More hard news

Noah went to hem/onc yesterday AM for a venofer (IV iron) infusion.  Since we went in knowing he'd need the venofer, the doc didn't wait for Noah's labs to come back before starting it.  It's a great idea in theory since it minimizes our wait time and the length of the visit, but in this case it didn't turn out as well.  His infusion was nearly over before they pulled up his labs and realized that his hemoglobin had dropped very significantly and that a transfusion would have been more appropriate than the venofer.  Ugh.

The idea behind the venofer is to give Noah's body the tools to build his own red blood cells and boost his own hemoglobin, but this hasn't worked the last three times it was given - in fact, each time he had to be transfused within a couple of days of receiving the venofer.

One of the quirky aspects of hem/onc is that we never know which doctor will be in clinic that day, and yesterday was the first time that a doctor really realized that the venofer hadn't been working.  Dr. S said that he feels that due to disease progression, Noah's body is no longer able to use the iron to build his hemoglobin.  It doesn't matter that we're giving him the tools - he just can't use them.

As a result, we're going back to hem/onc on Thursday for a transfusion. (Thursday is William's 17th birthday and we hate the fact that we'll be spending the day away, but this was the only day this week we could really do it.  We'll just have to do a big family bash on Friday night.)   Noah was typed and cross-matched before we left yesterday and that will shave a little time off of a VERY long day.  Dr. S also said that given Noah's health, the fact that he's a hospice patient, etc., they are now going to view transfusions as a quality of life issue.  By Thursday he should have come up with a target hemoglin (probably in the 10.5 - 11 range), and they will transfuse Noah every time he drops below that number.  This could mean transfusing him every other week or even more often, BUT it should also buy us some energy and quality of life. 

We got a call from Dr. T, the urologist yesterday as well.  Noah's bladder issues seem to be worsening rapidly and he showed no improvement at all from our discontinuing the oxytrol patches.  Due to a lot of different factors, several of our previous treatment options have been taken off the table.  Some aren't agressive enough and others are just too risky.  We were left with 2 choices:

1. A vesicostomy, which is a surgical opening from the abdomen out to the bladder.  Urine would dribble constantly through this opening into a diaper.  There are infection risks due to that opening being constantly exposed to the wet, warm environment of the diaper.  Noah would have to be catheterized every time we needed a urine specimen.

2.  A supra pubic catheter, which is a closeable plastic tube (similar to his g-tube) which would be surgically placed from his abdomen into his bladder.  We would open the tube and drain his bladder on a schedule and could attach the tube to a bag when he was sleeping or spending a lot of time in his wheelchair.  There is a risk of infection due to the indwelling plastic, but we would be able to irrigate (wash out) his bladder with antibiotic washes.  Noah would never need to be catheterized, and he might actually be able to wear underwear.  He would not wake up soaked in the AM.  The tube would need to be changed every few weeks, but this could be done in the office and would not require hospitalization. (NOTE - I mentioned a supra pubic catheter of a different kind in my last post - that kind, also called a mitranoff, is made from bowel tissue and is just too risky for Noah.)

We've decided to go with option #2 - the supra pubic catheter.  Noah will be having his surgery October 20th, a week from Thursday.  He'll be in the hospital for a couple of days.

We have several very specific prayer requests right now:

1.  Noah is seeing his cardiologist tomorrow - please pray for clear, helpful results.
2. Please pray for safety for Noah's transfusion and for wisdom in setting his new transfusion threshold. 
3. Please pray for safe, successful surgery and for peace for him when we tell him about it.
4. Pray for grace on the part of the hospital staff.  There is an excellent chance that all of Noah's admissions will need to be in the PICU (intensive care unit) from now on due to his narcotic pain meds and the level of care he needs.  It's frustrating to think that I can do this in my living room but they might not be able to do it in a children's hospital without putting him in intensive care.  Hospice is working with the hospital to verify which unit(s) can accept Noah and to do all they can to work things out for him to be on one of his two regular units when he has his surgery and when he's admitted in the future for any reason.
5.  Please continue to pray for all of us as we process all of this.

Yesterday was a hard, hard day.  It still seems shocking somehow that this disease is continuing to destroy body systems and organs like this, and it is agonizing to see the pace picking up like this . . .

10 months ago Noah had one line coming from his broviac and was hooked up to TPN 14 hours a day.  He wasn't hooked up to anything the remaining 10 hours.  He had a flat, button-style g-tube used for medicine and tiny amounts of formula.  He could eat.  If he hurt, we gave him some motrin.  He hadn't needed blood or venofer in months.

Today he has 3 separate lines coming off of his broviac.  2 of them have large IV pumps that run around the clock and the other one is used on and off all day for IV meds.  His little g-tube was replaced with a large hanging tube connected to another bag to drain everything from his stomach.  He can't eat.  His gut is friable and oozes blood. He's on 4 prescription painkillers, 2 of which are narcotics, and we're still increasing those meds.  He's needing increasingly frequent transfusions.  His liver numbers are almost always up.  Within a few days he'll have yet another tube taking up precious real estate on his little tummy - a tube that symbolizes the failure of yet another organ.

When I stop and think about it, it takes my breath away.  He has gotten so much sicker so fast.  His condition 10 months ago was very little changed from how it had been 4 years ago - yes, his disease was progressing, but not in these massive, "lose-the-use-of-body-systems-or-organs" ways.  I found some newborn pictures of him the other day and they ripped my heart out.  I remember my all-consuming joy and gratitude at holding this seemingly perfectly healthy, beautiful, longed-for child.  I was so innocent, and so naive, and it's breaking my heart that yet another huge part of this perfectly precious child has been destroyed.  I cherished him so much and I don't have regrets, but I would give almost anything to go back and hold him just one more time with no tubes and bags and scars and pain - to just be innocent and unknowing for a moment - to enjoy him in a fully sweet moment without sorrow lurking in the shadows. 

It's true when they say that the more things change, the more they stay the same, and the only thing keeping me afloat tonight is remember what hasn't changed - the nature and goodness and sovereignty of the loving God Who hasn't changed in the last 10 months.  He isn't surprised.  His plans haven't changed, and He is still working things for His glory and our good.  He loves Noah with a greater love than my soul-wrenching, heart bursting love, and He loves me that way too.  He was there in the beginning, He's here now, and He'll never leave.  I'll be honest - sometimes, like tonight, I have to content myself with knowing these things.  I'm not feeling them.  It's hard to hear the whispers of God through the waves crashing so hard in my heart right now . . . but that's OK.  Tonight it's enough just to know He's there. 

Blessings,
Kate
P.S. THANK YOU to the 100+ of you who commented, posted, or emailed about my last post.  Many of you had never commented before, and some of your stories moved me to tears.  I never meant this blog to be a ministry and it was so encouraging and humbling to hear story after story of how God used it in your lives.  He also knew what a hard day I'd have yesterday, and that I would be blessed by that steady stream of precious words from so many of you.  NEVER underestimate the power of your words, and please know that every comment brings strength and encouragement to our family, even if we've never met.