Fabry Disease and Anesthesia
People with Fabry disease may need, at some point, to undergo medical procedures that require anesthesia. Here is information about the different types of anesthesia, how Fabry disease may complicate their use, and precautions doctors should take before and while using them.
What is anesthesia?
In general anesthesia, doctors give the patient medication intravenously or via inhaled gases, or a combination of the two, to render them unconscious and unresponsive to pain. They continuously monitor the person, watching for changes in blood pressure, oxygen levels, breathing, heart rate, and the like.
In regional anesthesia, anesthesiologists inject a nerve-blocking agent into a peripheral nerve or the spinal column of an awake patient, in order to prevent pain signals to a large region of the body, such as the lower torso or a limb.
Local anesthesia is applied to a small part of the body, such as the gums during dental procedures or the skin when getting stitches. It can be given as injections, sprays, or ointments.
Sedation is used for minor surgeries that require more than local anesthetics. It can vary in strength, and range from making patients feel slightly drowsy to affecting their ability to remember the procedure. Sedation is typically combined with painkillers.
When might a Fabry patient need anesthesia?
People with Fabry disease require anesthesia for the same procedures as the general public. But they may need it for procedures specific to this disease, such as:
- removal of skin lesions, called angiokeratomas (small red or purple wart-like structures)
- corneal opacity (scarring of the cornea, causing it to become cloudy)
- cardiac surgery to repair a valve, install a pacemaker, or for a heart transplant
- surgical intervention for a stroke
- correction of arteriovenous fistulas (abnormal connections between arteries and veins)
- kidney transplants
- procedures related to fractures or injuries sustained in a fall or seizure
Possible complications with Fabry disease
General and regional anesthesia may present issues for Fabry patients, due to the multiple organ systems that the disease affects. Those with heart disease and kidney dysfunction are at greater risk of complications.
Patients may also be more prone to hemodynamic impairment (lack of blood flow to the brain). Anatomical facial features of Fabry patients, such as prognathism (jutting lower jaw), may make airway intubation more difficult or interfere with mask ventilation.
What precautions should doctors take?
As Fabry affects many different organs, doctors should recommend preoperative evaluations of organ involvement, with particular testing of the brain, heart, lungs, and kidneys. They should also screen patients for possible airway complications prior to surgery.
Finally, they should be careful of patient positioning during procedures to limit the risk of hemodynamic impairment or of aggravating pre-existing pain.
In patients with organ dysfunction, regional anesthesia may be safer than general anesthesia. If general anesthesia is necessary, patients should be monitored closely by an experienced team, preferably at an institution that is familiar with Fabry disease.
Last updated: Oct. 23, 2020
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