A sweet day of rest before starting "Boot Camp"

Oliver took a day of rest yesterday to recover from his big attempt to breathe on his own, and today they started him on "Ventilator Boot Camp." 

Yesterday he actually woke up for a few hours and we got to "play" a bit.  I talked and sang to him, and showed him all his animals and toys, and they let us put up a mobile for him.  He was kicking and squirming about, but was very peaceful and not frantic as he sometimes has been when he's woken up some from sedation.  His eyes were much more open than you see in the photo - he hates the flash. 

Then he fell asleep holding his billy goat. 

It was a sweet time to get to interact with him following such a discouraging time on Monday.  It was so reassuring to me that my little guy is still in there and still has some kick in him.  It's true that we cherish the sweet times so much more right after a time of grief. 

Today he mostly slept but has done well as they started weaning the ventilator again and pushing him to exercise his lungs and diaphragm.  They make him do "sprints" of two hours on minimal pressure support, and then a rest of two hours on the full ventilator support, but with less and less help each time.  They'll build him up to longer times of "sprinting" with minimal support wach day.  "Sprinting" is a good word for it - that's probably what it is like for him even though he's just lying down!  His main doctor this week is optimistic this exercise routine will work for Oliver, and so far he seems to be responding well. 

I got to meet with one of the metabolic specialists late yesterday, and, while it was quite informative about mitochondrial diseases and how they could relate to Oliver's condition, he communicated that the preliminary test results were inconclusive (of the two markers they looked for, one was positive and one was not).  We're still waiting for the full genetics panel (about 400 genes) to come back in a month or so. 

For now he remains a puzzle, and we'll keep helping him as best we can.  Please pray that his lungs and diaphragm will strengthen.  

I was really pleased to learn that we could start working with a physical therapist while were in the hospital.  They tried to start with us today, but Oliver wanted to keep sleeping, so he just got to sit up a bit instead. 

Also, we've chosen not to do Halloween, but we do love to dress up, and my mother (who arrived yesterday, Tuesday) was smart to bring a surprise outfit for Elsa in case there were other children dressed up at the hospital.  There were lots of costumes around today, including three fairies that visited Oliver.  I don't know if Elsa was more excited about getting dressed up herself or seeing Oma (my mother!) as a fairy too!  

And fairies have to fly of course, so we took the girls for their first ride on the OHSU sky tram.  Elsa was pretty quiet and pensive the whole time we were "flying," which means she loved it.

I was content not to dress up, but enjoyed being outside for a bit, walking with my girls, and taking pleasure in Nora's cheerful demeanor and Elsa's fluttery merriment.  It was a lovely diversion after the last few days in the PICU.  (Nora was starting to get tired in the photo below.)

The leaves continue to change color - look at the difference in the two weeks we've been here!  (This may seem like nothing to y'all back east, but we have mostly evergreens here!)

We keep hearing of new prayer lists that our family has been added to, and I will attest that we can so clearly feel the support you all are giving us.  When I step back and reflect it is a miracle how stable we have been able to remain through this, how our family has been provided for in so many ways, and how Oliver has persevered through so much in spite of his limitations.  Thank you for interceding for us. 

Fiery arrows

I was reading in Ephesians today about the shield of faith, and how it helps protects us from fiery arrows sent by the enemy.  Thank God for this shield, because I feel like we had some arrows shot our way today.

They tried to take Oliver off the ventilator today but had to re-intubate him because he was not breathing effectively on his own.  They tried all the other options of breathing support before starting him on the ventilator again, but none of them were quite enough for what he needed. 

Re-intubation is not terribly uncommon, but Oliver is, so this is not a simple "we'll try again in a few days" setback.  We've known since he was born that he has low tone (muscle weakness, hypotonia) and fatigues easily.  He also doesn't recover well from situations that cause muscles to atrophy from disuse, like hospital stays, and, as we are learning, ventilators.  Being on a ventilator even for strong adults causes the diaphragm and other muscles that work in respiration to begin to atrophy in even just a few days.  All of these factors, plus his underlying pulmonary hypertension, were all playing against him being successful breathing on his own.  He had done well on the "transitional" CPAP settings on the ventilator, but we think he may have been extra worn out from that too because it required him to initiate all the breaths on his own.

Working under the idea that his diaphragm needs to be strengthened, the doctors are going to be putting him through what they call "ventilator boot camp".  Tomorrow, after he recovers from all the hard work he did today, they will begin to put him on successively lower CPAP settings for longer amounts of time, and letting him have "resting" intervals on full ventilator support.  Just like training in the gym - successive sets with heavier weights and more reps, with rest time in between.  This "boot camp" will last about a week.  So we're looking at being here a minimum of two more weeks from now. 

If "boot camp" doesn't work then we'll be discussing a tracheostomy and a home ventilator with the doctors.  This would allow him to have a variety of respiration support as needed, would allow him not to be sedated, and would let us go home if everything is stable. 

We are very much anticipating getting the preliminary results back from the metabolic tests sometime later this week.  Knowing if he does have a mitochondrial disorder, and then what type, would greatly help us know how to help him.  We'd then have an idea of whether we should expect things to get better with therapy and treatment, or worse as his muscles and organs continue to weaken. 

So it was a sad, sobering afternoon for Joel and I as the last week of progress feels to have slipped into nothing as the nitric oxcide machine was brought back in to the room to help with the return of his acute pulmonary hypertension episodes and he was placed back on the warm air "bear-hugger" blanket again help bring his body temperature back up to normal.  He had to work so hard to breathe during that short time off the ventilator that it just completely wiped him out. 

Sorry, no new photos.  Oliver looks the same except with a new tape job moustache-ed across his sweet chubby cheeks.  Little babies should have kisses on their cheeks, not medical tape. 

We've known since January that we might only get to have Oliver with us for a short time.  As King David did for his son, I will plead with God for Oliver to have a full and strong life, and like David I will choose to praise Him even when I don't understand what He's doing.  But do we ever really understand what he's doing?  So when praise Him, isn't it always in the midst of a confusing, difficult world?

Weaning off the vent on "Oliver time"

We all had hopes of Oliver being extubated off the ventilator today, but Oliver had different ideas.  Early this morning (about 4am) they switched the ventilator settings to "CPAP" which essentially are intermediate settings between full ventilator support and being off the vent.  Shortly after this he had a big desaturation, down as low as 50%, and was not able to recover on his own so they intervened and went back up to full ventilator support with low settings.  His morning chest x-ray showed more fluid than the previous day, his electrolytes are still off balanced, and he's been having trouble processing the carbon dioxide out of his system - all factors that work against him being able to breath adequately on his own. 

So, as the doctors put it during rounds this morning, this will not be a "typical" extubation procedure. 

Oliver is overall still doing well, at least compared to few days of acute illness that caused his admission here, and we're hoping that we'll be able to have him off the ventilator by Monday. 

On one hand this setback was a bit dissapointing, but we're getting used to Oliver taking his own time to stabilize, so on the other hand it makes sense that his body will need a gentler, slower transition.  Contrary to my natural inclinations regarding hospitals, for Oliver I am in favor of longer hospital stays, slower transitioning, and more monitoring because of the puzzling nature of his underlying condition, so thankfully I'm not stressed out about being here longer each time he takes a step back.  Don't get me wrong - I can't wait to get home, but I'm very happy to wait until we are as sure as possible that he's stable enough to get there.

Thank you all for your continued prayers.

Weaning off the ventilator

Yesterday, Thursday, Oliver took  "a step back" because the doctors had to go back up on some of his ventilator settings to keep him stable, but today, Friday, he had a very good day.  He tolerated a few "big steps forward" on weaning off the ventilator, hasn't had any significant desaturation or bradycardia spells, and slept all through the day.  I think his nurse even got a little bored, but I think he would agree that boring days in the PICU are good!

Tomorrow will be a big day - if all goes well the doctors plan to take Oliver off the ventilator!  He has responded well overall to the weaning process, and the doctors seem optimistic that he will do well with the extubation (vent removal) too.  I am somewhat apprehensive about it considering that his heart stopped after he had a strong gag reflex on a breathing tube previously, but I know the doctors will be prepared for that possibility and that God continues to hold our little boy. 

The doctors have started weaning Oliver off the sedatives, but we learned today that this process will take some time too.  I hadn't thought about my dear baby going through withdraw as he woke up, but he's been on opiate sedatives and pain killers for 10 days now - long enough that he's gotten used to them and will need some support and time to wean off of them.  

