Ebook Pediatric palliative care: Part 1

(BQ) Part 1 “Pediatric palliative care” has contents: Pediatric palliative care in infants and neonates, paediatric palliative care in resource-poor countries, palliative care for children in hospital - essential roles, integrative approaches in pediatric palliative care, supporting parent caregivers of children with life-limiting illness,… and other contents.

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Pediatric

Palliative Care

Stefan J Friedrichsdorf Edited by

Printed Edition of the Special Issue Published in Children

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Pediatric Palliative Care

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Special Issue Editor

Stefan J Friedrichsdorf

MDPI• Basel • Beijing • Wuhan • Barcelona • Belgrade

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Stefan J Friedrichsdorf, MD, FAAP

Department of Pain Medicine, Palliative Care & Integrative Medicine,

Children’s Hospitals and Clinics of Minnesota

Minneapolis, Minnesota, USA

Editorial Ofﬁce

MDPI

St Alban-Anlage 66

4052 Basel, Switzerland

This is a reprint of articles from the Special Issue published online in the open access journal Children

(ISSN 2227-9067) from 2017 to 2018 (available at: https://www.mdpi.com/journal/children/specialissues/palliative care)

For citation purposes, cite each article independently as indicated on the article page online and asindicated below:

LastName, A.A.; LastName, B.B.; LastName, C.C Article Title Journal Name Year, Article Number,

Articles in this volume are Open Access and distributed under the Creative Commons Attribution(CC BY) license, which allows users to download, copy and build upon published articles even forcommercial purposes, as long as the author and publisher are properly credited, which ensuresmaximum dissemination and a wider impact of our publications The book taken as a whole

is c 2019 MDPI, Basel, Switzerland, distributed under the terms and conditions of the Creative

Commons license CC BY-NC-ND (http://creativecommons.org/licenses/by-nc-nd/4.0/)

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About the Special Issue Editor vii Stefan J Friedrichsdorf and Eduardo Bruera

Delivering Pediatric Palliative Care: From Denial, Palliphobia, Pallilalia to Palliactive

Reprinted from: Children 2018, 5, 120, doi:10.3390/children5090120 1 Brian S Carter

Pediatric Palliative Care in Infants and Neonates

Reprinted from: Children 2018, 5, 21, doi:10.3390/children5020021 14

Harold Siden

Pediatric Palliative Care for Children with Progressive Non-Malignant Diseases

Julie Hauer

Feeding Intolerance in Children with Severe Impairment of the Central Nervous System:Strategies for Treatment and Prevention

Timothy A Warlow and Richard D.W Hain

‘Total Pain’ in Children with Severe Neurological Impairment

Julia Downing, Sue Boucher, Alex Daniels and Busi Nkosi

Paediatric Palliative Care in Resource-Poor Countries

Ross Drake

Palliative Care for Children in Hospital: Essential Roles

Martha F Mherekumombe

From Inpatient to Clinic to Home to Hospice and Back: Using the “Pop Up” Pediatric PalliativeModel of Care

Andrea Postier, Kris Catrine and Stacy Remke

Interdisciplinary Pediatric Palliative Care Team Involvement in Compassionate Extubation atHome: From Shared Decision-Making to Bereavement

Kate Shafto, Suzanne Gouda, Kris Catrine and Melanie L Brown

Integrative Approaches in Pediatric Palliative Care

Lindsay J Blazin, Cherilyn Cecchini, Catherine Habashy, Erica C Kaye and Justin N Baker

Communicating Effectively in Pediatric Cancer Care: Translating Evidence into Practice

Kendra D Koch and Barbara L Jones

Supporting Parent Caregivers of Children with Life-Limiting Illness

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Katherine E Nelson, James A Feinstein, Cynthia A Gerhardt, Abby R Rosenberg, Kimberley Widger, Jennifer A Faerber and Chris Feudtner

Emerging Methodologies in Pediatric Palliative Care Research: Six Case Studies

Katharine E Brock, Joanne Wolfe and Christina Ullrich

From the Child’s Word to Clinical Intervention: Novel, New, and Innovative Approaches toSymptoms in Pediatric Palliative Care

Tiina Jaaniste, Sandra Coombs, Theresa J Donnelly, Norm Kelk and Danielle Beston

Risk and Resilience Factors Related to Parental Bereavement Following the Death of a Childwith a Life-Limiting Condition

Meaghann S Weaver, Cheryl Darnall, Sue Bace, Catherine Vail, Andrew MacFadyen and Christopher Wichman

Trending Longitudinal Agreement between Parent and Child Perceptions of Quality of Life forPediatric Palliative Care Patients

Sara Mu ˜noz-Blanco, Jessica C Raisanen, Pamela K Donohue and Renee D Boss

Enhancing Pediatric Palliative Care for Latino Children and Their Families: A Review of theLiterature and Recommendations for Research and Practice in the United States

Jean Kelly, Jo Ritchie, Leigh Donovan, Carol Graham and Anthony Herbert

A Retrospective Review of Resuscitation Planning at a Children’s Hospital

Larissa Alice Dreier, Boris Zernikow, Markus Blankenburg and Julia Wager

(SNAKE)—Concordance with a Global Rating of Sleep Quality

Taelyr Weekly, Nicole Walker, Jill Beck, Sean Akers and Meaghann Weaver

A Review of Apps for Calming, Relaxation, and Mindfulness Interventions for PediatricPalliative Care Patients

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About the Special Issue Editor

Stefan J Friedrichsdorf, MD, FAAP is an Asssociate Professor of Pediatrics at the University of

Minnesota and the medical director of the Department of Pain Medicine, Palliative Care and Integrative Medicine at Children’s Hospitals and Clinics in Minnesota, Minneapolis/St Paul (USA)

—one of the largest and most comprehensive programs of its kind in the country He is the elect of the Special Interest Group on Pain in Childhood of the International Association for the Study

president-of Pain (IASP) The interdisciplinary pain program is devoted to prevent and treat acute, procedural, neuropathic, psycho-social-spiritual, visceral, and persistent pain for all pediatric patients in close collaboration with all subspecialties at Children’s Minnesota The palliative care team provides holistic care for pediatric patients with life-limiting diseases and adds an extra layer of support to the care of children with serious illness and their families Integrative Medicine provides and teaches integrative, non-pharmacological therapies (such as massage, acupuncture/acupressure, biofeedback, aromatherapy, self-hypnosis) to provide care that promotes optimal health and supports the highest level of functioning in all individual child’s activities Dr Friedrichsdorf sees pediatric patients as inpatients, in the interdisciplinary pain clinic, or in the palliative care clinic He

is the sponsor of the system-wide lean value stream “The Comfort Promise: We do everything possible to prevent and treat pain” at Children’s Minnesota to minimize and prevent pain caused by needles for all in- and outpatients, reaching more than 200,000 children annually In 2015 the department opened an award winning, unique 10,000 square feet “Healing Environment” Pain, Palliative and Integrative Medicine Clinic Dr Friedrichsdorf received the 2016 “Elizabeth Narcessian Award for Outstanding Educational Achievements in the Field of Pain” by the American Pain Society and the 2011 “Hastings Center Cunniff-Dixon Physician Award” The department received the

“Circle of Life Award” by the American Hospital Association in 2008, was selected as a Palliative Care Leadership Center for the Center to Advance Palliative Care (CAPC) 2008-2015, and the 2013 recipient of the “Clinical Centers of Excellence in Pain Management Award” by the American Pain Society In 2016 Children’s Minnesota received the prestigious ChildKind International pain relief certiﬁcation He is associate editor of the Journal of Pain and Symptom Management, the principal investigator of a 2010–2017 National Institutes of Health (NIH) / National Cancer Institute (NCI) multisite study on the creation, implementation and evaluation of a Pediatric Palliative Care Curriculum (EPEC-Pediatrics) and he founded and continues to direct the annual Pediatric Pain Master Class, a week-long intensive interdisciplinary course for health professionals Dr Friedrichsdorf has presented more than 650 lectures about pediatric pain medicine and palliative care

in 28 countries on all six continents and has a track record of research and publications in the field, including contributions to more than 20 books on the subject Dr Friedrichsdorf received his MD degree from the Medical University of L übeck, Germany, completed his pediatric residency at the University of Witten/Herdecke, Germany (Children’s Hospital in Datteln), and undertook his fellowship in Pediatric Pain & Palliative Care at the University of Sydney, Australia (Children’s Hospital at Westmead) He is board certified in Pediatrics, a Fellow of the American Academy of Pediatrics and trained in hypnosis

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Editorial

Delivering Pediatric Palliative Care: From Denial, Palliphobia, Pallilalia to Palliactive

1 Children’s Hospitals and Clinics of Minnesota, 2525 Chicago Ave S, Minneapolis, MN 55403, USA

2 University of Minnesota Medical School, 420 Delaware Street SE, Minneapolis, MN 55455, USA

3 Department of Palliative Care and Rehabilitation Medicine, The University of Texas,

MD Anderson Cancer Center, 1515 Holcombe Blvd., Houston, TX 77030, USA; ebruera@mdanderson.org

* Correspondence: stefan.friedrichsdorf@childrensMN.org; Tel.: +1-612-813-7888; Fax: +1-612-813-7199 Received: 23 August 2018; Accepted: 28 August 2018; Published: 31 August 2018

Abstract: Among the over 21 million children with life-limiting conditions worldwide that would

beneﬁt annually from a pediatric palliative care (PPC) approach, more than eight million wouldneed specialized PPC services In the United States alone, more than 42,000 children die every year,half of them infants younger than one year Advanced interdisciplinary pediatric palliative care forchildren with serious illnesses is now an expected standard of pediatric medicine Unfortunately,

in many institutions there remain significant barriers to achieving optimal care related to lack offormal education, reimbursement issues, the emotional impact of caring for a dying child, and mostimportantly, the lack of interdisciplinary PPC teams with sufficient staffing and funding Data revealsthe majority of distressing symptoms in children with serious illness (such as pain, dyspnea andnausea/vomiting) were not addressed during their end-of-life period, and when treated, therapywas commonly ineffective Whenever possible, treatment should focus on continued efforts tocontrol the underlying illness At the same time, children and their families should have access tointerdisciplinary care aimed at promoting optimal physical, psychological and spiritual wellbeing.Persistent myths and misconceptions have led to inadequate symptom control in children withlife-limiting diseases Pediatric Palliative Care advocates the provision of comfort care, pain,and symptom management concurrently with disease-directed treatments Families no longer have

to opt for one over the other They can pursue both, and include integrative care to maximize thechild’s quality of life Since most of the sickest children with serious illness are being taken care of

in a hospital, every children’s hospital is now expected to offer an interdisciplinary palliative careservice as the standard of care This article addresses common myths and misconceptions which maypose clinical obstacles to effective PPC delivery and discusses the four typical stages of pediatricpalliative care program implementation

program implementation

1 Introduction

The special edition “Pediatric Palliative Care” in Children (http://www.mdpi.com/journal/

children/special_issues/palliative_care) has collated 20 outstanding articles from many of the leadingpediatric palliative care researchers and clinicians worldwide allowing us to present an overview ofadvances, research, and challenges in pediatric palliative care (PPC) As the guest editor, I thank theauthors for their strong contributions to this edition, in assisting children with serious illness and theirfamilies, as well as to moving our ﬁeld further along

Over 21 million children 0–19 years would beneﬁt annually from a palliative care approachworldwide, more than eight million needing specialized PPC [1,2] In the United States alone, more than

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42,000 children died in 2013, ﬁfty-ﬁve percent of them infants younger than one year [3] The leadingcauses of pediatric deaths include accidents (7645 children), suicide (2143), and homicide (2021).Leading life-limiting conditions include congenital malformations and chromosomal abnormalities(5740) followed by malignancies (1850) Minorities, such as Latinos, appear to have higher barriers toaccessing PPC [4].

PPC is about matching treatment to patient goals and is considered specialized medical carefor children with serious illness It is focused on relieving pain, distressing symptoms, and stress

of a serious illness and appropriate at any age and at any stage, together with curative treatment.The primary goal is to improve quality of life for both the child and his or her family In the words of anill child: “Palliative care no longer means helping children die well, it means helping children and theirfamilies to live well, and then, when the time is certain, to help them die gently.” (Mattie Stepanek,1990–2007) Sadly, advances in the control of symptoms in children dying of diseases such as cancerhave not kept pace with treatment directed at curing the underlying disease The majority of distressingsymptoms in children with advanced cancer (such as pain, dyspnea and nausea/vomiting) were nottreated, and when treated, therapy was commonly ineffective [5 9]

In this editorial we will address the four steps of PPC program implementation [10], followed by

an evaluation of common assumptions, myths and barriers, which may hinder the implementation ofPPC into the care of a child or teenager with serious illnesses [11,12]

2 Implementing Institutional Change: The Four Stages of PPC Program Implementation

Although the majority of children’s hospitals in the United States do have a pediatric palliativecare program, most of them appear to be understaffed and underfunded A survey by Feudtner et al in

2013 among 226 US children’s hospitals (of which 162 responded) showed that 112 (69% of respondents;

at least 49.6% of all children’s hospitals) have PPC program [13] However, most programs offer onlyinpatient services, and most only during the work week

A universal reality is that, overwhelmingly, in places where palliative care has not existed before,will require major cultural adaptation [10] We have adapted the following four stages, initiallydescribed by Bruera in 2004 [10], to pediatric palliative care program implementation

2.1 Stage 1: Denial

Clinical leaders suggesting the implementation of a new PPC program in a pediatric institutionare very likely to face signiﬁcant denial by clinical colleagues and hospital executives, who may not beaware of the need for a PPC program Often, there are limited or no measurement of the amount ofphysical and emotional distress suffered by children and their families with serious illness The limiteddocumentation on the need of PPC is complicated by perceptions (which are unfortunately not based

on reality) such as “Our patients here usually have very good symptom control” or “We here at ”(insert the name of any pediatric primary service or subspecialty here) “ already got it and wecover everything and don’t need an extra palliative care service.”

An important approach to expect and address denial, would be to carefully and rapidly documentthe level of unmet need in patients and families in the pediatric institution, including in the patientsunder care by individuals, who are in denial Simple surveys of uncontrolled symptoms or emotionaldistress might be extremely useful Parents’ testaments, in writing or in a short movie clip, might beparticularly helpful to overcome denial during a presentation to colleagues and leadership

2.2 Stage 2: Palliphobia

The second stage of PPC program implementation in a pediatric institution can be best described

as the recognition that there is a problem, but usually this meets consistent fear about consequences ofthe problem and the possible solutions it entails Although this might represent fear of the unknown,

it is not unheard of that sometimes people react with great anger towards it In fact, many individualscan react in negative way to developing a PPC team Physicians, nurse practitioners and other health

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professionals may feel their professional competence is being questioned or even threatened by thenew PPC team In fact, they may be afraid of referring patients to the PPC service because of concernsover criticism of their symptom control, communication or overall treatment strategies they haveused for many years Hospitals executives may be afraid of being found lacking in compassion andholistic care among all the competing priorities in a shifting landscape of pediatric healthcare andscarce funding.

Common phrases PPC teams may hear include “The parents are not ready”, “We asked them andthey said no” (although surprisingly few parents are asked for permission to include other services,such as infectious disease services),“She is not dying now”, “There is always something else we cantry”, “It is too early”, “Hospice? That’s wonderful, but that is for other people”, “You talking to thefamily means destroying hope”, “We are still ﬁghting”

Unfortunately, further exacerbation of Palliphobia can be expected, if the PPC team tries too hardand/or is too fast in making changes in patient care After a major confrontation with the neonatologist,oncologist etc (a.k.a the bull-in-the-china-shop-strategy—“move aside, the palliative care doctorhas arrived ”), the PPC usually succeeds in changing and improving analgesia, communicationstrategies or changing discharge planning, but likely will not receive further consults/referrals fromthis team or unit, resulting in a negative reputation among colleagues in the institution

A useful technique is to approach a limited number of possible referring clinicians and ask them

to become supporters and mentors of the program Once a signiﬁcant number of patients have beentreated, it is then safe to make a presentation in Grand rounds or Medical staff meetings showing theresults, ideally with the presence of the referring clinicians In absence of data, all opinions (includingpalliphobic ones) are good, but the presence of data on positive outcomes and testimonials fromthe initial team of referring clinicians can be very reassuring to those who are still uncommitted torefer patients

“Palliphobia” is more difﬁcult to overcome than “Denial”, and requires disciplined planning andrapid conﬂict resolution: Useful strategies to overcome this stage may include making great efforts atreassuring the existing clinical pediatric team that the PPC of course will work in an integrated fashioncollaboratively with them Importantly, members of the PPC team will not disqualify their patientcare plan, but rather enhance them by focusing on aspects not addressed so far “How can I help?”

is commonly a useful question posed to the primary clinician, followed by a detailed discussion ofwho does what (“Would you like me to prescribe the methadone, or do you prefer me simply givingrecommendations and you take care of it?”)

2.3 Stage 3: Pallilalia

A large number of PPC programs worldwide appear to be stuck in the 3rd stage “Pallilalia”.Usually about two to four years after implementing a PPC program, repetitive absurdities are spokenabout palliative care in general and the PPC program speciﬁcally, without anything being done toadvance its development This is in fact the most dangerous stage, as it has a high risk resulting inburnout among PPC professionals

Hospital leadership and executives describe PPC as “very important” or “a major priority”,but actually there’s no signiﬁcant allocation of personnel, space, money, curriculum time etc.Colleagues within the pediatric institution frequently talk about how useful they feel PPC is,and how happy they are with the PPC team, but as a matter of fact refer only small minority oftheir patients—usually the ones with terrible psycho-social-medical and/or mental health problems.With this attitude, a PPC program is simply ﬁnancially and administratively not viable

Children’s hospitals at this stage frequently “appoint a committee to discuss PPC”, or propose

“a major study whether PPC works in our institution” or, suggest the PPC lead applies for externalgrants, so “funds can be obtained for a pilot program in a year or two” There have also been cases,where hospital leadership decided that the 6-ﬁgure donation to the PPC program were used to offsetgeneral losses in other departments instead

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Most understaffed PPC programs (commonly lead by a 0.2 FTE physician, who was on-call 24/7and during his/her vacations), have disappeared at this stage Clinical colleagues and institutionsbecame used to the beneﬁcial presence of the PPC team, while having made no major commitment tosupport it.

