Wedding Day

We've made it to the wedding day in Wabash.  Only 24 hours before we relocate to Cincinnati for the baby and it has been very quiet on the pregnancy front.  Last night we enjoyed talking about my sister and her fiance at their rehearsal dinner and were very happy to be able to be present for the festivities.  My sister is marrying a great guy and we are happy to (finally) welcome him into the family.  I would consider it a major success to make it though today and safely to Cinci before the baby makes his arrival.

Speaking of arrivals, due to this amazing country we live in and the incredible healthcare we have here (particularly at Cincinnati Children's Hospital), we know so much about Ephraim going into the delivery that would have been unknowable only a decade ago.  Lung size, kidney size, heart function are just a few of the organs which the doctors have been monitoring.  While they are unsure of much until he makes his arrival, Megan's maternal fetal specialist (MFS), Dr. Jaekle, has given us a very realistic rundown of what he is watching and where we should place our expectations.  The top three organ functions they are watching during and after delivery are: 1. lungs, 2. kidneys, 3. bladder.

1. Lungs - This probably goes without saying but if he doesn't have the lung function/development he can't make it and there is no substitute for this.  If he is premature, there are steroids they can administer to further development and help his lungs work but this is not the same as the lungs developing during the pregnancy.  As of tomorrow, Ephraim is 34 weeks and well past the stage of basic lung development.

This is the most important function and also what Dr. Jaekle has continuously warned us about.  While he specifically states that he does not predict what will happen, he thinks the probability that the lungs have not developed is very high.  For this reason, he doesn't believe there are going to be many hard decisions.  Due to the size of the bladder and the crowding of the chest cavity, Dr. Jaekle has given us a prognosis of minutes to months.

2. Kidneys - As a non-science guy, I am amazed by the kidney.  These organs are incredible and incredibly complicated!  The doctors have spent hours looking at Ephraim's kidney's and assessing function.  We have discussed dialysis and the impact that has on babies.  Now the nephrologists (kidney docs, for you other non-medical people), have a different tone than the other docs we meet.  After all, you only need 15% of your kidney's to function to do their job and keep you alive, and even less if you rely on a machine.  In fact, Dr. Goebel said we really don't need the kidneys as we can keep people going completely on a machine imitating the role of the kidney (dialysis).  However, he cautioned us that the outcomes are not great, there is still a relatively high loss rate.  Also, it is a 24/7 job for 1-2 people for 2 years until the baby is ready for a transplant, of which he would have multiple throughout his life!  Dr. Goebel has been fantastic dealing with our case passing us the latest studies showing life expectancies and outcomes of baby and children kidney cases.  While he is a specialist dealing with just one organ, he witnesses the incredible turmoil and stress on families dealing with infant dialysis has made it known that this involves enormous sacrific on the part of the family.

Ephraim's kidneys have been dilated the entire pregnancy, They have been filled with urine the entire pregnancy which impairs development.  It is estimated there is another 700 ml of urine in his kidneys. Everyone will be watching the kidneys the first few days.

Baby kidney work is a fairly new field.  Cincinnati has been doing this work specifically on bladder outlet obstruction babies since 2004 and has had 47 cases come through.  Although the statistics are far from rosy, there are success stories here.

3. Bladder - Due to the unprecedented size of Ephraim's bladder, this is a major concern.  While the bladder is normally not a critical organ and usually can be repaired or reshaped, the presence of ascites prenatally indicates there are real challenges here.  Ascites is a collection of urine outside the bladder in the abdomen where it has leaked through some outlet/hole.  On a normal baby with bladder issues the docs put in a stint or valve to the stomach to drain the bladder.  For Ephraim this is a major issue because if his bladder doesn't work he is not a transplant candidate, foregoing all decisions made on the kidney.

It has taken us a few months to understand and process these concerns.  One of the questions I've had for the docs is what happens to these babies not born in a rich country in one of the best hospitals in the world.  Their unanimous answer? They die.  The doctors at Cincinnati have been excellent in setting expectations and allowing the parents to be a key team member in the care of their kids.  Megan and I have delved into ethical concerns and the "gray" laws mandating certain treatment and not others.  I've told the doctors that just because we can I'm not sure we should.  I've been expecting the doctors to talk me into treatments and to jump in and be superheros, but they've been remarkably respectable in this case as only people who deal with life and death on a daily basis can be.  Their body language has told me more about our case than what they have said.

I have a new appreciation for quality healthcare and the people who practice it.