Good practice where no adult services are available Charlotte Dawson Consultant in Adult Inherited Metabolic Disorders Queen Elizabeth Hospital Birmingham (with outreach clinics in Bristol and Taunton)

What is transition Barriers to transition Transition in areas with no commissioned adult service Providing specialist care in areas with no commissioned adult service

Transition is a transfer of care: Paediatric team to Adult team

Transition is a transfer of care: Parent to Patient If this doesn’t happen effectively there is a risk of non-adherence to treatment, failure to come to appointments etc

‘Good’ Transition Planned Gradual process Adjusted to patient maturity Agreed by patient, family, and both paediatric and adult health teams

Barriers to transition Protective Vulnerable adult with complex needs Attachment to paediatric team Fear of the unknown Fear of losing control More competition for resources More dispersed care with no single care coordinator Different system for accessing benefits Larger hospitals More dispersed care Healthcare teams unfamiliar with the condition Centralised and hospital-based Long relationship through difficult times Familiar environment Paediatric care Adult care Patients and families Community and social care

NHSE-commissioned regional IMD services Adult IMD services Paediatric IMD services

Barriers to transition in areas with no commissioned adult service Protective Vulnerable adult with complex needs Attachment to paediatric team Fear of the unknown Fear of losing control More competition for resources More dispersed care with no single care coordinator Different system for accessing benefits Larger hospitals More dispersed care Healthcare teams unfamiliar with the condition Centralised and hospital-based Long relationship through difficult times Familiar environment Paediatric care Adult care Patients and families Community and social care

Healthcare teams may be unfamiliar with the condition Providing care in areas with no commissioned adult service Attend at least one appointment at Children’s Hospital before transfer of care Enthusiastic local contact(s) Excellent communication with diagnostic departments (biochemistry, radiology) Emergency management plans and contact numbers in patients’ notes Flexible and pragmatic approach to appointment frequency Telephone consultations available if preferred Patients with life-limiting conditions or complex needs are seen in home environment with involvement of community teams Contribute to educational opportunities Larger hospitals More dispersed care Healthcare teams may be unfamiliar with the condition More competition for resources Adult care

Community and social care Transition in areas with no commissioned adult service Excellent communication is essential Paediatric care Adult care Community and social care Patients and families This happens anyway, it’s a little harder over the phone / by email but not that much harder

Case study Providing care in areas with no commissioned adult service 20 year-old male San filippo syndrome (MPS Type IIIa) Lived in Wiltshire Paediatric care at GOS Care transferred to adult services aged 18 Parents no longer able to bring him up to London Lost to follow-up for two years GP contacted department to say he was having frequent seizures, severe movement disorder, recurrent chest infections and hospital admissions

What happened next? IMD consultant and CNS visited patient at home Providing care in areas with no commissioned adult service What happened next? IMD consultant and CNS visited patient at home Community nurse, GP, parents and carers also present Discussed prognosis, likely complications and agreed that they could be managed out of hospital Involved local palliative care and respiratory teams IMD team produced document detailing how to manage complications at home signed and agreed by all involved in his care

Providing care in areas with no commissioned adult service And finally….. Regular communication between GP and IMD team to advise on medication Seizures and movement disorder settled Chest infections treated with oral antibiotics via PEG and home oxygen No further hospital admissions Died peacefully at home eleven months later

Providing care in areas with no commissioned adult service “Dear Dr Dawson, Just a note to convey our thanks to yourself and Jane Lodwig for coming to see X at home. Your recommendations were really appreciated and I’m sure that X’s excellent care towards the end of his life was in part due to yourselves. “

Case 2 18 year-old with classical homocystinuria Providing care in areas with no commissioned adult service Case 2 18 year-old with classical homocystinuria Mild learning disability Paediatric care under Bristol Royal Hospital for Children (BRHC) Seen by consultant and nurse from Birmingham adult team in his final paediatric appointment Transitioned in early 2016 First adult appointment attended by nurse from BRHC Came with both parents

Homocysteine level always highly satisfactory in paediatric care Providing care in areas with no commissioned adult service Homocysteine level always highly satisfactory in paediatric care Result on blood taken at clinic surprisingly high despite no apparent changes to medication

What happened next? Adult IMD team phoned patient and spoke to him directly not taking medication when at college eating burgers etc during the day Adult IMD team made arrangements with local hospital to have blood samples taken and sent to Bristol Maintained regular communication to help him understand the importance of adhering to treatment and simplified his treatment regimen Homocysteine level now satisfactory on less prescribed medication

Summary Adult care is always less centralised than paediatric care Difficulties with coordinating care are greater in areas where there is no commissioned adult service Providing care in these areas requires: Excellent communication Detailed understanding of patients’ individual needs Consideration of alternative models of care

But it’s not perfect…… Transition occurs at a later age in SW England Access to IMD services is inequitable based on ability to travel to a clinic Many patients with IMDs in SW England are seen by non-specialists and do not have access to 24 hour cover and other services available in specialist centres No overall accountability for care

General practitioner School / college Hospital teams Patient society Community health professionals Nurses Physiotherapists Dieticians Family / carers