Ouch! Quit it! Ouch! Quit it....

Strange is the word I use to describe the previous 36 hours. Aurelia's condition steadily improves, but at the same time managing her care becomes increasingly difficult. Since last writing, Aurelia lost her breathing tube, ended all IV pain medications (minus a small dose of morphine through her g-tube), resumed normal feeds, lost her central line, avoided any fevers, and is weening off oxygen.

I should jump for joy! At the same time, this is no three month old largely devoid of personality; Aurelia is nine months now and tremendously capable of self-expression. Discomfort, impatience, pain, exhaustion, irritability, all these and more our girl now shares, vociferously, with entire pod of babies.

Let me clarify. As Aurelia came off her breathing tube she expressed joy, but suffers irritably from a terrible cough and partially collapsed left lung (not something unusual - it often occurs in little ones who are kept sedated for some time). Angrily, Aurelia fights against her high flow nasal canula, the treatment for her lung problems. Fed up as she was with the breathing tube, the canula must feel like torture - she had this big tube removed, she fought to breathe on her own, and now they are forcing even more air into her lungs.

Now that the majority of her pain medication has stopped, Aurelia's color and personality return vigorously, but with that comes increased jaw sensitivity and discomfort in her leg from her central line. I would almost trade away the smiles that arrive as her pain medication stops, especially if it meant no longer watching her twitch, shake, and moan constantly from narcotic withdrawal. For some reason watching her filled with addiction fueled agitation and ache breaks my heart near as much as seeing her sedated into a stupor.

And if any of you know an addict, you know how terribly difficult it can be to rest. Aurelia has managed only six hours of sleep in the last 36 and that came thanks to diazepam's ability to take the edge off her craving. Hopefully, the combination of getting her central line out and maximum doses of ibuprofen will give her some rest.

Of course I know that this is all preferable to where she was four days ago, but seeing her so distressed, watching those emotions play across her face, it breaks my heart in ways surgery and long term stay hasn't in the past. Here's to hope! Maybe the step down unit soon. Maybe a chance at getting home, even for a few days, before we have to return for her open heart surgery. It's possible!

Like my new blanket?

Can you spot the differences?

Before

After

Can you spot the differences? The top picture was taken yesterday and the bottom one just a while ago. What do you see as differences? :)

Get this thing outta me!!

We had another interesting evening here in Philadelphia. Unfortunately Aurelia's last IV infiltrated and we had already left for the day. Jillian, Madison, and her mother spent the night in Gettysburg, much like I did last week, caring for the cats, cleaning up from lack of use, and doing some shopping. Dr. Davies, the fellow on duty, called at midnight to tell me that unless a viable vein could be found we would have to place a central line.

Those lines enter an artery at the groin and, while more permanent than an IV, are still less i9nvasive than a PICC line. I gave my consent, knowing that the procedure itself is fairly low risk, and today she looks far more comfortable.

Medicinally, Aurelia is down to 1.5 mcg/hr of Dexmedetomine and 2mcg/hr of Fentanyl, both steps in the right direction as we move toward extubation. Our plan is still to extubate today, and her nurse, Jessica, is weening Aurelia off the ventilator. Aurelia's temperature is fine, or low grade, as she wrestles with whatever it is she has in her lungs.

I'm hopeful today. Maybe we will have this tube out of our girl's lungs and we can get down to the business of accurate and adequate pain management for her distractors. Ideally I want plastics confident enough in Aurelia's status to train Jillian and me to move her jaw and thereby release us before we have Aurelia's open heart surgery. There is a chance they will want to keep us here until then, anyway, and that frightens me terribly. One day at a time, though, so we'll see.

Telling time

You can tell time with hospital monitors. Sudden beeps, chirping machines, and intermittent cries mark milestones, victories, achievements, and setbacks alike. Nurses come and go with the changing of the guard and every so often doctors appear to mark the noon and midnight of hospital life, rounds. Parents greet the day like so many commoners swept away in tidal events beyond their control, pulling with or pushing against, the rushing daily currents.

So much is beyond your control as a common cardiac parent. Every day is toil; exhausting, unyielding trudging toward whatever end. Aurelia is a warrior amid this struggle and her care team the aristocracy, grand and minor nobles alike, but we parents are commoners. We search for whatever ways we can to better our lot and the lot of our daughter in a world largely beyond our control. Maybe it's through an observation we share with the care team, or some piece of knowledge gleaned from experience as her day to day caretakers, or even questions we ask to keep ourselves and everyone else on their toes.

