Home with Caveats

We are home.

This morning they released us from the hospital and a couple hours ago the whole family reunited. It was heavenly sweet to finally all be together and free from the constant beeping and tethering of the monitors. My eyes have still not adjusted to the brightness of life outside the hospital.

Miles is a miracle baby. Countless doctors have told me equally as many times that there will be papers written about him. (How do you respond to that? Oh..? Smile and nod...) From the moment he was born they expected to intervene - surgery seemed imminent, and unless his heart showed drastic improvement we would not be able to go home. But one by one they removed various wires and tubes and though his heart hadn't improved he was still doing great. They couldn't justify doing a risky surgery when he wasn't showing any symptoms of cardiac stress and knowing that over time the tumor should shrink and he should grow. So they've started him on a chemotherapy drug that has proven in a handful of cases to reduce cardiac rhabdomyomas with the hope that the drug will accelerate the reduction of the tumors and Miles will keep trucking along proving to everyone just how strong and miraculous he is. He will be seen twice a week by doctors with echograms once per week and the cardiologist let me know not so reassuringly that they would be sitting on pins and needles between appointments until they started seeing improvement.

The drug he is on is an immunosuppressant and so the greatest risk to Miles right now is even the smallest of infections. If he were to get a cold we'd be right back at the hospital. The doctors have given us strict orders to put in place at our home to help keep Miles safe.

1. When you come into the house, phones, purse handles etc. must be cleaned with Lysol wipes.
2. If you've been in public areas or around sick kids, clothing should be sanitized if you'd like to hold the baby.
3. Following accessory cleansing, everybody must sanitize hands and arms.
4. Everybody sanitizes again directly before holding the baby.
5. If you are at all sick or potentially sick we ask that you do not come in the house.
6. If you have to be with Miles but have even an inkling of a cold, we have masks for kids and adults to wear. 

Given these complications we probably won't be up for playdates for a while but maybe some outside activities once we get settled and I figure out the daunting task of juggling two kiddos. 

It's a long road ahead with many doctors in our future but we are home. 

Chapter 1: Hot Tears

Cool tears tend to sneak up on you in moments when you are so absorbed in the emotion, the drop of water on your cheek is surprising but tender.  Grieving tears tend to have no noticeable temperature as you aren't aware of anything but your own unrelenting flow of dolor. The worst kind of tears are hot tears, when your eyes are so swollen from days of anguish and sorrow, and your skin is cool with hypotension and shock, that every tear seems to come from the deepest dredges of your soul and burns like bile as it drips onto your skin.

***

Miles Ray Dipo (read here for why we chose this name) was born on the 16th of May. He surprised us all by coming early, coming fast and screaming with health and ruddy skin at birth to a room full of all types of panicked doctors and nurses expecting the worst. With a vigorous debut we were allowed to snuggle Miles for ten minutes before he was whisked away to the NICU for evaluation. We knew he would most likely need surgery within a few days after birth, we knew that the worst case scenario was that he might need emergency surgery immediately upon delivery. We knew what to expect, we were prepared, we had hope. But we were wrong.

(Mama seeing Miles for the first time in the NICU)

Miles has 8 tumors in his heart. Miles has many tumors in his kidneys. Miles has 7 large tumors in his brain. Miles has tuberous sclerosis.

The diagnosis, made at Seattle Children's Cardiac Intensive Care Unit after a series of echograms, ultrasounds and an MRI, meant an entire new plan of care and prognosis for his future.

Miles' most pressing need is still the life-threatening heart tumor. In tuberous sclerosis patients, the heart tumors (called rhabdomyomas) tend to regress over time. Sometimes it's within a few weeks and other times it's over many years, but unfortunately, Miles' heart is working so hard unless the regression happens quickly and aggressively we will have to intervene. After a few exhausting days in the CICU Miles' heart continued to surprise and impress everyone because even though the tumors were not regressing, he was thriving. The team had determined the tumors had not grown into the aortic valve, and were concerned that heart surgery could damage his valve and turn one disease into two with a lifetime of heart medications and surgeries. So the current plan is to experiment with a few medications that might be able to accelerate the regression of his heart tumor. Unless they see vast improvement they will need to perform the surgery, but they are trying the best they can to avoid that.

