A Farewell to Arms (or the IVs in them, anyway)

Ford's pic line was pulled out this afternoon after it had steadily started to leak over the last few days. Although he wasn't completely weaned off the two medications he was still getting intravenously, the risk of infection was too great to wait. So they discontinued them and out it came. While his long-term fate still sits in the 'it depends on him' category, delivered daily by shrugging, erudite neonatologists, we are happy to see it go. As there is now only one pump left, which supplies him with increasing amounts of breast milk, they've attached it to his bed and wheeled the IV pole away.

The IV pole was a leaning tower of terror, a Borg(es)ian (could be both the writer as well as the unstoppable race of cyborgs for whom resistance was so futile) tree of bubbling, blinking, dinging, glowing, and occasionally dripping "life". Its absence has changed the lay of the land (which we can finally see) as it no longer shields us from the others in our row. Its presence as identity affirming flagstaff is gone and we are left needing to reconsider Ford's aboriginal name: "One who takes much Midazolam." We almost feel naked without it. While Ford, who was basically naked with it, can now finally get dressed.

Unbundled after the event he promptly assumed a "Jesus Christ pose", savouring the languor of arms outstretched for the first time since birth. Three cheers!

one month old and the pump room tales

Ford was one month old on Friday! And as of today, Sunday, is still rocking the CPAP. His lactates are slowly climbing, but they're not nearing a danger zone yet, so hopefully they'll plateau or inch back down. There is still a chance he'd have to be intubated again, though today they started the cpap wean, changing the PEEP from 10 to 8, so that's a step in the right direction. Otherwise his oxygen needs fluctuate from 21% (room air) to 28% and his breathing rate from 40s-80s.



We've noticed that Ford often sleeps with one eye cracked open, which gives him a pretty dopey look (tres adorable). Sometimes that eye rolls up so you just see the white, which is a bit more demonic than dopey. Not sure it comes across in this picture - our one eyed monster as I like to call him:





Here he is with both blues open, and loving his soother:



Another good step this week was getting his stitches out. Also, now that only one of his limbs has a tube in it (the pic line), he's able to be bundled up which he quite likes:

AND he's up to full feeds (20ml/hr)! Phew! It's been a good few days for Ford. In the next couple days they'll try moving the end of the feeding tube from the intestines (nasaljejunal or "NJ") up into the stomach (nasalgastric or "NG") and continue to wean the CPAP. They're also weaning the midazolam which will be down to zero this week.

In the first week we were here, I asked Dr. Joynt how many other "heart babies" were in the NICU. She couldn't tell me (it's all very confidential you know) but asked if I'd been in the pump room yet. I hadn't, and she said I'd likely get all the babies' stories in there from the moms.

And it's true. The pump room is a quiet place with soft lighting and the constant whir of the "Medela Symphony" breast pumps. Often there's a mom crying - it's not easy to cry in the open of the NICU, the beds being close together with always a handful of other parents and nurses nearby to witness your meltdown. In the pump room, there's only ever 3 or 4 moms in earshot, each with a set of sympathetic ears.

Sitting next to Moms in there is where I get the scoop on the other babies and it turns out there's quite a few with heart defects. It also turns out, with all of Ford's progress and set backs, we've been pretty lucky so far. Lily has HLHS, like Ford. But she also had a few other complications and isn't going to survive at all. Caleb's heart stopped a few hours after his norwood procedure and he's had two other surgeries since. He then developed a blood infection. It looks like that might be clearing up, but in the meantime his heart function isn't going to hold and he's being put on the transplant list. During Cameron's surgery for pulmonary atresia, her heart was punctured (a one in a million chance apparently). A second surgery was unsuccesful in repairing that and she was put on the transplant list. But then she also got an infection, and died. Joseph got a clot after his heart surgery and his head ballooned. Last I saw his mom he was in the OR for that, and I haven't had an update since, though I know they're still in the PICU. After a month in the hospital doctors still don't know why Olivia isn't able to breathe on her own but they're narrowing it down to a rare mitochondrial disease... and so on... it's heartbreaking.

