March 2013: The Norwood
During Isla's life, Mo used a blog on www.islajeanjohnson.simplesite.com as a means of communicating with friends and family for frequent updates about her condition. The following entries were transferred from the “Isla’s Updates” section of her previous website. VISIT FROM THE MORPHINE BUNNY Authored March 31, 2013
Isla's had a tough weekend so far. She has been very fussy and the nurses suspect she is having some withdrawal symptoms from being weaned off methadone (which, incidentally, she was given to prevent withdrawal symptoms from morphine). So, they gave her a wee bit of morphine to help ease her pain. Don't ask us how this circuitous reasoning makes sense...we are just trusting their judgement and of course don't want our little girl uncomfortable! Hopefully this will help her relax a little bit. She's also been moved into another room to give her a little more peace & quiet. She's clocked in at just over 9lb and is still on track to move upstairs this week!
8th FLOOR ON THE HORIZON! Authored March 29, 2013
The doctors say their goal is to have Isla up to the 8th floor early next week. As long as her numbers remain stable (which, except for her single episode of tachycardia, they have since her Norwood), she will be moved to the step-down unit to continue to work on feeding and gaining weight! She's still struggling to bottle feed and will continue to work with speech therapy and Dan as long as she is in the hospital. They will take another look at her vocal chords to see if there has been any improvement. She certainly sounds louder than before, but we aren't sure at this point if that's just because she is no longer hoarse, her one vocal chord is compensating, or her paralyzed one is healing.
When she moves upstairs, we will be staying with her 24/7. The nursing care is about 4:1, and it is our understanding that she will be disconnected from all her monitors, so we can learn to watch for signs of distress ourselves. There is a pull out bed and private bathroom, so we'll be rooming in with her, for as long as we need to feel comfortable before discharging her from the hospital. She will likely go home with a feeding tube, but we are working towards the goal of all feedings by mouth. That may take several weeks or months to achieve. More pics in album!
JUST HANGIN OUT Authored March 28, 2013
CHEST TUBE Authored March 24, 2013
Isla's chest tube has finally been removed, and she is being monitored closely for the next couple of days to ensure she is not leaking/draining fluids into her chest cavity. Her chest drainage has tapered off in the weeks since her surgery, but seems to kick up again every time they make an adjustment on her feeding composition or schedule. If it turns out she will need another drainage tube, they will install a much smaller, more flexible one. She could go to the 8th floor with a tube, but not be discharged from the hospital. Speech therapists are still working with Isla once per day to work on her bottle feeding. She also boasts a music therapist, massage therapist, physical therapist, respiratory therapist, and a couple other care team members. She will be very bored when we get her out of there!
Isla is doing well. After throwing up most of her feeds for a couple of days, Pops insisted she be given more time to digest her new food mixture, which consists of Tolerex (the fat free formula), rice cereal to thicken it, and now soybean oil for lipids. Why milk is bad for her has been explained to us, but still elusive and frustrating. She will likely be on the formula blend for another 4 weeks or so before re-introducing milk. Feedings through her gavage (feeding tube) have kept her throw-up episodes to a minimum. She's also been given daily medication for reflux. The Johnsons are busy educating themselves on her medical necessities as required when she leaves the hospital, learning as much as we can about her medicines and what "red flags" to look for. Pops even installed her new gavage feeding tube in her nose last night. That involved approximating where her stomach would be, forcing a tube up her nose,(hoping it didn't enter her lungs), then blowing an air bubble into her stomach with a syringe and listening for a burp with a stethoscope to verify correct placement. She was of course shrieking the whole time, so it was tough. We're hoping she will be on all oral feeds when she gets released, but we will still be required to demonstrate we know how to correctly place a feeding tube in the event that she is not feeding well. Her chest tube is still in, and had to be re-sutured to make sure it doesn't slip out, as the original stiches from 3 weeks ago were getting loose. Her chest drainage is sporadic and the medical team in charge of her are being extremely conservative, but she needs to get it removed before moving upstairs.
