End-of-life decision-making for children with severe developmental disabilities


Research in Developmental Disabilities 49–50 (2016) 235–246

Contents lists available at ScienceDirect

Research in Developmental Disabilities

End-of-life decision-making for children with severe

developmental disabilities: The parental perspective

I.H. Zaal-Schuller a, *, M.A. de Vos a , F.V.P.M. Ewals b , J.B. van Goudoever c ,

D.L. Willems a

a Section of Medical Ethics, Department of General Practice, Academic Medical Centre, University of Amsterdam, Amsterdam, The


b Intellectual Disability Medicine, Department of General Practice, Erasmus Medical Centre Rotterdam, Rotterdam, The Netherlands

c Department of Paediatrics, Emma Children’s Hospital—Academic Medical Centre, Amsterdam & Department of Paediatrics, VU University

Medical Centre, Amsterdam, The Netherlands




Article history:

Received 4 December 2014

Received in revised form 28 November 2015

Accepted 8 December 2015

Available online




Critical treatment decision

Severe developmental disorder


To advocate

Quality of life

Integrative review

Background and aims: The objectives of this integrative review were to understand how

parents of children with severe developmental disorders experience their involvement in

end-of-life decision-making, how they prefer to be involved and what factors influence

their decisions.

Methods and procedures: We searched MEDLINE, EMBASE, CINAHL and PsycINFO. The

search was limited to articles in English or Dutch published between January 2004 and

August 2014. We included qualitative and quantitative original studies that directly

investigated the experiences of parents of children aged 0–18 years with severe

developmental disorders for whom an end-of-life decision had been considered or made.

Outcomes and results: We identified nine studies that met all inclusion criteria. Reportedly,

parental involvement in end-of-life decision-making varied widely, ranging from having

no involvement to being the sole decision-maker. Most parents preferred to actively share

in the decision-making process regardless of their child’s specific diagnosis or

comorbidity. The main factors that influenced parents in their decision-making were:

their strong urge to advocate for their child’s best interests and to make the best (possible)

decision. In addition, parents felt influenced by their child’s visible suffering, remaining

quality of life and the will they perceived in their child to survive.

Conclusions and implications: Most parents of children with severe developmental

disorders wish to actively share in the end-of-life decision-making process. An important

emerging factor in this process is the parents’ feeling that they have to stand up for their

child’s interests in conversations with the medical team.

ß 2015 Elsevier Ltd. All rights reserved.


1. Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 236

2. Methods . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 237

* Corresponding author. Tel.: +31 020 566 0220.

E-mail addresses: i.h.schuller@amc.nl (I.H. Zaal-Schuller), m.a.devos@amc.nl (M.A. de Vos), f.ewals@erasmusmc.nl (F.V.P.M. Ewals),

h.vangoudoever@amc.nl (J.B. van Goudoever), d.l.willems@amc.nl (D.L. Willems).


0891-4222/ß 2015 Elsevier Ltd. All rights reserved.


I.H. Zaal-Schuller et al. / Research in Developmental Disabilities 49–50 (2016) 235–246

2.1. Literature search and study eligibility. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 237

2.2. Data evaluation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 237

2.3. Data extraction and synthesis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 237

3. Results . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 238

3.1. Involvement experienced by parents . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 241

3.2. Involvement preferred by parents . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 241

3.3. Guiding factors in parental decision-making . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 242

4. Discussion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 243

5. Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 244

References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 245

What this paper adds?

This is the first integrative review that describes the experiences of parents of children with severe developmental

disabilities regarding end-of-life decision-making for their child. The objective was to understand how parents experience

their involvement in end-of-life decision-making, how they prefer to be involved and what factors influence their decisions.

Nine eligible studies were selected after an electronic database search. The main outcome was that the degree of parental

involvement within and across these studies varied widely. This degree ranged from parents having no involvement at all to

parents being the sole decision-maker. Parents also differed considerably in their personal preferences of how they wished to

be involved in the decision making process for their child.

Parents indicated that they felt guided by two main factors: their desire to do what they considered to be the best for their

child and their perception of their child’s quality of life. Some parents added that it seemed as if physicians regarded the life

of their child as less valuable than the life of a normally developing child. This last factor often made parents feel that they

had to advocate for their child’s interests.

1. Introduction

Medical End-of-Life decisions (EoLDs) can lead to or hasten death, intentionally or not. These decisions include

withholding or withdrawing life-prolonging treatment (such as mechanical ventilation, tube feeding, or dialysis) and

alleviating pain or other symptoms with drugs that may have a life-shortening effect. Administering, prescribing or

supplying drugs to end a patient’s life at the patient’s explicit request also falls within the scope of EoLDs (van der Heide et al.,

2003). In the Netherlands – similar to other countries – this is prohibited in neonates, infants, children and people with

intellectual disabilities who are mentally incompetent to make an explicit request (Termination of Life on Request and

Assisted Suicide Act, 2002). Yet, a legal provision exists which allows physicians under strict conditions to deliberately end

the life of a newborn who experiences severe suffering without any prospect for improvement. These conditions are

described in the Groningen protocol (Verhagen & Sauer, 2005; Willems, Verhagen, van Wijlick, & C, 2014).

Sixty-eight percent of all deaths of Dutch children between 0 and 1 year of age are preceded by an EoLD. In children

between 1 and 16 years of age, 36% of all deaths are preceded by an EoLD (Vrakking et al., 2005, 2007). In children with severe

developmental disabilities (SDDs) this percentage is still unknown. SDDs are defined as severe chronic disabilities that can be

cognitive, physical or both. They are characterized by a delay in development, based on the expected stage of development at

a certain age. The disabilities appear before the age of 22 and are likely to be lifelong (Schalock et al., 2010). Two studies

investigated the prevalence of EoLDs among deceased intellectually disabled adults who lived in care-providing agencies in

the Netherlands (van Thiel, van Delden, de Haan, & Huibers, 1997; Wagemans, van Schrojenstein Lantman-de-Valk, Tuffrey-

Wijne, Widdershoven, & Curfs, 2010). While the first study showed that 44% of all reported deaths were preceded by an EoLD

(van Thiel et al., 1997), the second study reported this percentage to be 57% (Wagemans et al., 2010). This implies an increase

of 13% over a period of 15 years. A study by van der Heide et all investigated end-of-life decision-making (EoLDM) in the

general Dutch population and concluded that 44% of all deaths were preceded by an EoLD (van der Heide et al., 2003).

Children with a SDD have a greater chance to suffer from complex medical problems (Chaney & Eyman, 2000; Oeseburg,

Dijkstra, Groothoff, Reijneveld, & Jansen, 2011; van Schrojenstein Lantman-de Valk et al., 1997) and generally have a shorter

life expectancy (Bittles et al., 2002; Strauss, Shavelle, & Anderson, 1998). Hence, parents of children with SDDs will be

confronted with medical decisions more often than other parents.

A challenging question in medical decision-making, and especially in EoLDM, is in what way and to what extent parents

should be involved. Different degrees of involvement are possible: parents may be the sole decision-makers and bear the

final responsibility; they may participate in the decision-making process and share in the final responsibility; they may have

considerable input while the doctor makes the final decision; or they may have no involvement at all (Gillam & Sullivan,

2011). In the United Stated and Canada, parents are expected to be the primary decision-makers for their children in EoLDM,

based on prevailing moral and legal traditions (Choong et al., 2010; Eden & Callister, 2010). By contrast, in European, Middle

Eastern and South American countries, the medical and public opinion dictates that parents lack sufficient medical

knowledge to make these decisions and, even more importantly, should be protected from the burden of this responsibility

I.H. Zaal-Schuller et al. / Research in Developmental Disabilities 49–50 (2016) 235–246 237

(Cremer et al., 2007; da Costa, Ghazal, & Al Khusaiby, 2002; Devictor, Tissieres, Gillis, & Truog, 2008; Lago et al., 2005; Moro,

Kavanaugh, Okuno-Jones, & Vankleef, 2006; Vrakking et al., 2005; Ward, 2005). Nation-specific cultural and religious

traditions are therefore important predictors of the role given to parents in EoLDM (Devictor et al., 2008; Vrakking et al.,


Another interesting issue is the question of which factors influence parents in EoLDM for their child with an SDD. When

proxy decision-makers have to make an EoLD for an adult patient, they feel guided by the patient’s past decisions and

behaviour, even if they never specifically discussed EoLDs directly with the patient (Lipstein, Brinkman, & Britto, 2012). By

contrast, parents of a child with an SDD must rely on other sources of information because their child is not able to express

his or her preferences and will never be.

The number of studies exploring parental views on EoLDM in these situations is limited, and no attempt has been made to

review them collectively or identify gaps in our knowledge. The purpose of this article is to systematically trace and review

original studies in which the perspectives on EoLDM of parents with children with SDDs are investigated. Our main questions

are (1) how do parents of children with SDDs perceive their involvement in EoLDM, (2) how do they prefer to be involved and

(3) by which factors are they guided throughout the decision-making process?

2. Methods

2.1. Literature search and study eligibility

Four electronic databases were searched: MEDLINE, EMBASE, CINAHL and PsycINFO. We limited our search to studies

published between January 2004 and August 2014. We chose this period because parental experiences regarding EoLDM in

children with SDDs are subject to change, making older papers less relevant. In the past ten years, the level of parental

involvement in medical decision-making has especially been influenced by the growing attention for the concept of shared

decision-making (Kon, 2010).

Inclusion criteria were articles in English or Dutch of quantitative and qualitative studies on the EoLDM experiences

of parents of children with SDDs. Included studies had to report data on EoLDM even if it was not the primary focus of

the study. If less than half of the children in a study had an SDD, the study was excluded unless a distinction had

been made between children with and without an SDD. In those cases, we were able to extract and use the information

concerning the children with SDDs. The following key words were used and combined (1) end-of-life decision-making

and related terms, (2) intellectual disability and related terms, (3) parents OR patient participation and related

terms and (4) children (0–21 y); each key word was mapped to the appropriate medical subject heading of the

United States National Library of Medicine. The search strategy was developed in collaboration with an experienced

clinical librarian. An additional search was carried out using a broader strategy by excluding ‘end-of-life’ and

children’. This search did not produce any new articles. In addition, we hand-searched the reference lists of the

included articles.

