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An overview of sickle cell disease

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Sickle cell is a disorder of the haemoglobin in the red blood cells.

Haemoglobin is the substance in red blood cells that is responsible for the colour of the cell and for carrying oxygen around the body.

People with sickle cell disorder are born with the condition, it is not contagious. It can only be inherited from both parents each having passed on the gene for sickle cell.

The main symptoms of sickle cell disorder are anaemia and episodes of severe pain. The pain occurs when the cells change shape after oxygen has been released. The red blood cells then stick together, causing blockages in the small blood vessels.

These painful episodes are referred to as sickle cell crisis. They are treated with strong painkillers such as morphine to control the pain.

People with sickle cell are at risk of complications stroke, acute chest syndrome, blindness, bone damage and priapism (a persistent, painful erection of the penis).

Over time people with sickle cell can experience damage to organs such as the liver, kidney, lungs, heart and spleen. Death can also result from complications of the disorder. Treatment of sickle cell mostly focuses on preventing and treating complications.

The only possible cure for the disorder is bone marrow transplant but this is only possible for a limited number of affected individuals who have a suitable donor. A medicine called Hydroxyurea, can significantly reduce the number of painful crises.

1 SCD is inherited from both parents; sickle cell trait is inherited from one parent.

2 SCD can affect anyone, although it predominantly affects people from African and Caribbean backgrounds.

3 1 in 76 babies born in the UK carry sickle cell trait.

4 Approximately 15,000 people in the UK have sickle cell disorder.

5 Approximately 350 babies with SCD are born in the UK every year.

6 A simple blood test will tell whether you have sickle cell trait or the disorder

7 Children with SCD are at increased risk for stroke, the risk is highest between the ages of 2 and 16.

8 Episodes of pain may occur in sickle cell disorder and are generally referred to as a crisis

The Sickle Cell Trait

You are born with sickle cell trait. It is inherited when only one of your parents has passed on the sickle gene, and will never develop into sickle cell disorder.

You do not have symptoms from sickle cell trait, so it is a good idea to have a blood test to see if you have sickle cell trait.

If you have the trait, the majority of red cells in the blood are normal round shaped cells. Some sickle shaped cells may be present under certain conditions.

Sickle cell trait is found in 1 in 4 West Africans and 1 in 10 Afro-Caribbeans, and is also found in people who originate from the Mediterranean, Asia and the Middle East. It is less common in white European's, although with the ever growing diversity of the population this will change.

Most people who have sickle cell trait are healthy. However, anaesthetics can cause problems. If you have sickle cell trait Always notify your dentist or doctor before treatment commences to be on the safe side.

There is a small chance that you may experience pain at high altitudes (generally above 10,000 feet), including long-haul flying in unpressurised planes and mountain climbing. It is important you say you have sickle cell trait before undertaking such activities as you may need to breathe oxygen. Extreme exercise may also precipitate problems and if you are a professional athlete you should have a training programme that takes account of this.

The trait is not an illness, but if you are planning to have children, then certain factors have to be considered.

If your partner does not have sickle cell trait, then any children you have will not have sickle cell disorder, but they could have the trait (50% chance).

If you and your partner both have the trait, there is a 25% chance that any child conceived may have sickle cell disorder and 50% chance they will have the trait.

What to do next?

If you want to know your sickle cell status you can ask your GP for a blood test.

In some parts of the country there are also local sickle cell centres that can arrange a blood test for you.

You may have been screened for sickle cell disease. Screening is offered .

To all newborn babies as part of the newborn bloodspot (heelprick test) when your baby is five days old. The key reason for offering newborn screening for sickle cell disease is because babies with sickle cell disease are vulnerable to serious infections. By identifying babies early in life , they can be prescribed penicillin and be referred for specialist care, so that they stay healthy.

Newborn screening also detects babies who have the trait (also known as a carrier) for sickle cell disease.

To all pregnant women early in pregnancy (ideally by ten weeks) . Antenatal screening identifies parents to be who have the trait (also known as a carrier). If the mother is identified with the trait, the baby's father is offered a screening test.

There are several treatments available to help manage sickle cell (HbSS) and prevent or treat its symptoms. Your GP, hospital doctor or specialist nurse will recommend appropriate treatments for you or your child. Their recommendations may include:

Penicillin (or erythromycin in the case of allergy) is prescribed to all infants from the age of 3 months to prevent overwhelming pneumococcal sepsis. [1,2] Other infections, for example chest infection or osteomyelitis (infection of the bone) are treated with the appropriate antibiotic.

All infants should receive immunisations according to the universal schedule. In addition, those with sickle cell disease should receive extra vaccinations against Meningitis (Men ACWY) and Pneumococcus (PPV). The latter is given 5 yearly throughout life.

Mild to moderate painful episodes may be managed with over-the-counter painkillers like ibuprofen and paracetamol which should be taken regularly until the episode is over. It is sometimes necessary to go to hospital for severe pain as stronger painkillers such as opiates may be needed.

Blood transfusions are used in the management of sickle cell disease in different ways. In the case of a sudden drop in haemoglobin, such as acute splenic sequestration or aplasia due to parvovirus infection, a simple top-up of non-sickle cell blood is given. In acute emergencies, such as stroke or acute chest syndrome, an exchange blood transfusion is needed to reduce the amount of sickle cells in the blood. Regular, usually monthly, blood transfusions are indicated in the long-term management of stroke or if there is no response to other medications such as hydroxycarbamide (see below). Blood transfusions come from donated blood, which is collected, carefully tested for infections such as hepatitis and HIV, and then closely matched to the recipient to prevent reactions.

Also known as hydroxyurea, hydroxycarbamide increases the amount of foetal haemoglobin (HbF) in the blood and reduces the frequency of painful episodes and acute chest syndrome.[3] It is important to remember that hydroxycarbamide must be taken every day for it to work well and regular blood tests are necessary both to monitor benefits and possible side effects. It is now recommended that hydroxycarbamide should be discussed with parents of all children with sickle cell disease from the age of 9 months in order to prevent the onset of long-term complications., improve quality of life and maintain healthy growth.[4] However, while conversations should start from the age of 9 months, hydroxycarbamide is only licensed for use in children over the age of 2 years.

The mortality in childhood sickle cell disease has fallen from 10% in the 1980's to <1% now. This is almost entirely due to new-born screening, early registration into care and penicillin prophylaxis. Advice and support to parents is also crucial in insuring early recognition of symptoms and good access to medical care. The morbidity from stroke has reduced significantly with the introduction of routine transcranial Doppler screening from the age of 2 years. The adoption of the British Society of Haematology guideline to discuss with parents the early use of hydroxycarbamide and starting treatment from the age of 2 years, should improve quality of life further and prevent long- term complications before they develop.

For further information about the treatment and management of sickle cell, speak to a healthcare professional.

The following standards and guidelines are also available

Standards for the Clinical care of Adults with Sickle cell disease in the UK https://www.sicklecellsociety.org/wp-content/uploads/2018/05/Standards-for-the-Clinical-Care-of-Adults-with-Sickle-Cell-in-the-UK-2018.pdf Sickle Cell Disease in Childhood: Standards and recommendations for Clinical Care https://www.sicklecellsociety.org/wp-content/uploads/2019/11/SCD-in-Childhood_Final-version-1.pdf

1. Prevention of infection 1 Oligbu G, Collins S, Sheppard C, et al. Risk of invasive pneumococcal disease in children with sickle cell disease in England: a national observational cohort study, 2010-2015. Arch Dis Child 2018; 103: 643-7.

2. Gaston MH, Verter JI, Woods G, et al. Prophylaxis with oral penicillin in children with sickle cell anemia. N Engl J Med 1986; 314: 1593-9.

3. Rees DC. The rationale for using hydroxycarbamide in the treatment of sickle cell disease. Haematologica. 2011 Apr;96(4):488-91.

4. Qureshi A, Kaya B, Pancham S, et al. Guidelines for the use of hydroxycarbamide in children and adults with sickle cell disease: A British Society for Haematology Guideline. Br J Haematol 2018; 181: 460-75.

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Haemoglobin is the substance in red blood cells that is responsible for the colour of the cell and for carrying oxygen around the body. People with sickle cell disorder are born with the condition, it is not contagious. It can only be inherited from both parents each having passed on the gene for sickle cell.

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An inquiry into the avoidable deaths and failures of care for sickle cell patients in secondary care

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An All-Party Parliamentary Group (APPG) inquiry report published today highlights shocking failures as cross-party MPs call for major changes into care for sickle cell patients.

The groundbreaking inquiry, led by Rt Hon Pat McFadden MP, Chair of the APPG on Sickle Cell and Thalassaemia , has found "serious care failings" in acute services and evidence of attitudes underpinned by racism.

The 'No One's Listening' report, which is based on the inquiry's findings, is jointly published by the APPG on Sickle Cell and Thalassaemia and the Sickle Cell Society , a national charity that supports and represents people affected by sickle cell disorders.
Download the full report here: No One's Listening - A Report (PDF)
Support the report by writing to your MP here: www.sicklecellsociety.org/resource/write-to-your-mp/
Key findings from the inquiry include:

evidence of sub-standard care for sickle cell patients admitted to general wards or attending Accident & Emergency (A&E) departments (including a widespread lack of adherence to national care standards)

low awareness of sickle cell among healthcare professionals and clear examples of inadequate training and insufficient investment in sickle cell care

frequent reports of negative attitudes towards sickle cell patients and a weight of the evidence suggests that such attitudes are often underpinned by racism

The inquiry also found that these concerns have led to a fear and avoidance of hospitals for many people living with sickle cell.

