I met Anila on Zoom to ask a few questions to find out a bit about the role her faith has played in her relationship with her medical condition, thalassaemia.

Thalassaemia is an inherited anaemia which prohibits the body from making the required amounts of haemoglobin, a vital part of red blood cells for carrying oxygen around the body.  In its most severe form, like the one Anila has, the only way to survive is through regular monthly blood transfusions at the hospital.  For many patients including Anila the accumulated iron from the transfusions can be toxic to key organs in the body^*.

Like most other people with thalassaemia, Anila grew up aware of her illness because the transfusions started from the age of 6 months.  Unfortunately Anila’s first nurse was not very empathetic and she remembers having to wait for her arrival every evening to receive a painful injection to take away the iron.  The timeframe when the nurse would come could be anything up until midnight, which crippled her social life.  She remembers since the age of 4 or 5 thinking of the hospital as her second home.  She grew up in a Hindu household and follows the Sri Sathya Sai organisation:   https://srisathyasai.org.uk/uk-organisation/

As a child she was a member of a group that’s like spiritual scouts, and this taught her many human values and spirituality, which continue to govern her every day.

Anila wanted to become a doctor but was rejected 5 times.  She was told that opening up about her condition on her application form meant that the committee thought the illness would shorten her life and would make training her as a doctor a poor investment. Rather than stop at this obstacle, this drove her to get her science degree and a PhD at Newcastle University.  Many years later at a lecture she saw the professor who rejected her, and she thanked him because the rejection had only made her more determined.  This plays into her belief that things happen for a reason and that there are no ‘mistakes’ in the way events unfold- this is the belief of karma.  Her scientific work happened in hospitals until 2010 and she became a tutor working from home, which she describes as a magical experience to get to help young people in understanding science.  “Life is all about giving what you can, doing your best and being grateful,” she says.

Anila seeks comfort in her faith to deal with the pain and difficulties that thalassaemia has brought into her life.  She believes that you have to go through what is sent along your way.  Anila says she prays for the ability to tolerate and bear the pain and that “it works wonders when you are genuine” but it is also her firm belief that “hands that help are holier than lips that pray.” She has first hand experience with the power of prayer.  She tells a story helping in a medical camp in St Petersburg, Russia, when a mother came with a baby with a severe eye infection- what was needed was penicillin eye drops but there were none in the supplies that had come to the camp.  However, one of the volunteers found a parcel that no one recognised or knew how it had arrived, and on opening it, found penicillin eye drops. 

Her friends and family have always remained supportive and positive, staying by her side through every difficulty and every celebration, and are always prepared to make her laugh.  She is adamant that “laughter is one of the best medicines” and that “it is important to stay grateful for what you have because there is always someone else worse off”.  Affirmations and acceptance work to give her piece of mind even now whilst tutoring- she advises others not to label themselves negatively because those thoughts hold you back. 

I feel really privileged to have had the chance to meet and speak with Anila.  Her story resonated with me and learning about she responded with courage and commitment to what others would see as unfortunate obstacles in her life is truly inspiring.

*nowadays, most people can avoid this toxicity by taking regular tablets, which were not available when Anila was growing up

• interview by Maya, year 11

Summary of CAN Q&A on March 27^th 2020
 
These are the key points that came out of the live Q&A.  The information is correct as of 27.03.2020- further Government guidance may be issued and reviewed by the National Haemoglobinopathy Panel.  Please contact info@togetherwecan.uk if you have any queries.
 
Introduction
Please remember the guidance discussed here is specific to the United Kingdom.  Some of the guidance issued here will not reflect national guidance given elsewhere.  Please check with you care providers what guidance is applicable to you.
 
Hand hygiene 
This cannot be over-emphasised. There is a lot of information about how this should be done:
https://www.nhs.uk/live-well/healthy-body/best-way-to-wash-your-hands/
https://www.youtube.com/watch?v=bQCP7waTRWU&feature=youtu.be
 
Social distancing
This is the advice given to everyone in the UK at the moment:
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
 
Self-isolation/ ‘shielding’
This is much more strict in terms of limiting contacts with anyone outside the home (including not having visits from family members who live elsewhere).  Someone who is shielding should NOT go outside at all for 12 weeks, not even once a day.  They should stay alone in their room, and if they share a bathroom or kitchen, then they should use this on their own and the people who have used it before should make sure they clean everything with sanitiser before the patient enters the room.
 
More detail can be found here:
 
https://www.gov.uk/government/publications/guidance-on-shielding-and-protecting-extremely-vulnerable-persons-from-covid-19/guidance-on-shielding-and-protecting-extremely-vulnerable-persons-from-covid-19
 
If someone is shielding, does their partner also need to shield?
No, their partner or other people in the same house do not need to shield.  They just need to do social distancing.
 
