another appointment, another diagnosis

No, unfortunately I’m not joking.

On Tuesday I saw my regular perinatologist. The detailed u/s showed some growth discordance.

On Wednesday I drove to the University of Minnesota. After another detailed u/s, the new perinatologist and I had a nice chat. He’s a Washington native, too, and very nice. He said he agreed with the original diagnosis, but “we’ll let the cardiologist confirm it.” We discussed delivery and while he wasn’t thrilled with the idea of me delivering locally then transferring the baby, he said, “We’ll keep our options open.”

Then it was off to pediatric cardiology for another fetal echo; this time, on both babies. The new pediatric cardiologist came in to look at the pictures, then we went to talk.

There is a FOURTH diagnosis. Double Outlet Right Ventricle (DORV). So, now we’re dealing with DOVR, TGA, VSD and PS. Devastating news. The newer Arterial Switch Operation is completely off the table, not even an option. The course of action now is an injection of Prostoglandins immediately after birth. Within a week, placement of a BT Shunt (3 weeks in the hospital.) She said these kids have major issues with suck, swallow breathe and reflux, so he needs to demonstrate that he can gain weight and hydrate. He’ll be followed closely by cardiology and at some point, have the Rastelli procedure (she said likely between 5-8 months). They’ll build a Gore-tex conduit in the heart, which of course brings another set of issues, introducing a foreign material, which the body fights, plus the conduit has to be replaced as he grows and with buildup. Estimated hospitalization time for this is 4 weeks.

I asked about causes and she said nobody knows. I told her that I’d heard there’s a higher incidence in IVF babies and twins. She said that was true. I also told her that I’d read things online such as poor maternal nutrition, alcohol use during pregnancy, diabetic mother, etc. She said none of that is true and that I certainly wouldn’t find it in medical/ cardiology literature. As for how common something like this is, she said, “About 10 cases a year.” DORV is very rare.

The cardiologist wants additional chromosome testing, ultrasounds of the head and kidneys, and a few other things, right after birth. She also said the lungs can be compromised, so throughout life, the slightest runny nose means a trip to the pediatrician, concerns about RSV, etc. Six and a half hours later, I left. Then drove the four hours back, in the first snow of the year. Don’t I have all the luck?

Yesterday I was a confused mess. I called the original pediatric cardiologist and he met with me within hours. The discussion was very informative, and he clarified the need for additional chromosome testing (which hadn’t made any sense to me since the amnio was normal.) Apparently there are some cardiac lesions that are associated with chromosome issues that aren’t included in the amnio. He named one in particular, which sent me marching upstairs to my perinatologist’s office (they’re in the same hospital.)

My perinatologist’s nurse showed me the test results, negative. She also said my doctor was in, if I wanted to see him. I waited for him to finish seeing patients, then he and I sat down for another long, very helpful discussion.

This morning I left a message for my high risk OB’s nurse, and had asked that she call me. My doctor called instead; she’d just read the reports from the U. We had another long talk. She also informed me that she’d spoken with the neonatologist and he said that if I wanted to deliver there, he was okay with it. One of her partners brought up the issue of transferring the baby to Minneapolis, in February, the concern being weather/ blizzard related. Of course with the new diagnosis, my doctor doesn’t think anyone will be comfortable with delivering locally :(

So, where does this leave us? Well, obviously in the hands of some fantastic doctors. My high risk OB, original perinatologist and original pediatric cardiologist have to be some of the most caring, compassionate providers I’ve ever met. Ever. Of course we are devastated, beyond words, devastated. For now I’m scheduling appointments with other providers, researching like mad, and making a quick out of state trip for additional opinions.

Baby A is a very sick little boy, and we could use all the good thoughts and prayers you have.

First appointment in Minneapolis is scheduled

I received a call from the University of Minnesota. It seems that both my perinatologist and pediatric cardiologist contacted them. U of M wants to see me ASAP, so they have me scheduled for an ENTIRE day of appointments later this week. Repeat fetal echo, another detailed U/S, meeting with another perinatologist, another pediatric cardiologist, then a meeting with the “team” to discuss the delivery plans. She said they want to discuss the delivery plan since I’m “getting so close.” Err, I’m only 21 weeks, and considering my goal is 38 weeks (though probably not very realistic), I don’t feel that I’m getting close, at all. Oh well. The appointment is scheduled and this will be the first of many ‘leave the house by 5am, drive 4+hour’ appointments.

20 weeks 2 days, appointment with my high risk OB

On Wednesday I saw my high risk OB. It was a fantastic appointment. Labs and blood pressure are perfect! We discussed the appointment with the pediatric cardiologist and the previous appointment with the perinatologist. After telling her some of the highs and lows of the appointments, she shared some personal experiences, which helped TREMENDOUSLY.

I nervously sat in the chair as I told her of my desire to deliver at her hospital, with her. Much to my surprise, she didn’t say no. Things could still change (much depends on the next fetal echo), but she’s going to move forward as far as discussing it with her partners and speaking with the head of neonatology. Last time I went into labor, and delivered, in the middle of the night, she wasn’t on call. I fear that will happen again, so she’s going to request permission to deliver me, even if she’s not on call. She doesn’t think this will be a problem since they’ve granted special requests for her to deliver other doctors and nurses, in the past. Plus, she’s basically the head of the department, so hopefully speaking with her partners is just a formality. She also wants the neonatologist in the OR when I deliver, so she needs to get his okay too. The BIG problem with all of this is that she’s going to be out of town for most of February (medical conferences). The timing couldn’t be worse.

