To shield or not to shield: the challenges of covid-19 for patients with rare disease

The covid-19 pandemic has significantly increased health and social divides within the NHS. Those with complex health needs and/or chronic health conditions who have been shielding have been hugely affected by this challenging undertaking on social, emotional, and economic levels. However, the process of ascertaining individuals’ risk from covid-19 and the precautions they should undertake is complex and has been fraught with inconsistencies, errors, and controversy. [1,2] Many individuals suspected of being at high risk who have been advised to shield have what is termed a rare disease often with associated co-morbidities. Individually, these diverse conditions affect less than 1 in 2000, but collectively they affect 1 in 17 of people. We need to ensure that people who are shielding such as those with rare diseases are not forgotten or left behind as society re-opens. [3]

The announcement that shielding is set to pause on the 1st August has received a mixed response. The reality is that the risk of severe covid-19 disease varies significantly between people who are shielding. We must accept that our scientific understanding of what does and doesn’t make a person susceptible to severe disease response is still in its infancy. Data are beginning to emerge confirming that those with respiratory, renal, and autoimmune diseases such as diabetes are particularly affected by covid-19. However, somewhat unexpectedly, obesity and ethnicity also appear to be significant risk factors. More data is urgently needed to definitively categorise individuals’ risk.

The uncertainty of risk assessment is especially pertinent in the rare disease community where prognoses and disease pathologies remain largely unelucidated due to the small number of people affected with individual and usually very unique conditions often with associated co-morbidities. We have attempted to collate and summarise predicted risk for people with rare disease through production of a covid-19 Rare Disease Risk Assessment Grid and patient-friendly covid-19 Rare Disease Risk Interactive Tool. [4,5] We are fully aware that more evidence is needed to assess risk level more accurately so as to better advise individuals of the precautions they should be taking in order to mitigate or minimise their risk.

Many people with rare diseases are understandably anxious about what re-opening society and withdrawal of the framework of support on the 1st August means for them. None of us can reasonably allay their fears because we don’t have the answers. Many people with rare diseases have a learning disability and struggle to learn complex new concepts around enhanced hygiene and social distancing. Others, who are sight impaired, cannot practice social distancing because they are unable to see people around them or read social distancing guidance. Many parents of shielded children worry about the cessation of food and medicine deliveries and being forced to go back to work where they may inevitably be exposed to covid-19 and inadvertently infect their extremely vulnerable child.

The only justifiable course of action is to ensure that shielding benefits can continue for those people who, in partnership with their healthcare provider, feel that the risk to them remains high. We cannot solely rely on volunteers and finance-depleted charities to shoulder this important responsibility; patient support organisations for rare diseases are already being overwhelmed by patients and families understandably expressing significant concerns.

Let us commit to learning from the ongoing first wave of covid-19 and create a plan for future waves and pandemics. In this pandemic, many extremely vulnerable people did not receive a shielding letter, and some less vulnerable individuals unnecessarily received a shielding letter. Testing and personal protective equipment was rarely available for people with rare diseases and carers who needed it at the height of the pandemic. Many creative solutions have emerged from the NHS despite the challenges faced by the fast changing covid-19 landscape to help and support those who we look after. For the predicted and likely inevitable second covid19 wave, people who are shielding must be prioritised to have a robust framework of continued support and have access to a vaccine when this becomes available. We are all longing to return to some level of normality, including the most vulnerable in our society.

Above all, clear and consistent guidance must be made available for families living with rare diseases. Healthcare providers must be consulted and the evidence base for advice made available so that we can work in partnership with the government to provide the best care for those who need it most.

The health gap will continue to widen so long as the pandemic exists. It may be months, it may be years. There may potentially be a second, third, and fourth wave. We all know at least one person with a rare disease. We have a responsibility to our friends, colleagues and loved ones living with a rare disease to help bridge this gap and ensure that the most vulnerable in society are protected.

Lara Menzies, ST5 clinical genetics SpRs at Great Ormond Street Hospital, London. Twitter: @lara_menzies

Elizabeth Forsythe, ST5 clinical genetics SpRs at Great Ormond Street Hospital, London. Twitter: @GeneDrElizabeth 

Shehla Mohammed is a consultant clinical geneticist at Guy’s and St Thomas’ Hospitals, London. Twitter: @snmcb

Frances Elmslie is a consultant clinical geneticist at St George’s Hospital, London and President of the UK Clinical Genetics Society. Twitter: @clingensoc

Eamonn Sheridan is a professor of clinical genetics at The Leeds Institute for Molecular Medicine and is Chair of the British Society for Genetic Medicine. Twitter: @MedeshEamonn, @BritSocGenMed

All authors declare they have no conflicts of interest.


  1. Davey Smith, G and Spiegelhalter, D. BMJ 2020;369:m2063
  2. Torjesen, I. BMJ 2020;369:m2173
  3. Genetic Alliance. The Rare Reality of Covid19. July 2020
  4. Covid19 Rare Disease Risk grid:
  5. Covid19 Rare Disease Risk Interactive patient tool: