Rett Syndrome and access to critical care during the COVID-19 crisis


Since Reverse Rett published this article on 24th March 2020, NICE have made some amendments to the guideline on critical care.

In particular the use of the Clinical Frailty Scale in people with disabilities was updated due in large part to pressure from patient organisations like Reverse Rett and other disability advocates. 

At Reverse Rett, we remain highly concerned about the guideline, even with the amendments.

Here are the key issues:

1. Whilst the guideline now states:

Be aware of the limitations of using the CFS as the sole assessment of frailty. The CFS should not be used in younger people, people with stable long-term disabilities (for example, cerebral palsy), learning disabilities or autism. An individualised assessment is recommended in all cases where the CFS is not appropriate.

Rett Syndrome is not likely to be considered a stable long term disability, like autism. 

The guideline still also states ‘consider comorbidities and underlying health conditions in all cases.’ 

This fundamentally places people with Rett syndrome of all ages at a huge disadvantage due to the inherent health problems in Rett syndrome, which exist alongside the learning disability element of the condition.Despite the fact that the SCFN and the guideline states that the CFS shouldn’t be used in groups including people with learning disabilities and autism, we are still worried about how the guideline will be used in highly pressurised situations.

2. There is no mention in the guideline of reasonable adjustments which need to be made, when isolation processes may separate vulnerable adults from their families and carers and when someone does not have either the ability and or the capacity to discuss whether they want critical care.

3. The algorithm still shows that if you are considered to be frail, you will not be able to access critical care.

4. The Specialised Clinical Frailty Network (SCFN) website and literature still states that the CFS can be undertaken by any appropriately trained healthcare professional (doctor, nurse, health care assistant, therapist etc.) which means that judgement of frailty which will drive decisions about critical care can be made by staff without knowledge understanding or expertise in Rett Syndrome or rare diseases at large.

5. There is still no requirement to work with families in decision making about access to critical care.

We believe these remaining issues will lead to situations which contravene UN Convention on the Rights of Persons with Disabilities Article 25 which state that:

States Parties shall:

Provide persons with disabilities with the same range, quality and standard of free or affordable health care and programmes as provided to other persons and prevent discriminatory denial of health care or health services or food and fluids on the basis of disability.

We continue to monitor the situation and push for further, more clear and categorical amendments to the guideline.

We will keep you informed of developments.