WHAT IS THE SICKLE CELL BLOOD DISORDER?
The Sickle Cell Disorder/Disease is an ‘Anaemia’, there are many types of anaemias the more common known are General Anaemia, Thalassaemia, Leukaemia, Hypoglycaemia and the most common and fastest growing worldwide is Sickle Cell Anaemia.
Sickle Cell Anaemia is a serious inherited blood disorder where the red blood cells, which carry oxygen around the body, develop abnormally causing the extreme deprivation of Iron Fluorine, which can cause over 20 other diseases and conditions to manifest.
Under the heading of Sickle Cell Anaemia there are many types; such as Haemoglobin SS Disease, Haemoglobin SC Disease, Haemoglobin SB+ Beta Thalassaemia, Beta-Zero Thalassaemia, Haemoglobin D-Pun-jab plus others.
These Sickle Cell disorders mainly affects people of African, Caribbean, Middle Eastern, Eastern Mediterranean and Asian origin, however it is manifest in people of all origin.
So.... What causes Sickle Cell Anaemia?
Sickle cell anaemia is caused by a mutation (abnormal change) in the gene that instructs the body to produce haemoglobin.
Haemoglobin is the protein molecule in red blood cells that carries oxygen from the lungs to the body's tissues and returns carbon dioxide from the tissues back to the lungs. Haemoglobin is made up of four protein molecules (globulin chains) that are connected together.
The sickle cell gene is inherited.
To have sickle cell anaemia you will have inherited the defective gene from both parents.
If you only inherit the gene from one parent, you have what's known as sickle cell trait. It's likely that your blood will contain some sickle cells, however you will be able to produce normal haemoglobin and won't usually experience extreme symptoms.
However, you would be a carrier of sickle cell anaemia and may pass the abnormal gene on to your children.
In England, about 250,000 people are thought to have the sickle cell trait, with those of African-Caribbean origin primarily affected.
If two people with the sickle cell trait have a child, there's a one in four chance that the child will be born with sickle cell anaemia.
In the UK, about 20,000 people are thought to have a full blown sickle cell disorder, with 80% living in the London area and is most commonly seen in African and Caribbean people.
Normal red blood cells are flexible and disc-shaped, but in sickle cell anaemia they can become rigid and shaped like a crescent moon (or sickle).
The sickle-shaped cells contain defective haemoglobin, Hb for short, ‘that’s the iron-rich protein that enables red blood cells to carry oxygen from your lungs to the rest of the body’.
The abnormal cells are devoid of oxygen and are also unable to move around as easily as normal shaped cells and can block blood vessels, resulting in tissue and organ damage and episodes of severe pain.
Such episodes are known as a Sickle Cell Crisis or a Vaso-Occlusive Crisis, a Crisis can be triggered by cold damp temperatures, stress, dehydration, and infection & bruising or no obvious reason at all.
A Crisis can last from a few minutes to several months, although on average most last from around five to fourteen days.
The lifespan of a normal blood cell is 120 days however the abnormal Sickle blood cells have a shorter lifespan and aren't replaced as quickly as normal blood cells, which is on average just 20 days.
This shortage of red blood cells is known as Anaemia.
Sickle Cell Anaemia is a painful & debilitating condition and symptoms of anaemia include severe joint pains, lethargy (a complete lack of energy), dehydration, tiredness and breathlessness, particularly after exercise and a consistency of feeling very cold.
This is very serious condition and amongst others ailments it manifests symptoms such as iron over-load, jaundice, swollen joints, migraines, breathing difficulties, strokes, permanent damage causing removal of spleen, reduced bone density, ulcers, blindness, priapism, (painful swollen and lumpy penis which can cause erectile dysfunction) other ailments connected are aneurysms, pneumonia, organ failure, weakened immune system, gallstones and death
Treatments can vary and are tailored to each patient, however what is commonplace for all Sickle Cell patients are the daily need of Antibiotics, Strong Pain Killers sometimes Blood Thinners & Folic Acid, when admitted into hospital the patient will also require Oxygen & in majority of cases Blood Transfusions with many needing Exchange Transfusions.
CALL FOR ACTION:
The North Middlesex Hospital NHS Trust, in Edmonton North London is in desperate need of a 'Cell Separator' machine for its haematology department.
This machine is needed for the use of the Sickle Cell patients and is highly necessary to separate contaminated/ dysfunctional and damaged blood cells from the healthy ones whilst patients are undergoing lifesaving Exchange Blood Transfusions.
Currently this process is done by the hands of our overworked nursing staff and is very time consuming causing amongst other issues, additional expense.
This machine costs £50,000 so far around £20,000 has been raised via various community initiatives.
We the Members and Supporters of the Sickle Cell Care, Advocacy, Unity, Support with Empathy Group (SC Cause), has pledged to donate 40% from the proceeds of our 3rd 'Community Miles for Sickle Cell' Annual Sponsored Walk to help with the purchase of this machine.
After the walk we will continue to campaign and fund-raise until this machine is purchased.
We ask that you assist our efforts by either joining our walk or by donating directly.
You may click here to register to join our walk.
C/O Healthy Living Int.
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