Maggie Breen-CNS CYPOONS RMHBev Barclay-RGN/RSCN Nursing Director

J’s Hospice

Lives at home Father - prison officer

Mother - teaching assistantBrother Jed 13yearsSister Alex 17years

Radiological (MRI) diagnosis July 03

1yr prior history of GP contact/paediatrican and neurology review due to blurred/ double vision

Joe’s favourite subject sport - completes long distance running on regular basis

- Know experiencing right sided squint after exercise for prolonged periods

Treatment Cranial radiotherapy

Completed GCSE/ A levels Participates in sports Works at Barclays bank (Clerk &

computers) Qualified car driver Has girlfriend-close intimate relationship Lives at home and has close relationship

with Alex (sister) who has a young son Clinical and radiological (MRI) evidence

of disease progression

• Prior to oral Temazolomide Joe decides he wants to sperm bank

Chemotherapy Oct 06 - Sept 07 Clinical signs of progression Dec 07 Joe 19 years• Temazolomide (different regime) Dec 07- March 08 - Letter to employer-request break from

computer during working day

Review in clinic - atypical course

? Potential for adult clinical trial - biopsy for histological diagnosis (June 08)• Anaplastic Astrocytoma WHO Grade 3

PCV chemotherapy regime June 08-Oct 08- July 08 letter for A/E department local

hospital (no shared supportive care)

• Referral to adult phase 1 clinical trial drug development unit but:

- LFTs elevated, thought to be drug (chemo) related not disease SO

• Liver biopsy Nov 08

• LFTs remain elevated so not eligible for clinical trial (concern at wait by Joe & his parents for improved LFTs, as Joe’s physical health declining)

Joe’s weekly LFTs check Dec 08 - LFTs stabilising therefore OPD

appointment late December (Joes request) Jan 09 drug development unit-

performance status (scale) makes Joe ineligible for open phase I clinical trial

Jan 09-Feb 09 oral Cyclophosphamide/ Retinoic acid/Doxycycline

March 09 Joe at home- RIP

Paediatric service - Family centered care

TYAC service- Individualised & family centered care

Adult service

- Individualised care

Joe was referred to an adult palliative care team Lead care from a paediatric tertiary cancer centre District General Hospital (adult service) did not

know Joe – refused to share supportive care needs - District Nurses unable to access his Portacath

Adult palliative care team: ◦ desire to care and palliate◦ Tertiary hospital - (expertise on tumour type),

Clinical trials (benefits / risks) ◦ On treatment at time of death◦ Potential stress between expectations (of

clinicians/ Joe and/or parents?) and the reality of what was possible.

Challenges (cont)

Important when working with young people we do not replicate the paediatric model

Joe given every opportunity to discuss death and dying-it was his choice not to engage

Important to consider capacity in brain tumours

He chose to maintain hope and talk about the future

• Health:– Feeling of weakness– Loss of energy–Body image (physical) changes– Less able to participate in sportsJoe felt that the changes lowered his standing

with his peer group

• Independence– anger at his increasing dependence on his

parents (loss of control)–missed driving his car–Relationship with siblings (?unequal)

• Relationship with girlfriend– broke off his relationship – protected her from the

pain of separation caused by death– did not want to be pitied

• Absence from work– interfered with his vocational plans– financial concerns– Social role

• Effective team working was central across care boundaries

• Joe’s trust in paediatric neuro-oncologist/paediatric neurosurgeon?– reflection– adaption– full team participation

• The skills of the MDT team were recognised and valued

How:-◦ effective communication (not always given the respect

it deserves)

◦ Support

◦ Information

◦ appropriate advance care planning

◦ access to equipment

◦ 24 hour symptom control

Palliation aimed at young people needs to be flexible and responsive to their (and parents) needs

For Joe to die at home the necessary services and infrastructure were available.

Involvement in decision making wanted his parents involved (Parents have always been involved) wanted to be at home independence very important to Joe wanted opportunities to do “normal” things

Important: although young people may have the same physical symptoms as adults, not all will be in a position to cope with them or to accept that death is unavoidable.