I'm Alright...Nobody Worry About Me

(perhaps a little Kenny Loggins running through your brain). For all my lovely friends who take the time to read my blog, thank you, thank you, thank you...I really do it to get the feelings out, but it does help so much to know that you care.

In particular Roseanne and Cathi always follow up to make sure I am doing o.k., so I just wanted to take a second and let you know that most of the time I really am doing o.k., once in a while even good, and on the odd occasion fantastic. I tend to be motivated to post here when I am having a particularly bad moment (which still does happen, and will probably be accelerating over the holidays). But we are enjoying Annie so much (and Rachel too now that the pain and suffering of finals is over) and I am actually kind of sort of looking forward to Christmas (Happiness with a small side order of dread). Heck, I might even start shopping this week!!!!

There are still some really difficult times, and writing them here allows me a chance to "talk about it" because it has been almost 8 months, so there is some expectation that we be better and people who love us don't want us to be sad, so they can get really uncomfortable if we are...in a way this happened to all of us--that was really the message we got when Sophie died, our friends, our sisters and brothers, our kids, our nieces and nephews, coworkers, etc...this is the unthinkable, unspeakable thing and it sent shockwaves through all our lives. But everyone else didn't have Sophie in their daily lives, and so have been able to move on ---which is good---no it is great, and it is the way it should be. Mike and I, Annie and Rachel, we will change, and things will get different, but we will never "move on" We will live full lives and seek to honor Sophie's memory by remembering that life is too short for "someday" But for as long as I need to or want to, I'll be writing here when things are tough.

I thought it was worth saying that I am doing pretty good and that I value and appreciate every friend, family member, and even stranger who has supported us.

Happy Holidays!

Dr Evil (Note the Capital E)

Oh--I almost forgot to post about our horrible doctor appointment at the National Jewish Immunology Department on Wednesday. All we wanted was a simple blood test of the antibody levels in Annie's blood to see if her body had an immune response to her vaccinations. This is sort of the last check off on the list--and was recommended to us by 3 doctors...well the fellow takes our background information, consults with the doctor, who then proceeds to come in and tell us that we are hysterical and chasing shadows and we need to stop. We explained what we were looking for and he said we already had all the information we needed because we know Annie has a spleen and we know it is functioning (no howell-jollies). We explained about the pit count being elevated and he said that wasn't relevant. He berated me for EVER looking at the Internet...I explained to him that I was able to find quite a bit of relevant information on the internet, and that since he and his colleuges in different areas have such differing opinions, I, as my child's advocate have every right to seek out information. He said the other doctors were making us chase shadows...Mike was so good--asking why we should trust him over all others--he basically (and I am not kidding here) said that he is the most knowledgeable....I find it interesting that the NIH itself (see link above) thinks that the antibody titer test is useful but he doesn't. In the end we determined that the test would not harm Annie and had him do it.

The other recommendation we had was to get Annie a stronger vaccination against Pneumococcus..this came from Dr. Cordero and our pediatrician. Dr. Evil said that that vaccine does nothing for children. The APA and CDC both recommend that vaccine for kids 2-5 who could be high risk.....we were fuming...I have never felt so attached by a doctor...he chalked everything up to us being irrational grieving parents....we kept asking him why we should trust him over the other doctors....and that we didn't follow our gut when it came to Sophie (at least Mike's gut that the hemivertabra was somehow more serious) We have to now, we aren't turning Annie into our science experient by any means...she has had an echo and a spinal e-ray and a few blood tests, but all at the recommendation of doctors....I was happy to point out to Dr, Smarter than Everyone Else, that a simple Google search would have clued in the osteopath that hemivertabrae can be linked to asplenia......And I also got pleasure from telling him that I happen to know of a few parents whose kids have heterotaxy who diagnosed their own children with information they found on the Internet, because their damned doctors wouldn't believe them that something was wrong....look at a woman I know at work whose daugther was born with Congentital Heart Defect....often linked with other abonormalities of the intestines, spleen, liver, etc.....she was over 6 months old before her Mom had to REQUEST an ultrasound of her organs, and they found a kidney problem. So yes, I know doctors aren't perfect and that medicine is an art as well as a science---but I sure as hell wish that some of these doctors would stop pretending they know everything....there is a TON of evidence that they don't.......

We are still waiting on the results of the echo and the x-ray...been over a month now, I assume no news is good news but have put in a call just in case.....In the end we did get the test and the vaccine for Annie

Anger Management

If you are someone who really wants me to be o.k. and thinks I should be better by now....I'm great...so read no further and have a Jolly Holiday!

I have been managing to be very angry lately....It is tough, because I am trying hard not to, but I can't seem to contain it. I am snappish at work and at home and easily frustrated, irritated and pissed off. Thankfully my family is very forgiving.....and what is at the root of this anger? I can't say exactly, I know it is related to Sophie's death, but it I can't quite pinpoint the source. Am I mad that she died, hell yes I am...but that doesn't do any good....Maybe I'm mad because the whole world keeps right on moving along and I want it to stop. I want everyone and everything to come to a screeching halt and think about Sophie and what happened to her and how awful it is...of course I know that is unreasonable, life goes on and I must also, but boy how petty and out of proportion so much of life seems.....If only people could know how little I want to care about egotistical tug-o-war pissing matches at work, about who said what to who about whom, about whether we ever get a renter in our condo.....I hate that I have deal with these things. I just want to wrap myself and my family in a giant down comforter and hide away from everything. Especially Christmas.....I'm like the grinch I wan't to stop Christmas from coming...but rather than take every body else's Christmas and dump it, I would just climb to the top of Mt. Crumpet and stay there.....

At the very least it would be so nice not to have any other stress in our lives right now...If the real estate market would turn and we could dump both our rental properties....neither of us has the energy to be landlords right now and we are going to pay the price for that later....if we didn't have worries other than ourselves and the kids, what a relief that would be. Laundry and a teenager who won't do her homework...that I think I could handle--I'd even give up my cleaning lady! A mountain of real estate rapidly losing value, not so much....hard to deal right now.....

OK...time to put on the happy face and make gingerbread people (how PC)....

Paranoia May Destroy You

Or at least cause your kid to have to spend thousands on therapy as an adult....Generally the fear that Annie will die randomly in the middle of the night has been contained, but I still do have moments. This afternoon we were driving in the car, just Annie and me and she fell asleep hard, for a long time. After a while I tried waking her up and I couldn't...I was reaching back and grabbing her leg, calling her name and she wouldn't wake up. I pulled the car over on the shoulder, right there on I-25, turned around and shook her and she didn't move---I screamed really loud and then she woke up. That can't be good for her, but she isn't a deep car sleeper and I was so scared. If there is an area that I could use help with this is it. I used to generally have faith that if my kids went to sleep they would wake up...now I don't and I need to get it back. All our lives are going to suck if I constantly worry....Annie might have some sleep disorder as an adult because she has been conditioned by me....I don't want that to happen.

Everytime I read something about a parent that has lost more than one child I get so panicky....OK, I'm going to say it...it is a possibility, right?..it could happen to any one of us. But, I can't live my life in fear that I am going to lose one of the other kids. So, if anyone has any advice on how I might deal with this I would love to hear it. I'm not paralyzed, but I sure would like for the thought to cross my mind less often.

The holidays are making me anxious again...I may have to get reacquainted with my friend Ambien. I have pondered why the holidays would be any harder than any other day that I don't have Sophie in my life...the all suck. But I have concluded that gatherings of people really highlight her absence, gift buying brings to mind the things Sophie won't open this year, the mall reminds that she won't get to sit on Santa's lap (although if last year was any indicator she might not have done that anyway), even the damn snow reminds me that she doesn't get to play in it....Every year I get the girls Christmas PJ's and they open and wear them Christmas Eve...I was shopping for those yesterday and just melted down....no cute cuddly PJ's for Soph-Soph....I honestly don't know how I am going to make it through this....the amazing people in my life I guess....although it is tough to keep leaning on all of you, and I know I should be standing on my own more.....

On another note, my dear friend Jennifer is having surgery on Tuesday...for those of you who are the praying type, please include her this week. She's very scared, but she is a tough, brave, chick and I know she will be o.k.

On the brighter side, we do have a decorated tree up in the house and I have bought exactly one present, so progress is being made..... take a look at the cute little video of Sophie's 1st time in the big bathtub!

Ba-Humbug

Well, sort of. I am not anti-Christmas, but just not motivated to get into it this year. Mike and the girls got a tree today so that is a step in the right direction...I think the holidays will be a bit of mixed emotions this year. I have always loved Christmas...giving presents, being with family, peace on earth and all that crap. This year it may just be a reminder of all we lost in 2007 and Sophia's absence will be glaring....I miss her.

We had our last night of grief group last night. I was amazing to see everyone laughing at the end....a wonderful sight and a far cry from the first night when we were all crying before we could even get started. Anyway, I hope we all do stay in touch--we are different people for sure, but we have had the worst thing that could happen happen to us and we are all members of the same crappy club.

Annie really lost it last week....she was yelling and screaming for an hour or more just saying over and over again how hard it is, how her head is filled with bad thoughts, how she wants to play with Sophie but she can't---it isn't fair. Up until recently we have been trying to make her happy whenever she gets sad and missing Soph, the past few weeks we have been trying to let her know that it is fine to be angry, upset, sad sometimes...well she really took that to heart. It was heartbreaking to watch, but I think good for her. She has a fever tonight---I wonder if we will ever get over being terrified everytime she is sick...I'm sure neither of us will get much sleep tonight. She really needs a little sibling to play with...not sure what to do about that, although I know we won't have another one...anyone out there have a baby or two-year old they don't want, drop me a line....seriously, if you are thinking about starving, beating, or doing any other horrible things to your children (Baby Grace...) think again...think there are parents out there who would give anything to have what you are so carelessly throwing away (Whoa..where did that come from)

Ok-that's all for this post....thanks to all the amazing people in our lives (you know who you are)

Cry me a River

Or rather I have been crying myself a river these past two days. I don't know what is going on, but I really haven't been able to get a grip. As a result, Mike and Annie went to his brothers without me yesterday. I think the quiet time has been good and I am feeling somewhat better today. A big problem for me is that I'm not sure exactly what is making me so sad. Could be the holidays, but T-day was actually fun. There is some other stuff going on with friends that is stressful, so maybe that is contributing, but I am starting to feel bewildered again (Did this really happen? How did this happen? Why did this happen? Have I learned ANYTHING from this experience) I have also been angry the past few days....and damn do I miss our little Sophia Noel. You know, that night, that terrible, awful, haunting night, when we put her to bed for the last time, because that is what the doctor told us to do. She didn't look right, her color wasn't good, she was floppy--why the &(*#$@ didn't I know she was slipping away, why didn't my instinct tell me she needed help right then. I mean for fuck sake, how is your child 15-30 minutes away from their last breath and you have no earthly idea. I would give ANYTHING to have that night back again, to do something different. The docs we talked to after said it would have been too late at that point anyway, but how do we really know, do they just say that to try to allieviate some of our guilt, some of theirs.

I've got to get it together, I know.....Annie and Mike deserve better. I'm in a strange place, a bit of a "poor me" place and I really am feeling like no one can quite understand what I am going through (except, of course, Mike, who is wonderful and amazing, and much better than I at holding everything together). I do know I have so much to be thankful for in this life, it's out there on the periphery....Annie, Mike, Rachel, amazingly loving and supportive family and friends, a nice house, our health, etc.....but all of that doesn't replace the gaping hole where Sophie belongs, does it? I can't dwell in the past, can't stop living just because Sophia died, but how do I stop being so sad..........Mike and Annie come home in about 6 hours, I hope I can figure it out by then!!!!

Snowy days and Turkeys always make me cry?

Wow---I wouldn't have thought that Thanksgiving was going to be hard for me, but I was sad the whole week. The day itself was good, lots of family and friends, a wonderful bird, if I do say so myself (alas the much anticipated Turducken never showed...perhaps a light fingered Fex-Ex employee had a wonderful dinner on us....Regardless it was a lovely day. The only crying I did was on a walk I took earlier in the day before our guests arrived...just general missing Sophie sadness....