Meanwhile our doors have been perpetually rotating with a slew of other doctors. Once we get out of the woods with the heart tumors, Miles' greatest challenge will be his brain tumors. His brain is scattered with tumors, but there are 7 prominent ones of concern. He had an EEG done where they discovered that though his tumors weren't currently causing seizures, he is expected to start having seizures in two or three months and will battle them for his whole life. The tumors will also impact his cognitive abilities and general brain function - but to what extent, nobody can predict.

 (Rastafarian Baby - during the EEG. So excited to do this with a toddler... said nobody ever)

Now we begin the waiting game. The game we will be playing for the rest of our lives. Nobody knows what to do. All the doctors on all the relevant teams (cardiology, neurology, nephrology, surgery, genetics and pharmacy) are meeting daily to discuss Miles' course of care. They are watching his heart at every beat with baited breath. The medicinal approach is somewhat unusual and exploratory, and as one cardiologist referred to one of his drugs, "voodoo" for its lack of physiological understanding. But we are hoping and praying it works. We are beyond ready to go home and we fear if the medicine doesn't work after a week of close observation they will end up needing to operate on him thus extending our stay many more weeks.

In spite of this sad news, we have seen many small miracles along the way. For the first few days of life, Miles did not need any sort of assistance for his breathing, circulation, eating, etc. Heart surgery, while certainly still a possibility, is seeming less and less likely, and even if it does become necessary, it looks like the aortic valve will not need to be replaced. For the last few months, the thought was that surgery was the only option, and would be done within a day or two of birth. The doctors and nurses in general have been amazing, and a few in particular have treated Miles as well as their own. Morgan woke up one night to find Miles crying, and a nurse trying to rock him to sleep in the rocking chair. Feeling like that was really the job of parents, Morgan got up to take over, only for the nurse to shoo him away, telling him to get some much needed rest. TS is a scary diagnosis, but we have caught it at the earliest possible moment with some of the best doctors in the country working on the forefront of children’s medicine. Miles has a long road ahead regardless, but there will be plenty of early intervention with very motivated parents to give him the best possible chances to impress and surprise everyone.

Miles is beautiful. He looks perfect in every way and it is nearly impossible to believe that a child so pure on the outside is so terribly broken on the inside. We have no idea how this horrible disease will impact his life yet and we honestly never will as each stage of his life will uncover new challenges for him, but we are ready to enjoy every moment as they happen and pray for more good days than bad. We do expect to be spending a couple days a week at Seattle Children's for the first few months once we are released for MRI's, echograms, EEG's, therapy and such, but hopefully it will quiet down and we will find a rhythm and discover that the worse of the TS (tuberous sclerosis) symptoms appeared at birth and he is otherwise on the low end of the spectrum. That's our prayer, and perhaps it could be yours every once in a while.

Introducing Miles Ray Dipo

What's in a name? For Miles by any other name would be just as sweet. Yet we wanted to give him a somewhat unique name that alluded to his upcoming struggle. My little brother, who was born very premature at 28 weeks when our family lived in Honolulu, was given the middle name “Kekoa,” which loosely means “brave warrior” in Hawaiian. Keeping with that tradition, but also recognizing Miles had no business having a Hawaiian name, Jenny found his name, which is Latin for “soldier.” Soldiers of all nations don’t win every battle, but regardless of the side they’re on, they all fight for a what they believe is a greater cause. And even when the odds are stacked against them, they still fight for honor and to be remembered. We’re not sure what all lies in store for Miles, but he has already proven to be a strong soldier, and his fight will be honorable and memorable, regardless of the ultimate outcome.

We also gave Miles the middle name of “Ray,” which is my dad’s middle name. My dad’s life so far would be a great one for him to emulate, and we felt it especially appropriate to give him that name as my dad was likewise born with a heart defect, albeit one that didn’t impact him much until it ultimately required him to get heart valve replacement surgery back in February this year. But the timing of my dad’s surgery (one month after we found out about Miles’ heart tumor) gave us a good sneak peek into how routine heart surgery has become today and how quickly one can recover to full function. Granted, we are still holding hard to the possibility he won’t need heart surgery yet, or maybe ever, but it was one of many ways I feel a higher power has prepared us very well for this trial.

