Neuro Surgeons: Investigated/Replaced his shunt, but thought it was working just fine. Not sure why he is having this cranial pressure, maybe med related, could be poor vein drain from head (but think he'd be more symptomatic if it was a vein blockage). They said the brain is very good at developing good drainage if one avenue is blocked. They can operate if other tests demonstrate a need to.
Hematology/Oncology: Kelptyn's doing better than he's ever been. Making red cells, platelets on his own, showing normal calcium levels, normal sized liver/spleen, and no symptoms of Osteopetrosis currently. Yay! Still waiting on his white cell production to pick up and maintain 100% donor level over time. Doctor thinks Kelptyn may have drainage vein problem because we've been weaning steroids super slow.
Endocrinology: They have seen some increased cranial pressure when weaning steroids before. It could be too fast of a wean or poor recovery of his own glands to regulate. Think if it was a vein drainage problem then Kelptyn would be more symptomatic even when on steroids.
So there are a few pieces of the puzzle. Next steps, are for Hema/Onc to continue to follow up with doctors and radiologists in Minnesota that have seen more cases of Osteopetrosis and get their insight as to what they think of Kelptyn's scans. Is it a vein drain issue or not? In the mean time, we are treating it as an or not and testing his sodium level every 4 hours to maintain his lower cranial pressure while we very slowly wean steroids. ICU NP will be graphing his steroid weans and his sodium levels for a historical picture to see if we can discover any correlations as this is not the first time we've seen cranial pressure increase as we've weaned steroids.
We might be in the ICU for quite some time as we do this because the ICU is the only unit in the hospital that can bolus sodium and keep a watchful eye on it (or at least this is what the nurses have shared with us). We are trying to get him to maintain a higher sodium level with oral meds and get him off of the IV sodium. This is easier said than done. It upsets his stomach and makes him want to barf/poop at the same time. :(
Sorry if all this information is overwhelming and/or confusing. I've tried my best to simplify the information. Kelptyn's pretty much taking this all like a champ, as usual, as long as his cranial pressure is within normal limits. He's playing with his toys, requesting songs, and resting peacefully which is a medical blessing.