Hospital Management Guidelines for Normokalemic Periodic Paralysis Patients

Hospital Management Guidelines for Normokalemic Periodic Paralysis Patients

Patient's Name:

Birthdate: ________Height: _______Weight: _______

Address:

Primary Physician:

Emergency contact:  Your emergency contact should be someone who can speak for you when you cannot advocate for yourself.
Name:                                                                               Relationship:
home phone#                                         cell #                            FAX:

COMMUNICATION: If the patient is unable to speak and can blink, ask yes or no questions, say, "Blink once for yes, twice for no."

PRECAUTIONS: Place patient in coma position to avoid aspiration during weakness/paralytic attack. Weakness can rapidly progress to paralysis and respiratory failure, monitor the patient closely. Paralyzed patients may appear to be unconscious, but are awake and aware. Do not assume they cannot feel pain or hear conversations.

Normal BP: ________Normal heart rate:_______

Common symptoms during attacks;

Age at onset of symptoms;

Frequency of attacks;

Genetic mutation if known;

Family history?

Other Diagnoses:

Dietary Requirements: High carbohydrate, high protein diet

Normal range of K+ when asymptomatic:

Target range of K+:

Medications: Describe your schedule of medications and/or other management (such as high carb snacks)

MEDICATIONS TO AVOID: 

Food and Drug Allergies: a) b) c) d)

PAIN CONTROL:

CARDIAC INVOLVEMENT: Monitor cardiac activity and respiration.

IV USE: for maintenance when periodic paralysis is not the primary issue: Avoid IV use if sufficient fluids, medication and hydration levels can be maintained orally. Saline with added glucose is the the preferred suspension for IV if the patient is normokalemic;

GENERAL ANESTHETICS: RISK of MALIGNANT HYPERTHERMIA: Patients with Periodic Paralysis are more than usually susceptible to malignant hyperthermia. During surgery, avoid use of depolarizing anesthetic agents including suxamethonium and anticholinesterases that increase myotonia, which can result in masseter spasm and stiffness of respiratory and other skeletal muscles, and interfere with intubation and mechanical ventilation. Avoid all halogenated ethers (isoflurane, ethrane, sevoflurane, and desflurane) as well as halothane (which is an alkane). All are potential triggers of MH.

a) patients with any form of Periodic Paralysis often take longer to come out of anesthesia and may experience post-anesthetic paralysis and an extended period of post-anesthetic weakness.

b) While there is no standard protocol for anesthetic use in NormoKPP, the following have worked for some patients: Propofol, Fetanyl, Versed - Rocuronium, Toradol.

OPERATING ROOM: TEMPERATURE CONTROL: Chilling is a primary trigger in all forms of periodic paralysis. Patient should be kept warm during procedures, but both chilling and overheating are attack triggers in many patients.

MOVEMENT:

a) If feasible arrange for breaks from electronic monitoring (or other items that inhibit movement) periodically to enable patient to move around to lessen and/or prevent attacks.

b) If confined to bed periodically encourage as much movement of limbs as possible from bed to mitigate/recover from weakness, and prevent embolus.

Physician's List: (Include any that apply with contact information)

Pharmacy:                   phone#:                                             FAX#

Notes: