*Hospital Management Guidelines - HypoKPP - Periodic Paralysis International

Patient’s Name: ______________________________________________

Birthdate: _________________________Height: ____________Weight: _______

Address:________________________________________________________

_________________________________________________________________

Primary Physician: _____________________________________________

Physician Phone: _______________________ FAX: ______________________

Emergency contact: Your emergency contact should be someone who can speak for you when you cannot advocate for yourself.

Name: ____________________________________________ Relationship: ________________________________________

home phone# __________________ cell #______________________

Name of Pharmacy:

phone#: ______________________ FAX# ___________________________

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: Low carbohydrate (under _______ grams daily), high protein, low sodium diet.

Normal range of K+ when asymptomatic: _________________________

Target range of K+: ___________________________

Medications: Including dosages and timing of doses: _______________

___________________________________________________________________

Medications to Avoid: Epinephrine, drugs which reduce serum K+. Antibiotics which reduce neuromuscular transmission: aminoglycosides: Strepto-, Neo-, Kana-, and Clindamycin Macrolides: Erythromycin, Telithromycin, Azithromycin; Fluoroquinolones: Ciprofloxacin and others

Food and Drug Allergies:

Pain Control:

Potassium Supplementation: (Describe your schedule of K+ administration or other management.)

Monitor K+ frequently as needed. Labs should be stat and avoid use of tourniquet if possible.

Cardiac Involvement: Monitor cardiac activity and respiration.

IV USE: for maintenance when potassium is not the primary issue: Avoid IV use if sufficient fluids, medication and K+ levels can be maintained orally. IV K+ must be administered in dilutant. Avoid glucose drip, use dilute saline or Ringers.

General Anesthetics: In patients with HypoKPP, hypothermia, hypokalemia, sodium chloride and glucose infusions as well as myotoxic substances like succinylcholine in the OR often lead to flaccid muscle weakness and respiratory distress in the recovery room [Siler and Discavage 1975, Melnick et al. 1983, Rollman and Dickson 1985, Lema et al. 1991]. The hypokalemia is induced by operation-induced stress that leads to K+ uptake into muscle via release of catecholamines, insulin, and other hormones. Keeping the patients warm and serum K+ at high level and avoiding hyperglycemia are essential measures in preventing such attacks [Lema et al. 1991]. Patients often take longer to emerge from anesthesia and may experience post-anesthetic paralysis and an extended period of post-anesthetic weakness. While there is no standard protocol for anesthetic use in HypoKPP, 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.

Local Anesthetics: USE NO EPINEPHRINE Lidocaine works inconsistently in many PP patients, and not at all in some patients. If lidocaine does not work use an alternative such as articaine, bupivacaine or mepivicaine without epi. In children the injection site may be numbed with Emla Cream to reduce syncopal response to injection.

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.

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

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

Notes: