Mom to Edward Malcolm Joseph October 29, 2013
Baby M #1: 11 weeks, July 2014
Baby M #2: 9 weeks, November 2014
Posterior Urethral Valves (PUV)
Three years after marrying my amazing husband Michael, we decided to start a family. After a few months of unsuccessful trying, I had a feeling there had to be something wrong… I called my mom and luckily she knew a fantastic fertility doctor who could see me right away.
It was a pretty quick diagnosis – it only took one ultrasound to discover I have polycystic ovaries. We tried a couple cycles of Clomid and then opted for surgery to get my ovaries to respond. Two months later I was pregnant with our absolutely gorgeous and wonderful little girl, Halina.
As a family of three we did lots of fun things but couldn’t wait to grow…
In January 2013, I went back to the clinic. Our 2nd cycle was a bit wonky and we had booked a trip for the three of us and my parents to go to Cuba for a week before I went back to work so the doctor said to stop all medication and just go enjoy the sunshine…
When we got home I went for a pregnancy test at the clinic and it was negative. We were disappointed but not overly surprised. The nurse said to come back once I got my period. 10 days later – no period. So I went back for blood work on the Saturday of Easter weekend. When I got home, Michael and Halina met me on the porch. I asked if the nurse had called and Michael said, “No, not yet”. I was a little annoyed because I was anxious to hear back – when I turned around Michael was holding Halina in the sunshine and crying. I asked him what was wrong and he said, “You’re pregnant… I was going to tell you in a different way but the clinic called and you’re pregnant”. I hugged him and Halina and then called the clinic to confirm. It was true I was pregnant with baby #2. Michael said right away that he thought we were having a boy this time – I secretly agreed, but didn’t say because I hate to jinx things.
The next day we hosted Easter and Halina’s first birthday party. We had an Easter Egg Hunt for Halina and our nieces and nephews. There were a couple of bigger eggs with letters on them that spelled out: “Happy Birthday Big Sis Halina”.
At first they couldn’t figure it out and my dad said “oh big Sicilian” (he is Sicilian, lol). Then my mom hugged me with tears in her eyes and said “I got!! Congratulations!” Then my sisters came in the room and read it and looked at me confused because 2 days before I told them I had gotten a negative test result after our trip.
Because I am a fertility patient I had extra ultrasounds – at 6, 9 and 12 weeks – each of them are very exciting! At 6 weeks we saw and heard his little heartbeat, at 9 we saw his profile for this first time and the shape of his little body and at 12 we saw him move around and flash the ultrasound tech! She asked us if we wanted to know the baby’s sex but we said we wanted a surprise but her offer kinda gave it away… We were very much convinced we were having a boy.
We had one additional ultrasound just after my 9 week scan because I had some bleeding that scared the heck out of us! I remember crying and yelling for Michael just as I was about to put Halina to bed. I felt a gush and went to the washroom. I thought I might be losing my little Cuban Sunshine.
My parents came to watch Halina and we went off to the emergency room. After much fuss and a very helpful nurse they agreed to give us a bedside ultrasound. And there was his heart beating nice and strong. I went back to my doctor and he did another ultrasound and said the bleeding wasn’t very much at all and nothing to really worry about – just to take it easy for a few weeks. PHEW!
The pregnancy went on normally – I continued to work out and teach fitness one night a week. I was often emotional – crying to the Pina Colada Song on the radio and laughing hysterically by myself to the silliest things. I told Michael that this was definitely our emotional baby because I was all over the map. I started to feel some real movement around 16 weeks. I remember thinking the movements felt low but everyone reminded me that my uterus wasn’t that big yet. At my regular OB appointments the baby’s heart rate was strong and everything seemed to be normal. The heartburn started around this time too.
Then came our anatomy ultrasound at 19 weeks… It was July 9th, 2013.
I got called into the room pretty quickly and was a little surprised when the woman said she was a student and that her supervisor would be in after she completed all the measurements to check her work. I thought this was going to take forever and poor Michael was in the waiting room with a 16 month old!
At the start the tech was swirling the ultrasound probe around and didn’t settle anywhere – I asked her if she was having trouble finding the baby. She said ‘oh no, everything’s fine’. I said ok, chalking it up to her being a student. I think I may have even dosed off slightly it was taking so long! I asked if everything was ok and she said ‘yes, the baby is just in awkward position’.
