Onto the recap!
December 31, 2009 - I was unusually somber that evening. We went to my mother's house for a Pajama Party to ring in the new year, but I was feeling "off". I felt the same way last year too. Can you believe it has been almost TWO YEARS since Ethan passed away? That means we've spent two holiday seasons without him. That night I missed everything that wasn't meant to be. You'd think that I'd be excited since this was Niki's first NYE, but I was just plain sad. Nevertheless, I tried to perk myself up and managed to ring in the 2010 in better spirits.
1. To increase my self study habits. (In case I can return to school in October.)
2. To be more positive by ignoring any negative energy around me or my family.
3. To take better care of my health. (Take my asthma meds consistently 'cause I don't want some other b*tch raising my cubs. Lol!)
4. To be more a more patient mother and pseudo-wife. ('Cause having a head full of stress-induced white hairs ain't a good look.)
5. To blog more. (But I obviously broke this resolution already so I've added "from now on" to it. LOL!
Epin Lion Lion wearing a toot-ta-root from the PI
January 1, 2010 - The events from the night before had us absolutely pooped. Our entire den slept in and I spent most of my day churning out invitations for Niki's party. Here's sneak peek.
Saturday/Sunday, January 2/3, 2010 - It was my goddaughter/little sister-in-law's 10th birthday party. My MIL put together a cute little party and as always, we ate all day long. Later that night was my bro-in-law's birthday bash at a new club in SF. To be quite honest, John and I haven't gone out much since Niki was born so it was nice to have the opportunity to finally get out and let loose. We had a little too much fun, I think. John had much more fun he originally anticipated so I had to drive home. We got home and fell asleep a little before 2am.
I blame it on the a-a-a-a-alcohol!
I contacted the on-call Pediatric Hematologist so he could inform the ER that we were coming. When we arrived, the nurse Niki had on Christmas Eve/Day was working triage. (I'll call her Nurse Stat.) So, Niki got the VIP treatment and was immediately put in the room. Unfortunately, the nurse that took over wasn't the greatest in the world. Don't get me wrong....she was nice, but I only had about 80% confidence in her ability to care for Niki.
Call it Mother's instinct, but I was right. More on that later...
As always, they drew blood cultures and started infusing antibiotics (Ceftriaxone). They also performed a nasal swab to check for RSV since she never quite recuperated from the flu. Her nose was already runny and I noticed that her clear snot became very lightly pink-tinged after the swab. I didn't think anything of it because she had a nasal swab done when she had the flu and didn't encounter any problems/bleeds.
The ER visit was very "run of the mill" because unfortunately, we're getting used to this shit. We were in a room by 6am and got discharged by 12:30pm. Aside from the blood cultures to see if the line was infected, an antibiotic infusion, and a RSV swab, Niki also ended up having IV fluids because her heart rate was a fast. Her nurse was really starting to tick me off because she erroneously told us that we were going to be discharged and unhooked the line. I thought her pulse was a little alarming, but I tried to be patient and let the nurse do her job. Then when the doctor came in to give us our discharge instructions she asked the nurse where Niki's last pulse rate was. Apparently, she didn't document it so the nurse then scrambled to get Niki hooked up to check her pulse.
Thank God the doctor was on top of his game.
Although she was still very nice, she clearly didn't take Niki's condition seriously enough. IFirst off, the alarms on the pump kept beeping like crazy because there was tons of air in the line! We had to call her twice (for the antibiotics and fluids) so she could take the air out. Second, the nurse didn't screw the line onto her Broviac correctly because it started leaking all over the place.
I had to unscrew and fix it myself.
I'm not normally such a harsh critic, but when it comes to my daughter (or any of my cubs for that matter) I have high expectations. Besides, I also had a very mild hangover so I was cranky and wasn't on top of my game that day. It would come to haunt me a few hours later...
Fast forward to 7pm that night and Niki's nose suddenly started gushing blood. I immediately sprung into action and grabbed her Nosebudd (one of the many freebies we got from the Conference) so I could put cool pressure on her nose. John was at work so I called him to come home immedfidately. Everyone was panicking. There was blood everywhere! My MIL and SIL helped me hold down poor Niki (who was screaming and crying) so I could apply pressure. 15 minutes passed and Niki was crying and fighting with us the whole time. When I removed the Nosebudd the bleeding appeared to stop, but a few seconds later it started to bleed again. I called the on-call Hematologist (I'll call him Dr. Grumpy) and he me advised me to give as much direct pressure as Niki would allow. The conversation was short, but he instructed me to call him back if the bleeding didn't stop within the hour. It was a factor day.
Too much pressure would dislodge any clots that were starting to form so we pretty much had to let her bleed. We were both soaked in blood.
ONE HOUR passed and the bleeding still didn't stop. There was blood everywhere--all over Niki, all over several towels, and all over my shoulders. I called Dr. Grumpy again and he gave me the go ahead to administer factor. You may be wondering why I didn't give her factor in the first place, but nosebleeds clot differently that a "regular bleed" anyway. Extra factor may or may not have worked to stop the bleeding.
Her NovoSeven is close to $5,000 a vial so this was one expensive nosebleed!
I mixed her factor and was just about to administer it when the unthinkable happened... I couldn't push anything in! I couldn't even pull back fluid. This meant that the line was f*cking clotted!!!! I couldn't force the factor in because too much "push" could create a hole her Broviac. This my friends, is NO BUENO. Niki's first Broviac was defective and had a hole in it. They had to cut her open again to replace it less than 48 hours after her first surgery. This type of surgery isn't a big deal for a "normal person", but any type of surgery for a person with a bleeding disorder is a HUGE deal.
You may be wondering how her blood could have possibly clotted the Broviac when she has a bleeding disorder. Well, the answer is really ironic. The one thing we DON'T want to clot (her Broviac) can clot very easily while the rest of her body has difficulty clotting.
Get ready for some heavy duty medical info...
When Niki was first born, Dr. T (her NICU doctor) and Dr. Awesome set a "No Heparin" rule. Heparin is a BLOOD THINNER that is normally placed in central lines to prevent clotting whenever the line isn't in use. This is also called patency or keeping the line patent. When they had the "No Heparin" rule, Niki was on IV fluids around the clock to prevent clotting in her Broviac. She was always tethered down by something in the NICU. Initially, it made sense though.
Why would you give a blood thinner to a person with a bleeding disorder?
That rule changed after they consulted with a specialist at CHOC. You see, Niki was/is a very complicated case and even though Dr. Awesome learned a lot from Ethan, she was still consulting with Hematologists all over the nation as she developed a plan of care for Niki. So, after Niki had her second Broviac installed they decided that Heparin (Hep-Lock) was okay as long as it was given in small amounts.
We've been HepLock-ing since Niki was 3 weeks old. Keep in mind that using a Hep-Lock (Heparin) is standard protocol for patients with central lines.
But I digress, the fact that her line was CLOTTED told me that the nurse did follow standard protocol. The nurse probably didn't Hep-Lock after Niki finished her IV fluids in the ER otherwise the line would have stayed patent. I was out of it that morning so I didn't notice and I was kicking myself in the head for going out the night before. Niki's baby bag is a "mobile hemophilia unit" and I always carry factor and Heparin with me. Ordering factor or Heparin from the hospital pharmacy can take hours so it's just easier for us to have some on hand.
I always inform the nursing staff that I have Heparin if they need it. And they've always used our personal stock in the past. Earlier that day I informed Niki's nurse about the Heparin I had on hand. When Niki was about to be discharged that same nurse was the one who disconnected the line. I was utterly exhausted so when her nurse didn't ask me for Heparin, I figured she had some already. I was holding Niki while she was disconnecting the tubing. This oversight proved to be detrimental.
I mean, why wouldn't she follow standard protocol and Hep-Lock? But, she didn't.
Ultimately, I blamed myself for missing the fact that the nurse didn't use Heparin. If I didn't go out the night before, I would have been more alert. The ER was stressful, Niki was crying, and I was distracted. When I realized that the line was clotted and I couldn't give her factor, I really started to freak out. I called Dr. Grumpy again to tell him what was going on and he pretty much told me that we would have to wait until the morning to be seen. If the line was indeed clotted, then they would use TPA to break down the clot. That plan didn't sit well with me because she was still gushing blood, but he was the doctor and I didn't want to argue.
I felt utterly helpless and my MIL saw that I was about lose my composure. I cry when I'm stressed.
There was just so much blood. I desperately wanted Niki to stop bleeding, but there was nothing I could do. Poor Niki looked awful, but she was fine as long as we weren't putting pressure on her nose. All of our shoulders were really bloody. I didn't want to hinder any clots from forming so we left her nose alone. I called John crying like a blubbering fool to update him on my conversation with Dr. Grumpy. She was bleeding for close to TWO HOURS by that time. Luckily, John was only about 10 minutes away from home so when he got there he tried to access the line.
But still, no luck!
This time John called Dr. Grump and got a better response. (Maybe because he's a dude and his voice is scary? Who knows...) Anyway, he told us to take Niki to SF Kaiser so they could administer factor through a PIV . They would also evaluate Niki's Broviac for clotting and start TPA if needed.
John's little brother came with us to help.
When we got there, the same triage nurse (Nurse Stat) was there working a double. Again, Niki got the VIP treatment and was immediately put into a room. Luckily, the nurse who screwed up earlier that day had already gone home. A different person was taking care of Niki and she was much better. There were a total of three nurses and one ER doctor in the room with us. One of the nurses started man-handling the Broviac and I began to worry that he would create a hole. He screwed on a saline flush and began pushing and pulling so much that the veins in his arms started to bulge out! Niki was crying the whole time, but it wasn't because she was in pain. She just wanted to be held. A few minutes of pushing and pulling passed, but the line was still clotted. I started to think that they were going to have to do a PIV when...
...the nurse pulled back one last time and a blood appeared in the saline! He unclogged it!
The nurses started to ask me how to reconstitute the NovoSeven, but I didn't have time to explain. I nicely told them that I would do it instead. I mixed her factor as the nurses and ER doctor watched. Now, normally I would feel very self-conscious if "medical people" were watching me, but I was way too focused on hoping that the Novo would stop the nosebleed. Once the factor was in, John and I finally let out a sigh of relief. We changed Niki's shirt for the billionth time that day and tried to calm her down by playing Wondergirls on John's Blackberry.
She half-heartedly danced, smiled, and looked so sweet despite her bloody nose. Even the ER doctor said so. :) My BIL took this picture right before we cleaned her up. We document EVERYTHING about her life and what she's gone through so far. Maybe she'll appreciate it later when she gets older. Or not. We'll just have to wait and see...
Most people don't ask us questions. ( I think it's because they're afraid they'll offend us. I say, ask away!)
The bleeding slowly, but surely subsided. It completely stopped about 45 minutes after we administered the NovoSeven....THANK GOD! They discharged Niki and instructed us to follow up with Hematology in the morning so she could get her second dose of antibiotics. We went home and got some much needed rest. We also pretty much left her nose alone after that point. We didn't want to dislodge the clot and have the bleeding start over again. Eventually, the bloody boogers stopped bothering me.
Monday, January 4, 2010 - At around 5:30 am we got a phone call from a Call Center MD. Apparently, they received a critical lab result showing that Niki had a line infection! Her blood culture showed "gram positive rods" which could be the reason why she developed a high fever. Or so they thought. The Call Center RN contacted the Dr. Grumpy who was still on-call (after I advised her to) so he would be aware of the result. We were then instructed to report to Dr. Awesome's office at 8:30am so they could draw more blood cultures to confirm the results and give her another dose of Ceftriaxone. Naturally, we were worried.
A line infection is dangerous and usually translates to a one week stay in the hospital.
Dr. Grumpy was good, but he wasn't very pleasant to speak with and I felt like we annoyed him because we called him so much in the past 24 hours. I felt super relieved when we finally got to see Dr. Awesome. But...there was more bad news. Niki's RSV swab came back positive! In case you didn't know, RSV is some serious shit. Niki has what Dr. Awesome calls a "depressed immune system" because she has a Broviac. So, aside from worrying about a potential line infection, we had to worry about how the RSV would affect Niki.
But...I don't call her Dr. Awesome for nothing.
Dr. Awesome is very up-to-date and informed me that she recently read a European article about some radical new treatment options for immunocompromised patients. She decided that the plan would be to draw a second round of blood cultures to see if any more bacteria would grow, administer one more dose of Ceftriaxone (to fight any bacteria that may have been brewing in the line), and to start her on oral antibiotics (Clindamycin) to hopefully knock out the RSV.
Niki got her Cefriaxone infusion right there at Dr. Awesome's office. It was oh-so-nice to see Niki get treated by people who were familiar with her condition. Everything went smoothly with her infusion and they even remembered the Hep-Lock. :) I stayed home for a few days to take care of Niki. We were also waiting for the results of her second blood culture to come back. If it grew more bacteria in the next couple of days then she was going to be admitted to Oakland Kaiser's PICU.
By Monday afternoon, all THREE cubs were on oral antibiotics. Anthony developed an ear infection so Dr. Sweetheart started him on Amoxicillin, Kevin was taking Keflex for some dental work that he was going to have, and Niki was on Clindamycin. Twice a day the kids lined up for a medication administration assembly line. Fun, fun, fun!
Thankfully, bacteria didn't grow in the second culture (the first dose of antibiotics must have done its job) and I was able to return to work by Thursday. All of the cubs were completely off antibiotics by 1/13 and I am happy to report that they are ALL perfectly healthy as of this date.
After all of the medical stuff died down, I've been keeping myself busy with party planning for Niki's 1st Birthday. Our baby girl has beat the odds so we're throwing a celebrating fit for a Queen. This dang party has been excitingly stressful to plan. My computer also decided to crash which is another reason why I haven't blogged in a while. Luckily, my mom is lending me her laptop until mine gets fixed.
I desperately needed some blog-therapy. :)
In more recent news, Niki saw Dr. Awesome on Monday 1/20. Niki has some residual diarrhea (a side effect of the Clindamycin) so I was given the super fun task of scraping poop off of her diaper so Dr. Awesome could rule out some other underlying thing like C. Difficile. Oh the joys of motherhood! When I finally got around to doing the stool test, the boy cubs witnessed me performing the poop scooping. They held their noses and gagged the entire time. Even I almost lost my lunch. Another thing we discussed at her appointment was her surgery date. The port surgery is set to take place on March 8th. Niki be admitted to Oakland Kaiser on Sunday 3/7 so Dr. Awesome can titrate up on her factor in preparation for the surgery. Port surgery isn't typically a major procedure, but it is for someone with a bleeding disorder. I know I said this earlier, but I've given you guys a LOT of information in the post.
Please bare with my redundancy. :)
If all goes well, Niki will be discharged in one week. Then for two weeks John, Niki, and I will have to go to Dr. Awesome's office every other morning. We'll have port training with Nurse Richard. I'm excited and terrified at the same time. A port will give Niki more freedom, but it also means we're actually going to have to poke her to give her factor. Kinda like this. I hope Niki will be this good when she has her port. :)
Some day, I'll post the home video footage of what we do at home with her Broviac. Some day.