So much has been going on, I have been wanting to post but just haven't felt the inspiration. Anne Marie had an echocardiogram and a full spinal X-ray last week, just to make sure that she doesn't have any of the other symptoms of Heterotaxy, which the genetics doc seems to think is what is going on with both girls..a mild expression of those genes. I am thankful we are not planning on having any more children, I can't imagine how stressful that pregnancy would be. The doctor thought a 1 in 4 chance of the same thing or worse happening again...yikes. I am hoping Sophie's case will make it into the big Heterotaxy study at Baylor, but I am not hopeful...both kids have "mild" expressions. So little is known about these conditions. I have read that Heterotaxy may account for up to 1% of all newborn deaths...that could be up to 40,000 babies per year in the US alone. I know there is so much competition for research dollars...but this seems huge. I sure hope Annie can get some answers before she had kids (if she has kids). I must get going on the asplenia screen. I would LOVE to hear from other parents whose kids have asplenia but none of the other common symptons...how many are there?

I guess in the end I am thankful that both girls haven't experienced major illness (OK you don't get much sicker than Sophie did...but I mean prolonged) I am thankful that I had Sophie to hold for almost 16 months (plus the 10 months I carried her)...and I am thankful that I have Annie and Mike to love and to hold and to get me through all of this.....Rachel too, it's just that she is being so "teenager" right now (I hear you snickering Mom!)

It's Not All About Us

Today's post is dedicated to someone else's tragedy...a family of children who are suffering in an unimaginable way....A family that I don't know personally but can't stop thinking about. Last week one of my sister's employees did not show up for work. Her son, who also works at the pharmacy, did not show up either...this was strange and everyone was worried. In the end, it turned out that Blia, the Mom, had been killed early that morning in a house fire along with her husband. Thankfully none of the kids, who range in age from 9 to 20, were at home at the time. The fire is currently classified as suspicious and it is thought to be a murder/suicide, but that is still under investigation.

These kids have not only lost both their parents, but also everything they owned in this world, clothes, money, identifying documents, books, toys, bedding...everything is gone. In addition to this tragedy, these kids lost a sibling earlier this year to suicide....it has to be almost too much to bear.

I know I don't have any direct connection to these kids, but I want so badly to do something. I am sure they are overwhelmed right now, just sorting out the details, so there probably is nothing that I can do directly. Instead, I've decided to do everything I can to get the word out that these kids are suffering and they need help.

A fund has been established to collect money for the family's expenses. Please contribute if you can, if not, please pass the information along to someone who might....if you go to church, please ask your church members to pray for these children, to help these kids. Also, if you know of any resources for fire victims that would be helpful too.....

To contribute you can send a check made out to:
Denver Water Credit Union
The Lee Family Memorial Fund
PO Box 40035
Denver CO, 80204

I know you all to be amazing and generous and I beg you to help support this family through this crisis!

Sweet Sophie Blue Eyes

Sophia's amazing blue eyes, but they were amazing, a mile wide and blue as the Mediterranian off the Greek Islands. Mike had a birthday party for me on Friday (40--oh no) and it was great, I had a wonderful time and really enjoyed myself--until the end when I just got really sad and regaled the last few guests with the tale of the death of Sophie--way to leave everyone in a depression, but you know..I really didn't get to talk to too many people about it, what happened, how I felt, etc...so I think it was therapeutic to say everything out loud...but I have been feeling blue ever since. And I told Mike, the really hard part is that I don't want to feel un-blue or better or whatever you call it right now, I just want to crawl into bed with my husband and kids and stay there under the down comforters and not have to deal with all this crap we call life. I don't know, everything just seems so insignificant to me right now. Which I know is bad, I have an amazing family and spectacular friends and am so blessed...some of the other families in our class talk about how they feel like some of their family and friends think they should be over the loss of their children by now or at least shouldn't be talking about it, we don't have that so I am really glad about that. It's just that Soph is gone and she is not coming back, and I am thinking about her, still every day, but not as much as I was, and there is distance growing daily between when we lost her and now (master of the obvious, I know) but it sucks...I can't remember everything about her, I don't have 16 months worth of memories, I just have snapshots, this trip to the zoo, her jumping, holding her, rocking, her, but I don't remember every single little thing.

This morning I put Annie's winter jacket on for the first time (snow yesterday) and the second I got it on her she burst into tears and started crying "I want my baby sister, I miss my baby sister" I'm not sure why and she couldn't verbalize it, but I think it is probably related to one of the pictures we have, which is of Sophie in that jacket, which was 2x too big for her....I'll post it here later, along with a great pic of her eyes.....

I'm going to post her little video on You Tube with a link as well......thanks for all your love and support...I wouldn't survive without you.

You're not in Kansas Anymore!!!

Little by little things are getting better...I went to a party last week and was actually able to tell new people about losing Sophie without totally losing it...I worry about being "the mourning mom" though and that people might think it is an inappropriate thing to talk about... I am learning to be more private with my grief, but I also need to talk about Sophie, I can't pretend she didn't exist, that I didn't just lose her a mere 5 months ago...I told Mike I'm scared about the time when it will be longer that we haven't had her than we did have her (about a year from now). It seems crazy, it's all crazy this new "surreality" as we like to call it.

Mike and I started attending a bereavement support group at Children's Hospital this week. It meets Monday nights. It was very emotional...I started crying when we sat down--before anyone had spoken a word. I was the only one crying for a long time, and that scared me...the group is for parents who have lost a child in the last 18 months, and I know things get better with time, but I had a hard time believing that things got that much better..in time everyone cried as we shared our stories. I guess it is shitty to say that it made me feel better, but it did. It was hard to listen to everyone's story....horrible things are happening to our babies, it is hard to make sense of. The facilitator told us to be sure not to take the burden of anyone else's grief home....Right...no problem...of course I could hear the story of the parents whose little baby got sick and was in the hospital for 4 months--getting sicker and sicker and finally dying and they still don't know what happened--and not cry for them when I cry for me. Of course I can hear the story of the Mom who has lost not 1, not 2, but three beautiful children in the past 3 years and not have my heart break for her...We may come out of this stronger by all being together, but we will be weaker at times knowing the pain and sadness we all bring to the room. I think the group will be a positive experience for Mike and I, but it scares me too...next week we "tell the story of our children" That will be heartbreaking I am sure.

Something that struck me so deep was the parents that do not have answers about what happened to their children. That the doctors would ever stop searching for happened. How can a parent go forward not knowing? Not being able to make the tiniest bit of sense out of such a devesating event? One parent begged her doctor for additional tests, but eventually the doctors just gave up. It all goes back to what the doctors are taught in medical school ---When you are on the prairie and you hear hoofs, don't look for zebras, look for horses--- I can't tell you how many doctors we have heard this from. I think what some doctors have lost sight of may be that they aren't always on the prairie. I mean when you have a baby in the hospital and that baby is rapidly deteriorating....are you really on the prairie in Kansas? Or might you just be on the savannah in Kenya????????

