Best General Conference prep ever

Early on the first Saturday in April, we walked up to the elementary school to participate in the First Annual Color Me Woodway 5K Color Run. I planned to jog ahead with Ellen and Jack Jack, and Jack planned to walk with Rose while pushing Elijah in the stroller.

When it was our turn to start the race, we ran around the corner of the school and started down the street. At this point, most people started walking. Ellen and I slowed down, but Jack Jack just kept going. So I had to encourage Ellen to keep up the pace, keep an eye on Jack Jack up ahead, and try not to kick myself for not running any substantial distance in the last 15 years.

We ran through several color stations and got to the half-way point, had a drink of water and turned around to run the same course back to the school for the second half of the race. A few minutes after we had turned around, I saw Rose running along all by herself. I stopped for a second to check in with her. She was supremely confused to see me running "in the wrong direction" and said Daddy was somewhere behind her. I told her to keep going, and I would see her soon. I figured that with the 600 people running, mostly kids and their parents, she wouldn't get lost, and I could always find Jack and send him to get her or run back to her once I finished with Ellen and Jack Jack.

Jack was waaaay behind Rose, and it turns out that she started with him, but as soon as she saw everyone else running, she took off running, too. 

Elijah was a particularly attractive target for all the teenage female volunteers throwing colored chalk at the color stations. He was cold because I didn't anticipate how cool it would actually be, and he rode the entire time, so he wasn't really feeling it by the end.

I finished the 5k with Ellen and Jack Jack (who ran most of the way. That's three miles!) Then I went back for Rose, who also was still running along, looking very earnest and intent, and maybe a little worried. But she also ran most of the way.

For our first family 5k, it was amazing. All of us had so much fun (well, maybe except for grumpy there in the middle), and we were set to go home and watch eight hours of General Conference over the course of the next two days. I cannot wait for our next race!

Thankful

My sister-in-law started a blog documenting her experience with her recent surgery for a cochlear implant, including input from her husband (my brother) and her parents. Amazing. If you want to be inspired, be grateful for your hearing, be grateful for the amazing physical bodies we are blessed with, and maybe even cry a little, click on over and check it out. www.hegavememyears.blogspot.com

The future of Hypoplastic Left Heart Syndrome

By RON WINSLOW

New Haven, Conn.

Four-year-old Angela Irizarry was born with a single pumping chamber in her heart, a potentially lethal defect. To fix the problem, Angela is growing a new blood vessel in her body in an experimental treatment that could advance the burgeoning field of regenerative medicine.

Doctors at Yale University here implanted in Angela's chest in August a bioabsorbable tube that is designed to dissolve over time. The tube was seeded with cells, including stem cells, that had been harvested from Angela's bone marrow. Since then, the doctors say, the tube has disappeared, leaving in its place a conduit produced by Angela's cells that functions like a normal blood vessel.

"We're making a blood vessel where there wasn't one," says Christopher Breuer, the Yale pediatric surgeon who led the 12-hour procedure to implant the device. "We're inducing regeneration."

Angela, who had little stamina before the operation, now has the energy of a regular kid. She is on several medications, but Dr. Breuer and her parents think she'll be able to start school in the fall.

Scientists have long been captivated by the ability of animals such as salamanders and starfish to regrow body parts lost to injury. It was long assumed that developmental forces that create a human being in the womb are lost at birth. But recent advances in stem-cell research and tissue engineering suggest that regenerative forces can be reawakened with strategically implanted stem cells and other tissue.

This notion is fueling research at many academic laboratories and dozens of start-up companies where scientists are hoping to identify effective ways to treat maladies including heart muscle damaged from heart attacks, paralysis due to spinal cord injuries and poor-functioning kidneys and bladders.

Angela's condition, known as hypoplastic left heart syndrome, affects some 3,000 newborns in the U.S. each year. With just one pumping chamber, or ventricle, instead of the usual two, the babies can't deliver sufficient levels of oxygen to their organs and extremities, compromising their development and causing them to turn blue and suffer from a lack of energy. Without a surgical repair, says Dr. Breuer, 70% of them die before their first birthday.

