Blog #16: Thank You, Thank You, Thank You and What is Next

Wow.  For starters, you people are amazing!  I don't know the exact amount we all raised for TGen but I think it is near $16,500!  I haven't even finished my final thank you notes because we had donations coming in the day of the race!  Helping others is such a beautiful gift, something I hope you can all feel very proud that you participated in, whether you donated, said some prayers, or just thought about some of these amazing families that TGen serves.  We are so thankful for your generosity!  This is the first pic that was taken right after I finished (note Dave had already been done for an hour :)

I'm not sure many more half-marathons are in my future.  It was a good time and pretty exciting but I did get a bum right knee at about mile 7 and it oddly hurt worse and nearly froze if I tried to walk on it or stop..which meant I just had to keep running.  We had about 10 runners on our team and they all did an amazing job and it was so sweet of them to sacrifice so much of themselves for this cause.  We did a horrible job taking pics because people just roll in at such different times that it was impossible to get us all together, but I am hoping at some point to piecemeal the pics we do have together to share.

There were a couple of moving moments I experienced during the race .  One was when I got to the top of the hill at about mile 9 (on McDowell road in the midst of the Papago Mountains).  The view of the city from the top that morning was beautiful.  I actually stopped and took a couple of pics.  At that point on my ipod was a song that contained the following lyrics, "Greater things have yet to come, and greater things are still to be done in this city!  Greater things have yet to come, and greater things are still to be done in this city!"  I listened to it at least a couple of times for inspiration, but perhaps not-so coincidentally I couldn't help but keep thinking about my hope, my dream for premier medical care in Phoenix.

I know I'm going to pour out the cheese on this next paragraph, but it is honestly how strongly I feel about this.  I'm a fourth generation Arizonan and there are so few of us that I can't help but be filled with pride when I think about my family making its way to this state before it was even a state (and long before AC).  I even had a great-grandmother that had to swim across the Rio Grande in order to get in because Ellis Island was full, and then as a young teenage girl make her way to Arizona.  I know how desolate that trek is with I-10 in existence, can't imagine what that was like before.  I can't help but think about those people that built the CAP (the Central Arizona Project) long before we were one of the fastest growing cities in the country.  This has inevitably made us the "water-envy" of the west (granted not exactly the water-envy of the world, but it's good for the west!) and where would we be without it??  I have definitely drank the ASU President Michael Crow kool-aid and believe that we Phoenicians can take over the world!

Perhaps "the world" is a bit of an extreme, but can Phoenix be a premier center for 21st century medical care, now that would be my dream!  I was on the Board of Regents some 13ish years ago, and let me tell you that if someone had told me then that the UA would have a medical school in downtown Phoenix, I might have thought he/she moderately insane, and look at it now.  It resides in the old Phoenix Union buildings that my grandfather actually built...how cool is that!  And that ASU would take over downtown!  Even more surprising, it has essentially been that many years that we have been sequencing any person's genes, let alone thousands in any given year.  And that it would be offered for free to families desperately in search of a diagnoses for their sick child! Who would have thought!  I continue to be impressed by every article I read coming out about Barrows, about TGen...and throwing out that buzz word of all buzz words, I tell you Phoenix is creating some amazing synergies around our health!

So what are we going to do about it?  Some of you may gawk at my commentary and say we need an ivy league school in our backyard to make this real, or perhaps an endless supply of government money.  But in this day and age, all I have to say is that all you really need are the right people.  I already wrote about how surprised I was at the facilities in both NY and at the National Institute of Health...it really opened my eyes to how if we put the focus on the right people then facilities may not quite mirror the Taj Mahal, but we will have premier care.  There is a lot of talk in the media about access to health care, and don't get me wrong access is important, but it's only been 3 short years since we realized that access can only get you so far. When we REALLY needed medical care like we never had before, it wasn't about access, it was about finding the RIGHT, premier doctor to help us..and we went through a lot of ones that couldn't help us to get there.  It doesn't have to be this way.  We can build up organizations that truly strive for personalized, research-based care....greater things are yet to come!!

Our hope is obviously to continue to support efforts in gene therapy because at this point this is our best hope.  We hope that you will help us make that investment in Phoenix and anywhere there is promising research going on.  I don't know if this means at some point we will set up a separate foundation, or perhaps directly support causes that already benefit the many worthy non-profits that already exist.  We are still brainstorming the best approach.  I do know that we turned down numerous donors that came forward the last month that are insisting on us setting up an organization...only time (and some serious research) will tell.  In the mean time, we are happy to keep you updated on Emilia's progress every now and again and we are soooo thankful for your interest in her endeavors.  Your thoughts and prayers have been so comforting over the last few years.  Thanks for reading/running with us these last 6 months!! - Love, Mary

Blog #15: The Finish Line

To donate to our campaign, please click here: https://tgen.fundly.com/hall-of-famers/donate

Emilia and Ethan on Christmas Eve, aren't they so cute together :)

This particular blog has taken me quite a while to pen.  Although I always have planned to write on the topic of what it was like when I decided it was time to stop working, I couldn't quite organize my thoughts and memories..honestly some of it is a blur.  I kept starting and stopping, starting and stopping...just couldn't seem to get the discussion right.  I hated the idea of dragging you all along with a bunch of rhetoric. Strangely, I think the material has finally come to me while I was in the midst of cleaning the bathroom...strange, funny, but strange.

Since Emilia was a repeat c-section and I apparently lack all hormones that enable birth to proceed naturally, we were able to pick her delivery date that last week prior to her due date.  Things at work were quite a mess.  The previous six-months our entire organization had been in the midst of one of those "wonderful" externally-led reorganization processes..whew, was that a mouth full!  By the end of it, the department that I managed had been re-orged to a different organization.  This was such a stressful process.  I have to admit to losing my nerves at least a couple of times, and I don't think knowing that I was pregnant with a child that was having some difficulties helped throughout that process.  This meant that the department that I managed was no longer under a man that I whole-heartily trusted and respected (and had worked for 8 years).  I was only planning on coming back as part-time, so this also meant that this new boss was having to replace me with a new manager.  This meant there were some very nervous and uncomfortable employees, people that I have wonderful friendships with and who I really care about.  The worst part was probably that with all of the re-org madness, there was a lot of work that had been left wayside and I knew that if I wanted to leave the office in even a remotely respectable condition, I needed a few extra days before I delivered.  I tried to convince Dave to go with a date later and later.

Cooler minds prevailed (plus the schedule worked out that we could only deliver on the first business day after Thanksgiving).  Work was going to have to be a mess..so hopefully I could make a pretty quick recovery and help clean things up.  I have already written about Emilia's birth and the additional level of sensitivity it brought to my thoughts surrounding my future.  Well, after being home about six-weeks, things took a pretty drastic turn for the worst.  Emilia essentially quit eating.  She would nurse for maybe a minute or two to subdue her hunger I assume, but then she would scream bloody-murder like I had never heard before and refuse to eat any more.  We tried everything.  There came a point that I was just numb to it.  I know I have a couple of friends that have experienced something similar, and they have affirmed this reaction that I had.  Having your child refuse to eat, knowing quite well they cannot stay alive without eating, can pretty much drive you crazy.

As it came time for me to go back to work, at first the only person that could stay with Emilia was Dave.  And yes, he has a job so he was taking a day or two here and there to make this all work because he knew how stressed I was about how messy things were at work.  Let's add to this web that a new manager had been hired and everyone in the department had a new job in the works.  At one point my mom came in a couple of days to try to feed her while I worked for a half-day.  I knew things were getting bad because there were two-days in a row that I came home and my mother was teary-eyed.  She said it was just too stressful because Emilia would not eat anything all day and there had to be something wrong.  The last day we attempted to make a work day work out Dave decided to stay home.  By noon-ish he called me and said I'm driving the baby down to the office so you can feed her, she had not eaten all day.  I recall thinking he was crazy to think that I was going to be able to feed her, but I went along with it.  Of course she wouldn't eat...so we scheduled a pediatrician appointment.  The next day we went in and of course she had lost quite a bit of weight.  For any of you that have had that happen, I am so sorry.  They sic the wolves on you and you can tell the sound in their (the doctor's) voice has a heavy dose of fear.  Off to the gastroenterology doctor (GI doctor) we went.

Emilia and I went to see the GI doctor while the boys were at school and Dave was at work.  They weigted her in, it was bad.  We waited in the room for about 10 mins for the doctor to join us (a resident had already came in and asked us questions).  The doctor walked into the room and here is how the discussion progressed: Dr. "How quickly can you get to the emergency room?"  Me. "Uhh, what?  Um, well I have 2 other kids at school right now and I need to make arrangements, but I guess pretty quickly, how quickly do I need to get there?"  Dr.  "You have time to go home and pack your stuff.  You are going to be there for quite a while."  Me. "Um, what is wrong?  How long?"  Dr. "A couple weeks.  I'm going to go call the emergency room right now and make sure they let you right through to a room"  (It was winter and he said it would be horrible in the emergency room and she couldn't afford being exposed to it.)  I'm sure there was more commentary, but I was mostly in shock and didn't ask much, just did as I was told.  We were there (in the hospital) for about two-weeks.

I recall calling my new boss the second day or so we were in the hospital just explaining the circumstances and that I will probably be out a few days.  I called again in another two days..and again.  I eventually just said I didn't know when I could make it back in.  At this time I was also in the middle of a year of Tempe Leadership classes that I really enjoyed.  I was sitting on a couple of boards/committees for non-profits.  I recall leaving the hospital for the first time during that long stay somewhere around day 10.  Just thinking about what that felt like makes my stomach turn.  We really are very easily institutionalized beings.  I had grown so use to my 5 ft by 5 ft space and feeling like a zombie that those few hours of freedom to go hang with the boys and take them to Mass felt so awkward.  I'm pretty sure I cried through most of the Mass.  Driving home (I mean back to the hospital) that night I recall thinking to myself that everything is going to have to cease.  This is going to take all of what I have to offer.  From that point forward, I didn't even correspond with all of these baskets I had been juggling (work, organizations, Tempe Leadership), I just disappeared.  Every few weeks someone would check in and make sure all was okay and I feel horrible but I rarely responded...I simply had tunnel vision.  I was inspired to go even deeper in my research when I was put in touch with a woman that had two children with a rare neurological disorder that was essentially solved by a simple medication once they discovered the genetic error.  She was in research mode for a number of years and said she eventually solved it (and found the right doctor to get to) about 37 pages or so into a particular research paper.  I thought to myself about all of the research I had only been reading at a surface level.  My "home-work" just kept growing and growing.

The funny thing is that the world just keeps moving forward, as it should.  My old department moved back under my old boss.  They hired new employees and a new manager.  My former co-workers and friends started new jobs.  They changed the direction of the department.  My Tempe Leadership class completed an amazing group project, a community garden at the Escalante Center in Tempe. Etc, Etc.  I eventually went back in for a couple of weeks on a part-time basis to help them train some new employees.  Nobody ever mentioned anything about how messy things were.  And then I turned in my badge and parking pass (the premier downtown parking pass was really hard to let go of :).  Not to be dramatic, but it was a bit surreal walking to that parking garage for the last time...I really did love the people in that building.

