Hello All,
This is an article written and created with a compilation
of information, excerpts, references and links related to the problems and
issues of the use of anesthesia with individuals with the various forms of
Periodic Paralysis. There is quite a bit of technical information, which can be
shared with your doctor, anesthesiologist or dentist (though most of them
should know this information).
Periodic Paralysis and Anesthesia
Article and Compilation
by
The
Periodic Paralysis Network (PPN) February 2014
Periodic
Paralysis (PP) is a mineral metabolic disorder, also known as an ion
channelopathy, which is a dysfunction of the ion channels. There are four types
of PP, Hypokalemic Periodic Paralysis, Hyperkalemic Periodic Paralysis,
Normokalemic Periodic Paralysis and Andersen-Tawil Syndrome. Ion channels
transport the electrolytes, such as sodium and potassium through the cells.
This transport is faulty in individuals with ion channel dysfunction and
extreme care must be used when anesthesia is going to be utilized. This is due
to the possibility of developing serious symptoms such as breathing issues or
failure, arrhythmia, blood pressure issues, choking, muscle weakness or
paralysis, longer recovery after surgery, malignant hyperthermia or death.
Managing the use of anesthesia in individuals with Periodic Paralysis is mostly
aimed at preventing attacks of paralysis or the other symptoms during or after
surgery. The manner in which the situation is handled for the individual
depends on which form of Periodic Paralysis is involved. 1, 2, 3, 4, 5
Hypokalemic Periodic Paralysis and Anesthesia
For
individuals with Hypokalemic Periodic Paralysis, anesthesia is a known trigger
for paralytic episodes. According to research, in order to successfully manage
the patient there is need for an evaluation before surgery, avoidance of known triggers,
careful monitoring during the surgery and immediate and proper treatment if an
issue arises.
According to the National Journal of Maxillofacial Surgery, “pre-operative
stress along with necessary fasting and administration of dextrose containing
fluids” precipitates attacks. “The guidelines for care include control of
plasma potassium, avoidance of large glucose and salt loads, maintenance of
body temperature, acid-base balance, and careful use of neuromuscular blocking
agents. Good pre-medication to allay anxiety, avoidance of stress and adequate
analgesia is vital in preventing an attack. Fluctuations in electrolytes,
infection, and pain can lead to paralysis in the post-operative period.
Hypokalemia manifests earlier than paralysis and so its correction can prevent
paralysis. Dextrose containing solutions administered during surgery should be
avoided...” 6, 7, 8
This information was found in Miller’s Anesthesia written by Ronald D. Miller:
”Management of HypoPP patients should focus on avoiding triggers and
medications causing shift of potassium. General anesthesia, postoperative
stress, glucose-containing intravenous solutions, and long-acting neuromuscular
blockers are associated with postoperative paralytic events.138 Epidural
analgesia has been shown to reduce both pain-related hyperventilation and serum
catecholamines, thereby minimizing changes in serum potassium levels.138” 4
Hyperkalemic
Periodic Paralysis and Anesthesia
Nothing was written about the use of anesthesia and Hyperkalemic Periodic
Paralysis before 2002. Early research concluded that anesthesia might be used
without complications if the potassium levels were with-in normal levels prior
to surgery, if the carbohydrate levels were up, if anesthetic drugs, which
released potassium, were not used and if normal body temperature levels were
maintained.