His x-rays still show some fluid in his lungs, and he still is "poufy" as Elsa described to me when I asked how she thought he looked.  The docs are using several means to help him diurese this remaining fluid off, so hopefully he'll look normal soon. 

Here he is tonight, with his puppy propping up his vent tubes. 

With Oliver having such an uneventful, sleepy day, I actually had a quiet, restful day.  Betsy and the girls came in the morning, and Joel and I met for dinner after his work (he's been able to do some work remotely from a company office in Portland), but otherwise I had the day to myself.  In spite of being in the PICU it was rather nice!  I'm trying to rest and sleep now expecting that Oliver will probably need lots of mommy time when he starts to wake up from sedation. 

We continue to focus on the blessings and provisions.  I was reminded while meandering through the 8th floor courtyard today that beauty can be found even in drainage pipes.    

Another good day and a bath

Oliver had another good day overall.  He's now only on "moderate" ventilator settings, and has had only a few brief episodes of low blood pressure and oxygen desaturation.  When these happened they went back up slightly on the settings, but that's typical progress - two steps forward, one step back.  He's looking a lot more normal, but still has some extra fluid that he needs to get out still.  The doctors continue to tweak all of his meds and rebalance his electrolytes as he progresses.  His morning chest x-ray showed improvement again, and the echocardiogram today showed that his pulmonary hypertension has fallen back to his "mild" baseline level. 

One unexpected step of progress was that Oliver got a bath today!  Even though he is still sedated, it must have felt good to be sat up for the first time in a week.

The nurse also had fun putting some clothes on him, which Joel and I actually didn't really like because he still has so many wires and tubes attached to him, and they'll just have to take it all off for the x-ray tomorrow morning anyway.  But it was nice of her to let him sit in a baby bouncer for a bit of a change for him. 

And at least his shirt matched the dinosaur tape around his IV.  Isn't he handsome in green?

Unfortunately the girls were still sick today, so they haven't been in to see Oliver yet, but it is so much better to have them at least in the same city as us.  We really hope they are better tomorrow to at least come visit at the hospital, even if not to Oliver's room yet. 

We've been blessed with so much help and encouragement in so many ways in the last week, thank you all so much!  We've gotten help with places to stay, food, nanny-ing for the girls, visits, toys and activities for the girls, and so many prayers.  Thank you, thank you.  We couldn't get through this without you all!

And Joel got me flowers, even though we're not allowed to take them into the PICU room.  He always knows which ones will make me smile.  Thank you, dear.

Progress report

Oliver has had a quiet, mostly un-eventful last 24 hours.  His blood pressure and parts of his regular lab work still are mildly volatile, but they are thinking that it may be due to his underlying condition and not the infection.  He has been waking up and becoming more alert, and looks for us when we talk to him.  He is still moderately sedated, but his calm wakefulness is a great sign of his improvement.  As long as he remains calm and comfortable they won't increase his sedation.  He's finally not looking quite so puffy as the diuretics have taken effect.  They have been slowly weaning his off the ventilator and nitric oxcide (for his pulmonary hypertension) and overall he is doing well with that.  Depending on which nurse you talk to he is on moderate to moderate-high ventilator settings, so he still has a ways to go to be fully weaned.  His morning chest x-rays showed a slight improvement, but again, he still has a ways to go.  The doctors continue to tweak all his meds as his body stabilizes and restabilizes with the slow improvement.  Praise God for all your continued prayers and for his gradual progression. 

The girls have been sick on and off for the last few days, but seemed improved enough this morning that Betsy (my brave and wonderful mother-in-law) loaded them in our minivan, along with more clothes for us, and began the trip to join us in Portland.  We may not let them come to the hospital yet, but we can't wait to have them with us. 

I have been so blessed to have several visits from friends, particularly some who have experience with long hospital stays with children involved (as patients and children of patients).  Y'all have been so encouraging to us and have given us some great support and advice for adjusting to life in the hospital.

