Overdue Update...

It's hard to believe that nearly 2 years have gone by since my last post here. My sincerest apologies. We've been enjoying life, which is something I couldn't have even imagined being able to do at this time 4 years ago. The truth is, Rosemary has defied the odds and is living as full of a life as any other child her age.

Having HLHS does not define her, it has become her bridge to other people, and quite frankly from theirs to mine. Her life has inspired her family, friends, teachers and community to reach out to those suffering from heart disease and congenital heart defects. Last year, she was chosen as her school's "Heart Hero" for their Jump For Heart campaign through the American Heart Association. The pictures below are from 2013. Her school has been the #1 fundraiser for this event in our county, and one of the top 100 schools in the state of Maryland. Her school, alone, raised over $20,000 last year, something she was very proud of. Bringing awareness and raising funds for pediatric heart research is one of our goals as a family and this is part of our motivation to utilize Rosie's life to benefit others.

Rosemary and her PE teacher, Letty McNulty

Rosemary sits quietly as her PE teachers educate her classmates about her heart condition and that she is one of the many reasons the AHA raises money and awareness to promote healthier hearts.

Rosemary has been doing so well since her last surgery, so we decided to skip her annual heart catheterization last year. This decision was made partly because she wasn't showing any new signs of cardiac deterioration, and currently, the only thing left to do for her is to attempt the Fontan again or talk transplant. Her cardiologist and I were in agreement that because she was doing so well, we would revisit that discussion in a year. That year has passed, and at her 6 month follow up appointment last Wednesday, it was apparent that we needed to investigate exactly where she stands.

She had the usual routine, vitals, ECG, and an echo. During her ECG, I noticed that her heart rate was in the 40's and she usually sits in the mid 70's. I made a mental note and decided not to immediately worry but asked the nurse to let her doctor know. During the echo, I saw the same. Her heart rate fluctuated from the 40's back to the 70's. So her doc decided to put her on a halter monitor for 24 hours and get some labs done. He also mentioned that it would probably be a good idea to get her cathed again and once the results were back from all of the tests, to sit down with her surgeon, the cardiologist and Drew and I to discuss how to move forward.

......How to move forward.....

The realities of what this means for our family is something that I have been ignoring for the last 3 years while Rosie has been stable. I have distracted myself through nursing school, an accomplishment that is directly attributed to Rosemary's life. Having seen the amazing care that she received that kept her alive  was the internal catalyst that has led to a double inspiration to become an RN. It has been my goal since Rosie got out of the hospital. I knew it was something I could do and I wanted to do for her. 

Our family has regained a new sense of normal since March 2010. My other two children have been involved in school and sports and it's been wonderful to attend school events with Rosie in tow. We haven't had to do to much adjusting in our lives at all really, it's been smooth sailing. Auto pilot, if you will. It's been so nice watching her ride her bike up and down the street, each year riding a little further down the hill and she always makes it back up again.

As heart parents, we can't control much of anything with regards to our kids' hearts, they tell us what they need and when they need it. Aside from that, we're at the mercy of a seemingly nebulous free fall that will catapult us into any direction at any time, leaving us to constantly worry that the first sign of any illness or distress is a signal that there is something wrong with their heart. 

I choose to process her inevitabilities by intellectualizing the information that I can make sense of through my studies. Her numbers, her symptoms, symptom management. I mostly keep myself in a safe bubble of denial, which has worked for the most part. It's like wearing a pair of those horse blinders, I only see what's in front of me. It works until I hear of one of our own HLHS'ers suffering through the same struggles that Rosie has faced, and many times much more. It reminds me, daily, of how blessed I am to look into her eyes, to see her smile, run, play, laugh, smack her brother and sister, scream in anger...to see her live. 

Her continued quality of life is at the forefront of my desires for her. And I understand that even my best intentions will not be able to provide that for her forever. But while I can, I will. I have today. WE have today. 

God has a perfect way of revealing His timing. I just received an incredibly thorough report from all of Rosemary's specialists and teachers at her school. From Psych evals to every ED eval under the sun, she's gotten it and it will be reviewed on Tuesday of next week during her IEP meeting, two days before her catheterization. Looking at all of the information we will have, the absolute, most comprehensive information on how she is doing physically, mentally, socially and emotionally...we will have it all. We have time on our side right now as well, which makes going into a meeting with her surgeon feel...as prepared as possible. He's given us 4 extra years we didn't think we'd have. We're here, she's strong, and she's still fighting. In fact, I feel like she is the best she has ever been.

I expect that because she is bigger and stronger, that she will take to the Fontan again. I hope that her little lungs can handle the pressures. This was the surgery that caused her chronic pleural effusions and a 6 month hospital stay that nearly ended her life, again and again.

Her cardiologist mentioned the name of the doc who saw Rosie in January when she visited the ED at Children's, she is also the head of the new transplant department. I swallowed that with a side of denial, hoping that we won't have to visit that reality just yet. It is seemingly inevitable, but I want to give HER heart one more chance to keep her going. Knowing that someone else's child will have to pass for mine to live is an everyday reality that I cannot escape. It's not just that thought that makes me cringe at the idea. It's the reality that she will again have to succumb to such a deteriorating condition and endure such extreme pain and suffering in order to be pushed higher on the transplant list. It's a balancing act of keeping her here and keeping her comfortable.

We very well may get the report that everything is virtually the same and that we can continue to wait as we've been until something changes. I don't know. This is the part of being a CHD parent that I've gotten rather good at. Ninety percent of the time, I don't think about the inevitabilities because they're not urgent right now. I don't waste time and energy on things that I can't do anything about. Instead, I let my everyday family life blissfully consume me, I continue to forget about it while I can. 

We waste a lot of energy on the things we cannot change, even the struggles of yesterday. Doing that takes away from the energy we need to ENJOY today. Try to be a ray of sunshine for your loved ones, sick or not. 

I will update with more information when we have it. Until then, smile, laugh, love and make someone else's day better, because you can.

This was a long one. Thanks for reading.

Where We Are With Rosie

My day started knowing that at 4pm this afternoon, the cardiology case conference would be discussing Rosemary's failing heart. This has been the most difficult week of my life. Rosemary's little heart is not working so great, she is experiencing Fontan failure, this was the supposed to be her last repair. She has been on TPN nutrition for about 6 weeks, food through an IV, to rule out her pleural effusion problem stemming from a GI issue. She has had a GJ tube placed and replaced for feeds 3 times in one month. When I got here last Monday, her face was puffy, chest & abdomen full of dark blue veins and unable to speak or be comfortable. They restarted her formula feeds on Wednesday afternoon with hopes on sending us to a step down facility in Baltimore. By Friday morning, she was in respiratory distress again and her pleural effusion had returned. They gave her a total of 5 doses of IV lasix Thursday and her xrays that night were better. But Friday morning, she was working to breath again and she just wasn't doing well. Her xrays looked bad so they gave her another 5 doses total of IV lasix to combat the effusions that had again returned.

They moved us to a flexroom in the Heart & Kidney Unit, which has the ability to be a CICU room if necessary. There are basically two options: 1) Another open heart surgery to try to repair the narrowing of her atrial septum, which they felt was the root of her effusions. The pressure gradient across the septum and Fontan have been slowy increasing and not going in the direction we had hoped. This surgery has a 50% chance of survival with the possibility that she would need a pacemaker, and at best, that surgery may only have a 10% chance of improving the overall condition of her heart, 2) Heart Transplantation.

The surgeon is not crazy about the first option, he is thinking that transplantation would be Rosie's best chance. Her cardologist agreed. Because they would be sending us to NY Presbyterian where she had her first 2 open heart surgeries, her Cardiologist there would oversee the transplant evaluation. She was not excited about the transplant option for Rosie.

That's where we stand. It's been difficult coming to terms with these options as they are the only ones we have. Each day is a little harder, facing the possible outcome of losing her either way...well, how do I begin to accept that one. I'm dealing. Accepting. Praying. Knowing that God has complete control and whatever his will, will be done. She was born with half of a heart. Twenty-five years ago, she wouldn't have mae it 3 weeks. We intervened medically to try to repair it. She has done everything that she is supposed to do, and she is still suffering. If she stays, I get to be her Mom. If she doesn't, I'll have to wait to see her when I get Home.

Ultimately, my main concern is her quality of life. It has been slowly but steadiy slipping out of our grasp. She is working so hard just to breathe. I could feel this moment coming around the corner last week, all of the doctors and nurses became much more attentive to Rosie and my needs. Their faces were undeniably readable. Sorry smiles, asking if they could do anything for us. This has increasingly become the norm for me as I walk through the hallways that have been my home for the last 5 1/2 months.

During rounds this morning, I told the doctors again that I am a reality based Mom. I like to know the facts. I want to know exactly what they are thinking and feeling in their gut in the 3-5 seconds when they leave my door until they get to the next. I need closure on the facts so that I can begin to deal with the emotional overhaul that deciding on whether or not to try for a transplant is going to be the absolute best decision for Rosie's quality of life. There are a few other major elements that are playing into this picture for us. Her lungs have not been doing so great with these effusions. She now has a pneomothorax, air pocket, developing in her left lung and she still has an unknown pocket of fluid or substance in between her two right lobes. Her liver has been put to the test because of the Fontan, but the 6 weeks of TPN nutrition has also now taken its toll. Her liver is swollen and has dropped. She has never really been able to eat normally and has thrown up just about every day of her life. All of these things will weigh in on whether or not they will even consider her for a transplant.

