Congenital Heart Disease Awareness Week – “The Three Musketeers”

This journey is tough.  This heart stuff – not for the faint of – erm – heart!

I’m incredibly blessed and have Craig to do life with and go on their journey with.

What if…

What if I didn’t have him to lean on?  Call on?  Be there for me?

What if I was doing this…on my own?

 

“I know God wouldn’t give me more than I can handle- I just wish he wouldn’t trust me so much” –Mother Teresa

“You’ve never met a mom like me!” – Me


To be able understand my life as a single heart mom and the strength I have found within myself- you have to have a glimpse into what makes my family of Chase (17 months), Julie Anne (almost 3) and myself (25)- the self-proclaimed “Three Musketeers”- who we are. I, myself as a child was born with a life threatening illness- It was told that I would have three months to live, then six months and so on and so on. I just kept beating the odds and beating the odds, doing activism and awareness for my illness all along the way- until one day I became undetectable and I’ve been undetectable ever since. Growing up as a child, never knowing if tomorrow would come or not- I learned to never take life for granted, I lived every moment as if it was my last. When you live by that mantra you can’t ever accomplish things quick enough- therefore after graduating college in three years I moved two counties away from my hometown to be with my then fiancé. After trying for a miracle (it was doubtful of whether or not I’d be able to have kids) – God blessed us (or me) with Julie Anne. My entire pregnancy with Julie Anne- I went through without the support of my partner. He didn’t go to Dr’s appointments or ultrasounds for fear he would curse the baby- as he had had a brother who was severely handicapped. In March 2010, Julie Anne was born and upon birth aspirated fluid in her lungs- was a code blue in the nursery and God wrapped his arms around her and brought her back to me. She then spent 10 days in the NICU recovering but something else was very different about Julie Anne- she was born with craniofacial anomalies- they said it was birth canal bruising, a specialist called it “utero squish” and that it would work itself out- but my instinct told me it was something worse. Whenever life presents challenges for me- the best way I survive is by inundating myself with knowledge- I researched and researched and settled on the diagnosis that my daughter had right coronal craniosynostosis- a condition where one of the sutures in the skull closes prematurely in utero and if left untreated can cause brain pressure and therefore seizures. (1 in 2000 are born with Craniosynostosis) I took my daughter to Children’s Hospital of Philadelphia for a second opinion and to confirm that my diagnosis was right- and so be it- I was sadly right. My daughter was born different but she was perfect to me- I shook off the stares of strangers but there was one thing that wasn’t so easy to shake off- and that was the abuse of her father. When Julie Anne was born “different” he wanted nothing to do with her since she wasn’t “perfect.” (She was perfect to me.) He said and did horrible unspeakable things to me and every night when I put Jules to sleep, life was a living nightmare. He had had a brother who was severe special needs and his childhood was spent being put on back burner as his parents focused on their sick son- so now here he had had me who made him my world but then once our daughter was born the natural occurrence was she become our world but once again just like his childhood was in a situation where someone was taking attention away from him and he resented his own daughter for that. I couldn’t stay with him- I was the one paying the bills, buying the cars, paying for the house and the trips, etc- with zero support from him. I had been an admirable person to look up to but if I would have stayed with him I wouldn’t have been. I refused to let my daughter be susceptible to that. Two weeks before her surgery- I ended the engagement.

In November 2010 Julie Anne had her surgery. I, alone, had to stand and watch my daughters beautiful baby hair get shaved off, stood beside my child alone as she laid in bed recovering from major cranio and neurosurgery, her eyes swollen shut, in agonizing pain, with a zig zag incision from ear to ear that was eye-startling. I’ll never forget the day of discharge they needed to do a cat scan to make sure the operation was a success and they wanted to put her under again so she would lay still and I begged them no, no more medicine and I sang “Silent Night” again and again as it was the only thing that allowed her to remain calm so we could get that test done. When it was over- I pushed my daughter out of that hospital in tears- so proud of us- it was her and I against the world- we had overcame so much together and there was nothing we couldn’t do together. (Julie Anne today is one of the brightest kids in her class, she makes long sentences already (I’m in for it!), she’s always singing (knows entire song lyrics), dancing, horseback-riding, etc. Her birth defect has had no long term effects on her. She has had an eye surgery since then and could decide to have future surgeries if she feels she is still asymmetrical but bottom line is she is safe from a medical standpoint.)

