Zara is a 3-month-old baby who attended the GP surgery regularly with fever and infection. Zara received the BCG vaccine at birth, administered to the left upper arm. This was appropriate, as her grandparents were born in India, where the incidence of tuberculosis is reported at 167 per 100,000. Zara was taken to A&E by her mother who was becoming increasingly concerned with the large lump under Zara’s left arm and her worsening cough. Zara was admitted to the children’s ward. A thorough medical history was taken.

Zara was born at 40 weeks’ gestation by vaginal delivery. She was the second child of Sumaya and Mohammed who reported no medical complaints. Sumaya was worried as some of her relatives had children who died in infancy, but as they lived in India there was limited additional information.

Zara appeared to feed well but was not gaining weight and had dropped from the 75^th to 25^th centile on a growth chart since birth. Blood results indicated that Zara had no B and T lymphocytes.

• What is the likely diagnosis in this scenario  
• How will you nurse Zara on the children’s ward before transfer to an immunology centre?

Answer: Zara has Severe Combined Immune Deficiency (SCID) due to signs and symptoms including recurrent infections, local lymphadenopathy following BCG vaccine, family history of childhood death.

Nurse Zara in strict isolation using an incubator to reduce exposure of infection.

The importance of adherence to treatment has been highlighted. What would need to be considered before initiating treatment? What reasons may lead to poor adherence in children and young people? How would you support a child and family due to start lifelong treatment?

Answer: Pre-treatment: understanding of HIV, understanding of how medicines work, lifestyle, identifying who will support/give/observe medicine taking.

Reasons for poorer adherence:

1. Taste, size, frequency and amount of tablets or liquids
2. Beliefs, culture, religion
3. Past experience
4. Family life, support, role model
5. Routine, stress
6. Young carer
7. Exams
8. Social life, want to be ‘normal’, weekends, alcohol, recreational drugs
9. Don’t feel unwell
10. Reminder of diagnosis
11. Understanding of diagnosis and medication
12. Side effects

Things that may help:

1. Contact details of team if any worries or questions
2. Calendars
3. Alarms
4. Texts
5. Parent/carer support
6. Routine, e.g. brushing teeth
7. Labelling syringes
8. Home support
9. Direct observational therapy by family member
10. Education/knowledge of diagnosis/understanding how medicine works
11. Support groups/1-to-1 work

Susan is 13 years old. Susan was treated at an HIV specialist family clinic, where her mother also received her treatment and care. They both attended their appointments regularly, their HIV was well controlled with medication and they presented as essentially well.

The local children’s social care team received a referral from Susan’s school due to her poor attendance. A social worker made contact to arrange an assessment.

Mum was very reluctant to allow the social worker to visit. When the social worker visited, their home was found to be in an extremely poor state of hygiene. Medicine was scattered all over the floor and empty take-away food containers were piled high. It was evident that Susan slept in the same bed as her mother as her room was inaccessible due to piles of clothing and belongings. The assessment revealed that mum no longer cooked and they relied on take-away food.

The health information revealed that Susan’s physical health had been consistently good. The high level of school absences appeared to be related to mum’s need for her daughter to be at home. Susan’s mother had become very dependent, and Susan presented little independent identity. It was clear that mum had mental health issues, which were previously unrecognised.

The family’s health professionals collaborated in the safeguarding assessment and support plan, which included ongoing community visits to support the family in their home as well as clinic-based care.

The case demonstrates the complex social and psychological impacts HIV can bring to families who struggle to cope, where access to wider support and understanding can be difficult. It reinforces the importance of awareness of how in certain situations the welfare of the child is at risk and needs prompt intervention and support to ensure that the child’s welfare is paramount.

(Amanda Ely, social worker and projects manager, CHIVA website)

• What would be the priorities for the care team supporting Susan and her mum in this case study?

Answer: Support Susan and mum by addressing the home environment, healthy eating, clinic engagement, developing social skills for Susan and mum, accessing local services, engaging with healthcare team, communication between professionals involved.