Down Syndrome Awareness — 31 for 21 — Penoscrotoplasty: The Decision and The Surgery!

On a personal note, thanks to all of you for commenting and specifically expressing your interest in this topic. It is for all of you that are experiencing that sinking feeling that something is just not quite right “down there” that I am writing this story. I find the shroud of secrecy that exists regarding the male genitalia keeps such conversations out of the mainstream. For mothers of sons, we need to be able to talk about it, to confirm our suspicions and to take action… if we choose to! I’ve received many comments from Moms regarding their sons’ penes and this problem. Some with Down syndrome and some without. So, for the record, this is not necessarily or specifically an affliction for those with Down syndrome. I’ve heard from 1 Mom whose son with Down syndrome outgrew the condition. I’ve heard from several Moms whose sons with Down syndrome did not outgrow the condition but learned to live with it… with modifications (and assistance in some cases). The choice to surgically correct may or may not have been presented to these Moms… or they may have made a choice not to surgically correct. I do not know! And, I’ve heard from 1 Mom who chose the path I’m on now and, though her son’s recovery was difficult, she has absolutely no regrets and one year post-surgery, her now 6-year-old son has been potty trained and urinates using the stand-up method and is infection-free. Every body’s child is different. Every body’s journey is different. This particular saga is mine. The decisions I made were made specifically for my children. I’m telling it to get the word out and to give those who walk in my shoes — or shoes that are similar in any way at all to mine — that there are options. My old boss used to say, “It’s all about choices!”

If you happened to miss Part 1: So What Kind of Surgery Did My Boys Have Exactly? Click here!

Part 2: Penoscrotoplasty: The Decision and The Surgery!

The Decision: I went home from the new Urologist/Surgeon thinking I would not put my sons under anaesthesia or the knife unnecessarily again. Our post-surgical Tonsillectomy/Adenoidectomy experience last March was horrendous and I did not want to risk their well being… Besides, I wasn’t sure my heart could take their pain again either. But, it was their well-being that had me investigate the problem in the first place. I wasn’t sure that “doing nothing” was doing anything… for them and their well-being. So, as with every tough decision put to me, I over analyzed it! I did online research. I asked everyone I know (much to the male listeners’ discomfort). And I put pen to paper… writing out several pros and cons lists. In the end, with all the information I gathered, my list looked something like this:

If I do NOT correct the boys’ inconspicuous penes problem through surgery NOW:

PROS:

1. They don’t have to undergo surgery. They suffer no pain, no risk of delay due to potential surgical/anaesthesia complications or prolonged absence from school etc.

2. They might outgrow the fat pad themselves so the surgery would have been undertaken for nothing (or would it?).

CONS:

1. They undergo surgery again. Anaesthesia introduces the potential risk of brain damage and/or delay. Pretty much guarantees they will have pain, discomfort and absence from school. Worry about post-surgical recovery again (went south last time, could happen again).

2. Toileting. Cannot potty train them in a “normal” way which could make them the object of ridicule in school going forward (in diapers or toileting in a modified style). Will take longer than the extended length of time I’m already expecting AND complicates the procedure because I’d have to teach them to pull back the foreskin prior to urinating… not likely they’ll ever do this given past trauma. Want them trained, if possible, for kindergarten next year. Is that possible?

3. Hygiene. Given the foreskin tearing and bleeding scene with the first Urologist, the boys won’t let me touch their penes, let alone pull back the skin to clean. If I don’t correct, the urologist says I have to teach them to do this themselves. NOT very likely… which means introducing irritation, infection and potentially life-long dependence on others for good hygiene.

4. They’re not likely to outgrow the fat pad so this will not likely “fix itself”. They will have inconspicuous penes for life.

5. Inconspicuous Penes increases the likelihood of social ridicule. Just another difference that will potentially draw negative attention to them and single (or double, I guess) them out from everybody else: Identical twins… with Down syndrome… and small penes… AND they sit backwards on the potty to urinate. Not a good forecast!

I spoke with the Sarge, listed the pros and cons as I saw them and he felt strongly that we should go forward with the surgery. I guess size does matter (LOL). I discussed it with our Early Intervention Social Worker and she posed the deciding question: If your boys did not have Down syndrome, would you correct this? The answer was ABSOLUTELY YES!

Why hadn’t I thought of it that simply? The decision was made… Penoscrotoplasty!

The Surgery: The surgeon was surprised to hear from me after my less than enthusiastic demeanor with regards to the surgery upon my departure last visit. I expressed our interest in going forward and he introduced me to the surgical scheduler. She gave us a surgery date of July 7th. But, I ferreted out that the follow up included no submersion in pool water for 6 weeks. There was no way I was going to rob the fish, I mean the boys, of their summer. They LOVE the water! And since they were approved for the Extended School Year (ESY), they would miss school regardless. So, they rescheduled us for September 4th, right before Labor Day. I passed on that time frame too. I didn’t want them to miss the beginning of the school year when classroom routines were set in place. So we finally agreed to Tuesday, October 13th. I was given instructions to schedule a pre-op appointment with our Pediatrician the day before surgery to receive clearance and I’d be provided with specific pre-surgery instructions and the surgery times the day before, October 12th (Columbus Day).

The summer passed quickly and we were full swing into the start of the school year when the surgery date crept up on us. I scheduled a pre-surgery consult so that the Pediatric Urologist/Surgeon could show me exactly what he planned to do. He explained that he would re-open their circumcisions to open up the foreskin, he would “clean-up” Michael’s uneven circ and release the skin bridges. Then, he would pull back the foreskin and put two stitches on top on either side of the penis, and one stitch on the bottom in the center, all to hold the skin back at the base of the shaft. I also wanted to ensure that my expectations were in order with what the result was actually going to look like on the other side of this endeavor. Much to Michael’s chagrin, he pulled back the foreskin and demonstrated to me that their post-surgical genitals would look like “normal” circumcised penes. Satisfied, I confirmed our appointments and went camping….

I took the kids on a long-planned weekend camping trip (camper not tent) with about 15+ of our closest friends and, wouldn’t you know it, Brian came down with a mild post-Hershey Park, PA cold. I pulled both boys from school in an attempt to ensure good health for surgery and imposed a self-quarantine “no one in/no one out” rule in our home. Though I had significant doubts on Friday before the surgery, by Monday morning our trusted Pediatrician cleared the boys for surgery on Tuesday. The rest and relaxation and quarantine worked! We were finally going to get this behind us.

After receiving the “all clear” I took the kids to the petting zoo and Hay Play Yard at Atlantic Nursery and then out to lunch at Wendy’s… fully expecting to receive my pre-surgical instructions and surgery times via a message on my home phone or a call to my cell phone. It didn’t happen. I began calling around to the surgical center, our Pediatric Urologist/surgeon’s office and the Outpatient pre-registration nurse that called on Friday to confirm the boys’ names, address and other pertinent information. Each said it was the others’ responsibility to inform me and that I should wait for the call. I called again and again and each time was told to wait for the call. I was massively uncomfortable when each of their offices closed at 5 and/or 6 pm and I still hadn’t heard anything. I finally reached the pre-reg nurse at 7PM and she apologized saying she didn’t have access to the schedule information and, “technically, the nurses have until 8PM to call you with the information!” 8PM came and went. The phone never rang. Had I gone through all of this quarantining only to be put off due to administrative mistakes? At 9PM I called the Pediatric Urologist on-call emergency number and explained that my boys were scheduled for surgery in the morning but that I had no pre-surgical instructions or surgery times. He called me back. Listened. Apologized. Gave me very high-level instructions — Don’t eat 6 hours before surgery. Apple juice and water only up to 3 hours before surgery. Then nothing — but he did not know and could not access the surgery times. He instructed me to call the surgical center at 6AM when they opened and request the scheduled surgery times. To say I was disappointed or that I expected that doctor to handle it for me, I think, was natural. To say I was furious that I couldn’t plan my night-before-surgery and keep things calm and flowing on my end. I wanted to give the boys a bath at the last possible moment knowing it’d be at least 4 days before they could bathe again. But, with all the calling around, it got late and I wasn’t sure we were even scheduled for surgery at that point. I was worried that we might not be able to go forward because of their oversight. I thought if we were scheduled for the afternoon, maybe I could bathe them in the morning. No information was available to help me. So, I bathed them late Monday night… Not knowing. Having your children undergo surgery is stressful enough without being subjected to this kind of oversight. I began to worry about the competence factor. If they couldn’t remember to call me, would they remember to perform the correct surgery? Fortunately, each of my guys only has one set of genitals so they couldn’t amputate the wrong leg or anything like that. God help them if anything got amputated! (LOL… sorry, humor is what helps me through these situations). Between the worry and the frustration, I didn’t sleep a wink that night!

At 6:30 AM I reached the surgical center and when I mentioned I’d never received the call, I was told “I think one of the nurses left you message yesterday. Sorry, we must have had the wrong phone number!” No comment. I didn’t want my reaction to affect their treatment of my children. “Your boys are scheduled for surgeries at 11:30 and 12:30. [pause, muted discussion in the background] No, no wait. 10:30 and 11:30. [pause, more muted discussion in the background] No, no wait. Oh, just be here at 9:30 and DON’T LET THEM EAT OR DRINK ANYTHING!” Am I the only one having trouble understanding how to apply these instructions given the lack of information? Fortunately, the boys were still asleep so I showered, got the Old Soul off to school, got everything ready for a 9:00 am departure and then waited. They woke, dressed and we were off. No one talked about or asked for food or drink. There was no time and it wasn’t allowed anyway. As a matter of circumstance, Sarge and I fasted with the boys. Seemed the right thing to do.