We are learning a lot about the search for zebras...doctors seem to poo-poo pushy parents that have researched on the internet, instinctively feel there is something more seriously wrong with their child, or push for more tests. I have heard so many tales in the past 5 months of parents who have provided their children's doctors the diagnosis--sometimes of a very serious condition. Mike's gut told him Sophie's breathing was related to the hemi-vertabrae and that the hemi-vertabrae and associated scoliosis were more serious--but evolution and education have taught us not to listen to our instinct--that the experts must know it all. We have found a chart in a medical textbook that links hemivertabrae and asplenia....how about that? We parents MUST advocate, push and trust our instinct because our beloved doctors are out wandering around the prairie admiring the horses!!!!! (Speaking of which, I'll get off my high one now!) I don't think doctors have any bad intentions, this is what they are being taught in school.....anyway, I'll save it for another day. BTW--I am going to try allowing anyone to post comments so you don't have to register......

Some days are better than others....

I guess I haven't posted in a while...I've just been taking some time to breathe and to focus on work and family and put some of the Sophia related anxiety aside for a bit...not that I don't miss her every single day, I do.

There have been a lot of good days lately. Last weekend we were in Divide at Mike's brothers, their neighbors have a little girl who was born a week after Sophia...it was fun to watch her and imagine what Sophia might be like at 19 months. The strangest things catch me, like when the kids were playing emergency with these toy defibrillator paddles...it made me so sad--I don't even know if they defribillated Sophia or not, but it is the same feeling I get if I hear a fire truck or ambulance siren in the morning. You just never know....you think you are fine and then...boom. However, it was a really good weekend. Mike and I got a night to ourselves (thanks Thom and Jodi) and went and stayed at a bed and breakfast in Cripple Creek for the night. The place is gorgeous and really quiet and I highly recommend you go...click the link above to see more.

Lately the toughest thing has been seeing people who don't know Sophie died. The other day I was leaving work late and the night security guard who I haven't seen in a long time asked me how the baby was doing, and then we met with our financial advisor this week and he didn't know...hopefully all those encounters will taper off. I'm thinking of changing dentists just because she always asks about the kids.

On the "Quest for Answers" side, we got some good news this week. There is a research group at North Carolina doing some research on PCD, which is linked to asplenia and could potentially explain Sophie's early breathing problems. A gentleman on the yahoo group is trying to determine if he has PCD and he put us in touch with the researchers. They are interested about learning more about Sophie's case, which makes us happy. Not that knowing whether she had PCD changes anything....but being able to learn more and the potential for her death to contribute to the knowledge base and maybe prevent future similar deaths is huge for us.

Side note---got a letter from Dear Abby (actually it was from Steve, but it was on Dear Abby stationery) apparently the whole thing is just a bizaare coincidence as the "Grieveing Mother" lives in Missouri and they verified her existence...

Dear Abbey...mind your own business

On Tuesday, my wonderful neighbor Norma stopped me when I was on my way to work, she was teary eyed and told me that she had ready my letter in the paper and wanted to help me. ????? Well it turns out that someone wrote into Dear Abby and said that they lost a daughter named Sophia in April from a complication from a birth defect and were struggling to write thank you cards (http://www.denverpost.com/search/ci_6643339) My neighbor thought it was me and was offering to write my thank you cards for me...it wasn't me. Abby's advice was to get those cards out, even if friends and family had to be enlisted to help. So what an amazing coincidence....or someone who is mad because they didn't get a thank you? Or my psycho former employee stalker? Who knows....if you didn't get a thank you card from me....well put yourself in my shoes. I truly appreciate all the amazing support in so many forms we have gotten from everyone and it is what got us through...I tried to thank everyone by phone, email or in person if I didn't get to you, please know I appreciate every penny, word, hug, errand, morsel of food......

We have an appointment with the geneticist in October, Annie is getting another blood test soon....we are healing slowly.....

The kids are alright

Now that Mike and I are feeling a little better the girls are starting to have some trouble coping, we wonder if maybe they can sense us lightening up a little on some level and now feel like they can let go...Rachel broke down and confessed to feeling pretty blue about everything...I won't say much about it here because she is a teenager and I want to respect her privacy. Anne Marie has been pretty sad and wanting to talk about Sophia a lot. She asked me the other day why there was tape on Sophie's mouth at the hospital when she died. I don't remember her ever mentioning it before so I can't help but wonder if this question has been floating around in her little head for 3 1/2 months....it was a trach tube and it really bothered me, although my memories of the hospital are foggy enough that I can't remember whether she was with me or not when I asked the nurse to take it out (she wouldn't). Annie told me that she didn't kiss Sophie at the hospital because her lips were purple and that she was sad she didn't kiss her. I told her she kissed her so much when she was alive and that was what really mattered. Annie is also worried that Sophia is all alone, it really seems to bother her. I tried to tell her that Sophie has lots of playmates in heaven but she wasn't buying it (She can't move, Mom). It is so hard not to be able to take the girls pain away...not to have the answers to their questions. One of my biggest regrets about this whole thing is that Annie had to witness it all. She was with me when we went to get Sophie out of bed, must have been totally bewildered by my screaming for Mike, his giving Sophie CPR while I was on the phone with 9-1-1, the police and paramedics coming and taking her away. I am so grateful that Rachel wasn't here, but I wonder if she might feel bad that she wasn't...I hope in time the trauma will fade for both of them and they will be left with happy memories of their baby sister (who as Annie points out was a "very, very, very, very, very, very very, very, silly baby)

On the Anne Marie health front, we are working with her doctor to get another blood test to make sure her vaccines have done their job, that will be good information as there seems to be a great deal of controversy over what an elevated pit count (above normal but not in the dangerous zone) means. Hopefully that will come back with good news and we can worry a little less...I hope that I am not paranoid about losing one of the other kids for the rest of my life...We also have a medical geneticist on our to-do list. And I got a response from the Department of Public Health, which I will share here soon.

Mike and I are doing pretty good, I'm fighting a sinus infection and fever, yuk....but we are getting stronger every day, just miss our little angel more than you can imagine....