We scramble around like this out of love, of course, but also to fight off fear and its potential powerlessness. This is a good and worthy struggle, but it can make you so tired. Everything slips behind an omnipresent haze, clouding conversations, thoughts, even feelings. Some days little can clear away that haze except for the passing of time, one nurse, doctor, beep, or cry, at a time.

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Today Aurelia made some progress, baby steps size, but progress nonetheless. Her labs came back mostly clean, so she's off contact separation and whatever she does have brewing in her lungs is so not concerning that folks are going to let her fight it off on her own. Her fever is not sticking - if it shoes up, it goes away again with some tylenol (and even that is not always needed) - and she continues to tolerate her jaw movements reasonably well.

The tough part about that is avoiding any jiggling or jostling of the screws while attaching the screw driver. Her jaw is still mighty sore, so attaching that to her can be the hardest part of the job. We are slowly weening her off the respiratory machine and we hope to extubate her sometime tomorrow. I'm very excited about that as Aurelia will no longer need to be restrained. Watching her flail about because her arms are tied down to the crib is heartbreaking. Still, she is so brave, and so strong, and so determined. When she's awake now it isn't to have those silent screams every time, it's to make eye contact and to furrow her brow and to blink, as if to express her will and effort to overcome this struggle. Or, more likely, she's trying to fart. She's been really gassy lately.

Speaking of which, she's only had two bowel movements in over a week. We're not worried, but man, you ever seen a 17 lb. baby unleash a breast milk diaper? I swear to you that my time in Purgatory is greatly reduced because of what we've seen, smelled, and cleaned. To that end, in an effort to be sure all of Aurelia's insides are working right we did a hypoglycemia test today, hoping to see if her retching at the end of meals is related to a drop in blood sugar. We'll keep you posted. We'll also be doing a hearing test to dot some 'i's" and cross some "t's" with our doctor. I'm inserting a picture below of her jaw apparatus, via x-ray, so you can get a clear understanding of how this all works.

Here you can see a number of things on, around, and in Aurelia. From the bottom up: You can see the wires wrapped around her sternum from her previous open heart. Just above that you will see some of the sensor wires on the outside of her skin, wrapping around her torso. Above that are two separate marks on the x-ray; to the right is the band around her pulmonary artery, restricting the blood flow to her heart. On the left, moving up her throat from that location, is the breathing tube intubated through her mouth. That brings me to her jaw. You can clearly see the mandible device attached to her jaw in two places on each side. Between those two anchor points is the incision that splits her lower jaw. As you follow the metal bracings up behind her ears, you see the two screws that emerge from her skin. As we turn those with a special screw driver, the jaw splits along the seam between the anchors, moving the lower portion further forward and bringing the tongue along with it at the same time. This will help her breathe by enlarging her airway.

And she looks like a bad-ass combination of Skeletor, Megatron, and the Terminator. My daughter is a ass kicking cybernetically enhanced warrior. Bring it on, open heart surgery! I HAVE STEEL JAWS!

Ugh

It was not a good night. After attempting to lower Aurelia's pain medication to give her a little more consciousness as we move toward removing her intubation, we had a serious setback at about 3:00 this morning. During a diaper change Aurelia used ninja reflexes to yank her sutured breathing tube out of her nose. As the team scrambled Aurelia held her own for a few minutes before crashing. Her heart rate and oxygenation plummeted and the team had to do chest compression for 3.5 minutes as they attempted to circulate a variety of medicines through her body.

Aurelia did not lose her pulse or stop breathing, but in order for the paralytic and stimulants for her heart and lung to reach the important locations the team needed to be sure her body kept its blood and oxygen moving. Once paralyzed they picked her up to a standing position and intubated her through her mouth. Things became more complicated when she did not come out of her medicated paralysis the way everyone hoped, so we stopped all pain medication, using the pain to stimulate her body into moving again. As of now, we are back at 1/2 the dose of pain medications we were using yesterday.

Of course this morning Aurelia lost her remaining IV and needed to get a new one in her right leg. She also has a fever again and we drew labs and sent off some of her phlegm to be cultured. There is some thought that an infection could be causing Aurelia's difficulty breathing without assistance. I personally believe this is a reflection of her typical difficulty coming off oxygen. When she had pneumonia this spring we needed to keep her on 1/32 a L of oxygen for a week just to keep her airway open. That's nothing - it's like a whisp of air that barely does anything. Aurelia has always taken a tougher track when it comes to breathing on her own. Another thought could be swelling from her adenoidectomy. We'll see. Either way, it doesn't look like we will be removing her breathing tube tomorrow.  I hope only for a good response from the labs and culture so that we can just worry about getting this tube out of her throat.

