Duchenne UK responds to UK Parliament consultation on Coronavirus (Covid-19) and the impact on people with protected characteristics
12th May 2020
Last week Duchenne UK submitted a response to the UK Parliament consultation on Coronavirus (Covid-19) and the impact on people with protected characteristics.
The document brings together all the problems patients and families living with Duchenne have been telling us about. Click here to read the full document as a pdf, or scroll down the page.
These were our key concerns:
- Duchenne UK is concerned that COVID-19 could lead to a reversal in progress with a negative impact on morbidity and mortality as well as a delay to research, clinical trials and development of new treatments for DMD
- Shielding may result in a bigger burden for people with DMD in terms of their education, employment, finances and mental health
- Not all patients have received notification of their vulnerable status which has led to difficulties accessing much needed support
- In some cases, patients and parents have struggled to get support from their employers to be able to shield
- Patients with DMD require a team of multi-disciplinary experts to monitor and manage their condition, they are struggling to access specialists e.g. physiotherapists, endocrinologists, cardiologists and respiratory consultants
- Access to treatments such as testosterone therapy, bisphosphonate treatment and physiotherapy interventions such as splinting will be impacted, having an impact on the health and quality of life of patients
- NICE COVID-19 guideline for critical care: we remain concerned that clinicians and critical care units may be unaware of the revisions to this which do not appear to have been publicised
- Some parents are now providing the personal care of their adolescent and/or adult children because the risk of allowing carers in the home without testing and PPE is too high
- The Coronavirus Act 2020 allows for fundamental changes to the obligations on Local Authorities to provide social care during the COVID-19 emergency, which could last for two years
We would like to thank the patients and parents who contributed to this consultation. We will update you once the government provide any updates.
Duchenne UK response to UK Parliament Inquiry
"Unequal Impact Coronavirus (Covid-19) and the impact on people with protected characteristics: Duchenne UK"
Duchenne UK is a leading charity dedicated to funding research and supporting patients and families affected by Duchenne Muscular Dystrophy (DMD), a rare life-limiting progressive condition.
DMD is a genetic disease that causes muscle weakness and wasting. Children born with DMD have a fault, known as a mutation, in their dystrophin gene which means that they cannot produce dystrophin, which is a protein that protects muscles. Without dystrophin, muscles get damaged more easily and so muscle strength and function are weakened. DMD almost always affects boys, with only about 1 in 50 million girls affected. Boys tend to be diagnosed before the age of 5. There are an estimated 2,500 patients in the UK. For children with DMD, muscle weakness starts in early childhood. It is usually first noticed between 2 and 5 years old.
Often the earliest indication that a child may have DMD is their difficulty in getting up from the floor, walking or running. Gradually, the child’s other muscles will begin to weaken as well. Without dystrophin, muscles become inflamed over time. As the muscles try to repair themselves, fatty or fibrous material builds up in the muscle and causes them to stop working altogether. This is called fibrosis and causes the muscles to become rigid and hard.
Some children with DMD also have learning and behavioural difficulties. This is because dystrophin is also found in the brain, although we are not yet sure of its purpose there.
Advances in research and knowledge of the disease are increasing life expectancy. Now, more and more young adults with DMD are leading full lives – going to university, having relationships and living independently. However, men and women with DMD do have a reduced life expectancy. Most adults with DMD will not live beyond their 40s.
At a time of continued advances in medical research and the promise of disease modifying treatments on the horizon, Duchenne UK is concerned that COVID-19 could lead to a reversal in progress with a negative impact on morbidity and mortality as well as a delay to the research, development and availability of new treatments for DMD.
How people have been affected by COVID-19 or the response to it
Boys and men with DMD may be particularly vulnerable to the effects of COVID-19:
- Reliance on family and carers to help with personal care can make social distancing from caregivers impossible
- Learning and behavioural difficulties may also make social distancing and self- isolation more challenging especially for children who may respond poorly to changes in their routine.
- Compromised immune systems due to long term corticosteroid treatment can make those with DMD more susceptible to infection
- Compromised lung function could result in more severe COVID-19
- Cardiac symptoms could result in more severe COVID-19
- We know that males may be more severely affected by COVID-19 and the overwhelming majority of those with DMD are male
Most DMD patients have been advised by their clinicians that they are vulnerable and should self-isolate for a minimum of 12 weeks, and most likely far longer. DUK recognise that this degree of isolation may be necessary to shield those with DMD who are some of the most vulnerable people in society. However, not all patients have received notification of their vulnerable status which has led to difficulties accessing much needed support. Other patients and caregivers have expressed concerns that they will not be able to access food and medicines as they have not been able to register as clinically vulnerable - they have been unable to access priority food deliveries. There has also been a financial impact. As Vici Richardson, mother to a son with DMD says “disabled people and their families have been literally forgotten about .. in work benefits are having a rise of £20 … [but there is] nothing for the Carer's Allowance or PIP etc.
