SICKLE cell disease is a common genetic condition. It affects between 12,500 to 15,000 people in England.
This is a group of inherited diseases that causes red blood cells to die prematurely, resulting in a shortage of healthy red cells.
Sickle cell disease can be a life-long, serious condition. It can cause fatal complications but most people who have it live normal lives.
While sickle cell can be affected by anyone, it is more common in those of African or African-Caribbean descent. However, sickle cell can also be found in families from the Middle East and parts of India as well as the eastern Mediterranean and South and Central America.
What is sickle cell illness?
Sickle cell disease refers a range of conditions that affect red blood cells.
Sickle cell anaemia, the most serious form sickle cell disease, is the most serious.
People suffering from the disease have red blood cells that are unusually shaped.
Medical problems can result from this abnormality, as the red blood cells don’t live as long and can get stuck in the blood vessels.
This can cause serious complications like strokes, eye pain, and even death.
Who can get sickle-cell disease?
Anybody born to parents who have sickle cell-causing genes can get the disease.
There is a 25% chance that both parents carry the defective gene.
As they may not be suffering from the disease themselves, parents often don’t know they are carriers.
According to NICE, one in every 2,000 babies born in England has sickle cell disease.
The reason it occurs in some ethnicities more than others is because of a history of malaria, or migration from a malarial area.
Positive selection has resulted in the discovery of the gene mutation.
What are the symptoms for sickle cell disease?
The symptoms can appear as early as a few months after the initial diagnosis.
It is important to start treatment early as children often have fewer or no ongoing symptoms.
Sickle cell crises, also known as episodes of pain, are one of the most frequent warning signs. They occur when the abnormal shape or blood vessels causes a blockage.
These “crises”It can impact many areas of the body including:
- Hands or feet, especially for young children
- Breastbone and ribs
- Arms and legs
These episodes vary in frequency and severity from one person to the next.
On average, there is only one episode of bad behavior per year.
Although it isn’t always easy to determine what causes crises, common causes include weather (wind, rain, cold), dehydration and stress.
People with sickle cells are more susceptible to infection, especially if they’re young. This is because their body can’t fight viruses and bacteria as well.
Sometimes, small infections like a cold can lead to life-threatening conditions such as meningitis.
Sickle cell disease can cause many other symptoms and problems. You can find a complete list at the NHS website.
What treatment is available for sickle cell disease?
The primary goal of sickle cell treatment is to avoid pain crisis triggers.
A doctor may recommend that a patient drink plenty of water and wear warm clothes to prevent them from getting cold.
There are other ways to deal with a pain crisis at home.
However, patients may be offered medication like hydroxyurea if they have severe or repeated episodes.
The NHS announced that Crizanlizumab, a new drug to improve the quality of life, will be available to thousands of patients across England in October.
Anyone over 16 with multiple sickle cells crises will have the opportunity to receive this new treatment.
It will be delivered by transfusion drip and will work by binding to a protein in the blood cells to prevent the restriction of blood and oxygen supply that lead to a sickle cell crisis.
The new drug is set to reduce the number of times a patient with sickle cell goes to A&E by two fifths.
Amanda Pritchard (Norwegian chief executive) announced the new treatment. She said that the NHS had struck a drug deal that would allow as many as 5,000 people to enjoy a better quality of living over the next three-years.
Only way to treat sickle cell disease is through stem cell transplants or bone marrow transplants.
Because of the risks involved, transplants are rarely used.
Extreme treatment like this is only recommended when the long-term health benefits of transplants outweigh the risks.
What is sickle cell and hemophilia?
Anemia due to sickle cell disease or iron deficiency is different from anaemia caused in part by sickle cells disease.
Nearly all people with sickle cell disease will have anaemia, which is a condition in which the hemoglobin levels are low.
The oxygen transporter Haemoglobin can be found in red blood cells.
Although this is not likely to cause other symptoms, it can lead to severe complications.
If children experience a swelling in their spleen, which is another sign of sudden onset anaemia, the same treatment can be used.
You may also experience headaches, fast heartbeat, dizziness, fainting, or other symptoms due to lack of oxygen.
Sickle cell anaemia patients are at greater risk for stroke, hypertension, high blood pressure, organ damage, blindness and skin ulcers.
Who has sickle-cell disease?
Prodigy, a 42-year old rapper, was diagnosed with sickle cell anemia.
He developed complications from sickle cell disease and was unable to respond to treatment.
Evan Nathan Smith, a football stats analyst, died in his 21st year after calling 999 from the hospital bed. ‘staff refused him oxygen’.
An inquest was held in 2021 by doctors. They stated that Evan, who was admitted to North Middlesex Hospital for sepsis in north London and which caused a sickle cell crisis, most likely would not have died if he had been given the right treatment.