Coronavirus (COVID-19) & Sickle Cell Disorder
This page provides the latest information and guidance regarding coronavirus (COVID-19) and sickle cell. [Updated 26/06/2020]
What is Coronavirus (COVID-19)?
The main symptoms include high temperature and/or new and continuous cough.
The current risk level in the UK is high.
As the illness is new, there is limited specific information for people living with sickle cell. However, below we have included all the available specific information there is and included the official guidance on avoiding catching or spreading germs and what to do if you need medical help, which all apply.
- For everyone: Staying alert and safe (social distancing) – this includes anyone with sickle cell trait (sickle cell carriers)
- For all patients with a sickle cell disorder (e.g. HbSS, HbS Beta thalassaemia, HbSC, HbSD, HbSO): Shielding
More details around each section of guidance can be found further down in this article
Initial Data – Patient information on COVID-19 in haemoglobinopathy and rare inherited anaemia patients
Since the start of the COVID-19 pandemic, some groups of patients have been classed as ‘clinically extremely vulnerable’ and advised to ‘shield’ in order to avoid becoming infected. Many patients and patient support groups would like know how frequent and how severe COVID-19 has been for people with inherited anaemias, including:
- Sickle cell disease
- Thalassaemia (transfusion dependent and non-transfusion dependent)
- Diamond Blackfan anaemia
- Congenital dyserythropoietic anaemia
- Congenital sideroblastic anaemia
- Pyruvate kinase deficiency (with and without a spleen)
- Hereditary Spherocytosis (with and without a spleen)
- Other types of rare inherited anaemia
Since March 2020, a national group of doctors and nurses looking after people with inherited anaemias (called the National Heamoglobinopathy Panel) have been meeting via videoconference once a week to discuss how COVID-19 has affected their patients. This discussion is entirely confidential, with no patient-identifiable details discussed at all. The group reviews guidance issued by NHS England and advises NHS England and patient support groups about specific COVID-19 risks. Hospitals across the England have sent in anonymised data to the group regarding the number of cases of COVID-19 and what happens to people who get it.
How many people with inherited anaemias have had Covid-19?
As of May 6th, 195 people had been reported with proven or suspected COVID-19. Of these, 175 were adults and 20 children. Most had Sickle cell disease, but a small number of thalassemia and rare inherited anaemia patients were also affected.
How many people were admitted to hospital?
About three quarters of patients were admitted to hospital (about 146 people), and the rest managed at home. Just as many women as men were admitted to hospital. Of the patients admitted to hospital, about a tenth needed intensive care (about 15 people). Of the 20 children suspected or proven to have COVID-19, none required intensive care.
What was the outcome for people admitted to hospital?
The picture is not yet complete, because some people are still being treated for COVID-19. However, for the moment we can say that:
- the available results show that for every 100 patients infected, 92 (92%) have recovered and 8 (8%) have died
- the people who died were more likely to have other medical problems such as heart disease, cancer, kidney disease, diabetes and high blood pressure
- it is too early to say whether patients with inherited anaemias do any worse than the general population
- children with sickle cell disease, thalassaemia and rare anaemias who do not have other risk factors, do not seem to be at increased risk of having severe disease
- adults with sickle cell disease should still be considered ‘clinically extremely vulnerable’ and follow government guidance about shielding
- in the case of adults with thalassaemia and rare anaemias, the advice on shielding should be assessed for each person by their doctor
- it is important to note that there have been many people with inherited anaemias who have had few or no symptoms with their COVID-19 infection, and who have fully recovered
The data gathered in the national survey will be updated as more information becomes available.
What do I do if I think that I or my child may have Covid-19?
Please do not stay at home without telling anyone– it is important to contact your medical team and discuss whether or not you should be assessed in hospital.
Isn’t coming to hospital dangerous?
There are many people who have been extremely concerned about presenting to hospital, some have delayed at times when they have been very unwell. If someone with these important health conditions is unwell, please seek advice from your specialist team. Hospitals have been re-organised to protect people who come in with non-COVID admissions.
What can I do to stay well?
We recommend that continuing to focus on physical and mental health in all areas is also important eg taking prescribed medication, improving your diabetes, exercising, or eating healthy foods.