In the midst of the madness, one day my boss (the one I worked for nearly all my career at APS) and I grabbed some coffee to discuss how I might be able to help them transition things.  He said something pretty profound that day that at the time I am not certain I appreciated.  When he asked how things were going and  I explained the madness he said the following, "Man, I'm just really glad it is you and Dave.  I'm just really glad it is you guys."  Uhhh, what...I thought to myself, among other thoughts.  I eventually internalized what he meant and I do know it is a really nice complement.  He is right.  We have been blessed with the skills, the resources, the initiative, and the energy to solve this.  These things don't just happen on accident, I don't care what science says.  Everything that has happened in my life has prepared me for this new journey.

So, will I work again?  Hum, probably.  I have to admit to being really interested in the business side of hospitals...well, because they are a mess.  I once told a nurse that I would be happy to develop an algorithm for them that could limit the number of nurse changes a patient had to endure during a stay (we once had 22 different nurses when there was only a possibility to have 26 different nurses during that stay)..wowsers, what a mess.  I don't know that it would ever be full-time again, that sure was complicated.  People always ask if it is harder to be at home with the kids instead of work.  I always respond that there is nothing harder than doing both.  In retrospect, working with no kids was easy.  Staying at home with the kids is not that difficult (painful sometimes, but manageable, and sometimes rewarding), but working with kids full-time was exhausting, often rewarding but it is not for the faint of heart.

We are just 4 days out from the race.  We have both completed our playlists for the run...ah, the important things!  Thank you all so much for your support these last few months.  For those that are not aware, our team actually raised almost $16,000 for TGen.  The entire TGen race family raised over $54,000..which is apparently the most for any charity affiliated with the race this year.  We are so thankful to have such generous friends and family.  You guys are really the amazing ones!  We will check in on race day and let you know how it went once we recover :)

Blog #14: Divine Intervention

To donate to our campaign, please click here: https://tgen.fundly.com/hall-of-famers/donate

While we were at the NIH (National Institute of Health) a few months ago, we had one day that we were able to sneak off campus and spend the afternoon in DC.  We took the subway to the nearest exit to the National Mall and walked through a few museums, and per Emilia's request, we played in the grass for a good hour or so.  As we walked aimlessly as it got dark, as I previously noted, we literally ran into the presidential motorcade.  We stood around at least 30 minutes as the crowds of people that were not permitted to cross the street while the motorcade was en route grew bigger.  After a while, I noticed a man staring rather intently at Emilia.  Granted this happens often.  Dave says the guy's name was Ken, I can't quite remember but lets call him Ken.  He was dressed in a very nice suit.  I kept looking at him and then at Dave to see if Dave was going to say something.  Sometimes when this (prolonged staring) happens, Dave is much braver than I and acknowledges the person and asks he/she how they are doing.  After a while, Dave looked at Ken and asked him how he was doing.

Ken was startled and you could tell a little embarrassed...he had honestly been looking at Emilia with a very perplexing face for at least a few minutes.  He fumbled his words for a few minutes and said something about his granddaughter being about the same age, this conversation going on while we were walking among the crowded street.  At one point, he just stopped and grabbed one of our arms and said the following (I'm paraphrasing the discussion from memory), "There is something about your daughter."  To which Dave replied, "Yeah, we know."  Ken: "No, there is something very special about her."  Dave:  "Yeah, we know."  Ken: "No, I mean something really special about her, I can  just tell.  I don't know what it is but she is going to do something amazing."  Dave:  "No, we get it, we know she is very special."  Ken:  "Can I pray over your daughter?"  Dave:  "Right here?"  Ken:  "Yes, right here, right now."  Dave:  "Of course."

So, in the midst of one of the busiest sidewalks this man proceeds to pray over Emilia for at least 5 minutes right in the middle of the sidewalk (for the record, 5 minutes is an eternity on a busy street in DC).  Emilia was such a pro about it, just sat there in the stroller listening intently.  I think that was the case because she really is such a pro at being prayed over.  This experience actually reminded me how we found out so early that Emilia has pulmonary hypertension, and I mentioned previously that I would tell that story..so here goes.

Some of the first people we told that Emilia was having lung problems in utero that could be very problematic were some very good friends of ours, the Welps.  We told them for the same reason I think people instinctively start doing the sign of  the cross in traumatic situations...we knew we needed a miracle.  We knew they and their small children would pray incessantly.  A few weeks after notifying them, Christian Welp suggested he wanted to arrange a "prayer meeting".  He had planned to invite a group of people who he knew to be charismatic prayer warriors.  This was something new to both of us and maybe even a bit out of our comfort zone, but we knew Christian knew what he was doing.  These people were amazing..so considerate, so prayerful, so hopeful and determined to have a profound impact.  And, it worked!  Each time we went back for an ultrasound to see if the fluid in the pleural of her lungs had waned and each time it was getting better.  Given the statistics going against this happening, it truly was a miracle.

 When Emilia was about 15 months old, Christian decided it was time for another prayer meeting.  She had started having these week-long episodes of throw-up and diarrhea and each time it was getting more and more difficult to keep her hydrated.  This "meeting" had a number of the same warriors but also included the priest from our parish (Father Charlie), a good friend who was actually the priest at our wedding.  That morning, Emilia started one of her episodes but this was worse than anything we had seen before.  Everything we put through her g-tube was coming back up, she kept nothing done.  Strangely, she was in good spirits and as long as you didn't feed her she was a happy little girl.  Again, the prayer meeting was amazing.  Emilia slept the best she had ever slept that night.  I recall that she didn't even wake up until after 10am the next day...which was unheard of, and to be honest with you, a little unsettling.  We thought for certain this meant that she would have a good day and hold down her food.

