A Broken Heart

Incidence:
35,000 children are born with heart defects every year (American Heart Association)
Only 1% of those children have truncus arteriosus (many sources, including the Herma Heart Center where Turnip may be treated)
That means about 350 babies are born in the U.S. with the same condition as Turnip every year. (330 according to the Morbidity and Mortality Weekly Report from jan 6, 2006)
Treatment:
Surgery usually happens at 6-12 weeks (EPA, page 4)
15-50% of children require another surgery within the next five years (i.b.i.d.)
All heart valves are replaced after 12 years(i.b.i.d.)
Newborns with truncus arteriosus stayed in the hospital for an average of 30 days during 2003 (CDC, table 2)
The hospital charges for this stay (not including lab tests and doctor fees) ran to $200,000 (CDC, table 2)
Mortality:
In a small study published in 1996, 6% of infants died before surgery and 10% died during surgery (EPA, page 4)
In a national survey, 20% of newborns with truncus arteriosus died in the hospital in 2003 (CDC, table 1)

Tuesday, September 30, 2008

next check up

The folks from Milwaukee called today. Kevin and I will go to the Herma Center on the 27th of October for Turnip's next echocardiogram and growth ultrasound. We'll meet their cardiologist, one of the doctors who may deliver Turnip, and a social worker. We'll also get to schedule the induction date. The woman I spoke with said I get to have some say as far as dates go. I guess that's a no brainer--Dec 31, here we come!!!!!!

Monday, September 29, 2008

Badger Care

Today, I signed us all up for BadgerCare: Wisconsin's version of Medicaid. I signed up to make sure we don't run into problems once Turnip begins treatment. Our private health insurance has a lifetime cap of $2 million per person. If Turnip's surgeries are uncomplicated, I estimate he could hit that cap around his 3rd birthday. If he struggles, he could max out much earlier. Medicaid will pick up any costs above and beyond what our insurance will pay. According to the people I spoke with today, Medicaid has no lifetime spending cap.

Talk about sucking up the pride. I'm on public assistance.

Sunday, September 28, 2008

warning: religious

This post has been moved to my personal blog.

Saturday, September 27, 2008

Tell all

Kevin and I have started telling people in Wisconsin about Turnip's problems. Of course, my Aunt Mary already knew and our religious leader knew. Yesterday, Kevin told his boss and I told a woman I've gotten to know through church.

I've been dreading, not so much the telling, but the re-telling...especially at church. In my church, there is a lot of emphasis on looking out for each other. This has a few outcomes: some wonderful, some I find a bit intrusive. If a crisis occurs, the entire congregation will mobilize to help. However, in order to mobilize a congregation, the congregation needs to know about the crisis. As a result, there is a lot of chatter about the personal struggles and problems of the congregants.

I fear that as soon as we make this knowledge public, I will get bombarded with questions, comments, and condolences...from people with whom I do not feel close. People who have heard about our situation second- or third-hand and feel compelled to respond.

This is the price of being part of a community. You are PART of the community. You cannot control how other people respond. You can only let other people in. In my life, I have found much more joy in letting people in than in keeping them out.

Friday, September 26, 2008

good day

Today, for the first time, I was able to imagine playing with Turnip. Before dinner, Evelyn went out in the front yard to play with our next-door neighbors. Watching them all running and laughing, Turnip finally became a person instead of a problem....at least for a moment.

Wednesday, September 24, 2008

ghost hearts

Today I read an interesting article in Popular Science. A scientist in Minnesota has brought the hearts of dead rats back to life. Now, she has begun trying to do the same with pig hearts. Her research holds promise for patients with TA, not because they need new hearts, or rat hearts for that matter, but because of her elegant technique.

According to Popular Science, Dr. Taylor kills all of the living cells in a heart. Then, she replaces them with living cells from a second animal. The new cells move into the "ghost heart" and start dividing. Within a few days, they start beating. Within a week, the entire heart has revived--but with a twist. Now, all of the cells are from the second animal.

Dr. Taylor has used the same technique to revive a dead aorta (one of the major blood vessels leaving the heart). If an aorta, why not a pulmonary trunk? If an entire heart, why not a heart valve or two?

