Down Syndrome Awareness -- 31 for 21 -- So What Kind of Surgery Did The Boys Have Exactly?

First let me thank you all for all the prayers and words of support. I'm hoping that today is over-the-hump day. Praying that Michael wakes up happy and pain-free and ambulates himself out of bed and downstairs all on his own. I'd like to open up that morning diaper and see an unswollen, healing "pee-pee". That would truly just make my day. It also doesn't hurt that today's rain removes all the pressure to attend the LI Buddy Walk... Rain was the line in the sand I wasn't going to cross so I'm off the hook -- though I really wanted to attend, support, network and show my beautiful kids off -- though with all that swelling, I might have been showing too much of them off (LOL).

I've been beating around the proverbial bush with regards to their surgery and promising a candid discussion for all you Moms who are thinking you might be experiencing the same thing or something similar. So here goes:

Brian and Michael were born 8 weeks premature, almost 5 lbs each. Thank God they didn't stay in there those last 2 months b/c I think their size would have just about broken my back... which was already in spasms carrying these two little linebackers. They were long and wicked skinny with barely an ounce of meat on them but all of their pieces and parts were in tact and functioning on their own. Thankfully! This included their penis-es. Not sure how to pluralize that word... peni? penis'? penises? LOLing... just looked it up in my trusty Webster and though penises is correct, I think I like the penes -- the 2nd e pronounced as "ee" -- best... so that's what I'll use going forward... And, BTW, that's my learned-something-new for today!

The boys were circumcised just prior to their separate departures from the NICU (4 weeks apart due to Michael's NEC infection). As such, their circs were done by different individuals. Brian's by the MD on rounds and Michael's by a midwife on rounds. In the weeks and months that ensued the boys gained weight and I began to think, despite limited experience with little boys' penes (LOL) that maybe Michael's circ was not quite right. Very uneven and a bit of extra skin left around the top. At about a year, with both boys well into the "typical" developmental growth chart, their penes were rapidly disappearing into the foreskin... Michael's leading the retreat. The progression was like blowing up one of those long and skinny balloons. As the boys grew, or the balloon was blown up, the protruding end of their penes was disappearing. I brought my concern to our Pediatrician's attention and he explained that the entire penis was there and in the "normal" size range, but the penis was being overshadowed, so to speak, by the baby fat pad developing in their groin areas. Generally, this condition would resolve itself as the boys grew up and the fat pad dissipated. The official diagnosis was "inconspicuous penis". Now that's the most apropos medical term I've ever heard! That's exactly the term I would have chosen.

Of course, I researched to find out as much as I could about the diagnosis online... but that's just me. I confirmed everything my Pediatrician had told me and garnered additional information about surgical correction which was available if needed and warranted in some cases for various reasons.

A year later, the problem had become much more pronounced -- or less pronounced depending upon how you look it. In the world of Long Island Clam diggers -- my world, BTW... living in a bayman's cottage originally built in 1826 and occupied by clam diggers and baymen -- the boys' penes looked to me like very small "piss clams" (or, for a wider frame of reference, like the snout on what is known as a "steamer" in the culinary world). Or, like the little turtle had pulled his head in but left the neck of his turtle-neck shirt hanging empty out there. Please bear with me, I am not making light of this... ok, I am... but I want to give as accurate a description and visual as possible so that anyone who has a son with this issue will be able to recognize it and choose their own path. In my vast experience (LOL), their penes didn't look right so I asked our trusted Pediatrician for a referral to a Pediatric Urologist.

So, I see the man and provide him the boys' urological history (which is everything I've just told you all). He examined them and told me that the penis is a self-cleaning piece of apparatus and I shouldn't touch it... Wait! He'd never met a teenager with an inconspicuous penis (and in his walk of life, if anybody would have it'd be him!). They'll outgrow it.