Boy were we wrong.  She had a horrible day, throwing up everything we put in.  We considered the emergency room but she was so happy and in good spirits that we decided to wait it out.  It lasted another day or so when I finally looked over at her sitting up and realized she was struggling to hold herself up...it was time for the emergency room.  While we waited in the emergency center for a room upstairs, I started inquiring about who our presiding physician would be.  I was REALLY hoping it would be the same one we had a year prior.  He was so considerate and thoughtful (for instance he stayed a good 2 hours after his shift the evening we were admitted to make sure everything went okay).  Obviously, all of the people told me the chances were slim because there are a lot of physicians.  You can imagine my surprise, joy, etc the next day during morning rounds when I looked out our room and saw this doctor.  I nearly tackled him, inquiring whether he would be taking care of Emilia.  He was definitely confused about my enthusiasm because he didn't remember us, but I trusted this man to listen to us.  We spent a few days to treat her dehydration but before he discharged us he stated the following, "I just feel so bad that you still don't have any solid answers after the last year, what else can I do, is there any test that hasn't been done?  I stated that we had not done an echo.  A doctor back in Cleveland recommended we see a cardiologist but we just hadn't gotten around to it.  I told him I didn't think she had any symptoms to justify it, but I would love to just know all is okay with her heart.  He said, "consider it done, I will order it now and you can leave once you have the results."

I think both, we and he, were surprised what they found.  She had pulmonary hypertension, which is something you generally find because people are symptomatic and quite progressed in the disease.  Finding this out as early as we did has given us the ability to seek out the right care and treatment, treatments that we are hoping have a preventative nature to them.  I thought about why we had such a horrible week coming out of that prayer meeting and I really started to rationalize this by saying to myself that this pulmonary hypertension must be an important data point, we must need to know this before it became obvious for a reason.  Now here we are nearly 2 years later and it is definitely what we consider the most pressing issue  and I think the second most important data point we  have (second only to her genetic error).  The doctors in New York confirmed that she may require gene therapy on her lungs someday, which has solidified for us our need to support advancement in this technology because the ability to do what she would need doesn't actually exist yet...wow.  There are times I get distracted by other issues she is having, but I am reminded of what data came out of that prayer meeting and each time I put my focus back on her heart and lungs.  We are so thankful for people's willingness to pray over our child, both perfect strangers and dear, dear friends.

Blog #13: Forgiveness

To donate to our campaign, please click here: https://tgen.fundly.com/hall-of-famers/donate

I’ll warn you that this blog is definitely some raw material...full of blood, sweat and tears.  Not an easy subject for me to discuss, but it is a reality not only for families with chronically ill children, but for all families in search of that perfect school.  I think I have reached the point that it is necessary for me to purge this subject and this memory, from my system.  I do this in the name of forgiveness because this is who, what I need to forgive.  I think we all know that the road to any type of healing is a long road to forgiveness...it is often a circular reference in my opinion.

When Ethan began Kindergarten, I recall having to leave the hospital to attend his meet the teacher night.  I also recall just feeling kind of whimpery that night.  Whimpery is not something I would say I am very often, in fact I’m usually awkwardly hard-core, but honestly the stress had just kind of gotten to me.  I was so sad because I just knew that my heart just wasn’t there with poor little Ethan.  I felt like a shell going through the motions because that it the effect of an extended hospital stay when you finally get a moment out in the real-world.  I was only going to be in town for another week or so then Emilia, Isaac and I were going to be off to Cleveland, Ohio for a three month stay at the Cleveland Clinic.  My mind was wrapped in the stress of that fact, and of course, as I mentioned, we were in the midst of another week-long hospital stay that could suck the life out of anyone.

Now I will be the first to admit that Ethan was by no means over prepared for Kindergarten these days.  We had the most stressful last year of our lives and all of the grandioso ideas I had for preparing a kid for school just kept getting pushed to the side.  Emilia’s health was all over the place that year.  We were in and out of the hospital.  I was trying to stop working at a place I had been 10 years…which is a blog in and of itself.  The kid had been read to every night of his life, he had been adequately loved, and even attended an average pre-school..I just hoped it would be enough to get him through those months I would be gone and then we would work hard again.  The part that was difficult to reconcile was that Ethan really just is a sweet, sensitive kid.  I can only imagine all of the terrible ideas going about in his creative head as the majority of his family set off without him.  He had to have been so confused.

For the eight months preceding Ethan’s first Kindergarten class, Emilia was throwing up at least 5 times per day…when I say at least this means that there were many days that it was as many as ten times per day.  Just catching it, cleaning up, washing the clothes, the blankets, the carseat sometime daily was enough to make me crazy.   We did have a system and the boys were pretty well trained to listen for the sounds. They would run and retrieve blankets for us to catch “it”…get us what we needed to clean it up…sometimes they were just the alarm to let us know.  They were pretty amazing, but needless to say we were all in survival mode.  Once school started, he was doing fine.  He was very comfortably adjusted and had tons of friends…he loved it.  The homework was intense and I know Dave struggled those first three months, both of them adjusting to the new circumstances.  I think we both questioned whether the type of rigor was necessary for our happy, high-energy 5-year old and did struggle a bit to take it seriously.  He was definitely initially behind, but not by an insurmountable amount.

The last 3 months of that school year, his progress had been huge...the slope of his curve had started to skyrocket.  As a side note, although I am no education expert I will offer up a couple of observations.  As a teacher, look at the individual slopes of each of your kids.  Perhaps graphing kid’s progress is not something we do, however there is so much to be deduced from seeing a slope as opposed to just a bunch of numbers…ps everyone in the business world uses a graph to evaluate information.  I know his slope from the last 3 months of school had drastically shot up, which meant I felt pretty confident the direction he was going.  You can imagine my shock when his teacher essentially recommended that he might benefit from another year of kindergarten.  Really?  He was reading?  I thought he was doing awesome in math??  He wasn’t overwhelmed.  He doesn’t have any social issues…what is the deal??

