Changes to personal and social circumstances during the COVID-19 pandemic

All patients were reported to have shielded during the first lockdown period (figure 3); over half started shielding before being formally advised to do so.

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Figure 3

Reported patterns of shielding.

Changes to personal care assistance arrangements were reported by 67.5% (n=27) to minimise interaction with non-household contacts: of these 59.2% (n=16) stopped all visits from external carers for between 2 weeks and 6 months, when all care was provided solely by family members and 25.9% (n=7) reduced the number of individual carers or the frequency of carers changing over. Changes were typically made from March 2020 and usual care assistance arrangements typically resumed between June and August 2020. Changes to patients’ social and professional/academic activities were reported in 95.0% (n=38) and 77.5% (n=31), respectively. Financial difficulties were infrequently reported.

Risk of infection and disease severity

Concern about the risk of COVID-19 to patients’ health was associated with the perceived likelihood of severe or fatal disease. Perceptions were based on (a) previous experience of severe or prolonged illness due to respiratory infections and/or (b) vulnerability due to respiratory muscle weakness or failure:

…if a child cannot cough and clear their airways, any respiratory infection is going to be hard work. If your child has to have a PICU [Paediatric Intensive Care unit] or an HDU [High Dependency Unit] stay due to things like rhinovirus and been intubated because of a common cold, then COVID-19 obviously to me would pose the same risk. (Parent, male, spinal muscular atrophy (SMA) I, 0–5 years)

Because of my muscular dystrophy I’m classified as clinically extremely vulnerable. And I also sleep on a night ventilator because I have a compromised respiratory muscle system, so that concerns me quite a lot because COVID-19 is a disease that attacks your respiratory function quite quickly. (Patient, female, congenital muscular dystrophy (CMD), 32–38 years)

Uncertainty about the potential severity of COVID-19 was frequently reported, exacerbated by a lack of evidence specifically in NMDs:

…it seems a little bit like a lottery as to how [COVID-19] may affect individuals. Obviously I’m classed as clinically extremely vulnerable. But even in some of those cases, it seems a little bit hit and miss as to whether you would kind of see the full effect… (Patient, male, CMD, 32–38 years)

However, participants did not generally consider people with NMDs to be more likely to contract COVID-19 than others. Some considered COVID-19 to be of little risk to them, feeling they could entirely avoid infection, that is, through strict shielding.

Perceived accessibility and availability of acute care for suspected COVID-19

Reluctance to contact medical professionals in the event of suspected COVID-19 was not reported but unwillingness to attend hospital in the event of acute respiratory illness was common. This was because participants felt equipment and expertise was accessible at home:

…in some ways I’m quite lucky cos I’ve got all the equipment at home, so I’ve got the ventilator [and] cough machine, I’ve got all the things that they would do in the hospital anyway.” (Patient, male, CMD, 32–38 years)

In addition, many thought their physical care needs would be less adequately addressed in hospital compared with at home, particularly if carers or parents were unable to stay due to restrictions on hospital visitors:

…I know that during the pandemic, they’re not letting people stay with you. So that would put me in a very, very risky situation. (Patient, female, CMD, 39–50 years)

Several respondents reported being anxious about the risk of COVID-19 transmission in hospital, particularly if they were in close proximity to other patients. Others reported concerns based on pre-pandemic experiences about the ability of hospitals, particularly those without specialist expertise, to manage the complex needs of patients with NMD; some considered that a personalised ‘crib sheet’ they or treating clinicians could follow would be useful:

I think it would be useful… to have a guide as to if something did happen, in terms of like my lungs… what the best tips are… (Patient, female, CMD, 32–38 years)

Perceptions of prioritisation for ICU level care

Concerns about ICU care not being considered or available were based on (a) the perceived lack of ICU capacity particularly during peaks of COVID-19 infections, and (b) perceptions that people with disabilities and underlying health conditions were considered lower priority:

…there was quite an outcry from the disabled community that hospitals were deciding not to put certain people, certain disabilities on a ventilator… that was quite a worry. (Patient, female, SMA II/III, 39–50 years)

…it’s almost like they talk about like the death of a vulnerable person is just a bit inevitable… like they’re the people that do die. (Patient, female, SMA II/III, 26–31)

Some adult participants had been advised by their specialist teams during the first wave of the pandemic that they would not be considered for ICU level care: some considered this an understandable stance aiming to prioritise those most likely to survive, but for most it increased anxiety, stress and anger:

…even in the best of times I would not go to hospital, but seeing this written, saying to you “if you get it, don’t go to hospital”, and it’s the hospital saying that to you, that really brings your level of trust to zero. (Patient, female, CMD, 39–50)

There was a perceived lack of clarity in the criteria or process of prioritising patients for ICU level care. Some reported an awareness of the Clinical Frailty Scale (CFS) and were concerned that they would automatically be excluded or considered lower priority by virtue of their physical limitations. There was however doubt whether exclusions based on underlying conditions would be upheld in reality:

I would have hoped that if in the most severe cases, anybody that is suffering from COVID-19, regardless if you’re classified as highly vulnerable… if you were suffering severe symptoms, I would have hoped they would have had space in ICU… (Patient, male, congenital myopathy,19–25 years)

Shielding and minimising social contact

Initially patients and families aimed to avoid infection at all costs; patients’ homes were considered places of safety where social contact could be controlled. In addition, virtually all patients reported having received adequate support to shield, for example, from friends and family, employers, schools, healthcare services, and/or government support schemes.

…I haven’t really left home since it all started. I get my shopping done online, the other half still works from home, so we’re keeping away from people. (Patient, female, congenital myopathy, 32–38 years)

However a lack of clarity in confirming that people with NMDs were considered to be clinically extremely vulnerable to COVID-19 meant some only received formal advice to shield several weeks into the first lockdown, and several felt frustrated or let down by the lack of guidance provided by both the government and their clinical care teams:

No one really knows if it’s the GP that’s supposed to… or whether it’s the clinical teams that are meant to decide. No one actually really knows who is meant to be telling them if their child is extremely vulnerable. (Parent, male, SMA I, 0–5 years)

Patterns of behaviour encompassed in ‘shielding’ varied greatly between respondents; deciding on when and how strictly to restrict activities and social contact were based on (1) national and local advice, (2) availability of activities, (3) perception of infection risk and/or (4) perceived impact on physical or mental well-being.

I was watching how the cases were starting going up again, and I thought that’s it, I’m going back in. (Patient, female, CMD, 19–25 years)

Fundamentally shielding could be described as a state of mind informing acceptability of activities and social contact:

…I’ve just carried on doing what I have been doing since March [2020] going out when I really need to or when I want to, but avoiding contact with people… I’m still shielding, in my head, but I’m doing it according to what I feel intuitively is the right thing. (Patient, male, SMA II/III, 39–50)

Most patients continued shielding in some form outside periods where it was officially advised; household members generally returned to school or work, supported by strategies to minimise contact with others. However, some felt unsupported where they felt the household continuing or returning to shielding was in the patient’s best interests:

…it’s only guidance… it’s not law… all I’m asking you to say, is that [I am] in a specific situation that requires a different sort of approach…. (Patient, male, SMA II/III, 26–31 years)

Many planned to continue shielding to some degree until vaccines were available, and often beyond to evaluate effectiveness:

…hopefully the vaccine is kind of successful and that’s the end of it, but the reality of it probably isn’t… we can’t be shielded forever… (Patient, female, CMD, 32–38 years)

Generally, participants were reassured by emerging evidence that young people and children were at less risk of severe COVID-19, but others remained uncertain:

…so obviously now a lot of children have been taken off the shielding list [and] he’s been going to school. And every day I question myself, am I doing the right thing? (Parent, male, SMA II/III, 6–11)

Adaptations to home care arrangements

For patients requiring physical care assistance and support, complete isolation from others was not possible during the pandemic. Temporary cessation or reduction in external care provision was often undertaken to reduce the risk of COVID-19 transmission, and involved consideration of the risk posed by individual carers, for example, where they also provided care to other clients or in care homes:

I’ve been isolating, shielding, all this time but actually I can’t truly isolate or shield, because I have full time care, so I’m very aware that every time any one of my six or seven people come to the door, they could be bringing it in. (Patient, male, SMA II/III, 39–50 years)

Such measures increased anxiety about the fragility of care arrangements:

If we were to get ill how do we look after him regardless of whether he gets ill or not? (Parent, male, SMA II/III, 12–18)

Pre-pandemic care arrangements resumed either when provision of all care by family became physically or emotionally unsustainable, rates of COVID-19 had reduced or protective measures, for example, PPE were available:

I think [the carers returned] when we realised there’s PPE to help reduce the transmission, and it then got too much for my parents as well. ‘Cos it was round the clock care so, it was too much to keep up with really. (Patient, female, CMD, 19–25 years)

It was also important for patients and families to trust carers to minimise their own infection risk and report potential exposure to COVID-19. However, this heightened concerns about establishing trust in new carers which exacerbated long-standing difficulties in recruitment:

…you know if somebody new was coming in I obviously couldn’t be totally sure that they had done all the right things. (Patient, male, Duchenne muscular dystrophy (DMD), 26–31)

Practical approaches to minimising COVID-19 risk

Practical approaches to reduce the risk of transmission predominantly related to visitors, for example, non-household family members, external carers. Strategies included increased hand hygiene and PPE. The type and form of PPE varied based on (1) availability of equipment, (2) available guidance, (3) perceived infection risk and (4) the presence of potentially aerosol generating procedures.

I mean social services have given us PPE that we’re using… we’re just mainly using gloves… My carers are basically taking precautions. (Patient, male, SMA II/III, 39–50 years)

Participants considered the risks of potential transmission of COVID-19 between carers and themselves or their child, particularly where carers worked with other clients. In addition, there was particular concern where the patient or family directly employed carers and were therefore responsible for their safety at work. Hesitancy of carers to use PPE, for example, due to discomfort was infrequently reported. When well, some adult patients did not feel PPE was required in their homes, believing it would be of limited benefit in preventing transmission or would impact on the quality of support or interaction:

I said that I’d prefer that [carers] didn’t wear masks and aprons unless they absolutely felt they had to… Just purely because it felt very uncomfortable… very clinical. (Patient, male, DMD, 26–31)

…once they’re indoors and once they’re actually with me, the care is so intimate like, they’re taking me to the toilet, taking me to shower, putting my mask on, actually PPE isn’t really going to do anything. We’re in the same space, breathing the same air for the whole week. (Patient, female, SMA II/III, 26–31 years)

Many participants reported trying to maintain social distancing with others, including carers, whenever possible. A minority reported ongoing concern about the potential for fomite spread of SARS-CoV-2 and had established extensive cleaning routines, for example, of groceries, the home, which increased physical and psychological burden:

…whenever we get something, I always ask my mum to wipe it and she gets annoyed at times, obviously, because I’m constantly saying it again. (Patient, male, congenital myopathy, 32–38 years)

Remote access to community and hospital care

Generally remote (virtual) access to healthcare services was viewed positively by participants as an interim measure to maintain contact with services, particularly for discussion-based consultations, and for patients who had maintained clinical stability over preceding months or years:

The thing is my condition is fairly stable, so my annual check-up is very quick usually. It’s like me going in and saying “yep everything’s ok” and then leaving again. So, it was better to do it online. (Patient, male, SMA II/III, 39–50 years)

There was significantly less support for remote consultations where physical examination, procedures or diagnostic tests were deemed essential:

Basically he has been growing so quickly and his eyes, ears and teeth are constantly changing. And obviously those are the things we would like the medical person to have a look at, rather than just discussing over the phone. (Parent, male, congenital myopathy, 0–5)

Some participants preferred remote consultations reporting that they increased convenience and comfort, and mitigated logistical problems, for example, parking difficulties, limited wheelchair accessibility.:

…I found [remote clinic] a lot better, ‘cos obviously, when you’re talking from your own house, you’re a lot more relaxed than when you are in hospital surrounded by everybody. So I mean you have to step into the room, and then you wonder if the room’s a bit too crowded, or if you can fit through the door with the wheelchair. (Patient, male, SMA II/III, 32–38 years)

However, it was the preference of most participants that some, if not most, appointments return to being in person to facilitate communication and/or the performance of tests and procedures:

I think with the sleep clinic it’s important to hopefully get back to coming down and having an appointment face-to-face because it means we can do the blood gas test and it gives you a view of whether the NIV’s working properly. That’s obviously something that we can't do via teleconference. (Patient, male, CMD, 32–38 years)

Psychological impact on patients and families

Anxiety, fear and worry were the most frequently reported emotions during the COVID-19 pandemic (figure 5) although the level and pervasiveness of these fluctuated:

Honestly, it has varied across the past 8 months… I think in the beginning I was very worried, I was very anxious… (Patient, female, CMD, 39–50 years)

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Figure 5

Responses to the question ‘How have you felt during the COVID-19 pandemic?’ by (A) patient respondents and (B) parent respondents.