If you have any concerns about you or your child’s individual risk please discuss it with your specialist team.
Continue reading for the official guidance and advice
Staying alert and safe (social distancing) – for anyone with sickle cell trait
- People and employers should stay safe in public spaces and workplaces by following “COVID-19 secure” guidelines. This should enable more people to go back to work, where they cannot work from home, and encourage more vulnerable children and the children of critical workers to go to school or childcare as already permitted
- You should stay alert when you leave home: washing your hands regularly, maintaining social distancing, and ensuring you do not gather in groups of more than two, except with members of your household or for other specific exceptions set out in law (click here to find out more about staying alert)
- You must continue to stay home except for a limited set of reasons but – in line with scientific advice – can take part in more outdoor activities
It is still very important that people stay home unless necessary to go out for specific reasons set out in law. These include:
- for work, where you cannot work from home
- going to shops that are permitted to be open – to get things like food and medicine, and to collect goods ordered online or on the phone
- to exercise or spend time outdoors for recreation
- any medical need, to donate blood, avoid injury or illness, escape risk of harm, or to provide care or to help a vulnerable person
Shielding – for all patients with a sickle cell disorder (e.g. HbSS, HbS Beta thalassaemia, HbSC, HbSD-Punjab, HbSO-Arab)
The latest government guidance strongly advises people with serious underlying health conditions (click here to see the full list of clinically extremely vulnerable groups) to rigorously follow shielding measures in order to keep themselves safe. Please see below for more information on shielding and how the measures are going to be relaxed.
This includes all patients with sickle cell disorder (e.g. HbSS, HbS Beta thalassaemia, HbSC, HbSD-Punjab, HbSO-Arab).
Please note that this does not apply to people with sickle cell trait (sickle cell carriers, HbAS) – read more about trait here.
Some of this guidance is specific to England, you can find more specific guidance for Wales, Scotland and Northern Ireland here:
- Wales: https://gov.wales/shielding-extremely-vulnerable-people
- Scotland: www.gov.scot/publications/covid-shielding/
- Northern Ireland: https://www.nidirect.gov.uk/articles/guidance-shielding-extremely-vulnerable-people
Latest Government update on shielding:
For now, the guidance remains the same – stay at home and only go outside to exercise or to spend time outdoors with a member of your household, or with one other person from another household if you live alone (see below for more information) – but the guidance will change on 6 July and again on 1 August , subject to clinical evidence.
Shielding and other advice to the clinically extremely vulnerable has been and remains advisory.
From 6 July, the guidance will change so you can meet in groups of up to six people from outside your household – outdoors with social distancing. For example, you might want to enjoy a summer BBQ outside at a friend’s house, but remember it is still important to maintain social distancing and you should not share items such as cups and plates. If you live alone (or are a lone adult with dependent children under 18), you will be able to form a support bubble with another household.
From 1 August, you will no longer need to shield, and the advice will be that you can visit shops and places of worship, but you should continue maintaining rigorous social distancing.
More information on this guidance can be found below.
What is Shielding?
Shielding is a measure to protect clinically extremely vulnerable people (including sickle cell disorder) by minimising contact with those who are clinically extremely vulnerable and others and reducing the risk of infections.
Shielding guidance is being relaxed in stages, subject to clinical evidence. See below for the updated advice and the dates it comes into play:
- Now until 6th July
- From July 6th to 1st August
- From 1st August onwards
From 22nd June to 6th July
If you fall into a clinically extremely vulnerable group (have sickle cell disorder), you are strongly advised to avoid any face-to-face contact with people outside your home as much as possible until 6th July. If you become unwell during this time you must contact your hospital care team for advice as usual to be sure that you receive the right treatment for your sickle cell disorder. In a medical emergency call an ambulance on 999.
People who are shielding remain vulnerable and should continue to take precautions as stated on this page. The latest government guidance says that those shielding can now leave their home if they want to, as long as they are able to maintain strict social distancing.
Spending time outdoors can be done with members of your own household, or if you live alone with one person from another household (it is best if this is the same person each time).
The choice to go outside is up to the individual. Time outside in the fresh air is good for you but you can choose to remain home at all times if you do not feel comfortable with any form of contact with others.
