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

FOP and Life-threatening Emergency:
What to Do About Anesthesia?

Frederick S. Kaplan, M.D. and Michael A. Zasloff, M.D., Ph.D.

An emergency is defined as a condition in which irreversible harm or death can result if immediate care is not obtained. In FOP there are two major types of emergencies: those directly related to FOP and those that may occur in anyone.

The most common emergency directly related to FOP is that which threatens the airway. Other emergencies, such as those that may occur in anyone, include an infection or abscess requiring surgical drainage, acute appendicitis, severe injury requiring surgical attention, renal colic from a kidney stone, a bleeding ulcer, etc.

We will first discuss the management of a threatened airway in patients with FOP. Drs. Connor, Evans, and Evans reported in the journal Thorax (Vol. 36, pp. 419-423, 1981) that patients with FOP have marked restrictive chest wall disease that does not progress to chronic respiratory failure unless pulmonary infection develops. Dr. Connor reported at the First FOP Symposium in Philadelphia that complications related to pulmonary infection occurred less frequently now than they did in the era before antibiotics. Acute airway obstruction may occur rarely when a new FOP lesion involves the front (not the side) of the neck including the floor of the mouth, thus affecting the ability to swallow or breathe. During such an episode, a brief course of high dose steroids (prednisone) may be helpful in decreasing the swelling. The prednisone should be tapered and discontinued within three to four weeks (at maximum), as the steroids do not affect the ultimate appearance of new bone.

Longterm use of steroids in patients with FOP (as in any patient) can be associated with other complications. There are no studies that show the effectiveness of steroids in altering the course of heterotopic ossification in FOP, but steroids do appear to have some benefit in decreasing the acute swelling associated with new flare-ups. Generally, we do not recommend the use of steroids except in life-threatening situations when the swelling involves potential compromise to the airway. During such periods of neck swelling, semi-solid foods are safer than plain liquids, as they are less likely to "go down the wrong tube" and to be aspirated. If the swelling in the front of the neck is severe and begins to compromise breathing, then the patient may need to be hospitalized. In the extremely rare instance of airway obstruction resulting from severe swelling in the front of the neck (I have only heard of one such case in over 100 patients), then every attempt must be made to reestablish the airway, even if a surgical procedure such as a tracheostomy must be performed. Obstruction of the airway is an acute medical emergency in anyone and will rapidly lead to respiratory arrest and death unless remedied. In such an emergency, consideration of FOP comes second to consideration for reestablishing the airway and thus reestablishing the oxygenation of the blood. We must emphasize that this is an extremely unusual (if life-threatening) complication of FOP.

The other class of emergency involves the necessity of general anesthesia in an FOP patient. The best preparation for this unusual contingency is a well-informed patient and family and a well-prepared anesthesiologist. A suggestion that many have found helpful to reduce anxiety is to be better prepared for such a contingency. Ask your family doctor about obtaining an elective consultation with an anesthesiologist at your local hospital so that he or she may evaluate you and plan an approach for managing an emergency anesthetic situation should such a necessity arise. An elective anesthesia plan should be written out and filed in your hospital chart. A copy should be given to your family doctor and a copy should be retained by you, especially when you are travelling away from home. The anesthesiologist's note should include the warning that intramuscular injections should be avoided, if possible, and that care should be taken to avoid soft tissue injury and to pad bony areas during surgery to prevent pressure sores.

Several FOP patients have noted that elective removal of teeth once the jaw has fused may help provide oral access as well as help in eating and preventing aspiration of food. It is important not to undergo any aggressive dental procedure while the jaw is still mobile, as such procedures could rapidly lead to ossification about the jaw and further limit movement at the temporomandibular joints. However, once the jaw has fused, there is little additional risk of tooth removal; in fact, such procedures might help airway access and routine eating as previously mentioned.

Anesthesiologists are well-trained experts in handling emergency situations. Even if you have not had an initial evaluation, and even if your anesthesiologist has never seen a patient with FOP, a well-trained anesthesiologist is capable of rapidly evaluating difficult airway access. They will know how to deal with it. So, if an emergency should arise, and you have not previously seen or been evaluated by an anesthesiologist on an elective basis, do not panic. The anesthesiologist will be able to deal with it, much like an airplane pilot who has never flown a particular route will know how to deal with an emergency situation.

In summary, although surgery is generally avoided in patients with FOP, as it can stimulate new areas of heterotopic ossification, there are certain indications for surgical intervention and careful and appropriate airway management. Steroids (prednisone) should probably be used only for a brief period of time during an emergency of acute airway swelling from an FOP flare-up. Elective evaluation by an anesthesiologist is a prudent measure that might alleviate considerable anxiety should emergency anesthesia ever be necessary. Of course, you or your physician should feel free to call Dr. Fred Kaplan with any routine or emergency questions. Dr. Kaplan's office phone number is 215-349-8727. If he is not in his office, please leave a message with his secretary Kay, and he or another member of our FOP team will promptly return your call.