The throat area has a number of muscles all of which have the potential to spasm. This syndrome results from a spasm in the adductor muscles - the muscles closing or bringing the vocal folds together. Laryngospasm is a normal response to prevent drowning or keeping fluid out of the lungs. If water enters the airways of a conscious victim, the victim will try to cough up the water, or swallow it, therefore involuntarily inhale more water. Upon water entering the airways, conscious or unconscious victims show laryngospasm In most victims, the laryngospasm relaxes some time after unconsciousness. However, about 10-15% of the victims maintain this seal until cardiac arrest, a so called “dry drowning”.
 

   If you truly were drowning, this is a great reflux to have functioning. That clamping shut of the vocal cords may save your lungs from a bath or long enough for you to get back to the surface. However, laryngospam can happen even when only the sensation is present of something other than air entering the windpipe.
The symptoms are very characteristic: abrupt, sudden onset; may occur anytime, but often noticeable when eating and talking simultaneously and something feels like it went down the wrong way; may be awakened in the middle of the night unable to breath. Typically, it lasts less than 30 or 60 seconds. Faster “breathing in” makes it worse. One should never assume a diagnosis of laryngospasm without a history and physical exam.
 

   There is evidence that you become more susceptible to laryngosapsm after an injury to the nerves of the voice box. Nerves heal by regrowing new endings, and these new nerve endings seem to be hypersensitive. The condition of being susceptible to frequent laryngospasms may go on for some time.
 

   Layrngospasms are often associated with the throat irritation caused by being intubated during surgery. If they happen at all, these spasms normally occur in the first hour immediately after surgery and then stop. In people with CLS however, layrngospasms can occur as much as 24 hours after being intubated. This makes post-operative monitoring more critical. The spasms are further complicated by the unusually small jaw structure and narrow airway that normally accompanies CLS.
 

   In a person with no other underlying condition, each individual episode is a self limiting disorder that will resolve on its own and is generally not life-threatening. In CLS, the condition may be life-threatening because they do not understand what is happening and won’t be able to control their panic. If drop episodes are also present, the spasm may trigger those as well.
 

What to do
   If a person with CLS has a laryngospasm, call 911 immediately. If possible, attempt to clear the airway with your finger. Position the head as shown in the diagram. Administer mouth-to-mouth or mouth-to-nose artificial respiration. Mouth-to-nose has been recommended for babies and may be preferable if there is foreign matter in the mouth. If you are the caregiver for a person with CLS who is susceptible to laryngospasms, be sure to get training in how to properly administer mouth-to-mouth artificial respiration.
In the case of larygospasm, attempting to breath in slowly works better than trying to gasp in air. The Bernoulli principle - the one you may have learned about in high school physics that keeps airplanes in the air - is the reason laryngospasm worsens with stronger attempts at breathing in. The faster the air flow through a narrow area, the lower the pressure. The voice box is the narrowest part of the windpipe so it has the lowest pressure during rapid breathing.
 

   With the Bernoulli principle in mind, one can see that slower breathing in will effectively get more air into the lungs than rapid breathing in. We spend most of our life breathing out, as in talking, then we take a quick breath in and spend more time talking. When one has an episode of laryngospasm, one can reverse this usual trend and take most of your time to breath in slowly and then a quick breath out can be followed by another slow breath in. This can be repeated until the spasm stops.
 

   There may be a medication that would reduce the “tickle” sensation that triggers the episodes. Amitriptylline has sometimes been helpful in reducing throat tickles.
 

   Reflux can contribute to laryngospasm. If reflux is suspected, a long-term treatment with stomach acid reducers may be in order.
 

   For patients who are having a great many episodes, a tracheostomy button can be placed into the windpipe from the front of the neck. This may remain capped until an episode occurs and then the patient may open it temporarily. This procedure requires some ongoing maintenance and effectively eliminates swimming as an activity.

Jacob Walter, Age 11

   Jake choked on a peanut butter sandwich at lunch on Thursday, June 12th but he recovered and seemed okay. A couple of hours later he started coughing, vomiting and he had a fever of 101º. I thought he had the flu.
 

   Friday he was still vomiting but the fever was lower. By Saturday he was still unable to keep even Kool-Aid down, so I brought him to Urgent Care. They prescribed a suppository for nausea to stop the vomiting and amoxicillin for bronchitis. When I got home, I gave him the prescriptions then some cough medicine so he could get some rest. He still seemed to be having trouble breathing and coughing so I used the nebulizer—that is when he got really bad!
 

   I took him to the hospital emergency room because his breathing was rapid and shallow, and his heart was racing—he was so pale! They took him straight back to get him on oxygen and started talking about cardiomyopathy¹. The ER doctor had a cardiologist come in and do an echocardiogram which showed his heart was enlarged and a couple of the valves were regurgitating. They transferred Jake by ambulance to the Children’s Hospital in Minneapolis. He was given IV fluids and oxygen. The next day they diagnosed him with congestive heart failure and he will now be on 2 new drugs, Lisinopril (ACE Inhibitor) and Furosemide (loop diuretic).
 

