Cruz’s Story

Cruz was born on November 19th, 2013. He was thought to be healthy, passing all his APGAR / newborn screenings, and was sent home from the hospital on the morning of November 21st. That evening, I noticed Cruz seeming extra lethargic, but passed it off as normal baby-sleepiness. When he turned down his 10pm feeding, I decided I better set my phone alarm to go off at midnight, in case he didn’t wake me with normal hungry cries. When the alarm went off and he was still asleep, I looked in his bassinet at the end of my bed, and noticed him making a grunting sound… but couldn’t decide if it was a grunt or a snore. I tried to feed him, but again, he was uninterested. I set the alarm to go off at 2am, and I woke up to my phone a 2nd time… I was highly concerned. What baby doesn’t cry!? When I picked him up to nurse, I noticed he felt really cold, so after another failed attempted at feeding, I kept him in bed with me and watched him intently. I scooted him close so he would feel the warmth of my body, but after an hour of cuddling under my covers he was still cold and clammy, and his grunting had now become more dramatic. “THAT’S IT. I’m gonna do everything I can to wake him up, and if he doesn’t wake up, I’m calling for help!” I turned on my lamp and aggressively tried to wake him up (trying things like flicking the bottom of his feet, and being as “mean” as I could), and he just continued to sleep. I woke my husband and told him I started to get an awful feeling in my stomach, but he took a look at him and said he was sure everything was fine. I got up off the bed and stood holding Cruz against my chest and stared at him with my heart pounding… and i’ll never forget… he opened his eyes for just a moment, and I heard the words, “Mama, I don’t want to leave you yet”, crystal clear. I knew right then that he was dying, and I dialed for help. My firefighter brother-in-law said my 911 phone call came in at 4:01 am… and this is where everything gets worse.

The paramedics arrived at my house and asked where Cruz had been born, but upon hearing which hospital they decided it would be better to take him to a hospital within my insurance plan that had better emergency care. (For personal protection and until some legal matters are resolved, this hospital shall remain nameless and referred to from here on out as Hospital X). By the time the ambulance arrived at the ER entrance, Cruz was pretty much non-responsive, and when they poked him with needles to run labs he didn’t cry. They decided to do a spinal tap and warned me he could get upset, but he didn’t even flinch. He was admitted to the ICU, and over the course of that day they slowly eliminated viral/bacterial infection from their list of probable causes, but he was getting progressively worse. Late that night, someone (I believe it was a cardiologist) suggested that his blood-ammonia should be checked, and when the test results came back, his ammonia was found to be around 750 (normal range for a newborn is under 150). He was now becoming highly unstable, with heart rate and blood pressure going crazy, and we were told most likely he would not make it through the night. They attempted to put him on dialysis to rid his blood of ammonia, but we were told upfront that they didn’t have the proper dialysis machine he needed, and they made us very aware of all the complications that could arise. For reasons we are still investigating, they did not get the dialysis machine up and running properly for MANY hours, and by the time they started to lower his ammonia levels, his number had climbed from the 750s to the 1500s. He was now in an ammonia-induced coma.

He remained on dialysis overnight, with many starts and stops due to machine malfunctions, but I was SO happy and relieved when he was not only alive the next morning, but his eyes were opening! Our joy was immediately squelched when the dialysis machine stopped working all-together, and they didn’t attempt to restart it. We were called into a conference room to discuss Cruz’s prognosis. It was in this room that we were told Cruz had an inborn error of metabolism, most likely a Urea Cycle disorder, but they were unsure of which enzyme within the urea cycle he was missing. The metabolic geneticist sitting at the table with us explained that there are several enzymes within the urea cycle, and that it was possible he could be missing any of these enzymes… but because of Cruz’s extremely high ammonia levels, he was concerned Cruz could be missing an enzyme towards the beginning of this cycle. He went on to say that there are cures for many of the urea cycle deficiencies, but that an OTC enzyme deficiency is the one deficiency that still has no cure. Of course we left that meeting terrified, but still in high hopes that Cruz was missing an enzyme later into the cycle, and that we would be able to come up with a low-protein diet and supplements that would get him back to normal so we could go home. But of course – the tests came back showing he WAS in fact missing an enzyme from the very beginning of the cycle, and that enzyme was OTC.