Sigh of Relief???

Last Tuesday Mike and I met with the ER doctor, his boss, the head nurse and a hospital administrator. I knew it would be tough, but I definately underestimated how difficult it would be. I started crying when we first walked in and pretty much didn't stop, thank goodness for Mike's strengh and ability to ask the really tough questions....and they were tough. First let me say that the doctors were very caring, and it was emotional for all of us. We did get answers to questions like why didn't you take blood...Apparently the advances in vaccinations that prevent so many of common bacterial infections (Pneumococcus, meningitis...) made it very unlikely that Sophia would have had anything like that (less than .3% apparently) and a blood test would have taken at least 12-24 hours to come back, so it would have been too late to save Sophia anyway.

Sophia also wasn't showing signs of being a very sick baby, she was interactive, alert and not lethargic when we were at the ER, so she didn't give the doctor indication that she was on her way into septic shock. The doctor told us that in his 20+ years of practicing medicine he has never seen a baby get a fever and die as quickly as Sophie did. Not having a spleen just made it so fast and overwhelming.

We are still processing everything, I do feel much less anxious now though, and I believe that the hospital staff is sincere, that they had done an internal review and belive that they did the best they could given the circumstances. I miss Sophia so much, the feel of her cheek against mine, what it felt like to hold her, and even her streaking down the hall when I was trying to dress her. Anne Marie, especially, seems to feel the loss of her sister's presence lately and I wish I could take her pain away. We have coffee cups that have a little cartoon of each of us on them and yesterday Annie asked me if we shouldn't wash Sophie's picture off because she is dead--it took everything I had not to lose it.

We are still working on getting some answers regarding the status of Annie's spleen(s) and to make sure she has all the protection she needs. I think the Children's doctors may be able to help us with that, so I am going to follow-up with them right now......

Government at work....Seriously

Well, in less than 24 hours I got a response from Senator Windels which is posted here:

Grace:

I read your letter and you have made me aware of something I never dreamed could happen, much less something that happens time and again, although infrequently.

I have called my liaison to the Dept. of Public Health and asked that the dept. brief me on whether adding screening for asplenia has been discussed before and whether they would support adding the test to the newborn screening. She said she'd get back with me after talking to those involved with newborn screening.

My children are young adults but your letter brought me right back to memories of their early childhood and I found myself thinking about how devastated I would have been had they suffered the same birth defect and fate as Sophia.

Thank you for trying to save some other child and parent the heartbreak you have experienced. I admire your courage and will do what I can to help you with your goal.

I'll be in touch with you after I hear back from the Dept.

Best regards,

Sue Windels

So that's fantastic, hopefully we will make some progress there. We did discover something about Annie that makes us a bit worried though, even though they told us her pit count was nothing to worry about (7.4%, normal is 2% or 3.5%) I came across something that said that anything about 3.5% is considered hyposplenia, which according to the literature increases the risk for sepsis. I called the hematologist at Children's Hospital and she told me that she was sure it what would cause an elevated level, but she was sure it wasn't anything to worry about. But of course, we are worried. I faxed a bunch of the articles to our pediatrician, I don't know what she will have to say. But I also got the following email from a researcher who I contacted:

Thank you for your email. I am sorry to hear about your loss and I empathize with your concerns about your daughter. I will be glad to answer your questions to the best of my knowledge. Let me first start by saying that I am an internist (I treat adults and I have no clinical experience with children) and second, any opinion I provide for you here is a general guideline, like what you would otherwise obtain from medical literature, and cannot be regarded as a medical advice and doesn't establish a doctor-patient relationship. Agreeing to this, I will be glad to proceed to answer your questions.

1-The "normal range" for pitted red count is a very controversial area and a lot of it depends on the method used to make those counts specially the number of cells counted. Saying this, most patients who had functional hyposplenism and infectious complications reported in literature have pit counts > 15%. Which leaves this range of uncertainty of counts between 4-15%.

2-The definition of functional hyposplenism, according to the literature, is very controversial and an elevated pitted red cell count on its own doesn't establish the diagnosis of functional hyposplenism and cannot on its own justify prophylactic measures. Yet, it would warrant a search for an occult cause specially celiac disease. Celiac disease typically manifests late in life and is very unusual to present in children.

About congenital asplenia specially in relation to the Ivemark syndrome (in which asplenia is associated with congenital heart disease and anomalies in the intestine), recent research had suggested an abnormality in the the connexin 43 gene. It may be worthwhile to see a specialist in Medical Genetics. Although I doubt that testing for connexin 43 gene mutations is available in clinical laboratories. I hope you found these answers useful. If I can be of any further assistance, please don't hesitate to contact me.Sincerely,Basem M. William, M.D.

So we have that in our pile of information too...We basically have three general groups of questions now:

1. What happened in the ER the night Sophia died--did they do everything they should have in reference to the sepsis...were there signs she needed antibiotics.


2. Were there clues in Sophie's medical history that could have alerted someone to her asplenia. We want to raise awareness of the potential connections, especially between hemivertibrae and asplenia so that the doctors might thing to look in the future.


3. What does Annie's two spleens and elevated pit count mean for her in the near term (susceptibility to infections) and the long-term (having children and their chances of having Asplenia or some other related conditions.

We are still hoping to meet soon with the doctors from Children's--hopefully they can answer the first set of questions and give us the right direction for the others.

Destiny is not a matter of chance, but a matter of choice?

First, Anne Marie is fine, thanks to everyone who called to keep me from falling off the hairy edge. Second, I just love this sleepy picture of Sophie when she was just a few months old. I was getting ready for work and she was sound asleep in our bed.

Well, it has finally started....I think. Here is a letter I wrote to my state senator Sue Windels (http://www.suewindels.com/) urging her to get a blood test for Asplenia added to the Colorado Newborn Screening Program

Dear Senator Windels:

I am a constituent writing to ask for your assistance in changing some current legislation regarding newborn screening in Colorado. In April of this year we lost our precious 15-month old daughter, Sophia Noel, to an overwhelming septic infection. She was fine at 7:00 p.m., developed a fever around 9:00 p.m. went to the ER and were sent home, and she was dead by 3:00 a.m. The autopsy concluded that she was born without a spleen and because of this her body was unable to fight off the encapsulated bacteria attacking her system—this despite the four doses of the recommended vaccine against this bacteria.