I met with an administrator to discuss what our options were.  This administrator was armed with every difficult task the 1^st graders would be asked to do…I guess to instill some fear in to me.  As a rookie, unaware of what 1^st graders could really do or what a normal school would ask, have to admit to being a little shocked and asked myself could he really do that next year.  Ironically there is nothing that she threw at me that isn’t an expectation of the boy’s current school’s first grade classroom.  It is easy to create fear in the inexperienced, luckily you are only inexperienced the first time around J.  After the display of premier 1^st grade expectations I said, “You do realize that my other son, my daughter and I were all gone across the country at a hospital while Ethan was here by himself with his dad for the first 3 months of school?”  There was even a point that I just started to cry.  This is hard for me to admit because it really doesn’t happen that often, but my stress level had pretty much maxed out and I wasn’t thinking all that clearly.   To their credit, after my blubbering she eventually said, "we’ll do whatever you want us to do.  If you think he will be fine, we will trust you."  Like I said, I was obsessed with his slope of progress..that should have been enough for me to be confident enough to say, he will be fine.  But I let the bitterness fester in me for a few days.  I reanalyzed the way in which I felt like she arrogantly displayed all the difficult work he was going to be expected to do in 1^st grade.  I also did some serious soul searching and a wise person said to me, “why do you think you need a school to max your kid out?  You don’t need that.  You can and will encourage your child to grow in areas he is interested in…you can’t do that when a school asks the max.” 

Well, there it was.  I had to let my own failures go…not having him adequately prepared for what I knew to be a rigorous kindergarten, the sadness I felt for having to leave him for those months when he probably really needed the rest of us more at that point than ever before.  I recall thinking to myself that a school that prides itself on instilling truth, beauty and goodness can’t lack the awe and respect for children that endure some tough circumstances.  If we are honest with ourselves, I think we know that it is these type of life experiences that foster those qualities..a school and it’s rigor will only do so much.  And the kid (Ethan) does have heart, more heart and fire and passion than I ever had. 

The irony is that years ago I set out to write a book on how you instill truth, beauty and goodness into young girls these days.  It was in writing this book that I actually ran across this school.  Ironically it is my sons who will learn the lessons of truth, beauty and goodness through Emilia’s life..it won’t be from a school, it won’t even be from me.   What I mean to come to terms with through this commentary is forgiveness.  Thinking of that year brings some serious strain to my heart, and it is time for me not to care anymore and to just forgive all the people involved.  After all, he is doing awesome.  He is light years ahead in his school work now, and he still has that fire and passion to boot.

I see these people enduring similar struggles as our family, some far worse.  I know they worry about whether they can make adequate money to keep their families comfortable or just provide.  I know they worry about the time they can spend with their children, their parents, friends, etc, whether it is quality.  I have to say that all you need to do is read the majority of “success” stories across our history, the Bible, any presidential biography or famous well-respected political figure…and they endured like no other.  In fact I hear the statistic for how many US presidents lost a parent at a very young age is incredibly high.  Greatness often arises out of the ashes.  Let’s not let ourselves feel guilty about teaching our children to endure and value suffering for another’s sake and let’s not feel guilty about the trials we may have to let them endure, whatever that thorn may be…it will be what inevitably makes he/she great.  After all, it has been said that if St. Paul had not endured “the thorn in his flesh”, he may have been too busy to sit down and write Romans….and what a loss that would have been.

On a side-note, we started Emilia on her heart medications today to help treat the pulmonary hypertension.  It is scarier than I thought.  We really don’t know what is going to happen…the side-effects could be painful (headaches, nausea, vomiting, etc), but they also suspect that it could cause her to have some other issues that would require another medication.  Needless to say I am supposed to be monitoring everything.  I hardly let her out of my sight today and so far so good.  We could use your prayers as we continue to increase the dosage over the next couple of weeks and monitor its impacts.  I probably won't write again until after Christmas.  We hope you have a wonderful Christmas, great time with family and friends, lots of thankfulness and of course an abundance forgiveness.  Thanks for reading and for all of your support!

Blog #12: Birth-Day!

To donate to our campaign, please click here: https://tgen.fundly.com/hall-of-famers/donate

We celebrated Emilia's 3rd birthday last week so I am going to go with the obvious and discuss some reflections on her birth-day.  Here is a picture we took the morning Emilia was born, in fact the picture was taken right before we left for the hospital.  Am loving how nice and swollen I was :)

I do recall after taking this picture thinking to myself that it would capture a "life before" moment, knowing that life would never be the same afterwards.  Now granted it is true every time you go to the hospital to bring another child into this world.  That eerie feeling you have when they let you leave the hospital with this tiny infant.  That first night home from the hospital when you begin to realize that you don't actually know what you are doing :).  But having known for 4 months that Emilia already had a very rare problem with her lungs in utero, there was just something different about this trip to the hospital.

We were scheduled for a repeat c-section.  For the record, I found the scheduled c-section much more awkward than the emergency one.  There is just something so very unnatural about being able to say, "Wow, I will have a new baby in exactly 7 minutes."  Plus it is very unnerving and I always found myself just so anxious for the process to begin so that you would know that all is okay.  That particular sense was really elevated with Emilia.  I really just wanted to know if she was okay.  It was a quick "retrieval" process and although I do recall seeing her face and noticing how small she appeared, the first thing I recall was the doctor saying, "Her head is large, someone should get an x-ray of her head."  Now before you are annoyed with our doctor's blunt commentary, he is a very dear friend and we love him and he would only say such thing because he wanted to make sure Emilia gets the best care.  In spite of her being ever so teeny-tiny (with a large head), she passed the simple tests do they always do and they sent us off to recovery together.

