Two weeks ago today we almost lost my 9 yr. old niece to sudden cardiac arrest. I haven’t been able to sit down and formulate one coherent sentence since that day. I thought maybe a week after the event I might be able to write a few words but there were more pressing things that needed my attention like calling family, washing my niece’s blankie and praying. Lots of praying. I have felt the urge to share the experience as I’m sure you may have wondered why my blog when silent but a part of me is still very much in process mode. Nothing could have prepared me for what happened on July 8th. My sister and I talked about me writing again and initially I thought no way I just don’t think I want to share something so private but that probably would mean that the blog would just sit here and get stale because right now I really can’t think of ANYTHING else I would rather write or talk about. With the blessing from my family I would like to share with you what happened on Sunday July 8th.
There was clearly a plan that day.
The day before on Saturday I received a call from my sister, she wanted us to come over for a last-minute BBQ, we were celebrating a belated birthday for my husband. We went back and forth with messages about whose house and what time would be best because I was supposed to be in Los Angeles early Monday morning. Honestly I came ‘this close’ to asking for a rain check but apparently someone above had other plans. This makes me think of the movie The Adjustment Bureau, if you have seen it you will understand how it felt like my attempt to deviate away from my fate was rerouted and we were exactly where we were supposed to be at 4:24pm Sunday afternoon.
We decided to push up the time and meet earlier instead. My family and I arrived at my sisters home, they live about 30 minutes from us, bathing suits on and ready to get wet. I brought my instant cameras to take pictures of my nieces and slapped on my new Lifeproof (underwater) case for my phone so we could film the kids swimming underwater. Lily LOVES taking pictures with my phone. I can remember a few years ago I brought an underwater camera to her house and all we did for hours was film Lily swimming, she is our a little dolphin girl. I gave Lily my phone that afternoon and she took photos of her dad and I and a bunch of feet shots while all hung out in the jacuzzi. Dad and Lily were taking turns showing us their jack knife dives. I video taped Lily as she jumped off the edge of the waterfall and into the pool. She would land smack on her back and still come up laughing. She is one tough girl.
Some of Lily’s shots that day:
Lily continued to do this until after one dive as she swam to the edge of the pool and let out a big yell. My 15 yr old was standing above her at the pools edge and saw her appearing to freeze up as she leaned backwards towards the water. A nano-second later her dad dove across and grabbed her out of the water and rushed her into our arms. She looked like she was having a seizure. I was holding her as she moaned, her arms and hands folded in as she began to curl into a fetal position. (later we found out this is called posturing which if becomes a permanent condition is an indicator that traumatic brain injury has occurred.) I thought this was just a seizure and expected her to come to and start crying but within seconds we could see that something was very wrong, she didn’t appear to be breathing. As she laid there on the grass my husband and her father on either side, I hurriedly pleaded for 911 operator to send an ambulance. She seemed unresponsive, my husband started CPR. The 911 operators (my sister and I were on separate 911 calls) were adamant that we NOT apply chest compression if there was even the faintest of heart beats. I can not find the words to describe how difficult this was, so many things are coming into your mind at once and your natural instinct is to save a life and to not perform CPR when you can’t tell if someone is breathing or has a pulse was excruciating. The top of my head felt like it was on fire. My husband was doing his absolute best to keep it together. Every time my brother in law would yell at Lily “TAKE A BREATH!” she seemed to respond with a breath, our fear is that this is the only oxygen she got those eleven minutes that elapsed from the time of our 911 call to the paramedics recording a pulse. We anxiously listened for the faintest sound of sirens. Time seemed to stop. When they arrived on scene it was all I could do not to run and grab them by their shirts and drag them through the entryway. I started to pray as I watched the team calmly work in synchronization to get Lily’s heart to regain a steady rhythm. It was out of our hands. I grabbed my sister and told her to go put on dry clothes. She grabbed Lily’s comfort blanket and her rainbow rosary and drove in the ambulance to the ER.
Getting her to an Emergency Room
My brother in law and I jumped in his car and followed them to the hospital. We were placed in a small examination room next to Lily’s. We were also joined by a neighbor of Leslie and Jim’s who is an anesthesiologist who was able to translate in laymen terms everything that was happening as it unfolded, I can’t tell you what a blessing it was to have him there. Within 30 minutes of arriving the doctors wanted a cat scan to see what they were dealing with and rule out a head injury. I stayed in the ER while my sister and brother in law went in another direction. As I sat there on the gurney I heard an alarm sound over the loud speaker the words “Code Blue, Cat Scan… Code Blue Cat Scan” I felt the rush of the blood as it left my head. I thought to myself ‘Oh my God, that’s Lily’. In one big whoosh every doctor and nurse in the ER went running I saw the neighbor run so I followed, tripping over my flip flop sandals I stumbled to keep up. Rounding corner after corner people in white coats seem to join the group as it passed entryways. The group was growing. There she was on the table, a team of people all trying to resuscitate her…. again. Her little body was enduring heavy chest compressions. Her ribs bounced back like a coiled spring. I clasped my hands, prayed and pleaded with God. I was willing to give up anything to have her stay with us and as the seconds passed I had an even more urgent message to God, I told him he couldn’t take her, not yet, and NOT WITHOUT MOM AND DAD HERE. I begged the Lord to please wait for her parents to arrive, no one knew they were in the lobby. I looked at the security officer and told him you HAVE to find her mom and dad, you HAVE TO and he did. Within seconds they appeared and were able to be there and hold her hand as they called out her name. It was a miracle. Seconds later her heart settled into a rhythm and they were able to quickly get a scan of her brain. The conclusion, no obvious signs of head trauma, it was our thought that the doctors though maybe we didn’t notice her hitting her head on impact with the water. We knew it wasn’t head trauma.
Lily was immediately placed in an ambulance for transport to Mission Hospital a few miles up the freeway, a trip that I’m sure seemed like forever to her parents. My sister rode up with her in the ambulance as we followed behind. Once we arrived at the new trauma center a local fire fighter named Kevin (bless him) kept us updated every five minutes as to what was happening, no one was allowed to see her not even her parents. Here we were only an hour out from swimming and playing, sitting in a room with our bathing suits still on being greeted by the hospital chaplain, this my friends is not a good feeling. A pediatric intensive care doctor from CHOC Orange came down to be with Lily and never left her side her first night.
I went home around 10 pm and sent out a prayer request, “no codes in ICU and safe transport for Lily in the morning.” I clutched my phone and asked God to please not let it ring. The next morning I awoke at 6am the sun was shining and my phone was still silent, PRAISE GOD I said. A few minutes later my sister sent me a text message as they were passing by my neighborhood with Lily on their way to CHOC Orange. Soon she would be settled into her new home in PICU.
Within the first 24 hours both my parents flew in from the east coast. I hadn’t seen my dad in over two years, it was a bittersweet moment to see him walk down the hallway. My mom able to grab the last seat on the last flight into our local airport and arrived just after the 11 pm airport curfew Monday night. We all went back to my home, a few miles from the hospital and talked as we tried to wrap our heads around what had happened. We were beginning to learn from the doctors that she would most likely be sedated for a period of time to give Lily’s heart a chance to rest. At the time we had no idea what caused this and doctors were anxious to gather as much information from us as possible about the events leading up to the moment of her cardiac arrest. I wonder if the cardiologists suspected CPVT from the beginning given her age. We stayed up late searching the internet for answers, at this stage you are running on pure adrenaline which turns out to be the antagonist in this story.
Sleep doesn’t come easy for those who wait.
View from Lily’s room at CHOC, 6th floor PICU St. Josephs church in the distance.
The days in between that first and today kinda melt together into one long one. We have been through rough patches but each day seems better than the last. We quietly celebrate the little things like clear chest xrays, coming off the high dose drugs, extubation, removing oxygen tubes and then the biggie, her heart study last Monday where doctors went through her femoral arteries (entry point where you leg attaches to the body below your hip joint) with wires and cameras that looked at all the various arteries and cardiac structures to rule out the possibility of any physical impairments. There was nothing to see but what appeared to be a healthy heart. Doctors also stimulated the heart and in the absence of sedatives and heart soothing medication, agitated the heart to the point of arrhythmia so they could witness from the inside what was happening as she went into this potential fatal rhythm. There were no findings that indicated she had a structural defect which further suggested the initial suspicion of CPVT.
CPVT = Catecholaminergic Polymorphic Ventricular Tachycardia
CPVT affects 1 in 10,000 kids (source) and usually presents itself between the ages of 7-9. And although this seemed to appear out of nowhere, my sister recalled some very acute instances that were possibly precursory to that fateful day. Two years ago when Lily was 7 she fainted while on a ride at Knotts Berry Farm. Her dad was with her and said he recognized the same signs as she swam towards the edge of the pool. That morning of our BBQ Lily also said something very interesting, out of the blue while she rode in the car, she reminded her mom of that time she had a seizure at Knotts Berry Farm, if that does not give you goosebumps I don’t know what will. Doctors speculate that at the very early stages of her episode, Lily most likely experienced a sensory cue that triggered a memory of the same feeling she experienced at Knotts Berry Farm. We were told some epileptic patients can tell when they are about to have a seizure because they some times experience a visual aura or detect a certain taste or smell. We may never know the events that lead up to her sudden cardiac arrest but it would seem that Lily’s memory recall was undoubtedly a bit foreboding.
This is the couch turned sleeper bed where we sit and watch miracles happen. All around the room are photos of my niece Lily.
What we do know now is that the only way to truly diagnose Lily is to do genetic testing which is in place and set to happen as soon as she becomes an outpatient. Yes, due to health insurance policies the only way she is covered for this very expensive blood test is if she has the blood drawn as an outpatient. Mind boggling. Her family is anxious to start the process so they can figure out the best plan of action to make sure that her sister is treated in the event that she too has the same genetic pattern. Trust me I have been reading up on how you get CPVT and its complicated, it has to do with a genetic mutation.
- CPVT can result from mutations in two genes, RYR2 and CASQ2. RYR2 gene mutations cause about half of all cases, while mutations in the CASQ2 gene account for 1 percent to 2 percent of cases. In people without an identified mutation in one of these genes, the genetic cause of the disorder is unknown.
- The RYR2 and CASQ2 genes provide instructions for making proteins that help maintain a regular heartbeat. For the heart to beat normally, heart muscle cells called myocytes must tense (contract) and relax in a coordinated way. Both the RYR2 and CASQ2 proteins are involved in handling calcium within myocytes, which is critical for the regular contraction of these cells.
- Mutations in either the RYR2 (Lily was diagnosed with RYR2 mutation) or CASQ2 gene disrupt the handling of calcium within myocytes. During exercise or emotional stress, impaired calcium regulation in the heart can lead to ventricular tachycardia in people with CPVT.
In about half of cases, an affected person inherits an RYR2 gene mutation from one affected parent. The remaining cases result from new mutations in the RYR2 gene and occur in people with no history of the disorder in their family. This means that there is a 50% chance that the gene mutated on its own in my niece and that her sister or related family does not carry the mutation. There is no history on either side of her family to suggest we carry the mutation. Only genetic testing of her mom and dad will confirm its true origin. *Later test confirmed that the gene spontaneously mutated in Lily’s case.
So much to think about while you worry about your child. I know that my sister and brother in law are taking each day as it comes. We set up a Lily’s Caring Bridge site if you would like to follow her on the road to recovery. You will see that I have been busy with updating her site, its very therapeutic for this Auntie and she has over 4000 visitors already. The site has been a wonderful way to keep everyone updated on Lily’s condition as well as have a place where people can leave messages to remind my sister and her husband that they are not alone. My sister and I have talked about building Lily a more permanent site, like her own blog where they can share their journey as well as reach out to other parents going through similar circumstances and spread awareness for the need of CPR certification and the installation of AEDs in ALL SCHOOLS, GYMNASIUMS, FIELDS, PUBLIC SPORTS PARKS, MALLS you name it. If there is one thing you can take away from my story its this… knowing how to perform CPR may save a life, possibly YOUR OWN FAMILY MEMBER’S.
I know it saved Lily’s.
We are facing a week of changes as they prepare Lily to move out of intensive care and into an area of the hospital that can focus on her neurological recovery. This is time where we need patience and as an auntie I get breaks for extended periods of time so I can see progress whereas subtle differences in days may not seem so apparent to mom and dad. My new role is hair washer, bath giver, hand holder and reader of the stories and messages left on her guest book. I love my time alone with Lily where I can connect with that little light inside that is trying to figure all of this out. I know its not easy on her and our job to is reassure her that she is always safe and surrounded by her family and friends. I know the road will be a long one and I’m ready for this new chapter in my life. Lily has certainly given me the gift of perspective through all of this.
I can’t wait for the day we are able to look back on this as a distant memory, just a blip on the radar as we move forward to a full recovery. My heart has only room for hope.
updated: July 2013 Lily has her own site Lilyneedham.com please come see how Lily and her TEAM of angels continue to defy the odds!!