Five years ago today, Keegan received a miracle.  An angel heart that saved his life.  In fact at this moment five years ago, the surgery itself was just getting underway, and we had no idea if he would come back to us out of the OR, yet alone be here today.

I read an article on a support site for congenital heart defects called Heartwaves.  The author discussed the idea that most CHD families can identify a “moment of impact” where the reality of having a critically ill child hits you.  I know exactly when my moment of impact occurred.  It was September 18, 2007.  Dr. Guleserian had just told us the day before that Keegan could not come off of ECMO unless he miraculously received a heart transplant, the odds of which were stacked against us.  We had a million meetings with different doctors and staff members over the next day.  We were supposed to sit down with a social worker a little after noon to discuss our family support systems for caring for Keegan if he received a new heart and what preparations we were making if he didn’t.  I wasn’t leaving the ICU very often, if at all, but Gray begged me to go down to the basement cafeteria to pick up a lunch tray, even if I didn’t eat it.  As we waited at the elevator bank on the lobby level with trays in hand, I watched the other families swirl around me, coming and going, in the normal parts of their days.  I sank against the wall and knew that “normal” no longer existed for us.  I was drowned by a feeling that my son would never leave the hospital alive.

Of course twenty four short hours later, that would prove not to be true.  Our miracle would come in the form of a little boy named Johnston.  But for his parents, Kevin and LaMonica, my moment of impact would become their reality.  In their weakest moments, they found the strength to bless our family with the gift of life.  I fear the day I might again be faced with the decisions they made on this day five years ago, but I pray that I may have the courage they did.  I pray that I will never forget to be grateful for every breath Keegan takes for as we are keenly aware, it is given just as quickly as it is taken away.  More than anything, I pray that in five years, I can look back and say once again how blessed we are for each day and continue to be humbled by the gift of life we have been given.

I have tried and tried to start this post, to restart this blog actually, a million times now.  Quite literally, I’m afraid, over the course of the last few months.  It is easy enough to update when Keegan is in the hospital or when we have concrete answers.  Unfortunately, those answers are hard to come by.  They always have been with Keegan and still are, even though we are getting closer to them.  I dread answering the question, “how is Keegan doing?”  It seems impossible to answer at times.  I dread almost as much when someone asks how I am doing.  That also seems somewhat out of my grasp these days.  So, I usually answer “oh, ok” in person and have been avoiding any attempt to answer them on the blog just as much.

Instead of avoiding everything, I think it’s time to start over.  The first and foremost goal of this blog was always to be a chronicle of Keegan’s medical history.  Even if no one ever read it or understood some (or even most) of what I wrote, we wanted to have an accurate recounting of Keegan’s medical issues.  It kept our family and friends apprised of Keegan’s condition from the very beginning, as Gray actually started the first version when Keegan was but a few hours old.  It has helped us prepare to take him to Boston and to Cincinnati and to refresh our memories from trying times when doctors asked us for detailed information about his history.  It allowed those near and far to bless us with the power of prayer on Keegan’s behalf for the last nearly five years, and in rarer circumstances, it helped connect us to other families on a similar road.  Here and there, I felt the need to share my personal feelings and insights from this life also.  Partly because I felt it may help other heart/transplant/medically-complicated families, partly because it helped me process the complexity of the situation, and partly because at times I desperately feel the need to explain to others what this journey is like.  Whether that be challenging, rewarding, crushing, enlightening, or simply tiring.

However somewhere along the way, I started to feel the pressure of the “blog world” creep in to my “medical journal”.  This pressure that extends from blogs to Facebook and beyond.  To portray a perfect life, a perfect family, a perfect home, perfect crafts, perfect food, etc, etc, etc.  Shiny, happy people everywhere you look.  Keegan’s health and developmental state often keep us from doing many normal activities, even simple crafts at home.  We don’t have a spare dime to spend on making improvements to our house or taking trips, and I am certainly the furthest thing from a gourmet cook.  On the flip side, I didn’t want to be another family with medical/special needs that lives nothing but that world, all the time.  Nor do I have any deep philosophical truths to share.  I’m honestly not sure my brain is capable of even shallow truths these days.  I suppose I felt at some point that if I didn’t have something well-written with flawless photos that was (a) perfect, (b) creative, (c) brilliant, or even (d) mildly comprehensible to share, I might as well not share anything at all.

