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Friday, January 29, 2010


I've known about hemophilia all my life.

I don't remember how I knew what it was, but I did. As a child, I figured that only Caucasians could be hemophiliacs. I also envisioned that hemophiliac children were sickly pale because they lost so much blood from paper cuts. I didn't know jack squat about spontaneous bleeds or how hemophilia could be deadly. As far as I was concerned, they (hemophiliacs) might as well have been mythical creatures. I simply thought they didn't exist in real life, only in books.

But believe it or not, I knew more about hemophilia than other people.

John had no idea what hemophilia was. So, when I uttered "This could mean he has hemophilia" to John that fateful day, he didn't even know what I was talking about. There are so many people like him too! People in the medical profession even. Believe it or not, I've been asked "What is hemophilia anyway?" by people wearing scrubs. "I never knew it existed" is what John told me once.

And there are still people in this world that have never heard the word "hemophilia" before.

Never in my wildest dreams would I have guessed that the blood I had coursing though my veins carried the FVII Deficiency gene. It it has been almost two years since we learned that our den had a direct link to the bleeding disorders community. Hemophiliacs weren't mythical creatures after all. After Ethan passed away, we thought that this link was only meant to be short-lived. Then almost exactly one year later -- two hours after Annika was born -- we learned once again that our family was going to be a part of the community, but this time it was going to for good.

We were terrified and we thought she would die. And oh how we feared the unknown! We felt guilty because we produced another child that would "suffer". At the same time, we felt blessed because our link to "Holland" was here to stay.We grieved briefly for the normalcy that was never meant to be and marched onward to the trip that lay ahead.

What an adventure these past two years have been.

You read all about where this road has taken. Two years ago I learned that there is "no footprint so small that it cannot make an imprint on this world". Ethan left such a beautiful legacy behind with every breath that Niki takes. My two special little bleeders have taught me a lot about myself. This blog has taught me a lot about you too. You've laughed and cried with me with every step that our family has taken. Every message, every comment, and every question has made an imprint on my heart and I am absolutely humbled by how much you all care about our little corner of the universe.

Thank you for wanting to walk with us.

If you will, walk with us some more. I wrote last month that I was serving as a Team Captain for our local chapter's Hemophilia Walk in April. Please take a moment to donate or join our team's fund raising efforts by walking or sharing the information to our team's website. I've set the goal high, ($7,000 to be exact. Can you guess why? Lol.) but I have no doubt in my mind that we can reach this goal if we all join forces.

So, walk with me and let's make some footprints together. :)

Support Lucky Factor Seven and visit our team web page to learn more.

Wednesday, January 27, 2010

More Therapy...


I just went back and read my post from yesterday afternoon and....WOW. It was totally not like me to be so upfront about my feelings. However, I feel so much better now that it's off my chest. Some of those things have been bugging me for years. I must say that was the most therapeutic entry that I've written in a while.

Anything beats a long update post, huh?

I know that particular entry struck a chord with a few people because I had a lot of in-basket messages today. And, for those of you that approached me (whether directly or indirectly), I know it took a lot of cojones to do that.

So, I commend you. (LOL...but I'm still not telling which one of you are "guilty".)

No matter how much a person can piss me off or hurt my feelings, I'm a softy. Ultimately I just want to have a peaceful existence, ya know? I don't want anyone talking about me, my family, or my cubs. I'm no stranger to the not-so-nice things that people have said about us. My first reaction is to get mad and think "Well, f*ck 'em all then", but at the end of the day that's just not me.

I care what people say.

If someone directs negativity toward me or my family, I mentally dissect it so I can figure out why. It's hard for me to let go. As far as I'm concerned, my "wouldn't-say-boo-to-a-goose mentality" means that I've never *intentionally* done anything to hurt anyone. And if you beg to differ, please let me know because I don't want to be a hypocrite. I like to learn from my mistakes. I've thought long and hard about why people do hurtful sh*t when life is so short. Sometimes, people do or say things that they don't realize will be hurtful to others. I believe in the goodness of man and I like to think that people aren't intentionally malicious.

With the exception of the people that have intentionally said some insensitive things about Niki. May God have mercy on your soul.

Nevertheless I've forgiven, but not wholeheartedly. And I say this because honesty is the best policy. Lord knows I want to completely forgive, but it's hard. Hurt can last forever no matter how much you want it to go away. Some things can't be taken back or manipulated to make it better. Despite everything -- regardless of all the "hurt"-- I still treat people the way I want to be treated in hopes that I'll have some reciprocity.

And maybe one day that reciprocated consideration can blossom into genuine friendship.

