Deliberate

termination

of

life

of

newborns

with

spina

bifida

a

critical

reappraisal

Objects

Deliberate

termination

of

life

of

newborns

-LRB-

involuntary

euthanasia

-RRB-

with

meningomyelocele

-LRB-

MMC

-RRB-

is

practiced

openly

only

in

the

Netherlands

Unbearable

and

hopeless

suffering

is

the

single

most

cited

criterion

for

this

termination

together

with

the

notion

that

there

are

no

other

proper

medical

means

to

alleviate

this

suffering

In

this

paper

both

-LRB-

and

other

-RRB-

statements

are

questioned

also

by

putting

them

in

a

broader

perspective

An

infant

with

spina

bifida

can

not

suffer

unbearably

Infants

might

be

able

to

experience

unbearable

pain

but

spina

bifida

does

not

cause

it

-LRB-

Chervenak

-RRB-

Introduction

and

historical

overview

of

the

treatment

of

newborns

with

MMC

Before

the

most

newborns

with

meningomyelocele

-LRB-

MMC

-RRB-

were

not

treated

given

the

consideration

that

it

was

in

the

best

interests

of

the

child

and

family

in

most

cases

resulting

in

the

death

of

the

child

They

died

from

sepsis

meningitis

hydrocephalus

or

renal

failure

Contraindications

to

operation

included

hydrocephalus

irreparable

deformities

paralysis

of

the

sphincters

complete

paraplegia

or

any

ulcerative

process

in

the

region

of

the

spina

bifida

-LSB-

-RSB-

The

influence

of

the

surgical

approaches

since

the

allowed

gradually

effective

early

treatment

of

the

complicating

hydrocephalus

resulting

in

many

patients

surviving

into

childhood

Early

treatment

of

the

spinal

lesion

and

hydrocephalus

resulted

in

decreased

mortality

and

morbidity

-LSB-

-RSB-

Wait

and

see

changed

into

active

treatment

Lorber

-LRB-

-RRB-

published

the

first

large

series

of

patients

treated

between

and

-LSB-

-RSB-

He

found

that

only

of

the

survivors

had

less

than

crippling

disability

most

had

a

quality

of

life

inconsistent

with

self-respect

learning

capacity

happiness

and

even

marriage

To

spare

children

and

families

prolonged

suffering

he

proposed

the

well-known

Lorber

selection

criteria

These

four

major

adverse

criteria

are

severe

paraplegia

gross

enlargement

of

the

head

severe

kyphosis

or

scoliosis

and

associated

gross

congenital

anomalies

-LRB-

such

as

heart

disease

-RRB-

or

major

birth

injuries

No

active

treatment

was

advised

for

children

who

had

one

or

any

combination

of

these

criteria

The

result

of

this

proposed

selection

was

to

treat

only

those

with

lesions

on

lumbar

and

sacral

level

without

any

other

major

complication

Others

would

according

to

Lorber

die

quickly

Since

the

Lorber

era

there

have

been

tremendous

successes

in

the

treatment

of

patients

with

MMC

-LSB-

-RSB-

Over

the

last

years

continual

progress

has

not

only

been

made

in

the

neurosurgical

-LRB-

closure

of

the

back

defect

shunting

or

endoscopic

techniques

for

the

complicating

hydrocephalus

treatment

of

tethered

spinal

cord

and

Chiari

II

malformation

-RRB-

orthopedic

-LRB-

treatment

of

scoliosis

correction

of

club

feet

-RRB-

urological

-LRB-

preserving

normal

renal

function

securing

social

continence

-RRB-

and

psychosocial

treatment

of

patients

with

MMC

but

also

in

support

for

independence

by

modern

rehabilitation

programs

Nevertheless

early

decision

making

-LRB-

for

restriction

-RRB-

and

selective

treatment

of

newborns

with

MMC

is

still

propagated

-LSB-

-RSB-

resulting

in

approvals

but

also

in

critiques

-LSB-

-RSB-

What

is

considered

ethical

at

one

time

or

in

one

country

can

be

considered

unethical

or

unacceptable

at/in

another

The

ethical

debate

on

treating

children

with

MMC

is

unique

in

the

sense

that

these

children

will/can

have

a

life

of

substantial

and

sometimes

severe

physical

disability

but

often

with

normal

mentation

this

in

contrast

to

severely

psycho-motor

handicapped

children

Children

and

adults

with

MMC

can

be

aware

of

their

deficits

Lorber

-LRB-

-RRB-

stated

that

untreated

children

will

die

quickly

-LSB-

-RSB-

However

he

delivered

the

children

anticonvulsants

to

prevent

seizures

opioids

and

chloral

hydrate

to

prevent

pain

-LSB-

-RSB-

One

pediatrician

has

said

that

these

untreated

children

received

the

same

care

and

attention

as

others

being

fed

and

loved

but

they

also

received

mg/kg

body

weight

of

chloral

hydrate

four

times

a

day

This

was

being

administered

for

pain

but

the

children

who

were

operated

on

did

not

receive

this

regimen

Another

physician

just

said

we

do

n't

feed

them

-LSB-

-RSB-

Consequently

the

duration

of

life

seems

to

depend

on

the

attitude

of

the

responsible

doctor

In

the

United

States

untreated

children

were

not

given

morphine

or

anticonvulsant

drugs

and

were

fed

every

h

resulting

in

many

survivors

with

severe

handicaps

On

the

contrary

in

the

United

Kingdom

the

children

died

quickly

However

the

question

remains

if

these

children

were

really

in

pain

or

suffered

from

seizures

perhaps

not

Not

all

physicians

did

follow

the

Lorber

criteria

but

treated

all

children

unselectively

regardless

of

the

level

of

the

lesion

This

approach

resulted

in

multidisciplinary

MMC

clinics

and

MMC

teams

The

nonselective

treatment

of

newborns

with

MMC

started

in

the

early

and

gives

us

a

picture

of

long-term

outcome

of

newborns

with

MMC

-LSB-

-RSB-

and

also

of

the

quality

of

life

and

health

status

of

adults

with

spina

bifida

-LSB-

-RSB-

Some

physicians

became

bitter

after

treating

children

with

MMC

for

years

-LSB-

-RSB-

In

the

early

the

Baby

Jane

Doe

-LRB-

Stony

Brook

baby

-RRB-

case

resulted

in

the

United

States

in

an

ethical

debate

on

the

prospect

physicians

make

about

the

future

life

of

handicapped

children

born

with

MMC

This

girl

was

born

with

MMC

complicated

with

hydrocephalus

-LRB-

although

diagnosed

by

the

attending

physician

as

microcephalic

-RRB-

The

team

of

physicians

told

the

parents

that

the

child

would

spend

her

life

lying

in

bed

bottle

fed

She

would

experience

no

joy

sadness

or

any

such

emotion

except

response

to

pain

and

would

develop

no

cognitive

skills

She

might

survive

for

years

-LSB-

-RSB-

Based

on

the

given

information

the

parents

agreed

not

to

treat

their

child

However

the

physicians

turned

out

to

be

wrong

Although

the

child

developed

meningitis

sepsis

and

hydrocephalus

-LRB-

which

were

only

treated

to

some

extent

-RRB-

she

survived

all

these

complications

was

taken

home

became

ambulating

and

communicating

on

an

acceptable

level

of

intelligence

all

this

despite

the

partial

treatment

-LSB-

-RSB-

After

the

introduction

of

alpha

feto-protein

-LRB-

AFP

-RRB-

screening

in

the

late

-LRB-

followed

by

amniocentesis

-RRB-

and

ultrasound

diagnosis

many

fetus

with

MMC

can

now

be

detected

early

in

pregnancy

Nowadays

after

being

counseled

most

pregnant

couples

decide

to

abort

a

fetus

with

MMC

-LRB-

in

many

Western

countries

resulting

in

an

abortion

rate

of

resulting

in

fewer

children

with

MMC

born

-LSB-

-RSB-

-RRB-

seen

by

many

as

prevention

of

MMC

But

what

advice

should

neonatologists

neurologists

or

neurosurgeons

give

the

parents

when

a

full-term

child

with

MMC

is

born

Is

the

advice

not

to

treat

or

to

terminate

the

life

of

the

newborn

deliberately

-LRB-

involuntary

euthanasia

-RRB-

an

ethically

defendable

one

Deliberate

and

active

termination

of

life

in

newborns

with

MMC

is

only

discussed

and

practiced

in

an

open

way

in

the

Netherlands

-LSB-

-RSB-

The

judgment

unbearable

and

hopeless

suffering

of

the

newborn

and

the

prospect

of

unbearable

and

hopeless

suffering

in

the

future

together

with

the

notion

that

there

is

no

other

proper

medical

means

to

alleviate

the

unbearable

suffering

form

the

basis

for

this

decision

-LSB-

-RSB-

Because

these

judgments

are

questioned

and

to

quantify

the

level

of

discomfort

in

these

neonates

the

Rotterdam

Prospective

Study

on

Discomfort

in

Newborns

with

Spina

Bifida

