Questions from other groups which are relevant to the Myotonic Dystrophy community.

Does the CV actually kill the lungs? Are the lungs able to recover? (I appreciate these are almost impossible to answer!

Covid-19 causes an atypical pneumonia and interstitial lung damage in some people. There is recovery but I understand there may also be continuing lung damage if it is severe, although It is too early to know if this is permanent

I’m worried that my son will lose ambulation during the isolation period. What can I do to stop this?

Try and keep active indoors

COVID-19 is in the same family as SARS and MERS
-        Do we have any data relating to DMD patients (and NMD patients) and the MERS/SARS from the last epidemics, and what treatments were used?
-        What if any effects did we see?
a. There are no data for these disorders and DMD
b. THESE VIRUSES HAD A VERY SMALL CIRCULATION

As many adults with DMD have healthy lungs are we still at higher risk should we get infected? 
Yes

Are adults with DMD who have healthy immune systems any more likely to get infected than the average person on the street?  Do steroids have an effect on this?
The risk of being infected without isolation or social distancing is the same as the general population but there is a risk that the disease will have more severe consequences on DMD people who have reduced breathing capacity and who are on steroids ie the whole DMD community.

Does Cardiomyopathy come into play with COVID-19?
Yes, people with cardiomyopathy have a worse outcome, the virus can attack the heart muscle

Should our carers be wearing masks, or will it not prevent them infecting us?
Your carers should only wear masks, gloves and aprons if you are infected. If you are infected they should wear a full protection if you are actively using NIV. All carers should wash hands or gel before touching you. They should not come into your home if they have a fever or cough or if they have had contact with someone unwell within the last 14 days

Would it be likely to be safer for us to be treated at home, especially those already on NIV?
Yes

Is using a cough assist helpful with COVID-19?

If there are secretions it will help but it will also result in aerosol spread of virus so carers need full protective gear.

Are antibiotics any help at all?
a. No

b. ONLY IN CASE OF SECONDARY, SUPERIMPOSED PNEUMONIA. ALSO BE AWARE THAT NOT NECESSARILY ANY COUGH IS CORNAVIRUS. PEOPLE COULD STILL GET A PNEUMONIA FOR OTHER REASONS

 
Oxygen use is always a concern for patients with DMD (AND DM), considering it may be needed if someone is infected with COVID-19 what advice would you give?
a. Oxygen is ok, it is only dangerous if you have respiratory failure and you are not using NIV

b. AND IN HOSPITAL CAN BE GIVEN SAFELY EVEN TO PEOPLE IN RESPIRATORY FAILURE, PROVIDED THE CO2 LEVELS ARE MONITORED CAREFULLY

Looking at the NHS NIV guidelines seems to suggest that we shouldn't use a humidifier if we are infected, would this be your advice?
Follow the guidelines

 
Looking at the NHS NIV guidelines seems to suggest that we should use a full face mask, is this just in a hospital situation to prevent spreading or is there a patient benefit?
Follow NHS guidelines

In the event of being admitted as an older adult with DMD, is there a risk we won't be treated because we are deemed frail? Could our ventilators be taken away to be given to "healthy" people?
Patients with Covid-19 will be assessed using a frailty score. They may not get critical care if the condition is advanced. Ventilators can only be used in a hospital setting in an isolating room as they spread infection

If a person with Duchenne got Covid-19, would they be admitted to their specialist centre or would they would be treated locally and the consultants would liaise remotely?
They should be managed locally, their specialised centre will give advice.

Individuals are now receiving texts from NHS England advising that they are  in the high-risk of severe ilness group. Are you involved in deciding who should receive texts and letters or has this come from another source? GPs?
See answer above

What is your interpretation of the NICE Guidance on Critical Care and the ‘frailty assessment’ in relation to people with a neuromuscular condition?
The frailty score was designed for elderly people, but it is currently being used for all people presenting to hospital to decide upon whether they get critical care or palliative care.

Are BIPAP machines going to be enough to keep people safe if they get seriously ill with COVID-19?
Increasing the time spent on bipap during infection may be helpful the alternative is intubation and ventilation on ITU but if a patient is already on BIPAP their frailty score may mitigate against going to ITU.

How do you think ventilated children and adults (like those will MTM/CNM) will cope with coronavirus - are they more at risk?
Yes they are more at risk

How could we expect the virus to impact someone who is ventilated and has a weak cough - are there any other symptoms or signs we need to look out for in this patient population, other than rise in temperature, especially when they may be too weak to cough?
The symptoms and signs are those published on NHS, GOV and press websites. The condition can lower oxygen levels.

Would you recommend that people who have MTM/CNM and have symptoms go to hospital early for treatment?  Or stay at home until they cannot cope?
a. Everyone with mild symptoms should stay at home to prevent overwhelming the hospital services so that the sickest patients can be prioritised.
b. ALSO IT IS MORE LIKELY TO BE EXPOSED TO CORONAVIRUS IN HOSPITAL THAN AT HOME, ALTHOUGH CLEARLY ALL EFFORTS ARE BEING TAKEN TO AVOID THIS.

