Dancing Queen

It was around midnight on November 6, 2007. I laid in bed taken over by fear. My husband tried to calm me down, but it was no use, I knew there was something wrong with my baby’s heart. I was 20 weeks pregnant and at my 18 week ultrasound, the tech told my husband and I that she couldn’t see all of the structures of the heart because the baby was too small. I had to return in 2 weeks by myself. While she never indicated that anything was wrong, I knew my baby, my precious Dancing Queen, had a heart problem and I cried myself to sleep.

Later that morning, the same ultrasound tech took an extremely long time examining DQ’s heart. After what seemed like an eternity, she brought in the doctor who also looked for a long time. Then he told me my darling baby had tetralogy of fallot (“ToF”). He explained the 4 heart defects that make up this condition and he showed them to me on the screen. I was amazed at how large the VSD was, I could easily see it myself. Then he explained that he could not find DQ’s pulmonary arteries, but they may be there. Finally, he told me that it was possible that DQ had a chromosomal abnormality as well. I heard none of it as all I could do was concentrate on not crying. I only shed one tear in that room. Then the doctor led me to the consolation (consultation) room to sit while he searched for one of my OBs. As soon as the door shut, the flood gate opened. My life had changed forever.

During the next couple of days, I cried a lot, I learned a lot, and I started to plan for this new life that dropped into my lap without warning. I filled the last 19 weeks of my pregnancy with more ultrasounds, an amniocentesis, meetings with doctors, sessions of research, and a mountain of worrying and planning. We learned that DQ also had a microdeletion of chromosome 22q11.2 (VCFS or DiGeorge’s Syndrome), but we did not know what to do, so I learned and I planned. My baby girl would start her life fighting to keep it and my job was to get her the best tools to win that fight. Planning was my refuge. It allowed me to feel proactive and prepared for what lay ahead. Only later did I learn that you can never prepare yourself for the helplessness that is seeing your child in pain.

DQ was born on St. Patrick’s Day 2008 looking pink and perfect. The nurses in the operating room informed us that, without the prenatal tests, DQ would have been declared a healthy baby, ready to go home. Instead, she was whisked off to the NICU where she had an echocardiogram. My husband and I didn’t even get to hold her.

Within a couple of hours of DQ’s birth, the cardiologists confirmed that she had severe ToF with pulmonary atresia, “millions” of tiny collateral vessels supplying blood to her lungs, and no PDA, which usually keeps babies like DQ alive once out of the womb (luckily, DQ’s collaterals saved her). I still had not seen my baby, and as I had planned, she was due to be transferred to the children's hospital, but on the way they brought her to me. She was several hours old and by now connected to many tubes, but I got to hold her for the very first time.

I was not able to hold DQ again for several more days. She had her first heart catheterization when she was 1 day old. At 2 days old, DQ had several episodes of SVTs (extremely fast heart rate). She was intubated and hooked up to countless wires, tubes, and monitors. I joked that she had more wires than an HD TV, but seeing her that way was the hardest thing I had ever done prior to that point in my life.

At 7 days old, DQ underwent her 1st major heart surgery. The wait during surgery was agony, but after, she was alive and the surgery declared a success. The next day, I returned to DQ’s room to find dozens of scrub-clad people huddled around my baby’s crib, spilling into the hallway. DQ’s blood pressure had dropped dramatically and they had to use the pace-maker to keep her going. She had almost died. I have never been so scared in my life. At one point, the unit chaplain offered support to my husband and me, but in our frightened state it seemed as if he were offering condolences for our loss. The brief episode seemed to last forever, but DQ eventually stabilized. Afterward, she looked so awful. She was weaker than a newborn. She was ashen and gray. She was extremely swollen from the surgery. And she was sedated by heavy narcotics to keep her from feeling the pain. When she was awake, my heart broke with the vision of her silent cry. The ventilator helping her to take in oxygen served a second purpose of reducing her already weak cries to inaudibility. My husband and I quickly learned to hate this tube.

As the days went by, DQ still was not progressing as expected. Exactly 1 week after her surgery, DQ had her 2nd heart cath. That cath confirmed that the surgery had worked. The surgeon had placed a BT shunt from her aorta to her pulmonary arteries, bypassing the normal circuit for blood flow. The placing of that shunt allowed DQ’s miniscule pulmonary arteries to double in size during that 1 week. The only thing left to be done was for DQ to grow. Unfortunately, that did not happen quickly.

The next several weeks were a roller coaster. DQ was on a ventilator for over a month, during which we could not hold her. She would appear strong enough to be extubated, and we would be excited. Then she would go backward and have to be re-intubated. My heart would break all over again. DQ did get off the ventilator eventually but still had to have a nasal canula to provide a mix of nitrogen and oxygen for her to breathe. On the day she was supposed to be transferred back to the NICU, she had several episodes of SVTs and took several steps back again. It took many more weeks before she was stable enough to transfer out of the PICU.

On May 8, 2008, DQ went home and met her big brother, TRex, for the first time. I was ecstatic and scared to death. DQ was on a feeding tube, ten medications, and had two to three doctors’ appointments each week. I did not know how we would cope. In the beginning, each day was a challenge, but much like bringing any new baby home, we fell into a routine. In June, my maternity leave ended, so I returned to work. DQ joined her brother at day care. She did really well and started to flourish. She loved seeing all of the kids.

We had to work hard to get DQ to drink agonizingly small amounts of milk. After several trials, including multiple formula changes once my milk was gone, a second surgery unrelated to her heart, and a hospitalization for sharing the same cold virus as the rest of her family, she turned a corner. By the end of June, DQ was off of the feeding tube (we still had to work hard to get her to eat).  There were some roadblocks along the way, such as contracting the adenovirus in November 2008 and almost dying once again, but overall DQ was a very happy baby. She started early intervention at 5 months and started to overcome her developmental delays from being hospitalized so long.