TACHYCARDIA & WEIGHT GAIN Authored March 21, 2013
Isla's weight gain since her Norwood 2 1/2 weeks ago has plateaued, so her medical team has been trying a variety of different things. She's still having some difficulty with oral feeds, and has been throwing up a lot. As it stands, she is not tolerating the high-calorie formula very well, and is having trouble keeping it in her belly, regardless of whether she takes it by bottle or my feeding tube. Right now, she is being fed over a span of two hours through the feeding tube, then alternately by bottle. This will likely change a few times as the doctors fine-tune the method of getting the most food in her belly so she can continue to gain. Weight gain is the single most important indicator of how she is doing in this higher-risk interstage time between her Norwood and Glenn surgeries, so she will probably stay in the CICU until they figure this part out.
SWALLOW STUDY RESULTS Authored March 18, 2013
During her swallow study, the techs found Isla did aspirate a little bit of her feed into her lungs, so they thickened the formula with some sort of agent and administered it again, and she did fine. As I said on an earlier post, the formula she is on right now is still the fat-free stuff to prevent her from metabolizing fat, which could make her increase tissue swelling and drainage into her chest cavity. So, here's a breakdown of the trade-offs of the various things she is facing right now:
Watery, foul-tasting fat-free formula to prevent chest drainage
Chest drainage tube still inserted in case she does increase swelling
One-sided vocal chord injury that affects her suck-swallow-breathe pattern during feeding
Thin consistancy of formula being thickened to help her swallow and prevent aspirating goop into her lungs
It's all relatively small potatoes, but cumulatively mean a longer stay in the CICU. She is back on oral feeds with the thickened but still fat free formula with the help of a speech therapist to ensure she is swallowing properly.
OUR LITTLE GREEN GOBLIN Authored March 17, 2013
Isla is still pretty hoarse and is scheduled for a "swallow study" on Monday. She will be fed a bottle of some solution behind a screen and the tech will look to see that her injured vocal chord isn't affecting her ability to breathe, suck and swallow correctly. The test won't tell us how badly her voice was injured, but will focus on the far more important issue of whether she will be able to eat properly (or will need a lot more help from speech and occupational therapists before she gets discharged). She continues to be a little louder each day, so we are hoping it isn't too serious of an issue. Related to the vocal chord issue, her chest tube is still in and she is still on formula. She is being weaned off oxygen support and is taking all of her medications orally, both necessary milestones to get her to the 8th floor. Now we just need to get her back on oral feeds and she needs to prove she can gain weight steadily on them, and she will move to the step-down unit!
At two months old, we decided it was high time to get Isla in some clothes before she develops an aversion to them. She wore her first onesie a couple of days ago and promptly puked on it, but seemed to rather enjoy the feel of cotton on her skin.
SPEEDBUMP Authored March 15, 2013
Isla is now taking all of her medications by mouth and losing more of her cables and wires every time we see her. She still has a big drainage tube in her chest, and hopefully that will come out in the next day or two.
She's still very hoarse, and we found out today that one of her vocal chords is not working properly. The area where they did the surgery (mainly around the aorta) is right next to a nerve that feeds the vocal chords, and it's possible that it could have gotten stretched, nicked, or even severed during the procedure. She's been off the ventilator for enough time now that her voice should be back, so they ran a test and found out that one chord is working, but the other is completely still. More tests will follow on Monday to see the extent of the damage, and most importantly, to see if she is aspirating oral feeds into her lungs instead of taking them to her stomach. Until they can figure out what is going on, they have her back on all tube feedings (and still on formula). The nurses tell us that it's a very common complication of the Norwood, and it's a good sign that she has gotten a little louder over the past couple of days, but we likely won't know how severe the damage is for several days or weeks. We're hoping it will heal itself and won't have any long-term effects.
TWO MONTHS OLD TODAY Authored March 14, 2013
No big news to report, Isla is sill recovering very well and hopefully we will be moved to the step-down unit on the mythical 8th floor very soon. She's worked up to a full volume feed via feeding tube, but has been given formula for the past week. Apparently ingesting fat makes her at a higher risk of swelling and reduced draining, so they're giving her a fat-free formula that's easier to tolerate and adding lipids via IV. Sounds kind of goofy to us, but hopefully they'll get her back on the real stuff soon. She's off the sleepy drugs and is very alert again--we're playing with her and eagerly awaiting that first smile!