What are your recommendations to prevent cross infection to that vulnerable individual within a household, especially if another family member has it?
Please see government and NHS guidance

If another family member has this virus - is 7 days quarantine enough?  Should we be isolating ourselves for longer than 7 days - as there is some research to suggest that we could contaminate others for a matter of weeks after having COVID-19.
a. If another member of the family has the condition they should self isolate for 7 days, if you have been in contact with an infected person but have no symptoms you should isolate for 14 days to avoid passing it on
b. THERE IS NHS ADVICE IN CASE OF A FAMILY MEMBER IN THE SAME HOUSEHOLD. IDEALLY, IF THERE IS SOMEONE AT A HIGH RISK OF COMPLICATIONS SUCH AS A NEUROMUSCULAR PATIENT, THE SEPARATE SELF ISOLATION IS THE PREFERRED CHOICE. IF THIS IS NOT POSSIBLE, ON THE NHS WEBSITE THERE IS ADVICE ON HOW TO SELF ISOLATE AT HOME AND “INCREASE THE DISTANCE” FROM THE FRAGILE CHILD

 
If family members within a household with someone with MTM/CNM gets sick can they insist on being tested?  If not, how can they get this done privately and how much would it cost?
a. No they cannot, I am not aware of a private testing laboratory

b. AT THE MOMENT THE TEST IS ALSO NOT WIDELY AVAILABLE FOR FRONT LINE PROFESSIONALS. THIS WILL CHANGE IN THE COMING WEEKS

Should ventilated patients be doing anything else preventatively if they are symptomatic? For example - should they start a regular airways clearance regime straight away? Or wait until they are symptomatic with breathing difficulty before taking action?

I WOULD THINK THEY SHOULD DO WHAT THEY USUALLY DO IN SIMILAR CONDITIONS

We are told ‘cover’ ourselves with antibiotics if we get sick - even if they are not needed necessarily. Is this also recommended for covid-19 to help prevent deterioration to pneumonia?
Unlikely to be of benefit in covid-19 however not all infections will be due to this virus, if there is any possibility of a bacterial chest infection EF coloured secretions, take antibiotics

 
(This is from a question from a family outside UK). Do you think it’s safe to be with family over our holiday that’s coming up in a couple of weeks?
Advice is to self isolate and not mix with too many people. Having contact with new people in coming weeks will increase the risk

What contingencies can we expect to be put in place by CCGs for individuals needing high-level care if their main care assistants (parents or nurses) are sick and need to self isolate? For example, for those parents who are normally expected to fill voids in care - are the local CCG obliged to step up care provision if parents are unwell?  How long should this extra help with care be provided for - 14 days?
a. I do not know, please ask carers and care agencies
b. THIS IS A COMPLICATED ISSUE FOR WHICH IS UNLIKELY THERE WILL BE A GENERAL RULE, SO BEST CHECK LOCALLY BUT BE AWARE ADVICE MIGHT CHANGE DEPENDING ON AVAILABILITY OF WORKFORCE

If someone who has SMA starts to show symptoms of Covid-19, at what point do they call for healthcare intervention and who do they contact? Should this be via 111, the GP or direct to the specialist team? Does this advice vary according to the groupings above?
If they are short of breath or feel unwell ring 111 who will give advice and decide if a GP needs to be involved. Be sure to tell them of your underlying health condition

Some people won’t have an up to date healthcare plan or emergency care plan that they have with them in case their specialist team cannot be contacted for advice. Do they need to do anything about this now and, if so, what?
a. No need to do anything now, the correct action for everyone is to ring 111 for advice.

Many people who need respiratory support at home may not, until now, have been able to get things like cough assist machines. Do clinicians have any advice about this, e.g. can anything be done now to get these to families / adults.
a. Now is not a good time to get a cough assist machine as the hospital would need to assess you and show you how to use it. Going to hospital increases your risk of catching covid-19. You need to stay at home

b. ABSOLUTELY.

What if some complication of my or my child’s SMA arises while this is all going on or, e.g. scheduled surgery for a gastrostomy or spinal surgery was due. Will I be able to get advice – by phone / email / skype consultation? Can you give some guidance about what you consider to be an issue that reasonably needs clinical advice and how to ask for it? Many people have direct contact with their teams directly (e.g. if on treatment) but certainly not all.
a. We are continuing to provide advice to patients by telephone. All non urgent surgery is cancelled. It would not be safe to put a peg in at the moment. Patients with SMA should keep away from hospital
b. ABSOLUTELY, EACH PATIENT RISK AND BENEFIT IS DISCUSSED BY YOUR TEAM BUT NON-URGENT PROCEDURES ARE LIKELY TO BE POSTPONED, FOR GOOD REASONS

