Living Will and Advance Care Planning in Patients With Amyotrophic Lateral Sclerosis Admitted to Specialistic Home Palliative Care

Abstract

Objectives: In Italy a recent law was approved for providing patients’ wishes regarding end of life issues, commonly referred internationally to as “living wills”, (Dichiarazione anticipata di trattamento, DAT). Regardless of this official document, advance care planning (ACP) is often used in a palliative care setting to share the treatments to start, to continue, to withdraw, thus preventing the stress on an acute decision. The aim of this study was to assess DAT and ACP in patients with amyotropic lateral sclerosis admitted to home palliative care. Methods: Patients consecutively admitted to speciliazed home palliative care were prospectively assessed. The presence of DAT or ACP was recorded. Results: Sixty-eight patients were enrolled in the period taken into consideration. No patient had drown up DAT, and only one patient provided his ACP prior to home palliative care admission. Along the course of home palliative care care assistance, 30.9% of patients provided their ACP. Discussion: In Italy DAT resulted scarcely widespread, despite an existing law, as no patient officially provided their indication on end of life issues. In addition, ACP was given only after starting specialized home palliative care in less than 1/3 of patients. Home palliative care seems to be a fundamental resource for improving communication and soliciting expression of patients’ wishes regarding end of life issues.

Full text

Alzheimer’s/Dementia and Neuromuscular Disorders
American Journal of Hospice
& Palliative Medicine
®
2025, Vol. 0(0) 1–5
© The Author(s) 2025
Article reuse guidelines:
sagepub.com/journals-permissions
DOI: 10.1177/10499091241312906
journals.sagepub.com/home/ajh
Living Will and Advance Care Planning in
Patients With Amyotrophic Lateral Sclerosis
Admitted to Specialistic Home Palliative Care
Sebastiano Mercadante, MD
1,2
, Pietro Petronaci, RN
2
, and Alessio Lo Cascio, RN
1
Abstract
Objectives: In Italy a recent law was approved for providing patients’wishes regarding end of life issues, commonly referred
internationally to as “living wills”, (Dichiarazione anticipata di trattamento, DAT). Regardless of this official document, advance
care planning (ACP) is often used in a palliative care setting to share the treatments to start, to continue, to withdraw, thus
preventing the stress on an acute decision. The aim of this study was to assess DAT and ACP in patients with amyotropic lateral
sclerosis admitted to home palliative care. Methods: Patients consecutively admitted to speciliazed home palliative care were
prospectively assessed. The presence of DAT or ACP was recorded. Results: Sixty-eight patients were enrolled in the period
taken into consideration. No patient had drown up DAT, and only one patient provided his ACP prior to home palliative care
admission. Along the course of home palliative care care assistance, 30.9% of patients provided their ACP. Discussion: In Italy
DAT resulted scarcely widespread, despite an existing law, as no patient officially provided their indication on end of life issues.
In addition, ACP was given only after starting specialized home palliative care in less than 1/3 of patients. Home palliative care
seems to be a fundamental resource for improving communication and soliciting expression of patients’wishes regarding end of
life issues.
Keywords
amyotrophic lateral sclerosis, living will, advance care planning, home care, palliative care
Introduction
Amyotrophic lateral sclerosis (ALS) is an incurable neuro-
degenerative disease associated with a life expectancy of 2-
5 years after diagnosis.
1
Patients experience paralysis, dys-
phagia, respiratory failure, and loss of communicative func-
tion, making the wishes of patients on difficult decisions at end
of life of paramount importance.
2
This disease typically
represents the experience of loss, not only of physical function
but also of mental, social, and emotional well-being so
common in incurable neurological disease.
3
There has been an increasing awareness of the inadequacy
of end of life care and of the poor knowledge of wishes of
patients with ALS about their medical treatment at a time
when they lose the capacity to make decisions.
4,5
This results
in patients being cared for in a way they would not have
chosen,
6
and many patients at the end of life may receive care
that is inappropriate or futile or simply unwanted. A recent
retrospective study showed that early specialist palliative care
input and advance care planning (ACP) may increase the
likelihood of death outside of hospital for persons with motor
neuron disease.
7
Internationally, ACP encompasses a range of formalized
documents which vary in terminology, content, and legal
standing.
8
Advance directive documents are often synony-
mously called a “living will”in the English literature.
9
In Italy,
the intention to provide or negate consensus for some end of
life issues, could correspond to the term “living wills”.A
recent law was approved by the Parliament in 2017, and came
into force in January 2018.
