Sickle Cell Society

A Message from Lord Pickles and Lord Blunkett, followed by Sickle Cell Society's best practice article

The ability to listen and learn from one another has always been vital in parliament, in business and in most aspects of daily life. But at this particular moment in time, as national and global events continue to reiterate, it is uncommonly crucial that we forge new channels of communication and reinforce existing ones. The following article from Sickle Cell Society is an attempt to do just that. We would welcome your thoughts on this or any other Parliamentary Review article.

Blunkett signature Rt Hon The Lord David Blunkett
Pickles signature Rt Hon The Lord Eric Pickles

www.sicklecellsociety.org

THE PARLIAMENTARY REVIEW
Highlighting best practice
THE PARLIAMENTARY REVIEW
Highlighting best practice
14 | SICKLE CELL SOCIETY
CEO John James OBE
Sickle Cell and Thalassaemia All-Party
Parliamentary Group presenting the
End the Blood Tax
report
In 2019, the Sickle Cell Society celebrated 40 years as a national
charity. Back in 1979, it registered as a charity because of an
acute lack of awareness, understanding and clinical knowledge
regarding sickle cell disease. Its founders include Professor Dame
Sally Davies, a former consultant haematologist and chief medical
officer who was the first professor of haemoglobinopathies;
Sherlene Rudder MBE; and Professor Dame Elizabeth Anionwu,
the UK’s first sickle cell nurse, who helped establish the first
UK Sickle Cell and Thalassaemia Counselling Centre in Brent in
1979. Forty years on, CEO John James OBE explains just what
sickle cell disease is, and how the Sickle Cell Society continues
to be instrumental in raising awareness and improving care
outcomes for the condition.
Firstly, let me explain what the disease is.
Sickle cell is a genetic blood condition affecting the haemoglobin which is passed
on by both parents and – as the name suggests – causes blood cells to be sickle
shaped, rather than biconcave discs with a flattened centre, as is the norm. It
typically, but not exclusively, presents in those who have African, Caribbean,
Mediterranean, Indian or Middle Eastern heritage.
Blood cells contain haemoglobin, which carries oxygen to all parts of the body. At
low oxygen concentrations, the sickle haemoglobin forms abnormal clumps, and
the red cell can become deformed or sickle shaped. These cells are rigid and cannot
pass through small capillaries. This leads to obstruction, as they get stuck, which
FACTS ABOUT
SICKLE CELL SOCIETY
»CEO: John James OBE
»Established as a national
charity in 1979
»Head office in Brent, London
»Services: Helpline, children’s
holidays, educational
events and resources, policy
development, campaigning
and lobbying
»No. of employees: 12
Sickle Cell Society
15SICKLE CELL SOCIETY |
HEALTH & SOCIAL WORK
can lead to acute pain, as inflammation
occurs and oxygen cannot get to vital
organs. This pain can sometimes be
treated at home but typically cannot
be fixed by painkillers like ibuprofen
orparacetamol.
When sickle cell patients experience the
effects of the condition, they typically
go to hospital. In London, for example,
sickle cell painful episodes are one of
the top 20 causes of A&Eadmissions.
In the long term, sickle cell damages
the organs, such as the kidney and
lungs; reduces life expectancy; and
means that, on average, 20 per cent
of children with the disease will have
had silent strokes in the brain before
they are 20 years old. Sickle cell is a
devastating condition.
The condition is passed on by healthy
“carriers” of the gene who do not
experience its effects. When two
carriers have children, there is a 25 per
cent chance – for every pregnancy – of
giving birth to someone who has sickle
cell disease.
Awareness, understanding,
clinical knowledge
Our three key aims at the Sickle Cell
Society are as follows.
When you compare cystic fibrosis and
sickle cell disease – both of which are
genetic blood disorders – sickle cell
affects considerably more people;
in the UK, this is around 15,000,
compared to 10,500 with cystic
fibrosis. Both are debilitating, but
perhaps because they affect different
ethnic groups, there is less awareness
of sickle cell, the country’s largest
genetic blood disorder. It is therefore
vital that the Sickle Cell Society
continues to take action to raise
awareness of this condition.
We also want to ensure that people
living with sickle cell disease and
their families have access to high-
quality care, positive interactions with
clinicians and good health outcomes.
Therefore, as part of our drive to
raise clinical knowledge, in 2018
we were instrumental in producing
a set of updated adult standards of
care for people living with sickle cell
in the UK. In 2019, we finalised a
set of paediatric sickle cell standards
and recommendations in partnership
with the NHS and the Sickle Cell and
Thalassaemia Screening Programme
run by Public Health England. Patient
surveys and peer reviews have shown
that despite having specialist sickle
cell centres across the country, sickle
cell care is variable and improvement
isrequired.
Thirdly, we campaign and lobby for
improvement in services and health
policy. We run the secretariat of the
All-Party Parliamentary Group for Sickle
Cell and Thalassaemia, chaired by Pat
McFadden MP. We helped to develop
a number of policy initiatives, including
one for the Department for Work and
Pensions that introduced personal
The Sickle Cell Annual
Children’s Holiday, 2019
In 2018 we
were
instrumental
in producing a
set of updated
adult
standards of
care for
people living
with sickle cell
in the UK
BEST PRACTICE SPONSOR 2020
15SICKLE CELL SOCIETY |
HEALTH & SOCIAL WORK
can lead to acute pain, as inflammation
occurs and oxygen cannot get to vital
organs. This pain can sometimes be
treated at home but typically cannot
be fixed by painkillers like ibuprofen
orparacetamol.
When sickle cell patients experience the
effects of the condition, they typically
go to hospital. In London, for example,
sickle cell painful episodes are one of
the top 20 causes of A&Eadmissions.
In the long term, sickle cell damages
the organs, such as the kidney and
lungs; reduces life expectancy; and
means that, on average, 20 per cent
of children with the disease will have
had silent strokes in the brain before
they are 20 years old. Sickle cell is a
