World Sickle Cell Day🩸 is held annually on the 19th of June🗓️ as an awareness to increase public understanding of sickle cell disease (SCD) and the challenges faced by patients themselves as well as their family members/caregivers.👨👩👧👧
According to the World Health Organization (WHO), SCD affects almost 100 million people worldwide and more than 300,000 children 👦👧are born with SCD each year! It is considered the most frequent genetic disorder worldwide which affects predominantly people of African, Indian, Middle Eastern, and Latin American descent.
What is Sickle Cell Disease?
Sickle cell disease (SCD) is a hereditary blood disorder that causes red blood cells in patients to become ‘sickle’ or crescent-moon 🌙 in shape, making them sticky and rigid. These deformed red blood cells would be stuck in small blood vessels which in turn blocking blood flow and oxygen from reaching all parts of the body.
For a clearer picture,watch this video🧡
Causes
As mentioned earlier, SCD is inherited 🧬. It is an autosomal RECESSIVE disorder, meaning that in order to develop the condition, a person must inherit two sickle cell genes (one from each parent). If a person has a faulty gene from just one parent, they will have sickle cell trait but not sickle cell disease. People with SCT usually do not have signs of the disease and live a normal life but they could pass the sickle cell gene on to their children.
As depicted in the diagram above, if two carriers (AS) have a child, there is a 25% chance the child will be unaffected (AA), a 50% chance of having a child that is a carrier (AS), and a 25% chance of having a child with sickle cell anaemia (SS).
Symptoms & Complications
SCD patients usually begin to experience signs and symptoms at around 5 months of age. These symptoms vary from person to person (ranging from mild to severe) and may change over time.
- Swelling of Hands✋ and Feet🦶🏼. Normally the swelling of hands and feet is the first symptom of SCD. This swelling, often accompanied by a fever, is caused by sickle-shaped red blood cells blocking blood flow to the hands and feet.
- Anaemia🩸 . Normal red blood cells usually live for about 120 days but sickle cells tend to rupture within 10 to 20 days, leaving a shortage of red blood cells (anaemia). Without enough red blood cells to carry oxygen around the body, causing fatigue.
- Pain Episode/Crises😖. A periodic episode of pain, called pain crisis, is the most common complication of SCD as well as the primary reason for patients going to the emergency department in the hospital. Pain may develop at the chest, abdomen, joints and even bones when sickle-shaped red blood cells get stuck and clog the tiny blood vessels at these sites. The pain differs in intensity and could last for a few hours or even up to a few weeks. Some adolescents and adults also suffer from chronic pain (multiple on-off pain crises per year).
- Frequent Infections🦠. Sickle cells could cause damage to the spleen, leaving SCD patients more vulnerable to infections. Doctors usually prescribe antibiotics and provide vaccinations to infants and children with sickle cell anaemia to prevent potentially life-threatening infections, such as PNEUMONIA.
- Delayed Growth or Puberty. Red blood cells carry oxygen and nutrients needed for growth throughout the body. Hence, a shortage of healthy red blood cells could retard growth in infants and children and delay puberty in teenagers.
- Stroke💓. Getting a stroke is possible if the sickle cells clog blood vessels leading to the brain. There is an estimated 10% of children with SCD will have a symptomatic stroke which causes learning problems and lifelong disabilities.
- Leg Ulcers🦵🏼. Leg ulcers more often occur on the lower part of the leg. It affects males more than females. The possible reasons for leg ulcers include trauma, infection, inflammation, and interruption of the circulation in the smallest blood vessels of the leg.
- Vision Problems👁️. Vision loss and even blindness could occur due to blocked tiny blood vessels supplying nutrients to the eyes. When this occurs, the retina (the thin layer of tissue which processes visual images) would be damaged.
Treatment
There is no cure for most people with sickle cell anaemia. However, there are medications available to relieve pain and help prevent complications associated with the disease.
Blood transfusions🩸💉 may be needed to provide healthy red blood cells to replace the sickle cells when patients experience a sickle cell crisis (severe anaemia or infections).
Lifestyle Measures
There are simple measures that people with SCD can take to help prevent and reduce the number of pain crises, including:
- Attend regular follow-ups with the doctor👨⚕️.
- Ensure adequate water🥤 intake (8-10 glasses of water per day).
- Try to avoid places or situations with exposure to high altitudes (flying✈️, mountain⛰️ climbing, or cities with a high altitude).
- Try to avoid places or situations with exposure to low oxygen levels (mountain⛰️ climbing or too strenuous exercises extremely like in military boot camp or training for an athletic🏃♂️🤼🚴 competition).
- Follow a healthy, balanced diet🍎🍊🥑🥦🥕🌽 to boost overall well-being.
- Avoid extreme temperatures (too hot🔥 or too cold❄️) as this may trigger a crisis.
- Avoid smoking🚭 as this reduces the flow of oxygen through the lungs.
Prepared by: Chew Ee Wei, a HTM Pharmacist.
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