A multidisciplinary approach to monitoring neurological & functional outcomes in paediatric HIV infection

Contents.

Practice Issues

Service Issues

Appendices

Ethos of developmental care

Echoing the 'Every Child Matters' paper in 2004 the ethos in the care of HIV children is to enhance wellbeing and quality of life. To improve outcomes for these children, careful attention needs to be given to monitoring all aspects of progress (physical, cognitive, behavioural, learning, social and emotional). Monitoring should include addressing the changing demands of daily life e.g., self-care, independence, growth and appearance, routines, relationships and school achievements etc., as the child moves through childhood and adolescence.

Background

HIV encephalopathy has been reported in paediatric HIV since the first cases were reported in the late 1980s and children most at risk were those who have an early AIDS diagnosis. In the pre-ART era the prevalence of encephalopathy reported in cohorts of children with HIV in health resourced countries was between 25-50% and resulted in significant morbidity and mortality with both static and progressive presentations being reported (European Collaborative study 1994, Wachsler Fielder & Golden 2002, Willen 2006). Significant co-factors have been identified in pregnancy, birth and early life which increase the risk of neuro-developmental consequences. These include prematurity, failure to thrive, maternal intravenous drug use and other early infections such as CMV which may arise because of a compromised immune system (Foster et al 2006). For those progressing past the early years, children who acquired HIV in the peri-natal period were found as a group to have more developmental weaknesses than children who acquired HIV later in life.

Data available from the UK population have confirmed findings from other studies that the most common neurological presentation is hypertonia of the lower limbs (similar to that seen in cerebral palsy and usually presenting as a diplegia). This results in impaired mobility of varying degrees. Other common developmental difficulties include early delays in talking, later poor expressive skills and sometimes sensori-motor weaknesses; these can exist with or without neurological signs (Foster et al 2006, Biggs & Melvin 2007).

Following the introduction of ARTs there has been an increase in survival status and improved health, growth and wellbeing (Gibb et al 2003, Judd et al 2007). The prevalence rates of severe encephalopathy and neuro-developmental consequences in children with HIV have reduced with better care and treatments both for positive pregnant women as well as for those children diagnosed in infancy (Willen 2006, Patel at al 2008). ART treatments known to have good central nervous system penetration have been found to be particularly effective in reducing the progression of neuro-developmental difficulties for children with vulnerable health and immune systems. Whilst ARTs cannot reverse neurological damage they may minimize effects on functioning by improved health and wellbeing and possibly enabling alternative neuronal pathways to develop which in turn enhance cognitive functioning.

As more children with HIV live longer, other developmental and learning weaknesses are being identified; these can sometimes be quite subtle and difficult to measure on global tests at young ages. Increased reports of difficulties with attention and concentration, remembering or applying skills in learning situations, or organising or sustaining effort are being reported (Koekkoek et al 2008, Patel et al 2008). These kinds of difficulties are suggestive of impaired executive functions (neuro-cognitive processes governed by prefrontal and midbrain areas in the brain) and their effects on performance and behaviour may increase as learning and social demands become greater with age. It is unclear yet what are the relative effects on these difficulties of vulnerabilities caused by early exposure to HIV and later chronic effects from the condition, treatments and other adverse experiences. It is thought that maintaining health and good viral control may help reduce effects of any executive functioning difficulties on performance but more has to be learnt about this.

It is becoming increasingly important to gather systematic longer term developmental and psychological data on the UK population who have grown up with HIV, not only to plan effective interventions and services to help optimise future outcomes, but also to try to understand risk and protective influences from health and other experiences.

Aims of developmental monitoring

The primary aim is early detection of any developmental weaknesses in a child in order to facilitate interventions which prevent and/or reduce the impact of these weaknesses on functions and progress.

Further aims:

• To provide ongoing data about general developmental needs of the population to allow for planning for service provision
• To help in understanding effects of treatments (particular combinations, length of treatments, timing of starting etc.)
• To promote a greater understanding of the chronic influence of HIV infection on developmental processes and identify associations with risk and resilience factors which may help develop new interventions.

Recommendations for developmental monitoring in paediatric HIV clinics – A tiered approach

Children with HIV with known significant problems or delays in development or functions
should be linked into appropriate services providing assessment and interventions for neurological or developmental problems, either within paediatric health or by referral to community child development or educational services. This needs to be done with appropriate consideration of how to protect the confidentiality of the child's diagnosis (see below).

