Home of the Four Trans

“What did you say, Gong Gong?”

“That was hilarious!”

“I want to do it again!”

And its lack thereof.

We were at Babies R’ Us today per Nathan’s request. He was looking for a present for himself. But, along the way, he found something for Justin, too–a multi sport balled musical mobile. Justin’s a little too old for that, so we had to put it back. After 30 minutes of searching and debating between the Chocolate Percy train DVD or the Sounds Trains Make DVD, we ended up with nothing and headed over to Target instead. But, I digress.

Justin’s baby book. While I was there, I also perused through the aisles in search of an animal themed blanket/comforter. None really fit my taste. Or, if it did, I had to buy the whole set, bumper, sheets, everything for 80 bucks. No thanks! I came across many other cute animal themed baby things such as lamps, baskets, picture frames, toys, calendars…baby books.

It suddenly hit me that Justin is one month shy of 1 year old and I have not updated his book since, uh, I don’t know when! A little freaked, I brought this to the attention of Loi.

“You blog for him,” he responded. “That’s just a more tech-y version.”

Hmmm….I thought about it. Nice reasoning, but not really. I haven’t recorded his milestones and the dates he’d achieved them–sitting, rolling, ahhing, reaching, smiling, responding to his name, recognizing familiar voices. Maybe there are more, but I haven’t really thought about it. It’s not that I don’t care–I call to tell the world when he’s done something great such as voluntarily moving his right hand to feel pinto beans for the first time. I just haven’t thought about recording it…until now.

I did it for Nathan, why not Justin? I’ve been too busy recording doctor’s appointments, surgery dates, medical notes, my notes on his medical condition, calls to to the Regional Center of Orange County and California Children’s Services, insurance bills, medical bills, etc., that I haven’t really carved a piece of time or energy to create a nice keepsake for little Justin. As I type this, I wonder if I had made a mistake in not recording this info because his neurologist may be interested in knowing his progress at our next checkup 4 months from now.

Everything I do from day to day with my little Justin is for mere practicality, thinking of how I can better prepare for his physical health today, tomorrow, the future. The baby book is just fluff that will end up in storage…Or, so was that mind-set of the past of mine. Do I really want my child (or myself) to look back at his childhood as all work and no play? Do I want medical records and notes to define the life that I had/have with him?

We share many beautiful moments every day. Today: I loved the way he grabbed my hair and pulled my head to his mouth to eat my face after being handed back to Mama by Physical Therapist. He cracked me up this morning when he demonstrated how he can recognize familiar voices and faces, now. Just when he heard from his bedroom Nathan’s tutor greeting Nathan with, “Hiiiiii, Nathan,” at the front door, he “ahhhhhhh” really loud. When I went in to pick him up, he kicked his legs wildly, batting his arms up and down, with an intent smile on his face. When he saw the tutor, he kicked and bounced in my arm while batting his (good) left arm, and scrunched his little face into a wrinkled, toothless grin. Playing the clapping game is his favorite game–the knows that at the end of the song I would bring his arms up and pat his hands on his head. He excitedly brings his left arm and hand up to his head right before I finish the song.

He has some amazing traits other than his half functioning heart: In physical therapy, he cannot seem to concentrate when there are other patients around him–he loves observing and socializing. He smiles and ahhs at other people when they don’t pay attention to him…but, they eventually do because he is so freakin’ cute. He smiles all the time, at almost everybody. He lights up the room. He loves to grab things, anything, especially Mama’s spoon and food. He rotates his wrist from side to side, back and forth, with an object in his hand to observe it. He knows how to hide toys from his big brother: Nathan coming? Must bring toy to side, underneath high chair tray. Otherwise, risk losing toy.

What else will he or I be missing/forgetting in the future of this beautiful little boy’s life because I forgot to capture it–on note, photo, or film? No use looking back at this point. It’s only been a year, and there will be many more (I hope) to come. The beginning of Justin’s baby book is the beginning of another chapter in our lives, one filled with more joy than angst…

I came across this press release from June 2007.

“The same genetic defect that causes a rare respiratory disease may also lead to some types of congenital heart disease, according to a study from the University of North Carolina at Chapel Hill School of Medicine.

The link between the two diseases starts with cilia, the tiny, hair-like extensions that help the lungs clear of mucus and remove contaminants such as dust. The researchers first noticed the connection in children with a respiratory disease that affects cilia, primary ciliary dyskinesia (PCD). A genetic mutation that impairs cilia movement causes the disease. A few children treated for PCD at UNC-Chapel Hill also had heterotaxy – an abnormal position of the heart and lungs associated with congenital heart disease.

The research team wondered if PCD and heterotaxy were related because other types of cilia, called nodal cilia, are known to play a role in directing and organizing organs in developing embryo. Results of the new study indicate they are connected.

The prevalence of heterotaxy and congenital heart defects was 200-fold higher in people with PCD than in the general population (one in 50 versus one in 10,000), the study found. The research appears in the June 5, 2007, issue of the journal Circulation.

“This should spur physicians treating patients with congenital heart disease to be more vigilant about testing for and treating respiratory defects,” said Dr. Michael R. Knowles, professor of pulmonary and critical care medicine and study senior author. “It is critical for families and physicians to recognize when congenital heart disease develops.”

Children with both heterotaxy and congenital heart disease typically undergo surgery to repair heart defects, and if they have respiratory complications after surgery, it’s often assumed that the cause is their heart problems, Knowles said. But the study indicates these children’s respiratory problems may also have an underlying genetic cause.”

Now, here’s a possible clue as to how heterotaxy and congenital heart disease occurs. What’s nice about this piece of information is it also allows me the knowledge to ask my cardiologist or pulmonary specialist or any doctor that can and will listen for testing for Justin. There are many types of genetic testing that may be done. But, if we not know what to ask for, we won’t get it.I would also like to note that I was never offered this information in the past year while dealing with Justin and his heart issues, while my friend over at Chloe’s blog has been offered some about it. So, it may be that this is not a widely recognized explanation to heterotaxy and congenital heart disease. Nonetheless, it is worth finding out if my child has respiratory disease that I should be aware of. Then, when a cold arises, I will be better equipped as to how to approach it.In more recent news, National Heart, Lung, and Blood Institute is sponsoring a study that will “examine genetic material obtained from blood and tissue samples of patients with CHD and heterotaxy to gain a better understanding of these disorders and of a lung disease called PCD. CHD is prevalent in patients with heterotaxy. It is believed that certain forms of CHD or heterotaxy may have the same genetic origin as PCD.” Read here to learn more.On the way to answers? 

My younger sister made this comment after noting how happy Justin was even after throwing up. Instead of freaking out about how gross it was to have all this gunk come out of his mouth and smother onto his (and Mommy’s) clothes, he smiled and scrunched his face in jubilation.

“He was probably thinking how much better he felt now that he’s thrown up whatever made him feel icky.”

There’s a thing or two to be learned here: 1) My sister is a genius 2) My Justin is a model of how life should be perceived–with optimism. He’s been through more physical trauma than I could ever imagine (he has the scars to remind us of it), been poked and prodded at by people who approach him with such friendliness (who can he trust?), has rotten egg smelling medicine (Captopril) injected into his mouth 3 times a day, along with other not-so-pleasant tasting medicines….Yet, he always has the endurance and spirit to smile.

There’s this picture I have of him a few days post-Glenn: his scar is deeply bruised, purple, he had just been extubated, there’s a (unexplainable) bruised eye, suffered a stroke. He turns to Loi’s I-phone, as if there were not a care in the world, and smiled, this lopsided smile with one eye squinting (because half of his face was affected by the stroke). It’s heartbreaking, but so beautiful. His smile is always a reminder to appreciate the what is, the light of every moment of every day.

Justin’s first birthday bash plans are underway. The invitation promises of a party “fit for a king.” A lion and crown adorn the tangerine and green card. I’m excited. I love parties, love my Justin, still can’t believe he’s turning one. There’s still 3 more weeks till the big day that I am almost afraid of believing it. Anything can–wait, I don’t want to say it.He has this cold that went away and came back. I’m assuming that he got it from Nathan. But, I’m still concerned. Called the cardiologist yesterday because his eyes had been getting progressively puffier for 3 days. But, then, it went away. I guess Justin didn’t want to take that extra dose of Lasix the doc ordered for him.

 Today, I read through a blog about a mother trying to find answers to her 5-year old daughter’s heterotaxy condition. This child was not diagnosed until she was 5 years old. After pushing for 3 years, this mother finally got a diagnosis for her daughter’s, Chloe, condition. Fortunately for Chloe, she has a good prognosis and does not show any signs of congenital heart disease that is often associated with heterotaxy. I read through this blog and left inspired.

