Sickle cell anaemia is an inherited red blood cell disorder. Normal red blood cells are round-shaped and flexible to move quickly through blood vessels and carry enough oxygen, which is not the case in this chronic medical condition. Instead, the blood cells are sickle-like or crescent-shaped, making them rigid and sticky, which in turn find it difficult to move through blood vessels. The result? They slow down or block the blood flow, resulting in not enough healthy red blood cells to carry oxygen throughout your body.
Sickle cell anaemia causes:
Sickle cell anaemia causes can be characterized by acute anaemia. Normal red blood cells live up to 120 days, but sickle cells die in 10-20 days leading to a shortage of red blood cells. Consequently, the body experiences fatigue because of not getting enough oxygen.
- Anaemia: Sickle cells break apart easily and die, leaving one with too few red blood cells. Red blood cells usually live for about 120 days before they are replaced. But sickle cells usually die in 10 to 20 days, leaving a shortage of red blood cells (anaemia). Without enough red blood cells, your body can’t get enough oxygen, causing fatigue.
- Pain: Sickle cell anaemia patients experience episodes of pain that may vary in intensity. This is due to the blockage of blood flow caused by sickle-shaped cells while following through the vessels in your chest, abdomen and joints. Pain can also occur in bones. A severe pain crisis requires a hospital stay.
- Swelling of hands and feet: The swelling is caused by sickle-shaped red blood cells blocking blood flow to the hands and feet.
- Frequent infections: Sickle cells damage the spleen, leaving those affected more vulnerable to infections. Vaccinations and antibiotics to prevent potentially life-threatening conditions, such as pneumonia, are administered from time to time.
- Delayed growth or puberty: Oxygen and nutrients needed for growth are facilitated by proper blood circulation. A shortage of healthy red blood cells can slow growth in infants and children and delay puberty in teenagers.
- Vision problems: Sickle cells plug the tiny blood vessels that supply your eyes, thus damaging the retina, which helps in visual processing.
- Chronic symptoms: Some adolescents and adults with sickle cell anaemia also have chronic pain resulting from bone and joint damage, ulcers, and other causes.
Sickle cell anaemia treatment:
- Hydroxyurea: Daily doses reduce the frequency of painful crises and might reduce the need for blood transfusions and hospitalizations. It is, however, not advisable for pregnant women.
- L-glutamine oral powder: The FDA-approved drug for sickle cell anaemia treatment helps in reducing the frequency of pain.
- Crizanlizumab: The FDA recently approved this drug for sickle cell anaemia treatment. Given through a vein, it helps reduce the frequency of pain.
- Pain-relieving medications. Your doctor might prescribe narcotics to help relieve pain during sickle cell pain crises.
Other treatment options include blood transfusions, wherein fresh blood is introduced into the patient’s bloodstream, or Stem cell therapy, wherein a portion of bone marrow stem cell from a healthy donor is introduced into the recipient (mainly children). However, both treatments are advised for patients with chronic symptoms or complications.
When to seek a second opinion
Sickle cell anaemia is usually diagnosed in infancy through newborn screening programs. One can approach a haematologist, gynaecologist, or pediatric haematologist for treatment.If you or your child develops any of the following problems, search for a haematologist near me or seek emergency medical care:
- Unexplained episodes of severe pain.
- Swelling in the hands or feet.
- Abdominal swelling
- Pale skin or nail beds.
- Yellow tint to the skin or whites of the eyes.
- Signs of stroke such as one-sided paralysis or dropping or contortion in the face, trouble walking or talking, sudden vision changes or unexplained numbness.
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