Friday, July 24, 2009

Counting Down

It has been nearly 5 days since I've landed in Philly (really Camden, NJ since that is where I'm boarding).  As usual, it has been a whirlwind of a week.  One would think I would get used to this rollercoaster ride experience of carrying a child with an unknown syndrome, but I am not numb to it yet and they certainly catch me by surprise when something new or unexpected is thrown my way.  Let me explain...  My sister, Gina, and I went to CHOP for an appointment with the maternal fetal medicine (MFM) Dr. on Tuesday.  This was just for a routine growth sonogram to check the baby's development and amniotic fluid levels.  The Dr. said my ears should have been ringing lately because Ella has been the talk of the hospital.  There has been great debate over whether or not to do the Exit procedure as a mode of delivery.  Though they initially thought it was not necessary, the airway team seemed to have second thoughts.  Their argument is that it is the most controlled situation, since the baby will be oxygenated through me while they are trying to access the airway.  They also are worried that if I go into spontaneous labor that they can't guarantee that the right docs will be available to help her.  On the other side, are the OBs/MFMs and the hospital's chief of surgery who feel they will have sufficient time to access the airway and that an Exit is not necessary.  Each team is coming from different perspectives, with the airway team considering doing what's best for the baby and the OBs considering what's least risky for the mother.  And of course, my husband and family were adamantly opposed to the Exit since it is a rare procedure with higher chances of blood loss, hysterectomy, and morbidity.  This put me in a most difficult position because I want to do everything I can to protect Ella, but I also have to consider the feelings of my husband and family. 

I would be lying if I said I wasn't scared...if we don't do the Exit there's a chance that the baby will suffer the effects of oxygen loss and if we do it there's a chance that I will suffer the effects of blood loss.  After our meeting on Tuesday, I had this completely unsettling feeling.  Nothing had changed with the baby in the last 4 weeks for them to turn the tables on me.  Ella grew significantly (now weighing approx. 4lbs) and the amniotic fluids were normal, suggesting that she's still swallowing.  So we met with the airway and OB docs again yesterday.  I expressed my concerns much more clearly this time, as I was no longer caught off guard and could gather my thoughts and articulate myself in a better fashion.  It was obvious that the two teams were on different pages.  It was also obvious that one of the major reasons that this was even being discussed was that the ENTs were not able to guarantee that their airway experts would be available upon normal delivery.  We came to a compromise - if an Exit is the most controlled situation and a vaginal delivery the least, a cesarean was somewhere in the middle. 

On Thursday, August 6th, I will be having my baby via c-section.  I am praying to God that they will get her breathing right away and that the worst case scenario is that they will have to trach her.  There are so many unknowns with this child, however, one thing we do know is that we already love her dearly and we cannot wait for her arrival.  In less than two weeks, we will meet her for the first time.  The waiting and anticipation will be over with and we will just begin to know better what her issues are.  Her birthday will be the first day of a very long journey, equally beautiful and challenging I am sure.  I just ask that you continue to keep this little girl in your heart, praying to whomever your God that her delivery goes smoothly and that she has a chance at having a good quality life.  Also, Erik, myself, and my family are not nearly as strong as everyone thinks we are.  In fact, there are many of days that I feel like buckling, like I cannot handle one more stitch of bad news.  So we just ask that you pray for our strength and courage to get through this adversity.  We still have faith that a miracle can happen, that God can heal baby Ella.  If that is not His will we will cherish her and love her with all of our hearts just the same.    

Wednesday, July 8, 2009


My mother and I just returned from our second trip to Philly.  It was a mentally exhausting two days, meeting with doctors, midwives, social workers, and digesting the reality of what our lives will be like for the next year or so.  We first met with the maternal fetal OB and it looks like things are status quo with the baby.  Fluid was seen in her stomach, which is a good sign because it suggests that she is able to swallow.  Though she is still extremely small (below the 10th percentile), she did grow from our last visit and is now weighing in at 3lb 2oz.   Docs will continue to monitor the fluid levels and her growth, as it will determine whether or not we do an Exit procedure and when she will be delivered.  If her growth plateaus then they may deliver her early because she’ll have a better chance of growing outside the womb.

We also met with the craniofacial specialist.  Without a normal size jaw, Ella would never have a chance of breathing on her own.  They have several ways to correct the jaw, however it completely depends on how much bone they have to work with.  They do what is called a jaw distraction: Under anesthesia, the surgeon cuts the jawbone on both sides of the face.  He then anchors an appliance to the bone and turning a screw on the distractor gradually increases the distance between them daily. New bone is formed in the gap as the gap widens. Once the desired length is achieved, the turning stops and the device is usually left in until the bone is stabilized. This is usually 6-8 weeks, but it varies depending on the child.  If there is enough existing bone, the distraction surgery will take place a 1-2 weeks after birth.   

If there is little to no bone to work with, then the surgeon must wait until there is some growth or they have to borrow bone from another part of her body.  In that case, she would have to be trached, because intibation is not sustainable over a long period of time. 

The lovely people at CHOP gave us a tour of the Special Delivery Unit as well as the NICU.  It was definitely a reality check for us.  The room where I will deliver has a window through which they will quickly pass Ella once she is born.  Waiting on the other side will be an airway team made up of ENTs and other docs, who will first try to intubate her.  If the intubation is unsuccessful, they will then try to trach her.  The number one priority, of course, is getting this baby oxygen.  Unfortunately, we learned that we will not be able to hold her for some time, at least until she is off the ventilator.  This is extremely upsetting to us because as parents-to-be you long for those first moments when you can look into your baby’s eyes, cradle her in your arms, and caress her.  However, we know it is for her own good and, luckily, we will still be able to look at her at touch her.  

Equally upsetting was seeing all these sick, tiny, helpless babies in the NICU and knowing that my baby will soon be one of them.  You can’t help but want to pick up each and every one of these children and whisper in their ears that everything is going to be all right.  Though I know my baby will be in the hands of expert doctors and compassionate nurses (you have to be to work in a NICU), I still wish I could hold her in my arms and take her home like most other parents can.      

And so the decision was made that I am due to move down to Philly on July 20th, when I’ll be 35 weeks.  It seems I will be there for quite a while, roughly a month before she is born and likely several months or more after her birth.  Before birth, I will be staying at the Ronald McDonald House in Camden, NJ.  Once the baby comes I will be moving to the Ronald McDonald House in Philly, which is only six blocks from the hospital.  I will have some time on my hands and rather than letting all this worry consume me, I would like to find things to occupy myself.  If anyone has any suggestions (a good book, movie, hobby) or would like to come visit I would be absolutely welcome to that.    

Finally, let me end by saying thank you for the outpouring of love and support.  Thank you for your many phone calls, emails, comments, prayers, and offers to help.  If I haven’t responded to you, I apologize.  It is still very difficult for me to talk about without breaking down.  I thought with each passing day that it would get easier, but with the reality of our future setting in it remains tough.  I know this will eventually change and that once this child arrives we will not only accept our circumstance, but truly embrace it.  Please know that hearing that so many people care about Erik and I and about this little one, whom you have yet to even meet, brings us great comfort. 

Love and God Bless…


P.S. A few people had mentioned they are having problems posting a comment on the blog site.  I amended the settings to allow all people to post, regardless if they have a Google account.

Monday, July 6, 2009

33 Weeks

I decided to follow my gut instinct and go down to Children’s Hospital of Philadelphia (CHOP) to get a second opinion for baby Ella.  We originally decided to go down to find out whether an Exit Procedure was necessary as a mode of delivery.   An Exit Procedure is performed mostly in cases where the baby’s airway is obstructed.  In our case, it is the micrognathia (severely small chin) that made Dr.s in NY think that the baby would have a hard time breathing on her own.   It is similar to a C-section, however the mother is put completely out, an incision is made, and while the baby is still partially inside the uterus and connected to the umbilical cord they try to intibate or trach her.   So if all goes well the baby is not without oxygen for even a second.   However, the mother is given a specific anesthesia to keep her uterus big and relaxed - which in turn increases the chances of heavy bleeding.  This is an uncommon procedure, with the risk of morbidity mostly to the mother due to possible hemorrhaging.   CHOP has done over 80 of these surgeries, whereas hospitals in NY have only done a handful.  

So the good news (at least for now) is that CHOP docs did not think that I needed an Exit.  The sonograms and fetal MRI showed that the baby is swallowing amniotic fluid, indicating that she may be able to breathe on her own.  They will have to reassess the need for an Exit if the amniotic fluid in my uterus starts to rise.  In a few weeks, I will be relocating to Philly so they can monitor this situation more closely and so that if I go into labor early I will be close to the hospital.

The bad news is that this baby likely has one of three syndromes – all of which are extremely rare and serious.  They mostly involve facial and limb defects.  However, one of them, Fanconi Anemia, can be fatal as it is associated with blood and other types of cancers.  Right now they think that her right thumb is underdeveloped and they don’t see a thumb on her left hand.  Her ulnae are also way behind in growth and she has a few vertebral anomalies.  They also are concerned about part of the back of her brain.  They are not sure if the black on the scan is an indication of fluid or underdevelopment. 

As you can imagine, Erik and I are shocked and devastated by this completely unexpected news.  Our feelings are so volatile right now, one moment feeling proactive, the next angry, and the next sobbing.  And while I know that this baby is still blessing and will serve a very special purpose in this world, I cannot help but feel pain for her already.  I am her mother and it absolutely kills me to think that she will suffer or that we will lose her.   We are trying to take it day by day and think positive, but this is hard to do when all we read or hear about these syndromes is terrible.    

This is the most difficult thing I have ever had to endure in my life.  I know that God has a plan in all of this, though I am not yet sure what it is.   Perhaps it is to bring us closer to Him, to each other, or to show His love through this little spirit.   I truly feel he is giving us this baby because he knows we will not forsake her.  He knows that through all the trials and tribulations we will face we will continue to love and care for her, showering her with compassion and affection every step of the way.   

So we thank you all from the bottom of our hearts for your continued love, support, encouragement, and most of all for your prayers.   Erik and I are extremely grateful to have one another and to have such loving family members and friends.   There is no way we could get through all of this on our own.  While the road ahead of us will not be easy, I know in my heart we will get through this and that we will eventually smile and laugh again.   No matter what illness plagues this baby, she is still ours and she is still a precious human being, a beautiful blessing sent to us by God.  We already love her so much and will continue to love her unconditionally.