From DIAGNOSIS to BIRTH - most recent update at the top

NOVEMBER 15, 2006 - Week 37 - OUR BABY IS HERE!
Jonathan Rafael Purdy-Flacks was born at 11:45 am Tuesday November 14, 2006 at Mount Sinai Hospital by C-section after nine hours of labour. He came week 37, one week earlier than the scheduled cesarean, but looks beautiful and weighs a solid 6.2 pounds. His omphalocele is as large as expected but his blood saturation is better than expected which bodes well for later heart surgery. He has been transferred to the Sick Kids NICU. Mommy is recovering at Mt Sinai and getting to him as much as she can. Mama is busy taking care of Eli, Mommy and Jonathan. We are consulting with the OB, cardiac & general surgery teams as well as the neonatal care team. He has long light hair, beautiful dark eyes and is handling all the interventions and intrusions with dignity and heart-breaking patience. It's already been quite a roller coast ride and we are trying to take one day at a time, and sometimes one moment at a time.

NOVEMBER 9, 2006 - Week 36 - "PRACTICE LABOUR?"
Okay, this "false labour" thing. Whassup with that? Poor Janis was up since midnight (why does it always happen at midnight) with odd intense pain. By early morning, we were pretty sure it was some stage of labour. We paged our midwife and we called Mt. Sinai triage and they told us to come in. In my case, when I was in labour with Eli, because we were under the midwives' care, we just blew past triage. Lucky me. By the time we arrived, it seemed very obvious that it really was the beginnings of labour. Poor Janis was strapped to a machine and just had to wait to be assessed. Finally, about an hour later, a resident came in and checked her cervix. Not dilated at all. In the meantime, after a few real doozy contractions, it all stopped. Urk. Then our high risk OB, Dr. Ryan, finally came over to tell us that it's actually good for the baby to experience some of the stress of "practice labour". The contractions help him empty the fluid from his lungs, and make Janis release a good steroid for him. Since the labour stopped, we returned home, where Janis immediately fell asleep. Our midwife Joyce had come by also, and advised us to go home, too.

In the meantime, we've cancelled most appointments for the next few days, knowing that it really could happen any time. The other thing was that Janis concocted a surprise for me for my birthday (which is tomorrow). If you know Janis, you know that she is not so good at keeping surprises, but she did with this one! She booked us both snazzy massages, and me a haircut for this afternoon at a spa. Well, we had to cancel. So, that's a drag. But what a lovely lovely idea and it really is the thought that counts at this stage.

On another note: yesterday we had an ultrasound and the baby scored 8/8 on tests! Go Raspberry! His practice breathing and swallowing, and his movement were all excellent!

So, Raspberry might be a Scorpio yet. Although we felt at the time, "hey, we're not ready!" Now, we also feel like we hope Janis doesn't have to go through too much more of this. That was the silver lining of the planned C-section: no labour pain. Hmmm, who invented this practice labour, anyway??

NOVEMBER 3, 2006 - End of Week 35
Well, we are almost at week 36, and we are so pleased with Raspberry for staying in utero this long. We are now passed the danger zone for a premature birth. Lung development is complete at 36 weeks, and he is growing very well. This is something to celebrate!
Today we had an echo cardiogram at Sick Kids with Doctor Jaeggi, the head of cardiology. He'd last seen Raspberry at 28 weeks. He told us that although the diagnosis of Tetralogy of Fallot is still consistent, there is some unexpected good news. The pulmonary arteries, although small, are bigger than he expected. This means that it is possible that the baby won't have to have heart surgery until he is six months old instead of three months old! Also, he said that if Raspberry's oxygen saturation in his blood is above a certain percentage (I think he said 70%) they won't have to give him any drugs at all when he is first born. This would be lovely.
We also found out that his head measurement is 36 weeks. He is only 35 and a half weeks, so we think that means, again, that he is growing well. We're so grateful that he's staying put, where he belongs, and growing! We have been calling him the "beat-the-odds" baby lately, bless him. He's also kicking the heck out of Janis, go baby go!

OCTOBER 24TH, 2006 - Official Tour of Sick Kids NICU (long day, long post....)
We had a big day today. We did a tour of the NICU at Sick Kids Hospital, and met with Hazel Pleasants, a medical Nurse Practitioner; Nicole DaSilva, a surgical NP; Eden Dale, a social worker ; and briefly with an Occupational Therapist named Sandy. Were also introduced to a nurse on the medical team named Kelly Roddy, who may end up being our nurse. Our friend Leslee, also an O mom (to a lovely six year old) came with us and Kelly had been her "core nurse". Everyone seemed experienced and knowledgeable and it was reassuring in that respect. We got some medical details that we hadn't known up until now. Firstly, let me (Diane) say how grateful I am to Janis for getting us as up to speed as we are, so that the NP's could then go more in-depth with the medical information.

The NICU (Neonatal Intensive Care Unit) of Sick Kids is located on the 3rd floor and is a very intense place. Access is restricted to medical professionals and parents. Siblings of the baby can come for brief visits. Other family are also allowed access but only with a parent - only 2 adults at bedside at any given time. There are up to 30 babies at the NICU at any given time. We are encouraged to bring our own baby's receiving blankets, hats, socks, stuffed animal, mobiles, pictures and anything else that might help make it more "homey".

Some new medical info - The baby's omphalocele is "giant". It will probably contain liver, intestines, stomach, gall-bladder, spleen, and maybe even bladder. They cannot know until he's born. They also won't know where the different organs are located within the sac. The major issues in ICU will be getting him to breathe (well oxygenated), grow and take in nutrition. According to the NP, the average stay in ICU for this condition is 3 months but could be longer.

The baby's lungs and diaphragm have not had any organs pushing them up into the chest cavity thus far. As a result, it may be that his lungs are long and skinny and his chest is narrow, instead of bell-shaped. As the liver moves back into the body, it can squish some of the alveoli in the lungs and compromise the flow of oxygen to the baby. We want to avoid that and other conditions like pulmonary hypoplasia. This is, in part, why he may need supplemental ventilation. He will also need energy to grow, so that his heart can be big enough to repair (if needed) and so that the skin can grow over his ompahlocele. So, he needs to find a way to get nutrients - probably an IV with TPN (total parental nutrition), and breastmilk through a nasal tube to begin until they can assess his bowel functioning. If all goes well, he may be able to just be on breastmilk and our hopes are that he will be able to take it orally sooner than later. Janis will be pumping and freezing at the hospital.

Given the size of our baby's O, it seems that replacing the organs surgically is not an option. Even the silo method would probably put too much pressure on, especially given his heart condition. The third option, called "paint and wait", involves coating the sac, keeping it moist and intact until the baby's own protective skin can grow up and over it. This can take a long time and the baby must be carefully monitored for complications that can arise, his breathing and feeding managed carefully. The covering of the omphalocele (the sac) is very delicate, and can be damaged or ruptured. Something to consider in our case is that a giant O has the whole liver in it and, if nicked or damaged, it can easily bleed. We were told this is why they generally won't let you take the baby home until the skin has granualized over the whole sac.

Health Insurance - We also found out about some costs after the baby comes home - for drugs, nursing care, medical equipment etc. They'll help us access programs, but we'll also have to buy some extended health care coverage. ACTRA, my insurance, doesn't cover it, or any pre-existing conditions. Janis needs to go back into CTYS and have a longer discussion about extending health benefits if necessary.

Big Brother Eli - We also got some great advice about how to make Eli a part of all this. Keeping him involved, happy and integral. Our hope is to maintain the most important routines for Eli: bedtime, morning, and his school (which he loves). They have a scrap-booking program for siblings every two weeks and suggested that Eli be responsible for making him a picture and/or sending a message to "his baby" everyday. We can also use cameras.

OCTOBER 11, 2006 - 32 Week Ultrasound
Diane here. Janis had an ultrasound today. She's at 32 weeks. All the growth seems normal. Lots of amniotic fluid. We were reminded of how happy the baby is while he's in utero! The O measured at about 6.6 centimetres. Janis' appointments for ultrasounds now start to speed up to once a week, plus we have ICU tours, cardio ultrasounds, and other consults. So, we're glad that Janis will be finishing work soon. Take some stress away. 6 weeks until C section...

OCTOBER 4, 2006 - Tour of Sick Kids by a Parent and her Daughter
My friend D. alerted me that she and her daughter S. were at Sick Kids. S. is an "O" baby who is now a beautiful and sweet 4 year-old suffering recently from very painful abdominal blockages and digestion problems. She was born with a giant O and a four-part heart defect so there are a lot of similarities to Raspberry. I went by Sick Kids at my lunch today to say hello. S. & D. then took me on a tour that was amazing. All through their room and level 5, to the baby nursery, the NICU (although we couldn't actually go in there) to the library/resource centre, the childrens playground (for siblings), the kids library, the stores and the cafeteria area. It's like a small town! I got to see their room and watch D. manage her daughters care with intelligence and aplomb (with what seemed like dozens of nurses, social workers, therapists, caseworkers, etc). I am totally inspired by her example. The more time I spend with little one's and parents who are dealing with so much, the more I think they are heroes.

OCTOBER 1, 2006 - Riverdale Farm Visit
Today I met in person two mom's who I've either talked with on the phone or on-line. And I met their children. L. has a 5-year-old "O" daughter. D. has three girls who are 2, 4 ("O" daughter) and 7. I can barely put in words how valuable it was to see these wonderful families in action and to meet littles ones who were also born with omphaloceles. Diane and Eli came along as well. Each of our children have (and will have) their own unique gifts and challenges. But there was a connection that was instant. I loved that the two girls got to meet each other and show off their "princess tummies" and different g-tubes. And I enjoyed watching the sibling dynamics as I have a lot of questions about Eli related to his new brother. All in all it was a priceless day. I will always be grateful to have found these two women who I now think of as friends.

SEPTEMBER 26, 2006 - Treatment Options
Here is an interesting post I've taken from the MOO list serve from a mother who is also expecting. "All the information is very overwhelming and we want to make the best decisions for our new babies and grandbabies - I know that everything depends on what happens at birth and if there are other abnormalities but this is how I understand things so far: It appears that if the omphalocele is small or medium size and contains intestines and maybe some liver that the preferred method of repair is immediate surgery as soon as the baby is born. A mesh or Aladerm may be used to help with skin growth if needed. If the omphalocele is large to giant and contains more than the intestines and there is not enough room to return everything inside it appears that either the Silo method is used or the Paint and Wait method. If I am missing something or completely wrong- :) about any of this please post a correction. We are trying to learn so that we are knowledgeable and can make informed suggestions to our surgeon".
Expectant Mom scheduled for delivery Oct 11th (I wonder how things are going for her now?)

SEPTEMBER 18, 2006 - Week 28 Ultrasound
Just finished up another afternoon at Mt. Sinai with Dr. Ryan and the high risk pregnancy unit. The latest ultrasound, like the echocardiograph, indicates that all is as it was - no new surprises thank goodness. The O is big, there is no question. But spine, lungs, head, femurs (sp?) are growing on schedule and no other indicators of trouble areas. That's good because the heart defect and O are going to be enough!

SEPTEMBER 15, 2006 - Second Echo
Had a second echo-cardiograph at Sick Kids with cardiologist Dr. Jaeggi. The Tetralogy of Fallot diagnosis remains and the heart condition is consistent with what they saw at week 19. Of course I was hoping they'd see improvement but the good news is that nothing has gotten worse. Although it is difficult to say for sure, it looks like the heart surgery will be able to wait until three to six months and that repair of the omphalocele will come first. He cautions that they will not know fully until the baby is born and the surgical teams consult. I am scheduled for a next look at 35 weeks. On a lighter note, I was feeling nauseaus all morning and acutally had to leave the table twice to throw up during the echo. The technician was very patient with me but I think the site of me rushing down the hall to the bathroom with my big belly covered in green gel was a shcok to the people in the waiting area (espeically that early in the morning). I felt better right after - although with slightly less dignity :)

SEPTEMBER 8, 2006 - General Check Up
Had appt with the midwife Aug 30th and it went well. Baby is growing on schedule - as am I - and heart rate, blood pressure and baby size are normal. He even gave a good couple of strong kicks right at the point where her stethoscope touched my belly which was fun. We have two big appts coming up: September 15th with the cardiologist at Sick Kids and Sept 18th with the obstetrician at Mt Sinai. In the mean time I continue getting as much info as I can and preparing for the fall.

AUGUST 28, 2006 The Internet
Can't say enough about the yahoo list (emailMOOs) and how it has helped in connecting with other parents and learning so very much. Feeding issues and eating disorders seem to be a big deal for many O babies. One mother that I have met from this list is also from Toronto and lives downtown like we do. She has been very excellent to talk with (through e-mail). It's invaluable to be able to compare notes about the same hospitals, same doctors, same city, etc. I now know three "O" Moms in Toronto.

AUGUST 16, 2006 - 24 Week Appointment
Just got back from another long appointment at Mt. Sinai. We seem to be getting some mixed messages about the baby's prognosis - from different doctors - even in the same team. Today's appointment was not encouraging - a lot of talk about the possible complications and risks - and we are not feeling our best. Nothing new was detected, though, and the baby is growing normally. Janis has now been recommended to leave work at 32 weeks which is the week of Oct 16th.

AUGUST 3, 2006 - Meeting with another "O" Mom
Through Dr. Chong at Mt Sinai I was connected with another mother who has a two-year-old daughter who was born with both an omphalocele and Tetralogy diagnosis. She graciously agreed to meet me for lunch as we work near each other. There is nothing like talking with another parent (mother)! I seem to have a bottomless pit of questions. She was very generous with her time. I saw pictures of her daughter who is beautiful and doing well. I am coming to really appreciate that every situation is different and every child is different and there is only so much I can understand without our little one here in front of us.

JULY 28, 2006 - Full Amnio Results
No genetic complications were detected. Janis describes this feeling as: almost getting hit by a bus on the highway. You're relieved, but then you know that you still have to make it across a highway. This is our first hurdle down in this new journey. We definitely don't underestimate the ones to come - especially that the baby makes it to term. We're doing more research, consulting with parents and sick kids doctors. This might be the last update for a bit. These appointments and tests have been intense, and there will be more to come, but now, we might have a bit of a breather. Thank you for hanging in as well.Onwards, Diane P.S. We felt raspberry's first kick this morning.

JULY 26, 2006 - Care Teams
This is what I understand: I will be cared for during the pregnancy at Mt. Sinai. The baby will be born at Mt. Sinai. Once he is stable, he will be moved across University Ave to Sick Kids Hospital (there is a tunnel between the hospitals) where his NICU care will begin and where any surgeries will take place. Needless to say I am away from work a lot these days and using up all my sick days and overtime.

JULY 25, 2006 - Talking to an "O Mom"
Through a friend I was connected to a mother whose daughter was born with an omphalocele 5 years ago. We spoke on the phone after dinner. I was very grateful to speak with her, learn about her experience and get good advice. I was also happy to hear that her daughter made it through a difficult first two years and is now doing well. But there were complications and challenges that they faced. I learned a lot and was full of admiration but at the end of the call I was feeling very tired and a bit overwhelmed. I worry that I am not up for this.

JULY 21, 2006 - First Consult at Sick Kids
We met with a surgeon and neonatologist at Sick Kids today. The neonatologist was Dr. Moore and the surgeon was Dr. Annie Fecteau. Over all, we learned that omphalocele surgery, although major, can have generally good outcomes, and success rates. She was unable to tell us much about the two in combination (heart and omphalocele). It was very reassuring to us to hear that kids don't seem to suffer from long term difficulties. Potentially not much at all from the omphalocele, and maybe some exercise limitations for the heart surgery (no rugby or football). An omphalocele generally does not affec brain and nervous system development. The organs that are in the sac are developing well and proportional to organs growing in the abdomen. We learned about the different surgical options for the omphalocele, which will depend on: 1. how close to term the pregnancy ends up being (lung development), 2. the size and health of the baby (bigger is better!) and 3. the size of the omphalocele. Looks like The baby might need two surgeries that will have to be spaced carefully by the various surgeons in consult with each other (the one team handles the omphalocele and the other the heart).

JULY 19, 2006 - Mt Sinai High Risk Pregnancy Program
Just passing on a bit more info from today's appointments. We met with the high risk OB (Dr. Ryan) and also met an ICU doctor (Dr. Young Thai) who directs the Level 3 paediatric nursery at Mount Sinai. We saw the omphalocele on the ultrasound again and it is, indeed, very large. Dr. Ryan was measured, but positive. He felt that the heart problem was serious but correctable and that lots of kids do very well with omphaloceles. The baby is doing well in utero, all the organs are developing normally. The ICU doctor was able to be a bit more specific about some possible early complications that could occur immediately after birth - mostly to do with breathing and eating - nothing that we could predict at this point. We need to talk directly to the Sick Kids surgeon and see the ICU there to find out about the specifics of our baby's surgery. We do that on Friday. Then we wait for the amnio results to rule out a few other things, and that should arrive within 10 days. So, we're waiting, at the moment. This is a totally new world, that we're in, and we're getting a lot of info at once.