ONE WEEK POST-OP Authored March 12, 2013
Isla is continuing to get stronger every day. Tomorrow morning she is scheduled to have her intracardiac lines and pacemaker wires removed, so hopefully by the afternoon she will be in our arms! She is weighing in at just over 8lbs--small for a healthy 2 month old, but big for Isla. The nurses say cardiac babies are usually small for their age because they expend so much energy doing basic bodily functions that they don't have much leftover energy to grow. Also, I learned today that her nasal cannela gives her oxygen to help her grow. She is perfectly capable of getting enough O2 from the air around her to saturate her blood, but the extra help from the nasal tube allows her body to save a little energy and invest it into growing. She is still making tiny sounds like a baby tetradactyl, but is staying awake for longer periods as they slowly reduce her methadone. Her cry is getting stronger, but it will still be several days before it returns to its original force. We're cool with that :-)
OFF THE VENT Authored March 11, 2013
Isla is off the ventilator and stirring quite a bit. She's being slowly taken off her diuretic meds and painkillers, but is still pretty woozy. She hasn't made much sound yet, because she is hoarse from the ventilator in her throat for 5 days. We've been assured this is normal, and that she will regain her characteristic holler in a few days' time. Next up to come out are her chest and tummy drainage tubes, and hopefully her cardiac lines (so she can get out of bed and raise hell in the rocking chair with us) by Wednesday or Thursday. She's also been started on a larger volume of feeds through her feeding tube.
EXTUBATION TODAY Authored March 10, 2013
Baby Isla is on schedule to get her breathing tube removed in the next couple of hours, a procedure known as extubation. She's been taken off the sedative/painkiller Phentynol and is starting to come alive! The doctors want her wide awake for the ventilator removal, I guess so she can scream and holler and get some air in her lungs. All labs and numbers still look great. Four to 6 hours after she comes of the ventilator, she'll start feeds again--probably initially through her tube, then hopefully by bottle tomorrow. She's been given a few milliliters of milk continuously every hour for the past day or two to wake up her digestion, since Phentynol inhibits it quite a bit.
Fun fact of the day: as she gets taken off Phentynol, Isla will be given moderate doses of Valium and methadone, the same drugs given to wean heroine addicts! We trust the doctors know what they're doing. Can't wait to get our baby home and get her off all the drugs in favor of doggy therapy and good old-fashioned love from mom & dad.
STIRRING BUT SLEEPING PEACEFULLY Authored March 10, 2013
Baby Isla is still heavily sedated, but was taken off the paralytic early Saturday morning and started moving a little bit by this afternoon. Apparently she tried to pull her ventilator out after past procedures, so they are being extra cautious to keep her quiet until she is off the machine. Since she's no longer paralyzed, she can move her lungs on her own, and the plan is to slowly wean her off so she is breathing on her own. If she does well she may be off the ventilator by Sunday night, another big step towards graduating to the 8th floor. They've also re-inserted a feeding tube and are feeding her a tiny bit of breast milk directly into her tummy to supplement the IV nutrition and start to wake up her digestive system. Once she is off the ventilator, she'll be taken off sedatives, have the rest of her tubes taken out, and start to build up her feeds again. Hopefully her cardiac lines will be taken out soon, too, so we can hold her. Once all that happens and she can tolerate a full serving of milk (either by bottle of feeding tube), she will graduate to the 8th floor step-down unit, where we will be he primary care providers instead of the nurses. We can't wait!