This inconsistency is likely to have a more significant impact on those who are in their teens or older and therefore whose disabilities are more severe and who have been wholly reliant on carers. We have heard from patients and parents who work that in some cases they have struggled to get support from their employers to be able to shield. They have not been given the option to work from home or their employer won’t furlough them. They have had to take unpaid leave and move onto universal credit.
Clinicians at specialist centres have worked hard to minimise the impact on routine care for people with DMD. Outpatient clinics for DMD have been cancelled with telephone appointments offered instead although these cannot fully replace the physical assessment by physiotherapists and routine tests used to monitor disease progress and inform the day to day care of patients. These include blood tests, echocardiograms, electrocardiograms, bone densitometry (DEXA) scans, lateral spine x-rays, checks for scoliosis, respiratory function forced vital capacity (FVC) measurements and mental health screening.
Patients with DMD require a team of multi-disciplinary experts to monitor and manage their condition. Patients are managing to have access to their neuromuscular consultant but are struggling to access sub-specialists e.g. physiotherapists, endocrinologists, cardiologists and respiratory consultants.
Access to treatments such as testosterone therapy, bisphosphonate treatment and physiotherapy interventions such as splinting will be impacted, having an impact on the health and quality of life of patients. Most families have not had emergency care plans put in place, something that is particularly important for the management of corticosteroid treatment, an important part of the standard of care for those with DMD. New diagnoses may be delayed, with boys and their families waiting longer for much needed support and clinical care. There may also be delays in access to psychological support compounding the effects of DMD and social isolation.
As well as missing out on important appointments while we wait for the system to move to online, our son was meant to have infusions of zoledronate to help improve his bone density. Without this, he is at far greater risk of fracture. He broke his leg last year and has broken his arm twice. We are very worried about the risk to his bone health as a result of delays caused by Covid-19.
Boys and adults with DMD have entered an extended period of isolation and are unlikely to be able to return to school, work and everyday life at the same time as the general population, indeed many expect to have to wait for a vaccine to be available before they are able to return to school or work. This additional shielding will result in a bigger burden for people with DMD in terms of their education, employment, finances and mental health – impacts felt by individuals with DMD and their families.
There is no real thought on how this is going to pan out long term … I’ve already started preparing [him] to not be back at college until there is a vaccine – I can’t do that with [my unaffected children] though. (DMD mother)
Prioritising vulnerable patients and caregivers once antibody tests are available could help if it allows for some relaxing of strict isolation.
Specific impacts on people due to their having protected characteristics
Disability, age and sex are protected characteristics and some of the impacts on people with DMD are connected to these characteristics, primarily disability. Duchenne UK is concerned about the potential for adverse impacts of the government and NHS response to COVID-19 on disabled boys and adults with DMD.
COVID-19 has already resulted in delays to clinical trials for promising new treatments for DMD. Recruitment to trials has stopped and in some trials patients have been temporarily suspended from the trial because they cannot be monitored due to staff being redeployed to care for COVID-19 patients. We know of patients who have had to stop taking a drug that has shown positive results in trials, which is causing significant distress to families and, given the progressive nature of DMD, could result in an avoidable worsening of their condition.
My son Alex is 14 years old and suffers with Duchenne Muscular Dystrophy. He is on the cusp of losing the ability to walk. He has been on a clinical trial for a new potential treatment for Duchenne for over a year. We were devastated when his trial was stopped due to COVID-19. Since stopping potential treatment the company has released interim positive data adding to our anguish he can’t access the treatment. Alex’s trial has initially been stopped for 12 weeks because of COVID-19. This is time we don’t have to sit and wait whilst we watch our son decline. (DMD mother)
The majority of trials are designed for ambulant boys only. Given the progressive nature of DMD boys who were expecting to take part in a clinical trial will no longer be eligible to participate if they lose ambulation before trials begin. DUK are also concerned that the understandable focus on COVID-19 will result in a delay to the licensing of new treatments for DMD and could ultimately delay access to new treatments, with NICE already delaying the assessments of many new treatments.
People with DMD require specialist equipment and adaptive technologies to manage their daily lives. These requirements will change as the disease progresses and the needs of patients in March may not be the same as their needs even 12 weeks later. For example, patients losing the ability to walk move quickly from being able to walk a few steps and support their weight to being unable to stand even with support. This period is associated with an increase in the need for home adaptions and supportive equipment such as hoists.
Home adaptations may become urgent and delays in approving these adaptations, reduced household income and limited builders able to undertake the work could have a negative impact on the independence, health and quality of life of those affected. Changes to requirements for care assessments and reduced requirements on the level of care provided (see below) could have a significant impact on disabled children and adults with DMD either delaying meeting increased needs as their condition worsens or removing some of their current care package. This could have a significant impact on their physical and mental health. As one mother told us:
[My son] has gone from being included and supported in his community to isolated and told to stay in his house and that only essential care can be given from the outside. (Vicki Richardson)
We have been contacted by people living with DMD, or by their family members, who are very concerned that the assessment of frailty will disadvantage those living with DMD, and other disabling conditions who may score poorly on standard measures of frailty.
