Team Turtle Heart

V Celebrating her first year

V celebrating her first year.

Our Journey with NeoNatal Lupus and Congenital Complete Heartblock

In September of 2010 at a routine ultrasound for our first child we discovered two things. Our baby was a girl. And that our little girl had a heartbeat of just 55 beats per minute. It should have been around 120 beats per minute. She had a condition called congenital complete heart block, the worst possible complication of something called Neonatal Lupus. They didn’t tell us much more at the visit. But we could tell by the look on their face that it was serious.Following the ultrasound, we suffered through two excruciating days waiting on an appointment with a specialist. During those two days we cried. We worried. We goggled. The information we found online was neither reassuring or timely. It was all clinical and out of date. Very little relate-able or personal information was available. We were in the dark and terrified.

So I decided to start this page to share our journey, the good parts and bad parts, with any other parents out there who might find themselves in this situation. It would have been good to know then that  we were not alone.

It sounds very complicated. But basically a layer of electrically conductive tissue that lives between the top and bottom chambers of the heart fails to conduct the current. It insulates instead. It is porcelain instead of copper. That means that the top and bottom parts of the heart cannot talk to each other. So they beat at different rates. It can be caused by Neo-Natal Lupus. It is also often referred to as third degree heart block. This symptom in adults, regardless of the cause, is often called congestive heart failure.
In short, there isn’t one. Once it has developed it is permanent. But they can treat it. Our daughter, assuming the constraints of current technology, will require a pacemaker for the rest of her life.

Because she has a medical applicance, our daughter will have to wear a medical alert bracelet when she is old enough. Her father and I and her school/care provider will have to have copies of her pacemaker registration on us at all times.

She can never have an MRI. She cannot play contact sports like football for fear of knocking a lead wire loose. We have been told soccer and basketball are OK. She can not stand in the security systems in retail stores or in the metal detectors in airports. They can reset her pacemaker. She can not use a chainsaw, arc weld, or work on a running motor. Otherwise she has very few restrictions. Her outlook is for a very normal life.

She will have to have several more surgeries to upgrade the pacemaker and lead as she grows. She will need larger, more advanced generators and leads. But that is with today’s technology. Who knows what her treatment will be in 20 years.

About 4 million people in the U.S. have Sjogren’s Syndrome according to the Sjogren’s Syndrome Foundation. I could not find numbers from the CDC on the predominance of Sjogren’s Syndrome. About 1.5 million people in the U.S. have lupus according the Lupus Foundation of America. The CDC puts that number at approximately 322,000 with SLE.

One to five percent of children with mothers possessing autoantibodies will develop congenital heart block. This accounts for about 1 in 10,000 live births. How does that stack up against other birth defects? Check out the CDC chart for common birth defects per case of birth. But for an easy comparison, your overall chances of having a child with Down’s Syndrome are 1 in 691.

Neonatal Lupus does not mean that your child has Lupus. Auto immune diseases like systemic lupus erythematosus (SLE) and Sjogren’s Syndrome (SS) cause the body to produce things called autoantibodies. In Neo-Natal Lupus, a mother who carries these autoantibodies, which can be harmful to a fetus, passes them along with other antibodies that are good for the fetus through the placenta. These autoantibodies then attack some or all of the following in the developing fetus: heart tissue, skin, liver, and blood elements. Temporary skin rashes, liver abnormalities, and low white blood cells can result from this process. But the most serious problem is permanent congenital complete heart block.Many times mothers carrying autoantibodies have no idea they have them and either their condition or status as an autoantibody carrier is discovered when their child presents with symptoms of Neonatal Lupus.

You can learn more about Neo-Natal Lupus from the experts at theResearch Registry for Neonatal Lupus.

Sjogren’s Syndrome is a chronic autoimmune disease found predominately in women in which white blood cells attack the body’s moisture-producing glands. It is in the same family of autoimmune diseases as Lupus. To find out more about Sjogren’s Syndrome check out the About page of the Sjogren’s Syndrome Foundation.

Getting Her Here

Getting Her Here
As soon as we discovered our daughter’s condition I began taking 4mg of Dexamethisone (a steroid) daily. The hope was to prevent further damage to her heart. 55 beats per minute is exceedingly low for a fetus. Often congenital complete heart block is discovered with rates in the 90s.

We were told to expect delivery around 30 weeks of gestation. As the pregnancy progressed with little drama those estimates were pushed out to 32 then 36 weeks. All along the doctor’s told us the delivery would be by C-section since they could not monitor the baby’s heart rate during a normal delivery and they weren’t sure how she would handle the stress of a normal birth.

From 20 weeks gestation to 28 weeks gestation, I visited an OBGYN specializing in high risk pregnancies twice a week for an ultrasound. At these visits they counted the baby’s heartbeat to make sure that it was not slowing down and that there was no evidence of fluid collecting around the lungs, heart, or brain ( called hydrops).

At 28 weeks I started visiting the doctor three times weekly for ultrasounds. They added a BPP or biophysical profile to the test they ran each time. These visits were supplemented monthly with a visit to a neonatal cardiologist.

At 33 weeks the neonatal cardiologist noticed a downward trend in heart beats per minute. 50 beats per minute was the minimum acceptable threshold. Terabutaline which is often used to delay labor and to treat asthma was used to increase my heart rate thereby increasing the baby’s heart rate. The side effects were not fun.

At 34 weeks the by then routine ultrasound found the baby’s heart beats per minute at just 46 despite the terabutaline. We were scheduled for a C-section later that day.

Welcome to the World Little Turtleheart

Our daughter was born that evening as pretty and perfect as a picture breathing fine and with no other problems than a heart rate of about 55 beats per minute. She was 4 lbs 15 ounces and 17 inches long. No extreme intervention was needed though we had been told to expect a ventilator and the possibility of an external pacemaker immediately after birth.

Our daughter spent her first 12 days in the NeoNatal Intensive Care Unit (NICU). Most of her very limited difficulties came from being slightly premature.

At eight days old her heart rate had steadily slipped to about 48 beats per minute. She received her first epicardial pacemaker. She came back to the NICU on a ventilator which is not something a parent can be adequately prepared for. It was the scariest moment of my life. She recovered from the anesthesia well enough to be removed from the ventilator later that night. We went home three days later.

The Unluckiest Baby

At 13 weeks old we noticed a nasty purple/gray bruise developing on our daughter’s chest where you could feel the bump created by the pacemaker. By the time we could get her in to see her doctor, the bruise had started weeping clear fluid. She was diagnosed with the rare complication of erosion of the pacemaker pocket. It had come loose from where it was supposed to be and was working its way out of her chest. She was admitted immediately for IV antibiotics to prevent infection. When we asked the surgeon what could have caused it to happen he said “she must just be one of the unluckiest babies in the world.” But he assured us the revision would be simple and we would be home in a day or two.

The next day she went in to surgery for a “pacemaker revision”. They were moving it to a different location. The skin at the bottom of her wound did not close properly because of poor skin quality caused by the pressure the pacemaker had placed on the tissue. She basically had a pressure ulcer. This caused complication and seven days later the wound opened and fluid from her chest cavity and the new pocket began flowing from the gap at the bottom of the incision.

She was taken in to surgery the next day for another revision. About an hour in to the surgery we were taken to a consultation room. The surgeon told us that the lead running from the pacemaker to her heart could be seen clearly through the gap in her incision. It was open to the outside air and considered clinically contaminated. The whole system had to be removed. She would be placed on an external pacemaker which would require her to be in the ICU until the tissue healed enough to tolerate another operation and successful closure. We were devastated.

Viv went to the ICU on a ventilator again after this surgery. She was removed from the ventilator later the same day and did just fine. She had an external pacemaker that laid in the bed with her and three wires sticking out of her chest wall that were connected to her heart. One was actively pacing and two were redundant leads in case the primary lead was knocked loose.