Two months later, I heard from her father. He was “a changed man, he wanted to be there for our family.” I saw him ONE NIGHT and God gave me Chase (also showed me writings on the wall as all their father wanted was money again- and mind you, this was a man I was in a relationship for 4.5 years with/engaged for two of those!) God really wanted Chase to be born to me. I believe that with all my heart and soul. When I found out I was pregnant- once again I encountered on a journey alone. I was doing the normal Dr’s appointments and ultrasounds- when I went for the anatomy scan- they told me what I was hoping- that Chase was a BOY and I was so ecstatic. But the next sentence after that was that there was something seriously wrong with his heart. The weeks ahead led to an amniocentesis, consultations by CHOP’s Fetal Heart program, three hour ultrasounds revealing not only the complexity of his heart but the absence of an anal dimple, a stomach, small kidneys, missing vertebrae, etc. I’ll never forget them taking me into a room and explaining that they felt it was VATER/VACTERL association (a non-random association of birth defects)

(Mindy Tipton’s beautiful angel of a daughter Abby had this defect- this little girl is so special to my family and I have no doubt she has helped watched over Chase in his darkest moments. Love you Abby!)

So the Dr’s laid the diagnosis down and said that maybe I should consider the implications of a child “like this” and how it would affect my life and Julie Anne’s life. Never ever did I consider for a minute that this wasn’t God’s purpose for me- I would see this child through and even if I had three days with him- I would be lucky because I had been his Mother. The heart gave the Dr’s the biggest puzzle to figure out was it Truncus Arterosis? Was it d-tga with tricuspid atresia/aortic atresia? HLHS? Or HRHS? It was unfathomable for them to see a heart in the condition his was. However, we were going to proceed with normal birth plan for a cardiac kiddo.

(A month before delivering Chase I received an email from my ex stating he wanted to give me full custody so he never had to be bothered with any medical consent or so forth. Had my lawyer draft the paperwork, both parties signed and we never heard a word from him since.)

I delivered Chase at UPenn (right next door to CHOP) with the plan that after delivery he would go to the CICU at CHOP. I’ll never forget delivering Chase- delivering in the OR since it was right next to the resuscitation room- having them sweep him away never even seeing his face but only the top of his head as the door swung open and close to the next room, as he lay on the table. They took him over to CHOP and I was unable to see him for close to six hours after birth. Asking the nurse on the phone what did he look like- lots of hair and webbed toes (runs in the family- I don’t have them but cousins do.)

When Chase was born he had over 26 echo cardiologists scan him puzzled at his heart defect but he had geneticists diagnosing him within hours of birth. When I finally saw my son I was met at his bedside by a world-renowned CHOP geneticist who pointed out anomalies on my beautiful baby boy- and proceeded to take me into a room to discuss the long term implications/quality of life factor for my son. She explained that Chase had Rubenstein-Taybi syndrome. A genetic disease that includes but not limited to these symptoms:

  • Broad thumbs and toes (Just going to make him a better baseball pitcher)
  • Excess body hair (Yep, his leg and back hair looked like an old man’s)
  • Heart defects (Yep, that’s for sure.)
  • Mental retardation- Avg IQ not higher than 75. (Defying the odds with that one!)
  • Short stature (Not so much, he’s a string bean)
  • Low muscle tone (Yes but through therapy he is overcoming.)
  • Low-set ears or malformed (Yes, but hey it gives him character!)
  • Prominent beak-nose (I love my Little Who from Whoo-ville.
  • Undesencded testicle (A man’s nightmare that’ll get fixed after third heart surgery.)
  • Tethered spinal cord (Will be fixed after third heart surgery.)
  • ETC ETC ETC!

As I sat in that room and I have an entire room staring at me with sympathy- she got done her spiel and asked me if I had any questions or comments- I said- “You’ve never met a mom like me.” And with that mindset went head-first into each problem and developing a solution. But three days into Chase’s life his heart was deemed inoperable. The aortic valve was so small that the world’s best surgeon Dr. Spray had never even operated on one as small as his.  His heart defect was defined as single left ventricle, tricuspid atresia, aortic atresia and d-tga. His heart defect is 1st in our country and 4th in the world. Dr’s would literally have to draw out his heart for each other because it was unfathomable that his heart was formed like it was.

It was decided Chase was inoperable and there was nothing they could do and that they were going to have to turn off the meds and let him go. This was unthinkable- as I went home to collect all the things I had bought for him that I wanted to see him with if he were to pass- I went back to the hospital with a rigorous fight and refused to accept this was his fate. I said if he didn’t have a fighting chance I would have miscarried- he’s got a fight- we’re doing something. Dr. Swast and amazing cardiologist along with Dr. Spray and I came up with a plan- we would do a hybrid surgery to allow a stent to be placed to allow his aortic valve to grow so he would be ready for a Norwood surgery down the line- Dr. Swast and Dr. Spray went up against the entire group of Dr’s/Surgeons and put their reputation on the line and moved forward. (Texas side note- you will see why this is important- Night before Chase’s 1st heart surgery CJ Wilson was pitching in the playoffs for the Rangers- and the whole stadium was chanting “CJ! CJ! CJ!” little did they know they were chanting for my miracle.) The night before Chase’s surgery I made molds of his feet and his hands, I gave him his first bath and I took pictures- (in the pictures orbs of light surrounded him- wasn’t the first time God had shown his light was shining on my son) The night rounds before that first surgery the awesome Dr. Byrd at CHOP said to me, “I’m not sure about tomorrow’s outcome but I’m sure of one thing Chase is lucky to have a mom like you.” The next morning, as they rolled him down into the OR- he pulled the ultimate Philly-pride symbol- he got his little hand out of his blanket and made a fist like Rocky Balboa in the air- he was going in for a fight. He came out of the surgery miraculously- we did it. The weeks ahead were filled with victories and disappointments- all I wanted was to be able to take my miracle home but his oxygen levels kept preventing even his ability to go to the step-down unit (we actually lived in the CICU our entire CHOP time.) It was apparent that Chase had chronic lung disease and it kept him on and off oxygen, CPAP, etc. The need for a trach arose a month into life and we did all the necessary tests and it resulted at that time that it was not medically necessary so we pushed on hoping his sats would turn around.

I would spend my day’s day-in/day-out at hospital alone with Chase and come home to my beautiful little girl- it had always been her and I against the world and the guilt of not being with her too day-in and day-out wore hard on me. But I knew she was already such a compassionate little girl and she would of wanted me to be by Chase’s bedside as I was for her- she proved this to me by as I would leave saying “Ahhh Chasey” which I translated to “It’s okay Mommy go take care of Chasey.” I gave it my 100% effort to nurture their bond as siblings- brining her in every weekend- spending Halloween dressed up together as Dorothy & Toto- etc. At this point both of my kids were miracles and both of them deserved the world. It had always been a dream of mine to move to Texas and give my kids the childhood I had always dreamed of for them- living in a place with a small town square charm, where family values and religion are rooted deep, where music fuels the society and culture, etc. So that fall as my son was going through one of the most critical time in our little family’s life- I decided to put my long-time dream in motion. In November I flew down to meet with Children’s in Dallas’s team of Dr’s/Surgeons, set up insurance, look at real estate- because I couldn’t move my medically dependent children across country blindly- when I returned to Philly- it was all coming together that this was going to be our future and all of the Dr’s/Nurses knew the long term plan- get him healthy move to Texas.

(I met Stacie Jackley one night in the parent lounge who’s amazing cardiac warrior Kellen’s story inspired me and kept me going. She had experienced the same situations of a lack of support system and desire to move their family too.)

The Dr’s and nurses became my family- my own family visited when they could but it wasn’t as much as I had wished they’d be there- everyone in the CICU knew my entire life story, we laughed and cried, shared “look at Chase now” stories and he was already so remarkable- spent the holiday’s together and so much more.

My own relationship with my family was tested as I watched other little kiddos be surrounded daily by people coming and going- and all Chase had was me. (And his sister when I would drive out and drive back into the city to have her visit her brother) I held a lot of resentment and a lot of anger because I couldn’t understand why my family weren’t more involved in his journey (my whole family lived 25 minutes away from the hospital and they couldn’t even visit him on Christmas day- he was our very own Christmas miracle!) Relationships were tested, some broken, words exchanged but I have come to forgive them and realized the only person you can worry about is you. They would come to realize that they missed out on witnessing the miracle that is Chase.

Sure I watched the other babies be born, and come and go and wished it was our turn.

(Jennifer Clauss Skerels precious daughter Emma was Chase’s girlfriend at birth and we saw her go home and come back a few times and Jennifer would be like “you’re still here!”)

The greatest gift I received from the extended hospital stay was the gift of witnessing daily, the power of the human will and the desire to fight. I have never saw such proof in God’s existence then in those halls of that CICU. I also never felt such magic, hope, love, etc- it’s a remarkable place.

Chase’s episodes would happen and it would be in these moments that I would fill with such hurt and anger that I was in this alone- that their father had neglected his children. I believe God chose my ex just like he chose me to be Chase and Julie Anne’s parents- and I answered that call and did the righteous thing but he ran. And like I said my ex had had a brother who had special needs and who knows maybe he didn’t want a life repetitive of his childhood and that’s why Julie Anne’s condition scared him and then he never even wanted a thing to do with Chase. Whatever the reason- the pearly gates will hold his fate. He would have benefited immensely to be their father- it was God’s ultimate blessing on his life that he never accepted. Like I said, I’m the lucky one. I am so fortunate and blessed.

 (A little boy named Weston- Julie Harless Keeton’s beautiful son- was a beacon of hope for me for my little miracle man. I’ll never forget Weston rolling down the hall in his wagon bragging how Chase had a mowhawk like him. Weston’s unbreakable spirit kept me going- he and Chase were staples in that CICU.)

We spent Christmas in the hospital with one visitor- and anxiously awaited his second heart surgery which was to be a Norwood/Glenn combination surgery. When his sats were no longer manageable it was time and on January 17th, 2011 (the last surgery before we could finally get out of hospital and move to Texas to start the second lease at life we’d all been given.) Chase went in for his surgery- this was the riskiest one- I had been hammered by statistics that most hybrids hadn’t even made it to their second surgery and if they did there was a 5% chance of survival. Well Chase came thru- but the surgery was not a 100% success- his chronic lung disease which had hovered over us- keeping him on and off oxygen for most of the fall- was the culprit for the Glenn part failing. Ten days after surgery Chase went to be extubated from his surgery and we lost him- for 1 minute and 50 sec. As I stood there, throwing prayers over the Dr’s heads I couldn’t cry, I didn’t want consoled or comforted- I just stayed focus on my prayers. Chase came back to me by the grace of God and CPR performed by two Dr’s who had been a staple of our journey. One I had nicknamed Dr. Doom since she was so real with me non-stop (she is the one who not only saved my son but made our Texas dream become reality) and the other who deemed-himself Chase’s best friend (they had watched world series together- you know men- bonding over sports!) Chase had came back to me- he was reintubated and now the decision needed to be made- to trach or not to trach.

I sat in a room once again with over 10 faces staring at me telling me how trying it would be and the implications of a decision and I said once again- “you’ve never met a mom like me.” What other choice did I have- try again to extubate and risk not getting him back- I wasn’t willing to chance it. So we went for the trach- it was decided that it would be possible for me to receive the necessary medical training for the trach in Texas- since the healing time/training time was 8-12 weeks and why keep waiting around for our forever- let’s head to texas. So Dr. Doom became Dr. DreamComeTrue and on February 14th, 2012 (Valentine’s day- Chase’s first holiday out of the hospital) we flew to Texas! J Chase was admitted to Children’s where once again I was met with ICU Dr’s who hadn’t ever saw a case like Chase’s and assumed he was a hospice case- and once again I had to go into a room by myself with people who placed limitations on my son until I was reunited with the amazing cardiologist I had flown down to meet who, along with the pulmonologist who went onto join the team of my son’s life-savers- fought for Chase.

(We were moved from the ICU to the cardiac floor and placed next door to the only heart mama I had known in Texas- Czarina Hounsel and little Evan- who was in the midst of his hardest hours. We never got a chance to meet face to face because of his rough patch there but I had advised Czarina pre-delivery to have faith- you were chosen to be his mother- you can do this- she has handled everything that’s came her way with such an honest approach- never masking her fear or emotion and being true to the realities of a heart mom- and that’s what makes her the anchor she is in the heart mom community.)

Weeks ahead we played with vent settings and trained on his equipment and so forth. Julie Anne’s life was balancing back to normal- she was in daycare for the first time in her life, doing new activities, new place, it was her and I and Chase against the world, etc.

On April 18th, 2012 (after 6.5 months spent in the hospital) my miracle man came home! Adjusting to life at home with a vent-dependent kiddo wasn’t normal- but what’s so great about normal- bottom line is he was here. Sure it killed me that he had a radius for range of movement- I couldn’t sit on the couch with him or just carry him outside- or go to the bathroom and take mirror pictures with him like I once saw other cardiac mom’s doing- but HE WAS HERE. Weeks and months ahead he just beat the odds and beat the odds. We made the most of being together as a family- The Three Musketeers and our animals were reunited and in a brand new place to explore, make memories, sew our family’s roots- “The Miracle Family”.

Chase slowly but surely was weaned off the vent in between zoo trips (we adopted a giraffe for his birthday- we collect them because after we were unsure of what his brain function would be like- a giraffe stuffed animal was the first thing he fixated on- I’ve been collection/had a slight obsession for anything and everything giraffe since- hey, it’s a metaphor for him to remember there are no limits of how high you can reach!) anyway so we did trips to the pool, sightseeing trips, and so on. I refuse to keep Chase isolated from the world he was given a chance at life for. (I never ever want to look back and say I didn’t do this with Chase or didn’t do that with Chase- LIVE EACH DAY TO THE FULLEST.) I, being sick as a kid, knew what it was like to feel different and all I had ever wanted was to feel normal- so I refuse to put that on my son. It’s also important that Julie Anne experiences the childhood she deserves to experience. I never want her to develop any resentment towards her brother for not being able to do this or that- so I am constantly checking to make sure her needs are being filled as well. She’s amazing with him- she’ll say things like “Mommy, Chase needs suctioned” or check his heart with her little stethoscope. Now sure there are things she does that he can’t like when she goes horse-back riding that dust would not be good for him so I call in a nurse to watch him so she has that Mommy and me time. And accepting help is NOT an easy thing for me to do. As you’ve read I’ve went through this entire experience alone and I am extremely territorial over my son and his care. But I am only one person and sometimes I need to accept its okay to ask for help (this is still a struggle for me) because most people do have a partner or a family member to alleviate pressure time to time. I am a work in progress with this one…but I have to let my guard down and accept that after all my hard work I need to let life be normal for myself too.

Since moving to Texas both my kiddos are living the life I always dreamed of for them, we have an amazing created family, I started my own business, landed my dream job of being a radio DJ (which is amazing by the way to have a platform to share the causes, stories and music that makes a difference) bought our first home, took in two more pets, and sooo much more. Life is a new normal but it’s amazing. I have to check myself a lot of times because I think about how far each one of us has came. We are living the dream. We are living proof that miracles happen! The anniversary of every significant day of our journey just meets me with shock and awe for where we are. I followed God’s path for me- I never ever gave up hope in this was going to pan out the way I had always hoped and prayed my life would be. Sure, I am a single mom (full custody and no involvement from ex) and it does get lonely wanting that partner- but one day I hope to meet a man who would feel privileged and fortunate at the chance to be in our lives but I have faith God has that in his plans too. You can’t spend your life thinking of what you don’t have, you have to count your blessings for what you do have. And remember, no obstacle is too big for you to tackle. You just have to have faith in the designs of his plan for you- you might not understand why he would have you go through so much pain but you have to have faith it’s for a greater purpose. I didn’t think I could get any stronger than I already was after my illness, then Julie’s and then God sent me Chase and his fight isn’t over- he will have a trach-reversal surgery and third heart surgery which will be a Glenn/Fontan combo but we just celebrated a year of his trach being placed and he is 100% non-ventilator dependent and that is a miracle.

I know that Chase wouldn’t be here without me. God gave me Chase because he knew he wouldn’t need just a mother- he needed an advocate too.  It literally says on the discharge paperwork “non-palliation preferred, upon Mother’s wishes palliation proceeded with.” I will never stop fighting for my kids, there is no obstacle that we cannot tackle together, never ever place limitations on my brood- we are after all- The Three Musketeers.

So remember,

Never ever give up fighting.

Never give up hope.

The next miracle is on the horizon you just have to will it to you.

God bless,

Stephanie

 

Comments

  1. What a story…. I already follow all Czarina’s blogs with a smile and a tear, but I feel so much respect for Stephanie too! Sending all heart-mama’s and their loved ones lots of positive vibes and thoughts!
    Much love from Holland, Maaike