So we feel lucky that Ford is doing as well as he is and cry for the babies that aren't.

Third time's a charm?

Ford is down to only one IV now, although it's his pic line, so it's like an IV on steroids. He's only getting two medications via infusion - Milrinone and Midazolam, and a whole lot of food (relatively speaking). He's still on a host of other medications that they are administering at certain times throughout the day. I've been meaning to write a comprehensive list to post up here, for anyone who has the insight/interest to figure out exctly how they are keeping our little guy running (I know there are some pharmacists in the family, although I'm not sure if you read the blog).

As far as food goes, he's up to 12ml of breast milk an hour (increases by 1 ml every 8 hours), which is the highest it's gotten to so far. But everytime he throws up they stop feeding him and restart him back at 1ml/hr. Sounds like punishment to me, to get your food taken away everytime you misbehave, but apparently it's for his own good (classic disciplinary parenting tactics). So far it's been advancing in fits and starts and we are pleased he is doing so much better with it now, despite hearing each morning that he is loosing weight (down from 4.2kg to 3.8kg).

Update on the diaphragm is that it's still partially paralyzed. While the usual course is to wait it out for at least 6 weeks for any signs of self recovery, in his case (in the case of HLHS kids) they can't afford to wait that long. I'm not yet sure why. So this morning they delivered him an ultimatum: cope with extubation or go in for surgery.

This afternoon was exciting and stressful and sad and overwhelming and far too busy, but a lot has changed since this morning and some wonderful milestones where reached. Since the plan was to extubate him for the third time today, they had been carefully weening him off the vent to the point where he was basically breathing on his own. And then they waited for several hours, as opposed to the last two times where they quickly weened and then pulled the plug. Funny thing was, as Christa was rocking him in her arms he decided to puke on her (milestone #1 - a benign puke on mommy, so he still gets his food) and then start making his awesome Exorcist noises (wheezy grunting). Apparently he was starting to breathe around the intubation, which I gather was kind of startling for everyone who witnessed it - I did not. So they decided that he'd made the call himself this time and promptly extubated him right there in Christa's lap.

In the past he's fought like mad against it, but this time he's doing really well. With a minimal amount of fuss he was put onto CPAP and comfortably put to rest in his bed. He's been sleeping for the last several hours and they have managed to ween him back to room air already (when they switched it over they upped his oxygen to 30%). Last time they extubated to CPAP he required upwards of 60% oxygen almost constantly, which they were unable to ween - this gave rise their observations that he was 'hanging on by his fingernails'. In addition his respiratory rate has stayed in the 30s - 60s, which is good for him (it was up in the 70s-80s last time), and his blood gases looked unstressed.

When I left to come and write this he was just waking up and despite everything he'd been through he seemed genuinely happy. He was yawning alot, had a bout of hiccups, and was really getting his rocks off on his soother. He was clear eyed and calm and that's certainly milestone #2 for today. He wasn't comfotable at all the last time they extubated him and so they basically sedated him the whole time. That he seems in good spirits is pretty exciting as we are desperatly wanting to avoid another surgery. So please send him all your good breathing thoughts over the next few days as they are pretty critical for him. We are not really going to celebrate this until they wheel the ventilator out of the room. He could loose the fight again at any point.

some recent photos

the two times they've extubated ford, and when they try again in the future, they put him on "cpap", which looks like this:
the little yellow hat with strings keeps the 1" prongs in place in his nostrils. he hates the prongs.

here he is re-intubated and with one of his tent-hats to shield the light out of his face: getting to hold him again for the first time since his surgery:

Damn Diaphragm

So Ford was re-intubated late last night after an unsuccessful 30-hour struggle. The initial thinking was that he just tired out, but that he was gaining ground and strength. The next time they tried to extubate him, after a brief reprieve, he would do even better, maybe make it all the way (we were told some kids need 3 to 4 tries).