Of all places to "stuck in the hospital," I am so thankful we are stuck in Portland, even if it is at the beginning of rainy season.  For example, the room service menu offers selections like grass-fed beef, goat cheese, and steamed kale!  I can get kefir in the cafeteria!  Also, at least at the children's hospital, there are outdoor, rooftop, courtyards nestled on each floor - some for playing, some are "quiet" courtyards, and all of them are full of green planters and benches.  And the views from the rooms and skywalk are filled with hills of evergreens sloping down to the river.  These are the views from Oliver's window:

I've missed my garden all year, but am happy to enjoy growing life anywhere.  Fall comes to hospital courtyards too. 

Oliver looks about the same as in the last post, but I'll get some more photos of him for the next update!

Slowly stabilizing

Oliver has been slowly stabilizing and improving last night and all through today (Saturday).  He is still heavily sedated, receiving heavy doses of pain meds, getting full support from the ventilator for breathing, and requiring a diuretic to help keep the fluids moving out of his body.  His blood pressure has been pretty stable, his heart is doing its job, and his color is so much better. 

He still is on a 1:1 ratio with a nurse, but it was encouraging to us that he was one of the last patients that the doctors visited when they made their rounds this morning.  That means that his condition is not quite as critical as it was yesterday when they visited him first!  This morning his lungs still looked to have about the same amount of cloudy fluids, but the doctors were not surprised at this and increased his diuretic to see if it would help.  They also started the slow process of trying to back off of the ventilator.  He had one major dip in his oxygen saturation this evening so they gave him the ROC (paralyzing med) again and went back up on the ventilator again, but not quite up as high as it was.  That's is how it's going to go - two steps forward, one step back.  We're praying for more steps forward than back!

So, Oliver is improving, slowly, and stabilizing, but is still critical.  The doctors are working hard to keep his medical conditions, medicines, and ventilator support all in balance to help his body fight the infection as best as it can. One doctor yesterday explained that it is very encouraging that Oliver is responding well all the treatment, but was honest with us that, in his estimate, 20% of medically complicated kids like Oliver still don't survive a severe infection like this.  We'll be in Portland for at least another week, best case scenario. 

So far no results have come back to indicate which bacteria or virus is making him sick, and we may never find out, but the doctors are suspecting some sort of virus at this point.  They explained that the bug Oliver has is not necessarily a serious or deadly virus, and Oliver is not necessarily more susceptible to getting sick and he has a good immune system, BUT because of his medical condition, when he DOES get sick, he gets REALLY sick.  In other words, the same bug may have given you or I a runny nose, but it sent Oliver to the ICU. 

Oliver still has some atypical symptoms that the doctors are puzzling over, like his inability to regulate his temperature still, and the lactate levels in his blood work.  They've started us talking to a specialist in metabolic disorders, which was interesting to me because his cardiologist has also mentioned the possibility of Oliver having metabolic disorder, specifically a mitochondrial disease, because of his sleepiness and fatigue.  It could also explain some of the abnormalities with his vision, heart, kidney, respiration, short stature, low tone, and difficulty fighting infection.  We are obviously very interested in this new possibility, especially since all the genetics tests have come back normal.  Unfortunately there is not a lot understood about mitochondrial diseases, but I'm going to do my best to not read up on them too much until we know anything for sure!  We'll get preliminary results from blood work in two weeks, and full results in 6-8 weeks. 

Joel's dad had flown to Medford to visit while Betsy was taking care of the girls, and we're so glad he could make a quick change of plans and join us for a day in Portland too. 

We are doing well, Joel and I, but it is still difficult.  I still cry as I hold his hand and soothe his forehead, but our foundation is firm.  We are sleeping, eating, taking walks to the cafe, and even enjoying time to talk and sit together.  God has blessed us with some very sweet moments as he carries us through this.

We thank you all so much for your prayers - we feel so supported and upheld, and oh, how desperately we need the upholding!  We know we are on prayer lists all over the country, and we are so thankful. 

Elsa and Nora know my favorite hymn well, and Oliver probably too for the many times I sing it to them in the nightlight glow as they fall asleep. 

How firm a foundation, ye saints of the Lord,
Is laid for your faith in His excellent Word!
What more can He say than to you He hath said,
You, who unto Jesus for refuge have fled?

Fear not, I am with thee, O be not dismayed,
For I am thy God and will still give thee aid;
I’ll strengthen and help thee, and cause thee to stand
Upheld by My righteous, omnipotent hand.

When through the deep waters I call thee to go,
The rivers of woe shall not thee overflow;
For I will be with thee, thy troubles to bless,
And sanctify to thee thy deepest distress.

When through fiery trials thy pathways shall lie,
My grace, all sufficient, shall be thy supply;
The flame shall not hurt thee; I only design
Thy dross to consume, and thy gold to refine.

The soul that on Jesus has leaned for repose,
I will not, I will not desert to its foes;
That soul, though all hell should endeavor to shake,
I’ll never, no never, no never forsake.

---

Unstable but not acute

Oliver had a good night, under sedation, and by morning had good color again.  He is pink and puffy.

He had a quiet morning.  This morning's chest x-ray showed that his lungs have gotten worse (as the doctors expected).  By midday his blood pressure kept dropping really low and they began working pretty intensely to stabilize him.  Even under sedation he was trying to move and fuss a bit (we're glad that he's a little fighter!) and they think that when he does he exacerbates his condition, so they are giving him a "paralyzing" drug, or ROC, to keep him from moving and to let his body really rest.  All of his vitals responded positively to the ROC meds.  They are waiting now for his blood pressure to normalize a bit more so they can place an arterial blood pressure probe. 

So, he is "unstable" - they are monitoring intensely and constantly tweaking his meds to support him, and he has a 1:1 nurse devoted solely to his care and monitoring (our nurse, David, is highly experienced and rarely leaves the room!), but he is not "acute" - his current condition is not immediately life threatening and he is responding well to treatment. 
We're still waiting for results from the blood, urine, respiratory, and spinal fluid cultures. 

Thank you for all your prayers and encouragement!

PICU

We knew our trip to Portland would be eventful, but not this eventful.

The surgery follow-up appointment went well - we now know how to replace Oliver's g-tube ourselves. 

The IV placement for the DMSA scan did not go well, but after eight tries and trying all three of the top teams of nurses they finally got and IV into Oliver.   It took so long that we had to reschedule our doctor appts. for the next day.

The DMSA scan went very well - you can see the gamma camera in the photo above; Oliver is the burrito bundle in the middle.  The scan showed that Oliver's right kidney has grown slightly and is functioning at full capacity.  His left kidney, however did not show up at all on the scan, meaning that it has absolutely no functioning kidney tissue.  This is good news - the doctors have agreed that we will not be removing the bad kidney because in situations like this it will atrophy, shrivel up, and eventually go away.  It is also good news because the doctors expect this means that Oliver's hypertension will go away too. 

During our time in Portland, Oliver grew increasingly cold, listless, and had greater and greater spells of turning very pale.  At first we didn't think too much of these symptoms because cold feet is a common side effect of one of Oliver's medications, he is normally a very sleepy baby (and often gets overstimulated and "shuts down" on days like a big hospital visit), and he had been poked so many times that I wouldn't have been surprised if he turned pale!  But these symptoms worsened over Tuesday night, and by the time we saw the phlebotomist team on Wednesday, they confirmed that he looked worse than when they had seen him on Tuesday and helped us get checked into the pediatrics clinic across the hall.  We were seen quickly, and the nurses hooked him up to monitor how fully his blood was saturated with oxygen.  Healthy saturation is 100% and means that your lungs and heart are working properly to get oxygen to all parts of your body. 

Oliver's saturation would dip as low as 70% during a severe spell of going pale and getting cold.  This was not good. They soon admitted him to the Pediatric ICU (PICU) and he was visited by swarms of doctors, residents, nurses, cardiologists, and others. 

I won't go into the full details, but basically they think that all of Oliver's symptoms indicate that he is fighting sort of severe infection, and because of all his medical conditions his body is having a more difficult time battling the bacteria or virus.  They are doing a host of blood, urine, and respiratory cultures to try to discover the culprit. 

In the meantime, Oliver requires a ventilator with oxygen and nitric oxcide to breathe (the nitric oxcide keeps the ventilator from worsening his pulmonary hypertension), a "bear hug" warm air blanket to maintain his body temperature, and a blood transfusion to keep the IV fluids from watering down his blood too much (his red blood count was dropping).  They are doing regular chest x-rays and echocardiograms and additional blood cultures every time he spikes a fever.  Since he is so difficult to put and IV into they've also given him a PICC line; a semi-permanent, larger IV line that taps into a larger vein than a normal IV.   He's also heavily sedated and being given pain med so he can tolerate the ventilator breathing tube and to help him rest.  (Now there are even more monitors/equipment hooked up to him that you can see in the photos.)

He looks terrible.  Bloated and pale and sick.  Even so, we are very encouraged with the excellent team of doctors and nurses that are caring for him (and for the handful of other medical personel who have taken a liking to him, like the phlebotomist who came over to his PICU room today just to give him the puppy dog you see in the photo below). 

It is also encouraging that he is behaving and responding in a manner very typical of a severely infected (or septic) child, so we can have a bit of an idea of what lies ahead.  As the infection runs its course and the antibiotics and meds kick in they expect him to get worse  before we start seeing any notable improvement.  We expect to be in the PICU until Monday, and in the hospital probably a few days more (with a strong emphasis on the words expect and probably).

We are so thankful that we were already in Portland when this happened, and that Joel's mother was already in Medford to care for the girls.  We are doing well, resting the peace that passes all understanding, remembering the God of all comfort, and meditating on II Corinthians 1:3-11.  Please keep us all in your prayers.

CDRC Developmental Assessment

Two weeks ago Oliver was seen by the Child Development and Rehabilitation Center (CDRC).  Like our cardiologist and nephrologist, they are based in Portland but thankfully have clinics in Medford.  The geneticist who saw Oliver back in July was also with this group, but this was our first time seeing them for a developmental assessment. 

Their evaluation was simultaneously disheartening and encouraging.  I guess I'm getting used to having confused and conflicting emotions.

Oliver is now 6 months old, but was born a month early so that puts his adjusted age at about 5 months.  For gross motor abilities he is developmentally comprable to a 7 week old baby.  I have known that his gross motor abilities have regressed since his hospitilizations, particularly that he doesn't lift his head near as well as he used to, but this was still difficult to hear stated officially.  The good news accompanying this evaluation is that he seems to be moving like a NORMAL 7 week old baby.  In other words, it does not appear, at least so far, that he has some condition that impairs his motor ablility; he is simply delayed and they expect that he will eventually be able to catch up.  This may make sense considering his persistent sleepiness and disinclination to be on his tummy after have his g-tube placed.  We'll start working with Early Intervention as soon as the paperwork goes through.  They'll come to our home to do therapy with him and teach me how to do it too.  We're also looking into working with a private therapist in the meantime. 

See how tangled up the emotions are?  Our son is pretty far behind BUT so far it appears to be simply a delay and not a disorder.  So there is great hope that he can catch BUT it means a lot of therapy appointments, home therapy to add to my daily routine, and even more paperwork.  But he is totaly worth it. 

It was also difficult to have the doctors confirm that he has a vision problem (more conflicting emotions: yep, one more thing to fix on the baby! - that's the dissapointed sarcasm, but it is accompanied by the wave of hope of thinking "that may be why he hasn't smiled yet!  He can't see us well!").  His extropia (opposite of cross-eyed; his eyes look apart) seems to have worsened rather than improved as he's gotten older, so we've added an appointment with the Casey Eye Institute at OHSU in Portland when we'll be there in a few weeks (yea, I just love coordinating even more appointments! / I'm so thankful we have so many experienced specialists working with our son and that we're catching so many of theses issues right away).  You can see the problem with his eyes in this recent picture:

His recent check-up with his cardiologist showed that his heart contiues to function well in spite of all the abnormalities. 

When I took him in for his bi-monthly pediatrician last week I realized I have been so preoccupied with managing Oliver's medical care that I forgot to take the girls in for their annual check-ups!  Our doctor and nurses had a good laugh with me over that today.  There'll all scheduled together now for one big visit next week.

We didn't quite miss out on summer...

 

Thanks to the warm September weekends we finally made it to go play at a lake before "summer" was over.  Since school had started already we practically had the place to ourselves!

 

 

 

Elsa did not want to leave.  Her pout was classic.

 



Maid Marian

For several days lately Elsa has refused to answer to anything but "Maid Marian."

 

 

Even poor Nora, who has finally started calling her "Essa" instead of her endearing "Tata," is now referring to her sister as "Essa Mare-ian."

What a drama queen.  I can't imagine where she gets it.