While the inquiry did find that specialist haemoglobinopathy services are generally felt to be of a good standard, the report shows that this is far from the case on general wards or A&E departments. Care failings have led to patient deaths and 'near misses' are not uncommon. The inquiry heard that awareness of sickle cell among healthcare professionals is low, with sickle cell patients regularly having to educate healthcare professionals about the basics of their condition at times of significant pain and distress and that there is routine failure to comply with national care standards around pain relief when patients attend A&E.

Among the most notable of cases reviewed by the APPG was the tragic death of sickle cell patient Evan Nathan Smith in North Middlesex University Hospital in April 2019, which received renewed focus following the publication of the coroner's inquest in April 2021. The inquest found that Evan's death would not have happened were it not for failures in the care he received. The report highlights that high-profile cases of failings like these are sadly not isolated incidents and sickle cell patients all too often receive sub-standard care.

Worrying evidence of deep racial inequality in the healthcare system was found as part of the inquiry process. Sickle cell is a condition that predominantly affects people with African or Caribbean heritage and patients often reported being treated with disrespect, not being believed or listened to, and not being treated as a priority by healthcare professionals even during cases where urgent medical care was needed. Some patients shared examples of particularly overt racism. One patient said he has "had to deal with doctors and nurses openly being racist towards me and others ... I've been called the 'n' word to my face and much worse".

The report includes a number of recommendations and the APPG is calling on the Secretary of State for Health and Social Care, NHS England and NHS Improvement to prioritise taking urgent action.
Rt Hon Pat McFadden, Labour MP for Wolverhampton South East and Chair of the APPG for Sickle Cell and Thalassaemia, John James, CEO of the Sickle Cell Society, added: said:

"This is the most comprehensive report on sickle cell care that our All-Party Group has ever published. It has shown us that whilst people living with sickle cell generally had high levels of confidence in specialist haematology departments, there was a big gulf between this and the experience of A&E departments and generalist wards.

One of the key findings in the report was the lack of communication between these two areas when treating people with sickle cell, this, along with tragic and avoidable deaths , has caused a serious and damaging impact on the levels of trust in the health system among sickle cell patients.

In preparing this report we took extensive oral and written evidence from sickle cell patients, clinicians and others. The report calls for a comprehensive transformation in the care of people living with sickle cell. It also reflects the frustration that many of these arguments have been made for a long time without a proper response.

I hope this report is taken seriously by the Department of Health and Social Care and by the NHS. This time, we want people to listen because the inequalities in health care highlighted in our report and the inconsistencies in treatment for people living with sickle cell have been going on for far too long."

Download the full report here: No One's Listening - A Report (PDF)

"The APPG inquiry's findings are appalling but will sadly not come as a surprise to many people who have found themselves in need of urgent medical help for the symptoms of sickle cell.

I'd personally like to thank everyone who has given evidence and shared their experiences as part of this inquiry and particularly acknowledge the extraordinary bravery of the parents of Evan Nathan Smith who continue to fight so hard to prevent other families suffering from the loss of a loved one in such tragic and avoidable circumstances.

This report is an urgent call to action and should be a turning point that addresses these serious failures of care and secures long overdue improvements."

The inquiry is the first of its kind as it featured oral evidence sessions with testimony from expert witnesses including sickle cell patients, patients' carers and family members, clinicians and representatives from relevant healthcare bodies and over 100 submissions of written evidence from key stakeholders.

Support the report by writing to your MP here: www.sicklecellsociety.org/resource/write-to-your-mp/

Westminster Hall Debate

On Wednesday 8th December, the Rt Hon Pat McFadden MP, Chair of the All-Party Parliamentary Group for Sickle Cell and Thalassaemia (SCTAPPG), secured a Westminster Hall Debate to discuss the Treatment of Sickle Cell following this inquiry report.

The debate saw MPs discuss the findings and recommendations of the inquiry report, personal testimony from MPs with experience of family members and constituents with sickle cell, and a discussion of plans to make improvements.

Watch the full debate here:

No One's Listening Explained (Event)

On Monday 17th January our Give Blood Spread Love project hosted a free online event 'No One's Listening Report' Explained.
Notes to editors: About the inquiry and the 'No one's listening' report:
People from across the UK joined to hear from Seyi Afolabi, our Parliamentary & Policy officer for SCTAPPG, who presented on all you need to know about our inquiry and how you can support, followed by an open opportunity for questions.
About the All-Party Parliamentary Group on Sickle Cell and Thalassaemia (SCTAPPG):
Watch the full event here:
About the Sickle Cell Society:

Following the publication of the Coroner's report earlier this year, the APPG held three evidence sessions, hearing from patients, clinicians and politicians. The APPG took evidence from a wide range of witnesses and received over a hundred written submissions. The report is a result of that evidence.

The inquiry, chaired by the All-Party Parliamentary Group on Sickle Cell and Thalassaemia (SCTAPPG) Chair Rt Hon Pat McFadden MP, featured three oral evidence sessions held in June 2021, with SCTAPPG members receiving testimony from expert witnesses including sickle cell patients, patients' carers and family members, clinicians and representatives from relevant healthcare bodies. In addition, the SCTAPPG issued a call for written evidence which resulted in the receipt of over 100 submissions from key stakeholders.

The report includes a number of recommendations based on the evidence received and calls on the Secretary of State for Health and Social Care, NHS England & NHS Improvement to prioritise taking action.

About Sickle Cell Disorder:

Cross party members of the SCTAPPG are: Chair Pat McFadden (Labour), Vice Chair Janet Daby, (Labour), Dawn Butler (Labour), Baroness Benjamin (Liberal Democrat) and Bambos Charalambous (Labour). The late Sir David Amess (Conservative) was also a serving member.

Sickle Cell Society acts as the group's secretariat.

The Sickle Cell Society is the only national charity in the UK that supports and represents people affected by a sickle cell disorder to improve their overall quality of life. First set up as a registered charity in 1979, the Sickle Cell Society has been working alongside health care professionals, parents, and people living with sickle cell to raise awareness of the disorder. The Society's aim is to support those living with sickle cell, empowering them to achieve their full potential.

Email: info@sicklecellsociety.org

Sickle cell disorder is a long term inherited condition that causes excruciating pain, can be life threatening, causes multiple problems such as specific severe infections, strokes, chronic fatigue, delayed growth and progressive tissue and organ damage.

People are often admitted to hospital when they have a sickle cell crisis, given strong painkillers such as morphine to control the pain, intravenous therapy and antibiotics. Some require regular blood transfusions to help reduce the number of sickle cell crises as well as prevent life-threatening problems.

SCD is inherited from both parents; sickle cell trait is inherited from one parent.

SCD can affect anyone, although it predominantly affects people from African and Caribbean backgrounds.

Approximately 1 in 79 babies born in the UK carry sickle cell trait.

Approximately 15,000 people in the UK have sickle cell disorder.

Almost 300 babies are born in the UK with sickle cell each year.

A simple blood test will tell whether you have sickle cell trait or the disorder.

Children with SCD are at increased risk for stroke, the risk is highest between the ages of 2 and 16.

Episodes of pain may occur in sickle cell disorder and are generally referred to as a crisis.

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No One's Listening - A Report " Sickle Cell Society

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An All-Party Parliamentary Group (APPG) inquiry report published today highlights shocking failures as cross-party MPs call for major changes into care for sickle cell patients. The groundbreaking inquiry, led by Rt Hon Pat McFadden MP, Chair of the APPG on Sickle Cell and Thalassaemia , has found "serious care failings" in acute services and evidence of attitudes underpinned by racism.

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You will have to register for free to access the module for free

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This programme is in partnership with...

This programme is in partnership with...

About the programme

This unique interactive course will give you an understanding of the pathophysiology of sickle cell disorder, its treatment models and offers insight to the impact of sickle cell disorder on daily life for patients. It will also explain the importance of red cell depletion exchange as a treatment option and where you can access further resources.
Primary and secondary audiences
This module is aimed at healthcare professionals working within haemoglobinopathies specialities

How to access

In order to access any elfh programme, you will need an elfh account. If you do not have one, then you can register by selecting the Register button below.
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To view the ARCEx for Sickle Cell Disorder programme, select the View button below. If you already have an account with elfh, you will also be able to login and enrol on the programme from the View button.
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If you already have an account with elfh, then you can enrol on to the ARCEx for Sickle Cell Disorder programme by logging in to the elfh Hub, selecting My Account > Enrolment and selecting the programme. You can then access the programme immediately in the My e-Learning section.

NHS healthcare staff in England - ESR

The ARCEx for Sickle Cell Disorder programme is also available to NHS healthcare staff via the Electronic Staff Record (ESR). Accessing this e-learning via ESR means that your completions will transfer with you throughout your NHS career.

Further details are available here.

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If you are not an NHS health or care organisation and therefore do not qualify for free access elfh Hub, you may be able to access the service by creating an OpenAthens account.

To check whether or not you qualify for free access via OpenAthens, you can view the eligibility criteria and register on the ' OpenAthens ' portal.

Registering large numbers of users

If you are a HR, IT or Practice Manager and would like to register and enrol large numbers of staff within your organisation for access onto the ARCEx for Sickle Cell Disorder programme, please contact elfh directly.

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For HR departments wanting to know more about gaining access to courses using an existing Learning Management System please contact elfh directly to express interest.

More information

Please select the following link for more information on how to use the elfh Hub.

elfh is a Health Education England programme in partnership with the NHS and professional bodies

© elearning for healthcare 2022

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ARCEx for Sickle Cell Disorder - elearning for healthcare

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This programme is in partnership with... This unique interactive course will give you an understanding of the pathophysiology of sickle cell disorder, its treatment models and offers insight to the impact of sickle cell disorder on daily life for patients.

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Living with a bleeding disorder is never black and white. And it's not all blood-red either. Life after diagnosis may start out hard, but we learn by doing, making mistakes, asking questions and surrounding ourselves with people who understand.

Then, slowly, it just becomes life again. Because a bleeding disorder shouldn't define who we are, what we do or how we feel.

Through our community, we find the freedom, opportunity and fun in life after diagnosis.

Newly Diagnosed

Diagnosis & Treatment

Day-to-day Living

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The Haemophilia Society - Together For Life - The Haemophilia Society

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Living with a bleeding disorder is never black and white. And it's not all blood-red either. Life after diagnosis may start out hard, but we learn by doing, making mistakes, asking questions and surrounding ourselves with people who understand. Then, slowly, it just becomes life again.

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Patient Pathway

Introduction: Paediatric Haematology Team

Acute Management of Sickle Cell Disease

Management of Thalassaemia (Acute & Chronic)

Chelation Guideline and Iron Overload

Ferriscan Referral Form

Transfusion and Exchange

Surgery and Anaesthesia in Sickle Cell Disease

Hydroxycarbamide in Sickle Cell Disease

Chronic Complications in Sickle-cell Disease

Long-Term Management and Followup

Transition

Treatments

Referral, MDT and Workup

Trials

Links

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OUH Transfusion Website

Sickle Cell Disease Guidance

Sickle Cell Disease Reports

Thalassaemia Guidance

Patient Information

Patient Videos

Education

GP Haemoglobinopathy Information

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The Paediatric Haemoglobinopathies page for Thames Valley Cancer Network Haematology & OUH Department of Clinical Haematology

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1. We want patients with rare inherited anaemias to be able to meet each other (virtually or in reality) and help and support each other. Find us on Facebook

2. We want to give out reliable information about rare inherited anaemias and get your questions answered by doctors in the field. Ask us a question about one of these conditions on Facebook or by email, and we'll answer it.

3. We want to raise money to fund research so we can better understand these conditions and improve the diagnosis and treatments available. Help us fundraise - contact info@togetherwecan.uk.

CAN is a registered charity in England and Wales (5105120).

Have you or your child been diagnosed with a rare inherited anaemia? You can read more about:

Are you or your child receiving blood transfusions regularly but you have been told it has not been possible to make a diagnosis about the condition?
Are you worried you or your child might have a rare inherited anaemia but you have not had any tests yet from blood specialists?
Please contact us on info@togetherwecan.uk for help and advice.

Anaemia is not having enough red blood cells. Red blood cells carry oxygen from the lungs to the rest of the body. Not enough red blood cells (anaemia) makes people feel tired, weak and short of breath. There are dozens of causes of anaemia. Some people worry that anaemia is like leukaemia. Leukaemia is a cancer of the white blood cells. Some patients with leukaemia do get anaemia. But if you are told you or your child has anaemia it does not mean they definitely have leukaemia.Learn more >>

Inherited anaemias are ones which are carried in the genes and can be dominant (the mutated gene is carried in one parent and causes anaemia), recessive (each parent carries a mutated gene and only when they are found together in the child does the anaemia happen) or de novo (neither parent carries the mutation but it has arisen by chance in the developing fetus) These can either occur on their own or in the context of bone marrow failure.

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We are a group of patients, carers, doctors, and scientists who are all interested in patients with rare inherited anaemia.

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We want patients with rare inherited anaemias to be able to meet each other (virtually or in reality) and help and support each other. Find us on Facebook We want to give out reliable information about rare inherited anaemias and get your questions answered by doctors in the field.

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An overview of Thalassaemia

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Thalassaemia forms part of the haemoglobinopathies, a group of genetic blood disorders affecting the production of haemoglobin, when the body makes less haemoglobin than normal.

Blood is a red fluid freely flowing in the body, it is essential for maintaining the health and life of the human body. Blood is a mixture of a liquid called plasma about 55% and solids 45% blood cells (red blood cells, white blood cells and platelets). Blood has various important functions such as, supplying oxygen to cells and tissues, providing essential nutrients to cells, such as glucose, removing waste materials, such as carbon dioxide, protecting the body from diseases and infections, regulating body temperature. Any abnormalities, disorders and diseases of the blood can damage the many functions that the blood performs. There are various blood disorders, such as blood clotting, blood cancers, sickle cell anaemia and thalassaemia.

People with thalassaemia produce either no or too little haemoglobin. Haemoglobin is an iron-rich protein in red blood cells. It carries oxygen to all parts of the body. Haemoglobin is made up of four subunits: two subunits of alpha-globin and two subunits of beta-globin. Alpha-globin is a component of both fetal haemoglobin, which is active only before birth and in the new-born period, and adult haemoglobin, which is active throughout the rest of life.

Thalassaemia is inherited from parents; it is not contagious, or a virus acquired from blood transfusions. It is passed on equally by men and women.

How is thalassaemia inherited?

Thalassaemia is passed from parents to children through genes. A gene is a small packet of information that controls a characteristic in your body, by making very specialised protein molecules.

It is a section of DNA, the unique molecule that makes up your chromosomes. Genes make us who we are; they determine our characteristics, for example, the colour of our hair, our height and so on.

There are two copies of a gene in each cell. Each copy is called an allele: for everything we inherit, we get one allele from our mother and one allele from our father. In some cases, a gene can be missing, defective or altered from the usual form. This can cause disease.

In the case of a person with beta thalassaemia major, they have inherited two altered ß-globin genes from their parents (one from the mother and one from the father).

As a result of the altered - sometimes termed 'mutated' genes, the person cannot produce enough ß-globin, which forms part of haemoglobin. This is the substance in the red blood cell that carries oxygen.

Illustrated example:

Aisha and Hassan each have one gene that makes the usual amount of red blood cells and one altered or 'thalassaemia' gene that makes less red blood cells than usual. Aisha and Hassan are thalassaemia carriers. Being a carrier is sometimes also referred to as having thalassaemia trait or thalassaemia minor.

People who are carriers are well in themselves. They are healthy and in fact they may not even know they are carriers. But they can pass on the altered gene to their children.

A child can only get beta thalassaemia major if they get TWO altered genes - one from their father and one from their mother.

Each time Aisha and Hassan have a baby, there is

A: - 1 in 4 (25%) chance that their baby will inherit beta thalassaemia major.

B:1 in 2 (50%) chance that their baby will be a thalassaemia carrier.

C:1 in 4 (25%) chance that their baby will be completely free from thalassaemia.

These chances are the same each time Aisha and Hassan have a baby. Their next child could have beta thalassaemia major like their child Imran, be a carrier or be completely free from thalassaemia.

Due to the autosomal recessive pattern of inheritance, a person living with thalassaemia trait may pass the condition on to their children. If both parents are living with thalassaemia trait, there is a risk that their children will be carriers for thalassaemia or have thalassaemia major.

How prevalent is thalassaemia?

It used to be the case that thalassaemia was confined to certain geographic regions. This is because it initially developed as an evolutionary response to malaria; thus, it mainly affected people from malarial regions such as Asia and South East Asia, the Mediterranean, South America, the Caribbean, Northern and Central and the Middle East.

Map 1: Highlighting regions of the world where thalassaemia is most prevalent

Copyright UKTS

If a person was a thalassaemia carrier, it appeared to give them some protection against malaria. The human body had developed an effective evolutionary defence but had not anticipated what might happen if two carriers had children. This is where the issue arose, in the form of beta thalassaemia major.

In fact, it is estimated that 700,000 babies, severely affected by thalassaemia, are born globally each year.

Sadly, this underestimates the real number because in many poorer countries, cases are not always reported. According to a publication written by the Royal College of Obstetrics and Gynaecologists on the management of beta thalassaemia in pregnancy, (2014) it is estimated that there are 100 million people worldwide living with thalassaemia trait (asymptomatic carriers).

Whilst it was initially prevalent in the regions mentioned, this is no longer the case, and anyone can be at risk. Due to several socio-economic and demographic changes - for example, the migration of communities over the centuries and the rise of inter-racial families - thalassaemia is no longer confined to people from certain ethnicities.

This is why everyone should get tested. As said earlier, because carriers are generally healthy, you may not know if you are a carrier unless you have the test.

The prevalence of thalassaemia varies across different regions in the UK. Data from the review published the review published in May 2019 by National the Haemoglobinopathy Registry (NHR), indicates that there were 1100 people living with beta thalassaemia major in the UK, 250 people living with beta thalassaemia intermedia, 123 people living with beta thalassaemia/Hb E disease, and 266 people living with Haemoglobin H disease. Each year, around 20 - 30 couples are identified as being at high risk for having a baby with alpha thalassaemia major in the UK. In the UK, 60% of the 1100 patients living with beta thalassaemia major come from a South Asian background.

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The grey side shows the lack of education avaliable to people all around the world.
The colourful side represents the kind of future I want to see when I'm older. This section shows that education and access to school is avaliable to anyone wishing to go. This reflects a "Whole New World"as it will allow new opportunities to people who come from disadvantaged backgrounds like my family. Education shaped the success that we have today and I want that for everyone in the future too.

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I drew this picture to show the beauty of our world, which was forgotten by us because of the hardships we all went through during the last couple of years. When we are drowned by all of the bad news we hear on social media, and the personal issues in our lives, we tend to forget about all the wonders of our world, the greatest one of all being nature. The picture shows the smaller parts of nature, like the animals, flowers, and us - humans on the left, and the stunning sphere of our planet on the right. I really hope that just like me, with all the difficulties slowly retreating, many people all over the world will finally be able to sigh with relief, and let themselves look up, and see this precious planet with all of its colours.

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Title: Friends
Summary: In another world, I want to be able to understand what my dog Lucy is saying and feeling.

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I decided to do this 3D drawing to represent renewal, optimism and peace from the Coronavirus Pandemic and the unnecessary war in Ukraine. The Cherry Blossoms which was presented to United States by Japan after World War II represents hope and friendship. The boy and his dog represent enjoyment of life. The Big Ben represents time and grandness of human achievement. I am hoping my drawing overall demonstrates peace and prosperity for humanity.

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Hello, My name is IŞIKNAS, There is something we can all do for a fairer and more equal world. Every human being born into this world has the right to be happy. The more happy people there are, the better the world will be.

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I miss the old days when I can dress up and going to Disneyland with my friends and families to see my favourite princesses. During the pandemic lockdown period, everything was closed and we can only stay at home, meeting friends and families using Zoom. But I know on the other hand, the animals were having a great time. Without the human disruption, the whales can enjoy interact with each other in a quiet environment, the
penguins can explore more places around where they live and even can go fishing for twice a day. In this drawing, I wish the pandemic will end soon, and we can find a good balance and live together with the animals happily ever after.

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In a whole new world, I hope I can see happy faces without mask for everyone. I love swimming, I love to go to the beach with my family. This is my father, mother, elder brother and me. I love to see my mother and she dresses up every day. She can remove her glasses and I think she is the most beautiful girl in the world.
I love my yellow shirt. Life is simple but difficult. In the whole new world, everyone can find their beautiful lifestyle.

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Reflective explanation of Christian's submission.
'Once upon a time there was a boy called Christian and a dog. Christian was sitting by a river with lilly
pads, holding a ball...'
Christian's dream is to have a dog and take it for walks, picnics, feed it, give it bones 🙂 etc...He
modelled from plasticine his story and a picnic with green pepper, some citrus some soup etc. There
is also red teleporting machine, which helps him to teleport where he wants: to the sea, back home
etc.
This dream come true will be a whole new life in a whole new world for Christian!

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Upon completing an MSc at the London School of Economics, Neelam worked in the City as an Investment Analyst for several years before moving across to the third sector. Ever since graduating, she has worked on a range of freelance writing projects for a variety of publications.

In April 2019, Neelam assumed the role of Medical Writer / Magazine Editor for UKTS (working on a freelance basis). She is responsible for creating and producing Thalassaemia Matters, the charity's quarterly medical magazine. For each publication, this involves project planning its creation, securing stakeholder input, graphic design, writing content and final production. Neelam will research highly complex new medical treatments such as gene therapy and present the information in a way that is comprehensive and accessible to all.

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Teresa Choudhary joined the UKTS team in January 2020.

She obtained her degree in Computer Science from the University of Greenwich and has worked as an IT consultant, nationally and internationally, for a wide range of companies including British Airways, GSK and Lockheed Martin.

She is married with four children and has also been actively involved in fundraising for their schools, holding both secretary and vice chair roles for the PTA. She also manages, in her spare time, to assist with the various activities of the local Brownies and Guides.

Teresa is passionate about education and will be working with schools, colleges, universities and community groups to spread awareness of thalassaemia.

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Sophia is a Fashion Marketing graduate from the University of Leeds. She has previously worked for a luxury skincare and lifestyle company where she specialised in communications and public relations.

She is quite passionate about film photography, screenwriting, thrifted fashion and all things wellbeing. An avid yogi and advocate for meditation, pursuing a healthy lifestyle is something very important to her. Currently she is undertaking an extracurricular course teaching and practicing the core skills used in a counselling relationship.

The UK Thalassaemia Society is a charity very close to Sophia's heart and family. Honoured to be a child raised by a parent with Thalassaemia major, she has first-hand experience witnessing how the charities research impacts the lives of patients and their families.

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Hello, my name is Shaista Ismail, I am 30 years old and I live in Leeds/Bradford

Thalassaemia can be very challenging however I believe with love, support and understanding it can be regulated.

Having been involved in voluntary charity work and organising activities for the community these experiences allow me to adapt and change within my surroundings. Having studied childcare, psychology and graduating in animal management I have gained a better understanding of my illnesses and struggles, these experiences and knowledge have allowed me to help others with managing and understanding their illnesses. I highly believe in self-help but I also believe if you need help you should ask.

When I have some spare time I like to read and listen to music. I love spending time with my friends, family and animals and love to gain as much knowledge as I can.

As a patient representative please feel free to approach or message me on ambassador@ukts.org

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My name is Despo and I am mum to two wonderful children, a son and daughter. I work full time in a school in Harrow. My son was diagnosed with Thalassaemia Major in 1999 and attends Whittington Hospital.

It was a shock finding out about Thalassaemia and having to start his transfusions when he was just 5 months old. Despite this, my son has developed into a lovely, confident young man, achieving a 1 st at Cambridge University in Chemical Engineering. He plays for the local football team and is also a member of the university's team.

My husband and I are also fortunate enough to have had wonderful support from family and friends, through the tough times and the good times. I would like to pass this along by becoming an ambassador with the UKTS to help support any new parents and family members.

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Marathon Runner Robert Fletcher, 48 Husband/ Father/ Police Officer
Hello everybody! My name is Robert, I am 48 years old and originally from Yorkshire, but have lived in Leicestershire since 1998 due to work and family commitments. Currently, I work as a Police officer for Leicestershire Police.

I have been married for just over 6 years to my wonderful wife, Maria Fletcher nee Gavriel. Some of you may already know her from when she undertook the role of committee secretary for the UKTS. During her two years working for the society, she organised many successful and profitable charity events.

I am a father to my fifteen year old teenage daughter and owner of three dogs and a cat, so you can only imagine what our home is like sometimes!

Since leaving the Army way back in 1997, I have been invested in my fitness. Prior to lockdown, I had been undertaking training at Leicester Kickboxing Club. Martial Arts is one of those exclusive sports that has changed my life and helped me to continuously learn about myself and other people. For the last four years I have been a member, training in K1 Dutch kickboxing style. Running and cycling are also a favourite pastime of mine. Recently, I have proudly completed a charity event raising money for the Motor Neurone Disease Association. Partaking in the '7in7' challenge, which consists of running seven miles everyday for seven consecutive days, I ran a total of 49 miles.

One of the main reasons why I have decided to run the London Marathon for the UKTS is because of my wife Maria, who lives with thalassaemia major. Prior to meeting her, I had never heard of thalassaemia as well as being unaware of its further complications and how it impacts the day to day lives of sufferers. I have been truly inspired and privileged to see how Maria goes about maintaining some normality within her daily life. She attends many medical appointments and maintains and runs a busy home life; all with a smile and a positive can do attitude. Through Maria, I have become friends with some of the Greek Cypriot thalassaemic community. They have been my inspiration to get myself off the sofa, put my best running shoes on and get out there to raise as much money as possible because they all deserve the best quality of life. I have been truly humbled by their positivity and great sense of humour that derives through personal battles and ongoing adversity.

The only marathon I have ever ran was a half marathon back in 1991, in West Berlin. Aged 17 and a soldier. I remember my Platoon Sergeant the day before saying to me 'Fletcher, make sure you are dressed in civilian kit and parade outside the guardroom prompt and sharp Sunday Morning! Someone's dropped out through injury'. Back then, my training consisted of consuming less quantities of German beer than normal for a Saturday night.

Now it is not that easy. Simply looking at my trainers for too long will cause me to end up with blisters and break into a sweat. Anyway, it's all in the planning, so I will be starting a structured training plan in June to get marathon fit for October 3rd and do the UKTS proud.

At the moment, I run about twice a week with the distance varying from 4 to 8 miles. I also mix some kickboxing training sessions into my routine that consist of skipping, shadow boxing and finishing off with punching and kick combinations on the punch bags.

I feel very honoured and privileged to represent and raise money for the UKTS during the London Marathon. Hopefully, you are there on the day to cheer me on, it would be great to meet you all after to celebrate!

I encourage you all to donate what you can, a little really goes a long way and I will be so grateful to every one of you.

