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Sickle Cell - information and support

What is Sickle Cell?

Sickle Cell is an inherited blood disorder that affects red blood cells which contain a special protein called haemoglobin (Hb for short). Haemoglobin carries oxygen from the lungs to all parts of the body.

There are different types of Sickle Cell: 


Sickle Cell Trait

Everyone has two copies of the gene for haemoglobin; one from their mother and one from their father. If one of these genes carries the instructions to make sickle haemoglobin (HbS) and the other carries the instructions to make normal haemoglobin (HbA) then the person has Sickle Cell Trait and is a carrier of the sickle haemoglobin gene.

This means that this person has enough normal haemoglobin in their red blood cells to keep the cells flexible and they don’t have the symptoms of the sickle cell disorders. However they should be careful when doing things where there is less oxygen than normal for example scuba diving, activities at high altitude and if undergoing a general anaesthetics.


Sickle Cell Anaemia

People with Sickle Cell Anaemia do not have any normal haemoglobin A and may have Sickle haemoglobin (HbS) which is different from the normal haemoglobin (HbA).

When sickle haemoglobin delivers oxygen to the tissues, it sticks together to form long rods inside the red blood cells making them rigid and sickle-shaped. Normal red blood cells bend and flex easily. Because of their shape, sickled red blood cells can’t squeeze through small blood vessels as easily as the donut-shaped normal cells.

This can lead to small blood vessels getting blocked which then stops the oxygen getting where it is needed which can lead to severe pain and damage to organs. 

In addition to HbS there are other haemoglobin varients which can mix with HbS to cause a sickle cell disorder such as HbC, HbD, HbE and beta thalassaemia.


Sickle Cell explained

If both copies of the haemoglobin gene carry instructions to make sickle haemoglobin then this will be the only type of haemoglobin they can make and sickled cells can occur. These people have Sickle Cell Anaemia and can suffer from anaemia and severe pain. These severe attacks are known as Crises. Life expectancy is reduced but many people with Sickle Cell go on to live long fulfilled lives.

Other difficulties that may be more commonly seen in people with Sickle Cell:

  • Chronic Pain in Joints (Avascular Necrosis)
  • Gallstones (Cholecystitis)
  • Chronic Renal Failure
  • High Blood Pressure (Hypertension)
  • Stroke
  • Medication
  • Bed wetting
  • Interventions / blood transfusions

Red blood cells containing sickle haemoglobin do not live as long as the normal 120 days and this results in a chronic state of anaemia.

People with sickle cell disorder can attend school, college and work.

People with sickle cell disorder need regular medical attention particularly before and after operations, dental extraction and during pregnancy.

Many hospitals arrange follow-up appointments and it is advisable to discuss with the doctors questions concerning schooling, strenuous exercise, family planning, suitable types of employment and air travel.

When a person is found to have a sickle cell disorder it is important that all members of the family be tested. This may show they do not have sickle cell disorder but may be healthy carriers of a sickle cell trait.


 

Other types of Sickle Cell Disorders (SCDs)

There are also other different types of haemoglobin such as HbC, HbD and beta thalassaemia that can combine with sickle haemoglobin to cause sickling disorders. When someone carries the gene for beta-thalassaemia they cannot make as much HbA as they should. If this is combined with the HbS gene then more of their total amount of haemoglobin is HbS and they can suffer from what is usually a milder form of sickle cell disorder than sickle cell anaemia.

Who is affected by Sickle Cell Disorders?

The different kinds of Sickle Cell Disorders (SCD) and the different traits are found most commonly in people whose families come from:

  • Africa
  • the Caribbean
  • the Eastern Mediterranean
  • Middle East and Asia  

People who's families are not from these areas may also be affected but this is less common. 

Sickle Cell Myth busting

Some people in society may not respond appropriately to people who disclose that they have Sickle Cell because they have been given incorrect information about Sickle Cell Disorders.

Fact: People are born with Sickle Cell, just as they are born with their eye colour or hair texture. It is a condition inherited from both parents.

Fact: Sickle Cell Disorders are not; leukaemia, cancer or white blood cells eating up red blood cells. They are not infectious – you cannot `catch it’ as you would catch measles or a cold.

We encourage those with Sickle Cell, their immediate and wider families, friends and professionals to make sure the information they are acting on is from an appropriate source. This NHS (National health Service) guide from 2013 is a useful document for parents and carers and others.

Find out more: NHS - Sickle Cell - A Parents Guide 

5 Key points for schools on Sickle Cell and the effect it has on pupils and students

Comparing – Comparing one student with Sickle Cell to another student in the school is unhelpful as it affects children and young people differently. This means that associated behaviours will be different. Sickle cell is an umbrella term used to describe a range of related conditions but the type of Sickle Cell a child or young person has will dictate the way that they are affected. Comparisons can quickly erode the self-esteem of a young person who is already trying to cope with pain and treatments.


