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.

Praying for Remission

It's been a while since I've posted an update, especially about Annie's ulcerative colitis, so I thought I'd share a bit about what's been going on lately.  Annie went into remission for several weeks in December and we felt like we had started to see the light at the end of the tunnel after 4 1/2 months of chronic diarrhea, a terrible c-diff infection, and a 6 week hospital stay.  The couple weeks around Christmas were a huge blessing--we had Annie home, she was happy, healthy, and in remission.  However, as the new year rolled around, Annie started experiencing symptoms of another flare-up.  After doctor appointments and phone calls and medication changes, her flare-up continued through January and well into February.

Last week we were desperately hoping and working to get her off the steroids completely.  She has been on steroids to manage her ulcerative colitis for 3 months straight now, which is not safe and causing terrible side effects.  So finally, last week we managed to wean her down to a very small dose and were just a couple days away from having her off the steroids completely when Annie became quite ill.  Of course Annie throws up with any kind of illness, and with the steroids suppressing her immune system, we held out a couple days to see if all the vomiting was just due to a cold she might have picked up at school. (Vomiting with a cold is very normal for Annie since she can't tolerate any kind of congestion due to her inability to swallow normally.)

However, the vomiting continued, changing from phlegm to food and bile.  Her diarrhea didn't increase (she's consistently having about 4 loose stools a day since the beginning of January) but her diarrhea became much more bloody than normal.  She started vomiting blood, most likely due to the stress on her stomach and esophagus from so much retching, and she even started leaking blood out of her stoma (the hole in her stomach where her feeding tube is).  We had no choice but to increase the steroids, this time back to the dose she was on while in the hospital last fall.  We're becoming so discouraged that she can't seem to get off the steroids.  They are not meant to be used for long-term use and are toxic drugs with bad side effects.  If steroids continue to prove ineffective, the doctor will prescribe cancer drugs that will be more powerful in suppressing the immune system and helping the ulcerative colitis go into remission.

After being on this large dose of steroids for about a week now, Annie has seemed to respond.  We're seeing less blood and fewer episodes of diarrhea in a day.  So again, we will be soon working to wean her off the steroids.  We are especially praying that this round will put her into remission because of her upcoming surgeries in March for her salivary gland ablation.  It would be best for her to be off the steroids before having her surgeries, so we will work for that goal and hope that she doesn't start with another flare-up before her procedures.  We prefer to have her off prednisone for surgery, but the alternative (vomiting and increased bleeding) are not good symptoms to have before surgery either.  The prednisone is also causing an increase of fat around Annie's neck and chin which put her at an even higher risk of airway restriction after her surgeries which cause an incredible amount of swelling.  She already has a floppy airway and easily obstructs (she uses a CPAP machine at night to keep her airway open) so reducing the amount of fat around her chin and neck before surgery would be ideal.  So we pray that the prednisone will somehow bring about remission this time around, and that her other slew of drugs will be able to maintain her remission for her procedures.

I'm so excited for Annie's surgeries in March!  I can't wait to have once less thing to worry about, and can't help but wonder if this salivary gland ablation will prevent her from vomiting so easily and help her stay healthier.  We have high hopes and expectations, and hope this procedure brings her a higher quality of life!

Worsening Scoliosis

Annie had her 6 month scoliosis check-up on Monday.  Ever since she was about 2 years old she has had scoliosis.  It held steady at 11 degrees for a couple years, then held steady around 18 degrees for a couple years, but last July her curve jumped to 37 degrees.  They are wanting to monitor her more frequently now, so on Monday she had another x-ray. 

Now, just a note about how scoliosis x-rays are taken... Annie has sometimes had her x-rays laying on a table, and sometimes she's been sitting up.  With an 11-18 degree curve, the difference in the curve sitting versus laying hasn't been much.  However, generally speaking, the curve degree is more accurately measured if the patient is sitting up, which also usually shows a more severe curve.

In these images below, the one on the left is from July 2010 and was taken laying down.  The one on the right is from Monday and was taken sitting up.  Last July her curve was measured at 37 degrees.  We have not gotten an accurate measurement from her x-ray on Monday, although the computer model calculated it at 69 degrees.  (However, the same computer model calculated her July curve at 52 degrees.)  So, taking into account that she is laying down in the first one and sitting up in the second, we're guessing that her current curve would measure close to 52-57 degrees.  All of that is just our guess--the doctor really couldn't tell us an exact measurement now since it hadn't actually been accurately calculated.

