Fontan "Anna's Fontan Story"

Hi Everyone,

For those of you short on time surgery was on Wednesday July 23, 2003.We were released to Ronald McDonald house on Tuesday the 29th and we left Edmonton on Thursday the 31st. Today is Saturday and she is a bit grumpy and clingy at times but she is mostly happy and sleeping well. For those of you with a little time, here is the full story.

We left for Edmonton at 7:30am on Monday the 21st. We dropped Jesse, Emily and Justin off at their Uncle Scott's in Saskatoon around 9:30 and were on our way to Edmonton around 10:00. We arrived early in the afternoon, had a nap, did some shopping etc. We had to be at the hospital Tuesday morning at 8:00am to do all the pre-admit stuff including meeting the anesthetist(?), surgeon, an echo and last but not least blood work. When Anna was smaller she was really hard to poke and we were hoping that had improved but sadly it has not. The one tech tried in her left arm and finally gave up after what seemed like forever and asked someone else to try her other arm. As soon as they tied the little blue band around her right arm Anna said "UH OH". She was initially quite upset but by the time they finally found a vein in her second arm she had stopped crying and Chris actually had her (along with everyone else in the lab) laughing as he was jumping all around the room pretending her book was biting him in the butt.Her surgeon (Dr. Ross) said he was planning on performing the extra-cardiac fontan but he wouldn't know until he got in there if she was big enough to insert a large enough gortex tube that she wouldn't outgrow it. If she was too small he said he would do the lateral tunnel instead. We left the hospital around 1:00pm. We spent an uneventful evening just hanging out and playing. We had to give her three steri-baths, she loves playing in the tub so that wasn't a problem. We actually made a quicktime movie on our digital camera of her dancing which we showed to everyone at the hospital so they could see what a silly, happy baby she normally is.

We had to be back at the hospital at 6:00am. As soon as we arrived they put Emla cream on both her hands and wrapped them up so she looked like a little boxer. Then we basically just waited around in the clinic playing in the playroom until just before 7:00 when a porter came to take us up to the OR. Unfortunately our nurse was new and she forgot to give us the sedative Anna was to take before going with the porter so after a few minutes up stairs we went back down to the clinic where they gave her some Medazzlin(?) . We then went back up to a little play area just outside the OR. By the time we got up there the Medazzlin was kicking in and Anna was just a hoot. She thought everything was the funniest thing ever. We have some great pictures of her making faces and laughing her head off. It was so nice to turn her over for surgery so incredibly happy. Only one of us was allowed to go with her into the OR so Chris waited and I took her. She was extremely goofy but not sleepy so they put the mask over her face for just a minute and she started to go out. A nurse walked me back to where Chris was. I was holding it together just fine until she said "You were so good with her". At which point I lost it. Last surgery I actually fell on the floor bawling, this time at least I managed to stay upright. We stayed in the little waiting area for a few minutes while I had a good cry and then went to the waiting area in front of PICU to wait for the rest of the surgery. Dr. Ross had told us to be sure we were in the PICU waiting room at 11:00 but not to be too worried if he wasn't there until closer to noon. It was actually closer to 1 before he finally came and we were really starting to worry. I had tried to read to pass the time but after reading the same page 15 times I gave up. The day before when we were waiting for Anna's turn to have her blood drawn I had walked next door from the blood clinic into Admitting because they had a few toys and one of those tables with wires and beads that they always seem to have in hospital waiting rooms. Anna was playing with the bead thing and I noticed it was disgustingly filthy. The yellow beads were actually black with grime. When I couldn't read and was starting to go stir crazy I went down to admitting and asked if I could take the table back upstairs with me and clean it while I waited. You could tell the receptionist thought I was absolutely insane. She said , "We don't have any cleaning stuff here". I told her baby wipes will clean anything and I had plenty with me. She said she would have to ask. So she went in the back somewhere and came back a few minutes later and said it would be alright for me to take it she just needed my name. It took me probably 45 minutes to get it clean but it looked like new when I was done and it did make me feel better to have something to do. When Dr. Ross finally came he said things had gone well, he was pleased. THe reason it took so long was he decided to do the tunnel rather than the extra-cardiac. She was oozing a bit more blood than he would like but not enough that he was too concerned. Her sats as they were leaving the OR were 100 but he expected that to drop. But overall he was pleased. About a half hour later we saw them wheel her into PICU and about 20 minutes after that we were able to go in and see her.