Medication – The impact that taking different medications has on children and young people with Sickle Cell may be significant. Children at Primary school level may not be confident in advocating for themselves with regard to this issue. This means that it is up to the people working with the child or young person to be sufficiently aware and informed of the possible impact of medication.

Staff should feel confident in their knowledge and ability to take appropriate supportive actions for example ensuring the child is warm and has access to a drink initially.

Details of how best to respond should be included in the child or young person’s Care Plan which also includes a Pain Scale. This is a tool developed by the school nurse in conjunction with the Sickle Cell Service that helps the child communicate their level of pain.

If it can be manged in school the child should stay in school avoiding missing out on education and creating a learning gap.

Remember there will be days that many children and young people will also have appointments at clinics where their condition is monitored and they receive treatment. This will already have an impact on their education so it is best where possible not to add to it. Review the Care Plan with the child or young person and their parent/carer so that actions are agreed by all and there is a shared understanding of what to do.


Being believed - Some children and young people are not believed when they indicate they are experiencing pain. Please ensure staff working with them know about the Care Plan and Pain Scale tool so that any response is consistent. Medication identified in the Care Plan can often be administered in school by staff in agreement with young people themselves, parents/carers and professionals involved in the child’s care.


Coexisting conditions - It is possible for children and young people to have other conditions that coexist with Sickle Cell. It is important that concerns are appropriately investigated so that the underlying cause can be identified. Some children and young people with Sickle Cell also have autism, ADHD or other conditions. Concerns about aspects of a child or young person’s development should be explored using the existing appropriate services e.g. Educational Psychology or Occupational Therapy, Speech and Language Therapy.


More complex Sickle Cell - A smaller number of children and young people have a form of Sickle Cell that affects their cognition, memory and associated behaviour. This can be because there has been an occurrence of a stroke. Children and young people with Sickle Cell are offered regular scans from the age of 2 years old to monitor and identify any risk.  If the scan indicated the child is at risk of stroke the child will be put on a blood transfusion programme.


How can schools find out more? 

Schools are routinely contacted by the Sickle Cell nurse with an offer of training on Sickle Cell, it’s impact on students and tips for managing the condition in the school setting.

If you are a school professional and would like to find out more please contact:

Lead Nurse - Natasha Lewis

Homerton University Hospital NHS Foundation Trust

Sickle Cell & Thalassemia Centre

457 Queensbridge Road

London E8 3AS

Phone: 0207 683 4570

Fax: 0207 254 3364

e-mail: natashalewis1@nhs.net

How are Sickle Cell Disorders tested for and diagnosed?

Testing and diagnosis

A blood test can tell you whether you have a Sickle Cell Disorder or are a healthy carrier of Sickle Cell trait.

Routine screening should take place for all pregnant women, all newborn babies, and before anaesthesia, either at hospital or dental clinics.

Tests for pregnant women are offered by midwives before 10 weeks of pregnancy.

A test is offered to the baby’s father if the woman is found to be a carrier for sickle cell disease.

If both parents are carriers of sickle cell disorder the unborn child can be tested from 11 weeks of pregnancy to see if it has a sickle cell disorder. 

Tests can also be arranged by your doctor / general practitioner or at a local sickle cell centre.

After a blood test you may be told that you or your child is `sickle positive’ or has `sickle cell’. It is important to ask whether this means sickle cell trait or a type of sickle cell disorder.

If a child has a “trait” you will get a letter that explains more. 

If the result has come back as positive the Sickle Cell nurse will contact the family to discuss treatment and support and to provide further information. 


 

GP assessments

If GP has concerns and wants to refer children or young people for further investigation there are two options.

  • If the child is less critically ill, they are referred to the paediatric team at the Homerton Hospital.

Note: There is no specialist children’s sickle cell service at the Homerton Hospital.

  • Children and young people with more complicated issues and need for specific Sickle Cell treatment are referred to the paediatric haematologist via Accident and Emergency (A&E) at the Royal London Hospital.

Patients needing specialist management will be transferred to the Royal London Hospital.

Treatment, management and support for people with Sickle Cell...

Management and support of Sickle Cell

Management and support of Sickle Cell is overseen by the consultant haematology team at the Royal London and Homerton Hospitals.

Young People aged 16 and over receive support and treatment at Homerton Hospital.

Children and young people aged 0-16 receive support and treatment at Royal London (Homerton A & E )

The specialist Sickle Cell nurses work between both hospitals.

Find out more:

Homerton Hospital – Sickle cell and Thalassaemia service

City and Hackney Paediatric Sickle Cell Illness Pathway


Outside of a crisis, patients are seen at regular intervals a similar way to an annual review in the Paediatric haematology Department of the Royal London Hospital

 


Acute painful crisis

If a patient is in an acute painful crisis, they usually take pain relief medication at home. 

