Most doctors have never heard of Familial Dysautonomia (FD). While many doctors are open to learn from and collaborate with an FD family, there are some that do not listen. Unfortunately, when a doctor does not heed instructions provided by parents of an FD child, the doctor can put that child in medical jeopardy.
A few years ago, our son, Andrew, a young adult, was scheduled for an endoscopy at a hospital. Prior to this outpatient medical procedure, my husband and I met with the anesthesiologist to review all medication options. We told the doctor to treat Andrew as if he were taking an MAOI (monoamine oxidase inhibitor, a class of medication) and supplied research confirming that Andrew’s body is MAO- depleted due to FD. Why the warning? Because any medication that is contraindicated with an MAOI can cause a life-threatening hypertensive crisis in an FD patient.
The three of us agreed upon two safe anesthetics. However, unbeknownst to us, the doctor used a third medication, Lidocaine. Lidocaine is contraindicated for anyone who is MAO compromised. When Andrew woke, he immediately started retching and went into a full-blown hypertensive crisis, with a blood pressure of 189/140, heart rate in the 150’s, and violent retching. When we approached the doctor about his error, he completely disregarded our fear and outrage, emphatically shouting “Lidocaine cannot cause this reaction!” To the detriment of our son, he was wrong.
Doctors need to listen. Scheduling a routine test, like an endoscopy, for an extremely medically fragile child raises the already high level of parental vigilance to the next level. Knowing that your child will be treated by someone who does not understand or have experience with the FD body is scary, especially when a doctor, ignorant of necessary FD patient precautions, could cause harm. Parents must explain to doctors that each medication, e.g., Lidocaine, must be cross-checked to make sure it is not contraindicated for those taking an MAOI. The good news is that if you have a collaborative doctor willing to learn about this rare disease, you can avoid unnecessary danger.
To learn more about MAO deficiency in those with FD click here.
Ann Slaw, JD
President, FD NOW
Parent to young adult with Familial Dysautonomia