A useful technique to overcome Pallilalia is to use benchmarks for clinical and time burdenthat might allow for fair comparisons While some of the referring teams may see a mix of moreand less time demanding encounters, palliative care teams always see time demanding patients andthey frequently allow the referring teams to shorten their own encounter with the patients they refer.Therefore, clinical time may be a better measure than number of encounters Since this is not alwayseasy to measure, one palliative care team compared data from parking records to determine theadditional burden on the palliative care team

It is important to anticipate this developmental stage by gathering data of provided clinicalservices, number of patients seen, details of teaching conducted, research studies, etc The PPC teamshould aim mostly at leaders of hospital and medical schools, rather than just immediate supervisors orpeers, when providing documentation of work They should request, that information be compared tooutput and resources of other programs within the institution and on a national level PPC teams mayask for external review by national or international PPC leaders in the ﬁeld Sadly, not infrequently,only through resignation of the PPC leader, institutions come to realize that PPC has been badlyunder-resourced, and start corrective measures during the process of recruiting a successor

2.4 Stage 4: Palliactive

A PPC program has reached the ﬁnal level of development, when recognized by robustappointment and funding of professional interdisciplinary team PPC members It is recommended toseek a designation of an administrative structure equal to oncology or infectious disease programs(department or division) Other important components of this stage include allocation of space, formalcurriculum space and medical training program rotations

PPC is truly recognized when colleagues actively refer patients to PPC and encourage othercolleagues to do the same Unfortunately, regression can happen at any time—frequently due tochanges in administrative leadership within the institution Also, in large children’s hospitals oncertain units is not unlikely that a robust established PPC team may encounter all four stages on thevery same day, depending on which physician they encounter

3 Common Myths and Misconceptions in Pediatric Palliative Care (PPC)

3.1 Myth 1: PPC Is Primarily for Children with a Malignancy

The majority of children dying of serious illness do not have cancer [14] In 2013 a total

of 42,328 children 0–19 years died in the United States, more than 55% (23,440) of them infantsyounger than one year [3] The leading causes of pediatric deaths include accidents (7645 children),suicide (2143), and homicide (2021) In 2015 a total of 11,933 children, adolescents and young adults0–24 years died due to a life-limiting disease: Leading conditions include congenital malformationsand chromosomal abnormalities (5965), followed by malignancies (2688), and heart disease (1354) [15].Although the prognosis for children with cancer has improved considerably over the last decades,malignancies now remains the leading cause of non-accidental death in childhood only in children olderthan one year of age In pediatric cancer units, the presence of “trigger diagnoses” (triggering automatic

referral to PPC) increased likelihood of palliative principle introduction 3.4 times (p < 0.003) [16].

That said, when providing interdisciplinary PPC services for children, it can be expected thatmost children do not have a malignancy [17] and about half of the patients would be infants Despitethe great need in neonatology, more than 45% of institution in Canada and the United States not haveneonatal comfort care guidelines, and of those reporting institutional neonatal comfort care guidelines,19.1% do not address pain symptom management [18] More than 90% of respondents in the same

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study felt that their institution would beneﬁt from further education and training in neonatal EOLcare Carter elaborates on PPC for babies in his article [19] in this special edition.

3.2 Myth 2: PPC Begins when Curative Treatments Stop

Sometimes clinicians, patients and families incorrectly assume that PPC is only appropriate whenall curative treatments are exhausted and discontinued and/or when a child is close to dying In fact,PPC is recommended to commence at diagnosis of a life-threatening disease, to continue through thetrajectory of the illness, and does not equal end-of-life care (but certainly includes it) PPC servicesextend beyond the child’s death to the family during bereavement [20] Earlier recognition by bothphysicians and parents that the child had no realistic chance of cure led to a stronger emphasis ontreatment to lessen suffering and integrate PPC in pediatric cancer patients [21]

The overall improvement in the prognosis of serious illnesses, and the emotional involvement

in trying to save the life of a child may prevent both physicians and parents from discontinuingtherapies The pursuit of such therapy modalities may overshadow attention to advanced preventionand control of distressing symptoms and to quality of life and, which unfortunately may result inincreased suffering during child’s end-of-life period However, it is sometimes not possible for parentsand/or the child to forgo further disease-directed treatment, and this should not be required in order

to achieve optimal palliative care The need to ensure that everything possible has been done may bethe only way that some parents can live and cope with their child’s death [22]

The 2010 “Concurrent Care for Children” requirement of the United States Affordable Care Acthas this at heart: The 2016 brieﬁng of the Mary J Labyak Institute for Innovation at the National Centerfor Care at the End of Life [23] described concurrent care for children with serious illness as follows:

“Until 2010, parents in all but a few states in the United States were faced with forgoing curativetreatments for their children to be eligible for hospice services Or conversely, they were not eligible forbeneﬁcial interdisciplinary hospice services while getting curative treatment The Patient Protectionand Affordable Care Act (ACA) changed that situation It requires all state Medicaid programs topay for both curative and hospice services for children under age 21 who qualify On 23 March 2010,President Obama signed ACA into law enacting a new provision, Section 2302, termed the “ConcurrentCare for Children” Requirement (CCCR) Section 2302 states that a child who is eligible for and receiveshospice care must also have all other services provided, or have payment made for, services that arerelated to the treatment of the child’s condition.1 This provision affects children who are eligible forMedicaid or the Children’s Health Insurance Program (CHIP) In its simplest form, implementation ofthis provision could be accomplished by the state Medicaid agency eliminating any provider claimsthat deny or delay concurrent curative care and hospice claims [23].”

Pediatric oncology providers in a recent survey [24] issued a highly favorable opinion about theirinstitution’s PPC service and agreed that early consultation is ideal However, they report formallyconsulting PPC is extremely difﬁcult because of what the PPC symbolizes to families and the emotionallabor that the oncology provider must manage in introducing them

3.3 Myth 3: Pediatric Palliative Care Involvement Shortens Life

Clinicians trained in palliative care will never issue a statement such as “There is nothing else

we can do” Quite the opposite, advanced PPC teams may say “there is always a lot we can do”.Even when the underlying life-limiting disease cannot be cured, sophisticated medical technology will

be utilized to improve the quality of life of the child and his and her family and to prevent and treatdistressing symptom As such, PPC is therefore a very active and advanced approach to symptommanagement and family support

It appears, that a palliative care consult for patients with serious illness is associated with longersurvival and better quality of life: In the ENABLE III study, patients who received early palliativeoncology care had signiﬁcantly longer 1-year survival rates than those who received delayed palliativecare [25] In another innovative randomized controlled trial adults with advanced lung cancer receiving

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a palliative care intervention (providing appropriate and beneﬁcial treatments) at the point of diagnosis,

in fact increased the quality of life, decreased depression, and led to a prolonged life (11.6 months vs.8.9 months) [26] These results underscore the need for palliative care early in a serious illness andrefute the notion that palliative care means giving up Patients received palliative care alongside theircurative treatment There is now emerging evidence that the inclusion of PPC specialists improves theoutcome of children with advanced serious illnesses, and sometimes represents the means to allows forcurative care through advanced symptom management provided by PPC [27] Children who receivedpediatric palliative home care were more likely to have fun (70% versus 45%) and to experience eventsthat added meaning to life (89% versus 63%) [9] In addition, families who received PPC services reportimproved communication [28] and children receiving PPC experience shorter hospitalizations andfewer emergency department visits [29] Parents of children with cancer who received PPC reportedless distress from pain, dyspnea and anxiety during the end-of-life period [8] Undertaking research inPPC is inherently difﬁcult, and Nelson et al in this special issue address this in their article [30].The advanced pain and symptom management may explain the increase of survival in patientswith serious illness enrolled into PPC Brock et al describe in this special edition describe emergingmethods of symptom and health-related quality-of-life assessment through patient-reported outcomestools [31] Data has shown, that distressing pain is very common among inpatients referred to palliativecare and three-quarters of patients with pain improve and improvement in pain is associated withother symptom improvement [32] The involvement of PPC team with adolescent and young adultoncology patients is associated with the receipt of less intensive treatments during the last month oflife, such as being on a ventilator, invasive procedures, and fewer deaths in the intensive care unit [33].Advanced pain management for children with serious illness often requires multimodal analgesia.This describes an approach of utilizing multiple analgesic agents (such as basic analgesia, opioids,adjuvant analgesia), regional anesthesia (such as nerve blocks or neuroaxial analgesia), rehabilitation(such as physical therapy, motor graded imagery), psychological (such as cognitive behavioral therapy)and integrative (formally known as “non-pharmacological”) therapies (such as massage, hypnosis)which usually act synergistically for more effective pediatric pain control with fewer side effects than

a single analgesic or modality [34–37]

Harlow and Hain [38] explore the concept of total pain in the largest group of children withlife-limiting diseases, pediatric patients with severe neurological impairment

3.4 Myth 4: PPC Can Only Be Provided Comprehensively within a Children’s Hospital

Currently most children in resource rich countries die in a hospital, most commonly in an intensivecare unit In a recent study [39] reviewing all pediatric intensive care unit (PICU) admissions over

15 months of 89,127 children in the United Kingdom, children with life-limiting conditions constituted57.6% of all admissions Of the 4821 children who died on the PICU during that period, 72.9% had

a life-limiting condition Since most of the sickest children with serious illness are being taken care

of in a hospital, every children’s hospital is now expected to offer an interdisciplinary palliative careservice as the standard of care [40]

One of the early PPC pioneers, Dr Ann Goldman from the Department of Hematology/Oncology

at Great Ormond Street in London, UK implemented a “Symptom Care Team,” a team of nurses,who were introduced at cancer diagnosis to the child and family All children received homevisits after their ﬁrst discharge Children with high-risk cancer or relapses then already knew the

“Symptom Care Team”, which provided a 24/7 service, from the time of diagnosis Between 1978–1981,before the implementation of the “Symptom Care Team” only 19% of children with cancer died athome Then, between 1989–1990, after implementation of the team 77% of the children dying frommalignancies did so at home [41]

The death of a child with a serious illness at home may promote better family adjustment andhealing [42,43] This could be related to decrease helplessness and increased family intimacy by being

at home On the other hand, some have reported that family relationships appeared to be better when

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the child died in the hospital [44] While it is often suggested that most children prefer to die at home,this has actually not been systematically evaluated.

Parents of terminally ill children often wish for home care [45,46], and there is a not surprisingly

a positive correlation between availability of palliative home care and the number of children dying athome [9,47–49] The interdisciplinary PPC Team involvement in compassionate extubation at homehas been explored by Postier et al in this special issue [50] Most families regard caring for theirdying child as a positive experience [45] It is critically important to discuss preferences regarding theprimary location of care as early as possible A parental decision to care for their terminally ill child athome involves consideration of medical, psychological, social and cultural factors together with suchpractical considerations as the availability of respite care, physician access, and ﬁnancial resources.Children with advanced cancer who also received PPC home care or hospice were signiﬁcantly morelikely to have fun (70% versus 45%), experience events that added meaning to life (89% versus 63%),and to die at home (93% versus 20%) [9] Whatever the decision is regarding the primary location

of care, families should be reassured that they can change from one option to another and that theprimary team will remain closely involved [45]

There appears to be a growing consensus of most pediatric palliative care specialists, that advancedPPC for children with serious illness needs to be offered and coordinated primarily by aninterdisciplinary team within tertiary pediatric hospitals, aided by an outpatient PPC clinic andmust include a palliative home care service [51] When these services are in place (but not instead),

a freestanding hospice and respite house represents an excellent addition (but not substitution) of theservices provided inpatient, in clinic, and at home The World Health Organization (WHO) describesthat palliative care can be provided in tertiary care facilities, in outpatient clinics, in community healthand hospice centers, and in children’s homes [52]

A key component of family support, in addition to addressing the needs of the sick child [53],must be geared toward the siblings [54,55] and parents/caregivers [56]

3.5 Myth 5: Patients and Their Parents Need to Make a Choice between “Fighting for a Cure” or “to Give

up Hope”

Even when there might not be a realistic hope for cure, pediatric patients with a serious illnessand their parents may opt for continued disease directed treatment [21] This might be motivated bythe desire to extend life, to palliate symptoms related to progressive disease, or the hope for a miracle

In discussions of treatment options with families, one might choose statements, as suggested by Wolfe,such as “The very nature of miracles is that they are rare However, we have seen miracles, and theyhave occurred both on and off treatment” [57] In other words, a child does not have to continue ondisease-directed therapy in order to preserve hope, especially when the therapy signiﬁcantly impactsthe child’s remaining quality of life In fact, a large adult study could demonstrate that chemotherapyfor end-stage cancer does not prolong life, however reduces the quality of life [58]

Caring for a child at end-of-life is emotionally very difﬁcult It may be particularly challengingfor clinicians and parents to consider the early integration of PPC because this may be perceived

as ‘giving up’ As a result, the emotional cost of the recognition that a child may die could impedeplanning for optimal pain and symptom management and psycho-social-spiritual support In fact,the “hope for cure” and “pediatric palliative care” include and complement each other PPC translatesinto advanced management to maintain or improve quality of life and children can graduate fromPPC No matter the treatment goals, despite the prevailing myth, disease-directed care and excellentsymptom relief must and can be provided simultaneously

Date has shown that the integration of PPC explicitly does not result in giving up hope Engaging

in advanced care planning increases the patients knowledge without diminishing hope, increasinghopelessness, nor inducing anxiety in patients with advanced cancer [59] The disclosure of a terminalprognosis does not mean loss of patient hope: Instead, hope was redeﬁned on a goal other thancure [60] In pediatrics, there is no evidence that prognostic disclosure makes parents less hopeful

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Instead, the disclosure of a prognosis by physician can support hope, even when prognosis is poor [61].Parents who are upset by prognostic information are no less likely to want it The upsetting nature

of prognostic information does not diminish parents’ desire for such information, its importance todecision making, or parents’ sense of hope [62]

During a goals of care discussion with a family of child with a serious illness, a question such as

“Considering what your child is up against, what are you hoping for?” may be posed by the clinician.Not surprisingly, the response may be “Cure from the disease” or “A miracle” The clinician maythen respond “I hope this too Just in case the disease cannot be cured—What else are you hopingfor?” By exploring the extend of hope further, families may wish for very advanced treatment andprevention of pain and distressing symptoms, the possibility to go home, for grandmother to visit,

to hold their child more often, or many other things Blazin et al in this special edition explore how totranslate evidence into practice and communicate effectively [30] Even when the underlying conditioncannot be cured, PPC will never give up hope Sometimes, it appears that the best chance for thesechildren to truly live is for their parents and treating clinicians to accept the fact that this child mightdie [63] Not surprisingly, data reveals, that religion, spirituality or life-philosophy play an importantrole in the life of most parents whose children receiving PPC [64]

3.6 Myth 6: Administering Morphine Causes Respiratory Depression and Hastens Death

To paraphrase Sykes [65] “Morphine kills the pain, not the patient A physician killing a patient

in name of pain or dyspnea relief is not merciful, just incompetent.” An enduring misconception isthe belief that in the management of pain and dyspnea, opioids will hasten death and should only

be administered as a last resort This was contradicted in the adult literature [66] and our PPC teamscommonly observe that administering opioids and/or benzodiazepines, together with comfort care

to relieve dyspnea and pain, not only improves the child’s quality of life, but also prolongs life [67]

A retrospective cohort study (n = 223 adult oncologic patients) reviewing the mean survival in relation

to opioid use found less than a two-fold increase in their initial opioid dose resulted in 9 days survival,and more than a two-fold increase in 22 days survival [68]

One of the most common sources of pain and distress in children is visceral hyperalgesia andfeeding intolerance, addressed by Hauer in this Special Issue [69]

3.7 Myth 7: PPC Teams Are Too Expensive

A pediatric palliative care team is now expected standard of practice in every children’s hospital.Every single one of the “U.S News & World Report honor roll children’s hospitals” have palliativecare teams [70] Overwhelming evidence now demonstrates, that children’s hospitals in fact cannot

afford to not have a PPC team anymore PPC team basically “grease the wheels” of a clinical institution,

such as reducing burnout and as a result staff turn-over among pediatric staff For instance, in theUnited States more than 30% of all new nurses elect either to change positions or leaving nursingcompletely within the ﬁrst 3 years of clinical practice [71] Leading reasons include emotional distressrelated to patient care The average cost of turnover for a bedside nurse ranges from $36,900 to $57,300,resulting in the average hospital losing $4.9 million–$7.6 million per year Each percent reduction innursing turnover will cost/safe the average hospital an additional $379,500 [72] PPC services can helpreducing turnover and improving overall job satisfaction and performance important in to assuringpatient and family satisfaction while promoting quality care

In addition to providing advanced clinical care, pediatric palliative care services are alsocost-effective (although arguably not a single PPC team member worldwide provides clinical care

“to safe money” to the institutions or health insurers, but rather, because it provides superior clinicalcare to children with advanced serious illnesses PPC involvement results in better outcomes andlower costs: Palliative care program reduces stress, costs of care for children with life-threateningconditions [73–75] A retrospective study of 425 children receiving pediatric palliative home care inthe US comparing pediatric hospital resource utilization before and after PPC enrollment decreased in

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non-cancer patients the length-of-hospital-stay by 38 days and decreased hospital charges $ 275,000per patient [76].

4 Conclusions

Despite signiﬁcant growth and advances over the last two decades, a large number of children’shospitals currently either do not have a PPC service, or it signiﬁcantly under-resourced andunderfunded When implementing a PPC service, the development often goes through four steps intheir development These should be anticipated, and an action plan might include

external review

payments environment; include clinical and administrative innovators in program development

High-quality pediatric palliative care for children with serious illnesses is now an expected standard

of medicine However, even in resource-rich settings, there remain significant barriers to achievingoptimal care related to lack of formal education, reimbursement issues, the emotional impact ofcaring for a dying child, and most importantly the lack of interdisciplinary PPC teams with sufficientstaffing Whenever possible, treatment should focus on continued efforts to control the underlyingillness At the same time, children and their families should have access to interdisciplinary careaimed at promoting optimal physical, psychological and spiritual wellbeing Persistent myths andmisconceptions have led to inadequate symptom control in children with life-limiting diseases.Pediatric Palliative Care advocates the provision of comfort care, pain, and symptom managementconcurrently with disease-directed treatments Families no longer have to opt for one They can pursueboth, and include integrative care to maximize the child’s quality of life

Conﬂicts of Interest: The authors declare no conﬂict of interest.