Turnip will have repeat open heart surgeries because he will outgrow the pulmonary trunk the surgeons give him after birth. This replacement part will be dead. It will not grow as Turnip grows. If, however, a pulmonary trunk could be revived using his own cells, then, not only would it be alive, but it would also grow with him...and relieve the need for re-operation.

The bad news: Dr. Taylor has only revived rat hearts---not human hearts. Human clinical trials take quite a while to get approved and then another long while to run. This will not benefit anyone this year, but maybe in five years--if the human trials work out.

Still, I think I'll read Taylor's research in Nature Medicine.

Tuesday, September 23, 2008

conceding the point

I just joined an online support group for the parent's of kids with TA. It feels like giving up or conceding the point. My profile name is mamatytonidae. First person to correctly guess the origin of this profile gets a special delivery Cow Pie all the way from Baraboo, WI (if I have your mailing address)!

Monday, September 22, 2008

ambivalence

I have mixed feelings about Turnip. Some days, I want him to survive. Other days, I wish the whole ordeal would be over. Yesterday was one such day. Yesterday, the treatment seemed as bad as the disease. Death would only happen once. Open-heart surgery will happen over and over again. If he survives, I will have to hold my little boy and explain to him why we are letting the doctors hurt him again...and again...and again.

I guess I just want to protect him. And I can't.

Friday, September 19, 2008

What next...

The pediatric cardiologist (Dr. Wilson or Dr. Allen) says that Turnip's prognosis looks good. He said that I will most likely give birth in Milwaukee...where the heart surgeon works. He also said that I would most likely be induced at 38 or 39 weeks. By the doctors calculations, 38 weeks falls on my grandfather's birthday (Dec. 24) and 39 weeks falls on my birthday (Dec 31--which also happens to be my dad's birthday). If he's born on John Poppy's birthday, I think we will name him John Michael. If he is born on my birthday...I don't know. Any requests, dad? I'm not sure I like Roosevelt for a first name, but maybe Roosevelt Jesse...Hmmmm....

Anyway, the doc said that Turnip would most likely go into surgery between 1 week and 1 month of age. When the baby is born, he will switch from a fetal circulation pattern (getting all of his oxygen from me via his umbilical cord) to an adult circulation pattern (getting all of his oxygen from his lungs). This switch is very stressful for the defective heart. The surgeon will wait for this circulation pattern to stabilize before doing the surgery. He may wait longer, if Turnip seems to be doing o.k. This will give him a chance to grow and will make the recovery easier.

After the surgery, Turnip will be on a diuretic, like Lasik, for about a month, to reduce the swelling caused by the heart-lung machine. He will also be on digoxin (I think) to help his heart stay calm for about 6 months.

Turnip will be having an echocardiogram and ultrasound every four weeks until he is born. Our cardiologist is trying to set up the next round of appointments in Milwaukee, so we can visit the hospital and meet the physicians. He said that the doctor who does the prenatal cardiac imaging in Milwaukee is (if I remember correctly) Michelle Frommelt.

A few weeks ago, a friend in the imaging business emailed me saying he know two cardiologists in Milwaukee. Dr. Frommelt was one. I'm so glad we told my friends...who happen to live in California. I had forgotten what he does for a living. The connection comforts me.

The devil...

...is in the details. Today, I learned that we went to the only pre-natal imaging center in Madison that would have picked up Turnip's heart defect. Dr. Droste (yes, she's related to the chocolate family...I asked) said that 90% of pre-natal ultrasound facilities only check for a four-chambered heart--they don't check the the vessels that take blood away from the heart. Turnip has a beautiful, normal-sized, normal-shaped, four-chambered heart.

The Perinatal Diagnosis Clinic at Meriter Hospital also routinely checks to make sure the fetus has normal vasculature. Dr. Droste said she wasn't aware of any other imaging clinics in Madison that check outflow.

Turnip's early diagnosis means that specialists will be on hand when he is born. They will know exactly what is wrong. They will know what to monitor. They will know how to help him if something starts going wrong.