If we decide to go transplant, we will be sent back to NY to Children's Hospital in Manhattan. We will have to make sure that we are within a 3 hour distance from the hospital so that in the event that a heart became available, we could be there in a minute.

We have a lot of extremely difficult life changing choices to consider. I am scared out of my mind to make any decision. I selfishly want my daughter to live because I love her and I want her to be well. But as I sit here typing, I can hear her breathing getting louder and faster, she is working harder everyday just to maintain vital signs. It's not what I want for her. I don't want her to struggle anymore. I want her to be the strong, full of life child I knew a year ago.

So I will pray tonight that wisdom in this situation be evident, that my gut feeling be my guide. That whatever is down this pike for Rosie, be the absolute best decision possible for her quality of life. Please pray too, for Rosie's strength to fight, our strength to continue to be a light for her and keep her spirits as best as they can be. For our families. I have two other children that love her. All of our hearts are mending at the mending that's been done to her's.

Thank you for reading. If you have any thoughts, advice, words of encouragement, I'm all ears. This is difficult. I am finding peace and small answers in the quiet of the night.

One Week

It's Monday morning, October...26th. I had to double check on the computer because I have no idea what the days and dates are when I'm here in the hospital. Rosie had her 3rd open heart surgery on October 2nd. Since then, it has been a constant roller coaster for her. This last week has been eventful. After a series of days of Rosemary's oxygen stauration levels dropping and daily x-rays not really showing anything, the last of the x-rays revealed a developing pleural efussion, or fluid around the lungs. So last Monday they decided to place what are called, "Pigtails" in the right side of her chest. These drainage tubes can last anywhere from a few days to a few weeks or even months. On Tuesday, her morning x-ray showed a developing pleural effusion on her left side, so they placed a pigtail in her left chest, as well. On Wednesday, I was so sick and tired of the pokes and prodding that I asked for a PIC line (central line) to be placed into her chest through a vein in her left arm to forego anymore pain associated with blood draws and IVs. This would enable them to draw blood and administer IV meds and fluids as needed through one location. She had gone through 3 IVs in 5 days over the weekend, so she was done, and so was I.

These pigtails can sometimes become clogged or kinked, so they often need to be flushed or adjusted. Thursday evening revealed a kink in her right chest tube and a twist in her left, so the cardiovascular surgeon fellow came in to unkink the lines and eventually pull off 230cc from her right lung, and 170cc off of her left. Friday, more of the same. By Saturday, her pain was becoming hard to manage so they put her on scheduled doses of morphine, on top of the oxicodone and torodol she was getting. And last night, Sunday night, the nurses noticed the tubes were not draining as they should be. As they attempted to drain and pull off more fluid, the right chest tube broke away from the drainage port and the entire tube had to be pulled out of her chest. Doctors said that this happens all of the time, all over the world, it's just the nature of chest tubes. I'll be writing to the manufacturer later...once I've got nothing better to do.

So today, we are re-evaluating the need for another chest tube and the placement of a PCA, Patient Controlled Anesthesia. Rosie has seen so much pain medication over the last 2 years of her life that the normal doses of drugs do little to nothing to help her with the pain. Hence, the need for a little more than normal on the side of narcotics. I'll be meeting with the Pain Management Team today.

All of this to say, it has been one heck of a week. Just one of the three since we've been here. I can tell you this, I am the luckiest Mother alive. To have my baby girl being taken care of in a state of the art facility, to have my sister Leslie's family watching over my other two children, to have a loving partner who supports me, to feel the love all around me from two families that never stop believing in Rosie and in us...I am very lucky.

My cousin Kristen works in the ER downstairs. This morning, she brought me a load of gifts from my family who had gathered at my Aunt Jackie's house to celebrate a Steeler Sunday. The gift bags were hoisted onto an antibacterialized gurny, it looked ridiculously silly, I got a good laugh. It felt like Christmas morning. My sister, my cousins, my Aunts and Uncles, collaborated a host of Halloween gifts, costumes for Rosie, inspirational books, clean clothes, a beautifully hand made card from my Shea-signed by all-and food and more love than you can imagine, all put together so that I could feel a little bit of home while I'm here. After she left, I sat there and cried.

All of this insanity that comes with being here, I know it will end. One day, maybe in one more week, I'll remember this time and not feel anything but relief and the same love that I feel right now. Thank you, family. Thank you for bringing me home again.

Whatever it is that makes you feel at home...harness it. It's what you got!
Here's a musical place that takes me home too...(click on "Home")...
http://www.veltzmusic.com/Home_Page.php