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This photo made my day so I thought I would share.  

These two little ones both had heart surgery similar to what Jacob will be having.  Ian, on the left, had his surgery when he was 15 weeks old and he’s doing so great (obviously)!  His Mom posts pics on FB all the time and he’s such a happy active little guy – always makes me smile.  His little buddy Noah on the right had his surgery ---- *three and a half weeks ago* ---- and I think that smile (and the fact that he’s out of focus because he’s wiggling so much) speaks for itself!!! 

Thank you precious Ian and Noah for providing happy reminders of what post-surgery looks like to help us be brave until we get there.

(...and thank you to Ian's Mom and Noah's Mom for all the helpful and encouraging information as we prepare for this!)

Heart Repair One Step at a Time : Step 1 - March 7

First - Baby Jacob continues to be happy, happy, happy and the come-back of his developmental skills (from his pre-spasm progress) is impressive (and awesome!).  Both of his therapists this week (he does OT and PT once a week) were super excited and impressed at how well he did.  Miss Em who takes such good care of him during the week and had only met Jacob post-spasm diagnosis...  is now seeing the old baby Jacob emerge and kept saying this week…he’s so strong!

About his little heart - the short version:
Jacob’s congenital heart defect consists of two holes in his heart - a small ASD and a small VSD.  Both of which contribute to his heart functioning less efficiently than it could/should.  His little heart is still doing it’s job - the oxygen levels in his blood continue to be good but the concern is increased pulmonary hypertension that was identified at his last follow-up. Fixing this is important because if left untreated it could cause long-term damage to the lung arteries. The pulmonary hypertension was possibly exacerbated by the steroids but the most likely root cause is  the resulting effects of the ASD and VSD. [Note: While not a cardiologist, I am pretty good at hydraulics - pumps and pipes - so I *could* further explain with some confidence the interplay between the differential pressure in the heart and resistance in the lung’s arteries but I doubt anyone else finds it that interesting].  Bottom line - fixing the holes in his heart should fix the pulmonary hypertension - and his heart will work better in general.

March 7 ---- Step one is to have a cardiac catheterization performed.  Jacob is scheduled to have this procedure on March 7.  They perform two a day (three times a week - every week) and Jacob’s is the first one of the day. It will be performed by his pediatric cardiologist who specializes in these procedures.  It does require general anesthesia and will take a couple hours.  Unbelievably - assuming no complications - he will come home the same day with nothing more than a band-aid on his hip where they accessed his vein.  This procedure will give them valuable data to prepare for the repair surgery…the most critical of which will be pulmonary artery pressure measurements and his lung’s response to vasodilators (oxygen and/or nitric oxide).

The heart surgery itself has not yet been scheduled and we continue to work with the team on a timeframe.

Repeat to Self: The cure is not worse than the disease. [sometimes it has been hard to remember]

There are two parts to this update. Connected, obviously in one little boy...but different stories in my mind.

Part A : Happy Baby (!)
The steroid treatment for the infantile spasms is still working/worked - no spasms for weeks! - and we are now weaning him off the steroids which is slow and scary (with us worrying the spasms might come back) but going well. However, the baby started to be getting very sick, in pain, and more unhappy than we have seen him.  So... the last two weeks have been fun (read sarcasm) and included one 9 hour odyssey in the ER, 3 pediatrician appts, an appt with his GI specialist, one with his cardiologist, and an echocardiogram. We will soon be adding a nephrologist to his team of specialists. All that gave us results showing a kidney stone (a kidney stone!), a UTI, and gastritis....Jacob just can't catch a break.... all most likely side effects related to the treatment of the spasms. [Repeat to self: the cure is not worse than the disease.] 

So...new medication, antibiotics, etc. followed...  and yes, of course he got a yeast infection from the antibiotics treating the UTI. Oy!

BUT this past weekend....somehow the combo of reduced steroids, most of the above well on its way to being resolved, and his increased calories (per GI appt)...resulted in an active happy baby! Who knew. Overnight, baby Jacob seemed to reappear.  We certainly saw glimpses of improvement the previous weekend, but this past weekend - he's a different baby.  Saturday morning he had three toys in his hands at once as if to make up for lost time.  And wow that does wonders for his parents' mental state. Big smiles. Yay.

[I'm including evidence - happy baby pictures!]

Part B: Oh Little Heart
In the midst of the insanity the last couple of weeks, we also had a cardiology follow-up.  And....well... not so great news there.  It looks like Jacob's pulmonary hypertension has increased - maybe as a result of the steroids but maybe just as a result of his general growth.  This in turn is making his heart work harder and is leading the team to think we should do the repair sooner rather than later.  They make open heart surgery sound so routine with the word 'repair' (although for them it is - routine, that is). As Jacob's case is complicated by all these other factors (read Part A), the team is consulting - putting their heads together to figure out the best timeline for Jacob.  Likely Jacob will have a cath procedure and heart surgery in the next couple of months.  Stay tuned.

I've said it before but worth saying again. His team of specialists are all rock stars and in situations like this it is never more clear. They all know each other, are talking to each other and coordinating well together.  Many meeting confirmation emails with lots of people copied (some we know well and extended team members we don't) - but we are copied on everything and the conversation is smart, responsive and always with what's best for Jacob in mind. 

SO... This Wednesday is Jacob's last dose of steroids (!) and we hope that's the last we have to think about EEG's and infantile spasms.   Follow-up with neuro will happen after that.  There are some instances of relapse and a second round of steroids but we are hoping/praying that is not the case for Jacob.

Much more consultation with cardiology ahead... and more about that when we know.

For now - happy to keep the medical what-not in perspective and just enjoy Jacob being back to happy little Jacob.

All things Jacob

This is the place for the latest updates for our lil boy Jacob.