Justin has heterotaxy. This was diagnosed by the wonderful Dr. Michael Rebolledo while inutero. The early diagnosis saved us a lot of heartache and emotional turmoil because we were allowed some time to grieve this loss of a physically perfect child before he came into this world. When he came into this world, we were prepared, not shocked. Our minds were on straight, enabling us to make the best decisions for his health and well being. We knew that with this complex heterotaxy condition came a set of congential heart defects. We knew the set of congenital heart defects he may be born with: double outlet right ventricle, unbalanced atrioventricular canal, pulmonary valve atresia, situs inversus with levocardia. We knew that he would need open heart surgery in the first week of life to have a modified Blalock-Taussig shunt put in. We knew that it would be likely that he’d need a minimum of 3 heart surgeries to maintain his life, including the Glenn and Fontan; heart transplant will eventually be part of the plan when his own heart could no longer work for him. We knew that his complex heterotaxy would mean his stomach was on the left side as was his heart. He had the potential of having malrotated intestines (which was confirmed through a GI exam at CHLA after his first heart surgery, and has been “corrected” with the LADDS procedure). He would be asplenic (no spleen).

For the past year and a half, we have learned so much about Justin’s condition. In addition to those stated above, Justin’s general surgeon, Dr. James Stein, also found that he had a preduodenal portal vein and an annular pancreas. His liver is larger than normal. We also learned that Justin’s asplenic condition would have him with double AV (atrialventricular) nodes, while a normal person would have one. Having 2 AV nodes would lessen his chances of getting an artificial pacemaker put in because when one node doesn’t work properly, the other would kick in–not in Justin’s case. He developed arrhythmia sometime between his first heart surgery and second. He ended up needing an artificial pacemaker put in during his Glenn procedure.

Loi and I have accumulated more medical jargon than we had bargained for. We’ve developed a greater compassion for children and the disabled. We’ve learned of the various heart foundations and where to donate to. We’ve experienced the hospital life. We’ve met other heart families and joined in their journeys. We’re learning about the “system” and how to get it working for us in getting therapy and aid for Justin.

We’re moving along, getting through day to day, changing, trying to make a difference, to spread awareness…Yet, I realize, we’re still where we started from…clueless. Chloe’s blog reminded me that there is so little known about the heterotaxy condition. I’ve realized this, but have somehow placed it secondary to Justin’s heart defects, as those raised an immediate threat to his life; without proper function of his heart, he’d cease to exist.

But, this heterotaxy condition is no less important. It is the very basis of his heart condition. Somewhere during the embryonic development, something went horribly awry (but what?) that caused the rest of his development to mess up. In Chloe’s case, there may be a genetic link. In Justin’s case, we’re still not quite sure. We’ve been told that it’s not genetic. We’ve had genetic testing. But, I’m considering more.

Whenever I mention heterotaxy to the doctors, I’m made to feel like it’s not a big deal, that I should just concentrate on his heart. But, with heterotaxy, there are a group of health problems that I should be aware of depending on whether he’s situs inversus or situs ambiguous. Knowing which category he falls  in would better direct me to find answers.  According to my readings, Justin’s heart anomalies correspond with the situs ambiguous group.

Because Justin’s condition is so complex, I will be contacting a specialist in Immunology, Infectious Diseases, and possibly Pulmonary to obtain a better understanding of Justin’s condition and how to keep him healthy. While I agree that Justin’s heart is priority, I also believe that I need to know as much as I can about how the rest of his body works. How the rest of his body works affects how his heart works and vice versa. I need to know if his colds are normal and with a child with his set of anomalies, what is normal, for him?

His birthday party is approaching. I was looking forward to this lull in the year prior to his Fontan. Yet, I know, in this life with Justin, there is no such thing as a lull. I’m forging forward to find some answers for my baby boy. 

Nathan is sick…again. For the past 2 days, he had some wheezing and breathing issues which we alleviated with his inhalers. Watching his throat and stomach retract as he struggled to breathe freaked me out a bit. But, he is back to his usual self today, playing with CARS and talking too much. Even as he struggled to breathe and was almost breathless yesterday, he still talked. He’s constantly moving also. Where does he get all his energy from?

I think I changed my clothes at least 5 times yesterday, between Justin’s messy, poopy diaper and spit up and Nathan’s throw up. It’s gross, I know, but this is the life of a mother. Oh, and I did not want to carry any of Nathan’s germs on me when I went in to carry Justin, so I changed between holdings, too. Now, we have a whole basket full of laundry ontop of the already existing two just from yesterday.