Studies of children with various conditions (such as a neurologic, metabolic or genetic condition not further specified)

which are usually accompanied by an SDD were also included when the presence of an SDD was not explicitly mentioned but

could be assumed from the objective of the study. In some studies, it was not clear whether the EoLDM experiences of

parents of children with SDDs were included. In these cases, we asked the authors by e-mail to provide more detailed

information on the child’s diagnosis. With this additional information, we determined whether a study could be included.

2.2. Data evaluation

The final sample for this integrative review included qualitative and quantitative studies in which various methods,

such as in-depth interviews or structured questionnaires, were used. Due to the heterogeneity in methodology, the nine

selected studies were critically appraised regarding data relevance and credibility (Whittemore & Knafl, 2005)bymeansof

a checklist of Cochrane Netherlands (Cochrane Netherlands, 2015). In addition, studies with a quantitative design were

appraised by a tool designed for observational studies (Berger et al., 2014). The process of appraisal was done by two of the

authors (IZ and MV). As a result of this process, studies of low relevance and/or low credibility contributed less to the

analytic process.

2.3. Data extraction and synthesis

Two researchers (IZ and MV) independently completed a standardized data extraction form for each included study.

Differences were resolved by discussion and, if necessary, by consulting a third reviewer (DW). Extracted information

included citation details, setting, number of participating parents and study design. The results of each study were

subdivided into our three themes of interest for this review: (1) parental experiences regarding their actual involvement in

EoLDM; (2) parental preferences regarding their involvement in EoLDM; and (3) guiding factors during the EoLDM process.

The results of each individual study were reported in tabular forms per theme and combined to form a narrative. Given the

heterogeneity of the included studies, no meta-analysis was performed.


I.H. Zaal-Schuller et al. / Research in Developmental Disabilities 49–50 (2016) 235–246

3. Results

The literature search identified 2933 discrete citations. After screening the corresponding titles and abstracts, 35 studies

seemed to be eligible for inclusion according to our criteria. The full texts of these studies were then reviewed in-depth.

Thirty studies had to be excluded: 6 studies that included no, or too few, children with SDDs; 13 studies that did not focus on

EoLDM; 5 studies that did not focus on the parents’ perspective; and 6 studies that appeared to be case reports. An additional

4 studies were subsequently found by searching the reference lists of suitable studies. In the end, nine studies were eligible

for inclusion in our review. The flow of information for our review is summarized in Fig. 1.

In Table 1, we present the characteristics of the 9 included studies. The studies were inconsistent in the terminology used

to describe EoLDs. We encountered, for example, ‘limit or withdraw life support’ (Sharman, Meert, & Sarnaik, 2005),

decision-making in the PICU’ (Madrigal et al., 2012), ‘choosing plan of care’ (Guon, Wilfond, Farlow, Brazg, & Janvier, 2014)

and ‘critical decisions’ (October, Fisher, Feudtner, & Hinds, 2014).

All but two studies had a qualitative design, although in some studies questionnaires were used (Guon et al., 2014;

October et al., 2014). The remaining two studies had a quantitative design (Friedman, 2006; Madrigal et al., 2012).

Three of the nine studies prospectively followed parents throughout the decision-making process (Madrigal et al., 2012;

October et al., 2014; Sharman et al., 2005). The other six studies retrospectively investigated parents’ experiences and

preferences. In these studies, the period of time that had elapsed between the EoL decision and study participation varied

considerably, from several months up to nineteen years (Brotherton & Abbott, 2012; Friedman, 2006; Guon et al., 2014;

Morrow, Quine, Loughlin, & Craig, 2008; Rapoport, Shaheed, Newman, Rugg, & Steele, 2013; Reilly, Huws, Hastings, &

Vaughan, 2010).

All studies were conducted in the United Kingdom, United States, Australia or Canada. In Table 2, we present regulations

of parental involvement and conflict resolution in these countries according to the professional guidelines. One study also

included a small percentage of parents from other countries (Guon et al., 2014).



decision making



Parental or













3635 Hits


2933 Articles

identified by

screening on title

35 Potentially relevant

studies retrieved for

review in full

5 Relevant


30 Articles were excluded:

• 6: No or too few children with an SDD

• 13: Not related to EoLDM

• 5: No report of parents

• 6: Case reports


• Articles identified from a

reference list search

9Articles in


Fig. 1. Flow diagram for study selection.

Table 1

Characteristics of included studies.



participants, n


Patients, n



Underlying diagnosis of

patients, (n)



described d Timing of

data collection

Study design (data





—Qualitative Health


Friedman (2006)—

Journal of the

American Medical

Directors Association

Guon et al. (2014)—

American Journal of

Medical Genetics

Madrigal et al. (2012)—

Critical Care Medicine

Morrow et al. (2008)—

Archives of Disease in


October et al. (2014)—

Pediatric Critical

Care Medicine

Rapoport et al. (2013)—


Mothers, n =20 UK b n =20

Age: 1–15 y

Sex: 11 girls

and 9 boys

Parents and/or

guardians, n =60

Mothers, n =98

Fathers, n =30

US c n =60

Age: 2–32 y

Sex: 31 girls

and 29 boys

83% US, 5%


5% UK, 7%

from 12 other


n = 107

Age and sex

not specified

Parents, n =87 US n =75

Age: 0–19 y

Sex not clearly


Mothers, n =17

Fathers, n =2

Foster mother, n =1,

Sibling, n =1

Mothers, n =25

Fathers, n =18

Mothers, n =6

Fathers, n =5

Australia n =21

Age: 4–19 y

Sex not specified

US n =29

Age: 1–10 y

Sex: 16 girls and

13 boys

Canada n =7

All children died

Age and sex not


Cystic fibrosis, global Gastrostomy

developmental delay, placement

faltering growth, cerebral

palsy, congenital



disorders (numbers not


Severe developmental

disabilities and complex

medical problems

Trisomy 13, Trisomy 18





(2 mo–7 y 8 mo

after gastrostomy


Qualitative study—

(in-depth interviews;

discourse analysis)