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Sree Marathon Bio 2021

Hi Everyone! My name is Sree Sarkar and I am a 35 year-old accountant who has been born and raised in London.
I am honoured to be running the London Marathon for UKTS. My sister has Thalassaemia major and I've grown up watching her deal with the challenges associated with this chronic disease. I've been fascinated at how her treatment has developed positively over the last three decades and I have seen first-hand how the advancement of treatment can change a patient's daily life. The work that the UK Thalassaemia society do is so beneficial for both patients and their families, by providing information, raising awareness and funds for innovative research and new technologies.

My running journey started when I completed the couch to 5k a few of years ago and have been running fairly regularly ever since. I have not run anything longer than a half marathon, so this will be a challenge for me but one I am really looking forward to complete. It's been on my bucket list for years! I run about 3-4 times a week, usually in a park - Burgess, Southwark and Richmond park are the ones where you are most likely to find me!

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Marathon Runner Ward Stepman, 51 Husband/ Father/ Active role in biological crop protection and bumblebee pollination.
Hello everyone! I am Ward Stepman, a 51 year old who was born and raised in Belgium. Since 1991, I have been working for different companies active in biological crop protection and bumblebee pollination, spending 14 years working for the Biobest Group N.V based in Belgium. Privileged enough to travel extensively with this work, I have visited and lived in Belgium, Canada, the UK and I am now in Austria. I'm married to Marlene and have two children, my oldest is Helene aged 19 and Arno who is 17 years old.

I have been involved in sports for most of my life but my main focus is athletics. Living in the Austrian Alps allows me to partake in trail running which is a combination of running and hiking on differing land and gradients. Previously, I have completed some long runs, having participated in several half marathons and a 28k with 1500m elevation gain. The London Marathon however, is going to be my first ever full marathon!

For me running is the perfect way to switch off from everyday stresses especially when running through woods and in the mountains. Besides running, I'm a keen gardener and photographer so I have plenty to do to fill my free time.

My colleague and friend Julien Mourrut Salesse told me about the condition his brother-in-law Ashkaan Bandoui has and the opportunity to raise money and awareness by participating in the London Marathon. This is a fantastic opportunity to participate in something as spectacular as the London Marathon and raise money to help those with thalassaemia.

Together with Julien, we have already been able to collect quite some funds but it's of course never enough to support an incredible and hardworking charity like the UKTS. To support me and the society, please donate by using the link below.

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Gina is a 61 year old proud mother of three beautiful children and sister of the late beloved Costa Kountourou who worked tirelessly for the cause. She is an Executive Assistant in the city and has worked in the Insurance industry for 10 years. Prior to this post, she worked in an Independent Girls School as School Secretary/Registrar and part time teacher in Design and Textiles. With over 15 years experience in fundraising and charitable work, Gina also has vast experience in Event planning. She is passionate about the work she does and is very much looking forward to sharing her expertise with UKTS and helping to make a difference.

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Ashkaan Bandoui is a 31-year-old thalassaemia patient, originally from Iran but living in London since he was two months old. He majored in graphic design at university but is currently working as an estate agent. He also enjoys playing the Iranian drum called the DAF which originates from Kurdistan and has been performing with a band for over nine years. Ashkaan joined the board of trustees in 2017 and has been a great addition to the team to date.

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Peter has starred in the world's most successful musicals of all time and his acting career is now in its 40 th year. Amongst numerous other roles, he was the Phantom in Sir Andrew Lloyd Weber's Phantom of the Opera. More recently in 2018, he played Ahmed Qurie in OSLO for which he was nominated for an Olivier award. He has had various TV roles but is most famous for playing Chris Theodopoulopoudos in Birds of a Feather and even today it is shown on TV. Peter is a director of The Royal Theatrical Fund and also teaches in schools, colleges and universities across the UK.

Peter has supported UKTS for over 25 years and now as a patron he is keen to hear about all aspects of the new medical research currently being undertaken. He is particularly excited about the huge advances in gene therapy and feels they offer real hope to patients with thalassaemia.

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Tonia Buxton is a Greek Cypriot television presenter and author. She is the host of the Discovery Channel Travel & Living show My Greek Kitchen and is also the author of several publications. Although extremely busy balancing her career and family life, Tonia is determined to support the charity in raising awareness of the inherited disorder.

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Adil Ray OBE is best known as the star and creator of the hit BBC1 sitcom Citizen Khan. More on his work can be found via Adil Ray OBE .

Adil is happy to support the society in its future work towards educating the public and improving treatment for patients living with thalassaemia.

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Zoe Panaretou joined the UKTS team in July 2020.

She has recently graduated and obtained her degree in Nutrition and Food Consumer Science from the University of Reading. She is now a registered Associate Nutritionist (ANutr) which is accredited by the Association of Nutrition.

Zoe is passionate about health and fitness and will be helping patients to make small adjustments to their lifestyle to improve their quality of life. In her spare time, she volunteers at another charity, specialising in eating disorders.

As part of her role as information officer, she will be helping the team to provide nutrition advice that is specific to the individual's needs and assisting raising awareness of Thalassaemia.

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Thalassaemia forms part of the haemoglobinopathies, a group of genetic blood disorders affecting the production of haemoglobin, when the body makes less haemoglobin than normal. Blood is a red fluid freely flowing in the body, it is essential for maintaining the health and life of the human body.

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Sickle Cell is the name for a group of different but similar inherited health conditions that affect hemoglobin. It is the most common and the fastest growing genetic condition in the UK. Sickle Cell gets its name because the red blood cells - which are normally round and flexible - become shaped like a crescent moon or farmer's sickle. The unusually-shaped red blood cells are rigid and they can block blood vessels. This reduces the blood flow to that part of the body resulting in excruciating pain known as a Sickle Cell crisis. This can sometimes cause damage.

Sickle Cell can cause the following symptoms:

Sickle Cell crisis

Stroke (common between the ages of 2 and 16)

Blindness

Organ failure

Pulmonary hypertension

Acute chest syndrome

Leg ulcers

Gallstones

Headaches

There are different types of Sickle Cell

These depend on the genes a baby inherits from their parents. All babies are now tested for Sickle Cell in their first week of life using a spot of blood taken from a heel-prick. There are over 1000 different types of unusual hemoglobin but the ones that are most commonly seen in the UK are hemoglobin S, hemoglobin C, hemoglobin D and beta thalassaemia. Did you know it is estimated that 1 in 7 adults of African and Caribbean descent in the UK carry the Sickle Cell gene. In the UK it is estimated that there are over 15,000 people with Sickle Cell and over 250,000 with Sickle Cell trait (Sickle Cell gene).

Things that can cause sickling of red blood cells include:

Dehydration

Infection

Excessive exercise

Organ failure

Stress and anxiety

Sudden change in temperature

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Sickle Cell is the name for a group of different but similar inherited health conditions that affect hemoglobin. It is the most common and the fastest growing genetic condition in the UK. Sickle Cell gets its name because the red blood cells - which are normally round and flexible - become shaped like a crescent moon or farmer's sickle.

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All healthcare professionals involved in the screening programme for sickle cell and thalassaemia ( SCT) are advised to keep their knowledge updated; and an e-learning resource is provided to support practitioners.

1. Normal haemoglobin

Haemoglobin ( Hb) is the substance within red blood cells which carries oxygen around the body.

Normal haemoglobin is made up of different globin (polypeptide) chains with heme molecules containing iron. The globin chains combine to make different types of haemoglobin. The structure of each globin chain in haemoglobin is genetically determined.

Normal haemoglobin (see illustration below) is called haemoglobin A and consists of:

2 alpha (α) globin chains

2 beta (β) globin chains

Adult red blood cells normally contain the following haemoglobin chain combinations:

haemoglobin A (α2β2) >95%

haemoglobin A₂ (α2δ2) 2% to 3.4%

fetal haemoglobin F (α2γ2) <1%

Laboratory tests can quantify normal haemoglobin and identify variants by their different characteristics.

2. Haemoglobinopathies: an overview

Haemoglobinopathies are a group of recessively inherited genetic conditions affecting the haemoglobin component of blood. They are caused by a genetic change (mutation) in the haemoglobin . More than 1,000 mutations have been identified that result in either haemoglobin variants or thalassaemias.

Most of these unusual genes are clinically insignificant. However, there is a genetic relevance to some haemoglobinopathies which, when combined with other variants or thalassaemias, may cause a significant clinical condition resulting in illness and potential death.

The most significant haemoglobinopathies result in either a change in the structure and quality of the haemoglobin or a reduction in the quantity of haemoglobin produced.

Haemoglobinopathies are:

not gender (x) linked

more prevalent in some parts of the world

Sickle cell disease is most common in West Africa and India. Thalassaemia major is more common in Asia and Mediterranean countries.

The likelihood of a person being a carrier of a haemoglobinopathy depends on ancestry. The type of mutation varies between ethnic groups.

It is possible to inherit mutations in both alpha and beta globin genes at the same time.

It is also possible (although rare) for an individual to have a 'de novo' haemoglobin mutation. This is a genetic mutation that is not directly inherited from parents but is present only in that individual.

See illustration above for summary of haemoglobinopathy categories. See the NHS SCT Screening Programme adult carrier information leaflets for more information.

2.1 Change in structure and quality of haemoglobin

Haemoglobinopathies, where the mutation results in a change to the structure and quality of haemoglobin, are known as haemoglobin variants. The most important of these is sickle cell, Hb S. Other haemoglobin variants which have a genetic and possibly clinical significance (in combination with another haemoglobinopathy) are:

Haemoglobin E ( Hb E) is technically a haemoglobin variant. However, Hb E also interacts with beta thalassaemia to cause a significant clinical condition, so it can also be classified in the 'thalassaemias' category.

2.2 Reduction in quantity of haemoglobin

The thalassaemias is the name for a group of related conditions where the amount of haemoglobin that the body produces is reduced, and this impacts on its oxygen carrying capacity. These usually affect either the alpha or beta globin chain. The most significant of these are beta (β) thalassaemia, delta beta (δβ) thalassaemia and alpha (α) thalassaemia.

3. Inheritance of haemoglobinopathies

The genes for haemoglobin production are inherited from both parents. Please refer to the inheritance risk table for further details.

Haemoglobin disorders such as sickle cell disease or beta thalassaemia major are recessively inherited. If one unusual beta chain gene is inherited from one parent, the individual will be a carrier of the condition but will not be affected. This is sometimes called having a trait.

Carriers of haemoglobin variants are healthy and are unaware of their status unless screened. A carrier of a haemoglobin variant will usually have approximately:

50 to 65% normal haemoglobin A

30 to 45% unusual haemoglobin (for example Hb S, Hb C or Hb D)

a small amount of haemoglobin A₂ and F

If 2 unusual beta chain genes are inherited, one from each parent, the individual will have a haemoglobin disorder. The most common clinically significant conditions are beta thalassaemia major and sickle cell disease.

It is also possible to inherit a benign haemoglobin disorder, where the individual has 2 unusual haemoglobin genes but does not require treatment for a clinically significant condition. However, these conditions are genetically relevant.

For more information about haemoglobinopathies, please refer to the:

If both parents carry a significant haemoglobinopathy, the baby is at risk of inheriting a haemoglobin disorder. There is a:

1 in 4 (25%) chance of being completely unaffected

2 in 4 (50%) chance of being a carrier

1 in 4 (25%) chance of inheriting the condition

It is important parents are aware that the risks of inheriting a haemoglobin disorder are the same in every pregnancy (see diagram above).

4. Sickle cell disease

4.1 Sickle cell carriers

Sickle cell carriers have inherited one normal haemoglobin A gene and one sickle haemoglobin gene.

These individuals should be cautioned to be careful in situations where there may be a reduction in the supply of oxygen, for example when having a general anaesthetic or during activities such as deep-sea diving or mountain climbing. However, they do not have a condition which requires treatment.

See the NHS SCT Screening Programme leaflet for sickle cell carriers.

4.2 Sickle cell disease

Sickle cell disease is a recessively inherited genetic condition of haemoglobin. It occurs when both parents pass unusual haemoglobin genes to their baby.

Sickle cell disease most commonly affects people of West African, Caribbean, Middle Eastern and Indian ancestry. However, it can affect anyone from any population.

The most common types of sickle cell disease seen in England are:

In an individual with sickle cell disease, the red blood cell becomes misshapen and rigid, resembling the shape of a sickle, when the haemoglobin is de-oxygenated (releases the oxygen to the organs). This process is called sickling and causes a wide range of clinical complications.

4.3 Prevalence of sickle cell disease in England

Sickle cell disease affects around 1,000 pregnancies per year and there are between 260 to 350 babies born with a sickle condition each year. Between April 2007 and the end of March 2017 there were 3,206 babies born with sickle cell disease in England.

The National Haemoglobinopathy Register (NHR) has a record of more than 11,000 individuals with sickle cell disease in England. However, registration is voluntary, so the actual number is likely to be higher.

4.4 Clinical characteristics of sickle cell disease

Normal red blood cells move freely in the circulation and have a life span of 120 days. De-oxygenated sickled red blood cells can get stuck and cause blockages in capillaries (small blood vessels) and have a shortened life span of approximately 20 to 30 days, sometimes less. These blockages are known as vaso-occlusive episodes and are sometimes described as a painful crisis.

A sickle cell crisis can be triggered by:

sudden changes in body temperature

dehydration

shortage of oxygen

infection

'Sickling' can result in:

intense pain

severe anaemia

tissue damage

infections

strokes, particularly in individuals with Hb SS

shortened life expectancy

Appendix 3 has further details of the clinical consequences of sickle cell disease based on specific genotypes.

5. Management of individuals with sickle cell disease

Sickle cell disease requires specialist consultant haematologist or paediatrician management. Early diagnosis is vital and screening for sickle cell disease is incorporated in the NHS newborn blood spot (NBS) screening programme in England. Children who are diagnosed with sickle cell disease at birth should be referred for specialist care by 3 months of age.

Standards have been developed for appropriate care of children and adults with sickle cell disease.

Management of individuals with sickle cell disease incorporates:

regular specialist outpatient reviews and easy, direct access to specialist medical care when unwell

prophylactic antibiotics and regular immunisations to prevent infections

consideration of hydroxycarbamide for children with sickle cell anaemia (Hb SS) over 9 months of age or if the individual has significant and frequent symptoms

care with administering general anaesthesia which should only be undertaken when full medical support is available (this also applies to dental treatment)

blood transfusions for relevant complications, for example stroke or severe life-threatening anaemia

annual transcranial doppler scans to ascertain increased risk of stroke for children and adolescents with sickle cell anaemia (Hb SS) and Sβ⁰ thalassaemia, up to the age of 16 years

Sickle cell disease can be cured by bone marrow or stem cell transplant, but the genetic profile of the individual does not change. One potential side effect of undergoing this procedure is infertility.

Much of the care required by individuals with sickle cell disease is preventative and supportive care. Families and individuals need specialist support to understand the condition and be proactive in their personal care, to recognise potential problems and try to prevent or minimise the effects of a sickle cell crisis. Preventative care includes:

eating a balanced, healthy diet

maintaining a good fluid intake

keeping warm in cold conditions

complying with recommended immunisations and prophylactic antibiotics

administering simple analgesia at the start of a sickling episode

seeking urgent medical care when required

6. Thalassaemias

Thalassaemias are usually recessively inherited genetic conditions which affect the quantity of haemoglobin produced. A change occurs in the genetic code responsible for production of either the alpha or beta globin chains that are present in normal haemoglobin A. The 4 main thalassaemia conditions that have clinical significance are:

alpha thalassaemia major, which is clinically significant to the fetus and mother

haemoglobin H disease (alpha thalassaemia 3 gene deletion), which is clinically significant after birth

beta thalassaemia major, which is clinically significant after birth

beta thalassaemia intermedia, which has variable clinical significance after birth

Further details on the clinical impact of thalassaemia conditions is in Appendix 4.

7. Alpha thalassaemia

Normal haemoglobin A has 2 alpha globin chains. The production of these alpha globin chains is controlled by 4 alpha globin genes and 2 genes are inherited from each parent. In alpha thalassaemia there is either reduced or absent production of alpha globin chains, caused by a defect or mutation in one or more of the alpha globin genes.

Alpha thalassaemia carrier status cannot be definitively diagnosed by routine laboratory screening methods, due to its complexity. It must be confirmed by DNA analysis.

Below is a graphic illustration of the alpha globin chains (2), and the full complement of alpha globin genes, (4) that individuals with normal Hb A inherit. This is usually written αα/αα in medical literature.

7.1 Alpha plus (α+) thalassaemia carrier

Individuals with alpha plus thalassaemia have inherited either one or 2 faulty alpha globin genes (-α/αα) or (-α/-α). Although this can affect alpha globin chain production, there is usually minimal change to the haemoglobin level.

Alpha plus thalassaemia carrier status is not clinically significant but can be confused with iron deficiency anaemia in the antenatal period.

Alpha plus thalassaemia is most often seen in African, Caribbean, Indian, Pakistani, Bangladeshi and Middle Eastern populations.

7.2 Alpha zero (α°) thalassaemia carrier

Alpha zero thalassaemia carriers have not inherited any alpha globin chain genes from one parent (- -/αα). The individual is generally healthy but there is a reduction in alpha globin chain production and they may have a mild anaemia with a mean cellular haemoglobin (MCH) that is usually less than 25pg. This can be confused with iron deficiency anaemia.

If a couple are both identified as potentially being alpha zero thalassaemia carriers, then their carrier status must be confirmed by DNA analysis as there is a 1 in 4 (25%) risk to all their children of inheriting Bart's Hydrops Fetalis, also known as alpha thalassaemia major.

7.3 Haemoglobin H disease

An individual with haemoglobin H disease (- -/-α) has only one functioning alpha globin gene but usually retains the ability to produce sufficient haemoglobin for life. The haemoglobin level usually ranges from 70g/l to 100g/l, with a mean cellular haemoglobin (MCH) of 15pg to 25pg.

Haemoglobin H disease is a mild to moderate condition. It does not usually require treatment or lifelong blood transfusions, however short-term transfusions may occasionally be required during critical periods, such as pregnancy or illness or if the individual has an infection.

7.4 Alpha (α°) thalassaemia major

In alpha thalassaemia major, also known as Bart's Hydrops Fetalis, there are no functioning alpha globin genes (- -/- -). As a result, no alpha globin chains are produced, which results in a severe life-threatening anaemia in the fetus and, without intervention, is incompatible with extra-uterine life.

The hydropic fetus can usually be diagnosed by ultrasound scan during the second trimester of pregnancy. Very occasionally, babies survive with intra-uterine transfusions. However, there is a high risk of significant disabilities after birth.

