Welcome to Ezekiel's blog! I assume you've only wandered onto this page if you already know Jenny and I, and have more than a passing interest in Ezekiel's well-being. For that, we thank you. We plan to keep this page updated and hope this proves to be an effective medium to keep everyone informed. This first post is a little lengthy because there are multiple factors affecting Ezekiel’s health, but I’ll try to keep it as concise as I can. Now, onward to the tale of Ezekiel James Zart, Zeke, or Double-Z, whichever you prefer!
At our 20 week ultrasound, Ezekiel was diagnosed with a Posterior Urethral Valve (PUV) and bilateral clubbed feet. A PUV is a type of obstruction that occurs when there is an extra flap of skin in the urethra, blocking urine from leaving the bladder. This leads to an engorged bladder and excess liquid in the kidneys, a condition termed hydronephrosis. So much urine was backed up in Ezekiel that his right kidney had ruptured, leaking urine and forming an urinoma (cyst containing urine). Since this blockage traps all the fluid inside the body, that also means there was little fluid in the womb for Ezekiel to “breathe”, which is critical for lung development. There was also concern about his enlarged kidneys putting pressure on the rest of his organs and possibly impeding development. To help with both of these issues, we traveled to Cincinnati’s Fetal Care Center for an amnioinfusion and kidney aspiration (draining of the urinoma) at 22 and then again at 26 weeks gestational age.
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| Jenny geared up for her first fetal surgery. She may have a promising career as a lunch lady! |
The amnioinfusion went well both times, but the kidney aspiration was only successful the second time. Sadly, the urinoma refilled by the next morning, which meant his right kidney was still leaking fluid. After the second intervention, the added fluid disappeared in just two weeks and it became too risky to attempt another intervention. This means Ezekiel has been without any measurable amniotic fluid since week 28. He had already passed the most critical weeks for lung development, however, so we remained hopeful. Sadly, at 34 weeks gestational age, an MRI revealed that Ezekiel’s lungs were much smaller than they should be, a condition called pulmonary hypoplasia. This diagnosis carries a wide range of possible issues. He may be able to breathe on his own, but there is also the possibility that he may not have enough lung function to support life even with medical assistance. Hopefully, he'll land somewhere in between those two.
His prognosis is still uncertain since it's impossible to be certain how well his lungs and kidneys will function while in utero, but after countless appointments, ultrasounds, specialist consults, and those two fetal procedures, this is what we know...
The first hurdle he must overcome is his lungs. There are lots of potential treatments, and what they do will depend on how much support he needs. We've been told to expect him to need a breathing tube at the very least. Once he is stable, we can move on to his kidneys. He’ll need a surgery to remove the PUV and then they’ll assess his kidney function. It's likely that his right kidney will not function, and if it does, it will be at a significantly decreased level. His left kidney has a better chance, but retaining extra fluid for extended periods of time causes damage to the nephrons in the kidney, decreasing function. If he has little to no kidney function, which we've been told to expect, he will need peritoneal dialysis. This would require surgically installing a dialysis catheter in his stomach cavity and using that catheter to pump a dialysis solution in and out of that cavity on a regular basis to remove the waste from his body that the kidneys normally filter. There are risks associated with this treatment of course, but we've been told its infant success rate is as high as 80%! He would continue on dialysis until he’s big enough to get a kidney transplant, which would be around age 2. The last concern is his clubbed feet, which can be corrected with orthopedic therapy and sequential casting with no lasting negative affects.
With all of these considerations in mind, we've been scheduled for caesarian section on November 17th, just 3 weeks shy of his original due date of December 6th. He’ll likely be in the hospital for a while, but we can’t wait to get him home so he can enjoy his awesome room.
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| Han and Chewie have promised to guard Ezekiel's dialysis machine with Wookiee-like fervor. |
Thanks to everyone for your support, especially our family and friends who we've already been leaning on through all these doctors visits and changing diagnoses.
-Caleb