While I was trying to feed her, they kept trying to hook her up to get her oxygen levels, bp, etc.  The oxygen alarm just kept going off, but they kept just saying it wasn't getting a good reading.  We were very uncomfortable because we thought there was at least a decent chance she might have some oxygen problems, but it just kept being dismissed.  After about an hour, a very diligent and considerate nurse came in again and said that she was with us, there might just be something wrong and she put in an order for a doctor to come by.  It took some time..which of course was distressing, and the doctor that came spent about 3 minutes with us and said it can be normal to have some intermittent oxygen levels after a c-section birth (which is true) and we will just have to wait and see.  Wait and see...are these people nuts!  Don't you always wonder how they would feel if someone told them that??  I know they have to say it because I'm sure it does work itself out in most cases...but it sure does feel terrible to hear that phrase, "Wait and see."  On a side note, I later got the bill for this specific doctor visit and I considered writing "wait and see" on it.  PS - Saint Joseph's nurses have truly been our favorites throughout our hospital stays.

After that doctor left, that same diligent nurse said she was as uncomfortable as we were with that approach, especially in light of her size and she said she was going to work some magic to get a lung x-ray.  I don't recall how long it took, but I know that it was the last I saw of her for 24 hours when they took her for the x-ray.  She had a spontaneous pnuemothorax (essentially a collapsed lung) and her and Dave were off to the NICU.  I was stuck in recovery because of the c-section.  My job was apparently to recover and pump to supply the milk.  I'm pretty sure I was out of bed walking around by midnight that day so I could get the go ahead to head up to the NICU in the morning.  Dave was so comforted by the NICU because he just wanted to know that someone was watching her all of the time and that all types of care could be administered very quickly.  They put the first (of many) NG tubes down her nose so they could feed her so that she wouldn't scream and damage her lungs, and they hooked her up to some oxygen.

Some times I hate and some times I love smart phones and the ability to google search from anywhere.  I spent a fair amount of time that first night trying to understand if there was some sort of connection between these two pretty rare lung findings (one in utero and one at birth).  I honestly couldn't find a connection.  It was hard to find anything, including research papers, that mentioned them both...nothing was popping out to explain what was going on.  I really just wanted to understand what we should be looking for next, but google can only be so good at deciphering rare medical conditions on the spot (aka garbage in, garbage out). This is the reason I love teaching hospitals...the next day Dave and I were both able to sit in on the discussion between the lead doctor (for lack of a better term) in the NICU and a group of medical students as they discussed Emilia's case.  The doctor eventually asked the students what I had already been researching, "So do you think there is any connection between the unilateral pleural effusion in utero and spontaneous pnuemothorax at birth?"  A very eager student immediately raised his hand and said he had researched last night and read a tremendous amount about them both and he determined there is no connection.  The doctor said in sort of game show style, "Yoooouuuu'rrrrrreeeee wrong!  I don't care what your medical text books say, there will be a connection between the two." 

How amazing to be present for this conversation.  Hence began our research campaign to find a connection (and my love for mice and their willingness to endure endless tests :).  I was assured there had to be something underlying, and we needed to know what it was because only that could tell us what to expect next!  Emilia spent a few days in the NICU waiting for her lungs to heal and oxygen normalize then it was off to a continued care nursery to monitor how slow she was eating.  At this point they were feeding her the milk I supplied through a bottle and would put whatever she didn't drink through her NG tube.  NICU nurses are beyond experts at getting babies to eat what they need to so she was doing fine there.  It was a little more complicated in the continued care nursery, and although she started eating the volume they wanted her to after a few days, it was way slower than they were comfortable with.  We (being such rookies) insisted we were experts and argued that it had to be hard for her to eat with that damn tube up her nose and down her throat.  We wanted them to remove it so we could see if she would do better without it, but they just weren't comfortable with that.  Fortunately for us, Emilia was on our team.  She yanked it out in the middle of the night and the night nurse just went with it and surprisingly she ate everything they threw at her.  As a side note, when you stay in the hospital after a normal birth and you breast feed...the babies eat very little.  They are so tired.  In fact you leave the hospital thinking that breast feeding a baby is going to be quite easy because it really isn't that often (boy does that change once you are home :).  In this situation, when the hospital controls the health and well-being of your child, they are put on a strict feeding schedule and volume.  Boy was this unnerving, and although hindsight is 20/20 and we would obviously approach this stay differently now, at the time we just did not agree that she needed to be eating the type of numbers they were throwing around.

The next day we fought quite hard with that same "game show" doctor from the NICU to get her released.  At one point he even raised his voice at us and said, "If we don't get this feeding thing right, you will be back here in a month!"  (For those of you that don't know, he was right...we were back in the hospital in a month).  We kept pushing and pushing and finally he submitted.  We ended our time together with asking him, "What do we do now?"  (We were meaning to ask, are there other doctors we should be seeing, what should we be looking for, etc).  He was such a quirky man (don't get me wrong, we loved this about him) and he said, "I don't know.  Take your child home and love her.  She probably has some type of condition.  Maybe she'll need some special education, who knows."  Wowsers was that more information than we wanted...but we appreciated his honest commentary.

Off to home we went...only to discover that it would be nearly 8 months before Emilia would sleep more than 20 minutes consecutively at night.  Oh my gosh, what was going on??!!  I have filled in some of the holes already and I still have some more but I will let you off the hook with this blog.  The last 3 years have been some of the most exhausting and yet most fulfilling of times.  We wouldn't change a thing about her...she is just perfect the way that she is.  And for the record, I don't think she is going to need any special education.  Here is commentary from a discussion we had this weekend (yes, she just turned 3 years old.)

Boys:  "Why do we have to clean our room?" Me: "Because there are people coming over on Sunday for a party to help raise money for the doctors that are trying to help Emilia."  Emilia: "What did you say!?"  Me:  "There are people coming over on Sunday for a party to help raise money for the doctors that are trying to help you."  Emilia:  "I don't want to raise any money for those doctors.  I don't want to help them.  All they want to do is give me shots!"  See what I mean, nothing gets by this girl!

Blog #11: G-tubes and Bikes

To donate to our campaign, please click here: https://tgen.fundly.com/hall-of-famers/donate

For Emilia's first birthday (11/29/11) we had a soup puree party.  We had just gotten back from spending 3 months in Cleveland at the Cleveland Clinic and she had been occasionally eating pureed foods by mouth in decent amounts.  As many of you are aware, she had an NG tube placed at 2 months and a G-tube at 8 months, so I would be making a major understatement if I said eating by mouth has been "complicated".  I don't think I realized just how complicated it was until experiencing just how easy eating can be with our 4th child Gigi (more to come on this).  These next two pics from her first birthday party are some of my favorites.

Here she is before we tried to feed the soups to her.  You should notice the 3-1oz cups of the different soups.  At the feeding clinic, you had to measure all volumes of food so we could keep track of calories..a time consuming task that we still essentially do but I have gotten much better at estimating after the last few years.  Like I referenced above, it is amazing how easy it is to feed a child if he/she actually likes to eat.  With Emilia's eating, we honestly feel like we are slowly peeling back each layer of an onion until we finally arrive at a state in which she may actually enjoy eating.

 Aha, and here is a pic right after we tried to get her to eat the soups :).  Doesn't the face say it all...she looks like we are trying to cause some serious harm to her.  Now back to the onion.  Here are the layers we have either scientifically solved for or divinely discovered or simply arrived at by sheer LUCK in chronological order:  Aspiration (milk going into her lungs), volume sensitivity, rate (how quickly you pump formula into her tube), day-time versus overnight volumes, texture sensitivities, caloric needs (versus doctor wants), numerous bouts of dehydration, gluten sensitivity (celiac), lactose (we are just limiting not eliminating this) and little to no appetite (this may never be solved, but we are really in a major testing phase of this right now)..wowsers, and I always thought babies/kids just ate when they were hungry.  Each of these was solved with trial and error, there was/is no easy answer.

Getting back to Gigi and food/drink.  The child (10-months old now) will eat or drink just about everything she can get her hands on.  For instance, I don't think we ever gave juice to our other 3 children until they were 2+ so we could keep their milk intake high.  Gigi will find any random juice box and chug it down before you can even find her.  Sometimes I watch her chug...all ~7oz in less than a minute, then I think about how many hours it takes to get 7oz of any type of liquid into Emilia.  Good news is that we noticed that Emilia has experienced an interest in juice boxes (she would never drink juice before) because she watched her sister chug them down.  Needless to say, both girls are now drinking at least 1 (and an occasional 2) juice boxes a day.  Poor Gigi (aka the fourth child)..we will no doubt ruin her teeth by allowing this, but have to be honest with you that if by chance we can get Emilia to drink an entire juice box (a feat I would have previously said would require a miracle) by allowing Gigi this treat, we are going to have to run with it at this stage.

I am slightly embarrassed to admit this next crazy accommodation I have made to get calories in Emilia.  After learning at the NIH that Emilia doesn't seem to have major blood sugar issues at this point (although she does have low blood sugar) and that her caloric needs appear to be closer to 80% of age appropriate, we decided to take on an aggressive change to her diet.  We stopped giving her any formula through her tube during the day (just give her water or pedialyte) and decided to see if she would develop any sort of appetite.  We still feed her formula through her tube all night long but we essentially cut her calories in half.  It took more than a week before I saw any hint of an appetite, and even at that it was minimal.  She has definitely enjoyed not having to get the formula during the day.  So much so that now that I have added back just a couple of day-time formula feeds (she just wasn't making up enough calories by mouth), she is REALLY making me work for them.  It started with her telling me that I could put the milk in her tube (we do this via a 2oz syringe) only if I she was able to run and I chased her while doing it.  Some of you that are familiar with G-tubes might be able to imagine how difficult this is.  One, I have to hunch over to reach her cord and put the syringe in.  Then, I have to run within 6 inches of her as she mazes throughout the kitchen, family room, an occasionally run outside without pulling the syringe out (and worse would be her cord or tube), all while pushing down on the syringe so that it actually goes into her stomach but not too fast to make her throw-up.  This is very difficult, and let's just say that the one time she made Dave do it over the weekend, he wasn't terribly pleased :).  She has escalated it now.  Yesterday her new rule was that she would only permit the formula if I chased her on her bike.  At first I thought she was kidding, but then she went and got on her bike and said, "I'm ready".  She is super fast on her little 3-wheeler/ big wheel...and let's just say that if she keeps up this requirement, I may just be ready for the half-marathon long before I thought I would be.  It is a true test of my agility/ability/sanity/will/etc....the kid is such a crack up.

Dealing with these feeding issues is where I have really learned to appreciate subtleties....something you are just thankful for one bite or one sip.  In life, there are so many times that we want to check something off a list, and it is usually something substantial so it is these subtle successes that we just overlook.  Being a food/meal LOVER, when Emilia first got her G-tube, I remember thinking how sad life would be if you couldn't enjoy a meal with a friend or a starbucks break from work (oh how I miss the starbucks at the AZ Center)...food is so social.  The food table is where/when we learn to communicate, to gather and sort out the day, where most people give thanks and often say the only prayer they may pray that day.  We have done everything we can to be sure that Emilia experiences all of this "living" that takes place at the table, but it has not been easy.  To end on a funny note, she loves to say the prayer before dinner and this is generally how it goes, "I am thankful for momma, dadda, ju-ju (Julia), molly (these are her cousins), gianna sofia, and then usually picks one brother to name."  This usually makes the one she leaves off pretty angry and we can't help but assume that she is doing it on purpose because she rotates which one she leaves off but always says all of the other names.  Something tells me she is not far from normal in many regards :)..love that girl's feistiness.

