Annie's Salivary Gland Ablation Surgery

For years we have debated whether or not to put Annie through an invasive procedure to have her salivary glands removed.  Our doctors have continued to tell us that they don't think she's a good candidate, not because she doesn't drool heavily, but because Annie has never had pneumonia and such an invasive and risky procedure would grant her little health benefit unlike it does the children who are repeatedly suffering from aspiration pneumonia.

However, last summer at our Aicardi Syndrome Foundation Family Conference, I attended a session taught by an interventional radiologist, Dr. William Shiels, from Nationwide Children's Hospital in Columbus, Ohio.  In that session, I learned that just recently Dr. Shiels has devised a salivary gland ablation procedure that is only minimally invasive and does not require the removal of the glands.   I sat in awe, and got more and more excited about this procedure for Annie the more he taught us about it.  I remember feeling such a powerful sense of hope that this procedure could change Annie's life.

Currently, Dr. Shiels is the only doctor in the United States, maybe even the world, who does this procedure.  He hopes to start teaching this procedure to other doctors around the country to make it more available to so many children who could benefit from it.  After we had been home from the conference for a couple months, I went to work figuring out what we would need to do to get this procedure done for Annie.   I spoke with two other mothers in our group whose daughters have had this procedure done by Dr. Shiels.  I felt prepared, informed, and at peace with our decision to move ahead with it.  However, Annie quickly became very ill and landed in the hospital for 6 weeks, exactly during the time that I had planned to be with her in Ohio having this procedure done.  But again, we are now moving forward with it and finally have her scheduled for this surgery in March!  I can't wait!  I'm so excited to see positive changes in her life!

About this procedure... Dr. Shiels has devised a technique that will kill the salivary glands without actually removing them, thus eliminated excessive drool.  Under ultrasound guidance, (with the patient under general anesthesia), he injects the glands with a medical detergent to open up the cells in the salivary glands.  He then injects alcohol into the glands which immediately kills the cells in the glands.  This is a very effective treatment, but causes extremely severe swelling of the neck and face.  Because of this, only one side of the face can be treated at once.  Annie's first procedure will treat the glands on the right side of her face, then a week later, the glands on the left side of her face will be treated.

Everyone needs at least a little saliva, so only 5 of the 6 glands will be treated.  The submandibular gland is the gland that produces 70% of your saliva when at rest, whereas the parotid gland produces the most saliva when stimulated--like when you start thinking about a nice creamy milkshake or juicy burger.  So Annie will have both submandibular glands, both sublingual glands, and one parotid gland treated, leaving one parotid gland to function normally and prevent dry mouth.  Because the alcohol that is injected into the glands is so toxic, if the doctor over-fills a gland, that alcohol can seep out of the gland and kill surrounding tissues.  So to avoid that, only about 80% of the cells in each treated gland will actually be killed.

So compared to completely removing the glands, this is a much less invasive procedure with fewer risks of nicking facial nerves and other complications related to removing the glands.  Also, because there are just needle pokes and the killing of cells, it is also a relatively painless procedure requiring only advil for comfort.  Most discomfort is due to the tight, stretching skin as a result of the severe inflammation.

Most children have this procedure done on an out-patient basis, but because Annie is at a higher risk due to her floppy airway, sleep apnea, and need for CPAP, she will stay at least one night in the hospital after each treatment to monitor the swelling and protect her airway.  This is yet one more reason why we are fighting so desperately to get her off her prednisone which is only increasing the amount of fat around her neck and jaw.  We're hoping to eliminate some of that to make it easier for the doctor to even find her glands with the ultrasound, but also to reduce her already high risk of airway constriction.  

Some stats from the doctor (and why we are so excited)...

• 96% of patients having this procedure respond well to it with a decrease in saliva
• The average amount of decrease in saliva is 66%
• We will see a dramatic improvement in secretions within 48 hours after even just the first procedure and even more dramatic results after both sides are treated.

Why are we going to such lengths to take Annie to Ohio for this procedure?  Why are we working so hard with our insurance company and state medicaid programs to get this new procedure covered?  Here are our reasons:

• Annie soaks through several bibs a day--maybe only 3 or 4 on a good day, but up to 12 or more on worse days in addition to a vinyl bib with a pocket that we pour into the sink throughout the day to empty.  She never has a moment when she's sitting up that she does not have a bib on.  
• Annie's clothing is usually wet by the end of the day regardless of how diligently we change out her bibs.
• An 8 year old should not be wearing baby bibs.  This procedure will improve her dignity and help her be more age-appropriate and fit in better with her peers. People tend to shy away from interacting with people who regularly drool.
• Annie regularly develops a rash on her chin and under her neck from constantly being wet with saliva.
• Because of Annie's weak and uncoordinated swallow, Annie easily chokes and gags on her saliva sometimes resulting in actually throwing up.  This is why even a little cold or other congestion causes her to vomit.
• Annie aspirates on liquids (meaning she easily gets fluids into her lungs) because of her weak swallow, and therefore is at an increased risk for aspiration pneumonia.
• When lying down and sleeping at night, Annie still manages to drool, even when laying on her back.  She often wakes up in a large puddle with her pajamas and bedding soaked.

Annie's bed soaked with secretions one morning.  The wetness extends from halfway up her pillow down to where the ruler is on the chux pad, with the wetness extending slightly wider than the length of the ruler.

We are fortunate enough to be able to stay at the Ronald McDonald house during our 11 day trip to Ohio for this procedure.  We are so blessed and incredibly grateful for the help of parents in going to Ohio and helping with the other children at home.  We are excited and anxious for this procedure to be done and to see such a positive change in Annie's quality of life.  The surgery is not without risks, but we have felt peace and hope as we've prayed about this procedure for her.  Please keep Annie in your prayers, as well as Dr. Shiels, that this salivary gland ablation will be successful and go smoothly without any complications.