Guidebook Home

Table of Contents

Preface

Introductory Comments

General Questions About FOP

Genetics of FOP

How FOP Affects the Body

Care and Treatment

Activities

Feelings About FOP

Helpful Addresses

Family Resources

Ideas for Independence

Medical Articles

Acknowledgments and Contributions

A Guide for Emergency Situations Involving People with FOP

Brief Definition of FOP: Fibrodysplasia Ossificans Progressiva is a rare disease in which bone forms in abnormal locations, leading to stiffness, locking, and permanent immobility. Extra bone growth can occur without any warning or as a result of trauma, which can be as minimal as a bump or a fall. Malformations of the great toe are commonly noted at birth. Ectopic bone formation usually begins in the first decade of life and progresses episodically in characteristic anatomic patterns.

Emergency Precautions:
An emergency is a problem that needs immediate attention, and people who have FOP may have emergencies just like anyone else. But remember: no intramuscular injections! Subcutaneous injections, blood tests, and IVs are permissable. People who have FOP should avoid surgery to remove extra (ectopic) bone, but other types of procedures may be necessary and even life-saving. People who have FOP may develop a toothache, a tooth abscess, appendicitis, gall-bladder attack, heart attack, blood clot, fracture, infection, or head injury &endash; problems that need immediate attention and occasionally operative involvement in order to avoid more serious and potentially life-threatening complications. It is critical to seek medical attention and to make sure that these problems are properly evaluated and treated.

A good rule to follow is to have your local doctor or emergency room evaluate the problem as if you did not have FOP. Most emergencies that people with FOP have are not related to their FOP, but to common problems that anyone would have. After the problem is thoroughly evaluated, have your doctor contact one of the following doctors for further advice and consultation, if possible:

Frederick S. Kaplan, M.D. Medical Advisor, IFOPA 215-349-8727 (Work)	David L. Glaser, M.D.| Medical Advisor, IFOPA 215-312-8953 (Pager)
J. Michael Connor, M.D. Guthrie Institute of Medical Genetics University of Glasgow (Scotland, UK) 041-339-6996	Eileen Shore, Ph.D. FOP Laboratory Director University of Pennsylvania 215-898-2331
John Rogers, M.D. Victoria Clinical Genetics Service Royal Children's Hospital (Melbourne, Australia (03) 9348 1391

During the course of emergency evaluation, remember the following simple rules:

1. Intramuscular injections are generally not permitted as they may cause a flare-up of     FOP.

2. Medications can be safely administered subcutaneously or intraveneously, if needed.

3. Special care should be taken to avoid unneccessary trauma.

4. Please have your doctor contact one of the doctors listed above for further advice and     consulation during the evaluation process.