Poster Presentation 33rd ASM of the Australian & New Zealand Bone & Mineral Society 2023

Quality of life (QoL) and transition care for patients with Osteogenesis imperfecta (OI) and X-linked hypophosphataemia (XLH) (#224)

Lucy Collins 1 2 , Angelina Lim 2 3 4 , Sara Al Waheb 4 , Natalie S S Chee 4 , Chee K Chew 4 , Giovanni G R Farag 4 , Katrina M K Gurdev 4 , Margaret Zacharin 1 2 5 , Peter Simm 1 2 5
  1. Department of Endocrinology, Royal Children's Hospital, Melbourne, Victoria
  2. Hormone Research, Murdoch Children's Research Institute, Royal Children's Hospital, Melbourne, Victoria
  3. Centre for Medicine Use and Safety, Monash University, Melbourne, Victoria
  4. Department of Pharmacy, Monash University, Victoria, Australia
  5. Department of Paediatrics, University of Melbourne, Melbourne, Victoria

Background

OI and XLH are the two most common metabolic bone conditions seen in the Bone Health Clinic at the Royal Children’s Hospital (RCH). The transition period for these patients represents a current challenge due to the lack of guidelines and standardised processes.

 

Aim

To evaluate the 1) QoL outcomes using a disease-specific questionnaire for patients with OI (1) and 2) Strengths and challenges of the current transition model.

 

Method

A cross sectional survey using questionnaires was given to OI and XLH patients of the RCH and staff involved in transition care.

 

Results

118 questionnaires were sent; 5 were returned to sender (incorrect address). 42 questionnaires were completed (33 patients with OI, 7 patients with XLH and 2 staff members). Patient characteristics are reported in Table 1.

 

In the OI cohort, increasing age correlated with lower “being careful” scores (r=-0.23, R2=0.0698) and higher “pain” scores (r=0.35, R2=0.0688). There was no correlation between QoL and the number of bisphosphonate doses received.

 

Themes surrounding the transition period for patients with OI and XLH included “unfamiliar”, “frustrating”, “no continuity of care” and “lack of understanding”. Suggestions for improvements included “5-year plan”, “valid referral”, “education”, “clear transition plans” and “consistency”.

 

Following transition from the RCH, patients received care (patient-reported) from a private endocrinologist (9/17, 53%) or public hospital (2/17, 12%). 6 patients (35%) received no ongoing care. Referrals to adult care (patient-reported) were organised by the RCH (6/17, 35%), GP (4/17, 24%) and emergency department (2/17, 12%).

 

Conclusion

The transition period is a challenging and frustrating time for our patients, with over one third no longer receiving care. A prospective trial is required to evaluate outcomes following the implementation of consistent guidelines and/or a personalised transfer tool.

 

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  1. Hill CL, Baird WO, Walters SJ. Quality of life in children and adolescents with Osteogenesis Imperfecta: a qualitative interview based study. Health Qual Life Outcomes. 2014;12:54.