Finally she went to get her supervisor – it felt like she re-did the entire ultrasound. Meanwhile Michael started to notice that the waiting room was full of all new people and that women who had come in after me were gone with baby photos in hand. He asked another tech if she could check on me. Eventually they brought him into the room and he said ‘I thought there was something wrong this was taking so long’. No one really said anything and we told the tech we didn’t want to know the sex and she said that was good because she couldn’t tell anyway – she wasn’t the friendliest person (or she was just concerned and I took it as her being unfriendly). She said the baby was in funny position too and that she couldn’t get a good profile picture so printed us the one she could get and gave it to us for free – we had our $5 ready but didn’t need it.
On the walk home we were quiet and Halina fell asleep. We decided our silence was because we were hungry. Michael told me how he thought something was wrong and that the picture looked funny. I told him not to worry because it was a student and she probably screwed things up and I’ll have to go back to get the measurements re-done and hopefully get a better pic! I sent the baby’s photo to our friends and family and explained that he was being a pain and covered his face with his hands! My good friend Kari and I laughed that he ‘punked us’ and was going to be a kid with a personality. She also thought we were having a boy.
July 10- There was a message from the doctor’s office who was covering for my OB to call them that day on their back line or to come in first thing in the morning.
I immediately started to panic. I called my mom and started to cry when she picked up. I told her about the message. She didn’t want to alarm me but said it must be something serious for the filling-in OB to call and give a backline number. When Michael walked in the door I told him there was a message from doctor and started to cry again. We played the message a million times. He hugged Halina and me and then went for a jog to think… I played with Halina and tried to stop crying. He came home and we just went over and over what it could be.
I did not sleep that night.
We decided to go see our fertility doctor in the morning before the other doctor. We knew he would have received my ultrasound report and if it was bad news we wanted to hear it from someone we knew. On the way there Iris by the Goo Goo Dolls came on the radio. We cried. And then we heard it pretty much every day. It is now Edward’s song.
He explained that the ultrasound revealed that the baby had a very distended bladder; it could be prune belly syndrome or a number of other things. He said that he knew about a shunt procedure that was once done to help with the problem but he wasn’t sure if it was still performed. Either way we’d need a chromosomal test and further ultrasounds to confirm a diagnosis.
Having seen him first prepared us for what the other doctor said, which wasn’t much more. She referred us to the Pre-natal Diagnostic Clinic. The appointment was for Monday. We left through the back door so the waiting room full of pregnant bellies wouldn’t see what a wreck we were.
I told my mom that I couldn’t wait the weekend to find out. She used to work at the hospital in radiology so she called a few people she knew and I was in the next day!
Friday, July 12th. We went to the hospital for an ultrasound. We asked the tech if Michael could come in and at first she said no, but after looking at my chart said it wouldn’t be a problem. She had to re-do all the measurements and it could take a while. So Michael came in and rubbed my feet the whole time. When she finished getting all that she could she asked if we wanted to see the baby. We, of course, said yes. She showed us all the “normal” things she could then his bladder. It was a huge black spot on the screen. It was full of fluid. We started to cry and she asked if she should stop we said no, that we wanted to see what was happening. At first she couldn’t see the genitalia but then it was visible – she zoomed in and there was little Edwards’ bits! As she went on showing us Edward, the 3 of us started to cry… It was bad and we all knew it.
Our next stop was the Pre-natal Diagnostic Clinic. Before it became the worst experience of our lives Michael felt Edward kick for the first time (it was a beautiful moment). We were given an intake form requesting family histories, etc and then a section for “anything else you would like discuss”. I wrote in that section “Please do not mention termination. This is not an option for us so we would like to discuss anything but that.”
We saw a genetics counselor first. The first words out of her mouth were “I see you’ve written that you don’t want to discuss termination but it is an option for you”. I want to scream “Ummm, hello, can you read? I clearly stated that it was not an option for us and that this was our son she was talking about and we hadn’t even seen a doctor yet to get an official diagnosis and/or possible treatment options!” But instead we told her it wasn’t an option and to move on please. She then went on to say that our son had an obstruction in his urethra causing a block in his kidneys and based on all the soft markers she thought there was a 20% chance it was chromosomal. We had no idea why we she was telling us this or why it mattered but she went on. Michael pressed her on her stats and she kept talking about the nuchal fold and clubbed foot. I didn’t even know Edward had a clubbed foot – what did all this mean? We asked her about the shunt procedure and she said Mount Sinai in Toronto used to perform it but she wasn’t sure if they still did – she would ask the doctor. She then said if we went that route we should “really consider the quality of life and that termination was still an option”. WTF?! Seriously? Michael told her it really wasn’t an option and said we were Catholic. I just can’t understand why she would mention it again – there was this baby boy kicking inside me and fully alive and she kept mentioning the worst possible “solution” to me.
We waited a while before the doctor came in to tell us that Edward had been diagnosed with Posterior Urethral Valves and termination was an option. OMG! We told her no and that we wanted other options like the shunt or what carrying to term entailed. Would the baby make it to term with no fluid and this condition? Would he feel pain?
She told us she had called around before meeting with us and that Mount Sinai no longer performed the shunt procedure because of the “poor outcomes for the babies” but she could look into further for us. But she left me with the firm impression he would die in utero and to look out for when fetal movements stopped. She asked if we wanted to see a social worker – we said no, that we had a great network of support including our parish priest and we wanted to get home. The incidence of PUV is about 1 in 8,000 not all of those are fatal.
I went back to work on July 29th. That afternoon I googled “PUV shunt success stories” and discovered that the Children’s Hospital of Philadelphia still performed the surgery! I called their 1-800 number and the receptionist put me through to one of their nurses. She confirmed that the procedure was still done and that they did one last month. She didn’t know what procedures were or weren’t happening in Canada but that a Canadian doctor used to work at CHOP and was a researcher in this area. She gave me his name and contact details. She knew he wasn’t close to me but he would have better information on what was happening in Canada. I emailed Dr. Wilson who is in Alberta and he emailed me back saying that the procedure could be useful on the right candidates. He gave me some more contacts and wished me luck.
Then I found Dr. Greg Ryan at Mount Sinai… He had performed this surgery in 2009 and it was on the Mount Sinai foundation website. What the heck?! I cried and apologized to Edward that I didn’t investigate sooner. In the morning I went to my regular OB – not the high risk clinic who I was scheduled to see on July 31st (yes, 2.5 weeks AFTER my initial app’t). I told him how the other doctor had told us the procedure was no longer done at all and how I was still waiting to get in to see them but that I really wanted to see Dr. Ryan!
He said he would make that happen and he did! That Friday we were in Toronto with the specialist and seeing signs of hope finally instead of total despair. Dr. Ryan told us that Edward’s kidneys looked a bit bright but not in terrible shape and that doing a bladder tap was definitely worthwhile – the tap would give us an idea of Edward’s kidney function and confirm whether or not there was a chromosomal abnormality. He gave us three studies about the shunt procedure as homework, set up appointments with an urologist, a nephrologist and a neonatologist so we would have all the information we needed in order to make a decision about the shunt if Edward was a candidate. We did two bladder taps. Preliminary results were in on Friday August 9th. The first bladder tap numbers were not very good but the second one showed improvement into the shunt candidate zone! Chromosomal tests were not in yet but Dr. Ryan felt that we should move ahead with the procedure anyway – the appearance of a clubbed foot and increased nuchal fold could be explained by the lack of fluid and the baby being squished. And if it was chromosomal there was no risk to doing the shunt – it just wouldn’t be very effective since the baby would likely have more issues than we were aware of.
Dr. Ryan suggested laser ablation of the valves instead of the shunt because Edward’s case looked like such a classic case of PUV… this would be the first such procedure in Canada. We said let’s do it! He said he’d make the arrangements and get back to us.
Tuesday, August 13th. Surgery day! It was a long day and we finally got to the OR it had nearly 20 people in it! It was like a Grey’s Anatomy episode –fellows, doctors, nurses, etc are all gathered and everyone was trying to decide how best to approach this. Michael got to come in the room, thank goodness as I was only slightly sedated for the surgery and needed him.
After what felt like an eternity and some poking around, I started to gather from the doctors’ tones and back and forth that something had gone wrong. I thought there was something wrong with the baby. They had to abandon the surgery and I was convinced it’s because something happen to Edward but when the lights came on and I was removed from sedation, Dr. Ryan showed us the tube that the laser and camera were meant to go through to reach the valves – and it was all mangled with a hole in it. The camera couldn’t reach the valves because it kept slipping through the hole.