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2 Children’s Mercy Hospital, Kansas City, MO 64108, USA

3 Division of Neonatology and Bioethics Center, 2401 Gillham Road, Kansas City, MO 64108, USA

Received: 7 December 2017; Accepted: 1 February 2018; Published: 7 February 2018

Abstract: The application of palliative and hospice care to newborns in the neonatal intensive care unit

(NICU) has been evident for over 30 years This article addresses the history, current considerations,and anticipated future needs for palliative and hospice care in the NICU, and is based on recentliterature review Neonatologists have long managed the entirety of many newborns’ short lives,given the relatively high mortality rates associated with prematurity and birth defects, but their ability

or willingness to comprehensively address of the continuum of interdisciplinary palliative, end of life,and bereavement care has varied widely While neonatology service capacity has grown worldwideduring this time, so has attention to pediatric palliative care generally, and neonatal-perinatalpalliative care speciﬁcally Improvements have occurred in family-centered care, communication,pain assessment and management, and bereavement There remains a need to integrate palliative carewith intensive care rather than await its application solely at the terminal phase of a young infant’slife—when s/he is imminently dying Future considerations for applying neonatal palliative careinclude its integration into fetal diagnostic management, the developing era of genomic medicine,and expanding research into palliative care models and practices in the NICU

Keywords: neonatal; palliative care; comfort care; pain

1 Introduction

The provision of palliative or hospice care for newborn infants was ﬁrst introduced in the 1980s

At the time, hospice principles were being disseminated in the US and their applicability to certainnewborns and young infants was noted by Whitfield et al [1] and by Silverman [2] Since 1982,there has been increased growth in the field of palliative care in general, and in both pediatric andneonatal palliative care specifically Indeed, the specialty of palliative and hospice medicine has gainedrecognition in North America and internationally with the attendant growth of training programs inboth the clinical and academic arenas

2 Materials and Methods

A brief, but pertinent, review of the past 10 years’ clinical literature was conducted to examinethe breadth of neonatal palliative care as it is currently practiced Throughout this process,additional clinical needs, potential research ideas, and future considerations were determined and areherein addressed

3 Results

As the specialty of neonatology has grown around the world in the last three decades, the rolefor palliative care (PC) has likewise been recognized in the neonatal intensive care unit (NICU) andpediatric intensive care unit (ICU)—both common locales for neonatal and infant mortality [3 5].Over the past 15 years, marked improvements have occurred around the circumstances in which

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newborn and young infants die in North American hospitals There has been attention drawn

to patient needs such as pain and symptom management [6,7], spiritual support [8], honoringcultural practices [5], understanding grief [9], and employing a breadth of bereavement activities andservices [10,11]

Some particular features of providing PC for newborns and young infants include how pregnancy

is unique among human experiences and the veritable uniqueness of each pregnancy—and child—fromall others Mothers and fathers generally enter into pregnancy with anticipation and hopefulness,having a future-oriented idea of their yet to be born infant That a newborn may be critically ill,extremely premature, or born with signiﬁcant birth anomalies that threaten his/her life and wellbeing

is never truly anticipated When discovered—at birth or even beforehand with fetal diagnostics—thepregnancy generally takes a dramatic turn as hopeful anticipation is replaced by fear, joy may beeclipsed by guilt, and the experience of pregnancy and childbirth becomes medicalized—often withobsessive thoughts surrounding each clinic visit, imaging study, or test In addition to cure-orientedand life-extending neonatal intensive care, the provision of concurrent PC may provide supportivecare for the patient and family, and may help in decision-making [12,13]

As is true in other areas of pediatric PC, neonatal PC has had a somewhat divided history In theNICU, there have long been a number of patients who have been treated for weeks to months only toreach a plateau or stagnation in their progress toward growth and healing They remain ventilated,perhaps dependent upon intravenous nutrition, have endured infections and maybe bowel problems,their livers are impacted by cholestasis, and they may even have parenteral nutrition-associated liverdisease Some have endured brain injury Each day their care is challenging, and on any given day theymight have an acute decompensation for which escalating support is required—often accompanied byanalgesia and/or sedation to minimize cardiopulmonary instability or neurologic agitation Theseinfants may further decompensate or develop secondary pulmonary arterial hypertension alongwith their bronchopulmonary dysplasia (BPD) Any given infection may take their life For theseinfants—some of whom may linger, and others may in fact be dying—neonatal PC may be an adjunctivecare paradigm that is added to their continued intensive care For some, the cure-oriented care mayyield to a palliative paradigm after intensive counseling of parents, exploration of options with theinterdisciplinary neonatal team, and thoughtful reﬂection Psychosocial support for the family isessential and may attend to anticipatory grief; spiritual support may increase, and a decision tolimit ongoing life-support may result in a mutually agreed upon redirection of care towards comfortwith a reduction in vital-sign and invasive monitoring, phlebotomies, and imaging tests In time,

a compassionate withdrawal of life-supportive technology is performed, and caregivers work withfamilies to orchestrate a meaningful time with extended family, rituals, and allow for the infant’spassing In such cases, a period of focused palliation culminates in veritable hospice care in the NICU,typically associated with the withdrawal of life-sustaining medical treatments

As neonatal palliative care has developed, and an expanding literature is at the disposal ofclinicians (Figure1), there has been greater consideration for PC in the NICU In recent years,especially as prenatal diagnostics have improved and life-limiting conditions are diagnosed prenatally,some newborn infants will receive concurrent palliative care (even beginning with conversationsbefore birth) while being cared for in the NICU [14–16] The care may be oriented toward conﬁrming aprenatal diagnosis and exploring care options, or be directed at the baby’s comfort while not taking

on intensive or invasive technological care In the latter situation, time with parents, human contact,warmth, and symptom relief may be the predominant mode of support [17] For others, such as

a newborn with hypoplastic left heart syndrome, there may be intensive care provided while theneonatal, cardiology, and cardiovascular surgical specialists confer about palliative surgical options

or perhaps limits or confounders (such as prematurity, additional birth defects, or the severity ofanatomical size constraints that make surgery more difﬁcult) [18] When concurrent palliative care

is made available, the added value of an interdisciplinary team, psycho-social-spiritual support,and expert pain and symptom management are acknowledged as enhancing the baby’s and family’s

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quality of life even while cure-oriented or disease-modifying treatments are offered The focus is onthe quality of life and relationships while living with a life-limiting condition [19,20].

34 30

24 3531

14 7

13

19 13

Figure 1 Increasing publications addressing neonatal palliative care.

A working deﬁnition for neonatal palliative care can be found from the organization Together for Short Lives, a children’s palliative care group in the UK:

“Palliative care for a fetus, neonate, or infant with a life-limiting condition is an active andtotal approach to care, from the point of diagnosis or recognition, throughout the child’s life,

at the time of death and beyond It embraces physical, emotional, social, and spiritualelements and focuses on the enhancement of quality of life for the neonatal infant andsupport for the family It includes the management of distressing symptoms, the provision

of short breaks, and care through death and bereavement” [21]

Note here that this involves far more than care of the imminently dying newborn Indeed,the many domains of PC outlined by Ferrell in 2005 [22] are present and require consideration and

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action in the neonatal and young infant population as much as they do in adults or older children [21].

In recent years, referrals for PC from the perinatal and newborn period have often been the startingpoint for long-term follow-up of children with complex chronic conditions and special health careneeds by PC clinicians long after the infant has left the NICU

The types of patients for whom PC may be anticipated and offered in the newborn period aregenerally in one of three categories: those born at the threshold of viability or who are similarlyvulnerable by virtue of prematurity, those with birth anomalies that may threaten vital functions,and those for whom intensive care has been appropriately applied but are now burdened withinterventions that no longer are deemed beneficial, but come to be seen as burdensome, inappropriate,and only prolonging the infant’s dying There are guidelines published from the National Institute

of Nursing Research (NINR), National Association of Nurse Practitioners, the National PerinatalAssociation (NPA), the National Association of Neonatal Nurses (NANN), the National Hospice andPalliative Care Organization (NHPCO), and the Center to Advance Palliative Care (www.capc.org) thatspeak to these categories and potential triggers for neonatal PC [23–27] For some of the commonlyincluded conditions (below) a diagnosis may arise in the neonatal period and PC care may beinitiated, whereas others may lead to a more long-term follow-up with PC as an adjunct to other ICUfollow-up services:

• Genetic/chromosomal: Chromosomal aneuploidies with complex and life-limiting prognoses;severe metabolic, storage, or mitochondrial disorders; severe forms of skeletal dysplasia

• Organ-system problems: Severe central nervous system (CNS) malformations (neural tube defects,migrational disorders); hypoxic-ischemic encephalopathy; spinal muscular atrophy type-1 andmyotonic dystrophies; epidermolysis bullosa; Potter’s syndrome, fetal oligohydramnios sequence,fetal-neonatal chronic renal failure; short-gut syndrome with parenteral nutrition dependence;multi-visceral organ transplant under consideration (e.g., liver, bowel, pancreas); biliary atresia;total aganglionosis of the bowel; severe feeding impairment with feeding tube dependence thatmay be permanent; complex congenital heart disease, especially if functionally univentricular;extracorporeal membrane oxygenation (ECMO) patients; severe pulmonary arterial hypertension;consideration for heart transplant; congenital diaphragmatic hernia; severe pulmonary hypoplasia;congenital central hypoventilation syndrome; asphyxiating thoracic dystrophies; multi-organsystem failure

• Infection and immune disorders: Perinatal human immunodeficiency virus infection and acquiredimmune deficiency syndrome (HIV/AIDS); severe combined immune deficiency (SCID); severeperinatal herpes simplex virus (HSV), cytomegalovirus (CMV), toxoplasmosis or Zika virus withmeningoencephalitis or severe encephalopathy

• Complications of prematurity: Periviable gestation; severe intraventricular hemorrhage(IVH, grade IV) or periventricular leukomalacia (PVL); refractory respiratory failure;ventilator-dependent BPD; severe necrotizing enterocolitis (NEC) with resultant short gut;liver failure

4 Discussion

Today, newborns and young infants may receive PC that is initiated prenatally, introduced

at delivery, or acquired in a consultative manner in the NICU The acceptance of this paradigm

of care, and the capacity for it to be rendered as needed, however, varies across units of care andinternationally A number of investigators have spoken to barriers to neonatal-perinatal PC such assuboptimal interdisciplinary collaboration in hospitals, unacceptance of PC broadly in society andspeciﬁcally as it applies to children, poor clinician communication skills, and prognostic uncertaintiesamong others [28–31] Clinicians today must strive to advance PC education, employ successfulmodels of neonatal-perinatal PC, and incorporate PC competencies into those considered fundamental

in neonatal-perinatal professional education [15,32–36]