Resuscitation G-F Before and after Quantitative study—

provision of written (structured interviews by

information phone and review of

records; statistical


Continuance of


Choice of plan of

care (full


comfort care,

combination of full

interventions and

comfort care)


Medical decisions

respiratory, congenital or in PICU a setting

genetic, neuromuscular,

metabolic, malignancy

(numbers not specified)

Quadriplegic cerebral

palsy with feeding



(10), respiratory (6),

neurologic (4), trauma (4),

gastrointestinal (2),

metabolic/genetic (2),

sepsis/shock (1)

Genetic or suspected

genetic conditions

Interventions for

feeding difficulties

Critical treatment

decision (decision

to initiate, escalate,

withdraw lifesustaining


Forgoing artificial

nutrition and

hydration (FANH)

G-F Retrospective Mixed methods study—

(a computer assisted


questionnaire with

10 open-ended questions

and 12 demographic

questions; statistical

analysis and thematic

qualitative content


P-I Prospective Quantitative study—

(interviews and


instruments; statistical





Qualitative study—

(focus groups; constant

comparative method)

G-F Prospective Mixed methods study—

(structured interviews by

means of Good Parent

Tool and Good Parent

Ranking Exercise;

thematic qualitative

content analysis)




(1.5–5 y after the

death of the child)

Qualitative study—

(in-depth interviews;

interpretive description










I.H. Zaal-Schuller et al. / Research in Developmental Disabilities 49–50 (2016) 235–246 239

Table 1 (Continued )



participants, n


Patients, n



Underlying diagnosis of

patients, (n)



described d Timing of

data collection

Study design (data






Reilly et al. (2010)—

Intellectual &



Sharman et al. (2005)—

Pediatric Critical

Care Medicine

Mothers, n =6

Fathers, n =6

(all couples)

Mothers, n =9

Fathers, n =5

UK n =6

Down syndrome with Decision-making

All children died

Age at death: 2–18 y

Sex: not specified

congenital heart condition regarding medical


including heart


US n =10

Age: 3 mo–14 y

Sex: 3 girls and

7 boys

a PICU = Paediatric Intensive Care Unit.

b UK = United Kingdom.

c US = United States.

d E-I = Experienced Involvement; P-I = Preferred Involvement; G-F = Guiding Factors.

Malignancy (2),

respiratory failure (2),

neurologic condition (5),

metabolic condition (1)

Limit or withdraw

life support







(8–19 y after the

death of the child)


Qualitative study—


interviews; interpretative



Qualitative study—

(in-depth interviews;

not specified)



I.H. Zaal-Schuller et al. / Research in Developmental Disabilities 49–50 (2016) 235–246

I.H. Zaal-Schuller et al. / Research in Developmental Disabilities 49–50 (2016) 235–246 241

Table 2

Guidelines in EoLDM in each involved country.

United Kingdom—Consent to the withholding or withdrawal of treatment may be given by anyone who holds parental responsibility. However, if there

is parental opposition or a disagreement as to what is in the child’s or young person’s best interests, an application should be made to court for a

determination as to best interests. The High Court has overarching authority in decisions about children. When individuals ask for treatment that the

health care professional has not offered and which he or she considers not clinically appropriate for the patient, the professional is not obliged to

provide it a

United States—The decision to withhold or discontinue health care may be considered by providers in conjunction with the parents acting in the best

interests of the child b . In the small number of cases where agreement is not possible, physicians are not obligated to offer therapies that they believe

cannot achieve the goals of care. A process-based approach is recommended to resolve conflicts over end-of-life issues. Central to this process is

mediation by the hospital ethics committee c . If the ethics consultation process fails to resolve the dispute, the hospital must try to arrange transfer of

the patient to another physician or institute willing to give the treatment requested by the family. If no such provider can be found, the hospital and

physician may unilaterally withhold or withdraw therapy that has been determined to be futile d

Canada—Physicians are not obliged to provide treatments that would not be of benefit to the patient; however, unilateral physician decision-making is

not endorsed. If the substitute decision-makers insist on a course of treatment that the physician considers medically inappropriate, the physicianis

obliged to assist in finding a treatment team that will agree to treat according to the consent given. When all mediation attempts have failed, the

physician may apply to a judicial body to challenge the substitute decision-makers’ decision. This option should be considered only after alternative

methods of conflict resolution have been exhausted e

Australia—The senior treating clinician should not merely outline treatment options then delegate decision-making responsibility to families, but

rather they should make recommendations for management based on their understanding of the patient’s medical condition and prognosis, while

continuing to work with, and support, a family and reach a consensus decision. Where the process fails to bring a consensus about the appropriateness

of treatment limitation, resolution will require application to the Court f

a Larcher, V., Craig, F., Bhogal, K., Wilkinson, D., Brierley, J., Royal College of, P., & Child, H. (2015). Making decisions to limit treatment in life-limiting and

life-threatening conditions in children: A framework for practice. Archives of Disease in Childhood, 100 Suppl 2, s3-23.

b Eden, L. M., & Callister, L. C. (2010). Parent Involvement in End-of-Life Care and Decision Making in the Newborn Intensive Care Unit: An Integrative

Review. Journal of Perinatal Education, 19, 29-39.

c Truog, R. D., Campbell, M. L., Curtis, J. R., Haas, C. E., Luce, J. M., & Rubenfeld, G. D., et al. & American Academy of Critical Care, M. (2008).