This information was found in Miller’s Anesthesia written by
Ronald D. Miller:
”Potassium, cholinesterase inhibitors,
and depolarizing muscle relaxants will aggravate the myotonia in HyperPP
patients.65 Prolonged muscle weaken ss has been reported when succinylcholine
is used.141 Although one third of patients had no signs of myotonia,142
masseter spasm and respiratory and skeletal muscles stiffness could still occur
during intubation and ventilation.65 Therefore, neostigmine and succinylcholine
should be contraindicated in HyperPP patients. Ideally, all patients with
HyperPP need to be admitted preoperatively so that proper preoperative fasting
can be accompanied by the administration of dextrose-containing potassium-free
maintenance fluid.143 Postoperatively, HyperPP patients may remain paralyzed
for up to several hours. Preventive measures such as maintaining normal body
temperature and low serum potassium levels and avoiding hypoglycemia are
helpful in limiting such paralysis.144 Although patients with sodium channel
pathology have often been considered to be susceptible to MH, (Malignant
Hyperthermia) there is no increased risk for MH in these patients.145 General
anesthesia with and without nondepolarizing muscle relaxants has been shown to
have satisfactory outcomes.141,143,144,146 Regional techniques may also be
appropriate for this patient group.142,146 Abortion of the hyperkalemic attack
may be accomplished by administering glucose, insulin, epinephrine, and calcium
supplements, or alternatively, glucagon may be used. β-Adrenergic treatment
with metaproterenol has also been shown to prevent attacks and facilitate
recovery.” 4,9,10,11
Malignant
Hyperthermia (MH)
As mentioned previously, individuals with Periodic
Paralysis are at risk for developing malignant hyperthermia during or after
surgery. All forms of Periodic Paralysis are the result of mutations on
Chromosome 17. Malignant hyperthermia is also the result of a mutation on
Chromosome 17, thus creating the potential for those with Periodic Paralysis,
including, Normokalemic Periodic Paralysis, to develop the serious and
life-threatening symptoms involved with the use of anesthesia. 12, 13
The following information about MH is an excerpt from an
article from CINCH (Consortium for the Clinical Investigation of Neurologic
Channelopathies), which may be shared with physicians, dentists and
anesthesiologists:
Malignant
Hyperthermia
“Patients with
ion channel diseases are at increased risk of malignant hyperthermia reactions
with general anesthesia for surgery. In malignant hyperthermia, muscle cells
become overactive (hypermetabolic) in response to the most commonly used drugs
for general anesthesia. With inhaled agents (such as halothane, isoflurane,
enflurane, desflurane, sevoflurane and even agents such as ether, cyclopropane
and methoxyflurane ) the ion channels in the muscle cells open and remain open.
This results in excessive calcium release causing muscles to continuously
contract, producing massive amounts of heat, rapid rise in temperature and
disruption of nearly every organ system in the body. In the past this reaction
was fatal in 70% of cases. However, with the introduction of the medication,
dantrolene, the mortality has now fallen to 5% of cases. It is essential that
anesthetists and surgeons are made aware that a patient has periodic paralysis
or even that a parent suffers with the disorder, if a son or daughter is going
for surgery. The anesthetists and surgeons should be familiar with
neuromuscular disorders and the associated risks. If they are not in a
specialist center, the patient may need to be transferred to such a center or
at the least the patient's neurologist should be contacted prior to surgery.
Prolonged Paralysis
Other medicines used
during general surgery are called depolarizing agents (such as
Succinylcholine). These medicines act by blocking the signal from the nerve
(Acetylcholine) from reaching it's receptor on the muscle. This causes
temporary paralysis in patients during surgery, relaxing the muscles to make
surgery easier. In patients with ion channel disorders, such as periodic
paralysis, this can result in prolonged paralysis.
Normally,
when people are given the drug succinylcholine to relax muscles, a little bit
of potassium from inside the muscle cells leaks out into the bloodstream. This
is because succinylcholine, like acetylcholine, docks on the receptors and
opens some gates in the cell, letting certain molecules flow in and out.
Normally, this poses no problem. In the periodic paralyses, ion channels that
normally open when acetylcholine docks on membrane receptors don't function
normally, and people experience temporary paralysis attacks in response to a
variety of dietary, exercise, stress-related and other environmental changes.
After
surgery, patients with periodic paralysis have found they woke up in the
recovery room and couldn't move; they may not get full strength back for hours
or days. The exact cause of the prolonged paralysis with anesthesia in some
people with periodic paralysis isn't known. It could be the stress of the
surgery and/or any of the anesthetic drugs. It is critical for the medical team
to distinguish between a periodic paralysis attack and a malignant hyperthermia
reaction as the treatments are completely different.” 14,
15, 16
Important link for more information related to Malignant Hyperthermia"
Andersen-Tawil
Syndrome and Anesthesia
Some research
indicated that malignant hyperthermia is not usually an issue for individuals
with Andersen-Tawil Syndrome. However, it is an issue because individuals with
ATS have shifting of potassium into both high and low ranges causing symptoms
and paralysis. The other issue with anesthesia use and ATS is a need for
special precautions due to the serious issue of the long QT interval heartbeat,
a diagnostic marker for the condition and Torsades de Pointes another extremely
serious arrhythmia. There are many medications that must be avoided, which are
used routinely in preparation for surgery and during surgery including the
glucose and sodium IVs, as well as most forms of anesthesia.7
General Anesthestic considerations for patients with LQTS Management
From Drexel
University:
Avoid triggers of QT prolongation and Torsadesde
Pointes (TdP)
Provide Peri-op:
Anxiolysis
β-blockade
Analgesia
Maintain:
Normothermia
Normoxia
Euglycemia
Normocarbia
Avoid hemodynamic extremes:
Bradycardia
Tachycardia
Hypotension
Hypertension
Correct serum electrolytes esp:
Potassium
Magnesium
Prophylaxis beneficial even with normal serum
concentrations:
Prevent and treat arrhythmia:
Continue ECG monitoring in more than 1 lead
If ICD/pacemaker, ensure proper functioning
Have defibrillator and temporary pacemaker
available
Consult cardiology as
needed 18
More important information
available for ATS and Anesthesia at:
http://acibadem.dergisi.org/pdf/pdf_AUD_108.pdf
Lidocaine
Topical, regional and local anesthesia may cause potassium to drop in
individuals with Hypokalemic Periodic Paralysis, Normokalemic Periodic
Paralysis or Andersen-Tawil Syndrome if it contains epinephrine. The most often
discussed and utilized local anesthesia is lidocaine. For some individuals it
may work well if the epinephrine is removed. For others it may cause hypokalemia
or arrhythmia regardless of the epinephrine being removed. For others still, it
may not work at all or the usual amount may be needed during a procedure.