STILL HEALING Authored March 8, 2013
Isla's bandage and some of the AED pads got removed from her today, and her stitches are getting some air. Right when I got there this morning she was having a pretty freaky oxygen desaturation episode. The doctors had been trying to suction her ventilator and there was a big plug of snot in it that got stuck in the tube, blocking her air. They had to detach it and "bag" her, giving her air from the handheld thingy, while they called in a respiratory therapist to put in a larger tube that will hopefully pass the chunks of mucous better. It was kind of scary walking into her room and finding 10 medical personnel with all kinds of alarms ringing, but they got her fixed up quickly and the head nurse assured us it looked more serious than it was and she shouldn't have any long-term effects from it.
Isla was scheduled to come off the paralytic this morning, but after her breathing incident they will keep her still another day or two, and on the ventilator until around that time, too. We felt a surge of parental pride when one nurse commented that Isla has "anger management issues" and the anesthesiologist told us he had to use a much higher dose of medicine than he expected to keep her under for the chest closure. It's like she's angry and trying to wake up. It's tough seeing her like this, unable to move, and the stress is starting to wear on us, but we're hopeful that the coming days will bring lots of steps forward for her. She sure is sassy!
CHEST CLOSURE THIS AFTERNOON; OUR FIESTY BABY; BEADS OF COURAGE Authored March 7, 2013.
Isla is still working hard to recuperate from her Norwood. The surgeon and anesthesiologist today noted how she needed a ton of meds to put her under for the procedure. she wakes up often and tries to wake from her slumber. We can tell by her elevated heart rate and blood pressure. Next we're looking at loosing the paralytic, and the breathing tube.
The first big step, sewing up her chest, is scheduled to take place this afternoon. They were prepared to do it in the morning, and Dan graciously got up at 6am to get to the hospital to sign consent (and let me sleep in!). He waited around for a few hours before the doctors told him the anesthesiologist that would be helping the surgeon with the procedure was in surgery all morning and that the hospital would call later this afternoon to obtain consent over the phone. Will let you know when she is sealed up!
The hospital has a program were patients get beads for a variety of procedures they undergo during their visit. We didn't ask about this when Isla first got there (although during her visit Nana noticed signs about it around the CICU). Isla's nurse just started, and she already has a long string of beads. Had we started in January, she would probably fill a gallon jug by now with all her courageous episodes! From now on, we're staying on top of it! Pics of Isla's beads added to the photo album.
FIRST DAY POST-OP Authored March 6, 2013
The head surgeon visited Isla this morning and decided to keep her chest open another day to give her more time to drain the swelling. They've added a diuretic to her medicinal cocktail to help her pee out the extra fluid that's most visible around her hands, feet, and belly. We called a few times during the night and the nurses said she was stable and doing well.
A couple interesting points I wanted to share are about pain management and medical intervention at this stage. First, the way Isla's pain is controlled is by watching her heart rate and blood pressure. If those numbers trend upwards, it indicates that she is in pain, and the nurses give her anything from Tylenol to morphine, depending on the severity. Because she is sedated and paralyzed, the main thing that would make her heart rate go up is pain; all other indicators and markers are watched through labs (drawn every 4 hours) and the various sensors and electrodes that monitor her continuously. It's amazing that they can measure pain by watching these numbers. As we said last night, she is not moving and sedated, but is not "under" anesthesia, so she can still feel touch and hear sounds.
Another strange phenomenon is that at this point, the medical; team is letting her body heal itself and just watching everything, intervening when they feel it is necessary. They watched her fluid retention all night, and after several hours decided to help her out be administering a diuretic. It's amazing that after all the trauma of surgery, the best thing they do now is let her body heal itself and intervene when necessary. It helps us understand why doctors don't give us timelines for anything; they always say, "we'll see how she does". They can operate, but it's ultimately up to the patient's body to heal appropriately. Sometimes that takes a little longer and can be unpredictable. Isla has already impressed everyone with her recovery thus far. She may still be in her post-op "honeymoon" phase, or maybe she is just healing slowly but steadily. We're waiting eagerly to see what happens next. Will keep the updates coming! If you need a happy baby fix, see Isla's "Mozart Shuffle".
