Every Heart Has a Story: Pulmonary Hypertension

My first time in the cramped, dark office, I was eight months pregnant, listening to him explain truncus arteriosus, the congenital heart defect they thought my unborn baby had, and the surgery she would undergo to help her survive.

My second time in the office, I was in a wheel chair, having recently given birth. My baby, the Dancing Queen, had just had her first heart catheterization at one day old and he explained to us the heart defects she actually had (tetralogy of fallot with pulmonary atresia) and the surgery she would need at three days old.

My third time in the cramped office, he was letting us know that he had no answers. He did not know why DQ was not getting better, why she was still so sick several weeks after surgery. He explained that he may have to go in and replace the shunt he had placed, even though he'd never done it before. He had no answers . . .

So, a year later, when I entered his office for a fourth time, with my one-year-old on my hip, I was understandably shaken. I sat on the worn couch, looking out the window, hoping not to cry. Today, I can't remember if I did cry or not, but what I do remember is his answer to my question of whether DQ would have more energy, eat better, be more like an average kid after her surgery. His response was "no". He said she was already a pretty active child and likely wouldn't have the 180 turnaround that so many other parents saw with their children. I chose not to believe him. I wish I would have.

DQ a week before her surgery in 2009.

In April 2009, a 13 month old Dancing Queen underwent her "full-repair" (patched the huge hole between her two ventricles, shaved her stiff and thick tricuspid valve, widened the pulmonary arteries, and patched the aorta so it didn't open above the outflow and the inflow of the heart), but the surgeon merely called it a tune-up. If only I had heard that statement for what it really meant. I assumed the doc called it a tune-up because the homograft they used to connect her heart to her pulmonary arteries would need to be replaced, not that she would need any other type of surgery. And even after DQ was in the hospital for 3 weeks post op, was on a ventilator for a week, and had to be re-intubated after the vent was first taken off, I still thought she was going to be amazingly better after her repair. I held an amazing optimism.

DQ immediately post-op April 2009

Instead, after getting home, DQ remained fairly weak and actually got worse. She spent a lot of time that summer in the hospital, sick. Finally, in August, it was determined that DQ had pulmonary hypertension (elevated pressure in the pulmonary arteries, which was causing her heart to work extra hard, damaging it, and causing damage to her already small pulmonary arteries). I didn't know much about PH at that time, so I googled it. That was a bad idea. (Much of the data on the general web about PH gives grim prognoses--less than 2 years to live after onset.)  After reading a couple of pages, I stopped, too scared to even think of the possibility. I held onto the one positive nugget I found: if the underlying cause can be corrected, PH can go away. I figured the docs would determine why her CHDs were now causing PH. DQ's cardiologist had mentioned that DQ's right pulmonary artery had pinched down following surgery and that could possibly cause the elevated pressures. She did indicate that would be highly unusual, but I ignored the doubt and hung on to only positive possibilities.

DQ learned to stand in a hospital bed in June 2009 at 16 months old.

In July 2009, DQ perfected standing in a hospital bed.

In September, DQ had her fifth heart catheterization and the doctor ballooned and stented her right pulmonary artery where it had been pinched. It was believed at the time that the simple stent had reduced DQ's pulmonary pressures. We were told for the first time after that cath that DQ had moderate pulmonary vascular disease. Her pulmonary arteries are like a tree without leaves rather than a tree with leaves as they should be. We were told the stent, with the increased blood flow, should help those PAs grow along with lowering the pulmonary pressures.

A follow up echocardiogram confirmed the lower pressures. I was ecstatic at the numbers. I held on to those beautiful numbers, waiting for the miraculous turnaround. But all that I saw was DQ not doing well. She continued to do worse. I kept having to call the cardiologist because DQ was swollen. We kept having to up her diruectics when the numbers said there was no need for the medicines. Yet, I still let myself believe the positive.

In December 2009, DQ saw the ENT because she kept getting ear infections. The ENT wanted to place tubes, but needed cardiac clearance for the anesthesia. It was at that appointment that we learned DQ still had pulmonary hypertension (the previous echo had been wrong). It was at that point that I could no longer hide behind the positive.