10
To summarize the main state-
ments of the law, in anticipation of a possible future inability to
self-determine and after having acquired adequate medical
information on the consequences of one’s choices, each person
1
Main Regional Center for Pain Relief and Supportive/Palliative Care, La
Maddalena Cancer Center, Palermo, Italy
2
SAMOT Regional Home Care Program, Palermo, Italy
Corresponding Author:
Sebastiano Mercadante, MD, Main Regional Center for Pain Relief and
Supportive/Palliative Care, La Maddalena Cancer Center, Via San Lorenzo
312, Palermo 90146, Italy.
Email: terapiadeldolore@lamaddalenanet.it

can express their wishes (Dichiarazione anticipata di tratta-
mento, DAT) regarding health treatments, as well as consent or
refusal on diagnostic tests, therapeutic choices, and individual
health treatments. DAT is renewable, modifiable and revo-
cable at any time. The drafting of the DAT can take place in
different forms: by the notary or a civil office (both with a
public deed and with a private deed in which the person
independently writes their wishes and has the signatures
authenticated by the notary). In both cases the notary keeps the
original at the civil registry office of the municipality of
residence (with private deed) which takes care of the anno-
tation in a special register, In the absence of official data from
the Minister of Health, an association defending human rights
(Associazione Luca Coscioni) has announced that approxi-
mately 0.5% of adults have adhered to this law. These figures,
although increasing, remain quite low.
ACP does not correspond to DATin Italy. This norm in Italy is
not an official document, like DAT, but it is just an agreement
between the team, the patient or his delegate. This term is often
used in a palliative care setting, in patients with a limited
prognosis, either at home or in hospice, to share the treatments to
start, to continue, to withdraw, thus preventing the stress in
making an acute decision.
11
ACP and the importance of im-
proving end of life care are both endorsed by professional bodies
and medical associations, and supported by legislation in Italy, as
well in many other countries.
12,13
ACP and end of life discus-
sions reduce stress, anxiety, and depression in surviving rela-
tives.
14
Inarandomizedcontrolledtrialperformedinafrail
population of elderly patients,ACP improved end of life care and
patient and family satisfaction, and reduced stress, anxiety, and
depression in surviving relatives.
15
Barriers to DAT and ACP include social and personal
taboos about discussing the dying process, avoidance by
medical professionals of responsibility for initiating, coordi-
nating, and documenting discussions about ACP, lack of a
trained staff with the time, competence, and confidence to
discuss with patients, lack of standardised procedures for
recording and retrieving ACP documents across multiple care
settings, as well as legal and ethical concerns about the validity
of such documents.
4,16-20
Most patients with ALS live at home with support of a
palliative care team, with escalating complexity of care over
the trajectory of the disease to mitigate negative physical,
social, and emotional outcomes.
21
This setting could be op-
timal for improving communication and soliciting expression
of patients’wishes regarding end of life issues.
22,23
Studies
reported in a systematic review and meta-analysis that data
was heterogeneous and international differences were de-
tected. Living will, that should correspond to DAT in Italy, was
signed signed in 60.4% of records. The number of DAT
decreased in the last decade.
8
The aim of this study was to assess DAT and ACP rate in
patients with ALS who were admitted to a specialized home
care team in a country like Italy where a recent law promoted
DAT, as well ACP.
Methods
A cohort of a consecutive sample of patients with a diagnosis
of ALS admitted to specialized home care was assessed from
January to September 2024. The characteristics of the spe-
cialized home care team have been described elsewhere.
24
At
admission, the Amyotrophic Lateral Sclerosis Functional
Rating Scale (ALSFRS-R), awareness of disease, fronto-
temporal dementia (FTD), and the use of vital supports
(modalities of artificial nutrition and ventilation), were
recorded. The ALSFRS is a validated rating instrument for
monitoring the progression of disability in patients with
amyotrophic lateral sclerosis (ALS). The Revised ALSFRS
(ALSFRS-R) consists of items of functional rating scale which
include speech, salivation, swallowing, handwriting, cutting
food and handling utensils, dressing and hygiene, turning in
bed and adjusting bed clothes, walking, climbing stairs, and
incorporates additional assessments of dyspnea, orthopnea,
and the need for ventilatory support. Each item is scored from
0 to 4 where four is given if person is normal, and 0 for poor
performance. With 48 points, more than 90% of patients are
expected to have more than 9-month survival. ALSFRS-R
retains the properties of the original scale and shows strong
internal consistency and construct validity.
