Introduction

Most children seen in primary or secondary care have an acute illness and most will make a complete recovery. When this does not happen, and ill health drags on, it impairs a child’s natural, lively, sociable lifestyle. Care of these children and their families is as much about making life as normal as possible as about treating disease. This requires thoroughness, understanding and teamwork.

Chronic disease

The prevalence of chronic disease in childhood is between 6% and 11%. Common chronic childhood diseases are listed in Table 3.1 and details of these conditions are found in the appropriate chapters. They produce a wide variety of clinical symptoms and require specialist assessment and management. Principles underlying the care of all children with chronic disease are highlighted in this chapter.

Table 3.1 Common chronic childhood diseases by system

Some conditions are present at birth and result in lifelong disability. Others, e.g. Perthes’ disease, asthma and epilepsy, may have an impact on health for a few years before resolving. Many conditions are life-threatening and a few lead to progressive disability.

Most children with chronic disease are managed as outpatients. It is better for children to be at home or at school as much as possible, and treatments are increasingly geared to this goal. Even highly complex interventions such as intravenous treatments and assisted ventilation can be managed at home by well-supported and trained carers and through the use of specialist home nursing teams.

The child’s perspective

Try to view the problem from the child’s perspective. What professionals, parents or teachers might consider to be the major issues may well be at odds with the child’s concerns and fears. Listening to the child is vital. Children will tell what it is that’s bothering them if professionals take the time to ask and to listen. Children will often feel better simply for being listened to. If they will not talk to doctors they will often pour out their hearts to other team members such as nurses or therapists. Health professionals are in a powerful position to advocate for children and – sometimes – change their lives for the better.

An approach to chronic illness

An holistic approach views the child’s needs in five domains: medical, educational, social, developmental and emotional.

Initial management of chronic childhood illness – breaking bad news

Telling a family that one of their children has a chronic disease or a chronic neurodevelopmental problem demands skill and empathy. Traditionally, we have referred to this process as ‘breaking bad news’, which has strong negative connotations, so the term ‘sharing the news’ is now preferred. The ground rules for these conversations are clear:

It is not appropriate for a senior house officer in a busy clinic to blurt out to a mother that her 4-month-old baby may have cerebral palsy. Once the conversation starts doctors should:

Although the family wants to understand the problem, their level of distress may make it hard to listen. Despite careful news sharing interviews, parents may walk away believing the very opposite of what has been said. Repeated advice and explanations are likely to be needed until the truth ‘sinks in’.

When parents imagine their yet-to-be-born child, they expect to meet a healthy infant. If the child is born prematurely, or with a developmental or medical problem, they face an uncertain, perhaps frightening, and certainly daunting future. There is a psychological need to move on from the child they wanted, to the child they have. Parents often need much support during this transition.

Part of the way forward for all families is to grieve for the loss of the child who perhaps never was. This is hard. The parents may be thrown into the hectic life of caring for a child with special needs, and not have adequate time for themselves. If grieving does not take place, some parents embark on a quest for the miracle solution that will restore their ‘lost’ child, leading to excessive referrals and unnecessary, inappropriate and unproven treatments. Care and compassion may ‘unlock’ normal feelings of guilt, anger, sadness, failure, and lead to the ‘discovery’ of their special child.

The perceived vulnerability of the sick child may lead to the parental response of seeing their child as very special and precious. This sick role can persist throughout childhood, curbing normal social development and promoting dependence. Parents may need encouragement to allow the child to experience the hurly-burly of normal life.

The question of causation and blame will sometimes cause difficulties. The family may blame obstetric, paediatric or other health professionals, with or without just cause. If with justification, then explanation and apology are appropriate. Most parents want the truth, and an expectation that lessons are learned. Only if they meet a wall of silence do most families become entrenched. A sense of ‘genetic’ guilt can be destructive. Is it the mother’s fault or the father’s? Does it run in one side of the family?

Paradoxically, it may be as hard to cope with an apparently more minor problem. Sympathy and understanding from the broader social network may be lessened if it is not clear that the child has a difficulty, e.g. the hyperactive child may be thought of as naughty, and the blame laid at the parents’ door.

Working together for children with chronic disease

For many families, accessing the care and support they need is a struggle. Teamwork is the key to planning and delivering services for these children and their families. Teams may be multidisciplinary health-based teams or multi-agency teams that include social services and education, according to specific needs. These teams must work with the primary health care team.

The asthma team in Case 3.1 constitutes the paediatrician, the asthma nurse and the school health adviser; the GP and practice nurse will also be involved. A cystic fibrosis team might include a paediatrician, dietician, physiotherapist, nurse specialist and psychologist. When children suffer complex neurodevelopmental problems lots of professionals may become involved. See Table 3.3 for a list of those involved in the care of chronically ill children.

Table 3.3 Professionals involved in the care of children with chronic disease

The list in Table 3.3, although long, is by no means exhaustive or unrealistic. If families are to make any sense of this, the agencies must coordinate their activities and offer sensibly planned care. Usually care is supervised, at least in the early years, by a child development team – often staffed by a community paediatrician, a group of therapists and, on occasion, staff from education and social services. A key worker as the main link to the team is useful in helping families navigate the complex maze of health and social care. Combining professionals into mini-teams accessed from the same venue at the same time is also good practice. For example, an orthopaedic team might consist of an orthopaedic surgeon, a paediatrician, a physiotherapist and an orthotist. At school age, care may be planned by a school-based team, and in adolescence transfer to adult services needs further planning and teamwork.