Saturday, July 31, 2010

What is normal?

The usual or expected state = Normal

Normal is pretty much a textbook misnomer. Is there such a thing as a usual or expected state? Of course there is, however, there are infinite normals. Your normal is unique to you. The funny thing about normal is its ability to transform itself and take on a new form. It's an unstable phenomenon. Its foundation is built on shifting sands.

As events, circumstances, interruptions of fate, tragedies, happenstance, and other interventions occur, normal reinvents itself. The alteration of one's normal gives birth to a new normal.

Our precious Zoe Madison brought with her, into our lives, a new normal. A new normal filled with twice daily medication administration, daily vital sign checks, bi-monthly cardiology visits, monitoring, obsession over food intake, and a myriad of worrying.

The added attention to Zoe provides Emerson with unsupervised time to work on her arts and crafts (yikes!):

Zoe's most recent cardiology visit was moved up a couple days due to a recent vomiting spell. On nearly a half-dozen occasions in the last week, during or after a feeding, Zoe has projectile vomited large quantities of milk.

At the visit, as is custom, Zoe underwent a blood pressure check, EKG, and ECHO. After the tests, the doctor relayed to us that Zoe, and her heart, looked good. Precisely the news we were looking for.

Every cardiology visit is fraught with nerves, anxiety, hope, and fear - all at once. The collection of these emotions represents our new normal. Granted, we have the same joy, excitement, and deep love with Zoe that is present with Emerson, but Zoe's condition carries with the known risks of loss and thoughts of worst-case scenario. Not that we don't fill ourselves with hope for Zoe's path, but the reality of her condition is ubiquitous.

At the recent visit, the cardiologist switched Zoe from Zantac to Prevacid, in an attempt to quell her reflux and vomiting. He also reported that her ECHO looked good and there was no need to do a repeat ECHO at the next visit. Waiting for the ECHO results always generates a recipe of fear, worry, anxiety, and hope. But receiving good news is like an injection of optimism, which immediately overpowers the uneasy pre-ECHO emotions.

After leaving the appointment and going about our day, several hours later we received a call from the cardiologist. He said he reviewed the ECHO in more detail and spoke to Zoe's cardiologist in Philadelphia and wanted to give us an update. First thought - this can't be good! What could possibly be the problem? These and about a dozen other questions ran through our mind in the split second pause between the doctor's sentences. He explained that the velocity of the blood flow through the heart is higher than normal. This is an issue because it can signify a blockage or narrowing, also known as coarctation, of the aortic arch. A coarctation of the aortic arch can cause the single left ventricle to become overworked, thereby increasing the load within the already fragile shunt. With coarctation of the aortic arch, in addition to increased velocity, symptoms can include increased blood pressures in one or more limbs, inability to palpate pulses in the lower extremities, or symptoms of heart failure.

Fortunately, Zoe did not display any symptoms other than the increased velocity. Therefore, neither cardiologist is too worried, however, they agree that we need to monitor the issue closely. In order to do so, an ECHO will now be performed at each cardiology visit until the second surgery in mid-September. If it were to develop (now is the time to knock on wood), treatment for coarctation of the aortic arch includes a catheterization with an angioplasty, where a balloon is inserted into the arch and blown up to expand and eliminate the blockage. Let's hope we don't get to that point.

On Friday, we had an appointment at Phoenix Childrens' Hospital for an ultrasound of Zoe’s clotted left leg. Her left leg developed clots in the iliac and femoral arteries when she had her cardiac catheterization in Philadelphia in late-May. CHOP wanted a follow up ultrasound to determine the integrity of the of the arteries, primarily for future catheterization procedures. Zoe was a trooper during the ultrasound, although she did manage to land several solid kicks on the ultrasound technician’s arm.

The radiologist spoke to us afterward and said the left iliac and femoral arteries remain occluded, but this won’t affect the leg itself because several collateral arteries developed which allow for blood flow through the leg. At this point, the occluded arteries will never function as intended and will probably not allow for another catheter procedure at that site.

The radiologist also indicated that her femoral vein on the right side, where a catheterization procedure was also performed in late-May, appeared to be partially occluded. As with the occlusions on the other side, however, the blood flow also continues to be properly directed due to the development of collateral blood flow. This shouldn't affect Zoe’s well being, but again could complicate future catheterizations.

For the next couple months the focus is on getting to the second surgery, the Glenn, which should occur in mid-September. Until then, we continue to keep a watchful eye on Zoe and hold our breath. Her weight fluctuated a bit this past week and the cardiologist suggested he might like to hospitalize her in order to insert an NG feeding tube in her nose as a way to increase nutrition. He indicated that he’s only had one patient who made it from the Norwood to the Glenn without a feeding tube. Our goal is to give him a second patient, so we’ve been working hard trying to get Zoe to eat as much as possible. The obstacle is her reflux and occasional vomiting. Every bottle we feed her carries with it some mild stress, as we constantly count the amount of liquid intake to make sure she hits her daily quota, at the same time hoping she doesn’t take in too much and vomit.

We also worry everyday about any change in her symptoms, i.e. increased fussiness, increased bleeding from her daily Lovenox injection site, increased oxygen sats, decreased heart rate, increased sleeping, decreased sleeping, labored breathing, vomiting, etc… In a child without a congenital heart defect, many of these things would not be a concern or out of the ordinary. In Zoe’s case, however, with her HLHS, we need to be overly sensitive to all changes in symptoms, particularly as we are in the inter-stage period between the Norwood and Glenn.

Everyday is stressful and carries the thought that Zoe’s heart condition could take her at any time, which feeds the stress. We're not complaining about this as the joy Zoe adds to our family makes it more than worth it. She is strong and inspires us everyday.

Although we are still adjusting, we love our new normal.

Thursday, July 29, 2010

Zoe loves Mia...

Zoe and the rest of the Lihn Clan are wearing their Pink for Mia today.

After Zoe's diagnosis (in utero), I was blessed to find some wonderful moms who were dealing with my exact situation - a prenatal diagnosis which rocked our worlds. Jennifer Marrone, Mia's mom, is one of those moms whom I've come to know very well. We shared our fears during our pregnancies and delivered our babies within a week of each other at CHOP. We fought through surgeries and complications. Once we both returned home with our CHD fighters, we communicated about our daily lives and how they've changed. We continued to speak of our fears. The fear of losing a child. Although we knew the risks, we never thought (or perhaps were in denial) that one of our CHD Warriors would lose their battle. Sadly (a gross understatement), Mia Grace Marrone lost her battle with HLHS just a few days shy of her 3 month birthday.

We love you Jen, Ray, Madden and Cole and will never forget Miss Mia. She's touched so many in such a short time. You'll always be a part of our family.

With our deepest sympathies,
Stacey, Caleb, Emerson and Zoe

Wednesday, July 28, 2010

Pink for Mia


Please wear pink tomorrow in memory of Mia Grace Marrone - it is her 3 month birthday.

She fought a courageous battle against HLHS in her short 87 days of life.

You can easily participate in making this memory for her parents.

Let's show her parents, Jennifer & Ray Marrone, how many lives a 3 month old can touch!

Take a picture of yourself wearing pink (shirt, outfit, headband, tie, etc) - include your kids, dog, cat, entire family! You can choose to hold her name written on a piece of paper or you can get creative and spell it out however you like... take a picture of that, too! Please just participate!

Please email all photos to

Please invite ALL of your friends, regardless of whether or not you know this family personally - let's make this HUGE! Post it as your facebook status, tweet about it, blog about - just get the word out!

Thanks for remembering Mia Grace. We love her and her family who need a lot of prayers and support.

The Lihns

Monday, July 26, 2010

Heavy Hearts

Tonight I have few words as our hearts are extremely heavy and sad. Our dear friends lost their baby girl tonight, Mia Grace. Mia was born at CHOP a few days before Zoe bearing the same diagnosis - HLHS. Please pray for Mia's parents, Jen and Ray, her surviving twin, Madden and brother, Cole. You will forever be in our hearts sweet Mia.

Friday, July 23, 2010

The Big Ten!

At this rate, Zoe will weigh more than her older sister in no time! Zoe weighed in today at 10 pounds! She continues to do well eating and gaining weight. At the beginning of our journey, even before Zoe was born, I was certain weight gain would be our biggest hurdle. Emerson ate terribly (and still does) but Zoe turned out to be just the opposite.

So here she is, our 10-pound CHD warrior. She doesn't look so tough in this photo, but boy is she CUTE!

Life around the Lihn household remains stressful, but we're enjoying every moment we spend with our girls. They're such a joy!

Emerson seems to realize that Zoe is here to stay. I catch her checking to see where Zoe is during the day. Or perhaps she's just trying to hijack her things. I've caught her more than once...

Emmy, who apparently doesn't get out enough, has become really good friends with Lambsie (x3), Frog and Monkey. She loves to read them stories, push them around in her grocery cart and take naps with them.

Please continue praying for our 10-pounder. She seems to be struggling with reflux the last week-plus. To be 100% certain her issues are gastrointestinal and not cardiac, we'll be taking her to the cardiologist on Monday for a check up and ECHO. While you're praying or meditating, just picture this sweet little face and send a special message for Zoe's continued healing and happiness.

Much love,
The Lihns

Saturday, July 17, 2010

Our Trip to Holland

Okay, so we didn't actually travel to Holland (yet), however, according to this well written analogy, which we did not write, raising a child with a disability is analogous to an unexpected trip to Holland. We are enjoying every minute of our trip with Zoe.

I am often asked to describe the experience of raising a child with a disability - to try to help people who have not shared that unique experience to understand it, to imagine how it would feel. It's like this......

When you're going to have a baby, it's like planning a fabulous vacation trip - to Italy. You buy a bunch of guide books and make your wonderful plans. The Coliseum. The Michelangelo David. The gondolas in Venice. You may learn some handy phrases in Italian. It's all very exciting.

After months of eager anticipation, the day finally arrives. You pack your bags and off you go. Several hours later, the plane lands. The stewardess comes in and says, "Welcome to Holland."

"Holland?!?" you say. "What do you mean Holland?? I signed up for Italy! I'm supposed to be in Italy. All my life I've dreamed of going to Italy."

But there's been a change in the flight plan. They've landed in Holland and there you must stay.

The important thing is that they haven't taken you to a horrible, disgusting, filthy place, full of pestilence, famine and disease. It's just a different place.

So you must go out and buy new guide books. And you must learn a whole new language. And you will meet a whole new group of people you would never have met.

It's just a different place. It's slower-paced than Italy, less flashy than Italy. But after you've been there for a while and you catch your breath, you look around.... and you begin to notice that Holland has windmills....and Holland has tulips. Holland even has Rembrandts.

But everyone you know is busy coming and going from Italy... and they're all bragging about what a wonderful time they had there. And for the rest of your life, you will say "Yes, that's where I was supposed to go. That's what I had planned."

And the pain of that will never, ever, ever, ever go away... because the loss of that dream is a very very significant loss.

But... if you spend your life mourning the fact that you didn't get to Italy, you may never be free to enjoy the very special, the very lovely things ... about Holland.

By: Emily Perl Kingsley.

(Zoe with her new customized step-stool, courtesy of the Nolasco's. Thanks Tom and Stacy.)

Wednesday, July 14, 2010

A Hearty Performance

Today, Zoe had a follow up cardiology appointment. Our nerves were on edge for this visit. As you recall, nearly two weeks ago, the echocardiogram revealed decreased heart function. As a result, after Zoe's Arizona and Philadelphia cardiologists put their heads together, Zoe was prescribed Digoxin, a medicine used to treat heart failure.

So, today's plan included a follow up echo to evaluate if the Digoxin improved the heart function. After an EKG, a dozen attempts to obtain blood pressure, and the echo, we learned from Dr. Stock that Zoe's heart function was indeed improved. In fact, he said it appears to be within normal range. Way to go Zoe! Or way to go Digoxin! Either way, today's visit yielded only good news. What a nice change.

Zoe continues to eat very well, which means she continues to gain weight. Our little chunker is already a tad over nine and a half pounds. The Glenn surgery will be scheduled for sometime in mid-September. The goal weight for that surgery is eleven pounds. Zoe is well on her way to exceeding that goal.

All in all, Zoe is kicking butt. Please continue, however, to pray and send good thoughts for an uneventful couple months, as we approach the Glenn.

Tuesday, July 6, 2010

Still Smiling

We're keeping our chins up (no, that is not a pot shot at Zoe's double chin.)

