In Lieu of Flowers

Yesterday I contacted Reece's Rainbows to let them know that we chose their charity to honor the memory of our beloved Jameson. I just want to share with you all that all donations given in honor of our Jameson with go into the adoption fund of their little Jamison. He is also 3 yrs old and I believe he is in a Russian orphanage, waiting for someone to want him. He is waiting for a family to love him and claim him and bring him home. I feel so much joy in my heart as I see the way God is using our pain in such a beautiful way to help others. This little Jamison deserves to experience the same love and life that our Jameson knew and I love that we have the opportunity to help in that process.
http://reecesrainbow.org/jamison-3g

Little Man asked if we could bring him home since J is in heaven. He is having such a difficult time understanding what is happening and why he feels the way he does. He was extremely concerned that Jameson has no toys in heaven. We've started a little "game" with him whenever he gets really worked up about Jameson. We think of what J is doing in heaven right now and present Little Man with wonderful pictures of his brother playing on the beach, making sand castles with Jesus or eating chocolate milkshakes with Great Grandmas and Grandpas. It is actually nice for me too. Everyone keeps telling me that this is the hardest time and it will get easier. I hope it gets easier for Little Man too.

Home

Jameson went home this morning. He is now in heaven; a prince living with the Prince of Peace. We rejoice in knowing that his suffering is over.

We want to thank all of you from the bottom of our hearts for your prayers and support over the past 4 months. As soon as we make arrangements, I will post information. Today we are with our families remembering and celebrating Jameson's life. God bless you all as He has blessed us. And Jameson, welcome home.

Jameson is still hanging in there. He has horrifying amounts of edema all over his body but we are able to keep him comfortable with the pain medications. He is still breathing well and able to crack his eyes open and squeeze our fingers once in a while. Yesterday Little Man opened most of his presents at the hospital and we both came home with him for a bit last night to play and tuck him in. I am still at home and we skyped with Daddy when Little Man opened more presents and his stocking from Santa this morning.

I am trying to focus on the true meaning of Christmas more than ever this year. We celebrate this day because our Savior was born on this day. Because He came and lived on this earth and died for us, we have redemption. God is the only one who can redeem us, redeem this nightmare we are living, redeem our broken hearts. And so today, I will do my best to overlook the pain and sadness that accompanies this Christmas and focus instead on the beauty and love of Jesus' birth. Merry Christmas; may you find peace and love in your hearts on this special birthday. Love, Meghan

Jameson continues to amaze everyone with his strength and determination to do this his way. He always has been an independent child and that hasn't changed one bit. He is quite sedate, but today he has been squeezing our fingers and trying to open his eyes to check us out when we talk to him and sing to him. Hubs and I spent hours last night cuddling with him and remembering all of our amazing and wonderful adventures over the past three years. Jameson has had such a full and wonderful life; while we will always regret J not having a future here, we have no regrets for the past three years. Jameson fully knows love and happiness and fun and adventure. He has camped, swam in oceans and lakes and rivers and fished and canoed and enjoyed smores and dinner parties and campfires and rainbows and friends and fireworks and walks and waterfalls and cookies and State Fair milkshakes and Disney and Gator football. He's been to Alaska and seen whales and bears and moose. He has a passport and has been to Canada and gone boating in some of the more beautiful places on this earth. He has a best friend in his brother and life has been one big sleep over party for the two of them. He's enjoyed cheesecake and souffle and eggs florentine and cheese curds and birthday cake. He's had too many dance parties to count and spread more sunshine and smiles than I ever thought was humanly possible. Last year he even had a modeling shoot with Target. Today a wonderful person at Target emailed me the photos from his shoot. The picture above is my favorite. He is a natural in front of the camera.
Here are a few things from this week that have made us smile. We let him pull out is own NJ tube. He has been trying to rip it out since it went in. We also gave him some chocolate ice cream. He liked licking his lips but wasn't able to swallow it. And every day this week he has gotten to snuggle with his brother and watch movies together.

Yesterday morning our pain specialists did indeed have some new ideas and Jameson has been much more comfortable. We added versed back and went up on the pentabarb and the fentanyl. While this could never be easy, we are glad to have him pain free. He is -in true Jameson fashion- doing this his own way. Hubs has been reading his favorite books to J and we have been holding him and snuggling him all of the time. Again, thank you for your continued prayers and support. God has blessed us to have such wonderful friends and family.

We've had a lot of family in over the past two days and it has been good and sad and helpful and sad. Little Man has spent a lot of time here and he loves being with his brother. Jameson and Little Man are best friends and it just shatters my heart when I think of what we are going to have to tell him. I hope he is able to heal and have happiness easier than we will. I feel like for me the fog started to roll in yesterday and I am just so very sad. So very sad. I have this thing in my gut and sometimes it moves into my heart. It feels like a black hole and at any given moment I feel like I'm either going to vomit or implode. And it is so hard to breathe. I look at J and I just don't how we will make it without him here. He has been nothing but joy in our lives and I don't see how the world will ever have the same appeal ever again.

Last night and today have been painful for Jameson. We've had to up his pain meds and give him a lot of bumps. It is so hard to see that he is suffering. Even more then I want him to live, I want him to not suffer. And every time he grinds his teeth I feel my heart breaking and I'm always surprised because it already feels like it has been crushed in a grindstone. How are there even pieces left big enough to break any more? Tomorrow our pain team may have some new ideas to keep him more comfortable. I just don't want my baby to suffer any more.

We have had some good moments over the past two days. Hubs held him yesterday, I slept with him last night and probably will again tonight. Auntie K and Little Man both had turns snuggling with him in bed today too. And Little Man got to take his brother for a wagon ride. It was beautiful and heart wrenching. J is still sleeping in the wagon right now. He loves it! Geez, I wish we would have though of this sooner. We are savoring every moment we have left.

I think this might be the second most difficult entry to write, the most not being here yet. Jameson's doctors met with us today and we talked about his declining condition and his failing liver and kidneys and there is nothing left they can do. My baby can't be fixed. He is broken and only God can fix him and it looks like His plan is to fix him in perfection where I can't go yet.
We aren't sure how much time we have left; he may be in the beginning of multi-organ failure or it may be an infection that is causing his liver and kidney distress, but either way, it isn't good for Jameson.

We are still praying that God will heal him miraculously on this earth and we are still treating him and hoping he is able to muster the strength to come back to us. My God is good all of the time and I am so thankful and honored that he chose us to have Jameson. I don't blame Him for wanting him back so soon; my son is the best gift ever given. Thank you for your prayers and support; we need it now more than ever.

Jameson's effusion did not grow so he will not being having a chest tube placed today. The intensivist checked it out on ultrasound and the fluid he did find appears to be loculated, meaning there are little pockets of fluid, not just one big one. That makes it much harder to drain. Since Jameson is ventilating well, it isn't worth the risk to go after the fluid today.
The docs are going down on one of his IV diuretics today. He is peeing great, but his body isn't doing it the right way. His kidney function is decreased and he is intravascularly depleted. Basically, the diuretic is pulling the fluid from his veins and he is peeing it out and his body is supposed to be then pulling fluid into his veins from all of the extra places he has it to replenish the veins but it isn't doing that. The edema is still there and he needs blood to replenish his veins. He has gotten platelets, chryo, and packed red cells this morning. His body isn't cooperating and it is hurting his kidneys to dry them out this much. Hopefully his kidney function will improve now they we've backed off on the diuretic a bit.
His coags were all off this morning and the intensivist said it can be caused by a few different things. Infections can cause coags to go crazy and so can liver disfunction. I'm really hoping it is just infection. That liver scares me and so far it has been big but still functioning fine; that has made the docs scratch their heads, but no one has been really scared about it it yet. However, if his liver function is truly diminished, that is another story. The liver isn't something we can mimic with technology and it would be very scary and devastating if his disease is causing his liver to fail. We are all hoping and praying that is not the cause. Again we wait and pray and hope.

Jameson has been running a fever all day today. It got up to 39.6 and has been above 39 all afternoon even with tylenol and ice packs. His heart rate has been high all afternoon despite bumps and his CRP is up again today. The docs have switched around his antibiotics a bit. The vancomiacin is on for 9 more days and they've added mirapenum as well. They also added an antifungal but I forgot the name. Sorry.
He also lost his NJ tube (clogged up) and is in the process of getting a new one. Right before the new one went in, we were suctioning and he vomited. A lot of what came up was mucous and secretions and he may have aspirated some of it but Dad kept his cool and kept right on suctioning through it all. He seems to be doing better now; he may have gagged when coughing, not sure.
He has been fairly agitated all day long. The upside is that J is no longer alarmingly lethargic. Everyone is pleased to see him awake and alert; he no longer seems to be "out there" all of the time. I just hate that agitation and pain seem to be what brings him back to reality.
We anticipate the surgeons putting in a left chest tube tomorrow but we won't know until we see his morning chest xray. They may try to do it bedside, which is preferable. Hopefully we won't have to take a field trip to the OR.
Hubs was so relieved to come back and see Jameson and not have this big test looming over him. He passed the test, praise Jesus! I just sent him home to eat, relax, and sleep.

Take Two. The effusion is up on the left side so a different intensivist is going to try to put a new chest tube in in a few minutes. Please pray it works so we don't have to head to the OR today. I will update when I get news. Thank you so much for all of the prayers this morning!

Update:
 He couldn't get in it. Sigh. But the ultrasound didn't show a large pocket of fluid anywhere and he is still saturating well so the plan is to wait and see how it looks tomorrow and if it is bigger, Jameson will most likely head to the OR. He has been poked so many times and has had three attempts to get chest tubes in in just the last 24 hours. I'm so glad we have great pain meds.

The intensivist was able to rewire the subclavian and put in a new right femoral line to replace the left one. He couldn't get a chest tube in the left side and when he ultrasounded it, he didn't see a lot of fluid so we are going to monitor carefully for the next few days and if he needs another left chest tube, he will try again or let surgery try in the OR.

Sigh. Jameson's left chest tube fell out sometime in the last day and he is in the process of getting a new one put in. This will certainly set him back a bit; chest tubes are excruciating.
He is also having his subclavian central line rewired and a new femoral line placed. If he can't get it in, the intensivist will rewire the existing femoral central line. The infection is culturing from both lines and not clearing even with high doses of vancomiacin, so the lines have to go. Replacing the subclavian is too risky with his edema so hopefully rewiring it will do the trick.
He slept most of the night but was agitated while stooling this morning. He had another big one today with more tissue. His temp also spiked up again this morning to 39 and he still is having green goobers from his trach when suctioning.
Little Man is home with a cold and Hub's final exam starts at 8 am tomorrow morning. I'm doing fine, just tired and wondering when we get to catch a break. When you carry a burden this heavy, it still weighs you down, even when someone else is carrying you. I know God is carrying us through these times and we will survive, but will my shoulders ever feel light again? Ever?

Jameson is still sleeping all of the time. The intensivist is a little nervous about it as he is so sick and has that infection. The nurses are taking cultures from both central lines daily to check on the staph. (He has a triple lumen in his left femoral and a double lumen in the right subclavian.) If this infection doesn't clear up soon, they may have to give him all new central lines. He's had a few since we've been here so it won't be an easy task; his edema makes it even more difficult and riskier. Hopefully we won't have to cross that bridge.
Jameson is still way up on fluid, but thanks to stooling over 500ml today he is negative on the day. He passed a good amount of tissue in his stool. Everyone is always so disappointed whenever there is tissue as we all keeping hoping the worst is behind us. The only good thing I can report is that there was not noticable pain this time. No rise in heart rate, writhing, cramping, rumbling, nothing. In fact, he just slept through it. Because he sleeps all of the time. We have gone down on all of his pain and sedation drugs and it doesn't seem to have made much of a difference. The fentanyl will be gone tomorrow, the pentobarb will be done on the 28th. The precedex is getting weaned and is scheduled to be off on Christmas day. I don't think we will be off the ketamine(which will be weaned the slowest of all) for a long time. But at least we are able to start the weaning process. I just wish is meant he would be more wakeful, not more sedate. Wish we knew why.
We have maxed out the bumex to get him peeing and it wasn't enough so dopamine was added to raise his pressures up enough to make him pee. But it wasn't enough so a second iv diuretic was added. And now he is peeing. Fluid is much harder to take off than put on...just like those christmas cookies are for me. :) I digress, but I think the Ronald McDonald House's goal is to make all of us moms fat and happy. We've had 2 fluid negative days in a row and his weight was down to 17.9 kg this morning from 18.5 yesterday. Now to get back to that 15kg weight... He still is super puffy and his lungs are still having a hard time working as well as they were before; he really needs to dry out!
In liver news, his ferritin level just came back today at 9899. If you remember last Thursday it was 2850. Hmmmm. No idea. Haven't had a chance to talk with the docs about it yet.

Still huge belly, still not peeing well, still very, very sleepy. Docs went down on the precedex again tonight to wake him up a bit more, added dopamine to raise his blood pressure to hopefully help him pee, and we are venting his NJ at awake/moving times to hopefully help gas escape. His girth was up to 70 this morning. I gasped when I heard that. When we were first in St. Paul, I remember getting close to 60cm and thinking holy huge belly! and now here we are at 70cm. I think that is almost my waist. And I'm like 3 feet taller than him. We will probably be getting an ultrasound to check out the contents of the abdomen; if there is ascitis in there, a belly drain would be a quick and slightly easy way to relieve pressure and take off fluid. Every time they've looked in the past two weeks there hasn't been much ascitis- which is great- but that means the rising belly is a little more complicated and not as easy to shrink. I think Jameson gives his doctors headaches. His parents too. :)
I do have some good news to share! The staph infection is not a bad one!! Thank you, Lord, for answering this prayer! The vancomiacin should knock it out soon and hopefully he will recover quickly. Amen!
I am back at the hospital and plan to stay here until Friday so Hubs can have more time to study and concentrate for his test. His big final is Thursday(16th) and he needs all the prayers he can get. Thank you for praying for all of my boys. I feel somewhat rejuvenated after spending a few days at home. Playing in the snow, snuggling with my Little Man, Mom and the dogs on my own couch, and sleeping 12 hours a night(seriously) fed my body and soul. Your love and prayers have helped as well. We are always so humbled and amazed by the love and support you all give to us. From stangers praying for our family, to neighbors we barely know but already love snowblowing out our driveway 3 times in one morning thanks to the plows, and everything in between, we are so utterly thankful. God has surely blessed us; through you, we see Him as we walk through this valley. Thank you and may God bless you as you continue to bless us.

Jameson is not having a great weekend. I'm at home with Little Man and getting short reports from Hubs, so I can't share in great detail, but from what Hubs has said, he is +300 fluid positive today and he keeps desatting even though he is now up to 55% fiO2. He has been at 35% fiO2 for weeks and saturating around 99%. And now 55% fiO2 isn't good enough to keep his saturations above 88%. Gah! Part of that is becase he has so much extra fluid in his body, but certainly part of it is because he is sick.
His blood culture from yesterday grew staph. We won't know what kind of staph it is for a day or so. Some staph is very resistant to antibiotics and that would be really, really bad. He just started vancomiacin again today to hopefully treat the staph. God, please let it work.
He also isn't peeing well. Part of it may be pressure from the extra fluid. He has never peed well when he has a lot of pressure from a full belly. But it could also be because he is sleeping all.of.the.time. Part of that is probably because he is sick and needs his rest. But we need him to pee so the docs are planning to go down a bit on his precedex. He is very sensitive to precedex changes...he is so sensitive to all sedation/pain changes, God help him.
He was doing so well and now things are kinda scary. Please pray that he heals and pees and breathes well and his liver shrinks and his radiation enteritis goes away and his bowels heal and that radiation worked.......... He is still in a critically somewhat stable place, but he can crash so fast. It just haunts me to think how fast we almost lost him in September the day before his birthday. He was doing okay and 4 hours later he was on ECMO and the doc was telling us he just barely made it.
Thank you for your prayers. I know God is in control and I know he listens when we pray! Lets make sure he hears us with J's health needs tonight more than ever!!

Yesterday Jameson slept away the day. Everything was great except that he was fluid positive. Hubs and I have been very concerned about keeping him even or negative every day; the fluid has to go somewhere and his abdomen is already really full of bowels and liver, so that leans towards fluid going into the lungs. His breathing volumes are down already today so SuperDoc is ordering a chest xray to see if that shows anything. We also realized this morning that he wasn't getting the correct amount of bumex over the past few days and that is one reason he has been fluid positive. We are so frustrated that our child has gained over 5 extra pounds of fluid on his tiny body in the past two days because the pump wasn't running at the correct rate. SuperDoc upped the bumex and hopefully it will come off soon. Fixable, but also totally avoidable. Grrrr.
On the liver front I need to pretty much retract my entire last post except for the edited part that it could be something different. SuperDoc, hematology, and a liver specialist from a different hospital have all looked at his info and tests and none of them think hemochromatosis is a slam dunk at this point. They aren't sure what is going on, but since his liver function is fine and some of the other more worrisome labs are coming back fine, they all feel that we have the time to wait and watch for a while. But a liver biopsy is still in the back of their minds. So we wait.
A trach culture taken yesterday came back positive for gram positive cocci. He is on antibiotics and hopefully they will work quickly.
Today is day 100. Why is it that the round numbers always seem so momentous and heavy? We are so tired and weary from worry and stress and med school finals and this roller coaster of good days and bad days and not sleeping and it goes on and on and on. In the hospital, it is hard to keep track of time; hours and days are lost and jumbled together and sometimes it feels like this has all been just one really long, bad day. Other times it feels like it has been years and I find myself straining to remember what Jameson's voice sounds like, what it feels like to sleep in my own bed with my husband next to me, what laughter is. And I miss being barefoot and having a private shower and not feeling self-conscious if I wear my pajamas all day long. And I miss holding my baby and living out the simple happily-ever-after fairy tale life we had been living. I long for the way it used to be. 100 days is just a really long time to have everything backwards and up-side-down.

