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.

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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.