If the pain does not settle they can come to the local A&E department where they are assessed for triggers like infection or dehydration.  Stronger pain relief medication will be given at the hospital.

Patients over 16 in a crisis and who are receiving their care at the Homerton can attend Medical Day Unit as part of a drop in service. 

The Medical Day Unit opening hours at Homerton Hospital are

Monday -Friday 09:00am -6:00pm (excluding bank holidays) 

You will be treated and assessed as if you were in A&E.

 


Chronic disease management

Chronic disease management is carried out through an Annual Review that includes:

  • a Healthcare and disease activity review
  • mental health screening
  • medication review
  • lifestyle advice and
  • immunisations

Blood Transfusions

Blood Transfusions are offered as treatments for patient diagnosed with Beta Thalassaemia  Major (different from Sickle Cell).  This is also a haemoglobin disorder where you have the genes to make your red blood cells but not the genes needed to keep your blood alive.  This results in needing blood transfusions treatments every 2-4 weeks for the rest of your life and is managed by a specialist team.

Currently there is a need for more people to donate blood. NHS Blood and Transplant (NHSBT) wants to recruit 7,000 new black donors by 2020 – and currently half the country’s black population lives in the capital.  Donations of rare blood types, which are more common among black communities, are particularly needed. 

Theo Clarke, NHS Blood and Transplant National BAME Marketing Manager said: “We need more black donors as some rare blood types are more common among black communities. Donations are used to treat blood disorders, such as sickle cell anaemia, which are more likely to affect the black community. Sickle patients ideally need blood from a similar ethnic background. "

“We’re incredibly grateful to those already donating, but it’s vital we encourage more black donors, particularly in London where such a large black population lives. And we need a new generation of donors to give blood and help save lives. If you’ve not given then why not start, and if you do give then please keep up the good work.

Book an appointment or find out more...

Phone: 0300 123 23 23

Web:  www.blood.co.uk 

Support for young people...


Sickle Cell and Thalassaemia Service

The Sickle Cell and Thalassaemia Service is a multidisciplinary team providing specialist services to people living City of London and Hackney. Services include:

  • counselling
  • testing
  • advice and support to individuals with or at risk of having Sickle Cell, Thalassaemia or other red blood cell disorders 
  • advice and support to parents/carers

Find out more: Sickle Cell and Thalassaemia Service

Lead Nurse

Natasha Lewis

Homerton University Hospital NHS Foundation Trust

Sickle Cell & Thalassemia Centre

457 Queensbridge Road

London E8 3AS

 

Phone: 0207 683 4570

Fax: 0207 254 3364

e-mail: natashalewis1@nhs.net


Psychology Support

Our psychology team can support people living with sickle cell disease & thalassaemia and help to promote well-being and improve quality of life. Psychologists are trained healthcare professionals who help people manage their emotional concerns over a specified period of time. They do not prescribe medication.

The psychologists can provide support and help with a range of problems, such as:

  • Coping with pain at hospital and at home
  • Adjusting to being in hospital or having to come into hospital frequently
  • Managing other life problems, which affect how you cope with your illness
  • Dealing with worries about your future
  • Managing family and relationship problems
  • Improving your self-confidence
  • Coping with memory and concentration problems
  • Dealing with a fear of needles or blood transfusions
  • Improving communication with nurses, doctors and other hospital staff

Referral - You can self-refer or you can speak directly to the psychologist on the ward. Alternatively, you can talk to your doctor or nurse about a referral.

Phone: 020 8510 7202

e-mail: huh-tr.homertonsicklepsychology@nhs.net


 

Solace Sickle Cell patient support group

Solace are a Sickle cell patient support group that meet monthly (3rd Tuesday of the month, 17:30-19:30) at the Matthews Duncan Seminar Room at Homerton Hospital.

Solace run events, offer support and practical advice on pain management and other related topics. Keep informed by signing up for their newsletter on their website.  

Dalston Practice, Madinah Road.

Web: http://solacesg.org.uk/


The Huddleston Centre

The Huddleston Centre provides a fun and creative safe space for young disabled people in Hackney.

Making friends providing support:

Regular weekly groups provide a variety of social opportunities and fun activities:

  • Immediate Theatre - Mondays
  • HenPower - Tuesdays
  • Cool Gang Club – Tuesdays (for those aged 9-15years)
  • Access Project - Wednesday
  • Social Club - Fridays (for those aged 16-25years)

Find out more: Huddleston Centre


Targeted Health Outreach Team

Targeted Health Outreach Team run services for young people who have additional needs e.g. Learning Disabilities, autistic spectrum condition including Aspergers or those with physical disabilities. 

