Friday, May 31, 2013

Today I'm mad.

I've mentioned Wyatt's edema here occasionally and our struggle to find anything to slow it down from accumulating. He receives Lasix routinely multiple times a day, which certainly pulls off more fluid than if we wouldn't use any at all. But clearly, you can see by the pictures, our efforts are not entirely successful. Day to day I often don't notice to what degree he has changed. It's usually when I begin looking back at pictures from a week, a month, 6 months or a year before that I am stunned. The majority of Wyatt's edema accumulates from his thighs up, typically. His feet are a bit swollen, but the largest areas collecting fluid is his belly, his groin and thighs, his chest, hands, face and head. Even his ears are swollen.

Last year- Spring 2012.

In the picture above, from last year, we were already battling the shifting fluid. He was considered clearly "puffy" then. It's amazing how our perspective of "puffy" has so drastically changed in one year.

This year- Spring 2013. (Photo taken a few weeks ago)
I love him the same no matter how much fluid is trapped in his little body. But today I'm mad. It's not fair! To him, to me, to his family, to all the other children and families that have gone through this before us, who are experiencing this now or will at some time. It's just not fair. Mitochondrial disease has changed my son in so many ways, and now even his physical appearance.
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Wednesday, May 29, 2013

He is writing his own story.

"Help him live to the last breath." "Make every second count." "Give him all our love." These are simple sentiments I shared with the chaplain and our hospice nurse this morning as we discussed my feelings and our son's health. Dr. N, the hospice attending came to our home yesterday and in our discussion she prepared us for the moment that Wyatt has been dodging for the last year. Could this be "it"- the infection that won't respond to antibiotics, the pain that will cause suffering, the decline that he won't rebound from. We made a couple plans, tossed around multiple theories, talked about the boy who continuously confuses yet amazes us in the same day. Our hospice nurse, Gwen, and the chaplain, Tom, visited this morning to talk more, to try to prepare me more. It's difficult to prepare ourselves as we've settled into this as "normal" and have adapted to this pattern. We've become tolerant to a certain level of "sick." The truth is, there will always be doubt in the back of my mind as he's proven so many wrong in the past. Since the day he was born, he went against everything the medical books said would, could or may happen.

I love him so much for that. He is making his own way. He is writing his own story.

Though, I do hope I won't let myself avoid the truth because of that lingering doubt. I don't know how to give up. And I don't think I ever will. But I also, as his Mommy, can't possibly allow him to be in too much pain. I can't put into words what "too much pain" is, I just know it when I see it. We made the decision to add Methadone back into his complex cocktail of medicines this week, to help control the pain. Methadone works a little differently than other opioids, having the ability to reach different receptors and types of pain. We're hoping in combination with his Fentanyl PCA we can find him relief.

SuperWy, our super boy, is sick. His body is struggling. Is this "it"? I don't think anyone would dare say for sure anymore, but we can't deny that it very well could be. Today he is sleeping soundly and looks peaceful. Tomorrow we will redraw a new round of cultures (if nothing changes between now and then), to be sure we're not missing something, anything.

And we continue to look at life one day, one hour and one minute at a time, just as we have all along.

Family, friends, loved ones, anonymous followers and those that just drop in from time to time- thank you for all the thoughts, prayers, love and support. We appreciate it so much. If you have it in you, our super boy could use a few more right now.
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Tuesday, May 28, 2013

Anything is possible.

In the fight of his life Wyatt teaches us how to keep going, how to live. An unbelievable year it has been. Anything is possible.
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Saturday, May 25, 2013

A rough week.

Our super boy could use some extra super powers this week. The bacteria has been beating him up pretty badly. All week I've not-so-patiently been waiting for pending cultures to turn into final results. I've been very close to packing my sick boy up for a field trip to the lab to introduce them to "sick Wyatt", with the thought in mind that it could possibly speed up the process. Sick Wyatt is quite good at scaring people into action and reality. His body doesn't play around when it's declining.

So, my silence this week was related much to my need of infusing many doses of PRN meds as often as we could to keep him as comfortable as possible. While trying to suppress my frustration toward the lab that was seemingly moving at a snail's pace. Come the end of the week we did get the right antibiotics started. Now we wait for them to do their work...
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Thursday, May 16, 2013

A hard talk to have.

As I was fastening Wyatt's wheelchair down to the floor of our accessible mini-van, he misheard something that was said. He said "Please don't say that." I asked what he was talking about and he explained he thought he heard the word "dead". I didn't say the word dead, but asked why he didn't want us to say it. He explained further that he doesn't like to hear those words- dead, die, death- because he doesn't want to die. I then curiously asked him why. "I don't want to die because I'm afraid I'll miss you and you'll miss me too much."

"Oh." I thought to myself as I tried to find the right words to say out loud. These conversations are hard.

But I explained to him that I didn't think he would feel like he misses us when he's in Heaven. I hope that he'll be so happy, free of sadness and pain, no worries or fear, filled with love and a sense of peace.

I really, really, really, REALLY hope for these things.

I told him that I understood why he would feel the way that he does and a lot of people are scared to die. I elaborated by saying, I don't know that anybody ever wants to die, but it isn't always up to us. Our plans aren't always the best plans and we have to trust in God's love, his plans and that he knows what is best for us.

I reassured him that I would be so very, very sad when he dies and I will cry a lot. But I will be ok and I will love him forever. Daddy and the girls will be ok too. It will take time and our hearts will hurt a lot, I explained. I will miss him more than I've ever missed anyone and I will be waiting for the day I will see him again. But I will be ok.

He looked at me, needing a little more reassurance, and said "Really?' And I said "Yes. really."

What I won't say to him- I may eventually be ok, but I doubt if I'll ever be the same. My love for him is so great, so powerful.

We finished the conversation after asking if he had anymore questions or worries. I gave him a kiss on the forehead and said "Are you ready to go?" He replied "Yep." And we were on our way to the Adventure Aquarium for a field trip.

I thought of our conversation all day, all night and still this morning. We've had conversations about dying and heaven in the past, though he typically shuts us down pretty quickly. This time he didn't.

I can only hope he heard what he needed to hear.
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Tuesday, May 14, 2013

A different route.

Mania, anxiety, fear, agitation, fatigue, restlessness, pain....rinse, lather, repeat.

Wyatt has always gotten one med via his Jtube, Risperdal. There is no IV form. We give it late at night, only while he's sleeping, to avoid the increased pain and retching that giving meds enterally can cause for him. Risperdal has been to blame for causing the frequent and even constant tic'ing and myoclonic jerks (or questionable seizure activity). We tried weaning off this medicine, about a year ago, to see if the movements would slow or even stop, but as we were doing so he began to hallucinate.

I remember, we were awake most of the night with our boy who was shaking from head to toe, seizing, his temperature fluctuating, but not consistently high. I prepared myself for another infection. I emailed Dr. R that morning about our long questionable night. It was seconds later after I responded to his emailed reply that Wyatt began screaming, he was out of his mind terrified. He didn't recognize Bryan or I, we were robots coming to hurt him through his eyes. We couldn't move closer without him kicking, hitting and flailing his body around, screaming loudly and trying anything to get away from us. Pulling away abruptly causing his top heavy IV pole to tilt precariously, his lines stretched and I feared he would hurt himself. We hadn't seen that much movement in him in weeks up to that point. It's difficult to think about- he was so very scared of us that morning. As I continued to try to rationalize with him, talking him down. Bryan was able to get close enough to his IV pole to push a PRN dose of IV Valium through his med line. After that didn't work as well as we hoped, we were instructed to give him more. We eventually were able to get him to calm and sleep.

He was admitted to the hospital that day, Sunday. The next morning we stood on either side of Wyatt's hospital bed, Dr. R was rounding with his group of residents, he was just called in to our room following a smaller hallucination event turned seizure. I believe Wyatt was only half coherent, but aware we were close and cautiously staring us down, weary. Dr. R asked us what was worse- the shaking and tics or the hallucinations. Bryan and I looked at each other and quickly responded "the hallucinations." He agreed and we decided to give him an injection of Zyprexa, a med similar to Risperdal but only lasts for 24hrs. At the time the hospital pharmacy didn't have the Risperdal injection available.

I don't think I've ever shared these details here before. At the time I was unable to form the thoughts and words to do so, there was so much going on and our hearts were hurting deeply.

We discussed the intramuscular version of Risperdal multiple times since then, but have been hesitant to start it. Once it's injected it is in his system for 2 weeks. And we weren't sure how his body would react. It's also painful, which is not a plus. In a sense, Wyatt has his very own time release system within his intestinal tract. We don't know how much or if he will absorb any of the medicine on any given day. His body's ability to absorb is so unreliable due to the severity of intestinal failure. We were ok and making do with the unreliability up until this spring. I think some days he must have been absorbing some of the Risperdal, but now Wyatt's intestinal tract has seemingly gone on complete strike. We suspect he's not absorbing the Risperdal at all, or not enough to see benefit, which makes things very unpredictable around here of late. (Thankfully, he's not hallucinating or scared because of hallucinations!)

We lovingly refer to Wy as "the king of sleep" because, well, he sleeps a lot typically. But now the king is unable to sleep for days. This has been going on for a few weeks and has been gradually escalating to the point we're at now.

I realized last week he's acting a lot like my iPhone. Bryan laughed when I casually mentioned this to him, but he couldn't deny the similarities when I explained it further- the battery life of my phone is quite long and I keep using it and using it until I'm in the middle of a text and it hits 0%. I then have to plug it in and it begins to recharge from the wall or my computer, but it still doesn't work. I still can't turn it on. I must wait while it recharges for a bit before my phone will turn on and begin to resume functioning, while recharging its battery by an external source. Wyatt can be in the middle of a sentence and fall asleep. His body just stops- he hits 0%. His temp drops quickly to a low degree, he becomes whiter than white, his oxygen saturation drops, his machines start alarming, etc. and I then scramble to figure out what his body needs until its "recharged" enough to pick up a little on it's own. I've figured out through trial and error these last couple weeks that eventually his body can find a balance once it builds up enough "charge". But it's stressed.

Sleep is a good thing and his body needs it. It's tough for me, as his Mommy, when he doesn't wake for long stretches or days on end, but the opposite of that isn't a good thing either. I feel if we can get him back into an actual sleep cycle again the other issues- fear, anxiety, agitation and possibly even some of his pain, may fall back in line. That's my hope, anyway. So, with that being said, we made the decision to use the intramuscular Risperdal injection.

We won't know how it'll work for him until we try, right? That's what I think too.

So, here's hoping it works well by calming his mind and body, providing him restful sleep and, in turn, better...or should I say, more balanced days.

I don't think I've shared this video clip with you all- it shows a small portion of the shaking I've tried to explain multiple times. I constructed this video last spring to show his doctors what we were witnessing and to what degree. It's more than just a shaking or minor twitching limb, but it's difficult to put into words how so. I'm sharing this because, if anything, I want this space to be an open view of our life and our sons rivalry with Mito.

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Saturday, May 11, 2013

I like grey.

I've been reminiscing recently- it's been nearly a year since Wyatt was in room 8 on 3F, our second home, at A.I. duPont Hospital for Children. That day in June he, and all his equipment, was carefully and strategically moved onto a transport bed. I became nervous as they discussed what would happen if he began to die en-route to our home. They loaded him into the ambulance, I climbed into the front passenger seat and held back tears as we drove away from the hospital that we've come to feel so comfortable in through the years. We arrived home from the hospital a little more than an hour later, for what we believed would be the last time. Can you believe it? Almost a year.

Clearly this year has yet to play out like we thought- which is difficult to wrap our brains around some days. I remember last July emailing Dr. R feeling confused as to why this process wasn't happening like we all thought, feeling awful for questioning it, but wanting to know if he saw it too before getting my hopes up too high. It's now May 2013, which means July is not that far away. July 23rd will be our boy's 11th birthday. We are closing in on his 11th birthday when we didn't expect him to make it to his 10th! A miracle. A blessing. It could be considered both of those things. I do know it's unexpected, unimaginable, unexplainable and so amazing all tucked into one really cool kid. He knows he's proving us all wrong! He's proof of his own words- "anything is possible!"

I'm unable to put into words the actual feelings we've experienced within this year- it's been so different than any other. The highest of highs to the lowest of lows. But I can say it's been the richest year for our family. The actual hospital life that encompassed our daily lives was put aside. We left the hospital in June not expecting to return with our son. We have returned for visits, but not to stay. Though we dearly miss those that we came to know and looked forward to seeing, we have come to accept that they can no longer help our son with medical interventions. We're doing everything here, at home, that they would be doing there. And we have such respect for the honesty and love that was shown to us. It's not easy as parents to hear the news that your child is dying, but know it must also be very hard to come to the decision and deliver that news too. I trust his team more now than I've let myself trust any other doctors. And that's been life changing for me. I'm not sure he realizes it (or maybe he does), Dr. R has changed our family's life. He's changed my life, as Wyatt's Mother. And hospice has given us the ability to be together, to do "this" as we carry on as a family. Are they all perfect? No. Do I get frustrated with each of them at times? Oh, yes. And I'm sure the feeling is mutual occasionally. But I don't believe there is any better team for our son.

This year Wyatt's care was centered around comfort and quality of life. The worry of what was to come faded away, as we now know. This year he has been living on the brink of death- the questions don't linger overhead anymore. My focus began to shift to making memories, to fully embracing a moment, and not repeatedly swallowed up by medical research, advances and terminology. Wyatt's body is much like a puzzle, one that is missing a few key unsolvable puzzle. In 10 years he has made me second guess everything I've ever learned, everything I ever thought I knew. I emailed a similar sentiment to Dr. R and I received a simple reply back: "You and me both." So, at least I know I'm not alone.

Living in the moment has been taken to a new level- there has been more than a few times that I've said out loud "His heart is beating, he looks comfortable- we're doing good!" The thought, 'it is what it is' sums up a lot.

Oh goodness, and let me tell you, this year Bryan and I have become uninhibited, having no shame, completely silly and ridiculous even, just to get our son to relax, smile and laugh. There have been times we succeed so well in our antics that he laughs so much his oxygen levels drop and his alarms start blaring. And then Bryan begins his "victory dance", which makes Wyatt laugh a little more (You should ask Bryan to show you sometime.)

Quietly singing a fake song, in our own phony "french" words, in a key that one of us should not be singing, in the middle of the Ophthalmology clinic of the children's hospital is not beneath us desperate parents hoping to lighten our son's mood. It did happen- I can't deny it! (The door to our exam room was closed, at least!) Being in the moment and not caring what the people on the other side of the door, the wall or building was thinking when hearing us sing our fake song in our made up language- those are the moments I actually love. Happy to be able to sit in that exam room with our boy, with the feeling of being carefree when our life is anything but, just to see his shoulders relax and hear him try to hold back a laugh. We now just might be as crazy as we seem!

In some ways knowing and accepting what his future holds and what our family is facing has been freeing. It's difficult not to feel the need to cram in a lifetime of experiences in an unpredictable amount of time, but really just being together feels perfect, complete, right where we are all supposed to be. Some of the most simple moments of hanging out in the living room watching a TV show together can be the most comforting. We have found a "normal" and even happiness (at times) in spite of the difficult circumstances. We've learned a different way of life this year. And though it may not be desirable, it's one we can call our own. And it's been so special in so many ways.

 I've learned very little is black and white- thankfully, I like the color grey.
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Tuesday, May 7, 2013

What's what.

Through the years I've found some of the most helpful suggestions come from families like ours, who are living or have lived similar lives and share what worked best or even worked the least for them and/or their child. I'd like to say thank you to those of you who have offered suggestions or advice for us and Wyatt's care. I hope you know that we greatly appreciate every comment or email with offers of prayer, words of encouragement and the great amount of support that's been been extended to us along the way. We love hearing from all of you who read here! Please continue to comment or email us if you have something to say. These days we rely heavily on prayer (and a lot of caffeine!) to get us through.

I've received several emails or messages offering suggestions of draining Wyatt's G-tube continuously or to try Farrell bags and such to help with his belly pain. So, I felt it was probably appropriate to dedicate a post to explaining what Wyatt has attached to him at all times and why. Including some of the equipment that we use and to share what we found works well for us. I realize I don't always mention certain aspects of Wyatt's care because it's so routine and "normal" for us, it gets forgotten. So, here it goes:

Wyatt uses a Trilogy ventilator for respiratory support. Due to respiratory weakness, respiratory failure and/or respiratory depression (you may pick a term to use), he relies on the Trilogy to do the work of breathing for him and receives 25 breaths per minute. Wyatt has used multiple types or settings of non-invasive ventilation since he was about 5 years old due to apnea and hypoventilation. Despite relying fully on ventilation he still has a very significant amount of atelectasis, or non inflated/collapsed lung tissue. But we do the best we can.

We use a few different types of masks that we switch between to avoid breakdown and relieve pressure in different areas on his face. Some masks are more comfortable and some are just more reliable. We use nasal pillows while he is awake and a nasal mask while sleeping. Right now we really like the Swift FX nasal pillows for when he's awake. With this type of mask he can wear his glasses, which is a big deal! I like using this type of mask on him because I can see more of his face, which always brightens my day. We use the Mirage micro for nighttime or while he's sleeping. It's a reliable, easy mask and of late we get less leak with it than we do with the nasal pillows. Though, today I just ordered a new one- the ComfortGel blue. With the huge amount of swelling around Wyatt's face we've been struggling to get a good fit with the Mirage micro without leaving huge marks and indentations. We've had to tighten the mask against the edema so much his face is becoming flattened and it's pushing his jaw back, which concerns me. So, I'm on a mission to find a better fitting and reliable sleep/nighttime mask. We've used the Mirage micro for years now, so it's about time to switch it up. We also have a full face mask, the Mirage quattro, that he uses in times of great distress.

We bleed supplemental O2 into the Trilogy at all times. At this time he requires anywhere between 3-6 liters of oxygen continuously along with the support of the Trilogy. When at home we use a high flow concentrator that can deliver up to 10L of O2. And Wyatt has tested the limits of this on occasion- indeed, it does work! When we leave the house we use liquid oxygen in portable Marathons. Liquid oxygen lasts longer on a higher continuous liter flow than the E tanks that we were using- we love that! We have a large canister in our home that holds the liquid oxygen that we use to fill the marathons or we can use it as a backup oxygen supply should we need it. It's not pretty, but a necessity. We do keep a small back up supply of E size oxygen tanks in our home, as well, just in case we need them.

The pulse-ox sensor is the little red light that's attached to one of his toes. The pulse-oximeter on the other end of that sensor tells us his pulse (heart rate) and the amount of oxygen in his blood. He's attached to this at all times to monitor both of these things. His heart rate and oxygenation can tell us a lot.

Suction machine and oral care-
Wyatt requires suction when he's unable to clear his airway of secretions, mucus or, at times, blood. The frequency he requires suction varies greatly depending on his level of alertness and health. We can also brush his teeth by using suction toothbrushes on the end of the tubing. So nice! We have a portable suction machine for in our home and when we leave the house. I also use a lot of toothettes to swab his mouth, as well as lemon glycerine swabs and aquaphor for his lips.

We utilize nebulizer treatments to open up his lungs/airway as much as possible. We use them as we need to. Today he's wheezy, so he's already received albuterol and saline nebs. We have a tee adapter on his vent tubing that we connect the nebulizer cup to and the vapor/medicine is then pushed into him with every breath the Trilogy gives. And if Mommy is standing nearby and gets a breath of Albuterol she will be buzzing around the house for the next few hours- that stuff is potent!

The vest system-
The vest system we don't always use routinely, but have relied on it heavily when in times of great decline to his respiratory system. We call it his "shaky vest," as it shakes his body so rapidly, giving pulses to his torso, to help break up mucus and clear his lungs and airway.

GI system and IV-
Wyatt's G-tube and J-tube are both set to drain continuously- we use leg bags for this. We drain secretions, bile and decompress his intestinal tract to relieve pain and pressure by draining these tubes continuously.

Due to the severity of intestinal failure Wyatt is unable to receive any nutrition or hydration via his GI tract. He receives all of his nutrition, hydration and medicines through a double lumen broviac, or known as a central line. This is an IV that is threaded through a large vein in his chest. We've had the most success using steri strips to secure and IV3000 as a dressing over his central lines- this combo has been the most gentle to his skin.

A double lumen central line means it is split into two separate IV access points that come out of his chest. For Wyatt, we then need to divide each lumen into more IV access with trifurcated (tri-fuse) extensions on the end of each of those lumens to accommodate all of his nutritional and medicinal needs. He has three IV pumps, two Curlins and a CADD pump, that are running continuous infusions, 24hrs a day everyday- TPN, Fentanyl and saline. We also have a med line that is accessed continuously and we use a syringe pump to infuse his medications each day. One day a week we add a fifth IV pump to infuse Omegaven, a fatty acid supplement that is used in place of the lipids (fat) in his diet. Due to the severity of his liver disease he was granted approval for compassionate use of Omegaven which is still in the trial phases for FDA approval. The Omegaven is also a continuous infusion. Up until recently he received it more frequently than once a week, but at this time his liver is unable to process any more than a very small continuous infusion one day a week.

We wrap all 5 IV lines in a 42" line tamer to keep them together and more secure. These have been very helpful for us- I highly recommend them if you have multiple IV lines hanging around! We use a regular backpack that we adapted to hold his TPN, saline and both IV pumps. Wyatt's particular backpack is from Hanna Andersson- it's held up remarkably well for holding a lot of weight everyday for the last couple years and enduring its fair share of abuse from us! When we infuse Omegaven, the IV pump for it also goes in the backpack, while the glass bottle that it comes packaged in hangs in a hanger we constructed ourselves on the top of the IV pole or on a carabiner clip inside the backpack when we are on the go. His Fentanyl bag and its pump gets gently shoved into the same backpack when we leave the house, but at home it is in its own bag so that it's easily accessible for us and the hospice nurses. At home we hang both the backpack and Fentanyl bag on the top of the IV pole, along with the Omegaven. The syringe pump is attached on the pole by a clamp, as well.

Wyatt has a supra pubic catheter that drains his bladder continuously. This is an opening, a stoma, below his belly button that a Foley catheter passes through and into his bladder. We secure the foley to his abdomen with duoderm and tegaderm to prevent pulling and irritating the stoma. Wyatt is very sensitive to any movement of the catheter, so securing it well is necessary! The foley catheter is attached to a foley bag that collects the urine. We change the foley catheter at home every few weeks and prior to completing a course of antibiotics for infection.

Well, I think that covers everything that's attached to him or that we use frequently. I hope this helps to explain a part of what we use, what you may see in our pictures or anything you may have been curious about. Believe it or not, there is actually more that I'd like to share someday- maybe I'll do a 'part 2' of this post- but for now I must stop. Wishing you all a good day!
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