Chapter 8: A Happy Ending - Part 1

Josh's recovery after the surgery was truly remarkable and far exceeded everyones expectations. In fact, Josh was doing so well that they started tapering him off a lot of his medications. By the end of the first day post-op Josh was off the morphine infusion (but he could have a dose as needed for pain) and off the milrinone. With the sedating effects of the morphine gone, he was able to wake up and look around. He was still intubated and was fighting a lot with the respirator - but this was a good sign. He was successfully extubated on Wednesday evening - incredible!

I actually got to hold him Wednesday evening after he was extubated, but I was so nervous. I was so scared that I was going to hurt him or move in a way that would cause him pain. I don't think I moved my arms an inch from the time that the nurse placed him in my arms to the time that she placed him back in his bed (which was close to an hour). My arms were dead but it was well worth it.

Each day Josh got stronger and more of his lines and tubes were removed. By Thursday evening his right and left atrial lines were removed, his pacing wires were gone (thank goodness - I hated to see those poking through his skin), and his chest tube was removed. Even the small dressing over his chest had been removed, giving us our first glimpse at the incision. I think all that remained at that point was his PICC line, his arterial line and his NG tube. I had started breastfeeding him again, but they were still topping him up with a couple of ounces of breast milk through his NG tube after each feed. Our goal was to get him solely breastfeeding again but it would take a little bit of time and a lot of patience.

By Friday it was clear that Josh was ready to leave the ICU. I was so happy that we were finally moving upstairs to the surgical floor. Josh had spent 26 long days in the ICU and finally he was stable enough to get out of there. I will be forever grateful to all the doctors, nurses and other allied health professionals that took care of Josh (and myself) while we were in the ICU. They were all fantastic and I knew that Josh was in good hands when I went home at night - giving him more hugs and cuddles than he could ever hope for.

My mom had picked up a big box of chocolates from Laura Secord to give to the ICU staff to show our thanks and appreciation and I included a thank you card from Josh. I didn't really know what to say - I mean what do you say to the incredible staff that saved your sons life? I decided to write a poem [from Josh's perspective] and it went like this:

I came to the ICU in a really bad state,

but all you doctors and nurses treated me really great.

You all worked so hard to make me well,

and to look at me now you could never tell,

Just how much I had to go through,

the ups and downs and surgery too.

So thank you for all the cuddles and thank you for all your great care,

but most of all thank you for not shaving off all my beautiful hair!

During Josh's stay in the ICU he had to receive 2 CHEO haircuts where the sides of his head were shaved for IV access. He had such a full head of hair, I was sad to see it go. I thought it was a cute ending to the poem :)

Late Friday afternoon, on Oct. 5 - 3 days post-op, Josh was transferred out of the ICU. To be honest it was a little tough to adjust to being on the surgical floor. The staff there is great but we had been in the ICU for so long and we were familiar with the nurses and use to their routines. I don't know how to explain it, I just found the transition a little difficult.

One good thing though about being on the ward was that I could now spend the night with Josh at his bedside, which meant that I could breastfeed him through the night. His NG tube had been removed so we were now breasfeeding full force and it was actually pretty stressful. I had been so used to knowing how much milk he had been getting but with breastfeeding I had no idea. I had no idea how many ounces he was taking and so I often wondered if he was getting enough. That first night was rough, neither of us got much sleep, but we got through it.

On Saturday, Josh's PICC line was removed which meant that he was officially IV free. They we even able to take him off the monitors so he no longer had any lines or tubes attached to him. I wasted no time in dressing him up in a sleeper. Finally he was able to wear clothes! Saturday was my mom's last day in Ottawa - she was flying back to Vancouver Sunday morning and though she was disappointed that she never got to see Josh go home, she knew that he would be going home soon. Thanks again mom for everything. I couldn't have gotten through all this without you.

On Sunday morning my mom came by the hospital one more time to say goodbye and to get in one more cuddle with Josh before heading to the airport. When Robb returned later that morning we got some amazing news from Dr. Maharajh - Josh was being discharged! Josh had lost a little bit of weight from the previous day, but Dr. Maharajh wasn't concerned. He was breastfeeding well and felt he would start gaining weight soon. We were elated. We couldn't believe that after all that he had been through Josh was actually able to go home; 5 days after open heart surgery no less!

So on Oct. 7 after getting our discharge instructions from our nurse and the needed prescriptions for Josh's discharge medications, Robb and I took our little guy home. The only medications he needed after discharge were Lasix (a diuretic) once a day, phenobarb (to prevent seizures) three times a day, and enoxaparin (a blood thinner) twice a day. The enoxaparin was given as an injection - I had to give Josh 2 needles a day :( At the time of discharge Josh weighed 8 lbs. With all that Josh had gone through it's hard to believe that he was able to gain any weight at all, never mind one whole pound ( he was 7 lbs when we first brought him to emerg).

He had spent a total of 4 weeks in the hospital - 26 days in the ICU and 2 days on the surgical floor. During his course in the hospital there were 10 different teams involved in his care including:

Cardiology

ICU

NICU

Neurology

Hematology

Respiratory Therapy

Genetics

Infectious Disease

Vascular Access

Physiotherapy

And he received so many different medications. These are the ones that I know he received. He received other meds in emerg and in the OR but I'm not sure what all he was given. At any rate it's a pretty impressive list:

Prostaglandins

Morphine

Versed

Phenobarbital

Heparin

Enoxaparin

Lasix

Dopamine

Epinephrine

Flamazine

Potassium

Magnesium

Calcium

Glycerin Suppository

Gentamicin

Ampicillin

Vancomycin

Ancef

Steroids (high dose - not sure what kind)

Albumin

Milrinone

Ranitidine

I just want to take this opportunity to once again express my undying gratitude and appreciation for each and every individual who helped care for Josh in some way, shape or form. CHEO is an amazing facility and it will always hold a special place in my heart.

This picture was taken the same day Josh was discharged from CHEO. Clearly he is happy to be home!

CHAPTER 7: Surgery Day

On October 2 my mom and I arrived at the ICU bright and early - I think it was around 0600. Josh's OR time was 0800 and we wanted to spend some time with him before he was taken in for surgery. (I forgot to mention in the previous chapter that my mom had extended her trip once again, so instead of leaving on Sept. 30, her flight left on Oct. 7) When we arrived Josh was fast asleep and he looked so peaceful. I wanted to pick him up and cuddle him so badly, but I knew that I should let him sleep. He had a rough road ahead of him and I wanted him to get as much rest as possible. I figured that I would just wait until he woke up. He never did wake up though - he slept the whole 2 hours :(

As our remaining time with him was drawing to a close I considered waking him up for a little cuddle time, but eventually decided against it. Josh wasn't allowed to have anything to eat before surgery and I knew if he woke up he would be hungry and I really didn't want to cause him any undue stress. He looked so calm and comfortable, I thought I should leave him be. And so I just sat at his bedside watching him sleep, wondering when I would be able to hold him again.

Around 0730 the anesthesiologist came by to check on Josh and to give me a run down of his role during the surgery. Shortly after, Dr. Maharajh came by. All looked in order - the surgery was a go. Finally, no more complications; no more delays.

At 0800 the team arrived to escort Josh to the OR. My mom and I were able to follow them as they wheeled his crib to the operating suite and wouldn't you believe just as they were pushing him down the hall, he woke up. He wasn't crying - just happy and content. Once we arrived at the operating suite the team paused to allow us time to say goodbye. That was one of the hardest things that I have ever had to do. I wanted to be strong - I didn't want Josh to see me as the emotional wreck that I was, so fighting back tears I leaned in and gave him a kiss as I softly stroked his hair. And with that he was gone - whisked off down the hall while my mom and I were left standing there, tears running down our cheeks. We slowly made our way to the Cardiology parent room where we would wait for the duration of the surgery. And so began the longest and most stressful day I have ever had to endure.

After I pulled myself together I called Robb on his cell (who was sitting in the van in the parking lot as usual - the poor guy) to tell him that Josh had been taken to the OR. Tara (the CV surgery nurse) would be stopping by periodically to give us updates on the surgery and to tell us how everything was going. I told Robb that I would call him after our first update.

Tara came by for the first time around 0930 to give us an update. The prep for surgery had been completed, Josh's chest was opened, his heart exposed and they were working on the final preparations before the arterial switch could be performed. So far everything had gone smoothly and we were quite pleased. Tara headed back to the OR and I called Robb with the update.

About an hour later Tara returned with some more news, but this time she came with Claudette (the CV surgery social worker). That should have been my first clue that something was wrong, but oblivious I greeted them both with a big smile. Tara sat down, her tone steady and somber - I was beginning to realize something wasn't right. I held my breath as I waited for what would surely be bad news.

Tara began by telling us that they had run into some complications. The team was trying to heparinize Josh's blood (heparin is a blood thinner) before putting him on the heart and lung machine, however his blood was not responding to the heparin. After giving him a large dose of heparin through his central line, the ACT (Actual Clotting Time) of his blood had not changed. [They later learned that the central line was actually in his pleural cavity rather than in his circulatory system and was unusable]. They gave him a second dose of heparin through one of the lines going directly into his heart, but again the ACT did not change. At this point Josh was already hooked up to the heart and lung machine but they couldn't start it and begin the surgery until they could effectively heparinize his blood because otherwise his blood would clot as soon as it hit the machine.

Dr. Maharajh suspected that Josh had an Antithrombin III deficiency and as a result his blood would not react to the heparin. We were informed that they gave Josh a transfusion of fresh frozen plasma (which contained the missing antithrombin III), they gave it a little time to mix into Josh's circulation and then they gave him a third dose of heparin. Finally the ACT was starting to respond.

Before the heart and lung machine could be started the ACT needs to be over 400 seconds. Anything less than that carries the risk of the blood clotting when it runs through the machine. At some point while they were waiting for Josh's ACT to reach this the ideal range, Josh's condition began to deteriorate. We were told that there was 20-30 seconds where Josh "really didn't look good" and with his ACT between 300 and 350 seconds [and trending upwards] Dr. Maharajh made the quick decision to turn on the heart and lung machine and "see what happens".

Now for Dr. Maharajh to make that kind of call leads me to suspect that they must have almost lost Josh. If his blood were to clot as it cycled through the machine they would have lost him, which makes me think that they would have likely lost him had they done nothing at all. It was a very scary thought - too scary to even confirm my suspicions with Tara. Luckily though Josh's blood did not clot in the machine and they were able to move on with the surgery.

It goes without saying that I was a nervous wreck. The transition onto the heart and lung machine did not go smoothly and while I knew that he was fine for the time being as they repaired his heart since the machine was taking care of everything, it was the transition off the machine that I was worried about. Dr. Maharajh had told us from the beginning that this type of cardiac surgery would either go very very well, or very very badly. It was all or nothing and there really wasn't anything in between. I tried so hard to stay positive and to remain optimistic, but my mind couldn't help but wander towards the unimaginable - what would I do if I lost him? The stress was unbelievable. My stomach was in knots.

The arterial switch operation took about three hours to complete. Tara had come by about half way through to let us know that everything was going well. She also talked to us about the available options for taking Josh off the heart and lung machine. If Josh's heart was unable to handle a full circulatory load then they could slowly reperfuse his body by gradually taking him off the heart and lung machine. So basically his heart does some of the work and the machine does the rest. This process takes about half the total time that was spent on the machine. Josh was on the machine for 3 hours so slow reperfusion would take 1 1/2 hours. It was suspected that Josh would require slow reperfusion so it was comforting to know that this was an option should Josh need it.

Around 1430 Tara returned with some great news. The arterial switch was completed and Josh was off the heart and lung machine. His heart started beating again on its own and unbelievably was able to handle the full circulatory load right from the get-go. Slowly reperfusing his body was not necessary. Josh was doing way better than expected. It was a HUGE relief to hear this and I could instantly feel an enormous weight being lifted off my shoulders. I cannot even begin to explain the happiness that I felt. I knew that Josh was still in surgery and that he still had a long road ahead of him, but this was one giant step in the right direction.

Josh was out of surgery and back in the ICU around 1700. Dr. Maharajh and his team joined us in the cardiology parent room around 1800 to give us a debriefing on the surgery. Even Robb was there! He came up shortly after we heard that Josh was off the heart and lung machine and was doing well. Dr. Maharajh talked a little bit about the difficulty he had when trying to heparinize Josh's blood and said that he suspected the antithrombin III deficiency was due to the fact that he's been on blood thinners for a few weeks which depleted the amount of the protein available. Other than that the surgery itself went smoothly which resulted in a good surgical repair. The only negative thing that he had to report was that he had a little bit of trouble ligating the PDA and he was pretty sure that he damaged the nerve innervating Josh's left vocal chord, which would result in a paralyzed vocal chord. Josh would still be able to speak, but his voice would be hoarse. Dr. Maharajh said that his choices during the surgery were to do what he needed to do to ligate the PDA and risk damaging the nerve, or don't and his aorta would bleed. Yeah, I'd say he made the right choice.

As it turns out the nerve must not have been damaged like he thought because Josh's voice is perfectly normal - no hoarseness. What's even more amazing is what little support Josh's heart needed to maintain systemic pressure. As you may recall there was great concern as to whether Josh's heart would be strong enough to maintain systemic pressure due to physiologic decompensation. Dr. Maharajh (as well as the ICU docs) were certain that Josh would need milrinone, epinephrine and dopamine to support his heart. It was just a question of how much of each medication would he need. Being the little fighter that he is, Josh only required milrinone which apparently is a standard medication after open heart surgery. What an amazing little guy!

After the debriefing we were able to go in the ICU to see Josh. I think it was around 1815. It had been a very long day. As I have mentioned before Robb hates hospitals and didn't like to see Josh hooked up to machines, tubes, etc. Tara had told us several times about how good Josh looked. She said that he was awake and looking around, wasn't swollen or puffy and really looked great. Since we were expecting Josh to be quite sick for a while after surgery (he was likely to get puffy again, require the ventilator, and so on) I encouraged Robb to come into the ICU to see Josh. Not knowing what the next several days had in store I thought it would be a good time for Robb to see him.

Robb was agreeable but I could tell that he had some reservations. As an added incentive I reminded Robb that the ICU has multiple points of entry/exit so if as he was walking up to Josh's bedside he lost his nerve or felt that he couldn't handle it he could just keep on walking right through one of the alternate exits. This seemed to clinch it, Robb decided to come in with us.

As I entered the ICU and approached Joshua's bedside, I was speechless. I couldn't believe how many tubes, wires and lines were attached to his little body. There were all sorts of IV lines, there was blood transfusing, he had a chest tube, he had pacing wires, he had two atrial lines, he was intubated, and he had two large strips on his forehead measuring the O2 sats in his brain and despite having just endured open heart surgery he was awake and looking around. I'll take Tara's word for it that Josh looked "really good". They do more than 100 heart surgeries a year so she would be the one to know, but he didn't look to good to me. It broke my heart.

As the initial shock of seeing Josh for the first time after surgery began to dissipate, I realized that Robb too had come in to see him. I turned toward where he had been standing but he was gone. No surprise really, but I suddenly felt bad for putting so much pressure on him to come in and see Josh.

Though it was nice that Josh was awake and able to see me right after surgery it quickly became apparent that he was uncomfortable and in all likelihood in considerable pain. He couldn't move his body, he couldn't cry, he couldn't communicate in any way but he didn't have to. His eyes told it all - the discomfort, the panic, the fear. That's a lot of emotion for a 5 week old. Tears still well up in my eyes just thinking about how awful he must have felt.

Thankfully one of the ICU doctors also sensed his discomfort and decided to double his morphine, which almost immediately put him to sleep. It was a relief to see him close his eyes because it was an indication that his pain was being better controlled and maybe he could get some rest.

Robb and I both spent the night at the hospital. It was something that we had planned on doing since the beginning - to spend that first night post-op at CHEO. We don't live far from the hospital, maybe a 10 minute drive, but a lot of things can happen in 10 minutes and we wanted to be right there should something happen. Luckily it didn't. Josh had a great night - no complications. Finally things seemed to be on track.