They specifically work with:

  • those who have no input from social work services. 
  • are aged 14 - 19
  • have special educational needs (SEN) or a disability
  • would like some support around developing their independence and improving their health wellbeing

Find out more: Targeted Health Outreach Team

Support for parents and carers...

Sickle Cell and Thalassaemia Service

The Sickle Cell and Thalassaemia Service is a multidisciplinary team providing specialist services to people living City of London and Hackney. Services include:

  • counselling
  • testing
  • advice and support to individuals with or at risk of having Sickle Cell, Thalassaemia or other red blood cell disorders 
  • advice and support to parents/carers

Find out more: Sickle Cell and Thalassaemia Service

Lead Nurse

Natasha Lewis

Homerton University Hospital NHS Foundation Trust

Sickle Cell & Thalassemia Centre

457 Queensbridge Road

London E8 3AS

 

Phone: 0207 683 4570

Fax: 0207 254 3364

e-mail: natashalewis1@nhs.net


Psychology Support

Our psychology team can support people living with sickle cell disease & thalassaemia and help to promote well-being and improve quality of life. Psychologists are trained healthcare professionals who help people manage their emotional concerns over a specified period of time. They do not prescribe medication.

The psychologists can provide support and help with a range of problems, such as:

  • Coping with pain at hospital and at home
  • Adjusting to being in hospital or having to come into hospital frequently
  • Managing other life problems, which affect how you cope with your illness
  • Dealing with worries about your future
  • Managing family and relationship problems
  • Improving your self-confidence
  • Coping with memory and concentration problems
  • Dealing with a fear of needles or blood transfusions
  • Improving communication with nurses, doctors and other hospital staff

Referral - You can self-refer or you can speak directly to the psychologist on the ward. Alternatively, you can talk to your doctor or nurse about a referral.

Phone: 020 8510 7202

e-mail: huh-tr.homertonsicklepsychology@nhs.net


Hackney Carers Coordination Service

Hackney Carers Coordination Service is a service is for unpaid carers (a person looking after a child or adult who is ill, disabled, has a mental illness or has substance misuse issues). Access to this service includes parents/carers in receipt of Carers Allowance as this is a benefit and does not mean you are a paid carer.

The Hackney Carers Coordination Service can:

  • Register you as a parent carer
  • Give you a carers cards (cards which give discounts for shops and leisure centres for carers)
  • Run information and training opportunities for carers e.g. First Aid, Moving and Handling courses, information sessions about adult services and personal budgets
  • Send you a quarterly newsletter
  • Give you to an invitation to Carer’s Rights Days & Carers Conference events,
  • Make referrals to organisations within the Carers are the Bedrock partnership who can then provide services e.g. benefits advice, counselling, support groups

Find out more: Hackney Carers Coordination Service


Disabled Children’s Service

Hackney’s Disabled Children’s Service provide social care support for families with disabled children.

A Child and Family Needs Assessment to see what support the family needs could lead to a support package in the form of Direct Payments, or through an approved care agency. Support through a care package is for:

Hackney's Disabled Children's Service does not accept new referrals themselves. All referrals come through Hackney First Access Screening Team (FAST)

Phone: 0208 356 5500

e-mail: Fast@hackney.gov.uk

 


Solace Sickle Cell patient support group

Solace are a Sickle cell patient support group that meet monthly (3rd Tuesday of the month, 17:30-19:30) at the Matthews Duncan Seminar Room at Homerton Hospital.

Solace run events, offer support and practical advice on pain management and other related topics. Keep informed by signing up for their newsletter on their website.  

Dalston Practice, Madinah Road.

Web: http://solacesg.org.uk/


 

City & Hackney Carers Centre

City & Hackney Carers Centre is a service is for unpaid carers (a person looking after a child or adult who is ill, disabled, has a mental illness or has substance misuse issues).The person being cared for must live in Hackney or the City of London. The Centre works closely with The Carers Coordination Service.

Find out more: City & Hackney Carers Centre

 


As1 - parents support group

As1 (as one) are a parent led support group for parents and carers of children, young people and young adults who have special educational needs and or disability (SEND). As1 offers a community of like-minded individuals with shared experiences who provide friendship, support and understanding for one another.

Find out more: As1 – parent support group


 

Hackney Independent Forum for Parents/Carers of Children with Disabilities (HiP)

HiP is the official forum for Hackney parents/carers, run by parents and carers for anyone whose child (0-25) has any kind of disability, SEN or additional needs.

HiP is part of a wider network of parent carer forums known as The National Network of Parents Carer Forums (NNPCF). A network of local forums who meet in local and regional settings and who work together to effect local and national service improvement through participation and coproduction with parent carers.

Find out more: Hackney Independent Forum for Parents/Carers of Children with Disabilities (HiP)

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