was

started

in

in

the

Erasmus

Medical

Center

-LRB-

EMC

-RRB-

Sophia

Children

's

Hospital

-LRB-

Rotterdam

the

Netherlands

-RRB-

Although

in

due

time

the

results

of

this

study

will

be

published

some

important

aspects

will

already

be

addressed

in

this

paper

by

elaborating

on

the

case

of

a

newborn

with

a

very

severe

form

of

MMC

and

hydrocephalus

Deliberate

termination

of

life

in

newborns

with

MMC

in

The

Netherlands

The

Dutch

neurosurgeon

De

Lange

analyzed

the

selection

criteria

for

treatment

of

newborns

with

MMC

in

the

Netherlands

already

in

the

-LSB-

-RSB-

It

became

clear

from

an

enquiry

among

Dutch

neurosurgeons

that

a

number

of

them

advised

against

treatment

on

the

basis

of

physical

handicaps

alone

Others

regarded

the

expected

mental

abilities

as

an

overruling

factor

When

basic

communication

skills

could

be

expected

most

children

were

treated

At

the

time

deliberate

termination

of

life

was

never

practiced

De

Lange

-LRB-

-RRB-

also

concluded

If

we

were

to

decide

that

in

our

opinion

patients

with

spina

bifida

who

are

below

a

certain

level

of

physical

or

mental

ability

should

not

be

kept

alive

it

should

be

illogical

not

to

apply

the

same

rules

to

other

categories

of

handicap

-LSB-

-RSB-

In

the

the

Dutch

ethicist

Hertogh

-LRB-

-RRB-

published

his

paper

Ethical

considerations

on

the

dilemmas

caused

by

the

selective

treatment

of

newborns

with

MMC

in

the

leading

Dutch

medical

Journal

-LSB-

-RSB-

He

elaborated

on

the

philosophical

and

ethical

aspects

of

the

Lorber

selection

criteria

The

objective

medical

fact

of

a

physical

shortcoming

is

something

completely

different

than

the

subjective

reality

of

being-handicapped

itself

Studies

have

shown

that

living

as

being-handicapped

does

not

correlate

directly

to

the

severity

of

physical

shortcomings

The

question

as

to

what

can

be

considered

as

an

acceptable

handicap

transgresses

in

other

words

the

scope

of

what

can

be

ascertained

in

a

medical-scientific

way

and

this

therefore

refers

inevitably

to

the

subjective

experience

It

has

to

be

questioned

whether

the

quantification

of

qualitative

aspects

of

medical

treatment

perhaps

offers

nothing

more

than

pretence-rationality

caused

by

the

used

decisive

terminology

In

conclusion

up

to

date

the

criterion

quality

of

life

appears

to

be

more

deceptive

than

clarifying

in

the

medical

decision

about

the

possible

continued

existence

of

a

newborn

with

spina

bifida

-LSB-

-RSB-

He

also

referred

to

the

ethics

of

the

philosopher

Levinas

Ethos

can

not

be

founded

in

scientific

facts

it

is

founded

in

the

human

relationship

itself

Mutual

dependency

which

is

characteristic

for

human

being

makes

us

responsible

just

by

itself

and

therefore

defines

the

disposition

of

ethos

as

a

rational

fact

This

relationship

always

precedes

facts

themselves

-LSB-

-RSB-

In

other

words

human

beings

just

because

of

the

fact

that

they

are

human

beings

have

an

intrinsic

and

mutual

responsibility

for

the

well-being

of

others

this

responsibility

itself

preceding

physical

qualities

This

thoughtful

paper

however

was

not

given

much

attention

in

the

Dutch

medical

press

In

baby

Rianne

was

born

in

a

small

town

in

the

Netherlands

with

a

MMC

at

level

of

the

first

and

second

lumbar

vertebrae

complicated

by

hydrocephalus

The

lower

limbs

were

deformed

and

paralyzed

Computed

tomography

-LRB-

CT

-RRB-

scan

of

the

head

confirmed

the

diagnosis

of

severe

hydrocephalus

On

the

first

day

of

her

life

the

decision

was

made

by

the

pediatrician

and

the

gynecologist

in

consultation

with

a

neurologist

neurosurgeon

and

pastor

not

to

operate

the

back

lesion

and

the

hydrocephalus

On

the

fourth

day

of

her

life

at

the

request

of

the

parents

the

gynecologist

Prins

terminated

the

life

of

the

baby

by

a

lethal

injection

In

an

interview

Prins

stated

-LSB-

-RSB-

Closing

the

defect

and

fighting

infections

with

maximal

treatment

could

enable

the

child

to

survive

for

years

No

one

knows

how

long

On

the

question

of

what

an

operation

could

have

accomplished

Prins

answered

The

defect

would

have

been

closed

The

possibility

of

secondary

infection

which

was

an

immediate

threat

to

her

life

would

have

been

diminished

Her

brain

pressure

would

rise

however

and

it

would

have

been

necessary

to

implant

a

shunt

On

the

question

of

how

he

would

calculate

the

benefit

of

treatment

he

answered

It

-LRB-

the

operation

-RRB-

would

have

prolonged

her

life

without

treating

the

symptoms

and

consequences

of

the

defect

because

the

operation

would

not

have

alleviated

them

The

operation

would

only

have

minimized

the

possibility

of

infection

but

it

would

have

created

the

necessity

for

repeated

operations

involving

the

shunt

and

the

urological

system

because

there

were

obstructions

in

the

passage

between

the

kidney

and

the

bladder

Her

brain

damage

was

so

extensive

and

the

possibility

of

movement

so

limited

She

would

never

have

been

able

to

walk

or

sit

unsupported

she

would

have

been

incontinent

in

her

bladder

and

bowel

There

was

sensory

damage

as

well

she

could

not

feel

sensation

in

her

lower

body

She

would

have

started

life

with

repeated

operations

need

for

persistent

medical

attention

no

possibility

for

self-support

and

very

low

communicative

ability

-LSB-

-RSB-

As

a

definition

of

a

severe

handicap

he

stated

This

can

relate

to

the

estimated

possibility

of

becoming

happy

-LSB-

-RSB-

and

he

further

referred

to

the

Dutch

Association

of

Paediatrics

who

developed

criteria

including

the

lack

of

capacity

to

communicate

lack

of

capacity

for

self-maintenance

persistent

need

for

medical

assistance

and

the

existence

of

pain

All

these

criteria

grouped

together

gave

a

picture

of

the

baby

's

quality

of

life

Because

most

of

the

criteria

were

satisfied

the

baby

was

considered

to

be

severely

handicapped

resulting

in

a

very

poor

expected

quality

of

life

the

child

was

not

operated

on

because

of

the

severity

of

the

expected

handicaps

On

the

necessity

of

life-termination

Prins

-LRB-

-RRB-

explained

The

baby

was

in

a

great

deal

of

pain

and

the

normal

analgesics

did

not

work

well

So

it

would

have

been

necessary

to

use

narcotic

analgesics

morphine-like

drugs

And

that

would

have

caused

depression

of

consciousness

It

is

clear

to

me

that

the

use

of

such

drugs

would

have

depressed

normal

functioning

behavior

expressions

and

sensibility

Using

morphine-like

drugs

would

have

caused

a

situation

of

floating

between

heaven

and

earth

It

would

have

been

dishonest

and

should

be

considered

as

unethical

and

unjust

There

is

no

moral

difference

between

killing

and

withholding

useless

medical

treatment

There

is

an

emotional

difference

And

there

should

not

be

a

legal

difference

-LSB-

-RSB-

Although

elements

of

the

offense

of

murder

were

proven

the

physician

was

acquitted

based

on

the

unavoidable

conflict

of

duties

between

his

duty

to

prolong

life

and

his

duty

to

alleviate

unbearable

suffering

-LSB-

-RSB-

In

three

Dutch

child

neurologists

and

one

neurosurgeon

questioned

in

the

leading

Dutch

medical

journal

the

active

termination

of

life

of

newborns

with

MMC

-LSB-

-RSB-

They

defended

the

opinion

that

-LRB-

severe

-RRB-

handicapped

life

could

also

be

meaningful

Deliberate

termination

of

life

was

not

considered

appropriate

sedation

and

the

delivery

of

opioids

were

mandatory

in

the

case

of

suffering

Not

treating

a

child

with

MMC

should

not

result

in

a

more

severe

handicapped

life

the

decision

to

withhold

treatment

was

only

made

in

the

case

of

severe

pulmonary

or

cardiac

complications

with

the

prospect

of

early

death

The

possible

suffering

of

the

parents

should

not

be

an

indication

for

deliberate

termination

of

the

life

of

a

newborn

with

MMC

They

referred

to

the

study

of

Steinbok

et

al.