We spent the night at the hospital and saw Dr. Ryan in the morning. He said to come back Friday to check on our healing. I went back on my own that Friday so Michael could go to work…. BAD idea. Edward’s bladder didn’t look like it was filling, Dr. Ryan seemed a bit concerned and told me to come back the following Wednesday. Originally he said we’d probably do the surgery on Tuesday or Wednesday so this was not a good sign. He told me there should be more fluid in Edward’s bladder by this time but we’d check again next week. I was a wreck, called Michael and told him he had to come to all further appointments.
I went home to pray for pee. Unfortunately the fluid in the bladder wasn’t increasing. This time Michael was with me and Dr. Ryan said he was finding it hard to be optimistic for little Edward. His kidneys were going into failure and without urine production there would be no saving the lungs and even if his lungs were somehow developed his kidneys were in very bad shape. He said to come back one more time to make a final decision – he would support us if we wanted the shunt.
We went back on Friday, August 23, and again no real change in urine production. We decided not to proceed with the shunt and said good bye to the team at Mount Sinai… all of whom were terrific in their care for us. They were all compassionate and really wanted to help us give Edward the best chance at life. We also got Edward’s full karyotype back – 46 chromosomes, XY. A perfectly normal baby with this awful fluke of PUV.
On Oct 29th, 2013 at 6:17am, 35 weeks on the dot Edward arrived weighing 5lbs 10oz. He was perfect in every way. He had lots of curly hair, not quite as much as his big sister or as dark, but still lots for a newborn. He had his mama’s nose, slightly smooshed because of his cramped quarters but definitely the same. He had his daddy’s chin, same as his big sister’s and blue eyes. When I was first pregnant and eating like crazy my friend Kari said “I bet you’re having a bruiser of boy!” and she was right – nearly albs at five weeks early!! His hands and feet were quite big giving us the impression he would have been quite a tall boy.
We were blessed with 2+ hours with him. He came out and cried – the greatest sound we’ve ever heard. The nurses put him on my chest immediately and we got some skin-to-skin time while Michael kissed his head and kept a hand on his back. This was the calmest moment and one we’ll cherish forever. He coughed a little and continued to take a number of breaths. All three of us, happy to finally be face-to-face after everything we had been through.
Fr. Michal arrived and Edward was baptized and received the anointing of the sick. Then Gaga and Nono arrived with big sister Halina. Our little family of four received one of the greatest gifts and that was time to bond. We prayed to Pope John Paul II for a miracle and, as my aunt said, this was maybe his gift – time. Halina held and kissed her live little brother and we got to hold our two gifts from God for quite some time.
Gaga held grandbaby #6, Nono gave us hugs. Next Zia Dianna arrived, held Edward, and then whisked Halina off to Toys ‘r’ Us for a well-deserved spoil. We had more alone time with our baby boy before Babcia arrived and held and kissed her first grandson. Auntie Weezie flew down the 401 from Windsor and although she was not able to see him breathing, his spirit was still there and she gave him lots of cuddles.
Edward had his formal photo shoot with a very kind photographer from Now I Lay Me Down to Sleep. She captured some beautiful moments and we got his hand and foot molds.
We stayed with Edward for the rest of the afternoon before going home to be with Halina.
Edward was buried in the morning, October 31st, with my maternal grandparents. It was a beautiful ceremony and honoured our baby boy in the perfect way.
It’s been a year and half since we had Edward and in that time we have lost 2 rainbow babies. We got pregnant with Baby M#1 just 6 months after losing Edward and were so excited and nervous to go through nine months of pregnancy… sadly, at 13 weeks I went for an ultrasound and the baby did not have a heartbeat. I had never miscarried before – the shock was unreal. I was so confused and still grieving Edward that I found the grief for this baby almost unbearable. Baby M#1’s due date has come and gone and I still wonder if I would have been holding a three month old boy or girl… Our first cycle after Baby M#1 was a success! Pregnant again – would we lose a 3rd baby? Sadly, yes. At 9 weeks I went for an ultrasound and no heartbeat. That baby’s due date was June 20th… Would I be feeling kicks by now?
My doctor has run every possible test for recurrent miscarriage and he can’t find anything wrong – just bad luck. Edward’s PUV is completely unrelated to the miscarriages.
Will we try again? Yes. Every moment I have had with all 3 of the babies I have lost was worth the heartache and grief. I wish I could have had them longer. And having Halina makes me ache for another one. Children are a gift. I thank God every day for making mom to Edward and the little ones and for helping me through the loss of them.
In heaven, we will have quite the family reunion. Until then my loves, watch over mommy, daddy and big sister Halina. Loving you always.