Some people in the SMA community have been reading reports from other countries that suggest people with SMA will be left untreated if they get Coronavirus and are unwell. This is justified in such reports by SMA being considered ‘a terminal condition’, therefore other individuals get priority. We understand some European  countries are releasing critical care protocol: ‘covid 19 and access to critical care’ and that access by those with rare conditions has been of concern and an urgent discussion point at Eurodis today (27th March).
a. Please see my comment above resulting frailty score. People with mild SMA should get full treatment

b. THE WORLD MUSCLE SOCIETY HAS JUST RELEASED A CONSENSUS DOCUMENT STRESSING THE NEEDS OF PEOPLE WITH NEUROMUSCULAR DISORDERS AND THE OVERALL GOOD OUTCOME THAT THESE PATIENTS CAN NOW EXPERIENCE SO THAT THIS COULD ALSO BE USED AGAINST ANY DISCRIMINATION. WHATCH THE SPACE IN THE WMS WEBSITE

Questions from other groups which may be less relevant to the Myotonic Dystrophy community.

According to government advice, patients are ‘at risk’ if they are “on immunosuppression therapies sufficient to significantly increase risk of infection”.
Are corticosteroids (deflazacort & prednisone), at the dose they are given in DMD, considered to be immune suppressing?
a. Yes
b. HERE I WOULD SAY THAT THE ANWSER IS THAT PROBABLY YES ALTHOUGH THE TYPICAL DOSES USED FOR FULL IMMUNSUPPRESSION ARE SIGNIFICANTLY HIGHER THAN THE DOSES OF BOYS AND YOUNG MEN. THERE IS LACK OF INFORMATION ON THE CHRONIC EFFECT OF THE STEROIDS ON THE IMMUNE SYSTEM EVEN AT THE LOWER DOSE USED, SO WE AGREED THAT IT IS BETTER TO BE ON THE SAFE SIDE

Should patients stop taking steroids?
No

Can you explain what stress dosing is and when it should be used?
a. Stress dosing is to prevent adrenal suppression during moderate to severe illness. If you do not have a plan in place for this, the advice is to give an extra dose of steroids in the evening for 3 days then gradually reduce this dose over another 10 days. Only do this for illness that is moderate ie worse than a cold

b. AND TO DISCUSS THE PLANS ALSO WITH YOUR LOCAL TEAM WHEN THE ACCESS TO CLINIC IS STARTED AGAIN. DOCUMENTS OF THE VARIOUS PROTOCOLS IN USE IN DIFFERENT HOSPITALS HAVE BEEN CIRCULATED TO THE DMD CONSULTANTS, AS THERE ARE SUBTLE DIFFERENCES BETWEEN DIFFERENT PROTOCOLS, BUT THE BOTOM LINE IS THAT STEROIDS SHOULD NOT BE STOPPED ABRUPTLY, NEVER, AND THAT IF PEOPLE ON CHRONIC STEROIDS ARE UNWELL, MORE STEROIDS MIGHT BE USED, AND ROS SUGGESTION IS A VERY GOOD, PRAGMATIC ONE IF PEOPLE ARE UNWELL BUT NOT VERY UNWELL. IF PATIENTS ARE VERY UNWELL, THIS IS A 999 ISSUE.                                      

Should people with some neuromuscular conditions get rescue packs? If so, how should people go about accessing one?
IF RELATED TO STEROIDS, FOLLOW ADVICE ABOVE

Given we are aware medical teams won’t be in a position to have individual in depth conversations at the moment  should we e.g. alert all the community to  have a copy of chapter 10 emergency care in the SoC or would this be too generic and counterproductive?
a. The hospital teams will appreciate this information as SMA is a rare disorder
b. THE SAME APPLIES OUTSIDE THIS EMERGENCY, WE ALWAYS ENCOURAGE FAMILIES TO BE FAMILIAR WITH SOC AND SHARE WITH THE EXTENDED TEAMS

Should everyone anyway have a copy of the family guide to the standards of care at home during this time? (Note SMA UK has hard copies and could post out if people request one. It’s also online here along with links to the actual scientific articles: https://smauk.org.uk/international-standards-of-care-for-sma:
a. Will do no harm
b. ABSOLUTELY, AND, AS ABOVE, THIS SHOULD BE A CONSTANT BEHAVIOUR ALSO OUTSIDE THIS EMERGENCY

Would you urgently advise us what the position is for people who have SMA in the UK. We understand that critical care decisions are made by clinicians, we assume in consultation with the patient and their family. The SMA community needs reassurance that the fact they have SMA will not be a reason to preclude their access to critical care services should this be necessary.
a. Currently all patients are being assessed according to their degree of frailty.

b. MANY CENTRES ARE IN THE PROCESS OF SENDING A LETTER TO EACH INDIVIDUALS DEEMED AT RISK, AND THE CRITERIA HAVE BEEN CIRCULATED TO ALL THE UK PHYSICIANS.