Muscular dystrophy (MD) is a set of 30 genetic conditions in which individuals muscle get weakened, shortened and degenerated gradually due to lack of protein named dystrophin.1 This protein is essential for the growth, structure, and function of the muscles. Due to weakened muscles, the patient cannot move freely and may become dependent on a wheelchair. The disease ranges from mild to severe. It becomes severe and fatal when it progresses to heart and lungs resulting in heart failures and respiratory failures that may lead to sudden unexpected death. There is no cure for muscular dystrophy.
Is Muscular Dystrophy Always Fatal?
Muscular dystrophy is an inherited disease that causes disabilities due to weakened or degenerated muscles.1 It is a slowly progressive disease that affects usually voluntary muscles of the body. This happens because defective genes are passed to the individuals from one or both the parents. These genes codes information for the production of a protein named dystrophin. The main work of this protein is to build and maintain the healthy function of the muscles. When the body becomes deficient of dystrophin, then the muscles get weakened and shortened gradually. In a course of time, they get degenerated.
Muscular dystrophy is many times mild and involves only voluntary muscles such as muscles of extremities and tongue. This affects one’s ability to walk or move the extremities and swallow. The disease has slow progress to the degeneration and wasting of muscles. But it can involve involuntary muscles of vital organs such as the lungs and heart. This may cause inadequate functioning of heart and lungs. This may result in problems related to the supply of blood and breathing. The symptoms become worse with time and the affected individual may die due to lung and heart failures. Thus, muscular dystrophy is a fatal disease.
However, muscular dystrophy is not fatal always. In few types of muscular dystrophy, the course of the disease is slow and mild. However, it cannot be reversed or treated completely. But its symptoms can be managed with medical intervention. Many gene therapies are on trial to tackle the disease.
Muscular dystrophy can appear at any age and progress of the disease is gradual and slow. The severity of the condition depends on the age of onset, its type, the speed of progress, affection of heart and lifestyle of an individual.
The types of muscular dystrophy that can be fatal are-
Duchenne- It develops in early childhood in the age of 2-6 years. It leads to muscle wasting bounding the patient to use a wheelchair to move as the deformity of arms, legs and spine have developed due to muscle wasting.2 It can become more severe in the later stages as it can cause heart and respiratory ailments. The child dies in 15 -20 years of his age.
Becker- It begins in the 2-16 years of age. It affects males only and can cause ailments related to the heart. The patient dies in adult age.
Myotonic- it appears in between early childhood and adulthood. It leads to weakness of muscles and affections of the nervous system and other organs. Life expectancy of this muscular dystrophy is short
Congenital- it is present from birth. The affected child is born with impaired motor functions and muscle control. The disease can be mild or severe. Many of them die in infant age. Only a few survive up to adulthood.
Emery –Driefuss- this muscular dystrophy appears in childhood and causes muscle weakness, heart and breathing problems. The life expectancy of such patients is short up to mid-adulthood due to lung or heart failure.
Muscular dystrophy is a slowly progressive degenerative disease of the muscles. There are more than 30 types of muscular dystrophy. Every type of muscular dystrophy is not fatal always. The severity of the disease depends on the age of onset and speed of progress of the disease and others discussed above.