Recommendations for end-of-life care in the intensive care unit: A consensus statement by the American College of Critical Care Medicine. Critical Care

Medicine, 36, 953–963.

d Fine, R. L., & Mayo, T. W. (2003). Resolution of futility by due process: Early experience with the Texas Advance Directives Act. Annals of Internal Medicine,

138, 743-746

e Choong, K., Cupido, C., Nelson, E., Arnold, D. M., Burns, K., & Cook, D., et al. & Accademy. (2010). A framework for resolving disagreement during end of life

care in the critical care unit. Clinical & Investigative Medicine, 33, E240-253.


Office of the Chief Health Officer (2012). Guidelines for end-of life care and decision-making (pp. 1–17). Sydney: NSW Department of Health.

3.1. Involvement experienced by parents

Four studies investigated how parents experienced their involvement in the EoLDM process (Brotherton & Abbott, 2012;

Morrow et al., 2008; Reilly et al., 2010; Sharman et al., 2005). The degree of involvement ranged from parents being the sole

decision-maker to the physician being the sole decision-maker. The latter cases were described in more detail, and parents

indicated that the experience of not being included caused negative emotions (Brotherton & Abbott, 2012; Morrow et al.,

2008; Sharman et al., 2005).

Two qualitative studies described the experiences of parents who faced the decision about a gastrostomy placement to

achieve adequate nutritional intake for their child (Brotherton & Abbott, 2012; Morrow et al., 2008). In the first study, some

mothers identified their physician as the sole decision-maker while several other mothers described themselves as such,

even though, from a clinical perspective, they all appeared to be in the same situation: all of their children required enteral

tube feeding to achieve adequate nutritional intake (Brotherton & Abbott, 2012). These difference in experienced

involvement could be explained by the fact that in this study only the personal perspective of how much choice they received

from the physician was described. In the second study, which included parents of children with cerebral palsy, parents often

indicated that they felt their opinion was not considered important. Moreover, some parents indicated that they felt

pressured to accept the gastrostomy. They believed they were made to feel guilty or ashamed, particularly with regard to

their child’s nutritional status (Morrow et al., 2008). A similar feeling of pressure was described by some parents who felt

they had to accept an EoLD (Sharman et al., 2005).

In a study by Reilly et al. (2010), six couples who had lost a child with Down syndrome and a congenital heart condition

were interviewed. All couples mentioned that they had felt highly involved in their child’s care and in decisions regarding

their child’s medical treatment, but they did not specify the extent to which they had been involved in the EoLDM for their


3.2. Involvement preferred by parents

The way in which parents preferred to be involved varied widely and seemed to be highly influenced by the type of

decisions. One study investigated whether parents’ preferences regarding EoLDM for their child were influenced by the

child’s disease characteristics. Child’s disease characteristics, such as diagnosis, the presence of chronic conditions or

previous hospital admissions, were not significantly associated with parents’ decision-making preferences (Madrigal et al.,

2012). Most parents included in this study preferred shared decision-making with their physician (40%) or (largely) making

the final decision on their own (41%). A still-sizeable minority of 19% preferred the physician to be the sole decision-maker.


I.H. Zaal-Schuller et al. / Research in Developmental Disabilities 49–50 (2016) 235–246

One study described the preferences of parents in the decision-making process regarding a gastrostomy placement

(Brotherton & Abbott, 2012) and another regarding withholding artificial nutrition and hydration (Rapoport et al., 2013). In

the first study, most mothers who had been allowed to make the final decision evaluated this as very positive. Several

mothers underlined that this leading role had enabled them to make a true child-centred decision. Mothers who had not had

any say in the decision-making process described feelings of resentment and guilt, even if considerable time had elapsed

since the gastrostomy placement (Brotherton & Abbott, 2012).

In the second study, 11 bereaved parents were interviewed about their experiences regarding the decision to withhold

artificial nutrition and hydration. All parents stated that this decision should only be considered when they were ready, i.e.

when they viewed their child’s quality of life as very poor. Parents highly appreciated it when the option to withhold artificial

nutrition and hydration was discussed with them in a sensitive, non-judgemental and supportive way. They also found it

helpful when professionals shared their knowledge and past experiences. Finally, parents greatly appreciated the

information that withholding artificial nutrition and hydration would not lead to an uncomfortable dying process. All

parents included in this study believed the decision was theirs alone to make (Rapoport et al., 2013).

In a fourth study, parents did not clearly specify their preferred way to be involved. However, this study described the

different decision-making styles parents had, with some parents relying solely on information and others on values and

feelings (Sharman et al., 2005).

3.3. Guiding factors in parental decision-making

In making an EoLD for their child, parents felt guided and motivated by a combination of factors, with as most important

factors: their assessment of their child’s suffering; their assessment of his or her quality of life; their wish to do what was best

for their child; and their urge to advocate for their child’s interests.

One study investigated whether the specific medical condition of the child, such as an SDD, was an important factor for

parents in the EoLDM for their child. However, parents in this study described that putting the child’s needs first, advocating

for their child’s interests and focusing on their child’s quality of life were the most important factors in the decision-making,

regardless of their child’s diagnosis or the presence of a chronic condition (October et al., 2014).

The question by which factors parents felt guided in their EoLDM was investigated in all studies except in one (Madrigal

et al., 2012). The child’s perceived poor quality of life (Rapoport et al., 2013) and doing what was best for the child

(Brotherton & Abbott, 2012; Sharman et al., 2005) appeared to be the most important guiding factors in EoLDM in three

studies. In defining quality of life parents concretely named the child’s ability to interact meaningfully with his/her

environment and the ability to enjoy things (Rapoport et al., 2013).

According to two other studies, the parents’ personal observations of their child’s suffering and pain weighed heavily in

their wish to withdraw life-prolonging treatment (Rapoport et al., 2013; Sharman et al., 2005). Parents also felt guided by the

will they perceived in their child to survive. They based this conviction on their observations of the child’s behaviour, his or

her body language and eye contact (Sharman et al., 2005). Parents of children with trisomy 13 and 18 especially weighed the

child’s anomalies, such as brain and cardiac defects, in their wish to withhold life-prolonging treatment (Guon et al., 2014). In

the two studies regarding the decision of a gastrostomy placement, parents reported different considerations. Extreme

feeding difficulties appeared to be an important reason for the parents who agreed with this placement (Morrow et al., 2008).

By contrast, the loss of everyday mothering activities was an important reason for other parents to not want a gastrostomy

placement (Brotherton & Abbott, 2012). Moreover, many mothers regarded the placement of a gastrostomy as a personal

failure to adequately feed their child (Brotherton & Abbott, 2012; Morrow et al., 2008). The side effects of artificial feeding,

such as pain, nausea, and vomiting, contributed to the decision to withhold artificial nutrition and hydration and in all cases

resulted in the child’s death (Rapoport et al., 2013).

Parents often felt they had to advocate for their child’s interests (Guon et al., 2014; Morrow et al., 2008; October et al.,

2014; Reilly et al., 2010; Sharman et al., 2005). They did this by explaining to the professionals that a decision had to be based

on the needs of their child at that point in time and not on the fact that their child was disabled (Morrow et al., 2008). If

parents could still experience their child’s life as positive and enriching, regardless of his/her life expectancy, they strongly

favoured the continuation of life-prolonging measures (Guon et al., 2014; Sharman et al., 2005).

The role of religion was investigated in two studies. In both studies, parents indicated that religious beliefs had not been a

guiding factor in their decision-making (Guon et al., 2014; Sharman et al., 2005). However, according to one study, several

parents felt strengthened by their belief in God. This helped them to better cope with the painful decision-making process.

Some of these parents also felt that only God had the final decision-making authority (Sharman et al., 2005).

Additional guiding factors in EoLDM that were mentioned in various studies were: the opinion of the other parent

(Rapoport et al., 2013; Reilly et al., 2010) and previous experiences with EoLDM for a loved one (Sharman et al., 2005). One

mother stated that the financial burden of having to care for a severely disabled child had convinced her to agree to withhold

life-prolonging treatment (Sharman et al., 2005).

Most parents were well aware that they were highly dependent on the information provided by the physicians. One study

showed that the provision of written explanatory information about resuscitation in a non-acute setting led to a significant

increase in requests to withhold this treatment (Friedman, 2006).

Five studies indicated that parents felt that the information provided by the physician had been insufficient because, for

example, they had not been informed about other treatment options (Friedman, 2006; Guon et al., 2014; Morrow et al., 2008;

I.H. Zaal-Schuller et al. / Research in Developmental Disabilities 49–50 (2016) 235–246 243

Rapoport et al., 2013; Reilly et al., 2010; Sharman et al., 2005). This had made their decision-making more difficult (Reilly

et al., 2010).

In two studies, parents stressed the importance of how information was given (October et al., 2014; Rapoport et al., 2013)

and the importance of open communication between parents and the medical team (October et al., 2014). Parents sought

information from various sources when making a decision for their child (Guon et al., 2014; Sharman et al., 2005). Conflicting

information obtained from the physician and from the Internet could lead to mistrust of, conflict with and distress regarding

the physician (Guon et al., 2014). Two studies mentioned the desire of parents to speak with other parents who had gone

through the same process (Morrow et al., 2008; Rapoport et al., 2013).

Time was also an important factor for parents in EoLDM (Friedman, 2006; Rapoport et al., 2013; Reilly et al., 2010;

Sharman et al., 2005). Lack of time made decision-making more difficult (Reilly et al., 2010) and could make parents feel

pressured into making a decision (Sharman et al., 2005). Sufficient time was appreciated by parents because it gave them the

opportunity to gradually come to terms with the fact that withholding treatment was in their child’s best interest (Rapoport

et al., 2013).