25
Awareness of
diagnosis and prognosis was deducted by home care team
through discussion, not directly enquiring about that.The
presence of DAT, according to the definition given above, was
recorded. As routine, at admission to specialized home care,
patients were encouraged to share a ACP for vital support,
including artificial nutrition and ventilation, or end of life
choices. This process is gently introduced at different times of
home care assistance when physicians feel that this discussion
would been appreciated, according to the cultural, social, and
religious aspects of each patient and caregiver, respecting their
sensitivity in discussing such issues. The use of palliative
sedation, that is the administration of sedative agents, even-
tually combined with opioids in presence of dyspnea, to lower
the level of consciousness and suffering, was recorded. Data
were collected until September 2024. Informed consent was
obtained. The study was approved by the local ethical com-
mitte of the province of Palermo. Identifying details were
omitted. The study was conducted according to STROBE
Statement of cohort studies
Results
Sixty-eight patients started home palliative care and were
enrolled in the period taken into consideration. Prior to home
palliative care admission, at initial assessment, no patient had
drown up DAT, and only one patient provided his ACP during
a hospital admission, At time of home care admission
40 patients (58.8%) did not have any vital support, and
22 patients (13.2%) had ventilation by tracheostomy and PEG
(percutaneous endoscopic gastrostomy).
2American Journal of Hospice & Palliative Medicine
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Along the course of home palliative care care assistance,
patients were encouraged to provide ACP after admisssion to
the home care program, taking into account the sensitivity of
patients and caregivers in accepting such a discussion: twenty-
one patients (30.9%) provided ACP. Specifically, 15 patients
agreed or refused tracheostomy: 15 (71.4%) and 6 (28.6%)
cases, respectively. Ten (50%) and ten (50%) patients agreed
or refused PEG respectively, and one patient did not provide
any indication about PEG. All patients who provided ACP
agreed to be sedated in case of suffering. In some cases,
delegates, particularly for patients with FTD, were involved in
end of life choices (Table 1). The remaining patients did not
afford the discussion within the study period.
Thirteen patients (19.1%) died during the period study,
almost at home. Of these, six patients had provided their ACP.
In three patients ACP was respected (no tracheostomy, no
PEG, palliative sedation, home death). Three patients died
without sedation, because they died unexpectedly. Of those
without ACP, two died in hospital on emergency. For the other
five patients who died at home, delegates for FTP agreed with
palliative sedation in four cases, while in one patient palliative
sedation was not performed for unexpected death.
Discussion
This study showed that, despite a recent law allowing DAT, no
patient officially provided their indication on end of life issues
before being admitted to a home care program. Moreover ACP
was almost never discussed previously, and was performed in
less than one/third of patients, only with the palliative care
team at home. Most patients were still living at study ter-
mination, thus further ACP were not recorded yet. Starting end
of life discussion in patients who have never received such an
input, is not so easy, particularly in a Mediterranean pop-
ulation with specific sociocultural values. These aspects are
taken in consideration very gently by the team to find the right
time and words to start a discussion. Of interest, all patients
with ACP agreed with palliative sedation in case of suffering.
Table 1. Characteristics of Patients and End of Life Choice.
Frequency %
Age (years, mean, SD) 68.1 ± 8.54 (range 45-88)
Gender F 24 35.3
M 44 64.7
Education Primary 15 22.1
Secondary 26 38.2
High school 21 30.9
Degree 6 8.8
ALS diagnosis Bulbar 19 27.9
Mixed 4 5.9
Spinal 45 66.2
Interval from ALS diagnosis to start of home palliative care (days, mean, SD) 955 ± 941 (15-3703)
ALSFRS-R at admission 21.7 ±20.5 (2-42)
Awareness diagnosis-prognosis None 2 2.9
Partial 35 51.5
Complete 15 22.1
Not evaluable 16 23.5
Frontotemporal dementia (FTD) No 52 76.5
Yes 16 23.5
Previous DAT/ACP ACP during hospital admission 1 1.5
DAT 0 0
Vital support at admission None 40 58.8
Ventilation-tracheo –PEG 22 32.3
NIV 3 4.4
NIV-PEG 1 1.4
PEG 2 2.9
End of life choices Not provided at study termination 22 32.4
ACP 21 30.9
None 6 8.8
Delegate on emergency 4 5.9
Delegate for FTD 11 16.2
Delegate for FTD on emergency 4 5.9
PEG: percutaneous endoscopy gastrostomy; NIV: non invasive ventilation.