Remembering that this blog was not to serve my own purposes but Keegan’s is the main reason I am recommitting myself to updating.  It is a disservice to him, both medically and for his own knowledge, if I fail.  Keegan deserves to have this information about his medical battles in order to understand where he has come from and to make future decisions about his care.

If I am to be brutally honest with myself and any reader who happens here though, I do have the selfish reason of writing as therapy.  Congratulations, you are now my new therapist!  God and everyone else knows I don’t have the money for the real stuff.  As I felt that perfection pressure increasing, so did my inability to cope constructively with just about every aspect of my life.  I am pretty good at soldiering on and keeping a straight face.  It served me extremely well once upon a time in a courtroom.  (Remember that little piece of history where I was a somewhat successful trial lawyer?  Yeah, neither do I.)  The truth is that behind the facade was the crushing feeling that I was failing at absolutely everything.  Failing at taking care of Keegan and making sure that I was giving him every possible advantage.  Failing at making a home, at being a wife, at being a mom, at meeting Audrey’s very distinct needs compared to her brother’s.  Failing because I was not earning a living to contribute to our financial situation, failing to repay the debt of a law degree that I worked so hard to receive.  I dreaded getting on the internet and seeing others tell of successes that seemed so far away to me.  And then, well geez, I was even failing at keeping up the blog!  The endless cycle just kept going.

I wanted to break free of it, but I felt bound by the looming uncertainty of Keegan’s remaining genetic labs that we were due back last week.  How could I commit to changes if with one test we could be thrown into seeking treatment for a new diagnosis or starting a new round of drugs that would change everything.  If the tests confirmed a form of Severe Combined Immunodeficiency Disorder (SCID), we would be pursing the possibility of a form of bone marrow transplant.  If they did not, we were hoping that the research labs being performed by the rheumatology group would give us guidance for a stronger immunosuppressant that would help us wean Keegan’s steroids and prevent flares, but also make him even more at risk for infection.  How do you pull yourself by the ol’ bootstraps if you don’t know what tomorrow is going to bring?

Unfortunately, we learned last week that the lab in Canada that was supposedly working on the genetics never received the blood we sent in June.  12 weeks of waiting utterly down the drain.  I refuse to go into details here.  Suffice it to say that policy at Children’s has been changed so that this mistake will not happen again to another family.  If Keegan stays stable for the next 10 weeks or even stays able to fight flares as successfully as he has recently, the only harm caused by the lab error will be more anxiety from the wait.  We decided to cautiously tackle a painstakingly slow steroid wean so that the remaining wait is not a complete loss.  One milligram per month, starting last Thursday.  Currently, Keegan is back to what we have come to call his baseline.  I hesitate to call it stable, as the slightest upset can capsize the whole boat, but right now, there is nothing going on that we can’t handle at home.  Some days are worse than others.  This weekend was a rollercoaster ride; Keegan was extremely tired and had a 36 hour rise in his heart rate and blood pressure.  Not good signs for him, but he ultimately came back to more comfortable numbers by last night.  He is powering through his therapy sessions and making small gains each week.  He is developmentally very similar to Audrey and started showing some classic two-year-old behaviors that are very encouraging.

The truth though is that the anxiety of the unknown and the grief of an unrealized dream for my family robbed me of joy.  I admit and acknowledge that, and I am confessing it here in order to keep myself accountable in the future.  It is not the first time in the last few years that I have succumbed to this worry and fear.  It just seemed more acute this time.  It felt as if there was more on the line with Audrey added to our family and being on the cusp of a five-year quest for answers for Keegan.  The truth is that anxiety stems from a need for control.  (A constant source of issues for me since childhood.)  But I was never in control in the first place.  This life and purpose belongs to God and no other.  If I know that I have placed my trust in Him, if I truly believe that He will make these struggles glorified, then my anxiety is a waste of energy that could be put elsewhere.

People often quote the adage “God will not give you anything you can’t handle.”  Any person who has been told that as a means of comfort knows how it can stab at your heart.  But the missing ending of that phrase is that He will not give you anything you can’t handle if you place your trust in Him.  He doesn’t expect me, nor want me, to shoulder this burden on my own.  That is the truth.  One I am putting great effort into remembering daily and am renewing a desire to seek daily.  If you stuck with me through this post, I can’t thank you enough.  Heck, if you stuck with me through the last few months, I really owe you.  I hope that I can live up to my promise and desire to post here more regularly.  For Keegan’s sake and mine.