Last night sucked. He was up writhing until well after midnight and it wasn't until we gave 2 pentabarb boluses that he was finally able to calm down. Then he started right back up with the cramping and pain at 6 am this morning. Finally around 9 am, he passed a giant hunk of tissue in his stool and the cramping and rumbling finally went away. He has been sleeping since 9:30 or so now. I am so glad that he is able to rest!
Yesterday SuperDoc ran a serum ferritin test and he came back off the charts. His level is 2,850 and normal range is 7 to 142. Yikes! This could mean he has hemachromatosis, which is basically iron overload. He may have this due to the number of blood transfusions he recieves. A side effect of hemachromatosis is enlarged liver. Cha-ching! Abdominal pain is another. Cha-ching! There are medications they can give J to chelate his blood so he poops the excess iron out. And then, hopefully my baby will have a normal liver again and his stomach cramps will be less intense or totally go away! Based on the tissue sloughing, I think he is still in the throes of radiation enteritis, but hopefully this will resolve some of the pain and cramping. I have no idea idea how long it takes before his iron is at normal levels and his liver starts shrinking again. Wikipedia didn't help much with that and the docs haven't been by since we got the lab results. Not like it matters- J never plays by the rules anyway. :)
Thanks for all of your liver prayers and keep them coming. There can be some serious side effects to hemachromatosis and I sure hope he doesn't have any lasting bad ones! And just for the record, I'm done with "tosis" illnesses, thank you very much!
EDITED: Um, just talked with the doctor and it could still be something other than hemochromatosis...I'll update when we figure it out for sure. Sorry for jumping the gun!!

We started out alright this morning but the day got rockier as it went on. And I HOPE we are finished with everything but sleep for the rest of it now. J slept, sat in his red chair, and sat on the edge of the bed again this morning and did well. But.
His heartrates and pressures have been higher today, his saturations have been lower, his lung haven't sounded as great, and he is having some green/yellow/blood-tinged secretions out of his trach. Yesterday we sent cultures and so far nothing has grown. It could be a virus or something else that we will never have the answer to. My child is an enigma more often than not.
This afternoon and tonight he is finally stooling and we are finally having a fluid negative day. If only it didn't also mean that he is having cramping and stomach pain. This evening it was so bad that he was actually crying real tears as he writhed around and his stomach rolled. We gave him a ketamine bolus and it lasted about 30 minutes. It should last waaayyy longer than that. His heartrates were still up in the 190's and he was still writhing in pain and it still felt like an alien was going to pop out of his stomach and then he pooped again. It gave him a minute of relief and he started right back up with the rolling belly, pain in the eyes, high heart rates. We have him chlorol and he also had benadryl (to pre-medicate for platelets) and a fentanyl bump. 30 minutes after all of that, he finally fell asleep. I hope and pray he can sleep all night long now.
We didn't get a chance to talk liver today with any of the docs. Maybe tomorrow. Please pray for J to have a pain-free night. I'm going to try to go to bed now so I can get up with him whenever he needs his momma. Good night, my friends.

The ultrasound yesterday showed an epididymitis and a hydrocyl but no hernia. I haven't seen the urologist today but I'm sure he/she will come by sometime in the next day or so. From what I have read, the epididymitis can be very painful. Our Infectious Disease specialist was surprised to hear he had that with all of the antibiotics J has been on. Hopefully it will clear up soon.
The CT scan went well this morning; Jameson was on best behavior. I think he liked being awake while we rolled down and back. During the procedure he was on propophol and he woke up well before they even got him off the scanner. This child chews through his sedation! Our SuperDoc looked over the scans and said there isn't much ascitis in his belly. His growing belly is all bowel and liver. His liver is huge. Not quite sure why, but J just had an echo cardiogram done to make sure the enlarged liver isn't a result of heart problems. Haven't heard back yet.
Our hematologists/oncologists will look over the scans as well and see how the lymphangiomatosis is doing as well. It is too early to really see any differences from radiation, but maybe we can find out something from the scans that will be helpful and hopeful.
Jameson was transfused this morning with platelets, packed red cells and chryo. Part of the reason his heart rate was up yesterday was because he needed blood. It helped and now he is sleeping in the 120/130's. Much better.
He is still having bouts of pain and cramping when he stools and he is still passing tissue. The bumps only work so much. I feel so bad for my sweet little boy. It is hard to watch him have pain and not be able to do anything about it. I just pray Jesus stays in that bed with him all of the time. Thanks for checking in. I will be sure to update when we get more info. God Bless!!

My child has almost been Sleeping Beauty this weekend. I'm glad he has been sleeping a lot because his very brief awake sessions have not been that great for the most part; he is having a lot of high heart rates, has been fluid positive for two days, last night and this morning had painful stools and stomach cramps that brought on even higher heart rates(over 200). Last night we had to give him a pentabarb bump and he's gotten lots of fentanyl bumps all day long. He's has some fevers up to 39.4 that coincide with his heart rates and they go away after getting pain meds, namely his beloved pentabarb. Even sleeping he can't get his heart rate below 150 today.
As a result of being so fluid positive( he is up almost 900 over the past two days) he has a bigger effusion on his left side and for some reason it isn't draining welll through the chest tube. And his right upper lobe(lung) collapsed again. He is also up on his weight -over 3 kilos above his dosing weight. His girth is the biggest it has ever been, 67 cm this morning. The intensivist ordered at CT Scan for tomorrow morning(7:45am) so we can see what is going on with his fluid distribution and see where the fluid is and why it isn't draining. The night nurse is going to have fun trying to figure out how to condense his pumps. He currently has two trees with four brains and fifteen pumps. And a CT scan is a short road trip so I'm sure they will want to get him down to one tree. It is hard enough to take this act on the road with one tree and the vent and the chest tubes and everything else. The nurses are amazing.
Adding insult to injury, Jameson may have another hernia. His testicles have been swollen and bruised and (unrelated) full of blisters( no idea on the blisters except that he is fluid overloaded). He had an ultrasound this morning and we will get the results when we speak the urologist tomorrow. Forgive me, because they did find something and the intensivist did tell me what is was but I can't remember for the life of me. It isn't anything too serious. He isn't stable enough to head to the OR unless it was absolutely critical, so I imagine we won't be doing anything extreme.
Right now he is sleeping in his little red chair, sucking on a toothette. Every once in a while he opens his eyes and gazes at his Christmas tree. Picture included! So adorable. I'm so glad that he is able to have good peaceful sleep with so much going on.

Okay. As of this morning we are off the norepi, (blood pressures have improved some) but he is still fluctuating so it could go back on at some point if needed.
This morning he lost an IV and his femoral line was iffy. They were able to rewire the femoral central line and it is now working well. They attempted to put in another IV but it wasn't working, so in addition to his femoral line, he now has a subclavian central line. At least he is able to get his medications reliably. Which is good since he is on a lot of stuff.
Sedation and pain meds seem to be doing much better. He is off the dilaudid and on fentanyl. And his dose is only 10mcg/hr! That only means something if you remember that before the pentabarb coma he was up to 250 mcg/hr...which is enough to take down three Hubs. Granted, he is also still on 2.5mcg pentabarb and on a hefty dose of methadone and they upped the precedex, but still, I'm pleased with that. Today we are going to start knocking down the pentabarb, but very slowly. He seems to be incredibly sensitive to even tiny changes in his medications. He was down to 2.0 but was back up to 2.5 when we ran into trouble the other day and we will be going down to 2.25 today. With that change the methadone was upped again. (When he was in his pentabarb coma he was getting 4.0 pentabarb.)
Jameson is also getting an anti-nausea medication but we aren't sure if it is helping or even if nausea is an issue. It is so hard to tell what is bothering him when he can't tell us or even really show us. He does seem to calm down more when he has it in his system, but it also has sedative qualities, so maybe that extra bit of sedation is what is making the difference. The only problem with giving it is that is can also soften his blood pressures, and we've had enough issues with that, thank you very much. We had gone down on his feeds from 20 to 10 two days ago and back to 15 yesterday without issue so today we will also be going back up to 20.
Today we've also been trying to use the power of distraction during wakeful periods. His vision is much too blurry from all of the drugs to really see the tv across the room, but Hubs held his laptop right in front of J and let him watch Dinosaur Train this morning. Sure is better than loking at the ceiling all the time. I think he even liked listening to it when he was too tired to keep his eyes open.
Over the past few days his belly tube output has been a brighter and fresher looking red. We aren't quite sure what to make of it and neither are the docs. Last night he also had about 2oz of bloody bile coming from his NG tube. It hasn't continued and we aren't quite sure what is causing it. Hubs is slightly concerned that is could be radiation enteritis. I hope not; it sounds yucky and we don't need any other problems for our sweet Jameson.
The belly tube and chest tube output has decreased over the past few days, but not dramatically and not enough to convince anyone it is a result of radiation. I pray this trend continues and it IS a result of radiation. I want nothing more than for Jameson to be well and come home.
Thanks for checking in. I'm sorry the updates are always so long, but there is always so much changing all of the time and it is all so very complex. We truly appreciate your willingness to continue reading about and praying for our smiley pie J. God Bless you all!

Jameson is doing alright. Yesterdays labs showed his white count up to 0.5. Wahoo! Keep going, baby! It is so hard to not be hopeful that this is a sign of his body healing and radiation working. Patience.
Going back up to 1.4 on the pentabarb has helped and he has been sleeping well. As a matter of fact, he is still asleep now from last night. It's like we have a teenager today. In other good news, the rash is clearing up. Amen. His skin is creamy and beautiful and he looks like the sweetest and most perfect angel sleeping in his big bed with all of his pillows and blankies. An angel in handcuffs because he is so strong and has such sassy hands. I love it.
Yesterday GrammyBear spent the day here and he sat in a red chair and loved it. He even played a drum. Can't wait to get him sitting again when he wakes up. I will post pictures tomorrow if I can.
Little Man brought a Christmas tree in and we decorated it and put a few festive things in the room. It is nice to see the holiday spirit in the room. Have a nice weekend!

Sigh. Jameson started having a rough time about 5 minutes after I posted the last post talking about how great he was doing. Sigh. He was struggling with withdrawal and pain and poop all day long. It is so hard to seem him writhe in pain, grinding his teeth, watching his heart rate stay in the 180's and 190's and watch him desat as a result of pain. He passed more tissue in his stools today and the cramping and rumbling never stopped. Auntie K was right on when she said it feels like he has a motor in his tummy. His temperature climbed up to 39.5, too, so we took more cultures today. We maxed out his fentanyl bumps and tried chlorol to calm him down so he could catch his breath, but it only worked for 25 minutes. The intensivist thinks we are weaning him from pentobarb too quickly so he gave him a pentobarb bolus and increased his drip to 1.4. It was 1.5 yesterday morning and 1.25 yesterday afternoon when we went down. Pentobarb was really, really great when we needed it, but now it is a headache, especially for J. Hopefully weaning him slower than molasses will work. After getting the bolus, he did calm down and fell deep asleep for the first time all day. Hubs just emailed me to let me know he is now saturating well and his heart rate is down in the 140's. Whew. Hopefully he will sleep all night long so Daddy can get some studying done!! Thank you all for your love and prayers!

I got a talking-to from SuperNurse this morning about waiting too long between updates. :) Sorry. Overall, Jameson is having a good week. His white count went up from 0.2(yes I wrote it wrong before) to 0.4. That is still low, but better. Fingers crossed that it keeps going up, up, up!
We haven't had any more really bloody stools and he seems to be having a more manageable time pooping. It still causes discomfort and pain, but not like it was before. Again, hopefully this trend continues and we are over the worst of it.
Our eye doctor was in this morning and said his eyes are doing well enough to only use drops and salves when he is really red or has discharge as opposed to lubing his eyes with a Vaseline substance every.two.hours.around.the.clock. J hates eye drops, so this is great news!
The rash is still here and looking angry. It has spread and it just looks like it hurts. They ran cultures but nothing has grown. It may be viral. It is worrisome to say the least and we all want it to go away.
We were able to go down a little more on the pentobarb and he is more alert every day. He is strong, too. Everyone is surprised at how strong he is considering how long he was inactive and how many sedation drugs he is still on. He needs wrist restraints now because he can lift the z-flo pillows right off the bed to get his hands free. I LOVE how feisty he is and can't wait for him to keep us even busier with his sass.

We are so thankful that our little guy is still fighting and maybe making progress. Being here all of the time is so hard. "Living" in the ICU is really hard. But leaving the hospital is harder. It is hard to leave J's room and sometimes it is even hard to let go of his hand. Especially when we see people walking down the hallway lost in tears and we know they are walking away for the last time. Walking away without their child. And we watch knowing that every day it is a miracle that we are not that family. And also knowing that any day we still could be that family. But not today, God willing. We have much uncertainty in our world. But one thing I am sure of, is that no matter happens, God will give us our daily bread.

I sit and I think about that. He never promises to give us leftovers, never an extra helping for that midnight snack. He certainly doesn't offer a Costco membership to get us through the month in one foul swoop. He says not to worry because He's got our back. No matter what, I know I can have reservations at the finest table in all of eternity anytime I need them and I will be served what I need and leave full. I can bring all of my fears and worries and anger and pain and tears that leave me empty inside and just leave them in the coat room. And when I leave I will be full. Full of love. Full of grace. Full of mercy, confidence, joy. But I have to go there each and every time and ask for that table for two and sit across from the date who paid the ultimate bill so I may satiate my hunger and leave full. And I know that every time I go to the table, no matter how hungry I am, no matter how long it has been since I was last there, He is enough. I will not leave hungry. I will not leave empty. I will not leave alone. No matter what. And today I sit in this room and I thank God for giving us our daily bread, for giving us the strength we need, for promising to be enough. No matter what.

Jameson has been doing so well with his sleeping and waking the past two days. Last night he slept through the night and he is still sleeping now. He wakes up and looks around for a few seconds and drifts off again. He is so stinking cute. We has also been having decent awake time during the days without a lot of pain. He still has his moments of stomach cramping, but overall, he is doing well with pain management. We've been holding him every day for an hour here and there. It is quite a production and takes a few people to hold lines and limbs and tubes to get him from the bed to the chair and back again, but it is well worth the effort. Holding him is such a gift and we are so thankful for every moment with our sweet baby J.
I jinxed him by talking about not needing blood products in that last post. Sigh. Later that day he had a bloody stool and needed packed red cells and platlets. He is getting platelets often, as well as chryo to help with clotting. He is still having bile and some bloody drainage out of his NJ (feeding tube) at times. Feeds are still stopped but I think the plan is to start up very slowly today and see how his gut handles it. I guess we'll see how that affects belly pain and cramping.
His white count is down to .3, which is really low. We suspect tomorrow he will be under protective isolation and everyone coming in will have to gear up in masks, gloves, and gowns.
Jameson has developed some kind of rash diffusely over the past few days and we aren't sure what it is or what is causing it. Go away, rash! We have enough to deal with already!
We still haven't seen any definitive signs that radiation has worked and Hubs and I have decided to hold off on chest radiation until we see some results from abdominal radiation. Jameson has so much wrong, but right now his lungs aren't part of the problem. And radiating them may very well take away his ability to ventilate well. At this point it seems the that the risks of radiation outweigh the benefits. Hopefully in a week or two or five we will start seeing positive results from the abdominal radiation and we can then start plans to move forward with chest radiation. We just pray Jameson can just keep fighting and give us the time we all hope will help. Hubs has read that it can take up to 6 months for radiation to start helping. God, please give us 6 more months.

Jameson likes his ketamine; it seems to give him that little something something to take the edge off, although I think he may be watching purple elephants dancing on the ceiling. He is dazed and confused for sure.
The GI doc came by yesterday and gave us a few more drugs to hopefully help with the abominal cramping and pain that amazingly still breaks through his arsenal of drugs. He is now on octreotide to help with the diarrhea and lomodal to help with the cramping. Feeds are still on hold but he may start trophic feeds again tomorrow or the next day. He'll be the only turkey in the room tomorrow. At least he is still allowed to have his lollipops and he loves them.
His NJ clogged and needed to be replaced yesterday. I was hoping the nurse would let him yank it himself but that would only reward sassy behavior. :) Maybe when he doesn't need it anymore.... The new NJ took forever to get in; J just wasn't cooperating. But we did get it in so he can at least get his oral meds. He is still bleeding out of it but the GI doc thinks scoping it would be too much of a risk at the moment; radiation has made everything inside very fragile. And he must not be losing too much blood because his platlets and hemoglobin were both surpisingly good this morning. I can't remember the last time we've gone 2 or 3 days in a row without getting something transfused. If you are looking for an inexpensive, yet priceless gift to give this season, please consider donating blood.
On Sunday Hubs and I both had a chance to hold Jameson. He was in a lot of pain that day and we were all hoping extra cuddles might help. I hope you enjoy the picture of me holding Jameson. I haven't held my baby since the beginning of September and it was wonderful and scary all at the same time. Then he got too hot and pooped so cuddle time was over. Hopefully we'll be able to hold him again when he is more comfortable.
Thank you for your prayers and love. We wish you all a Happy Thanksgiving!

J slept through the night last night now that his pentabarb is back up to higher levels. He needed three huge boluses to get him back down...it probably wouldn't even take one of them to put me out for days, but my kid chews through his sedation. He's getting bigger pentabarb boluses, on a higher pentabarb drip, and on a ketamine drip, plus they went up on his scheduled methadone and he is still on the highest dose of precedex available. And the fentanyl drip and boluses are also bigger...tripled, I think. Special K seems to be making the difference...that and the bigger fentanyl boluses.
The underlying issue- abdominal pain is still there...hopefully it will go away soon.
SuperDoc ordered an echo today and it turned out fine. We found out the tentative schedule for radation....next Monday he will have the planning CT scan and radiation will start on Wednesday. I think it is only 8 sessions this time. He now has two trees with 4 brains, holding 16 pumps, plus his vent so roadtrips to Abbott should be that much more interesting. Last time he just had one tree. I guess that is why the nurses get paid the big bucks. Ha!
He was in a somewhat happy place this afternoon and Hub's parent's loved on him while Hubs, Little Man and I went out to lunch with some family. It was so great to get out and have a nice lunch at our favorite restaurant, knowing that J was in an okay place. Hopefully the new drug combo will allow more uneventful days and nights.

J's had a really rough day. Sedation and pain have been a major problem over the past 24 hours. Jameson spent most of last night and today with heart rates above 180, sometimes up to 210. He has moments were he just writhes and is in so much pain and it happens so fast. It all seems to be related to abdominal pain. He seems to have some relief after having bowel movements but the relief doesn't last long.
We've tried upping his fentanyl and methadone; we have given chlorol, vallium, ketamine and nothing gives him relief for more than an hour. Even with both vallium and ketamine in his system at the same time his heart rate didn't get below 160 and less then an hour later he was back up to 210 and writhing in pain. Our SuperDoc is putting him back under with pentabarb. Which means we will probably be back on norepi for soft pressures shortly.
Jameson also started having blood in this urine last night and tonight he started having blood bubble(slowly but still a significant amount) out of his NJ (feeding tube). He is no longer being fed anything and they are running cultures on the blood coming out of him. He is also getting platelets and packed red cells right now.
We've done extra abdominal xrays tonight and SuperDoc looked at his heart with an ultrasound as an informal echocardiogram. She thought it looked alright so we aren't having a "real" echo done anytime soon.
Please pray that our sweet Baby J can get some comfort and relief soon and rest. Thank you for checking in and God bless.

Jameson slept all night long with only a few brief and calm wakings and he put himself back to sleep without needing any bumps or even hand holding.
Right now he is sitting up in his bed watching a movie. The intensivist last night found a pocket of fluid in his belly and went to put a new tube in but very little was actually in there so she just drained it with a needle.
Enjoy the picture and may God bless you!
 

The care conference went okay today. We had both our SuperDoc and SuperNurse there and it was wonderful to have their support and knowledge. It was good hear that everyone is on the same page and hopeful that radiation could still work.
Jameson's biggest issue is that his immune system and bone marrow are both compromised, but not from the radiation as I originally thought. It is from the lymphangiomatosis. He is leaking too many essential things into the chyle and then it leaves the body from the chest and belly tubes. As a result, the rebound we have been waiting for before radiating his chest won't happen. Instead, until his disease in stopped/slowed he will continue to become more and more immune compromised, which is already a tough thing as he is almost bottomed out. Infection may be one of our biggest fears and problems; if he were to get a bad one, it would be extremely difficult for him to recover. The plan is to start radiation of the lungs and chest cavity after Thanksgiving. Waiting longer only lessens survival chances.
We are also starting back up on the rapamiacin (sirolimus). I am at home with Little Man but it may have already been started back up today. This drug is immunosuppressive, so there is risk but we have to try everything to stop this disease. He has been taking the inferon as well over the past few weeks.
There are no other options left on the table. If sirolimus and/or radiation do not work as we hope they do, we will put a plan in place to keep him as comfortable and happy as possible and enjoy every precious second God gives us with him.
Please pray that Jameson stays well and infection-free. At this point, infection may even be a more dangerous enemy than lymphangiomatosis. And please continue to pray that radiation works and heals our sweet Jameson. God bless and thank you all for your prayers and love.
Addition: I just got off the phone with Hubs and J's belly tube has stopped working and was pulled out. He will most likely be getting another one put in tonight or tomorrow morning. Poor J.

Jameson has done alright over the past day. Still off the norepi with decent blood pressures. We are going down to 2.0 on the pentabarb today. Hopefully it will be a smooth transition. He does still have bouts of irritation and agitation and they do seem to correspond to rumbling and gurgling in his precious tummy. He is obviously experiencing pain in that region. But usually after a few minutes, it calms down a bit and he settles. He does need occasional bumps and he gets tylenol and chlorol at times and his precedex was upped again, but overall, he's handling the detox and pain fairly well.
Yesterday he was opening his mouth really wide and we noticed he has a brand new molar on the top. And later I noticed that he has two more on the bottom just breaking through. Poor kiddo. We tried baby orajel and he is not a fan, but I don't blame him; it tastes awful. He much prefers the jolly rancher suckers we've been giving him to lick whenever he sticks his tongue out. :) I don't know anyone who deserves a little sweetness more than Jameson.
He has needed blood about every other day and the docs are thinking that it is related to the belly chyle's brighter red color. It seems he is leaking a little more than usual. Not much we can do about it and I'm not sure if the output has actually gone up or not. Sorry.
Tomorrow we have a care conference in the afternoon with all of Jameson's doctors. I am a little anxious about it. Overall, we are in a better place and Jameson seems to be in better health than he was at the last care conference- which had me in tears for days afterwards- but we cannot deny that fact that his disease is still uncontrolled. Pray for us and for our doctors as we discuss J's health, options, and future.

Sedation and blood pressure issues....again. His blood pressures are still very soft -when he is asleep- but when he wakes up they jump up too high so he's on and off the norepi a lot(like 60s at rest and 160's minutes after waking). Last night SuperDoc started to change up the meds again so he is a little more even keel and the pressures won't change quite so fast.
Methadone was increased and dilaudid was also increased and is now given continuously instead of a just bumps when he gets agitated. They also went up on the precedex and down on the pentabarbitol. When he wakes up, he doesn't seem like he is in pain, but irritated and agitated. He seems itchy too and his eyes are really blood shot. Some of it may be withdrawal symptoms. Some of it may also be an inrritation to the dilaudid. Dilaudid is in the morphine family and morphine makes J throw up, so... The pain docs doubled his oral narcan to see if that will take away some of the itchines sand irritation. They are also looking into dilaudid a little more and possible solutions and alternatives if this is indeed causing his problems. The pain and sedation plan is a work in progress, as always.
Yesterday his belly fluid grew a culture. He may have a gram positive rods infection and we should find out more today. It could really be an infection or it could have been from a contaminate somewhere when culuturing. I guess this type of infection isn't very strong so it seems unlikely for him to have this on the antibiotics he is on. They took more cultures of the fluid and also took viral and fungal cultures to see if anything grows. Additionally, he had abdominal xrays last night looking for pockets of air but they showed nothing.
Mid-update- sedation woes continue. We're in the throes of pentobarbital withdrawal, sensitivity to dilaudid in the form of itchy eyes/face and probably nausea (similar to his vomiting episodes in St. Paul with plain morphine, but knowing for sure he is wretching with a trach and NG tubes is not perfectly clear). Yes, a crappy day so far as his breathing rate gets into the 50s, heart rate in the 180s, blood pressure in the 160s. Now it's a matter of adding more meds to try to mitigate the side effects before abandonig his current regimen of pain killing and sedation.

Jameson is having a bit of a rough day. He is having low blood pressures, 60's/20's so he is back on norepinephrine. He also having higher heart rates and fevers (above 39C) and showing some signs of discomfort during his more frequent awake times.
We all think it is highly unlikely that this is a result of an infection. Jameson just completed 8 days of vancomiacin and is still on mirapenum. But just in case, the intensivist ordered one more dose of vanc today.
It is a possibility that this is pentabarb withdrawal. He spent a long period of time in the pentabarb coma and it could be that we need to wean him a little slower. His methadone was upped today and he's been getting a lot of dilaudid bumps so we are able to keep him comfortable most of the time. He also his getting to spend more time on his belly, which helps(yay for flexitrach!). He saturates better and seems more comfortable whenever he has tummy time.
Hubs just took Little Man home to make snowmen and play outside in the winter wonderland and I'm going to stay here with J tonight. As we were driving in this morning and trying to stay on the unplowed roads, I was thinking about how the last time Jameson was at home it was "diaper weather." And now it is snowpants weather. This coming Tuesday marks 11 weeks in the hospital. 11 weeks since we've all been a family under one roof. 11 weeks since I've gotten to hold my baby and see him smile and make him laugh. I am so grateful we have this time here for Jameson to heal and get better, but, man, I'm really looking forward to bringing him back home as soon as possible.

Flexi-trach is in! As in, in Jameson's throat. And everyone loves it. Especially Jameson. And it is just in time since he is starting to have some awake periods. Not much yet but we are lightening sedation this week. It will take a full 8 days to wean off the pentabarb. He was up to 4 mcg/hr and they can knock off 0.5/day. He's now on methadone and we gan give him boluses of diluadid if he needs a little something something every once in a while. Hopefully the waking up of our comatose baby will go smoothly. The last time we switched everything up was a disaster but I'm hoping that now that his trach is finally healing well and more comfortable that he won't be in as much pain. He will still have loads of pain for a while- chest tubes are excruciating and I imagine a belly tube isn't much better.
Oh and speaking of that peritenial drain (aka belly tube), the nurses have had a hard time pulling fluid off so they put on a gravity drain so it can flow as needed. It is different than the chest tubes because there is no vacuum seal, no extra help. Just a tube with a urine bag at the bottom.
And speaking of urine -he has been a peeing machine. We are getting out good amounts; so much so that the bumex has been dialed back down. The stool is also on the move so J is doing well with removing the fluids, etc that need to go. He still has some cheeky jowls but the cankles aren't bad. And for the record, the cankles were never all that bad, but the word is way too fun to pass on... In just two days, he is down 1 kilo. Still has some to go to get to his normal weight, but this is a great start.
Still no change on the chyle output. I am trying my darndest to be patient but that one is difficult. I suppose as long as he is venting well and staying stable, I'm glad we have the time and happy to wait but at the same time, oh, it would be so sweet to see it go away and not come back. But we do have time right now. His lungs are happy; not happy like he can come off the vent happy, but he has been satting well. Today he has been down to 40% fiO2 and he was satting in the mid/high 90's. At one point when Little Man was telling him stories about crazy legomen getting eaten by sharks he actually got up to 98% satuation. I took a picture of the monitor to prove it but left the camera at the hospital. Patience isn't my virtue of choice, but I'm happy to wait and see as long as we are in this good place. I am learning a bittersweet benefit to the patience lesson; waiting is good because that means J is still here and in a critically stable place.
We had a really special visitor today. Very special. One of our intensivists introduced us to a healthy, beautiful, and vibrant teenage girl and her sweet father today. And it was so wonderful. Wonderful because she is beautiful. And vibrant. And healthy. And last year she was in the PICU with 6+ chest tubes and almost on ECMO. And not able to even speak. And she has lymphangiomatosis. And today she was here for a check up and xrays, etc to see how she is doing. But you don't need to see her bones and pleural spaces to see she is alive and well and thriving. She is a glorious miracle. The same miracle we are praying for. And while every case is so different with this nasty disease, it gave us another glimmer of hope. Some people do pull through the darkest times. Some people can survive. Miracles do happen.
And no matter what happens with us, no matter how this all turns out, please pray for a cure. Because we are just one family that you know of out there, struggling to understand, struggling to survive, struggling to hope. But there are many more out there fighting this daily battle and hoping for a miracle too. So in 6 months, and even in 6 years, please still think about J. But not just J. Think about this other beautiful, amazing young woman and countless other families who are losing their mothers and fathers and spouses and sons and daughters. Think about the families who have already had to say goodbye to their beloveds. And please, stop and say a prayer for peace and healing and for a cure.

Today was the last day of abdomen radiation and it went well, with no hiccups. Now we wait and hope for the best. We don't have a plan for the chest yet; I think it really depends on how fast his body rebounds from this radiation.
His weight is up even more today. Yesterday he finished the day fluid positive(over 700mL) and even with his bumex doubled he still isn't peeing as much as we had hoped. He has so much extra fluid in his body and the edema is just awful. His skin feels like jello; it is the first time my skinny boy has ever has jowls and rolls...rolls of puffy, fluid-filled skin. Everywhere. To the point that he may even be bruising from the edema. Cankles, jowls, shoulders, arms, legs...you get the point. Poor J.
One of our intensivists who was not working today stopped by to chat and get some candy this morning and Hubs mentioned a new belly tube might help since the old belly tube is old and not working. He popped right back in the room with an ultrasound machine and put a new one in right then and there. And promptly pulled off 200mL plus whatever initially came out when they put it in. That candy basket is worth the money! :) Hopefully this new belly tube will make Jameson a bit more comfortable.
Sedation is hopefully going to be lightened tomorrow. We really want to get Jameson as awake as possible and get him using his body again. He has been heavily sedated and paralyzed for most of the last 2+ months and has very little muscle-power left. Even his bowels have fallen asleep from the sedation drugs and we need to get this boy up and moving a bit so we can start back on the road to recovery. The physical therapist and I were talking this morning and she said it typically takes kids 2-3 times as long as they've been sedated to get their muscle strength back to where it was. That means it will take about 8-12 months to get J walking and running and climbing again. We start slow with baby things, like tummy time and learning how to roll over again. I can't wait to get to the point where that is what we can focus on.
One of our specialists came in to talk with us this morning and wanted to get a feel for how we are feeling about everything. Basically it came down to wanting to know if we were still hopeful or giving up on the idea that this could still turn around. While that may sound really callous, it is a valid question. A few weeks ago we had a care conference with all of our specialists and there was very little hope left for our sweet Jameson. It was to the point that they flat out said they would understand if we decided to let him go that day. Yes, it has been that bad. And every day, we are aware that he is here by the grace of God(as we all are...) and it could take a turn for the worse at any moment. Her questions kind of caught me off guard because -while nothing with his disease has actually improved- J is doing okay and we are still in experimental treatment. Of course we still have hope! I guess in a way, getting grounded once in a while isn't a bad thing but it does carry a nasty sting. I do still hope and believe that we can beat this -even if only for a few more years- but I want to be realistic too. J has been diagnosed with a terminal disease and he has been fighting for his life for over two months. How do you come to terms with that? Right now, hope and faith are just about all that keep us going. We hope radiation works. We hope his body heals. We hope his lungs strengthen. We hope a freak infection doesn't sneak in and ravage his body. We hope he keeps being the amazing fighter he has been. And we hope this buys us enough time for a cure. It is a tall order and he has already defied all the odds by making it this far. Jameson is a living, breathing miracle and I have faith that God can continue this miracle. And I hope that is the plan.

Jameson did well on the radiation trip yet again. We made it there and back uneventfully and tomorrow is that last day of ab zapping!
He is saturating well- 95% at 55%fiO2. Not too shabby.
He is still showing signs of infection even though nothing has grown on any of the cultures sent out. He is now ruddy again just like the last time. He hasn't had a fever in over a day now. We just don't know what is going on with him. He is on vancomiacin and mirapenum (heavy duty antibiotics) and it is causing some kidney distress. His creatinine was up to 1.2 and BUN was also high...I'm blanking but I think it was in the 60's or 70's. And I can't spell medical terms- sorry to all of Hub's friends.
The past few days he has been very fluid positive which isn't great. He is getting bumex(to make him pee more) but every day for the last three days it has leaked at one point or another...we've had these problems in the past and J's bed and floor have been heavily medicated during our stay. He also isn't pooping much. We had a few days where it was looking better but not enough is coming out of J. His chest tube sites are putting out about 1/2 of what they used to hourly and today they haven't been able to get anything out of his belly and just 2 days ago they were pulling out 400mL at a time(every 8 hours). While this at first seems great- maybe he isn't making the fluid- it isn't the case. He is up almost 2 kilos in weight. So, the pee, poop, and chyle are in there and just not wanting to leave his body. I don't get it.
Sigh, at least he is breathing better... With Jameson, if it isn't one thing, it is three others. My poor baby. The other day when he was in radiation I just dropped to my knees in the middle of the room and begged God to please heal Jameson and let us keep him for so much longer. On the way back to his room from that trip he started breathing better. I believe with all of my heart that God hears me and loves me and loves Jameson. I believe that Jameson will live. But I can't help feeling bad for all of his suffering in the mean time.
After radiation, Little Man and I went to the Como Zoo for the afternoon. We enjoyed watching the polar bears and giraffes. The last time I was at the zoo, Jameson was with us and we had a wonderful time and picnic lunch with great friends. I can't wait to get back there with both of my boys next summer.

 
Jameson has had a big morning! Daddy actually held him in his arms without even sitting in a chair. I think they both adored it equally; Jameson gave his first big smile since we've been in when Daddy put him down- almost like saying "I loved it, Dad!" It was the most beautiful thing I've seen in a long time. Then he watched Toy Story 3 in his little red chair. Okay, he slept through most of it, but I know he loved it. Then PT came by and we got him sitting up on the edge of the bed! He doesn't have the torso strength yet, but he was able to hold his head up by himself and move his arms and arch back a bit. He continues to absolutely amaze everyone with his strength and feistiness. I am SO proud of my boy! After that he fell asleep and slept through Santa's visit, but we can show him the picture later. :)
The liver is the biggest concern of the week. The echo turned out fine and today he had a dopplar of the liver and the flows were all fine. His liver function is fine, but it.is.huge. And it got huge fast. No one understands what is going on with it. Could it be a drug toxicity? Or a delayed reaction from radiation? Or could the lymphangiomatosis be the cause? Those are just a few of the many questions our team of doctors is researching and mulling over. SuperDoc really wants to get a biopsy to see if that tells us anything about it. We aren't so sure about that yet and need to think about it and hear a little more info from our specialists first.
His girth and weight are still way up. He is up to 17 kilos and his dosing weight is 15 kilos. (They actually just upped that yesterday, the original dosing weight when he was admitted was 13 kilos.) He hasn't been peeing well and had to get the catheter put back in last night. It is helping but he is still fluid positive again today. He hasn't stooled since yesterday. Hopefully we can empty him out a bit and get that girth and weight down. Please pray about that liver.
Hubs and I are humbled and grateful for your continued support and prayers and love thoughout this rocky time in our lives. We appreciate each and every prayer you say for our amazing Jameson and for our entire family. Thank you so much and may God bless you as well.

Just a quick update to calm nerves! J has had a great weekend. He has been breathing well on the vent and even got down to 35% fiO2 for a bit Saturday! He's been at about 55% fiO2 all day today and satting in the 90's. Good things! His blood pressures started to go low Saturday and last night they started to get too low so he is now on norepinephrine(sp?) and doing great on it.
His heart rate and temp also went up, up, up Friday night. He hit 38.9C and it now on the heavy hitting antibiotics again. They are also running cultures on everything, but just like the last time he was septic, no one is expecting anything to grow. J is a mystery! The only great thing about the fever is that his saturations get better. At one point when he was hot (38.9) he actually satted 100% on 60% fiO2. Wow. 100%. Awesome, except for the whole high fever thing. But today he has been good on temp and saturation. And the Vikings won in OT. We were so excited and celebrating so much the intensivist actually came in the room to tell us to quiet down. Not sure if he was kidding or not. And our Supernurse wore her AP jersey. Loved it. We actually have Supernurses on during the day and at night this weekend. Awesome. So yes, great news all around, minus the possible infection thing.
Really excited to get to Wednesday and not have fieldtrips for awhile. Not sure what the plan for lung radiation is yet but as soon as we find out, we will let you know. At this point, he is in slight bone marrow failure from the radiaiton and some of his medications so I think we have to wait until his marrow gets a little better before we can start the lungs. Hopefully by that time we'll see reduced belly fluid and see that radiation is working for our little man!!
Oh! I almost forgot! Other big news from the weekend is that when Hubs was gone, I cut Jameson's hair a bit. Hopefully this week on the road, people will stop whispering about "the sweet baby girl" in the hallways at Abbott. Seriously. Even with long hair he looks all man to me, but I caved and cut. He still looks super cute and I'll have to take a picture and post it to prove it. How could J ever not look cute. :) So much for a quick little note....hope you all had a great weekend!! Love, Meghan

J has a rocky night and morning as far as oxygen saturation goes; He was sitting at 100% O2 and still desatting this morning. Last night he spent most of the night on his tummy and even that way they couldn't get the O2 below 75%. But we still went to radiation as planned and now he seems to be doing a little better. He is down to 65% O2 and satting 89ish on his belly. No other news. We are hoping and praying for a very quiet weekend. Monday and Tuesday are the last radiation days for his abdomen. Hopefully soon we will start seeing signs that it is working.
We wish you all a blessed weekend and hopefully you won't hear anything from us until after radiation Monday. :) Happy Friday

Tummy time was good for J all afternoon and he was stable enough to head over to Abbott for radiation. Praise Jesus. We just got back and he's doing alright...still not great, but I'll take what I can get. He's not desatting but still at 80% O2. Hopefully we can get down to at least 60% by bedtime and then get to stay there or keep going down. The past few days I've been having awful dreams about coming back to see J and having ECMO back in the room. I sure hope his lungs can strengthen up and heal on their own so we don't have to head back down that road again.
Nothing new to add, still issues with poop, gas, and chyle. I know it can take a few weeks but I'm anxiously awaiting positive results from the radiation. This just has to work!
Our lovely nurses are doing their best to untangle Jameson and get him settled in for the night. I'm always amazed at how awesome the staff is here. Team J rocks, bigtime.

We are still having saturation issues. J is on 100% O2 and still desatting. While he isn't setting the alarms off he still hasn't gotten above 89 today. Crappy.
Even with colon cleaner he still isn't having great poop either. Right now he isn't stable enough to go to radiation so we've rescheduled for 4 pm today and the goal of the day is to get him stable and satting at least above 85 consistently and not on 100% O2 so we can keep his appt. His girth is still up- 61 cm. We are checking abdominal pressure right now and will probably be getting an ultrasound to see if there is a fluid pocket the abdominal drain can't get to. If so, he may take a quick trip to the OR to either get a belly tap or another belly drain put in. Joy. Okay, just got the pressure and it was fine, so this plan is on the back burner...we are going to turn him on his tummy right now to see if that helps and try to pull more fluid off the belly drain.
Today is green day at school for Little Man and we are both decked out in some great Irish green. I'm praying for the luck of the Irish to be with J today. We know that we have a great medical team and powerful medications and procedures to work with but only God can save. Ultimately, His plan will prevail and His plan is good. We cannot praise Jesus for the good in our lives and curse him for the suffering. He is the same God today as He was the day he gave us our Baby J. And I am thankful for that. I praise Him in this storm and pray that he sustains our family, heals our Jameson, and that all of this suffering and pain will ultimately glorify Him. And today we have extra prayers for lungs to work. Thank you for praying with us and for us.

Today was a little rocky for the travel. Nothing bad happened but his saturation levels have been down for the past 24 hours and we just can't seem to get him ventilating as well as we want him to. During his roadtrip, he was on 100% O2 and only satting in the 80's. Not that we are back in the room and settled in bed he is down to 70% O2 and satting high 80's/low 90's. I would love to get back down to the 35%O2 we were at a few weeks ago.
No one knows for sure why he his lungs are being slightlly naughty but it probably has a lot to do with fluid, gas and poop. His girth is up to 63cm and it needs to shrink! We need this boy to poop and fart. And stop making chyle. Hopefully today we will have some good luck with cleaning the system out and his saturations will improve.
Little Man asked me at lunch if today is Wacky Wednesday...I sure hope you are all having some wacky and silly fun. We are watching Veggie Tales with Jameson; he is sleeping through it and hopefully Little Man will follow suit. Have a wonderful day and God Bless!
Meghan

We are back from radiation again today. Today was day 7 of 12 and again it was a smooth ride. Tomorrow the Radiation Oncologist will decide if we need to complete all 12 treatments or only do 10 treatments. From the information she gathered at the Boston conference, it sounds like not everyone required as much radiation as Jameson is getting so she is hoping we can cut it short. However, in order for that to happen, we actually need to see improvement and see that his body is not producing the chyle in such large quantities, his girth goes down, and his overall condition starts to improve. As of now, that hasn't happened. Jameson still has a very large girth, is still requiring a number of transfusions(mostly platlets), and still have a lot of chyle pouring out of his chest tubes and belly tube. I would be shocked if they cut the treatments to 10. We are still optimistic that radiation may still work, though; sometimes it can take weeks after treatments have ended before things start really turning around.

Chances are we will start radiating the lungs before we even know if the belly radiation has worked. At this point, we are just so happy that Jameson has remained critically stable. This is the longest stretch we've had since he's been in the hospital where we haven't seen deterioration; there hasn't been any dramatic improvement, but as long as we don't start sliding backwards and his pain is under control, we are happy to have the time to wait it out and see if something begins to work. And pain and sedation have been managed wonderfully this week. Jameson is really under with his pentabarb and precedex and very comfortable. Most days, he just sleeps with an occasional eye opening, lip flutter, or finger squeeze. He isn't really awake at all, but he isn't in pain either. No teeth grinding, no grimacing, no writhing. It is a relief for everyone.
I'm at home with Little Man right now. He has really been struggling the past few weeks with our family situation. I think the stress of everything and the lack of normalcy has really caught up with him. It has been over two months now. Yuck. We are really trying to spend more time with him and have him at the hospital more to see his brother, but it is difficult to ask a 4 yr old to sit on a couch in a room and talk and read books to his sleeping brother for long periods of time. We have started to embrace family movie time at the hospital often; Little Man's brain may just need a little rotting in front of the TV during this time so he can be close to his brother and get extra snuggles with mom and dad. One of the things he seems to struggle the most with is sleeping at night. He and J share a room and he really seems afraid of sleeping alone. We have totally upped snuggle and story time, but I have a hard time deciding when to stick with the rules and when to be a softy...I just keep praying I'm doing the right thing and don't screw him up too much. It is so hard to have my babies in two places. I can't really be with both of them at the same time and I have such guilt and anxiety about the one I'm not with whenever I'm away. I logically know I'm doing a good job- at least the best I can, but it still pulls at my heart strings and I feel so badly for Little Man.
There really isn't any part of this whole thing that is easy. I'm so glad God is carrying our entire family through this time, because I'm not this strong on my own. Hopefully this radiation will start to work and we can start thinking about bringing J home and being a family under one roof again.

Again, sorry for not updating earlier. Jameson has had a good day and did really well on his field trip to radiation. We are now halfway done with ab zaps! My mom went back home for a few weeks so we tried taking Little Man along for the ride today and he did really well. They sweet ladies in radiation oncology even let him pick out some sweet treasures and stickers for himself and J. Then we ate lunch together at the cafeteria and Little Man watched a movie in J's room while I got in my kisses and love with J. The highlight of the day was putting Jameson on his tummy. It was awesome! First time since we've been at Minneapolis Children's that J's been flipped over. Our Supernurse made it look so easy too. Why, you may be asking, is it difficult? Well, first off his trach site still isn't healed all of the way and very sensitive and needs to be kept pretty still and straight on. Secondly, chest tubes with drainage lines hooked up to large suction/storage containers. Thirdly, IVs and lines everywhere. J's on a tight leash. But she did it and he promptly feel asleep and seemed oh so happy to be on his belly. Hopefully we can do this almost every day.
Thank you for checking in. God Bless! -Meghan

Happy Halloween! Little Man is going trick or treating tonight for candy and sweets. Jameson hopes for chlorol(HA!) as an extra special halloween treat. Hahaha. But he doesn't need much- the pentabarb and precedex are working well and he is even able to have some awake times without pain. Love it! Our little J-Rex has had an awesome weekend and we are looking forward to another great week of radiation and healing starting tomorrow! God Bless you all and praise Jesus for healing, stability and chocolate. Seriously.

 

Five down, seven to go! Radiation and travel seem to get better and smoother every day. Amen! J's team is always amazing. We love our PICU staff. Jameson is having a great day today and all is well in the world. He has the weekend off from field trips and we will go back on the road Monday around 11:30. Have a wonderful weekend and Happy Halloween!! Love, Meghan

Four down, eight to go! I'm at home with Little Man but Hubs said it went well. I saw J for a bit this morning and he was comfortably sleeping and looking good. Little Man has been needing some extra love this week so I'm trying to spend a few full days with him.
This morning was Little Man's Halloween party at school and the Trunk or Treat in the parking lot. It was so cold and there were snow flurries. I loved watching the 3 yr olds struggling to hold their bags going from trunk to trunk to get candy and treats and think about how cute Jameson would be in his costume as his teachers try to keep him from sitting down and eating the candy the second he gets it at each car. He was supposed to be in that preschool class; maybe he can join late or next year after he gets better. Little Man wanted to be a boat captain and he wore his daddy's Navy hat with some of his old ROTC medals on a blue blazer. He was so proud to be his daddy for Halloween. After getting the treats we all went inside and the kids sang some fall songs for us. It was adorable. Little Man didn't sing the songs during the program; instead he stuck out his tongue and made faces at me in true him fashion. Wish I hadn't forgotten the camera at home. God bless my big boy; he keeps me sane and gives me more joy than anything else could right now.
Stay tuned for super cute Halloween pictures this weekend! Have a great day and I'll update tomorrow after day five of radiation! We love you all dearly and every day we are so thankful for your prayers, love and support.

Three smooth days of transfers and radiation! Be still my beating heart! And it is totally a result of your prayers. And our Supernurse. And Superdoc. We are so blessed to have such an amazing team working with us. On the way back through the tunnels our radiation oncologist caught up with me to introduce herself. She was in Boston all last week at a radiation oncology international conference so we have only met her partners until now. She mentioned that she learned of five other new and unpublished cases of lymphangiomatosis being successfully treated with radiation. Hallelujah! Hopefully we will get a chance to meet with her soon and find out more information.
Jameson just had a chest tube put in a few minutes ago and he's already had about 80mL. The chest x-ray showed it needs to be pulled out a bit so they are working on fixing it right now. Hopefully his stats will improve now that his effusion is on the way down. Just to recap, he now has one chest tube in the right side, one in the left and one tube in the belly. This is the 4th one he's had on the left side.
No other news today...radiation is scheduled for noon every week day and we leave around 11:30am for the trek.
Oh, sedation! He is finally found his happy place last night after 4 pentabarb boluses and lots of hand holding. But they are again changing things up so hopefully it will go smoothly. He is off versed right now and they are dialing down the fentanyl and that will be off by the end of the day. Then he'll only be on precedex and pentabarb for at least 7 days. Hopefully this time the reset will work. Fingers crossed, knees bent. :)
I think Little Man is coming in and we may do a family movie on this cold, blustery day. I hope you all have a great and blessed day and thanks for checking in! -Meghan

So sorry for not updating sooner. Everything went great with transport and ab zapping again. Praise God.
J's left effusion has grown and he is probably going to get a pigtail(chest tube) or they will poke him and take some fluid off. He is satting in the high 80's.
We are weaning off propophol and probably going back to background pentabarb. And Hubs and I did trach cares and suctioning on J for the first time. Our nurse gave us gold stars for the day. Well not really but she said we did good.
Radiation will be weekdays at noon thru Nov 9 and we usually leave the room sound 1130. I'll be sure to check in and let you know how transport goes daily. Love, Meghan

And breathe. We made it there, did the first ab zap and made it back without a hitch. One down, eleven more roadtrips to go. Still not having great lungs, but we did make it through the entire trip without desatting. Tomorrow's treatment is scheduled for noonish. Hopefully it will go just as well. Thanks for all the prayers and God Bless!

Sedation issues -what's new? Off pentabarb and it doesn't look like the receptors have been reset. He's already at 100mcg/h of Fentanyl and it is giving like 5 minutes of relief after bumps. So propophol has been added. Holy canolli it knocked him out the second the line went in! Don't worry, we have way better doctors than Micheal Jackson had. Waaaay better.
Right upper lobe is collapsed again so we are working hard to get him ventilating better before he takes this act on the road. Right now he's at100% O2 and satting around 89. Yuck. They are getting some decent goobers at suctioning so hopefully it's a mucus plug and we can get it out soon.
Since we are off the pentabarb the EEG is gone; no more onion hat. Yay. But based on sedation issues and the fact that propophol is a short term thing, both pentabarb and onion hat may return. We'll see what the pain team comes up with after we get back from Abbott.
As of now and pending desatting issues we are all set to go ahead with radiation today. The trek through the tunnels is scheduled to begin at 11am. Please prays for smooth travels and throw an extra one up there for our nurse today. She just got back from a week in NYC; nothing says welcome back to work like having J plus a long field trip. I hope you all have a blessed Monday morning and I'll make sure to check in when we get back to our room this afternoon.

Today is a little better than yesterday was. Amen! Jameson completely cleared out his bowels yesterday and I think he is feeling so much better. Yesterday he finished the day with -1500 for his ins and outs. So he has a lot less fluid(and poop!) in the system which is a great thing. His overall edema seems less today too.
His chest xray looked a bit better today as well. He still has that huge belly but his pressures are low and the girth is 58, which is not bad for him. Labs were all improved this morning too.
Yesterday the intensivist started him back on Precedex and that seems to be helping. He may have been having some withdrawl from it. This morning he is also back on Fentanyl and Versed. The 30 mcg/h they started today are a far cry from the 225mcg/h he was getting before we switched to the Dilaudid and then the Pentabarb. Hopefully his receptors have been reset and we won't need to escalate too quickly. He was a little awake this morning and not really happy, but not in pain. No unhappy faces, just sticking his lower teeth out once in a while. Now he's in his fentanyl happy place and snoozing with dino and blankey love.
He is still on steriods, insulin, meds to lower his blood pressure(I always mix up the lowering/raising names so I won't even try...), miralax, and about 20 other things that escape my mind. And feeds have slowly started today. Again.
As of right this minute, the intensivist thinks he is stable enough to take his radiation roadtrips. Amen! Now we just have to hope he stays steady or improves over the next few weeks. Thank you for your prayers; the Lord has been so good to us and He is answering our prayers. Team J has been storming Heaven and God is listening! Every day with Jameson is truly a miracle. God Bless and Skol Vikes! -Meghan
 

Jameson is not doing great today. His fever is back. His heart rate is high. His bowels are huge and not working and possibly full of fluid. He seems to be bleeding in his belly and possibly in his bowels but no one is sure how much or where it is coming from. He is in rough shape and the doctors aren't sure if he will be stable enough on Monday to survive his daily roadtrips for the radiation treatment. Avastin is off the table right now due to bleeding, too. We are running out of options. I still have hope but I'm also very scared. I don't want my baby to go to heaven so soon. Please pray that he will stabilize enough to get the radiation.

Jameson had his CT scan this morning and did great. Now the radiation oncologists will work on the plan and hopefully we can start something soon. Still no culture growth and his fever has been down since yesterday. Hopefully whatever he had(has?) was mild and the drugs kicked butt.
Right now, Little Man is here and we just had a family lunch together in the cafeteria (obviously not J) and now we are all snuggled together on the couch overlooking J in his bed watching the Princess and the Frog together. Little Man has been wanting to spend a lot more time here and tells me often how much he misses his brother. Breaks my heart. At least we can all watch movies together. :)
I hope you all have a wonderful weekend; hopefully we won't need to update until Monday after we get the radiation plan.

No CT today, but it probably worked out for the best. Just as the were about to go on the road with the entourage, they found out the CT Lab had no power/lights. Instead, our SuperDoc went to work with the afternoon plan: removing the non-draining left chest tube and sticking a needle in the left effusion and pulling off fluid. The old left chest tube wasn't that old- it was the one put in for his pnuemothorax and it had never drained much and probably did nothing but irritate him so good riddance, chest tube! His left effusion was bigger this morning and needed some draining since it was affecting his ability to breathe so she stuck a needle in his chest and pulled off about 140mL of chyle(the fluid). He is now satting much better but he is still on 70% O2. His post needle-drain xray looked better but they are holding off on the CT until he is more stable.
On the infection front, Jameson has not gotten worse, he may even be a bit better. His fever has been down a bit and he hasn't had tylenol since 3 am. The cultures sent out haven't grown anything yet so we don't know what we are fighting. His white count was way up this morning, almost all nutrifills(90%).
He is now heading into his pentabarb coma today and off all other pain and sedation drugs. The EEG machine is monitoring him continuously and a neurologist will be coming by sometime to check on him.
SuperDoc is going to put in an Arterial Line shortly to continously monitor his blood pressure since it is still low and he's still needing the meds to pop it up.
Hubs and I finished our trach care class in record time. Suctioning and cleaning the trach on a fake, totally still baby was easy! So was the CPR. I have a feeling by the time we will be trying out our new skills on J, it probably won't be so easy. Just getting a diaper on him is challenging enough when he is well! They recommend swaddling hands in a blanket while doing trach care, I'm thinking handcuffs will probably come in handy...
So, maybe CT tomorrow if J feels up to it. Radiation probably won't start until the infection is treated and long gone. Always busy, always changing...hopefully only for the better from here on out! Thanks for checking in and praying. Love, Meghan

PS...Just found out, they are starting him on IVIG to boost his immune system and they are about to get a culture from his lungs through blind lavage. Should be safer and easier than a bronchoscopy right now.

So good to have the pain meds working and a calm and comfortable child. Although, calm probably isn't the right word....comatose works better. I'm not sure if he is technically in a coma, but he is low enough that they are doing continuous EEG monitoring to make sure they don't bring him too low.
All of the nurses who have looked at his trach site today think it is starting to look better. Having him still and not moving his head around and not being in constant pain and discomfort seems to be helping him to heal, which is a great thing. Back to the EEG for a minute: He has electrodes all over his head with a white fishnet stocking over the top of his head and all of the wires coming out of the top of his "hat" and it just reminds me of an onion. What a sweet onion he is. :)
The big news of the day is radiation. We met with the pediatric oncologist and the radiation oncologist this morning and are going ahead with arrangements to start radiation sometime next week. We are still thinking about the idea and letting it sink in, but from the reports we've read, it seems like it might be a promising therapy to try. We are in uncharted territory, prayerfully stumbling through the darkness, hoping we make it down the right path.
The first step is to get a CT Scan this week and the radiation oncology team will look it over and make a plan on what to radiate. His disease seems to be fairly diffuse and they can't radiate his entire body at one time. We all seem to think the disease is doing the most damage in the abdomen so he will mostly likely undergo radiation on his entire abdomen for 13 days at a low dose. If it works and he doesn't have really bad side effects, they will give him a week or two to recover and then start the process on his chest. The oncologists all seem to think this is a less risky option than Avastin at this point and think this is our best next course of action.
I understand that radiation is a risky procedure, but the part that scares me the most is the daily field trip to Abbott to do the procedure. Every day they will take him down to the basement and through the tunnels to Abbott, he will undergo his radiation treatment and then come back through the tunnels to his room. He is much more stable than he was the day he coded in the MRI room, but field trips still make me nervous. The doctors all assured me that he will have quite an entourage the entire time as well. Man, I hope and pray this works. I really want to bring my baby home again.
Again, we humbly ask and thank you for your prayers. We are so grateful for your enduring support and love as we continue on this journey. God Bless you all, Meghan