DQ's cardiologist explained that DQ's PH was likely caused by the pulmonary vascular disease (hypoplastic distal pulmonary arteries) and, accordingly, could not be treated through any means currently known to science. She suggested we see DQ's pulmonologist, but left us with a grim reality.

The next week, DQ and I waited two hours to see the pulmonologist. When he finally came in, he listened to my story, and said he had nothing. He ordered DQ to be placed on oxygen at night to help her lungs get a break, but he gave me no other hope. When I asked him the prognosis, his response was "you already know how bad this is. I have nothing more to offer you." Needless to say, our holidays were not that great in 2009. We grieved a lot and spent a ton of time together as a family, loving our very sick little girl.

This picture was taken the day after meeting with the pulmonologist. Ma tried to help distract us with cookies! Or was she just trying to soak up as much of DQ as she could? I think it was both.

While the holidays had a very grim shadow, we did still have some fun.

When I wasn't spending time with DQ, I was making calls. I was searching the internet. I was posting requests. I did everything in my power to find every pediatric PH specialist in the country. I contacted them all. Almost all of them responded to me with a fairly uniform answer: "PH caused by pulmonary vascular disease cannot be treated."

By the end of January 2010, I was desperate. I left messages for DQ's cardiologist, but the messages never seemed to get through. So, I finally sent a fax to DQ's cardiologist with a huge list of questions. Within an hour, she called me back. An hour later, DQ had a cath set up for the next week. The local docs were no longer going to give up on my girl and I wasn't about to stop trying to save her.

At the beginning of February, she had her 6th heart catheterization. The docs learned that DQ's lungs responded to nitrogen, so they started her on revatio (a.k.a. sildenafil, a.k.a. viagra). But, she developed pneumonia and stayed hospitalized for 10 days. Upon discharge, we weren't given any promises and were told good luck.

DQ in the hospital after that February cath and pneumonia. Here, she is watching "Cars" or "Baby Signing Time", her favorite videos then and now.

Through the next several months, I continued to search for another answer, as we grew more and more tired from having to give Revatio three times a day, meaning staying up until 11:00pm every single night to give the final dose of the day. And yet, each time DQ went to the cardiologist, nothing would change.

Finally, in April or May, as I stood by DQ's hospital bed (respiratory virus), my cell phone began to ring. It was a PH specialist from California. He was the first doctor who did not dismiss DQ's case as hopeless. He offered to see her.We just had to get her healthy, keep her healthy, and find a time to travel.

We waited and waited and finally, DQ went to see the PH specialist the end of July 2010. She had a cardiac MRI and examination. After it was all said and done, the PH specialist stated that DQ's pressures were likely originating from her heart because of her valveless conduit and a pseudo-aneurism in her conduit (things we knew about for over a year). He also explained that it was typical for someone with DQ's mix of CHDs to start declining without real explanation or solution, but just not usually as quickly as she had. Usually, the decline starts in the teenage years. He suggested that DQ's best possibility was replacing the conduit with a valved one. He didn't think that would solve everything and may not work at all, but it was the only idea. He pushed to have the surgery completed within a month so hopefully DQ's damaged heart could start to heal.

Just arrived at our hotel after a long flight.

Enjoying an ocean view.

My sweet girl, chillin after a long day at the ocean, following two very long days at the hospital.

Upon our return, I spoke with DQ's primary cardiologist.  She didn't think surgery was going to help DQ either, but didn't think it would hurt. She had no other ideas and left it up to us, saying she would support either choice. We opted to talk with DQ's surgeon, but he was on vacation.  So, we spent August on pins and needles, not knowing what to do.  Should we put our child through another open heart surgery? If we do it, will the local surgeons agree with it? Should we have the local surgeons operate if they agree, but don't believe it is necessary? Should we have the surgery done in California, so far from home with doctors and nurses we don't know and don't know DQ? There were so many unknowns and one sick little girl.