We were taken in quickly. Stashed in a room with a television and asked a myriad of questions regarding the boys’ medical histories… 3 people firing questions at the Sarge and I at once. We discovered the admitting nurse threw away our Pediatrician’s clearance papers but they were able to fish them out of the trash and get the information they needed. (Hmmm! The question of competence comes to mind again.) Our Pediatric Urologist/Surgeon stuck his head in to say hello as did the surgical RN. Two people I like and trust! And, then we met with the Anaesthesiologist. I explained the difficulties my boys had with anaesthesia in the past to which he responded, “I know Downs kids.” Uh oh! He’s using diagnosis-first language and he’s lumping my children into a group as opposed to listening to their individual reactions to anaesthesia, surgery and pain meds. Not good. I overlook it and continue to provide the pertinent and specific-to-my-boys information that I feel he needs to know in order to do his job well and, most importantly, to keep my boys safe and healthy and alive! We’re given 2 sets of mini-hospital gowns, surgical caps and non-skid socks for the boys and a one-piece suit for me. I’m on the surgery brigade and Sarge is on the recovery brigade. We’re told to dress Brian first. He looks adorable in his gowns. Sarge snaps a few pics and he’s called in for surgery. It is exactly 11:30 am.

It reminded me of the red carpet! Or, death row’s dead-man-walking (not a good reference). Patients and employees stopped and watched him, smiling, as he passed. He’s waving and saying hello. Wearing his Blueberry Beret (like Prince’s song, “Raspberry Beret” but his is scrubs-blue) and everyone was commenting on how cute he is. He spotted the surgeon in a side room and waved to him too. Hit the button to open the big automatic doors into the surgery hallway and stopped dead in his tracks at the doorway of the operating room. “NO MOMMY!” I carried him in against mild resistance — I think he knew resistance was futile — and laid him down on the table. He’d fallen silent but his big green eyes were pleading with me and both of his hands gripped mine tightly. “It’s OK, Brian.” They masked him loosely and through the mask he mouthed “no Mommy” until he faded away. Eyes rolled then closed. I personally find the anaesthesia piece of surgery to be a very difficult emotional experience…. especially because I’ve had the unfortunate experience of putting a beloved pet to sleep. Very similar except the pet doesn’t wake up. My mind replayed those past scenes with my beloved pets and my stomach did a flip. This is my baby! It’ll be all right. “OK, Mommy, kiss him one more time” and I did. Then I turned toward the door. Looking back at my sleeping boy I said to the surgeon, “Good Luck” and took my leave… walking back down that hallway alone. Without my brave and confident little man in his blueberry beret.

Back in our holding cell, Michael had retreated to a hiding spot behind the big chair. He was quietly chanting, “NO! Bro! Where Bro?” I bent the television arm so he could watch Dora The Explorer from his chosen safe spot while Sarge and I chatted. The 40 minutes passed slowly… and quickly. The surgeon came in smiling with his stars and stripes do-rag and told us everything had gone smoothly. They’d be moving Brian into recovery and Daddy could join him there. “We’ll be coming for Michael shortly!”

I dressed Mikey, against his will, in the gowns and had no luck keeping his blueberry beret in place. This one would NOT go quietly. I carried him through the halls which echoed with “No!” and “Bro?” and into the operating room. He fought me as I put him down on the table and, like his brother, continued to protest — a bit more vehemently than Brian — until the anaesthesia took effect. The final kiss. The “Good Luck” wish to the surgeon. And the lonely retreat back to our holding cell.

Not ten minutes later we were bedside to a quietly sleeping Brian. “Let him sleep!” the nurses said. Sarge ran out for a vending machine egg salad sandwich, coffee for him and Diet Coke for me. By the time he’d returned, Brian was awake. In short order, Michael joined us and though he’d been given “a little something extra” in his IV because he’d awakened so quickly after the surgery, our little fighter fought to stay awake and kept up a steady protest as the Sarge and I rotated back and forth between their beds. They drank their apple juice immediately and without repercussions… asking for 2nd and 3rd helpings. The local anaesthetic worked so well on Michael that he was under the impression the IV in his hand was the “boo boo”. Unfortunately, Brian was not that lucky. In addition to the discomfort of an IV in the top of his foot, the pain in his genitals quickly became unbearable and he was given additional pain meds that delayed our departure for home. The surgeon visited and explained that the surgery went exactly as planned. No complications. The recovery nurse provided written instructions for their post-surgical care and by 3:oopm we were cleared for take off. Have you ever tried to carry a 47 lb boy without having them straddle your hip? That was one of the restrictions we were thankfully spared by bringing in our big red double stroller. We ducked out a back exit and by 3:10pm the boys were strapped in the minivan, eating Tostitos, drinking apple juice and watching Monster House on the portable DVD player. We were on our way home. Happy to be out of there. The surgery behind us… Recovery ahead!

Stay tuned. Our Recovery Story is still unfolding and it has not been without incident, unfortunately. Wednesday is our post-op follow-up appointment so I’m going to skip a few days and give you a break from all this drama. (LOL) But, I’ll be back to let you all know the outcome… the good, the bad and the ugly! And oh yes, there has been some ugly stuff! And some scary stuff! (That’s my series cliff-hanger!)

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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!

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