I've been reading the blogs of other families who have children with the same defect. Many of them were not diagnosed until the babies started going into heart failure--several weeks or months after birth. By that point, the heart and lungs have already sustained damage.

I feel so grateful that I will have processed my grief and made the hardest decisions before Turnip is born. I feel so grateful that he will get treatment as soon as possible.

Turnip is growing...

Kevin and I just got back from the echocardiogram and growth check. The sonographers estimate that Turnip weighs about 1#10 oz., has a 23 cm-circumference head, and is indeed a boy (see image below). All of these measurements are right on target for his gestational age.


He's also started putting on some fat, so his face looks a bit more human. In the bottom picture, he's got his hand up over the top of his head.

Turnip co-operated with the sonographers much better today than last month. However, he told them to, "shove off," in no uncertain terms when they pushed too hard on the bit of anatomy shown below.

...but his heart is still broken

The pediatric cardiologist evaluated Turnips heart function. His heart looks normal size (see image below). The blood is flowing at a normal velocity. The blood vessels that go from his heart to his lungs (pulmonary arteries) are growing and developing appropriately. And, it looks as if the one outlet valve that he does have (the truncal valve) is in good shape.

In the first image, you can see Turnip's four-chambered heart, and, if you look closely, you can see the hole in his heart, aka, ventral septal defect (VSD). I pointed to it with a red arrow.


In the second image, you can see blood flowing through the heart. I can't remember how this is oriented, but, I do remember that the blue backwards "C" to the right of the image is Turnip's aorta/common trunk. It was easier to figure out the anatomy without the blood flow, but I didn't want to push my luck by asking for yet another image.

The doctor still couldn't figure out if Turnip's pulmonary arteries are growing directly out of his common trunk or if he has a separate pulmonary trunk. If Turnip doesn't have a pulmonary trunk, the surgery will just take an extra 15 minutes.

Tuesday, September 16, 2008

scared, or "things I'd rather never have done, II"

Friday, we go in for a follow-up ultrasound and echocardiogram to check Turnip's growth and development. We will learn one of two things. Either Turnip will be the same (normal size for age, appropriate development, etc...) or his blood vessels will have begun to deteriorate and he will have begun falling behind developmentally.

To prepare for these tests, Kevin and I met with our religious leader. We discussed the ethics and implications of termination, in case Turnip's condition has deteriorated so much that he would not survive outside of the womb. I hope the things we discussed remain completely hypothetical.

I could have gone a lifetime without that conversation.

who do we tell?

We haven't told anyone in our hometown about Turnip's problems (except our religious leader). I think we probably won't..for as long as possible. At first, I felt a bit dishonest. But, I must say, it's nice to just talk about the happy parts. I don't have to feel sad every time I talk about the baby...because no one here is asking me about Turnip's health, or looking at us with pity, or saying they're praying for us. Those things aren't bad, they're just reminders. Reminders I don't want every day.

(Plus, when I feel sad, I can call my family (who all know) or visit the blogs of other families who have faced the same challenges)

Sunday, September 14, 2008

things I'd rather never have done, I

So far, most of our heartache over Turnip is prospective and hypothetical. What will happen when...? How will this affect...? What will it mean, if...?

This week, the pain became more immediate. After my scare on Friday night, I realized that we hadn't prepared for Turnips birth. We hadn't thought about who could watch our three-year-old, when I go to the hospital. I didn't have the phone number for the hospital or the doctor or the after-hours advice nurse. We hadn't written out our wishes for Turnip's care.

Now, Kevin has decided who to call about Evelyn, I have the triage number for the birthing center we may deliver at, and I have downloaded the DNR form from the hospital website.

We plan to keep the DNR in effect until Turnip is cleared for surgery (Plans being, as always, subject to change). If he makes it that far, we will remove all restrictions on his care and go for the grand medical interventions.

I've never done anything that hurts more than signing a piece of paper saying, "if my baby dies, please, let him go."

Saturday, September 13, 2008

false labor

So, I've been having Braxton-Hicks contractions for months. I got them when I was expecting my three year old, too. I don't find them too painful, just odd. Breathing becomes difficult and I need to stop what I am doing for a moment until the knot in my abdomen unties.

