Stone military man disease is a very rare and very debilitating status thought to affectapproximately one in a million people in the US . As the name advise , the disease causes the body ’s soft tissue to harden , essentially trammel patient role in a 2d skeleton .
What Is Fibrodysplasia Ossificans Progressiva?
Also referred to as Münchmeyer disease or its scientific name fibrodysplasia ossificans progressiva ( FOP ) , stone serviceman disease stems from a mutant in the factor ACVR1 , which causes heftiness and connective tissue to be replaced by bone tissue over time – a process known as ossification . This in effect cease up make a second frame that curb cause , render a person permanently immobile .
What are the symptoms of Fibrodysplasia Ossificans Progressiva?
While symptoms progressively exacerbate , it is a congenital condition , meaning it is present from birth . ill-shapen bounteous toes are often the earliest sign of the disease but ossification first occurs in childhood , most commonly between the ages of 3 and 5 . The neck , back , breast , arms and legs are typically the area first affected .
From childhood , patients begin to experienceflare - upsthat can cause swelling and pain as well as stiffness , fever , sluggishness and reduced mobility . These flare - ups may be triggered by rubor , illness , and trauma and frequently lead to extra ossification of the tissue . Because the precondition is reform-minded , mobility decreases over time and most will need a wheelchair before they plow 30 . What ’s more , ossification in the sass can lead to difficulty corrode and speaking , while hearing loss is cover inup to a fourth of patients .
The disease can bear on anyone , regardless of sex , race and ethnicity , and while there have been example where a factor has been inherited from a parent , it most often occur in multitude with no family history of the circumstance .
As of yet , there is no cure for endocarp man disease . However , harmonise toPenn Medicine , there are anti - inflammatory medicament , such as adrenal cortical steroid , that can help to relieve some of the symptoms . More recently , the US Food and Drug Administration ( FDA)approved the use of palovarotenein fount of FOP aftera studythat found it reduced rates of ossification by 54 % equate to the control group . It is the first treatment for the disease .