   We met a new neurologist while we were there, and he actually has another patient in his practice with drop attacks! This doctor is having us increase the Lamictal, and Jake will continue with Clorazepate and Effexor. We have discontinued the Neurontin. We are glad to have a doctor who actually knows what drop attacks are!
 

   While Jake was in the hospital he retained his urine for 34 hours. His bladder and kidneys were checked out via ultrasound and it all looked normal so they couldn’t tell me why he does this or even if toilet training would ever be successful.
 

   Jake had an EKG² when the CLS was diagnosed but he never had echocardiogram. This heart problem would probably not be diagnosed without the echo, so I’d recommend to all CLS parents to have an echo done on their child.

Greg & Lissa Walter
East Bethel, MN
 

1 Dilated (congestive) cardiomyopathy is the most common form of cardiomyopathy. In it, the heart cavity is enlarged and stretched (cardiac dilation). The heart is weak and doesn't pump normally, and most patients develop congestive heart failure.

2 An Electrocardiogram (EKG or ECG) is a noninvasive test that records the electrical activity of the heart. An Echocardiogram is a test in which ultrasound is used to examine the heart.

Davis Illa, Age 17

   Davis has a history of laryngospasm after being intubated during surgeries. In October, he caught a cold and started producing a lot of secretions which he had difficulty clearing effectively. After coughing all night and further irritating his throat, he started choking on his own secretions to the point that it brought on a larygospasm which completely closed his airway. He then panicked and had a severe drop episode on top of everything else. Luckily I was in the room with him when this happened, or he would not have made it.
I called the paramedics and performed CPR on him until they got there. We very nearly lost him. Davis was intubated and taken to the hospital. This was on a Wednesday evening. The doctors put him on steroids to get the swelling and irritation in his throat improved, and then on Saturday morning, extubated him.
Within 45 minutes of being extubated, he started to choke on secretions, had another spasm, and had to be intubated a second time.
 

   Now the doctors were very concerned about his ability to sustain without the breathing tube. We waiting until Monday, and then performed a tracheostomy so that the other breathing tube could be removed.
Now that Davis has demonstrated that he can have layrngospasms any time he has a cold or other throat irritation, each cold he gets in the future is potentially life-threatening. The conservative treatment would be to make the trach tube permanent.
 

   A permanent trach tube has risks of its own - infection, cleaning and maintenance issues, scarring, inability to speak, etc. We need to weigh the risks of him having a layrngospasm against the changes to his quality of life that a permanent trach tube involves.
 

   A special thanks to all of you who provided encouragement and support to us online during Davis’s challenging time.

Mary Hoffman

Brandon Christian, Age 16

October 21, 2004

Dear Mary,
 

    Brandon’s condition has changed since the last time I wrote you. He was diagnosed with Cardiomyopathy. His main heart muscle isn’t functioning properly so he is now on Enalapril and Furosemide. His doctor said if he was to go through surgery he wouldn’t make it because his heart is too weak. If the medicine stops working we are dealing with congestive heart failure.
 

   This really make you look at life different it make you appreciate and tell your love one how much you love them, Mary I wouldn’t change a thing. I love my son. Having him around is such a joy. He always say I love you mom. That makes me feel so good, having a blessed child is a gift from God. He don’t put no more on us than we can bear. We all will be blessed for taking care of God’s little angels.
 

   He is doing well in High School. He is in the 11th grade. I'm looking forward to graduation.
I have a question for the parents with the children dealing with sleep apnea. Brandon started out sleeping with the C-PAP but now seems to get choked every time I make him sleep with the mask. How do you get them to relax? I have talked to him, laid beside him -he won’t wear the mask anymore.
 

   I am now dealing with the curving of the spine. He has 75% curving. He is always complaining about his back hurting. I'm not ready for this but I have to be strong for Brandon. He went to the doctor on Tuesday, October 25, 2004. I was told because of his heart condition if he was to go through surgery for his spine he would probably go into cardiac arrest.
 

   My heart goes out to all the parents for doing a wonderful job dealing with the trials and tribulations that we go through day to day. Mary I have moved please pass my new address on to the other parents. I hope all is well with the other CLS families, and may God bless you all .

Denise Christian
Richmond, VA
 

Allan Ellzey, Age 15

   Allen Ellzey had spinal fusion surgery on October 20th, 2004 in Dallas at Scottish Rite Hospital.  Allen only weighs 65 pounds and he is 15 years old.  His spine was at a 58 degree curvature and was progressing at about 2 degrees a month. He was in constant pain.  We tried to have the surgery done this summer, but they found a problem with his first vertebra in his neck and we had to correct that first. 
 

