Professor Frank Casey

Consultant Paediatric and Fetal CardiologistRoyal Belfast Hospital for Sick Children

Northern Ireland

PCICS 15th December 2018

Physical

Neurodevelopment

Social

Quality

of Life

Fetus Birth Newborn Infant Preschool age School age

Prenatal:

•Genetics•Dysfunction of fetal circulation•Dysfunction of cerebral vessel autoregulation•Delay of cerebral maturation

Preoperative:

•Cyanosis•Haemodynamicinstability•Microcephaly

Perioperative:

•Cardiopulmonary Bypass modalities•Circulatory arrest•Dysfunction of cerebral perfusion•Hypothermia•Haemodynamicinstability

Later factors:

•Persistent hypoxemia•Reoperation•Reduced physical fitness•Socioeconomic status•Genetics

Adapted from: Hövels-Gürich HH: Psychomotor development of children with congenital heart defects. Causes, prevalence and prevention of developmental disorders after cardiac surgery in childhood. Monatsschr Kinderheilkd 2012; 160:118-128.

Herberg U and Hövels- Gürich HH: Neurological and psychomotor development of foetuses and children with congenital heart disease –causes and prevalence of disorders and long-term prognosis. Z Geburtsh Neonatol 2012; 216:1-9

Fetus Birth Newborn Infant Preschool age School age

Diagnosis : Prenatal or Postnatal

ShockSense of LossAcceptancePreparation

Preoperative:Attachment IssuesFeeding Difficulties Medical TreatmentFinancial burdenRelationship Impact

Perioperative: Increased anxietyConcern about Mortality

Later factors:Lifestyle adjustmentEmployment issuesPhysical activity and SportSibling Impact

Brain and Heart Development occur simultaneously in the Fetus with congenital Heart Disease

CHD leads to derangements of fetal blood flow that can impair brain growth and development.

Delayed brain development leads to a unique vulnerability to white matter injury in children with CHD.

This in turn may lead to mild but pervasive Neurodevelopmental impairment commonly seen in children following neonatal cardiac surgery

• Early effects are mainly psychomotor and may which show recovery.

• Late effects may emerge – mild but distinct

difficulties for some in terms of other cognitive abilities

• Emergent evidence then is a “phenotype” with

planning, sequencing and organisation difficulties

• 41% (-v- 13% siblings) required extra help at school

• Cyanosis / LOT bypass predictive factors across time

BUT acyanotic conditions also at risk

• Maternal and family factors can either be protective

or exacerbate risk.

The first consultation with parents is the most important one.

Shock Anger Sense of loss Should allow for a period of emotional and

psychological adjustment to the diagnosis

Importance of role of Fetal Nurse Specialist

Emotional adjustment from healthy to sick child

Shock and anger

Attachment issues

Feeding difficulties

Medical Treatment and procedures

Financial strain

Relationship stress

Clinically elevated levels of psychological distress are apparent in one-third of mothers and almost one-fifth of fathers in the months following the birth of a child with CHD.

Maternal distress was not predicted by disease factors.

Maternal mental health as measured by outcomes on the BSI was predicted by:

(i) maternal coping skills. (ii) their understanding of the diagnosis. (iii) the degree of cohesive family functioning with poorer

cohesiveness being related to greater psychological distress.

Descriptive correlational study included 62 biological mothers of infants admitted to a PCICU within 1 month of birth who had undergone cardiac surgery for CHD.

Results—Mothers’ scores revealed that infant appearance and behavior was the greatest stressor, followed by parental role alteration, then sights and sounds. The combination of trait anxiety and parental role alteration explained 26% of the variance in maternal state anxiety. Mothers with other children at home had significantly higher state anxiety than did mothers with only the hospitalized infant.

Conclusions— Nurses are in a critical position to provide education and influence care to reduce maternal stressors in the PCICU, enhance mothers’ parental role, and mitigate maternal state anxiety.

“The ability to understand the meaning of the illness and its treatment: manage emotional reactions and continue to undertake necessary actions; fulfill responsibilities and provide necessary support”

Psychological Distress and Well-being

Severity of CHD

Poorer understanding of the diagnosis

Poor cohesiveness in the family

Gender differences- mothers more affected

Distress decreased post treatment of CHD

Illness related worry

Parental mental health

Family cohesion

Parenting styles

Sibling issues

Socio-economic status

Social Isolation

Financial stresses

The Psychosocial Impact on families of children with complex conditions may be determined by individual and family factors beyond the severity of the child’s condition

Familial factors such as cohesiveness and adaptive parental coping strategies appear to be paramount for successful parental adaptation to CHD in their child which in turn plays a significant role in parent’s and children’s long term well being

• Neurodevelopmental impairment in survivors of complex neonatal surgery is more highly associated with innate patient factors and overall morbidity in the first year than with intraoperativemanagement strategies.

• Improved outcomes are likely to require interventions that occur outside the operating room.

The C ongenital Heart

Disease Intervention

Programme

Early Psychosocial and parental education Initiatives in early

Infancy

Sponsored by The National Lottery Charities Board, U.K.