Her double chin is a good thing.

Here are some of our smiling faces...

(Mommy found Emmy "pumping" on the kitchen floor.)

And to keep the smiles going, Zoe had a spa day on Monday.

A completely relaxed Zoe emerged.

(Emmy seemed a bit jealous of Zoe's spa day.)

We hope ya'all are keeping your chins up and still smiling!

The Lihns

Sunday, July 4, 2010


Unfortunately, late yesterday afternoon, Caleb received a phone call from the skilled nursing facility where Jackie was transferred on Thursday. Jackie's nurse informed Caleb that his mom was once again non-responsive. Just earlier that morning she was up walking with assistance and conversing. Caleb requested that Jackie be taken to St. Joseph's Hospital (a request previously made and denied by Banner Thunderbird.) For those of you who do not live in Arizona, St. Josephs' Barrow Neurological Institute is top ranked in the country - sort of like the CHOP of the southwest for neurological issues.

Caleb drove to St. Joe's and spoke with ER nurses and doctors. Jackie underwent a series of tests in the ER and then was admitted to the neurotelemetry unit. The neurologists at Barrows hope to give us an answer - currently they are speculating some sort of neurodegenerative disease. We hope that in the next few days we'll know what is causing her rapidly declining health.

Zoe has taken two doses of her Digoxin and appears to be tolerating it well. She's been sleeping a lot today - so I think she's having another growth spurt.

Tonight, Caleb, Emerson and I are going out (yes, out of the house) to see fireworks. I'm so excited! Thanks to my parents for offering to hang out with Zoe while we get some Emmy time.

As always, we'll keep you posted on the Lihns and our rapid, turbulant rollercoaster ride.

Saturday, July 3, 2010

Game Plan

We have one as of this morning. Zoe will start on an alternative medication, Digoxin, in hopes that it will help her heart function. If it does not improve on the Digoxin, the next step is to admit her into the hospital (likely Phoenix Children's) and administer Captopril. The hospitalization is necessary to monitor her blood pressure while administering and balancing the Captopril. Mommy REALLY, REALLY does not want Zoe hospitalized, so let's all pray that the Digoxin does the trick. According to her cardiologists, some babies respond well to Digoxin, others do not. Some respond well to Captopril, others do not. With heart babes, its really a juggling act - as we know all too well.

I am glad we have a game plan in place, which will start this evening. We will know on July 14th (Zoe's next appointment and ECHO) if her heart function improves. We have a week and a half to pray people, so let's make it happen!

Happy 4th from the Lihns!

Friday, July 2, 2010

Emotionally Spent

With all that is going on in our world, the last thing we wanted to hear today was bad news. Well, this morning at Zoe's cardiology appointment, we received a rather unpleasant report. Zoe's ECHO revealed that her heart function has decreased, mildly. Not sure the "mild" decrease makes me feel any better. Her cardiologist cannot say for certain why the decrease in function, but that it's clearly discernible when compared to her last ECHO in Philadelphia. It could be that her heart is overloaded from the first surgery and after her second surgery, the heart will offload and function will resume as normal. It also could be that her muscle function is compromised, meaning the Glenn will not improve the function and she will need a heart transplant. There are a few other possibilities in between, but an ECHO will not reveal the cause, just the result. A heart cath may be informative, but with Zoe's clot and track record, a catheterization, at this time, is not worth the risk. So, we wait and see...

I ask for prayers, prayers and more prayers that Zoe's decreased function is not an indicator of muscle function and can be reversed with the Glenn, or better yet, by her next ECHO which is scheduled for July 14th.

A medication called Captopril can be used to assist in increasing heart function, however, Zoe's blood pressure sits on the lower side of normal (as does nearly all females in our family) and since Captopril can lower BP, he does not want to risk further complications - particularly given that it is a 3 day holiday weekend if some emergent situation were to arise. A call and email have been placed to our cardiologist in Philadelphia to relay the information and prepare a game plan.

As we sit currently, Zoe will likely have her Glenn surgery closer to the 3 month mark, the early side of the surgery range, particularly given the latest ECHO results.

Mommy's head hurts from stress and emotion - as does Daddy's.