37 Weeks: The Plan of Action

We've made it to that magical number: 37 weeks! Though baby is small he is full term and old enough for open heart surgery. Fortunately because of his big growth spurt those last few weeks, they will allow me to go until 39 weeks. So the countdown is on, because baby is coming on May 26 unless he decides he wants to come earlier.

On Monday we met with the heart surgeon, the Swedish neonatologist, and toured Seattle Children's Hopsital and the NICU at Swedish. Unfortunately, it seems we misunderstood the last doctor and our little boy will be swept off to the NICU at birth despite his size though I'm told I may get to hold him a few seconds... He won't be allowed to nurse, leave the NICU, or eat anything for at least 48 hours (or so that's the plan now) even if he is doing really well because there is so much uncertainty about what will happen when his ductus closes.  They will basically do non-stop echograms on him watching and waiting and deliberating the next course of action amongst the various pediatric cardiologists and surgeons that have all been watching this case the last few months.

If baby is still doing ok and the tumor is basically impacting him after birth as it was in utero, once I'm released from the hospital he will be transported to Seattle Children's Cardiac Intensive Care Unit - the place we will call home for the next 4-6 weeks.  They will continue to monitor him and try to prolong heart surgery as long as possible weighing the following risks:

• The younger/smaller he is, the greater the chance of brain damage from the bypass machine.
• The younger/smaller he is, the greater the chance they won't be able to close up his sternum after surgery and so he will need it left open for days or even weeks. 
• The younger/smaller he is, the smaller all the components of the heart are making for a more complicated surgery.
• The longer we wait, his heart will undergo more stress and continue to misshapen (as it showed in the last echogram).
• The longer we wait, the more we will know about the tumor including if it will start to recess which would indicate the possibility that it may go away completely over the course of the next few years and possibly - if recessing quickly enough - keep him from heart surgery. 

As I have gathered it, the most likely scenario seems to be they will operate on him about a week after he is born. After the surgery he will be intubated, sedated and broken for another week and then hopefully we will be able to start holding him and somewhere a few weeks down the line even start nursing him. A lot of things could go better than this and a lot of things could go worse, but that seems to be what we are mapping toward. 

What you can do.

All of the grandparents work but have graciously made time in their schedules to come help with our toddler as we embark on this awful journey. My in-laws from Spokane will be watching our toddler for a week and my mom will watch him for a couple weeks. After that, we figure we will probably both be so over the horrible terrible no good very bad hospital life that it will be a treat to be home and Morgan and I can swap being there and being home. It's going to be a long, exhausting and arduous haul and I know we are going to be lonely, scared and bored out of our minds because we won't even be able to hold or nurse our little one, just sit, touch, and watch those monitors and tubes. At this time, I feel like we will love visitors, at least after the first week or so... and bring snacks if you come. 4-6 weeks of eating out three meals a day is going to get old and expensive. No balloons or flowers though, apparently they want our little CICU room as dreary as possible. Also, please let us know if you live somewhat close to Seattle Children's and have a spare room. The bed in the CICU is a single and nurses and doctors will be checking on him all day and night, so I imagine there will be times both during the day and night when one of us may want to escape for some uninterrupted sleep without having to go all the way back to Bainbridge thus disturbing our toddler. 

Morgan says he is numb to all of this and is just ready for it all to be over whatever the outcome. I'm experiencing a different array of emotions as the time is now so near and so final. I am terrified. I am more afraid of May 26th and beyond than I have ever been of anything in my life. I'm afraid for Kai, for the unfair challenge this is going to be on him. I'm afraid for our baby for the horrible pain and suffering he is going to endure. I'm afraid for us that we will collapse under this physical and emotional trial. I'm afraid for life after it all... whatever that may be. But at least this prologue is almost over.

Chapter 1.