In The Beginning
I had been followed by a rheumatologist since I was four years old. Over the 26+ years, I had been on a variety of hazardous medicines. I knew most of them weren’t compatible with pregnancy. They had detrimental effects on fetal growth and development.
Five years prior to getting pregnant, I started to have the conversation with my doctors. I wanted to make sure 1) it was even possible for me to conceive a child 2) it was safe for me, and 3) it was safe for my future child.
I started taking the infamous Hydroxychloroquine, also know as Plaquenil. From my understanding, this medication was not only safe during pregnancy, but came highly recommended. I had eliminated all other unnecessary medications. The only prescriptions I had at the time was my Plaquenil, a baby aspirin, and an iron supplement.
As far as I could tell, my Sjogren’s was under control. I had no symptoms. My labs were great. I had just finished completing the AIP diet, and never felt better in my life. I consulted OB, Rhuem, and hematology. Everyone gave me the okay.
What I Never Knew
Again, I will reiterate I had followed Rheumatology since I was 4 years old. NOT ONCE. NOT EVEN ONCE. Was I ever informed that women with Sjogren’s syndrome risk passing on their SSA/SSB antibodies to their child during pregnancy. The risk here is that these antibodies can cause the baby to have congenital heart block.
I didn’t know this until I was already 12 weeks pregnant, terrified, sitting in the Maternal Fetal Medicine (MFM) office. Shame on me for not looking up the risk of my autoimmune disease on pregnancy. But also, shame on the 26 years of rheumatology that no one ever mentioned this to me. Especially because I clearly stated 5 years prior my goal was pregnancy and wanted to do all I could for a healthy pregnancy.
The very first MFM visit was such a blur. I was completely shocked. I knew I would be considered high risk, but I really didn’t know what it would involve.
I was finally educated on Sjogren’s syndrome, antibodies, and congenital heart block. The good news out of this scary news was that I had a 1-2% chance of my baby developing heart block. That seems like pretty good odds, right? The even better news was that I was already taking Plaquenil which is the medicine they give to women to prevent heart block in the first place. I was relieved, double check. I’ve been on that for 5 years at this point.
Most women don’t even know they have Sjogren’s until their baby develops heart block. Then they are given Plaquenil for future pregnancies.
The 1 Percent
The plan to monitor for heart block was to get a fetal echocardiogram every other week starting at 16 weeks, for me. First of all, it is amazing that they can even do an ultrasound of a baby’s heart when they are that small.
Each week I went in and I would see little Renee’s heart just fluttering away. I somewhat enjoyed getting these echos done, because it meant I got to see my baby! Things were moving right along just as expected. Every cardiologist would tell me the same thing. “Everything looks great, you are already taking the medication to prevent heart block, you don’t have to worry.”
We became so comfortable with these appointments, my husband even stopped coming with me so he could save his time off. I went in for my regularly scheduled 22 week echo, and my mom was with me this time. The tech put the ultrasound probe on my belly, and I could watch the whole thing live on a big screen in front of me.
Something wasn’t quite right this time. The little tiny heart wasn’t fluttering as fast as it normally did. The echo didn’t last as long as usual. I instantly knew something was wrong. That’s when the doctor came in and told me the news I’d been praying to avoid, Renee had developed heart block.
This was so rare. This was never supposed to happen. How did I become the 1%?
The doctor brought my mom and I back to his office to explain what this new diagnosis meant. To say we were all shocked is a complete understatement! I mean expressionless. The doctor furiously started rummaging through documents and my medical chart to try and figure out how this could have happened. At the same time, trying to console my mom and I.
There was chaos. It went from – I was going to be admitted, to I need to see MFM right away, to let’s just get some labs and start from there.
As if autoimmune disease isn’t a curse in itself and the most frustrating thing to deal with, I then had to live with the guilt that my disease was passed on to my child. She would suffer significantly because of something that was out of my control, yet in the palm of my hands at the same time. That is a feeling like no other that I hope you will never have to experience.
Congenital Heart Block
Heart block is when the electrical system of the heart stops working. Typically, our heart is meant to beat at a certain pace and is controlled by a series of nodes and fibers in the heart. In the case of complete heart block, this system shuts down entirely. One of Renee’s nodes became “inflamed” and was unable to relay the signal to her ventricles. Fortunately, our hearts have a “backup system” where there is an intrinsic resting rate and that was able to keep her heart beating until she was born.
This diagnosis meant Renee would need to have a pacemaker to keep her heart beating for the rest of her life.
This was obviously devastating news, as it wasn’t at all expected. However, we finally became comfortable with the fact that Renee would have a pacemaker when she was a toddler. She would be able to live a pretty normal life. There are lots of success stories of children born with heart block who are doing amazing. Most women carried their babies to term, and we were relieved.
Short Term Treatment
The heart block was actually caught very early. My anatomy scan was just performed one week prior, with no signs of heart block. The team got together and hoped that some early intervention could possibly reverse the heart block.
Step 1 – Dexamethasone
This drug was the death of me – it is a steroid. I was prescribed this to help with my kidney issues and in attempt to reverse Renee’s heart block. I was already taking prednisone in attempt to correct whatever kidney issue was brewing. However, I was switched to dex, because this steroid actually crosses the placenta. I couldn’t sleep, I developed gestational diabetes, I had SEVERE acne literally everywhere. My blood pressure was through the roof. Nothing was helping, everything was only getting worse.
Step 2 – IVIg
This one actually made me feel better, but its purpose was to fix Renee’s heart. Unfortunately that did not help. This is an infusion they give to boost the immune system. I had 2 rounds of this 6 hour infusion before doctors decided to throw in the towel.
Irreplaceable and Irreversible
Unfortunately, Renee’s heart block was not reversed with any of the treatment thrown at me. At this point, I was scheduled for a different doctor’s appointment everyday. I am not even kidding…
Renee actually ended up developing a second heart condition 2 weeks later. This one was even more serious and affected her heart and her lungs. The immediate cure for this was to deliver Renee early. This was the first time the possibility of early delivery even came up.
My doctor went over all of my medications again, and asked me several questions. He decided the best treatment was for me to stop taking aspirin.
I went back 3 days later for another echo, and this new heart defect had completely resolved! Thank God, because I couldn’t handle another roller coaster event.
God was laughing though, because only He knew what was to come…