The NICE COVID-19 guideline for critical care was published in March 2020 to help the NHS manage care during the pandemic. The original guideline did not clearly convey that critical care could be appropriate for those scoring poorly on the frailty measure. It also failed to highlight that this measure is only suitable for older adults and not for those living with long term disabilities. Whilst this guidance has been revised to address our concerns, we remain concerned that clinicians and critical care units may be unaware of these revisions which do not appear to have been publicised. Indeed, we are aware of clinicians giving advice to patients based on the original guidelines that have led to concerns that patients will not be treated for COVID-19 or any respiratory condition at this time. We are concerned that this could result in poor implementation of the guideline, particularly as patient numbers rise and capacity in critical care is stretched. We understand that difficult decisions will need to be taken by clinical teams during the pandemic but pre-existing disabilities should not be a determining factor in access to effective care for patients who could reasonably recover from COVID-19 and return to the same, or a similar quality of life as they experienced prior to contracting COIVD-19. Additionally John Chisolm the chair of the British Medical Association’s Ethics Committee has written that the question of treatment will “no longer be how best to meet individual need, but how to maximise benefits from severely stretched resources” indicating decisions may be discriminatory against those with underlying health conditions who may need extra support. (The Guardian 1 April 2020)
Jon Hastie, the CEO of DMD Pathfinders and an adult with DMD, has written:
It’s terrifying to think people with health conditions could be denied treatment based on the idea that we might take longer to recover than other people. That we could die even though our condition could be treatable. (Jon Hastie, DMD Pathfinders)
Additional isolation requirements to ensure clinically vulnerable people with DMD are shielded is increasing the isolation faced by boys and men with DMD and will impact on their mental health. Poor mental health, anxiety, ADHD and autism spectrum disorder are established risks amongst young people with DMD, which can sometimes lead to challenging behaviour. Increased anxiety of parents around behaviour issues has been established. Some parents are now providing the personal care of their adolescent and/or adult children because the risk of allowing carers in the home without testing and PPE is too high. Many parents are also doing other jobs so there is additional pressure on families that is unsustainable. Parents are struggling physically and emotionally to meet all the care needs of their children without their usual network of carers and other support. Patients and families are worried about the lack of support and policy regarding PPE and testing and feel abandoned by the Government. Improved access to PPE and testing may allow for external carers to resume supporting patients which would ease some of this pressure.
Most families have other children who do not have DMD. Government advice around disabled children and young people/adults appears to assume that those with underlying health conditions are older or live alone and therefore can self-isolate more easily. It seems little consideration has been given as to the impact on healthy children in families of disabled children who may be unwilling or unable self-isolate, for example needing to attend their school as soon as it opens because they have upcoming exams. Greater consideration of the impact on wider families and guidance to address the challenges these families face could help.
Whether there may be unforeseen consequences to measures brought in to ease the burden on frontline staff, for example relaxing the measures under the Mental Health Act and Care Act)
DUK recognises the need for emergency legislation to relieve some of the burden on frontline health and social care staff to help ensure that there is the capacity to respond to the COVID-19 pandemic. However, concerns have been raised within the DMD community that have highlighted areas where unforeseen consequences may fall disproportionately on those with protected characteristics.
Notably there is concern that hospitals will be unwilling to treat people with DMD, who may have an exacerbation of their condition unrelated to COVID-19, particularly those already requiring full-time ventilation or who may need increased respiratory support. We are concerned that as the pandemic continues, and staff are potentially redeployed to respond to urgent care and COVID-19 patients, DMD patients may find their care compromised with essential care delayed, for example boys requiring cough assist devices may have to wait to be assessed, requests for specialist wheelchairs may take longer to be approved, surgeries may be delayed and cardiac symptoms may develop unnoticed and unmanaged. Improvements in clinical care have led to marked improvements in life expectancy for people with DMD and we want to make sure these advances are not lost as the NHS inevitably focuses on COVID-19.
On occasion, those with DMD require urgent care as a result of their condition or treatment. They may be more prone to respiratory infections due to a compromised immune system or reduced lung capacity and may require additional respiratory support to recover from respiratory infections. DUK are concerned that this care may not be available to boys and men during the COVID-19 crisis or that patients and their families may avoid seeking treatment due to a fear of accessing hospital care at this time. There is a risk that respiratory patients may be put on wards with patients with COVID-19, increasing their exposure to the virus.
The Coronavirus Act 2020 allows for fundamental changes to the obligations on Local Authorities to provide social care during the COVID-19 emergency, which could last for two years. It reduces the required level of support for disabled adults and will likely delay access to assessment and resources, particularly for those undergoing transition from children’s to adult services. Similarly, this emergency legislation allows for periods when Local Authorities will not have an ‘absolute duty’ to provide Special Educational provision but will instead need to make ‘reasonable endeavours’ to do so. There may also be a suspension of annual reviews of need. This will impact on the educational opportunities offered to many children with DMD.
We understand 7 local authorities have already implemented the care act easements provided for in the legislation, while others have not. This highlights the absence of clear guidance or controls on when local authorities should use these new powers raising a concern that the powers could be used inappropriately and disproportionately around the country.
Emily Crossley & Alex Johnson