Laryngospasm, Additional Info on

Thanks to one of my regular readers with Kennedy’s Disease, I have some additional information on my recent post about Laryngospasm. Hearing the sound of the person gasping for air on the videos always brings me back to those initial few times when it jerked my awake at night.

Bastian Medical Media has a website on the subject with a couple of good videos as well as an audio description of the process. In the second video, Dr. Bastian introduces the straw technique that can be practiced ahead of time to help minimize the impact of the spasm when it occurs. Below is an introduction to the spasm. You can read the entire article and watch the video by clicking on this Bastian Medical Media link.

Laryngospasm

“A sudden reflexive closure of the larynx occurring when an individual is trying to breathe. Laryngopasm occurs more frequently in persons who have vocal cord paralysis or in those experiencing sensory neuropathic cough; it is also seen as an aftermath of an upper respiratory infection.A typical laryngospasm episode begins abruptly and lasts approximately one minute. The individual often makes loud inspiratory noises, the loudness of which abates gradually over the first minute or two. The voice may be choked off during the same time, making it difficult to speak. Laryngospasm is terrifying not only to the person experiencing it but also to family, friends, or strangers observing the episode. An attack may awaken its victim from sound sleep. Rarely, an individual will experience a series of laryngospasms, making it appear that they are having one much longer spasm…” 

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There is also a web page in Anesthesiology website on the subject. I was unaware this spasm can be an issue when a person is coming out of surgery. Below is an introduction to the topic. You can read the entire article by clicking on this Anesthesiology website link.

Laryngospasm-The Best Treatment

“Almost 40 years ago, Dr. N. P. Guadagni showed me a technique for prompt termination of laryngospasm, which I have used countless times with complete success. Because I have used the techniques so often myself and have taught it to hundreds of residents and nurses. However, a thorough literature search has not revealed any mention of it. The technique involves placing the middle finger of each hand in what I term the laryngospasm notch. This notch is behind the lobule of the pinna of each ear. It is bounded anteriorly by the ascending ramus of the mandible adjacent to the condyle, posteriorly by the mastoid process of the temporal bone, and cephalad by the base of the skull (Figure 1). The therapist presses very firmly inward toward the base of the skull with both fingers, while at the same time lifting the mandible at a right angle to the plane of the body (i.e., forward displacement of the mandible or “jaw thrust”). Properly performed, it will convert laryngospasm within one or two breaths to laryngeal stridor and in another few breaths to unobstructed respirations…”

Surgery Considerations for those with Kennedy's Disease

This topic has come to the surface again. A friend who is currently in the hospital discussed his concerns of using certain anesthesia after receiving some information from a doctor familiar with Kennedy's Disease.

"Important:  A known issue has been reported by Dr. Neil Porter. Do not used  SUCCINYLCHOLINE (generic name). Brand names include Anectine and Quelicin.  Please have your medical file updated.This drug could be life threatening for a person with Kennedy's Disease."

Certain types of anesthesia have been known to cause or create problems for those of us living with this condition. There is an entire page on the KDA website on this subject. Also on this page are several other items to discuss with your doctor and anesthesiologist prior to surgery.

The key message is to make the surgery team aware of your condition and to provide them with information on possible issues with certain anesthetics and also on potential post-surgery concerns.

When I was scheduled for surgery several years ago to repair a broken tibia and fibula, I brought in a copy of the webpage mentioned above. After my doctor read the information and discussed it with the anesthesiologist, they decided on a different course of action.

Being Prepared – Preparation is the key - Part II

Sunday's article provided the background on why you should have a plan for discussing with your doctors potential complications from certain types of anesthesia. In today's article, I will provide some actual steps you can take to minimize complications and delays for needed surgeries.

1.        Emergency Medical Card – This card or something similar needs to be carried in your wallet.  It will forewarn emergency response personnel and hospital staff that you have a health issue that needs to be considered when treating the patient.

2.       Medical Information Form - This form, when filled out, provides detailed information about your health condition, medications, doctors, health insurance, emergency contacts, etc.   I recommend carrying a copy in your car, giving a copy to your doctor, and having a copy at home.  Because I traveled quite a bit in my earlier life, I also carried a copy in my carry-on bag.  In the “Medical Concerns” section, add specific wording that you feel is appropriate.  A discussion with your doctor on what he would add to this section is most helpful.  Included in my form are the following:

·         Certain anesthetics could be hazardous to the patient’s health.  Of primary concern is how the anesthetic agents will affect the patient’s muscles (including the heart and lungs) during surgery and recovery.  Certain neuromuscular blocking agents are a major concern.  The use of syccinycholine should be avoided because of possible unpredictable side effects.  Reactions could include malignant hyperthermia (MH).  The best treatment is not to give this patient any MH triggering agents.

·         Patient needs to be kept well hydrated before during and after surgery. 

·         Patient does not tolerate changes in body temperature (especially cold).  Try to keep him warm.

·         Caution should be used since the patient chokes easily and could experience difficulty swallowing.

·         Patient may be predisposed to regurgitation and pulmonary aspiration.

·         Post-surgery pneumonia is a potential complication because the patient cannot easily clear his lungs. 

3.       Communicate your Condition – During your next doctor’s visit, discuss your medical condition and any potential issues that need to be considered by an anesthetist prior to any surgery as well as the hospital staff pre-during-and post operation.  Ask your doctor to document these concerns in your medical record should emergency surgery ever be needed.

4.       Planning for Surgery – If there is time (elective or an upcoming planned surgery); discuss potential issues with your surgeon and surgical team.  Make certain they are well versed in Spinal-Bulbar Muscular Atrophy and the potential side effects including those from certain anesthetics.  Ask if they have performed surgeries before on patients with similar conditions.  If you have concerns, discuss these with your regular doctor and neurologist.   

A key resource in getting your message out is your family doctor and you/your spouse.  Do not be shy and always error on the side of caution.  If you do not feel you are being listened to, call a “time out.”  Yes, you are dealing with professionals, but that does not mean they have experience with neuromuscular disorders (NMD) and in particular your, or similar, disorders. 

Be prepared ... it might save your life.

Readers, if you have some particular recent experiences with surgery, please share them.

“Be Prepared” ... A motto that might save your life – Part I

This last week I received an email. "I would like to know the position of general anesthesia and KD affected men. If you could cover how KD affects anesthesia, are there any options, at what stage in the KD lifecycle does this become an issue. I have this fear of having to have an emergency operation and because KD is such a rare disease the surgeons etc may not be aware of the risks."

The questions are excellent and it was a concern of mine a few years ago. Because of the importance of this subject, it will be a two-part article. This article is focused on comments from doctors. Part II will be about preparation and concerns in general as well as highlighting some of the comments from this article.

Background: I broke my tibia and fibula in a fall. The emergency room x-rays showed the two bones were broke a several places. The orthopedic surgeon wanted to operate and use some pins and screws to correct the problem. I asked my wife to go home and bring back the anesthesia information from my Kennedy's Disease file. The anesthetist reviewed the information and he recommended that the operation not be performed at this hospital. He had never assisted in a surgery on someone with spinal bulbar muscular atrophy.

There is some good information on the internet about anesthesia and neuromuscular diseases. The KDA has four articles on the subject that explain the concerns and potential issues quite well. The MDA also has information on their website about anesthesia and neuromuscular disease (NMD).

Dr. P.J. Halsall and Professor F.R. Ellis provide the following explanation.

"People with neuromuscular disorders must take great care if they are to have a local or general anesthetic. Even someone with very mild, or
non-existent symptoms, or someone who has a family history of a disorder, needs to let the anesthetist know well in advance so that tests can be carried out and proper care after the operation can be arranged. Many people are afraid of having an anesthetic, mainly through ignorance, but when we look at the rate of complications and even deaths arising from anesthesia we see that it is in fact very safe. This safety is the result of a thorough understanding of the patient's medical condition with a careful assessment before the operation, marked technical improvements in monitoring facilities such as High Dependency Units (HDU) and Intensive Care Units (ICU).

Patients with neuromuscular disorders (NMDs) deserve special attention when it comes to anesthesia because many of the agents used (gases and
chemicals) have effects on both muscle and nervous tissue. The main areas of concern are how the anesthetic agents will affect the muscle and how they will affect the heart which is itself a muscle.

Muscle relaxant drugs should only be used if essential because they tend to have a more profound and prolonged effect in NMD patients compared to
other patients. One type of muscle relaxant, called suxamethonium, should usually be avoided. It causes the release of potassium ions (K+) from the muscle tissue into the blood. In normal patients this is usually of little practical significance. In patients with NMD the muscle may normally leak K+ so that a further increase in the levels of K+ in the blood may cause abnormal heart rhythms. A preoperative blood test to check K+ levels is therefore important.

A local anesthetic works by preventing the normal electrical activity in the nerve around which the anesthetic agents are placed. For minor procedures, such as stitches for cuts, they are probably the first choice for patients with NMD because they have few if any side-effects. However for major local anesthetic techniques, e.g. spinal or epidiural, careful assessment of the patient is needed and the type of NMD considered well before the operation.

Changes in body temperature and preoperative 'starvation' are also a concern. Patients with NMD do not tolerate changes in body temperature or the
starvation often associated with anesthesia or surgery as well as normal patients, so steps need to be taken to minimize these problems by keeping the patient warm and well hydrated using drips.

To sum up ... clearly anesthesia in NMD is not to be undertaken lightly. Such patients should expect the anesthetist to make a careful and thorough assessment of their particular condition and their current state of health. They are not suitable to be treated as 'Day Cases' because doctors should
carry out preoperative investigations, and enough time and recovery facilities should be available after the operation. It is absolutely essential that the person affected by NMD should inform the anesthetist even if there are only minor symptoms, or no symptoms at all. Occasionally a neuromuscular disorder in a person who had no symptoms has come to light only because of an unexpected problem with anesthesia, particularly in young children."

Dr. Linton Hopkins, a neurologist at Emory explains other concerns.

"KD patients may have significant weakness of respiratory muscles and not know it, so they don't report it pre-op. Then after surgery, with the ordinary chest or abdominal pain that follows so  many operations, they find it impossible to take an adequate breath or cough well enough to clear secretions. This can quickly lead to hypoventilation & pneumonia. Everyone who is not confident about the strength of their cough and sniff should warn their doctors and ask for pre-op pulmonary function and instructions about incentive spirometry and other ways to minimize the risk. ... Surgeons and anesthesiologists know about myopathies and neuropathies, even though few will have heard of KD, which is such a rare "neuropathy", or anterior horn cell disorder."


Part II will be posted on Tuesday.