Also, please continue to keep Caleb's mom, Jackie, in your prayers. She is now in a skilled nursing facility, but struggles daily with dementia. Today when Caleb visited her she was confused, didn't know where she was, and was generally out of it. Just over a month ago she lived independently, drove, and visited us while in Phildelphia. Today, her body has left her unable to do any of those things. Doctors can't say for sure, but because these symptoms have been present for the past month or so, it's likely that this will be a struggle for the remainder of her life. Please pray that Jackie can recover as much ability to independently function as possible. She will probably never live alone again, but we hope that her quality of life can significantly improve.

Please keep the Lihns in your thoughts and prayers.

Thank you,

Thursday, July 1, 2010

Approaching Two Months

Zoe is fast-approaching a big milestone - she'll be 2 months old on July 11, 2010. To many of you, 2 months may not seem like a "big milestone", but for a heart baby, it is huge. We are halfway to her second open heart surgery, the Glenn.

The Glenn:

This second operation will replace Zoe's BT shunt with another connection (the Glenn shunt) to the pulmonary artery. In this operation, the Blalock-Taussig shunt is removed, and the superior vena cava (the large vein that brings oxygen-poor blood from the head and arms back to the heart) is connected to the right pulmonary artery. Blood from the head and arms passively flows into the pulmonary artery and proceeds to the lungs to receive oxygen. However, oxygen-poor (blue) blood returning to the heart from the lower body through the inferior vena cava will still mix with oxygen-rich (red) blood in the left heart and travel to the body, so Zoe will likely remain mildly cyanotic. The Glenn operation also helps to create some of the connections necessary for Zoe's final operation, the Fontan procedure (which occurs anywhere between age 2 and 3.)

I know what you're thinking. Oh my God, how is this possible? Each time I read about these three open-heart surgeries, I ask myself the same question. It is amazing how far medicine has come. For this reason, we've stored Zoe's cord blood and cord tissue, hoping that medicine will rapidly advance. Who knows, perhaps one day researchers will figure out a way to rebuild an artificial heart with stem cells and Zoe can have her own, rebuilt heart. A heart mom certainly can hope!

So, our precious little one goes for her ECHO tomorrow morning with Dr. Stock. I am very nervous about the ECHO. In Philadelphia, each time we underwent a test, or another procedure, some complication arose or became apparent. I would love to hear some good news tomorrow. Zoe's "numbers" are good, she continues to eat well and is gaining weight but the ultimate looking glass is the ECHO.

Emerson left this afternoon and is heading up north with my parents. They'll spend a few days at their cabin in Strawberry. I'm thankful for this, as Ms. Em has been locked in the house for 3 days. She's been such a trooper adjusting to our new bubble life.

Overall, the girls are doing well and are growing more and more used to each other. They're quickly become sisters - check Zoe out as she kicks her big sister in the butt as they chillax during an episode of Yo Gabba Gabba.

I have a very strong feeling that Ms. Zoe is shaping up to be much like her mommy - a stubborn, strong-willed fighter. This is not a bad thing people, particularly for a heart babe. At times she gives me a look, a mischevious look I know all too well. I guess the karma train is rearing up to smack me for the stress I put my parents through.

Update on Jackie:

Caleb's mom was discharged from the hospital today and is now back in a skilled nursing facility (a different one than before) where she will undergo therapy. She still has bouts of confusion and we're afraid that her dementia is here to stay. Some days are better than others. We hope that over the next several weeks, the therapy will build self-confidence and physical strength. Once we know her maximum abilities, we'll assess her next move (literally.)

And, the moment you've all been waiting for...some pics of our little ladies.

Zoe's new bath sling - she's not a huge fan of the bathing just yet.

Emerson's first swim in the pool, this summer, with Dad.

And, I saved the best for last.

Zoe's big smile (and big cheeks!) And, yes heart moms, that is a CHOP gown which I'm sure we've paid for a bizillion fold by now!

A Glimpse into Our World

I wanted to share the details for tonight's airing of Boston Med on ABC. They will be following the story of a couple whose baby is prenatally diagnosed with HLHS and delivered at Boston Children's Hospital - a top ranked children's hospital. We considered Boston Children's for Zoe, but after meeting with Dr. Spray at CHOP, felt Spray was our man.

Please consider watching tonight's episode and get a glimpse into our world and the lives of our large CHD family.