Symptoms of SCA are manifested in the first year of the affected child; it usually begins to manifest only 5 months after birth because fetal hemoglobin (f -Hb) is present in the child immediately after birth and this takes care of the new-born from getting affected.
Symptoms in infants and young children are fever, abdominal pain, bacterial infections, dactylitis and splenic sequestration, while young adults mostly present with eye damage, leg ulcers or aseptic necrosis. Pain episodes are also very common in SCA patients.
Symptoms and problems of sickle cell disease are due to the hemoglobin S (HbS) molecule.
Hemoglobin S (HbS) when it loses its oxygen becomes rigid rods and changes the red blood cells into a sickle or crescent shape from the normal disc or concave shape.
The sickle-shaped cells instead of the smoothly flow stick to the walls of the vessels and are unable to squeeze through the finer capillaries. This traffic jam in smaller tiny blood vessels deprives the tissues and organs of oxygen and leads to its damage.
The decreased oxygenation causes severe pain or sickle cell crisis and over a period of time it leads to gradual destruction in organs and tissues throughout the body.
Depending on the impact impaired circulation has on organs - ‘crisis’ episodes
may be manageably moderate or critical. Many of these symptoms are specific to a certain age group while others are common to all.
The main symptoms of SCA include- Anemia and fatigue
– Low hemoglobin results in less oxygen to tissues and causes shortness of breath, headache, dizziness, coldness in the hands and feet, headache, chest pain and palor. Similarly feeling easily tired and fatigued are all symptoms of anemia. Sickle cell crisis-
The pain may be acute or chronic, but acute pain, which may be mild or severe, is more commonly observed. This may be sudden and can range from mild to very severe. The pain, which usually lasts from a few hours to some days could also lasts for weeks to months. It affects lungs, abdomen and joints and is a medical emergency which must be dealt with.
If a patient is undergoing a surgical procedure and belongs to SCA endemic regions, they should undergo sickle cell screening to avoid such a crisis on the operating table.In addition to the above patients may experience-
Young teenagers may experience delayed puberty
Gums may bleed or get infected
Joints may show swelling and cause pain
Ulcers may appear on legs
Vision problems may occur
Anemia may get worse