She came out of the OR this time with a lot more lines than she did after the Glenn. She had an arterial line in her jugular vein, one in each wrist, one in her left ankle as well as a line into her Atrium to measure pressures there. She also had an IV in place in her left arm as well as in her left ankle. She had a foley cath. She was on the respirator and she had two sets of pace maker wires and was being atrially paced so there was also a portable pacemaker lying at her side. Plus of course the O2 sat monitor on her toe and three leads placed at various places on her chest to monitor her respirations and heart rate. And a chest tube. Her blood pressure kept going too low and they believed this was because her fluid level was too low so they kept pumping in more pack cells, and albumin. THis meant that even though she wasn't really swollen when she first came out of OR she was pretty puffy by the end of the night on Thursday. They were giving her morphine but they really wanted to get her off the ventilator so they turned it down and by midnight she was awake enough that they pulled it. She didn't even have her eyes open and she starting begging for a drink. Her first words were "drink of juice please". They wouldn't give her any liquids for four hours in case they needed to put her back on the vent. They were giving her chloral hydrate to help her sleep but she still woke up lots and would just beg for a drink. I told her the juice was all gone so she said "Water please" when I said no water she said, "I go bath". I guess she figured a drink of bath water would be better than nothing. Around 2:00am Chris came and I went to sleep for a few hours (we slept in shifts so she would never be without at least one of us there). So he was the one who was there at 4am when they finally let her have something but of course it was less than a teaspoon full. Chris said she just looked at him like "What the heck!". They kept her fluid restricted for what seemed like forever but I think it was only until around lunch time when they pulled the line out of her Atrium after that she was allowed to drink as much as she wanted and we could give her popsicles which was great as they lasted a lot longer than drinking. We have pictures of her eating one. She was so tired she wouldn't even open her eyes but she held it and ate it by herself. Thursday morning they turned off the pace maker but at some point (I can't remember the exact time, it all sort of blurs together) but I know it was after they pulled the AV line she had a tachycardia spell where her heart rate was over 250. IT was only for a minute, they shocked her with the pace maker and she went back into a sinus rhythm of her own. No one seemed to know why that happened but they decided to turn the pace maker back on and set it at 110 until Friday. Friday morning they pulled the chest tube and shortly after that all the arterial lines and the foley cath so that all she had left were the monitor leads, sat monitor, pace maker wires and pace maker and the two iv lines which were in but not being used and oxygen (she refused to leave the things in her nose alone so she had a 100% oxygen blow by) and we were allowed to move up onto the floor. She had been on heparin to keep her from clotting but before leaving PICU they started giving her a half a baby aspirin instead. She had also been taking something so her heart wouldn't have to work so hard but I can't think right now what it was called (it started with an m). She was on profonol,morphine, adavant(?)and chloral hydrate at different times to help with pain and sleep but once we left PICU she was only taking Tylenol and Advil for pain, Lasix and Aldactone to help her get rid of all her extra fluid and the baby aspirin.

