Can't we stop the world just for a minute?

Wow--it really has been forever since I posted. I have been doing really well, just the occassional stunned moment, or something catches me off guard and I lose it, but mostly things have settled down and I am doing o.k......That is until this week, things are really starting to get tough. I am so fortunate to be loved and supported by so many amazing people...that helps. I have read many stories of people who have lost a child whose family and friends think they should "be over it" Nobody in our life is like that--not our family, friends or bosses. I think everyone we know has been impacted and are not over it themselves, this is every parents worst nightmare, right....so anyway, I feel blessed to have so many kind, giving, generous, loving people in my life.

A friend of mine just had an amazing, beautiful little baby girl...she felt guilty talking to me about her and I told her that I don't have any jealousy of other babies. They still bring me the same amount of happiness and joy that they did before we lost Sophia. No one contributed to our tragedy, so no one has anything to feel bad about.

On the advocacy front...not much going on. I did find an article which said that the incidence of congenital asplenia is 1 in 2000....way higher than most of the diseases they test for. This same article also told of two siblings who developed sepsis and were found to be asplenic. They then tested the rest of the family and found that the mom and another child were also asplenic. It really rekindled my curiosity about whether Mike or I might be asplenic.... We decided to move forward with getting blood tests for us and abdominal scans if we can....

Mike wrote this amazing letter to the group savebabies.org that advocates for newborn screening. We got a letter back (which I can't find at the moment) from the head of research saying that we should keep pushing forward with this, the main obstacle seeming to be that the screen can't be done with a filter paper test. Anyway, here is the letter...I will probably be posting much more this week...it is shaping up to be a tough one....

To Whom It May Concern,

My wife and I are very interested in learning more about the Consumer Task Force on Newborn Screening as the recent death of our 16 month old daughter Sophia, in April, from undiagnosed asplenia (and the resulting overwhelming sepsis) has sent earthquakes through our life and family. We are committed to furthering knowledge and understanding, and have been in contact with many doctors, some on a national stage, even state legislature, to see if something can be done to minimize a repeat occurence for someone else's family. My father, Marty Wilcox, was a founder of ultrasound in obstetrics in the 70s, and he asked a simple question after Sophia passed. If someone who suffers from and does not have the means to combat simple bacterial infections due to the lack of a spleen, couldn't much be done to ensure survival if diagnosis can be made early through a simple organ checklist being taken when the mother is already most likely getting ultrasounds done for her unborn baby? Howell-Jolly bodies are indicative of a failed or missing spleen in the blood of such an infant. Can't an additional test be added to the blood draw every infant goes through in their first days of life? Polysplenia is another problem that could be ferreted out with such tests. The treatment is a simple one, antibiotics and parental awareness. Sophia was asymptomatic in most regards, and though her medical file is large for one her age, she didn't have the more severe syndromes associated with asplenia like heterotaxy or Ivemark's Syndrome. There were signs we have found after the fact like breathing issues and a hemivertebrae, but those connections are all the more sorrowful now.

We feel compelled to make her short life and death helpful to others. Please let us know any way we can help, and we will both become familiar with your Web site and efforts.

Thank you,
Michael and Grace Wilcox

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

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......

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

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....

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.