Battling Yet Again

      How hopeful humans can be!  I was hopeful after Jacob's last pneumonia, we'd be free from sickness for a while.  My hopes are dashed.  His lungs are full.  Again.   This evening he started bringing up coloured phlegm (not a good sign.)
  
      We're noticing all the regular symptoms; increased breathing, decreased oxygen saturation, higher heart rate, intermittent fever, secretions galore and super fussy.  His poor little brain and body.  It must be chaos inside his body.  Oh, but if he has peace in his soul, then ALL is well.  That's a daily prayer.  

      Jacob's nicely sporting his nasal prongs again.  He's sure not in agreement when we put them on, but eventually he gets used to it.  

Fighting hard.  Every breath is once again a struggle.  We need divine wisdom in how to use the medications we have in the house for such circumstances.  The meds which have been added recently is a whole new world for me, and one I'm far from confident with.  

Sleeping Beauty :)  He can look (and sound) so peaceful when asleep.  

Today I was privileged to be able to sit down with our case manager.  It was great to be able to talk with her, considering she was away for a couple weeks - a couple weeks where I truly missed her.  The Lord has blessed Paul and I far beyond what we deserve with all the excellent support we receive, regarding all aspects of Jacob's care.  I'll leave those details for another day :) 

Common Questions

     So often we are faced with questions by family and friends. 

      The first question is: 'How is Jacob doing?'  It seems like a simple question, yet most days it's quite complicated. We heard him labelled as 'stable but unpredictable.'  That sure sums it up quite nicely.   His lungs have been decent for the past week and a half (until this morning), but his brain continues to send wrong messages.  His right side can be cold, while his left side is toasty warm - odd.  He can spike fevers; we undress him, and his fever disappears.  It's all stems back to the signalling of the brain.  We are experiencing more of these abnormalities.


     Another question is: 'How are you doing?'  Honestly, many days I don't know.  It's like walking through fog, with not much sense of direction.  Some days are so grim, yet others are a pure joy.   The days of grim and darkness are often when I'm focused on the immediate surroundings, and not looking Up for strength, wisdom and contentment.  It seems like it's a constant battle. Yes, I can always say 'God has things in his control', yet to truthfully feel that and be content with that is another!  Daily grace is essential.

     'How are the other kids?' is another staple question.  Over all, kids are resilient.   It's a great blessing!  During Jacob's last serious bought the kids all handled the situation so different. Breanne thought she was watching her brother die.  Considering she is the one who holds in her emotions, it was tough to see her so upset.  Sheryl thought Jacob was sick and we'd probably bring him to the hospital, but truly didn't see the seriousness of the situation.  Caleb comes with the typical four-year-old mentality!  It was beautiful.  He kept talking about the hospital. 
       About a week after the situation, Sheryl realized the depth of the matter, and it hit her - and hard!!  Of all things, at school.  I'm so thankful for caring staff that took her (and Breanne) under their wing and stepped them through their thoughts and concerns.  Since then, there have been several conversations about Jacob.  Some make us laugh, while others bring sheer pain.  One of the funniest things I heard was Breanne saying one big advantage of having Jacob was that  she gets out of doing regular chores when she takes care of Jacob.  Even though she wants to get out of chores :) she's a huge help.  

The kids exploring in the woods.  They thought the outhouse was pretty neat.  It's beautiful to see them still be able to put their stress life behind them and truly enjoy themselves!! 

    
Another question which some ask is 'what is the prognosis?'  That leads to a bleak answer, yet one we have to face daily.  The chance of recovery is 0%.  Considering the condition is regressive, he'll never get better.  It's all a matter of how long will he plateau, and if he regresses more, will he plateau again.  It's a life of uncertainty!

     Paul and I feel Jacob was brought into our lives for a reason, so when people ask questions, we generally don't mind.  We will provide answers to the best of our ability.  The fact is there are days we just don't know how to answer the questions.        

Recent Pictures

Jacob falls asleep in the most awkward positions!  He's not normally on his stomach, but that's the only way he would sleep one day.  He's truly living up to his nickname, 'Mr Sometimes.'  

As things go on, it seems more and more meds are added :(  It's a reminder again how much our brain is responsible for.  So much for the body goes through the brain.  For Jacob, those signals are misfiring.  That explains much of his irritability.  It truly breaks a mother's heart - knowing that there's not a whole lot that can be done for all the misfiring and confusion going on his little head.

Oh, the ringlets :)  They're gone now.  He was in desperate need of a hair cut, yet I loved the ringlet look!!  Isn't the 'messy do' in now??!! :)

 Caught in the act!! The stethoscope is a hands-off tool in our house!  Caleb claimed he had a sore stomach.  I love the boy!!  He's such a good distraction (most days.)