4. Discussion

This review investigated the EoLDM experiences of parents of children with SDDs. The main outcome is that the degree

of parental involvement within and across the studies being included varied widely. A comparison between these findings

and research carried out in children in PICUs (which is not exclusively limited to children with SDDs) shows some striking

similarities. Two studies that explored the views of bereaved parents regarding the physicians’ roles in EoLDM also

reported strongly varying roles, ranging from physicians making the decision alone to physicians only giving advice and

leaving the final decision to the parents (Meert, Thurston, & Sarnaik, 2000; Sullivan, Monagle, & Gillam, 2014). A third study

which investigated parents’ experiences in PICUs in both France and Canada reported a similar broad variation in how

parents had been involved (Carnevale et al., 2007). By contrast, the majority of parents who had to decide about

extracorporeal membrane oxygenation felt that there remained no choice but to consent to the proposed line of treatment

(Curley & Meyer, 2003). The majority of bereaved parents whose children had died after withdrawal of life support also felt

that they had had little to no control over the situation during their child’s final days (Meyer, Burns, Griffith, & Truog, 2002).

None of the studies gave a conclusive explanation about this difference in parental involvement. Recent studies have shown

that physicians working within the same hospital and sometimes within the same intensive care unit may involve parents

quite differently in the decision-making process (de Vos et al., 2011; McHaffie, Lyon, & Hume, 2001; Meert et al., 2000;

Provoost et al., 2006). It is still unclear whether this diversity reflects the different personal styles of individual physicians,

or the adjustment of physicians to the preferences and needs of individual parents (deVosetal.,2011;McHaffieetal.,2001;

Meert et al., 2000).

Which degree of involvement do parents prefer? The study with the largest sample of parents indicated that the majority

of parents favoured shared decision-making, even if this involved a burdensome EoLD (Madrigal et al., 2012). This outcome

contrasts with the outcome of a study concerning gastrostomy placement, in which all parents preferred to lead the decisionmaking

process (Brotherton & Abbott, 2012). In the decision to withhold artificial hydration and nutrition, they even

indicated that they wanted to be the sole decision-maker (Rapoport et al., 2013). This preference may be explained by the

fact that parents consider feeding their child to be one of the basic elements of parenting. Overall, parents differ in their

personal preferences as to how they want to be involved. Yet, they very seldom prefer an approach in which the physician is

the sole decision-maker.

In a recently published review about EoLDM in neonatal and paediatric intensive care units, most parents indicated that

they preferred a shared decision-making approach. For some parents this implied that they wanted to make the final

decision together with their physician. For other parents it implied that they wished to be well informed and to be asked for

their approval. Especially if the prognosis of their child was uncertain, most parents did not want to make the final decision

(de Vos, 2015). In the studies included in this review, parents did not indicate that the uncertainty of their child’s prognosis

influenced their preferences regarding their involvement. It may be hypothesized that these parents still felt confident that

they knew best what decisions were in their child’s best interests. This hypothesis can be strengthened by studies carried out

in children in PICUs. In these studies, parents also indicate that in EoLDM they felt guided by a sense of their child’s condition

and needs (Carnevale et al., 2007), their own intuition (Michelson et al., 2009), or the assumed wishes of their child (Meyer

et al., 2002).

Our review indicates that parents feel guided by two main factors: their desire to do what is best for their child and their

perception of their child’s quality of life (Brotherton & Abbott, 2012; Rapoport et al., 2013; Sharman et al., 2005). It was not

always clear what parents actually meant by ‘doing the best for their child’. Further research is needed to better explore this

concept (Allen, 2014).

Research investigating EoLDM in all children admitted to PICUs also concluded that quality of life is an important guiding

factor for parents (Meyer et al., 2002; Michelson et al., 2009). Many parents also felt guided by the presence of pain and

suffering. In their opinion, both factors highly diminished their child’s quality of life (Curley & Meyer, 2003; Meert et al.,

2000; Meyer et al., 2002; Michelson et al., 2009).

Parents often reported that they felt the urge to advocate for their child’s interests (Guon et al., 2014; Morrow et al., 2008;

October et al., 2014; Reilly et al., 2010; Sharman et al., 2005). Some parents added that it seemed as though physicians


I.H. Zaal-Schuller et al. / Research in Developmental Disabilities 49–50 (2016) 235–246

regarded the life of their child as less valuable than the life of a normally developing child. This strengthened the parents in

their conviction to persuade the physician of the intrinsic value of their child’s life.

Several parents underlined the importance of having sufficient time and obtaining sufficient information during the

decision-making process (Friedman, 2006; Morrow et al., 2008; Rapoport et al., 2013; Reilly et al., 2010; Sharman et al.,

2005). Information from other parents who had gone through the same process was considered especially helpful (Morrow

et al., 2008; Rapoport et al., 2013). The importance of sufficient time and information during EoLDM applies not only to

children with SDDs but to all children with critical illnesses (Carnevale et al., 2007; Curley & Meyer, 2003; Meert et al., 2000;

Michelson et al., 2009).

A recent review describing the experiences of parents of medically complex infants (including children with SDDs) also

concluded that parents feel strongly guided by their wish to do what is best for their child and not for themselves. Other

factors by which this group of parents felt guided were the seriousness of the child’s illness and the existence of additional

treatment options, such as experimental therapies if the child did not respond to initial treatments (Allen, 2014).