This was an exhausting way to start the week considering our jam packed weekend with not a moment’s rest. Saturday was my high school friend’s wedding. I attended the ceremony myself as Loi was left with diaper duty with the two boys.  We met at Loi’s parent’s house to drop off Nathan. Then, lugged a sleeping Justin to the hotel where the reception was held at to wait for his ride and sitter for the night (Thanks Auntie Phuong! Since we know you can handle it, we know who to call next time…hehe) to pick him up. We finally had some much needed recreational conversation time that did not include Mommy-whining in the background or discussions about poopy diapers and feeding. We couldn’t escape the Justin and his condition talk, though. How it was brought up, I have no idea. Loi was immersed in that convo more than I. I was busy catching up with my other high school friend’s big news–she’s pregnant!

Sunday was Loi’s grandma’s dedication party. This is one of those rare occasions where Justin is allowed out of “his cage,” as his paternal grandfather puts it, to interact with the rest of the family. Loi’s family is huge. With lots of kids. Hello, germs! Needless to say, I was the primary caretaker, holding him with a bottle of Lysol Foaming Sanitizer right beside me. Nathan was on Germy Hands Watch, also. Whenever anyone approached Justin, including his paternal Grandma, he ran right up between Justin and the person to tell them, “You have to wash your hands first!” This rare Justin sighting prompted many to approach him to admire his chubby cheeks and smiley face.

My boys are party animals (should I start worrying now about their teenage years?). While at the sitter for 3 hours, Justin did not sleep one wink and forced his Auntie Phuong to play with him and look at him and hold him. He was still wide eyed when we came to pick him up at 10:30 at night. As for Nathan–he just doesn’t sleep. Everyday is a party for him and his CARS that come along everywhere he goes.

Hope you all had a nice weekend!

Justin’s medical history–dates for surgeries, doctor’s appointments, important events, changes in medications, notes–is all completed and up to date by my recollection. The doctor’s notes and discharge papers are somewhat of a mess in a separate binder in plastic coverings. This, I will work on next. Then, I have to order his records from Children’s Hospital LA and OC.

Though this is such a daunting task, keeping up with all these dates and events and paperwork,I know I have to do it. I realize this when every time I see a different specialist, even while in the hospital, they ask me for Justin’s (very long) medical history. I can proudly recite it from memory. Everything that ever happened from the day I received news of his diagnosis inutero just rushes out like water from my mouth. “Slow down, there,” a GI Doc once said to me. Everything is important. Everything that has ever happened defines the Justin today. And, I think it’s important for them to know the little pieces to see the big picture.

But, there will be a time when my memory of such horrific, sad, unpleasant events will fade. Then, there may be a time when Justin must know his own history to repeat to his healthcare providers while I may not be there to do it for him.

It is hard for me to believe that he’s only 2 months shy of 1 year old. Judging by the thickness of his 3 ring binder and his extensive medical history, you’d think he’s an old man. We’ve been through so much with him in the past 10 months, I feel like it’s been years….not months.

Sure, I don’t know what the future holds. But, it doesn’t hurt to prepare for it, right?

“Just a little better? Or, better?,” Loi asked when I called to tell him the good news about Justin’s leaky valve.

Even if it’s a little better, it’s better. That’s all I need to know.

“Why is it better?,” asked my younger sister when I told her.

The little voice in my head (yes, I have one. Scary, huh?) kept pressuring me to ask the cardiologist the same question: “Why do you think the leakage has improved? How would you label the severity of the leak?” I looked at him, attempting to concentrate on him saying, “This is very good news. I’m very glad,” while fighting the voice in my head.

I’m sure we all want to know how and why this leak that I was told could never be fixed (but, could be improved after the Glenn) and could only be maintained with medication got “better.”

But, is why the correct question to ask? At this point in my journey, and with all our past experiences with Justin, I’ve come to realize that there is no true explanation for the way things happen the way the do for Justin. Asking why will not help me learn any more information that would allow me to fix his leak. And, if the doctor really knew why, he’d share some of it with me.

The leaky valve has gotten better. That’s all I need to know. That, and my belief in the Lord and his plan. I’ve prayed for that leak, I’ve asked, pleaded even, with the Lord for my child. I truly believe that it is a miraculous, divine intervention that has allowed this to happen. I have to believe that even if the material world may not always work in my favor, according to the way I’d like for it to happen, I must let it be, listen to my inner voice, the Lord, to guide me toward my given path. I must also allow Justin’s path to unfold as it should. Though the reward may not be immediate or even of this world, there is a greater picture, a grander scheme that shall be achieved.

For this moment, the Lord has allowed things in this material world to work in our favor. For that, I am thankful.

We just came back from the pharmacy. Which is only 6 minutes away walking time, may I add. I love our new place!