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Looking ahead, I will outline what I see as three major considerations for the ﬁeld of PC as

it pertains to pregnancy, fetal, and neonatal patients in the next decade First, given the improveddiagnostic capabilities present with maternal-fetal imaging, and the expansion of fetal diagnosticand intervention centers, the integration of PC with prenatal counseling will likely increase For thepediatric PC specialist familiar with neonatal-perinatal care, this will not necessarily pose concerns.However, for those unfamiliar with neonatal-perinatal care (e.g., a pediatric oncologist who has beentrained in PC, or a family physician now boarded in Hospice and Palliative Medicine) there may be alearning curve to ramp up capabilities to address the often unique aspects of pregnancy and childbirthcomplicated by life-limiting conditions [37–39]

In such instances where a palliative medicine consultant has little experience with newborns,

it will be beneficial for neonatologists, pediatricians, and hospitalist pediatricians to be identified aslocal “champions” for neonatal PC within a given NICU, academic faculty, or practice group The NICUchampion will likely work with unit-specific interdisciplinary staff and the local PC-trained physician

or nurse practitioner to effect well-coordinated and effective PC in the NICU The coordination oftransitional care from the NICU to home-based hospice care, an inpatient hospice-house or facility,

or even outpatient management anticipated to be beneﬁcial for months to years that is shared with aprimary care pediatrician, specialty clinics, and interim contact with the PC team ﬁrst met in the NICU,

is of added value to both NICU staff and patient families

Related to this ﬁrst consideration is the expansion of fetal and neonatal diagnostic evaluationswith genomic medicine to better understand or explain multiple anomalies, metabolic disorders,

or complex central nervous system dysfunction [40–43] I foresee that pediatric (and neonatal)clinicians in general, and perhaps PC clinicians speciﬁcally, will need to develop a facility withboth new diagnostic genetic tests and the cautious compilation of new diagnostic groups with yet

to be determined prognoses While some such cases may be treatable with present regimens, othersmay require yet to be developed, personalized pharmacogenomics-derived treatments In the prenatalsetting, genetic/genomic diagnoses that can be made from chorionic villous sampling, amniocentesis,fetal blood sampling, or even non-invasive maternal (blood) testing will likely accompany phenotypicdiagnoses seen on imaging These developments will impose the need for expanded perinatalcounseling and open doors for PC clinicians to join neonatologists, geneticists, and maternal-fetalmedicine specialists in contributing to perinatal and neonatal decision-making Already, we see thepossibility of expanded genome-wide newborn screening being proposed in the care of all newborns,and even if this is constrained to symptom-driven circumstances, the need to address prognosticuncertainty will call upon the superlative communication skills of trained and informed neonatologistsand PC clinicians [37–39,44]

As a second consideration, PC material is increasingly being incorporated into the training andeducation of neonatologists This will likely result in an enhanced momentum for PC services to bebetter integrated into the newborn’s care concurrently with cure-oriented or life-prolonging critical care.Progress has been made in the establishment of PC teams in children’s hospitals in North America.However, due to their variable stafﬁng, composition, resource priority, and the limited number offellowship-trained PC specialists, the ability for these teams to concurrently provide PC throughout

a large children’s hospital and in the NICU may be limited This too provides an opportunity forneonatologists to become local champions for PC in their NICU—and in so doing building the capacityfor PC as they partner with PC team members—until capacity is increased with more fellowship-trainedpediatric or neonatal PC specialists Given the longstanding interdisciplinary nature of the care team inthe NICU, unit-speciﬁc neonatal nurse practitioners who may be PC champions, respiratory therapists,perinatal social workers, nurses, and others can work with chaplains, psychologists, and child-lifespecialists to create NICU-PC teams that address unit needs with input and assistance from pediatric

PC specialists elsewhere in the hospital Together these complimentary and concurrent teams can see

to patients, family, and staff needs [19,37–39,44,45]

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A third consideration that I would highlight in the next 10 years of PC provided for newborns

is the need to conduct research, qualitative and empirical, that can advance the derivation of bestpractice models for neonatal PC [15,32–36] Such research will advance the evidence base for theﬁeld, and likely result in increasing acceptance and utilization of PC in the neonatal-perinatal world.Investigations might address symptom assessment and management skills; studying commonly used,but poorly evaluated medications; looking further into the barriers and facilitators of PC in theNICU across staff/disciplines [28–31,39,44,45]; and evaluating effective communication strategiesand decision-support for families and staff [46,47] The utilization of large neonatal populationdatabases to make inquiries and construct studies to answer common problems would seem to holdgreat potential [48] What are the best ways to manage common symptoms? How should ventilatorwithdrawal be accomplished [49]? What do parents think about the limitation or withdrawal ofmedically assisted nutrition and hydration [50]? How can neonatologists, maternal-fetal medicinephysicians, geneticists, and others best inform a pregnant couple of unexpected ﬁndings on fetalimaging or address prognosis in the NICU [51]? Would a complimentary presence of an ethicsconsultant add value anywhere along the path from diagnosis to treatment decisions or life-sustainingmedical treatment withdrawal [52,53]?

or when being too premature and too small—or fraught with too many anomalies—confronts familiesand clinicians with the limits of medicine, PC should be at the ready (or already have been incorporatedinto a concurrent care model) to assist all stakeholders I believe that the foundation for the integration

of PC into the NICU is set, the need is present, and the progress made over the past 15 years speaks

to a bright future in the practice of humane, ethical, and family-centered care for our world’s tiniestand most vulnerable patients We can meet the challenges outlined here and others with continuedefforts, well-trained young leaders, forthcoming research, appropriate advocacy, and local hospitaland university support

Conﬂicts of Interest: The author declares no conﬂict of interest.

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Review

Pediatric Palliative Care for Children with

Progressive Non-Malignant Diseases

Harold Siden

Canuck Place Children’s Hospice, BC Children’s Hospital, University of British Columbia, Vancouver,

BC V6H 3N1, Canada; hsiden@cw.bc.ca; Tel.: +1-604-875-2776

Received: 22 December 2017; Accepted: 12 February 2018; Published: 20 February 2018

Abstract: A substantial number of children cared for by pediatric palliative care physicians have

progressive non-malignant conditions Some elements of their care overlap with care for childrenwith cancer while other elements, especially prognosis and trajectory, have nuanced differences.This article reviews the population, physical-emotional and social concerns, and trajectory

Keywords: palliative care; palliative medicine; terminal care; hospice care; metabolic diseases; inborn

genetic diseases; social support; disease progression; symptom management; emotional support

1 Introduction

In industrialized countries the majority of children cared for in Pediatric Palliative Care (PPC)programs have non-malignant diseases other than cancer This includes a very broad range ofconditions affecting the brain, the muscles, the heart and lungs Infrequently there are infectiousand immunologic conditions

In North America cancer comprises only 30–40% of the cases seen by pediatric palliative careclinical teams This is a testimony to the overall rarity of childhood cancer and to the overarchingsuccess of current treatments The most common of all the childhood cancers, acute lymphoblasticleukemia, also has the highest 5-year event-free survival rate, at over 90%

Cancer is widely identiﬁed with palliative care in part for historic reasons The ﬁrst modernhospice, St Christopher’s in London, was founded by a medical oncologist, Dame Cicely Saunders

A surgical oncologist, Balfour Mount, coined the term “palliative care” It was not until some 15 yearslater that the ﬁrst hospice for children was developed by Sister Francis Dominica with Helen House,Oxford Helen, for whom the program is named, indeed had cancer, however it was the centralnervous system sequelae of that tumor that made her a candidate for palliative care [1]

This article will describe the broad epidemiology of the non-cancer conditions seen by PPCclinicians, provide insights into aspects of care, and point out areas needing focused research

2 Terminology and Classiﬁcation

In order to understand the approach to conditions other than cancer followed by PPC teams,

it helps to understand terminology and current classiﬁcation schemes All of these are continuouslyevolving as we come to understand the nuances of PPC

One area of terminology that has led to some confusion is an ongoing discussion over the terms

“life-limiting” and “life-threatening”, as different groups use the terms differently [2] The focus

of pediatric palliative care has long been ameliorating symptoms, maintaining good quality of life,and supporting families when a child has a condition that is highly likely to end in a prematuredeath at any time prior to adulthood When programs, clinicians and researchers use terms such aslife-threatening, life-limiting, or any other, they simply need to be explicit about the term they are

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using and how it is deﬁned This approach will be more productive than attempting to ﬁnd the singleunifying language, given that all of them are constructs.