We were shocked. How is a child born with out an organ and for 15 months nobody knows? In the weeks since her death my husband and I have become avid researchers, seeking to find every bit of information we can on congenital asplenia, whether anyone could have known, and if anything could have been done to save her. What we have found out so far is that asplenia, while rare, is much more common than many of the birth defects and disorders covered in the Colorado Newborn Screening Program. Additionally, had her doctors known she didn’t have a spleen she could have been on prophylactic antibiotics and might have lived a normal, healthy life. Finally, we have learned that a simple blood test or an ultrasound can determine whether or not a child has a functioning spleen.

The relevant portions of the Colorado Revised Statues are:

25-4-1002. Legislative declaration.
Statute text
(1) The general assembly hereby finds and declares that:
(a) State policy regarding newborn screening and genetic counseling and education should be made with full public knowledge, in light of expert opinion, and should be constantly reviewed to consider changing medical knowledge and ensure full public protection;
AND

(c) The board of health shall use the following criteria to determine whether or not to test infants for conditions which are not specifically enumerated in this subsection (1):
(I) The condition for which the test is designed presents a significant danger to the health of the infant or his family and is amenable to treatment;
(II) The incidence of the condition is sufficiently high to warrant screening;
(III) The test meets commonly accepted clinical standards of reliability, as demonstrated through research or use in another state or jurisdiction; and
(IV) The cost-benefit consequences of screening are acceptable within the context of the total newborn screening program.
(2) The executive director of the department of public health and environment shall assess a fee which is sufficient to cover the costs of such testing and to accomplish the other purposes of this part 10. Hospitals shall assess a reasonable fee to be charged the parent or parents of the infant to cover the costs of handling the specimens, the reimbursement of laboratory costs, and the costs of providing other services necessary to implement the purposes of this part 10.
I believe that a test for congenital asplenia meets these criteria. I address each one individually below:

(I) The absence of a spleen is a significant danger to the health of the infant, in the absence of a congenital heart defect, Asplenia is often diagnosed at autopsy. However, if the condition is known the proper precautions can be taken and the child can live a healthy life. An asplenic child who reaches 5 years of age has a greatly reduced risk of succumbing to an overwhelming septic infection (see http://www.emedicine.com/ped/topic150.htm for more details)

(II) The exact incidence is not known, but is estimated somewhere between 1 in 2,000 to 1 in 15,000. Either of these are more common than many of the tests currently in the screening program. For example, Homocystinuria, MSUD, and Isovaleric Acidemia all have an incidence of 1 in 100,000. PKU has an incidence rate of 1 in 25,000 births and Sickle Cell Anemia occurs in approximately 1 in 5,000 births. These statistics are from the March of Dimes website.

(III) Obviously ultrasound at the hospital upon birth is an accepted clinical standard. When both of my daughters were born there was an ultrasound machine in the delivery room. However, there is also a blood test which can detect the condition. There is a certain type of body (Howell Jolly) that will be present if the spleen is not functioning properly.

(IV) Having suffered the loss of our daughter and knowing that she might have been saved if we had only known she didn’t have a spleen, there is no question in our mind that whatever the cost it would have been worth it. According to the CDC (http://www.cdc.gov/nchs/data/nvsr/nvsr54/nvsr54_20.pdf) there were 69,804 live births in Colorado in 2005. Assuming an incidence rate of 1 in 15,000, nearly 5 asplenic children could potentially be identified each year. This is more than are diagnosed with many of the other diseases for which we currently test. Identifying these children doesn’t just ensure healthy development, but can save lives. Our experience with Children’s Hospital was that the blood test cost $75.00. I can’t put a cost benefit on an infants life, but Sophie was certainly worth more than $75.00

Much of the academic research available in respected medical journals urges that more awareness of this condition can save lives. Physicians are busy, and as ours told us trained to look for quarter-horses, not zebras. In the time since Sophia’s death several of our friends have talked to their pediatricians about Asplenia and were all assured there was nothing to worry about. This without a blood test, without the physician even bothering to search Google or Pubmed. Our 2 year old niece spiked a sudden fever of 106 in the days after Sophia’s death and the ER physician wouldn’t even consent to a blood test. (Thankfully she was o.k.) My point here being that doctors are not going to look for this, the medical system is set up to let babies like Sophia die. The general attitude seems to be that some children are expendable. But if it were your child, I’m sure you would do anything to save his or her life. Without legislation, or a change in the program at CDPHE, doctors will not look for this condition.

As one of your constituents, who voted for you, and as a mother, I am asking you to pursue a change in policy or legislation to test for this condition. At the very least parents should be given the information and the option to pay for a confirming blood test or ultrasound should they choose. Many parents are paying thousands of dollars to bank their parents cord blood for a statistically unlikely event. Surely many of them would also be willing to pay $75 to save their child’s life. I don’t remember what the specific test was, but I know when Sophia was born her doctor recommended a test that would cost us an additional amount (somewhere around $25) and we paid it. If I can be the catalyst for just one parent to find out before it is too late that their child is asplenic, Sophie’s death will have some meaning for me.

I am willing and able to talk more to you, via email, in person or on the phone. I have volumes of articles from medical journals I am willing to share. I appreciate your help, your public service and all you do for Colorado.

So I sent that to her today. I might send it to the Governor too. I am really hoping to take this all the way and to get that test added, or at least made available. I'm a little afraid that if the fight is too hard I might run out of steam later (those of you who know me well might be nodding your heads) but since it isn't a constant fight and with everybody's support (especially Mike) I think I can do it. A special thanks to Nancy for putting the idea in my head in the first place.

Many of you have asked if there is anything you can do....you could alter the letter and send it to Senator Windels, or to your representatives in the State House (http://www.vote-smart.org/index.htm) and I might just ask folks to do that if I don't hear something back soon.....I love you all so much, I couldn't have made it this far without you......

It had to happen sometime....why this weekend?

Anne Marie got her first fever this weekend, well not her first, but her first since Sophie died. Of couse it had to be the weekend that Mike is out of town. She got up to 102.7, but Renee (my sister) came over and kept me sane. She's the only reason I got any sleep last night. She's had fevers before, of course, but I am nervous. She seems to be doing better now as she is fighting me really hard about going to bed...it's a good sign so I am trying not to be too irritated. The folks on the list serve seem to think that having two spleens means she has polysplenia, the diagnosis was "small accessory spleen", so I'm not sure what, if anything the difference is. Good question for her doctor.