Take this post as a simple reminder whenever you feel the urge to be unkind to not just me, but anyone in your life. Lord knows that people don't want to be mean, but things like jealousy, past grudges, insecurity, or even envy get in the way. Do unto others as you want others to do unto you. If I haven't done anything unkind to you, then please don't be unkind to me. In my life, some people have forgotten all about the Golden Rule.

It's simple, but often forgotten in all aspects of our lives.

I feel relieved now that my feelings are out in the open, but at the same time I feel...nude. I'm mentally nude right now people!!! This is uncomfortable for me because not too many people are allowed to see this side of me. I feel fine being in my "birthday suit" when I'm showering alone or in the --ahem-- intimate company of my "Honey Bunches of Oats", but I'm sure as hell NOT signing up for a "mental nudist colony" any time soon. Nudity ain't always pretty, but we should all try it once in a while. I've said my piece and I'm leaving it at that.

Now quick, someone get me a towel so I can cover up!!! :)

Tuesday, January 26, 2010

You Can't Please Everybody...

You know what I hate about having parties?

You can't please everybody!

I've been working on the seating arrangements for Niki's party and I'm officially stressed out. Configuring seating has taken way too much energy from me because I simply CAN'T please everybody.

But, oh boy do I try!

In case you didn't know, there are a handful of people in my life that I genuinely care about, but those same people can't stand each other. Ah, the joys of being neutral! I hate it, but it's true. The fact that certain people in my life don't like each other has posed a HUGE problem for me because I don't want anyone to feel uncomfortable during Niki's party. Frankly, I also don't want to witness or hear of any childish bullsh*t during Niki's special day. John says I should just put all the people that don't like each other in the same table so people will be forced to just get over themselves. Ha, if only it were that easy! Honestly, I'm thisclose to doing it because I'm so sick of worrying about the seating arrangements. But...I'm not cut-throat like John, so I'm trying my hardest to keep everyone content.

It is supposed to be a day of celebration, but all I can think about is how the hell I'm going to avoid making people unhappy so I can have peace.

I'm a "people pleaser" so I'm not satisfied unless everyone else is happy. Never mind the fact that I'm unhappy right now because people can't just grow the hell up and leave the past behind them. In fact, some of those people don't even have enough courtesy to be considerate of my feelings by keeping their negativity to themselves. It's no secret that certain people don't like one another, but I really don't need to see or hear about it. But...don't get me wrong because I'm human and I have the same ugly skeletons in my closet too.

I'm nice as hell, but there are certain people that rub me the wrong way.

We are all entitled to dislike a person every once in a while. I can only speak for myself ('cause this is my blog) but, I can honestly say that I've NEVER disliked a person just because I felt like it. If I don't like someone it's because they did something to me first. Don't mess with me or my family and I won't mess with you. Simple. I am far from "holier than thou" and I too have difficulty completely forgetting past wrongs against me.

However, the difference is I don't drag side parties into my negativity.

Yeah, I vent to blow off steam. Who the hell doesn't? However, I only share what few negative thoughts I have swimming around in my silly head with people who hold the same contempt as I do. I don't try to recruit people to join my way of thinking nor do I make it a point to plan malicious sneak attacks to piss people off.

We've all watched Mean Girls. Believe it or not Regina George could be male, female, young, and old.

I'm not clueless and I've been thinking A LOT about all of the potential petty bullsh*t that could ensue at Niki's party. This has happened at numerous parties and get-togethers that John and I have held in the past -- even Ethan's funeral for crying out loud!

You may think you have an idea about who I'm talking about, but you have no idea.

I'm not calling anyone out, I'm just getting these thoughts out of my system. It has been eating away at me. All of this "hidden drama" is happening all over the place (between family and family - friends and friends - young and old - male and female) and I am EFFING stressed out about it!!! My head hurts.

I'm unbiased by nature, but this neutrality has been a blessing and curse at the same time.

I can't change my family, friends, or acquaintances no more than I can change how certain people feel about me or each other. What I love about "making nice" with everybody is that it gives me the opportunity to appreciate the same things that they don't like about one another. Am I making sense? Everyone is a nice person if you allow yourself to see the goodness in them.

Can I get a Kumbaya?

What I despise about being neutral is the fact that it means some people get ticked off because I don't pick favorites. There is ALWAYS a common denominator when people don't get along. It's just that people often lose sight of the REASON why they didn't like each other in the first place.
I always give people the benefit of the doubt unless they give me reason not to. I've already heard everyone's version of the same story and I've made my own assessments. I honestly believe that if prior drama weren't involved, then the same people that dislike each other would've actually gotten along great. If only they had the opportunity to get to know one another then we could have all gone out for coffee, I swear!

It's simple magnetism, really.

Likes always repel likes and opposites attract. Perhaps certain people don't like each other because they're alike in some way, too. On the flip side, just because you associate yourself with a certain group of people doesn't mean that you're like them. Keep in mind that opposites attact and allow me to use myself as an example. I'm close with friends/family/aquaintances because of the gifts that their unique personalities bring into my life, not because we're clones of one another. Being closer with someone doesn't automatically mean that I think that person can do no wrong. I had a very personal experience with this a few years ago and it sucked. I really don't like it when people make inaccurate judgments about me.

But...who does?

I know that it's easy to succumb to the temptation to talk crap. In fact, it's hard for me to forget and fully forgive when someone has wronged against me. We're all this way and anyone who says they aren't is a damn liar....or Mother Theresa. It takes a very big person to "let go and let God" and I'll be the first to admit that I'm far from that inner strength. However, I do believe that way too much time and energy is spent campaigning against people that we don't like.

Why can't we follow Rodney King's example? Can't we all just get along?!

I'm tired of worrying about how a few people who I love (but dislike each other) could potentially spoil my daughter's beautiful day. That type of negative energy has NO BUSINESS at her party. Niki has beat all the odds and she's alive when science says she shouldn't be. We're having this huge party, not to show off or because we have money to burn, but because of what Niki has overcome in the past year.

Have you not been reading my blog? Why not celebrate it that we made it this far?

She has had it rough -- as a family we've had it rough -- and I'll be damned if I'm going to allow petty drama to ruin this day. Allow us (my family) the opportunity to celebrate what Ethan's passing has helped Niki accomplish, too. She wouldn't even be here if it weren't for him.

February 13th is supposed to be a happy day and now that I've gotten this off of my chest, I'm DONE stressing off of other people's drama.

I have a lot of friends, family, acquaintances that read this blog. Some might be guilty of what I'm talking about while others are not. So, for those that feel that the contents of this blog has affected them in any way, perhaps you are who I'm speaking of. Please be considerate of my feelings and follow my advice....

Let's leave the negative energy at home that day, shall we?

I'm not mad and I most definitely don't blame you for feeling the way you do, but Niki's party is not the time nor the place to talk crap about one another. You are entitled to your opinion, but I'd like you to leave that opinion at home. I don't want to see eye-rolling, whispering, or hear comments about how you can't stand blah-blah-blah-yackety-shmackety. We can do that privately some other time, k? Over coffee perhaps? I'm always open to hearing you out.

For that day, let's just be merry, drink tea, and celebrate Niki's milestone. :)

Since I'm in the mood to be so open about my thoughts, there's one other thing that's been burning a hole in my heart....

If any "frenemies" of mine (and I know of a few out there) happen to stumble upon this blog entry please, let go of whatever it is that irritates you about me. Oh dear readers, you might even know a "frenemy" of mine, too.

Socrates once said, "Well, if what you want to tell me is neither True nor Good nor even Useful, why tell it to me at all?".

Read the Test of Three if you want have some useful insight on how to handle rumors. My very good co-worker/friend told me about this story and I absolutely loved it. Yeah, Socrates is pretty awesome.

I guess what I'm trying to say is that if for whatever reason you've heard some random story about me, perhaps you should think twice about what the speaker's intentions are. I've never said a word about them so why do you think they would say anything about me?

Things aren't always as they seem.

To state it simply, I'm nice person and some not-so-nice people have felt the need to subliminally f*ck with me. However, I wouldn't say "boo" to a goose let alone confront a person about why they don't like me. This is my blog, my therapy, my outlet so it's really my only means of getting the word out there. In all honesty, I've got too much stuff going on with my family to be worried so much, but I do. I'm a people pleaser, remember?

All of this petty stuff is a distraction and I'm tired of being distracted.


There are signs everywhere if you keep your mind alert to them. I am a firm believer that our Creator gives us the opportunity to "see" what He has in store for us. There have been many signs that show me that what happened to us was meant to be, but a few in particular stick out:

1. The very first church John and I attended together was Holy Angels. The children's portion of cemetery where Ethan is buried is also called Holy Angels. Perhaps this was a small hint about how our faith would be important in the rough times ahead.

2. After Ethan passed away, I saw ladybugs everywhere (especially at the cemetery) and I couldn't quite figure out why. I gave birth to my very own LadyBug a few months later. The first woman I heard of with FVII-Def started the LadyBugs, LLC -- an awareness group for WWBDs. (Women with Bleeding Disorders)

3. Ethan was born 6:02am. I miscarried my first child on 6/2/2000. A doctor from Stanford theorized that I lost the first baby because he/she (we think he) could have been even more severely FVII-Def than Ethan and Niki. Our first cub and Ethan ultimately were both "incompatible with life".

4. Niki came home from the NICU on on 3/5/2009. Ethan was buried on 3/4/2008. We were cloaked in darkness in the year following his death and up until Niki came home. Sunshine had re-entered our lives one full year after the sunshine left.

5. Niki was born on Ethan's due date 2/16, but was due on Ethan's birth date 2/20. She entered our arms exactly one year after we expected him to enter ours. Maybe the fact that she was due on 2/20 meant that she and Ethan were going to be "twins" when it came to their condition.

All of these signs were more than coincidences. Ever since I realized how these small facts intertwined, I've tried to keep my eyes open. God is constantly speaking with us, we just have to pay attention.

Monday, January 25, 2010


The Golden Gate Regional Center gave me this story after we found out that Anthony had severe speech delay. It was a scary time for us, but most of you all know that Anthony's speech is doing quite well these days. I would have never guessed how valuable this story would be just a few years later. I've heard people tell me many times "I don't know how you do it" whenever we discuss Ethan's passing and Niki's condition.

All I can say is being in our shoes ain't so bad. Everyone goes to Italy, but "Holland" is absolutely beautiful.

by Emily Perl Kingsley.

I am often asked to describe the experience of raising a child with a disability - to try to help people who have not shared that unique experience to understand it, to imagine how it would feel. It's like this......

When you're going to have a baby, it's like planning a fabulous vacation trip - to Italy. You buy a bunch of guide books and make your wonderful plans. The Coliseum. The Michelangelo David. The gondolas in Venice. You may learn some handy phrases in Italian. It's all very exciting.

After months of eager anticipation, the day finally arrives. You pack your bags and off you go. Several hours later, the plane lands. The stewardess comes in and says, "Welcome to Holland."

"Holland?!?" you say. "What do you mean Holland?? I signed up for Italy! I'm supposed to be in Italy. All my life I've dreamed of going to Italy."

But there's been a change in the flight plan. They've landed in Holland and there you must stay.

The important thing is that they haven't taken you to a horrible, disgusting, filthy place, full of pestilence, famine and disease. It's just a different place.

So you must go out and buy new guide books. And you must learn a whole new language. And you will meet a whole new group of people you would never have met.

It's just a different place. It's slower-paced than Italy, less flashy than Italy. But after you've been there for a while and you catch your breath, you look around.... and you begin to notice that Holland has windmills....and Holland has tulips. Holland even has Rembrandts.

But everyone you know is busy coming and going from Italy... and they're all bragging about what a wonderful time they had there. And for the rest of your life, you will say "Yes, that's where I was supposed to go. That's what I had planned."

And the pain of that will never, ever, ever, ever go away... because the loss of that dream is a very very significant loss.

But... if you spend your life mourning the fact that you didn't get to Italy, you may never be free to enjoy the very special, the very lovely things ... about Holland.

Sunday, January 24, 2010

2010: The Bloody Shoulders Update

Happy New Year, ya'll! It's only been 24 days into 2010 and our den has already encountered mounds of things to keep us busy. So, here's another long post for your reading enjoyment or dismay. ;-)

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.
My resolutions?
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

Niki looking at her silly big brothers

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.

Niki at my little SIL's birthday party
Exhibit A: John and I having too much fun. He NEVER smiles and I NEVER throw up" deuces".
I blame it on the a-a-a-a-alcohol!
At 5am my sister-in-law (who was watching Niki for the evening) woke me up to inform me that Niki felt very hot. Lo and behold her temperature was 102.1!!! John's "fun" caught up with him so he was pretty much out of commission. I had to perform the arduous task of getting my barely-sober-self together so I could drive to Kaiser SF's Emergency Room. My SIL came with me to help.

My eldest SIL hiding behind Niki's lion

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.

She was not a happy girl

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...

Niki smiling happily after listening to Wondergirls...
That was the billionth shirt she changed into since her nose started bleeding
We played the waiting game. We changed her shirt again! Her nose was still bleeding but it was starting to slow down. One of the nurses came into our room with towels and warm water. Then he started asking us all sorts of questions about Niki. Apparently, he was very fascinated by her case and just needed to feed his curiosity. Girl bleeders are rare. I am all about educating the masses and dispelling myths so I appreciated that he was so interested even if it was just to gawk at her.

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 and Daddy waiting @ Dr. Awesome's office
In the exam room

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.