Mercadante et al. 3

Only few studies reported on DAT or ACP in patients with
ALS. Different terms and meanings, other than differences in
regulation in various countries may contribute to this con-
troversial data, particulalry between Anglo-Saxon countries
and European, Latin America, or Eastern countries of which
information is laking. In a systematic review and meta-
analysis of DAT and ACP, results regarding use and con-
tent of DAT were heterogeneous and international differences
were detected. DAT were signed in 60.4% of records. The
number of DAT decreased in the last decade.
9
Discussion of
ACP, care coordination, and symptom management was
common in patients treated at a multidisciplinary ALS clinic.
These topics were discussed more frequently in patients with
lower functional status, more bulbar symptoms, or lower
quality of life, without palliative care consultation.
26
Patients
reported high satisfaction with APC by a computer aid, and
low decisional conflict with decisions about end-of-life
care.
27,28
In a qualitative longitudinal interview study of nine dyads
in Belgium, although all participants thought about future
care, few of them talked about it. Coping with the disease and
relational dynamics shaped individuals’thoughts about future
care over time, as well as psychological, social, and medical
factors were interwoven in ACP.
29
In contrast, in the Neth-
erlands, ACP started when the diagnosis was given, by means
of a general outlook on the future with progressive disability
and immediate introduction to a customized multidisciplinary
team. Patients accepted this policy as careful professional
guidance.
30
In a large retrospective study performed in an ALS clinic,
less than one third of patients with ALS completed an ACP
document, suggesting a need for greater documentation of
goals of care in the ALS population.
31
In a survey performed during a routine visit to the mul-
tidisciplinary ALS clinic, a substantial proportion of subjetcs
initiated end of life conversations early. When feeling in-
formed, patients were more likely to make specific choices.
Most patients felt comfortable discussing to discuss these
issues. When specifically asked about a DAT or ACP, 91% of
patients responded, and 68.9% of them reported they had a
DAT or ACP. In most patients ACP was completed within one
year of diagnosis.
32
These findings suggested the opportunity
for providers to help facilitate conversations, ensuring patient
wishes.
This is contrast with the findings of this study, performed in
a country and particularly in a Southern region like Sicily, with
specific cultural peculiaritis, different from Anglo-Saxon or
Northern European countries. No patient had a DAT, and ACP
was initiated only with the home palliative care team, re-
flecting the reluctance of ALS clinics to encourage overt
discussion of ACP.
Most studies have been performed in ALS clinics, while the
present study was performed in home palliative care, pre-
sumably in patients with more advanced disease, as witnessed
by the low mean value of ALSFRS-R at admission to the home
palliative care program. Thus, end of life decision making is
often delayed. In a retrospective chart review of all patients
referred to the neuro-palliative care team in Hong Kong,
patients with advanced neurological diseases, including ALS,
were readier to discuss their end of life issues and signed their
advance directives. Thus, the neuro-palliative care teams can
play a valuable role in end of life discussions in patients with
advanced neurology diseases in collaboration with neurology
teams.
33
There are some limitations of this study. This is a single
center study, and not necessarily reflects the entire Italian
situation, also considering some cultural differences between
the Northern and insular areas of the country. Morever, the
home care team is characterized by large experience with
specific competence neuropalliative care.
24
The accomplish-
ment of APC was not recorded, also because most patients
were still living at study termination. However, in patients
who died no conflict has ever been observed between ACP and
end-of-life decisions.
In conclusion, in Italy DAT resulted scarcely widespread,
despite an existing law, as no patient officially provided in-
dications on end of life issues. In addition, ACP was given
only after starting specialized home palliative care in less than
1/3 of patients. Home palliative care seems to be a funda-
mental resource for improving communication and soliciting
expression of patients’wishes regarding end of life issues.
Barriers for ACP should be overcome with an early intro-
duction of facilitators, which include accumulated skills
typically belonging to palliative care culture. Public awareness
coupled with implementation of ACP programs, collabora-
tions between hospital and health services, and local and
residential care facilities are needed in making system-wide
ACP a reality.
Author Contributions
SM: Conceptualization, Methodology, original draft preparation,
review and editing. AL: Formal Analysis, review & editing. PP:
Investigation, review & editing.
Declaration of Conflicting Interests
The author(s) declared no potential conflicts of interest with respect to
the research, authorship, and/or publication of this article.
Funding
The author(s) received no financial support for the research, au-
thorship, and/or publication of this article.
ORCID iD
Sebastiano Mercadante https://orcid.org/0000-0001-9859-6487
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