-LRB-

-RRB-

-LSB-

-RSB-

for

their

argument

that

the

life

of

children

and

adults

with

-LRB-

severe

-RRB-

MMC

can

be

meaningful

-LSB-

-RSB-

In

the

Dutch

physician/medical

ethicist

Van

de

Vathorst

stated

that

whenever

it

is

decided

not

to

treat

a

defective

newborn

deliberate

termination

of

the

life

of

this

newborn

should

be

regarded

as

a

moral

demand

based

on

the

principle

of

mercy

-LSB-

-RSB-

She

illustrated

her

arguments

with

a

case

of

a

newborn

with

a

thoracic

MMC

Interestingly

enough

the

life

of

this

child

was

not

terminated

It

died

by

itself

a

few

days

old

because

of

pulmonary

insufficiency

Although

she

received

a

national

award

for

this

paper

it

resulted

in

several

disapproving

letters

to

the

editor

In

January

two

pediatricians

one

physician

and

one

pediatric

neurologist

reported

on

the

deliberate

termination

of

life

of

newborns

in

the

Netherlands

between

and

-LSB-

-RSB-

Explaining

the

fact

that

all

cases

were

newborns

with

MMC

and

hydrocephalus

Verhagen

et.al

referred

to

the

baby

Rianne

case

stating

that

her

life

was

dominated

by

unbearable

suffering

and

since

this

case

it

is

established

that

newborns

with

a

severe

form

of

spina

bifida

do

suffer

severely

-LSB-

-RSB-

According

to

the

authors

the

deliberate

termination

of

life

was

necessary

because

of

the

presence

of

unbearable

and

hopeless

suffering

acutely

and

long-term

with

no

other

proper

medical

means

of

alleviating

the

suffering

-LSB-

-RSB-

All

parents

consented

to

the

proposal

of

termination

of

life

in

four

cases

they

explicitly

requested

for

it

For

the

public

prosecutor

the

termination

of

life

was

acceptable

if

four

requirements

were

properly

fulfilled

-LRB-

-RRB-

the

presence

of

hopeless

and

unbearable

suffering

-LRB-

-RRB-

consent

of

the

parents

to

termination

of

life

-LRB-

-RRB-

consultation

of

another

physician

or

of

a

multidisciplinary

MMC

team

and

-LRB-

-RRB-

a

proper

and

careful

execution

of

the

termination

These

four

criteria

eventually

became

the

cornerstone

of

the

so-called

Groningen

Protocol

a

protocol

meant

to

insure

that

the

decision

for

life

termination

the

executing

itself

together

with

reporting

to

the

legal

authorities

are

all

conducted

in

a

proper

way

This

protocol

was

approved

and

adopted

by

the

Dutch

Association

of

Paediatrics

in

The

motivation

for

the

judgement

of

unbearable

and

hopeless

suffering

in

the

cases

of

spina

bifida

was

-LSB-

-RSB-

suffering

-LRB-

acute

and

chronic

pain

physical

pain

-RRB-

-LRB-

of

the

cases

-RRB-

the

lack

on

ability

to

live

or

do

things

independently

as

the

result

of

severely

disturbed

sensomotoric

development

-LRB-

of

the

cases

-RRB-

the

lack

of

possibility

to

verbal

and

non-verbal

communication

-LRB-

of

the

cases

-RRB-

the

prospect

to

dependency

to

the

medical

circuit

as

the

result

of

frequent

hospital

admissions

and

operations

-LRB-

of

the

cases

-RRB-

the

life

expectation

-LRB-

the

burden

of

severe

suffering

increases

by

a

longer

life

span

-RRB-

-LRB-

in

of

the

cases

-RRB-

of

the

cases

led

to

prosecution

all

cases

were

found

to

be

in

accordance

with

good

medical

practice

This

article

of

Verhagen

et

al.

-LRB-

-RRB-

gave

rise

to

only

a

few

reactions

in

the

Netherlands

Kompanje

et

al.

-LRB-

-RRB-

-LSB-

-RSB-

questioned

the

validity

of

unbearable

suffering

as

a

useful

criterion

They

also

stated

that

with

the

use

of

modern

palliative

care

suffering

always

can

be

dealt

with

in

an

adequate

way

as

did

Laane

-LRB-

-RRB-

-LSB-

-RSB-

De

Jong

-LRB-

-RRB-

-LSB-

-RSB-

made

a

plea

for

letting

them

die

instead

of

making

them

die

in

the

case

of

an

untreated

newborn

with

MMC

On

request

of

the

editor

of

the

New

England

Journal

of

Medicine

Verhagen

and

Sauer

-LRB-

-RRB-

published

another

article

concerning

the

Groningen

protocol

and

the

cases

-LSB-

-RSB-

and

a

similar

article

appeared

in

Pediatrics

-LSB-

-RSB-

The

reactions

in

the

international

medical

press

were

mostly

negative

In

his

article

Control

of

suffering

on

the

slippery

slope

of

care

in

the

Lancet

Feudtner

-LRB-

-RRB-

also

addressed

the

poor

quality

and

the

insufficiency

of

the

offered

palliative

care

and

questioned

whether

legalization

of

active

termination

of

life

might

not

lead

to

the

abuse

of

it

-LSB-

-RSB-

Saugstad

-LRB-

-RRB-

stated

in

the

Acta

Paediatrica

Neonatologists

must

be

extremely

careful

not

to

start

on

the

slippery

slope

ending

in

the

Dutch

practice

of

euthanasia

of

newborns

I

would

strongly

warn

against

this

attitude

which

I

find

non-compatible

with

Western

humanistic

traditions

-LSB-

-RSB-

Jotkowitz

and

Glick

-LRB-

-RRB-

in

the

Journal

of

Medical

Ethics

were

worried

We

feel

an

important

line

has

been

crossed

if

the

international

medical

community

consents

to

the

active

euthanasia

of

severely

ill

infants

and

are

concerned

about

the

extension

of

the

policy

to

other

at

risk

groups

-LSB-

-RSB-

Bondi

et

al.

-LRB-

-RRB-

in

Pediatrics

made

a

plea

for

an

open

discussion

Rather

than

performing

an

in-depth

examination

of

the

philosophical

ethical

and

moral

implications

of

this

protocol

the

authors

focus

on

its

technical

details

and

acceptability

within

the

European

medical

community

The

Groningen

Protocol

centers

not

on

patient

self-determination

but

instead

on

the

perception

of

the

patient

's

current

and

future

suffering

Although

the

authors

note

that

an

open

discussion

of

these

issues

is

extremely

important

they

fail

to

initiate

it

We

hope

that

thoughtful

ethicists

and

legislators

will

initiate

the

open

discussion

that

the

authors

advocate

-LSB-

-RSB-

In

their

article

Why

the

Groningen

Protocol

should

be

rejected

Chervenak

et

al.

-LRB-

-RRB-

concluded

that

The

justification

they

offer

is

woefully

inadequate

It

relies

on

appeals

to

the

concepts

of

hopeless

and

unbearable

suffering

the

best

interests

of

the

patient

and

medical-ethical

values

but

it

never

explains

these

concepts

and

it

gives

no

ethical

argument

for

their

clinical

application

In

fact

not

only

is

no

argument

for

the

Protocol

given

can

hope

to

succeed

The

Groningen

Protocol

should

therefore

be

rejected

as

the

basis

for

obstetric

and

neonatal

practice

in

The

Netherlands

and

throughout

the

world

-LRB-

-RRB-

Manninen

-LRB-

-RRB-

finally

made

an

important

contribution

with

her

article

A

case

for

justified

non-voluntary

active

euthanasia

exploring

the

ethics

of

the

Groningen

Protocol

in

the

Journal

of

Medical

Ethics

-LSB-

-RSB-

She

made

a

thoughtful

plea

for

the

ethics

of

the

Groningen

Protocol

What

the

Groningen

Protocol

proposes

to

do

is

both

ethical

and

also

the

most

human

alternative

for

these

suffering

and

dying

infants

Newborns

eligible

for

this

protocol

do

face

no

future

at

all

that

is

the

infants

must

be

terminally

ill

will

die

soon

no

matter

what

actions

doctors

undertake

modern

medicine

can

do

nothing

to

save

them

have

no

prospects

have

crossed

the

threshold

into

a

comparatively

valueless

state

of

existence

will

spend

the

remainder

of

their

lives

in

a

chronic

state

of

nothing

but

pain

and

suffering

-LSB-

-RSB-

Interestingly

enough

on

November

an

additional

letter

of

herself

Regarding

the

Groningen

Protocol

-LSB-

-RSB-

was

published

in

the

same

journal

Some

key

passages

of

this

letter

It

has

recently

been

brought

to

my

attention

that

the

physicians

of

the

Groningen

Hospital

who

originally

proposed

the

Groningen

Protocol

have

proposed

to

violate

the

first

two

conditions

of

the

original

Groningen

Protocol

-LRB-

-RRB-

The

suffering

must

be

so

severe

that

the

infant

has

no

prospects

for

a

future

-LRB-

-RRB-

There

is

no

possibility

that

the

infant

can

be

cured

or

alleviated

of

her

affliction

with

medication

or

surgery

For

example

Dr.