On the floor we were put into a room with a 15 year old girl who had surgery the same day for aortic stenosis. Her name was Nicole and this was her second surgery. She was pretty uncomfortable the first couple of days and didn't say much but we were both released the same day and by then she was much more talkative and seemed like a nice girl. I had requested that Anna be put into a bed rather than a crib once we had a room and so Friday night I slept with her and Chris went to Ronald McDonald house. We realized at about midnight that she hadn't peed since they had pulled the foley cath in PICU. So her nurse decided she would start up an IV. Earlier in the day she had flushed the iv in her arm to make sure it was still good , it was so she had taken the one out of her ankle so Anna would be able to walk around. Of course that night when we needed it the one in her arm didn't work so they pulled that one too. We didn't want to try and put in another one so we spent quite a bit of time squirting juice into her with a syringe and finally Saturday morning she peed. WE then had Dr. Ross come by and say that her last x-ray showed more fluid than he would like so not to force liquid on her only give it to her if she asked and not to worry about her not peeing. Saturday was when she really started acting more like herself, laughing and smiling and talking lots. The only negative thing on Saturday was they sent someone in to try and get blood. They had put Emla cream on her arm first thing in the morning but we missed the tech while we were gone to X-ray. He was suppose to be back right away but he ended up not coming for four hours which meant she ended up with a rash from the cream. Anyway he tried but couldn't find a vein. He actually gave up fairly quickly saying he wasn't going to torture her anymore. Apparently he is the one they call in for kids that are hard to poke and he said he never leaves empty handed but he just couldn't do it. He was apologizing left and right and gave her all kinds of stickers trying to make her feel better. He said he would check and see if she could skip bloodwork as she had just had it done in PICU the day before. I guess they decided she didn't have to have it because no one came back until Sunday. I actually saw the tech from Saturday in the hallway Sunday morning and he looked at Anna and said "Don't worry sweetheart, they couldn't pay me enough to go near you again". They sent a neonatal transport nurse instead who didn't even try her arms but went for her foot instead and got it on the first try. She had a hard time getting enough blood out but at least she didn't have to poke her for ten minutes first. She also made the nurse go and get smaller blood vials called microtainers before she even started so that not as much blood was needed. So remember that if any of your kids need bloodwork ask for "Microtainers" as opposed to the regular pediatric size vials. Anyway, when we moved out of PICU they turned her pacemaker down to 80 so as long as her heart was beating faster than that it stayed off but if it dipped below 80 it would kick in. All day Saturday and Saturday night she stayed above 80 so first thing Sunday morning they pulled the pacer wires. The pulling of the wires themselves didn't seem to bother her too much but she got really upset when they were taking off all the bandages and tape that were covering them. She was so upset they actually left one bandage partially on and told me to just take it off later when she was calmer. Throughout the day I picked at it and by bedtime I had all but one corner of it loose, that corner was actually stuck in with the bandages covering her incision and chest tube. When I pulled it loose a small amount of pink liquid leaked out around the incision bandage. I called the nurse and she said not to worry it was probably just a bit of fluid that had been just under the skin. At 4:00am when they woke us up to giver her her meds I felt a wet spot on the bed and thought she had peed out of her diaper but when I turned on the light I saw it was a pink spot about the size of my hand. The nurse still didn't seem too worried but taped some gauze over all the other bandages so we would be able to see it if it leaked any more. Anna woke up at 6:00 and wanted to go for a walk. I was standing in the hall talking with the nurse specialist when I realized the whole front of her shirt was wet. We went back to the room and they called the Cardiac Resident on call and him along with two of the surgical residents came to have a look at her. They thought she was probably leaking from the chest tube site and said that wasn't too much of a concern but they wanted to take all the bandaging off just to make sure. The Resident pulled everything off and fluid just gushed out of her. She was quite upset and everytime she would cry it would actually squirt out of her chest (the incision not the chest tube). By this time Dr. Ross was already in the OR for his first surgery of the day so they decided I should just keep covering it with gauze and wait and see what Dr. Ross wanted to do. Chris was still at Ronald McDonald house. They had actually told us Sunday night that she would have an echo on Monday morning and then they would likely release us to RM House and just have us come back on Wednesday for a few last tests and then send us home. He had set his alarm for 6 but had had trouble sleeping that night so he decided since everything was fine he would go back to sleep for a bit. He showed up about 7:30. By this time Anna did not want to be in her room (bad things happen in the room) so I was sitting in the hallway with her watching tv with a pile of gauze beside me that I was changing what seemed like every other minute. When Chris walked up I said sorry we're not leaving today she is leaking at which point I removed the gauze so he could see the liquid coming from her chest. He says he wasn't really grossed out so much as freaked out. He felt all the color leave his face and just couldn't believe that was coming out of his baby's chest. We just sat for the next couple of hours in what I can only describe as a controlled state of panic ( if that makes any sense). By the time Dr. Ross finally came (he actually wasn't coming to see us, he was going somewhere else but Chris saw him and basically dragged him to where Anna and I were to have a look at her) Chris was starting to look really sick. We were both trying to play it cool but not knowing what was going on was really starting to freak us out. having her run around and look so happy helped but we were still pretty worried. She wouldn't sit calmly so Chris took a receiving blanket and wrapped it around her chest and taped it in place so we could keep the gauze in place without tape (she hates tape) When Dr Ross came we got her all unwrapped and he looked at everything, said she looked fine and that probably when I removed the bandage it popped a stitch underneath and that there is always fluid in the chest she just maybe had a bit more than usual that they would just keep an eye on it and he was sure it was fine. He said it looks freaky but for us not to freak out and he would come back to check on her later. By this time Chris couldn't stand it anymore and he had to leave for a bit and go and buy her some more toys. While he was gone I went with her for a chest x-ray and an echo. She was happy and co-operative for both. When the echo was finished I changed the gauze and put her down for a nap. She slept about three hours and when she woke up the piece of gauze closest to her skin only had a small spot of fluid on it and it was actually stuck to her so I just left it.