Pediatric surgeons typically treat the condition with a series of operations called the Fontan procedure, designed to enable the heart to function without the missing ventricle. The last operation involves implanting a synthetic blood vessel made of Gore-Tex to reroute blood from the lower extremities directly to the lungs instead of through the heart. The device works, but it is prone to clotting, infection and in some cases, the need for additional surgery later in life as the child grows. The idea behind Dr. Breuer's project is that a natural conduit with the biology of a normal blood vessel would grow with the child and avoid or significantly reduce complications associated with a synthetic tube.

Angela's case "is a real milestone and broadly important for the field of tissue engineering," says Robert Langer, a researcher at Massachusetts Institute of Technology and a regenerative-medicine pioneer who isn't involved in the Yale initiative. "It gives you hope that when you combine cells with a scaffold and [put] them in the body, they will do the right thing."

Angela's heart defect was diagnosed in utero, when her mother Claudia was five months pregnant. She had her first operation when she was 5 days old, and another at 8 months. But her heart defect was taking a toll. She was shy, small for her age and lacked the stamina of a normal 3-year-old.

"If she ran from [the living room] to the kitchen, she got tired and she had purple lips," her mother, Claudia Irizarry, a church secretary in Bridgeport, Conn., recalls.

In four meetings with Dr. Breuer and other Yale staff, they discussed the pros and cons of the conventional synthetic tube versus the new approach. A tissue-engineered version can still narrow or become blocked, for instance, Dr. Breuer says. And more drastic complications such as cancer triggered by stem cell therapy or a bad immune-system reaction can't be ruled out. In the end, the choice to become the first patient in Dr. Breuer's study turned on three things, Ms. Irizarry says: the family's faith in God, their trust in the doctor, and the potential for a natural blood vessel that could possibly help avoid more surgeries. "Before, they were using plastic, now they're using this special graft that will grow with her," Ms. Irizarry says.

Today, Angela seems more like a regular kid, says her father, Angel Irizarry, who works as a carpenter. "It's a huge difference," he says. "It's like going from a four-cylinder to an eight-cylinder car in one operation."Before the surgery, he adds, "her eyes weren't as happy as [they are] now."

Development of the procedure has been painstaking. Dr. Breuer undertook four years of laboratory research after he joined Yale in 2003 before seeking approval from the U.S. Food and Drug Administration in 2007 to test the approach on patients. It took four more years and 3,000 pages of data before he got a greenlight. The study builds on the cases of 25 children and young adults successfully treated in Japan a decade ago with a similar approach.

Dr. Breuer, who holds several patents through Yale related to the technology, expects to implant a tissue-engineered blood vessel in a second patient soon as part of a six-patient study to test the safety of the procedure and determine whether the blood vessels actually grow in the body as a child gets bigger. The hope is that if these patients are treated without a hitch, the procedure may be available under a special FDA humanitarian device exemption, which would allow Yale to charge for it while conducting a larger study.

Write to Ron Winslow at ron.winslow@wsj.com

A version of this article appeared Mar. 20, 2012, on page D1 in some U.S. editions of The Wall Street Journal, with the headline: To Fix a Heart, Doctors Train Girl's Body to Grow New Part.

If you give...

If You Give Rose Sour Cream

by Ellen Starks

If you give Rose sour cream, she's going to want a taco to go with it.

If you give her a taco, she's going to want a lime to go with it.

If you give her a lime, she's going to want a lime squeezer.

If you giver her a lime squeezer, she's going to want a lot of limes.

You will have to go to the store to buy a lot of limes.

If you go to the store to buy limes, everyone will want to go with you.

You won't have room for everyone, so you'll all have to stay home and have a taco.

And if you give Rose a taco, chances are, she's going to want sour cream to go with it.

We've read a lot of giving pigs pancakes, mooses muffins, and mice cookies, in case you didn't recognize the rhythm. Ellen came up with that story about Rose tonight at dinner as Rose was finishing off a dish of sour cream.

That Rose is eating anything is a huge relief since she caught a nasty virus that took away her appetite for anything but water for an entire week. She ran a fever for days and carried around her blanket begging to be put down for a nap. She slept more than anything else. Jack and Ellen were also knocked out for a couple of days, ate little, and voluntarily took naps ("rested" according to Ellen), but nothing like Rose.

Elijah, fed a steady diet of liquid immunity and kept as far from feverish or snot-nosed kids as possible, has stayed completely healthy and weighed in at 13 pounds and 3 ounces at his recent visit to the cardiologist. His O2 levels have been great, his EKG and echo looked great. We love his new pediatrician, who is working on scheduling a follow up swallow study for as soon as possible. Please pray that Elijah will be able to swallow any consistency of liquid so he can have normal (unthickened) breast milk, and so when we start solid foods in a couple of months he'll also be able to start trying water or juice from a cup.

Elijah

Elijah is growing! He weighed in at 12 lbs. 11 oz. today at his appointment with his pediatrician. Unfortunately, his O2 sats continue to be lower than we want, so we took him home and hooked him up to the good air. He's on .5 liters of oxygen and the O2 monitor.

He'll have his 4 month check up and vaccinations in a week, a synagis shot soon after that, and then a trip to Dallas to see the cardiologist early in March. Elijah's operation day buddy already has his second surgery (the Glenn) scheduled for late April, so we expect Elijah won't be far behind. Except for the low O2, he's doing great!

The marathon begins

Thank you, thank you, thank you for your thoughts and prayers and messages.

Jack got to hold our beautiful Elijah David immediately after birth and brought him to my side so I could see and touch him. Then he was taken to the NICU for an echocardiogram, to start an IV, and to begin giving him prostaglandin in prep for the Norwood procedure.

After a couple of hours, a couple of phone calls, and absolute insistence on my part, Jack wheeled me down to the NICU, accompanied by Jack's parents and my dad, to see the baby. As a result of the echo, the surgeon and cardiologists decided to take Elijah as soon as possible to the heart catheterization lab due to anomalies discovered prenatally and prominently seen on the post partum echo.

Jack and our fathers gave Elijah a beautiful blessing, and we returned to our room . After two hours in the cath lab, the cardiologist and surgeon came to tell us that in addition to HLHS, it looks like our baby has TAPVR, a different heart condition that can appear in isolation. For a baby to have both is extremely rare, and Elijah has the added complication of obstructed coronary sinus.

The surgeon, Dr. Mendeloff, came to tell us that Elijah's best chance is to undergo surgery immediately to address all three issues, and he doesn't anticipate finishing before 6 or 7 in the morning. Since they won't know how Elijah is responding until the very end, we won't have any updates during surgery.

In light of the most recent news, I am grateful for several things. Most of all I am grateful for the gospel of Jesus Christ and the knowlege that no matter what happens, Elijah is now and forever our son. I am grateful that we looked for the best doctors we could find, and for spiritual confirmation that we were making the best choice for our family in completely uprooting and moving across the state. I am grateful for priesthood blessings. I am grateful for immediate and extended family who have made great sacrifices to help us. I am grateful for loyal and loving friends, old and new, who listen, encourage, counsel, pray, cry, hug, and love us.

I've thought before that sometimes our "trials" are in fact the very blessings we seek and pray for. I've also been learning that trials can be blessings we didn't even know we needed.

Thank you.

Scheduled

October 25th
9:30 a.m.
Cesarean
These next four weeks are going to fly by. I don't know if I'm ready to have a baby that soon!

High risk

It's still hard to believe that my pregnancy falls into the "high risk" category, since everything about being pregnant this time around has been and will continue to be pretty normal, except the frequent doctor visits, and the number of doctors who have a say in how and when I deliver.

Yesterday I hit the 32-week mark and took a trip to Medical City in Dallas, where I visited several doctors. This will be a recurring event every two weeks until I hit 36 weeks. Then I'll start going every week. That's still normal, except for the fact that I have to travel over 100 miles to get there.

We had an early morning because I was taking Ellen, Jack Jack, and Rose to play with cousins at my sister's house in Greenville while I was at my appointments.

So for those of you who have been waiting for more information about our baby boy, here it is.

Dr Laird (9:30)
At the end of my first appointment of the day with my pediatric cardiologist, Dr. Tracy Laird, she wrote this note to the pediatric cardio-thoracic surgeon who will be operating on our baby soon after birth:

"Hypoplastic left heart syndrome
-restrictive PFO
-possible cor triatriatum"

The good news is that she sees good blood flow from the baby's heart and umbilical cord, and she wants to make sure the blood continues to flow and doesn't start to back up where it doesn't belong.

