Fibrodysplasia ossificans progressiva (FOP)

FOP is a very rare disease that causes parts of the body such as muscles, ligaments and tensions from turning into bones when they are damaged. This causes the joints commonly are based avoiding movement. Removal of ossification is ineffective because it grows back again similar to a healing process. The disease is rare that so commonly is misdiagnosed as a potential cancer, leading doctors to perform biopsies, which enhances the growth of bone callus. No cure exists.
The most famous case is that of Harry Eastlack had ossified your body so that the end of life just moved his lips. His skeleton is exposed in the Mütter Museum, USA.
Treatment
There is no specific treatment for FOP effective and thus the measures are aimed at symptomatic improvement and prevention of worsening of this condition.

All kinds of accidental trauma or caused to be predictable. These include:

1) surgeries for removal of heterotopic bone. They are absolutely contraindicated, since it can cause the development of more heterotopic ossification by tissue trauma.

2) intramuscular injections, including intramuscular immunizations and mandibular blocks for dental procedures.

3) Manipulation of the joints or aggressive physiotherapy.

4) Falls.

5) Contact sports physical.

The current management of FOP can be divided into two categories: the management of symptoms and management of the underlying disease. Although the main hope is to develop better treatment for FOP (1), there are some alternatives that can bring relief to acute symptoms. The research to find drugs that act in the FOP is very difficult due to the rarity of the disease. However, as the natural history of FOP is well known, small controlled therapeutic studies have been done to determine the risks and benefits of various medications. Another limiting factor in the study of FOP is the lack of appropriate animal models. The identification of the defective gene and development of therapeutic agents will provide considerable advance in the research of new drugs effective and beneficial.

Currently there are three classes of drugs that can be used for the symptomatic management of FOP:

Class 1 - Prednisone is the choice in the early stages of acute episodes of FOP. His thumb is more effective for acute exacerbations involving larger joints. Not to be used for the management of acute exacerbations of neck, back, or chest wall.

Their introduction should be done quickly, ie, within 24 hours after the onset of the episode in an attempt to inhibit the acute inflammatory infiltrate and roughage that is characteristic of this phase and which causes the death of skeletal muscle. The dose used is 2mg/kg/day in a maximum period of four days. It is important to remember that chronic use of prednisone has no practical use and can cause undesirable systemic effects. After 48 hours of the beginning of the episode, the benefits of prednisone are very low. After use of prednisone can be introduced nonsteroidal anti-inflammatory agents and inhibitors of leukotrienes (class 2). The cyclooxygenase-2 inhibitors have anti-inflammatory and antiangiogenic properties, although not conventionally used for patients less than 16 years, its use should be accompanied by tests for liver and kidney function.

Class 2 - Montelukast (Singulair ®), a drug leukotriene inhibitor may be used in doses of 5 to 10mg / day, may contribute to reducing the symptoms of inflammatory episodes, particularly in those over 48 hours of evolution. Can be used in conjunction with NSAIDs or COX-2.

Class 3 - This class includes drugs under development, which can only be employed after their approval for clinical use. These include new antiangiogenic agents and BMP antagonists.

Noggin is a potent inhibitor of BMP-4 and is a drug with intense research laboratory. Since the attempts and efforts to develop a cure for FOP are closely linked to the discovery of the altered gene, their discovery will make it possible to complete the analysis of the disease, test new drugs and more effective testing of existing ones.
Prognosis
The FOP patients are generally healthy and also intellectually normal. The main cause of death is lung infection disease severe restrictive chest wall. Currently, however, with the advancement of antibiotics, there are already patients in the sixth decade of life.