And that is definitely the truth.

I ran.

Literally had to brush dust off of a dri-fit shirt and laced up some running shoes that should have been trashed a long, long time ago.  I made it 28 minutes before having to take a break for a call from the transplant team.  That was about 24 minutes longer than I thought I would make it.

But I ran.

It felt foreign at first.  I was training for a marathon when I found out I was pregnant with Keegan.  Ran regularly until I was eight months pregnant.  Started running again as soon as he was home from the hospital, but as he got sicker, I couldn’t figure out how to work running in to our crazy schedule.  I would get one in here or there over the years.  But then Audrey came and Keegan got sicker.  Excuses?  Maybe. But not today.

Today, I ran.

I felt alone.  Very alone without my Rusty.  My faithful running partner for 8 years.  The one who logged hundreds and hundreds of miles with me.  The one who was always ready to hit the road or trail, no matter the time, weather, or how crappy the day had been.  The one who tackled every brutal hill in middle Tennessee with a tail wagging with excitement.  The one who urged me to go on when I felt I couldn’t.  The one who made sure I was never alone.  Today, for the first time since 2002, I ran alone.

But I ran.

My heart felt like fire.  My legs felt like they would buckle at any minute.  But every time I thought of giving up, I realized how it must feel to be my son every day.  The pain and fatigue he carries with him daily but most often with a smile.  The pokes, the tests, the fever, the hypertension, the intestinal failure.  Rarely does he let it get the best of him.  And he gave me courage to go on.  To hold my shoulders back, loosen my hands, and feel the pavement beneath my feet.

And I ran.

My mind raced with all the uncertainties facing our family right now.  But in that moment, all that mattered was to put one foot in front of the other.  The only way we know how to go forward right now.

I ran.

And in the end, I felt better.  And grateful.  Because I could have been running away from everything, but instead, I ran home.  Home to my precious Bug who inspires me every day to keep going.  I felt energized to face the rest of the day, week, year.  I sure hope I can keep it up.  So maybe tomorrow I can also say….

I ran.

If you are local to the Dallas area or have read this blog for awhile, you may already know about and be a fan of a documentary series on Children’s Medical Center Dallas called Children’s Med that is currently airing it’s second season.  One of our dear little friends, Rylynn, received her angel heart last October.  She was featured on the first season of the show while waiting on a Berlin heart, and the first two episodes of this season showed the story of her transplant.  You can watch the second episode on the Children’s YouTube channel or by clicking HERE.

I wanted to share this tonight for several reasons.  First and foremost, we are so happy for Rylynn and her family and want to share her story.  Second, while we may have had some issues arise over the years, our transplant team (you’ll get to see Keegan’s surgeon, Dr. G, and our transplant coordinator, Susan, in the show) does amazing work every day that truly deserves to be celebrated.  Additionally, it is a real life view into the operating room during a heart transplant.  This is what our son endured four and a half years ago.  Dr. G explains the transplant process very well in this episode.  Keegan did not have the same defect as Ry, but he went from a form of mechanical life support (ECMO) to bypass to transplant.  He was also only 7 days old and weighed barely 5 pounds, with the heart the size of a walnut.

More than anything though, watching this episode brought on a wave of emotions and memories that I try hard to not think about too often.  I distinctly remember Susan walking into the waiting room (in the old CVICU) and falling to my knees with exhaustion and gratitude when she said Keegan’s donor heart was finally in and very “snappy.”  Although I have seen my son wheeled down the OR hallway post-surgery more times than I would like to count since then, I can still see his little face peeking out of blankets and tubes.  I remember saying, just like Andrea, “he looks so good, so pink!”  But I also remember realizing, as Dr. G describes, that my son would clinically die on that table that night.  For even the briefest of moments, no heart would beat in his chest.  No breaths would be taken as an entire cardiac team waited to see if his angel heart would bring him back to life.  Back to me.  And I am rocked with that grief and yet the renewal of knowing he was given a second chance all over again.

I don’t know what I would have done or what our lives would have been like if things had gone differently that day.  Or if a miracle in the form of a little boy named Johnston wouldn’t have come our way.  Still, I wouldn’t have chosen any other life…any other child.  I couldn’t.  He was chosen for me.