Last night, I experienced a beast of a different nature--false labor. It lasted for two hours, with painful, sustained contractions--some five to ten minutes long, some as close as two minutes apart. I thought I might meet Turnip a little sooner than expected. But, things settled back down to normal before I figured out who to call--my doctor, the on-call nurse, the hospital...

This morning, I got the triage number for the maternity ward at the closest hospital. Best to be prepared in case this happens again.

Friday, September 12, 2008

endurance sports

The doctor said that Turnip will need to avoid high-impact sports (think football and rugby) and will, most likely, not win any endurance events (think marathon and ironman).

I don't know about the second limitation. Last night, Turnip started practicing flips, pikes, twists, and tucks around 5:30. He didn't stop until 9:00. He wore me out. All I could do was stretch out on the couch, groan and try to anticipate his next jab.

Wednesday, September 10, 2008

grow your own heart valve

As Turnip gets older, he will have repeat surgeries to replace worn out heart valves. If, instead, scientists could grow him a new valve, using his own cells, he could skip these surgeries. Our pediatric cardiologist told us that scientists are trying to do just that. However, he couldn't tell us which scientists or where they work. Of course, I view this promise with a great deal of skepticism.

I've decided to learn a bit more about this research--to learn whether it may help us or whether this technology holds promise for patients in the far future. I get a lot of science magazines. Buried in the most recent edition of the HHMI Bulletin, Kevin found a profile of Kristi Anseth (picture and paragraphs two to seven). She studies this problem. I haven't read her research, yet. More to come...

Tuesday, September 9, 2008

role playing

Yesterday, I braved the virtual tour at the Herma Heart Center. When our three-year-old woke up from her nap, I showed her pictures of the NICU, the operating room, and the Echocardio Lab. She wanted to know all about the boy on the table in the Echo Lab. Did he have a broken heart? Was his name Turnip? Did he have to have surgery? Where was his heart?

For her birthday, we gave Evelyn an anatomy puzzle (I know, a bit strange...but she has been obsessed with "sick guys" ever since she saw a front-page-story about plastic surgery last November). I showed her where the heart was and explained that Turnip was missing a tube coming out of his heart.

For the next few hours, she wanted to play sick guy. She was the guy on the table in the Echo Lab. Using our Mardi Gras necklaces, I gave her an oxygen tube and a feeding tube. Then, we waited while she grew. After she got big enough, I used more Mardi Gras beads to give her an IV. I pretended to do surgery on her heart and then put in some monitoring lines and drainage tubes (more beads). Finally, we had to wait while she got better.

She wanted to play again and again. I don't feel quite as terrified of everything now. I think the purple Mardi-Gras beads helped!

Monday, September 8, 2008

best hospitals, I

We're trying to determine the best place for truncus surgery. Our current pediatric cardiologist said that Stanford, Boston Children's, Philadelphia, and Milwaukee would have topped his list in the past. However, the surgeon at Stanford just retired and the surgeon in Boston just moved to D.C.

Two conclusions:
1) So far, it looks like Milwaukee will be the most convenient place to get the best quality care. I plan to learn more about other locations before agreeing to anything, though. I don't have a job, so I have no problem relocating to get the best possible medical care.
2) The fact that our doc. talked about THE surgeon at Stanford and THE surgeon at Boston Children's drove home just how unusual this condition is. He also spoke in terms of THE surgeon in Milwaukee who does this surgery. Judging by the way our doctor was talking, each of these BEST places to get care for this condition has ONE surgeon who performs the procedure. Obviously, they'd have a whole team working with them, but still...it's not like a heart bypass or orthoscopic knee surgery, where there are hundreds, if not thousands of doctors who perform the operation every day.

Friday, September 5, 2008

angry

Yesterday, Kevin called home to tell me about next year's AAAS meeting. It is in February in Chicago--just a few hours from where we live. This is THE scientific meeting of the year. I planned to go to last year's meeting in Boston. I had hoped to get some smashing story ideas. I had to cancel my reservations due to pregnancy complications.

Kevin hopes to present a poster at this year's meeting. "My grant will pay for a hotel. We can go together," he said.

