URGENT Info for Emergency Room Personnel and Others

File folder with "URGENT" stamped on it in red

When FOD Families call 911 or come into an ER please FOLLOW the EMERGENCY PROTOCOL LETTER IMMEDIATELY that their specialist has given them and LISTEN to the PARENTS or FOD Adult! We are having some of our Families thrust into bereavement all because someone CHOOSES to BLATANTLY DISREGARD the Protocol and send Families home because their child/adult ‘looks’ okay or they think the blood sugar is sufficient [FOD children/adults can often present with symptoms even when the BS level is in the 70s and 80s]. DO NOT ASSUME you know how to treat FODs, especially if you have never treated an FOD individual in crisis ~ it is NOT treated like diabetes.

FOLLOW THE PROTOCOL that their specialist has individualized specifically for them!

**Why Emergency Protocols are NEEDED and why they NEED to be followed by Chuck Hehmeyer, practicing attorney

If hospitalized [ie., for illness, surgery etc], it is imperative, according to FOD specialists, that a 10% dextrose IV (D5% is NOT enough) is started immediately following blood chemistry samplings ~ waiting hours for the results before putting in the IV can be fatal when an FOD child/adult is in crisis. The 10% dextrose/glucose gives NEEDED FUEL to the brain and body that normal saline IV cannot provide. Also note that even though the child/adult may appear to be hydrated, it does NOT mean they are not heading toward a crisis ~ they may have fluids onboard, but they NEED CALORIES to help them prevent and/or get through a metabolic crisis/stress. Many experts also recommend the use of carnitine (Carnitor® or Levocarnitine – prescribed drugs) and if one cannot keep oral carnitine down due to vomiting, there is an IV carnitine available for emergencies.

Too many children/adults have been sent home from the ER because they didn’t LOOK sick ~ those with FODs do not have any kind of ‘look’ so INSIST that the blood and urine chemistries are done to determine what is going on INSIDE their bodies! We have had some NEEDLESS DEATHS due to being sent home TOO SOON!

[From our FOD Experts] A plasma glucose in the “normal range” does not mean it is safe to skip a D10 bolus if a medical protocol instructs that a bolus is necessary. When a patient is ill, some FODs require a glucose infusion even in the face of a glucose level that is normal or near normal as toxic metabolites can still accumulate.  Sometimes only a glucose bolus will reset the metabolic “thermostat” which regulates breakdown of endogenous (stored) fat. If a physician has concerns about a glucose bolus for a sick FOD patient based on a normal/near normal plasma glucose level, the answer is NOT to ignore the protocol. The patient’s treating metabolic physician must be consulted immediately or, if unavailable, another metabolic physician should be paged. For many FODs, it is much easier to deal with transient hyperglycemia than the consequences of continued decompensation.

Note: Specialists also advise to Avoid fat binding/producing anesthetics, lactated ringers, long term use of steroids, and products that contain aspirin or salicylates. These can cause possible complications.

Anesthesia Concerns: Propofol Infusion Syndrome

Emergency Protocol Letter Examples

Please be aware that the above Protocol Letters are just EXAMPLES that some of our Families have shared with our Group. It is important that you INDIVIDUALIZE your/your child’s protocol sheet in conjunction with your physicians and other professionals. Please also refer to the URGENT INFO posted above for Emergency Room personnel.