We flew out on Tuesday, the 24th.  This was Audrey’s first plane ride ever and only Keegan’s second on a commercial flight.  I am not a big fan of airplanes, especially small ones.  We only purchased three tickets, taking Audrey as a “lap child” (which by the way, is my new favorite phrase…seriously, what is she? A chihuahua?).  We got more than a few sideways glances for pulling out the clorox wipes and going over everything in an arm’s radius, not to mention for Keegan’s mask.  Overall, the kids did great, and the day went pretty smoothly.

We stayed with a friend of mine from high school, Amy, and her amazing family in a suburb outside of Cincinnati.  Talk about making us feel welcome and easing a stressful situation.  Keegan and Addie (2 months apart) hadn’t seen each other in over a year but were instant friends once again.  I think Addie had a difficult time understanding why Keegan wasn’t talking to her much, but they had a great time anyway.  Matt and Amy’s youngest, Jameson, is three months older than Audrey and a bone marrow transplant survivor.  It is helpful to have someone to talk to who has lived through what you are facing (bone marrow and solid organ are two vastly different transplants), but to have true friends who are honest and loving and really understand your fears and day-to-day life…priceless.  Absolutely priceless.  We stayed up way too late each night talking and sharing.  Maybe it is a blessing in disguise that we don’t live closer to each other.  We may never get anything done otherwise!

• Keegan’s last set of labs were very stable, and his HLH markers were actually a touch lower too.  We are trying to stretch out time between labs, so if all else seems ok, we won’t recheck until the 23rd!  We have continued to wean Keegan’s steroids by a paltry amount every other week, but at least we’re still weaning without too many big hiccups.
• He has been off of tube feeds since before Christmas and mostly maintaining his electrolyte levels with a few tweaks and supplements.  Since the last steroid reduction, we’ve seen some of his old tummy issues start to come back, but we’re not ready to draw conclusions from it.
• Keegan has an appointment with his nephrologist tomorrow.  His kidney numbers have looked okay, but we haven’t been able to reduce his diuretics or blood pressure medications.  We’re anticipating a repeat of the kidney function test we did last year sometime in the near future.
• Friday, he will finish his developmental/autism testing at Scottish Rite hospital.  The neuropsychologist at Children’s did the initial testing at the beginning of December, and the developmental specialists at Scottish Rite will help us round everything out.  I didn’t ever really explain all that went into the autism diagnosis.  Essentially, the neuropsychologist reviewed all the brain MRIs Keegan has ever had and used them to interpret the examinations and testing that she had Keegan do.  Ultimately, Keegan has suffered some extensive brain damage over the years from hypoxic events (lack of oxygen to the brain from his surgeries, ECMO, heart defect, etc), strokes, and the increased pressure on his central nervous system from inflammation due to HLH.  Much of Keegan’s “autistic” behavior can be directly related to a specific medical issue, however the autism label will help us get the best treatment and education for him.  All of that is currently taking a backseat to his medical issues though.  

We are still on the fence of making a diagnosis for what Keegan is facing.  If you happened to do a web search when I first mentioned Macrophage Activation Syndrome (MAS), you would have found that it is virtually indistinguishable at presentation from Hemophagocytic Lymphohistiocytosis (HLH).  In theory, MAS is a complication of juvenile rheumatic diseases (such as rheumatoid arthritis, vasculitis, etc), and HLH is a blood disorder that can be genetic, reactive (in response to an infection), or idiopathic (no known trigger).  Both are, to put it in hugely simplified terms, conditions where the immune system goes completely nuts.  The worst of both conditions results in what is called a “cytokine storm”, where there is so much inflammation in your body, that it feels like the worst flu you have ever had….times about a million.  Again, if you looked up cytokine storm when I first mentioned it, you probably would have seen that it was likely a cytokine storm that was the cause of death in otherwise healthy people after H1N1 exposure.

The criteria for diagnosis are the same; both cause raging inflammation in the body, decreased blood counts across the board (white, red, and platelets), coagulopathy, etc.  But the treatment differs widely.  MAS is treated with a variety of immunosuppressants, including high dose steroids and a drug called anakinra.  Keegan has completed the three-day steroid pulse and been on anakinra for two days now.  HLH, however, uses the same drugs, but also involves a round of chemotherapy and possibly bone marrow transplantation.  People with underlying rheumatoid disorders can have recurrent MAS flares that are not fatal.  Children with HLH rarely survive a relapse, prevention of which is the reason for transplantation.