devastating condition.
The condition is passed on by healthy
“carriers” of the gene who do not
experience its effects. When two
carriers have children, there is a 25 per
cent chance – for every pregnancy – of
giving birth to someone who has sickle
cell disease.
Awareness, understanding,
clinical knowledge
Our three key aims at the Sickle Cell
Society are as follows.
When you compare cystic fibrosis and
sickle cell disease – both of which are
genetic blood disorders – sickle cell
affects considerably more people;
in the UK, this is around 15,000,
compared to 10,500 with cystic
fibrosis. Both are debilitating, but
perhaps because they affect different
ethnic groups, there is less awareness
of sickle cell, the country’s largest
genetic blood disorder. It is therefore
vital that the Sickle Cell Society
continues to take action to raise
awareness of this condition.
We also want to ensure that people
living with sickle cell disease and
their families have access to high-
quality care, positive interactions with
clinicians and good health outcomes.
Therefore, as part of our drive to
raise clinical knowledge, in 2018
we were instrumental in producing
a set of updated adult standards of
care for people living with sickle cell
in the UK. In 2019, we finalised a
set of paediatric sickle cell standards
and recommendations in partnership
with the NHS and the Sickle Cell and
Thalassaemia Screening Programme
run by Public Health England. Patient
surveys and peer reviews have shown
that despite having specialist sickle
cell centres across the country, sickle
cell care is variable and improvement
isrequired.
Thirdly, we campaign and lobby for
improvement in services and health
policy. We run the secretariat of the
All-Party Parliamentary Group for Sickle
Cell and Thalassaemia, chaired by Pat
McFadden MP. We helped to develop
a number of policy initiatives, including
one for the Department for Work and
Pensions that introduced personal
The Sickle Cell Annual
Children’s Holiday, 2019
In 2018 we
were
instrumental
in producing a
set of updated
adult
standards of
care for
people living
with sickle cell
in the UK
THE PARLIAMENTARY REVIEW
Highlighting best practice
THE PARLIAMENTARY REVIEW
Highlighting best practice
16 | SICKLE CELL SOCIETY
independence payments for those
with sickle cell who are not in work.
Another has been a policy document
regarding the lack of a national
approach to teaching sickle cell in the
nursing curriculum.
Together with the UK National
Screening Committee, we have also
worked to improve access to the sickle
cell screening programme in the UK
through intensive patient engagement.
We won the tender, in conjunction
with the UK Thalassaemia Society,
to continue delivering the outreach,
engagement and development
programme for the next three years.
A lack of available treatment
options
We have been lobbying hard for the
pharmaceutical sector to carry out
more research and provide more
choice to keep people well and help
them to live longer. In comparison to
ten years ago, there are now a number
of large and small pharmaceutical
companies from across the world that
are carrying out clinical trials on new
drugs for sickle cell.
Future affordability will also be a
significant challenge. Going forward,
we will see more and more technologies
emerge – the likes of gene therapy and
genome work, which could potentially
alter the shape of a sickle cell. The
NHS may not be able to afford these
technologies – if clinical trials do come
to fruition, affordability will be the next
key question that the healthcare system
faces. It is vital that funding is dedicated
to the new treatments so that we can
see further improvements in the lives of
those living with sickle cell.
Securing our future
The last 10 years have been difficult
economically for our patients and also
our charity. We are aware that we
could do so much more if we had the
resources. We need to raise awareness
in order to raise funds, in addition
to applying for grants. We need to
learn from other charities that support
people with genetic conditions.
Forty years on, we still need to
improve understanding even though
this condition is no longer confined
to a small group of people. As the
population becomes more ethnically
diverse, there will be more relationships
between people of different ethnic
backgrounds. Soon sickle cell disease
will not be seen as a “black” condition.
Already, one in every 300 white babies
born today has sickle cell trait.
We look forward to our continued
partnership with NHS England and
Public Health England. We will continue
to work with the doctors, nurses,
psychologists and therapists who care
for those with the disease and lobby for
better and more consistent care across
the country. We will continue to be the
advocates for all those who have sickle
cell disease and their families and hope
to have your support in our mission.
We look
forward to our
continued
partnership
with NHS
England and
Public Health
England. We
will continue
to work with
the doctors,
nurses,
psychologists
and therapists
who care for
those with the
disease
»AVAILABLE TREATMENT OPTIONS
Outcomes in sickle cell disease have improved immeasurably in the
past 20 years, but there is still more to be done. Universal newborn
screening, prophylactic penicillin to prevent early mortality from sepsis
and the introduction of transcranial doppler screening to identify
those at risk of stroke have all been a huge advance. On the treatment
front, painkillers and blood transfusions continue to be the mainstay.
Bone marrow transplantation is available in children but only
for those who have a compatible sibling. The introduction of
hydroxyurea, a form of chemotherapy, has reduced morbidity from
painful episodes and acute chest syndrome significantly. However,
not everyone with sickle cell disease responds to hydroxyurea, and
patients have concerns about long-term implications.
We strongly believe that more research into effective treatments
is required to offer a wider choice that can improve the morbidity
and mortality as well as the quality of life of people living with sickle
cell. More resources need to be provided to support those with the
disease and their families, as having a painful and life-threatening
long-term condition has a wider psychosocial impact which affects
schooling, employment and family life.

