Can't Catch a Break!

Annie hasn't been back to school since her surgery a week ago last Wednesday.  We thought she'd be able to return to school last Monday as a happy, healthy, and much drier child, but of course life is never that predictable.  Annie hasn't been doing very well since about last Sunday.  Her salivary gland ablation surgery left her with lots of swelling and thick secretions that have been difficult for her to tolerate, causing almost constant coughing and vomiting bile.  We've been suctioning her regularly since surgery, even throughout the night. 

However, it was about Sunday when her cough started getting weaker and weaker and she just hasn't been able to clear all the junk that's collecting in her throat.  We usually just suction it out, but all the congestion is too deep for us to suction anymore--we just don't get anything.  Usually when we suction her, it also makes her kind of gag and cough up what's in there, but we can't even get her to cough or gag with the suction.  She's just not her usual self and the weak, congested cough is continuing.  Her drooling is almost just as bad as ever, but we're trying to not get too discouraged about that yet since we think she must have some other bug that has increased all her mucous and phlegm. 

We met with her GI on Tuesday, originally planning to discuss how to get Annie off prednisone which has been managing her ulcerative colitis.  Fortunately, we were able to wean her off the prednisone in preparation for her surgeries, and amazingly enough we've been able to keep her off the prednisone.  She still continues to have 1-3 very loose stools a day, but that's a huge improvement from the 8-12 she used to have without prednisone.  Blood in the stool has also been absent, even without prednisone, so we're hoping she can maintain this status.  The GI did say though that if she has another flare up with more frequent stools or blood present, Annie will most likely have to start taking stronger immunosuppressants--drugs that are typically used for chemotherapy.  That's made us very nervous, so we're praying that we don't have to go down that road.

On Tuesday I also had Annie into the pediatrician for her post-op follow-up, her UTI follow-up, and to talk to him about her weak cough, congested airway, and us starting to worry about pneumonia.  Her UA showed a possibility that her UTI is still present even after her full 10 day course of antibiotics, so we're waiting to see how the culture comes back.  The doctor said her lungs sound ok, like the congestion hasn't settled low enough that she has pneumonia yet, but he's concerned about her.  He wanted to start her on antibiotics to help prevent pneumonia, but with the urine culture still pending and the high risk of c-diff reoccurring, I asked him if we could hold off a little longer. 

So we should hear today or tomorrow about whether she needs another round of antibiotics for a UTI, and if her cough remains unproductive and weak by next Tuesday, we'll take her for a chest x-ray before considering antiobiotics for pneumonia since those are more likely to cause c-diff.  We also have an appointment next Tuesday with her pulmonologist, originally scheduled as her annual CPAP checkup, but it will be nice to talk to him about our pneumonia concerns as well. 

And as if it weren't enough to have Annie sick, Justin and I have each battled a short but nasty 24 hour bug this week.  At least the snow has finally melted--now I'm just hoping for some blue skies, sunshine, and temperatures to come out of the 30s and 40s.  We know to enjoy spring and summer when they eventually come, because in Nebraska they sure don't last very long!

The Ronald McDonald House

Staying at the Ronald McDonald house has been an incredibly huge blessing!  What a wonderful place!  This Ronald McDonald house here in Columbus is the second largest in the world (next to NYC) and has 80 guest rooms.  The guest rooms are very similar to hotel rooms and they just recently got TVs in every room (but no remotes--that's what Justin is for!) There are several "family rooms" or dens that are VERY nice with TVs or massage pillows, etc.  There's a gym, a movie theater, a very large library, two large laundry rooms, playroom, and a rec room with a pool table, etc.  On the main floor is a kitchen/dining area.  There are 8 full kitchens arranged around a large dining area.  There are refrigerators and freezers--one of each for every 3 guest rooms.  We knew that a full kitchen would be available to us, so we made sure to pack some canned and boxed food that we could easily prepare while here, yet so far all but about 3 meals have been provided for us!  Plus all the kitchen cabinets are already packed full of food for the guests.

Dinning room circled by 8 full kitchens.

One kitchen area where most of the meals are served.

Pantry area with freezers and guests' food lockers.

Outside organizations bring in lunches, dinners, and occasionally breakfasts as well.  Usually it's a church group of some sort bringing a meal in, but we've also been served by a girl scout troop and an honors society.  It has been so wonderful, especially with me being so sick, to not have to worry about preparing something.  These volunteers have been so nice and happy, and most of them ask about Annie and what they can pray about for her.  The entire Ronald McDonald house is run by volunteers which is pretty amazing, especially considering they have 1-2 people at the front desk 24 hours a day!  It takes special people to volunteer such selfless service!

