This document is a collaboration between the Haemoglobinopathy Co-ordinating Centres in England, offering guidance to patient with Diamond Blackfan anaemia (DBA) and their carers. It has been reviewed and agreed by representatives of the Haemoglobinopathy Co-ordinating Centres in England and the Clinical Reference Group for Haemoglobin disorders and has had input from patient representatives.
Date: 18/03/2020 Version 1.0
What is Coronavirus and COVID-19?
Coronaviruses are a large family of viruses which may cause illness in animals or humans. In humans, several coronaviruses are known to cause respiratory infections ranging from the common cold to more severe diseases such as Middle East Respiratory Syndrome (MERS) and Severe Acute Respiratory Syndrome (SARS). The most recently discovered coronavirus is called SARS-CoV-2 and causes coronavirus disease COVID-19.
The transmission mode is by spreading from person to person through:
respiratory droplets spreading when coughing or sneezing
close personal contact with an infected person (shaking hands or touching)
touching something with the virus on it and then touching your eyes, nose or mouth with unwashed hands
What should I do?
It is essential that to minimise its spread to take the following measures:
Wash your hands with soap and water often – do this for at least 20 seconds
Always wash your hands when you get home or into work/school
Use hand sanitiser gel if soap and water are not available
Cover your mouth and nose with a tissue or your sleeve (not your hands) when you cough or sneeze and put used tissues in the bin immediately and wash your hands afterwards
Follow NHS advice on https://www.nhs.uk/conditions/coronavirus-covid-19/
All patients with Diamond Blackfan anaemia should:
a. Avoid close contact with people who have symptoms of coronavirus
b. Refrain from non-essential travel and only travel on public transport if you need to.
c. Work from home, if possible.
d. Avoid social activities, such as going to pubs, restaurants, theatres and cinemas
e. Avoid events with large groups of people
Discuss with lead medical provided possibility of remote consultations and postponement of routine monitoring tests and clinical consultations that are not essential.
We encourage patients to let their specialist teams know if they have symptoms or have to self-isolate, or if are admitted to hospital.
Who needs to take stringent Social Distancing Measures?
Follow advice on https://www.gov.uk/…/guidance-on-social-distancing-forevery…
We should all refrain from non-essential travel, avoid social activities and events with large group of people to minimise the spread of coronavirus.
If you are a patient with DBA and either:
You are taking steroids in the form of prednisolone or equivalent at the following doses:
children: prednisolone ?0.5 mg/kg on alternate days or ?0.25 mg/kg daily
adults: prednisolone ?30 mg on alternate days or ?15 mg per day
have an immunodeficiency as part of DBA then Stringent Social Distancing Measures are strongly advised and these include:
Significantly limiting face-to-face interaction with friends and family if possible
Avoid attendance to nursery/school/university
Work from home
Please discuss with your specialist team whether you are in this category.
Other patients should follow current NHS advice.
Do I need to make changes to my treatment?
No changes to your usual treatment are required. However, you should avoid if possible changing to a new form of treatment unless essential.
Patients on regular transfusions should remain on the same schedule. Blood transfusions are safe. NHSBT are working to maintain the blood supply and will liaise with hospitals in good time if problems are likely to develop to make the necessary arrangements.
If may be that if the situation worsens significantly your transfusion threshold will need to be lowered from your usual Hb level. Chelation treatment and its monitoring should continue as usual, though you can expect MRI monitoring to be postponed if your iron control is good. If a fever develops, you should stop all chelation agents at once.
Patients on steroid treatment should remain on the same steroid regimen.
Patients in haematological remission do not require additional monitoring.
The exceptions are the following:
We do not recommend patients starting a steroid trial because of the suppression of the immune system with high doses of steroids. The steroid trial best postponed until the risk of Coronavirus has ceased.
We do not recommend undertaking a bone marrow transplant for transfusion dependent DBA and the likelihood is that your specialist team will postpone this. There may be circumstances in which a bone marrow transplant still needs to go ahead (for examples patients who have ceased to produce blood altogether or who have abnormal cells) but these circumstances are not common and will be discussed by the specialist team.
In case of diagnosis of COVID-19:
You may not necessarily need to be treated in hospital. It is important that you contact and inform your specialist team they have symptoms or have to self-isolate, or if are admitted to hospital. The aim is to identify those patients who require direct care because they have developed the infection in the lungs.
The use of paracetamol, and not ibuprofen, is at the current time the preferred option to treat the symptoms of coronavirus, unless your doctors has told you that it is not suitable for you.
If receiving chelation treatment: you should stop chelation treatment at once and discuss this with your specialist team, as with any other febrile episode.NHS advice about coronavirus (COVID-19), including what to do if you think you have it and how to reduce your chances of getting it.