The widely cited report of the U.K charity Together for Short Lives (previously ACT) and theRoyal College of Pediatrics and Child Health identiﬁed 4 groups of diseases that may receive PPCservices [3] The ﬁrst of the 4 categories were those diseases where cure was generally possiblebut for an individual patient failed Fortunately, the number of curable conditions is growing.The most prominent in this category is Cancer of course (congenital heart disease being a closesecond) This points out that the conditions seen in the other 3 categories make up the majority ofdiseases seen in PPC Category 2 are conditions whereby cure is not possible, but treatments directed

at the underlying disease pathophysiology greatly prolong the life-span and enable good quality

of life for a long period of time The classical example is cystic fibrosis HIV, if full-treatment isavailable, is another example In the past few years an increasing number of gene-based metabolicconditions, such as some lysosomal storage diseases, have moved into Category 2 Many of thegene-based metabolic and neurological diseases remain in Category 3 In this category, there is no cure,and no direct treatment for the disease The focus is on treating manifestations of the disease, such asseizures, inadequate nutrition, secondary osteopenia, etc The interventions for these conditionsnamely anti-convulsants, gastrostomy feeding, bisphosphonate infusion, all improve the quality oflife and arguably prolong life They do not, however, stop disease progression Fortunately, as cannow be seen with some storage diseases, and most recently with Spinal Muscular Atrophy, medicaladvances move diseases from Category 3 to Category 2 Lastly, Category 4 contains neurologicalconditions secondary to static encephalopathy In this category we find children who have experiencedhypoxic-ischemic brain injury, as well as those with congenital brain dysgenesis Until we find ways todirectly repair injured brains, the focus for care of these individuals is on symptom management andquality of life

Another lens is to use a simple classiﬁcation scheme of 7 categories of diseases seen in PPC;breaking them down into: Cancer; Primary CNS conditions; Biochemical/Metabolic diseases;Neuromuscular diseases; Cardiac and Pulmonary conditions; Infectious diseases and Immunologicdiseases; Multi-Organ conditions due to chromosomal aneuploidy or gene defects This is a simpliﬁed,practical 7 category system used for internal reporting purposes at Canuck Place Children’s Hospicethat has been found useful as a descriptive tool for the estimated 170 different diseases found amongthe children seen in the program [4] Others include the system developed by Richard Hain andcolleagues, as well as the Complex Chronic Conditions categorization developed by Chris Feudtnerand colleagues [5,6] These categorical systems enable researchers and health services planners to lookfor trends and evaluate different approaches to treatment

3 Symptom Management

The principles and practice of symptom management with non-cancer conditions are similar

to those for cancer, although with some nuanced differences Attention to these differences may beimportant [7] Much of what we know about symptoms in these children derives from our study of

275 children with non-curable, non-cancer conditions In these children we tracked 7 symptoms: pain,dyspnea, nausea and vomiting, dysomnia, constipation, seizures, and changes in alertness Strikingly

on average, their parents reported 3.2 symptoms of concern for each child [8]

3.1 Pain

When discussing symptoms in PPC, attention is immediately paid to pain Most non-cancerconditions can have pain, but generally not due to the generalized inﬂammation or space-occupyinglesions found in cancer, nor with the marked, ongoing escalation [8,9] Sources of pain includemusculoskeletal pain secondary to weakness, bone and joint conditions, and contractures Skin andorgan pain (due to enlargement) can be found in some genetic metabolic diseases Pain secondary toprocedures and interventions is a problem Visceral pain, especially gastro-intestinal pain arising all

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along the alimentary tract can be found in almost all conditions, especially if associated with non-oralfeeding, reﬂux disease and constipation Neuropathic pain can arise from neuropathy or in associationwith long-standing nociceptive, inﬂammatory pain Lastly, children with central nervous systemconditions can also be affected by a “central” pain or neuro-irritability that does not seem to have

a nociceptive-inﬂammatory source [10,11] This condition is challenging to diagnose and to manage;

it is an area of active research

Treating pain in these children, especially those who are very young or are non-verbal, requirespatience, sometimes tenacity, and attention to detail [12] One must search for readily treatable causessuch as hip dysplasia, chronic constipation, or poorly adjusted seating systems [9] When no cause isfound clinicians should pursue a screening approach that takes into account silent conditions, such asrenal stones, especially with a young or non-verbal patient In this circumstance pain is relieved whenthe underlying cause is directly addressed The treatment of pain (and many other symptoms described

in this article) often requires the input of many disciplines Medical and surgical interventions are onlytwo components The involvement of physiotherapists, occupational therapists, and speech-languagepathologists is often required to address pain and/or other symptoms

If no cause is identified, then families and clinicians are faced with “pain-like” behaviors thatlook like what happens with nociception or inflammation but may be induced by activation ofinternal central nervous circuits alone One then undertakes empiric treatment with environmentalmodifications (e.g., swaddling, massage) and with medications (e.g., gabapentin trial) [13] A step-wise,trial and error approach using validated assessment tools is needed, along with close communicationbetween clinicians and patients/family members The nature of this central pain—irritability andapproaches to its treatment are areas of active research

3.2 Dysomnia

The second most common symptom in our study was dysomnia Disturbances of sleep inchildren can be multi-dimensional and include problems with delayed onset, frequent awakeningwith fragmented sleep, inadequate sleep duration, and daytime sleepiness (so called “day-nightreversal”) For some children many of these features are mixed together Singly or combined theycan pose a major challenge to the child’s health and to the family’s overall wellbeing They are verycommon in children with neurological conditions, comprising a large number of the children followed

in PPC [14,15] There are many potential causes that need to be considered in the evaluation includingphysical health conditions such as upper airway obstruction; poorly-treated pain leading to poor sleep,and environmental factors Some conditions, such as Rett Syndrome and MucopolysaccharidosisType III are well known to have sleep disturbances, probably on a primary neurological basis [16–18].Treatment is highly empirical, and evidence is mostly based on either surveys of currentpractices (by both clinicians and parents), or on case reports There is a widespread agreementthat environmental and behavioral interventions are worth under-taking Examples include structuredbedtime routines, dark and quiet rooms, avoiding pre-bedtime stimulation with videos The drawback

is that such interventions need to be individualized for each patient’s particular circumstance andtherefore it can be time-consuming to ﬁnd the interventions that work best Similarly, there needs

to be consistency in implementing them There is evidence, albeit not very strong, for the use ofmelatonin [14] Melatonin appears to assist with sleep onset but may lead to a shortened total duration

of sleep; using a combination of immediate-acting and long-acting melatonin may avoid this problem.The environmental interventions and melatonin are considered to be safe Other medications usedare a wide variety of drugs with sedation effects—these include anti-histamines, chloral hydrateand benzodiazepines Chloral hydrate’s only indication is sedation but there are concerns about itslong-term use and side effects, especially the effect on the liver The other medications, as well as

a variety of anti-depressants and anti-psychotics, are all sedating but sedation/hypnosis is not theirmain indication Specialists however ﬁnd them useful, and the non-expert prescriber should becomefamiliar with their use and side effects and not hesitate to ask for expert input Almost every review of

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dysomnia in children with severe diseases emphasizes the need for family support, especially practicalsupport such as having a night-time nurse or sitter.

3.3 Feeding Difﬁculties and Constipation

In children with non-cancer, life-threatening conditions the 3rd and 4th most common troublingsymptoms were feeding difficulties and constipation [8] The evaluation and treatment of feedingdifficulties in children who receive artificial enteral nutrition has been well described Similarly,there are a number of approaches to treating constipation that can be considered There are two pointsworth noting: one is that treating feeding intolerance requires a step-wise, patient approach Similarly,treating constipation involves more than simply prescribing a stool softener and assuming the problem

is resolved It requires ongoing assessment of the situation The second is that in some circumstancesfeeding intolerance, especially intolerance of elemental formulas and electrolyte solutions in theabsence of signs of malabsorption, may be a harbinger of terminal decline This has been reported butnot widely studied [19]

Due to the preponderance of neurological disorders found in children with non-cancer diagnoses,artificial enteral nutrition is a common feeding approach Not only can these children have difficultiessecondary to reflux, delayed gastric emptying and constipation, but their parents also report a higherprevalence of pain and respiratory problems Pain may be a component of the gastrointestinalsymptoms, or another manifestation of the overall disease condition Respiratory difficulties againmay be due to reflux, perhaps exacerbated by the addition of a feeding tube, or it may be se secondary

to inadequate airway protection and ongoing aspiration of saliva and bronchial secretions In somecenters this may be an indication for tracheostomy, but it has not been the practice at our institution

3.4 Dyspnea

Dyspnea, the sensation of breathlessness, was also found in our longitudinal study Dyspnea is

a symptom best studied in the context of adult cancers, especially those involving the lungs, the airway,

or the muscle system While the sensation is well described, there are few commonly used tools toassess it, and a minimal literature on treatment—the literature emphasizes the use of airﬂow acrossthe face, and opioids to alter the CO2ventilation threshold so that there is reduced stimulus towardscompensatory hyperventilation

Dyspnea is primarily understood in the context of adult cancer and cardio-respiratory medicine.Scales have been developed in children, such as the Dalhousie Dyspnea Scales [20] These scales weredeveloped with verbal, cooperative children over 8 years of age, in situations such as severe asthma andcystic ﬁbrosis There has not been a similar scale developed for non-verbal, non-cooperating children.Overall there is a paucity of research [21] Clinically there is a challenge in determining whether

a child with severe neurological impairment, from whatever cause, is experiencing dyspnea Cliniciansmay assume that the child with tachypnea, use of accessory respiratory muscles, decreased oxygensaturation and other sings of respiratory distress are experiencing dyspnea They may empiricallyutilize dyspnea plans with interventions such as airﬂow, opioids and benzodiazepines with theexpectation of beneﬁt

3.5 Neurological Symptoms

There are other symptoms of concern that must be addressed in children with non-cancer,life-threatening illnesses, especially neurological symptoms [22] First among these are seizures.Closely working with the child’s neurologist will be the best step towards determining an optimalplan for seizure control In some cases, full control cannot be achieved Developing both an optimaltarget and a rescue plan for prolonged or frequent seizures is critical

Along these lines dystonia is a challenging symptom to contend with [23] The ﬁrst step intreating dystonia is to recognize that it is distinct from seizures, although to the uninformed observer,and especially with a non-verbal patient, the uncoordinated movements may suggest a convulsion

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Children may also have co-existing dystonia and seizure disorder, further complicating the picture.Diagnostic evaluation needs to include an EEG to evaluate and delineate seizure-related movementsand to treat those While treatment of dystonia may involve muscle relaxants such as baclofen,parasympathetic inhibitors (trihexyphenidyl), benzodiazepines and dopamine agonists are often tried;these all depend on knowing the etiology of the condition.

Two other symptoms warrant consideration as parents may raise them, although very little isactually known about them One is lack of arousal or easy fatigability, while the other is temperaturedysregulation (cold or hot extremities) These two symptoms have appeared in reports by parents

as troubling to them [24] The lack of arousal is suggested when a parent notes that the child “is nolonger him/herself” at times, or less interactive than usual While the children are in many cases veryimpaired in language, parents and other caregivers who know the child well learn the individualsignals that indicate preferences and emotions When these are absent, it is clear to the family andclose caregivers that something is different There may be many causes to be investigated, includingthe introduction of new medications, neurological changes—for example more seizures, or fatigue due

to lack of good sleep More ominously, it may indicate progression of an underlying disease, especiallyfor metabolic conditions related to energy or storage diseases Some clinicians have speculated that

a change in arousal might be related to a psychiatric depression; our current state of understandingthe emotional life of a child with neurological impairment is very limited, so we must consider thesespeculations with reservation

Temperature dysregulation is also reported by parents, especially in reference to cold and hotextremities This is also an area that has not received much attention in the literature One reasonthat hands and feet may be cold is that the child may lack large muscle mass due to inactivity,resulting in decreased thermal production When the extremities alternate between being hot and cold,then centrally based autonomic dysfunction may be the etiology We are learning more about the rolethat the autonomic nervous system plays in children and this may lead to useful interventions [25]

In the short term, simple environmental measures are useful paying attention to cold and hot handsand feet and changing clothing accordingly

4 Emotional Support

It is fundamental that emotional support for the affected child is a responsibility of pediatricpalliative care In this regard there is no difference between children experiencing cancer and thosewith non-cancer diagnoses In both situations the range of services must be broad and robust.There needs to be capacity to provide many different modalities adjusted to age, developmentalcapacity, and child/youth preferences Modalities employed include play-based therapy, art therapy,music therapy, and standard talk-based approaches such as cognitive-behavior therapy

Just because a child is very young or non-verbal it is wrong to assume that they are notexperiencing feelings that need to be addressed Many parents of non-verbal children provideconsistent descriptions of their child’s understanding that usually exceeds what a brief observation in

a clinic will demonstrate to a clinician Prolonged interaction and observation of a child by nurses,physical and occupational therapists, and others generally conﬁrms the parents’ assessment Therefore,taking the lead from parents and developing a strategy to address a child’s depression, anxiety,and fears, regardless of perceived developmental stage is important

5 Social Issues

It is here that we consider the family beyond the affected child; parents and siblings, and inmany cases, grandparents, aunts and uncles and other extended members Foremost is addressingtheir questions that may focus on prognosis; this will be covered further on under the Trajectorysection Social support for family includes both care for their emotional needs—anxiety, depression,guilt—but also the practical issues of daily life that are complicated by having a child with

a life-threatening condition

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Some observations about how non-cancer conditions can be different are warranted here One isthat while “cancer” is not a single diagnosis, the care of children with cancer is often provided by

a uniﬁed team at a single center Parents then beneﬁt from services available at a single site under thebanner of cancer care; this may include mental health services for themselves and for siblings, familysupport through activities such as camps, and a communal experience with other families encountered

in the clinic and hospital Simply having a recognizable condition such as cancer, as difﬁcult as that is,may create a framework for social understanding

Non-cancer diagnoses number in the hundreds and cover a wide variety of organ systems [26].There is no single team or clinic that may follow these children There is often not a support system forparents or siblings provided in an ongoing, organized fashion, and families may feel highly isolated

As most of these conditions are very rare, families may struggle to explain to others what exactly isgoing on in their lives

A particular challenge for families are one of a kind diagnoses where there are no other childrenwith a similar condition, and similarly where a child lacks a diagnosis In both cases families are leftwithout information, increasing the uncertainty New techniques in genome analysis, such as WholeExome Sequencing and Whole Genome Sequencing are now available at both the clinical and researchlevel However, not all gene-based conditions can be identiﬁed as due to a single gene mutation

In some cases, there are multiple gene interactions that lead to a phenotype Understanding how theprotein product of a gene creates the condition is a necessary piece of information Recent studiessuggest that families’ value a “diagnosis” even when it does not lead to any treatment [27]

Regardless of having the gene explanation, families with children whose condition is either veryrare or non-diagnosed can feel isolated This can occur both in their family and community as well aswith interactions in the health system It is not unusual for families to report confusion by health careproviders and sometimes reluctance to provide care It is important for clinicians to understand that inthe absence of a life-prolonging or curative treatment, the focus on symptom management will rely onbasic principles that are similar across conditions, until we learn otherwise

Because of their expertise in symptom management, familiarity with rare and undiagnosedconditions, and family-centered approach, pediatric palliative care teams can be important to families,sometimes akin to a “medical home” for families if they are resourced accordingly If not that wellresourced, they can still be valuable as consultants to primary teams caring for these children

6 Trajectory

In the model we teach to trainees and use day-to-day in practice, following the sequence outlined

as Physical, Emotional (child), Social (family), Spiritual (religion and meaning), and Trajectory, the lastitem keying into advance care planning Trajectory is a topic that uncovers child and family goals,hopes and concerns Based on these elements we then develop alternative pathways using a “what if”approach This is an iterative process

As described in the previous sections, non-malignant conditions are often hard to characterize

as they are rare, not well studied, and with a resulting low level of evidence As many of theseconditions have a genetic basis, the nature of the gene defect, balancing and unbalancing geneticfactors, and interaction with the environment all play a role in phenotype and therefore clinical course.These factors all combine to make disease course and prognostication very difﬁcult A high degree ofclinical expertise, experience, careful communication, and a healthy dose of skepticism are all needed.Figure1gives insight into an interesting ﬁnding; the majority of children followed at any giventime on the Canuck Place program have non-cancer diseases, whereas mortality rates show a highincidence of cancer deaths

This pattern suggests that there are differences in the way that, and the timing of, engagement ofchildren and their families with PPC services at least in one well-established program Similar data isfound when examining timing of death in relationship to referral, broken down by disease category asshown in Figure2 Children with cancer may die sooner after referral as curative attempts continue

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well into the disease course For many patients with non-cancer, non-curable conditions, cliniciansand families are seeking hospice, medical respite, and palliative care support earlier in the disease,and therefore constitute a larger proportion of patients on program at any given time This is onehypothesis to explain the differences In addition, the services accessed for patients and families withcancer as compared to a non-cancer diagnosis may differ Care intensity may be high in children andfamilies living with cancer, although for a shorter period of time In contrast, children with non-cancerdiagnoses, especially those with static encephalopathy (Together for Short Lives Category 4), receivedcare intermittently but over longer-time periods [28].

Figure 1 Children on Program and Deaths on Program Canuck Place Children’s Hospice.

Figure 2 (a) Intervals from referral to palliative care until death for cancer patients; (b) Intervals from

referral to palliative care until death for patients with non-cancer conditions.

As was shown in Figure2the unpredictable pattern of time from referral to death, observed inseveral studies, conﬁrms that the best approach is to prepare families, and oneself, both for suddenterminal events and simultaneously for a protracted course Preparing families involves both education

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and careful planning One mother aptly describes this as “only a (parent in a pediatric palliative careprogram) meets their kindergarten principal and funeral director in the same week”.

In addition to discussing advance care planning and advance directives with families as ongoingdiscussion, it is also advisable to explore scenarios Completing paperwork, for example a letter to beused in the Emergency Room, is also advisable so that one is prepared for the unexpected

The pediatric palliative care program at Canuck Place does not require an advance directivedocument or Do Not Attempt Resuscitation order It is practice however to have advance care planningdiscussions with families on an annual basis, at minimum In these discussions it may be sufﬁcient

to simply review the relevant concepts and terminology that families may encounter (e.g., what is

a “Code Blue”), without requiring any speciﬁc decision-making As we found in a retrospective review,this kind of conversation supports family decision-making, and one result is that even among thefamilies who self-described as choosing all resuscitation measures, at the time of their child’s deathalmost none asked for intervention

7 Future Directions

As the ﬁeld of pediatric palliative care matures more attention is being paid to the non-malignantconditions in research and knowledge translation An understanding that cross-communication amongseveral ﬁelds and subspecialties in medicine and other disciplines needs to occur in order to develop

a more comprehensive (holistic) understanding of how these conditions are experienced by affectedchildren and their families, and in turn to design interventions that support quality of life

Immediate targets include epidemiological and health services research studies to betterdelineate the population of interest and their service use; clinical intervention studies of physicalsymptoms, children’s emotional wellbeing, and family psycho-social health; and basic physiology andpharmacology studies to enhance our understanding of symptoms and symptom treatment.Children and their families with non-malignant conditions are a substantial proportion of thework of pediatric palliative care and will potentially increase in number in the future with advances inlife-prolonging treatments This is an area that deserves our attention especially for on-going research

Conﬂicts of Interest: The author declares no conﬂict of interest.

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© 2018 by the author Licensee MDPI, Basel, Switzerland This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).

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