Finally, the surgeon talked with DQ's primary cardiologist and indicated that he thought DQ should have surgery. He would do it and he would put DQ on his calendar, but first we had to come down to his office, that same cramped room with the same worn brown couch, for an examination.

And so, I entered that cramped office once again in late summer 2010, still not knowing whether the surgeon agreed to the surgery because we were going to do it anyway or if he believed it was the best option. I wasn't going to hand my baby over to anyone who didn't believe in what they were doing, even if I had trusted them over and over before. Fortunately, we left knowing that everyone was in agreement.

On Monday, September 20, 2010, I handed my sweet toddler over to the surgeons once again. She left me, drugged out on versed, thinking her pacifier was a riot around 7:40 am. Seriously, she was waving it in front of her face, giggling. Around 9:45am, we were told the surgery was starting. It takes a good two hours to get all of the lines in and anesthesia properly complete. It took over an hour to get through DQ's sternum because of all of the scar tissue she had from open her chest multiple times. She went on the heart-lung bypass machine around 12:30 pm and came off around 4:00pm. At 7:00pm, they came to get us so we could watch our girl on her way from the OR up to the PICU. I gave her one more kiss.

Playing in pre-op.

Giggling at her pacifier, doped up on tons of versed.

It took 11 hours in the OR to make our miracle happen. What wasn't evident from images produced by numerous echocardiograms, heart catherterizations, and an MRI was that DQ's tricuspid valve had a huge hole between two of the leaflets, her PFO remained open (small hole to upper chambers of the heart), and the stent that had been placed in the right pulmonary artery the year before, was blocking blood flow to the left lung. On that Monday, my favorite surgeon fixed all of those things and he replaced DQ's right ventricle to pulmonary artery conduit with a valved, porcine (pig) conduit.

DQ immediately post-op, September 2010.

My baby was able to come off of the ventilator the very next day! She was home by that Saturday. She was miraculously better! So much better than anyone had hoped.

A couple days post-op, looking ready to go home.

Exactly one week post-op, home and doing well.

Eleven months later, DQ's pulmonary pressures have decreased. They are still higher than normal, but no longer dangerous. She is still dependent upon a lot of medications that the docs wouldn't expect, but since they help her, we continue their use. DQ's last echo was a couple of weeks ago and her heart has continued to get smaller (a very good thing) and the leak in her tricuspid valve is almost non-existent. These are all signs that surgery was a success and my push to find answers so worth it. My baby's heart is the best it ever has been and will be.

The surgeon did explain that there is nothing he can do for her distal pulmonary arteries though. That he cannot make them branch and grow. And unlike most cases, DQ cannot have more stents inserted into her main pulmonary arteries to make them bigger so that the distal arteries can grow. Placing any stents will only cut off blood flow to the other parts of DQ's lungs because of their odd placement.

So, we hope with time and better health that DQ's pulmonary arteries will grow on their own. But we were warned that if they don't grow more than proportionately, we will be in the same place again, but without anything to fix. At some point, without extra growth, DQ's pulmonary arteries will no longer be able to sustain her and the pressures will increase again because her heart will start working harder to try to force blood to the lungs to get oxygenated better. And at some point, she will be desperately sick again. I'm holding out hope for new technologies and medications.

Practically speaking though, I am enjoying my time with my beautiful daughter, soaking her up. Trying not to let her precarious future take away from her love of life.

Dancing Queen in August 2011.

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The Dancing Queen's story from conception and my post from last year's Every Heart Has a Story can be found on the navigation bar at "Dancing Queen" and a fuller description of her heart defects is on the navigation bar at "I'm a Heart Mom". I also maintain a carepage for DQ that is just about her health and specifically her story. If you want to know more or want to follow DQ, send me an email at momonaline (at) gmail (dot) com.

Finally, if you haven't stopped by Stefenie's blog and read the other heart stories posted there, please do so. Or, if you are a CHDer or a family member of a CHDer, write up your heart story, and link-up with the rest of us.

Every Heart Has a Story--Relationships Take Two

I know that my posts tend to be on the introspective side and dark because this is my outlet.  However, I love my life and the people in it. I have been remiss since the day I posted my Every Heart Has a Story--Relationships.  I posted the day after the Dancing Queen was hospitalized, the day after we almost lost her again. I had not seen her since I left to pick TRex from school and when I left, she was doing extraordinarily poorly. And even though I had been writing my Relationships post for a couple of weeks, I finished it on that dreary morning.  As a result, it ended up with more of the negative, than the positive.  When I read the posts from other heart moms, I realized I forgot to write about the fabulous changes that occurred as a result of DQ's congenital heart defects. I am rectifying that oversight today.

One of the most wonderful things that has happened to me as a result of CHD and pulmonary hypertension is that I don't take my relationships for granted. I try to let the people I love know that I love them. There is no guaranty of a tomorrow for any of us. I know that now. It allows me to appreciate the ones I love for who they are. My core relationships are stronger as a result.

CHD has also deepened my belief in the goodness of people in general. I am an optimist at heart. I try to see the good as much as possible. I think we should all look out for each other, take care of each other. Unfortunately, with the political discussions that have been taking place in this country over the last several years, I didn't feel like society at large really cares to help each other.  Everybody was out for themselves.  But, because of DQ's CHDs, I've learned that perfect strangers care. They don't want to see my baby suffer. They will come together to help a little girl they have never met simply because she is sick. They will help our family because they care so much.

DQ's carepage has had close to 1,000 unique visitors and many who regularly read it. I know that the posts are also emailed from several people and forwarded on from them as well (I get emails from people well outside my circle that are a forward of a forward of a forward of a post).  Other people have added DQ to their webpages for children to pray for and she has been on countless prayer lists across the globe.  While I do not believe in a higher power, my heart is filled with gratitude and warmth for the care and love shown to my girl. All of these acts make me realize people do care and have made me appreciate everyone around me more.

The most humbling act of caring toward my family happened last year. It reaffirmed my faith in humanity more than anything. When we finally found a doctor to give us a second opinion regarding DQ's pulmonary hypertension and we had to fly across the country, my family jumped into action (and wouldn't let me say no) and threw a benefit for DQ. They raised a lot of money that allowed us to travel without the added worry of cost and to stay home with DQ as she prepared for surgery. We were beyond grateful for that help.  But more importantly, I felt we were part of a community who was working to give DQ a better life or at least a chance at more life. We had strangers from off of the street see the banner with DQ's picture, stop by and donate. We had people read the fliers and just want to meet the amazing girl who had been through so much. My entire family came together with their friends to work on this amazing event for my daughter. They laughed and they planned, they accepted my co-workers and DQ's therapists into their embrace. Everyone rallied around my girl. Everyone wore shirts with her name. Seeing so much love in one place for my family reaffirmed my belief that people are good at heart.

Without DQ's CHDs, I would never have started the carepage, I would not have needed the support of the community at large, and I am sure I would be much more cynical and angry with society.  Instead, I have hope.

Every Heart Has a Story--Relationships

I am participating in a blogging event for Congenital Heart Defect Awareness Week.  The task was to write about how the Dancing Queen's CHDs have changed my relationships, then link the blog post to When Life Hands You a Broken Heart so that all of the heart families can share and learn.

I have been thinking about this post for weeks now.  I even suggested the idea to Stefenie.  When I suggested it, I was certain what I was going to write about how I lost many friends, felt lonelier than ever, but my husband and I have grown together more.  This is all true and I thought I knew the reason too.  I thought my relationships faltered because people couldn't deal with my new life; couldn't handle the thought of a baby going through such agony and being around me made it too real.  Perhaps some of that rationale is true, but it is so much more complicated than I originally thought.

