Celebrate!

William enjoying his First Birthday present!

 On this day of celebration, I also wanted to share William's story with everyone.  Below is my blog I submitted to a blog that isposted in the EA/TEF community.  It is the story of William's first year of life.  Thank you for all the prayers and support.  Our sweet baby turns One!

Hope

Hope is the thing with feathers
That perches in the soul,
And sings the tune--without the words,
And never stops at all,
And sweetest in the gale is heard;
And sore must be the storm
That could abash the little bird
That kept so many warm.
I've heard it in the chilliest land,
And on the strangest sea;
Yet, never, in extremity,
It asked a crumb of me.

Emily Dickenson                                                                                  

 “You are miscarrying your baby” is the news we received at my 5 week ultrasound and doctor visit.  My husband and I were expecting our 3^rd child and knew there was something wrong when the ultrasound technician did the ultrasound.  When the nurse came in and gave us this news, I broke down.   My HCG hormone levels didn’t double during the week and they gave us this as our only explanation for what was happening with our unborn baby.  I was told to go home and consider a D&C the following week if nothing occurred over the weekend.  After talking it over with my husband, we knew we couldn’t wait weeks for nature to take its course; so I made the call on Monday to my doctor and asked to schedule a D&C.  I asked for a second ultrasound to confirm there was no baby and was told it was not necessary.  I let the nurse know I would call if necessary to have the second ultrasound and she conceded and I had an appointment for the following day.

The morning of the ultrasound, hope is what helped me get out of bed…Hope that they were wrong and that our child was still alive inside me.  My sister was the technician who did the ultrasound at a high-risk perinatal center and after rechecking we heard the beautiful words “there is a heartbeat”!  The three of us cried and celebrated. Our baby was alive – my pregnancy was a week later than originally thought – and our baby was alive!! 

At this point, we thought our worries were behind us even after switching to a new OB/GYN and having been told to “take it easy” for the remainder of the first trimester.  Little did we know there was so much more around the corner…

The perinatal center wanted to do my 20 week ultrasound as a follow up since I came in at 5 weeks.  The morning of the ultrasound appointment we were so excited to see our sweet baby and have relief in knowing how he or she was doing and growing.  During the ultrasound, we asked a lot of questions and started to get difficult news.  By themselves, the news we received were not concerning, but because they were seen together, it was of concern.  We did find out the wonderful news that our baby was a boy!  Our son had a single-umbilical artery and excess fluid on the kidneys.  Together these were a soft marker for downs syndrome.  We met with the genetic counselor and made the decision not to have testing to confirm downs syndrome.  The repercussions of doing an amniocentesis were too great and it didn’t matter to us; we knew God made him and he would be perfect, and that God would help us through the road ahead. 

At the time, we were devastated to hear this news and needed time to digest it.  As a parent, all you want to hear is that you are having a healthy child…it is heartbreaking to hear that your child will have physical or mental obstacles to overcome.  Due to the information gained in the ultrasound, the doctor’s wanted to observe William to see if the fluid levels decreased and to be prepared in the event they didn’t.  Looking back, had it not been for these soft markers, we wouldn’t have known about EA/TEF and would not have been prepared (as much as one can be).  Even more so, had I not been monitored as closely, I would have gone into labor earlier and William would have faced many more challenges than he already was facing.  Gradually, the fluid levels on the kidneys decreased and before he was born, they had been within the normal level for a few weeks.

I was to be monitored every 4 weeks at the perinatal center; but at the 24 week ultrasound they found my fluid levels had increased faster than expected.  I was scheduled to return every 2 weeks.  At 28 weeks, my levels entered a danger zone and I was put on modified bed rest.  I was also now scheduled to come in every week for an ultrasound, a non stress test, and fluid level check.  At 29 weeks, we had to make a decision to have a therapeutic amniocentesis.  It was a difficult decision because the risk of something happening to the baby is so high.  We felt it was the best decision though, because, in this case; the risk of doing nothing put our baby in greater danger.  The amniocentesis threw me pre-term labor and I was admitted to the hospital.  They were able to control my contractions and make them stop.

At 33 weeks, my levels got into the danger zone again and another therapeutic amniocentesis was necessary.  Again, I was thrown into pre-term labor and needed additional medications to make the contractions stop.  Magnesium-sulfate was used, and although it was awful from my perspective; it made the contractions stop.  After two nights in the hospital, I was released and knew if I could make it to 35 weeks; it would be safer to deliver.