PROGRESS Authored March 6, 2013
We grabbed some dinner and enjoyed a much-needed break at the Ronald McDonald house (it was therapy dog night!) then went back to see our baby girl. There are two nurses keeping a close eye on her all night. They told us that 3-12 hours or so after a major operation is usually the "honeymoon" period, after which the babies usually need a little more intervention as the full clout of the surgery hits them and the pain medication wears off. Isla is heavily sedated and on a medicine to paralyze her body as long as her chest is open, but the nurses tell us she can still hear and feel us. We touched, talked, and read to her for a couple of hours.
Two nurse practitioners came in to check on her while we were there and they both said a few times how great she looked. I suppose "great" is a relative term, but they assured us that her labs and stats that measure all the hundreds of important things invisible to the naked eye are all right where they should be at this post-op stage. They are monitoring her closely all night, and the surgeon will check on her in the morning. If she passes enough fluid to reduce her puffiness and her stats are satisfactory, he will close up her chest. This is done right in her room, so she won't have to go back to the OR again. Progress over the next several days will be measured by how quickly she recovers, starting with the lymphatic drainage, closing up her chest, weaning her off the ventilator, controlling her pain as she wakes up, removing cardiac lines, and eventually getting her back to the bottle feedings. Once she can tolerate an entire feed, they will move her to the 8th floor. It's a long journey but today she's taken an enormous step!!
Thank you guys so much for your kind words and constant encouragement. We love you!!
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NORWOOD PROCEDURE UPDATES - Authored March 5, 2013
ABOUT TO BE WHEELED AWAY
Isla is about to be wheeled away for surgery. We were here until late last night and came in early this morning to hold her as much as we could, because it will likely be another week or two before we can again. There are a series of steps that need to happen post-surgery to move her to the 8th floor and eventually home. The cardiac lines placed on her heart (which keep us from holding her) are one of the last things to come out. We left our phones in our room but will be updating the site throughout the day!
10am - HEART/LUNG MACHINE
They began surgery around 8:30am. For me the spookiest thing is that they actually stop her heart and re-route her circulation through a heart-lung bypass machine. The machine gets hooked up where blue (de-oxygenated) blood enters the right side of the heart. The blood is passed through the machine, where it is oygenated and pumped back into the exit arteries of the heart (pulmonary arteries and aorta). After they stop her heart, they cool down her body to keep energy requirements low as the main part of the surgery is completed. Afterward, they unhook the machine, restart the heart, check for leaks, and slowly warm her up before finishing. They'll leave her chest open for a day or two to aid the inevidable swelling that will occur by introducing the blood to the foreign machine.
The main part of this surgery is the construction of a larger aorta, which is the underdeveloped arch on the top of the heart that passes oxygen-rich red blood to the body. They will also ensure the chamber between the atria stays open permanently, and all the valves are working correctly to utilize the one good "pump", the right ventricle, as the only pump for the heart. The bands that were placed around her pulmonary arteries five weeks ago will also be checked. They typically do this as part of the Norwood, but chose to do it earlier with Isla to make this procedure a little less stressful and buy her some time to bulk up. These bands will likely remain in place her whole life.
For Isla, the main concern is swelling because she has poor lymphatic drainage, and her single kidney will be under a lot of stress to get all that extra fluid out. The next 48 hours are pretty critical. More soon. We are trying to take naps in the waiting room.
SURGERY COMPLETE
Isla's surgery is complete, and the doctors are monitoring her newly plumbed heart on an EKG machine for a period of time before they return her to the CICU. We are waiting for the surgeon to come talk with us, then will be able to see her wheeled back into the unit. We usually have to wait about an hour for the nurses to get her settled into her room before we can see her. The doctors have warned us that she will be sedated at least through tomorrow morning and given a paralytic medicine until her chest is closed in 1-2 days.
ISLA’S IN RECOVERY
We were just able to see her in her room back in the CICU. The surgeon said things went well, and as planned he kept her chest open to help with drainage. We can actually see her heart beating through her split sternum. May post pictures and video later with a disclaimer for those of you who want to see it. Some may find it graphic and disturbing, others fascinating. She was on the bypass machine for just under 2 hours. More after we get home tonight. the next 48 hours are critical.
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