Chapter 25: Care of children and young people with haematological problems

ANSWERS TO ACTIVITY 25.1: CRITICAL THINKING

Before reading more of this chapter, consider how a lack of one or more of the constituents listed in Table 25.1 could affect health and wellbeing.

Answer:

Lack of stem cells would lead to lack of all other blood cells leading to bleeding, infection and anaemia.

Lack of mature white blood cells (leucopenia) makes the child or young person at risk of overwhelming infection.

Lack of red blood cells (anaemia) reduces the ability of the blood to carry oxygen and causes fatigue, paleness, headaches, dizziness, fast breathing (tachypnoea), fast heart rate (tachycardia) and cold extremities. Prolonged anaemia impairs growth and development.

Low platelets (thrombocytopenia) leads to easy and excessive bruising, petechiae (pinprick red/purple spots), purpura (larger, flat purple patches), nose bleeds, bleeding gums, blood in urine, blood in faeces, and heavier periods in older girls.

Lack of blood plasma (hypovolaemia) may be caused by external or internal bleeding, severe burns, diarrhoea and/or vomiting. It causes tachycardia, diminished blood pressure, and reduced perfusion (the skin turns pale and there is reduced capillary refill). The individual may feel dizzy, faint, nauseated, or very thirsty. Children will typically compensate (maintain blood pressure despite loss of blood volume) for a longer period than adults but will deteriorate rapidly and severely once they do begin to decompensate.

Lack of albumin will allow excess fluid to move from the blood capillaries into the interstitial fluid causing the affected tissue to become swollen (oedema). 

Lack of clotting (coagulation) factors will lead to a tendency to bruise easily, excessive and prolonged bleeding from cuts, and a tendency to bleed into joints and muscles causing pain, swelling, limitation of movement and long-term damage. Women with bleeding disorders may experience problems during menstruation, pregnancy and childbirth.

ANSWERS TO CASE STUDY 25.1: LUCY

Lucy was a seven-year-old girl diagnosed with aplastic anaemia following a history of a petechial rash and pallor. Clinicians initially adopted a ‘watch and wait’ approach, to see if she would make a spontaneous recovery. When this did not occur, Lucy was admitted into hospital for insertion of a Hickman line and commencement of immunosuppressive therapy of anti-thymocyte globulin (ATG) horse and cyclosporin. The therapy was generally well tolerated, but Lucy needed many platelet and red blood cell transfusions and intravenous antibiotics to treat infections. This required Lucy to stay in hospital for prolonged periods, as she lived a two-hour drive from the hospital in a rural home she shared with her parents and younger brother.

Initially there was not a clear response to treatment, and a bone marrow transplant was considered.  Gradually an increase in haemoglobin and platelets demonstrated a slow but satisfactory response to treatment.  Weekly blood counts, reviews and platelet transfusions still meant regular trips to the hospital.

Cyclosporin doses were slowly reduced. Lucy was able to attend clinic every two weeks. To allow Lucy to stay at home, oncology nurses from the hospital taught local district nurses to manage her central line.

  • What support would help Lucy and her family cope with her hospitalisation?

Answer: Lucy and family would need the following support:

  1. Practical support – basic information regarding the ward and hospital amenities such as facilities, parents’ accommodation, café, bank machines, etc.
  2. Emotional support – time with multidisciplinary team to talk about her condition and treatment.  Play specialists for Lucy and brother.  Consider psychology input. Suggest notepads for questions or a diary for noting feelings,
  3. Spiritual support – offer support such as hospital chaplain.
  4. Financial support – consider social work input, grants from local charities, access to applying for government carer’s allowances.  Basic advice on budget options in hospital such as going to local supermarkets to save money, or suggesting relations bring in food for family to save money,
  5. Education support – information regarding disease, treatment and prognosis.  Information on caring for central venous catheters.  Play therapy to help Lucy come to terms with hospitalisation.
  6. Social support – encourage family to personalise room, bringing in own toys, linen, etc.  Encourage family to participate in care.  If family separated suggest Skype/Facetime calling to keep up regular contact.
  • How might the disease have an impact on her family?

Answer: Family may have to split up due to distance; brother may have to stay with other family members while Lucy and parents in hospital.  Financial implications may affect whole family and the lifestyle they are accustomed to.  Stress may cause extra family anxieties.  May have a long-lasting impact on the siblings as jealousy of parents’ time may become an issue.

  • What advice was required to help Lucy’s family promote her health while she was pancytopenic?

Answer: Health promotion in pancytopenia involves different approaches for different elements of the blood. If a child is low on any component of the blood, a healthy diet should be promoted to aid the recovery of the cells.

If a child has thrombocytopenia, they should be advised to avoid activities that may induce injury, such as contact sports.  They should not be given ibuprofen. Patient and families should be aware of symptoms of possible low platelets.  Older girls may need medication to stop periods.

Families of an anaemic child should be informed that the child may need to be encouraged to rest. The child and family should monitor for breathlessness or chest pain and should seek immediate medical assistance if this should occur, as a transfusion of red blood cells may be required.

In the neutropenic child, avoiding infection is vital. Children and families should be instructed to avoid others with infection, and to avoid large crowds. They should be advised on signs and symptoms of infection, and who to report this to. All foods must be cooked carefully, and the child should not receive unpasteurised products or anything that might cause food poisoning. Families should have a thermometer to measure the child’s temperature, as if a fever occurs, immediate medical assistance and intravenous antibiotics will be required.