Why have some people received letters from their GP or NHS England telling them they are high risk/vulnerable, and other people have not, even if they have the same condition?
NHS England has apologised to the National Haemoglobinopathy Panel for the errors in letters that were sent and the distress this has caused some people.  Part of the reason for some of the errors is down to what we call ‘coding’ which means that some people with sickle cell trait (who are perfectly healthy) have received letters saying they are ‘vulnerable’ when they are not.  If you are unsure about having or not having received a letter, please check with your clinical team.
 
So, who should have received a letter?
A letter should have gone out to:

• Everyone with sickle cell disease
• Some patients with thalassaemia- the ones with very high iron levels in the liver or heart, the ones with some heart impairment, or the ones with splenectomy AND another risk factor like diabetes
• Patients with DBA who are on high doses of steroids (check with your doctor whether the dose you are on qualities), or whose adrenal glands don’t work, or who have very high levels of iron, or whose immune tests show abnormalities, or the ones who have had a bone marrow transplant in the last 6 months or are still on immunosuppressive drugs
• Patients with other types of inherited anaemias (eg CDA, PK deficiency) who have had a splenectomy AND have a high level of iron

 
What does it mean if you fall in one of the categories above?
It means you should be ‘Shielding”.  However, we recognise that this is not always possible.  Some young children fall in that category and of course they will need contact with their caregivers.  Some people will live in a very small space where being completely isolated for 12 weeks would have a poor effect on their mental health.  In some cases, therefore, going out for a walk on your own and washing your hands when you come back would seem a better option than staying cooped up all day every day.
 
What if I am meant to be shielding but I live alone?
In that case you will be eligible for help- someone will be able to do your shopping for you, or collect your prescriptions.  You can sign up for this here:
 
https://www.gov.uk/coronavirus-extremely-vulnerable
 
What if I am meant to be shielding but I am meant to go to hospital appointments?
All appointments will be rationalised so that only the critical appointments are kept.  Your clinical team should be doing that, but if you are not sure whether an appointment is really necessary, do check with the team looking after you.  There is currently no need to change blood transfusion schedules to reduce hospital appointments.  Appointments which may not be needed include scans (eg Ferriscans) unless changes in treatment would follow the scan (eg change in chelation schedule).  Interactions with staff in the hospital are expected to be safe, and do not require masks.  However, it is perfectly acceptable to ask staff to wash their hands if you have not witnessed them doing so.
 
Many upcoming transplants will have been delayed by several months, so routine pre-transplant appointments should not be kept.  Please check with your medical team in case some of your medications need to have dose changes.  Patients with central lines that are not being used regularly and/or that are not critical should be removed to reduce the risk of infection.
 
It is important that you do not take public transport to your appointments.
 
Do you need to shield if you have HS (hereditary spherocytosis) and you have had your spleen removed (splenectomy)?
This is very very confusing, and we don’t blame anyone for being unsure about this, especially as the UK Government guidance specifically mentions people without a spleen as being very vulnerable.  However, the National Haemoglobinopathy Panel (experts in red cell disorders in the UK) has reviewed the guidance and decided that as long as people without a spleen are up to date with their vaccines and take their preventive antibiotics (if prescribed), they do not need to shield.  Some patients strongly disagree with this, and if you want to shield, you absolutely can do so.  Anyone can shield if they want to.  It is just something that is very difficult to achieve, and on balance people with HS and a splenectomy probably don’t have such a severe risk of viral infection to require this level of protection.  Some people have pointed out that they are asked to have a flu vaccine every year so that must mean they are at increased risk of viruses.  However, the reason for having the flu vaccine is to prevent flu which could bring you into hospital, where the lack of a spleen would put you at increased risk of bacterial infection.
 
If I am meant not meant to be shielding, does that mean I can go to work?
No!  We have had this question asked a lot, some of you are key workers and want to carry on helping, and we can understand that.  However, you will not be helping anyone if you get Covid-19 and become very unwell.  So if you are just social distancing, you should not be going to work.  Speak to your employers and see if there is something helpful you can do from home.  The only exception to this would be people with HS and a spleen, who would be safe to carry on working if they are a key worker.  However, please discuss with the team normally looking after you to confirm.
 
What if your employer is threatening not to pay your salary because you are not going to work?
In many cases, the employers are not following their duties.  Please speak to your medical team- they will be able to provide you with a letter that you can show your employers.  You can also get one from this website:
 
https://111.nhs.uk/isolation-note
 
 
 
 
Dealing with a fever or other symptoms that could be Covid-19
It is very important not to self-isolate in the case of a fever, because the fever could result from a bacterial infection that requires antibiotics.  On developing a fever, it is important to put a hold on chelation, as in the short term it can do more harm than good.  In a very small group of patients (those with iron in the heart), chelation must not be stopped- these patients will know who they are because your team will contact you and let you know.
 
