Tuesday, January 15, 2013

The Water is Chasing Me!

Look at me, posting twice in the same week!  I wanted to be sure I saved this story for posterity, it is that funny.

C was running around sans diaper a few days ago.  From across the room I heard a panicked shriek.  "The Water", he cried "The Water".  I looked over and he was peeing.  He started backing up and then he whimpered "The water is chasing me" as he frantically backed away from the stream of urine he was generating.  He looked so forlorn and confused, it was absolutely adorable. I took this to be a sign that we are NOT ready to potty train yet.

Monday, January 14, 2013

2012 – A Year In Review

The dawn of 2012 (or February as we'll call it in this post) saw us heading toward the tail end of another cold and flu season. Since our life continued to revolve around C's many medical trials and tribulations, the cold and flu season continued to be particularly troublesome. Toward the end of February as my husband and I approached our first 100% anniversary, C came down with a Very. Bad. Bug. Even though we were on Synagis for a second year, he had RSV. We were at the tail end of the effectiveness of the shot for the month when he got it. As soon as we got next month's dose, he got a whole lot better. All in all, not a lot of fun. It was the start of lingering respiratory issues that would plague us for most of the year.

The end of February also saw TheMan and I reach our first 100% anniversary. TheMan is only required to do something for our anniversary when we hit 100%, so it was a big year for him. He made us reservations at the melting pot. The Saturday night of our celebration rolled around and C was sent off to visit Mimi and Papa. TheMan and I dressed up and had a lovely 3 hour long dinner at the melting pot. It was a lot of fun.

After we had our bout of RSV, life became a series of illnesses and ER visits. I began to fear we were facing asthma issues. Nothing seemed to control the flare ups. Anytime C experienced even a mild fever (99.3 degrees) he'd start retracting. He was wheezy with any illness and all in all, anytime his system took a hit, he had no reserves. C continued to gain and lose the same pound or two. Even though he grew in height much of the year, he didn't gain weight and in the end, tumbled completely off the weight chart. By the end of April, we had a rescue inhaler and had been on steroids multiple times. I think the final straw was the last weekend in April. My MIL called me at 1:30 in the morning. As a respiratory therapist, she simply wasn't comfortable keeping C any longer. She brought him home and when I heard how he was breathing (stridor and wheezing) I knew we needed to take him in. We ended up in the ER under observation until 9 in the morning. Finally, after hours of breathing treatments, we were allowed to go home. It was a close call between admission and going home, but we were pretty comfortable assessing his breathing, so in the end, they let us go.

After months of soul searching and second guessing, I decided I had to do something different. I gathered our medical information together in early May and sought a second opinion from Children's Hospital Boston (CHB). CHB is the number one children's hospital in the country and the top hospital when it comes to pediatric cardiac care. Researching the issue of mitral valve regurgitation lead me to their congenital valve program, the only program of its kind in the country. The surgeon leading the efforts of that program is Dr. Chris Baird. For months, our local cardiologist had been counseling patience. He wanted us to wait until C was larger and have the valve replaced. He was convinced that repair was not a viable option and that an eventual repair was a foregone conclusion.

Just days after I delivered our medical records to Dr. Baird's assistant at BCH, I received a call back from Dr. Baird himself. It was a Friday night about 7pm, which is 8pm in Boston. He called to tell me he had reviewed our records along with Dr. Gerald Marx, our newly assigned cardiologist and from what he saw, he thought that a repair was our best course of action. He advocated for the repair to occur very soon. He explained that he could see an open cleft in C's mitral valve, and that closing that valve would almost certainly resolve our weight gain issues, lung issues and most likely ensure a permanent solution to C's problems. The odds were heavily in our favor that he'd never need surgery again.

I was shocked. I honestly expected to be told we should do a repair a few years down the road. I had believed our cardiologist's theory that the weight gain, repeated illnesses etc were unrelated to the “mild to moderate” mitral valve regurgitation we were told C had. The surgeon and cardiologist at CHB had a markedly different opinion. They felt the issues C was experiencing were most certainly related and absolutely should be addressed quickly. Unlike our local cardiologist they did not see a benefit in allowing C's heart to enlarge as they didn't want to place a prosthetic valve. They wanted to repair the valve he had, which was growing larger, less elastic and more fatigued with every passing month.

Our local cardiologist urged restraint. He counseled me that we could still end up with a valve replacement and if we did, we'd have to do it again in just a few years. He stressed how much of a gamble he felt this was and how much he felt our medical issues were unrelated to C's cardiac status. I began researching mitral valve replacement and Dr. Baird. Researching him and the congenital valve program at CHB lead me to a paper entitled “Mitral valve operations at a high-volume pediatric heart center: Evolving techniques and improved survival with mitral valve repair versus replacement”. At this point, our occupational therapist (OT), pediatrician (ped) and gastroenterologist (GI) all felt that the cardiac issues C continued to experience were negatively impacting his health. In the end, I felt that the opinions between CHB and our local hospital were so different that we had to seek an impartial third party.

Between January and May, C had gained .25 to .5 lbs. You could clearly see his ribs and count all the nobs of his spine. He was eating as much as he could, and he was starving. In June of 2012, we undertook a 3 day nutrition challenge where we recorded every calorie C ate. He'd been on an appetite stimulant called Periactin for almost 3 months at that point and though his appetite was much improved, we still seemed to yo-yo wildly and not gain ground. The nutritionists calculated the calories C ate over that 3 day period and found he was eating 120% or more of his caloric needs and still wasn't gaining weight.

I contacted Children's Hospital of Philadelphia (CHOP) in June following the nutrition challened. CHOP is the second ranked pediatric cardiology program in the country. Our local cardiologist had cautioned that BCH was an aggressive facility that treated sooner rather than later. He explained CHOP could go either way, but was somewhat less aggressive. I spoke with their second opinion coordinator and explained that I needed an impartial third party that would tell me what course of action they'd advocate with the understanding that we wouldn't be following any course at that hospital, but rather be choosing between 2 courses of action at other hospitals.

CHOP created a wonderfully diplomatic and amazingly informative letter that taught me more about my son's current condition than any information I'd received in the year preceding that. They corrected a few errors in the report information provided by our local heart program. Their explanations highlighted the misunderstandings our local program had regarding my son's condition. They also confirmed the opinion of BCH, our OT, GI and Ped, the tremendous issues we were having with respiratory health, weight gain and growth were a direct result of the severe mitral valve regurgitation that C was experiencing. Both BCH and CHOP suspected that C was also experiencing some degree of Pulmonary Hypertension due to the 2 jets of back-flow shooting across his atrium with every beat of his heart.

Given my new information and the fact that CHOP firmly agreed with BCH that we should repair that valve. I once again contacted Dr. Baird's personal assistant in early August. I sent the latest echo which I hoped was clearer as it was performed by our favorite sonographer. Both BCH and CHOP had commented on the quality of the previous echo and the problems they had viewing the study and by not having all the views needed.

Dr. Baird called me back 2 days after he received the latest echo. He again called late in the evening and he spoke with me at length. He stressed the fact that he truly felt C's current valve was repairable. He explained how he'd handle the repair and his thoughts that it would be the last open heart surgery (OHS) we'd ever need. TheMan and I talked it over and faced a circumstance that we'd thought would occur in the distant future. Originally, when we were told of C's regurgitation immediately following his first surgery, the surgeon said he could last a “long long time” with that level of regurgitation. At the subsequent follow up 3 months later, our cardiologist told us it would be well into his teen years before we needed to address it. 3 months after that, he said it would be the early teens. 6 months after that, he said he thought he'd get to his teen years and the final appointment before we decided to go to Boston, our cardiologist said he hoped we'd get 3 more years. Deciding it would be this year instead of 3 years from now was actually still pretty wrenching, but the chance of having this be the last surgery and finally being able to put some weight on C lead us down that path.

C's second birthday was August 9th. On August 10th, BCH called and we scheduled his open heart surgery for September 26, 2012. The week that followed I was a whirlwind of activity as I gathered documentation for the insurance company, secured lodging through patient and family services at BCH and transportation through the Angel Flight network. Once that was all handled, I was stuck with waiting and worrying. Once we decided to have OHS, I did contact my insurance company and asked for a dedicated coordinator. Calling Customer service did not work, as they didn't know what I was talking about. My benefits coordinators through my work were able to help me and a complex case manager was assigned. She was fantastic and helped me navigate the insurance maze with ease. She complimented me on my knowledge of my son's defect and told me I had educated her on the condition more than she ever could me. In fair trade for that, she certainly educated me on the ins and outs of insurance.

Coventry is an amazing and wonderful insurance company who surely helped my son receive the appropriate medical treatment using their national network. I provided documentation from my cardiologist, pediatrician, the third party opinion from CHOP and a personal letter explaining why I felt the appropriate level of care wasn't available locally. Coventry took that information seriously and sought to help me meet my son's needs. After the many horror stories we've seen on the news, I'm incredibly pleased to say my experience has been anything but a nightmare. All of my claims and issues have been handled calmly and easily. I can't thank the employees of Coventry enough for their tireless work.

C caught yet another upper respiratory infection the first week in September. He again started wheezing and his respiratory rate was elevated. We went to urgent care (because they won't keep us) and they gave another nebulizer (nebs) treatment. More than anything, I wanted to ensure he didn't get a lingering ear infection and didn't have pneumonia. His respiratory rate was 58, 2 short of what it takes to get sent to the ER... so they let us go home. A quick check of his ears did confirm yet another ear infection(we get them with every head cold). We got a prescription for antibiotics and we were on our way home. It was 3 days before the beginning of the 3 week window in which any illness can cause surgery to be rescheduled.

I pulled C out of daycare and I started working from home on September 10th. I became a germaphobe for the second time in my life. I invested in hand sanitizer and bought 10 (yes 10) buckets of sanitizing wipes. I wore masks when I left the house and didn't let anyone come within 10 feet of C unless I knew exactly who they'd been around in the previous days. I'm sure I was neurotic and my husband had the patience of a saint to put up with it. I even called the Yawkey Family Inn (which is like the Ronald McDonald house) and asked if they had any sick people there. I was beside myself with anxiety and looking for anything I could control.