Blog #10: Progress in New York

To donate to our campaign, please click here: https://tgen.fundly.com/hall-of-famers/donate

<---- Brothers and sister(s) reunited!

Well we have been home for a week, but I must admit to still trying to catch up and get things back to normal, hence my delay in summarizing our New York adventures.  I'll just start where I left off, now isn't that novel :).  One of the last things the doctors at the National Institute of Health (NIH) said to us was, "The best advice we can give you right now is that you need to go see the best cardiologist you can find that understands Emilia's condition."  Luckily, we had already arranged that visit knowing that her heart/lung problems are our most pressing issue right now.  So we were off to New York.

Small diversion...I think this is a good time to note some general observations I made over the two weeks.  The first I already alluded to when I noted that the NIH was somewhat surprisingly not the Taj Mahal of medical institutions.  Well, neither was the children's hospital at Columbia.  I know they are just older facilities than us west coasters are accustomed to, but I also found some of their practices to be surprising.  For instance, sharing rooms was the norm at both facilities.  Their doctors seemed much more like "normal" people than I experienced at many of the other facilities (this is a compliment :).  And rather than feeling like we were trapped in a prison with little control over our destiny (and child's well-being), everyone really treated us like we knew what we were doing and gave us so much flexibility.  I think what I learned from this experience is that quality of care isn't always correlated with my personal comfort, bells and whistles and fancy "anything" (including doctors).  And yes, I get that resources are generally finite and if you spend your money on your facilities and fancy autonomous systems, you may not have enough to hire the right people.  I definitely have an even grander appreciation for the right people.

Here is what we learned in New York.  Her lung tissue looks very similar to a handful of other patients that they have seen with a similar genetic error, but she is in the very early stages and they suspect that her tissue will get progressively worse as the other patient's had.  They were most fascinated to see a person at this early stage.  Generally, patients with pulmonary hypertension go see a doctor because they are symptomatic.  They are running out of breathe, tiring easily, etc.  We can't say definitively that she has any of these symptoms at all.  She certainly is more reserved, does tire more easily than the boys (but they are crazy)...but as many of you know, she is generally pretty high-energy.  They are hoping to find out if early treatment will help delay some of the progressiveness of the disease.  In case you don't know, the drug that is most commonly used to treat this disorder is viagra...yes, it was actually developed to treat pulmonary hypertension but just happen to have this interesting side effect that I am certain has made the pharmaceutical company far more money :).

There were some other interesting findings:  her heart pressures responded most positively to oxygen, and for part of my conversation with the doctor she was really contemplating putting her on oxygen.  Although this would be far from the worst thing that could happen, I did find the idea pretty daunting at this point.  Even if it were just at night.  She is already hooked up to a G-tube pump all night.  She is a horrible sleeper..wowsers, another cord??  Anyhow, the doctor eventually decided we would skip the oxygen for now.  They also did what they called a "fluid challenge" during the heart cath.  She did really bad with this and all of her numbers worsened.  To be honest, I am still trying to understand what this means.  Essentially all they did was very quickly put ~8oz through her IV and measure what her heart does with it.  The doctor initially suggested she take a "water pill" to resolve this particular issue.  But after discussing how low her fluid volume intake is right now (we are lucky to get in the age appropriate daily recommended amount), she decided to hold off on the water pill until we can get her fluid levels up higher (that will require a miracle).

We also learned, as I angrily posted on facebook that she is lactose intolerant, her intestines "just don't look right"...unsure of what this means but hoping to find out soon, and we learned that the long-term solution to her pulmonary hypertension may just be a lung transplant...wowsers, I had no idea that if you have a genetic deficiency like hers that a lung transplant would hold up (meaning not be over-run by the same error).  This was good news.  In fact they described that the patients they treated with the similar error went through all of the various hypertension treatments on the market.  They all worked for a while then they transitioned to another level of treatment and a few of them received lung transplants.  Apparently we were in the right place.

Some of the more entertaining moments were: riding the New York City bus over 100 blocks for at least an hour and a half to get into mid-town...don't ask me why we took the bus as opposed to the Subway...we are idiots.  Watching Emilia be completely alert and respond to numerous questions in the operating room despite the fact that they had given her medicine that was suppose to make her loopy and fall asleep??  When we had to the stay night after the heart cath, we entered our shared room only to see the most frightened looks you can imagine on a mother and father of a very small child (maybe a few months old).  You could see the fear once they realized they had to share a room...and thus internalized that they are suppose to somehow miraculously keep their child from screaming all night.  I must say if felt quite good to be able to say to them, "Please do not worry about us.  We have been in your shoes.  It will not bother me if your baby screams all night.  Do not worry about us."  I must admit to feeling quite prideful in their relief.  St. Patrick's Cathedral.  The Milk Bar.  Trick-or-treating in the City...

The things I might try hard to forget:  the plane rides, listening to Emilia tell us over and over again, "I want to stay at the "hotel", I want to stay at the "hotel"".  She's a smart girl and knew it was her safest place.  Driving in New York.  Letting Gigi and Dave walk to the apartment 12 blocks away from the hospital at 10 pm.  Being exhausted nearly everyday.  I wouldn't tend to say I am faint of heart.  I generally think I am pretty tough, but those two weeks were some of the hardest and most mentally, physically and emotionally draining thus far.  Not sure we would plan a double-visit like we did this time, next year...unless I successfully forget about the exhaustion.

One thing I didn't explain quite yet is how we came to know about her pulmonary hypertension at such an early time.  We have actually known since March of 2012 when she was just about 15 months old.  It was a miraculous chain of events that led to its discovery that I will have to explain later, perhaps next week.  I have always believed that it was going to be key to both her diagnosis and her over all well-being since it took such a curious set of circumstances to discover it....more to come later.  Thanks for taking the time read about our adventures.  We really appreciate your love, support and prayers.