Did I mention that staying at the Ronald McDonald house is completely free of charge?  I hate to think how much an 11 day stay at a local hotel would have cost!  But before you think we've been TOO spoiled... (well, we really have been very spoiled)... let me explain that we do have our own house responsibilities by being guests here.  Guests have to make up their own beds and are responsible for washing their own sheets and towels, taking out their own trash, and cleaning their own rooms and bathrooms.  Guests are also responsible for a daily chore--ours has been keeping the library clean.  We have to dust the shelves, pictures, and blinds, straighten the furniture and vacuum every day, but since the library doesn't seem to get used a whole lot, it's a pretty easy chore to do. Oh, and if you start a book from their library and just don't get the chance to finish it before you go, no problem, just take the book with you--it's yours.  They say they get new books donated all the time.  (Although Justin and I packed our own libraries with us, so we haven't needed to grab anything from the library here, but it has made a nice place to go for a change of scenery.)

The library

Library

One rule is that no food is allowed in guest rooms, and I was a little sad about that at first, but it's worked out just fine.  There was one night I just wanted to watch a movie in bed and eat popcorn and I couldn't, but the rooms feel so much cleaner than a hotel, maybe because of this rule alone.

It's been interesting to be on the opposite side of so many service projects this week instead of being in the church group usually providing the service.  In the guest bathrooms there are even hygiene kits in ziplock bags--I can't count all the times I've helped assemble hygiene kits with my church group for various organizations, and now I'm actually seeing how some of those are used and appreciated.  This isn't a hotel, so there's no little bottles of shampoo and lotion--but it's all been made available in the kits that have been donated.  Seeing all the ways to serve make me want to help out at our Omaha Ronald McDonald house--I think it's definitely an experience that would be neat to share with our church youth. 

One way RMH raises money is by collecting pop tops from soda or formula cans.  When Annie was on pediasure, we easily collected 4 pop tops a day, but now we don't seem to come across many with her on a powdered formula (and because we don't tend to drink a lot of pop).  But if you feel so inclined, it's an incredibly easy thing to do to help support the Ronald McDonald house.  Just collect your pop tops and drop them off at your local RMH.

Here's some other pictures from around the RMH.

This is our third floor foyer as we get off the elevator.  They clearly love their sports here!

This is a family room on the third floor where Justin spent most his time working.

Family room

The playroom

Another family room--actually called the meditation room.  The massage pillows work quite nicely!

A family room in the basement

One of the laundry rooms

Basement playroom

Rec room.  The basement is all themed after the Blue Jackets hockey team.

Rec room

Basement hallway

Here's some pictures of Nationwide Children's hospital.  I'm just now learning that not all Ronald McDonald houses are near the hospitals.  The ones I've seen before always have been, but I just learned a few days ago that Omaha's RMH is about 50 blocks away from the children's hospital.  So we are very fortunate to have the hospital so close.  We've been able to easily walk back and forth to grab meals and take naps.  Although we made sure never to be out alone after dark--all the workers here as well as at the hospital have made a point to let us know how unsafe this neighborhood is and they even provide escort services to help people walk more safely the short distance between the RMH and the hospital.

Main entrance hospital foyer

 If I had any photo software available to me now, I would have glued these following to pictures together to show a panoramic shot of the entire hospital.  It's rather large.

Left half of Nationwide--the section furthest left (the tallest building) is a new addition that should be open in a year.

The other half of Nationwide

A school collected these pop tabs during the year and brought them in one day for the RMH!  The money earned by recycling these pop tabs helps run the RMH and produces enough money to pay the entire electric bill for this 4 story building each month!  So collect your pop tabs for the RMH!

Salivary Gland Ablation, Part 2

Annie had the second half of her salivary gland ablation yesterday.  Fortunately this time she wasn't having crazy seizures beforehand.  We also explained that she was probably too heavily sedated last week since it took her 3 hours to get out of recovery and then another 4 hours after that before she woke up. 