Toward the end of the antibiotics for C's ear infection, I noticed what might be thrush in C's mouth. I semi-franticallyy called the nurse line and asked if I could start nystatin. She called a prescription in for me and we determinedly worked to ensure C would not have thrush when we headed to Boston.

Four days before we were to leave for Boston, the pilot who was going to fly us there called with bad news. Some part on his plane had failed and wouldn't be fixed for 3 weeks. He also had a cold. There was no way he could get us to Boston. In the 4 days leading up to our leaving, I researched and learned everything I could about flying with a toddler and with a sick child. It wasn't my original plan, but it ended up working OK. Delta Airlines and the TSA in Kansas City were fantastic. I'd called ahead and informed them we'd be there and that I was attempting to ensure he didn't get exposed to any germs on the way to Boston.

TheMan and I had a slick system for traveling. He'd pre-board with the special needs boarding. He'd wipe down all the hard plastic surfaces with clorox wipes and install the car seat. Meanwhile, I'd wait until absolutely everyone had boarded and then I'd bring C on last and get him settled. C was “All Done” from the moment we sat him in the car seat. He proclaimed this fact loudly and repeatedly for the first 10 minutes we were in the air. Luckily things settled down for him after that and it was a smooth and quiet flight.

The day after we arrived in Boston, we spent an exhausting and exhaustive day at preop. C was checked for everything under the sun and signed up for several different studies going on related to OHS and congenital heart defects. We tried to get as much fresh air as we could and spend time enjoying the world without exposing C to anything unnecessarily. On Tuesday, my mom and sister arrived and provided amazing emotional support. Surgery was bumped and ended up being September 27, 2012. It was as breathtakingly terrifying as the first one. We got through it, the surgeon, doctors and staff at BCH work tirelessly and are absolutely the best in the business. They amazed me with their skill and professionalism. I cannot say enough good things about our stay there, nor can I express how happy we were to leave. Much love to all, but seriously, hospital life sucks.

When we were discharged from the hospital we were left with two major issues, C's sinus node was not functioning correctly after the surgery which led to bradycardia and a persistent junctional rhythym. Electrophysiology (EP) is the department that handles the electrical system within the heart. At our consultation with them, they said not to worry about his heart rate and to give it is at least 4 months before even thinking about interventions such as a pacemaker (cue immediate and persistent worry about a pacemaker now). C's heart rate would dip into the 30's during the night and routinely was lower than 60 during the day. I learned something I never knew. If any person's heart rate is below 60, that is one sign to start asssessing that person to see if they need CPR. This is a very disconcerting to be in when you leave all those lovely and reassuring monitors behind.

My fondest memories of Boston are of C playing in the green courtyard of Harvard's Medical School. The days were beautiful and he had so much fun. I couldn't believe my child was running full speed through the yard seven days after he had had his heart stopped, reconfigured and restarted. We headed back home from Boston the following Tuesday after a followup with Dr. Marx. Who, along with Dr. Baird, continues to be one of my favorite people in the world. 

After returning home, we kept C out of school for 5.5 weeks until just before the expiration of the restriction on lifting him under his arms. The first 4 of those weeks he remained in isolation to ensure he had plenty of time to heal before we risked an upper respiratory infection. It was surprisingly hard to let that go and start living again. But it became liberating in its own way. I was so thrilled to be able to take C to the zoo. Before surgery, I was so scared that he'd never get to see it.

In October, C went to wonderscope with his Mimi, on adventures with his Grandma and went to the zoo twice. Once with just Mom and Dad and once with Katie and Jake for Boo at the Zoo! Much fun was had on both occasions.

Thanksgiving was at our house this year. We had a huge turnout with 22 people spending the afternoon with us. It was a lovely day and we were so happy to have our family with us. That Saturday we celebrated my nephew's birthday, which was a quieter affair, but no less enjoyable.

Over the 7 weeks since we let C out of his bubble post OHS he's almost consistently had an upper respiratory infection along with a sinus infection, multiple ear aches and 2 stomach flus. Just before the end of the year we received word that we do indeed need to get ear tubes and I'm working to get us into the ENT.  With all that, we've had no major respiratory flare ups and C's maintained his appetite and continued to gain weight.  His sick is a lot like his healthy used to look.   I continued to breast feed C 3-6 times a day through the end of the year.  I can only imagine this has helped, I can't picture how often he'd be sick without it.

C had a lovely “Word World” Christmas this year. He doesn't really understand Christmas, or gifts, but he loves having time with mom and dad and playing, so he counted it as a win. We end the year with a academically advanced kiddo who could care less about the other skills he should be growing. He's on the verge of reading, he LOVES to spell words, can count as high as he feels like, but doesn't jump and rarely throws stuff. He has no interest in cars or other “age appropriate” activities. But he can spell his classmates names, knows the days of the week and the months of the year. A bit lop sided, but we're working on it.

On a work front, the husband and I are both happily working to keep modern in a rapidly changing programming landscape. We enjoy it.