We are still waiting on getting to meet with the ER doctors. We were gone, then they were, so maybe the first week in August. I have been writing down my questions and reviewing her medical records so I can make sure I don't leave any questions unanswered there. We still need to decide what to do about talking to the pulmonologist and the orthopedic doctor, we want to make sure they know that there can be a connection.

I'm still doing pretty good....thanks everyone for so much support...

Open foot insert mouth

The online support group has turned out to be actually an amazing source of information. Some people have lost children to asplenia, polysplenia, heterotaxy, etc and others are battling it every day...these people have more information than any of the doctors....and they are amazingly supportive. I was just being impatient as usualy.

Just wanted to let anyone who is reading know that I had a very bright and happy day today (anything to do with the venti iced latte this morning?) And that I miss the little beetle bug but am feeling happy.......

Good days and bad.....

Had a very weird moment yesterday where I had been fine all day and then just lost it, sobbing in my office. I decided to call Mike and when he answered the phone he was crying too...I wonder what happened to us both at that same moment....strange. Some days I am fine and some I don't know if I can stand it...never seeing her again, our little beattle bug....

Kristin's brother Evan has gotten us some amazing contacts and has written a letter to the former director of the National Center for Birth Defects and Developmental Disabilities http://www.cdc.gov/ncbddd/ asking if he could provide any more information about asplenia for us.....he also gave us the numbers and names for some local experts. The director of the state program here in Colorado has called me, her husband died unexpectedly in an accident recently, I felt so heartbroken for her. When she gets back into the office whe is going to get some information for us.

It may seem obsessive to anyone who hasn't been through this, I really don't spend all my days and nights thinking about the ER situation and asplenia, but I also have questions that I need answered, questions that I am hoping can shed some light on this mystery, can help us help doctors help patients, can prevent even one family (preferably more) from having to have to endure this heartbreaking, wrenching pain....

I also joined an online support group for Asplenia/Herotaxy/Ivemark's Although so far it hasn't been too helpful. At this point it seems like a group of people who have become good friends and don't talk too much about the condition or associated loss, but rather about their friendship, and everyone has ignore my posts (poor me) but at least I have tried.

Enjoy the picture from last Halloween....she was the most adorable little bat, but of course hated the hat

Life Returns to.....Normal?

Will life ever be normal again? I can't imagine that it will, we did go on vacation last week and it was nice to get away, very relaxing. Right before we left we got a call from the director at the ER where Sophie was seen the night she died....they are willing to meet with us to talk, which is helpful. We are trying to schedule that now. She answered some of my questions like:

Why didn't they take her blood pressure? Apparently they don't take it in kids that young, they don't find it to be useful, in her case it seems like it would have been one of the few things that could have saved her, surely her blood pressure was dropping by then, only a few hours before she died? She didn't answer my other question about why they didn't take blood, I still struggle with trying to understand how an asplenic child EVER survives a sepsis infection, because what would prompt the doctors to ever check for it...I will want to ask about it for sure. She told me that she didn't think that even if they had for some reason started IV antibiotics right then they could have saved her...I find that somewhat hard to swallow, when we got to the ER she had a fever for 2 hours tops......if she wasn't sick enough for them to register something was seriously wrong.

Anyway, I am happy to have a chance to meet with them, although I know it will be very hard, at least we don't have to sue them just to get some answers. And I am sure they must have cleared it with their legal department who must feel that they did everything right, so that gives me some peace too.

In general, 11 weeks from the single most horrible day of my life, I am less anxious, sad but not as much, and maybe moving toward acceptance, I don't know......

More questions than answers

Well, we now know Annie has a spleen and that it appears to be functioning (see previous post), but we are still waiting the results of two blood tests...we aren't really worried, but it has been two weeks so it would be nice to know.

I have been really struggling the past few days, frustrated by the lack of information about a) Sophie's condition and b) what happened in the ER that night. I finally took the step of going to the library and pulling all the journal articles that we keep seeing on Pub Med. One of the references was to a text book Human Malformations and Related Anomalies which had a chart showing that Asplenia and hemivertabrae are linked. That was a gut punch for both of us, and we want to get in front of the orthopedic doctor she saw to make sure he knows that. If it could prompt him to recommend an ultrasound for even one child with hemivertabrae and it saves them it would be worth everything. Another article that I have been curious about is one that suggests that when a child has recurring sepsis infections a doctor should check for asplenia. My question still is what would prompt them to check for a sepsis infection in the first place? If high fever with very sudden onset, shaking, vomiting, lethargy doesn't do it, how does a child survive a septic infection (I know they do).

I have been fighting with this last question for a week...what happened in the ER...is it what should have happened (please, please, please) If it isn't, will anyone ever be honest with us about that? It would help so much.....Mike suggested that I call the hospital and ask them to sit down with us, which I have. I left a message for the care center director on Tuesday, just asking if it was possible to sit down with someone and review her chart, what happened that night, the decisions that were made, the doctors notes. I don't think they will, but it is worth a shot.....I have stopped obsessing over it and will just wait to hear, if I don't then I will decide what to do next. We have also requested her medical records....anyone know a pediatrician who would want to review them for us?

Most recently I have read that asplenia happens 1 in 2000 births....if it really is that common this might be the beginning of a long crusade for me to raise awareness...for now, I just miss Sophie so much, I ache, all the time. Thank God for my amazing family, my strong and loving husband, our beautiful daughters and the incredible support for family and friends. I know Soph is at peace and I try to cherish the time I had with her.....Please if you know anyone who can help with our questions have them email or call us

You want answers????

Well, we finally got the answers on Anne Marie's test, and her two spleens appear to be working fine, so finally I don't have to go in every night and make sure she's breathing....and the morning anxiety attacks have lessened. The hematologist at Children's hospital still wants to do one more test to confirm that the spleen(s) are not working overtime, but they said they have no reason to suspect it, so we are not too worried at this point. Breathing a little easier on that front helps.

We are still questioning the genetic component of it all, but in general the doctors don't seem to think there is a connection...Mike and I find it strange that of our two biological children (Rachel was adopted by Mike when he married her his ex--her Mom) one didn't have a spleen and the other had two....not really sure where to go with that, but we will probably keep hunting in the future.

