Emergency Care

Anaesthesia and peri-operative care in the primary Periodic Paralysis Disorders

Adverse reactions to anaesthesia, including life-threatening events, are a serious concern for patients with Periodic Paralysis Disorders. Many of our patients report serious events associated with anesthesia and side effects which may linger for days to months.

There is rarely time for a surgical team to comb through journals and texts for the specialized knowledge needed to care for the patient with periodic paralysis.

Converting Mg of Potassium to MeQ or MmoL

 

Source: Levitt, Jacob O, Practical aspects in the management of hypokalemic periodic paralysis. Journal of Translational Medicine 2008, 6:18 doi:10.1186/1479-5876-6-18:

Akut behandling af et hypokalemisk paralytisk anfald

Akut behandling af et hypokalemisk paralytisk anfald

Første skridt:
1. Åndedræts- eller hjerteanfald er mulige
2. Tjek hjerte og vejrtrækning - dilatation i hjertet kan forårsage høj puls og lavt blodtryk.
3. Foretag måling af hjerterytme/EKG.
4. Mål elektrolytter for at bestemme serum K+
5. Giv ilt - blodprøve fra fingeren afspejler måske ikke iltmætningen pga. dilatation
6. Anbring patienten som ved koma for at undgå kvælning.

Hospital Management Guidelines for Paramyotonia Congenita Patients

 

Hospital Management Guidelines for Paramyotonia Congenita 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:

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:

Severe respiratory phenotype in a patient with HypoKPP

Severe respiratory phenotype caused by a de novo Arg528Gly mutation in the CACNA1S gene in a patient with hypokalemic periodic paralysis.

Authors Kil TH, et al.

Journal: Eur J Paediatr Neurol. 2010 May;14(3):278-81. doi: 10.1016/j.ejpn.2009.08.004. Epub 2009 Oct 12.

Affiliation: Department of Pediatrics, College of Medicine, Konyang University, 685 Gasoowon-dong, Su-goo, Daejun, Choongnam 302-718, South Korea.

A Quick Overview of Hypokalemic Periodic Paralysis

 

1) Patient has "attacks" of weakness and/or floppy paralysis.

2) First attacks occur by the age of 16 in 65% of patients.

Hospital Management Guidelines for Thyrotoxic Periodic Paralysis Patients

Hospital Management Guidelines for Thyrotoxic 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:

Emergency Care for an Attack of Andersen-Tawil Syndrome

First Actions:

  • Respiratory and cardiac arrest are possible.
  • Call for Cardiac Monitoring/EKG
  • Draw electrolytes to determine serum K+
  • Provide 02 as thimble may not reflect 02 saturation due to cardiac dilitation
  • Lie patient in coma position to avoid aspiration.

Anesthesia in Hypokalemic Periodic Paralysis

Provided by Frank Lehmann-Horn MD, PhD

From
Lehmann-Horn F, Rüdel R, Jurkat-Rott K. Chapter 46: Nondystrophic myotonias and periodic paralyses. In: Myology, edited by AG Engel, C Franzini-Armstrong. McGraw-Hill, New York, 3rd edition, 2004, pp. 1257-1300.
and
Klingler W, Lehmann-Horn, Jurkat-Rott K. Complications of anesthesia in neuromuscular disorders. Neuromuscular Disord, 15:195-206, 2005.

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