A roller coaster of a ride

 

I have a beautiful friend who always says that dealing with CCHS (Congenital Central Hypoventilation Syndrome) is a roller coaster of a ride.  That’s my opinion also, and it’s 100% true.  Life with Mighty Z has been a roller coaster on so many levels.

Just like a roller coaster car climbing the rails does, Mighty Z has brought me so many butterflies in my stomach: butterflies of proving the doctors wrong, butterflies of the inchstones that Mighty Z has met with her gorgeous smile on her face, and of course the butterflies I still get every time she opens those amazing eyes in the morning ready to seize the day.

Just like you feel being on the very tippy top of a roller coaster, able to see the whole city, so I have felt many times that we are at the pinnacle of this disease and can see how far we’ve come, how hard we have worked just to breathe.  I am in awe of not just Mighty Z, but also of the family God has given to us to help us achieve these goals.

Yes, and then come the scary dips and it doesn’t matter how many times you have ridden that roller coaster, those dips still take your breath away.  The same is true with CCHS.  No matter how long we have walked this road, those dips, dives, and sharp turns always jar us. However, then we start climbing right back up and hit the top once again.

When the ride is over, instead of getting off and lamenting about how fun and yet scary the ride was, I get to ride again and again. Yes, CCHS is a roller coaster of a ride; however, it is the best ride in the world.

 

01 02 12

Educate and Advocate

My partner and I started a organization. called Hope's Seed. Hope's Seed was founded in February 2013, to meet the needs of parents of special needs kids by focusing on local, face to face support and care.


Hope's Seed plants a seed of hope in parents by telling them "You're not alone!"  We all know what it is like to experience difficulty on the journey of raising our children with special needs, and so we created this organization to support and encourage each other, and to offer hope.


Each month Emily and I come up with a topic to talk about this month (March) we are talking about advocacy. Here was one of my post.


Please like us on Facebook and help show other parents of special needs that they are not alone.






Sometimes, advocating for your child can be difficult.  Standing up to a doctor and telling him or her how to care for your treasure can feel intimidating.

Many times I still feel intimidated when I speak my mind. Lots of things go through my head, like maybe I am wrong- after all they are the doctors they should know what they’re doing. I have come to the understanding that many times doctors have no idea how to handle our children and so they are just winging it.

 

In the last post we talked about how gut instinct mixed with knowledge is your biggest weapon. One of our parents who has a child on a ventilator felt that he was on the wrong ventilator.  Her gut was telling her so; however, she read up and asked other parents with children with the same diagnosis as her child. She took a deep breath and went and told the doctors she wanted her child on another ventilator. You know what?  She was right!! Her child improved quickly and drastically.

 

Standing up to the white coats (aka doctors) is hard, especially if you are questioning yourself.  However, remember that the doctors are working for you (you are giving them a paycheck every time they walk into your room), and there is a reason they say they are practicing medicine and they do not say they have mastered medicine.

 

Think of it like this: your child is your business and you are the CEO of the business, and the doctors are paid contractors. They cannot do anything to your child without your consent.  In fact, you can choose to move your child to another hospital if you do not feel that your child is being properly cared for.

Here are some key points:

Your Doctor does not know everything.

You are the leader of the team that is taking care of you child.

What you say carries more weight than what the doctors say.

Gut instinct and knowledge are the keys to caring for your child successfully.

Take a deep breath.  Know that you are right; tell the doctors what you want and do not back down

 

01 02 12

I Love Liam Lyon

I started following I love Liam Lyon right after he got his trach ( I have a passion for trachies since Mighty Z had one) I was amazed by this little cubs fight to survive and the family that surrounded this Miracle. I hope it touches your heart like it touches mine.

 

February 18, 2011 – It was a day like any other – and then the world turned upside down.  In my mind I have likened the news like watching the events of September 11 unfold.  Whitney was supposed to have a baby shower that weekend.  Liam wasn’t due for another 5 weeks but he wasn’t waiting another minute.  I sometimes wonder about that because it just happened a special doctor was on duty that quickly recognized Liam was in trouble.  He was immediately supported and transferred to Tulsa from Ft Smith – the first of many flights in his short life.  And Whitney left the hospital with Grandma Nanci and they drove – just hours after giving birth.

I remember the call to me in Houston – the first time in my life I had ever heard of a condition called Hypoplastic Left Heart Syndrome or HLHS.  I could hear the fear in Nanci’s voice.  I could feel the fear in me as I read about this condition trying to understand what they meant by palliative surgeries and comfort care and heart transplants.  I didn’t want it to be true.  Your head swims and your heart breaks and then you have to try and regain control because the fight has just begun – and you will need all your strength.  So you decide that hope is your only course and a positive outlook is the only one you will permit.  And you believe.

Liam went from Tulsa to Arkansas Children’s in Little Rock where he spent most of the first year of his life.  Nanci works in adult cardiac cath labs so she had a good feel for what was happening.  Liam had repeated open heart surgeries – sometimes his chest was left open – he was hooked up to what seemed like a million lines and wires and banks of syringe pumps.  We became fluent in a language no parent should ever have to learn.  We slept in waiting rooms and in hospital chairs – we met other parents – we celebrated with some and we grieved with others.  When he was just 5 months old, it was apparent his palliative surgeries were not working.  He had the Norwood and the Glen – but he was only improved for a few days and started to decline.  Nanci had to head back to work and when she left, she wasn’t sure she would see Liam again.  Almost as soon as she got home, Whitney called and said she “got the call” – which in transplant families means it’s your turn.  Nanci had a friend with a small plane fly her back that night and Liam got a new heart.  The waiting room was filled with friends, family, and prayers – again it was a time to grieve for another family – and there was a soul engulfing fear as you realize the enormity of the situation - that you were having your baby’s heart removed and replaced with another.  But when they said he had some through with flying colors we were so filled with hope – we knew it would not be easy – but he had a chance!

He looked better within days.  He was pink!  But sometime after this, they began to realize he had some other issues like bronchial and tracheomalacia.  He was also very drug dependent from the constant sedation and chemical restraint.  After a few weeks he came off the ventilator for the first time in his life.  Up until then he had always been taped up and sometimes even in restraints.  He was such a fighter.   He had this incredible smile – to be sure some days he made you work for it!  And he had the most enormous soulful and penetrating eyes – and he talked with those eyes.  And he loved to hold hands – especially with his grandma Nanci who never let go.  He started having fluid retention problems and abdominal swelling.  And we found out that the drugs and the bypass and a number of things had severely compromised his kidney function.  And the repeated heart caths had cause an occlusion of many, many veins and most importantly his inferior vena cava – the main venous drainage for all parts below the liver.  They speculated this was the cause of the fluid in his belly that would ultimately back up and make him swollen all over.  They “tapped” his abdomen and it gave him some much needed relief but it did not fix the problem.   I remember a week Nanci and I spent with him in Little Rock in the step down unit.  Whitney and Brody and Cheyanne got some much needed family time and we got some very needed quality Liam time.  We took turns holding him and rocking him.  It was a great week – it was the best week.  He was transferred back to Tulsa – which was closer to home and Grandma Nanci.  He had a window of time around Thanksgiving where he was well enough to go home and a very careful and nervous Nanci drove Whitney and Liam home.  There was a welcoming community with signs and balloons and fire trucks.  It was so touching.  There were so many tears.  The local paper even covered the little Lyon’s homecoming. 

He was not able to stay home long before he had to go back to Tulsa and from there to ACH again.  The word from ACH was his venous blockage was so serious; he would not be able to survive.  Whitney and Brody made the decision to get another opinion.  We would find out, his is not as easy as it would seem, or as it should be.  Eventually he was transported to Boston Children’s Hospital.  They tried to open the vein but were only partially successful.  He was sent back to ACH, and then again back to Tulsa. 

Back in Tulsa they told us we needed to let them place a trach.  It took a while to get our heads wrapped around that – but eventually we did.  We hoped he would only need it a few years at most.  It was a hard decision to have this done, very hard.  But he was able to have all the stuff off his face again – finally.

In April 2012 Liam had become so swollen in his belly and then all the other tissues that he was rigid.  The doctors there would not consent to tapping his abdomen to relieve the pressure.  We begged and pleaded – we cried – we consulted with one and all of them and they would not.  They told us to call the family in – so we did – and we called upon the prayer warriors.  He was baptized and we brought all his little and big cousins in to meet him in person.  We were devastated.  And we were broken.  And then – Liam started to turn around – very slowly his tummy started to decrease.

He improved a while – and in July we celebrated his first ‘heart birthday’ with an amazing outpouring of support – it was themed “Liam’s Day at the Zoo”.  But Liam was not doing well – he was swollen and he had some terrible skin issues going on.  We tried everything to help his skin but were told it was related to the excessive swelling and then the drying effects of the diuretic.  No one really focused on the little black spots he had started to develop.  No one knew those spots would ultimately consume him and end the much too short life.

Since before Liam went to Boston, we tried to find doctors and hospitals that would consider taking him as a patient.  We talked directly to Lucille Packard in California, we talked directly to Boston Children’s, and we searched for 1 doctor to accept Liam as a patient in Houston so he could be around family.  We found a doctor in Houston, Dr. Benjamin Ho, but still Texas Children’s wouldn’t accept him as a patient.  But we finally found what we believed was the miracle we had prayed for - the miracle was Dr. Morales in Cincinnati.  When we were down to our last hope, the doctor’s at Cincinnati said they would try.  They flew him there and tested him and we were so hopeful - so very, very hopeful.

But the black spots were bigger and erosive and the belly was bigger than ever – and the tests we thought showed us a glimmer of hope – well, we misunderstood.  The tests showed that the venous occlusion he should be able to compensate for – the black spots on his skin had worsened – and he lost the tip of a finger and we knew.  He had a systemic fungal infection – bad in most patients, but often deadly in transplant patients.  Once you lose the overall integrity of the body’s greatest barrier to infection in an immune-suppressed patient, you have greatly decreased your odds of survival.  The skin couldn’t support the peritoneal dialysis tubes; all of his life support was maxed out.  God called him home and we shattered in a million pieces.  It was devastating and unbelievable – and something no one should have to go through.  Somehow we got him home and all of us home from Cincinnati to Oklahoma.  And somehow, we planned a baby’s funeral.  With a tiny casket, and songs and pictures for videos, a hundred decisions – I don’t know how we got through it.  We then celebrated Liam’s life and courageous battle with 400 of our closest friends and family and thousands more online for the “miraculous soul” that he was – and we laid the cub to rest in quiet country cemetery in Cameron – the balloons and butterflies we released have been symbolic for us ever since.  The entire community was so respectful and loving – we will never forget.

You go through a range of emotions with a child this ill.  Most days you are terrified.  Some days you want to walk away and never come back.  Some days you are so angry this happened to your child and your family.  Some days you are incredibly thankful for the caregivers and some days you hate them beyond measure.  Some days you are perfectly reasonable and the next day you are clinically insane.  The crazy part is its all normal but there is no “help” at many hospitals for families – only for the patients.  If you are staying there at the hospital you don’t really ever sleep and if you are away, you live in fear of that one phone call.  I started every day like a lot of people, checking on the cubby.  Many days you wonder how you can possibly ask your child to undergo even one more procedure.  But when it comes down to it, if there is hope, you have to try.  And when it was over – it was a mix of profound sorrow, sadness and an unimaginable grief – and a sense of relief.  I am sure many people will take issue with that comment but it is a relief to know he is not suffering and not bedridden – that he is finally out of the hospital and out of that bed.  The old adage hindsight is 20/20 has never been more true – if we knew how it would end for him, would we have done any more than comfort care?  Maybe, but your heart and soul join together in a strength you cannot imagine and you believe with everything you have that together you can overcome all of it.  We believed and nearly 95,000 people believed with us.

I once remember my mom telling me that she would give up everything they owned if she needed to, just to save one of her children.  Well it is true – you will.  Whitney and Brody lived the way many families have to – one hanging on to a job, and the other living at the hospital.  Cheyanne saw her mommy as much as possible but spent nearly 18 months in hospitals and cities all over the US.  Grandma Nanci ended up quitting her job, selling her home, cashing in her retirement, and terminating an eight year relationship – you see, she had a grandson and a daughter to try and save.  We, as a family, all incurred enormous debt that will take us years to recover from.  But we will recover.  We would give it all for one more smile and one more little grasp by that tiny fist.

 

In Liam’s memory, we have started a foundation to help other families faced with critically or chronically ill children.  We hope to educate about heart disease and organ donation.  We hope the foundation will keep his memory alive and inspire others to fight like Liam – and Pierce and Glory, and so many others.

Thank you for reading about Liam –I love you Liam Lyon, Aunt Susan

 

 

01 02 12

Bailey's Story

When My beautiful friend told me her story I knew that it needed to be told again and again. It is proof of what a little seed of hope can grow into.....

Only twice in my life have I ever had dreams about my father. He passed away when I was three. I remember waking up from the strangest dream about him. Like most eight months pregnant women, I woke up to once again waddle to the toilet. Slowly lowering myself onto the toilet I had a gush of water. I was startled by this unexpected gush; it was gushing out with each move I made. I yelled for my husband saying, “Kyle, my water just broke! This is it.” He rushed into the bathroom sliding in the amniotic fluid and slid into the wall. I started laughing hysterically-- it was like something from out of the movies. As I laughed, more and more water was rushing onto the floor. I stood in the bath tub barking out orders to my wide-eyed hubby. Thank goodness our bags were already packed from a labor scare the day before.

We arrived to the hospital where the nurses checked me and tested the fluid to make sure it was amniotic fluid. (So silly and a waste of time, I thought. This was my third baby and I knew what water breaking felt like). However, I was only one cm dilated, had very small contractions, and our sweet baby girl was still moving around. Hours later and still I was progressing slowly.  My doctor came in all smiles. She gave me the decision that would forever change my life. She said I could stay in our local hospital and deliver our baby, go to a bigger hospital locally, or to Dallas. Her reasoning was that I was progressing slowly and if I needed anesthesia for any reason they would have to call the “on call doctor”. It could take him to an hour to get there. I talked it over with my husband and we both agreed to go to Dallas.  We wanted to be so careful and I wanted the best for our little girl.  We took the ambulance to Dallas and got into our room. The meds where started, pushing me deep into labor and then I had the wonderful epidural. My mother and my best friend had arrived and were patiently awaiting Bailey’s arrival.
After around 36 hours of labor I started pushing. Our doctor stopped mid push to tell me Bailey’s head was tilted and she would need to use forceps to pull her out. She explained that if we didn’t I would need a C-section. As she proceeded to pull Bailey and turn her, she said “I see hair.” I pushed with all I had and out Bailey came. The doctor laid my baby onto my chest and all we heard was silence. Bailey wasn’t crying and I touched the top of her hair and said in a panic, “She is not crying!” That is when everything went crazy.

Someone grabbed my baby from me. A wave of people flooded my room. A tall grey-haired doctor began working on my beautiful, lifeless baby. He pumped her chest and from time to time he said breath. I counted each time he said breath. It was a total of three. I looked to my family in the room and saw sheer terror on their faces. Everyone was so still. No tears, no words, no comfort. The OB was still between my legs working and was not looking towards my baby. This is not how it was supposed to be. My husband was supposed to be cutting the cord. I was supposed to be counting her fingers and toes. Instead they were rushing her from the room. My husband was close behind. I’m not at all sure where time went from there.
I know my best friend left, my mother was with me, and my husband was not. I think someone must have told me they were taking my baby to the Children’s hospital next door to be placed on a cooling blanket. I remember the nurses not looking me in the eyes. I remember the room they put me in. I don’t remember time. I felt like I was watching myself from some other place; this wasn’t my life. This was a nightmare.

All the while my husband was dealing with the worst moment of his life all alone. He told me later how Bailey flat lined on the way to the hospital. This was the second time our daughter died.

Some women were parked in the medic bay and the ambulance took off their mirror. They were yelling at the ambulance and my husband yelled “That’s my daughter in there!” He sat outside of the ER room where doctors and medical staff worked diligently on Bailey. People kneeled down in front of him checking on him. I’m sure he was in a hellish daze; the Chaplin was brought down to comfort him.

A doctor finally came and spoke to my husband. She explained that Bailey had suffered a severe hypoxic event. It happens to 1 out of a 1000 babies. There is no reason for her to be basically born dead. She had a heartbeat all through labor. The moment she came out, she stopped breathing. A child that is born with lack of oxygen suffers a condition called Hypoxic-Ischemic Encephalopathy (HIE).  The doctor told my husband about how that lack of oxygen most definitely will have affected her brain. She also said that Bailey had a 50/50 chance of making it that night. My husband asked her about placing her on the cooling blanket and what that meant. The doctor asked what time Bailey was born. Kyle said almost 5 and half hours ago. Knowing that a baby needs to be put on the cooling blanket by 6 hours of age to reduce brain damage up to 20%, the doctor rushed out to get our baby started. The cooling blanket basically puts a baby into hypothermia thereby reducing the damage and helping prevent cells from dying. The child’s core temperature is lowered to between 33.5 and 35 degrees Celsius from the normal temperature of 37 degrees. This process takes 72 hours.

I eagerly awaited my husband. I’m not sure what happened in those six hours my husband was away. I don’t remember if I even spoke a word. I was in complete panic and terror. When my husband finally returned, he was pale and frail looking. He was forever changed. He explained to me what all had happened. We were in shock; all we had was each other in this horrific moment. I melted into him; I thought about what a good man he was and how all I wanted was to share our love with our child, how he deserved to be a father. I was hysterical on the inside. I’ll never forget that at the moment I was questioning internally Why, God, is this happening?, a nurse walked into my room. She started asking if I was okay. I told her how our baby was only given a 50% chance of living through the night. She said “You know what? You got yourself a praying nurse tonight. If you would like we can pray together.” My husband, the nurse, and I formed a circle of hands and she prayed aloud. She prayed that God’s will would be done and prayed for strength for Kyle and me. What I wanted to do was fall on my knees and pray for my baby-- the baby I had carried and dreamed of for the past 8 months. The baby whose room my dad had painted the perfect shade of pink. Her little swing that I put together and the crib her big brother helped build.

Each hour that we hadn’t received a phone call was another hour my baby was alive. That was the longest night of my life. I was still feeling the effects of the meds and the exhaustion of labor. I think I slept; I’m really not sure. What I am sure of is we didn’t receive a phone call that night. By the next morning, Bailey was off all heart medications and had experienced no seizures. Seizures go hand in hand with brain damage. They can be a side effect of brain damage and they can cause more damage as well. I felt that tiny seed of hope grow. My husband went to visit Bailey as did as our family. I got to see pictures of my beautiful baby girl. A nurse had put a crocheted pink flower on the side of her mask. She looked like a little girl. She had round chubby cheeks and her daddy’s toes. Her little body was bruised from the CPR and she was intubated for safety. She was on room air which was wonderful. I got to watch videos of her daddy talking to her. She would move her feet when she heard his voice, just like when he would talk to her in my tummy.

I couldn’t even process the emotions I had. Where to start?  I was angry that I wasn’t with her, happy she was here, jealous others where with her, sad, so so sad, exhausted, and fearful. I tried to just make it, tried to push myself to get up and walk. I started pumping milk for Bailey. The only thing I could do for my precious sick baby was make her milk.

Doctors came and went. The doctor that delivered Bailey came to see me. She had a few others with her in a team. I’m guessing she must have needed support as well. She came and held my hand and told me she was a realist and that something would be wrong with Bailey due to her time without oxygen. I just nodded and thanked her for doing all she could. I just wanted to be alone. Every moment I had to talk to someone was a moment I was not praying and begging God for my daughter’s life.

Nothing else mattered but my endless rambling prayers and making milk. It was day two, the day I would get to meet my baby. I still had the IV in my arm as my mother-in-law supported my shaky body down the long stretch of the hospital. It was a beautiful hospital just for children, filled with bright colors, butterflies, and twinkling lights that looked like stars. It was the longest walk of my life. I wanted to run to Bailey. We went through the security of the NICU. Walking through the automatic doors, the first bed on the left was Bailey’s. I turned the corner and there she was. She had her little arm sticking straight up in the air and it looked like she was waving. My mother-in-law gasped and said, “Look, she is waving!” Inside the little bed was my sweet baby.

She had tubes going everywhere. Machines beeped and flashed. I can’t describe how she looked after having two rounds of CPR. Her head was scabbed and shaved on each side where they tried to insert an IV. My heart was beating hard as I got to touch her little hand. The soft baby skin of her hand was so perfect. I got to count her little fingers and toes. I was so afraid to breathe and time was racing by. My hospital had only given me two hours to go visit my Bailey. It’s the most horrific feeling walking away from your baby. There is nothing right about it. Every part of my body ached for her. I shouldn’t be walking out the door. Bailey should be with me, nursing and sleeping. My hospital room should be full of balloons and flowers. People should be calling and coming to visit, not me heading empty back to my hospital.

This night I screamed and cried for my baby. My body shook as I cried hysterically. My IV had blown and the nurses wanted to put a new one in. That seemed so minute compared to what was happening to my baby. I was angry. So I prayed. This was the night I started meditation. I would picture in my head God’s light healing my baby. If I couldn’t be with her I would send my constant prayers.

The next day Bailey was taken off the cooling blanket and the vent. I got to hear her little cry. The most natural thing, it was what she was supposed to be doing!  Crying!  Her little face and body had started to swell from all the medications and her color was getting a bit darker. She was urinating blood and her lab work was starting to look bad. Her doctor had an EEG done on her little brain. It showed normal brain activity and no seizures! My husband and I were so excited. The doctor gave us a sad look and said, “Yes, this is good but we are not out of the woods. I have a feeling that her MRI will not be good, and I’m sure she will start to have seizures.”

Our little seed of hope and happiness was quickly dashed away by the harsh words. I prayed. Bailey soon got worse. We had a meeting over her MRI. They told us how damaged her brain was. That she would most definitely have cerebral palsy and seizures, that she would not be “normal,” and that she could still die at any time. That night my husband basically had to carry me out to the car. My legs felt like Jell-O. This nightmare had somehow become hell. As my sweet husband wrapped his arms around me and held me, I felt a hand on my shoulder. It was the doctor. He helped my husband get me to the car. He said “Pray.” God had sent him to help my husband in that moment to pick me up. He had been the one to deliver the news, but God had sent him to remind me it wasn’t up to the doctor. Bailey was in God’s hands. The doctor urged me to hold Bailey. I didn’t know that I could. I was afraid it would stress her out. He said “What she needs is to be held by her mother. There is nothing better.”

The thought of holding my baby got me through that night. It seems that when you have an extremely sick child it’s the little moments that keep you going. I learned to live for those moments.  Though we may have been in the hospital, in my mind we were home. I got to hold my sweet baby. Finally I kissed her little forehead. We could see the dimples in her shoulder blades. We debated over her nose and her lips. I had never been more in love with my husband than in that moment. We were a family.

Bailey’s lab work continued to get worse. Her liver was failing and possibly her kidneys. Her stomach was possibly dead from the lack of oxygen. We didn’t know because she had only had IV fluids and no milk. Her heart had two holes in it from not taking the initial first breath a baby takes when it is born. When a baby takes that first breath it closes those holes. She was having problems with her white blood cells and clotting. She was receiving blood products and was put back on the vent. Her bilirubin's were extremely high from the liver problems and she was turning neon yellow. One nurse practitioner stated that Bailey was the sickest baby in the NICU. I sat next to her little hospital bed. I prayed. I meditated envisioning God healing each new part of the body her doctors said was failing.

The only bright spot we had was the nurses. Each day a nurse would let us have some little bit of normalcy. My husband got to change a diaper for the first time. I got to pick out Bailey’s socks. We got to suction her nose and check her temperature. Every three hours we had touch time. Though we were helping medically, it gave us a deeper purpose.

Around this time is when we started getting the “Looks”. The staff would try to avoid our gaze or have this pity look on their face with a fake smile. I avoided rounds in the morning. I wanted to hear what was going on just from the nurse, not a whole team of doctors with no bed side manner. Hours and days ticked by.  We became pros at watching the monitors. I held our sweet baby as often as I could. Her little body was so swollen and yellow. I found ways to comfort her by stroking her hair or kissing her forehead. My husband sang to her (sometimes a bit too loud, lol), read to her, and cuddled her. Her blood work got worse; it was possible kidney and liver failure. Her doctor ordered an ammonia test to see basically how fast her cells where dying.

I looked on the internet to find what normal ammonia levels where. I waited patiently, okay, not so patently for the results. The nurse told me the number. I knew before she could tell me that it was in normal range! The test to show how fast she was dying was NORMAL! As I walked to the elevator I ran into her doctor. I was so excited I’m sure I was rambling about the test and how happy I was. She gave me “the look”. My heart went into my throat. She said “Will you be back in her room in about an hour?” I said “Yes, of course.”

An hour later I walked past the nurses’ station. Bailey’s doctor was sitting at the desk. She was crying. I looked down to the floor praying for whatever baby this doctor was so upset about, thinking this couldn’t be my baby. Soon she rounded the corner and entered our little room. I was holding Bailey’s hand and my husband was on the other side of her bed. In that moment I wanted the truth. I didn’t want to continue with this back and forth of info. This is good and this isn’t, but this is okay but maybe this test will show this, blah blah blah. The doctor sat next to me with tears in her eyes. I asked her the question no parent should ever have to ask. Is she dying? Her doctor said “I believe she is.” I asked “Is she in pain?” The doctor said, “I think she is suffering and it would be the humane thing to take her off life support.” I couldn’t breathe. I felt nauseated. I cried looking at my baby sleeping so sweetly even with the monitors beeping. The doctor said that liver failure is a horrible death. She told me how she had treated other patients in this situation and there wasn’t a good outcome, and that since Bailey had brain damage she would never qualify for a liver transplant.

My husband and I wept and held each other. We took turns falling apart and being strong. We made the calls for anyone who wanted to meet Bailey that this would be her last weekend with us. Our friends and family came. Everyone, including us, spent as much time with her as possible in, what we thought, would have been her last 2 days on earth. Bailey’s nurses helped us make foot prints and hand prints. It was such a normal thing to be doing with your baby. My husband decided he would get a dedication tattoo of her prints on his arm so she would always be with him. I was empty. I was dying on the inside. I couldn’t eat. I stopped pumping milk. Why should I pump anymore? She would never drink it. I struggled with every breath. This isn’t real. This isn’t right. Nothing will ever be right ever again.

Sunday night we sat in Bailey’s room watching her every movement. The night nurse was talking to us about our decisions. I wanted to know how it would all happen. She explained to me they would put us in a private room. They would make her comfy on meds and let her pass. We could bring her little clothes and her blankets with her sweet name Bailey Faith monogrammed on them. We could give her a real bath and put lotion on her skin. The nurse said if I wanted I could hold Bailey as she passed. I told the nurse that I brought Bailey into this world and that I would hold her as she left. I know it sounds odd but the thought of dressing her and bathing her gave me such comfort. I could enjoy my baby for a minute. We could take pictures of her sweet face not covered by the vent. We would be able to kiss her and smell her hair. We would be able to kiss her little toes and cuddle her little body.

Our mothers decided they would be there as well. I told them I completely understood if they couldn’t be. I would never ask anyone to witness what we were about to. The nurse called for funeral pamphlets to be delivered by the morning.

Monday morning we were to have our “Care” meeting. I hate that they named those meetings “Care”. I woke up from a restless night of sobbing and nightmares. Kyle called my original OB and had her call in a prescription for me in case I needed to be calmed. I have never screamed in my head before. I screamed at God in my head! I screamed that this wasn’t what was supposed to be happening. This wasn’t right. We were good people. My husband is an amazing step dad and husband. I wanted to give him a healthy baby-- not any baby, THIS baby. I couldn’t understand how the world could still be going on as normal as could be while this tragedy was happening. How could people be laughing and smiling when this was happening!?  I was empty. I had nothing to say to anyone. I called the hospital to check on how Bailey’s night had gone. One of our normal nurses answered. Instead of her saying the normal that Bailey was “ok”, she said “You need to be here for rounds this morning. How fast can you get here?” I told her we were on our way.

Kyle, his mom, and I walked into Bailey’s room right as the cluster of doctors rounded the corner. They went through their normal routine of her list of complications and birth. Then they went to her blood work. My mother-in-law was holding Bailey. We where half listening to the doctors when we heard the words I will never forget. Her liver has improved! The doctors all looked up from their computers to see our reactions. They confirmed it again and again and probably again. Her liver had started to heal itself over the weekend! The same doctor who advised on Friday to take her off life support stated that she had never changed her mind in her entire career until now.

That moment forward we could see the miracle unfolding. We all three sat there staring at each other for what seemed like hours. This is our miracle. The charge nurse came to us and told us she had only witnessed one other miracle like this in 20 years. After the liver starts to heal it rarely goes back. From that day forward Bailey was in control. She healed her liver. She came off the vent. She started eating and then pooping! Her stomach was working. She started opening her eyes. Bailey would cry and wanted to be held. All the things a healthy baby would do. Bailey did. She could track things with her eyes. She held our fingers. We could smile again.

So, even though we were told there is a chance she would live, which we were so thankful for, Bailey’s doctors still gave us the constant doom’s day speeches about how she would have severe mental issues and/or physical disabilities. At around week 6 a doctor measured her head and it hadn’t grown a centimeter. He said she would never walk, talk, eat on her own, would basically be in a vegetative state, and would be in a wheel chair. However, over the next two days her head grew! She started slapping at toys! So we bought more and more toys, anything that could stimulate her brain. I will never forget in our MRI meeting the doctors kept telling us how horrible the news of her brain was. All we asked was “Will she live?” The head doctor said “Well yes.” My husband said “That’s all we need to know.”

The doctor didn’t think we understood, but we understood perfectly. When you have been faced with a life without a child, things like normal tests results don’t matter if they are livable. My husband told the doctor that it was okay: he would be the biggest cheerleader they had ever seen at the Special Olympics! I still at times envision him in a skirt and pompoms.

Ever since the decision from week 2 not to take her off life support, and to give her a chance to live, her organs kept improving.  Lab results, while still in a cautious state, started to slowly improve, which made doctors and everyone agree that she really was going to survive. Slowly, over the course of being in the NICU from week 2 to week 7, medicines to keep her alive started going away, including blood products, nutrition IV’s, and anything else that was keeping her alive. She started feeding on bottles, started receiving physical and occupational therapy in the hospital, and the therapists were blown away by her progress because of what she went through.

Bailey had an NG tube, which is a tube that went from her nose into her stomach. We were advised that it would be best to have a surgical procedure to where there would be a hole made into Bailey’s stomach that a machine would hook onto to. This would feed her. I thought about this and it didn’t seem right. Every time we had given Bailey a chance to do something, she did it. They hadn’t even worked with her on bottle feeding but two times! I understood the risk in both but after her rough start there was no way I wanted to have surgery without giving her a chance. So we started speech therapy. It was slow but she was taking milk. We had a swallow study done and she was drinking perfectly.

Cardiologists started coming into the picture around this time. It was around week 6, and the holes in her heart have not closed. Normally they do by now and after this age don’t close on its own. The doctors thought that we would need to have a surgery done involving a small catheter to close the two holes. A few days later, an ultrasound was preformed. Guess what?!?! One hole had closed itself and the other closing as well.

By week 7, it was agreed by all doctors and therapists that Bailey was healed and recovered enough to go home. I finally got to nest. I went home and washed everything that would stay still long enough to let me clean it.  I rewashed her little pink sheets and footy pj’s. I arranged her toys. It was one of the happiest moments of my entire life, cleaning for Bailey.

Around week 8 Bailey came home. She came home to her two older brothers and her pink room. (She has never slept a night in that room.) Upon discharge nursing was arranged as well as therapists. Nursing came one time to make sure we were comfortable inserting her NG tube into her stomach. Physical therapists came and played with her and watched her. They decided she didn’t need therapy. We thought that when we left the hospital our lives would be constant therapist and doctor appointments. The only therapy she received was speech to help her with bottle feeding. At five months she was completely orally feed. She was discharged from cardiology right away with no more holes in her heart. Her liver is normal, just annual tests. She only has one kidney but as the doctor explained, millions of people walk around every day with only one and don’t even know it. Her kidney is growing with her.

Bailey is now 10 months old. She has met every milestone on time. She crawls and sits. She drinks bottles and eats food. She plays with her toys. Bailey said momma at around 5 months, followed by dada. Now she says baba (for bottle), momo (for no, no) duh duh (for her favorite bath toy, a duck). She has recently started pulling up to her knees and she can push her baby walker. Bailey will walk and she will talk. She knows who her momma and her daddy are. Lol.  I still pray every day but boy, have my prayers changed. We don’t know what the future holds for Bailey but when we go to her doctor appointments, they call her the miracle baby.

 

Writing my daughter’s story was one of the hardest things I have ever had to do. The reason I agreed to write it was to give hope. In our darkest moments I would have given anything for one story of hope. I hope her journey can help not only others going through this but also prove what God’s power can do when we pray.

 

01 02 12

Keeping My Respiratory-Comprised Child Healthy During RSV Season

I was excited and scared when we finally got to bring our youngest daughter, Mighty Z, home from the hospital.  It had been six long months since she had been born with Congenital Central Hypoventilation Syndrome (CCHS), and during that time she had been trached and vented. Before the hospital would allow us to bring Mighty Z home, they prepared us to care for her and taught us how to troubleshoot problems that might arise.  Her doctors and nurses made sure we understood how important it was to keep Mighty Z, a baby who had been intubated and was still trached and vented, away from every contagious “bug” that tried to bite her.  RSV, the flu or even the common cold, could result in not just sickness for Mighty Z, but in her death.

Handwashing Is No Joke

As Mighty Z’s primary caretaker, it was up to me to keep her safe in a sneezing, coughing, runny-nose world, and I took that responsibility seriously.  After her long-awaited homecoming, everyone who graced my threshold was interrogated as to whether they had a fever, a cough, the sniffles or anything else that might land Mighty Z back in the pediatric intensive care unit (PICU) before being allowed inside our home.  Those who gained entry to the foyer were requested (instructed!) to remove their shoes and sanitize their hands before they could go any deeper into the house.  Despite my precautions, I knew it was impossible to keep all contagious illnesses away from Mighty Z, and I lived in fear of one illness in particular, RSV.

Educate Yourself About RSV

Though RSV infection can occur in people of all ages, preemies, infants and very young children are particularly susceptible.  Older children who are infected with RSV usually have only mild, cold-like symptoms such as a cough, stuffy nose or low-grade fever, while infants under age one are more likely to experience more severe symptoms that result in trouble breathing.  A child who has been trached and vented, or even intubated for even as little as one day, has scar tissue from those procedures in the lungs.  That scar tissue makes such children especially susceptible to having an ordinary childhood illness become something substantially more catastrophic than it might otherwise.
Outbreaks of RSV begin most often in the fall and run into the spring, and the virus is spread through tiny droplets that go into the air when an infected person blows their nose, coughs or sneezes.  In general, RSV symptoms usually appear 4 – 6 days after coming in contact with the virus, and they include:

•       Bluish skin color due to a lack of oxygen (cyanosis);
•       Breathing difficulty or labored breathing;
•       Cough;
•       Croupy cough (often described as a “seal bark” cough);
•       Fever;
•       Nasal Flaring;
•       Rapid breathing (tachypnea);
•       Shortness of breath;
•       Stuffy nose; and,
•       Wheezing

Antibody Shots Buy Time

Even healthy, full-term babies and toddlers can wind up with a breathing tube and on a respirator due to RSV, and her doctors knew what it could do to a child like Mighty Z who had scar tissue in her lungs as a result of the respiratory treatments necessary to care for her rare disease.  In order to help keep Mighty Z RSV-free, her doctors prescribed monthly Palivizumab (Synagis®) shots for her between the ages of 6-months and 24-months, after which point her doctors felt she would be “out of the woods” and better able to fight infections.  Synagis®, a man-made antibody to RSV, is costly both to obtain and to administer, as it is typically given in the Synagis® clinic at a pulmonologist’s office.  In my experience, it costs approximately $1,500 per dose now compared with about $3,000 per dose 10 years ago. Because of the cost, our insurance didn’t want to pay for Mighty Z to get monthly Synagis® vaccinations, and it was only after much arguing that I persuaded our insurance company to pay for the cost of the antibody itself.  There was a catch, though, as their agreement to pay for the antibody cost was conditional upon my agreement to administer the vaccinations to Mighty Z myself instead of taking her into the pulmonologist’s office every month to get shots.
After we negotiated our agreement, once a month our insurance company sent a syringe and two vials to our door.  One of the vials contained powdered Synagis®, and the other contained sterile water.  I mixed the powdered Synagis® antibody with the sterile water to reconstitute the shot, drew it up with the syringe, and then gave the shot to Mighty Z once a month.  Although giving Mighty Z her monthly Synagis® shot was almost unbearable (giving your own child a shot is even worse than watching someone else do it), I did it anyway.  Even though Mighty Z and I both hated the shots, I knew she needed them.

RSV is Serious Business for Respiratory-Compromised Children

When Mighty Z turned two, I was over the moon! Not only had Mighty Z doubled the one-year life expectancy her doctors had foretold when she was initially diagnosed with CCHS (Yippee!!), but also I would no longer have to give her Synagis® shots (Yay!).  Unfortunately, my elation about no longer having to give Mighty Z monthly shots was short-lived, as she became infected with RSV after only a month without shots.  Though she had to spend the next two months in the PICU fighting for her life, Mighty Z won her battle.  Looking back now, I believe that Mighty Z survived RSV infection at age two because of the precautions undertaken to keep her RSV-free until she was old enough and strong enough to win the fight.
If your child has ever been either intubated or trached and vented for any amount of time, tell your guests with sniffles that you’ll see them later. Require those that make the cut to take off their shoes and wash their hands, and put on your chemist and nurse hats to administer RSV shots if prescribed by your doctor and that’s what it takes to get the cost covered.  In short, if your baby has ever been considered medically fragile, sometimes you’ll have to do whatever it takes to increase your child’s chance of beating the bugs.



Sources:
Respiratory Syncytial Virus – Accessible from PubMedHealth, A.D.A.M. Medical Encyclopedia, U.S. National Library of Medicine on September 20, 2012.
MedImmune – RSV Protection - This website, which is sponsored by the maker of Synagis®,  provides information about RSV including who is at risk, how to prevent the virus, and when RSV season occurs.01 02 12

The Well

The problem with special needs parents (me included) is that we desperately want to live a normal life, to fit into society.  It’s not so much the normal we crave, but the fact that no one understands the pain and the hopelessness we feel. No one understands the strength, the determination, and the fight we, as parents of a special needs child, must face on a daily basis.



Special needs moms are so alone and we try so hard to wear a brave face at all times, yet in our own minds we are not as brave as we appear to the world.   Let me describe it this way…It is like we are in the bottom of a very dark Well. At first we don't want to get out because it is too hard to climb. We want to resist the fact that our child has something wrong with them. I remember when Mighty Z was in the NICU…in the beginning I didn’t want to associate with the other parents, simply because I didn’t want to belong to the special needs sorority of mothers. When I go up to the NICU now, that is the same reaction I get from the new moms. They say, “I didn’t want to call you because I didn’t want my baby to be trached and I was hoping this would all be okay.” That, my friends, is when we are at the bottom of that dark Well and we just want to sit there for awhile, to believe, and hope, that we will not have to buy the special needs awareness T-shirt.  



Finally, when we start to accept this journey, and we want to start climbing, we are already so weak from sitting at the bottom of the Well in all of the muck and mire, that we can’t even get half way up out of the Well before we keep sliding back down, landing with a great big plop in a heap of tears and pain. But this time, when we are sitting at the bottom, we feel so alone we want to get out of the Well, but we have no idea how to start to climb up and out. We try again -- digging our fingers into the sides of the Well, breaking finger nails, sweating, straining, and begging to get out, only to slide down once more.



This is when one of two things happens. We either slowly and painfully climb out of that Well by ourselves, or a seasoned  special needs mom that has been walking this road for years, stops to  throw down a rope or a ladder and helps to pull us out and starts to teach us how to navigate this new normal journey that we have been thrust to travel down.



I had to climb out of my well by myself and it took years to reach the top.  I navigated how to walk down this hard road by myself with no map, no compass, and no translation book. I got lost along the way, having to double back many times, and I certainly didn’t know this new language of medical terms. Everyday I would have to go home and translate the day’s conversations.

 I think it is up to us as the older (or more seasoned) special needs moms to help the new special needs moms. To give them the maps, the compasses and the translation books. We need to help pave the way, to shine the light – to throw down the rope-- because we all know that a simple rope to help to climb out of the Well is priceless.



The seasoned special needs moms have to teach that this “new normal” is just a different place. It's slower-paced than the old normal world. It may be less flashy this new normal, but after you've been there for a while and you catch your breath, you look around... and you begin to notice that this new normal is full of beautiful smiles, inch-stones instead of mile-stones…it’s filled witha sense of pride and accomplishment that few children feel at such a young age, and maybe most importantly, the deepest love most humans will never feel.



 But... if you spend your life mourning the fact that you didn't get to be in that old normal world, you may never be free enough to enjoy the very special, the very lovely things  about this new normal world that your little miracle opened your eyes to see and experience.

edited by Linda Kruger

01 02 12

Mighty Z's story of congenital central hypoventilation syndrome

01 02 12

Sloane's Story

One of the most wonderful parts of walking down this road is the friends you meet along the way. I have been very blessed to meet one of the fiercest little girls in the world. Her Momma and I have never laid eyes on one another however we have laughed, prayed and shared many bitter tears together. This beautiful family has had the hardest year but has managed to walk down this road with grace, fortitude, prayers, smiles, and many tears. This is Sloane's story………

January 19, 2012, 6:38 p.m.  I waited…and waited…and waited. I blocked out everything else that was going on in the room.  The bustling of the nurses in the corner, the sound of my husband getting ready to take the first pictures of her, the beeping of the machines.  It all disappeared.  At this moment it was just me and her and I was holding my breath and waiting for a sound that would never come.  This was it.  This was the moment that the Earth stopped spinning and my universe shifted into its new position where it will stay for the rest of my life.  This was the minute that I would draw my last real breath and my daughter would begin fighting for every single one that she will ever take. 

From the minute she was born she was gorgeous.  She looked like no other baby I had ever seen.  She had a mop of black hair and eyes that were entirely too mature for a brand new baby (her first gift from her daddy).  Her mouth looked like a tiny little rosebud placed perfectly under a precious little button nose.  Her hands were tiny but her fingers were long.  She was perfect.  As the doctor placed her on my chest, I cried.  I cried because I was so elated to finally meet this girl and I wept because deep down I knew that something was not right.  I still hadn’t heard the sound that I so desperately needed to hear.  I was waiting on that outburst that newborns have.  That first big wail that says “I’m here and everyone needs to know it”!  I was allowed to hold her for only a few seconds and then she was rushed to the nursery where the nightmare would all begin.

A few hours later we were told by a specialist who had been called in to the hospital that Sloane was having trouble breathing.  Her respiratory drive was erratic and at times nonexistent.  Her heart rate was also an issue.  It would drop down to dangerous levels very quickly and without any warning. They couldn’t seem to figure it out.  She had been intubated and placed on a ventilator.  The next time I saw her she looked like a teeny tiny baby bird placed in a nest of machines, tubes, wires and probes.  No beautiful hair exposed anymore.  It had been replaced by a cotton cap that held medical equipment in place. No wise eyes peeping out.  They were lost under a cloud of sedation.  Her beautiful hands were covered in IVs and tape and dried blood.  That rosebud mouth was pried open by tubes that were held in place by a massive piece of plastic that was spread across that button nose and her little round cheeks.  How could this be my child?  How could this be happening?  How did I make it through a peaceful, uneventful pregnancy to find myself standing over her vulnerable, weak and sad little body in the nursery of this hospital? How, after years of trying to have a baby, losing a baby, fertility treatment, tests, procedures and those awful drugs. How could this be?  Hadn’t we had enough?  I stood watching her fight for her life and she seemed so alone.  We couldn’t even get close enough to really touch her. This is not how this was supposed to happen.  Her daddy and I should be getting to love on her and learn how to take care of her while friends and family poured in to see her and ohhh and ahhh over how beautiful she is.  I had bags just down the hall with her brand new monogram on them that were full of outfits that she should have been wearing.  We should have been getting ready to take that first “baby is all cleaned up and looking warm and cozy and mama has showered and has on makeup and cute pajamas” picture.  The one that you send to everyone you know announcing that your perfect little bundle was here and that she is, in fact, perfect.  I don’t have a single picture of myself holding my baby after she was born.   In fact, I don’t have a picture of me holding her until she is many, many days old.  I wanted to pick her up and untangle her from all of that mess that they had created around her.  I wanted to put her in that precious little pink cashmere outfit that her daddy picked out for her.  I wanted to go home and introduce her to our dogs and start our lives as a family.  I wanted the life that I had been dreaming about for the last few years to start. 

The next day came and in what initially seemed like a string of coincidences, the neonatologist that was in the nursery the night Sloane was born had just transferred from our state’s only children’s hospital.  She had a feeling that she knew what was going on.  She said that she had seen this only two other times in her career and that we needed to get to the children’s hospital right away. In fact, the helicopter was already on its way.  Again, not part of my plan.  We should have been taking her home where she would live happily ever after. Fairy tales come to an abrupt halt when the last thing the doctor says to you before your tiny baby gets on a helicopter is “I’m just not very encouraged by Sloane” and “She just seems sort of lazy, like she doesn’t really care to breathe”.  It’s ironic that in just a few days a friend of mine would create a Team Sloane logo that has the words “Girl Is Fierce” across the front.  Sloane wasn’t lazy, she did care and she WANTED to live.  She’s proven that every minute of her life since then. 

As Sloane makes her way to the children’s hospital via helicopter, my mother, my husband and I make the nearly 3 hour drive.  They didn’t bother letting one of us go with her in the helicopter.  Yet another scary event in my daughter’s life that she had to endure alone.  I remember saying to my mother on the car ride to Little Rock, “What if she has to be here for like a week or something”.  Wow - If I knew then what I know now. Once we got to the hospital we went straight to the NICU and the first thing I see is a tiny baby being wheeled down the hall.  The poor thing was covered in electrodes and there was barely room for her in that bed because most of the space was being used by machines and monitors.  As that baby got closer I realized she was mine.  She had already been attached to more stuff.  She was almost unrecognizable. 

The next few days are a total blur.  I don’t remember sleeping or eating.  I don’t remember talking to the countless friends and family members who waited in the waiting room.  I don’t remember bathing or ever leaving the hospital although I know I did.  I had to.  Because you see, they don’t let you spend the night in the NICU.  So every night parents have to say goodnight to the most precious thing in their world, walk away and hope and pray that someone who is sweet and kind and gentle will be assigned to keep your child alive that night.  Within a couple of days the doctors had tested everything and they concluded that Sloane probably had CCHS (Congenital Central Hypoventilation Syndrome or if you prefer medical slang, “Ondine’s Curse).  Luke and I were confused.  What on earth was CCHS?  What did that mean for Sloane?  And could we just go home?  The doctors did the best they could to explain CCHS but with less than 1500 people in the world who suffer from it, they weren’t experts.  CCHS in very short terms is a respiratory disorder that is fatal if not treated.  It’s a very rare and serious form of central nervous system failure as it relates to the autonomic control of breathing.  In English?  It means that those with CCHS do not breathe when they go to sleep.  While most of us don’t even think about continuing to breathe when we go to bed at night or take a nap on the couch, if those with CCHS do this without the proper life support, they will die.  CCHS never goes away.  It’s genetic; it’s part of every single strand of DNA. It’s not something that is outgrown. So with our permission they sent a sample of Sloane’s blood to a lab in Chicago that would test for it.  It took over two weeks to get the results. 

A lot happened within those two weeks.  Luke and I were allowed to “help” give Sloane her first bath.  She was almost a week old and up until this point was too unstable to bathe.  So there we were: Luke, me and a nurse giving our baby a sponge bath.  I picked glue and goop from the electrodes out of her beautiful hair for what seemed like hours. We carefully wiped her skin with rough hospital issued washcloths.  Then the nurse put a new diaper on her and allowed us to pick out a pair of socks for her.  No cute pajamas, as they would interfere with all of her IV’s.  How’s that for a first bath memory?  It was a far cry from the scene I had created in my mind.  No cute whale shaped bathtub, no delicious smelling Kiehl’s bath wash and certainly no monogrammed towels.  But none of that really mattered because for the first time, we really got to touch her.  We got to hold her tiny fingers and rub her sweet little back.  We got to pat her bottom and tickle the bottom of her chubby little feet.  I held on to that memory for a very long time and it ended up getting me through many sleepless nights.  I wanted so badly to nurse her and have those very intimate bonding moments with her but that was out of the question with an intubated baby. So I started to pump in order for them to at least give her some of my breast milk.  Perhaps one of my saddest moments (as if there hadn’t been so many before) was after a particularly unproductive and painful hour of trying to pump and producing only a few drops.  A nurse explained to me that I probably wouldn’t be able to produce enough milk to sustain her. Due to the lack of interaction and bonding with her, my body didn’t really recognize that there was a baby in the picture.  In order to stimulate my body and remind it that I had a baby, they suggested that I take something with me that smelled like her when I tried to pump. This was devastating to me.  Sloane and I had missed out on something really special and extremely important.  We didn’t get those first few days cuddling and bonding when all of those “hormoney” things happen and as a mama your body makes all of those magical changes that God put in place so that we can take care of our babies.  I didn’t know if Sloane knew that I was her mother but I did know that my body didn’t even know she existed.  I was heartbroken and this was the start of a very hard journey that Sloane and I would go through in order to form the bond that I so desperately wanted but could not, no matter how hard I tried, feel. 

Also during the time that we waited on the results of the test, Luke and I started researching CCHS.  We read everything we could get our hands on and reached out to other parents who have children with CCHS.  We needed to know what we were up against if the test came back positive.  What we found was that while it was not the best diagnosis that we could get, it certainly wasn’t a death sentence.  Unfortunately not everyone felt this way.  We were asked (more than once) if we wanted to let Sloane go.  This is a question that no parent should ever have to answer.  Yes, she was broken and tattered and a complete mystery to everyone, but to her daddy and me she was our miracle and our hero.  The question itself is horrendous but what the question does to the mind of a parent is so much worse.  Imagine being asked this and then think about how that automatically forces you to look at your child.  You can’t help but think that her life is going to be so grim that it’s not worth saving. It’s a good thing this thought was fleeting.  Sloane hadn’t given up and we refused to give up on her.  Her heart rate issues had also started to get more concerning.  She was seen by a cardiologist who determined that a pacemaker should be implanted immediately.  The following morning my baby girl, on her eighth day of life, had a pacemaker placed into her tiny little abdomen.  She sailed through the surgery and was sent back to the NICU within 24 hours. After all of our research, Luke and I knew that Bradycardia (the condition that caused her to require the pacemaker) was a side of effect of CCHS.  In the meantime, Sloane’s bathroom habits were becoming a cause for concern as well.  She was not pooping on her own, yet another sign pointing to the diagnosis that we really hoped she wouldn’t get. 

On February 7, Sloane was diagnosed with CCHS.  In addition, she was diagnosed with Hirschprung’s disease (the CCHS side effect that caused the bathroom issues).  Hirschprungs is a disease of the abdomen which is caused by the lack of nerve cells that help the large intestine function properly. The next day my husband, being a direct match with Sloane, gave several units of his blood because the next morning Sloane would undergo three surgeries at one time and would more than likely need a blood transfusion.  The doctors sent several nurses to Sloane’s bedside to explain to us exactly what to expect the next day.  One nurse brought a book to show us what a tracheotomy would look like.  I couldn’t bring myself to look at it.  Later that night I begged my mom to tell me how the pictures of the babies looked in that book.  I could tell she didn’t want to be honest with me.  Tears streamed down her face and she whispered the word “awful”.  Later on I found out that the book that the hospital was using to show parents how their children would emerge from the operating room with a trach was completely outdated and didn’t really paint an accurate picture of the medical equipment used today.    

February 9 was the most horrific and most beautiful day of my life.  Sloane was walked down to the operating room by her mama and daddy and both sets of grandparents.  She had quite the parade through those halls.  We all wept and prayed over Sloane and for the next few hours sat huddled in a waiting room with about 80 other strangers while Sloane was somewhere down the hall in a cold, sterile operating room getting a tracheotomy, a colonoscopy and having a feeding tube placed into her stomach.   The questions “Why me?”  “Why us” and most of all “Why Sloane” played over and over in my head.  When everything was over and she had been taken back to the NICU we were allowed to go see her.  When we walked into the pod where her crib was, it was like a spotlight was on her.  It was one of the most amazing moments of my life.  I could finally see her face.  No tape, no big plastic piece holding her mouth wide open, no tubes shoved down her throat.  She was laying there sleeping peacefully and we finally got to see all of those precious features that had been hidden since the night she was born. She was even more beautiful than I had remembered and I sat next to her rubbing her cheeks for the next 5 days as they kept her “snowed” so that she wouldn’t move and irritate any of the surgical wounds. 

After that, the remainder of Sloane’s four month hospital stay was really about her getting big enough to use a home ventilator.  Babies have to be at least 5 kilograms (11 pounds) before the doctor will even try to put them on it. On April 14 Sloane hit 5 kilos and on April 18 they put her on the ventilator that would end up coming home with us.  Before they transitioned her to this vent they warned us not to get our hopes up.  No child had ever transitioned the first time it was tried.  The minute they put Sloane on it she never looked back.  So about a month later, after Luke, my mother, his father and I had been through extensive training on how to care for her, we got to take Sloane home.  We were scheduled to be discharged on May 15. On May 14 I packed up Sloane’s belongings and cleaned out the only “home” she had ever known.  I didn’t take much.  I didn’t want to keep anything that reminded me of our time there.  I wanted to start fresh when we got home.  So I packed the bag with her name on it - the one that had once held newborn outfits and her beautiful going home outfit.  It now held medical supplies: a pulse oximeter monitor, a CO2 monitor, a portable suction machine, a home suction machine, all the supplies to change an ostomy bag, a spare g tube for feedings, extra trachs, trach ties, drain sponges and the list goes on). 

Because we live so far away from the hospital, she had to be taken home in an ambulance.  Her daddy rode with her and I followed closely behind.  It was during this three hour ride that things kind of got weird for me.  I had almost convinced myself that when those ambulance doors opened, all of this would be behind us and Sloane wouldn’t have a trach and a vent and an ostomy bag and a g tube.  She would be able to breathe when she slept and she would eat from a bottle and she would poop in a diaper, not a plastic bag adhered to her tummy.  But – when we got home and they unloaded her, nothing had changed.  What was going on?  We’re supposed to be taking pictures next to a stork in the front yard with her name and birth date on it.  We’re supposed to be carrying her in to meet her 4 legged sisters Bayona and Clancy.  We were supposed to take her on a tour of her house and show her the nursery that we worked so hard to make perfect for her.  I didn’t want all of those machines to come in my house with her.  I wanted to take her off of that God awful vent and Pulse Ox machine and throw them back in the ambulance where they belonged.  I wanted to take my daughter in the house and slam the door on CCHS.  If only it were that easy.

We spent the next few months learning how to have a very medically complex baby live with us.  I spent those months just trying to keep her alive and healthy.  I was her nurse now.  From the time Luke went to work until he got home in the evening; I was in charge of her life.  I had the ultimate responsibility of keeping her alive all by myself.  I took my new job very seriously.  In hindsight, I took it too seriously.  I didn’t read to her, I didn’t cuddle with her.  I cleaned her supplies and kept very meticulous notes about what she was doing almost every single minute of the day.  I took her temperature and checked her CO2 every 30 minutes.  There were days when we never left her room.  My husband would come home from work only to find me in the same place that he left me when he went to work. I was scared to death to move her around.  I was scared to death of her.  Period.  As the days and weeks went on, Sloane got stronger and stronger and I got worse and worse.  The heaviness of this life was suffocating me.  I was losing it.  I began to resent Sloane.  I began to resent my husband for getting to leave every day to go to work.  I wanted to escape from this situation.  I knew that I wasn’t good for Sloane.  Yes, I was taking extraordinary care of her.  She was amazingly healthy but that’s all she was getting from me.  I was still nothing but a nurse.   I didn’t feel like a mom. During those days I used to think about things my girlfriends had said about their new babies - Things like “I can’t imagine my life without her”.  I didn’t have these feelings.  I could imagine my life without her.  It was easy.  It was happy.  I wasn’t scared to death every second of every day.  I could breathe. I missed that life.  These are awful feelings to have about your child and even today I cringe thinking back on that time of my life.  I used to beg to God to either give me the things I needed to be a good mother to Sloane or just take me out of her life.  She deserved better.  For several months I didn’t leave the house much.  I only took a quick trip to the grocery store every now and then or a doctor’s appointment for Sloane.  I distanced myself from my friends. I didn’t want to see them.  I was ashamed of the person I was and I was afraid to introduce them to Sloane for fear that they would think she was some sort of weak and fragile freak.  I stopped answering the phone; I made excuses for people who wanted to come see us and her.  Looking back, I’m so embarrassed and ashamed that I behaved this way because these are the people that I needed the most.  These were the people who would have helped me crawl out of that dark hole in which I had placed myself. 

A dear friend who is a psychologist (and more importantly Sloane’s Godfather), connected me with a good therapist which turned my life around.  I began to get used to my new life and embrace our version of “normal” which, by the way, is a word I hate. You see, when you have a medically complex child like Sloane, the word “normal” changes drastically. Our society puts so much weight on being ”normal” but who really knows what that means?  Do I think Sloane is normal?  Yes. She is just like a typical child her age; she basically just needs help breathing when she sleeps.  To me, it’s no different than a child with asthma that needs an inhaler or a diabetic child who needs insulin.  Sloane is going to go to school, do all of the activities that her friends do, go to college, get married, have babies and live a full, meaningful, important life. So yes, my daughter is normal. Well actually, she’s extraordinary and she’s my best friend.  And she is such a blessing to us.  She’s more than I ever could have imagined and way more than I deserve. 

As I type these last few thoughts, my precious baby is still asleep.  I can hear on the intercom.  Over the steady rhythm of the ventilator, I can hear her.  She’s dreaming and that makes me smile.  Every night when her daddy tells her goodnight the last thing he says to her is “Dream big little girl”.  And you know what?  She is.  And so are we.  We’ve just celebrated her first birthday and I know with all of my heart that this child is meant for very big things.  She is going to change lives and make this world a better place.  She’s already started.  The answers to so many of the questions that I had in the beginning – why me? why us? why Sloane? why didn’t I get my fairy tale? are unfolding every day.  And the best part, those don’t really feel like questions anymore. Sure, there are things that will never be answered but I’ve accepted that.  It’s ok because now I feel blessed, not burdened. My heart is full.  My heart is happy.  My baby is perfect. 

I’ve learned a lot of very hard lessons over the last year.  Some that I hoped I would never have to learn and some that I never knew I would be lucky enough to learn.  

Here's what I don't know:

1.  Why God entrusted us with such a fragile child.
2.  Why our Sloane was chosen to bear such a heavy burden.
3.  Why I will never experience the sheer joy of pregnancy, labor or holding my newborn baby in my arms
4.  What it feels like to have a baby sleeping in my house that isn't on life support
5.  How to give up.  I saw a quote recently that said:  “We’ve got three choices in life. Give up, give in or give it all you’ve got.” The first two are not an option. 


Here's what I do know:
1.  My child is a miracle.
2.  Life is fragile yet the human spirit is fierce.
3.  Friends, true friends, will be there.  At the beginning, during the worst of it, and when you decide to

crawl out of that dark hole and come up for air.  And they will be just the same as they were before you found yourself in this place and won't fault you for being forever changed.

4.  Doctors don't know everything.  It's up to us as parents to advocate, research, study and be diligent about the care our children receive.
5.  Life is hard. It's best to take it one day at a time.  Sometimes one hour at a time or one minute.  The challenges put in front of us can be overwhelmingly difficult and scary. 
6.  You have to be kind to yourself.  Beating yourself up isn’t good for anyone around you and the bruises take a long time to heal.
7.  Its ok to cry. - A lot
8.  We absolutely are given more than we can handle sometimes.  But we are never left to fend for ourselves.  He will provide what is needed to get through it.

9.  God is good.

 

 

 

 

 

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