But, in January 2009, DQ started to decline. She was outgrowing her shunt. On April 13, 2009, DQ underwent her “full-fix”, where the doctor patched the extremely large hole she had between the ventricles, made her aorta go over the correct part of the heart, and placed a conduit from her right ventricle to her pulmonary artery. DQ’s natural pulmonary valve is useless, but could not be operated on because DQ’s coronary artery splits in 2 and 1 of the branches is directly above where the surgeon would have to cut DQ’s heart to fix the pulmonary valve. So, they didn’t do that and instead put a homograph from a donor to supply blood from the heart to DQ’s lungs. DQ’s surgeon described the surgery as a tune-up, rather than the traditional full-fix. We all knew she’d at least need her conduit replaced before she was 6.

DQ’s recovery was difficult once again. She was intubated for almost a week post-op and had to be re-intubated after a couple of days because her lungs couldn’t adjust well. She was pink though! After 3 weeks, DQ came home. Unlike most kids who get the full-fix for ToF, DQ didn’t get markedly more energetic. She continued to progress, but she still remained fairly weak. The summer following her “tune-up”, DQ was hospitalized twice for 10 days each with respiratory illnesses, she was diagnosed with asthma, and she just had a rough go of it.

In August 2009, DQ was diagnosed with pulmonary hypertension (“PH”). The blood pressure in her pulmonary arteries was much higher than it should be, causing the heart to work too hard, thus damaging it, and damaging the lungs. The first course of action was a heart cath. She had that in September 2009 and they inserted a stent in DQ’s right pulmonary artery. We were also told at that time that DQ had moderate pulmonary vascular disease. While normal lung vasculature look like a tree with leaves, DQ’s looks like a tree without leaves.

We thought that the stent helped DQ’s PH, but she continued to have symptoms of right-sided heart failure (swelling, shortness of breath, severe leak in the tricuspid valve). The week of Christmas 2009, DQ went to the cardiologist to get clearance to have ear tubes placed. At that time, we were told that she still had PH and that because of the pulmonary vascular disease, the doctors had no drugs, no surgery, nothing they could do. They put her on oxygen at night to give her lungs a break, but left us feeling lost and devastated.

Not accepting such an answer, I began contacting pediatric PH specialists. Most of them gave me the same answer, but doctors at Stanford agreed to take a look. In preparation for that second opinion, DQ underwent her 6th heart cath in February 2010. At that time, they started her on sildenafil (viagra) for the PH and tried to find something to stent. While all of DQs pulmonary arteries are way too small (or severely hypoplastic in medical terms), they are all grouped too closely together for stents to open them (they look like an octopus).

We went to Stanford in July 2010 after DQ had spent much of the spring in the hospital with respiratory infections. We were told then that DQ’s PH does not likely come from the itty bitty pulmonary arteries, but from her heart. Those cardiologists thought DQ’s right ventricle had a aneurysm. That added to her conduit not having a valve could cause her PH, so the Stanford doctors suggested that DQ undergo another open heart surgery to replace the conduit with a valved conduit, fix the aneurysm, and fix the tricuspid valve.

DQ had that surgery on Monday, September 20, 2010. Turns out, she did not have an aneurysm, but a huge gap between two leaflets of her tricuspid valve. The surgeon closed the gap, closed her PFO (small hole between the upper chambers of the heart), widened the opening to her left pulmonary artery where the stent in the right pulmonary artery was blocking blood flow to the left, and replaced DQ's conduit with a valved pig conduit. She now has a mechanical valve that is really loud.Nobody knew before the surgery if it would work and we were told not to expect too much, but she did wonderfully and was home in 5 days. Her heart started improving and her pulmonary pressures dropped considerably. DQ still does not have great heart function and she still works very hard to oxygenate her blood. As she grows, her pulmonary arteries will likely not keep up, meaning her heart will have to work harder and harder to maintain her. But, today, her heart is the best it can be. And DQ's cardiologist will tell you DQ's weakest point is her lungs. (DQ's pulmonologist will say its the heart!)

You would think with all of these clinical problems (DQ has a lot more than her heart issues) that DQ would be grumpy. She is not grumpy. She is one of the happiest kids you will find. She turned 3 in March and talks up a storm. DQ loves music! She loves to sing and dance. She must sleep with Elmo and her big brother is her best friend. DQ is a goofy kid who loves to color (even on me and my floors). She LOVES going outside. The swings are her favorite, but slides are starting to surpass her love for swings. DQ  loves books and throws temper tantrums. If you saw DQ, you wouldn’t know she was sick, except for the scar that peaks out of most shirts or if you look at her medicine drawer. DQ can tell you the names of all 10 of her meds. But most of all, DQ is a fighter. We have hope that DQ’s fighting spirit will keep her with us for a very long time, well into adulthood and beyond.  After all, she constantly tells me she will be a mommy and a ma (grandma) when she grows up.  That along with being a Lightening McQueen, a Mickey, and a Thomas the Train. In the mean time, we get in as many hugs as possible.

If you want to follow DQ's journey, I maintain a carepage for her. Send me an email at MomOnALine (at) gmail (dot) com and I can send you the link. A fuller look at DQ's life from 2009 to 2011, is at my post for Every Heart Has a Story.
The Dancing Queen in the fall of 2011, shortly before we learned she has started to decline again.
Having a child with a CHD is like being given an extra sense---the true ability to appreciate life. Each breath, each hug, each meal is a blessing when you've watched your child live off a ventilator, trapped in an ICU bed, being fed through a tube. Each minute is a miracle when you've watched your child almost die and come back to you.
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