That, and the note above, means that I will have appointments every two weeks to monitor the condition, and that I will probably be scheduled for a C-section at 39 weeks (around November 4th) if all things stay the same.

We want all things to stay the same, or get better.

Dr. Weiss (10:30)

My perinatologist or maternal-fetal medicine specialist does an ultrasound every time to measure growth, movement, amniotic fluid, and also wants to see me every two weeks. The baby has been growing normally and moving a lot, just like he's supposed to.

When I asked him if I could just assume the contractions I was having were Braxton-Hicks, he laughed and said it was better not to assume anything, and that he'd have the OB check out the situation. We don't want any surprise deliveries with this baby.

Incidentally, the perinatologist in Lubbock (who we were going to see for a "routine" ultrasound to find out we were having a boy) is the doctor who first discovered the heart problem and referred us to a pediatric cardiologist.

Dr. Seddi (11:30)

Next I went to the Specialty Obstetrics Referral Center a few floors down to see my OBs, who only see high-risk cases. My blood pressure was a record 80/60, probably due to the fact that I had eaten nothing but a granola bar that morning and was super hungry. Dallas traffic did not leave me time to stop for a McMuffin.

They weighed and measured me; I'm growing as expected. They hooked me up to a monitor for baby's heartbeat and contractions; the baby looks and sounds good; I am not in labor. They did a protein test and checked my cervix; they are more than 90% sure I am not going into labor anytime in the next 14 days. That's a relief.

Lunch (12:30)

I ate in the hospital cafeteria and read Art of War by Sun Tzu. Why the interesting choice? After seeing it on our bookshelf for the past six years (Jack had a copy from a college class), I thought it would be interesting to take a look. It was also free to download to my phone, as the actual book is now in storage or a second-hand bookstore somewhere. Also, it is completely unrelated to any topic currently occupying my mind and heart.

I do sometimes feel that I'm going to battle for my kids, especially this little one, and although the book is written literally, I like to read literal things in a figurative way. My degrees ruined me like that, so I can't shut off the wandering thought about what else this could mean.

Pre-admission/Tour (1:00)

Pre-admission: done.

Tour of the Cardiac Surgical Center and Pediatric ICU: lots of machines, several questions about logistics, breastfeeding, and baby care answered. This was probably the hardest part of my day, but I am happy with the nurse's explanation of hospital policies and the level of involvement they want and expect from parents, and the great lengths they go to to support breastfeeding.

Dr. Treen (2:00)

The director of the Pediatric ICU and a neonatologist, Dr. Treen gave me a straight forward and detailed explanation of what to expect from the time I deliver until the baby's first surgery, including what tubes, monitors, medications, and interventions are normally done on a baby with HLHS. It was all very overwhelming, and I don't remember much.

Following my appointment with Dr. Treen, I had to go to the lab for a glucose tolerance test (very late in this pregnancy), get some signatures for paperwork, and then I finally headed back to Greenville at about 4:00. Traffic wasn't bad. I picked up pizzas for dinner on the way to Heather's house, and the kids were ecstatic.

While I was having my fun day, they were playing trains, play-doh, princesses, taking a walk, riding bikes, eating snacks, and witnessing the very exciting losing of a tooth: cousin Eva's third. Ellen assured Eva that she'd be back in two weeks, when they could continue playing the game they had started. And she gave her a hug. It was very cute.

We left Heather's at 7:00 and got back to Nana and Pa's house just before 9:00.

And I have all that to look forward to again in two weeks, plus a visit with the surgeon. If you're in the Dallas area, Medical City is on Forest and 75, a couple of blocks from the temple. I'd love to see friends!

Hypoplastic left heart syndrome

I realize that writing a blog post is a very impersonal way to communicate very personal information, and I apologize for not being able to speak in person with all of you who read this. That said, I would still like to use our blog as a way to keep family and friends updated on some recent and ongoing events in our life.

A couple of weeks ago, Jack and I went to a routine ultrasound to find out if we were having a baby boy or a baby girl this coming November. We found out it's a boy! And we also found out that he has a serious heart defect and were referred to a pediatric cardiologist.