It was a nice thought. Only, if Turnip survives birth, he will be in quarantine until he goes into surgery. We'd want to minimize the risk of him getting sick. And who do you find to watch your actively-dying baby while you take a trip to Chicago?

"Oh, yeah, if he starts turning blue, just call 9-1-1. No big deal."

costs

I just read the story (scroll down to Thurs Aug 9, 2007) of a truncus baby who had racked up 1.1 million in medical bills during his first five months of life. And the bills keep piling up--regular visits to the cardiologist, medication for reflux and blood pressure. Like us, this family's insurance caps lifetime spending on each individual patient at 2 million dollars. When you reach the cap, the insurance company drops you. Good luck finding coverage with such an expensive pre-existing condition.

I've already applied for state-sponsored children's medical insurance where we live. Apparently, Badger Care will pick up any bills not covered by our private insurance (I'll have to do more research to find out if this is true). We're also fortunate in that 1) I can go back to work and get coverage through a different company, and 2) Kevin should be done with graduate school in two years--so we should be getting different insurance before Turnip reaches the 2-million mark anyway.

Still, the thought is a bit overwhelming

Thursday, September 4, 2008

innocent questions

I can no longer hide my growing bump. People look at me and say, "congratulations." Then they ask when I am due, if we have picked a name, and how the pregnancy is going. The questions are innocuous--like asking about the weather.

The answers, however, are far from simple. Every time I say, "thanks," "the turn of the year," "we haven't decided yet," or "oh, it's fine," my heart breaks. I know, but I cannot bear to tell.

Wednesday, September 3, 2008

perspective

I read this article back in April. I don't remember why I saved it--maybe because the author lives in Madison, maybe because I liked the writing. In any case, I found my copy a few days ago while cleaning out my "in box."

When I re-read it, I realized that the author has been where I am now heading...if we are lucky. All I could do was cry.

Tuesday, September 2, 2008

breast milk

Before we learned about Turnip's problems, I decided that I wouldn't go out of my way to feed him breast milk.

My daughter never mastered the art of breast feeding, so I expressed milk and fed it to her from a bottle for her first six months. Of course, she was my first child, so I had the time and energy to do this. My mom found the whole thing ridiculous and kept telling me to just use formula. The more she pressed, the more determined I became to do things "my" way.

This time around, I decided that Turnip could either do things the old-fashioned way or drink formula. However, in light of recent developments, I've rethought my decision.

Turnip will have less energy than a healthy baby AND he'll have to use more of his limited energy to send oxygen around his body than would a healthy baby. Which means, he'll have less energy for fighting infections (Not that any baby has much to speak of in the way of an immune system any way).

However, I can fight infections for him. Before he is born, my infection-fighting powers travel through his umbilical cord and keep him healthy. After he is born, those same powers (called immunoglobins) get mixed into my breast milk. No formula that I am aware of has active immunoglobins. Hello, Medela, here I come again!

Monday, September 1, 2008

quad screen

I had blood drawn two weeks ago. My doctor ordered some tests that estimated my chances of having a baby with Down's Syndrome, trisomy 18, or a neural tube defect (spina bifida, etc...). I got the results back yesterday.

All of the tests were very normal--which is reassuring and bolsters my decision not to get an amniocentesis. Based on these tests, Turnip has a much lower than average risk for any of these complications. However, these are only screening tests--there is a margin of error--so I'm trying not to get too excited over this little bit of good news.

decisions

When we first learned about Turnip, I saw two decision points. The first: to continue the pregnancy or to terminate. Then, if Turnip survived birth: to pursue aggressive surgical treatment or to allow him to die peacefully.

Now, I realize other decisions will impact my family far more. I can treat insurance agents, who have never before had to deal with this particular condition, with humor and respect or I can vent my frustration on them. I can actively include Kevin in my frantic search for information or I can blame him for not instigating the search. I can help Evelyn and Kevin come to know and love Turnip and his daily acrobatics or I can ignore his antics.

The first two decisions still loom large. We will not make either one until we understand more about Turnip's condition and have a better idea of his chances for survival.

The other decisions may seem trivial. However, I believe the sum of such trivial decisions will either bind our family together or tear it apart.