If you do go out, you should take extra care to minimise contact with others by keeping 2 metres apart (this includes other members of your household).
Below is further guidance on the updated advice:
- If you wish to spend time outdoors, this should not be in other buildings, households, or enclosed spaces and you should take extra care to minimise contact with others by keeping 2 metres apart.
- If you choose to spend time outdoors, this can be with members of your own household. If you live alone, you can spend time outdoors with one person from another household (ideally the same person each time).
- You should stay alert when leaving home: washing your hands regularly, maintaining social distance and avoiding gatherings of any size.
- You should not attend any gatherings, including gatherings of friends and families in private spaces, for example, parties, weddings and religious services.
- You should strictly avoid contact with anyone who is displaying symptoms of COVID-19 (a new continuous cough, a high temperature, or a loss of, or change in,sense of taste or smell).
- Visits from people who provide essential support to you such as healthcare, personal support with your daily needs or social care should continue, but carers and care workers must stay away if they have any of the symptoms of coronavirus (COVID-19). You may find this guidance on home care provision useful. All people coming to your home should wash their hands with soap and water for at least 20 seconds on arrival to your house and often whilst they are there.
This advice applies until the 6th July
From 6 July to 1st August:
- You may, if you wish, meet in a group of up to 6 people outdoors, including people from different households, while maintaining strict social distancing
- Remember to avoid sharing items such as cups and plates
- You no longer need to observe social distancing with other members of your household
- If you live alone (or are a lone adult with dependent children under 18), you will be able to form a support bubble with another household.
From 1 August:
- The advice to ‘shield’ will be paused. From this date, the Government is advising you to adopt strict social distancing rather than full shielding measures. Strict social distancing means you can go out to more places and see more people but you should still try and minimise contact with others outside your household or support bubble.
- You can go to work, if necessary, as long as the business is COVID-safe. If possible, it is still safer to work from home.
- Children who are clinically extremely vulnerable (have sickle cell disorder) can return to school or college, when this is possible, in line with their friends and classmates. Where possible children should practise frequent hand washing and social distancing
- You can go outside to buy food, to places of worship and for exercise but you should maintain strict social distancing
- You should remain cautious as Covid-19 can be a severe infection, and may cause more complications in some people with sickle cell disorder
- Some people with particular complications of sickle cell disorder might be advised to continue to shield after 1st August by their doctor. If you are particularly worried about stopping shielding you should discuss this with your sickle cell doctor or nurse specialist.
Why is the guidance changing now?
Current statistics show that the numbers of people catching coronavirus continues to decrease. On average less than 1 in 1,700 people are estimated to have the virus, down from 1 in 500 four weeks ago.
Unless advised otherwise by your clinician, you are still in the ‘clinically extremely vulnerable’ category if you have sickle cell disorder and should continue to follow the advice for that category, which can be found here.
The Government will monitor the virus continuously over the coming months and if it spreads too much, they may need to advise you to shield again.
If you are in receipt of Government provided food boxes and medicine deliveries, you will continue to receive this support until the end of July.
See further down this page for more information on the support that is available.
What support will be available after July?
From 1 August, people with sickle cell disorder will still be classified as clinically extremely vulnerable and will continue to have access to priority supermarket delivery slots if you have registered online before 17 July for a priority delivery slot.
NHS Volunteer Responders will also continue to offer support to those who need it, including collecting and delivering food and medicines.
The NHS Volunteer Responders Scheme has been expanded to offer a new Check in and Chat Plus role. This new role has been designed to provide peer support and companionship to people who are shielding as they adapt to a more normal way of life.
If you are vulnerable or at risk and need help with shopping, medication or other essential supplies, please call 0808 196 3646 (8am to 8pm).
You can find further Government support here: https://www.gov.uk/find-coronavirus-support
The updated shielding guidance should not affect any social care or support you were receiving prior to the start of shielding.
Individuals should continue to contact their local council if they have any ongoing social care needs.
Given the very unusual circumstances that shielding creates, it is important to be aware of ways to keep oneself as fit and healthy as possible. This includes eating a varied diet which should include plenty of fresh fruit and vegetable. Foods that contain Vitamin D such as oily fish and eggs are important as Vitamin D deficiency is very common not only in the general population but also in sickle cell disorder and may exacerbate bone pain. Sunlight on bare skin is a good way to increase Vitamin D intake so every opportunity should be taken to benefit from the sun, if only at an open window or on a balcony, if sitting in a garden is not feasible.
Taking regular moderate exercise is not only good for physical health but also improves general mood and helps overall mental health. Very rigorous exercising is not recommended in sickle cell disorder and if the weather is hot care should be taken to drink plenty.
If you are anxious about your health whilst shielding or want more advice you can contact your specialist health care team . If you become unwell you should contact your GP and in an emergency phone 999.
Living with Other People
We know that many people live with others and may be concerned about how to effectively shield whilst sharing a house or flat. Below is guidance if you have a sickle cell disorder and you live with other people.
Although the other people you live with do not need to follow shielding guidelines (unless they also fall into an clinically extremely vulnerable category – click here to see the full list) everyone in the house should do what they can to support you in shielding and strictly follow the specific advice below and general social distancing advice (found later in this article).
- Try to minimise the time spent with other people in shared spaces (kitchens, bathrooms, sitting areas)
- Keep shared spaces well ventilated
- Aim to keep 2 metres (3 steps) away from people you live with and encourage them to sleep in a different bed where possible
- If possible, use a separate bathroom from the rest of the household
- If you do share a toilet and bathroom with others, it is important that they are cleaned after use every time (for example, wiping surfaces you have come into contact with)
- Use separate towels from the other people in your house, both for drying themselves after bathing or showering and for hand-hygiene purposes
- Another tip is to consider drawing up a rota for bathing, with you using the facilities first
- If you share a kitchen with others, avoid using it while they are present
- If you can, you should take your meals back to your room to eat
- If you have one, use a dishwasher to clean and dry the family’s used crockery and cutlery. If this is not possible, wash them using your usual washing up liquid and warm water and dry them thoroughly
- If you are using your own utensils, remember to use a separate tea towel for drying these.
We understand that for many people it will be difficult to separate themselves from others they live with. Try as best you can to follow this guidance and everyone in your household should regularly wash their hands, avoid touching their face, and clean frequently touched surfaces.
Getting help with food, shopping and medicine
To continue to get food, shopping and medicine you should ask your family, friends and neighbours to support you and use online services.
If this option is not available then the public sector, business, charities, and the general public are gearing up to help those advised to stay at home.
During this time of staying at home (shielding) please speak to those you trust on how they can support you (family friends, carers, neighbours, and community groups).
From Tuesday 24th March 2020, if you are in the clinically extremely vulnerable group (sickle cell disorder) you will be able to apply for support (including food, shopping and additional care).
Please visit https://www.gov.uk/coronavirus-extremely-vulnerable to find out more.
During this period, prescriptions will continue to be provided as usual. If you do not currently have your prescriptions collected or delivered, you can arrange this by:
- Asking someone who can pick up your prescription from the local pharmacy, (this is the best option, if possible)
- Contacting your pharmacy to ask them to help you find a volunteer (who will have been ID checked) or deliver it to you
- You may also need to arrange for collection or delivery of hospital specialist medication that is prescribed to you by your hospital care team
Care support from your local authority or health care system will continue as normal, with additional precautions being made to make sure that you are protected.
Hospital and GP Appointments
Where possible, try and access medical assistance remotely. Hospital care teams are offering most patients telephone or video consultations during the COVID-19 outbreak.
If you have a scheduled hospital or other medical appointment and have not been contacted by your GP or specialist about this, call them before attending to confirm that the appointment has not been canceled or postponed. Talk to them about how best to ensure you can continue to receive the care you need.
Children and Schools
The latest government guidance states that from the 1st June there will be a phased return of children and young people to nurseries, schools and colleges in a way that is measured, reduces risks and is guided by science.
For children in the clinically extremely vulnerable group
The government guidance states that children and young people who are considered extremely clinically vulnerable and shielding (sickle cell disorder) should continue to shield and should not be expected to attend.
Children and young people who live in a household with someone who is extremely clinically vulnerable and shielding (sickle cell disorder) should only attend if stringent social distancing can be adhered to and the child or young person is able to understand and follow those instructions.
Post-Covid Inflammatory Syndrome
Specialists in Italy and England have described an increase in cases of an paediatric inflammatory multisystem syndrome (similar to Kawasaki disease) following the beginning of the pandemic with COVID-19*. This is a very rare syndrome characterised by a high temperature, rash and swollen glands. The treatment includes the use of high dose steroids and the outcome is usually good.
However, this treatment could be detrimental in children who have sickle cell disorder. It is important therefore that a distinction is made between a sickle cell episode and this very rare disease. There can be confusion as symptoms overlap. It is important that you talk to your specialist paediatrician or haematologist if you are concerned about new symptoms occurring weeks after a Covid infection to ensure the right diagnosis is reached.
*BMJ 2020; 369 doi: https://doi.org/10.1136/bmj.m1990 (Published 15 May 2020)
Sickle Cell Trait
If you have sickle cell trait please follow the guidance given to the general public. This doesn’t make you more vulnerable to coronavirus infection but do check the full clinically vulnerable groups list in case you fall into another category such as if you have had your spleen removed, are pregnant or aged over 70). The full clinically vulnerable groups list can be found here.
General Advice about Feeling Unwell
If you are mildly unwell with a cough or fever below 37.8 oC you should manage your illness at home and not go to your GP or A&E. If you are worried about your symptoms you should call your hospital care team for advice.
If you suspected you have any symptoms of COVID-19 (high temperature and/or new and continuous cough) please contact your centre (even if you have called 111) of care to ensure that you receive the appropriate specialist advice on the need for further assessment.
Due to the impact other infections can have on people living with sickle cell (including pneumonia and acute chest syndrome) anyone with a sickle cell disorder who has a worsening cough, difficulty in breathing or fever above 38 oC should urgently contact their centre of care or in an emergency 999. Mention you are worried about coronavirus but also that you have sickle cell disorder. They will then instruct you further.
Sickle Cell Pain (Crisis)
If you are experiencing severe sickle cell pain (crisis) then go to hospital as normal. However, if you also have a cold, a high temperature or new and continuous cough contact your centre of care (or 999 in an emergency) first.
Taking medicines during the COVID-19 outbreak
If you are taking hydroxycarbamide or iron chelators (drugs to remove excess iron) it is important these are continued. Your centre of care will make arrangements to monitor this treatment through the hospital or your GP. There is no evidence these drugs affect the risk of COVID-19 one way or the other. However there is some concern that anti-inflammation pain killers like ibuprofen might make coronavirus infection worse. During the COVID-19 outbreak it is recommended that you take paracetamol (unless you have an allergy to it) which is safe instead.
Official guidance and information can be found here:
Here are a few important bits of guidance which you can follow:
To avoid the catching or spreading of germs:
- cover your mouth and nose with a tissue or your sleeve (not your hands) when you cough or sneeze
- put used tissues in the bin immediately
- wash your hands with soap and water often – use hand sanitiser gel if soap and water are not available
- try to avoid close contact with people who are unwell
- do not touch your eyes, nose or mouth if your hands are not clean
The NHS have a specific service for people who think they might have coronavirus. You can find out more about it here: https://111.nhs.uk/covid-19
Responding to Covid-19: Reasons for Hope
Sickle Cell Society Chief Executive, John James OBE, writes for the King’s Fund’s Leading through Covid-19 series to reflects on the challenges the organisation has faced and shares what he has learnt and what gives him hope for the future. Click here to read the full article.
111 Online Service: https://111.nhs.uk/covid-19
Extremely Vulnerable Group: https://www.gov.uk/coronavirus-extremely-vulnerable
Government Response – https://www.gov.uk/government/topical-events/coronavirus-covid-19-uk-government-response
Isolation Advice: https://111.nhs.uk/isolation-note
NHS Guidance – https://www.nhs.uk/conditions/coronavirus-covid-19/
Social Distancing: https://www.gov.uk/government/publications/covid-19-guidance-on-social-distancing-and-for-vulnerable-people/guidance-on-social-distancing-for-everyone-in-the-uk-and-protecting-older-people-and-vulnerable-adults
Universal Credit and Coronavirus: https://www.understandinguniversalcredit.gov.uk/coronavirus/