   One of the hardest parts is that I cannot tell Allen what we are doing, or why we are doing it.  I also cannot tell him why he is hurting when he wakes up. 
 

   Allen is deaf and only has the mentality of a 2 year old.  He does not know enough sign language to communicate on that level. He only knows simple commands.  Please put us on your prayer list and remember us.

Thanks
Lanette Sessions
Jacksonville, TX
 

Phillip Barnes, Age 18

   Having been there nearly two years ago when Phillip [then 17] I know how you feel. Phillip does understand to a point what was going to happen as he can hear and talk nearly normally. He was in surgery for nearly 12 hours and in the hospital 16 days. The only reason he was in so long was that the brace he had to wear for six months afterwards. The hospital staff just seemed unable to get the thing to fit as it sudden until the third attempt. Phillip himself was fine. He has gained some weight since the op. now we have to watch he does not gain too much. The only thing he his not allowed to do is contact sports. He swims, goes horse riding, been absailing, canoeing [with medical consent] and many other things he would have found difficult in the past The surgery, though hard, if it works is well worth it. Your family will be in my prayers Lanette.

Wishing you and your family well.

Fiona Barnes
Bedfordshire, GREAT BRITAIN
 

Brett Smitth, Age 20

Hi, Mary!
 

   Sending a quick update and some questions about Brett. Brett is still attending public high school. He can stay in school in Texas through his 21st year.
 

   Brett's overall health has been good. He has been fortunate not to have been afflicted with drop attacks or some of the other CLS related problems. The only thing we are facing now seems to be unique. Brett has had scoliosis for several years now and wore a brace for a couple of years. When he stopped growing the progression of the scoliosis also seemed to stop. However, within the last 1-2 years his head has continued to tip to one side. This has some affect on his swallowing and breathing . We are not sure if this problem is compensation for the body curve to kind of 'upright his world' or if some other problem is occurring. We are in the process of having MRIs, CT scans, etc. but it is looking now like they will be suggesting to perform the spinal fusion surgery for Brett.

   I know your son, as well as other CLS kids, have gone through this experience so any hints, warnings, or other information you can provide would be greatly appreciated!! I plan to go back through several old newsletter issues, too.
  

Thank you again for all of the time and effort you put into the foundation to benefit all of us. You are an angel!

Steve & Sherri Smith
Garland, TX
 

Dear Sherri,
   There is a link on the website to some information about scoliosis and spinal fusion.  Or, just go to http://www.google.com and search on "spinal fusion". You'll find lots of information.   It makes sense that the head tilting could be related to trying to balance against the curve in his back. If you stand with your hips our to one side, you naturally compensate with your head in order to maintain your balance.    Tips. Don't freak when you see him right after surgery. It's a long surgery and he might come back all puffed up from lying on his face for hours. He may have to have his hands restrained to keep him from pulling on IVs or other tubes.
Tell the anesthesiologist to use the smallest intubation tube possible - CLS kids tend to have small jaws and narrow tracheas. He'll most likely have to wear some kind of protective body jacket for some months after the surgery.  

-MCH

Tracheomalacia
 

   When Davis was in the hospital recently, the respiratory specialists concluded that what he had was not layrngospasm as previously thought, but tracheomalacia, a condition that is often overlooked or misdiagnosed because it mimics asthma very closely.
 

   Tracheomalacia is a softening of the cartilage of the trachea, which makes getting air in (and especially out) more difficult. Think of a garden hose that normally holds it’s circular shape, but collapses when it gets soft.  It is most often seen in premature infants, whose cartilage is not fully developed, and disappears over time on its own. The condition can also be acquired from chronic tracheal infections or prolonged intubation. Tracheomalacia can be associated with a variety of congenital anomalies, including cardiovascular defects, developmental delay, esophageal anomalies, and gastroesophageal reflux.
 

   Symptoms include stridor (also called laryngeal crow), which may worsen when lying down, and respiratory infections.  Feeding difficulties are reported sometimes. Hoarseness, loss of voice, and labored breathing also may be reported.
 

    I am curious how many other kids might have this same problem and don't know it. How many kids have a history of reflux, stridor or asthma, cardiac problems and chronic respiratory infections? I suspect this may be more widespread in our population than we know.
 

I’ve also found that Davis gets withdrawal symptoms if he is on narcotics even for a short time. A single dose of cough syrup with codeine will trigger an episode of sweating and agitation the next day.

-MCH

Andrew Anderson, Age 17

   We have had a lot of changes in the last year! We are now living in southern Alabama. Andrew is in 11th grade now. He attends a wonderful all inclusive school that he has adjusted to well. The busses here are almost a regular bus size, and he loves to ride 'the big bus', as he calls it!  He has a new 'girlfriend' named Kiki—she rides the bus with him, but isn't in the same class. Every morning he asks to ride the bus, then asks about Kiki! What about school!!

