The illness history for a child with a malignancy may be relatively short; parents may take their child to the GP with a history of a persistent infection, as with Jonathan in the following case scenario. GPs are usually the first professional encountered by the family. A common investigation for children/adolescents with a history of recurrent infection, abnormal bruising or bleeding, lethargy or abnormal lumps is a blood count for the assessment of their haemoglobin, platelets and white cell count including a differential.

Activity

• What are the normal ranges for a full blood count and differential?

• What do the terms anaemia, neutropenia, thrombocytopenia and pancytopenia mean?

GPs are likely to see only one or two cases of childhood malignancy in a 20-year practice but education regarding the signs and symptoms of these conditions and the need for prompt intervention has seen a significant improvement in GPs’ responses to such symptoms over the last 30 years (Pinkerton et al 1994).

It is likely that the admission to the district general hospital will be for a short period of time, in some cases just an overnight stay while arrangements are made for transfer to the regional paediatric oncology centre where the diagnosis and prognosis will be confirmed and treatment commenced. Transfer to another centre will raise the patient’s and parents’ anxiety levels. They require written details and an explanation of how to get there, the name of the ward and staff that are expecting them. It is also useful to be able to advise them what to expect when they arrive at the centre. This can help the parents prepare themselves and their child to face a children’s ward where they will see children/adolescents who are undergoing treatment and who have altered body images such as alopecia.

Information booklets about the regional cancer unit are useful for the patient and family to enable them to prepare for the transfer.

Due to a greater knowledge about childhood malignancies, the confirmation of the diagnosis is rarely a medical emergency. However, for the child/adolescent and parents it will be a time of great emotional distress and uncertainty (Kars et al 2008). It is vital, therefore, that the investigations are planned to cause the minimum amount of physical and psychological stress for the child and family. However, due to the neutropenia, children/adolescents can become acutely ill very quickly, therefore nurses need to be able to identify children/adolescents at risk of developing septic shock, recognising subtle changes in their condition, and be able to prioritise appropriate emergency care.

Establishing venous access is often the first priority, both for assessment of blood values and to facilitate the administration of medications, hydration and the correction of haematological and electrolyte abnormalities prior to further investigations being carried out. The type and variety of investigations required will be decided upon depending on the suspected diagnosis. Bone marrow aspirates and trephines are carried out to confirm and determine the type of leukaemia (acute lymphoblastic leukaemia and acute myeloid leukaemia), lumbar punctures are required to identify the presence of leukaemia cells in the cerebrospinal fluid. Children/adolescents with solid tumours are likely to undergo a combination of:

Effective communication between the different departments involved in the investigative procedures is essential. It is also important to remember, however, that the patient may have to experience the investigations numerous times throughout treatment – the preparation for the procedures is, therefore, crucial.

Consideration for the use of pharmacological interventions, such as sedatives or anaesthesia, and non-pharmacological methods, such as distraction, play and hypnosis, for procedures that are invasive, painful or that require the patient to remain still should be given (Christensen & Fatchett 2002). Assessment and intervention by the play therapist is valuable as relationships are just beginning to be established. Carrying out many procedures under one anaesthetic or sedative requires expert planning and negotiation skills. If the investigation is deemed not to be invasive or painful, or the patient is considered to be able to comply with instructions, it will still be necessary to assess that the patient is free from physical and/or emotional pain/discomfort and establish appropriate therapeutic interventions where required (Pinkerton et al 1994).

This is an extremely difficult time for the patient, parents, other family members and the members of the healthcare team. Effective communication is vital to minimise additional stress and to establish trusting relationships that are essential to assist the child and family as they start this journey (Clarke et al 2005).

Parents see themselves as advocates for their child (Holm et al 2003), making medical decisions, limiting the actions of healthcare professionals and learning about their child’s condition. Their education in order to participate, therefore, is paramount.

The confirmation of the diagnosis will bring many mixed reactions and emotions. The family whose child has had a short history of a mild illness may find it difficult to accept that their child has a life-threatening illness, whereas for the family who have encountered a delay or complications in the confirmation of diagnosis and whose child’s condition has continued to deteriorate, it may come as a relief in that they can now begin treatment. Buckman (1992) advises that, although specific details of what was said will be forgotten, the way in which the news of the diagnosis is given to the family will be remembered. It is important, therefore, that the confirmation of the diagnosis is a planned event. Privacy, an appropriate environment, time and honesty are key issues. Parents will search for a cause for their child’s illness and a meaning of the illness (Ruccione et al 1994). Parents feel responsible for their child, yet at this stage they lack knowledge, authority and power to participate in healthcare decisions. It is important that they receive the right amount of information and feel listened to (Clarke & Fletcher 2003). Interestingly, Clarke-Steffen (1993) identified that once parents felt they had been told the details of the worst things that could happen to their child, they felt more able to trust the professionals to be honest with them about subsequent information or news.

It is recommended that children/adolescents are informed of their diagnosis, treatment and prognosis (Eiser et al 1994). However, many factors need to be taken into consideration, such as the existing communication strategies within the family, cultural and religious beliefs, and the child’s age and level of development (Price 2003).

It must be remembered that parents will react in different ways depending on their coping strategies, previous experiences, relationships and culture. The grief that the families experience when they hear the diagnosis has been likened to Kubler Ross’s (1969) model of grief.

Although it is recognised that children/adolescents have a right and a need to know their diagnosis (Eiser et al 1994), negotiation with the parents will help to decide the best way this is carried out for their child. Parents may need time to come to terms with the diagnosis first, but delay in confirming the diagnosis with the child/adolescent might result in finding out via an alternative source, which can subsequently lead to difficulties in establishing trusting relationships. Telling the patient and family together may be valuable for some families, for others the parents may prefer to break the news themselves. Price (2003 p 38) identifies the following strategy to assist information giving to children:

It is recognised that the confirmation will come as a shock and the ability to remember information will be reduced; it is vital, therefore, that the patient and parents feel reassured that they can ask staff to go over information as often as is required for them (Pinkerton at el 1994).

An outcome of the establishment of the UKCCSG in 1978 and the organisation of the 22 regional centres has been the increase in the number of children/adolescents that are entered into clinical trials, which evaluate current treatments, test new drugs or test existing drugs in different dosages, ways of administration or combinations.

Although recruitment of adults with cancer into trials is low (approximately 30%) the figures for children/adolescents is much higher; 90% of children/adolescents with a malignant disease are registered with the UKCCSG, 80% of these are eligible for entry onto trials and 70% actually participate in trials (more details are available at: http://www.cancerresearchuk.org). As previously mentioned, the rarity of childhood cancer makes this vital because the significance of findings would be difficult to evaluate if the numbers entered onto trials was low.

The aim of treatment is to remove or to kill the malignant cells and achieve a cure. The term ‘cure’ refers to the patient who can be expected to have the same life expectancy as any other child/adolescent who does not have a malignancy. Quality of life (QoL) for patients is an important consideration when deciding upon treatment approaches. QoL for cured patients is also very important and will be discussed in more depth in the cure/survivorship section. Cure usually refers to patients that have not been receiving treatment for 5 years and have no evidence of disease.

The main treatments for childhood malignancies are:

Children/adolescents undergoing treatment for a malignancy are likely, therefore, to receive a combination of therapies, which may last for months or, in the case of leukaemia, years. Some patients will undergo long periods of hospitalisation, others will receive much of their treatment as outpatients, attending both the regional centre and the shared care hospital. The effective management of side effects, therefore, is vital.

Effective management incorporates the physical and psychological care for the patient and the psychological care of family members. Symptom management for a patient undergoing treatment for a malignant disease requires nurses to have knowledge of the: