Half Full

While it may not seem like it, I am always trying to look at the full part of my glass.  Well, this morning, my coffee cup!

Cole is actually drinking his instant breakfast as I type, instead of throwing it at the dog and he is up 10 ounces in the last week and a half.  Although he's still off the charts, he's moving in the right direction.  I'm not exactly sure what he's liked this past week but at least I've cooked or baked some winners.

Vials sent, sealed, and delivered fine.  Fingers crossed, blood marker results from Philly could be in as early as this afternoon.  From there I can only hope if the anticipated genetic tests are ordered the paperwork goes smoothly and approval is swift.  Cinci blood work will be a little longer, but I can be patient for a week, that isn't all that long these days.

Miraculously the car repair was not as bad as we feared, thus no more worries of being stranded on the side of a hot road with three kids.  Luckily, Nick's got a great partner at work who he happened to be carpooling with all summer, so there was no scrambling for a vehicle for our multiple medical commutes this week.  

In addition, it has been nice to spend some time outside in the sun with some very healthy friends.  After a long lonely winter, with little more than phone calls, a few of the girls' friends have come to swim.  They've had to stay outside, just in case, but Cole's afternoon nap has given the girls a little bit luxury where we have not had to say no for a change.  Due to my own appointments, they've even had a wonderful day with kids away from home, thanks to my friend Lori, brave enough to take them to art class, the playground, and then let them invade her house for the day with her own two kids, even though my #2 can be quite a challenge with hers.

We are still hopeful that I will be able to get back into my classroom this fall.  It is such a challenge to make decisions when you are still waiting for information.  I am so fortunate to be in a career where I have been able to take as much time as I have, and am optimistic to jump right back in with my colleagues, who I miss tremendously.  Again, I know I have changed over this journey, and know these individuals will treat me the same as before, which is all I want.  I don't want to be looked at as a sob story, because you know what, I don't feel sorry for myself.  We've welcomed one really cool kid into our family and he has opened our eyes to a different world.  I would never change that.  He just happens to have some hurdles to jump and needs our help, that's what parents do.  The team I work with is amazing, like a family, and I've been lucky to have them as co-workers and friends, and love that when I see them, it as we are picking up where we left off.  Looking forward to it on a regular basis somewhere on this road.

As a very proud, independent individual I have always wanted to do things myself because I could, and maybe I just don't want to seem weak or dependent.  I know many of you have generously asked what you can do, and honestly, I believe we are doing ok.  But the few times I have needed something and was just too darn stubborn to say anything, I am lucky to have people who just said they were doing this or that, whether watching the kids, dragging me out, understanding I was too tired for plans, or volunteering to take me to appointments for myself.  I am so thankful for you, and for those who really want to help, don't worry, I am not so shy or proud anymore that I will not ask, but am saving my requests for the days when I just may not be here to help if and when we are finally sent to Houston or Boston.     

Right now, your love, friendship, support, and understanding is all I need.  Knowing what I have in so many of you and the laughter of my children is what keeps me smiling past the rough patches.  That and, while it is cold, my coffee cup is still half full.

Long

Doing what I do when I'm stressed - cooking.  Planning to make spanakopita and cannolis.  I know, odd combination.  But one I love, the other should put some more meat on Cole's bones.

After spending the morning at the hospital, supposedly for blood tests, we were sent on our way.  According to the technician, there was an issue with how the specimens were to be transported that needed to be worked out before the draw could be done.  Really?  They've been coordinating this draw for two weeks, we have our out of network approvals and were told to show up today.  We have been jumping through hoops for a year now, still with no end in sight.  At this point if I could wave a wand to get answers other than "we need more tests" I would.  Actually, I'd settle for driving the blood to Philly and Cincinnati myself!

Honestly, my overall frustration level at being backed into a corner where I can do so little for my child is beyond words.  I know and appreciate that the doctors are doing what they can, especially by seeking other experts and looking into all their suspicions, but inside I'm irrationally screaming because when it is a child it nothing should take this long.

Needless to say, we are still waiting, not for answers, but for the draw itself.  Then the real wait starts.  For this moment, while my girls play and my boy sleeps, I will cook.  Hopefully the kids approve of today's treats.

Hurry Up

There are times lately where I just am at a loss for words.

Whirlwind doesn't begin to describe our last month.  Between the start of soccer and end of the year festivities, we survived a very long viral infection with the boy.  Luckily, while it was tough and fast, the flare that came after only lasted a few days.  However, we are now combating the after effects of the weight he has lost.

On the other hand, a few weeks ago we participated in the Take Steps Against Crohn's and Colitis in honor of Cole.  Not only was it powerful to see so many other people there in support of IBD, but heartwarming to be joined by so many people on our team in support of not just Cole, but our family.  It has been a challenging journey and this day reminded me that we are not alone, even though there are days it sure feels like it.

Throughout our medical odyssey, there has been tremendous "hurry up and wait" in terms of testing and moving forward.  It seems we've returned to the first part of the cycle once again.

Once again, we are heading for numerous blood tests as ordered by a world renowned immunologist whose specialty is nk cell function.

Late Friday afternoon we received a call from our immunologist outlining some of this doctor's concerns as well as his desire to have a number of new tests run that should offer answers, or at the least, further direction.  Due to the impressively low function, yet not the absence of function, this man believes there must be an underlying cause, not just Cole's IBD.  Again, the blood markers he is looking for in combination with the other panels being run should confirm or discredit some of his thoughts.

As before, some of the blood drawn will go to Cincinnati Children's.  Additionally, a portion will be sent to a lab in Philadelphia.  Some of the tests take a few days, some others, a week or two.  These waits aren't really all that long, considering the lengthy wait last December for Cole's genetic panel for NEMOS. Still, depending on the results we already know further genetic testing may be done and I am not looking forward to that wait.

But, no matter what, I will continue to be patient, one day at a time.  I will continue to share here, just know that there are days neither Nick or I have the words to express where we are and are just trying to keep things as normal as possible for the kids as we move down this road.  This new normal has taught me more than I could ever describe, and my heart goes out to those parents and families whose adventures are much harder than ours.  It is in talking to these families I realize how truly fortunate we are, no matter how difficult our path has been, it could be so much steeper.

Pictures

What a way to end winter!  Like a blanket on a cold day, we have been engulfed in warmth and have shaken the chill of winter from our bones.  This week has just been amazing!  From a picnic in the park, followed by countless walks, as well as chalk drawings and sprinkler runs it has been the best March I can ever remember!

Despite all the fun of the week we also had a number of appointments for Cole as well.  We started Monday with a seven vial draw.  Initially the immunologist told us her test results would take a few weeks, however they have already contacted us.  Unfortunately, Cole's tests show he is "naive" to all of the viruses they were looking for.  In other words, he has never had experience with these viruses, thus has not made any antibodies against them.  The hope was that the tests would show some antibodies against one of these viruses as it would indicate Cole's ability to handle these kinds of infections without serious problems.  Since they do not, we will continue keeping our man in his virtual bubble, prolonging the avoidance measures we have been employing for a while longer.  We'd rather be safe than sorry since it has been explained that any possible contraction of these illnesses could not only be serious, but life-threatening for Cole.  The sooner we know about any likely exposure or infection the sooner aggressive treatment with antivirals or IVIG can begin.

A year ago I think my head would have spun with the thoughts of intravenous treatments with one of my kids, but now, I have come to expect the unexpected in terms of what all of Cole's doctors might tell me and accept any test or treatment without blinking an eye.  When the girls were his age, I cringed at the idea of their shots, now, I realize shots are a piece of cake!  Honestly, right now I am more concerned with Cole's continued weight loss than anything else.  Losing weight tangible while all the rest is merely a matter of if.  Maybe that's an odd way to look at all this, but we are more or less no longer social exposures are pretty limited and that eases my mind a tad.

After the blood draws, Cole saw his pediatrician for a routine appointment.  Other than the additional weight loss, things looked ok.  At this point, Cole is now down about two pounds in the past month.  At least our doctor reassured us that we were doing all the right things to promote healthy eating, and ultimately, weight gain for Cole.  It would be a matter of whether or not he actually wanted to eat that would determine whether he would gain soon.  Since everything looks good right now, we just need clearance from g.i. and we will be able to move forward with a minor surgery we have had to put off for Cole.  Hopefully we can schedule soon so he is recovered before summer.

Once we were done with the back to back appointments, I wanted to enjoy the day with my two littles.  While we have to avoid crowds, as well as enclosed spaces, Cole's stroller on a quiet trail is just fine.  Since we have not been out as much as we were with the girls, it's funny to see Cole's entire face blaze with amazement as he discovers bits and pieces about his world.  Both Gwyn and Cole were fascinated with a pair of ducks in the park.  Gwyn's quacks and Cole's claps and giggles while the ducks waddled, flapped their wings, then splashed into the pond were absolutely priceless!  Just like the sounds of laughter floating through the neighborhood while the kids rode their bikes and ran through their artificial rain, these tiny moments have etched themselves alongside my other favorite memories from my time as mom, creating pictures of how I always imagined life with kids would be.  This is how I will choose to think of these days, even though indelible images of antiseptic waiting rooms and offices often monopolize my mind.

But

Never has there been a contradictory word that drives me crazy like the word 'but'.  It's a nails on the chalkboard kind of crazy or like my apostrophes gone wild pet peeve with my middle school students who sprinkle their writing with them as if they are topping an ice cream sundae.

So when our immunologist started off with a positive, I waited for the other shoe to drop after she uttered the word 'but'.

To begin, we were told the good things Cole has going for him, that most of his immune system was wonderful, but there was a key part of it that does not function.  This means that there are a particular class of viruses that could be life-threatening to him if he were to have exposure to them either directly or by the rest of the family having contact with them and bringing the shedded germs home.

However, due to the rarity of this nk cell dysfunction, the relative new nature of studying this disorder, as well as the limited amount of research in kids Cole's age, the actual diagnosis may not be solidified for another year. Before they will confirm this disorder with any kids, they usually have a least a year of poor nk cell tests, or at least six tests.  At this point, Cole has only had two.  Doctors also want to rule out the possibility that these results could stem from his g.i. disorder or class of drugs they are treating him with since there is a study that links the two with decreased nk cell function.  Once again, our physician is unclear since there is only an old study dealing with adults and the information regarding kids like Cole and their nk cell studies just are not available so it is tough to truly tell us if their is a link or if he can just be diagnosed.  For now, they are just classifying Cole as having an immunologic disorder. 

With the information we have, we also need to make some decisions about the level of risk we are willing to take with Cole's immunizations.  The majority of them are will be safe for him as they are killed vaccines, but any live vaccines can have life threatening complications for our little guy.  For example, if we do go ahead with the chicken pox vaccine and see one little dot, we need to take action immediately without waiting to see what happens because things could go downhill quickly.  At the same time, the benefit of the shot could outweigh the risk. Ultimately, we are gambling whether we get the shot or not.

Yet again, our immunologist has consulted another researcher.  This doctor is in the process of leaving the east coast for another position in the southwest.  If Cole's blood work next week shows he has not started to form and antibodies against the herpes class of viruses like chicken pox, Epstein Barr, etcetera we may need to meet with this doctor as we move forward.

In the meantime we are to do what we've been doing, keep up the limited exposure to everything, monitor what our older kids are exposed to, and wait.  At least the weather is incredible so we pace a different perimeter.

While we continue in the holding pattern, we are desperately trying anything to bulk up this boy.  In the past week he has continued to lose weight.  Not much, but it is still a loss despite the various high calorie/ high fat foods we have been offering him.  There's about a week and a half until his next weigh in at g.i.  At that point, without a significant gain we may be discussing other measures to get him moving in the right direction.

Overall, despite the long appointment and time our doctor spent with us, the news was about what she had already told us.  On the flip side, there wasn't anything new or worse, so we are pretty thankful today.

Once again, I cannot explain the gratitude I have for family and friends who are there when we need you yet also understand when we have just needed breathing room.  Your messages and phone calls have often come at times when I've doubted my strength or patience in all this waiting and while I know all our changes and sacrifices are for the right reasons, when I'm frustrated at them you've reminded me this is where I need to be now.  Please know, in my heart I know this and I am absolutely sure we are doing the best we can for Cole, and if faced with these choices again, I would make them all.  Everything happens for a reason, and your love, kindness, and prayers are appreciated so much more than I can say.

Love to all.

Gains

There's nothing like a taste of spring topped with sunshine to sweeten the start of any day.

Today was Cole's follow up appointment from his last colonoscopy.  Since they had already called us to discuss the discovery of the granuloma we figured this would be a relatively short visit.  At this point I'm come to realize anything less than an hour is just abnormal.  And just as our visits seem to be getting longer, they are also getting closer together.

Now that the biopsy shows a granuloma, the physicians are closer to truly diagnosing Cole with Crohn's Disease.  They are still hesitant because the presentation just is not quite right and they are trying to be entirely sure the nodules and inflammation are not symptoms of something else. There is still the fleeting thought that with Cole's immunological issues the gastro problems could be a side effect of another problem.  So, we continue to wait.  This is the same position we have been in for the past few months, as if our iPod is stuck, repeating the same song over and over.

Even though Cole is flaring right now the doctors are trying to stay away from another course of steroids due to the issues with his immune system.  In addition to suppressing his immune function further, the prednisone could also begin to impact his growth, which at this point is not going in the right direction.  Once again, Cole is losing weight.  This time he is down a little over a pound in the past three weeks.  Unless his symptoms begin to worsen, we are just to continue with our usual medications and work on getting Cole to not only eat, but to put on and maintain his weight.

To help with this task, the g.i. nutritionist will be in touch with us tomorrow to discuss how to bulk up Cole's diet.  One thing I do know, there is no weaning in my near future.  From the last visit I thought I might be able to nurse less, but the doctors have told me I cannot at this point.  I'm ok with this, I just was ready to know there would be no more unexpected bites in my future.

So as we wait to hear what immunology has to share with us next week, we keep working on trying anything and everything we can to get the little man growing.  Here's hoping for big gains in the next few weeks.

Deep Breathing

Experts say that deep breathing is one of the best exercises for staying calm and relieving stress.  Sure, it works, but so does driving down the Thruway with the radio blaring. Which happened to be the best medicine for me Wednesday morning.

It had been a long night with Paige, her fever had continued to spike over 104 even with medicine.  Initially when I called the doctor we were told to go to Children's, but she would have to contact them first to let them know we were on our way.  Before we could get out the door, the nurse called us back, telling us Children's did not want her there spreading whatever infection she had and that we needed to give the antibiotic time to work.  Even though we were not going, we needed to watch her closely, so I settled in the recliner with my book ready for the night shift.

High temperatures are frightening.  Even after popping her in tub, her temperature only fell a few tenths of degrees.  In between each bout of sweats as the fever broke and before its inevitable rise she would talk in her sleep. This would have been much funnier had I not been so worried about her.  The last time it happened, she sat straight up, looked at me and told me she "couldn't write anymore, there was something on her eraser!"  Then she proceeded to lay right back down, snuggling Pooh Bear as tight as could be.  Not long after, the chills set in, and after the last fresh tub, set of jammies, and bedding for the night at about 4, it seemed she was finally on the mend.  Once I knew she was ok, I allowed myself to shut my eyes, hoping for a few hours before the little man called for me.

To say I was looking forward to a nap not long after I awoke the next morning was an understatement!  Still, I was pretty happy that after three days she was actually asking to eat.  I didn't care what she wanted, I would make anything.  After polishing off two plain pink pancakes she scurried off to play with her sister.

Balance was returning to our house when the phone rang.  It was our immunologist.  I was caught off guard.  Shouldn't examining the specimen and running tests take longer?  I knew she would be calling, but in my mind I was prepared for a call in the afternoon.  Without hesitation, I was informed that due to the biopsy findings the game had changed.  The presence of a granuloma in addition to his other labs meant that Cole matched criteria for a disease they were not looking at before.

CGD, or Chronic Granulomatous Disease is now the illness they are considering.  Our physician explained that CGD is another rare primary immunodeficiency disease that would require a lifetime of medication and limits to activities as well as a possible bone marrow transplant.  Luckily, the test for this disease could be done here in Buffalo.  We would need to go to Children's for a test sometime this morning or wait until Monday since the expert would need two consecutive days to work with the live cells.  If we went today, we would know by the end of the week.  Needless to say, running on adrenaline and caffeine I packed up the baby and off we went.

On my way, my head was just spinning with a million what ifs.  The happy chatter from my little man only made my thoughts race faster.  How could any of this be?  Honestly, in this moment I could feel the anger pumping its way through my veins in a way I have never felt in my life.  Maybe it was the lack of sleep over the past week, since it seems I've been able to divert this feeling for some time.  Logically I know, CGD may not be what he has, but I still couldn't stop the feeling of fury as I thought of what his future might be if this test comes back as a positive.

My red passion melted quickly with a good song and the giggling drifting through the car from the back seat. I'm convinced he was laughing at my singing ability or lack thereof by the way.  The more I thought about it, I realized and understood that I was not angry, just absolutely terrified of what may lie ahead.  Not just all the medical stuff, but afraid of the heartbreak that could hit everyone we love. While valid, these are not places I wanted to let my mind to inhabit.

As I arrived at the hospital and held my precious child in my arms I vowed that no matter what I was feeling, each day would be cheerful, not just for Cole, but for the girls and Nick and I as well.  We would be optimistic and trust that all of this is happening for a reason.  I've even gone back to reminding myself daily not to dwell on what I cannot change, to lessen the risk of being consumed by worry so I am not gasping for breath like a fish out of water.  Instead, I will not look too far down the road when I don't have to, just take things one day at a time, one moment at a time, one breath at a time so I can stay in the eye of this storm.

Don't worry, as we wait, I am practicing my deep breathing in between belting out some silly songs as all the kids giggle.

Walking Outside

Every day is a new adventure it seems.  Once we got the phone calls from both genetics and immunology informing us about Cole's results we were also instructed on the next series of steps we needed to take, immediately.

Now that Cole tested negative for NEMOS, there was still the mystery as to what was the root cause of his NK cell dysfunction.  Our immunologist explained not only the case studies she had been comparing him to, but her contact with a leading physician and researcher at Cincinnati Children's Hospital.  They stated we would need to take Cole for additional function tests as soon as possible.  Besides the repeat NK cell test they would also be looking at his perforin/granzyme B functions as well as his IL-2 receptors.  Just like the last time, the blood would be drawn here, then shipped to Cincinnati for evaluation.


In addition to the function tests, now that the genetic results were in, the gastrointestinal doctors wanted Cole's colonoscopy scheduled as quickly as possible.  With a few calls, OR time was found and the procedure was scheduled for next week.


As I drove to our blood work appointment, I dreaded the draw.  Not the screaming I knew was coming, but his face. The absolute look of betrayal when I have to hold him down, then the sheer terror in his eyes as he is pierced while tube after tube is filled with his blood just shatters my heart in a million pieces every single time!  Luckily, today's phlebotomist was the best we've had up to this point.  Unlike others, the first time was a charm and she was easily able to fill the seven tubes she needed without utilizing the syringes to suck additional blood from Cole's veins.  Within a few minutes my baby was back in my arms, twirling my ponytail in his hand, his head lying on my shoulder.


It is truly incredible just how forgiving a little one can be.  Here I struggled from the moment I knew he needed more blood work and in an instant it was like he had forgotten that I assisted the woman who had caused him such pain.  Immediately I thought about the quote that "having children is to decide forever to have your heart go walking outside of your body." 


Initially I realized what this meant not long after our first daughter, Paige, was born.  She was colicky and I just had no idea what to do to soothe her.  After a few days when nothing was working I felt like I was doing something wrong.  Unless we were hiking it seemed like she cried 24/7.  My heart ached terribly when I saw my friends with happy babies while mine was so sad.  Fortunately, as she grew, she also grew out of her colic.  About that time, I needed to return to work and leave her which caused me a worry like I had never experienced.  After a few weeks, I knew she would be ok, after all, she was home with her dad.  Still, the idea that as her mom, I could not always be there each and every second she might need me broke my heart.  I wanted to be able to keep her safe from anything that could cause her pain, like when she was safely growing in my belly.  In the end I knew I could not shelter her from the world, that in order for her to grow and learn she would have successes and failures, and in both, joy and pain.  So while I would worry and share in her disappointments, I would also be proud of her achievements as she grew into an independent child, one learning to make choices and live with the consequences, with and without my help as the situations would allow.  


While more of my heart left my chest with babies two and three, I realized this feeling would not subside and was a natural one for any parent.  There is nothing like the love a parent has for a child not to mention the lengths we parents will go to keep our children safe and free from pain.  


While I still ache when I can't stop Cole's agony or afflictions, I know he is surrounded by love and in good hands, which is the most I can ask for, not just for him, but for all my kids.  No matter what they may endure in their lives, whether I am with them physically or not, a piece of my heart will always be with each of them wherever they may roam.  For this week, it means mine will be walking around a few classrooms and in a hospital operating room while I sit nervously in the waiting room, anxiously hoping for answers.