Wants to Die

Willem continues to speak of wanting to die.  As morbid as it may seem to the reader (in fact it seems odd to me, the author, now that I look back upon it) I took the opportunity to discuss the particulars of what he wanted done with his body.  Willem stated that he did not want to be buried and wishes instead to be, as he put it, "burned."  When asked what we shall do with his ashes, he did not know.  I recommended that his ashes be spread at the "goo beach" at lake Charlevoix and he agreed that it was a good idea.  I asked if I could join him and he said yes and added that we could die together.  In the tired and depressed state I find myself of late, the notion doesn't seem all too terrible to me: to be walking hand-in-hand for eternity, with my son, on the beaches of Lake Charlevoix.  Forever catching tadpoles and frogs.

But alas...  "We both are destined to live" I tell him and add "You and I will suffer through this together and enjoy the goo beach once again as livers and lovers of life."

Erin is with Willem at the hospital -- still in PICU -- and informed me that he is doing better, physically, but stated that he did speak of funerals.  Because of this, Willem will now be getting a permanent sitter to be bedside to watch over him.

Willem did manage a ride around the 8th floor in his wheelchair today.  Which he complained about.

Staying in PICU

X-Rays this morning reveal that the right lung is worse. Doctors not sure if the x-ray is showing atelectasis (lung collapse) or inflammation. Willem will be staying in PICU.

Administering more pain meds then respiratory therapy.

PICU: 31 Jan 2012 05:30AM

Respiratory nurse and Willem's nurse took my laying down as a sign that it was time to speak loudly. Harrumph.  Respiratory nurse tried a device called an acapella that Willem used with some reluctance.  He said this too caused him pain -- pain in his chest in particular.  Respiratory nurse seems timid... Keeps asking me what we should do.  I want to say "Go get a friggin' candle and have him blow it out!?  I dunno... You're the expert!"  My take is to keep trying; go slightly past the point of comfort and to keep trying.

How did they mess this up?!

Now he's sporting a fever.  They're also administering antibiotics as a preventative measure because of the stomach contents that went into his lungs.

Vitals pretty much the same as before.

PICU: 31 Jan 2012 04:15AM

No change.  Resting. Going to try and get a little sleep myself.

PICU: 31 Jan 2012 02:15AM

Respiratory nurse woke Willem up to give him a back massage with a pneumatic vibrating massager.  Willem hated it; said it caused him great pain but he powered through it nonetheless. Like a champ.  Very tired boy -- hates this floor of the hospital the most and everyone on it as well. But... His breathing is a little better but still working for it.

I fear a possible intubation yet try to acknowledge the fear and then ignore it and enjoy the present.  I think that is what all this acceptance hubbub is all about: touch him; love him; comfort him; accept and enjoy that I have him.

BPM: 101
Ox: 98
RR: 45
BP: 104/62

Forgot to mention that he was given morphine a while before the respiratory therapy.  Or did I mention that?  Daddy getting sleepy...

PICU: 31 Jan 2012 12:15AM

Willem awakened by the respiratory nurse to massage his back and work on breathing.  Willem complained of intense pain in his back -- 'said he is at a ten on the pain scale; where ten is the highest. And that was the end of his respiratory therapy for the now.

BPM went as high as 150
Ox 96
RR  between 50 and 60
BP 104/66

Administering Loratab (like extra strength Vicoden I am being told) for pain.

Some talk of his bladder being full because he hasn't urinated since 3pm yesterday which could be the reason for the intense pain in his back and, to some extent, increased respiratory rate.

I told the nurse to get us a urinal and we'll make him pee.  He peed. He peed a good 300ml.  No change in RR yet: 50 to 58
BP 123/74

Would like to see his RR back in the 30s again.

Coughed a little bit.

PICU: 30 Jan 2012 10:15PM

Resting.  Occasional moans with no apparent correlation with changes in vitals.  Probably in a little pain. Now adding potassium as he is a little dehydrated.  Respiratory will be coming in to do cough assist and deep breathing to get his lungs to open up more.  In looking at him, I see a boy that's having a difficult time breathing.  It pains me.

Willem was supposed to have a friend from school visit him around five today but we had to call and cancel given the circumstances.  We hadn't the opportunity to tell Willem yet and while he was being wheeled up to PICU he wanted us to make sure to tell his friend that he was going to be in a different room.  He was crushed to learn that he would not be able to see his friend.  At this time I wish to thank Willem's friends who come to visit and play with him -- it means so much to him and to us as well.  Thank you for helping Willem be the boy that he is and for being a crucial part of his recovery.