At this point, I was going to the perinatal center 2 times a week.  The day of my 35 week ultrasound arrived and I felt it was the day our son would be born.  He wasn’t moving as much as he had been, and although he was making the required “kick counts” he was less active.  I was extremely worried there was something wrong….something just didn’t feel right.  The doctor spoke with me and let me know I was not in labor and our son was okay.  I could go home and wait until the next ultrasound…at my insistence and through my tears; the doctor said I could go to the labor and delivery floor for a few hours until I felt okay enough to go home.  After being on the floor for 2 hours, I went into labor.  The doctor on-call and all the residents tried to stop the contractions without the use of medicine but they continued.  That evening, the doctor came in and let us know we would do a c-section in the next hour.  My husband had just left to go pick up our daughters, I called him and told him to come back; that William would be here in an hour!  He thought I was kidding and the doctor, standing at my side, said “Brian, it’s no joke, come back”.  Our family came to the hospital and we were all joyously eager to meet William; we knew it was likely to be a difficult journey ahead; but so badly wanted to meet our child.

My husband and I already met with the Neonatologists, the pediatric surgeon, and had a tour of NICU.  We were as prepared as we could be in the event our son was born with EA/TEF.  I had hope that everyone was wrong and that he would be born completely healthy.  I remember visiting all of the specialists and the NICU thinking, I’ll take in all this information, but I’m hoping the whole time that I never have to see you again…odd, that in the delivery room the Neonatologist was there and all I felt was relief.  A team of nurses and doctor’s were there for our son, and after waiting what felt like an eternity, we heard the most beautiful sound a mother can hear – her newborn baby’s cry.  When the nurse brought him over, I saw his sweet face and let him know how much I loved him. After I was released from recovery, they wheeled me in to see our son.  He was so beautiful, and looked so small in the bed.  He was already hooked up to a few machines and had the ng tube to keep his airway safe. That night, I could not sleep.  I missed my baby and hated to be away from him even if it was just by a few floors.

The next morning, my husband and I made our way to NICU as early as we could and met William’s day nurse…a nurse who would come to be one of his primary nurses and who helped us so much during his stay in NICU.  She let us know the surgeon was going to my room to talk about the surgery he wanted to do that morning and that he would be back shortly.  She must have felt the pain in our eyes and asked if I wanted to hold William before surgery.  I eagerly said yes, and for the first time I was able to hold our beautiful baby boy.  I remember the surgeon coming to his room, talking to us about the surgery, and saying a prayer with us for William.  While tears rolled down our faces, I prayed for God to take care of William and for the surgeons to be able to make the connection successfully.  Brian got to hold William as well, and he went to the OR with the team of nurses while I went back to my room.  William wasn’t 18 hours old and was going into surgery; we didn’t say much during the 2 hours it took.  When we got the call that William was on his way back, we rushed downstairs.

I recently read a quote that said “You don’t know how strong you are until being strong is your only choice”.  The sight of your newborn hooked up to all those machines is startling and difficult.  We couldn’t hold him for a few days because he needed to remain still after surgery and to help not disrupt the life-giving machines he was hooked to.  The night before I was discharged from the hospital, I broke down during dinner and continued to cry for 24 hours.  I cried because I couldn’t handle the thought of leaving my baby in the hospital, I cried because I knew I had already been away from our daughters so much and they needed me too, and I cried because my heart broke for our son who had already gone through so much and such a long road ahead of him.  I cried so much the skin under my eyes was rubbed raw.  The day of discharge, my doctor came in and, at the sight of me, gave me a hug and talked to me about the road ahead in NICU.  He explained how hard it was going to be and let me know I could take my time leaving the hospital.  My husband and daughters came up and we lingered in William’s room for a while, ate lunch, and went back to William’s room.  I couldn’t bear to leave him there.  I felt as if part of my heart was staying at the hospital and part of my heart was in the car going home – my sweet daughters.  That night, we had dinner at home with our daughters and then my sister watched them so Brian and I could go back to William.  I practically ran to him, it was so hard being away.

The next month had its ups and downs.  We felt as if William would take 2 steps forward and 1 step back.  He had his 2^nd surgery before he was 3 weeks old; a g-button installed due to his left vocal chord being paralyzed.  He was aspirating on all liquids and had to be fed via his g-button.  I felt constantly torn between our daughters needing me at home and our son needing me at the hospital.  I just wanted our family to be together in one place – home.  Finally, we received great news – William could come home 4-1/2 weeks after being born.  On May 19 (his original due date) William came home for the first time – what a wonderful day!  Although William is our third baby, we both felt like we were coming home as new parents that night…and as excited as we were, we were scared. There is a safety of having your baby in NICU – the nurses and doctor’s are there 24 hours a day to help your baby and honestly, to help you.  We felt the weight of caring for William on our shoulders.  Even with all the scared feelings, we were thrilled beyond belief to have our family together for the first time.

In June, William had his first dying spell during one of his tube feeds.  I started CPR and by the time the paramedics arrived, he was breathing again and acting normal.  After a call to the doctor, we made our way to the ER and spent the night.  The doctor’s couldn’t figure out what caused his dying spell, and since he didn’t have another one in the hospital, we went home.  Brian and I are terrified of another dying spell, so William is never out of our sight – literally.  We have a baby monitor that is a video, we don’t shower unless the other one is home, the bathroom door stays open…you get the idea.  I feel like God has helped me get to this point.  The weekend before William’s dying spell, I told Brian “ I feel like we’re going to need to do CPR on William, something just tells me we will” and so I began to replay the actions we learned in class.  Thankfully, it was fresh in my mind that day…  I am also so thankful for our wonderful daughters.  When I knew I needed to start CPR, I told them firmly to go to their room – and they listened.  Without knowing what would happen, I needed them to be safe; safe from seeing William get CPR, safe from the front door being open and letting the world in, safe from what would happen when the paramedics got there…safe.

There are many ways children deal with having spent so much time in the hospital.  Our daughters accepted it as a way of life and never saw anything but love for William.  Frequently, they would play “house” with their dolls, keeping the bulb syringe nearby in case “their baby spit up” and they would rush in to save them.  The girls also checked pictures of themselves as babies for their g-buttons and saw William as nothing more than their new baby brother.  Love, it is so wonderful!

July brought a wonderful phone call…the team in Boston returned my call 20 minutes after I left a message.  Dr. Jennings, Dr. Manfreti, and Dorie were all on the call and listened with such care and love to William’s story and problems he was facing. His tracheomalacia was terrible, feedings were awful and seemed painful for him, and he continued to have constant blue spells (not including the dying spell the month before).  They listened and told me the best news a parent can hear “…we can help William; we can help him breathe and lead a better quality of life.”  Amazing!  We talked, had all of William’s files, photos, and scans sent to Children’s Hospital Boston and planned our trip.  We knew we had to go, but we weren’t exactly sure how we would get there.  In the end, we took from Brian’s retirement account and planned to use that as our money.  Brian’s grandparents offered us money and my company offered to pay for our hotel and gave us money for the trip.  We are beyond touched by this help for our son.  We were scheduled to leave on a Friday evening and drive through the night.  That afternoon, our eldest daughter started throwing up with the stomach flu…then I got a call from Dorie that William’s appointment needed to be rescheduled due to the surgery schedule…intervention from on high?!  Yes!  The following week brought the flu to our second daughter and, by the time we were scheduled to make the trip 2 weeks later, everyone was healthy again. 

We made it to Boston for William’s appointment on Halloween.  The girls Trick – or Treated with Brian through the hospital and William and I spent the day in and out of appointments.  He was scheduled for tests and dilation on November 2.  We spent November 1 sightseeing and having a great family day.  On November 2, William and I went to the hospital and he had his first dilation.  There are few feelings like having to give your child to the surgery nurse.  Your heart is pulled from your chest and you never want to let go.  William did well during the procedure and went to NICU for recovery.  He had a terrible time coming out of anesthesia; so much that we had to leave NICU so the nurse could help him come out.  That evening, while we were eating dinner, William had another dying spell.  The nurses helped him recover but the doctor’s wanted to do surgery to help his tracheomalacia – an aortopexy.  Because of the delay in the trip, Brian and I were able to meet with 2 surgeons in STL who were able to do the aortopexy.  We knew we wanted to return to STL so we could be closer to family during the surgery and recovery and so the girls could be at home.  William had to spend 24 hours in NICU from the time of his dying spell and we had to sign a lot of paperwork to allow them to release him.  We know the fear and concern the doctor’s had releasing William, we felt that fear daily…the fear of another dying spell and the fear of knowing we might not be able to reccessitate him…but we knew we had to do what was right for our whole family. 

The Boston trip was extremely beneficial as we found out that William had significant trancheomalacia – his trachea was completely collapsing during anesthesia…and it was clear this was an even bigger issue when he was awake and trying to move, eat, breathe…live.

The trip home was LONG – William had trouble eating and needed to go back on tube feeds (we worked hard in the late summer to wean him from the tube on he was fully breastfeeding by this point) and everyone was worn out from the trip.  We made it home and scheduled the aortopexy for November 16. 

On November 13 William had another dying spell, and this one was worse than the one in June.  It took a round and a half of rescue breaths and compressions before he started breathing on his own again.  We went to the hospital via ambulance and William had an x-ray the next morning.  The cardiothorasic surgeon came in on Monday and said we were moving the surgery to the next day.  Brian was home with the girls and I stayed with William.  I remember asking his wonderful nurse if I could go take a shower and if she would stay with William (we couldn’t leave him alone because of the dying spells).  I walked to the Ronald McDonald Family Room, had what seemed like a long tour (as I held back tears) and as soon as I closed the door to the shower; the tears began to fall.  I cried from fear of having the surgery moved up so fast, for fear of knowing our baby was having yet another surgery, and fear of another dying spell if he didn’t have surgery.  I love him so much and the thought of him not making it through surgery was too much to bear.  Those Family Rooms help in more ways than just providing a place for showers, laundry, and food…they give solace to families.  I felt better after a hurried shower and went back to William.  The next day, my sister stayed with our daughters and Brian came to the hospital to spend the day waiting for surgery.  The surgery was pushed to the evening due to complications of other surgeries.  Again, we had to hand William to the surgical nurse – tears flowing and heartstrings pulling.  We made our way to PICU and waited for the doctor to come out.

Shortly, very shortly!, the surgeon came to tell us the surgery was successful and we could go to William.  He was sleeping peacefully in PICU under pain medicine.  We were talking to his nurse when one of the doctor’s came in and told us one of his lungs was collapsed.  This had not happened before, and we weren’t sure what to expect for recovery.  The next day, William was having a hard time coming out of the pain medicine and dealing with the pain.  He started having dying spells and for the next 2 days had them anytime there was a loud noise, talking, or sudden sounds.  It was awful and we felt helpless knowing we had to give him time to heal; that more narcotics would be worse because he would eventually have to come out of them and have these same problems.  Finally, midway through the week he started to stop the dying spells and gradually recovered.  We went home that weekend with full tube feedings once again.  Thankfully William had the g-button because, without it, he would not have gotten to come home so quickly.

The aortopexy made a world of difference for William.  At this point (7 months) he had not started to roll, couldn’t handle lying on his stomach, and was constantly just trying to breathe.  After the aortopexy, he started rolling, he was silent (rarely could we hear the tracheomalacia), and he started smiling more!  He could finally breathe for the first time in his little life!  We thought he was in the clear and were looking forward to progression.

Little did we know, yet another surgery was waiting for William.  On December 15, William had another dying spell.  This was the worst one – 3 rounds of rescue breaths and compressions.  It was to the point that I remember thinking I wasn’t going to be able to reccessitate him and that I would focus on maintaining blood flow and oxygen to his body.  The paramedics could not get there fast enough.  This time was also different for William.  In the past, his body went rigid during his dying spell – like he froze in time.  This time, his body went floppy and even when he started to breathe on his own; it was an awful moan escaping from his body.  It was so awful, I didn’t stop CPR right away because I thought it was air escaping and not real breaths.  He finally started to act more like William and we went to the ER for the second time by ambulance. 

I am struck by the rareness of William’s anomaly when we go by ambulance and when we are in the ER.  The paramedics always ask me to spell and respell words like tracheomalcia and finally, they ask if I can talk to the nurses on staff as his history quickly fills the small piece of paper they are using to write.  The ER staff is similar with the exception that most nurses have heard of EA/TEF, an aortopexy, and know how to work the g-button.  We have had one nurse tell me (kindly, not trying to be mean) that she was glad for the opportunity to care for William as she had only read about EA/TEF in nursing school and had never seen a child with it.  White I am not sure how I should respond to this, I try to understand and answer the same questions over and over as different nurses come in the room. 

