Updates for support group at Guy’s & St Thomas Hospital

Please join us at the Weekly Virtual Sickle Cell Support Group for Guy’s and St Thomas’ (GSTT) patients and caregivers

The support group for patients and caregivers from GSTT is currently running virtually on Thursdays from 1-2pm.

If you are cared for by GSTT, and would like to join us, please follow the instructions below.

Click this meeting link: https://bluejeans.com/540502643/0189

You will be prompted to install the BlueJeans App – this will offer the best PC/laptop/device based experience. If you do not want to download, you can use your internet browser page each time.

 To enter the group, you will need to type in the meeting ID: 540502643  and the participant passcode: 0189.

Please note this is the same ID and passcode each week.

 Please email HPS@gstt.nhs.uk if you would like further instructions about how to join, or to be added to our email mailing list about the group.

Thank you, and we hope to see you soon!

 

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Advice to clinicians on risk assessment for severe COVID-19 in patients with haemoglobinopathies and inherited rare anaemias

Haemoglobinopathy HCCs advice on risk assessment COVID-19 28.08.20

This information has been produced following virtual meetings with representatives from the Haemoglobinopathy Co-ordinating Centres (HCCs) for Haemoglobin Disorders, the Clinical Reference Group for Haemoglobin Disorders, the National Haemoglobinopathy Panel, NHS Screening Committee and NHSBT as well as national experts on Sickle Cell Disease, Thalassaemia and Rare Anaemias and patient groups.

This updates previous advice from this group and should be read in conjunction with government guidance advising that shielding should be paused from 1st August 2020. This document offers advice to Haemoglobinopathy practitioners if the situation arises that shielding is re-introduced. It also provides support for clinicians in having conversations about COVID-19 risk with patients.

This document covers

  1. Background information
  2. Guidance for discussions with children
  3. Planned changes in risk assessment for adults
  4. Guidance for discussion with adults
  5. Advice to patients on employment/education
  6. Additional patient information

It should be read in conjunction with ‘Advice to clinicians on planning recovery from COVID-19’ 26.06.2020. REF

1 Background information

From March 2020 certain groups of ’clinically extremely vulnerable’ people were advised to ‘shield’ to reduce their risk of COVID-19. The National Haemoglobinopathy Panel advised that all people with Sickle Cell Disease (SCD) and some of those with Thalassaemia and Rare Anaemias should shield. https://b-s-h.org.uk/media/18244/hbp-hccs-response-to-covid-v9-200420.pdf

From the 1st August shielding was paused and those shielding have been encouraged to cautiously increase activity outside the home whilst maintaining social distancing. This governmental guidance is being regularly updated and the latest advice can be viewed 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.

 

Updated advice from August 13th states that:

  • Patients may be advised to shield again if the situation changes and there is an increase in the transmission of COVID-19 in the community
  • This may include local lockdowns
  • The risks of serious illness from COVID-19 is low for most children and young people. Children and their families have been advised to speak to their paediatric specialist or general practitioner over the summer to discuss if they should be removed from the shielding list
  • All members of the household of those who are identified as ‘clinically extremely vulnerable’ at the time of the flu vaccination programme are eligible for a free flu vaccination

2 Guidance for discussions with children

The government advice says that evidence indicates that the risk of serious illness for most children and young people is low. In the future they expect fewer children and young people will be included on the shielded patient list. If a child or young person is removed from the shielded patient list, they will no longer be advised to shield in the future if coronavirus transmission increases.

The Royal College for Paediatrics and Child Health has produced some updated guidance on shielding (June 12th 2020) which should be used for discussions with children and their families and advises that clinicians should discuss this with their patients over the summer.

https://www.rcpch.ac.uk/resources/covid-19-shielding-guidance-children-young-people

This states that in principle:

  • A small group of children who are ‘clinically extremely vulnerable’ due to their pre-existing condition will need to continue to shield.
  • A further larger group of children exists who, due to their underlying condition, may need to shield. The decision to continue to shield would normally result from a discussion between the clinician, the child and their family.
  • Splenectomy alone is not an indication for shielding

Conditions listed in the red categories confer the highest risk and individuals should be advised to continue to ‘shield’.

Conditions listed in the amber categories below confer a less well defined risk and will require a case-by-case discussion to decide whether, on the balance of risks, an individual should be advised to continue shield. Not all of those in the categories listed below will need to shield.  If following a discussion, the individual is advised not to shield, they should maintain stringent social distancing.

Individual risk assessment on which shielding advice is based should be undertaken by an experienced member of the local or specialist haemoglobinopathy clinical team (medical staff or clinical nurse specialists). This may be facilitated by a screening tool or checklist to ensure consistency. Input from other clinical specialties should be sought where relevant particularly if decisions on shielding are equivocal.  It is recommended guidance on shielding is communicated verbally in the first instance and a record of the discussion documented in the patient’s health record. A letter confirming the advice given should be sent to the patient/parent(s) and copied to the GP.

Conditions listed in the categories below will require a case-by-case discussion to decide whether, on the balance of risks, a child should be advised to continue shield. Not all children and young people in the categories listed below will need to shield.  If following a discussion, a child is advised not to shield, they should maintain stringent social distancing.

Any decision on shielding should balance the clinical and social impact of shielding – weighing the benefit of keeping children and young people with underlying co-morbidities safe whilst protecting the most socially vulnerable, due to family and social circumstances, who may risk additional harm from continued shielding.  There may be other patients who do not fit these categories below, but for whom secondary care clinicians feel, after discussions with families, that shielding is advisable. We advise contacting their tertiary specialists for advice.

  Clinically extremely vulnerable: shielding strongly recommended Case-by-case discussion on possibility of continuing to shield Shielding no longer recommended
Sickle cell disease Children who have had a BMT within one year or are on continuing immunosuppression Additional co-morbidities of concern e.g progressive critical neurovasculopathy, severe or symptomatic heart failure

A history, within the preceding 12 months, of either one or more chest crisis requiring ITU treatment or two of more chest crises requiring treatment

All other children with sickle cell disease
Thalassaemia Children who have had a BMT within one year or are on continuing immunosuppression Severe cardiac iron overload (T2 *<10 ms) plus additional co-morbidity causing concern (eg splenectomy) All other children with thalassaemia
DBA Children who have had a BMT within one year or are on continuing immunosuppression Associated immunodeficiency, severe iron overload (as per thalassaemia definition) or are on prednisolone (or equivalent) ≥0.5 mg/kg/day All other children with DBA
Other rare inherited anaemias, e.g. pyruvate kinase deficiency, congenital dyserythopoietic anaemia Children who have had a BMT within one year or are on continuing immunosuppression Severe cardiac iron overload (T2 *<10 ms) plus additional co-morbidity causing concern (eg splenectomy) All other children with inherited anaemia

Clinicians should discuss with children and their families/carers their removal from the shielding list and update the shielding lists accordingly. Patients can only be removed from the shielding patient list by their GP or specialist, following consultation (via letter, phone call or face to face discussion) with the child and their family, and other clinicians where appropriate.

It is likely that the majority of children and young people with haemoglobinopathies  and inherited anaemias will be removed from the shielding lists and will NOT need to shield if shielding is re-introduced. It is essential that the shielding lists are kept up to date to ensure we don’t cause harm by keeping children away from home unnecessarily.

3 Planned changes to risk assessment in adults

Current advice for adults (as of August 13th 2020) is that patients who are on the list of ‘clinically extremely vulnerable’ could be advised to shield again if there is an increase of COVID-19 in the community. This could be national advice or may be local advice if there are local lock-downs.

The government are developing a national risk assessment tool for adults. This is based on large data sets that identify risk factors for severe COVID-19 infection. This will include factors such as age, renal disease, diabetes and cardiovascular disease. Sickle cell disease will also be included as a risk factor. Patients will be given a risk score based on all of these risk factors.  It is expected that this tool will be available in September and once this is available clinicians who look after adults with Haemoglobinopathies and Rare Anaemias will need to work with their patients to produce a risk score and provide advice on risk.

The protocol of this risk assessment tool is given here.

https://www.medrxiv.org/content/10.1101/2020.06.28.20141986v1.full.pdf

This tool is based on large data sets and as we look after rare conditions and rare complications of these conditions, these may occur too infrequently to influence this national tool. We have developed some guidance on rare complications which should be taken into consideration when discussing risk scores. This is based on data from national data collection (Telfer et al 2020 Haematologica in press), from observational data and from expert opinion.

4 Guidance for discussions with adults

 

Whilst we wait updated advice on the updated risk assessment tool, it is important to remember that advice on ‘clinically extremely vulnerable’ patients is advisory rather than mandatory. The reason for the advice is to keep patients safe and allow them to best protect themselves from the risk of COVID-19.

We recognise that shielding and social distancing are going to become more and more challenging over time for social, employment and psychological well-being. They will also impact on the need of patients to access long term health care. The challenge is how we balance the ongoing risk of COVID-19 with the risks of shielding/social distancing. People will need to make complex assessments about the ongoing risks and benefits of shielding and social distancing and make a decision that feels right for them. This advice will take into consideration the growing experience of dealing with COVID-19 in this patient population and aims to help clinicians and patients to assess risk.

Haemoglobinopathy clinicians will need to advise their patients in two ways. Firstly, we will need to help interpret the new guidance (when available) with respect to each patient’s individual clinical scenario and advise them whether they fall into the higher risk category or not. Secondly, we will need to have conversations with them interpreting the impact of this clinical risk with respect to their social, educational, employment and mental health needs. Individuals should then be able to consider their own risk profile depending on age, ethnicity and their underlying condition, take into consideration their own personal circumstances and their own risk appetite and balance this against the current risk in the community (which may change).

We have listed below factors which (on current evidence) confer increased risk of serious effects of COVID-19 and may be helpful in supporting discussions about clinical risk. It is likely that these will be updated as more evidence becomes available.

 

Current evidence shows that the largest risk factor for serious outcomes from COVID-19 is age. Among people already diagnosed with COVID-19, people who were 80 or older were seventy times more likely to die than those under 40.  Risk of dying among those diagnosed with COVID-19 was also higher in males than females; higher in those living in the more deprived areas than those living in the least deprived; and higher in those in Black, Asian and Minority Ethnic (BAME) groups than in White ethnic groups. This latter fact is of particular importance as almost all patients with sickle cell and thalassaemia are within the BAME population and hence are at increased risk from COVID.

Public Health England have produced a document ‘Disparities in the risks and outcomes of COVID-19.’ 2nd June 2020. https://www.gov.uk/government/publications/covid-19-review-of-disparities-in-risks-and-outcomes

Conditions listed in the red categories confer the highest risk of serious effects of COVID-19.

Conditions listed in the amber categories below are likely to cause an increased but less well defined risk and will require a case-by-case discussion.

Conditions listed in the green categories are unlikely to cause an increased risk of serious effects of COVID-19

  Highest risk of serious effects of COVID-19 Likely increased risk of serious effects of COVID-19 Not likely to cause increased risk of serious effects of COVID-19
General risk factors Organ transplant recipient; on chemo or radiotherapy; being treated for a haematological cancer; HSCT in past 12 months/on immunosuppression;

Severe lung condition (cystic fibrosis, severe asthma or severe COPD); on

On immunosuppressants (such as high doses of steroids); have a serious heart condition and pregnant;

ESRF on renal replacement therapy

Diabetes; IHD; Obesity (BMI 40+); Increasing age**; Liver disease (hepatitis, cirrhosis); HTN

Pre-existing cardiac disease

Auto-immune disease, on immunosuppression

Splenectomy
Sickle cell disease Severe cardiac iron overload (T2 *< 10 ms) AND additional co-morbidity (e.g. diabetes, chronic liver disease) Patients 50 years and above

CKD3 and over;

Pulmonary hypertension by echo criteria > TRV max >2.5ms; (NB >2.9 ms places patients at most risk)

Chronic sickle lung – on ambulatory oxygen;

Liver – sickle hepatopathy

Acute sickle presentation requiring ICU support in past 12 months

Two or more chest crises requiring treatment in the past 12 months

Iron overload as per thal guidelines

Severe neurological disease eg progressive critical neurovasculopathy,

 

 
Thalassaemia Severe cardiac  iron overload (T2 *< 10 ms) AND additional co-morbidity (eg diabetes, chronic liver disease)

 

Patients 50 years and above;

Severe cardiac iron overload T2*< 10ms, with no additional co-morbidities and adherent with therapy;

Cardiac iron overload (T2* 10-20ms);

Severe – moderate iron overload (LIC > 30mg/g dw and cardiac T2* > 10 ms) PLUS additional comorbidity

 

Splenectomy
DBA Patients who have had a BMT within one year or are on continuing immunosuppression Associated immunodeficiency, severe iron overload (as per thalassaemia definition) or are on prednisolone (or equivalent) ≥20 mg/day*  
Other rare inherited anaemias, e.g. pyruvate kinase deficiency, congenital dyserythopoietic anaemia If they are at particularly high risk due to iron overload as per thalassaemia guidelines above.   Splenectomy

*Most patients with DBA responsive to steroids are on doses of prednisolone ≤5 mg/kg or ≤20 mg on alternate days and therefore do NOT fall in this group.

**Age >70 years has been highlighted in the general population, but in view of the high rates of co-morbidities in this patient group, age >50 years is likely to be associated with increased risk.

Pregnant patients should be managed in accordance with the RCOG and government guidance, with updated risk assessments undertaken with employers. It is acknowledged that evidence is only just being gathered in this population hence this criteria will remain under regular review and be updated based on it.