Implications for the mother during pregnancy with a fetus that has Bart's Hydrops Fetalis include:

pre-eclampsia

ante-partum haemorrhage

retained placenta

possible 50% maternal mortality rate if alpha thalassaemia major in the fetus is undiagnosed

During antenatal screening it is important to diagnose if both parents are alpha zero (α°) thalassaemia carriers. Essential components for this are:

accurate family origin information

blood tests (analysis of full blood count)

DNA analysis for confirmation of carrier status

The family origins at highest risk of alpha zero (α°) thalassaemia carrier status are from Taiwan, Laos, Vietnam, Malaysia, China, Hong Kong, Myanmar (Burma), Cyprus, Turkey, Sardinia, Greece, Singapore, Philippines, Thailand, Cambodia and Indonesia.

In the UK around 20 to 30 couples annually are identified as being at high risk of having a baby with alpha thalassaemia major.

8. Beta thalassaemia

There are 2 main thalassaemia conditions depending on the genotype of the beta thalassaemia gene mutation inherited. They are:

thalassaemia major

thalassaemia intermedia

8.1 Beta thalassaemia carriers

A beta thalassaemia carrier:

has inherited 2 haemoglobin A genes, one of which is faulty and can usually be classified as one of the following:

beta+ (beta plus) thalassaemia - where a reduced amount of Hb A is produced

beta⁰ (beta zero) thalassaemia - where no Hb A is produced by that gene

has changes to the red blood cell indices:

haemoglobin A₂ is usually between 3.5% to 8%

Hb F is greater than 1% but less than 10%

MCH (mean cellular haemoglobin) is usually below 27pg

is not usually diagnosed at birth using routine newborn screening methods, due to the high level of fetal haemoglobin which masks the inheritance of a faulty haemoglobin A gene. Babies who may have inherited a beta thalassaemia gene should be tested when they are over 9 months of age to confirm their carrier status

A beta thalassaemia carrier may be misdiagnosed as having iron deficiency anaemia because the red blood cells are smaller and paler than usual, and there may be a lower than normal haemoglobin level. However, iron supplements must not be given to correct the apparent 'anaemia' caused by carrier status, as they are ineffective.

Further details are available in the leaflet Information for adult haemoglobinopathy carriers - you are a beta thalassaemia carrier.

Thalassaemia intermedia occurs in an individual when the beta globin chain production is significantly reduced but not completely absent. Clinical implications vary depending on the gene mutations inherited from both parents. The individual usually has a degree of anaemia, but the condition is not as severe as beta thalassaemia major.

The individual usually manages without regular blood transfusions but there may be splenomegaly and the requirement for occasional blood transfusions during pregnancy or illness. Regular monitoring by a specialist is recommended.

8.3 Beta thalassaemia major

Beta thalassaemia major is also called 'Cooley's Anaemia' or 'Mediterranean Anaemia' and is most common in people of Pakistani, Cypriot, Italian, Greek, Indian, Bangladeshi, Chinese and other East/South East Asian ancestry.

In beta thalassaemia major there is severely reduced or absent production of the beta globin chains that make up normal adult haemoglobin, due to defective beta globin genes which are inherited from both parents. This results in severe, life-threatening anaemia which usually requires regular blood transfusions to sustain life.

8.4 Prevalence of thalassaemia conditions in England

It is estimated that thalassaemia major affects about 1 in 27,000 pregnancies. Although there is no specific newborn screening programme, using the current technology most babies with beta thalassaemia major are identified during the newborn screening process. There are approximately 20 to 30 babies born with beta thalassaemia major each year.

The has a record of more than 1,200 individuals with thalassaemia conditions. As inclusion in the registry is voluntary, it is possible the total number is greater than this.

8.5 Clinical complications of untreated beta thalassaemia major

Clinical complications of untreated beta thalassaemia major include:

failure to thrive in babies and young children

lethargy and fatigue due to severe anaemia

hypersplenism due to increased breakdown of red blood cells

over activity of the bone marrow and bone deformity

severe anaemia leading to early death

9. Management of individuals with beta thalassaemia major

Although beta thalassaemia major is not included in the NHS NBS Screening Programme, children with this condition are usually identified and should be referred for specialist care. Early diagnosis helps to monitor a child's condition until blood transfusions are required. It also gives parents the opportunity to learn about the condition before complications arise.

The management of individuals with beta thalassaemia major aims to correct the severe anaemia and includes:

blood transfusions every 3 to 5 weeks, usually starting from approximately 9 to 12 months of age

iron chelation therapy to remove the excess iron (either orally or by subcutaneous injection)

regular hospital appointments to monitor the condition

splenectomy for hypersplenism

Supportive care is important for people affected by beta thalassaemia major. They:

should be encouraged to avoid iron rich foods and have a daily vitamin C supplement

may be suitable for bone marrow or stem cell transplant to cure their condition, although their genetic profile does not change

may need psycho-social support in addition to medical care

There is a risk of early death unless patients adhere to the strict regime of blood transfusions and iron chelation.

Thalassaemia major can be cured by bone marrow or stem cell transplant, but the genetic profile of the individual does not change. One potential side effect of undergoing the procedure is infertility.

9.1 Clinical complications of treated beta thalassaemia major

If thalassaemia major is not well managed by transfusion therapy this could result in anaemia and over-activity of the bone marrow. However, complications are usually related to an excess of iron accumulated in the body due to the regular blood transfusions. This can result in:

damage to the pituitary glands which could affect:

growth and delay puberty

insulin production, resulting in diabetes

hypothyroidism

cardiac impairment/failure

liver damage

lethargy and fatigue

erectile dysfunction in men and amenorrhoea in women

change in skin colour due to iron deposits

Individuals may also have:

hypersplenism due to increased breakdown of red blood cells

increased susceptibility to bacterial infections especially if the spleen has been removed

Increased risk of blood borne infections due to regular blood transfusion therapy

10. Haemoglobin E/beta thalassaemia

Haemoglobin E/beta thalassaemia may result in a syndrome similar to beta thalassaemia major or beta thalassaemia intermedia. However, some individuals do not have any significant clinical symptoms.

As there is no way to predict the severity of the condition at birth, all babies should be referred to a specialist for assessment and follow-up.

11. References

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All healthcare professionals involved in the screening programme for sickle cell and thalassaemia ( SCT) are advised to keep their knowledge updated; and an e-learning resource is provided to support practitioners. Haemoglobin ( Hb) is the substance within red blood cells which carries oxygen around the body.

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Sickle Cell nursing care management presentation

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Sickle Cell nursing care management presentation

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Sickle cell anemia is a severe hemolytic anemia that results from inheritance of the sickle hemoglobin gene.

What is Sickle Cell Anemia?

Sickle cell anemia is an inherited form of hemolytic anemia.

Sickle cell anemia is a severe hemolytic anemia that results from inheritance of the sickle hemoglobin gene.

The sickle hemoglobin (HbS) gene is inherited in people of African descent and to a lesser extent in people from the Middle East, the Mediterranean area, and the aboriginal tribes in India.

Sickle cell anemia is the most severe form of sickle cell disease.

The HbS gene causes the hemoglobin molecule to be defective.

Exposure. The sickle hemoglobin acquires a crystal-like formation when exposed to low oxygen tension.

Change in the shape. The oxygen level in venous blood can be low enough to cause the erythrocyte to lose its round, pliable, biconcave disk shape.

Adherence. These long, rigid erythrocytes can adhere to the endothelium of small vessels; when they adhere to each other, blood flow to a region or organ is reduced.

Reversion. If the erythrocyte is again exposed to adequate amounts of oxygen before the membrane becomes too rigid, it can revert to normal shape.

The causes of sickle cell anemia include:

Cold temperature. Cold can aggravate the sickling process, because vasoconstriction slows the blood flow.

Tissue hypoxia. Tissue hypoxia and necrosis causes a type of sickle cell crisis called the sickle crisis.

Human parvovirus. Aplastic crisis results from infection with the human parvovirus.

Splenic infarction. Sequestration crisis results when other organs pool the sickled cells, just like the spleen.

Symptoms of sickle cell anemia vary and are only somewhat based on the amount of HbS.

Anemia. Anemia is always present; usually, hemoglobin values are 7 to 10g/dl.

Jaundice. Jaundice is characteristic and usually obvious in the sclerae.

Dysrhythmias. Dysrhythmias and heart failure may occur in adults.

Enlargement of the bones. The bone marrow expands in childhood in a compensatory effort to offset anemia, sometimes leading to enlargement of the bones of the face and skull.

Complications of sickle cell anemia include:

Infection. Patients with sickle cell anemia are unusually susceptible to infection, particularly pneumonia and osteomyelitis.

Due to the decrease in oxygen supply because of the sickling, stroke may occur.

Renal failure. Blood flow is reduced to other body tissues including the kidneys, which may lead to renal failure.

Heart failure. The heart compensates for the decreased blood distribution by pumping more blood, and it may ultimately fail if it wears out.

The patient with sickle cell anemia usually has the following laboratory results:

CBC: Reticulocytosis (count may vary from 30%-50%); leukocytosis (especially in vaso-occlusive crisis), with counts over 20,000 indicate infection, decreased Hb (5-10 g/dL) and total RBCs, elevated platelets, and a normal to elevated MCV.