The thing is, you might say, "Well, Annie is obviously not sitting up straight in the second x-ray."  Let me just tell you that both Justin and I were supporting her on the x-ray block, and when they took the x-ray, I commented to Justin about how glad I was to have her sitting so straight on the block--that way the curve of her spine wouldn't be influenced by how she was sitting, and I knew they'd get an accurate measurement not influenced by the way she was sitting.  I thought we had her sitting so perfectly straight, and from what we and the technicians saw, she WAS sitting as straight as she would go.  That's why I was so surprised to see the x-ray!

July 2010 X-ray (37 degree curve)         January 2011 X-ray (52-57? degree curve)

Just a couple other things about these x-rays...  We were rather appalled to see on these x-rays the amount of fat that Annie has gained around her chin and cheeks.  We know she's been gaining lots of weight being on prednisone for nearly 3 months now, but I was surprised at how clearly these x-rays show that weight gain in her face!

Also, one reason some of her past x-rays have been laying down is because Annie get her hips x-rayed every year to monitor for problems and another dislocation, so they seem to like to get both hips and spine in one image and be done with it.  So in the image on the left from July, you can clearly see her dislocated left hip and her rotated pelvis, which in turn is causing her scoliosis to worsen.  You  can also clearly see how that dislocated hip is causing her left leg to be about 3" shorter than her right.

So, now you know almost as much about Annie's insides as we do (at least orthopedically)!  The plan now is to continue to monitor her scoliosis, with x-rays every 4-6 months.  Annie is not a candidate for bracing because this is not the type of curve a typical child might develop who has normal muscle tone.  Bracing would only cause more pressure sores and potentially cause respiratory issues.  Any amount of bracing at this point also would never work to correct her curve.  It might prevent it from getting worse, but most children with Aicardi Sydrome have continued to have a worsening curve even with bracing, and for us, the side effects aren't worth it when results would be so poor.

So really the only option (if we do decide to treat it) is to surgically correct it with a major spinal fusion surgery where two rods are inserted on either side of the spine with loops threaded through the spine.  Most doctors don't like to do spinal fusions on children until they are 10 years old.  So right now we'll continue to monitor her curve and start discussing surgery in about a year, unless things start getting really bad really fast.  The doctors believe that Annie's curve is already at the point where it is going to start putting stress on her heart and lungs, so we're anticipating that within the next 18 months we're going to be facing some tough decisions.  For now, I'll just try to not think about it all and be grateful that we have Annie and that she is so happy and doing relatively well!

Moon Face

Annie has been on steroids for her colon inflammation for nearly 3 months now.  She's gained almost 10 lbs since starting the steroids, and she's developed the typical moon face associated with steroid use.  In addition to making her incredibly chubby-faced, the amount of fat Annie's collected around her chin has made itdifficult to prevent rashes from all her drooling.  Most of that skin doesn't see the light of day, but stays wet all the time.  We're working with a doctor in Ohio who will be doing Annie's salivary gland ablation in the spring.  Hopefully we'll get Annie off her prednisone and lose some of that moon face before her procedure, otherwise the doctor might have a hard time finding her glands at all!

Annie before steroid use

Annie after 11 weeks of steroids.

Staying Warm

It's been a challenge since moving to a cold, wintry climate to keep a coat on Annie in her wheelchair.  So I was thrilled when her sweet Aicardi sister Macey sent Annie this poncho!  It's made specifically for wheelchairs--it's really short in the back.  It's a breeze to get on and off and it keeps more of Annie warm than any coat we've ever managed to get on her.  Thanks, sweet Macey!  You're a doll!

This poncho came just in time for a bitter cold snap!  I was shocked to see these seven birds on our deck this morning when the temperature outside was 1 degree with a windchill of -16 degrees!  I have no clue how they are finding food or surviving at all!  I'm so grateful we have a nice warm house and plenty of food storage so I don't even have to go outside!

Annie's Wheels

Annie got her first wheelchair when she was just a year old to give her weak little body more support than a traditional baby stroller.  Little did we know then that ordering that first KidKart Xpress wheelchair was such a breeze!  Emotionally, it was one of the more difficult transitions, having to really face the fact that our daughter was so different and would require a wheelchair for the rest of her life.  Up until that point, she had looked like a normal baby--she didn't do the things a normal baby did, but she looked like a typical kid.  The wheelchair changed all that.  But it was a huge blessing for her, and we loved her KidKart.

Annie's KidKart--age 13 months

When Annie was 4 and had grown out of her KidKart, we ordered her a brand new Quickie Zippie IRIS.  This time, ordering her chair was a much bigger ordeal.  Little did I know about how detailed the customization would be.  Every single aspect of the chair was discussed with a physical therapist and our durable medical equipment supplier.  We were thrilled when it arrived!  It fit her so well!  (Of course at this point we had to get our modified van in order to now transport Annie in her wheelchair--at 85 lbs, the chair became impossible for me to lift into the back of our 4Runner alone.)

New Quickie Zippie IRIS (Intelligent Rotation In Space)

Annie--age 4

Which brings us to today in our little wheelchair history timeline.  Last July our orthopedist told us Annie's scoliosis curve had worsened from 18 degrees to 37 degrees.  Because of this, Annie had been developing pressure sores under her left arm as her spine has been tipping her body that direction.  The orthopedist ordered a wheelchair evaluation for us with the physical therapist and our DME supplier and instructed them to order a chair with a contoured seat back and larger lateral supports to help brace against Annie's curve more comfortably.

We thought we would be ordering a whole new chair, but our DME supplier explained how her current chair could continue to grow if we ordered just a new seating system for the frame.  Also, until we expanded this frame, insurance would not cover a whole new wheelchair (which costs over $8,000).  So again, we ordered a new seating system with a contoured seat back, larger lateral supports, a new butterfly harness, a new seat bottom cushion to help accomodate her leg length descrepancy, and a calve protector.  The entire chair was expanded by 2" for a wider seat, and longer foot plate extenders were put on to accommodate Annie's increasing height.  Last week, 6 months after ordering all this, the parts were finally delivered.

Our supplier picked up Annie's chair and took it for the day to install the new seating system.  He brought it back and we realized it still needed some adjustments, but we felt confidant in doing them ourselves, so we sent the supplier on his way.  However, even after making all our adjustments, Annie kept literally falling out of her chair.  It was just our luck that our supplier was off the next day, so Annie was without a chair for 2 1/2 days.  He came back the following day and spent 3 hours with me trying to brainstorm how to make this chair more comfortable for her and also supportive enough so that she wouldn't fall out.

Annie's chair, Jan. 2011, with the old seating system.

Annie's new seating system before our modifications.

Contoured back with very little support.

New calve protector so Annie's feet don't fall behind her foot plates.  (New foot plate covers to come soon!)

New seat cushion to accommodate Annie's leg length discrepancy.  The right leg cushion extension should have been about 2" longer than it ended up being--Annie's right leg is about 3" longer than her left, partially due to her dislocated left hip.  (This vinyl seat cover had to go too--otherwise Annie would be stuck to her chair in the summer!)

Front view of seat back cushion before we made our modifications.

Over a week later, we now think we have this chair working for her a little better.  We are still waiting on new foot plate covers to replace the ones Annie has put holes in.  And we just ordered a new tray that will better fit on her wider chair.  At least now when we do order Annie's next new chair, we'll know better what to get or not get. 

Our modified seat back cushion.  We lowered the entire seat back about 3" and offset the laterals in order to give her the most support against where her spine is curving.  We also cut out about 4" of foam from the seat back cushion to help get the lateral supports more snugly against her body for more firm support, thus the wrinkled cover now.  (I might be brave enough some day to cut into that cover and sew it so it fits better.)

The new neoprene seat cover so Annie won't be so sticky in the summer.  Wow!  It's so clean!

Never have we had so much trouble with a piece of equipment, and I would have never imagined that ordering a new seating system could be so difficult.  Hopefully we can either get Annie's scoliosis resolved eventually, or learn about an even better option for a wheelchair system that will provide her with the support she needs with a worsening curve.  Fortunately, we love our DME supplier and he's been so patient with helping us get what we need to make Annie comfortable, including making a dozen trips to our house!  It didn't help the stress level though when he moved to a different medical supply company in the middle of all this!  So once we get the new tray and foot plate covers, I think we'll finally be done with this chair.  For a while, at least...