Verhagen

has

maintained

that

the

Protocol

could

apply

to

infants

that

are

not

necessarily

terminally

ill

but

nevertheless

may

face

a

compromising

future

In

essence

the

Groningen

Protocol

has

begun

to

make

quality

of

life

judgments

If

the

Groningen

physicians

have

commenced

making

quality

of

life

judgments

they

have

strayed

away

from

the

original

purpose

of

the

Protocol

and

this

I

believe

may

have

concerning

ethical

implications

As

I

write

in

my

paper

the

Groningen

Protocol

does

not

make

quality

of

life

judgments

and

it

is

because

of

this

very

important

requirement

that

the

Protocol

strikes

me

as

humane

and

morally

permissible

for

it

seems

utterly

vicious

to

extend

the

life

of

a

suffering

infant

with

no

prospects

for

a

future

If

the

infant

did

have

a

possible

future

ahead

of

her

I

would

be

more

hesitant

to

condone

a

legal

practice

that

begins

to

make

quality

of

life

judgments

for

such

judgments

can

be

and

have

been

subject

to

error

-LSB-

-RSB-

In

her

opinion

in

these

end-of-life

decisions

quality

of

life

judgments

should

not

be

made

and

in

a

way

with

this

letter

she

also

highlights

the

inconsistencies

in

the

reports

by

Verhagen

et

al.

Being

of

crucial

importance

the

subject

of

quality

of

life

judgments

will

be

discussed

more

extensively

in

paragraphs

and

Finally

in

in

The

Netherlands

a

national

multidisciplinary

expert

review

committee

has

been

appointed

In

the

case

of

active

termination

of

the

life

of

a

newborn

the

responsible

physician

is

supposed

to

inform

this

committee

which

then

has

to

determine

whether

the

criteria

for

life

termination

have

been

met

Subsequently

this

committee

has

to

advise

the

Public

Persecutor

's

Office

-LRB-

OM

-RRB-

whether

the

physician

should

be

prosecuted

or

not

Although

such

a

committee

seems

to

guarantee

more

certainty

it

can

be

doubted

however

if

this

checking

on

afterhand

is

of

much

value

for

the

newborn

in

question

It

seems

to

be

more

sensible

to

change

this

procedure

to

a

checking

on

beforehand

Legally

this

might

perhaps

be

difficult

to

establish

but

it

seems

not

justifiable

that

legal

procedures

themselves

do

dominate

the

question

of

appropriateness

of

life

termination

errors

in

judgment

being

unacceptable

-LRB-

cf.

the

level

of

certainty

required

for

a

death

penalty

in

crime

in

e.g.

the

USA

-RRB-

Analysis

of

the

mentioned

criteria

for

non-treatment

and

deliberate

termination

of

life

Suffering

The

actual

presence

of

acute

unbearable

and

hopeless

suffering

of

the

newborn

and

the

prospect

of

future/chronic

unbearable

and

hopeless

suffering

as

the

result

of

handicaps

pain

and

discomfort

is

the

most

given

argument

for

deliberate

termination

of

life

in

neonates

with

MMC

In

the

recent

Dutch

series

this

suffering

was

stated

to

be

present

in

of

the

cases

-LSB-

-RSB-

Acute

pain

Not

any

convincing

evidence

can

be

found

in

the

literature

that

newborns

with

MMC

actually

do

suffer

from

untreatable

pain

in

the

days

after

birth

caused

by

their

back

lesion

or

by

other

complications

A

pediatric

surgeon

who

devoted

much

of

his

professional

life

of

more

than

years

to

the

treatment

of

those

who

have

been

born

with

spina

bifida

stated

I

personally

have

seen

little

evidence

that

the

babies

have

pain

in

the

newborn

period

nor

have

I

found

them

unable

to

sleep

-LSB-

-RSB-

Delight

and

Goodall

-LRB-

-RRB-

-LSB-

-RSB-

studied

the

experiences

of

parents

of

children

born

with

MMC

all

managed

without

any

surgery

All

children

died

before

their

first

birthday

Only

a

quarter

of

them

did

require

some

medication

such

as

analgesics

and

anticonvulsants

half

of

them

were

given

drugs

in

the

dying

period

Analgesics

were

given

in

a

low

dose

simply

to

enhance

their

quality

of

life

-LSB-

-RSB-

Chronic

pain

The

literature

on

chronic

pain

in

MMC

is

scarce

The

first

systematic

examinations

of

the

nature

and

prevalence

of

pain

in

children

with

MMC

was

published

in

-LSB-

-RSB-

Headache

is

not

uncommon

in

adults

with

MMC

and

is

associated

with

hydrocephalus

In

this

series

of

patients

reported

experiencing

pain

once

a

week

or

more

frequent

Of

the

shunted

children

reported

headaches

compared

to

of

the

children

without

a

shunt

-LSB-

-RSB-

Children

in

this

study

reported

frequent

and

sometimes

intense

pain

at

multiple

locations

associated

with

the

physiological

sequelae

equipment

usage

and

medical

management

of

the

disability

The

authors

concluded

that

children

with

spina

bifida

frequently

report

clinically

significant

under-recognized

and

untreated

pain

and

they

propagated

proper

care

and

adequate

medical

management

of

these

children

In

another

series

of

patients

underwent

investigations

for

one

or

more

episodes

of

chronic

headache

due

to

shunt

malfunction

ventriculostomy

failure

or

symptomatic

Arnold

Chiari

malformation

-LSB-

-RSB-

Although

all

these

problems

sometimes

can

be

fairly

serious

the

notion

of

unbearable

and

hopeless

suffering

is

never

mentioned

The

lack

of

ability

to

live

or

do

things

independently

as

the

result

of

severely

disturbed

senso-motoric

development

The

second

argument

for

unbearable

and

hopeless

suffering

is

the

prospect

of

lack

of

ability

to

live

or

do

things

independently

as

the

result

of

severely

disturbed

senso-motoric

development

experienced

by

the

patient

in

childhood

puberty

and

adult

life

-LSB-

-RSB-

McLone

reported

in

the

results

of

the

unselective

treatment

of

consecutive

newborns

with

MMC

-LSB-

-RSB-

After

a

follow-up

period

of

to

years

there

were

survivors

He

concluded

that

the

total

percentage

of

children

who

could

be

independent

and

competitive

is

approaching

Hunt

and

Oakeshott

-LRB-

-RRB-

-LSB-

-RSB-

and

Oakeshott

and

Hunt

-LRB-

-RRB-

-LSB-

-RSB-

studied

the

outcome

in

unselected

and

actively

treated

patients

with

MMC

at

age

The

patients

were

treated

between

and

without

any

attempt

at

selection

Forty-two

patients

had

a

severe

lesion

at

a

sensory

level

above

of

whom

died

most

before

their

first

birthday

They

died

mostly

of

cardio-respiratory

or

renal

failure

hydrocephalus

or

CNS

infection

Two

of

the

survivors

live

independently

drive

cars

and

have

an

open

employment

six

have

an

IQ

In

another

study

-LSB-

-RSB-

out

of

adolescents

with

spina

bifida

had

the

lesion

at

a

high

lumbar

or

thoracic

level

The

authors

studied

the

quality

of

life

in

these

patients

They

concluded

that

there

were

no

significant

relationships

between

the

level

of

the

lesion

or

spina

bifida

severity

and

the

overall

quality

of

life

which

appeared

to

be

to

on

a

scale

both

patients

and

their

parents

view

the

overall

quality

of

life

positively

-LSB-

-RSB-

Besides

these

results

criteria

such

as

living

independently

or

doing

things

independently

can

be

confusing

when

deciding

about

the

future

life

of

vital

newborns

Most

people

who

do

live

a

meaningful

life

are

dependent

on

others

or

interdependent

on

each

other

In

fact

interdependency

and

especially

the

willingness

to

care

for

other

-LRB-

perhaps

dependent

-RRB-

people

can

be

considered

as

a

criterion

of

a

truly

civilized

society

certainly

so

when

this

means

to

offer

something

for

nothing

-LRB-

this

being

real

mercy

-RRB-

The

lack

of

possibility

of

verbal

and

non-verbal

communication

In

of

the

studied

Dutch

cases

it

was

predicted

-LRB-

future

-RRB-

communication

of

and

with

the

child

would

not

be

possible

neither

verbally

nor

nonverbally

-LSB-

-RSB-

In

other

words

these

babies

were

predicted

to

be

or

to

become

in

a

state

of

deep

coma

or

in

a

persistent

vegetative

state

Neither

of

these

states

of

very

severe

disturbed

consciousness

however

is

applicable

to

a

newborn

with

MMC

and

hydrocephalus

Furthermore

several

publications

concerning

the

level

of

mental

functioning

of

adults

with

MMC

have

been

published

-LSB-

-RSB-

Intellectual

development

varies

between

normal

IQ

-LRB-

this

being

the

case

in

-LSB-

-RSB-

to

-LSB-

-RSB-

of

the

studied

population

-RRB-

and

severe

mental

disability

but

the

predicted

complete

absence

of

the

possibility

to

communicate

is

never

mentioned

On

the

contrary

Virtually

all

infants

with

spina

bifida

are

capable

of

meaningful

human

relationships

independent

of

the

level

of

the

lesion

Indeed

most

are

of

normal

intelligence

-LSB-

-RSB-

Aside

from

this

incorrect

medical

description

there

are

some

other

important

aspects

that

need

to

be

highlighted

When

all

communication

is

claimed

to

be

impossible

one

wonders

by

what

methods

at

all

-LRB-

the

amount

of

-RRB-

suffering

can

be

established

Furthermore

when

a

person

is

in

such

a

state

of

deep

coma

or

in

a

persistent

vegetative

state

there

simply

is

no

cognitive

experience

whatsoever

also

not

of

-LRB-

unbearable

-RRB-

suffering

This

was

confirmed

in

a

recent

conviction

of

the

Dutch

Medical

Disciplinary

Committee

against

a

general

practitioner

who

euthanized

a

comatose

patient

the

ground

of

the

verdict

being

because

of

the

comatose

state

there

was

no

longer

any

question

of

unbearable

suffering

nor

any

question

of

euthanasia

-LSB-

-RSB-

The

prospect

of

dependency

to

the

medical

circuit

as

the

result

of

frequent

hospital

admissions

and

operations

It

seems

hardly

possible

to

quantify

this

criterion

in

such

a

way

that

it

can

be

used

to

justify

life

termination

of

vital

newborns

When

compared

to

persons

with

other

congenital

malformations

or

congenital

diseases

or

some

acquired

diseases

patients

with

MMC

do

not

appear

to

need

substantially

more

medical

care

than

e.g.

patients

with

intracranial

tumors

or

with

chronic

diseases

such

as

severe

pulmonary

cardiac

or

neurological

disorders

Furthermore

the

claim

of

Verhagen

-LRB-

-RRB-

that

this

child

would

have

to

undergo

at

least

operations

in

the

course

of

a

year

to

temporarily

alleviate

its

problems

-LSB-

-RSB-

again

can

not

be

substantiated

a

retrospective

survey

of

treated

newborns

with

MMC

over

years

in

the

Sophia

Children

Hospital

showed

that

three

to

four

operations

were

needed

in

their

first

year

of

life

Life

expectation

-LRB-

the

burden

of

severe

suffering

increases

by

a

longer

life

span

-RRB-

It

is

clear

that

the

longer

a

patient

lives

the

more

medical

care

will

be

needed

in

a

quantitative

way

Whether

this

means

that

this

will

affect

the

experienced

quality

of

life

in

a

negative

way

remains

totally

unclear

and

can

not

be

predicted

Furthermore

patients

with

MMC

do

require

relatively

much

of

their

medical

care

in

their

first

two

decades

this

steadily

decreasing

when

they

grow

older

and

when

disabilities

and

coping

with

these

disabilities

are

gradually

stabilized

Can

suffering

be

applied

as

a

reasonable

criterion

in

newborns

Suffering

especially

the

unbearable

and

hopeless

aspects

of

it

is

a

complex

psychosocial

phenomenon

and

by

definition

strictly

individualized

only

the

individual

itself

can

experience

the

existence

the

intensity

and

hopelessness

of

it

Others

-LRB-

parents

physicians

nurses

-RRB-

can

only

to

some

degree

make

an

estimation

of

it

It

is

therefore

tentative

whether

suffering

can

be

applied

to

newborns

at

all

What

is

possible

however

is

to

establish

quite

precisely

the

level

of

-LRB-

dis

-RRB-

comfort

of

a

newborn

by

making

use

of

standardized

score

charts

such

as

the

Comfort

score

and

the

Visual

Analogue

Scale

-LRB-

VAS

-RRB-

-LRB-

see

below

-RRB-

The

Dutch

physician

Beijk

-LRB-

-RRB-

stated

it

as

follows

suffering

will

be

interpreted

by

every

patient

by

every

doctor

and

by

every

other

involved

person

in

a

different

way

and

therefore

can

lead

to

completely

different

conclusions

The

bearableness

is

especially

dependent

on

personal

and

environmental

factors

it

is

all

about

the

subjective

experience

of

the

individual

itself

who

is

suffering

about

the

durableness

of

the

suffering

and

about

the

wish

to

die

-LSB-

-RSB-

In

in

his

Ph.D.

thesis

also

Hamburg

-LRB-

physician

and

jurist

-RRB-

concluded

for

the

criterion

suffering

there

are

no

objective

or

scientifically

validated

criteria

available

with

the

consequence

of

decisions

of

life-termination

being

dependent

of

the

subjective

judgment

of

the

doctor

leading

to

inconsistent

decisions

-LSB-

-RSB-

Therefore

according

to

Hamburg

in

a

time

of

evidence-based

medicine

suffering

is

not

acceptable

anymore

-LSB-

-RSB-

as

also

was

suggested

by

Kompanje

et

al.

-LRB-

-RRB-

-LSB-

-RSB-

Although

Beijk

and

Hamburg

are

referring

to

the

practice

of

euthanasia

-LRB-

which

in

the

Netherlands

is

strictly

referring

to

competent

patients

wishing

to

die

-RRB-

their

statements

are

the

more

applicable

to

newborns

as

in

these

cases

predictions

are

being

made

about

future

suffering

of

others

According

to

Chervenak

et

al.

-LRB-

-RRB-

An

infant

with

spina

bifida

can

not

have

and

therefore

can

not

have

lost

the

ability

to

realize

intentions

desires

and

hopes

for

the

future

it

can

feel

pain

but

can

not

suffer

as

a

psychosocial

phenomenon

-LSB-

-RSB-

Laane

-LRB-

-RRB-

came

to

the

same

conclusion

having

no

biographical

consciousness

suffering

can

not

be

applied

to

newborns

-LSB-

-RSB-

To

date

it

is

unclear

why

this

improper

use

of

suffering

still

continues

in

this

debate

Can

physicians

predict

quality

of

life

Counseling

the

parents

In

a

time

in

which

most

fetuses

with

MMC

will

be

detected

by

AFP

and

amniocentesis

or

by

ultrasound

diagnosis

-LRB-

in

most

cases

being

followed

by

iatrogenic

abortion

-RRB-

the

birth

of

a

newborn

with

MMC

is

increasingly

becoming

a

rare

phenomenon

-LSB-

-RSB-

The

decision

to

terminate

a

pregnancy

in

the

case

of

a

fetus

with

MMC

is

usually

made

shortly

after

the

diagnosis

It

can

be

questioned

whether

there

is

enough

time

for

proper

counseling

about

the

prospect

of

living

with

spina

bifida

as

a

patient

and

also

as

parents

Freeman

-LRB-

-RRB-

stated

Decisions

are

made

by

the

parents

without

knowledge

of

the

child

's

future

without

knowing

whether

the

child

will

have

minimal

deficit

and

walk

with

only

short

leg

braces

or

walk

with

no

braces

at

all

and

without

awareness

of

whether

this

child

will

have

a

thoracic

lesion

with

high

paraplegia

and

spend

his

or

her

life

in

a

wheelchair

Few

parents

at

that

stage

in

the

pregnancy

know

much

about

spina

bifida

or

about

what

life

will

be

like

for

that

child

and

for

that

family

In

other

words

these

rarely

are

informed

decisions

-LSB-

-RSB-

He

points

in

the

same

direction

concerning

physician

experience

Today

few

younger

physicians

have

experience

with

the

decision-making

process

for

spina

bifida

either

before

or

after

birth

Thus

physician

advisors

will

lack

experience

with

the

joys

of

caring

for

a

child

even

a

handicapped

child

as

well

as

experience

with

the

pains

and

problems

They

will

have

little

experience

with

the

feelings

of

the

handicapped

children

themselves

With

the

lack

of

experience

how

should

the

physician

counsel

On

what

basis

can

the

inexperienced

physician

advise

uninformed

parents

What

decision

should

the

parents

make

-LSB-

-RSB-

In

their

article

Tell

the

truth

about

spina

bifida

Bruner

and

Tulipan

-LRB-

-RRB-

also

showed

their

worries

about

the

quality

of

counseling

Unfortunately

many

healthcare

professionals

are

equally

ignorant

of

the

current

prognosis

of

children

with

spina

bifida

who

have

ready

access

to

comprehensive

care

in

a

modern

multidisciplinary

clinic

As

a

result

much

of

the

information

initially

provided

to

couples

with

a

newly

diagnosed

fetus

is

biased

and

misleading

As

medical

ethicists

Bliton

and

Zaner

at

Vanderbilt

University

Medical

Center

observed

To

date

we

have

met

and

held

intense

conversations

with

more

than

pregnant

women

and

their

partners

Many

times

couples

admitted

the

initial

counseling

they

received

from

their

obstetrician

was

slanted

both

against

disability

and

toward

termination

of

pregnancy

What

they

remembered

was

how

the

initial

obstetric

consultation

portrayed

as

grim

a

picture

as

possible

about

their

future

child

's

prognosis

-LSB-

-RSB-

The

statements

of

Verhagen

et

al.

-LRB-

-RRB-

mentioned

earlier

together

with

other

incorrect

predictions

-LRB-

these

newborns

have

no

prospect

of

a

future

and

no

chance

to

survive

at

all

are

barely

able

to

breathe

these

children

face

a

life

of

agonizing

pain

that

can

not

be

alleviated

by

any

proper

medical

means

the

child

would

suffer

such

unbearable

pain

that

it

has

to

be

constantly

anaesthetized

-LSB-

-RSB-

that

they

all

have

spina

bifida

of

the

most

severe

form

at

level

of

the

neck

and

all

have

non-functioning

kidneys

-LSB-

-RSB-

-RRB-

unfortunately

seem

to

confirm

these

worries

also

in

counseling

the

parents

of

a

newborn

with

MMC

To

date

it

is

not

clear

why

such

incorrect

information

is

presented

to

parents

colleagues

and

society

as

a

whole

When

parents

are

told

that

communication

with

and

of

their

child

never

will

be

possible

that

their

child

will

face

a

life

of

agonizing

pain

and

will

suffer

unbearably

that

this

suffering

can

not

be

alleviated

by

any

means

that

annually

at

least

operations

will

be

needed

to

offer

some

-LRB-

but

only

temporary

-RRB-

relief

then

these

parents

are

left

without

any

hope

and

their

consent/decision

to

non-treatment

or

to

active

termination

of

life

almost

can

be

predicted

However

in

such

situations

the

fundamental

concept

of

informed

decisions

is

obviously

violated

Does

the

level

of

the

lesion

matter

Does

the

level

of

the

lesion

determine

the

amount

and

severity

of

disturbances

In

general

the

higher

up

the

spine

the

lesion

is

the

more

likely

are

severe

neurological

disabilities

to

be

found

Cervical

lesions

often

do

not

contain

neural

tissue

and

are

simple

meningoceles

At

lower

levels

-LRB-

thoracic

and

especially

lumbar

-RRB-

the

converse

is

true

the

infant

nearly

always

being

-LRB-

partially

-RRB-

paralyzed

below

the

level

of

the

lesion

the

cord

showing

the

classical

features

of

dysplasia

-LSB-

-RSB-

However

the

extent

of

handicaps

that

a

person

with

MMC

experiences

is

determined

not

by

the

site

of

the

lesion

but

almost

entirely

by

the

neurological

deficit

Verhoef

-LRB-

-RRB-

concluded

that

young

adults

with

a

mild

form

of

spina

bifida

-LRB-

and

subsequently

with

few

disabilities

-RRB-

did

experience

their

disabilities

more

often

as

problematic

than

contemporaries

with

a

serious

form

of

spina

bifida

-LSB-

-RSB-

Likewise

in

the

study

of

Seller

-LRB-

-RRB-

-LSB-

-RSB-

eight

patients

ended

up

with

minimal

handicaps

having

bony

lesions

in

the

thoracic

-LRB-

one

patient

-RRB-

lumbar

-LRB-

two

patients

-RRB-

and

lumbosacral

-LRB-

five

patients

-RRB-

spine

Three

others

with

lumbosacral

bony

lesions

were

among

the

most

gravely

handicapped

-LSB-

-RSB-

According

to

Hetherington

-LRB-

-RRB-

low

lesions

do

not

necessarily

indicate

a

mild

course

physical

well-being

was

not

related

to

the

level

of

the

spinal

lesion

-LSB-

-RSB-

Although

persons

with

sacral

lesions

showed

less

impairment

compared

to

persons

with

higher

spinal

lesions

Padua

et

al.

-LRB-

-RRB-

did

not

find

a

correlation

between

quality

of

life

-LRB-

both

physical

and

mental

-RRB-

and

the

site

of

the

lesion

-LSB-

-RSB-

According

to

Pit-ten

Cate

et

al.

-LRB-

-RRB-

lesion

level

type

of

spina

bifida

and

gender

were

not

significantly

related

to

the

quality

of

life

in

children

with

spina

bifida

-LSB-

-RSB-

This

is

in

agreement

with

the

results

of

the

study

of

Sawin

et

al.

-LRB-

-RRB-

-LSB-

-RSB-

no

significant

relationship

with

lesion

level

spina

bifida

severity

or

shunt

status

and

quality

of

life

was

found

in

adolescents

Schoenmakers

-LRB-

-RRB-

-LSB-

-RSB-

concluded

that

being

independent

in

mobility

contributes

more

to

quality

of

life

than

other

functional

abilities

such

as

whether

or

not

being

wheelchair-dependent

And

although

children

with

the

most

severe

forms

of

MMC

have

significantly

more

and

severe

disabilities

than

children

with

milder

forms

of

MMC

scores

about

self-esteem

did

not

differ

significantly

between

the

two

groups

-LSB-

-RSB-

In

conclusion

the

level

of

the

lesion

does

not

correlate

with

the

actual

experience

of

the

handicaps

themselves

Is

the

diagnosis

extensive

brain

damage

easy

to

make

There

is

little

doubt

that

surgical

procedures

are

unjustified

in

hydrocephalic

newborns

with

little

or

no

potential

for

independent

survival

the

decision

as

to

what

constitutes

as

irreversible

and

severe

brain

damage

may

however

be

difficult

to

make

Ventricular

size

alone

can

not

be

used

as

a

conclusive

predictive

criterion

It

has

been

repeatedly

demonstrated

that

there

is

no

consistent

correlation

between

the

thickness

of

the

cortex

and

the

eventual

psychomotor

development

Barf

et

al.

-LRB-

-RRB-

-LSB-

-RSB-

concluded

that

cognitive

status

was

especially

negatively

influenced

by

multiple

shunt

revisions

and

by

other

pathologies

associated

with

hydrocephalus

Amacher

and

Wellington

-LRB-

-RRB-

-LSB-

-RSB-

treated

children

with

hydrocephalus

in

operative

procedures

and

concluded

that

there

is

no

difference

in

results

based

upon

measurement

of

the

initial

thickness

of

the

cerebral

mantle

They

found

normal

intelligence

in

of

the

survivors

In

a

recent

study

also

Beeker

et

al.

-LRB-

-RRB-

described

the

difficulty

in

making

a

reliable

prognosis

of

the

intellectual

development

based

on

ventricular

size

-LSB-

-RSB-

Quality

of

life

to

conclude

It

seems

impossible

to

predict

with

certainty

the

future

quality

of

life

of

newborns

with

MMC

One

also

has

to

be

aware

of

the

fact

that

the

self-reported

quality

of

life

of

children

with

handicaps

does

not

differ

from

that

of

children

without

disabilities

and

health

care

professionals

are

known

to

underestimate

disabled

persons

quality

of

life

as

compared

to

self-reports

-LSB-

-RSB-

Generally

speaking

prognostic

judgments

about

quality

of

life

are

conceptually

plausible

their

failing

is

simply

that

given

the

available

evidence

they

do

not

appear

to

be

reliable

-LSB-

-RSB-

Finally

and

perhaps

the

most

important

quality

of

life

judgements

are

considered

as

being

ethically

unacceptable

in

end-of-life

decisions

-LSB-

-RSB-

Non-discrimination

principle

In

his

Ph.D.

