Intellectual disability
Intellectual disability (also known as learning disability in UK health services) is characterised by significant impairments of intellectual and adaptive functioning with onset in childhood. People with intellectual disabilities (PwID) have higher rates of physical, mental and behavioural disorders compared with the general population. Communication deficits and impaired cognition can impede people from identifying their difficulties or distress and communicating them to get the right support and advice. PwID can react to changes in their physical, mental and emotional states as well as to any changes in their social or environmental circumstances. This can be presented as challenging behaviours towards themselves, towards property or others. PwID are also disadvantaged by premature mortality. Increased prevalence of physical and mental health conditions, low health literacy, diagnostic overshadowing combined with challenges accessing healthcare are some of the factors that have led to premature mortality among PwID.
Intellectual disability and COVID-19
PwID represent one of the most vulnerable populations to any pandemics for many different reasons. These include multimorbidity, low levels of health literacy, difficulties in understanding and communication, reliance on other people for care, low compliance with complex hygiene rules, the strong need for routine/sameness and low adaptive skills. This became more evident during the COVID-19 pandemic. As the pandemic unfolded, an increased risk of hospital admissions and mortality from COVID-19 among PwID became clearly evident.Reference Williamson, McDonald, Bhaskaran, Walker, Bacon and Davy1,Reference Perera, Laugharne, Henley, Zabel, Lamb and Branford2 A threefold higher risk was recognised for PwID than the general population in succumbing to COVID.3 Studies looking at mortality in this cohort have identified several associated factors such as Down syndrome, epilepsy, mental illness, dysphagia and severity of ID.Reference Perera, Laugharne, Henley, Zabel, Lamb and Branford2,3 These alarming statistics have resulted in PwID being prioritised for the COVID-19 vaccination programme.
Apart from the direct effect of COVID-19 illness, the disproportionate indirect impact of the pandemic on the mental health of PwID and their carers has also become evident. Travel restrictions and change in human contact has led to increased levels of distress and behaviour change in these individuals. The impact of the pandemic on the mental health well-being of PwID is yet to be studied in large samples. Small-scale studies have shown the possibility of increased mental health difficulties as well as increased prescribing of psychotropic medications in PwID.Reference Naqvi, Perera, Mitchell, Rory Sheehan and Shankar4 One also has to acknowledge the impact on the mental health of PwID when their families, carers and staff get COVID-19.
Post-COVID-19 syndrome (PCS)
Although most patients recover completely after COVID-19 infection, some continue to have persistent symptoms or develop new symptoms which is known as PCS or long COVID. The National Institute for Health and Care Excellence defines it as signs and symptoms that develop during or after an infection consistent with COVID-19, continuing for more than 12 weeks (3 months), and not explained by an alternative diagnosis. Commonly reported symptoms are breathlessness, fatigue, neuropathic and musculoskeletal pain, cognitive difficulties and psychological distress. There are more than 200 symptoms reported involving more than ten organ systems making it a challenging multisystem condition to manage. It is estimated that there are more than 1 million adults in England alone with PCS and over 40 million worldwide. The underlying pathophysiology is not yet clearly established but an underlying exaggerated immune response, endothelial damage causing hypercoagulation, direct and indirect brain and central nervous system damage, dysautonomia and psychological response are all contributory and linked mechanisms driving these symptoms.
Studies have shown around 10% of people who was positive for COVID-19 developed PCS. The self-reporting COVID-19 Yorkshire Rehabilitation Scale (C19-YRS) has been validated in the general population.Reference Sivan, Halpin, Gee, Makower, Parkin and Ross5 It captures a range of subjective and objective symptoms.
Concerns about recognising PCS in PwID
Given that acute COVID-19 is worse in PwID, it can be argued that the rate of PCS in PwID is likely to be worse or at the least similar to that in the general population. However, there is no published evidence on the prevalence rate of PCS in PwID. Furthermore, recognising PCS in PwID can be challenging as the ability to articulate symptoms or distress can be very limited because of communication difficulties and lower intellectual functioning in this cohort. As neuropsychiatric symptoms including depression, anxiety, delirium and psychosis are already prevalent in PwID they can be difficult to separate out as sequelae of PCS. It also creates clinical challenges and diagnostic dilemmas in identifying whether the symptoms are a sequalae of COVID-19 or not. Key symptoms of PCS such as fatigue and pain are difficult to describe for people with communication problems leading to diagnostic overshadowing.
