Attack triggers

1. Introduction
2. Trigger overview
   

1. Introduction

In the literature, a host of triggers are mentioned that need to be avoided in the case of CPT 2 deficient patients, otherwise a rhabdomyolytic attack may occur. Conversely, if a rhabdomyolytic attack is already in progress, then it is essential that all triggers that are present and active should be contained, restricted or eliminated.

Please note that it is not the case that people invariably will get an attack if one of the triggers mentioned is at play. Nevertheless, prevention is always better than cure, and CPT 2 deficiency patients and their doctors should keep these triggers in mind, and avoid them if possible, or otherwise treat them.

2. Trigger overview

A (potential) trigger for a rhabdomyolysis attack in CPT 2 deficient patients is, in general, anything that increases the dependance on lipid metabolism. The following attack triggers are mentioned in the literature:

Illnesses:
Infections (viral)
Fever

Physical:
Prolonged physical exertion
Exposure to cold
Sleep deprevation
Heavy injuries
Operations (and other emergencies)

Dietary:
Fasting
High fat intake

Drug-induced (see further):
General anesthesia (most drugs, but not all)
Common drugs, such as Ibuprophen, Paracetamol, Diazepam
Alcohol

Emotional issues:
Stress
Trauma
Operations (and other emergencies)

In the first place all such situations will challenge an individuals (fat) metabolism.

They may also require medication, emergency measures and operations.

It should be born in mind that there are a host of medications that could trigger a rhabdomyolytic attack. The following are mentioned in the literature:

(from E.B. Larbi, MB, PhD, FRCP)

TABLE 1. Drugs which can cause rhabdomyolisis.

Antipsychotics and antidepressants
Amitriptyline²⁴
Amoxapine
Doxepine⁵²
Fluoxetine⁵³
Fluphenazine⁵⁴
Haloperidol⁵⁵
Lithium⁵⁶
Protriptyline⁵⁷

Phenelzine⁵⁸
Perphenazine²⁴
Promethazine⁵⁹
Chlorpromazine
Loxapine⁶⁰
Promazine⁵⁹
Trifluoperazine

The particular issue of concern is the use of certain anesthetics (such as mentioned in the above) that can trigger a similar response as in patients prone to Malignant Hyperthermia (MH).

Like in MH these anesthetics should be avoided in patients with CPT deficiency and the same protocol followed as with MH patients.

The protocol that follows is taken and modified from the protocol given by MHAUS (the Malignant Hyperthermia Association of the US)