She has concerns about preterm labor and said that she’ll likely give me steroid shots to mature the babies’ lungs, in case they come early. As for now, everything is stable and she doesn’t need to see me for two weeks. She gave me a big hug and I seriously left my appointment with a bounce in my step, for the first time in weeks.

Then I hit reception…

I told the new receptionist that I needed to schedule an appointment with Dr. Hopeful in two weeks. She told me it wasn’t possible; she was booked throughout the month, and asked who my second choice would be. My jaw dropped. Second choice? Um, no. I explained to her that I was high risk, and only wanted to see my doctor. The other receptionist overheard this and told the new girl to speak with my doctor’s nurse. The new receptionist said she’d do that, but told me to keep a second choice in mind.

At 8:30am yesterday morning, my doctor’s nurse called. The receptionist had spoken with her, she then talked to Dr. Hopeful, and Dr. Hopeful told her to fit me into her schedule in two weeks AND book my next SIX appointments. HOORAY!!!!!!!

As completely awful as this is, and it is AWFUL, I have such amazing doctors!

First fetal echocardiogram, first appointment with the pediatric cardiologist

Tuesday started with the echo. For over an hour and a half, I was on the table while the tech took hundreds, yes hundreds, of images of Baby A’s heart. It is clear that something is wrong, and the tech was an angel, absolutely as sweet as could be.

After that, I walked across the hall to meet the pediatric cardiologist. Everyone always asks, “Will someone be joining you?” “No, it’s just me.” A sure sign you’re about to get bad news, and that most people don’t go through this alone.

Unfortunately, the perinatologist’s diagnosis is correct, and lucky us, not one, but three heart defects.

Transposition of the great arteries (TGA)

Pulmonary Atresia/ Stenosis

Ventricular Septal Defect

The pediatric cardiologist drew pictures and diagrams, as we talked. Fortunately I know a bit more than the average bear about normal heart anatomy, so we were able to move through that portion rather quickly. As usual, causes were discussed. As usual, it comes down to a slightly higher risk of heart defects in IVF babies, twins, and sometimes really bad things just happen.

Moving on to the “what does this mean” portion of our discussion…

He started by saying that this is much more complicated because I’m pregnant with twins. They are very concerned about preterm birth, since I delivered a bit early with Kate, and twins generally come even earlier. The “goal”, in his opinion, is to get me to 35 weeks, anything after that is a big bonus. If I can make it to 35 weeks, they should be able to do open heart surgery within a few days of birth, as scheduled. Otherwise, we’ll have some NICU (neonatal intensive care unit) time before surgery.

The best-case scenario is that they’ll be able to do a newer (about 20 years old) procedure called the Arterial Switch Operation (ASO). He said they “think” this operation will ultimately provide the best quality of life, best long-term outcome for TGA patients. Unfortunately, since we have some other issues, the ASO might not be possible, in which case they’ll do an older procedure (plus end up building an additional structure with a foreign material), known to have more subsequent long term effects, additional open heart operations, and possibly leading to a heart transplant one day.

One of my major concerns is being on pump during surgery, as I know many adults have said they’ve never felt the same after being on the heart lung machine. We discussed the consequences, long term effects (not well studied in children), etc. This is one of the times I really, really wish I hadn’t worked in cardiac surgery. I know what it’s like to be in the OR, what it’s like to see a patient’s sternum cut open, to go on bypass, etc. Very different when it’s your child, your newborn. He completely agreed.

We discussed hospitals and surgeons at great length. The bottom line is that bad things can happen anywhere, and things can go downhill quickly in any OR, which I know. It’s important to me that we have a surgeon with a lot of experience, not someone who sees maybe five TGAs per year. He gave me the names of surgeons at Stanford, Boston Children’s, CHOP in Philadelphia, and a few others. Location wise, a hospital in Minneapolis would be ideal, as it’s only 4+ hours (each way) from where I just leased a townhouse (days before the initial diagnosis). There are three options in Minneapolis (U of M, Children’s and Mayo). The pediatric cardiologist doesn’t think that one is better than the others, though he personally knows the surgeons at U of M and is still on clinical staff there. The fetal echo films were already en route to U of M as we were speaking, though they can be sent anywhere, if we choose to go to a different hospital. They are making arrangements for me to meet the “team” at U of M sooner than later, in case of preterm labor, and in case I don’t think it’s a good fit, we’ll have time to go elsewhere.

Another topic I brought up is delivery. I’ve been told repeatedly that I need to deliver at a major cardiac center, which I completely understand the rationale behind. However, knowing that the baby has a severe VSD (which buys some time), and other circumstances, I asked the cardiologist about delivering locally, as originally planned. He said the “team” wouldn’t like it (which I know), but due to our unique circumstances, he’d push for it, if the VSD is still large, and everything else looks stable. Before the judgment starts, please know that I’ve put a lot of thought into this, and I know the risks. That said, I’m trying to figure out how to divide myself three ways, add in that I have a very sensitive, almost 16 month old, who’s already been through a lot over the past few months. I’d like her to have stability as long as possible, before her world is once again turned upside down. The other factor is that often times, TGA is not diagnosed in utero, so it’s a big surprise after delivery, chaos ensues getting the baby transferred, etc. In my case, everyone will know ahead of time, plans can be in place, and if we do end working with U of M, a medical transport from here to there is not a huge undertaking. Plus, I still have time to think about this and change my mind. He repeatedly said he doesn’t envy us, or the decisions we have to make.

The plan is to repeat the fetal echo between 28-30 weeks. He said he’s always available, if I have any additional questions or need anything. Three hours, well spent!