1. Narrative therapy – (CHIP DVD – Beyond the Diagnosis…)

2. Problem Prevention Therapy.

3. Psychoeducation and CHIP Manual

4. Facilitated Exposure – medical equipment, settings, professionals.

5. Maternal responsivity training – feeding and neurodevelopmental stimulation.

6. Generalisation of problem prevention therapy –family adjustment.

7. Community health professional education

Reduced worry and consequent reduced demands on health services

Increased knowledge and understanding

Improved coping skills on some dimensions

Improved infant mental, social and emotional development at 6mths

Some evidence for reduced feeding difficulties

Some evidence for improved behavioural adjustment

Mental Health - Mothers

Anxiety Scores - Mother

20

25

30

35

40

45

Baseline 6-Month 12-Month

Intervention Control

Mean Family Strain Scores from Baseline to One Year Follow-

UP

17.3

15.8

16.1

17

15

15.5

16

16.5

17

17.5

Baseline Follow-Up

Intervention Group

Control Group

Mental Health - Fathers

Anxiety Scores - Father

27

28

29

30

31

32

33

34

35

Baseline 6-Month 12-Month

Intervention Group Control Group

There is not a simple relationship between disease severity and long-term outcome for the child and family.

Socioeconomic factors and maternal worry significantly influence long-term outcome for the child with regard to health behaviour and physical activity

Early psychosocial intervention and multidisciplinary support to improve parenting skills and reduce maternal worry can impact positively on Infant Neurodevelopment.

Despite surgical and intensive care advances in the past couple of decades, rates of psychosocial difficulties have remained stubbornly unchanged.

Important to focus on Interventions to potentially modify outcomes.

Using modern technology to deliver interventions

Also important to focus on studying factors which confer resilience in research as much as factors which exacerbate risk and this should be a growing theme in our research.

First major separation from parents

Advice for parent and the teacher

Parent Information day - reinforce guidelines, answer questions

Patient specific information sheets-parent, teacher, GP

• Periodic developmental surveillance, throughout childhood may enhance identification of significant deficits, allowing for appropriate therapies and education to enhance later academic, behavioral, psychosocial, and adaptive functioning.

• Interventional programmes focused on behavioral adjustment, lifestyle education and family support can positively impact on the overall outcome for the child.

• Dedicated Developmental Follow-up Clinics for Children post surgery for CHD

Positive gains were found at 10-month follow-up in terms of maternal mental health and perceived personal strain in the family.

Children in the Intervention group missed fewer days from school and were perceived to have been sick less often than those in the Control group.

Although the proportion of children whose CBCL scores were in the clinically significant range nearly halved in the Intervention group, and increased slightly in the Control group, statistical comparisons were not significant

70 children from original cohort compared with nearest age healthy siblings

CBCL as rated by parents and teachers Independently.

Significant differences in◦ Social problems

◦ Thought problems

◦ Attention problems

Bringing home a child with major congenital heart

disease (CHD) is difficult

Anxiety levels are often high

As a regional service, patients may be distant from

specialist help if problems arise

Video conferencing codec

Tandberg 1000

Portable pulse oximeter

TelephoneVideo

Group

15.00

12.50

10.00

7.50

5.00

2.50

0.00

Red

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on

in

ST

AI sco

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35

p < 0.001*

Mean (s.d.) = 6.1 (3.0) Mean (s.d.) = 2.7 (1.6)

*Independent samples T-test

Family Impact

Paediatric Cardiology Relatives

Home monitoring Parent groups

Financial support

Clinical Psychology

Social work support

Nurse Specialists

• 14 month Neurodevelopmental outcome in 373 survivors

• Assessment by Bayley Scales of Infant Development

• Psychomotor Development Index (PDI)

• Mental Development Index (MDI)

• Mean PDI and MDI lower than normative means (p<0.001)

• Neither PDI or MDI associated with type of Norwood shunt

• Clinical Centre in which surgery performed

• Birth weight < 2.5 kg

• Longer Norwood Hospitalisation

• More complications between Norwood Discharge and age 12 months.

• Clinical Centre in which surgery performed

• Birth weight < 2.5 kg

• Genetic syndrome/anomalies

• Lower maternal education

• Longer mechanical ventilation after Norwood

• More complications between Norwood Discharge and age 12 months.

• NOTE: No relationship of PDI or MDI to perfusion

type or cardiac anatomy.

• The ever increasing cohort of surviving patients with complex CHD presents a challenge in optimizing overall quality of life.

• Many can have normal development but as a group these children are at increased risk of adverse neurodevelopmental and behavioural outcomes

• Aetiology is multifactorial

Childhood Health Screening

Two to Two and a Half Years

Nine Months to One Year

Six to Eight Weeks

One to Two Weeks

After Birth

ASQ-3 Questionnaire on Developmental Progress

• Early effects are mainly psychomotor and may which show recovery.

• Late effects may emerge – mild but distinct difficulties for some in terms of other cognitive abilities.

• Emergent evidence then is a “phenotype” with planning, sequencing and organisation difficulties

• 41% (-v- 13% siblings) required extra help at school

• Cyanosis / LOT bypass predictive factors across time BUT acyanotic conditions also at risk

• Maternal and family factors can either be protective or exacerbate risk.

Family stress, perceived social support and coping following the diagnosis of a child's congenital

heart disease.

Young Ran Tak, Marilyn McCubbin

Journal of Advanced Nursing Volume 39, Issue 2, pages 190-198, June2002

Journal of Advanced NursingVolume 39, Issue 2, pages 190-198, 27 JUN 2002 DOI: 10.1046/j.1365-2648.2002.02259.xhttp://onlinelibrary.wiley.com/doi/10.1046/j.1365-2648.2002.02259.x/full#f1

Poorer adjustment and anxiety in the child relates more to parental attitude than the severity of the incapacity - Linde et al 1982