www.sicklecellsociety.org

This article was sponsored by Sickle Cell Society. The Parliamentary Review is wholly funded by the representatives who write for it. The publication in which this article originally appeared contained the following foreword from Rt Hon Elizabeth Truss.

Rt Hon Elizabeth Truss's Foreword For The Parliamentary Review

By Rt Hon Elizabeth Truss

Even by the standards of the day –this has been one of the most exciting and unpredictable years in British politics.

The leadership election we’ve just seen marks a huge moment in our country’s history. This government is taking a decisive new direction, embracing the opportunities of Brexit and preparing our country to flourish outside the EU.

As international trade secretary, I’ll be driving forward work on the free trade agreements that are going to be a priority for the government. Free trade isn’t just an abstract concept bandied around by technocrats. It is crucial for a strong economy and for the ability of families to make ends meet. Free trade benefits people in every part of our country, as British firms export to new markets and people doing the weekly shop have access to a wider choice of goods at lower prices.

The essence of free trade is in the title: freedom. It’s about giving people the power to exchange their goods without heavy government taxation or interference. Commerce and free exchange are the engine room of prosperity and social mobility. I’m determined to tackle the forces who want to hold that back.

One of my priorities is agreeing an exciting new free trade deal with the US, building on the great relationship between our two countries and the Prime Minister and US President. But I’ll also be talking to other partners including New Zealand, Australia and fast-growing Asian markets.

And with the EU too, we want a friendly and constructive relationship, as constitutional equals, and as friends and partners in facing the challenges that lie ahead – a relationship based on a deep free trade agreement. Our country produces some of the world’s most successful exports, and the opportunity to bring these to the rest of the world should make us all excited about the future. It is this excitement, optimism and ambition which I believe will come to define this government.

For too long now, we have been told Britain isn’t big or important enough to survive outside the EU – that we have to accept a deal that reflects our reduced circumstances. I say that’s rubbish. With the right policies in place, we can be the most competitive, free-thinking, prosperous nation on Earth exporting to the world and leading in new developments like AI. To do that, we’ll give the brilliant next generation of entrepreneurs the tools they need to succeed. Since 2015, there has been a staggering 85 per cent rise in the number of businesses set up by 18 to 24 year olds – twice the level set up by the same age group in France and Germany. We’ll help them flourish by championing enterprise, cutting taxes and making regulation flexible and responsive to their needs.

As we do that, we’ll level up and unite all parts of the UK with great transport links, fibre broadband in every home and proper school funding, so everyone shares in our country’s success.

2019 has been the year of brewing economic and political revolution. 2020 will be the year when a revitalised Conservative government turbo charges the economy, boosts prospects for people across the country, and catapults Britain back to the forefront of the world stage.



Rt Hon Elizabeth Truss
Secretary of State for International Development