Joshua Is 1 Year Old!

I can't believe it but one year ago today at 5 am Joshua was born! A child's first birthday is always something special but I can't help but think that Joshua's birthday is extra special given what kind of year he has had. I still can't believe how well he is doing despite all of the challenges he has faced. He is such a strong little boy!

We had a little birthday party for him on the weekend which went quite well. There was a lot of people crammed into our small space and Josh wasn't too keen on the extra noise and rowdiness, but he handled it a lot better than I thought he would. He is a trooper after all.

So I just wanted to take a moment and say......

HAPPY BIRTHDAY JOSHUA!

Chapter 6: Still Waiting For Surgery

On day #16 in the ICU (a Tuesday), neurology performed a couple of neurological tests. The first was a VEP (visual evoked potential) where they placed probes on Josh's head and goggles over his eyes. There was flashing lights inside the goggles and the probes would record whether his brain reacted to the stimuli. His brain did react which meant that he was not blind. (Yay!) We still didn't know what he could see (light, shadows, blurred images, etc.) but he could definitely see something. Very positive news.

I should mention that the only way to find out exactly what Josh can see, is by him telling us. How he acts and behaves and how he reacts to his environment can give us clues as to how well he can see, but ultimately he will be the one to tell us what he can or cannot see.

I should also clarify that it's not his vision that is the concern. His eyes have the ability to see just fine it's how the brain interprets what the eyes see that's the concern. Incidently, Josh's vision has been tested and he is mildly nearsighted and will likely need glasses at some point. However his nearsightedness has everything to do with bad genes (I am badly nearsighted) and nothing to do with the brain damage.

The second neurological test was a SSEP (somatosensory evoked potential) of the lower limbs. Again probes were placed on his head and a little electrode thingy was placed on his lower legs (one leg at a time). The test was to see if whether electrical stimulation of the tibial nerve was transmitted to the sensory cortex of his brain. Both right and left tibial nerves caused a reaction in the brain. (Yay!)

Good news on Tuesday morning was followed by bad news Tuesday afternoon. Well I guess I shouldn't say bad news, it was disappointing news. Josh's surgery had been scheduled for the following day (Wednesday) however we were informed by the CV surgery nurse (Tara) that Josh's surgery had been postponed until Thursday morning. Apparently cardiac surgeries are usually performed on Tuesdays and Thursdays and staffing is arranged to better handle a cardiac post-op patient on these days. Josh's surgery had been booked for Wednesday to "squeeze him in" that week. The child booked for surgery on the Thursday had become ill and was not able to have surgery as planned so they moved Josh into their OR time. The news was a little disappointing as we were eager to move ahead with the surgery but figured at least he had one more day to get stronger.

My mom was really disappointed when she heard that Josh's surgery had been postponed another day. She was originally supposed to return to Vancouver on Sept. 25th but had extended her trip until Sept. 30th so she could be here for the surgery. A later surgery day meant less time here with us during his recovery.

When my mom and I arrived at the PICU Wednesday morning ( day #17) we were surprised [and amused] to see Josh sporting a mohawk. I guess the night staff were having a bit of fun with him and decided to style his hair. I think they may have even used a styling product because I couldn't get his hair to lie flat after the novelty of it was over.

Later that morning it became clear that something was going on with Josh. He was having a hard time maintaining his O2 sats in the preferred 65-85% range. His sats were often dipping into the low 60's and then it would take a long time for them to come back up again, even with oxygen. He had to be put back on oxygen via nasal prongs but this time it was set at 2L. This was a big change considering his O2 sats had often been above 85% without oxygen in the days previous.

The ICU doctors ordered a chest X-ray to take a look at his lungs to make sure that they weren't missing anything. The lungs looked fine although they were a little bit 'wet'. They ordered and extra dose of Lasix to help rid some of the extra fluid.

As the day progressed Josh's O2 sats continued to be an issue. He was also quite fussy - more so than normal - and it was difficult to settle him. I don't think he napped at all that day.

Around 1530 Josh's sats started to drop into the 45-50% range. Now something was clearly going on. The doctors decided that they needed to take a look at Josh's heart so a stat echo was ordered. The echo revealed that Josh's PDA had closed (again) and there wasn't enough mixing of oxygenated and deoxygenated blood through the ASD. The cardiologists had hoped that if the PDA were to close again the atrial septostomy that they created on Sept. 10 would provide enough mixing of blood to satisfy Josh's needs. Unfortunately it did not. It turns out that Josh had an atrial aneurysm and the septostomy had been created through this aneurysm. Whenever Josh's heart pumped the flaps of the broken aneurysm folded together creating a very small opening for blood to mix.

The flurry of activity around Josh brought back memories of when we had first brought him in to emerg. Doctors are calling out verbal orders, nurses are quickly drawing up IV medications, preparing boluses of fluid, and calling the blood bank for 0-negative blood, while another wheels over the crash cart. It was really quite overwhelming and I couldn't believe it was happening again.

Josh was given a large loading dose of prostaglandins to try and re-open his ductus arteriosis (hence the need for the crash cart - prostaglandins can cause respiratory arrest). Luckily it worked and Josh's O2 sats slowly began to climb and were soon back in the 75-80% range. Josh was kept on a continuous infusion of prostaglandins which he would stay on until his surgery the following day.