I tried not taking Ambien the other night--what a disaster--at 4:00 a.m. we were basically all up (thanks to me) and Mike and I had a long conversation about what might be going on. He astutely pointed out that although I keep claiming to want to go to therapy, I haven't taken any steps to that end. I think he is mostly right, but I am also giving some credit to the fact that I do not seem to be able to make even the simplest decisions right now. Anyway, we have an appointment for tomorrow.....

Things are pretty relaxed at home right now, I really miss Sophie so much..I hate that our mornings and evenings are quieter, less rushed....I'd give anything for her to be pestering me for dinner, bath and bed. So far the hope that his blog would lead to someone else who lost a child to Asplenia or some researcher who knows something hasn't worked, but I'll keep it up because it helps me....

Frustration so bad it makes you cry

It's been such a rough week. We are still waiting on Anne Marie's test results to make sure her spleen and liver are functioning normally...it has been almost two weeks since the first test and a weeks since the other two--results were supposed to be back in 1-3 days, so we are very stressed out. It might bode well that we haven't heard, assuming that bad news would travel faster, but we're not always thinking rationally right now and we need some peace of mind...losing a child makes you extra paranoid about the other ones, at least in the immediate aftermath.

Also frustrating is the lack of information about anatomic (congential) asplenia...oh there's all sorts of information out there about how to prevent sickness in death in a child that has anatomic asplenia...but HOW THE HELL IS ONE SUPPOSED TO FIND OUT IF THERE CHILD DOESNT HAVE A SPLEEN??????? There's several easy tests, ultrasound, blood smears that aren't all that expensive--but apparently the "cost-benefit" doesn't justify the $71 blood smear when they are infants, or the cost of an "organ check" ultra sound....does that seem right? I mean insurance pays for Viagra alot of times for *&%@*$ sake and lots of other things that might be important...but as important as a child's life? And anyone with the attitude that it wouldn't be worth saving one or two babies a year isn't a parent who lost their precious, amazing, sweet wonderful little girl way too early. Even if the incidence is .06% (6 tenths of 1 percent) that would be about 2,400 babies in the US alone who wouldn't die each year---2400....mon dieu. At 4 million US births for year, guessing $200 per screening it would be $8 million...a mere $3,000 per life saved--if I ever get that money I will start a foundation in Sophie's memory that will help children get screened for Asplenia at birth.

I guess I just feel angry today...maybe tomorrow will be better....

blood test, boxes, and bags (oh my?)

We are really hoping to get the results of Anne Marie's blood test this weekend so we can breathe easy knowing she will be o.k. They are doing three tests, one to test for the Howell-Jolly (http://www.answers.com/topic/howell-jolly-body) bodies, the presence of which indicate a missing or non-functioning spleen. Since we know Annie has a spleen this will tell us if hers is working. The other test is futher confirmation, something called a pit-count and the third is to test liver function to verify that something they saw on the ultrasound is not a problem. The chances are small that anything will be wrong with her, but after what we have been through it is difficult to have any peace without knowing for sure.

Last weekend I bagged and boxed most of Sophie's clothes, it was extremely difficult. I had to rush through it so I didn't stop and look at everything and remember. I couldn't part with the shoes, mostly because she loved shoes so much--she would bring her shoes, or Annie's (or mine or Mike's or Rachel's for that matter) up to you and have you put them on, then she loved to take them off....We don't want to eradicate her memory, and there is some stuff I will never be able to get rid of...like the little pink tutu that she loved to wear.

I miss her so much...which seems like such an obvious thing to say. An amazing little girl, I would love to kiss her, hold her, tickle her or even spend all night trying to get her to sleep again, it's really sinking in that this is real, that she is not coming back and it is so hard.

5 Weeks Tomorrow

It will be 5 weeks tomorrow since the death of our precious 15 month old daughter Sophia Noel. She died suddenly from pneumonoccal sepsis as a result of congenital asplenia, which means she was born without a functional spleen.

There are many reasons I decided to start this blog, one as a personal journal as we try to deal with this and resume a normal life (whatever that means), also in hopes that someone with some knowledge of asplenia will will reach out to us with information, and finally as a place where other parents who have been through this horrible nightmare can find a place to connect.

Sophie died on Friday morning, April 27th, 2007. I won't go into the details, but it was horrific, she had a wonderful day at daycare on the 26th and was fine when we put her to bed at about 7:00, she awoke crying and vomiting with a fever of 105 about 9:15 p.m. and we took her to the emergency room where they did a chest x-ray to check for pneumonia and a urine test to make sure it wasn't a kidney infection. They gave her some Tylenol and sent us home. I last saw her about 2:30 a.m. and in the morning she was gone.

We were in total shock. We found out the next day that she did not have a spleen. Apparently most children who are born with out a spleen (congenital asplenia) also have a congenital heart defect or some other type of syndrome that would lead the doctors to check for a spleen, but Sophie was completely healthy in that regard. To be honest I had no real idea of the purpose of the spleen before this happened and it didn't make sense to me at first because I know people have their spleens out all the time. But apparently in small children it serves a very important function and without one they are unable to fight off certain types of bacterial infections, especially encapsulated bacteria (http://www.answers.com/topic/asplenia) There is so much they can do to prevent overwhelming sepsis if they know a child doesn't have a spleen, but it is not common for doctors to check and without knowing she had no chance.

Most of the information available on the Internet is academic in nature, and although we and our families are fairly well educated, it is tough to get a sense of how common this is. I've heard 1 in 15,000 children and if this is the case I would advocate strongly for a post birth check of all children through a simple blood test. The State of Colorado requires several blood tests for syndromes less common than this. At the very least I hope to make more parents aware of the condition so they can ask or even demand the blood test. Insurance likely won't pay because the risk is so small, but given the option of saving your child's life, many parents would be willing to shell out the $75 for the test, I would imagine. Sophia had 4 doses of the Pneumococcus vaccine as recommended and it didn't make a difference for her.

We are also trying hard to get a sense of the genetic implications, and here there is really no information. We have found out through ultrasound that our three-year old has two spleens. Although we have been assured this is a "normal variant" with 17% of the population apparently lugging around an additional spleen, we are still pretty nervous and awaiting the results of some blood work to confirm that she is safe. What are the chances that both our children would have spleen abnormalities, if it is genetic how can we find out?, what does it mean for our other child's future?

I hope to post every day and let folks know what we are finding out and how we are doing.