Summary
Lipodystrophy and other severe insulin resistance (SIR) syndromes are rare causes of diabetes which are frequently associated with delayed diagnosis and severe metabolic disease. Diagnosis and management is complex and guidelines for management do not currently exist. The National Severe Insulin Resistance (NSIR) Service was commissioned by the National Specialist Commissioning Team for adults and children with lipodystrophy and/or SIR in England. It offers a multi-disciplinary clinic with consultants in adult and paediatric Diabetes and Endocrinology, specialist nurses and dietitians and support from the clinical genetics and obesity teams. The NSIR service uses a new model of cross-organisational working which includes the same team providing adult and paediatric management with several members of the same family often affected.
Results
The team has seen 130 patients, with four to six new referrals a month. The first 86 patients with SIR and/or lipodystrophy attending the clinic between April 2011 and March 2013 were audited. Analysis showed a reduction in median (IQR) HbA1c between the first clinic visit and at six-12 months follow up.
Patient satisfaction was high. There is encouraging feedback from referring clinicians, and referral rates increased after article publication conference presentations. Several teams have requested assistance with the use of Humulin-R U500 insulin. The service continues to be commissioned by NHS England.
Challenge
The rarity of severe insulin resistance and lipodystrophy leaves patients subject to poor clinical management and outcomes, but research has contributed to improvements. Since 1996, patients with severe forms of insulin resistance but not severe obesity have contributed to a unique collection (‘biobank’) of DNA and blood samples. The genetic basis for 10 previously uncharacterised syndromes have been identified to date.
Research regarding the effects of recombinant leptin therapy on severely obese children with congenital leptin deficiency provided experience for our current clinical practice. The clinical applicability of this research led to the Department of Health commissioning a national multidisciplinary severe insulin resistance NHS service based at Addenbrooke’s Hospital in Cambridge, the only such service in the world.
Objectives
The service aims to support patients and carers, and to establish and disseminate evidence-based therapy recommendations. Mechanisms include: provision of a precise diagnosis, patient education, raising the profile of severe insulin resistance/lipodystrophy as a clinical problem, and provision of treatment interventions such as dietary and pharmacological therapies.Feedback has been encouraging. There may be potential to set up centres elsewhere in the UK. It is hoped the service will be commissioned for patients in Wales, Scotland and Northern Ireland, although this is subject to Department of Health funding. It is likely that diagnosis in patients with apparent type 2 diabetes will become more complex and accurate in the future.
Solution
A multidisciplinary team was recruited and a weekly clinic arranged. Patients were admitted to the medical programme investigation unit and protocols developed for assessing insulin requirements.
Referrals have been encouraged by presentations at clinical meetings, and local teams remain involved in patient care. All patients are given a copy of their clinic letter, and a website and patient information leaflets were produced.
A shared care guideline and risk management strategy for the use of Humulin R U500 insulin were created. The NHS laboratory can now provide genetic testing in most common genetic causes of SIR. Biochemical analyses are performed in our laboratory and samples processed in the clinic by NSIR team members.
The service specification is available on the NHS England website and data is collected and reported to NHS England on a monthly basis, with six-monthly service reviews.
Learnings
When setting up the new service the team tried to engage potential users at the early stages, requesting a lot of feedback. They learnt that patients valued psychological support over long term metabolic data. Patients also liked having a copy of their clinic letter and an accessible team.
Feedback from the MDT was important, especially when working with a combined adult and paediatric team. Weekly team meetings were held and communication were maintained with GPs and other diabetes centres. NHS.net email was useful but not always available.
Persistence and robust data collection systems were important in getting funding. Constant review and flexibility in how the services was ran proved essential, and patient feedback helped highlight areas for improvement.
Evaluation
Feedback has been encouraging. There may be potential to set up centres elsewhere in the UK. It is hoped the service will be commissioned for patients in Wales, Scotland and Northern Ireland, although this is subject to Department of Health funding. It is likely that diagnosis in patients with apparent type 2 diabetes will become more complex and accurate in the future.
