in Thailand.
They are relatives and live nearby each other, which may pro-
mote conforming responses about advance care preferences.
These issues limit the generalizability of our results. However,
the results from this small scale study provide awareness about
the benefits of advance care planning in progressive ataxia.
Such planning not only offers opportunities for patients to
communicate their wishes to witnesses who can convey those
wishes with deteriorating patient capacity but also gives insight
to health-care providers about the need for continuity of care.
Providing increased availability of accompanied home health
care in this patient group might influence their decisions to
choose end-of-life care at home rather than hospital when the
patients advance in their disability.
Conclusions
Based on our sample, social engagement ability is the major
concern for Thai patients with SCA1. Those patients who pre-
ferred a hospital for end-of-life care exhibited more physical
disability and reported lower quality of life than those who
preferred home care. That preference for higher cost hospital
care may be influenced by depressed moods (as inferred from
their quality of life scores) and increased attachment to hospital
care due to familiarity. Increasing the amount and frequency of
accompanied health-care services for these patients in their
American Journal of Hospice & Palliative Medicine® 37(1)
homes could lead to improved decision-making in advanced
care planning. Due to the limitations of a small sample size and
to cultural factors, we encourage more studies, with larger
populations, in varied cultural settings to enhance the general-
izability of our conclusions.
Declaration of Conflicting Interests
The authors declared no potential conflicts of interest with respect to
the research, authorship, and/or publication of this article.
Funding
The authors disclosed receipt of the following financial support for
the research, authorship, and/or publication of this article: This work
was supported by Faculty of Medicine Research Fund, Chiang Mai
Univeristy, Chiang Mai, Thailand. FUND-25571029-03317.
ORCID iD
Patama Gomutbutra https://orcid.org/0000-0002-7360-8979
Supplemental Material
Supplemental material for this article is available online.
References
1. Coarelli G, Brice A, Durr A. Recent advances in understanding
dominant spinocerebellar ataxias from clinical and genetic points
of view. F1000Res. 2018;7:1781.
2. Sura T, Eu-Ahsunthornwattana J, Youngcharoen S, et al. Frequen-
cies of spinocerebellar ataxia subtypes in Thailand: window to the
population history? J Hum Genet. 2009;54(5):284-288.
3. Diallo A, Jacobi H, Cook A, et al. Survival in patients with spi-
nocerebellar ataxia types 1, 2, 3, and 6 (EUROSCA): a longitu-
dinal cohort study. Lancet Neurol. 2018;17(4):327-334.
4. Boonkongchuen P, Pongpakdee S, Jindahra P, et al. Clinical anal-
ysis of adult-onset spinocerebellar ataxias in Thailand. BMC Neu-
rol. 2014;14:75.
5. Moss AH, Oppenheimer EA, Casey P, et al. Patients with amyo-
trophic lateral sclerosis receiving long-term mechanical ventila-
tion. Chest. 1996;110(1):249-255.
6. Hogden A, Greenfield D, Nugus P, Kiernan MC. What influences
patient decision-making in amyotrophic lateral sclerosis multi-
disciplinary care? A study of patient perspectives. Patient Prefer
Adherence. 2012;6:829-838.
7. de Silva R, Greenfield J, Cook A, et al. Guidelines on the diag-
nosis and management of the progressive ataxias. Orphanet J
Rare Dis. 2019;14(1). https://ojrd.biomedcentral.com/articles/
10.1186/s13023-019-1013-9. Accessed April 18, 2019.
8. Barwise A, Juhn YJ, Wi C-I, et al. An individual housing-based socio-
economic status measure predicts advance care planning and nursing
home utilization. Am J Hosp Palliat Care. 2019;36(5):362-369.
9. McDermott E, Selman LE. Cultural factors influencing advance
care planning in progressive, incurable disease: a systematic
review with narrative synthesis. J Pain Symptom Manage. 2018;
56(4):613-636.
10. Murray SA, Sheikh A, Thomas K. Advance care planning in
primary care. BMJ. 2006;333(7574):868-869.
Gomutbutra and Brandeland
11. Shaw K, Clifford C, Thomas K, Meehan H. Review: improv-
ing end-of-life care: a critical review of the gold standards
framework in primary care. Palliat Med. 2010;24(3):
317-329.
12. Hamilton IJ. Advance care planning in general practice: promot-
ing patient autonomy and shared decision making. Br J Gen
Pract. 2017;67(656):104-105.
13. Yabe I, Matsushima M, Soma H, Basri R, Sasaki H. Usefulness of
the Scale for Assessment and Rating of Ataxia (SARA). J Neurol
Sci. 2008;266(1-2):164-166.
14. Winser SJ, Smith CM, Hale LA, Claydon LS, Whitney SL. Clin-
ical assessment of balance using BBS and SARAbal in cerebellar
ataxia: synthesis of findings of a psychometric property analysis.
Hong Kong Physiother J. 2018;38(1):53-61.
15. Ruff RM, Parker SB. Gender- and age-specific changes in motor
speed and eye-hand coordination in adults: normative values for
51
the finger tapping and grooved pegboard tests. Percept Mot Skills.
1993;76(3_suppl):1219-1230.
16. Strub RL, Black FW. The Mental Status Examination in Neurol-
ogy. 3rd ed. Philadelphia, PA: Davis; 1993:244.
17. Lim L, Seubsman S, Sleigh A. Thai SF-36 health survey: tests of
data quality, scaling assumptions, reliability and validity in healthy
men and women. Health Qual Life Outcomes. 2008;6(1):52.
18. Klinke I, Minnerop M, Schmitz-Hübsch T, et al. Neuropsycholo-
gical features of patients with spinocerebellar ataxia (SCA) types
1, 2, 3, and 6. Cerebellum. 2010;9(3):433-442.
19. Beusterien KM, Steinwald B, Ware JE. Usefulness of the SF-36
health survey in measuring health outcomes in the depressed
elderly. J Geriatr Psychiatry Neurol. 1996;9(1):13-21.
20. Nagaviroj K, Anothaisintawee T. Study of the association between
multidisciplinary home care and home death among Thai palliative
care patients. Am J Hosp Palliat Care. 2017;34(5):397-403.