But this morning the surgeon was concerned about Ford's difficulty and ordered an ultrasound of his diaphragm, to check for an unlikely post-op complication in which part, or all, of the giant muscle becomes paralyzed.

After waiting all morning for the results we finally met with one of the NICU fellows, who had not yet had a chance to consult in detail with the radiologist as to the results, but was able to generalize about what they saw. Turns out that half his diaphragm is not working, that it 'looks' like it's paralyzed, but not in the usual way that they see it. Apparently this can be caused by several things: post-operative swelling can constrict the muscle's ability to move, but this is generally a short-lived phenomenon and once the swelling goes down normal function returns. Ford isn't swollen anymore, he's two weeks post-op, so this probably isn't the problem. The other two problems involve damage to the nerves that feed it. They can be either temporarily numbed, or permanently cut. If they are just numbed from being poked during the surgery then they will recover - anywhere from a few weeks to a few months. But if they were cut then they won't recover at all and he will have to learn to cope with having only half a diaphragm.

If it turns out that the nerves were severed then there is a surgery they can perform to tack down the side of the muscle that is not longer working, which apparently helps him deal better with the problem (I'm a bit fuzzy on the exact details of this, we'll learn more over the next few days).

So the latest thinking is that this is why he is fighting so hard to breathe on his own. The good news: now that they have an idea as to what is wrong, the next time they try to extubate him (probably early next week) they will have a better strategy as to how to help him (apparently there is a kind of physio they perform to stimulate the area). The bad news: he might have suffered a pretty serious injury, which will set him back in his recovery and make things even more difficult for him in the long run. It also might mean another surgery.

On the plus side, Ford's catheter was removed a few days ago and that means one less tube in his body. It also means much more frequent diaper changes, something the nurses and Ford* are not as cheery about as we are. BUT... Christa, who surpasses me in so many ways, was first to step-up and offer to try her hand at changing, cleaning, and re-pampering little Sesame. Though I didn't witness the milestone myself, I was thrilled to hear about it after-the-fact and am thinking its the kind of routine I could easily settle into (her changing him while I'm out of the room).

Christa also got to hold him again - it's been over two weeks since she last got to snuggle and so, despite the threat of more trouble ahead, we really enjoyed our afternoon with Ford.

*Yesterday the nurse Bea, who was lovely in so many ways, and has undoubtedly been the most gentle and engaged and nurturing of all the nurses Ford's had, repeatedly pointed out that our little boy was "very intelligent, because he does not at all tolerate it if you do not change his diaper right away." While I was tickled pink at the thought of someone else acknowledging how brilliant our baby is (because he is obviously the smartest baby in the world), I am a bit reluctant to use the 'How quickly do you get angry when left to stew in a dirty diaper Scale' as the prerequisite for a Mensa mind. Maybe I've set my standards a little too high.

Once more with breathing

After a very calm and stable night/day, with his stomach settled and his feeding rapidly increasing, they again decided to try and extubate Ford. He had been weened down to normal room air (no oxygen assistance) and the rate of breath that the machine was supplying him was as low as it could go (which i believe is 6 per minute). He was doing very well on his own.

Extubation is a little different (at least for him) than just pulling the tube out and shouting hurray. He's still hooked up to the machine but the interface is different. Instead of a tube down his airway he has a little two-pronged device which sits against his nose and blows air up it. They call it CPAP (constant positive airway pressure).

He hated it.

I hadn't yet seen him as mad he was after they made the switch, his systolic blood pressure was up from the low 60s (where they want it) to 120, which is incredibly dangerous for him as it could burst the shunt in his heart. He was freaking out, going purple with rage and was writhing and struggling and really giving the nurses a hard time. For a while there were three of them pinning him down while the RTs adjusted the new rig. Because he's been intubated for so long we were told it would take him a while to recover the use of this vocal cords, which in this case was probably a blessing, so all he could do was grunt and wheeze. Sounded (and even looked) a lot like The Exorcist.