Stained RBC examination: Demonstrates partially or completely sickled, crescent-shaped cells; anisocytosis; poikilocytosis; polychromasia; target cells; Howell-Jolly bodies; basophilic stippling; occasional nucleated RBCs (normoblasts).

Sickle-turbidity tube test (Sickledex): Routine screening test that determines the presence of hemoglobin S (HbS) but does not differentiate between sickle cell anemia and trait.

Hemoglobin electrophoresis: Identifies any abnormal hemoglobin types and differentiates between sickle cell trait and sickle cell anemia. Results may be inaccurate if patient has received a blood transfusion within 3-4 mo before testing.

ESR: Elevated.

Erythrocyte fragility: Decreased (osmotic fragility or RBC fragility); RBC survival time decreased (accelerated breakdown).

May reflect decreased Po₂ (defects in gas exchange at the alveolar capillary level); acidosis (hypoxemia and acidic states in vaso-occlusive crisis).

Serum bilirubin (total and indirect): Elevated (increased RBC hemolysis).

Acid phosphatase (ACP): Elevated (release of erythrocytic ACP into the serum).

Alkaline phosphatase: Elevated during vaso-occlusive crisis (bone and liver damage).

LDH: Elevated (RBC hemolysis)

Serum potassium and uric acid: Elevated during vaso-occlusive crisis (RBC hemolysis).

Serum iron: May be elevated or normal (increased iron absorption due to excessive RBC destruction).

Total iron-binding capacity (TIBC): Normal or decreased.

Urine/fecal urobilinogen: Increased (more sensitive indicators of RBC destruction than serum levels).

Intravenous pyelogram (IVP): May be done to evaluate kidney damage.

Bone radiographs: May demonstrate skeletal changes, e.g., osteoporosis, osteosclerosis, osteomyelitis, or avascular necrosis.

X-rays: May indicate bone thinning, osteoporosis.

Treatment for sickle cell anemia is the focus of continued research.

Peripheral blood stem cell transplant. This may cure sickle cell anemia, however, this is only available to a small subset of affected patients, because of either the lack of a compatible donor or because severe organ damage is a contraindication.

Transfusion therapy. Chronic RBC transfusion therapy may be effective in preventing or managing complications from sickle cell anemia.

Supportive therapy. Adequate hydration is important during a painful sickling episode; aspirin is very useful in diminishing mild to moderate pain; NSAIDs are useful for moderate pain or in combination with opioid analgesics.

Monitoring pulmonary function. Pulmonary function is monitored and pulmonary hypertension is treated early if found. Infections and acute chest syndrome, which predispose to crisis, are treated promptly. Incentive spirometry is performed to prevent pulmonary complications; bronchoscopy is done to identify source of pulmonary disease.

Hydroxyurea has shown to be effective in increasing fetal hemoglobin levels in patients.

Arginine has antisickling properties and enhances the availability of nitric oxide, the most potent vasodilator, resulting in decreased pulmonary artery pressure.

Nursing management for a patient with sickle cell anemia focus on the following:

Assessment data for a sickle cell anemia patient should include:

Factors causing previous crisis. The patient is asked to identify factors that precipitated previous crisis and measures the patient uses to prevent and manage the crisis

Pain levels. Pain levels should always be monitored using a pain intensity scale.

Characteristics of pain. The quality, frequency, and factors that aggravate or alleviate the pain are included in the assessment.

Infection. Because patients with sickle cell anemia are susceptible to infections, they are assessed for the presence of any infectious process.

Based on the assessment data, major nursing diagnosis for the patient include:

Main Article: 6 Sickle Cell Anemia Crisis Nursing Care Plans

The major goals for the patient are:

Relief of pain.

Decrease incidence of crisis.

Enhance sense of self-esteem and power.

Absence of complications.

Nursing interventions for sickle cell anemia include:
Managing Pain

Use patient's subjective description of pain and pain rating on a pain scale to guide the use of analgesic agents.

Support and elevate any joint that is acutely swollen until swelling diminishes.

Teach patient relaxation techniques, breathing exercises, and distraction to ease pain.

When acute painful episode has diminished, implement aggressive measures to preserve function (eg, physical therapy, whirlpool baths, and transcutaneous nerve stimulation).

Preventing and Managing Infection

Monitor patient for signs and symptoms of infection.

Initiate prescribed antibiotics promptly.

Assess patient for signs of dehydration.

Teach patient to take prescribed oral antibiotics at home, if indicated, emphasizing the need to complete the entire course of antibiotic therapy.

Promoting Coping Skills

Enhance pain management to promote a therapeutic relationship based on mutual trust.

Focus on patient's strengths rather than deﬁcits to enhance effective coping skills.

Provide opportunities for patient to make decisions about daily care to increase feelings of control.

Increasing Knowledge

Teach patient about situations that can precipitate a sickle cell crisis and steps to take to prevent or diminish such crises (eg, keep warm, maintain adequate hydration, avoid stressful situations).

If hydroxyurea is prescribed for a woman of childbearing age, inform her that the drug can cause congenital harm to unborn children and advice about pregnancy prevention.

Monitoring and Managing Potential Complications

Management measures for many of the potential complications are delineated in the previous sections; additional measures should be taken to address the following issues.

Protect the leg from trauma and contamination.

Use scrupulous aseptic technique to prevent nosocomial infections.

Refer to a wound-ostomy-continence nurse, which may facilitate healing and assist with prevention.

PRIAPISM LEADING TO IMPOTENCE

Teach patient to empty the bladder at the onset of the attack, exercise, and take a warm bath.

Inform patient to seek medical attention if an episode persists more than 3 hours.

Emphasize the importance of complying with prescribed treatment plan.

Promote trust with patient through adequate management of acute pain during episodes of crisis.

Suggest to patient that receiving care from a single provider over time is much more beneﬁcial than receiving care from rotating physicians and staff in an emergency department.

When a crisis arises, emergency department staff should contact patient's primary health care provider for optimal management.

Promote continuity of care and establish written contracts with patient.

Promoting Home and Community Based Care

Involve the patient and his or her family in teaching about the disease, treatment, assessment, and monitoring needed to detect complications. Also teach about vascular access device management and chelation therapy.

Advise health care providers, patients, and families to communicate regularly.

Provide guidelines regarding when to seek urgent care.

Provide follow up care for patients with vascular access devices, if necessary.

Expected patient outcomes are:

Relief of pain.

Decreased incidence of crisis.

Enhanced sense of self-esteem and power.

Absence of complications.

Education is always the best way to impart what the patient and the family need to know for home care.

Vascular access management. Nurses in outpatient facilities or home care nurses may need to provide follow-up care for patients with vascular access devices.

Communication. All health care providers who provide services to patients with sickle cell disease and their families need to communicate regularly with each other

The focus of documentation in a patient with sickle cell anemia include:

Client's description of response to pain.

Acceptable level of pain.

Prior medication use.

Signs and symptoms pf infectious process.

Plan of care.

Teaching plan.

Responses to interventions, teaching, and actions performed.

Attainment or progress toward desired outcome.

Long term needs.

Here's a 5-item quiz about the study guide. Please visit our nursing test bank page for more NCLEX practice questions.
1. A nurse expects an adult patient with sickle cell anemia to have a hemoglobin value of:
A. Near 3 g/dl.
B. Near 5 g/dl.
C. Between 5 and 7 g/dl.
D. Between 7 and 10 g/dl.
2. Sickle-shaped erythrocytes cause:
A. Cellular blockage in small vessels.
B. Decreased organ perfusion.
C. Tissue ischemia and infarction.
D. All of the above.
3. A person with sickle cell trait would:
A. Be advised to avoid fluid loss and dehydration.
B. Be protected from crisis under ordinary circumstances.
C. Experience hemolytic jaundice.
D. Have chronic anemia.
4. On the basis of the knowledge of the inheritance of the sickle cell gene, the nurse expects the patient to be of what descent?
A. African.
B. Indian.
C. Middle Eastern.
D. Mediterranean.
5. On assessment, the nurse notes that the patient's face and skull bones are enlarged. She knows this is a compensatory response to: Answers and Rationale 1. Answer: D. Between 7 and 10 g/dl.
A patient with sickle cell anemia has a hemoglobin level of between 7 and 10 g/dl.
2. Answer: D. All of the above.

D: All of the options are correct.

A: Sickle-shaped erythrocytes cause cellular blockage in small vessels.

B: Sickle-shaped erythrocytes cause decreased organ perfusion.

C: Sickle-shaped erythrocytes cause tissue ischemia and infarction.

3. Answer: B. Be protected from crisis under ordinary circumstances.

B: A person with sickle cell trait experiences a crisis called sickle crisis.

A: A person with sickle cell trait would not be advised to avoid fluid loss and dehydration.

C: A person with sickle cell trait would not experience hemolytic jaundice.

D: A person with sickle cell trait would always have chronic anemia.

4. Answer: A. African.

A: The sickle hemoglobin (HbS) gene is inherited in people of African descent.

B: The sickle cell gene is inherited to a lesser extent from the Indian descent.

C: The sickle cell gene is inherited to a lesser extent from the Middle Eastern descent.

D: The sickle cell gene is inherited to a lesser extent from the Mediterranean descent.

5. Answer: B. Anemia.
The bones are enlarged as compensatory response to offset anemia.

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