Fibrodysplasia Ossificans Progressiva

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  • Topic: Fibrodysplasia ossificans progressiva, Connective tissue, Myositis ossificans
  • Pages : 9 (3011 words )
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  • Published : April 19, 2013
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Abstract
Fibrodysplasia Ossificans Progressiva (FOP) is a disorder with many names. It is also known as Myositis Ossificans Progressive, Munchmeyer disease, or just casually Stone Mans Disease. The term Myositis Ossificans, is favored to the term Fibrodysplasia Ossificans Progressiva, because the ectopic osteogenesis occurs in the connective tissue within muscles, fasciae, tendons, ligaments, and joint capsules rather than in the muscle fibers themselves. It is a very rare autosomal dominant disorder that is characterized by swelling of the muscles, which is very painful for the patient, and also swelling of the connective tissues. It is typically diagnosed early, within the first few weeks of life, because of the congenital deformities of the big toes, and heterotopic progressive osteogenesis, which are two key features that are typically present at birth. Early diagnosis and avoidance of trauma are the key principles in the effective management for these patients. It is particularly disabling in children, and the prognosis for the disorder isn’t very promising. This disorder only promises increasing disability throughout their life, with most FOP patients being wheelchair-bound by the young age of 30. There is no effective treatment for FOP, therefore it is vital for the patient to avoid any soft tissue trauma including biopsies, IM injections, surgical procedures, and even mandibular blocks for dental procedures, as well as any viral illnesses should be avoided if at all possible. Examples of any of these sources may bring episodes of rapidly progressive heterotopic ossification that could possibly result in permanent loss of function to the area that incurred the damage.

Imagine a person being slowly trapped inside their body, BY their own body. Their bones are quietly encasing their organs in a slow, unpredictable dance of a growing, encompassing, stone-like cage made of their own bones. Every bump, every scrape, every bruise or disturbance sustained to their body, has the potential to mummify them into a mass of muddled cartilage, muscle and bones; an eternally deformed and restrained body.

One might wonder what could possibly bring about such a debilitating and incapacitating condition. What exactly is it, and how can you treat it, or stop it? The questions that arise are many, including who is at risk, and what does the disease entail? One thing is known for sure, for those diagnosed with Fibrodysplasia Ossificans Progressiva that is what their future looks like: trapped by an ever enclosing cage of bone, produced by their own seemingly malicious body.

Fibrodysplasia Ossificans Progressiva, or FOP for short, is also known as Munchmeyer Disease, and is sometimes referred to as Stone Man Syndrome. Another scientific name for it is Myositis Ossificans Progressiva, and is a preferable term because ectopic osteogenesis (bone growth) occurs in the connective tissues within muscles, fasciae, ligaments, tendons and joint capsules, rather than in the muscle fibers themselves (Camargo, Moura-Ribeiro, Nucci, Queiroz, Santos, 2000).

Basically, what that means is that whenever an injury has taken place, the soft tissues of their body slowly (but sometimes rapidly) start to turn into bone. The soft tissues involved can be muscle, tendons, ligaments, and other connective tissues. This process of osteogenesis or growing excessive bone may take months, or even years, but eventually those afflicted with this disease will find that they aren’t able to move certain parts of their body anymore, and they become a person made of ‘stone’. Many of them, if not most patients become so overcome with excess bone growth that they are bedridden by time they are in their 30s (Schwartz, Majewski & Majewski, Jablonska, (2011).

When a child is born with Fibrodysplasia Ossificans Progressiva, an astute clinician that is knowledgeable about FOP should be able to diagnosis the condition, because there are very...
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