Treatment and therapy
Meulengracht’s disease cannot be cured in principle, as the metabolic disorder is genetically inherited and congenital. In most cases, those affected manage well even without treatment and do not require therapy. In severe cases, the symptoms and gastrointestinal complaints can be treated with medication, although the side effects of the prescribed medication are often more serious than the symptoms themselves.
Usually, the symptoms occur in relapses and subside quickly without treatment. However, those affected can ensure that the symptoms occur only rarely and in mild form. The eyes discolored by jaundice in particular are extremely stressful for patients.
By drinking enough, the body is “flushed” properly and the accumulated bilirubin can be excreted more quickly via the kidneys. The symptoms are caused more severely by certain behavioral patterns. An appropriate lifestyle, in which patients do not smoke, avoid alcohol and long periods of hunger and sleep sufficiently, prevents an increase in the bilirubin concentration in the blood and the associated symptoms. As a result, the disease can be well controlled and the health impairment remains low.
Bilirubin value
The concentration of indirect bilirubin is increased in Meulengracht’s disease and is above the normal value of 2-5 mg/dl. Indirect bilirubin is bilirubin that has not yet been conjugated with glucuronic acid in the liver and is therefore not soluble in water. In the blood, the indirect bilirubin is bound to a specific transport protein, albumin.
To check the bilirubin value, the doctor takes a blood sample. Laboratory diagnostics determines the total bilirubin value and the value of conjugated bilirubin in a blood sample in the patient’s plasma (cell-free blood) or serum (plasma without coagulation factors). The concentration of indirect bilirubin is the difference between the total value and the value of conjugated bilirubin.
Duration and prognosis
Normally, Meulengracht’s disease is completely harmless and patients live completely unaffected by the disease. Drug therapy with enzyme inducers (e.g. phenobarbital or rifampicin) is usually not necessary and is rarely prescribed due to the undesirable side effects. Only a few patients unfortunately suffer from symptoms and in general, life expectancy is not reduced by Meulengracht’s disease, as hyperbilirubinemia does not cause damage to the internal organs.
