FOP and you! Or...well Chloe!

[Chloe Andreu]

Chloe has not yet been diagnosed with Fibrodysplasia Ossificans Progressiva - so let's break it down what the disorder is, and how you can discover she has it.

WHAT THE HECK IS FOP?

Fibrodysplasia ossificans progressiva or FOP is a disorder in which soft connective tissue (muscles being the best example) turns into bone.

The process of ossification is defined as "the hardening or calcification of soft tissue into a bonelike material". This natural process occurs in children when they're skull is forming from soft cartilage into hard bone material.

In FOP, this manifests starting from the upper body and moving throughout the lower body. Every tear in someone's muscle structure causes the body to "heal" the wound by closing it with ossification, turning the gape from the wound into solid bone. These excess bones can be surgically removed, but only more bone growth occurs after a surgery.

People with FOP may not experience any excess bone growth for month or years at a time. When excess bones are growing, it is called a "flare-up". Flare-ups are painful, and usually accompanied by a fever. Flare-ups last usually for a month and a half to two months, and medicine is often prescribed to limit the pain.

When a flare-up is in progress, joint stiffness may occur overnight. The stiffness comes from swelling and pressure inside the muscle during the earliest stages of new bone formation.

FOP never goes away and is not curable. People are born with FOP, but extra bone may not be present at birth or recognized. Bone created by FOP does not disappear, and the condition worsens as people grow older.

Bone growth can happen without warning or from a serious bump or fall. However, there are times when even after serious trauma, FOP does not manifest itself! The disease is active and dormant at various times, though current reasons are not known.

FOP affects the neck, spine, chest, shoulders, elbows, wrists, hips, knees, ankles, jaw, and many areas in between. The progression of ossification follows a characteristic pattern. Usually extra bone forms in the neck, spine, and shoulders before developing in the elbows, hips and knees. The muscles of the diaphragm, tongue, eyes, face, and heart are characteristically spared. The well-documented and characteristic progression of FOP, as well as the regions not affected, likely hold important clues to the cause and development of the disease.

The time and rate of extra bone formation changes from person to person, as well as changes in how it limits movement.

Ex. an elbow could be ossified into a closed position, putting the hand permanently across the chest, or the elbow may be left with some range of motion.

FOP does not affect a person's intelligence or cognitive abilities. It does, however, negatively impact the child's ability to safely access the school premises, use and manipulate the school's materials, and function academically in a typical way.

What can be done to help a person with FOP:

1. Make sure tissue is collected during appropriate emergencies.
2. Arrange for bone marrow to be removed from the person within an appropriate time frame (before it has grown or solidified).

Certain risks to be cautious of:

Patients who have FOP are particularly susceptible to complications from the flu. This is due to the severe restrictive disease of the chest wall that occurs at an early age and leads to the subsequent increased risk for developing life-threatening complications of respiratory infections.

SIGNS OF FOP CURRENTLY IN CHLOE THAT HAVE NOT BEEN RECOGNIZED:

• Chloe is actively having a flare up
  
• She has just endured physical trauma from falling out of tree and shattering an upper vertebra of her back.
  
• Hidden beneath her pink slippers, Chloe has malformed big toes which are curled oddly against the other ones, must like what occurs in Chinese foot binding. Her toes seem welded together and she cannot move them.
  
• Excess bone growth (ossification) has begun on her upper spine and neck, causing her an inability to move them. X-rays will need to be taken to notice this.
  

CHLOE'S CURRENT SYMPTOMS THAT ARE BEING ANALYZED:

• She is running a fever
  
• Stiff neck, x-rays not currently taken.
  
• Inflammation is evident on her back
  
• Flush face

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CHLOE'S CURRENT DIAGNOSIS:

- None, believed to be recovering from her surgery. Her surgery took place to fix what was believed to be splintering of bone from her fall.

POSSIBLE INCORRENT DIAGNOSIS:

• Allergic reaction to the morphine
  
• Infection
  

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How to Reply:

In character, feel free to bicker over a possible incorrect diagnosis. Always put the incorrect diagnosis in bold tags so I make sure to put it on the list. Though this would probably be most fun to do in a roleplay thread. Merely copy and paste a quote your character said regarding Chloe here, and I'll translate it over to the list.

I don't want people to immediately guess she suffers from FOP. So do give it an honest House-y game of "No, that CAN'T be right!"

In jest, I'd like to say that FOP could easily be mistaken for Lupus.

Yes. That's right. The answer to everything: lupus. xD