Lidocaine and other local types of anesthesia need to be used with extreme
caution.4
In Summary
“Although
neuromuscular diseases are relatively uncommon, patients with these conditions
will present to the operating room and to non-operating room procedure areas
for diagnostic studies, treatment of complications, or surgical management of
related or unrelated disorders. Overall debility, with diminished respiratory
muscle strength and increased sensitivity to neuromuscular blockers (NMBs),
predisposes these patients to postoperative ventilatory failure and pulmonary
aspiration, and may slow their post-procedure recovery because of difficulty
with ambulation and increased risk of falling. A basic understanding of the
major disorders and their potential interaction with anesthetic agents is
necessary to minimize the risk of perioperative morbidity.”19
”The anesthetic management of patients with periodic paralysis first involves
knowing the patient's history and their particular disease characteristics. The
concurrent diseases must be ruled out (such as Andersen's disease). The primary
goal of the anesthetic is to avoid events (perioperatively) that are known to
precipitate muscle weakness. Electrolytes should be normalized, hypothermia
should be avoided and frequent monitoring of the serum potassium level is
indicated. The ECG should be constantly monitored for signs of arrthymias.
These patients can be considered at risk of MH, thus avoidance of MH triggers
is indicated. Use of nondepolarizing muscle relaxants is thought to be
acceptable, although abnormal sensitivity to these agents may be encountered
and adequate muscle strength must be assured prior to extubation.” 20
Anyone with
Periodic Paralysis needs to be extremely cautious when planning any surgical
procedures, which may use anesthesia.
References and Links
Periodic
Paralysis general info about care with meds and treatment
Pre-operative
Hypokalemic
Periodic Paralysis
Hyperkalemic
Periodic Paralysis
Malignent
Hyperthermia
Andersen-Tawil Syndrome
Until later...
Added September 4, 2015:PROLONGED PARALYSIS
Other
medicines used during general surgery are called depolarizing agents
(such as Succinylcholine). These medicines act by blocking the signal
from the nerve (Acetylcholine) from reaching it's receptor on the
muscle. This causes temporary paralysis in patients during surgery,
relaxing the muscles to make surgery easier. In patients with ion
channel disorders, such as periodic paralysis, this can result in
prolonged paralysis...
..After
surgery, patients with periodic paralysis have found they woke up in
the recovery room and couldn't move; they may not get full strength back
for hours or days. The exact cause of the prolonged paralysis with
anesthesia in some people with periodic paralysis isn't known. It could
be the stress of the surgery and/or any of the anesthetic drugs. It is
critical for the medical team to distinguish between a periodic
paralysis attack and a malignant hyperthermia reaction as the treatments
are completely different.
https://www.rarediseasesnetwork.org/cinch/learnmore/faqs.htm
http://www.mhaus.org/.../be-prepared/associated-conditions
http://www.ncbi.nlm.nih.gov/pubmed/11870726
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2900112/
Added February 25, 2017
Advances in Anesthesiology
Volume 2015 (2015), Article ID 562378, 6 pageshttp://dx.doi.org/10.1155/2015/562378
Review Article
Mutations
in Sodium Channel Gene SCN9A and the Pain Perception Disorders
https://www.hindawi.com/archive/2015/562378/