At this point in the process we were told there was absolutely NOTHING we could do, and that we needed to start making plans for how we wanted to let him go. In our disbelief we kept asking,”IS THERE ANYTHING WE CAN TRY!?“, but we were told that there was absolutely nothing. One doctor said that probably someday down the road science would be at a place where they would know how to treat it, but that Cruz was unfortunately born too early for that. One of my friends (who happened to be a nurse on a different floor) heard that we had been given this fatal news and decided to start doing her own research on OTC deficiency… and she found literature stating that liver transplant was the common cure. She organized a meeting with me and one of the specialists to discuss this information, but we were told that we “must not understand the complexities of his disorder“, and my friend’s findings were dismissed. Independent of this situation, I found out later that my brother had also done his own research and had found this same information as well, and independent from him, my aunt had also stumbled across cited articles about liver transplant as well. But every time it was brought up to the doctors at Hospital X, we were told that information was incorrect, and none of them would look into it any further.

Days crawled by, Thanksgiving had just passed, and Cruz was still alive… with his ammonia levels hovering between 150-450, being managed with an ammonia scavenger, as per my request. He was put on Comfort Care, which is just a tender way of saying “we will keep your child comfortable until he dies, but we’re not going to do anything to prevent him from dying”. He wasn’t being fed anything but intravenous sugar, and he dropped from 10 lbs down to about 7. There were nights when Cruz was choking and gagging on secretions around his breathing tube, and I would ring for the nurse and no one would come. I would go out into the halls or send my mom/sister to ask for people to help, but it would sometimes take 10 minutes before someone would respond, so I started using the suction tool all by myself and got used to taking care of him on my own. The doctors had suggested that I stop ALL treatment (including his respirator), as to not prolong the inevitable, but I couldn’t bring myself to stop everything because somewhere in my heart I was sensing something was off. I refused to be the one to make the decision, and I kept praying, “God, if you’re gonna take him THEN JUST TAKE HIM! Don’t make me be the one who has to choose whether he lives or dies!” But Cruz wouldn’t die, in fact, when you looked at him it was hard to believe he was dying, because with his ammonia levels somewhat under control (even though he should have remained on dialysis until his levels were within a safe range) he was acting like a normal baby. He had his eyes open, and he would look at you when you talked to him… needless to say… I wasn’t going to “pull the plug”.

I did however organize to have him dedicated, because I decided that was something I wanted done before he died. We brought in all the family and some elders from our church, and my father-in-law (who happens to also be our pastor) dedicated him to the Lord just like I had Ezekiel dedicated when he was a baby. While Pastor Jess was still praying, I saw a doctor take Jared out of the room. At first I was upset, because I couldn’t understand why they needed to interrupt an irreplaceable moment with our dying son when they had been so inattentive the past few days… But when Jared returned from speaking with the doctors, he came bearing the best news I’ve ever received: Cruz was eligible for liver transplant! A doctor had returned from his Thanksgiving vacation that had spent many years at Stanford University, and he knew about all the treatments available for boys like Cruz. I could hear the hesitation in his voice when he sat down to talk to me, because I think he knew that his help was coming 2 weeks too late. At this point Cruz’s ammonia levels had most likely done rigorous damage to the brain, and that was something that couldn’t be fixed. But he also said there was no way of knowing whether Cruz had significant brain damage yet or not, so he left it up to us whether we wanted to leave and go to California to pursue transplant treatment. OF COURSE WE SAID, “YES!“, IN ALL CAPS! So, he contacted Stanford to see if they had any rooms available, and arranged for the life-flight. I cannot tell you the amazing feeling I had in that moment… I could never describe it in words. For the first time in over two weeks, I actually slept in the bed next to his and got some rest. HELP WAS ON THE WAY.

But the strangest thing happened. When I woke up, the PICU team was outside Cruz’s room discussing the plans for the day, so I decided to join. They were talking about starting a bunch of invasive procedures, but there was no talk of Stanford coming to pick him up. I looked at the lead doctor with a confused look on my face, and she informed me that Stanford wasn’t coming until tomorrow, because “we like to get our patients ready in a neat and orderly package to hand off to other hospitals before transferring them”. Looking back at this moment, I wish I would have said, NO, I’m NOT ok with this decision, call Stanford right now and tell them to get here ASAP,  but at the time I didn’t think I was allowed to make such demands.

And this is the day that changed everything. This one extra day – I  firmly believe – cost him his life. They surgically inserted a catheter into his neck that was way too big for his vein (Stanford later informed me), and restarted dialysis. The dialysis machine had TONS of issues, and Cruz became highly unstable. He went from a normal looking baby, to a HUGE balloon baby in a matter of hours, his heart rate shot sky-high (higher than ever), and his blood pressure plummeted. My sister was the one who discovered the blood pooling under his head, which had been leaking from the catheter in his neck, but the surgeon who had placed his line had already gone home. Instead of having another surgeon come and help, they paged/waited for this surgeon to return before Cruz’s bleeding was fully stopped. I’m not sure how much blood Cruz lost that night… but the amount of blood was so excessive that it soaked through every layer of his bedding, and the puddle surrounding his head was deep enough that every drop made a splash.

By the time morning came, it was touch and go… every minute with him was fragile, every second uncertain. I was told that Stanford was on their way, but that the team was worried he would pass before they arrived. I stood there standing over his bed thinking, WHAT HAPPENED!? How could he have gone from stable these past 2 weeks to THIS condition overnight? Why did they start all these invasive treatments if they knew Stanford needed him stable for transport? Why would they try to dialyze him when they don’t even have the proper renal team? Are they trying to make it look like they’ve been treating him when they’ve actually been doing nothing? Why didn’t Stanford come yesterday? Why would they delay? OH IF ONLY they wouldn’t have delayed!!! I was looking at my baby, struggling to stay alive (boy was he a fighter), and feeling like somehow his shot at life had been compromised in the last 24 hours. All I knew was, Stanford was coming to rescue him from this place, whether he made it to transplant or not, and just knowing that someone was going to give him the fighting chance he deserved was all that mattered to me. The lead doctor came into the room and sat with me on my pull-out bed, and urged me to stop going forward with the transport. She said he probably wouldn’t make it from the hospital to the airport, and that if he didn’t die on the ambulance, he would most likely die on the plane because of the elevation, and that it might be a very dramatic experience for me. She asked if she could call Stanford to call the whole thing off and I said, “If he’s going to die, he’s going to die with me trying to save him. I’m going to keep going until his heart stops”. I couldn’t believe my ears! Why did she just ask me that? What parent in their right mind would call everything off on the CHANCES that their child might die, when the only other alternative is that they die FOR SURE? I was so perplexed. My sister Kenzi was sitting there with us as well, and after the doctor got up and left the room, Kenzi said, “Is it just me or did she seem frustrated with your decision to continue?” Both of us were highly confused at what was happening…

Once Stanford’s incredible transport team arrived, they safely moved Cruz from his bed to the isolette (like an incubator), and I left with them in an ambulance to head toward the airport. He made it from the hospital to the private plane, from the plane to the next ambulance, and all the way to Stanford Children’s (LPCH) without any scares. I walked through those double doors and Cruz was whisked off to the ICU while I was escorted to the parent’s room where there was hot tea, juice, snacks… I walked into that room and felt like I had entered an alternate universe. I’m surprised I didn’t break down right then and there… but I think I was on Cloud 9 realizing… HE MADE IT. He spent the whole night on dialysis, and he had 10-20 people doctors and nurses hovering over his bed at all times. It was so weird to think that just a few days ago, Cruz was laying in a hospital bed without an assigned nurse, and now he was the center of attention in the PICU. Did heaven just touch earth? How did my/his reality take a such a complete 180? By the next morning, Cruz looked like himself again, and he had re-stabilized. I was brought into a conference room and sat down at a table with the lead geneticist and probably 10 other specialists, and this is when I found out just how terrible the care in Oregon had truly been. They asked me tons of questions like: Why wasn’t he being treated? What were you told about current treatments? How high were his ammonia levels when they were first tested? On and on the questions continued, but there was one question that stood out to me the most: “Why did you postpone transport?” I told them I was not the one who postponed, and that I was of the understanding that Stanford postponed. They said, no, that they had there team all ready to go and right as they were about to leave they received a call from Hosptial X saying to turn around and not come until tomorrow….

Hearing this news devastated me. I could not figure out for the life of me why Hospital X would have postponed life fight and took it upon themselves to start treating him. Any why did they try to get my permission at the last minute to cancel transport indefinitely?

Long story short, the Stanford PICU team went from being quite confident that Cruz was going to live – in fact, very confident – to realizing that Cruz had many more complications than the average OTC patient. They told me that he was the 3rd boy they had treated just that month, and that their infant liver transplant success-rate was at 99%, but that Cruz was not proving to be a textbook case. He lived another 2 months in the Stanford Children’s PICU, but those 2 months were full of question marks; why can’t we keep his blood pressure stable? Why does he need so many transfusions? Why does he have so many infections? Why is he this? Why is he that? And none of these complications were normal symptoms of a typical OTC baby, which caused them to keep going back to the drawing board looking for answers. Eventually they came to us and said there was nothing they could do, because he was no longer a candidate for liver transplant. All his organs were failing… this was indeed the end. We made the decision to stop invasive treatment around 10pm on January 29th, 2013, and he passed away at 7:04 the next morning. (You can read more on that unforgettably painful night here). They asked me for permission to do a full autopsy, to which I said yes with no hesitation. He had arrived at Stanford when he was 14 days old, and died when he was 2 months 11 days, with no conclusive reason as why he couldn’t be saved. I know some parents don’t like the idea of their child being cut open and sewn back up, but I wanted answers.

A couple months later, we received a phone call from Stanford to discuss autopsy results, and it was just as I had feared. Cruz had multiple blood clots in his body, and the two largest clots were in his neck, where the dialysis catheters were placed by Hospital X. They said these clots were probably caused by those catheters, and by starvation/poor nutrition. The clots were completely blocking all blood flow from the heart to the brain and back, but his determined little body had grown several tiny veins around it to keep the blood traveling… these new veins were too small to allow for proper blood flow, hence, his extremely low blood pressure. Pieces of the clot (emboli) had broken off and traveled to his brain causing  a stroke, and they also found emboli in his lungs. When we received the official document in the mail stating the causes of death, it listed #1 cause of death being blood clots, and #4 being OTC deficiency. It was hard to see that OTC deficiency wasn’t listed as 1, 2, or even 3… but dead-last…….

I got in contact with the executive directer of the National Urea Cycle Disorder Foundation and discussed the ins/outs of my story, and she was blown away at the level of malpractice that took place here in Oregon. I also contacted the American Board of Genetics and Genomics to inquire about a certain doctor, only to find out that he was not certified, nor did he attend the 8 years of schooling required to become a geneticist. He was a pediatrician-of-sorts, wearing a hat much too large for his expertise. I was told that this “incurable” disorder has actually been curable since 1988, and that thousands of boys like Cruz have been rescued via liver transplant across the world. It’s such a known cure that doctors in the Philippines are educated enough to send their OTC patients to the U.S. for treatment. I was told that an OTC center of excellence was just 5 miles down the road from Hospital X, and that one of the top 10 OTC specialists in the country is located there. Why Hospital X didnt at least entertain the thought of transferring Cruz a few minutes down the road to a teaching hospital that is known for their research in genetic disorders is BEYOND ME. The only comment made about this teaching hospital by a doctor at Hospital X was a negative/daragatory one… so it’s clear to me that these two hospitals must be in direct competition with one another.

Regardless – Cruz was born with OTC deficiency, which requires immediate proper treatment and eventually liver transplant (at around 3-6 months old), but he was never given the opportunity to be put on the transplant list because he was at a hospital that didn’t have the proper team or equipment to take care of him, and his main doctor was practicing without a license. I have attempted to pursue legal action, but 4 lawyers have now declined the case. One lawyer said “an employer has the right to decide whether their employee is fit for the job or not, regardless of education”, and that hospitals will do anything and everything they can to cover their tracks and get away with it. Another said she didn’t think it would be worth my time and energy because after paying her 30% fee, she doubted I would come out on the other end with more than a few thousand dollars due to Oregon’s cap on death claims… which wouldn’t even come close to paying the medical bills that were now 50K+ (after insurance). The other firm didn’t really give an explanation, they just said it was not something they wanted to take on, and the 4th said that the likelihood of winning was too low, and after investigating they felt like there wasn’t enough evidence in the medical records to prove that anything done at Hospital X was less-than appropriate treatment.

I am still in the process of getting this whole mess figured out. It’s not about the money, it’s not about revenge… It’s the thought that one out of every 8 thousand babies born will have a Urea Cycle disorder, and Hospital X is considered to be one of the best hospitals in Oregon – therefore Cruz will not be the last baby to end up at this hospital being treated by a doctor who’s not educated in his field. I can hardly sleep at night knowing another boy could go through the same thing Cruz did, and another family could be losing their child. I have thought about going to the media and using that as a platform to get my side of the story told, but I was warned by my lawyer that they could turn around and sue me for slander. I feel stuck between a rock and a hard place, but my stubborn personality will figure something out. I can’t give up. Cruz’s tired little body may have worn out after fighting so hard for so long, but I will keep fighting for him… and for all the potential OTC babies born across the world ❤

You can see more pictures of Cruz during his journey from birth to death on my Instagram, @mycolonialreMODel or under the hashtag #iheartCRUZ

Advertisements