She's got a bath now, new linens, and she finally had a bowel movement, so here is to a day of no problems or troubles.

Here's our girl after her rough night. You can see the tube now taped down into her mouth as opposed to her nose, you can see her right arm restraint, keeping her from yanking at the tube, her new IV line behind the brace on her right leg, and a fresh diaper. 

I'm burning up in here!

Today has been a relatively quiet day. Aurelia went through the night with only a few hiccups, trying to turn over a few times, needing a pain management rescue now and then, and otherwise attempting to wiggle free of her intubation. Truly, though, she's doing relatively well. She's a wonderful fighter determined to beat all that comes her way. She inspires Jillian and me to keep faith and strong together.

Plastics came by this morning and moved her jaw two millimeters. Aurelia took it on the chin like a champ (da da dun, ching!) even after only 30 minutes lead in time with a pain med treatment. She did so well the plastics folks are intending to come back tonight for another 2 millimeter extension.

The day itself has been rather uneventful which oddly keeps me on edge more than a crisis would. Aurelia had some mild alterations in her pain medications to keep her sedated and from turning over (which she successfully completed early this a.m.) and subsequently aggravating her jaw. The IV she lost yesterday in her foot has yet to be replaced because they taped down and isolated the remaining IV in her left arm with a brace. We hope her veins are repairing themselves in case we need to drop a second line in for medicine administration should this last IV infiltrate.

Aside from that small adjustment to her pain medication, our daughter continues to gain strength. Her oxygen saturation levels are good, no longer suffering from momentary desaturation and she appears to be bouncing back well. We have a few concerns, such as a stubbornly resistant slight temperature, a fairly good amount of phlegm coming up from her lungs via suction, and a resulting junky sound over the stethoscope. The attending isn't terribly concerned; fevers are common after surgery and broken bones, and Aurelia's labs yesterday came back great. Similarly, if there is an infection there have been no substantial changes to Aurelia's vitals, meaning she is resisting the bacteria/virus well on her own. If there is a change in any vitals then we'll culture some of the phlegm from her lungs and draw more labs.

We are hoping for some more good news soon, however, particularly regarding her tracheomalacia. ENT did not mark malacia as present during the scope, so we don't know for sure, but we can hope that our little warrior has outgrown this airway debilitating problem. On a scale of 1 - 4, with 4 being, "HOLY CRAP, CALL IN THE REINFORCEMENTS," Aurelia's throat is listed as a 1, or a non-critical airway. That thrills us, since six months ago she'd have airway collapses from getting too worked up! We look forward to final confirmation from ENT, but we think the news will be good. We hope, anyway.

So that's all we have for now from CHOP. Maybe more later, if not, definitely more tomorrow.

Here's our girl this morning, resting after having her jaw advanced. Please no comments about her thighs. She's very sensitive about them. ;)

Who's Who

Our time in the CICU has been full of memories, good and stressful, as we see old faces and remember previous pod-mates during our past stays. Our attending is Dr. Naim, plastics is led by Dr. Taylor, Dr. Rizzi worked on her from ENT, and even her cardiac anesthesiologist has been someone we know from previous operations. All these people help Jillian and me feel at home here since everyone has a history treating Aurelia.

Dr. Naim in particular is delightful; she cared for Aurelia during our daughter's first few weeks of life and has remarkable background knowledge about our little one's needs. She so adores Aurelia that she requested her as a patient upon our admittance and routinely comes crib side just to 'get some love' from our daughter, who smiles and asks for hugs from all who come near. Dr. Taylor continues to impress us with his knowledge and affable nature, and Dr. Rizzi's bedside manner always keeps us at ease.

These are the miracle makers at CHOP. They have made this entire task seem remarkably less daunting, especially since we are looking at three very difficult months - three weeks of pain inducing jaw advancement (what is in essence daily resetting of a broken jaw, combined with additional bone growth), the subsequent final healing of her jaw and neck, open heart surgery, healing, and hopeful improvement.

Today Aurelia is much the same as yesterday. Three times she's tried to roll over (not an easy task with your breathing tube shoved down your throat and sutured to your nose) and other than that we need to keep the status quo - sedated and comfortable, not too riled up, work her feeds back up to normal amount, decrease her breast milk fortification while she's not moving around, watch for infection, and monitor her lungs and jaw.