That evening/night was a tough one for Josh. The stress of the day's events had taken its toll on his little body and you could tell that he just wasn't feeling good. He was so pale, he was irritable and difficult to soothe, and his heart rate was sky high (a side effect of the prostaglandins I think). If he was calm his heart rate was around 180 beats/min, but if he was crying and upset it would easily reach the 220-230 beats/min range. It really freaked me out to see his heart rate so high. That coupled with his need to be held and cuddled (probably because he felt so crummy) lead me to the decision to stay at the hospital all night. I knew it was going to be a rough night for him and I wanted him to be comfortable so he could get as much rest as possible before the surgery - even if it meant holding him all night long.

By morning Josh was doing a bit better. He didn't get much sleep but his heart rate was in a more reasonable range and his colour was much better. However he had started running a fever overnight of 39.1 Celsius and his temp remained the same at 0730 that morning. When Dr. Maharajh came by to check on Josh before surgery he didn't like that he was running a fever. Prostaglandins can cause a low grade fever, however he was to have received a large dose of steroids at 0200 in preparation for his surgery which would lower his temp. Because he was running a fever even after the steroids, Dr. Maharajh did not feel comfortable taking Josh into the OR for fear that he was developing an infection of some sort. Surgery was once again cancelled. (We found out a short while later that his night nurse had never given him his large dose steroid as ordered. She thought that they could just give it to him in the OR, but he was supposed to get one dose at 0200 and then a second dose at 0800 in the OR.)

Dr. Maharajh ordered blood work - what he was specifically interested in was his WBC (white blood cell) count and blood cultures. His WBC came back at a whopping 24.5. Two days earlier his WBC's had only been 13. It appeared that Dr. Maharajh had made the right call - it seemed as though Josh had some sort of infection, but the result of the blood cultures were needed to confirm it.

Understandably I was really frustrated and disappointed that Josh's surgery had again been cancelled. I was even a little upset because if the surgery had just gone ahead on the Wednesday like originally planned the whole issue of his PDA closing again (and the aftermath) could have been avoided. Dr. Maharajh must have sensed my disappointment because he took me aside and explained his concerns with taking Josh into the OR when running a fever. A combination of the high dose steroids and being put on the heart-lung machine would basically wipe out Josh's immune system for at least a few days after surgery. If Josh did have some sort of infection and the surgery had gone ahead on the Wednesday as planned before any symptoms had developed it could have had fatal consequences, as Josh's body would have no way to fight it off. After hearing this my frustration quickly turned to relief and as the day progressed that relief increased ten-fold as it was clear that infection or not, Josh was in no condition to undergo open heart surgery. He was exhausted, he was weak and his body was still struggling to recover from the stress of the day before.

So what did all this mean? The best case scenario: there was no infection - the fever was a side effect of the prostaglandins and the elevated WBC was a result of stress. If this was the case then the surgery would go ahead on the following Tuesday at the earliest. Worst case scenario: blood cultures come back positive for an infection and Josh would need antibiotics for 7-10 days to clear it before he could have surgery. Obviously I was praying for the best case scenario and a few days later when the results of the blood cultures came back those prayers were answered. There was no infection and surgery was booked for Tuesday October 2.

Once Josh had been cleared for infection he was placed in protective isolation to limit his exposure to germs until his surgery. I was still able to be with him but it just limited the amount of traffic around his bedside. I thought it was a great idea. Plus there was a little t.v. in there to help pass the time when Josh was napping :)

The evening of Monday October 1 - the night before his surgery - was one of mixed emotions. I was happy that Josh's heart was finally going to get fixed, but nervous about how the surgery would go and what the recovery process would be like. The ICU doctors had been preparing me for what to expect after surgery. Since Josh had a rough few weeks already they expected a lengthy recovery time and told me not to be surprised if he had to spend another 1 1/2 - 2 weeks in the ICU (at least one of which he would likely be on the ventilator) and then as much as a month (or more) on the surgical floor before being discharged. It wasn't really a process I was looking forward to, but I was prepared to do whatever was needed to get Josh through it.

I was also going to do everything in my power to make sure that his surgery wasn't cancelled again. I knew Josh was supposed to receive his first dose of the high dose steroid at 0200 and I realized that he didn't have an IV line. Since there was the issue of him not receiving it the last time around I wanted to make sure that it wouldn't get missed, so when Josh's nurse came by the room around 2100 to check on him I mentioned that he was to receive the IV steroid at 0200 but that he had no IV access. ( He had a PICC line but the prostaglandins was infusing through it and required a dedicated line). At first the nurse denied that he had any medications ordered for that time but after a quick review of his kardex corrected herself. She said that she would page vascular access to start an IV - which is ultimately what I wanted her to do, but thought it would be best to point her in the right direction and let her make that decision on her own. I was walking that fine line between being a concerned, well-informed parent and being an over-bearing, know-it-all parent.

The vascular access nurse came by about an hour later, Josh got a new IV line and his nurse knew to give him his steroid. Everything seemed in order, Josh had drifted off to sleep and so I headed home to try and get some rest before his surgery the following morning.

Now That I'm A Working Mom

Well, I'm happy to report that the transition back to work was relatively smooth - for me anyway. It took a few shifts to get familiar with the unit again and become comfortable with all the little things that had changed in the year that I had been off, but all has come together nicely and I now feel that I have found "my groove" so to speak.

The hardest part about being back to work (other than having to leave Josh of course!) is adjusting to even less sleep. After working the evening shift I generally get home around 11:45 pm and unfortunately Josh is not a good sleeper. He's extremely difficult to get to sleep (my fault I admit --I have spoiled him) and he still wakes during the night - once for a bottle and then few times before and after. He generally settles back to sleep with a little reassurance and comforting, but not always.