PICU: 30 Jan 2012 8:15PM

Mission tonight is to get him to cough.  Giving him Albuterol but it may have made him throw up so holding off on that.  Coughing a little bit on his own.

BPM 113
Ox 95
RR 37
BP 91/44

Being given oxygen through a mask but just switched to a... I forgot what it was called... It's a mask that allows for 50% pure oxygen and mixes it with air -- 50/50.  So next step is no mask as his oxygen saturation is good.

Man what a tough kid.

Willem asked if someone could spend the night with him and I raised my hand and jumped up and down exclaiming, "Oooh! Oooh! Pick me!  Pick me!"

He did.

Score.

ACCEPTANCE: PICU after Broviac

This word, "acceptance" -- and phrases and words like acceptance -- have been pushed on me of late by more than one person and from many different angles.  Reading, talking, trying, and crying my way ever so closer to this allusive state of being and I am now being tested -- all when I think I just might give my interpretation of acceptance a try:

Willem's surgery to put in his broviac was successful but he is having trouble breathing and his oxygen saturation levels are dropping.  He is currently in post op fighting any attempt to give him oxygen...
We told the doctor to let us go back there and help Willem and they finally agreed.  We arrived to find our boy struggling.  Struggling for air and struggling against attempts to give him air.

To make a long story short... They just needed mommy and daddy in there to do some tough love. He  kept the mask on after we got in the room.  We also did some visualization techniques and worked as a team.  Willem agreed to leave his mask on for x time to get y time in breaks -- even agreed to hold the mask so I could rest my arm. My ruse.

Heart Rate (HR)150 BPM; Oxygen Saturation (Ox) 80; and Respiratory Rate (RR) at 60+ means he goes to PICU.  Docs talked of ventilating and I got a little upset and said to give it some time because he started to get better with mommy and daddy in with him.

7PM
Where he's at now:  HR 119; Ox 98; RR 35-52; BP 93/20  (all good stuff)  RR needs to drop and stay dropped or he goes on the ventilator.

Check for MUNC Then Forward

Willem is ready for his bone marrow transplant (BMT) but transplant doctors want to check Liliana's and Willem's blood for the MUNC gene mutation before proceeding. The MUNC gene mutation is where HLH has affected the GI system -- like Willem's. There are five cases where HLH has affected the GI system and they're all in Pakistan. The genetic test will be done in Cincinnati -- which is good because  they still have Lili's and Willem's blood on file.  Time frame?  Not sure yet but we've just now been informed that the gene test is priority in Cincinnati.  When (if) the results come back that Liliana does not have the MUNC mutation, Willem will begin his BMT.

We got a brief lesson in BMTs:  Day zero is the transplant day, so days before are "day minus 7" and the days after day 0 are "day plus x." (where x = is the variable for the unknown number of days to engraftment).  The hardest days for Willem will be days minus 7 to whenever engraftment (incorporation of grafted tissue into the body of the host) takes place (somewhere between 0 and 30 is a number I got of the internet).  "Toughest days for Willem will be day -7 to day +16 (ish)" -- Dr. Foley. 

There is a possibility that we may get to  take Willem home - even though he's on TPN and lipids - before transplant.  More home care.

A1 Sauce

We lay awake in our beds and we dream of infinite possibilities; we fantasize and we wish for things.  Willem's overriding wish these two days past has only been for a taste of something with flavor. Anything. He begged and he pleaded. It was a day and a half struggle but we finally got approval for a spoonful or two of A1 sauce; Willem's favorite and the closest he will get to a steak for a long time.  Pray that it is only a long time and not forever.  I fear terribly for my boy but hope is not lost.

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Thank Yous
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Thank you Scott Barcheski for the unexpected and delightful visit yesterday.
Thank you Pam and mom Weston
Thank Bill Vreeke for the awesome party last night -- a necessary diversion.

On decadron and VP 16.  Ultimately doing the transplant sooner.  IO2 receptors lower and ferritin levels still high.  HLH in the duodenum and Willem not adequately responding to the treatment. There will be a transplant conference on Monday and the consensus is that there's no reason to wait because the surgical anastomosis is healed.

Willem is very hungry and crying a lot -- exclaiming that he just wants something with flavor.  Angry that the doctors cannot seem to make up their minds and that they need to hurry up and do something.  It was a fight to get authority to allow him to have sips of water.

Fall

An attempt was made to put a feeding tube into Willem's intestines, through his stomach but the HLH has caused a new stricture which is why he had been throwing up. The last bit of hope I had for his recovery went with this news.  All we have left to hang on to now is that the bone marrow transplant actually happens and then that it works.  I am feeling numb and hopeless.

Downward

Threw up 450 ml without having had food or drink so back to the abdominal drainage bag.  Add now TPN and lipids to his tower of bags.  He will also be getting a broviac.  Again.  More bags.  More tubes. I don't know if I feel like keeping this blog up anymore...

Emesis

Throwing up a lot today.  Back to nothing by mouth.  No solids, no liquids, no anything.  I hate HLH.  If only there was a way to give my life and health to Willem.

Celebrated Too Soon

Perhaps it best to forego any celebratory postings until a day has past for there always seems to be a step back or two not long after the joy.  Willem threw up his milestone sandwich three hours after eating it.  Out through his mouth go my spirits today.  My poor dear boy.

Monumental Day!

Subway has been at the top of his list for food that he wanted to eat the very moment that he was again allowed to eat solid foods.  He ordered a 6" ham on cheese bread with mustard.  This is, quite literally, a dream come true.

All Before 10AM

There was some concern for a few moments that last night's meal wasn't going to stay down but Willem powered through.  This morning's breakfast: tomato soup with a side of ice cream.

One of the doctors came in to take out Willem's drainage tube (from the surgery) and Willem asked that he be put out for it thinking that it was going to be terribly painful.  I had Willem hold my hand and then I said, "Staring contest. Go!" He stared at me and the tube was out before he knew what was going on.  My little hero.  So cool.

Great day.  An idea was brought up to make yesterday - his first day of being able to eat without throwing up - as an annual day of celebration.  I thought it a good idea.  Sort of like a birthday.

Yay Willem!

Up

I have come to fear posting the positive days for they seem to be followed by a negative or two -- or I feel as though I am tempting the fates.  Yet I must post that I walked into Willem's room to find him about to telephone me to tell me that he was having some chicken broth and a cola.  Happy news. The nurse then informed me that they clamped his stomach drainage tube at 1:30pm and it has been clamped since and no sign of nausea all day.  I fear to say it but it appears as though everything is working and Willem is recovering from the surgery.  Willem just told me he loved me in sign language.  That was awesome.

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Thank Yous

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Thank you Paul for spending the entire day with me and helping me get that monstrocity of an application packet put together.
Thank you Steve for looking over my resumes and offering some advice.

X-Rays for Breakfast

The morning routine seems to be x-rays now because Willem's stomach fluids are not emptying into his intestines -- which was the whole point of the surgery.  It's perplexing because we watched the stomach fill up with barium the other day and then proceed as planned through the bypass. Success.  We cannot figure out why the stomach fluids are not going through the process as well. We will occasionally clamp the drainage tube in hopes of having the process work only to find that Willem will develop a fever and then, as did happen last night, vomit the stomach contents.

Very discouraged.

At the risk of oversimplifying things, or demonstrating my ignorance, I've been wondering if maybe the stomach doesn't really feel like passing anything through because it isn't substantial.  They put barium in his stomach and it went through the system... Why not have him drink or eat something and see what happens?  Heck, why not put formula directly into his stomach - like they did the barium - and see what happens.  After all, he's not had any nutrition in days.

Small Update

Willem has another fever this morning.  Pulse is averaging 133.  He's sleeping a lot.  In addition to the morphine, Willem is now getting Toradol -- essentially liquid Ibuprofen.  Watching him sleep... He's twitching a lot.

Intestintal Bypass Surgery Successful

It was a relief, to say the least, to watch the barium stop at the opening in the bypass, and kind of sit there wondering what to do, and finally decide to go through and fill the next corridor in its journey through the rest of the GI.  Willem was just xrayed again an hour after the first photos and the barium is still traveling through the GI.  X-rays will be by again in another hour to check the barium's progress.  The hope is that it completes it course through the colon (which has a smaller, seemingly less threatening, stricture).

Ultimate goal of this surgery:  eat solid food; digest solid food; and, finally, poop.

Ignorance is Bliss

Willem is doing well today.  Surgeon said that he is pleased with Willem's recovery from the surgery thus far.  Willem appears to be in good spirits and is currently watching Tom & Jerry and laughing.

I've been avoiding reading about what is involved in a bone marrow transplant (BMT) for a good reason.  I should've avoided it a little longer.  My poor little man has a long way to go yet and his recovery from this surgery is critical.

Because we need more stress: Our insurance (Tri-Care) has refused to pay for Willem's bone marrow transplant.

http://www.cumc.columbia.edu/dept/medicine/bonemarrow/bmtinfo.html

So Far So Good

Willem's foley catheter was taken out this morning much to his relief.  He was happy to pee in the "unerall" (what he calls the urinal) again.

Still no fevers and the incision appears to be healing nicely -- an indicator (I hope) of the healing that's going on on the inside.

Willem walked a lap around the ward today and is in good spirits -- a change from last night.

Post Op

Surgery went well; "...better than expected" were the words the surgeon used.  Now we wait.  The big concern is how well Willem heals after the surgery.  Willem's particular HLH is an anomaly so everything being done comes with a degree of uncertainty.  For now we take it hour by hour and day by day.

Willem is alert and watching television.  He is in a lot of pain and counting the minutes before he can press his morphine button again.

This is my first time back at the hospital since I left in September and I'm trying not to feel like he's still as sick as he was back then.  Sleeping peacefully now... Such a beautiful boy.

Surgery Day: Waiting for His Surgery Prep Room at Helen DeVos Children's Hospital

Not a care in the world and very anxious for the surgery.  He is so excited at the prospect of being able to eat solid foods again and to be rid of the bag and tube pictured in the photo.  We cannot go anywhere without the bag and tube.  Not shown here is the backpack that holds the formula and feeding tube that also has to go with him everywhere. 

Not A Care In the World and Singing

Willem was smiley, extremely talkative, and, when he didn't think we were listening, he was singing.  When asked if he was excited about today's surgery he smiled and simply said, "yes." Willem could be seen skipping to the surgery prep room after being weighed.  The boy is anxious to eat real food again and anxious to get rid of some more tubes. We are anxiously awaiting the same.

The news came to us yesterday that his third of six genetic tests came back negative for Familial HLH.  We were also informed that Willem's six year old sister will definitely be Willem's bone marrow donor.

Thank you uncle Greg for driving up here from Chicago to be with us.  We love you!

NO WAR FOR DADDY

My time away from my son has been an emotional challenge unlike any I have endured and I reached my absolute limit.  With Willem's recovery reaching a plateau, and the upcoming surgeries, I requested to be sent home to be with my son and the Army obliged. No more war for me. The downside is that our family will now be struggling financially and my stress and guilt level increase. Nothing is ever easy: Go to war and suffer emotionally to provide financially or suffer financially to be home with my son. To the reader, I imagine that the decision is an easy one.

Surgery is tomorrow (January 17) at 1:10PM. It will take 3+ hours and Willem will be hospitalized anywhere between 5 to 10 days.  This surgery, if all goes well, will finally allow Willem to eat real food. The surgery in a nutshell: Imagine the intestine as an S. The surgeon will cut a hole in the underside of the top part of the S and in the top of the middle part of the S and then connect the two holes. The upper left (side) part of the S is the damaged portion of the intestine that is not currently allowing food to pass so, after the surgery, the damaged area will be bypassed -- thus allowing solid food to continue through the system. To use the doctors' repeated statement: I am cautiously optimistic.

Still awaiting the results of Liliana's blood-work to ensure that she can indeed be the donor for Willem's bone marrow transplant. My poor babies. To think that my little six-year-old baby girl may suffer in an attempt to save the life of her older brother. And it just occurred to me that we never even asked her if she wanted to be his donor. 'Wonder if we should.

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THANK YOUs

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• Thank you to the Palmer Lake VFW 3915 Ladies Auxilary for their donation. Thank you for thinking of us and thank you for your prayers.
• Thank you to the Ada Bible Church. Thank you for thinking of us.
• Thank you Kathy, Tom, Emily, Grace and Andrew.  Thanks a latte (sorry for the pun -- I couldn't resist).
• Thank you Mrs. Otto.  Wow!
• Thank you Mrs. Meltzer
• Thank you aunt Milly
• Thank you Rick and Mary
• Thank you uncle John and aunt Cynth
• Thank you aunt Ami

Thank you everyone!  To all of the contributors, throughout this entire ordeal, you have made our lives easier and more enjoyable in one way or another.  As you can imagine, our lives have changed dramatically and the stress has been unbearable at times -- you have helped ease the suffering.  Thank you.

Some of the Meds