Primarily, negative emotions were related to (1) uncertainty, including about the potential impact of COVID-19 on the patient’s health, (2) conflicting or changing guidance or advice and (3) the potential duration for which restrictions would be required. Depression and low mood were less frequently reported but were more common in those with previous mental health problems:

I’d say I’ve become a lot more depressed and I suffer from depression, so that adds on top of it. (Patient, male, SMA II/III, 32–38 years)

Some reported negative psychological effects on the family when they were shielding or providing all patient care:

Some days I’m okay, some days I find it hard… when it was just me and the kids for 4, 5 months… all day and no adult conversation at all, that was quite difficult. (Parent, male, SMA I, 0–5 years)

For some, these feelings were exacerbated by seeing the general population being able to resume more activities as restrictions were lifted:

I think when the restrictions eased, and you keep seeing people on social media, boasting "oh, we’re going there", it does kind of make you feel left out. Like the world is moving and you’re just in the same place. (Patient, female, SMA II/III 39–50 years)

Others were more comfortable being at home as they felt protected or could continue activities they enjoyed:

…I feel safe in my house, where if I went outside I don’t feel safe at all. (Patient, female, early-onset scoliosis, 39–50 years)

Most patients missed social interaction with non-household contacts. However feelings of loneliness and isolation were uncommon due to mitigating factors, for example, keeping busy with work or schooling, maintaining contact remotely and interacting with carers and household members:

I spent the lockdown with my parents who I have a pretty good relationship with. So I wasn’t on my own. I really feel sorry for people who or were living alone during the lockdown because how they managed to continue and keep their heads above water, and being completely alone, I have no idea. (Patient, male, CMD, 32–38 years)

Negative emotions generally improved during the pandemic, either due to (1) acceptance or adaptation to the situation, (2) better understanding of COVID-19 or (3) the resumption of some social contact or activities:

I have periods of stability where I feel quite safe. I think the uncertainty at the beginning did make me very anxious… I feel like I’ve got good coping skills on the whole so if I was anxious it wouldn’t last for days. I have gone up and down a lot, but I think that goes for everybody… (Patient, female, CMD, 39–50 years)

However, for some, negative emotions had been superseded by boredom, frustration and apathy. Particularly for those who shielded more strictly, there was anxiety about returning to normal activities and interactions;

…being at home, the [outside] world becomes a bit more of a scary place. And so I don’t know how I’m going to fit back in to doing things that I used to do. (Patient, female, SMA II/III, 26–31 years)

Several participants felt hopeful for the future or psychologically resilient and able to use coping strategies effectively. Some had taken proactive steps to improve their mental well-being, including seeking support from psychological medicine professionals:

I found that due to the sense of loneliness I was a little bit more sad, and maybe thinking in a negative way. And that’s when I asked for psychological support, and I’m now having like some sessions on a weekly basis with a counsellor. (Patient, female, CMD, 26–31 years)

Perceived positive outcomes from COVID-19 pandemic

Although predominantly a negative experience for most, almost all participants identified at least one positive aspect to come out of the COVID-19 pandemic. These were mostly related to (1) changes in personal circumstances, for example, spending quality time with family, or (2) impacts considered positive for wider society:

I think my relationships with family have become stronger… because we’ve been put into isolation, lockdown with our families for a really long time… (Patient, female, congenital myasthenic syndrome, 32–38 years)

…I think it’s brought to the surface a lot of inequality, which lots of people knew was already there. But now it’s a bit unavoidable. I think it’s come in to more of a public conversation and I hope that in terms of disability that continues… (Patient, female, SMA II/III, 26–31 years)

Of particular benefit was improved accessibility, as more activities and events were available online. Several participants reported that the pandemic had led them to re-evaluate their lifestyle and priorities, prompting the uptake of new skills, ways of working and increased focus on health and well-being:

…there were so many events that I used to go to that weren’t accessible, or, [hadn’t] got wheelchair space, and I had to miss out. Now they are online… equality was created, but at a price. (Patient, female, CMD, 39–50 years)