However there is much variability in how, who and when children are identified with developmental difficulties across different centres, and access to appropriate interventions may be delayed. This may be a particular concern in the case of children who are newly diagnosed or recently arrived from other countries and have varied health and developmental histories. Whilst it is known that early intervention is most beneficial for future coping, both for the child and for their carers, caution needs to be exercised when assessing children from other countries, particularly on tests of cognitive and verbal functions. Many available tests may not have ethnically appropriate norms and newly arrived children will need some time to adjust to the changes in culture between different countries. However, sometimes monitoring is still useful if only to provide a general view of the child's level of functioning in comparison to their age peer group in the UK, rather than provide a reliable reflection of underlying potential (see Section 2 & Appendix 2).

This document recommends a developmental screening approach for all paediatric HIV services in the UK as a first step in identifying which children need more detailed assessment or referral. Detailed developmental, cognitive or functional assessments are often difficult to obtain in clinics because of limited resources or personnel to carry out such time consuming assessments on all children. Furthermore, some developmental weaknesses in this cohort may not meet the referral threshold for assessment or intervention by already stretched local services but these still require regular monitoring because demands and circumstances change.

The following provides some guidance for paediatric HIV services or clinics to help both in the systematic collection of developmental information and associated psychosocial factors on all children attending clinics (a standard core set of outcome markers – Section 1) as well as providing an example of a developmental screening tool for use with all clinic attenders (Section 2). Suggestions are then made for key times and areas to be assessed in those services with access to resources enabling more detailed and specific developmental and cognitive assessments (Section 3 & 4).

1. Annual reporting of core developmental indices on all children attending HIV clinics in UK

Aim: To collect data on general neuro-developmental problems to contribute to UK database on outcomes and to identify significant changes.

The following table lists core presentations and referrals representing neuro-developmental concerns. It is suggested that these figures are collected each year and the population is reported on in terms of year of birth. See Appendix 1 for example of audit sheet developed by Biggs & Melvin (2007).

2. Developmental checklist (screening sheet for completion by carer and child)

Aim: To review concerns about progress from carer and older child to help with early detection of difficulties and referral to appropriate agency if needed.

A screening questionnaire, completed by staff at the HIV service with the carer and child and covering concerns about progress and a check list of observations would both help contribute to the above indices and help identify whether a child needs more in depth assessment or referral to another agency. Appendix 2A gives an example of a screening questionnaire developed by the PHP – UK Paediatric HIV Psychology group. This includes both a baseline checklist and a follow up review (see Appendix 2B).

A developmental baseline on newly diagnosed HIV children soon after engagement with medical care can provide a useful way of identifying whether some aspects of progress need further support or investigation. They can also be a helpful guide to monitoring adjustment to new systems and demands for those newly arrived in the country. However care must be taken in interpreting scores from those newly arrived from other countries as many developmental and cognitive tests may not be appropriately standardised for children from their ethnic backgrounds and they may still be adjusting to the changes in their lives.

3. A review at key times of change

Aims: To gather any evidence of developmental vulnerabilities at known key times of change or adjustment i.e. significant developmental stages e.g. starting school or end of primary school (10 -11years) or times of psychosocial change e.g. newly arrived youngsters or part of discharge planning prior to transfer to adolescent/adult services.

The kind of assessment possible at these key times of change may depend on the expertise of the professional, availability of tests and amount of time available to carry out such assessments. Appendix 3 highlights appropriate functions to assess at key age ranges as well as the kinds of interventions which may be appropriate should difficulties be identified.

Some examples of core assessment tests are suggested in this table with an emphasis on tests with UK norms. Screening for progress in literacy and numeracy is also helpful through school age either via a school report or using tests such as the WIAT (Wechsler Indices of Achievement Tests). Inclusion of a generic health related quality of life measure, such as the Pedsqol, and ratings from the screening questionnaire (Appendix 2) both of which reflect concerns of the child and carer, can enhance information collected during this review and should be considered the minimum to collect at these key times.

It is hoped that collection of some general developmental data at these key times becomes routine practice for all centres.

4. More specific or detailed assessment

Aim: To assess detailed developmental, learning and cognitive profiles associated with changes in health and treatments.