JULY 14, 2006 - Tetralogy of Fallot
We just got back from a long day at Sick Kids and Mount Sinai. Yesterday was a day of positive news, but today was more sobering. We got an echo-cardiogram. They knew there was a heart defect - they spotted what looked like a Ventricular Septal Defect (VSD) on the ultrasound, but were concerned that a more serious defect, called Tetralogy of Fallot could also be present. It turns out that is the case. In isolation, it's quite correctable with open-heart surgery sometime in the first six months, but in combination with the omphalocele, it presents a greater risk. We also have to wait for the full results of the amnio, because occasionally when there is more than one system with abnormalities, there may be a genetic factor, and then there may be other systems that are affected. We won't find out the amnio results for a few weeks.

JULY 13, 2006 - Another Update
We got the initial results of the amnio, and it is normal for the chromosomes they were testing. So, one of the big worries - that there might be three chromosomes (Trisomy) of number 13, 18, 20 or X or Y - is now past. And...the baby is a boy! Tomorrow we are having a fetal echo cardiogram at Sick Kids, and will get the results right away. The issue with the baby's heart is the next big concern, and we're really glad that we can get answers this quickly. Shortly thereafter we'll be meeting with the Natal ICU doctors, and the surgeons at Sick Kids. We know we have a long road, but are pleased that it doesn't end now.

JULY 13, 2006 - Lots of Medical Appointment
An omphalocele is an opening in the abdominal wall wherein as the baby developes in utero, some organs are growing outside the abdominal cavity in the umbilical cord. An omphalocele is serious in and of itself but can be an indicator of other problems and/or syndromes including heart and chromosomal abnormalities - that all have to be explored/ruled out. I've been going to lots of appts and having tests that are all urgent and that are scheduled on short notice.

JULY 12, 2006 - Amniocentesis
An amnio is quite an amazing procedure. They insert a long needle into your stomach and extract amniotic fluid from the uterus (from around the fetus). Living cells from the fetus float in the amniotic fluid. After a sample of amniotic fluid is removed, these cells are grown in a laboratory for one to two weeks, then tested for chromosomal abnormalities or various genetic birth defects. By the way, I found that this procedure didn't hurt at all. I was advised to take the day off and rest. It can take up to three weeks for the full results to come back but, as this is urgent, we will be getting initial results (a FISH test) hopefully tomorrow or Friday that will tell us whether there are trisomies present. [Genetic Trisomy Every human cell contains 46 chromosomes, arranged as 23 pairs, with one member of each pair inherited from each parent at the time of conception. A genetic trisomy means that where there should be a pair of chromosomes there are instead three and that indicates the presence of a genetic defect or possible syndrome. Trisomy 21 for example, is an indication of Down Syndrome].

JULY 11, 2006 - Genetics Testing
Had a level II ultrasound and an appointment with Dr. Chong of The Prenatal Diagnosis and Medical Genetics (PDMG) Program of Mt Sinai Hospital today. This ultrasound on revealed complications with the baby's heart. That is not great news and will need further exploration. She is also arranging for an amniocentisis. Omphalocele's often are associated with heart defects and chromosone abnormalities so it's important to do a full chromosone scan. Apparently some of these abnormalities are "incompatible with life" which would mean early termination. Very frightening.

JULY 5, 2006 - An Omphalocele
Had an appointment with Joyce Coombs at the Midwives Collective of Toronto this morning to discuss prognosis and next steps. I went with my mother as Diane is still away. Joyce has been a midwife for many years and is one of the best in the city but she has never herself had a client with an omphalocele baby, although the clinic has, many years ago. She has set me up an appt with the High Risk Pregnancy Unit of Mount Sinai for July 19th - which seems a long time to have to wait. In the mean time, we will try to get as many tests done in preparation.

JULY 4, 2006 - The First Sign
Had 18 week ultrasound this morning. Later in the afternoon I got an urgent call from my midwife (Joyce Coombs) to tell me that they detected an omphalocele, a defect wherein internal organs are growing outside the abdominal wall. The abdominal wall has not closed properly or has a hole(s). It's serious. And can be an indictor of other issues (like heart or chromosome irregularities). I have left work (very upset) and am at home where I have been able to talk more with the midwife, contact Diane (who is working in Montreal), and do more research on-line. I have an appt with Joyce first thing tomorrow and she is getting me an appt with a specialist at Mt. Sinai asap to assess the condition in more detail.