I drove the three girls and Elijah down to the children's hospital again today for their six-month GI doctor check-ups on liver and celiac diseases. My sister-in-law added Toby to her family today, so I had a little less stress.
The sun-rise was absolutely gorgeous on our drive down. The enormous neon sun glowed over the fog-patched fields and the delicate blossom-covered bushes and trees. Elijah slept peacefully most of the way, until he was fed-up with his car-seat the last 20 minutes. With two stretches of road construction there wasn't time to spare, but we arrived just on time.
The GI doctor was mildly concerned at Hannah's slow growth rate--after gaining 2 lbs in the last 15 months, she now weighs in at 38 lbs for a six-year old. Hannah's blood draw included seven tubes of blood to check various vitamin levels as well as thyroid, which is often deficient in people with auto-immune diseases like celiac. I was also told to feed her more fatty foods like hot dogs and ice cream, something Hannah found absolutely hilarious to hear a doctor say. Emma checked out well with no evidence of liver disease progression or growth problems. She had a couple tubes of blood drawn to check the basics.
Naomi's spleen had been slowly enlarging and her white cells slowly dropping as a result of the increasing pressure in her portal vein from her liver fibrosis. In October her spleen was felt 6 cms below the rib cage. To her amazement, the doctor could not feel Naomi's spleen at all today. It is apparently no longer enlarged. "I wonder if she's formed a spontaneous shunt, " the doctor remarked.
I had heard of spontaneous shunting before, but it is relatively rare. It is where a body with portal-hypertension (high blood pressure in the portal vein, which feeds the liver, because the blood has trouble flowing freely through the liver--in Naomi's case, due to the fibrosis) suddenly re-routes some blood another direction to relieve the pressure in the portal vein. I need to look into it more (I also posted a question to an online group of ARPKD/CHF parents) to find out exactly what this might be, but it looks like there are several different types of shunts that can develop and they come with benefits and risks. Sometimes it is an aneurysm that develops from pressure in one vein and lets blood leak into another nearby-vein. In any case, it isn't nature's first choice for blood-flow and can cause issues, but it can also solve a lot of problems, including improving Naomi's white cell counts and lowering her risk of having life-threatening internal bleeding from a ruptured vessel.
I am waiting for word on Naomi's blood work today to see if her platelets and white cell counts have risen, and also waiting to hear from other parents who might have experience with this to let me know how I should feel about this. I think I am happy, even amazed that God may have remedied a major problem Naomi was facing, without medical intervention at all! Praise God (I think) for this development. I will post again when I get further word.