Visit www.ifopa.org for more information on FOP!
Gabriel and Angela are happy to answer any questions you have regarding Ali or FOP to the best of their ability, please reference the Contact Us tab. The following information was taken from www.ifopa.org, the International Fibrodysplasia Ossificans Progressiva Association. There is a ton of information on this website and we encourage you to view the website and gain knowledge on this severe disorder.
FOP Fact sheet (www.ifopa.org)
What is Fibrodysplasia Ossificans Progressiva (FOP)?
One of the rarest, most disabling genetic conditions known to medicine, it causes bone to form in muscles, tendons, ligaments and other connective tissues. Bridges of extra bone develop across joints, progressively restricting movement and forming a second skeleton that imprisons the body in bone. There are no other known examples in medicine of one normal organ system turning into another.
An example of the typical progression of FOP:
Spontaneous flare-ups of the disease arise in defined temporal and spatial patterns, resulting in ribbons and sheets of bone that fuse the joints of the axial and appendicular skeleton, entombing a patient in a skeleton of heterotopic bone.
How would understanding the cause of bone formation in FOP help others?
The information obtained from studying this disease will have far reaching implications for the treatment of common disorders such as fractures, osteoporosis, hip replacement surgery, and other forms of heterotopic ossification that occur in trauma and burn victims.
Demographics of FOP:
- Genetic disease affecting 1 in 2 million people
- No ethnic, racial, or religious patterns
- 700 confirmed cases across the globe
- 185 known cases in the United States
Clinical Characteristics of FOP:
- Characteristic malformations of the great toe
- Flare-ups occur spontaneously or following bodily trauma such as: childhood immunizations, falls while playing, viral illnesses
- Misdiagnosed in a majority of cases as cancer
- Surgery makes the condition worse
- There are no effective treatments
Finding a Cure and Treatment for FOP:
- Researchers at the University of Pennsylvania School of Medicine, the only laboratory in the US dedicated to FOP research, announced the Discovery of the FOP Gene in Nature Genetics in April 2006.
- 10,000 sq. ft. of shared research space in the Department of Orthopaedic Surgery
- 3 principal investigators with 15 post-doctoral fellows, students, scientists, and staff
- Funds spent on research - Approx. $1.5 million/year
- 75% from FOP family fundraising and donations
- 25% from institutional support (NIH/NIAMS, Orthopaedic Research and Education Foundation)
FOP FAQ (www.ifopa.org)
Q - What does FOP stand for and when was it first documented?A - FOP or Fibrodysplasia Ossificans Progressiva (Fibro-dis-playsha Os-sih-fih-cans Pro-gress-eva) means "soft connective tissue that progressively turns to bone." The earliest documented cases date back to the 17th and 18th centuries.
Q - How many people have FOP?
A - It is estimated that FOP affects about 3,300 people worldwide, or approximately one in two million people. Such statistics may be better grasped by the following example: if a large football stadium holds 100,000 fans, one would need to fill nearly 20 football stadiums to find one person who has FOP. At the present time, researchers are aware of approximately 700 people throughout the world who have FOP.
Q - Can the extra bone be removed?
A - The technical answer is surprisingly "yes," but that does not tell the whole story. Surgical intervention often results in a worsening of the condition. New bone will grow back and further impair mobility.
Q - How is the extra bone in FOP different from normal bone?
A - The extra bone in FOP forms by a progressive transformation of soft tissue into cartilage and bone. This is the same process by which bone regenerates (heals) after a fracture (break) occurs and is nearly identical to the process by which bone forms normally in an embryo. The abnormality in FOP occurs not in the manner of bone formation but rather in its timing and location.
Q - What is a flare-up and is it painful?
A - A flare-up occurs when the body starts to generate new bone. No one knows what initiates this process, but once it begins, it leads to tissue swelling and much discomfort. Sometimes the individual will not feel well and may develop a low-grade fever. While there is no medication or therapy that can stop the process of bone formation once it has begun, a physician can prescribe medicine to help relieve the pain. A single flare-up may continue for as long as 6-8 weeks. Multiple flare-ups may also occur during an active period of FOP. 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.
While flare-ups are usually painful, the degree of pain can vary. Most people find that the pain subsides when a flare-up is over; it appears that it is the process of extra bone formation, rather than the extra bone itself, that usually leads to pain.
Q - Will FOP get worse? Does it ever stop or go away?
A - Unfortunately, FOP does not improve over time. The "P" in FOP stands for "Progressiva." That means that FOP will progress, or get worse, as a person ages. As FOP is part of a person's genetic make-up, people with FOP are born with the condition, even though the extra bone may not have appeared at birth. So people with FOP will not outgrow the condition. Nor can the extra bone that has been produced by FOP disappear. The body of a person with FOP does not make extra bone all of the time; a person with FOP may go months or years without a flare-up. Yet there is always a chance that extra bone can form, either without any warning ("spontaneous flare-up") or following trauma, such as a bump, fall, muscle overexertion, an injury, intramuscular injections, surgery, or even some viruses. It is unclear why the disease is active some times and quiet or dormant at other times.
Q - What parts of the body are involved in FOP? How does FOP affect mobility?
A - 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.
FOP affects mobility because the body's joints, such as the knees or the elbows, connect the bones and aid in movement. In FOP, extra bone replaces the ligaments (which cover the joints), as well as muscles and tendons (which move the joints). Consequently, movement in areas affected by FOP becomes difficult or impossible.
Q - What physical variations are seen from person to person?
A - The largest variation seen is the timing and rate of extra bone formation. Another common variation includes the severity of movement restriction. For example, an elbow could be fixed in a closed position, putting the hand permanently across the chest, or the elbow may be left with some range of motion.**Attention to all current and future followers** We appreciate everyone's want to help and the offers that have been made, but we need to make it VERY clear, that all merchandise being sold on behalf of Ali to raise money for her medical needs is featured on her website ONLY. If you do NOT see it on her official website at www.alis-army.org, then it is NOT official gear and the purchase of said items do NOT benefit her! I would advise everyone to make sure that any FOP purchases you make for the cause are made through OFFICIAL websites, as we have several people trying to pick up on Ali's story and use it for their own personal gain. The ONLY legal site for Ali's donations will be www.alis-army.org from this day forward. We will NOT be using any other website or page, on the web. Please be sure when making a donation that the site you entered in actually reads www.alis-army.org once the page loads. I am shutting down our giveforward.com page today and will NOT be using it in the future. Unless you are on her official website, with us in person or mailing donations to the addresses listed on her official page, or at a fundraiser that was featured on her website you are NOT giving to Ali. As you also know donations can be made directly to IFOPA as well, their official website is www.ifopa.org. If you ever have any questions regarding someone's official page please contact us, we have the means to find this information for you as we are connected with the FOP community, and can ask the individuals personally. To all of our FOP'ers please feel free to post YOUR official websites or links here. We are happy to share them & are striving to prevent any misleading to our supporters. Anyone infringing on any FOP charities and using our families medical issues for personal gain will be punished to the full extent of the law. Have some compassion people, our child is suffering, numerous others are suffering, how dare you try to use someone's disability as personal gain for yourself! On another note if you would like to actually help in a LEGAL way we would love to hear from you and guide you in the right direction. If you ever have any questions please feel free to contact us personally. We would be happy to answer ANY and all questions that we can.