thesis

Dorscheidt

-LRB-

-RRB-

raises

the

issue

as

to

whether

the

deliberate

termination

of

the

life

of

disabled

newborns

is

compatible

with

the

non-discrimination

principle

in

particular

the

legal

prohibition

of

discrimination

on

the

ground

of

disability

-LSB-

-RSB-

Deliberate

termination

of

the

life

of

newborns

with

spina

bifida

is

always

preceded

by

a

non-treatment

decision

-LRB-

as

in

the

baby

Doe

case

and

the

baby

Rianne

case

-RRB-

and

according

to

Dorscheidt

the

decision

of

non-treatment

should

be

questioned

with

regard

to

the

non-discrimination

principle

as

well

He

elaborates

on

two

fundamental

rights

of

the

child

being

the

child

's

right

to

life

and

its

right

to

health

-LRB-

care

-RRB-

mentioned

in

international

human

rights

instruments

such

as

the

Universal

Declaration

of

Human

Rights

-LRB-

UDHR

-RRB-

The

United

Nations

Convention

on

the

Rights

of

the

Child

and

the

European

Convention

of

Human

Rights

and

on

the

authoritative

views

of

international

human

rights

bodies

such

as

the

United

Nations

Children

's

Rights

Committee

-LRB-

UNCRC

-RRB-

and

the

Committee

on

Economic

Social

and

Cultural

Rights

-LRB-

CESCR

-RRB-

Some

examples

Article

UDHR

Everyone

has

the

right

to

a

standard

of

living

adequate

for

the

health

and

well-being

of

himself

including

medical

care

and

the

right

to

security

in

the

event

of

disability

All

children

shall

enjoy

the

same

social

protection

CESCR

par

According

to

standard

Rules

States

should

ensure

that

persons

with

disabilities

particularly

infants

and

children

are

provided

with

the

same

level

of

medical

care

within

the

same

system

as

other

members

of

society

UNCRC

In

its

examination

of

States

parties

reports

the

Committee

should

commit

itself

to

highlighting

the

situation

of

disabled

children

and

the

need

for

concrete

measures

to

ensure

recognition

of

their

rights

in

particular

the

right

to

live

survival

and

development

UN

General

Counsel

We

will

take

all

measures

to

ensure

the

full

and

equal

enjoyment

of

all

human

rights

and

fundamental

freedoms

including

equal

access

to

health

education

and

recreational

services

by

children

with

disabilities

and

children

with

special

needs

to

ensure

the

recognition

of

their

dignity

to

promote

their

self-reliance

and

to

facilitate

their

active

participation

in

the

community

Proposal

text

of

the

American

delegacy

for

the

UN

Convention

to

Promote

and

Protect

the

Rights

and

Dignity

of

Persons

with

Disabilities

States

Parties

reaffirm

the

inherent

right

to

life

of

all

persons

with

disabilities

shall

take

all

necessary

measures

to

ensure

its

effective

enjoyment

by

them

and

shall

ensure

that

disability

or

perceived

quality

of

life

shall

not

serve

as

a

basis

for

infringement

of

the

right

to

life

-LSB-

-RSB-

These

international

political

statements

and

agreements

are

also

reflected

in

the

resolution

of

the

International

Federation

for

Spina

Bifida

and

Hydrocephalus

-LRB-

IF

-RRB-

as

formulated

at

the

International

Conference

-LRB-

The

Right

to

be

Different

-RRB-

for

Hydrocephalus

and

Spina

Bifida

in

Toulouse

-LSB-

-RSB-

People

with

spina

bifida

and

hydrocephalus

can

live

a

full

life

with

equal

value

to

that

of

any

other

citizen

and

they

should

not

be

seen

as

a

medical

condition

Their

views

should

be

sought

and

heard

by

governments

and

health

professionals

who

should

acknowledge

the

right

of

people

with

spina

bifida

and

hydrocephalus

to

speak

for

themselves.People

with

disabilities

have

the

right

to

aim

for

lives

as

rewarding

as

those

of

their

peers

who

have

what

is

regarded

as

a

normal

life

Supportive

systems

must

be

in

place

when

they

are

needed.Experience

over

the

past

years

has

improved

considerably

the

medical

outcome

and

the

quality

of

life

of

people

with

spina

bifida

and

hydrocephalus.Most

adults

with

spina

bifida

underline

that

their

quality

of

life

is

not

automatically

and

should

not

be

given

as

a

reason

for

abortion

-LSB-

-RSB-

As

a

recognition

of

the

importance

of

non-discrimination

on

the

basis

of

disability

-LRB-

that

is

to

say

present

as

well

as

expected

disability

-RRB-

or

-LRB-

expected

-RRB-

quality

of

life

in

deciding

whether

or

not

a

newborn

child

with

e.g.

spina

bifida

should

be

treated

or

whether

or

not

it

is

justified

to

deliberately

terminate

such

a

child

's

life

Dorscheidt

-LRB-

-RRB-

recommends

to

involve

the

legal

concept

of

objective

justification

in

the

medical-decision

making

process

By

using

a

particular

questionnaire

the

physician

in

charge

may

take

advantage

of

this

concept

when

considering

-LRB-

and

deliberating

on

-RRB-

how

to

act

properly

and

professionally

Through

this

the

physician

can

ensure

the

disabled

newborn

child

's

rightful

claim

to

equal

protection

of

its

right

to

life

and

its

right

to

health

-LRB-

care

-RRB-

Doing

so

would

offer

possibilities

to

replace

the

usual

doctor-centered

approach

of

decision

making

by

a

more

patient-centered

approach

as

well

To

summarize

from

another

perspective

Prominent

theories

of

justice

emphasize

equality

of

opportunity

for

human

experience

and

development

if

society

has

an

obligation

to

provide

citizens

this

very

broad

equality

of

opportunity

then

it

has

an

obligation

to

shoulder

the

burden

of

care

associated

with

citizens

who

have

spina

bifida

-LSB-

-RSB-

Palliative

care

for

untreated

newborns

with

MMC

When

a

newborn

with

spina

bifida

is

not

treated

discomfort

can

be

expected

in

the

nearby

future

caused

by

the

lesion

itself

-LRB-

e.g.

by

becoming

infected

-RRB-

by

a

subsequent

meningitis/ventriculitis

or

by

raised

intracranial

pressure

caused

by

an

untreated

hydrocephalus

Therefore

the

decision

not

to

treat

should

always

be

accompanied

by

offering

proper

palliative

care

It

is

becoming

increasingly

evident

that

taken

modern

palliative

techniques

in

newborns

seriously

pain

and

discomfort

can

always

be

dealt

with

in

an

adequate

way

-LSB-

-RSB-

In

their

article

Strong

opioids

in

pediatric

palliative

medicine

Hain

et

al.

-LRB-

-RRB-

also

stated

that

clinical

evidence

is

accumulating

that

strong

opioids

can

be

used

safely

and

effectively

they

should

be

used

as

part

of

a

rational

approach

to

the

management

of

pain

-LSB-

-RSB-

This

use

of

strong

pain

killers

is

also

highlighted

in

the

recent

Oxford

Textbook

of

Palliative

Care

for

Children

-LSB-

-RSB-

In

the

highly

unlikely

event

of

insufficient

effect

of

modern

pain

medication

terminal

palliative

sedation

should

be

the

next

step

in

the

palliative

approach

-LSB-

-RSB-

With

palliative

sedation

the

possible

experience

itself

of

discomfort

can

always

be

dealt

with

adequately

In

the

Consensus

guidelines

on

sedation

and

analgesia

in

critically

ill

children

by

Playfor

et

al.

-LRB-

-RRB-

in

Intensive

Care

Medicine

some

guidelines

are

mentioned

by

which

pain

and

discomfort

in

critically

ill

children

can

be

treated

in

an

efficient

way

life

termination

not

being

mentioned

at

all

-LSB-

-RSB-

When

it

is

decided

that

surgical

treatment

is

not

a

feasible

option

in

a

newborn

with

spina

bifida

it

is

regarded

by

some

to

be

an

act

of

mercy

to

terminate

the

life

of

the

newborn

-LSB-

-RSB-

This

notion

however

is

a

misconception

they

are

not

terminally

ill

because

of

the

MMC

and/or

hydrocephalus

per

se

they

are

terminally

ill

because

of

the

decision

not

to

treat

their

congenital

malformations

The

needs

of

these

untreated

newborns

however

are

still

the

same

as

the

needs

of

all

newborns

proper

care

feeding

comfort

respect

and

love

till

death

-LRB-

and

apparently

not

death

itself

-RRB-

The

need

for

life

termination

in

these

patients

can

only

be

regarded

as

an

indication

of

insufficient

palliative

care

Life

termination

as

an

act

of

mercy

can

only

be

applied

to

hopeless

situations

that

can

occur

e.g.

on

battlegrounds

or

in

mountaineering

when

there

are

no

proper

means

indeed

to

alleviate

real

unbearable

suffering

This

is

obviously

not

the

case

in

a

modern

equipped

hospital

-LRB-

at

least

it

should

not

be

-RRB-

with

experience

in

palliative

care

and

pain

management

This

is

all

confirmed

in

the

paper

of

Sauer

in

Ethical

dilemmas

in

neonatology

recommendations

of

the

Ethics

Working

Group

of

the

CESP

-LRB-

Confederation

of

European

Specialists

in

Pediatrics

-RRB-

in

the

European

Journal

of

Pediatrics

-LSB-

-RSB-

In

this

paper

some

ethical

principles

applicable

to

every

newborn

are

being

formulated

like

decisions

to

withhold

or

withdraw

treatment

should

always

be

accompanied

by

optimal

palliative

therapy

and

dignified

and

comforting

care

and

every

form

of

intentional

killing

should

be

rejected

in

paediatrics

-LSB-

-RSB-

This

is

followed

by

However

giving

medication

to

relieve

suffering

in

hopeless

situations

which

may

as

a

side

effect

accelerate

death

can

be

justified

To

date

it

is

not

clear

why

these

recommendations

should

not

hold

for

cases

of

untreated

spina

bifida

and

hydrocephalus

in

the

Netherlands

and

why

the

Dutch

Association

of

Paediatrics

does

not

adopt

these

recommendations

Illustrative

case

After

an

uneventful

pregnancy

a

full-term

female

child

was

delivered

spontaneously

Apgar

scores

being

and

Physical

examination

revealed

a

huge

MMC

at

high

thoracic

level

-LRB-

Fig.

-RRB-

a

very

large

head

with

a

large

but

soft

fontanel

a

marked

deformation

of

the

thorax

with

scoliosis

and

clubfeet

After

counseling

the

parents

they

persisted

in

their

wish

to

treat

the

child

if

this

probably

could

save

her

life

The

MMC

team

decided

to

respect

the

parents

will

and

on

day

the

back

lesion

was

closed

by

the

neurosurgeon

in

collaboration

with

the

plastic

surgeon

making

use

of

extensive

rotational

flaps

together

with

free

skin

grafting

-LRB-

Fig.

