Cassidy's Next Step

Since my last post brought all of you up to date on Chloe, this one will do the same about Cassidy.  In some areas, we have made, or at least maintained, our progress.  In other areas, not so much.  As I always say, in the process of sorting out the causes and treatments for the kids various health challenges, we also work hard at trying to maintain a normal childhood for them.  It can be difficult to explain how hard this can sometimes be.  I recently saw a short video created to promote awareness of eosinophilic diseases, in which a mother of 2 EGID kids tried to explain how painfully frustrating it is when well meaning people make comments like "But they don't look sick" or "She looks fine to me".  The mother explained that it is more about what you don't see.  You don't see our kids when they are vomiting or have headaches, the fatigue, body aches, abdominal, chest & throat pain.  You don't see the lack of or loss of appetite or difficulty swallowing.  We keep our kids home when they are feeling this way.  So naturally, when most people do see our kids, they "don't look sick".  You also can't see the frustration that goes along with the strict dietary restrictions.  How would your child feel if they could never eat the cake at the birthday parties that they attended?  They can smell the pizza but cannot taste it.  They cannot have ice cream on the playdate or field trip to the creamery or amusement park when everyone else is trying to decide on one scoop or two.  As parents we struggle not only to keep our children nourished, but also to compensate for the things that can make them feel angry, frustrated or left out while also helping them to accept these aspects of their lives and not be defined by them. So while these comments may be well intentioned, they can be irritating to us as parents, and can make our children feel as though no one else really believes there is anything wrong at all.  To a child suffering with chronic pain, frequent tests and procedures, and extensive dietary restrictions, feeling as though someone does not believe that they have suffered is heartbreaking.




Now that I got that out of my system, on to the health update.  At the time of her scopes and biopsies in November, Cassidy's esophagus was clear, her stomach was normal (and had been prior as well) and the microscopic inflammation in her colon had gone from patchy to uniformly covering the colon.  Due to still having abdominal pain and the change in the inflammation, she started taking Asacol to treat her colon in February.  By late April, her lower abdominal pain was better but not completely gone, and she had added bouts of severe, intense mid and upper abdominal pain. On June 20th, one week after Chloe's biopsies, Cassidy went back in to be scoped and have biopsies taken again.  That evening after we came home and the next day she had abdominal pain so severe that we ended up back in the emergency room to rule out complications from the colonoscopy (there were none).  The lab reports showed that her esophagus is still clear (hooray!) but her stomach now shows some chronic inflammation.  It's microscopic, and a single finding of it at this point does not point to anything significant, so for now that information is just tucked away in case it changes down the road. Her colonoscopy still shows microscopic inflammation throughout, although there are some changes from last time.  I don't think it is really accurate to say that it is worse, but it isn't better either, just different.  As in the past colon biopsies, there were neutrophils present.  These are the most common type of white blood cell, and it is my understanding that they are commonly present most of the time where there is inflammation or infection, so that was not a surprise.  Also noted this time were eosinophils.  While that sounds alarming at first, it is important to note that about the only place in the body that it is normal to find small amounts of eosinophils is in the intestinal tract, and the amounts found in Cassidy's biopsies, while not specifically noted before, are within the normal range.  The last type noted were plasma cells. Plasma cells are also a type of white blood cell, and are usually present in cases of either a viral infection or an auto immune problem.  Again, these cells were noted in the lab report, but in small numbers that, at this point, are not specific or indicative of anything.  Maybe all of this will snowball into something worse, but maybe it won't.  Right now we have to take it at face value.  Wouldn't we expect to see less inflammation after months on the Asacol?  Probably.  But there is no way to know for sure if, had she not been on the meds, she would be much worse now.  There are no definitive answers as to whether it is helping or not.  Maybe without it she would have been in horrible shape.  Maybe she would have been fine.  No way to know for sure.  Regardless, while these findings are different, none of them really explain the level of severe pain that she battles from time to time.

So we have a couple of things that fall under "the next step".  First is ruling out any other possible causes of the newest pain.  Coming next week are CT scans of the abdomen and ultrasounds of the liver, pancreas and gallbladder.  Also on the agenda, which we actually started last Saturday, is removing gluten from her diet.  Cassidy does not have Celiac disease, but she could still be sensitive to or unable to tolerate gluten, which could, possibly, be to blame for some or all of her lower GI problems.  This does not change anything as far as her eosinophilic esophagitis, this is in addition to the EoE. So all of the previous dietary restrictions will remain in place as well.  We will be strictly gluten free for 6 weeks. She will either feel better a few weeks into this, or she won't.  Then we will have an answer to the gluten question either way.  Once that is worked out, we will address the Asacol.  She will stop taking it, and she will either stay the same (in which case it wasn't helping anyway, so we can safely wave goodbye to the side effects, such as headaches), or she will get worse, in which case we will have another piece to the puzzle and she will go back on the Asacol or maybe another similar medication that does not cause some of the same side effects for her.  We will wait until after the gluten test for that, because if we make both changes at once and she improves, we will not know for sure which treatment was effective.  Her siblings have been wonderfully supportive of the new restrictions, asking about the ingredients of everything that comes out of the grocery bag, and being careful, when spontaneously hugging their big sister, to not squeeze her "special tummy" too hard.

Maybe we will get some answers next week, maybe in a month, maybe later than that.   At this point, I find myself sometimes just hoping to ever know what is causing my child this chronic pain.  I believe that we are in excellent hands with our gastroenterologist.  I couldn't ask for a more thorough or devoted physician, and he and his staff are always patient with my endless questions.  Even when they are frustrated, the girls love Dr. A as well, which helps so much.  I am also endlessly thankful for the outpouring of support from our friends and family, always offering to help out and always being so understanding when we have to cancel plans at the last minute or need to vent.  Pretty much every ounce of our energy right now goes into helping all 4 kids cope with all of this, and that would be so much more difficult if we did not have the support system and medical team that we have.






Hopefully the rest of the summer will go smoothly.  Beyond the immediate needs associated with all of this, we are also looking forward to this time next year.  In July 2013, the American Partnership for Eosiniphilic Disorders (apfed.org) will be holding their annual conference in Philadelphia.  Only 45 minutes from my house?  Yes, please!  It will be wonderful if things are under control for both girls by then, but either way at least one of us will be attending.  Over the years, Cassidy and Chloe could improve greatly and require fewer, if any, medications and dietary restrictions.  Or they could get worse.  There is really no way to tell.  For now we will focus on getting through the next 6 gluten-free weeks, then the next couple of months after that.  Always moving towards the next step, one step at a time.

Chloe's Next Step

I realize that this post is long overdue, and that many of my readers have been waiting for an update on Cassidy and Chloe.  We have been busy with doctor's appointments, tests and procedures, new treatment attempts, and, just as important, trying to maintain some sense of just being a family and "regular" kids.  I believe my last post that included a significant update was this one, when Chloe went in for her first set of scopes and biopsies. I mentioned shortly after that post that Chloe was, in fact, diagnosed with eosinophilic esophagitis.  So here are the details on Chloe since then...

Chloe's first set of biopsies came back showing eosinophils totaling about 30 per high powered field, which exceeds the diagnostic criteria for eosinophilic esophagitis (greater that 15 eosinophils per field).  Given the fact that she was also having episodes of difficulty swallowing and food getting stuck (dysphagia) and not gaining weight well, and the fact that she has a sibling with the same disease, the gastroenterologist, Dr. A, felt confident in this diagnosis.  So he referred us back over to the allergy and immunology department to complete the 3 types of allergy testing that we went through with Cassidy, which you can read about here.  So we went ahead with the testing, and found that between the scratch testing and blood testing she showed a likely sensitivity or allergy to milk and eggs.  The patch testing was a little trickier, in my opinion.  As I have explained before, patch testing involves placing pure food samples against the skin for 2 days, them removing them.  Results are read 24 hours later.  Having gone through this with Cassidy, I remembered a few of her reactions being quite pronounced the night before the reading, but more mild by the time the doctor saw them.  In Cassidy's case, the mild reactions were still noted in her chart, and it turned out that there were 2 that she could tolerate (corn and bananas) but others that she reacted severely to when we tried to reintroduce them.  With this in mind, I wrote down the numbers of the test areas which had clearly visible reactions at bedtime the day we removed the test strips.  The next day, 5 of the 6 looked much better, showing only mild irritation, so I'm glad that I made note of them.  Hopefully they will not actually be relevant triggers for Chloe, but if removing the 2 foods with clear positive reactions isn't enough, I'll be glad that I have these additional foods to try.



The bottom line for the testing on Chloe is that eggs and milk were positive, and the additional foods that I noted were soy, wheat, celery, beef and sesame.  I learned later that during the test reading, while I thought that none of the mild irritants were noted, her chart does actually list wheat and soy as possibilities, in addition to the eggs and milk.  For now, none of that is immediatly relevant, so bear with me while I explain.

Because Chloe's cell counts were around 30, which is twice the number required for diagnosis but still on the lower end for this disease (to compare, Cassidy's counts were around 50-60 the first time), there are 2 possible treatment options to start with.  One is the method we used with Cassidy, allergy testing followed by removing all possible allergens turned up during that process for 6-8 weeks followed by repeat biopsies to monitor progress. The other option is not removing anything from the diet and just treating Chloe with PPIs (commonly a reflux medication, including brand names Prevacid and Prilosec).  Apparently, in some patients with lower counts this alone is effective.  We ended up trying the medication alone for 6 weeks, although I have to admit that shortly after this decision I just didn't have a good feeling about it.  But we crossed our fingers and hung in there.  2 weeks before the repeat biopsies (which was about 4 weeks on the medication), Chloe started eating less again, waking up in the night and eventually refusing solid food shortly before the procedure.  Mark and I went in the day of the biopsies and scopes, June 13th, with a bad feeling that things would not be much better in there.



We should have been wishing for things to be the same.  After 6 weeks on medication, Chloe's entire esophagus is inflamed to the point that the tissue looks grainy and is friable (bleeds easily),  the furrowing from the lower third of the esophagus is now extending up to include the middle third as well, and her entire esophagus is covered in little white patches, which lab reports confirm are eosinophilic abscesses. The lab listed the cell counts as high as 70, which is more than twice the number that was observed less that 2 months earlier.  No wonder she can't swallow!  Mark and I were horrified.  The gastroenterologist explained his plan of attack to us. Chloe is now on a steroid treatment for a minimum of 3 months.  After 4 weeks we will let the doctor know how she is feeling, and 4 weeks after that, she will have another scope with biopsies to confirm the effectiveness of the meds.  The point here is to help heal the damage enough that she has some respite for what she has been going through, and also to confirm that the medication is effective for her.  Once she is doing much better and has healed, we will address the issue of changing her diet, and hope that making such changes works well enough for her that she will not need to remain on steroid based medication.
Removing the food immediately may seem like a better idea, but the problem with that is that we would not know, once she gets better, which treatment is the one working for her.

So far she has been on the new meds for about a week and a half, and while she does not report any improvement in symptoms, it can take longer than this for enough healing to take place that she will feel noticeably better. She is eating a little better most days, thank goodness.  I should mention that once the allergy testing was completed, I realized that Chloe had been pretty much already on an egg and dairy free diet, mainly because of Cassidy's restrictions and also in case dairy was triggering Chloe's migraines, for nearly 6 months leading up to her first scope (when the cell counts were around 30).  We did reintroduce both in the couple of weeks before her scope, but she was no longer interested in consuming much of either.  Since she is still eating both now, it is possible that this could explain the worsening of her condition.  However, it should also be noted that while this may explain the increase in cell counts from 30 to 70, it also means that something else was causing a reaction that resulted in the initial 30 cell count biopsies.  Maybe milk and eggs will be enough, but I'm not holding my breath just yet.  I'm increasingly glad that I noted the other 5 possible allergens during her testing.

While we do our best to sort all of this out, we are also trying to make sure that Chloe and Cassidy (I'll get to Cass in my next post) are able to just be kids.  True, they don't feel great much of the time, but they still have to be encouraged to enjoy their lives.  Otherwise, it is easy to slip into a rut of feeling sorry for yourself.  Once that happens, you tend to let the disease define you rather that just be an aspect of your life.  So when they are feeling up to it, we encourage them to play, dance, create and goof off.  While they have always been close, we have noticed them growing even closer as they bond over their shared physical discomfort and and the way the disease effects each of them.  What makes them different from other kids is many ways is the same thing that makes them even more like each other, and that bond is proving to be as priceless as the sisterly bond that they have always had.