So this went on basically most of yesterday, them trying in vain to settle him and him going crazy every time anyone even gently touched him. Last time they tried he struggled a lot but very quickly failed to take up the challenge of breathing and so they intubated him again about 2 hours later. This time, although struggling, he seems to be coping (he's on to about 20 hours) and so they are hesitantly waiting. This morning during rounds we were told he was hanging on by his fingernails, meaning they are probably going to put him back on support again. But, we were also told that someone made a mistake yesterday, that he was supposed to be put on an assisted CPAP (where they supply constant air/oxygen and also give him bursts of increased pressure to prompt him to take bigger breaths) and hadn't been. Whether or not it would have made much of a difference is unclear, but in their step-down plan they jumped yesterday from 3-1, instead of 3-2, which probably made it more stressful on him then it had to be.

It sounds like he will eventually be intubated again, but that it isn't really a problem yet. The fact that he has lasted so much longer is a good sign that he is getting stronger and more capable if tolerating it. As frustrating as it is for us to get good news and then get it taken back, we are happy that he's a fighter, even though his fighting is in this case counter-productive (if he would relax he would breathe a lot easier).

Today we are instructed to not touch him at all, even the nurses are refraining from touching him and he is covered in a little blanket tent to keep as much of the noise and light out as possible.


Yesterday we met with one of the cardiology students who is going to be training us on Ford's home care, for whenever we get out of here. She brought a big black bag of gadgets (really big) and spent sometime showing us how to work them. The thought that we will be bringing part of the ICU home with us was pretty overwhelming and I started to freak out. With him being as irascible as he is, the thought of having to administer injections of blood pressure medication, insert feeding tubes through his nose into his intestines, measure daily oxygen saturation, weigh and constantly watch for signs of heart failure (that could be prompted by his tendency to panic), be prepared to instruct emergency staff on how to treat him, in the event we do need to make a run for the hospital (his condition being so rare), on top of all the regular drama of early parenting, she left us with our heads spinning, utterly drained, and feeling pretty hopeless.

What the heck have we gotten ourselves into?

'Lil Junkie's comb-over

Ford spent most the last 36 hours battling what appears to be (according to the most recent assessment) morphine withdrawal, constantly vomiting, shaking, a sleepless night, high blood pressure/temperature, not to mention his moaning and muttering - I just need a dime, man... Anything you can spare.

It was really upsetting to sit with him yesterday and watch him struggle with so much. With all the tubes down his nose (one draining his stomach fluid, one going to his intestines for feeding, and one for breathing) he is reduced to dry-heaving, silently gagging and choking. Eventually there was just too much coming up that it started bypassing the tube. He was drooling out thick yellowish-green goo and looking pained and incredibly distressed. Apparently he was puking out more than they were putting in.

So these withdrawal symptoms, on top of his recovery from the surgery, seem to be really taking their toll on him. They finally bulked and gave him more morphine this morning and he had a good long rest. He seems in much better spirits today. His stats on the monitor were hard to watch yesterday as his agitation had thrown them all out of whack and got us all concerned that he was going downhill again, but today things look a lot better. I think with a few more days of good rest he will be ready to try extubation again. Hopefully he will tolerate it this time.

The nurse today is quite lovely and calming and she seems very concerned that he stay settled. So we are instructed not to touch him too much and he is covered with blankets to keep the noise and light out. She took the time to wash his hair, which was matted with ultrasound gel from all the brain scans he's been getting (routine, we are assured). Then she combed his hair into something she felt resembled mine, a nice part to the side and an exagerrated wave. "Now he looks like daddy!" she cheerily exclaimed. I wanted to point out that I hadn't washed my hair in days, so in fact he looks a lot unlike daddy, a lot better than daddy, even. But I held my tongue.

He's been waking up for short periods of time today and seems much more alert than usual. He's had a big day of visitors with Anita coming up to visit from White Rock and her sister Cathy coming in from Sherwood Park. It's great to have some family come around, and more importantly to finally feel comfortable having them here. It's been so hard to think about him getting better and to feel good about introducing him to others while riding this crazy train.