Danon – The Disease

Danon disease refers to weakening of the heart and skeletal muscles, along with intellectual disability. It affects men much earlier than women, and the severity is also much more in men. The symptoms of Danon disease usually start surfacing as early as one’s childhood or adolescent years in males. In females, the signs and symptoms mostly start showing in early adulthood. Danon disease life expectancy in men is 19 years, while females affected with this disease can live up to the age of 34 years. While the exact Danon disease prevalence is not known, it is a very rare condition, and as a consequence, not many doctors are aware of its diagnosis.

THE HEART OF THE MATTER:

Focusing on heart, the major symptom of danon disease is Cardiomyopathy. This occurs invariably in all men with the disease. Hypertrophic cardiomyopathy, which refers to the thickening of the heart muscle, is also a common symptom in men that leads to complications in the heart in pumping blood. Some men have dilated cardiomyopathy, another type of cardiomyopathy that is characterized by the weakening and enlargement of the heart, thereby keeping the heart from pumping blood in an efficient manner. Any of these cardiomyopathy types prove fatal. They cause heart failure and lead to premature death of the affected person. Women with Danon disease can also suffer from cardiomyopathy and out of those who do, half have dilated cardiomyopathy while the other half have hypertrophic cardiomyopathy.

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Since Danon disease affects the heart, heart-related symptoms are an obvious occurrence. Some signs and symptoms include pounding in the chest, chest pain or abnormal heartbeat. Many people with Danon disease also have conduction abnormalities. Intellectual disability ranging from mild to moderate levels is also common in males with Danon disease but rare in affected females. These symptoms are the very basic ones. There can be a host of other symptoms of Danon disease. For instance, many people with the disease suffer from breathing problems, gastrointestinal diseases or abnormalities in vision.

TREATMENT AND CURE:
As far as Danon disease treatment is concerned, no cure to it has been discovered yet. Danon disease treatment only helps in delaying death in patients or preventing premature and fatal heart failure. People diagnosed with Danon disease are required to get their heart function monitored on a regular basis for any symptoms of cardiac insufficiency. An implantable loop recorder is an effective way to monitor the heartbeat continuously, and it can work up to 14 months. An electrocardiogram is another way to check heart function.

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To prevent sudden death due to arrhythmia, doctors may prescribe an implantable cardioverter-defibrillator. As the name suggests, it is a device that is implanted in the body. It continuously monitors the heart and gives a shock in the event of an irregular heartbeat.

Along with these options, medications are also used to control Danon disease, according to the patient’s condition. Some of these medications are given with the aim of providing relief from symptoms. A long-term Danon disease treatment could be heart transplantation. This is an especially good option for young men to increase their life expectancy.

Also read: Heart Attack- Know the risk

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