Robb has had a rough adjustment to my returning to work. Josh is not used to Robb putting him to bed and he has given him a hard time - lots of crying and protesting on Josh's part. Hopefully Robb and Josh will settle in to their own routine soon, preferably before Robb loses his mind :)

In other news, Josh has finally gotten his first tooth - one of the bottom front ones. He's almost a year old and I was beginning to wonder when that first pearly white would show itself. It seems like he has been teething since he was 4 months old but there has never been anything to show for it. But at last he has a tooth! Very exciting! I think a second tooth must not be far behind judging by the amount of chewing and biting he is doing. The tooth is still too small to get a good picture of, but as soon as I can snap a pic of that new tooth I'll post it for you to see.

Until then I'll leave you with a picture of Josh cruising around the furniture. It's one of his new tricks that he just loves to practice.

CHAPTER 5 : Waiting For Surgery

Josh was finally taken off the ventilator on Monday evening (September 17) after 8 long days in the ICU. Finally I was able to hold him again. It was a little difficult to negotiate all of his IV lines, wires and tubes but once I had him in my arms all of my stress and apprehension, fears and worries melted away, if only for a moment. But it's a moment I will never forget. Oh, and to hear him cry! It was the sweetest sound - his soft, squeaky little voice.




Josh did really well off the ventilator. He needed a little bit of oxygen via nasal prongs but just a whiff really - he was being titrated between 0.1 and 0.2 L of oxygen. After a few days he was taken off the oxygen altogether.


Our second week in the PICU was much calmer as we were just waiting for Josh to get stronger and for his brain to stabilize before surgery. Josh was looking and acting much like the little guy that I had at home. He was bright and alert and acting like a normal little baby. And he loved to be held. He was a real cuddle bunny (and still is!) and was most content when held in someones arms. Once he came off the ventilator I was holding him every available moment. I didn't want to give him up. My mom had been with me in the ICU a good part of each day and I'm sure she was just dying to hold him, but she never said a word. She let me have my time with him I guess waiting for the day that I would be able to hand him off to someone else. I don't remember how long she had to wait - a long time; several days. Thank you for being so patient - hopefully the wait wasn't too torturous. I just wasn't ready to share him. One of the few times in my life that I have been selfish.


Robb managed to make it into the PICU a few times that week to see Josh. He had a real hard time being in that environment (all the bells and alarms) and he didn't enjoy seeing Josh hooked up to all the machines and monitors. He wanted to be close by so he stayed on the hospital grounds all day - either sitting in the van in the parking lot waiting for me to call him with an update or sitting in one of the waiting rooms in the hospital - but he couldn't always bring himself to enter the ICU. When he did manage to psych himself up enough to come in, it was usually only for a few minutes at a time.


I remember once when he came in I asked him to stay with Josh for about 10 minutes so I could go and pump some breastmilk. Josh was awake at the time and I didn't like to leave him alone unless he was sleeping. Robb reluctantly agreed. I could tell he wasn't completely comfortable with the idea of being left alone with him, but I thought it would be good for them to have some time together. When I returned Robb was sweating like crazy and was really flushed. I guess the stress of being there longer than what he was comfortable with brought on a bit of an anxiety attack or something. That's probably the first time I realized how much of a struggle this was for Robb. I mean I knew it was tough for him, but now I knew how tough. I have no idea how he managed to sit in the van for hours on end, alone with his thoughts and his cigarettes but obviously we all deal with stressful times in our own ways - and that was his.


On day #10 in the ICU (a Wednesday) Josh was finally able to eat again. The cellulitis on his abdomen had cleared up (thanks to antibiotics) and the abdominal distention had decreased significantly. The doctors in the ICU had not been able to confirm whether or not Josh had NEC. None of his X-rays showed signs of it, but they did show that a normal gas pattern had returned indicating that whatever trauma the gut sustained had resolved. I was so glad that Josh was finally able to eat again. I still wasn't able to breastfeed him but at least he would be getting some food into his belly. He was one hungry baby! He had been receiving fat and nutrients through one of his IV lines but nothing had been in his stomach in over a week - and he let us know every chance that he got.

Josh was started on a slow infusion of my breast milk through his NG tube - and when I say slow infusion I mean 2 mL an hour. I understood the need to reintroduce food slowly in order to ensure no reaction from his gut, but 2 mL an hour? Geez, the guy was starving and we were just giving him a tease. The rate was increased 2 mL/hour every 4 hours so long as Josh was tolerating the feeds well, to a maximum of 27 mL/hour. Once Josh reached the maximum infusion rate and had tolerated it for 4 hours I was able to start breastfeeding him again. Yay!

The purpose of breastfeeding wasn't to satisfy his caloric needs but rather to maintain that mother-baby bond and to help Josh remember how to breastfeed. Josh tired really quickly when breastfeeding because of his heart defect and his respirations would often reach the 110-120 breaths/min range while feeding. So really the breastfeeding was more or less just non-nutritive sucking and then he would get 2 oz of breastmilk through the NG tube after every breastfeed. It felt nice to be able to breastfeed him again and I know Josh enjoyed it too.

The end of the second week brought the most activity as the results of two big tests were revealed. The first was a genetics test. A sample of Josh's blood had been sent for genetic testing on the second day of Josh's admission. Apparently congenital heart defects are sometimes associated with a genetic disorder called DiGeorge's syndrome so a full chromosomal workup was performed. The results came back normal, which was great - there was one less thing lingering at the back of my mind.