After this dying spell, Brian and I met with William’s pediatric surgeon and GI doctor to discuss tests he needed to have to determine if reflux was the cause of the dying spell.  In early January, William started off the year with his second dilation and tests to determine the need for another surgery (Nissen Fundoplication).  He had a pH probe (he managed to keep it in for 18 hours before pulling it out himself!), a gastric emptying test, and a swallow study.  The results told us that he had significant reflux and did not have delayed gastric emptying.  When we met with the doctor’s, they told us their greatest concern…that William would have another dying spell overnight, and his apnea monitor would malfunction and we would wake up in the morning to find William dead.  Honestly, that was all we needed to hear to make the difficult decision on the surgery.  Brian and I had been wavering due to the complications from the Nissen Fundoplication. We scheduled the surgery for the following week.

This was William’s 4^th surgery and he wasn’t 9 months old yet.  We were worried about the complications that go along with any surgery as well as the complications from the “Fundo”.  Prayers streamed in from family and friends.  These prayers helped us get to this point and helped us get through the difficult times.  William’s surgery was on Valentine’s Day.  His surgery was over quickly and was thought to be successful.  He started to recover fairly well and even tried to nurse a little that evening.  The next day, William started to take a turn for the worse and would continue down that road for the next 4 days.  Blood was taken to determine if he had a virus or a bacterial infection.  Finally, midway through the week we found out that William had HMPV – a nasty cousin to RSV.  Because of the HMPV, he developed pneumonia.  He stopped eating orally and even refused his pacifier.  He didn’t want anything in his mouth and was so listless.  It is heartbreaking to see your child like that and not be able to do anything more to help.  Over the weekend, William started to come around and by Monday was doing well enough to get to come home.  I tried to nurse him, and he started to choke – so we were back to full g-button tube feeds.  He was not completely himself, but we were eager to go home. 

We started working weekly again with William’s nutritionist (he had been doing well enough to go to a biweekly appointment) to make sure he was gaining enough weight. He lost almost 2 lbs during his hospital stay.  I was pumping to try to keep up the breastmilk supply and was successful so far.  The following Monday, we went to a follow up visit with the feeding team.  He did well during the swallow study and was doing well during the feeding visit.  He ate a cracker (even though I was terrified to try!) and stage 2 baby food.  We knew it was going to be a long road to get him eating table food but at least he was back on that road!

William also had trouble with sleeping at night.  On a good night he was awake every 2-3 hours.  On most nights, he was awake every hour or less.  Because he was on 24 hour tube feeds the week he came home (to maintain minimum calorie intake); he slept through the night for the fist time in his life!  Everyone was eager to have him sleep through the night – he needed the sleep to develop and grow.  Sadly, my milk supply was not strong enough to nurse him during the day and provide enough to fill the feed bag at night.  We had to supplement with formula – when William was in NICU he was on various formulas to try to get him to gain weight and all caused extreme distress.  This formula was no different and by the second week home, he wasn’t able to sleep through the night due to stomach pains.  We stopped the formula and saw some relief but no more sleep.

We started working on crawling with his OT and continued on trying to get more calories through table foods with his feeding therapist and nutritionist.  Roughly 1 month after surgery, William crawled for the fist time!!  He was 10 months old and it was wonderful to see!  He also started to eat more and more table foods – and gradually moved away from baby food.  We can not say how grateful we are to First Steps for the help they provide.  From day one, William’s therapist and nutritionist have been indispensible in helping William get to where he is today.

Tonight is the eve of William’s first birthday.  All week, I have been replaying last year’s events through my mind and heart and find myself very emotional.  I remember the pain of handing him over for all his surgeries and of having to leave him in NICU.  I also remember all the smiles and sweet laughter we hear from him.  I see the way his sisters love and care for him and how they have grown because of this past year.  It was a year of challenges but it was also a year where we grew closer as a family.  Hope is constant in my soul; God blessed us with a wonderful son and although life is now forever changed for our whole family – I know God always had our son in mind for us.

We know this is just the start of the path for William; but instead of having fear for the unknown, we try to have hope that God will help him through and help us as a family face the challenges ahead.  We have a wonderfully supportive network of family and friends and without all of the prayers, meals, sleepovers, hugs, and tears; I’m not sure where we would be.  One of the best memories I have of the past year – hearing William laugh and seeing him and his sisters play…seeing him enjoy life.  He is amazing, he is an inspiration, he is a miracle.