Gilbert’s syndrome is a benign condition passed down generations as an inheritance. The disease influences the way bilirubin (the orange-yellow pigment in the bile that forms as a product of haemoglobin) is processed by the liver, indicating the presence of jaundice in the body. That is why jaundice affected people complain of persistent yellowness of eyes and fatigue.
Gilbert’s syndrome is a common disorder,with eight people in every 100 being affected. It is mostly diagnosed at puberty or in adult life. Males are affected more often. The values of bilirubin may be higher than 3 mg/decilitre due to stress, fatigue, illness, fasting, and alcohol. To understand Gilbert’s syndrome, it will be useful to learn a bit more about bilirubin, and the condition, unconjugated hyperbilirubinemia.
Bilirubin is a pigment found in the bile, a digestive juice secreted by the liver and stored in the gallbladder. The process of the creation of bilirubin begins with the breakdown of Red Blood Cells (RBCs) in many parts of the body. RBCs contain haemoglobin, which in turn breaks down to two substances: ‘haeme’ (iron) and ‘globin’ (protein). Haeme is converted to bilirubin, which is then carried through the blood stream to the liver.
In the liver, most of the bilirubin is chemically attached to another molecule before it is released in the bile. This conjugated (attached) bilirubin is called direct bilirubin; unconjugated bilirubin is called indirect bilirubin. Conjugated bilirubin is secreted by the liver into the bile and stored in the gallbladder, or transferred directly to the small intestines. Bilirubin is further broken down by bacteria in the intestines, and those broken down products contribute to the colour of the faeces.
Unconjugated or indirect bilirubin is formed by haeme fragments, and they do not get attached to any other chemical. Because unconjugated bilirubin is soluble n fat and cannot be excreted in the urine or bile, it can escape to extravascular tissue, especially fatty tissue and the brain, resulting in a condition called hyperbilirubinemia. Unconjugated hyperbilirubinemia is the cause of Gilbert’s Syndrome.
Do You Have Gilbert’s?
The disease is mainly asymptomatic.However, certain signs and symptoms like the following can help in identifying the disease:
- Yellow tinge to the skin and whites of the eyes.
- Abdominal discomfort.
- Abdominal pain in the right quadrant.
- Increased blood levels of bilirubin.
- Mild jaundice
Does It Require Treatment?
Gilbert’s is a harmless disease. However, the recent awareness of increased toxicities with certain drugs, especially Irinotecan, has woken up the interest in this disease. Congenital unconjugated hyperbilirubinemia have parents other than Gilbert’s Syndrome – like haemolysis (premature destruction of red blood cells) – and their diagnosis is entertained if bilirubin levels are high. Hence patients with unconjugated hyperbilirubinemia are also checked for haemolysis. The bilirubin levels in hemolysis usually do not exceed 5 mg/dl. As Gilbert’s is a common disorder, sometimes haemolyis and Gilbert’s may co-exist. In these situations, bilirubin levels are higher.
Are Medicines Needed?
Administration of Phenobarbitone improves bilirubin levels. Certain drugs like Rifampicin, Oestrogens (in birth control pills), Paracetamol and certain HIV drugs as Indinavir may increase bilirubin levels. The intake of the cancer drug Irinotecan in Gilbert’s can cause severe toxicities like diarrhoea and bone marrow suppression.
Gilbert’s syndrome per se does not require to be treated. It is important to be aware of the condition though, and reassuring the patient of a recovery is mostly all that is required.