Showing Us What She’s Got

The options at this point were limited. A “theoretically possible” placement of an intravenous pacemaker system could be attempted since her body was having trouble accepting the epicedial system. This option posed lifelong complications due to the fact the vein would be lost permanently for future pacemaker placements. The second option was to let her heal at her natural fall back heart rate at home and come back at a later date when her tissue was in better shape and her body was a little bigger. The third option was to stay in the ICU with the external pacemaker until her body was bigger and her tissue in better shape.

Her fallback heart rate – or the rate her heart would beat unassisted – was 52. The doctors monitored her fallback rate a great deal and two days after her pacemaker was removed the external pacemaker was dialed back to 50 beats per minute. The doctor’s monitored how many times her heartbeat fell to 50 and needed to be paced. It did not. The external pacemaker was removed (not the wires) and she was moved to a regular cardiac floor for close monitoring. Her heart rate held, the external pacemaker wires were removed and she was sent home after 14 days in the hospital.

Her heart rate held steady for almost three months at home under close observation from her cardiologist. Her most pressing complication was an inability to adequately gain weight. At six months old she had yet to reach the 0 percentile of the infant growth charts even with three caloric increases to her formula. She weighed just over 10 lbs. A 24 hour halter study revealed that her average heart rates were again dropping in to the 40s and the decision was made to attempt another pacemaker.

Pacemaker #2

Our daughter had her second pacemaker and fourth surgery at six months and three days old. She was sent home the next day with just less that 24 hours in the hospital.

From six months to one year old our daughter set about the business of catching up. She had gross motor skill delays but was advanced in fine motor skills. Crawling came slowly. Sitting up unassisted finally came at 11 months. Weight gain remained an issue. But improvement was always visible.

Well Suprise

Shortly after our daughter’s second pacemaker was installed we discovered we were pregnant…again.

We called the NNL Registry for guidance and within an hour Dr. Buyon personally called back to offer advice and guidance based on the registry’s current research. We chose at that point to use the same great team that had ushered our daughter into the world. We enrolled in the NNL PATCH study which required I take a higher than normal dosage of Plaquenil in hopes of preventing the antibodies from attacking the baby’s heart tissue. I went for regular biweekly ultrasounds and monthly echocardiograms to monitor the baby’s progress. And he was A.O.K. all the way. Almost fourteen months to the day after his sister’s spectacular debut our son quietly made his way into the world and went home from the hospital two days later. An echocardiogram performed two weeks after he was born confirmed a structurally perfect heart.

Get Out of Her Way

Our daughter’s second year was full steam ahead. She finally mastered crawling and at 13 months took her first wobbly steps. Shortly thereafter she moved to the “big girl” class at school. She now runs, jumps, climbes, and throws tantrums like a normal child. Her developmental delays are all but gone. She is still small for her age (First percentile on the growth charts). She eats constantly.

At 22 months old her cardiologist adjusted her heart rate from 120 to 90. He told us to expect no change. However, the 25% decrease in her heart rate produced a 50% increase in her activity and energy levels. She is now bouncing off the walls.

From the Experts

We participate in this research registry. Dr. Buyon personally and her staff have been very helpful in keeping us informed and worked with our cardiologist to develop our daughter’s treatment.

The Pacemaker Up Close

From the Trenches

I have come to think of V’s ordeal with pacemakers as a very loose parallel to a story I follow with strict devotion – George Lucas’s epic, yet campy Star Wars saga. Maybe because I walk by a 7ft Darth Vader and beeping R2D2 in my office every day I see everything in shades of geekdom. In the first chapter of her story, we discovered youngling V Turtleheart’s one in a million condition. We watched her battle the dark side of the force and prevail. The story climaxed when she received her Jedi light saber…I mean pace saber…I mean pace ...
Read More

Hi. My name is Holli and I’m passive aggressive. And I’m southern. And I’m not ashamed of either of those facts. I think they are a very useful pair of tools to have in your toolbox. They compliment each other. I only say this because the other day I had occasion to make a woman almost crawl under the shelves at a local grocery store. And all I really used to do it was a smile and a drawl. Don’t get me wrong, I have bat poop crazy spider monkey in my toolbox too but sometimes that is just overkill ...
Read More

Got the latest insurance statements today from our last stay in the hospital and all I can say is… Pregnancy Test: $10.00 Lemonade and Wendy's cravings: $250 Baby Stuff: $500 High Risk Prenatal Care: $15,000 Labor and Delivery: $27,000 Five months of various and sundry medical expenses for an infant with congenital complete heart block: $275,000 Having good insurance: worth every penny of premium and deductible My little turtleheart: Priceless ...
Read More

V is 17 weeks old and weighs 8 pounds 10 oz. Yeah. We have what is called a failure to thrive. We don’t even break the 0 percentile. We moved up to 26 calorie formula this week. Normal baby formula is 20 calories. She is just not an eater. Never has been even pre-pacemaker, with pacemaker and post pacemaker. The doctor  said that some babies are just petite and that might be what we are working with on top of the three surgeries, the chronic neonatal steroid exposure,  and a slow heart rate. I must admit, I had a moment ...
Read More

Having had lots of time to overanalyze the inner workings of this hospital for the wee ones, I have determined it is run mostly by covens , herds, and cowboys. Think Charmed meets Bonanza. The real work is done by the covens. The covens are made up of nurses, care partners, etc…etc… and they handle all things rubber meeting road. I call them covens because they are primarily female, work all the magic, and when they have a rare moment to stop they laugh in unison like a coven of cackling witches. I have been woken from several naps by ...
Read More

With the removal of V’s pacemaker during the revision of the revision surgery, our options for an eight pound infant that needed a pacemaker began to narrow significantly and become infinitely more complex. One of the three options we were given came with the “theoretically possible” caveat for a baby this small. One was reattempting the same thing we have been trying to do that obviously isn’t working. The other was to leave the baby alone and let her show us what she’s got. Forty eight hours ago they turned V’s external pacemaker down to 50 bpm so that they ...
Read More

V’s second surgery in a week – we are calling it the revision of the revision – did not go well. The surgeons could see an exposed pacemaker lead wire through the hole that had opened in her incision from the previous surgery. Though the cultures for infection have been consistently negative, because the wire had been exposed to the outside air it was considered contaminated. Both the pacemaker and the lead wire had to be removed to prevent the possibility of a life threatening infection. They did repair the hernia and plastic surgery did close the wound. V is ...
Read More

V has had a setback. We were supposed to be going home today but after the drainage tube in her chest was removed yesterday morning, fluid began draining from the bottom of her incision where the skin tissue was already poor quality. Over the next few hours the stitches gave way at the bottom, the incision separated, and a lump formed between the pacemaker and the incision that had not previously been there. V needs to continue IV antibiotics. They attempted to set a pick line four times and were unsuccessful. That was followed by two attempts to place a ...
Read More

We have been closely watching a spot just under V’s rib cage where the edge of the pacemaker could be felt for about two weeks now. It began to bruise last week and started becoming more prominent as we neared the weekend. Over the weekend it became more discolored and the edge of the pacemaker could clearly be made out. By Monday morning, a small area of skin had become so thin it had started to weep clear fluid. The doctor had us come in and he referred us immediately to a surgeon. The pacemaker had become unseated and moved ...
Read More

I started a scrapbook for V when I was 21 weeks pregnant. That was the point that I started the twice weekly ultrasounds. By the time she was ready to enter the world, I had hundreds of “photos” of her that I needed to do something with. She is 5 weeks old today and there are more than a thousand pictures of her on my hard drive so far. I thought I had bought enough scrapbooking supplies to get me through maternity leave. I was very wrong. To make matters stickier, I had tons of time to work on the ...
Read More

(Visited 92 times, 1 visits today)