5 Advice to patients on employment/education

Patients may also have risk factors relating to their work/education environment.  For all patients clinicians should review continue social distancing, discuss their commute and work/school environment and the potential  risks:

  1. Patient in a public/client facing/interacting: such as healthcare, education, travel, childcare
  1. OH assessment advised
  2. Recommend as per PHE website – to work from home/redeployment to a less client facing area.
  • If patient willing to accept risk and return to work suggest we advise use of appropriate PPE.
  1. Travel
  1. Avoid public transport if possible
  2. If must use public transport then consider travel outside peak hours
  • Avoid transiting through busy thoroughfares (e.g. shopping malls at peak times)
  1. Parents with children returning to school – await government advice for Sept:
  1. Per government advice continued social distancing,
  2. Discuss staggered/distanced drop off for very young with the schools
    1. Continued good hygiene measures hand washing etc
    2. Keep outer wear separate

    As lockdown is relaxed it is likely that individuals will need to return to work or education. National recommendations will need to be followed but general principles are discussed below.

    Employment

    Workers should work from home if they are able to do so and employers are encouraged to support this.  Once shielding of highly vulnerable individuals is stopped and for non-shielded patients, if patients decide to return to work employers should make special accommodations for vulnerable individuals at work if working from home is not possible.  This will include robust social distancing within the work environment, the use of teleconferencing and the use of protective equipment.  As restrictions are lifted vulnerable individuals will be able to resume working normally but will need to maintain physical distancing while in public places.

    Education

    Children of school age should be following national recommendations on school attendance

    Carers

    In some cases carers of highly vulnerable children or adults, particularly those in high risk occupations, have asked to or been advised to work from home whilst family members are shielding.  As restrictions are lifted they should continue to follow national recommendations and should discuss concerns with health professionals.  Once shielding of highly vulnerable individuals is stopped and for carers of non-shielded patients, as they return to work employers should make special accommodations including robust social distancing within the work environment, the use of teleconferencing and the use of protective equipment.  As restrictions are lifted these carers will be able to resume working normally but will need to maintain physical distancing while in public places. Non-shielded individuals should avoid public transport whilst safety concerns remain.

    6 Additional patient information

    Refer patients to up to date advice on the NHSE and PHE websites. There are also resources on the UKTS and Sickle Cell Society web pages (https://ukts.org/heads-up/coronavirus-information/  and https://www.sicklecellsociety.org/coronavirus-and-scd/ ) and further resources will be added to the National Haemoglobinopathy Panel web pages as they are made available.

    Haemoglobinopathy Co-ordinating Centres 28.08.2020

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STSTN Red Cell newsletter Easter Edition issue 14

We are pleased to announce the release of our new STSTN Red Cell Easter edition newsletter. This edition has lots of informative information regarding COVID-19 and the latest news around the STSTN network.

Download and read here

We hope you enjoy this edition!!

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COVID-19 (A MESSAGE FROM PSYCHOLOGY AT GSTT)

As the world is facing the global uncertainty and threat of the COVID-19 pandemic people may find themselves experiencing a number of difficult feelings. In the face of a crisis and the changes to the normal way of living it is natural to experience worry, stress and feeling out of control. Every person will react differently and so it is important to listen to your body and notice what you are experiencing. Some people may feel angry that their plans have been cancelled or sad that they cannot see their family, whereas others might feel grateful for some enforced rest and time to stop. Many of us will be experiencing a mix of emotions that change from hour to hour. There is not a ‘right’ way to feel.

None of us will have dealt with something quite like this pandemic before and therefore it is normal to have some difficult feelings come up when adjusting to the new ‘normal’, For people who are living with a long-term condition like Sickle Cell Disease it can feel especially challenging when you have been advised to ‘shield’ for 12 weeks. It may take some time for your brain and body to adjust as our natural instinct is to go into survival mode when there is a threat or crisis which creates fear.

We have been thinking about some challenges that you might be facing, including:

  • Anxiety and uncertainty about the future and what it might look like
  • Low mood as a result of the challenges of the pandemic or as a result of previous challenges you have been facing
  • Feeling isolated as a result of being physically separated from friends and family
  • Not being able to do what you normally do to feel well, i.e. your hobbies, socialising, which might feel frustrating or upsetting
  • Feeling trapped or having a lack of control
  • Feeling overwhelmed
  • Ensuring your basic needs are met (e.g. food, shelter, medical needs)
  • Adjustments to the way you receive care in the hospital such as virtual consultation instead of face to face contact

Some patients have told us about some interesting positive aspects to the situation, including:

  • Feeling like the whole world being ‘put on hold’ is giving them a chance to catch up
  • Feeling less pressure to achieve things like work or studying or socialising
  • Having permission to rest and recuperate and take each day as it comes
  • Feeling like the rest of the world is getting a flavour of what it can be like to have sickle cell – having plans curtailed and limits placed on activity as well as having a heightened awareness of health concerns
  • Having an opportunity to pause and re-evaluate what is important in life and how to live life based on what matters to you

We have some ideas about how to cope with the current situation. Some of them might be helpful for you and you might have tried some of them already:

If you start to notice that you do not feel like you can cope, there are places where you can get additional support:

We hope you find these thoughts and suggestions helpful. Keep well and keep safe!

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