Studies about complex decision-making in children mostly focus on EoLDM, especially decisions to withhold or withdraw

life-sustaining prolonging treatment. However, in children with SDDs, almost every decision is complex. As noted

previously, children with SDDs frequently suffer from complex medical problems (Chaney & Eyman, 2000; Oeseburg et al.,

2011; van Schrojenstein Lantman-de Valk et al., 1997). Parents are therefore continuously faced with challenging decisions

(Tamburro, Shaffer, Hahnlen, Felker, & Ceneviva, 2011). Moreover, each decision has consequences for the next decision and

also has significant influence on the child’s quality of life.

Recent studies indicate that parents want to be involved in all aspects of care for their child: not only care in general but

also specific care, such as the management of epilepsy and necessary surgery (Rallison & Raffin-Bouchal, 2013; Redley et al.,

2013; Sheela, Latha, Liu, Lem, & Kaler, 2005; Vallenga, Grypdonck, Tan, Lendemeijer, & Boon, 2006). Moreover, most parents

regard themselves as the experts in the care of their child (Avis & Reardon, 2008; Iversen, Graue, & Clare, 2009; Rallison &

Raffin-Bouchal, 2013). At the same time, they have to face encounters with multiple health care professionals. They often

experience these encounters as negative (Iversen et al., 2009; Rallison & Raffin-Bouchal, 2013; Redley et al., 2013; Sleigh,

2005). Parents indicated for example that they were not listened to (Sleigh, 2005); that they were excluded from the

decision-making process regarding fundoplication (Fox et al., 2012), an operation predominantly carried out in children with

SDDs; and that they did not receive sufficient information (Ager, Downs, Fyfe, & Leonard, 2009; Iversen et al., 2009;

Marcovitch, 2005; Sleigh, 2005; Tandy, 2012). Several parents indicated that they would have especially appreciated

information from other parents who had already been through this process (Iversen et al., 2009).

The high prevalence of challenging decisions that parents of children with SDDs have to make over a long period of time,

and the fact that all of these decisions have serious consequences for the child and his/her family, make these decisions

‘critical treatment decisions’. In our opinion, using this one uniform term will facilitate further research.

This review has several limitations. First, the number of included studies is small, and all studies were performed in

Anglo-Saxon countries. This limits the generalizability of the outcomes.

Second, in several studies being included the sample size was small (Madrigal et al., 2012; Rapoport et al., 2013; Reilly

et al., 2010; Sharman et al., 2005) and/or there was a potential sample bias resulting from the self-selection of participants

(Guon et al., 2014; Madrigal et al., 2012; Reilly et al., 2010). Furthermore, the type of decision parents had encountered varied

greatly. This makes it harder to compare the different viewpoints of parents in EoLDM.

Finally, there was a considerable variation in how much time had passed between the decision-making and the data

collection phase. We do not know whether this may have affected the parents’ evaluation. We are well aware that because of

these limitations, the outcomes that we describe should be interpreted with caution and require further research among

larger groups of parents of children with SDDs making different types of critical treatment decisions.

The strength of this review is that it provides a comprehensive overview of the literature about the role of parents in

EoLDM in children with SDDs. An interesting theme for further research is the question what parents and physicians both

mean when they use the concept ‘the child’s best interest’. A second interesting theme is the influence of cultural, religious

and socio-economic factors on parental decision-making. This requires the inclusion of other, preferably non-Western,


5. Conclusion

Although the experienced involvement in EoLDM of parents of children with SDDs varied widely, parents were largely in

agreement about their wish to actively share in the decision-making process. Compared with parents of normally developing

children, the presence of an SDD does not seem to make a substantial difference in either parents’ preferences regarding

EoLDM or the factors by which they feel guided in their decision-making. Doing what is best for their child, seeing their child

suffer, and their child’s perceived will to survive were the three most important guiding factors mentioned by parents.

Parents often felt that the information provided by the physician was not sufficient, and they underlined their desire to

speak with other parents who had gone through the same process. Many parents reported that they felt that they had to

advocate for their child’s interests.

Parents of children with SDDs face very different types of decisions, ranging from surgery to the management of epilepsy

to decisions to withhold life-prolonging treatment, including artificial nutrition and hydration. Parents sometimes foresee

these decisions but may also be taken by surprise. We suggest the term critical treatment decisions as the overall term for the

I.H. Zaal-Schuller et al. / Research in Developmental Disabilities 49–50 (2016) 235–246 245

wide array of decisions that parents have to make for their child over time—decisions that almost always have important

implications for the child’s quantity and quality of life.

Funding sources

This study is part of a research project entitled ‘‘End-of-life decision-making in children with severe intellectual


This project is funded by the Rehabilitation Fund: R2011131 (het Revalidatiefonds), the Fund for Intellectual Disabilities:

L2011057 (het Fonds Verstandelijk Gehandicapten) and Erasmus Medical Centre, Department of Intellectual Disability

Medicine: SC200007. These funds did not in any way influence the data acquisition, data analysis, or drafting of this


Financial disclosure

The authors indicate that they have no financial relationships relevant to this article to disclose.

Conflict of interest statement

The authors have no conflicts of interest to disclose.


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