Neutropenic teenagers must be advised of the particular importance of using protection against sexually transmitted diseases.

The following website provides information, advice and support for families about Aplastic Anaemia: www.aplasticanaemia.co.uk/

ANSWERS TO SCENARIO 25.1: HOLLY

Holly is a 15-year-old with sickle cell disease. She takes regular penicillin to prevent infection, and folic acid to promote the production of red blood cells. She tries to drink plenty of fluids and stay warm and free from stress. However, recently, with school exams approaching, she has had a couple of painful crises. This has meant time off school and in hospital, where she required opioid analgesia, oxygen, intravenous antibiotics and fluids. Holly is worried that she might not do as well as expected in her exams. She is also aware that the severe pain she experienced was due to blockage of her blood vessels by the sickle-shaped red blood cells. She knows such blockages could cause permanent damage to her organs and bones. She is scared she might have a stroke and die. This happened to a relative. Holly does not feel able to discuss her fears with her family.

  •  Who do you think could provide support for Holly?

Answer: Holly may be able to speak with friends but she may find they do not understand her condition. She may not want to discuss her concerns with them as she may not want to be seen as different from them. She could access peer support through a local or online sickle cell support group.

A school nurse, a teacher, youth worker, or religious minister could provide support and advice and signposting to other sources of support.

Holly could speak with her GP/practice nurse and/or with a specialist nurse/team.

A recent report about the healthcare experience of adults and children with sickle cell disease and their parents indicates support varies across England: www.pickereurope.org/wp-content/uploads/2016/04/Sickle-Cell-Pilot-Report.pdf

These resources highlight best practice in care of children and young people with sickle cell and thalassaemia at school:
www.dmu.ac.uk/research/research-faculties-and-institutes/health-and-life-sciences/sickle-cell-education/resources-for-schools-and-parents.aspx

ANSWERS TO SAFEGUARDING STOP POINT 25.1

Multiple bruises on an infant should raise concerns about whether the child is being cared for in an unsafe environment or has been harmed. However, large amounts of bruising can also be a sign of low platelets (thrombocytopenia, see above) caused by rare conditions such as Glanzmann's thrombasthenia.

  • How would you respond to the parents of an infant who claim they have no knowledge of how such bruising has occurred?

Answer: In order to form and maintain an effective professional relationship with parents it is important to respectfully listen to and to document what they say. It is usually best to keep parents informed about who you will be passing any information onto, for example another nurse or doctor. As a registered nurse you may need to explain to parents if police and social workers need to be informed.

  • What blood tests do you think would be required?

Answer: Blood tests which are likely to be required include a full blood count to find out the number of blood platelets the baby has and also prothrombin time and partial thromboplastin time to see how long it takes for the baby’s blood to clot.

ANSWERS TO SCENARIO 25.2: KYLE

Kyle is a two-year-old with haemophilia A. His mother, Catherine, is a carrier. Kyle has been on prophylactic factor VIII therapy for a year. A decision was made to insert an implanted port and both his parents agreed to be trained in its use. His father, Bruce, is not as confident as Catherine at accessing the port, but Catherine needs his help to hold Kyle still. Catherine is frustrated that Bruce often arrives home late from work, which delays the time Kyle can be given his prophylaxis and settled to sleep. This causes frequent rows between Catherine and Bruce.

  • As a nurse how could you support Kyle, Catherine and Bruce?

Answer: You could help Kyle to find ways to cope more easily with the procedure. At the moment he is possibly responding to his parents’ anxiety and anger and may also be very tired when the procedure is taking place. For more advice about therapeutic holding see Royal College of Nursing (2010), Restrictive Physical Intervention and Therapeutic Holding for Children and Young People. Guidance for Nurses. Available at: www2.rcn.org.uk/data/assets/pdf_file/0016/312613/003573.pdf

Kyle is at an age when he is likely to enjoy routine and also want to be independent, rather than have things done to him. There may be things which he can safely hold during the procedure to make him feel he is playing an active role. Making up a simple silly song about what is being done, which he can join in, may help him feel less anxious. He may be able to lie still if he is allowed to cuddle a special toy or watch a favourite video or play a game on a phone or tablet whilst he is having the procedure done.

Catherine and Bruce need the opportunity to discuss their thoughts and feelings about the procedure. Caroline may be feeling guilty because Kyle has inherited the disease from her. She may also feel resentful that she is having to carry out the procedure every day. Bruce may feel he is working long hours to support the family and be reluctant to face an angry Catherine and Kyle at the end of a tiring day. It is possible that Caroline and/or Kyle may appreciate some further teaching/support from a nurse carrying out the procedure within their home environment. They may agree for another family member/close friend who they and Kyle trust to be taught to undertake the procedure or to help with it.

ANSWERS TO WHAT’S THE EVIDENCE? 25.1

Different centres have different cut-off levels for transfusing platelets for invasive nursing interventions, such as intramuscular injection and passing nasogastric tubes. What’s the evidence for this?

Answer: The practitioner should consider the child's condition and the cause of their thrombocytopenia, if the platelet count is recovering or likely to fall further and when the procedure is due.  This should be weighed up against the risks of a further platelet transfusion.  NICE (2015) recommend considering prophylactic platelet transfusions to raise the platelet count above 50×109 per litre in patients who are having invasive procedures.

Reference

National Institute for Health and Care Excellence (2015) Blood Transfusion NICE Guideline NG24. London: NICE. Available at: www.nice.org.uk/guidance/ng24/evidence (last accessed 23 May 2017).