Inherited Retinal Disease (Australia) PSP question verification form

The purpose of this Question Verification Form is to enable Priority Setting Partnerships (PSPs) to describe clearly how they checked that their questions were unanswered, before starting the interim prioritisation stage of the process.

The JLA requires PSPs to be transparent and accountable in defining their own scope and evidence checking process. This will enable researchers and other stakeholders to understand how individual PSPs decided that their questions were unanswered, and any limitations of their evidence checking.

Name of the PSP

Inherited Retinal Disease Priority Setting Partnership

Please describe the scope of the PSP

The aim of this PSP, herein referred to as the Inherited Retinal Disease PSP, is to identify the evidence uncertainties about Inherited Retinal Diseases. These evidence uncertainties will be from the perspective of:

patients, defined as individuals who are 16 years or older, diagnosed with any type of IRD (including syndromic IRDs such as Usher syndrome);
guardians and family members (e.g., a parent, grandparent, sibling, or partner) who support an individual (of any age) with any type of IRD (including syndromic IRDs); and
health and supportive care professionals, defined as those who have direct contact with the above defined patients in their role, and have a focus on addressing/supporting vision loss (e.g., ophthalmologists; genetic counsellors; social workers, and occupational therapists).

The scope of the Inherited Retinal Disease PSP includes questions about IRDs, that relate to:

prevention of disease;
diagnosis;
disease progression and control;
treatment and research into potential treatments;
epidemiology; and
management, which includes management of the physical, psychological, emotional, financial, and social aspects of living with an IRD, or caring for an individual living with an IRD.

We will exclude responses from individuals impacted by age-related macular degeneration, and exclude questions about:

Symptoms of IRDs that are not focused on vision loss (e.g., hearing loss for some syndromic IRDs)
Other eye diseases (even if they are concomitant with the IRD).

Please provide a brief overview of your approach to checking whether the questions were unanswered

In partnership with the Steering Group, we will collate and categorise all ‘raw’ submissions from our first survey into summary questions using the PICO format. We will undertake a literature search of each summary question (that is deemed in-scope) and categorise each as being:

Answered: Reliable, up-to-date systematic reviews, meta-analyses or clinical guidelines has already been published.
Partially answered:

Relevant, reliable and up-to-date systematic reviews, meta-analyses and evidence-based guidelines but do not address continuing questions.
Relevant systematic reviews but not up-to-date (i.e., published before 2013).
Current clinical trial but not exhaustive enough to answer the question

Not answered: No relevant systematic reviews, meta-analyses or clinical guidelines identified

All questions categorised as ‘2’ or ‘3' will be deemed unanswered and taken to interim priority setting.

Please list the type(s) of evidence you used to verify your questions as unanswered

We will verify questions as unanswered taking into consideration the following evidence types:

systematic reviews
meta-analyses
evidence-based national guidelines
clinical trials: clinical trials will be used to identify questions that may be answered in the coming years. An ongoing study may not mean that an uncertainty will be addressed. Steering group members will discuss ongoing studies and document any decisions made because of information found.

Please list the sources that you searched in order to identify that evidence

The following searches will be undertaken as relevant for each question:

Cochrane Reviews, using keywords specific to each question.
Database searches on MEDLINE, CINAHL, PsycINFO, Embase, PubMed and Google Scholar, using keywords specific to each question.
Royal Australia and New Zealand College of Ophthalmologists (RANZCO) guidelines
American Academy of Ophthalmology (USA) guidelines
Royal College of Ophthalmology (UK) guidelines
German Ophthalmological Society (DOG), the Retinological Society (RG) and the Professional Association of German Ophthalmologists (BVA) guidelines
Deutsche Ophthalmologische Gesellschaft, DOG/ Retinologische Gesellschaft e. V., RG/ Berufsverband der Augenärzte Deutschlands e. V., BVA joint guidelines
Optometry Australia (Australia) guidelines
British College of Optometrists (UK) guidelines
American Academy of Optometry (USA) guidelines
Human Genetics Society of Australia guidelines
American College of Medical Genetics guidelines
Australian and New Zealand Clinical Trials Registry (ANZCTR) and clinicaltrials.gov

What search terms did you use?

For our search on Cochrane Reviewers and database searches, we will include:

systematic review* OR meta analys* OR meta-analys* OR metaanaly* OR meta synthes* OR meta-synthes* OR review* OR guideline*) AND
(inherited retinal disease OR retinitis pigmentosa OR usher OR vision loss OR stargardt OR cone dystrophy OR vision impairment OR blind OR retinal dystrophy) AND
[additional search terms to reflect the concepts/focus of each question]

Please describe the parameters of the search (eg time limits, excluded sources, country/language) and the rationale for any limitations

Time limits

Searches will be limited to evidence published from 2013-2024. Where we are aware that older evidence has answered a question, and further research or an update may be unnecessary, this evidence will be presented to the Steering Group, with a consensus reached on whether the question is considered ‘answered’ or ‘partially answered’.

Country and language

Searches will be limited to publication in English in a peer-reviewed journal (or peak body, in the case of guidelines)
Evidence from outside of Australia will be included. However, some questions may require Australian-specific evidence (e.g., specific to the Australian healthcare system), which will be discussed with the Steering Group.

Sample

Scenarios where we find evidence that is not restricted solely to individuals with an IRD (e.g., includes a sample of individuals with an IRD and age-related macular degeneration) will be discussed with the Steering Group, with consensus reached on whether the question has been answered.
Similarly, there may be scenarios where broader literature in other genetic conditions may be included. Again, these scenarios will be discussed with the Steering Group.

Names of individuals who undertook the evidence checking

Dr Eden G. Robertson, with additional support from Associate Professor Lauren Ayton, Associate Professor Anai Gonzalez-Cordero, Professor Robyn Jamieson, Dr Alan Ma, Dr Meredith Prain, Professor Matthew Simunovic, and Professor John Grigg

On what date was the question verification process completed?

February-March 2024