If you get a fever and/or cough, you should consult the 111 website:
https://www.nhs.uk/conditions/coronavirus-covid-19/
 
In some cases you will be advised to attend hospital for testing.  Please follow this advice.
 
However, even if the advice is to stay at home, you must contact your team or attend hospital as you normally would.  Depending on where you live and your local arrangements, for some people this will be a phone call to a 24h line, for others it will be attending the ward, for others still it will be A&E.  Wards and A&E are likely to have been reorganised temporarily and if at all possible, a phone call is advised to check new arrangements.  It is probably a good idea to do this when well rather than wait and do this in case of an emergency.  Please bring your latest clinic letter so that the doctors who see you will have all of your clinical information and drug doses within easy reach.  You may be reviewed by doctors who are not familiar with your condition or your usual clinical pathway due to radical staff changes.
 
Blood transfusions
There are no changes to regimes at the moment, but this may become necessary if blood shortages happen.  Some people will have longer intervals between transfusions and running at a slightly lower Hb. This is not dangerous.  There are currently no blood shortages but please raise the profile in your networks to get people to give blood- known donors as well as new donors.  It is important that you do not take public transport to your appointments.
 
 
Should you wear masks and gloves if you go outside?
The only masks that really protect you from Covid-19 are the ones which will only be available in the hospital.  Some people find wearing a mask outside (bought in a pharmacy or even made at home) makes them feel safer and helps other people keep the 2m distance that is important to respect outside.  Gloves can help prevent you from coming into contact with Covid-19.  However, if you are outside for a walk and you don’t touch anything, and you wash your hands when you get home, this should be perfectly sufficient.
 
What if you have HS and you always get lots of haemolysis when you have a viral infection and you need transfusions?
It’s true that people with HS can get a lot of haemolysis (red cell breakdown) triggered by viral infections, and may require a blood transfusion.  It’s possible that Covid-19 will also cause that.  The way you would be able to tell if you may need a transfusion is getting more jaundiced (yellow around the pupils of the eyes) and getting more tired and breathless.  In that case, contact your usual team to see if you can be assessed for a transfusion.
 
What can you do to stay as healthy as possible and should you increase your dose of antibiotics?
To stay healthy, wash your hands (have we mentioned that?).
Stay hydrated.
Take all the medications you are meant to be taking. If you are not up to date with your vaccines, call your GP to arrange the vaccines as soon as possible.
Look after your mental health.
 
Please do not increase the dose of any of your medications without first speaking to your medical team.
 
Why is sickle cell disease such a risk for severe Covid-19?
As well as not having a functioning spleen (in sickle cell disease, by the age of 3yrs the spleen stops working), patients with sickle cell disease are prone to sickling in the lungs when oxygen levels get low- this is called a chest crisis, and it can be fatal.  Because Covid-19 can cause a lung infection, it can drop the oxygen levels and start sickling in the lungs.  Doctors would treat this by an exchange blood transfusion.
 
Has anyone with sickle cell disease been known to have Covid-19 and how did they do?
Yes, we know of several patients with sickle cell disease who have had Covid-19.  Like in the general population, some have been very mild (like a cold, not requiring hospital admission), some have required hospital admission and some have required intensive care.  Like in the general population, some patients with sickle cell disease have contracted Covid-19 and sadly died.
 
Has anyone with HS been known to have Covid-19 and how did they do?
We have not heard of any reports, but this does not mean no one has had it.
 
If someone has CDA-1 and they are on interferon, can this increase their risk of Covid-19?
We have no data on interferon alpha (the type used in CDA-1), but there are some reports that interferon beta can help improve Covid-19 infection.
 
Does having PK deficiency increase the risk of severe Covid-19 infection?
No, the risk is not increased, but if your spleen has been removed, you will be like the HS patients who need to make sure they are social distancing.
 
Updates
There are weekly meetings at high level so this guidance is being monitored nationally.  If the guidance needs to be changed more rapidly, it will be.
 
 
Finally
This is a scary time for everybody, and we understand that.  It can feel confusing to keep up with all of the advice and guidance.  All guidance will aim to cover as many eventualities as possible, but will never be able to suit every single person’s individual circumstances.  However, there is no need to panic.  Common sense must prevail, and looking after your physical and mental health more generally is very important.  Good food, enough sleep, plenty of laughter.  Oh, and don’t forget to wash your hands!
 
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