While Keegan meets every one of the criteria for diagnosis of either or both, his medical history makes it more difficult for us to decide at first glance which one this is, and thus, what the next step should be.  No one knows what either looks like under the umbrella of an immunosuppressive regimen after a heart transplant at 7 days of age.  I mentioned a few days ago that the many doctors here working on Keegan’s case where rethinking his many ups and downs over the last four years.  It appears that what we thought were a strange combination of isolated incidents could have been recurring flares of MAS/HLH that were mediated by his heart transplant anti-rejection drugs.  A few examples:

• Keegan has been cytopenic his entire life.  This means he has never had enough of any type of blood cell (hemoglobin, white blood cells/neutrophils, and platelets).  At times this has caused him to be dependent on blood transfusions, GCSF and epoetin injections, and platelets replacements.  
• Two of his three liver biopsies showed evidence of hemophagocytosis.
• We thought he had a rare form of hemolytic uremic syndrome (HUS) induced by one of his transplant medications, tacrolimus.  By reviewing some of the labs from that timeframe, it is possible that this may have actually been a MAS/HLH flare that we inadvertently stopped by switching him to a drug called Simulect.  High IL-2 levels in your blood are a criterion for HLH, and Simulect is an IL-2 interceptor.  This may explain why Keegan made such an unexpected recovery from HUS.  We also gave Keegan five doses 
• Most recently, Keegan’s bone marrow biopsy results show signs of hemophagocytosis.  He also has had some recent bleeds in his brain that may have been aggravated by the MAS/HLH.

Some of Keegan’s labs have improved with the current course; some have not.  His inflammation markers are slowly trending down.  One level, ferritin, has normal values of 0-75.  Levels over 10,000 are the cut-off for chemotherapy in general according to hematology’s protocols.  Keegan’s levels peaked on Saturday at 48,000.  His blood counts improved slightly with steroids and the blood transfusion, but they have already returned to their old levels.  He is still bleeding, and his blood has not regained the ability to clot properly, despite three transfusions of fibrinogen and a blood transfusion.  He is either asleep, completely agitated, or “zoned out”.  It’s this last development that has been of most concern.  Tomorrow, we may have to run an EEG of his brain activity in order to know for sure that he is not experiencing seizures of some kind or repeat his brain MRI to ensure the bleeds have not grown.

Tomorrow the entire team of doctors will meet again to discuss Keegan’s case.  This time, the oncologist specializing in HLH at the hospital will attend, as will the ICU attending.  These “team meetings” are astonishing to me.  This never happens at home.  I think we knew last week that what we were dealing with was extremely serious when we walked into a room of 20 doctors with drawn faces telling us how sick Keegan was.  And we hadn’t even seen the worst of it yet.  Yet, it’s really hard to admit that your child is sick when they are on the regular cardiology floor, not intubated, not on dialysis.  There is obviously no denying it now, but it still could be lightyears worse.  And that is what everyone is scared of, right now.  Because “lightyears worse” could happen tomorrow, two weeks from now, a year from now.  And what will that look like?  Will it be something Keegan can come back from the next time?  What should we or can we do to prevent a “next time”…. especially one that he can’t fight.

The decisions we will have to make tomorrow and in the coming days are ones that no parent wants or should have to make.  It is not nearly as clear cut as it was at first glance.  No one wants to do too much too fast if it isn’t necessary, but we have no crystal ball.  There is no way to know what will happen, no matter which path we choose.  It appears that no one thinks it is as much of an “if” as a “when”, but how much are we willing to risk now if we don’t know when the “when” will come.  We are trying our hardest to gather as much information as we can and surround ourselves with as many people who are experts in their fields as possible.  And right now, I think that’s the only thing we can do.  No one is going to be able to make this decision completely for us, and unfortunately, there are enough different ways of treating MAS and/or HLH that not all of the doctors will ever be in complete agreement.  Anything we do will be taking a huge risk.  I just pray that we choose the one with the biggest benefit because I can’t afford to lose him.  And I refuse to let him go through this ever again.

So once more, I will ask for your prayers not only for Keegan but also for me, Gray, and every doctor working tirelessly to help him.  We have no doubt that he is in the best possible hands.  Those of his doctors, his family, and every single pair of hands lifting him up in prayer to the Father.  Thank you for helping us to help him on this road.