The second test was the follow-up MRI of Josh's brain, the results of which would dictate what happened next. If the MRI showed that his brain was ready to tolerate the surgery and Dr. Maharajh felt that Josh's left ventricle was strong enough to maintain systemic pressure, the surgery would likely happen the start of the following week. If the MRI indicated that Josh's brain needed more time to recover and/or if Dr. Maharajh felt that the left ventricle had weakened too much, they would first have to perform a procedure where they band the pulmonary artery to increase the pulmonary resistance and therefore force the left ventricle to work harder/stronger. This was a procedure that would also require open heart surgery but is far less invasive then the surgery he would eventually need.


The MRI was done on Friday September 21 - day #12 in the ICU. A family conference was scheduled for late afternoon to discuss the results. Neurology, cardiology, the cardiovascular surgery team and the ICU team was all present at the meeting, as well as myself and my mom. The neurologist (Dr. Macmillan) started things off by sharing the results of the MRI. The MRI showed that the clot was beginning to dissolve and blood was now able to flow around the clot. The intracranial pressure had decreased and the swelling had subsided. All good news. Neurology gave the go ahead for surgery. Yay!


Unfortunately the MRI wasn't all good news. Now that the swelling had subsided it was evident that Josh had in fact sustained a moderate amount of permanent brain damage, particularly to the occipital and parietal lobes and the left side was damaged more than the right. Based on the areas of the brain affected neurology said that Josh may have epilepsy, may have difficulty learning, may have difficulty with movement and would most likely have problems with vision. With regards to the vision, there was the possibility that Josh would be completely blind. Or he may only be able to see light or shadows or blurred images. Or he may be able to see perfectly clear but then there was the possibility that his brain would not be able to interpret what he sees. At any rate, neurology could not give us a clear cut picture of what exactly the brain damage meant for Josh. He was still very young and there was great opportunity for the healthy areas of the brain to compensate for the functions of the damaged tissue. However, the amount of brain damage was "significant" and some deficits were to be expected.


I tried so hard to stay strong but the news that I was unwilling to accept a week ago was now very real. I broke down, tears streamed down my face and I found it difficult to collect myself. I did manage to pull myself together long enough to hear what Dr. Maharajh had to say with regards to Josh's cardiac surgery. He felt that in Josh's case, the less surgeries the better so he would go ahead with the ASO (arterial switch operation). If his left ventricle had decompensated too much they could support it with medications until it was strong enough. Surgery was booked for Wednesday September 26.

Once the meeting was over I was still pretty upset. I was glad that we had a surgery day and that we could finally move forward but I had a hard time dealing with the neurological issues. The ICU staff was nice enough to let us use their conference room for as long as we needed - I think it took me a least an hour to pull myself together. As I look back on it now I think everything hit me all at once, at that moment, and it was the first time that I really let myself grieve the loss of my healthy baby. It's probably something that all parents go through when they learn that their child has a serious medical condition. You go through the 9 months of pregnancy with a certain expectation - a certain life plan for you and your family and all of a sudden it all changes. The future of your child and your future as a parent is suddenly unclear. I needed to grieve that loss before accepting my new path in life.

CHAPTER 4: The First Week In The PICU

When I arrived at the hospital early Tuesday morning - I'm not sure what time it was, maybe 0730 - Josh's condition seemed to have stabilized. Well, at least his vitals were in a good range but I guess not much else had changed.

Tuesday was a busy day. Actually that whole first week was extremely busy. There were so many different teams involved in Josh's care and it seemed like there was always someone around assessing Josh and/or turning to me for information. I answered a lot of questions that week - many of them more than once. Questions about my pregnancy and delivery (specifically any complications), questions about our family health history, and questions about the day Josh got sick seemed to be the most popular. In retrospect even though that first week was so hectic and busy it probably helped distract me from dwelling on how sick Josh was.

At 1400 on Tuesday Robb and I had our first meeting with the cardiac surgeon (Dr. Maharajh) and his team. Dr. Maharajh went over in detail the nature of Josh's heart defect, and what would have to be done surgically to repair his heart. I think it was during this meeting that we first learned that surgery was booked for Friday September 14. Ideally Dr. Maharajh would have liked to perform the surgery Monday evening however Josh's body needed some time to recover from the stress of everything he went through. I wish I could remember exactly how Dr. Maharajh put it because I remember I found his choice of words shocking.....I think it was something to this effect: 'Ultimately I would have liked to do the surgery yesterday but when babies do what Josh did, that is when they try to die, they need some time to recover before they can have surgery.' It was the 'trying to die' part that shocked me - I wasn't expecting that and I found myself still lingering on that statement long after he said it.

During this meeting we learned that there is typically a three week window to perform the type of surgery Josh needed (Arterial Switch Operation), meaning the surgery should be performed within the first three weeks of life. Due to the anatomy of Josh's heart his left ventricle was pumping blood to the lungs which takes a lot less strength than pumping blood to the body, which is what would be required of it after surgery. The concern was that after three weeks outside the womb, the baby's heart begins to adapt to it's physiological workload. In other words, the left ventricle will weaken and will no longer be strong enough to pump blood to the body. At the time of our meeting Josh was two weeks and 3 days old - that three week window was almost closed. A surgery scheduled for Friday put us 1 day before he was 3 weeks old.