The cardiologist confirmed the diagnosis of hypoplastic left heart syndrome and discussed treatment possibilities with us, all of which require surgery almost immediately after our baby is born. A prenatal diagnosis is an advantage for us and our child, because the doctors and surgeon can have a better idea of what to expect and begin to make plans now for the necessary procedures.

After several weeks of research, discussion, consulting with family, meeting with doctors, attending the temple and lots of prayer, Jack and I have decided that the best course of action for our entire family is to move closer to our immediate family so that Ellen, Jack Jack, and Rose will have the stability they will need when life gets crazy in November.

We will be moving to live with Jack's parents, Jack and Cheryl (or Nana and Pa) Starks, in Gilmer, Texas, east of Dallas. Upon moving to Gilmer, we will meet with a pediatric cardiologist and other doctors based in Dallas and associated with a children's hospital there.

Answers to few questions you may have at this point:

I will have a relatively uneventful, but highly monitored pregnancy. The baby is completely safe while I am still carrying him, but ultrasounds and echos can give doctors the information they need to plan for the right type of surgery.

I will most likely have a C-section. Due to the particular abnormalities associated with the syndrome our baby demonstrates, he may need surgery immediately after birth.

I am okay. I am married to a healthy survivor of a different congenital heart defect who holds the priesthood and understands the miracle of modern medicine. I also know that Heavenly Father loves us and knows us. His plan is the best one of all. I have had experiences in the past several months that I feel have prepared me to understand and know that this particular "trial" can be a blessing to me and my family if I will stay close to the Lord and trust him. I have experienced an entire range of emotions that I am sure will repeat over the next year. For now, I feel peace.

Feel free to call, write, comment, ask questions, or offer suggestions. We will miss our Lubbock friends greatly and hope to stay in touch with as many of you as possible.

Alien arms: update

I started doing the hundred push ups training program at the end of January. I'm happy to report that I am such a weakling with alien arms that even though I made it through week four, I couldn't do enough push ups in an exhaustion test to move to week five, so I went all the way back to repeat weeks three and four.

Somewhere along the way, I hurt my neck and shoulders (either sleeping scrunched up on a kid bed or doing incorrect push ups) and had to take about a week off. I'm completely recovered (I know you were all concerned) and took my post-week-four exhaustion test again yesterday. I am also happy to report that I successfully completed my remedial work and am ready to progress to week five! And I am now seven weeks into the six week program!

Don't worry, I can still only do 35 girl push ups, so technically I guess I'm still doing remedial work. It's kind of nice to be at a remedial level. This way I can have really, really low expectations for myself. In fact, I fully expect to have to repeat week five, and then do week six twice as well before I'll actually be able to do the promised hundred push ups. I mean, girl push ups.

The long-term plan is to then repeat the entire program doing real push ups. Realistically speaking, this could very well take the rest of my life.

In my post life, I think I'll take up the hundred squats program. Do you think I have alien legs, too?

Alien arms

During my first semester of college, I was in the dorm computer lab working on a group project for Biology 100. It seems like all I ever did my first semester of college was study for Biology 100 tests and work on group projects for Biology 100. My group members had each written part of the final paper, and we were meeting to combine all the parts into a finished whole paper.

I happened to be sitting at the computer doing the actual typing. As I sat with my hands on the keyboard, ready to type the next transition sentence, a boy in my group said, "You have alien arms." The group project quickly digressed into a discussion of alien films, and whether or not my arms did indeed resemble alien arms. I'm pretty sure I wrote about it all in my journal.

Jack said something to me about my skinny arms once, and I told him the alien arm story. He thought it was hilarious, and has since warned me to be careful carrying my kids, pushing strollers, and opening doors and jars lest I injure or break my alien arms.

In an effort to reverse some minor wrist pain and strengthen my arms, which get very little working out other than carrying my heavy kids, pushing strollers, and opening doors and jars (which apparently has been working fine, since I won an arm wrestling contest on New Year's Eve) I recently started the hundred push ups training program.

The claim is that by following the training program, you can go from being able to do zero push ups to one hundred push ups in six weeks. I'm already on week two. I still have to do girl push ups because I'm not called alien arms for nothing. But I am definitely seeing progress. I'll let you know how it goes.