Everything went very smoothly yesterday.  We were really hoping that this time the doctor would be able to get into one of her parotid glands to treat it, but he just wasn't able to.  He explained that the only way to safely treat the parotids is to find the actual teeny tiny hole in the wall of her mouth where the duct from the gland empties into her mouth, then insert his even tinier tube up into the duct in order to get the solution into the gland that will kill it.  Unfortunately, Annie has built up thick flaps of tissue on the walls of her mouth from where she bites her cheeks during seizures.  The doctor said Annie has too much tissue cluttering up the area where the duct opens into her mouth and he just can't access the ducts.  We asked if he could make a cut in the tissues in order to get to the ducts, but I guess that would put her at too great a risk of having the alcohol solution get somewhere other than the duct and the gland which would kill good, normal facial tissues.  His only suggestion for treating the parotids if we feel like she needs them treated, is to have ducts tied off.  We obviously aren't going to even consider doing that until we get her well from these surgeries and see their full effect.

Annie was only in recovery this time about an hour and a half and was then taken to her room on the pulmonology floor.  Our doctor had told us that if we were comfortable, since she had no major complications last week, he would allow us to be discharged later today.  We were extremely excited about that since Annie's hospital room had absolutely no parent bed in it!  Annie did incredibly well all day and at 7pm we started working on getting discharged.  Finally by 11:15pm we were discharged and immediately went to bed at the Ronald McDonald house. 

Annie still has a lot of congestion and thick phlegm, but we're starting to wonder if that's more from a possible cold since her main salivary glands have now all been killed.  Her tongue and chin and cheeks are also so swollen that she's just having a hard time dealing with even the smallest bit of congestion and phlegm.  She slept fabulously through the night on her CPAP, but has required a bit more suctioning today.  We plan to rest today, do our cleaning for the RMH, pack everything up and drive home tomorrow!  I can't wait to get Annie all healed and see really how effective this procedure is going to be for her!  Hopefully she won't have to wear soaking wet bibs everywhere she goes anymore!

Before surgery--our room, even smaller than before with NO parent bed at all!

Before surgery

After surgery

Starting to wake up

The lovely view from our window

We were definitely grateful for a doctor who discharged us early from this room!

Back at the RMH

Napping this afternoon

Not the Week We Expected!

So here it is the night before Annie's second surgery for her salivary gland ablation and I'm just barely updating our blog!  This week has definitely NOT turned out to be how we expected!  Annie was finally discharged from the hospital last Thursday evening, much later than we had expected, because she spiked high fevers of 103.8.  It took them most of the day to figure out the source of the fever, and we were relieved to find out it was just a UTI.  Not something we wanted to deal with right now, but easy to treat.

We've been glad we thought to bring Annie's suction machine with us because we used it several times an hour around the clock for the first several days of recovery and even today are still using it rather regularly still.  But generally, Annie has done very well recovering from part one of her salivary gland ablation.  I actually caught this little smile last Thursday after we got her settled for the night at the Ronald McDonald house.

We're noticing just a small amount of paralysis on the right side of her face, but that's often expected and almost always temporary, lasting anywhere from 3 weeks to 3 months. 

This picture below was taken just tonight.  She still has a small amount of swelling under her cheek and chin on her right side, but the swelling under her tongue has gone down even more.  I was expecting the swelling to be nearly gone by the time they do the left side, but I guess this is good enough!

The big thing that threw us off this week was the flu that hit me Friday morning.  I think all my stress and lack of sleep lately have finally caught up with me, although Justin believes I caught a bug at the hospital last week.  The floors are on lock-down due to all the cases of flu, especially on the pulmonology floor Annie has to be on, so the visitor policy is restricted, and patients are not allowed out of their rooms for any reason.  I thought I was being careful, but not enough, I guess.  I've literally been in bed about 95% of the time since Friday.  Justin has been wonderful in taking care of Annie as well as me, all while trying to work away from his office.  This was definitely not the leisurely vacation we had expected!  But today has been looking up and I think I'm finally starting to improve--just in time to go back to the germ-laden hospital!

We're disappointed that we had to cancel some fun plans while we're here.  We had planned to attend church nearby on Sunday and visit with a friend from Austin, Texas while she was also here on vacation, but we clearly didn't make it to church!  I was especially saddened to have to cancel plans with a friend here who also has a daughter with Aicardi Syndrome.  Chances to visit with Aicardi friends are so rare, I was really looking forward to seeing Ronda and Marissa, but of course couldn't take the chance of spreading anything to her sweet daughter.

On Sunday Justin helped me muster enough strength to drive to the Columbus temple.  We knew it was in the parking lot of the stake center, but we didn't realize quite how small it would be.  It is by far the smallest temple I've ever seen.  We thought about how wonderful it would be to have our temple right there so close to our chapel--no excuses for not regularly attending the temple! 

Tomorrow hopefully I'll feel better enough to actually take some pictures of the Ronald McDonald house since it's the 2nd largest in the world and has been such a wonderful place to stay.  I'll definitely post some updates on Annie as well.  She's been rather congested and phlegmy today and has been throwing up some bile, but we're hoping that's just still from the UTI or just trying to deal with her congestion.  It would be nice, ideally, to get her a little less junky before surgery, but hopefully after tomorrow she'll have much fewer secretions leading to all this congestion. 

On a side note, we found out after her last procedure that her sublingual  glands were rather large for her age.  The sublinguals are the glands that produce mucous, so hopefully once she gets both sublinguals treated we can say goodbye to all this phlegm and congestion.  We appreciate everyone's prayers for Annie and also for me!  Please continue to keep her in your prayers as she undergoes her second surgery tomorrow!

Day One of Annie's Salivary Gland Ablation

We made it to Columbus, Ohio yesterday evening at 9:00pm after a 13 hour drive.  We had a room held for us at the Ronald McDonald house across the street from Nationwide Children’s, but we soon learned that checking into a Ronald McDonald house is not as quick or easy as checking into a hotel.  First they took us on a tour, then they explained all the rules, as well as the chores that are expected of us during our stay. (Pictures of the Ronald McDonald house coming soon.)  We filled out all the paperwork and then were finally taken to our room around 10pm.  Then we had to put our sheets on the beds and make up our room.  

By then Annie started having more myoclonic seizures even after getting a dose of Ativan in the car for seizures that lasted over 2 hours earlier in the evening.  By the time I got her in bed, she started with a grand mal seizure on top of the myoclonic seizures—something I’ve never seen her do.  I ended up giving her a dose of Diastat to stop it all, yet Annie continued to have several more grand mal seizures—another thing she’s never done!  Diastat has always stopped seizures almost immediately.  We starting thinking about taking her to the ER, but she finally calmed down around 11:30pm and we were able to go to sleep.

We woke up this morning at 4:45 in order to check into the hospital at 6pm for Annie’s procedure.  Annie had the first portion of her salivary gland ablation surgery around 8:30pm and was in recovery from 9:30am-12:30pm!  The doctor injected her right submandibular and right sublingual glands—the left submandibular and left sublingual will be treated next week.  He planned to inject one parotid gland today, either the left or the right—whichever he could more easily access.  However, the parotid glands are accessed through the inside of her mouth through the duct in her cheek, and Annie has too much thick tissue build up on her cheek walls for the doctor to get to the ducts of the parotid glands.  Annie usually chews up her cheek and tongue when she has seizures, so I guess 8 years of that has resulted in really thick tissue.

Annie remained on CPAP and oxygen until about 4:00pm when she finally woke up.  She started coughing and struggling with phlegm in her throat, so I took her CPAP mask off in order to suction her.  I noticed that without the CPAP she was keeping her oxygen saturations at 95-100%, so we left her CPAP off for the rest of the day while she was awake.  

She was pretty alert and content until about 7:30pm tonight when she started growing more irritable.  She got some Tylenol since she hadn’t had anything for pain up to that point.  Then by 8:30pm they discovered that her temperature had spiked to 103.2!  (At about 6:00pm her temp was 97.8).  They don’t know what would be causing the fever, so they’re calling a doctor to come check her out.  Everything was going so well earlier today—I was feeling so good about all of this and was thrilled at how smoothly everything was going.  Now I’m not even sure if we’ll be able to discharge her tomorrow.  We appreciate everyone’s thoughts and prayers for her.  Please continue to keep her in your prayers.

Here's a few pictures from today...

Wasted even before the procedure--from having so many seizures in the previous 12 hours.

Annie's room.  I feel bad I ever complained about the hospital room we were in for 6 weeks last fall.  It was a luxurious mansion compared to this, but at least we don't plan to be here long!

Annie on CPAP and oxygen several hours after coming out of recovery.  Bruising from the ablation.

Finally awake!

Annie's tongue is the upper portion of what you see.  The bottom portion is the tissues under the tongue that normally lay beneath the level of your bottom teeth. She received an injection through that tissue and now it is swollen up well above the level of her bottom front teeth, pushing her tongue to the roof of her mouth.  This is clearly why they only treat one side at a time.

Trying to use suction to clear her throat of all her thick phlegm is a little tricky with this much swelling!

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!