Chris came back and we put more gauze on top of the first piece, closed her pj top, rewrapped the receiving blanket around her chest and taped it back up and she spent the next few hours runing around and playing like there was absolutely nothing wrong. On Sunday they had changed one of her diuretics from Aldactone to Aldactazide. I noticed that night her eyes looked a bit red. By Monday afternoon they were extremely red and when we took her for a walk and passed by a window she screamed and covered them. They didn't seem itchy at all but were extremely sensitive. I mentioned this to the nurse and told her I thought it was either the lasix or aldaczide. She looked it up and photosensitivity was one of the side effects listed for both drugs (of course they had never seen anyone react that way). All the Doc's were gone by then so there was no one around to change or stop either drug but she did get permission to give her some Benadryl to try and help.(Dr. Ross never did come back by that night which we took to mean he really did think she was fine). Anyway they gave her the Bendaryl around 11:00pm, she went to sleep and Chris left. She slept for about an hour and a half, woke up screaming about "The GUY!" and would not go back to sleep. I walked the halls with her until 3:30 when I started seriously thinking about jumping on the stroller with her and pushing us both down the stairs. Instead I decided to call Chris. He came and we finally got her to go back to sleep at 5:00 and he went back to RM house. He had just left and I had gotten out of her bed and into mine when our roommate and her mother woke up. They turned on the light, each went to the bathroom and had a little conversation. I was lying there thinking surely any second they are going to turn off the light and go back to bed. They didn't. The Mom decided to go have a smoke. I got up went to the bathroom, explained to Nicole that Anna had just gotten to sleep (apparently they hadn't heard her at all) and asked her to turn off the light. She did and we both slept for two whole hours before Anna was up again. The nurse specialist came in shortly after we got up and I explained to her how Anna had not leaked since 3:00pm, had been up all night (they think she had a negative reaction to the Benadryl) and was scared to go into our room, could she please release us to RM House. She got right on it, her cardiologist came and stopped the Aldatazide, signed the release papers and by 11:00 we were at RM house an Anna was finally sleeping. She slept until 3:00pm. We got dressed and drove to the store to get diapers. She fell asleep on the way so we drove around another two hours and just let her sleep. We came home, had supper and she slept the entire night. Tuesday night she looked really horrible, her eyes were all red and sunken in with big circles under them but by Wednesday morning she was looking much better. We spent the day relaxing and playing in the playroom and playhouse at RM house. In the afternoon we took her to a pet store where she got to pet a bunny and watch some really playful kittens. They also had a lot of fish and when we went near them she said "OOOH STINK" and covered her nose which was too cute. We also were looking at the birds and I read the price tag for one, I said, 'Wow, 1800 bucks". And she repeated 1800 BUCK! it was very cute. After that we went back to the house and had a good sleep. We went back to the hospital at 9:00am.

