Laughing, Weeping, Living

Life happens. You laugh about it or cry about it, sometimes both.

Terminal

Yesterday Agnes took a turn for the worse. Her overall condition has been described to us as “critical stable.” And, “fragile.” And, “terminal.”

We have heard the words, “there is nothing more we can do except help her to be comfortable.”

Agnes is comfortable right now, and she is stable within her very fragile balance, but it is also clear that her body is failing.

After all this runaround about her shunt, it turned out not to be the deciding factor. Her ongoing trouble with pulmonary hypertension is what now her “terminal” condition.

In a nutshell what is happening is the pressure in the blood vessels going to her lungs is so high, that the right side of the heart has to work extra hard to move blood. The right side is enlarged, to the point where the left side is being compressed. Agnes’ heart isn’t able to keep up and so blood is backing up in her veins, unable to get to the heart to be re-oxygenated and sent through the arteries to her her body. Blood is backing up and with nowhere to go, fluid from her vascular system is seeping out into her tissues. The slowdown in circulation is affecting her organ functions. There is not enough blood flow to her liver, kidneys, and intestines. She is not absorbing nutrition from food, or her medicines. Her lungs are very stiff because of the inadequate blood flow so her ventilation needs a lot of support.

They could try to fix her shunt, but with organs in the process of failing what good would an operational shunt do for long term? The shunt is small potatoes. The biggest issue now is that the problems are affecting nearly every system in Agnes’ body.

She is dying.

There is always time for a miracle, and we haven’t given up hope for one. We are also talking with palliative care about Agnes’ end of life options and what we feel like we could or should do. We are always striving to make the best choices for Agnes. Right now she is behaving so we have some time to think and pray. There really is no telling how much longer Agnes will hang on. She is the boss lady. In any case it does not appear at this time that Agnes’ death is imminent–at least not in the next day or two. So that’s a relief.

Fr. Sal did allow her to receive Eucharist last night, in her own way. She got to lick the Body of Christ and I consumed the rest of it. I know she doesn’t need that sacrament because she is a baby and pure and sinless, but I wanted her to have it.

I do plan to write a beautiful post about what I’ve learned from Agnes, about the meaning of suffering and the power of prayer and all that stuff. When I feel like I can get through it! Hopefully I will be able to manage that soon.

Thank you all for your continued prayers.

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Agnes’ Tricky Shunt Surgery: UPDATE

Agnes’ shunt surgery yesterday afternoon was successful. The surgeons were able to place a V-A shunt on Agnes’ left side without a problem. This morning they sent Agnes to do a head CT scan just to check up and everything looks like it is working.

Of course, the big unknown factor is Agnes’ pulmonary hypertension and whether the pressure in her heart will prevent the shunt from draining properly. This may take a while to develop fully, but things appear to be working at this time. My feeling was that if the shunt didn’t fail right away, we have a pretty good chance that it will continue to work. Agnes has been more awake today and she got mad a few times, and the shunt still appears to work, so I  am encouraged.

In other good news, Agnes got back on her home ventilator today, her tube feeding is back to her normal rate and schedule, all her home medications are going without any problems, she is tolerating routine care like baths, diaper changes, and trach care. Her incision sites still look gross of course, but they are healing according to expectations. Her belly is soft again, though still a bit more distended than what her normal should be. No one is concerned because that distention she had was more than likely shunt-related, and now that issue is resolved. It will take a little time for her fluids to balance out again with all the recent changes in shunts and other drains.

To top off the surprising news, we were told today that Agnes will move out of the PICU and up to the regular floor for trach and vent dependent children. This is mostly because the PICU is full to capacity and they need Agnes’ room for an incoming, critically ill child, but it is all because Agnes continues to improve and the PICU isn’t doing anything for her that she can’t get on the regular floor.

And finally, word on the street is that Agnes will come home next week. Like, early next week. Like, if she continues to do well and the shunt appears functional, and all the home care gets organized, she could be home for the New Year. Whoa.

We were expecting Agnes to have a Nissen wrap surgery this admission, as well as an I.V. port installation, but those two things now seem to be optional at this point. The intensive care doctor is convinced that Agnes’ recent gastro- problems were a result of the extra fluid in her abdomen, and now she is tolerating food well, and everything seems to be normal in that area. So, a Nissen is not indicated. As for the I.V. port, that may be something for Agnes in the future, but it is not emergent and can definitely be scheduled if and when the need arises. Plus, the neurosurgeon wants Agnes to have a chance to totally recover and heal from the shunt surgery, so we’re looking at weeks at least if we decide she needs the other stuff sooner rather than later.

So.

Big news.

We are shocked still, and we can’t even feel excited or happy because this is so unexpected. I am convinced there was some miracle here. The story changed way too quickly to be entirely medical. Someone out there must be a really fervent pray-er. Or some saint intercessor is very powerful. I guess we’ll never know the agency of this miracle. God is good.

Thank you all for your prayers. Obviously they are working!

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Back For More

Today we had a big Family Meeting that included Jeremy and me, many people from the Palliative Care team, the attending PICU doctor and a PICU Nurse Practitioner, and the neurosurgeon and his Physicians Assistant. It was a full conference room.

We talked about a few major items.

Smiling in her sleep.

Smiling in her sleep.

1. Agnes’ shunt. This is the determining factor for her, going forward. It will be what limits her progress and dictates what further steps need to be taken. Tomorrow (Tuesday) at 12:00 noon, the neurosurgeon will replace Agnes’ current shunt with a V-A shunt, which will drain Cerebral Spinal Fluid directly into the right atrium of her heart. This type of shunt is already not a super good choice, and in Agnes’ case it could fail right from the get-go, because Agnes has increased pressure in the right side of her heart due to pulmonary hypertension. The pressure difference may not be great enough to allow the shunt to drain properly. If this turns out to be the case, we could know within a few hours to a day that the shunt will not work. If the pressures are kind of borderline, it might take a longer period to know whether the shunt will work or not. Or the shunt may work indefinitely. The neurosurgeon could not make a prediction. My personal feeling is, if the shunt does not immediately prove to fail, I think it has a pretty good chance of working out long term. That is not based on any medical facts, just my own gut feeling.

2. What happens if the V-A shunt does fail. There are two more obvious solutions for shunt placement. When I say “obvious,” I mean for a neurosurgeon. No one else has heard of them and there are a list of reasons why. The first alternate option is to drain the shunt to the gallbladder. The final option is to put the shunt in a major vein in the head. The gallbladder option may work for Agnes if needed, but it has some of the same concerns as the V-A shunt, infection risk and inability to place extra catheter. The brain vein option is not really available because as Agnes’ neurosurgeon put it, if the shunt doesn’t work in a major vein in her chest, what makes us think it will work in a major vein in her head? If those shunt options are exhausted, there is really no where else to go and we would be at the end of the line.

Her smiles are so sweet!

Her smiles are so sweet!

3. How close are we to the end of the line. As I said before, it really is dependent on the shunt. Agnes does not have any terminal condition at this point. Her respiratory failure and pulmonary hypertension are being managed with significant yet not extreme measures. There is no reason to think at this point that her conditions–apart from the shunt–cannot continue to be managed. There are a few other interventions that are possibly in Agnes’ future, such as a Fundoplication (Nissen) wrap to prevent reflux and a permanent I.V. port to facilitate access for medicine and blood draws. Those options will be open for discussion once Agnes has a chance to recover from the V-A shunt surgery. In the meantime, Jeremy and I have made it clear to all the doctors that all available methods are to be used in preserving Agnes’ life. We can revisit the question later if we start to feel like things are being done to Agnes rather than for her.

The meeting was good to have, even if no new information was uncovered, just to get all the teams on the same page. And now they all know they are supposed to do anything if Agnes has a crisis. I feel good leaving things at that point for now.

For a prayer request, could you please pray that the V-A shunt works? That seems to be the cutoff between not-extreme and extreme care. Mary, Mother of God, pray for us. Saint Agnes, pray for us. Saint Maria Goretti, pray for us.

