Blog #11: G-tubes and Bikes

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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

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<---- 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.