My mom testing her stethoscope skills :)  It's extremely difficult to hear Jacob's lungs due to a lot of upper airway noises.  If we actually get to hear his lungs, it's trying to differentiate between crackles, wheezes, rubs, grinding... I have a lot to learn yet.  

For those who know my dad, this picture is for you.  I've inherited his genes when it comes to the medical world.  His stomach turns with almost anything relating to Jacob.  This was quite the day though, he thought he'd be really helpful with the 'OCG monitor' (he has no idea what the monitors or machines are called, so he makes up names for them all :)  )  My dad informed me that Jacob's levels were all good (meanwhile he wouldn't know what's normal.)  I enjoyed the entertainment!!
My mom on the other hand, is a genuine help, day and night!!  We see it through the eyes of providence, that she was given the nursing heart years ago and pursued a career in nursing.  Little did she know 40 years down the road what an asset her nursing background would be.  When Jacob was really struggling two weeks ago, she still remembered what the breathing was called that Jacob was doing.  I was more than impressed.  What a blessing she is! 
To give my dad some credit though:), we deeply value my dad's spiritual insight and ability to step us through the tough times.  He's a mentor and a real leaning post!  
   

Confirmed Genetic Testing

    The phone rang today like any other ring.  It was Dr. Sui from 'Genetics' at the hospital.  We haven't heard from her in a while.  The last time we talked she explained that once again the Ontario government didn't approve Jacob's DNA testing.  Their simple answer stated that PCH type 2 (Pontocerebellar Hypoplasia) genetic test had never been ordered in Ontario and they are no longer approving any new DNA testing.  We enquired if we could pay it ourselves and the government turned that down also (that involves a lengthy explanation, but let's just say the government made NO sense with the whole matter.)
 
    So when I picked up the phone and it was Dr. Sui, I was really puzzled as to why she was calling.  She told me that the funding was approved and Jacob's results have been returned.  What??!! We've only been wondering for 22 1/2 months what the results would show us.  I still have a rather large knot in the pit of my stomach.  Yes, Jacob does indeed have Pontocerebellar Hypoplasia Type 2.  I don't know what we ever wished for, but I still don't think I was ready to hear the conclusion of the testing.  Considering our other children had a 25% chance of also having PCH2, I'm thankful beyond belief that we have 3 healthy children.  

     In a way it makes things clearer, yet, on the other hand I didn't want to know.   That probably doesn't even make sense, but it comes with a whole mixed bag of emotions. 

    A tidbit of positive information; Jacob's lungs are clear :)  Totally unreal!!  Miracles still happen in 2013!  His breathing was much more regular today.   Hopefully this becomes the norm.  Talking with Lisa Pearlman  today, she  stated again the seriousness of this condition.  With the brain not signalling things correctly, it causes MANY abnormalities, with many of them being serious.
  

Another Day

       To many people, today was just another day.  The same old.  For us, it was indeed another day; another day in which we were able to cuddle with, care for, and love Jacob.  A precious gift indeed. 

      As a whole, his lungs are on the upswing.  Every day now they are slightly better :)  :)  

      We continue to monitor him closely for all sorts of 'odd' things.  The only large scare I had today was when his heart rate was 55 - 60 beats/minute for several minutes.  Jacob's norm is around 100 beats/minute.  He didn't look in distress, so there wasn't a whole lot I could do.  I'm unsure what the irregularity means. 

     Today our visiting nurse came.  She truly is a gem.  She knows Jacob very well, as she's the one in the community who's followed him since the first full day we were home with him (back when he was 6 weeks old.)

Not sure how, but Jacob slept through the entire dressing change.  That sure makes life MUCH easier.

      She's extremely supportive of our decisions.  She's going to also be playing a much bigger role in the days ahead, as more treatment and assessing will be done at home, as opposed to the hospital.  Our night nurses and visiting nurses know him better than any nurse in the hospital, plus we pretty much have a hospital room set up in the house.  The palliative care team is always a phone call away and they can now direct the nurses and myself in what steps should be taken. 

     We know we have a dedicated nurse when...yesterday was her day off, but she still came to the meeting here, so she could take it all in, and be on the same page as everyone else.  That takes a special person.  Again, we are provided for, beyond what we deserve.  It is so humbling how God has provided us with such fantastic support.   “O the depth of the riches both of the wisdom and knowledge of God! how unsearchable are His judgements, and His ways past finding out!” Ro 11:33

The instant the stethoscope was on him, he woke up and started crying.  That's super loud in the ears.
Good thing the nurse is a good sport :) 

      The kids are struggling with reality.  It hurts to see them hurt.  Hopefully they can find submission with the road that they are called to walk.  The last week and a half has been a real eye opener for them.  If they can keep talking about their emotions and thoughts it's much better, but for some, to talk about their emotions, can be a chore.  As parents, we need much wisdom in dealing with these matters also.

Relieved

    As we reflect on the meeting we had this afternoon here, I must admit, I'm more than slightly relieved.  The palliative care team came and assessed Jacob.  It was good to hear 'the experts' take on things.  Yes, to some degree it was as I expected - not all clear cut answers. Yet, they clarified some points.  I didn't expect Lisa to come and say 'Jacob has __ many days to live', but I was hoping she would be able to shed some light on the subject.  The consensus of the assessment was that the new developments we've seen with Jacob may begin a new chapter in his life, but, he is NOT actively dying!  Music to my ears.  God's ways are higher than our ways.   

     He has developed irregular breathing.  Oh, how he can pause for far too long!!  It's a matter of how long can he continue to breathe so irregular?!   Yet, as human eyes see, he's not actively dying.  The many MANY prayers which have gone up on High, on our behalf have been answered.  So humbling indeed.  Just because Jacob isn't classified as actively dying doesn't mean Jacob’s a healthy boy.   Yet, God has spared him to this very second.  Praise God from whom all blessings flow.  Just as I stand in amazement at God’s compassion for Jacob, I also see His compassion to Paul and me.  We've had low times - brutally low times, yet, we've been upheld also to this very second.


     Jacob has also developed periodic mild swelling in his feet and face.  Unfortunately, there was no answer for this.  Hopefully this doesn’t develop into a major problem.   Jacob continues to be an extremely difficult one to figure out. 

      As we look into the future, we are extremely uncertain what life will bring our way.  Jacob may plateau at this point.  If that is the case, the cares and demands may be greater.  He may continue to regress, and the needs will escalate from here.  Time will only tell!

     Many tough decisions have been made, and now we also pray that we will have peace with those decisions also.  We’re so thankful for a great team walking this journey with us.  Their input is invaluable! 

     We thank you all for the many words of encouragement, but above all for your prayers.  He hears the needy when they cry.

Opa Boesterd last night with Jacob.  (Jacob's great grandfather)

  
 
    

Unknown Future

   
This weekend has proven to be a continuation of an emotional and physical roller coaster. So many ups and downs. So many worries that we can't even begin to wrap our head around. So many doubts.  So many....

It's became apparent in our situation that NO-ONE knows what the future holds for us. People that have been in the palliative care field for years don't know the path that we will walk. The only thing our dear palliative care nurse practitioner did say is that she's 'worried Jacob won't rebound.' Enough said!! So tossed with tempest!! We LOVE our boy beyond what words will ever be able to express. We are tired of watching him suffer, many times praying 'Lord, take him Home.' Take him to that place filled with heavenly mansions. A place where he will have perfect vision, a place where he will breathe in perfection, a place where his hearing will be accurate, a place where he will have full understanding and a place where his body will be whole.  Truly beautiful. Yet.... how can we part with our own flesh and blood. At 22 months, he's still so young. Yet... in God's timing his life will be complete.  It feels like we've just begun to know him (notice I didn't say begun to figure him out, because we don't feel like we've ever really figured out Mr. Sometimes.)

Tomorrow the palliative care team from London are coming for a home visit. Hopefully this will clarify some questions. We know enough of their thoughts, when they tell us they are going to supply us with a 'symptom management kit.' They want us to be prepared to deal with the road ahead. From now on, we are to treat the symptoms. Clearly, this is a scary road to be travelling.  One I had never even dreamt of prior to having Jacob. 

This morning was indeed trying. I called our case manager this morning, who has ALWAYS had a listening ear. I wanted direction from her. I wanted her reassurance. After some time, I got hold of her, just to hear her sounding more than slightly under the weather!! After a short conversation, she informed me that her father had suddenly passed away, and she would be taking a leave of absence. Wow. My sounding board was gone. Then I realized that God wants Him to be our 'sounding board.' He wants us to go to Him with all our cares, worries, concerns, apprehensions and fears.  Even if we can't utter a word, He knows the heart.   He is the one who we should be seeking direction from.  He has the perfect 'listening ear.'  I have so much to learn.

Back from Emerg

    We just got back from St Thomas Emerg, to find out Jacob's pneumonia has gotten substantially worse.  He's now on antibiotics, and we're more than hoping that they have the desired result.  It looks like it's going to be a tough road ahead. 
    We truly covet your prayers.

Diagnosed

  
At our doctor’s appointment yesterday, it was confirmed by x-ray that Jacob has a viral pneumonia. Seeing that it's viral, his body fight it out naturally. This path would be slightly easier if his brain would signal things properly.  At times, his brain doesn't even signal him to cough.  He attempts to cough, but his brain turns it off part way through.  I wish we could cough for him!!  The huge concern is that the pneumonia will turn into a bacterial pneumonia which requires antibiotics. Chest physio is a big part of his care now, to keep the stuff in his lungs moving. He's also back on a ventolin puffer to help with opening his airways. He's on oxygen, because he's not keeping his oxygen level high enough.

On Monday, Jacob continually was biting/grinding/crunching his teeth and gums. It sounded awful!! He kept biting his lips and cheeks, therefore causing huge blisters. The biggest one is almost the size of a dime. The blisters have since turned into a virus (similar to canker sores, but more painful.) The doctor increased his morphine dose to combat the intense pain. The more he's in pain the more he bites down and the more he bites down, the more pain he's in, as he opens up those blisters again. It's a vicious cycle. The morphine actually works as intended, which is a great reason for genuine thankfulness.

Putting him on morphine was a step we hoped that Paul and I didn't want to consider for a long time. Yet, it helps considerably, and when we see him 'somewhat' settled, we do believe it's for his good. We continually have to go focus on our goal for Jacob,  that he's as comfortable as possible.

Paul and I are feeling thankful more than ever for praying and caring family and friends. God indeed hears and answers prayers!! Not always in the way that we want, yet according to His good will.   

We also feel so blessed to have such a great medical team beside us. Our paediatrician and palliative care nurse make a terrific team!! We currently have 5 superb night nurses!! We feel once again God's leading hand. The managers that oversee what's going on in the home are caring and compassionate beyond words.  They truly walk this path beside us, helping in any way possible.

Jacob was scheduled to go to respite today, but that has been cancelled. That's hard on us, as we've had to postpone a little family vacation, yet we are content to be close by our little man, especially during this unsettled time. Every day with him is a great blessing. We've seen this through a different set of lenses this week, as we watched helplessly, Jacob battling for every breath, and watching his levels continue to drop. Miraculously he picked up and four days later we still are able to spend time with him.

Peacefully asleep.  The 'beautiful' frog is Caleb's pride and joy.  For him to give it to Jacob, really showed us how much Caleb loves his brother, and feels for him when he's suffering.

He snuggled right into the frog :) 

Ongoing Sickness

    Jacob has been suffering for much of this week.  Suffering in a different way than we've ever saw before.  We're unsure of the meaning of it all, but one thing we know, his days are in God's hand.  We do not know what tomorrow will bring (let alone what an hour can bring), yet we are comforted to know all things are ordered by Him who reigns above.  Our prayer is that we may have submission and peace with the day(s) that lie before us. 
    We have a doctor's appointment this afternoon to hopefully shed some light on the subject.  The doctors need wisdom and direction from above also.  We pray that Dr Kerr may provide us with a solution to the ongoing situation. 
     We covet your prayers!!

God's Compassion

Last week we read a portion from Mark regarding Christ’s compassion.  Christ’s compassion was present when He fed the four thousand.  Christ is the same yesterday, today and forever.   It became so personal, reflecting on God’s compassion to us as a family as we walk this difficult road, with a more or less suffering boy.  God promises He will never leave us nor forsake us.

God’s compassion is complete, perfect, sovereign and mighty.   What a blessing to serve a Compassionate God! 

Compassion is a feeling with and for others.  It’s to love, have pity and to be merciful.   God the Father provided a spiritual and eternal deliverance for sinners by giving up his own Son to die the death of the cross.  What glorious compassion!! 

“But thou, O Lord, art a God full of compassion, and gracious, longsuffering, and plenteous in mercy and truth.” (Ps 86:15)

“Unto the upright there ariseth light in the darkness: he is gracious, and full of compassion, and righteous.” (Ps 112:4)

 “The LORD is gracious, and full of compassion; slow to anger, and of great mercy.” Ps 145:8

“But though he cause grief, yet will he have compassion according to the multitude of his mercies.” Lamentations 3:32

“But when he saw the multitudes, he was moved with compassion on them, because they fainted, and were scattered abroad, as sheep having no shepherd.” Matthew 9:36

“And Jesus went forth, and saw a great multitude, and was moved with compassion toward them, and he healed their sick.” Matthew 14:14

“And he cometh to Bethsaida; and they bring a blind man unto him, and besought him to touch him.”  Mark 8:22

“For he saith to Moses, I will have mercy on whom I will have mercy, and I will have compassion on whom I will have compassion.”  Romans 9:15

Smiles are Gone

    It seems that Jacob's lost the ability to smile :(  For the previous month, we've been watching closely for that beaming happy face again.  He gave Caleb a few quick smiles after his last repite admission.   We always cherished those smiles, and now they're gone.  Those giggles warmed our heart, and now to have them gone is heart breaking.  So often amid the difficult times, he would still smile, giving us so much joy!!!  This past week, he's been overal content, but still providing us with no smiles.  We're really hoping that it's not a sign of the brain regressing!!
Thankfully we captured a few of his last smiles.  He LOVES his Oma!! 

 Taken the last week in December.