Since the Dancing Queen was born, I do not have the relationships I once had.  I don't speak with friends on the phone as frequently and instead of regularly speaking with three or four good friends on the phone (they all live in different states), I now only regularly speak to one friend. From early on, I felt like my other friends were abandoning me because of these less frequent calls and eventually never hearing from them.  It hurt a lot.  I was more alone than I had ever been.  I was hurting and the people who had said they would be there for me in the middle of the night if necessary weren't there.  I started a carepage for my daughter so that people could know how she is doing.  I know exactly who looks at it or at least opens the page.  And I know who doesn't.  A couple of people who I thought were my very best friends never read the posts and never call.  That hurt me the most. Neither was there for me when I needed it most.  With one of the friends, she actually came to TRex's 2nd birthday party and lied to be about reading the posts to make it seem like she cared.  The lying hurt even more.  I felt like she was rejecting me, adding insult to injury.

However, in writing this post, I learned that she wasn't rejecting me and neither was my other friend.  Neither of them had changed.  I had.  Before the Dancing Queen, I would call them.  Before the Dancing Queen, I kept up the relationships.  They only called me if I had left a message.  Nothing changed on their ends.  I just didn't have the time nor energy to be the only one making the effort, so the relationships fizzled. Having a child with a CHD is all consuming.  There are lots of doctors' appointments, therapy sessions, lab tests, and lots of time in the hospital.  There are surgeries, catheterizations, x-rays, pharmacy visits.  All of those things take time.  I simply didn't have the time to maintain friendships that were not reciprocating.  I couldn't be the emotional support any more.

It is nice to know this now, but three years ago, when I was all alone, it didn't help.  I was alone.  Nobody could talk about it.  Nobody would talk about it.  As a woman, I wanted to talk about my feelings.  I didn't have that with anyone.  My husband was in deep denial at the time.  My family wouldn't talk about it.  It just wasn't a topic of conversation.  Over time, I have forced the issue more for my sanity, but I still get cut off when I want to discuss my feelings many times with friends and family.  That is the part of girlfriend relationships that I miss.  (A caveat here is that I found wonderful heart mom friends who provide a forum for me to release these feelings.  They understand and I feel that they care as I care for all of them.)

On a positive note though, my husband now allows me to get out my feelings, no matter how much it hurts him.  He has moved beyond his denial.  I feel closer to him for it.  He tells me how he is feeling, at least in a rudimentary fashion.  We share this experience fully with each other.  By fully, I mean, we share the doctors' appointments, the therapy sessions, sitting in the hospital room, crying at the down times, rejoicing in the small miracles.  The one thing I love about this sharing though is that we generally don't fall down at the same time.  That allows him to hold me up when I most need it and I can hold him up when he most needs it.  The Mad Scientist is my rock, my partner, my best friend, and I am eternally grateful that he wouldn't let me run away.

All of that being said, I tried to think of how else my relationship with the Mad Scientist has changed, but I can't really remember.  It seems that we have been on this roller coaster for an eternity.  But when I think really hard, I would say that we don't get to spend nearly enough time together any longer.  Between both of us working and all of the Dancing Queen's appointments and keeping TRex on the right track, the Mad Scientist and I basically fall asleep on the couch each night rather than playing games together like we used to.  We try to take dates when we are able.  We have had a couple of weekends away from the kids.  We constantly talk about that trip to an all-inclusive beach resort we hope to take.  But, just getting a good strong hug after rough days is what I really need right now and he always has it.  Well, that and talking.  We talk.  We talk a lot about what we are feeling, our fears, our hopes, our plans.  That makes us closer.

Normally, I would do this sort of post on the carepage that I maintain for the Dancing Queen.  Mainly started to keep family and friends informed about our progress when I was 8 months pregnant, the carepage continues to be about the Dancing Queen.  I started this blog because there were things I wanted to say that were not appropriate in journaling the Dancing Queen's ups and downs (I really want her to be able to read it and not feel bad about her health's affect on our family because I love my baby and all that she has brought, even the bad because it comes with such GOOD). Since this blogging event was about my relationships and how my life changed, I posted it here.  If you found us from the blogging event, you may know us already from carepages, but I am protecting the innocent on the open blog by not using real names and faces.  If you found us from the blogging event and want to follow the Dancing Queen's story, send me an email at MomOnALine (at) gmail (dot) com.  If you didn't find us from the blogging event, you should check it out at When Life Hands You a Broken Heart.