At present some clinics have access to psychology, physiotherapy or other child development professionals and can provide more in-depth assessments for individual patients with concerns about progress. More detailed assessments are time consuming and require access to specific tests and staff trained in their administration and interpretation. Further, this is a culturally diverse population, with a wide age range, and careful selection and interpretation of results is needed to provide a reliable picture of children's functioning. Also the timing of assessment for children newly arrived into the country requires careful consideration.

It is suggested that a standardised test of global cognition such as the WISC IV should form the initial assessment tool, but it is also becoming increasingly clear that global tests of development or cognition such as IQ are not always sensitive in picking up more subtle effects on function or performance resulting from changes in health or immune status, treatments or new stressors. More specific neuro-cognitive tests, particularly those monitoring attention, working memory and information processing as in executive functions may provide more useful data. Items from tests such as the Test of everyday attention (TEACH), Working memory scales, NEPSY, Kaufman Assessment Battery etc., can be useful. Non–verbal items from these tests may also be more reliable.

There are also some computer based tests such as the CANTAB which are useful for executive functions and which are free from cultural or linguistic bias. Furthermore assessing speed of performance such as pegboard or tapping speed can also be useful e.g. Movement Assessment Battery for Children (Movement ABC). At present few paediatric HIV services have access to services or test resources which can carry out such in depth neuro- psychology and neuro-cognitve assessments.

In future, support for a multicentre resource with a specialist and multidisciplinary neuro-developmental/neuro-cognitive assessment team, may enable such neuro-cognitive and performance assessments to be available more widely to local HIV centres. This centre could also be a focus for interventions and research, e.g. monitoring effects of treatments, and could link with other services such as neuro-imaging. Such a resource could be shared across other chronic or paediatric specialties.

Referral to local services

The importance of referral for early intervention for developmental difficulties is recognised by parents as well as services. However because HIV remains a stigmatising condition and there is often a lack of up to date information about Paediatric HIV within general paediatric, educational and community services, there can be anxiety about a referral to a developmental or educational service. Conversations about the benefits for sharing the HIV diagnosis for the child's future care can be helpful, together with a discussion about confidentiality which includes to whom and how the information will be shared, and can help reduce parental concerns. Parental consent for referral is essential and the information being shared needs to be clearly shared. Sometimes several discussions will be needed with parents or carers as there may be considerable anxiety about the sharing of sensitive information. Explaining how services work in the UK and what they can provide can also be very helpful for families newly arrived who may have experienced different provision in their country of origin.

Whilst it is generally better to share full knowledge about a child's diagnosis when referring to new services it may not always be possible or appropriate to do so especially when making an initial enquiry about what services are available and what the referral pathway entails. Sometimes an initial referral describing the child's difficulties i.e. that they have mobility problems, are slow in talking, are struggling with work at school etc., can be used to make the first engagement with a new service. This can help parents and carers have time to build up trust and form a relationship with new providers and services before a full discussion about the diagnosis occurs. Sometimes identifying a key person within the service where referral is to be made can be helpful, and that staff at clinic have checked out how sensitive information is stored. It can often be difficult for anxious parents or carers to negotiate this by themselves.

There is no legal obligation to share children's HIV diagnosis with schools, nurseries or colleges and doing so should always be led because there is a need and benefit for the child (Conway 2005). It helps to remember that there are many children in schools who may have some difficulties without there being a known cause. Sometimes it can help to discuss that a child's difficulties with development or with school progress may have arisen because of early illnesses; a partial explanation. However in some circumstances, sharing the HIV diagnosis may be essential and will need to be explained to parents e.g. if the HIV service is asked to contribute to a Statement of Special Educational Needs for a child.

It helps to remember that many local child development, educational and community services in the UK have little up to date knowledge about children and HIV and often welcome some extra general information and support about the condition from the HIV clinic. Staff from the HIV clinic helping support the initial contact a family has to an educational or community referral, by a visit or telephone call, can be reassuring for both parents and the receiving service.

Summary

To ensure the best functional outcomes and wellbeing of the child, developmental monitoring should be a core part of the management of paediatric HIV at all centres.
Regular screening, especially at key stages for all children, should become part of clinical practice. It is suggested that a multi-centre resource is one way of making specialist multidisciplinary developmental and neuro-cognitive assessments available for those with more complex problems.

References