They had been concerned before we left because her platelets had really dropped post-op which they attributed to being on bypass but they wanted to do another blood test to make sure. She is such a hard poke they decided to just poke her finger and get the blood that way. IT still took forever to get enough blood out but she didn't get quite as upset as when they tried to draw it from a vein. Once again Chris had her laughing through most of it. I don't know what I would do without him. After the blood we went for an x-ray. The platelet count came back great but the x-ray was not so good. The right side seemed to still have quite a bit of fluid and they thought her diaphragm might not be working on that side so we needed to have an ultrasound. Apparently you usually can't get an ultrasound on short notice but one of the nurses went down personally and talked to someone and was able to get us in. Up until this point Anna had been very co-operative about everything. But as soon as we walked into the ultrasound room she started crying. The tech said , 'I don't think she is going to calm down" and I believe he was going to suggest having her sedated but we interupted him and said she may not be happy but she will be still. She was. She cried and fussed a bit but he was able to get everything he needed as was his boss who came in after him( I don't know why they both couldn't have been in there to begin with). Anyway he confirmed that there was fluid in her chest but not a lot and the right side of her diaphragm was not moving as well as the left but it was moving.He asked her if she wanted some stickers to which she screamed NOOOO!. Everyone gave her stickers and then poked or jabbed or did something to her so I guess she has started relating stickers to pain. Anyway,he wrote us a quick note and sent us back up to cardiology We held our breath as Dr. Dyck read the note, took one finally look at the x-ray , and said we could go. WHOOO HOOO!!!!!!! I guess as long as the diaphragm is moving a bit it will repair itself. The fluid he said was not too much of a concern, we just need to keep her moving and she should get rid of it on her own. We took a quick picture of the three of us in the hallway on our way out. I wanted to get one of her in front of the hospital sign but Chris was having a fit to get going and get out there before they changed their mind. We went back to RM house, fed her some soup, cleaned our room and hit the road. We made it to Saskatoon around 6:30pm. She was so excited to see the kids again. All the relatives were there waiting for us and had planned a small celebration to welcome us home. There was tons of great food and even presents. We left at 1:00pm yesterday and finally arrived home about 3:30pm. Anna has been rather clingy and a bit grumpy but she slept great and I am sure it won't be long until she is back to her normal always happy self. Hopefully she is young enough that she won't remember too much of the last two weeks . I don't think Chris and I will ever really get over it, we are just so glad to have this hopefully last big thing behind us. She is just so incredibly precious to us and we can't believe how blessed we are to get to keep her .

One last little aside to the story just to point out to those of you who don't know him how incredible my husband is. I guess when he came to the hospital Monday morning and I showed him all the fluid leaking from Anna's chest and he saw that all the bandages were removed he actually thought I had removed them and that was the reason she was leaking. He said he kept thinking to himself "Didn't Pam hear them when they said we should just leave the tape over her incision until it came off itself, what the heck was she thinking." For those of you that don't know me that well I am a bit of a cleaning nut and as soon as they said anything could come off her I would keep picking at it until I got it all cleaned off. He thought I couldn't stand it anymore and had decided on my own just to clean everything off her. He said he was saying to himself "I have got to be supportive" but he was so ticked at me he just couldn't believe it. It was until sometime later in the day that I said something about how upset she had been when the Three doctors had come in and removed the bandages that he realized it wasn't me that had done it. I am one lucky girl to have someone who would love me enough to still be nice to be even though he was thinking her leaking all over the place was my doing. By the way for those of you that are curious she ended up losing about 240 ml (almost a cup of fluid). Dr. Ross told us that if she hadn't leaked it from the incision site that much fluid combined with what is still in there would have meant they likely would have had to go back in and put in another chest tube. Which would have meant basically starting all over again. So even though it was a totally freaky experience for us as parents it ended up being a positive thing.

Pam and Anna (2 years 2 months)TA, HRV,ASD, 2 VSD's Glenn. Aug.11, 2001, Lateral Tunnel Fontan July 23, 2003 - POST FONTAN WHOO HOO!!!!!!!!