Today's x-ray came back looking good, no signs of anything unexpected, her swelling is reasonable, and it appears we will leave her intubated throughout the weekend. Even though Aurelia is not listed as a critical airway, plastics is hesitant to extubate our girl over the weekend when fewer staff is on duty at the hospital. We won't start moving her jaw today, but we will probably start tomorrow with 2 mm increments. This is an increase from 1 mm, but Dr. Taylor believes Aurelia can handle the increase. The plan is to move her jaw and keep x-raying her until we are comfortable with tongue placement inside Aurelia's mouth, relevant to the back of her throat. I'm terribly scared of the pain this might cause Aurelia. I think I fear this process more than anything else because it will last so long; even heart surgery's pain lasts roughly four days before it can be managed by tylenol; not this....this is something that could be hurting Aurelia for weeks on end.

The ear tubes went in without problems yesterday as well, and we are putting drops in her ears every now and then to prevent infection. She's not having any trouble with those drops because, well, she's unconscious. The only other thing we are waiting to see is the progress on her trachea malacia. Hopefully there has been improvement, but we are unsure of just what's going on with that as of this point and time. Our care team is going to get the report from ENT and then we will pass that along as we find the information.

Here is our baby girl just after surgery. You can see her breathing tube up on that contraption to prevent her from having anything on her sore and swollen face.

Here you can really see the screws we'll twist to move Aurelia's jaw

What a whirlwind

So. Here we are. I wonder why it is this blog only ever sees attention when I have difficult things to post? We came here Sunday night from Connecticut (after visiting parents on both sides) to participate in a sleep study. We wanted to see if Aurelia had sleep apnea. The test revealed severe apnea, to the point of her oxygen saturation dropping thirty to forty points into the 50s. The results astounded us, making me question my ability to care for Aurelia at home; how could I have overlooked something this dangerous? We gave her oxygen, measured her saturation rates and even monitored her O2 levels daily, so how did we miss sleep apnea this bad?

Little did we know, but babies with terrible apnea often learn to position their bodies optimally for improved breathing. Aurelia's preferred sleeping position was on her side, 3/4 turned, back arched, head back. She subconsciously moved her tongue forward in her palette and minimized the impact of enlarged adenoids every night. During the sleep test, wrapped as she was and forced to lay flat on her back, she sounded like a freight train rumbling on decrepit tracks, squealing under heavy load.

Since 3:00 a.m. that morning we've been reacting to constantly changing circumstances, expectations, assistance, and fears. Monday came and went without any surgery and only the beginnings of a plan to treat Aurelia and Tuesday brought more frustration as plastics, ENT, and cardiology attempted to develop a care plan for our little girl. We did our best as a family to stick together though we all felt completely helpless and unable to control any part of the situation. Jillian and I tried to get organized for what could be a multi-week stay at CHOP, and Madison persevered valiantly, trying to have fun and understand why her sister was so sick and we couldn't go home to see our cats, Ulysses and Shiloh.

Frantically, we canceled therapy and doctor's appointments for this week, made arrangements for friends to care for our cats, secured lodging at the Ronald McDonald House, and convinced Jillian's parents to come down for a few days during and after Aurelia's surgery. We completed these tasks amid ever changing plans for Aurelia. Doctors informed us that Aurelia's care could require weeks at the hospital, that we'd be having one surgery, or two surgeries, maybe repairing her cleft palette, maybe not, maybe looking at a tracheotomy, maybe removing her tonsils, maybe doing nothing, maybe moving her jaw. What registered clearly for us was that until these breathing treatments were resolved, Aurelia would not have her next heart surgery in July.

We trust the care teams at CHOP with our daughter's life, so really we knew that our preparations had to be for the most severe scenario - a two to three week recovery from multiple surgeries leading straight into her next open heart surgery in mid-July, with us getting home sometime right around the beginning of August. I cried, cursing how a stupid sleep test could turn into more than a month away from home. I fumed, infuriated that my family was thrown into tumult with little I could do to restore balance and order.

To make Aurelia's (and Jillian's) time in the cardiac intensive care unit somewhat easier - remember that these rooms are four pods a piece, with limited family members allowed bedside by each patient, I left Philadelphia with Madison Tuesday. To her credit, Madison rallied her spirits here wonderfully, but she is just a bit too loud for promoting appropriate care in an intensive care unit. At home I could both occupy Madison and take care of some loose ends at the house. Jillian remained with Aurelia, focusing her care entirely on our little one as surgery approached.

We went into surgery this morning with a plan in place. Aurelia would have tubes inserted into her ears, her throat and bronchial tubes scoped, her vocal chord paresis examined, her enlarged adenoids trimmed down, and her mandible advanced. The surgery would involve both ENT and Plastics, and last for several hours. Aurelia has come through that surgery wonderfully and now recovers. In future posts, I will talk more about the procedures and some of the people working on Aurelia during this most recent stay here at CHOP.