Warm Crusty Bread

There are only a few little pieces left of EEG glue stuck in Miles' hair, the smell of hospital chemicals is finally dissipating and that wonderful fresh baby smell - that delicious newborn smell akin to warm crusty bread - is starting to surface again.

Deep full breaths.

We made it. Miles is still seizure free and on Friday night, we began preemptively treating him with the Vigabatrin following the guidance from that positive study. The medicine makes him extremely sleepy (I think he slept around 20/24 hours of the day yesterday) but we are told the sleepiness factor should only last a couple weeks as his body adjusts.

His EEG lasted almost 30 hours and was absolute torture for both me and my little boy. Over the course of an hour, they placed about 30 leads all over his head - each time exfoliating the spot, covering the now raw wound in glue, attaching the lead, and blowing cold air into it until the glue hardened.  Miles screamed and screamed and got to the point where he would pass out for the few seconds between lead placements because he had been worn down past his limit.

Only about 1/3 of the way through and already passing out with each break

He was uncomfortable the entire EEG. We learned later that some of the exfoliating was much too rough and left open wounds on his head which contributed to the discomfort. The next day, when they were removing the leads (which is an awful process as well, albeit quicker) Miles barfed ALL over the EEG technicians and I felt proud, vindicated, bad for everyone involved.

The good news from the EEG is that Miles isn't having seizures yet. The bad news is it shows spikes and slowing which is apparently code for "seizures are coming" and "hey your baby's brain isn't working quite as well as it should." If the doctor was left to his own devices he would have said but we don't know anything, this doesn't necessarily mean anything, it could still be ok we will just have to wait and see... but because I am a fire breathing dragon and apparently like to dwell in fiery pits of negativity, I begged for a more realistic prognosis - but what does that mean? Be real doc, I can take it.

I can't take it.

Between the "heavy tumor burden in his brain" the fact he has Tuberous Sclerosis Complex, the results of the EEG and his lengthy bypass run during his open heart surgery, Miles does not have the odds ever in his favor. I think Dr. S's words were "I have a lot of concerns for Miles' future, this medicine may be his only fighting chance." He reminded us fifty times that preemptively providing the medication is NOT standard protocol, and that it comes with a plethora of risks but who needs eyesight anyway? Sigh.

We have given him a fighting chance. We are going to have an outstandingly miraculous healthy little boy who defies the odds and teaches us all new things. I wish very much I didn't need to learn these "new things" and my difficult decisions in life were how many diapers to pack, how to finagle the most amount of sleep out of my newborn and which snacks to pack for my toddler... but instead I get to decide if a 30% chance of vision loss is a better risk than an 80% chance of infantile spasms which leads to about a 90% chance of mental retardation.

Remember when I blogged about my horror dog that is a neurotic peeing maniac and escape artist on a maximum dose of Prozac? Remember when I worried about Kai because he wasn't really a people person for a few months of his life? Oh past Jenny... you had so much to learn. But for right now, I get to bask in the smiles, soak up the snuggles and enjoy some actual warm crusty bread (thank you Chuck and Judy Ogden!) and lean on hope and prayers with everything I've got. Please forgive me as I post a few of my favorite pictures from this last week - I'm allowed to indulge on occasion, right?

Something to Hope for

How are you doing? I always respond the same way trying to remind myself that we are home now, Miles is off the feeding tube, he is nursing and growing (over 8 pounds now), and if it weren't for the scar on his chest, a handful of meds and his diagnosis he would be a normal newborn. So we should be ok. We're fine. He's fine. Just normal newborn challenges now.

The truth is I struggle. The future is terrifying. I keep trying to think live in the now, focus on the now, but it's easier said than done. We were told Tuberous Sclerosis Complex (TSC) is one of those terrible diseases that is out of our hands. His tumors are too many to be removed; more will grow and we don't know how they will impact him anyway; there is nothing we can do. At the hospital about 8 weeks ago they discussed with us the tumors in his brain and his EEG results letting us know they expected infantile spasms to start between 2 and 3 months of age. Once they started they would get him on medication, but that only helps control infantile spasms in a small proportion of patients - most kids need to try a variety of medications that never really work and come with an arsenal of their own problematic side effects and many kids, even while medicated, still end up with hundreds of seizures a day. Infantile spasms have a terrible outlook with the grand majority of children that have them developing mental retardation and uncontrollable epilepsy for life.

There is nothing we can do. Sit and wait and watch. 

But yet... I began researching infantile spasms (IS) and reading up on the latest studies impacting children with TSC. In 2011 an article was published showing a brand new study that indicated infants diagnosed with TSC who showed a propensity for infantile spasms (abnormal EEG scans) benefitted greatly from starting a medication before the onset of IS. The study was small and only covered two years, but the results were astounding. Out of the 14 patients treated before the onset of infantile spasms, only two ever developed IS, one of which developed drug resistant epilepsy and they were the only two that developed mental retardation. The other 12 children retained their IQ. 

For the first time since Miles was born we found hope. I found this article on Monday night and Tuesday morning I called our neurologist at Seattle Children's (for the tenth time) begging him to do something preemptively for Miles. And for the tenth time I left a message with a nurse and never heard back. I then called the local TSC expert at Swedish, (Dr. S) who had agreed to meet with us back when Miles was born but only had availability in September. I told the nurse that I needed Miles to start this medication - referenced the paper - and that I would love for Dr. S to sign off on the plan but I was going to find a way to get him on this no matter what. We got a call back immediately and Miles will be going back to the hospital on Monday for a 24hr EEG and a full workup at the Tuberous Sclerosis Clinic at Swedish and if he is a good candidate we will start the medication.

The medication, Sabril, comes with serious side effects and risks - 15% of patients, for example, develop permanent vision loss of some form. However, in our opinion, the risks of not doing anything far outweigh the risks of the medication. We are giddy with hope. Now, our prayers are desperately focused on making sure Miles can go just a few weeks longer without seizures because as soon as they start we lose our edge. Morgan and some friends gave him a priesthood blessing the other night and we have fallen on our knees in desperation because finally we have something tangible to hold onto, something specific to pray for, some hope. 

We are beyond frustrated with Children's that they didn't know about this possibility... we could have started this medication 8 weeks ago and be far ahead of the game. But, is it fair to expect doctors to stay that closely informed of an extremely rare disease that they would be willing to practice a study published only a couple years ago? Probably not - but what a lesson this is for us. We can't just put our trust in the doctors. People kept saying we need to be our child's advocate, and yes, of course we thought... what a silly statement... but now we get it. If we hadn't read this article and pushed for Miles to be seen, we would have started having infantile spasms and it would have been too late. 

We beg of you for prayers, good vibes, energy and so forth that Miles can stay seizure free until the medicine has taken effect and that he will avoid infantile spasms all together. This is empowering, this is exciting, this is hopeful - maybe we are cut out to be Miles' parents after all.