Why capturing this and researching this area is important
Given the disabling symptoms or functional difficulties in PCS, the importance of estimating the scope of the problem in PwID is the need of the hour. It can be argued recognition of PCS in PwID may help to better understand the behavioural and neuropsychiatric manifestations of PCS in this population and improve management including reducing usage of psychotropic medications.Reference Naqvi, Perera, Mitchell, Rory Sheehan and Shankar4 Comorbid neurodevelopmental conditions such as autism may make it more difficult to accurately tease out the contribution of PCS to the clinical presentation in these individuals. A clear understanding of the impact of PCS in PwID will also help for future planning for social care given the high rate of support needed in this population. Complications seen in PCS such as pulmonary fibrosis, myocarditis, pericarditis, cardiac arrhythmias, renal and hepatic dysfunction, deep vein thrombosis/pulmonary embolism may contribute to increased mortality and morbidity. Appropriate and timely management can be put in place with better understanding of the condition. PCS is a long-term condition and is also likely to make these individuals more vulnerable to other infections and complications further accentuating morbidity and mortality. Finally, given the significant healthcare utilisation and economic impact of PCS in the general population, the financial implications need to be estimated in PwID, which will inform service planning and commissioning.
Recommendations for clinical practice and research
The World Health Organization has suggested the triple R approach to PCS – Recognition, Research and Rehabilitation. The Appendix outlines a logical approach to build evidence on the recognition and impact of PCS in PwID.
Recognising PCS needs intellectual disability clinicians and carers to look for subtle changes in behaviour and functional need. We need to develop PCS-specific screening tools for this cohort of people. Adapting the C19-YRS scale and validation in this cohort might help recognise the problem and get a real estimate of the burden. To do this caregivers need to acquire the skills to detect subtle changes in behaviour and function and identify changes in need for psychotropic medication where PCS could be suspected.
An earlier stage in building the evidence base may be through case reports and case series. Although a lower standard of evidence, these descriptions can give early clues on the consequences of COVID for PwID. Ealy case series of COVID deaths in PwID gave clues to the consequences of COVID-19 infection before larger studies were described.Reference Perera, Laugharne, Henley, Zabel, Lamb and Branford2,3
Change in carer burden can also indicate PCS. Without such recognition of the condition the residual manifestations would remain undetected and can lead to potential worsening of the presentation itself. Awareness of PCS could be achieved by bespoke training of front-line carers and family carers. Such training needs to be extended to front-line health professionals who are likely to meet PwID such as primary care staff, acute hospitals and specialist intellectual disability staff.
Research should focus on understanding whether the presentation of PCS in PwID is different from that being reported in the general population and whether the impact from symptom type, severity and functional is different. Research should also focus on long-term functional and neurobehavioural changes in prospective longitudinal studies. PwID are often excluded from research even for conditions to which they are especially prone, for example epilepsy. This exclusion from research should not prevail in PCS given the scale of the problem and potential implications for the patient, carer and the wider healthcare and social system.
Changes in prescribing before and after COVID infection may suggest the consequences of PCS. Case–control studies comparing patients who have had COVID and those who have not may help detect changes in prescribing that may be a proxy for PCS. Cohort studies examining changes prospectively would be ideal, but the changing nature of the virus makes this methodology very challenging. Ideally clinical rating scales such as the validated Royal College of Psychiatrists Health of the Nations quality of life outcomes tool for PwID (HONOS-LD) or similar, before and after infection could be used for assessing the effects of COVID-19 infection and repeated at 3–6 months intervals after infection.
Awareness communication, training and rehabilitation provided in a timely manner may reduce the long-term functional deterioration. This has been well proven for other aspects of chronic condition management and should be extended to PCS management in general, particularly for those PwID. An integrated rehabilitation approach that has input from a range of specialists with expertise in managing the complications seen in this multisystem condition will prove useful in reducing long-term functional decline and helping family and carers manage the condition better. We need to ensure these vulnerable individuals are not left behind in our response to this devastating pandemic.
Declaration of interest
B.P. has received speaker fees from Flynn pharma outside the submitted work. R.S. has received institutional and research support from LivaNova, GW pharma, UCB, Eisai, Veriton pharma, Averelle and Destin outside the submitted work. M.S. is a significant contributor to the development of the C19-YRS Scale discussed in the paper. No other author has declared any conflict of interest.
Appendix
Potential plan to enable research about post-COVID-19 syndrome (PCS) in people with intellectual disabilities (PwID) and challenges to realise them
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