Friday, August 12, 2016
A lot of people have asked over the last three months what Kreed had and what he died of. The answer is as complicated as his life was. Nothing with Kreed was every simple or easy. Neither was his death.
When we brought Kreed into the Children's Hospital at Denver, Kreed was already dying, just no one knew it. By the time we brought him to the hospital, it was too late. He was starting his three month decent toward his death. It felt like a tidal wave had hit our life and Kreed was caught in the undertow. Never in those three months did I think we were losing him until the day he stopped breathing on May 8th, 2016, Mother's Day, about four days before his heart stopped beating.
Kreed has been sick with odd things his entire life. Sometime later I will go in more depth. What's important for his diagnosis is the fact that he had megaloblastic anemia. Combined with developmental disabilities, peripheral neuropathy and an immune deficiency. Those four things led doctors to suspect that Kreed might have a biochemical imbalance, a metabolic condition. They ran one simple blood test. A test for about $90 we could have known something was very wrong with Kreed. That test came back high and it told doctors he had one of 14 colbalimum and folate disorders. They ran one more test and narrowed it down to 5. Each equally as rare. One disorder there are 14 cases. Another there are 48 cases. The one they suspected there were only 5 cases. All that had to be tested was his homocysteine. An elevated homocysteine level tells doctors that your body has a breakdown in the way your body is processing B12 and folate and there is a breakdown somewhere along the way. It's a remethylation defect. Further tests revealed Kreed had very low folate in his cerebral spinal fluid which was the cause of a lot of his issues including nonverbal and developmental disabilities. A lot of damage occurred but it was biochemical damage not seen on an MRI.
But flashback maybe four to five years ago, Carie and I were going through his labs and noticed he had this megaloblastic anemia in every single lab of his from doctors. We flat asked his geneticist at the time (in Phoenix) what those labs meant and why they were elevated and he just said Kreed has megaloblastic anemia- he just has large red blood cells, nothing to worry about.
Nothing to worry about as we sit with our child buried below ground. Nothing to worry about as he lay dying in our arms. Nothing to worry about as he has suffered for years and years with pain and breathing difficulties unbeknownst to us. Nothing to worry about. I will never worry about something more.
Flash forward and we spent 15 minutes with the metabolic doctors in Denver and brought them our files of labs and doctors notes and they saw the pattern and ran the test the next day and just like that they knew.
With so few cases, it was hard to pin point which disorder he had and what it would mean. Kreed still presented somewhat atypical.
When we brought Kreed to Denver it was because he stopped walking and his legs were in massive pain. What we didn't know was his nerves that ran from his brain stem to his lungs were short circuiting. That caused him to hypoventilate both awake and asleep. He had become hypoxic throughout the day starting in December but that's just when we decided to start testing him more regularly. Likely it started well before that. We also didn't know that the chemoreceptors in his brain stem were irreparably damaged. What caused that damage, we don't know exactly.
(First night at Children's, he was unable to walk and in pain)
(He continued to be in so much pain in the hospital- his oxygen was also dropping)
Chemoreceptors are what keeps your body in a state of homoestasis. It keeps your ph level in your body stable and monitors the oxygen and carbon dioxide in your body. A normal body will begin to hyperventilate when carbon dioxide rises in the blood. It's a protective factor that your body employs. Kreed did not have this protective factor. This is why he went into respiratory acidosis three times over the three months. It's now also believed that he went into respiratory acidosis way more than we ever knew.
Rewind to 2014 and we admitted Kreed to a hospital at the end of January. He was laying on the ground barely conscious. We have videos that shows it perfectly. We spent one week there- seizures were negative. Heart was fine except fast heart rate. They didn't check anything else. The main pediatrician said we were overreacting and it was just autism. Just autism. Autism doesn't cause a child to lay on the ground passed out every single day barely able to get. Doesn't cause his heart rate to soar to 190 just standing up. Doesn't cause his legs to look bright red and his feet to curl and have difficulty walking. Autism doesn't cause tremors in his hands and loss of bathroom functioning. We were never more horrified with this doctor. Blood gas was never checked. Despite the fact over night his oxygen levels fell. Every night. He got blow by oxygen. They didn't give him a sleep study then. We waited for weeks once we got out. We had to call them and tell them that we still didn't have a sleep study. We showed up for another outpatient procedure (nerve test) and the anethesiasolgist wouldn't put Kreed under without seeing a cardiologist and sleep study. Within a month he got his sleep apnea diagnosis, POTS and neuropathy. Blood gasses were never checked or mentioned despite the fact that Kreed needed ventilation at night for no reason. Nothing on brain MRI. No indication of why he needed nighttime ventilation. And they never checked his oxygen again after the study. For a year. I called and told them he looked blue. I could tell his oxygen was falling at night. They said if I was that concerned to go to the ER. I begged for another study and to match his sleep hours. They agreed to the study but didn't change the hours. Despite Kreed going to bed at 3am on average, the did the study only to 5am. Kreed never slept that night. I still told them his oxygen was low can't they just give us a monitor at home and supplemental oxygen. They said there no need for any of it.
(This is NOT autism...its hypercapnia, POTS, neuropathy and sleep apnea- all undiagnosed at the time).
We moved to Colorado and Kreed was inpatient for surgery and they immediately noticed he needed oxygen with sleep. Phoenix didn't. And he was hypoxic for an entire year. 365 days of not getting enough oxygen at night.
You know when Kreed's death spiral began? January 2014 when the doctor told us when we were afraid Kreed might die that we were over-reacting. Two years later we buried our child. So yes. Yes we were concerned for our child.
Fast forward to February 2016. Kreed couldn't walk. His legs were in immense pain. He had difficulty keeping his Bipap on at night. Probably due to high co2 levels. He was worked up, decided his neuropathy was flared up. Seemed to be doing better after two weeks, we were thrilled. He had his homocysteine test and the metabolic docs began their work up so we also had a looming new diagnosis that would hopefully change his life. He started some early treatments. He was hypoxic on and off so we just used supplemental oxygen more.
Six days later Kreed suffered a devastating seizure outside in our back yard. He had a full tonic-clonic seizure and I sat there as Kreed went lifeless in my arms and his heart stopped. I started rescue breaths and hit his chest. He gasped for air and came back to us. We thought his epilepsy had gotten worse. Three months later experts agree his co2 likely rose to 100% and triggered a seizure. Back to the hospital we went for another week to change seizure Meds, stop his new status migraine with painful DHE treatments and continue his metabolic work up.
He got better. We went home for several weeks. Things were difficult as his new seizure Meds caused severe issues. We switched and he got better. Then we noticed his oxygen was dropping a lot. At night. During the day. A blizzard was coming. We took him to the urgent care connected to the hospital. His oxygen continued to be difficult. They didn't know what was wrong. They ambulanced him to Denver. We stayed three days, got antibiotics, he seemed better and oxygen improved. We took him home happy. Four days later, his oxygen continued to drop. He wasn't better. We drove to Denver and they admitted him again. This time for 36 days. This was Kreed's true spiral.
His oxygen was always teetering on the edge. He had every test known to man. So much came back normal. But he couldn't breathe. A trach was talked about, but Kreed would never survive that. Then they thought pain was causing his breathing issues. He went on better pain regime. He seemed to get better. Then they tested his blood gas. Co2 was 70% in his body. We went to the ICU. He was ventilated with his Bipap for 24 hours and got better. Then for two weeks people missed the fact that he wasn't wearing his Bipap at night like he should have been until I caught it and added up his symptoms. I begged them for three days to check his blood gas. Things didn't make sense. He wasn't doing well but lungs were fine. On the third day they tested it. Then we grabbed Kreed and took him downstairs for some snacks on his scooter. Smiling. Excited for his food. His CA got a call that we had to immediately return to his room. I turned to them and said I bet you he has high co2. The doctors met us with frowns and started apologizing. His Co2 was 91. 91. 91 was his CO2. Back to the ICU he went. Ventilated for 24 hours and back to normal. They checked it twice a day for several days, he was right on target, wearing his Bipap. His oxygen levels improved. Still weren't great, but okay enough. He seemed awesome. For a whole week. We did everything we could at the hospital. We had as much fun as humanly possible. He was so joyful. So happy. It was like a bright light of sun shined down on Kreed. He seemed the healthiest he had ever been. I hadn't seen these smiles in years. I hadn't seen him swing and do his scooter in so so long. Never did I think that this was his energy burst before a sharp decline. But it was.
(His carbon dioxide level is 91 here. He is maybe hours from a seizure and possible death- this is why he was so complicated- do his favorite activities and he didn't care)
So we took him home. He did one more night at the hospital. He had some difficulty wanting to wear his Bipap but he did for five hours. So we went home. Things weren't great when we first went home. He had several rages. I did not have him hooked up to monitors at first. When we did, he was dipping low. So we gave him his Bipap on and off all day. He seemed to be in pain so we gave him some pain Meds and his medical cannabis. He fell asleep at 10pm. Exhausted we went to sleep across from him. We had him hooked up to his monitors and a camera on his monitors so we could see. Carie and I woke at midnight because his monitors were going off.
And for the rest of the night we were dumbfounded because at times Kreed wouldn't breathe at all, sometimes for 20 seconds or more. His oxygen would stay crashed even on five liters of oxygen, the most we had at home. It was the longest night of our life at the time as we were exhausted yet trying to make sense of what was happening. Finally he fell asleep in the wee hours of the morning and continued to sleep solid until late morning. What we didn't know was he had 68 apnea events an hour that night. His decent had started but we didn't know. Didn't think to pull his data from that night from his Bipap until later in the day.
It was Mother's Day the next day so I wanted to take him shopping for Carie. I took his monitor and decided to watch him continuously. And continuously his monitor went off. Oxygen at 75, then 83, then back up to 95. I took him to Five Guys and he happily ate French fries while his monitor silently beeped at me the entire time. Back in the car I gave him supplemental oxygen while we drove around. Next up was a hair cut. He did amazing as we did this, then back in car with oxygen. Finally we decided to go into Walmart and get a card for Mother's Day. We walked in, Kreed seemed to start to panic a little so I tried to comfort him and we walked over to the cards. He scanned a bunch and I questioned him on his device as to which one he wanted. He picked one out. Then we started to discuss gifts and all the sudden Kreed broke into complete panic. You could see it in his eyes- complete panic. I tell him we will go to the car.
Once in the car, I get a reading on his oxygen - 65%. Ugh. I put on his oxygen and we keep it on. At times he needed up to 10-15 liters of oxygen and other times 2. It varied so much. We decided to drive home. Once at home we hook him up to the pulse ox machine and watch him. His oxygen continued to fall. We hooked up a second machine to make sure they matched. Our house rang out with the sound of nonstop alarms. Carie and I just kind of look at each other and Carie starts packing. She saw the look in my eyes. I don't want to go back. That's all I can think. I don't want to go back, we had been home a total of one whole day. Then I turn to Kreed and he looks so bad and suffering so much. He's upset and alarms go off nonstop.
Kreed's brother and his wife also come home from work and sees the alarms going off on Kreed and his irritable mood. He also thinks we should go. He also asks if he can come because if Kreed loses it, he will be hard to keep oxygen on. I agree.
We pack up and hit the car with Kreed. We decide to go to Childrens Colorado urgent care rather than the drive to Denver. We just felt to unsafe and during our drive down the mountain, Kreed goes in and out of consciousness. We arrive at the urgent care center and they assemble a team for him, as they know he can be difficult. They immediately check his blood gas, as that would decide if we go to denver or not and what's wrong.
Ten minute later the nurse in and says, it's time to go to Denver. I ask him what his level is...his carbon dioxide rose to 96%. Kreed was suffocating. A helicopter transfer or icu ambulance was discussed. Meanwhile Kreed finally lost it and his brother and I had to restrain Kreed. Various medications and shots were given as Kreed began to sweat and fight us and throw up. It was a mess.
The icu ambulance finally arrives and we are off to Denver. His Bipap is put on to ventilate him and hope to get his levels back to normal.
By the time we arrive straight to his icu room and being ventilated for an hour, Kreed's levels began to go back to normal. He was in such severe respiratory acidosis. This current episode took its toll on his body. He fought and was exhausted. In the early hours of the morning his levels were back to normal and he could take his mask off. It felt like a whirlwind- from swinging and having a blast at him to the icu in less than 24 hours. What was happening to our baby?
Morning came and we all relaxed somewhat as Kreed seemed to be in better spirits and eating breakfast. Doctors told us we would definitely be staying in the icu for a while to figure out why this keeps happening. Another EEG was ordered as well to help see if seizures were causing oxygen issues. Kreed's stats still varied greatly.
In the late morning I stepped out to talk to his icu nurse about everything. While we were filling out paperwork, we kept looking at Kreed through the glass- he looked grey and just ill. We kept saying how bad he seemed to look, but all his stats seemed normal enough. We even ordered a blood gas that came back normal.
At about noon Kreed requested his Bipap and it seemed he wanted to take a nap. We put his Bipap on and thought it was great he wanted a nap after not sleeping all night and so much fighting. We all relaxed for a bit.
At 12:30pm the EEG tech came to put Kreed's leads on for his test. We would have to take his Bipap off to do so. I walked in and decided to take control of his blow by oxygen in case he had difficulty (his oxygen always drops when sleeping).
We slip his Bipap off. Maybe within a three second period Kreed's oxygen plunges- 80, 75- we move the blow by closer. 70-65- the nurse yells to seal the blow by oxygen around his mouth. 60, 55...45. The nurse yells that he is not breathing, grabs the oxygen from me and hits the code blue. Kreed's room is instantly filled with all available staff on the floor and they have bagged Kreed and giving him full breaths. We stand in silence while the nurses and doctors continue to bag him, stop and wait for a breath and continue bagging when there are no breaths. Kreed was not conscious or breathing. The main icu Doctor turns to me and tells us that we need to leave the room because they have to intubate him. We turn in silence and go wait outside the unit, stunned and shocked and heart broken. Our son was not breathing. He wasn't there. Wasn't conscious. Our boy was "gone" for the moment.
That morning, Mother's Day, will forever be the day we last saw Kreed breathing on his own, moving, fully conscious and aware.
For the next 36 hours Kreed continued to take no breaths on his own and did not regain consciousness. Tests were run. Meetings were held. Doctors were talked too.
At the 24 hour mark Kreed had double pneumonia, three virus's and sepsis.
Finally he began to stir and become conscious after a day and a half, but he still did not breathe and he was in so much pain we had to sedate him with pain medication. Kreed was hurting and out of breath. Anytime he would come to, he would start to fight again, his eyes were unclear and there was nothing but pain and confusion.
Later that night he reacted during suctioning and pulled his tube out. He had to immediately be bagged and a Bipap with a back up rate was put on him- essentially the Bipap was breathing for him.
(Kreed's best guys- Ty and Bill comforting him through the pain- Bill flew in the middle of the night from Arizona to be by his side)
He slept mostly on the Bipap and only a few breaths a minute were his own. Not enough to sustain life. Kreed simply lost the ability to breathe on his own. We were able to get him to drink a few sips of water and a couple of chips on the third day, but by the fourth, his swallow reflex was gone and he could no longer swallow. One by one Kreed's reflexes were shutting down as his nerves shut down.
By the fourth day, his kidneys no longer worked or his bowels. So no breathing, no swallowing, no kidneys and no bowels. Every system in his body was shutting down one by one. And he could not breathe. We couldn't even draw blood from him because his blood was too thick to come out.
Suppose for a second that Kreed could have overcome his illnesses and body shutting down by some miracle...he would have been left with a trach for breathing, a tube for feedings (no more food by mouth since he couldn't swallow), wheelchair bound and an ostomy bag and most likely a kidney transplant due to lack of oxygen to his kidneys causing too much damage. He would have never been Kreed as we knew him, but a boy who was gone, sedated most of the time from pain and unable to do anything he used to enjoy. But he would have never lived through future procedures. He couldn't even live through the infections raging through his body, and every system in his body shutting down. His genetic defect was terminal. There was no treatment in the world that was going to save him. Our Kreed was gone. He was gone really on Mother's Day when he stopped breathing and lost consciousness for 36 hours.
Eventually at 12:30pm on May 12th, Kreed's body could no longer sustain life and he crashed again and we would not get him back. He could not breathe and his heart finally gave out at 3:29pm. Our brave boy fought his last battle and lived his life to the fullest.
Three months later we are still working on answers. He has a genetic defect in the MTHFD 1 gene. Super rare. He is 1 in 7 billion. The only person in the world with that particular mutation. Five other people have a mutation in that gene but not his particular one (8 over all, three died). His gene and skin cells are continuing to be studied to find out exactly how those defects have affected his cells and ultimately what happened to his body.
We do know he suffered nerve death all over his body and without nerves to send signals to various parts of the body, it shuts down. His nerves from brain to lungs were damaged which also affected his blood sugar and everything else. The strongest part of his body was his heart. It beat so strong and for so long even after it shouldn't have been beating any longer with all the damage his body had incurred. I'm not surprised because that's how Kreed lived his life- with his heart, his love, his joy.
While for so many his death was a surprise, it was to us only up until Mother's Day May 8th. It was in that moment that we knew Kreed was not going to survive what was wrong with his body. But nothing will ever prepare you for the day a child's heart stops beating.
It's taken three months to write this, process this and re-live those three months, and that last week. We had discussions about Kreed and him not making it to a full term of life at various times, but nothing prepares you for the reality. Watching your boy suffer for his entire life until the end.
But through it all, the pain, the suffering, the frustrations and complicated life he lived, he truly loved life. He truly lived life. We tried to give him as epic of a life we could. Nothing was out of our reach and we lived with no limits- never knowing there was a limit on his life. Even in the last two weeks of his life- he lived without limits and was so happy and full of life and sharing his light, joy and happiness with so many people- whose lives have now been forever changed, just by knowing our boy for a few months. The outpouring of love for our boy in the days, weeks and months following his death have been incredible. We never knew the impact sharing our story would have and the impact of this one boy who loved to live his life and teach others there were no limits to the things they could do and accomplish.
And now nothing compares to our utter sadness. Our soul death. Our emptiness. The joy being extinguished from our life and we are left to pick up the pieces from a shattered life and live a life we never wanted. We prepared to take care of him for the duration and were looking forward to giving him even more life experiences. All of that was ripped away from us without our consent and without knowing. We sit in an empty house devoid of his sounds and hopping and happiness. We drive in an empty car without his rocking to music. We live in an empty life missing our joy and happiness. We lived with a truly amazing boy and human being and now our life seems cold, removed, empty and joyless. He brought joy to everything and every second counted. Now time drags on with every second tick screaming in our heads as another second ticks by without him. A life without him. A life without joy.
The best we can hope for is that he continues to change lives. And that somehow, someway, we will find a meaning to life- although that seems impossible now because he was our meaning and our everything and life seems so pointless without him and his celebration of life.
So now you know. On the three month anniversary of his passing, these were the events that so many didn't know about because we never knew we would be telling the story of his end. We always thought we would solve his mystery and give him this amazing, epic life. Instead we are solving the mystery of his death and hoping somehow it helps others, even though as it stands now he is alone with his genetic mutation. Kreed was always unique, we just never knew how unique.
To you all, our final wish has always been to live in Kreed's way.
One life. No limits.
Monday, August 1, 2016
So many people say they understand or want to be there for you, but the truth is, unless you have actually lost a child- you don't understand. Until you've lost a very special needs child that needed you every second of every day, you really don't understand.
Other people or family members didn't live this life with Kreed. We did. Every second of every day. Other people get to grieve for Kreed and then it's over. Their lives aren't filled with emptiness and heartache. Our grief comes over and over again every day we wake up from sleep and feel the pain all over again. Our grief begins every morning and lasts all day until we fall into unconsciousness where then the grief haunts our dreams.
So many people then walk out of lives because they don't understand. The soul death we now face, missing our joy and happiness that was Kreed. In the months following a death eventually the texts and calls slow, the check in's go away and life returns to normal for everyone else. But not us. The pain is just as great, if not greater almost three months later.
We still wake up with a hole in our life. We drive in a car without Kreed, we live in a house without him and have to go about our life with out the single greatest joy of our life. We would gladly take back the sleepless nights, and spending every second with him. To feel him again, touch him, smell him. We live with that hole, no one else does. Not even close.
And we realize no one else realizes the impact his grief still has on us. And we find out who the real people in our lives are, even if we don't respond or can't speak, they keep trying to show us they are there.
You don't always have to say the right thing or know what to do. Because the reality is no one does. No one could possibly know this pain and know what to do. Our grief is long and deep and keeps us from functioning most days. Most days we are lucky we get up and breathe much less do anything else.
So in the end we won't be able to change our child being gone, but we will be able to change who we allow back in our lives. Because those people will eventually try to walk back in once they think the coast is clear, and we should be getting on with our lives and being back to normal, and guess what? The Coast will NEVER be clear. We will have moved on from those people who didn't need us then and who we certainly don't need now because news flash...there is never getting back to normal after a child has died or moving on, there is only learning how to live a new kind of normal and moving on in spite of the pain and grief. We will NEVER be the same. Some people can handle this fact, and some people choose not to. To those people I say goodbye, as you go live your normal life and move on and live your life, and it is too bad you could never understand why that wasn't possible for us. We've been through too much to care anymore, and we've changed too much to notice.
But to the ones that continue to check in, talk to us, send funny pictures to try and put a smile on our face or annoy us with texts or messages- thank you. Even if we don't respond, even if it seems like we ignore everything and everyone, it's the little things that count. Some days we just don't have the energy to say anything or do anything more than breathe or exist. Just know that every day we experience that grief and some days more acutely than others- if we find a piece of him or a memory suddenly comes upon us unexpectedly. Our life is lost right now, unsure of anything we know to do- because we lived for him and every second was dedicated to him happily. He was our joy, our happiness and now we must find a different sort of life. That takes effort and willingness that some days are just too tough to muster.
Our grief is every second of every day just as our life was for him. And people drop out of people who grieve lives- maybe because they don't understand the depth of pain or they don't know what to do with someone who only knows how to get up and breathe because the pain is so great. Their life goes on, ours do not. But some try no matter what and you figure out who will be there no matter what. Even if you can't understand another's pain or even come close to understanding the depth of it, it's possible to be continue to try and be present in their life.
Life is short. You don't know what will take you or when that time will be. And when kids are taken from you, you are set adrift without a life raft and most of the time you're drowning in that grief. You never know what will be your lifeline...it could just be someone that sends funny messages or tells you that they are there even if you say nothing. Or the friend that says "shit I can't imagine what your feeling." At least we are seen. The depth of our pain may never been seen or felt, but our grief can, we can.
We get up and exist every day and thank you to those who acknowledge the fact that we get up and exist and know that we are still here, even if all we can do is breathe.