   He is now on a CPAP as well. Andrew's doctor also prescribed oxygen as his 02 levels drop dramatically while sleeping. He has done really well, sleeping a lot less and still full of energy! The doctor has the CPAP set at 10 and his oxygen at 2.
 

   We were here for hurricane Ivan and had to go to a shelter. Because of his equipment he and I were made to stay in the special needs area. I don't know which one of us was more nervous, he kept asking for his dad. I was glad when he finally went to sleep, he slept right through the worst of the storm.
 

   Andrew is currently taking 100 mg each of Topomax and Lamictal, two times a day with about a 90% seizure control, maybe 5 or 6 a week, from before(5 or 6 a day minimum).  Andrew recently spent 4 days in the hospital. He had an infected sore on his elbow and the doctors were worried that it was in his bones. He had a couple of sores similar to this before, but they had cleared up an their own. I think they were in reaction to an insect bite he had some time ago. The doctor kept Andrew on IV antibiotics over the weekend, sent him for a bone scan on Monday, and sent him home Tuesday, well enough to take oral antibiotics. I took him for his check up last week and everything is now ok.

   During this past summer Andrew had his first experience on the Greyhound bus, and with an airplane! I rode out to California with him the first of June to see his brother graduate 8th grade. We stayed with my mom for a month then his brother, sister and I came home and he had Grandma for a whole month to himself! They flew home the first of August. Grandma said he was a little afraid of the boarding with the "tunnel" but the flight attendants helped comfort him. He got a cute teddy, some wings, cards and a blanket, all for flying.

   He is getting anxious now because we are planning another trip for Christmas. He asks all the time about going, we have to call a lot!  Before we go I am taking him to a clinic called Children's Rehab Services. It is an assistive program that includes doctor appointments, therapy clinics and adaptive equipment, not sure what else. It is kind of like the CCS (Cal. Childrens services) we had in California. Unfortunately it has taken us almost a year to find them! Relocating was difficult but we are finally settling in. Andrew is doing well in all areas.

Kathy Davis
Saraland, AL
 

   Tantrums are triggered by disruptions of routine, changes in sleep and meal schedules, unrealistic expectations, and disappointments.

   When a child who regularly throws tantrums makes a request, parents are often tempted to put off a confrontation with, "I'll think about it," or "We'll have to wait until your mother (or father) comes home." This sets up a long and risky period when a tantrum is likely. For the moment the request is denied, but it was done in a weak way that tempts the child to fight for what he or she wants - plenty of time to try out a tantrum along with other obnoxious behavior.

   Also, putting off the child leaves him with nothing to do. It takes experience and creativity to put aside one line of activity and take up another while waiting for an answer to come down from the parental powers. Instead of switching to a new activity, the childish thing to do is cling to the present direction and push for an answer. Nagging is followed by complaining, then frustration and attack, and then the whole tantrum.
Many parents I know have used the "all stop" method with success. The term comes from the Navy when the ship captain commands, "All stop!" and all engines, whether in reverse, slow, or full speed, are shut down and the ship is dead in the water. For tantrums it means no progress is possible until the tantrum stops - no discussion, no alternatives, no argument. Mom merely says, "We're in "all stop" until you stop this tantrum."
The pitfall to this approach is that most of us will not really stop. We are tempted to continue to talk, cajole, plead and threaten - especially if the tantrum gets longer and louder. If this attention is part of the child's reason for tantruming, then we're going in the wrong direction again by providing attention only for escalation.

   And speaking of escalation, parents need to guard against escalating their own volume and anger, thus providing additional bad behavior to be imitated.
 

   Many parents have told me that tantrums occur at regular times - often when routine is disrupted by holidays or company, or when the competition from a sibling is the focus. Here's a good place to keep a behavior record. You may find that food shopping with your child right before dinner is likely to be a tantrum situation. Or that homework arguments right before bed produce the most tantrums.
 

   The best solution will come from patience with a child not experienced with the unusual stress of the holidays or not mature enough to handle frustration, hunger, or fatigue without emotion. And parents can help with a thoughtful and consistent reaction when tantrums do erupt.

Dr. McIntire is the author of Teenagers and Parents: 10 Steps to a Better Relationship and Raising Good Kids in Tough Times, available in our bookstore. His newspaper column appears in a growing number of newspapers nationwide.

New Year's Day is every man's birthday. 

~Charles Lamb

Thanks for Caring!

The following families or individuals have recently made generous donations to the Coffin-Lowry Syndrome Foundation:

Hiroatsu and Kaoru Hirayama

Kanagawa-ken, Japan

Send your donations to:  

Coffin-Lowry Syndrome Foundation

c/o Mary Hoffman

3045 255th Ave SE

Sammamish, WA 98075 

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