When I returned to the ICU after meeting with the surgeon I received some bad news from Dr. Foster. Josh had started having seizures, indicating neurological issues. When newborns have seizures it's pretty subtle. They don't have convulsive episodes like an adult. The only physical signs of Josh's seizures was occasional hand and lip twitching. An EEG (electroencephalogram) was performed to confirmed the seizure activity and an MRI would have to be done to investigate what was going on in his brain. When Dr. Maharajh heard of the seizure activity he decided to push the surgery to Saturday September 15 to give Josh's brain an extra day to rest. There's our three week window.

As I was talking with Dr. Foster about the seizures I noticed that Josh's belly was quite distended and that there was a red rash on the right side of his abdomen from his groin to his armpit. I think she noticed it at the same time I did. She grabbed a black marker and traced the border of the rash so they could see if it was getting worse. It turns out that the rash was cellulitis and they now suspected that Josh may also have issues with his gut. They had started to feed Josh my expressed breast milk through his NG tube (a tube in his nose that goes to his stomach) but had to stop until they could determine if his gut had sustained damage. Apparently the bowel is one of the first things to suffer during periods of hypoxia because blood flow is shunted away from the intestines in favour of the vital organs. The ICU doctors suspected that Josh may have NEC (necrotizing enterocolitis). I didn't really know much about it, but it didn't sound good. Abdominal X-rays were ordered every 8 hours to monitor for signs of NEC.

On Wednesday Josh received another EEG. It showed that he was still having seizures, sometimes lasting as long as a minute (not a good sign). He was started on a continuous infusion of Versed to get the seizures under control and once the seizures were stabilized a couple of days later he was switched over to phenobarb three times a day. An MRI was done Wednesday afternoon to take a look at what was going on with his brain. The MRI showed several things. First, it showed a small bleed in his brain. It was only a small amount of blood and it was old - it probably happened during birth. Not really a big deal as long as it didn't start bleeding again. They would have to keep an eye on it. The MRI also revealed that Josh had suffered a venous stroke. As you may recall the metabolic acidosis had caused sluggish circulation so blood was draining from the brain much slower than normal. As a result, a clot had formed in one of the veins of his brain (they call it a sagittal sinus thrombosis) occluding that vein. Intracranial pressure began to build which caused his brain to swell and the swelling caused brain damage. It was all this trauma that caused the seizures. Actually at that point we weren't 100% sure that Josh had sustained brain damage. It was possible that most of the blackened areas on the MRI images were simply cerebral swelling rather than actual damage. We'd have to wait for the swelling to subside before knowing for sure.

As upsetting as this news was I have to say that I wasn't devastated. Not yet anyway. There was a chance that there was minimal to no damage and I was holding out hope that the stroke hadn't caused any permanent brain damage. In fact, I was so optimistic that I didn't really even entertain the idea that he may have brain damage. I somehow managed to convince myself that it was just cerebral swelling and in a week's time we'd see that there was no brain damage. Perhaps it was foolish or naive to be so blindly optimistic but I think it may have been a defense mechanism. I had been through an emotional rollercoaster the last few days and I don't think I could deal with yet another complication as serious as brain damage. I was in denial. I wasn't ready to deal with it.

Since Josh's seizures were not yet under control and there was a significant amount of cerebral swelling neurology decided that it was not safe for Josh to have open heart surgery at this time. His brain would need at least 7-10 days to rest and recover from the stroke. Another MRI would be done in about a week, the results of which would dictate what would happen next. In the meantime Josh was started on a blood thinner to treat both the clot in his brain and the clot in his groin.

On Thursday a PICC (peripherally inserted central catheter) line was inserted into Josh's right arm. It was extremely difficult to get IV access on him and when they finally did get an IV in him it usually wouldn't last very long. The PICC was a godsend because at least it gave him one reliable IV line. By this point he was receiving TPN (total parenteral nutrition) as he was still not allowed to receive breast milk. He continued to receive abdominal X-rays every 8 hours and so far no evidence of NEC was apparent but the NICU had recommended to his doctors that he not be fed for 7-10 days in order to err on the side of caution.

On Thursday evening my mom arrived from Vancouver. It was a trip that had been planned for a while but it's purpose changed when Josh got sick. It was great having her there not only for support but she also made sure that I took care of myself. Up until her arrival I had hardly been eating and I hadn't been pumping my milk as often as I should have been. Having her there made dealing with everything that much easier. Thanks mom!

On Friday the doctors started weaning Josh off some of his meds so he was able to wake up and look around, although he was still pretty groggy. On the weekend he was much more alert when awake. I believe he was also off the prostaglandins at this point. I guess the prostaglandins have a lot of side effects and since it was still a while before his surgery his doctors figured that they would take him off of it. If his PDA were to close again he still had the ASD which they thought would provide a sufficient amount of mixing of oxygenated and deoxygenated blood.

The end of the first week was much quieter. Partly because it was the weekend and partly because Josh's condition was improving. The seizures appeared to be under control with medication and his vitals were consistently stable. However, the weekend wasn't without incident - on Sunday we learned that the upper lobe of Josh's right lung had collapsed, likely due to the ventilator. A physiotherapist came by twice a day to work on loosening the secretions so that the lobe could reinflate. They had started to wean him off the ventilator as Josh had been triggering more and more respirations but he wasn't quite ready to come off yet. Probably in another day or two. I was really looking forward to the day when he could come off of the ventilator. It had been a week since I was last able to hold him in my arms. I wanted so badly to pick him up and give him a big hug, and show him how much he was loved.