The pharmacist behind the counter commented on how little Justin is, not even a year old, and constantly on Amoxicillin.

“Why?,” she asked.

“He’s aspleenic,” I nonchalantly replied.

“Aww, poor thing.”

“Yeah, and he has some heart issues, too.”

“So, how is he doing?”

“He’s fine now, but his condition is not ‘fixable’.”

“How are you and your husband doing?”

“We’re fine, now. I think it’s most stressful when we’re in the hospital. But, now, everything is pretty normal.”

Now. Normal.

On our walk back, I contemplated about our meaning of normal, surprised at myself for realizing that we have gone back to a normal that does not involve constant, obsessive compulsive stress every single day.

Normal is:

1) Driving Justin to PT 2 times a week and setting aside 2 hours each day to drive to the sessions and to participate.

2) Giving medicines to a crying Justin every day, 3 times a day.

3) Taking him to see specialists: cardiologist, hematologist, neurologist, electrophysiologist. And, awaiting anxiously for “news”. Seems like every other week, but more like once every month.

4) Calling in for a pacemaker check once a month and listening to the click, click, click over the dial tone to make sure his pacemaker is working.

5) Monitoring how blue his lips get when he cries or while he is asleep or awake.

6) Wondering why he sleeps so much when he does…is it his heart? Or, did he just have a really productive day.

7) Keeping up with insurance and medical bills.

But, normal is also:

1) Seeing him smile, that wide mouthed smile

2) Listening to him ah, ah, ahhh and eh, eh, ehhh.

3) Kissing his chubby, chubby, cheeks.

4) Being amazed every day by his progress: jumping more and more in his jumperoo, lifting up his right hand, making new sounds, reaching for toys, fighting with his brother for a toy.

5) Waking up to see him smiling and ah, ah, ahhing.

6) Going to sleep to see his peaceful face.

7) Hearing his chuckling cry when he sees a bottle of milk.

Picking him up and holding his warm body against mine.

9) Our daily walks outside with brother.

10) Having him in our lives, everyday for the past 10 months.

Then, I catch myself, thinking don’t get too comfortable–things can change on a dime.

All my heart mama friends, you were right. There is a normal that we do return to after giving birth to a child with heart defects. There is a normal after enduring those sleepless, nightmarish nights at the hospital. The normal, of course, is never quite ever really normal. But, hey, what’s normal anyway? Boring, I think.

I find myself thinking about all our family and friends, too. Thanks for all your support. Without it, we would not have gotten through all the emotional turmoil.

This position I’ve taken as “Mama” means more than any other title I’ve ever possessed–eldest daughter, first American born grand-daughter, oldest sister, niece, student, friend, wife, wedding consultant–because this title creates a true sense of responsibility to another person. This is not to say my other identities did not also encompass a sense of responsibility–it did, immensely. With the other identities, though, I really did have the option of passing on some of the burden, abandoning it even; I just chose not to. In clarity, there could always be someone else to take my place even when I wanted to believe that no such thing could be true.

As “Mama” my children’s life depends on me. Sure, there are grandparents, aunts, and uncles; they can share the burden at times with feeding, entertaining, strengthening my children’s sense of self and independence. But, at the end of the day, it is “Mama” who knows them and molds them and confirms the very value of their existence.

“Mama” chose to give you life because I love you.

“Mama” chose to harbor your life because you are a gift and so gifted, too.

“Mama” does any thing to ensure the best for you, your life, and afterlife.

This is the first identity I’ve possessed that I do not expect for anything in return. My boys, they are the gifts, the reward. To be their Mama, to love them unconditionally is in all clarity my selfish want to give love.

“You need to have a girl, a daugther, ” I’ve been told, to be my caretaker when I reach old age.”

Your sons will leave you, “they said. “You’ll suffer.”

Maybe they’re right. Maybe my sons will forget their old mother on Mother’s Day, with not even a phone call to tell her how much they appreciated her. Maybe they’ll abandon me for their in-laws. Maybe my sons will be too busy to give me a ride to the doctor’s.

Maybe I will be left alone in an empty nest save for a few visits a year. Maybe I will regret not taking heed to their advice.

But, this is life. If there is one thing my boys have taught me, it’s this: in life, there is no certainty. I’d like to think I’ve raised my boys right to think of their Mother from time to time and in her moments of weakness.

Here’s the beautiful thing: I’d take it a compliment that they’d one day spread their wings and conquer the world. I’ve given them life, helped build them a foundation, and it’s up to them to live the life worthy called of living and embracing. My boys, they’ll move mountains!Here’s how our Mother’s Day went down: