John's Journey

John's Journey John was born with an extremely rare neurogenetic disease, Type 2 Gaucher's Disease. John and his family need your prayers and support.

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Day 20. John was actually fairly stable for the first half of the night with a pulse of mid 120s, a well controlled tem...
09/17/2026

Day 20.

John was actually fairly stable for the first half of the night with a pulse of mid 120s, a well controlled temp, and stable oxygen and vent needs that were not great but not increasing.

At 3:00 am I was woken by John's nurse saying that his FiO2 was now at 85% and still getting pretty low sats. His hemoglobin dropped a little more in addition to very wheezy lung sounds that are not responding to treatment at all. He is febrile again with a temp of 101.1 and a pulse of mid 150s. And we got a lung x ray indicating some fluid that we are trying to draw down from his lungs with Lasix.

A blood transfusion has been ordered and will be started pretty soon. The goal is to force oxygen throughout his body.

John is so pale. This photo is actually a pretty good indication of his true coloring right now.

We are so worried that he is worsening and especially that we cannot find the reason yet. He is just so, so sick.



Day 19. This is a long one. The past 12 hours have been *rough* for John and I have not had time nor known where to even...
09/16/2026

Day 19.

This is a long one. The past 12 hours have been *rough* for John and I have not had time nor known where to even begin with an update. The short version is that sometime in the wee hours of the morning, John became acutely ill, and now he is very, very sick.

We have taken SO many labs the past 10 hours. John's WBC has more than doubled in the past 18 hours, now at 33.21. His CRP is very high at 3.5. Lung x ray is indicating possible pnuemonia. Blood cultures have not yet grown anything, but John coughed up mucous from his lungs and we cultured that overnight. It is already showing bacteria growth, though it is too soon to know exactly what.

John did get a milk and molasses e***a last night, which produced 1 lb 9 oz of p**p. That is nearly a liter. Unfortunately abdominal x ray shows at least another liter of stool still sitting in his intestines. So John will get another milk and molasses e***a tonight.

Blood pressures are low. Fevers are high and only temporarily responding to both ibuprofen and tylenol before popping back up.

We starting two broad spectrum IV antibiotics, cephepine and vancomycin, to address any infections that have started, until we know more from cultures and know which specific antibiotic we really need.

Until we know more, John's PICU doctor is assuming this is sepsis.

Really truly hate how swollen he is, especially his face. He doesn't even look like himself. 😭

The most concerning thing about all of this is how high John's vent and oxygen needs truly are. My feelings about the trach have changed frequently, but today I am thankful we have it, because John's respiratory status would have required intubation this morning if we hadn't had the trach already. John is taking very few breaths on his own. His chest is retracting with every breath. The vent is doing almost all the breathing work for him. John's main PICU attending told us this morning that at this point, she is *very* concerned about John's lungs and vent settings.

He has been cyanotic around his lips and nails a lot of today. At the worst of it today, he was surrounded by about 15 doctors, nurses, and RTs very suddenly trying to help him. So thankful for such wonderful people here who care about helping our baby.

John's pulmonologist is not on call, but came by earlier to evaluate and talk with us between his own clinic patients, because John got so severe that the PICU team paged him. All he recommended was to test to make sure John's fungal skin infection did not pass into the blood. He also recommended that a blood transfusion would be a wise idea to help make oxygen flow throughout the body.

His pulm also assured us that he doesn't believe this is a trach complication. He has never seen this after a trach, and he not did expect it. He does not know what this could be besides some kind of severe infection turned septic.

We are SO worried about our sweet baby. This is the most emotional hospitalization and illness we have ever walked through with John. And the 10 week timeline to go home with the trach is completely out the window. At this point it could be 3 months or even 4 months or longer depending on how this illness goes. We cannot do anything else with this trach in preparation to go home until John is past this acute illness. So we are here for the long haul with no timeline at all.

Please pray.

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5:20 pm Update: John is getting a trach change for the second day in a row because doctors now think it might be occluded. They're not sure. This is just to rule out every possibility.

10:20 pm Update: John's oxygen sats have been well controlled for a couple hours now. Our doctors learned that John needs adult vent settings, not pediatric vent settings.

His heartrate is sitting mid 130s, which is the lowest its been all day by far.

And skin coloring is looking much better than before now that we can force him to oxygenate better. Still very pale, but definitely not blue anymore.



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Day 18. John seems a little more comfortable tonight as far as pain goes. Oxycodone is still a PRN med but we've been gi...
09/15/2026

Day 18.

John seems a little more comfortable tonight as far as pain goes. Oxycodone is still a PRN med but we've been giving it as often as is safe, and that's making the most difference.

Our biggest issue right now is that John has not stooled in 6 days now, since the day before his surgery. It is causing his abdomen to be distended and slightly hardened. His heartrate is consistently pretty high. I really think this is a huge source of discomfort for him at this point.

We have been giving glycerin suppositories every 12 hours, Lactulose every 12 hours, prune juice every 4 hours, pear juice every 2 hours, LOTS of water per usual.... we are now at the point of just having asked the doctor to submit an order for an e***a, and then if that doesn't work, we'll be trying an injection of methylnaltrexone. This drug is supposed to treat constipation specifically induced by heavy opioid use.

Please pray something works soon. I really want to get him to a more comfortable place.

You can see how well his face rash is healing though!

Today is supposed to be a big day for John so I will update later below!

$2 Tuesday if you'd like to help with daily expenses and our bills at home while we are hospitalized! 🫶🏻 We anticipate we'll be here about another 9 weeks, and Philip no longer has a job to go back to when we are discharged. Please do not overwhelm us with questions about what we'll do; there is no solid plan yet. We do know that we will be getting more paid caregiving hours from our state, but we don't know exactly how many, and we don't know how that will affect income since probably 50 hours per week will be going to a nurse to help us several days a week at home.
We also know that home nurses, especially night nurses, are in severe shortage in our area right now.
And we know that John is required to have at least one caregiver awake with him 24/7 now that he has a trach.
And in the meantime while we are here, Philip and I are both required to be here to go through extensive trach education and bedside practice. Two caregivers daily here are required, and we have no one else. Even if we already had a nurse, both of us as John's parents would still need to know how to care for him well.
Please pray the Lord gives us a clear path moving forward. ❤️

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✨️ 7:15 am Update: John did give us a small stool less than an hour after writing this post last night! It was very small, but we are so thankful! We did give a mineral oil e***a at 6:30 to help this along.

✨️ 5:30 pm Update: Big day for John!

First trach change by ENT, and then Philip and I did the first trach care under RT supervision! We completed our first trach class on trach intro, trach care, and trach change. So now we are allowed to do John's daily trach cares, and his weekly trach changes!

John started clonodine today to try to prep his body for weaning off all these heavy sedation meds.

And the plan for tonight is to start weaning off the paralytic.

And he was visited by ophthalmology just to make sure his eyes weren't affected by the fungal infection on his face!

✨️ 9:45 pm Update:

John did receive his weekly ERT infusion earlier today! Forgot to mention earlier.

Unfortunately he spiked a fever of 101.3 this afternoon. We drew cultures from his IV and his port, as well as mucous cultures from what we were able to suction. Those will grow for several days. Right now his team is assuming its another of his dysautonimic fevers, but will keep a close eye on it. Thankfully it did respond very well to ibuprofen.

John is off his paralytic completely, and he is moving! Definitely trying to roll over and suck his thumb, but can't quite get the hang of it yet after paralyzed for so long. I think by tomorrow he will for sure!

John's tummy is now very distended and very hard to the touch, and tender. Whenever we go to evaluate, he cries. There is so much backed up stool. Philip and I opted to give the methylnaltrexone injection over an hour ago, and at 10:30 he will receive a milk and molasses e***a. Of course he is still also receiving both prune juice and pear juice frequently, Lactulose, and glycerin suppositories. We are adding methods rather than switching because the situation is getting severe. John is in a lot of pain from this. Tonight during rounds we even discussed an abdominal x ray and ultrasound and then manual disimpaction. :(

I did get to hold John this evening for the first time in 6 days!! ❤️



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Happy 7th birthday to John's big brother, Mark! He is kind, helpful, generous, inclusive, and very protective of his bab...
09/15/2026

Happy 7th birthday to John's big brother, Mark! He is kind, helpful, generous, inclusive, and very protective of his baby brother. We couldn't ask for a better big brother to John.

We love you Mark!!! 🩵

Day 17. John is back on his full dose of vec because of the agitation. Oxygen needs are higher than the past couple of d...
09/14/2026

Day 17.

John is back on his full dose of vec because of the agitation.

Oxygen needs are higher than the past couple of days, but John's PICU doctor is not worried about that yet since he is on a lot of meds that are known to cause respiratory suppression.

John's now on a narcan drip too because of the high doses of many sedative meds and the paralytic, especially since he's needing so many boluses of morphine and vec as well as his constant med drips.

Our baby is on enough meds to take down an adult and still fighting through it.

A new doctor came onto John's service today, so we're hoping some fresh eyes might be able to come up with something that will actually keep our John calm and comfortable and pain free.

Please keep praying for him. He is miserable. 😭



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Day 16. Today has been a little rough so far. This morning during rounds it was agreed to start Ativan in addition to al...
09/13/2026

Day 16.

Today has been a little rough so far.

This morning during rounds it was agreed to start Ativan in addition to all his other meds because the pain and agitation are still not controlled. While I was trying to sleep off a migraine though, John's team changed their minds and increased his dose of Clonazepam instead, and our nurse didn't wake me to talk about it. She did apologize for not waking me to discuss a change in plan before administering the medication, and John's doctor did come back to discuss a new plan with me and Philip when he got here, which is trialing dilaudid instead.

They also cut the dose of vecuronium in half this morning, which has made the agitation worse since John can now move a little on his own.

We did not expect the first week post op to be SO rough. Our nurses and doctors keep telling us that after Tuesday or Wednesday, this should be a lot easier.

Really hope they're right.



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Day 15. John is on Norco, fentynal, and morphine, but still the pain is not entirely controlled. We're also using ibupro...
09/12/2026

Day 15.

John is on Norco, fentynal, and morphine, but still the pain is not entirely controlled. We're also using ibuprofen though I don't think its doing anything since not even the opioids are entirely successful right now. So we've been using vecuronium as a paralytic, but John's PICU doctor really wants him off the vec due to respiratory distress. Still on precedex drip too.

So we've upped the pain meds in hopes that will keep him comfortable enough we won't have to paralyze him. He pulled at his trach early this morning and pulled out a couple of his stitches. It happened very fast. It was all our nurse could do to hold him down while she asked me to push the staff assist button on the wall, and very suddenly we met nearly 20 more nurses, doctors, and RTs who all surrounded John trying to help.

Please keep praying for pain control. John seems to hypermetabolize these strong meds at an alarming rate, and his doctor explained to me this morning he's hesitant to go up on dosing much more. We're on such high doses right now.





09/11/2026

Practicing cough assist with our new trach! And since we weaned off his paralytic, get to see those pretty green eyes! 💚


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Day 14. John's night has been peaceful so far. We finally got a really good control of the pain. He is paralyzed and sed...
09/11/2026

Day 14.

John's night has been peaceful so far. We finally got a really good control of the pain. He is paralyzed and sedated so cannot move, to keep him safe.

2 weeks down, about 8 weeks to go, if all goes well!

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12:45 pm Update: Per morning rounds with John's PICU team, we will be weaning the vecuronium bromide today, which is the paralytic. The sedatives will remain on a high dose.

1:15 pm Update: The first class that Philip and I have to take, Trach 101, has been scheduled for Tuesday afternoon! John's first trach change will be done by ENT on Tuesday morning. After our trach basics class, we will receive our binder and education checklist, and will be allowed to start practicing bedside trach skills hands on!





I did update the last post this morning, but also deserves its own post. John is out of surgery and recovering safely ba...
09/10/2026

I did update the last post this morning, but also deserves its own post.

John is out of surgery and recovering safely back in his PICU room! Sedated, but he looks good.

And look at his sweet little face! 😍

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5:10 pm Update: John is being sedated with Morphine, Fentynal, Precedex, and Vecuronium Bromide. He is still in quite a bit of pain, and waking every so often and trying to pull on his new trach. So his med doses keep getting increased because we absolutely have to keep him sedated.

He is also on a 24 hour course of Tylenol to help with pain control and its a prophylactic against infection.

Please pray for no pain! We want John to be happy and comfortable and sleep for the next few days so he can heal.





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