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The Little Blue Pill, and other Agnes updates

Agnes continues to hang out in the Pediatric ICU at Akron Children’s Hospital. She seems to be very touchy and possibly in some pain; she fusses and starts to breathe really fast and her heart rate gets much faster anytime anyone does anything, however minor. She is receiving doses of morphine, versed (a sedative), and tylenol as she needs, which seems to be fairly often. Last night the PICU docs attempted to switch Agnes over to CPAP plus pressure support, and then to CPAP, but she didn’t like it and her lungs actually looked worse when they checked with an X-Ray this morning. She has lost some ground that now must be regained.

Meanwhile, we are still waiting for all the lab work and various cultures to produce results. So far, the only culture to show growth is the trach culture, but that is not unusual for kids with trachs and the doctors don’t think that is the source of her infection. The working diagnosis is shunt-related. I guess the shunt was malfunctioning slightly, which caused Agnes to vomit. Her neurosurgeon replaced part of the shunt in the OR when he externalized it. It is still draining to a bag outside her body while we wait for the Cerebral-Spinal Fluid (CSF) culture to show results. They do suspect the shunt is infected because the preliminary culture of the CSF showed elevated white blood cell counts there. We won’t know for sure until tomorrow.

Also a cardiologist saw Agnes and another echocardiogram was done. It showed significant pulmonary hypertension, worse than Agnes had before. So the cardiologist decided to start giving Agnes Sildenafil. This is the medicine used off-label to treat pulmonary hypertension in children. The FDA has recommended that it not be used in this manner because one study found that a few children died. The PICU doc I saw today explained that it is generally accepted that these children in the study died from their diseases, independent of the medicine. So, Sildenafil is used around the country to treat children with pulmonary hypertension. Sildenafil is also known as Viagra.

I wish I didn’t know that.

Anyway, I am glad Agnes is getting this medicine; I think it will help her. The risk of death for her will not be significantly increased by using this drug. The doctor told me that she has a very high risk of sudden death anyway, so…yeah.

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So Far So Good…

Today Agnes moved off the ventilator and off the CPAP and is now breathing totally on her own! Yay! She is getting oxygen just like she did at home, with a mist collar just like at home. She is now back to her “baseline” which is an extremely relief. She does get an inhaled bronchodilator medicine which she did not have before, but other than that her medicines are the same, too. I am so proud of her!

The results of the echocardiogram show that Agnes’ pulmonary hypertension is no worse than it was before, and since she appears to not need ventilator support, the doctors have decided to not use the experimental medicine. That’s fine, I guess, but I am worried what Agnes will do when she gets mad and starts to desat like before. She didn’t really while I was there today, but the nurses did nothing more than diaper changes. I’ll be interested to hear how she does with a bath and trach care and whatever else they try tonight. If Agnes desats a lot, they may put her back on the CPAP with or without pressure support, I don’t know. I seem to have misunderstood the debate about the pulmonary hypertension medicine. I thought that if the echo didn’t look better, they would consider the medicine. I thought it was maybe about the medicine or continued vent support. But what it now looks like is, the echo looks no worse, and Agnes is currently doing fine without the vent, so no medicine. It’s about Agnes needing no help versus needing the medicine and continued vent support.

So, we’ll see what happens in the next 24 hours. If Agnes keeps doing fine, she will move out of the PICU and back up to the floor, and she will be discharged at some point from the floor. If she keeps doing fine for the next day or two, I’m certain they will let us come home very soon since we won’t have to learn to use a ventilator and do a 24-hour care test. Agnes does need to get her feeds back to bolus rather than a continuous drip; that will take a day, maybe two days. And we will have to wait for the at-home nursing to get sorted, though hopefully we can have our previous nurses back. Agnes was approved for up to 12 hours of nursing a day, which will be a huge help! Thank you, Medicaid home care waiver! But other than the nursing, I don’t think we will have to wait for anything else before we come home.

Dare I look at the light at the end of the tunnel? Yes! I’m looking at it!

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