I wait yet another year. Uncomfortable with the situation but feeling like I've covered my bases. When the boys were nearly 3 and at the prime age for potty-training I finally accepted the fact that there was not a chance in heck that I could potty train them "normally" with the equipment in hand. There was nothing to aim. Their urine dribbled out of their penes and straight down their legs. With their construction, they wouldn't ever be able stand in front of a potty and urinate and when they sat down it's ran all over their thighs. So I held the pot beneath their withdrawn penes, against their legs to begin the process.... Quickly realizing it was just not going to work. Far more disconcerting than the potty-training issue was that the foreskin, which had now totally enveloped their penes such that they looked like uncircumcised boys, had tightened significantly over the head of their penes. The opening was tiny and I could not pull the foreskin back anymore without it straining and then popping over the head and causing the boys MUCH discomfort.

I made our annual appointment with the old urologist and explained the new problematic developments. After rigidly explaining again about the self-sufficiency of the unit, he immediately pulled the foreskin down over each of the boys penes in turn and, on both, the skin ripped and tore... splitting wide open and bleeding in vertical wounds reaching from top to bottom all the way around their penes. The boys were screaming, wailing, bleeding... A LOT! But, the urologist stayed the course saying they'll be fine and I shouldn't be pulling that skin back anyway. Then he gave me a prescription for a steroid cream, and instructions to apply to their penes for two weeks to loosen the foreskin so that, should the boys get an erection, the heads of their penes would be able to break free from the skin without the skin tearing and bleeding as it did here... Otherwise we'd end up in the ER with a "trapped penis" and emergency surgery. Scary thought! Fortunately, erections were not a significant part of our lives so that scenario didn't play itself out. He also agreed that Michael had a little extra skin and an uneven circumcision and then diagnosed "skin bridges". That is, probably after the circumcision, the cut fore skin remaining on the penis shaft had fused itself back to the head of the penis such that there was no ridge between the shaft and head (sorry if TMI but again, I'm going for candid accuracy). He tells me that's this is a common problem occurring in 1 out of 9 men (I'd never heard of it before and I'm curious if any of the 9 men in my extended family had it) and can be corrected any time with a simple office procedure... He went further to share with me that he'd even corrected his own skin bridges right here in his office (Whoa... too much personal information -- as in "I don't want to know anything about your genitalia, Doc!") I was advised to follow-up in a few weeks to check the effectiveness of the steroid cream.

I filled the script and used it according to the Urologists instructions but I was not comfortable at all with the wait and see approach anymore. Or with the doctor's approach in general. Time was passing. Kindergarten -- and mainstreaming -- is right around the corner and I want the boys to be potty trained for that milestone if possible. I feel confident that's an attainable goal since both boys had urinated in the pot when held against their legs. But, in my humble opinion, their apparatus was not working as God intended, so to speak. So, I went back to my trusted Pediatrician and explained. He referred me to a different Pediatric Urologist within the same group. But this man was also a surgeon in the field.

We met with the new doctor and he began immediately to explain the surgical process used to correct the problem. Because my boys have Down syndrome, he said that the likelihood that they would outgrow their fat pad in the groin area was not great. Possible! But not great. "People with Down syndrome are not tall." Though I didn't appreciate his generalizations (all based on a brother-in-law with Down syndrome), I did understand the best and worst case scenarios of not going forward with the surgery. They'd either grow out of it or they wouldn't. Certainly I could take the wait and see approach. Or, the good doctor explained, he could pull back the extra foreskin and place 2 stitches above and one stitch below at the base of the penis shaft to hold the skin back permanently. All done in a half hour as an outpatient in a surgical center. At that point, I had not quite set my mind on surgery and was not convinced that my boys could not outgrow the fat pad given they were already very tall for kids with DS -- in the 50th percentile for height on the "normal" developmental charts. I thanked him and said I would think about how I wanted to proceed.

The Saga Continues: Tune in tomorrow for Part 2: Penoscrotoplasty: The Decision and The Surgery!