-RRB-

Shunt

placement

could

be

postponed

till

day

a

medium

pressure

valve

being

used

Because

postoperatively

the

fontanel

did

not

soften

adequately

weeks

later

the

medium

pressure

valve

was

changed

in

a

low-pressure

valve

now

with

satisfying

clinical

results

Fig.

of

the

newborn

showing

the

extensive

MMC

extending

from

the

thoracic

vertebra

till

the

midlumbar

regionFig

at

the

end

of

the

surgical

procedure

The

MMC

was

closed

by

making

use

of

extensive

rotational

flaps

together

with

free

skin

grafting

All

period

long

from

birth

till

week

after

the

shunt

revision

the

well-being

of

the

baby

was

estimated

by

making

use

of

the

Comfort

score

and

the

VAS

and

also

medication

used

for

treatment

of

pain

or

discomfort

-LRB-

paracetamol

and

morphine

-RRB-

was

recorded

-LRB-

Fig.

b

-RRB-

Fig.

Comfort

scores

during

the

first

month

of

life

-LRB-

min

no

discomfort

max

extreme

discomfort

-RRB-

Green

bars

only

paracetamol

-LRB-

intermittently

-RRB-

being

used

Green/black

bars

low-dose

morphine

together

with

paracetamol

being

used

b

The

same

as

a

but

now

with

the

VAS

-LRB-

min

no

pain

max

most

severe

pain

-RRB-

Note

The

combination

of

a

Comfort

score

of

at

least

and

a

VAS

of

at

least

indicates

that

the

well-being

of

the

newborn

is

in

jeopardy

In

such

a

situation

the

attending

nurse

is

expected

to

determine

the

cause

of

the

discomfort

and

to

take

adequate

measures

-LRB-

changing

a

full

nappy

feeding

a

hungry

child

emptying

a

full

bladder

giving

adequate

painkillers

if

appropriate

etc.

-RRB-

to

secure

the

child

's

well-being

Studying

these

data

and

particularly

the

amount

of

medication

needed

several

conclusions

can

be

made

When

untreated

-LRB-

the

first

days

-RRB-

the

child

was

not

discomfortable.Closing

of

the

back

lesion

in

this

case

certainly

a

major

procedure

did

not

contribute

significantly

-LRB-

and

only

temporarily

-RRB-

to

more

discomfort

In

other

words

closing

of

the

open

spine

can

be

qualified

as

a

proportional

treatment

modality.The

same

holds

true

for

placing

a

ventricular

shunt.Treating

an

active

hydrocephalus

adequately

contributes

to

the

well-being

of

the

patient.In

month

's

time

this

active

treatment

resulted

in

a

stable

situation

of

overall

well-being

in

this

patient.Only

paracetamol

and

low-dose

morphine

were

necessary

in

the

treatment

of

actual

discomfort

In

summary

this

case

not

only

demonstrates

that

there

is

no

such

thing

as

suffering

in

these

newborns

but

also

the

incorrectness

of

the

statement

of

Verhagen

et

al.

-LRB-

-RRB-

-LSB-

-RSB-

that

the

suffering

of

these

newborns

can

not

be

alleviated

by

any

means

in

a

proper

medical

way

-LRB-

Fig.

-RRB-

The

overall

impression

of

this

case

is

in

accordance

with

the

preliminary

results

of

the

Rotterdam

Prospective

Study

on

Discomfort

in

Newborns

with

MMC

there

is

no

acutely

unbearable

suffering

in

these

newborns

-LRB-

Figs.

b

-RRB-

Fig.

same

child

at

months

old

fixating

and

following

with

the

eyes

vocalizing

normal

movements

of

the

arms

moving

both

legs

vigorously

-LRB-

perhaps

nonvoluntary

-RRB-

still

being

partially

fed

by

a

nasogastric

tubeFig

and

b

Preliminary

results

of

the

Rotterdam

Prospective

Study

on

Discomfort

in

Newborns

with

MMC

-LRB-

n

-RRB-

Although

in

an

interview

Verhagen

himself

reported

that

all

cases

had

a

lesion

of

the

most

severe

form

at

level

of

the

neck

-LSB-

-RSB-

in

their

published

articles

Verhagen

et

al.

-LSB-

-RSB-

do

not

quantify

the

level

of

the

lesion

However

it

seems

very

unlikely

that

his

statement

is

correct

or

that

the

levels

of

lesion

in

these

cases

are

of

the

same

level

and/or

magnitude

as

in

the

case

described

Synthesis

and

conclusion

There

is

no

evidence

that

newborns

with

MMC

and

hydrocephalus

do

either

suffer

unbearably

or

hopelessly

and

certainly

not

without

the

prospect

to

relieve

this

suffering

by

standard

care

Suffering

itself

is

a

nonconclusive

and

in

newborns

inapplicable

denominator

that

should

not

be

used

anymore

in

this

debate

Although

they

will

in

their

future

life

be

confronted

with

handicaps

sometimes

very

severe

their

future

prospects

and

their

actual

experienced

quality

of

life

can

not

be

predicted

with

such

certainty

at

birth

that

their

lives

can

be

regarded

as

hopeless

or

meaningless

-LRB-

quality

of

life

judgments

as

such

being

unacceptable

in

this

decision

making

-RRB-

Possible

discomfort

in

these

newborns

can

easily

be

treated

in

a

straightforward

way

by

active

treatment

-LRB-

closure

of

the

defect

and

shunting

the

hydrocephalus

-RRB-

and

when

necessary

by

the

use

of

a

professional

pain/symptom

protocol

The

decision

not

to

treat

such

a

newborn

when

based

on

expected

handicaps

possibly

violates

the

non-discrimination

principle

-LRB-

Dorscheidt

-RRB-

When

not

being

treated

they

are

not

terminally

ill

because

of

the

MMC

and/or

hydrocephalus

per

se

they

are

terminally

ill

because

of

this

nontreatment

decision

Not

being

terminally

ill

it

is

not

humane

or

merciful

to

terminate

their

life

this

also

being

not

in

accordance

with

international

legislation

and

international

medical

recommendations

When

untreated

and

when

it

is

the

intention

to

alleviate

actual

discomfort

-LRB-

this

question

of

intent

being

crucial

to

a

moral

and

legal

analysis

of

end-of-life

decisions

including

active

termination

of

life

-RRB-

this

can

always

be

achieved

in

an

effective

way

by

using

one

of

the

widely

accepted

palliative

protocols

Such

a

child

can

and

should

be

cared

for

in

a

respectful

and

dignified

way

providing

all

its

actual

needs

-LRB-

which

apparently

is

not

death

itself

-RRB-

This

being

the

case

there

is

no

indication

whatsoever

for

the

deliberate

termination

of

the

life

of

children

born

with

MMC

While

Verhagen

et

al.

-LRB-

-RRB-

must

be

credited

for

bringing

the

discussion

about

deliberate

termination

of

newborns

in

the

open

to

date

there

does

not

seem

to

be

much

in

their

qualifications

that

can

be

quantified

properly

especially

not

in

cases

of

MMC

which

is

prone

to

quality

of

life

judgments

-LRB-

and

thereby

neglecting

the

needs

and

rights

of

the

newborn

-RRB-

To

date

the

Groningen

protocol

therefore

can

not

be

regarded

as

very

useful

Being

a

tool

it

seems

to

have

been

changed

in

a

means

in

itself

To

gain

more

usefulness

at

least

it

has

to

be

extended

in

a

way

as

proposed

by

Dorscheidt

-LRB-

-RRB-

by

adding

charts

such

as

the

Comfort

Score

and

the

VAS

and

by

adding

palliative

medication

charts

Thus

it

should

be

changed

from

a

doctor-centered

protocol

to

a

patient-centered

protocol

To

throw

more

light

on

these

end-of-life

decisions

Verhagen

et

al.

should

consider

to

extend

their

retrospective

study

focusing

on

the

medical

aspects

of

the

cases

Thus

it

must

be

possible

to

get

a

basic

understanding

of

the

extent

of

the

malformations

themselves

of

possible

co-morbidities

whether

or

not

quality

of

life

judgments

were

made

how

unbearable

suffering

was

ascertained

how

palliative

care

was

offered

and

why

this

failed

Such

a

study

would

contribute

significantly

to

the

open

discussion

they

propagate

Verhagen

is

undoubtedly

correct

indeed

in

his

appeal

It

's

time

to

be

honest

about

the

unbearable

suffering

endured

by

newborns

with

no

hope

of

a

future

-LSB-

-RSB-

Finally

to

date

it

remains

unclear

on

what

grounds

the

Dutch

Association

of

Paediatrics

has

adopted

the

Groningen

protocol

and

why

international

legislation

international

human

rights

instruments

and

international

medical

recommendations

appear

not

to

hold

for

the

Netherlands

especially

not

in

newborns

with

MMC

meningomyelocele
newborn
suffering
pain
quality of life
termination of life
palliative care
groningen protocol