New Year....

You know its been crazy when a month has gone by without a post....Crazy is an understatement.

I wanted to thank so many of you for all the encouraging words when I went back to work. The long and short of it.....it was an EPIC FAIL. I lasted all of two days. Two days I won't get back. So, how amazing do you think I am now? Not very, I know.

Quitting was harder than deciding to start. All the hopes and dreams I had for myself and for my future went out the window. All the hard lessons that I told myself I needed to deal with...non-existent.

There were a few reasons behind it. One being my arrangements for the kids were falling through minute by minute. It was easy to see that two days into it, it wasn't going to work long term at all. But the hard and honest truth is that I just can't be away from her. I will never get the night of almost losing her out of my head. I can't no matter how hard I try. All I can remember is trying to make her say "I love you" while I tried to record it on my phone. I made her do it over and over even though she was weak and didn't want to. I needed to hear it.

I sat at work for two days wondering what in the hell I was doing? I know now that I needed to just get it out of my system or it was something I was going to obsess about constantly. I humbled myself and realized it was just something I wasn't ready or willing to do. That is the short of it.

Over the last few weeks, I have realized that it was what I needed to do. And being home with her (with breaks here and there) is where I need to be. I have a few things up my sleeve, that if they work out will be a much better solution for me, my family, and for Brigitte. In the meantime, the images, sounds, feelings of that night are to fresh in my mind and I just need to be "around."

We have been overwhelmed by the generosity of this community over the last few weeks. Just as panic was setting in about how we would make Christmas happen, everything just started to come together. It has been a sign that I did the right thing....and people really do want to help.

We were blessed by the generosity of Laurie Kolifrath and New Beginnings Nail Salon who set up a "giving tree"...I was astonished at how many things were delivered to our family. Thank you will never be enough. The day before Christmas Eve, we also visited North Londonderry Elementary and were overwhelmed with the donations made by North School Staff, families, and especially Danielle Blair...a 5th grader who made ornaments out of toys and sold them to help benefit our family. There are so many of you out there that I owe Thank You's to. I am hoping to get to them soon. I promise. Please know that we are so grateful to EVERY SINGLE PERSON who has helped. I may not have mentioned you here, but please know how much we appreciate all you have done for us. Really, my words are completely inadequate and I will never be able to express how we really feel.

So here we are, another New Year upon us. I am not hoping for a better year than the last. I refuse to do it anymore. Instead, I am just hoping to focus on what was good from 2010. My son, Sean, endured two brain surgeries and is playing out in the snow as I type. My daughter, Brigitte, wasn't expeceted to live and is now in the living room SCREAMING at the top of her lungs!! She even uttered a very appropriate "DON'T TALK TO ME" this week! My son, Jake, went undefeated in football and won the Mity Mite Superbowl! My daughter, Brenna, became a National Junior 3 Co-ed Champion two weeks ago in cheerleading!

Life is Good....and we will take that for however long it wants to stick around.

Happy New Year!!!

Changes.....

It's been a bit since I last updated. I am so sorry. Things are just busy here. I feel like I say that all the time. Not like its any different at your house, right? We are all busy!!!

Brigitte is holding her own right now and proving day in and day out that IV nutrition is truly what her body needs. I can say she is doing fantastic. Her new port has been wonderful, she has energy, her color looks good, and she doesn't look like a bag of bones anymore. Now, unfortunately, things aren't perfect and we've got issues we are dealing with....but seems as if this is about as good as it is going to get. So I will take it for however long it lasts. We are in the process of working with her TVI (teacher for the visually impaired) and her neurologist. Her brain MRI and her vision issues are making it pretty clear that she has a cortical vision impairment. For all intents and purposes, she is likely going to be declared "legally blind" but not in the sense that she can't see. She can see, she just doesn't process correctly. So this isn't actually an eye issue, but a brain issue. Its hard to explain, so I won't try to here, but it does explain alot of the issues we are seeing related to her learning style and why she has such a hard time moving around and being who she is. Once again....its a process.

Sean is doing remarkably well too. He has not complained once of a headache or neck pain. He isn't complaining about school. He is doing fantastic in school!! Best decision we ever made was having that second surgery. He was referred to Make a Wish and will be having his wish granted very shortly! He is beyond excited.....and so are we!!!

I made the leap into the working world again today. After almost 9 years of being home with the kids, I returned to work full time. This was one of the most emotional decisions I have ever had to make. Financially, it was a no-brainer. I am feeling much better about it tonight than I was yesterday, but it is hard no matter what. People have asked me time and time again since I got the offer if I "was excited??" Excitement was the last thing on my mind. Am I happy to be among adults again and earn my own paycheck? No doubt about it. Other than that....not much to be excited about. My reasons for doing this aren't purely financial...but are sadly, very emotional.

It is hard for me to explain how this felt. First of all, if it weren't for the availability of a nurse with whom I am very confident will take very good care of Brigitte in my absence, this NEVER would have happened. For her, I am very grateful. My life has become all about Brigitte and less and less about anyone else. That includes my other children, my husband, and more importantly, myself. Brigitte and I are one in the same. There would be no me without her and there would be no her without me. We are inseparable on so many levels. I know that sounds beautiful, but it really isn't. Its not good for either of us.

The big elephant in the room is that more likely than not, I will outlive my daughter barring some major breakthrough in medicine or a miracle which cures her. I have come to accept that over the last few months. Its been a hard realization, but it is reality. Until today, the last four years of my life have revolved around taking care of Brigitte. I have forgotten how to do anything else or be who I used to be. I honestly cannot for the life of me remember what life was like before Brigitte. I really can't. I have become a machine just going through the motions to get through every day.

So today, I did it. I cried the entire way to work. I cried on my break. I cried on the way home. But I did it. I got through the first day. All I can think about is that the time I am away is time I am losing with her. Hours and days that are limited are hours and days I will never get back. However, I also thought about all the things this going to allow my family and I to do. Next year, Brigitte will be in Kindergarten 5 days a week. The year after that 1st grade, full days. Then where will I be? That "something" will ultimately take Brigitte, but I can't allow it to take me too.

Merry Christmas!!

Breathing.....

Breathing. It's something I can't seem to do much of anymore. I am sitting here trying to fully grasp everything that has happened over the last 4 months and I truly cannot believe it.

This week was certainly no different. I am sure you have all heard about the "fight or flight response" at one time or another. My family is living in that constant state every day. I have been doing alot of reading about it as I know it must be detrimental....but what do you do about it? It’s important to learn how to recognize when your stress levels are out of control. The most dangerous thing about stress is how easily it can creep up on you. You get used to it. It starts to feels familiar – even normal. You don’t notice how much it’s affecting you, even as it takes a heavy toll. I am used to it, and have been for way to long. What is even scarier is that if I am feeling this way, how is Brigitte feeling? How are the other kids feeling about all of this?

I should let you know, that Brigitte was again rushed to Boston on Tuesday and underwent another emergency surgery. I had some trouble with her pump and some occlusion alarms in the early morning hours on Tuesday. Think like 2 or 3am. Yeah, makes sleeping impossible. I finally decided to shut the damn thing off because not only was I not sleeping, but Brigitte wasn't either. I flushed her line with saline and swore I heard a little "pop." WTF!!!! I checked her site, inspected the line and didn't see anything. I went back to bed. She woke around 7 and was crying a bit. At this point, you can imagine the chaos that takes place trying to get 4 kids up and ready for school. I got breakfast for the kids, got them all dressed and was ready for Brigitte. Let's just say her morning routine is a little less than pleasant. That's putting it mildly. She is a major witch in the morning, so I leave her for last. As I was attempting to get her dressed, I noticed that her dressing was red. At first I thought it looked like fleece stuck to her (she was wearing a fleece pajama set) but quickly realized that her PJ's weren't red and what I was looking at was clearly blood. Add to that, Jake and Sean both saw it at the same time and started freaking out. It took all I could to get them to hang up the phone fast enough as they had already dialed 91...and you know what would have happened next. I could see she wasn't actively bleeding so I knew that an ambulance ride would have actually wasted valuable time. In our town, they are not allowed to cross state lines, so we would have been taken to the local ED, stabalized, then transported to Boston. I knew that pop I thought I had heard was real and that we were already beyond the 4hr mark of blood starting to clot. So we needed to get to Boston fast. That's kind of funny, because it was rush hour traffic time, and Brenna was home sick. So she went with my dad and Brigitte and I made our way to the city.

It turns out that her line had totally blown and she was headed to the OR again. The surgery itself lasted about 3 hours. This time instead of a broviac CVL being placed, we went with a port. Although we aren't able to get the full benefits of it right now since she is on TPN 7 days a week, we are optimistic that at some time she will be able to have some days off and be deaccessed giving her the ability to bathe, swim...and just not be bothered. We are a long way from that, though.

So, as I said, we live in a constant state of "what's next?" It is becoming more and more difficult to just live normally. Clearly, this is affecting Brigitte more than anyone can imagine. She is having tremendous difficulty in school because of her anxiety. This poor child can't even wake up on a Tuesday and get excited about school and her friends because just when she thinks things are ok, she is in the hospital with a mask covering her face as she is forced into sleep to be cut open again, only to wake up with another scar and one more piece of plastic stuck inside her little body. One more thing to make her so unlike most little four year olds. She is being admitted again next week for all of her testing, and tonight I have had multiple email exchanges with her doctors who are also concerned about her emotional well-being. We have come to the conclusion that a psychological consult team will be part of every admission to deal with her ongoing anxiety. My poor baby is only four years old and will now have a TEAM of psych doctors. This just isn't right on so many levels.

I have to be honest and tell you that I am a very angry person right now. I am angry on so many levels. I am angry that this happens to Brigitte and Sean. I am angry that there isn't a damn thing I can do to make any of it go away. I am starting to get really mad at family and friends who don't realize that my life is nothing like theirs and they just don't get that. I dread the holidays and having to put on my happy face. It makes pretending all that much worse. I know a while back I promised a post on all the good things that are happening, but right now, I can't seem to find anything. I am taking a few days to sit here and feel bad for myself, my kids, my husband, my marriage. Next week, because it is Thanksgiving, I will pull myself up by my bootstraps and be happy for all that I do have in my life.....which I do know is alot. But right now, I just want to be mad.

For all my other mom friends who have children with special medical needs or worse, have lost your children. It's ok. It's ok to be mad, really mad.

What's not ok is to stay mad.

Updates

Well, things certainly have been busy around here. I honestly don't have the time or energy for a real heartfelt blog entry....but I know I owe all of you an update on how the kids are doing!!!

Sean ROCKED his surgery. I mean, truly ROCKED it!! I am beaming as I sit here and write it because I am still amazed at how amazingly strong he is. His surgery was about 4 hours long. He had a very difficult time going to sleep and really fought it. His surgeon got inside the dura and his cerebellum "popped" right up into place. He said that his post-op MRI was very deceiving for how much crowding was actually there and he definitely needed this second operation. Having known that Valium was Sean's magic drug post-op the first go-around.....we got the PACU nurses to give it to him right off the bat. We knew he was feeling pretty good when he said "Hey, is that thing (catheter) stuck in my balls again so I don't have to get up to pee?" Sean was back....as funny as ever!!! For those of you who don't know Sean personally, he is a boy of few words, but when he does speak, he usually leaves you shaking your head, laughing!! He was also started on Decadron in the OR and continued it for a week total. This helps with brain swelling and also with the junk that was in his lungs prior to surgery. He was eating and drinking immediately after surgery and had a great nights sleep. Saturday morning he was up and out of bed and already taking oral pain meds. We gave him the morning to see if he continued to do well and when his surgeon, Dr. Benjamin Warf, came in to check on him I asked if we could go home. There wasn't anything I couldn't do for him at home and lets just say that things with Brigitte were becoming a little more complicated and being home was best for everyone. Sean was discharged less than 24hrs after brain surgery and never took a dose of pain meds after leaving the hospital. He is AMAZING!!!!! He rested on Sunday and on Monday morning I had to make him come back in the house and sit on the couch. No hitting wiffle balls two days after brain surgery!!! Geeeshhh!

And then there is Brigitte. I will just say that if you looked at her today, you would say she looks probably the best she ever has. I had a doctor ask me today if she was healthy? My response; "Well, if you consider a 4yr old that is life dependant on TPN nutrition 7 days a week, has a heart defect, is immunodeficient, is in complete colonic shut down, and so on, healthy, than yes, she is healthy." It saddens me that I can honestly agree that she looks the best she has in months. It saddens me that it is a central line, TPN, and IV hydration that is keeping her alive. I think people don't get that. They see a little girl running around, yelling, playing as best she can, going to school and her brothers football games and they think "Wow, she is doing so awesome." Until you understand that if we take away the central line, the TPN, and the hydration....give her a few days and she would die from dehydration and malnutrition. So, yes, Brigitte is doing ok.

She is being admitted the week before Thanksgiving to have her manometry testing done. This will be a very difficult admission for her. We are hoping for only 3 days, but it could turn into a few days longer. She will go to the OR to have a colonoscopy, endoscopy, have probes placed in her rectum and in her gtube stoma.....she will also likely have an NG tube dropped from her nose into her stomach. She will then be attached to monitors to see what her intestinal motility looks like. She has to stay in bed while this is done, and no she cannot be sedated. Good Times. Feel free to visit and serve as a distraction. The thousands of stickers we have should come in handy, but I very well may lose my mind!!!! This testing will give us an idea of what surgical procedure may need to be done. She is still only having bowel movements every 12-14 days despite being on mega-doses of laxatives. Yes, she is still smiling!!!


So, those are the latest and greatest!! I have lots of pictures to show you of Sean, but we have been straight out and just haven't had the time to do much of anything else but auto pilot. We're getting there.

I have been thinking about another post to write soon, but I know I have alot of people reading thanks to the Thrive Blog post I did with Children's Hospital. What would you guys like to hear about?? Leave me some comments and I will try to come up with something good. I was thinking of "How to deal with family through the Holidays?" Let me know what you think!

Much Love Always!!!

Shared by a friend tonight.....

A friend shared this with me after reading my "Picture Perfect" post. It couldn't be more perfect. Enjoy and really listen to the words. Please see the post below as well if you have not already.

Picture Perfect????

If you were to drive by my house this morning when the kids were getting ready for school you would have seen a scene from a movie. Hugs and Kisses, High Fives and Good-byes. A pretty little red head with such bounce in her step carrying an umbrella while her brothers cram in beside her. One with Clark Kent glasses and a backpack almost as big as he. The other, grinning from ear to ear, wearing his Blaze jacket he earned playing on an AAU baseball team this spring. Their smiles are almost contagious. All three walking side by side in the rain, turning back every few seconds to give there momma a wave and blow a kiss goodbye.

Picture Perfect.

What you don't see on the inside of the house is the mom with tears streaming down her face and the 4year old asking "Mama, why you crying?" She cries because the picture on the outside looks so perfect and oh how she longs so badly for it to be so true.....even just for a day. She will go back up the stairs to start saline and heparin flushes, give GTube boluses to one sweet little girl and begin to prepare for major brain surgery for another sweet little boy. You also don't see that the dad hasn't been home in 3 days because he has worked 3 16hr shifts straight and stays at a relatives house close to his work so he can at least get an extra hours sleep each night rather than wasting that precious time sitting in traffic on a long commute to work. He will likely be to tired all weekend to be the dad or husband he really wants to be. His tears flow each day as well because he isn't able to tuck the kids in at night and can only text them each day to see how their day was or hear about the back handspring someone landed at practice last night.

This is just a small glimpse.

The mom and dads pain in this picture is so deep but they live in survival mode. There is no crashing because there isn't anyone to pick up the pieces. If they stop for a second and really take in the reality of their situation, surely it would break just about anyone. How can you break though when you have a family to love and care for? There are still bills that need to be paid and life has to go on. But that's just it...all this family does is exist. Existing in a world that from the outside looking in, looks as pretty as a picture. Nobody will ever understand what really goes on behind closed doors. Nor do I hope that you are ever in a position to truly understand.

All I want to do is cry and cry and cry. I can't. I do not write these things because I want your sympathy. Sympathy is the last thing I need. I write because it makes me feel better. What I really want to do is crawl under the covers and not come out for a few days...better yet, a few weeks. I do not have that luxury. I have snacks to pack, baths to give, homework to help with. I have appointments to go to, doctors to deal with, bill collectors to fight with. No matter how badly I want to break, I can't. Even if I did, then what would that say about me or my family. Then we would be the family with the two sick kids and the mother that went crazy. Its just not that easy.....and hard would be a major understatement.

I don't know what the answer is. I try to hope for the future to be better, but it truly is hard to dance through the storm when you never see any sunshine. I have to laugh at our reality sometimes, because I just don't know what to do anymore and honestly I am sick of pretending everything is great when it isn't. People ask me everyday "How is Brigitte?" My typical response is "Ummm, she's doing ok. Thanks for asking." What I would really like to say goes something like this: Actually, she's a 4 year old little girl who has spent more days in the hospital than she has in her own bed. She is not physically able to eat because her intestines are not working, so an IV in her heart is keeping her alive. Without it she would be dead. Yes, she is home from the hospital and yes, I let her go to school. That is so she can sort of be like a little girl. But most 4 year old little girls aren't eating with a Gtube in their belly or an IV in their heart. They aren't talking to surgeons about possibly needing a colostomy bag. They are running around splashing in puddles or going on playdates when mine only gets to go to PT, OT or Speech. She didn't get to got to the "friend party" at McDonalds today because the germs there could kill her. Instead she spent the day in the house driving her mother crazy because she can't do the things little girls do. She had to have two needles stuck in her legs and blood drawn from her arms twice this week along with having 3 appointments at Children's Hospital. In addition, she now has to be admitted to the hospital next week even when she isn't truly sick because her 7 year old brother has to have brain surgery and the only way she can be taken care of is by a nurse which the State of NH won't let us have because I don't "work"...so she has to be in the hospital when really she doesn't need to be. So, how is Brigitte doing? "Ummm, she's doing ok. Thanks for asking."

Here we go again....

I have been blogging alot about Brigitte lately and haven't updated all of you on Sean. Things have been very difficult for him since his surgery. He is having alot of pain in his neck, many headaches and still tingling in his arms and feet.

We have been back to see the neuro-surgeon over the last few weeks quite a few times. He has had a post-op MRI as well. Things just aren't any better than they were before the first surgery. He is waking up multiple times in the middle of the night crying and asking for his valium and oxycodone...this coming from a kid I have to chase around the house to give him medication on a regular basis. He is struggling quite a bit at school as well. I was at North School twice within an hour yesterday. Once to pick up Brenna as she wasn't feeling well, and then again to get Sean who just couldn't make it through the day.

We met with Sean's surgeon again on Monday and have decided that it is time to bite the bullet and go ahead and do a second surgery. This time he will actually have to do a duraplasty, which means his brain will be open and exposed while the doctor sews in a synthetic patch to help brain expand. It may not be as long a surgery since most of the bone has already been removed, but it will be more complex and there is more risk involved anytime the brain is manipulated. I got the call this morning and his surgery will be next Friday, October 22. His Pre-Op is scheduled for the day before.

I am so sick of all of this. Brain surgery twice in 8 weeks for one child and then almost losing another? Is this really happening? I know that it is. I think the thing that is hitting me the hardest is that I feel completely numb. I have shut off so many feelings so that nothing can hurt anymore. It is the oddest feeling in the world.

I am now trying to figure out how to manage having Sean in the hospital and be there 100% for him because he so deserves it, but also manage Brigitte's care....which nobody except me can do. On top of that Jake has his Mity Mite Superbowl the next day on the 23rd. It's just not fair. I cannot be in three places at once and so need to be. Actually, four places because Brenna needs her mommy too. How do you do this? I think there should be an unwritten rule somewhere that if you are blessed enough to be given the gift of an unhealthy child/children that you shouldn't have to worry about all the other stuff and it will just be taken care of! Yes, that was not a misprint, I do feel very blessed to have been given Brigitte and Sean. They are amazing children that have taught me what life is all about. They are MY teachers....not the other way around. I do not pretend to think this is an easy life, and there are many, many people who couldn't handle 1/10th of what we do. BUT, I really do know what matters.

My children are bringing together a community and teaching them what life is all about too. The Londonderry Wildcats,Kim Payson, and so many others have stepped forward and are helping to fill the void and take care of things that Bryan and I can't physically and financially. My children are leaving their footprints on so many people's hearts, it is truly heartwarming.

So although medically things are always in a tailspin in this house, we are getting through it with the love of family, friends and an entire community that is rallying around us. Sunday, Shory's Mexican Roadhouse in Manchester and Salem dontated $1000.00 from a fair share event to help defray some costs associated with the mini-makeover being done at the house. On Monday, Lenny and Lisa Santosuosso of Santosuosso and Son's Construction had a crew out to begin work to separate the master bedroom into two rooms that will now be for Brenna and Brigitte. Brigitte slept in her own room for the very first time in 4 years. It was bittersweet for me, but truly amazing for her. She thinks she is such a big girl now. Yesterday, Derek Wagner of Wagner Electric came out to do some electrical work in the rooms and hallway. He had read about our family and donated all of his time yesterday and completed all the work for free. Bill Mower a local plumber and friend of a friend has been over to evaluate some plumbing work that has been needed got all of his supplies donated and is going to start soon as well. Eric Buck of Pollock Land Planning was also out yesterday and has a grand plan of constructing a new deck and patio area that is safe for Brigitte to finally be able to play outside. I also found out that Steve Babine from Landscape FX will be starting a fall cleanup next week. From what I am hearing there are many other people that have stepped up to help and are committed to getting this project completed. I would urge anyone in the Londonderry area that needs to have anything done in their house, yard, etc. to use these companies. There is something to be said of people who donate their own time and materials to do good for others. I know that none of them want or expect any thanks and are doing what they feel is right in their hearts, but wouldn't it be nice to reward them with patronage for a job well done!!

I am headed to Boston with Brigitte this afternoon for a few appointments. She is seeing her GI doctor and the nutritionist. We are still having major issues with her intestines and has lost about a pound in the last week. Back to Boston again tomorrow for her follow-up with her General Surgeon as well.

I will continue to keep you all posted with news on Sean, Brigitte, and of course the house. Thank you as always for keeping our family in your thoughts and prayers. We feel it and appreciate it.

If you are looking for more information as to how you can help with the makeover, please feel free to contact Kim Payson at bkpayson@hotmail.com My understanding is that the need is for materials right now. If you know anyone who can help, it is much appreciated!!



It takes a village to raise a child~~~~African Proverb

Much Love Always,

Trust....

Is trust a good thing or a bad thing??

I used to believe 100% that it was an amazing thing!! Today, I'm not so sure. Tonight, its bittersweet.

I am in a rocking chair beside Brigitte's bed with a tiny little light so I can see what I am writing. The monitors are all on. Beep, Beep, Beep....the slow, steady hum of her heart. She's stable right now. Lets hope it stays that way.

Today marked day 14 of Brigitte not having a bowel movement. Keep in mind she takes a double dose of adult Miralax, gets an ex-lax square every other day, and also got a few doses of Milk of Mag. We got NOTHING!! Today her doctor decided it was time for a cleanout. Typically this is done inpatient over a few days in the hospital. This time, its being done at home.

This is the email I got today:

We also don’t typically do this at home. But I know you will be with her and know B very well and we are trying to avoid hospitalization more so for B than for your typical patient. Look out for distension, pain, vomiting, in which case we can ‘t do this at home.

I think this makes sense:
(1) Golytely st at 50ml/hour for 2 hours, then 75ml/hour for two hours, and if doing well, go to 100ml/hour. If seems she is barely holding on but not looking worrisome, stay at 75ml/hour. Max is 2L.
(2) chemistry check tomorrow (chem10) to make sure electrolytes are not altered with the treatment
(3) she can have clear fluids by mouth during this time, but best not to have solids or full liquids. She will certainly loose some weight...but that is to be expected.
(3) Hydration 250ml NS three times per week IV.

What does this have to do with trust you say? Well, I am flattered that her doctors trust me so much. It is by far one of the greatest compliments I ever get....aside from those from other parents. I have learned how to do ALL of her care as well, if not better than some of the nurses we have encountered. I know lab values, I can read an EKG, I can deliver meds and suction, I can put her on oxygen, I can give her shots, and infusions. You name it, I can do it. All I have is a HS diploma. I have learned my daughter's conditions and can recite her entire 4 year history much like I can my own social security number.

This is a letter I wrote to a cardiac surgeon in Miami:

Dear Dr. Burke,

My name is Shannon Kaiser. I have a 3year old daughter, Brigitte 8/18/06, who is currently a patient at Children's Hospital Boston. Brigitte's primary diagnosis is Noonan Syndrome, amongst a very long list of other issues. In 11/06 she had pulmonary valve dilation in the cath lab and only had mild relief of the RVOT. On 12/08/06 she had extensive pulmonary valvuloplasty whereby they opened the RVOT and reconstructed the valve with pericardial patches. She also had her main proximal pulmonary artery dilated. After surgery she had a residual gradient with no pulmonary regurgitation in the setting of a very thickened and restrictive right ventricle. This surgery was performed by Dr. Emile Bacha.

Present day she has severe pulmonary regurgitation. The right ventricular function is normal with a moderately dilated right ventricular chamber. She also has an ASD.

Brigitte has had a very extensive medical course. She has MAJOR feeding issues and has had a LADD's procedure for intestinal malrotation 03/07, she has a Gtube 03/07, and has also had a fundoplication 11/07. All of these were performed by Dr. Jay Wilson. As of 09/09 she has had a Broviac CVL placed to initiate TPN due to failure to thrive. Despite being on TPN 3x a week in addition to a very complicated feeding regimen which also consists of 5 60cc bolus' of Enlive Apple Juice daily, and 12 hour continuous overnight feeds of Boost Kids Essentials 1.5 at a rate of 40cc/hr....Brigitte continues to lose weight. In fact, she saw her GI doctor for follow up on 12/18 and was 12.3k and was also seen on 12/30, weighed on the same scale and was down to 11.3k. There is no explanation for this. Her labwork is totally normal. She has been worked up for metabolic issues, thyroid issues, and diabetes. Dr. K(GI) did order a CXR and it was noted that there was change since her previous CXR and was found to have some significant prominence in her pulmonary vasculature.

The reason that I am writing to you is I believe that Brigitte's weight issues are directly being impacted by the insufficiency in her heart. Her respiration's have increased over the last few weeks. Previously she has been in the low-mid 20's and she has been consistent in the mid 30's lately. She is acting pretty normal though. She seems a bit more dusky around her mouth and does get a bit tired after she has been moving around for a bit. She is not losing fluids through diarrhea, nor is she vomiting. Her reflux is completely under control at this point.

I understand that historically children with isolated PS usually do not need intervention until well into their adult years, if at all, but Brigitte has never played by the rules. We have done everything we possibly can to explain away her weight issues. I should add that at this time she doesn't have any oral aversions and will eat by mouth often throughout the day. Every time I consult with another specialist, they all say it "sounds cardiac related." We are on the verge of adding in more TPN to her regimen and I feel as if we are trading one set of problems for another. It doesn't make sense to me to pump her up with nutrition if her heart could be forcing her to burn it all off. Brigitte has enough medical issues, that I don't want to add liver damage to the list due to longterm PN use. My husband and I are well aware of the risks involved in possibly replacing her pulmonary valve and/or closing her ASD. We feel at this time, given the situation, it may be an option that is needed to help take the strain off her heart and allow her to grow.

We have spent well over 300 nights at Children's in the last 3 years. She has had over 25 major surgical procedures, and countless other studies including, triple scopes, MRI's (never a cardiac). She has been worked up for pulmonary lymphangiectasia, celiac disease, hirschprungs disease, amongst other things. She has been severely truamatized and needs to have her ECHO's done typically with Ketamine/Versed. She is extremely fearful of even a simple examination. I share this just so you are aware of how complicated her sweet little life has been, mostly all related to her inability to gain any weight. I also have 3 other children. Brenna is 8, Jake is 7, and Sean is 6. They are all healthy. You may also be interested to know that my father has Hypertrophic Cardiomyopathy and I have Restrictive Cardiomyopathy and Mild Pulmonary Hypertension.

I am hoping that you may be able to help us,or at least give me peace of mind that I have explored every avenue for Brigitte and we are or have done everything possible regarding her cardiac issues. We are very willing to travel to Miami to see you.

Thank you in advance for taking the time to read this. I look forward to hearing from you.

Regards,

Shannon Kaiser



Here is part of his response:

Dear Shannon,
Thanks for the email, I'm sorry to hear Brigitte has had such a difficult course. Your summary is excellent, you may have missed your calling in medicine. I can imagine how traumatic this has been for you and your family. We have three little girls, and it would break my heart to watch one of them go through what you described. If I may, I would like to show your letter to my CICU director, he has a good feel for babies who fail to thrive after heart surgery, and I value his opinion.

Sincerely,
Redmond Burke



While of course it is very flattering to hear things like this from doctors......it puts an unbeliveable amount of pressure on me as well. Kind of like when she was rushed by ambulance a few weeks ago....and I thought she was constipated and dehydrated. Of course as that morning went on, I realized quickly that things were not as simple as I may have thought.....but nonetheless, I should have brought her to the ED that Tuesday night, not Wednesday morning.

I am not blaming myself at all. Looking back, I know I would have done things the same way. But, you can bet your butt the next time she is vomitting up green bile, we will be going in for an eval instead of waiting it out. You just learn to do things differently.

Tonight, I am learning how to do things differently. I am learning how to do a hospital procedure in my house, by myself, with 3 other children who still have to go to football, get homework done, take baths, make lunches for field trips, run to the pharmacy to get medications.......and, maybe eat!! Bryan is working overtime again!!!

It is becoming more and more apparent that her doctors are valuing my need to be at home with my other children, but are also entrusting her life to me as well. That may sound like a no-brainer......but, the pressure is indescribable. At least in the hospital I have nurses checking on her every hour. So if something happens, its not my fault. Tonight, if something happens, it is my fault. How do you live with that for the rest of your life. Again, you try to make quality of life decisions for 3 older children, but put another's at risk....or you make one childs needs more important and sacrifice 3 others. I can't win.

So today just reinforces why I hate Tuesdays. I have a feeling there are going to end up being a few more days of the week I come to despise as well.


“Trust yourself. You know more than you think you do.” Dr. Spock

I hate Tuesday's......

Screaming. It happens alot in this house.

Brigitte's anxiety is at an all-time high right now. Its not about to get any better. Every morning when she wakes up, I have to disconnect her TPN and gtube. The procedure for anything that involves her central line has to be sterile. Which makes everything a production. Getting her out of bed in the morning starts with screaming. Can you imagine if you had to start each and everyday already being afraid? Brigitte does.

Tuesday's are usually the worst days of the week. Tomorrow I will wake her up. Disconnect the TPN. Disconnect the GTube. Then I have to scrub the end of her line with alcohol for the length of time it takes me to sing the ABC's in my head. I don't sing to Brigitte, because distraction makes it worse. After I am done with that I have to flush her line with 5cc's of Normal Saline. Then I have to scrub the cap again. This time I sing Itsy Bitsy Spider in my head. Then I can flush her with 5cc's of Heparin. She screams bloody murder the entire time. No word of a lie. She is so sick of people touching her.

We will go downstairs and I have to take her temperature. She hates this. I do it under the arm. I have to track her temp twice a day to watch for any signs and symptoms of a line infection. Then we eat breakfast....or I do, because she doesn't eat. We get Brenna, Jake and Sean on the bus....and then the screaming again. She only can bathe on Tuesdays; just to her waist because she can't get the covering to the central line wet; and we change her dressing on Tuesdays.

I tell her that Pam or Rhonda are coming, and she starts to cry again. She knows if they're coming, its not for a visit. It's for business. I time her getting out of the tub so that as soon as they get here, we change the dressing. Changing the dressing requires a sterile procedure. I have to hold her down and push her face away from her chest. She wears a mask (tries too), I wear a mask, and the nurse wears a mask. Her chest is scrubbed with chlorohexadine and skin prep. Gloves are a must!! This is extremely difficult to do. In the hospital it takes about 4 of us to hold her down. She is so strong. You can imagine how hard it is with only me holding her down. I don't put her on the cardiac monitor anymore during dressing changes because her HR is through the roof and that just scares everyone!! After a few minutes the dressing is changed, we apply a special giant band-aid to keep the line from hanging in her diaper area...a big, big No No!!

After that is done, I get to hold her down again while she gets her IVIG infusion every Tuesday as well. This involves sticking her with a needle in each one of her thighs and keeping her on the couch and still for 1h 48minutes...the time it takes for a blood product to infuse into her little legs, which will hopefully keep her from catching anything during the week....even though she rarely leaves the house anymore. Without the IVIG on board, she would be in the hospital every month for weeks at a time due to common viruses that just give you and I a cold....they are deadly to her.

On a normal day, we would be bolusing her high calorie formula through her gtube every hour on the hour. We give her a break on Tuesday's. I figure it is the least I can do for her.

Normally, we would distract her and talk to her about school and who she is going to play with when she gets there. That can't happen anymore either. That's a story for another day as well.

This may sound pretty boring....it is. Why am I writing about it? Because I hate Tuesdays...and its Monday night. I won't sleep all night, thinking about tomorrow. She won't sleep much because I have to wake her every few hours to give her medicine and change her diaper......before the torture starts for the day.

Do you know how hard it is to sit and listen to your child scream for hours and hours and know that there isn't a damn thing you can do about it. I don't lie to Brigitte. I don't tell her that it doesn't hurt. It hurts like hell. It hurts her and it hurts me. I am supposed to be the one protecting her.....and rationally I am. You can't explain to a developmentally challenged child that holding her down and stabbing her with needles daily is the best thing for her. She just looks into my eyes and screams "Don't touch me"...."Don't touch me."

Those are the saddest, and hardest words any parent wants to hear. I hear them all day long. It breaks my heart. It's not fair.

Years 2 and 3

Brigitte's second and third year brought much of the same as the first year. She was admitted countless times for mostly respiratory infections and failure to thrive. We spent countless days heading in for clinic appointments and testing. The second and third years brought many, many diagnosis for Brigitte.

There were many more surgeries and procedures done to her little body. Her current diagnosis list is long!!

Noonan Syndrome
Mild Cerebral Palsy
Autism
Failure to Thrive
Type 1 Laryngeal Cleft
Pulmonary Stenosis
Hypertrophic Cardiomyopathy
Constipation
Hypogammaglobulinemia (supressed immune system)
Hypotonia
Lung Disease
Asthma
Gastroparesis
Small Bowel Obstruction
Poor Gastro-Motility
Global Developmenatal Delays

and the list goes on........

Her big surgeries of those two years included an attempt to repair a Type 1 laryngeal cleft, which is basically a hole in her larynx that allows food/liquids to flow into the trachea vs. the esophagus. This was to be a very complicated surgery. Brigitte had other plans. She had a laryngospasm on the OR table and the case was subsequently cancelled. We can't find a surgeon willing to touch it now....so she still has a cleft. We have learned along the way, the Brigitte does things in her own way. As much as this drives me crazy and makes for some very scary situations.....it also shows me that she is her own little hot mess!! She dances to her own beat and will never be ordinary!!

The other big surgery was another cardiac catheterization. Brigitte still gets very tired easily. Her lips turn blue and she sometimes has trouble breathing. Add in to that the inability to gain any weight, and it was time for another cath. It's funny when you get to a point where it's "just a cath", no big deal!! She did well for the procedure, had a little trouble with anesthesia and regulating her temperature, but otherwise did ok. We did get some very alarming news afterwards. Brigitte is now missing her entire right coronary artery and all the vasculature that goes with it. The theory is that she must have had a stroke at some point and the artery clotted off. Her left side has made up for the right but this puts increased pressure on her heart.

The problem with having alot of problems is they all make the body work a bit harder, which in turn means the body burns more calories than it should. Brigitte also has ADD so she is on the go non-stop! For those of you who know her, you know what I mean. She does not stop talking all day long! The decision was finally made after multiple hospitalizations and the lack of really good answers or solutions to place a central line in Brigitte and start TPN nutrition. She got her first line in September of 2009. In six weeks, Brigitte gained almost 2 kilos (almost 4 pounds). It was our miracle. Then the first line infection hit. She was a sick little girl and we spent another 14 days inpatient trying to clear infections from her line. It didn't work, and she was emergently sent to the OR to have the line removed. She lost all the weight we worked so hard for and was worse off than when we began. We came home for a few weeks, and then went back in December to have a second CVL placed. This time she came home on TPN 4 days a week and 3 days of IV hydration. The next line infection struck in February. This time it landed her in ICU. She was septic and not responding to antibiotics to treat the line infection. Another emergent trip to the OR to have the line removed. No more lines for Brigitte. She is immunodeficient, so bacteria just proliferates in her system once it gets there.

All of this back and forth is difficult on everyone. If you go on medical message boards people often refer to their healthy kids as UA (unaffected). This is so far from the truth and it is one thing that gets my blood boiling. My other three children are anything but unaffected. They may not go through the physical pain that Brigitte (and now Sean) go through, but the emotional toll is just as devastating...if not more so. There are many days that I feel as if I am sacrificing the lives of 3 other children to save the life of one. Brigitte has spent 373 nights at Children's Hospital Boston. She is only 4 years old. That means I have not been with my other 3 children for 373 days. Thats alot to miss out on. For me and for them. I don't doubt that I am doing what I have to do for Brigitte. I am not strong. I am not brave. There is no choice. She is my daughter. End of story. Having said that, each and every time Brigitte is sick, I leave three kids home without their mom. My kids ask me everyday "Mom, your going to get us off the bus, right?" Everyday I answer "Yes." BUT....there have been so many days, when that just isn't true. They come home and someone is getting them off the bus and they will spend days sleeping over someones house, or having multiple people stay overnight with them here at home. Not only that, but last year was almost unbearable. Hospitals had severely restricted their visiting policy to parents/guardians and grandparents ONLY!! No children under 18 were allowed in due to H1N1. My kids get extremely nervous when they can't see Brigitte. In their mind if they see her and she is on the floor not the unit, then they know she is sick, but she'll be fixed and will come home. When they can't see her for themselves, all hell breaks loose. They really suffer. Tremendously. My kids know what all of her diagnosis mean. They know how to flush a gtube and give her meds. The biggest thing they know is compassion. There has been so much good that has come from Brigitte and her life. It makes my heart smile everytime I see my kids showing empathy and understanding to children with special needs. So with the bad, there certainly comes alot of good.

I hope that this little history lesson has given you some insight into why we fight so hard "Saving Brigitte." I do have to add that these are just some of the highlights. She has so many other issues, has had so many other surgeries. If I tried to list them all, you would be reading FOREVER. I think you get the point. Yes, of course we have suffered alot of heartache. She has endured more than most. My next post will be of all she has accomplished. Thats quite a list as well considering half her doctors told us she isn't going to live....and the other half tell us "She won't do this, she won't do that".....and she has, and she will!!!

Some History......

It has recently come to my attention that there are now alot of people following this blog!! Thank you for reading. Thank you for caring. Thank you for helping in "Saving Brigitte." We are so happy you are here.

Some of you are probably wondering "Why are they Saving Brigitte? What is wrong with her? She looks ok to me." Looks can be deceiving. A perfect example of that old adage, Never Judge A Book By It's Cover.....is Brigge.

Brigitte was born on August 18, 2006. She wasn't due until September 11th. We had found out early in the pregnancy that there was something that wasn't just right. The first indicator was that my quad screen was abnormal. Just slightly, but enough for the doctors to ask us if we wanted an amnio. Bryan and I decided that we would proceed with a Level II ultrasound and if there were some issues with that, we would have the amnio. You know I ended up with the amnio, right!!!

During the test there were a few things that weren't right, the biggest thing was with the heart. Having gotten not so great news, I needed to walk out of there holding onto something. We decided to find out the sex of the baby and were thrilled with the prospect of learning that we were indeed having a little girl. Perfect. 2 girls, 2 boys.

A few days later I had what would be the first of three fetal echocardiograms. The first one showed that the babies heart was fine.....so did the second. By late June I had begun to have quite a bit of trouble breathing. My belly was so big, I could barely move. We had just moved into our dream house and I couldn't unpack a thing. At my next OB appointment, I was diagnosed with polyhydramnios. It means too much amniotic fluid. No wonder I couldn't breathe....but the baby was having problems too. In late July I ended up in pre-term labor and was admitted to Brigham and Womens for 10 days. I was having daily ultrasounds, and was also having amniotic fluid drained from my abdomen every other day. 2 liters at a time. We were transferred to the High Risk OB department. It was at this time that developed my hatred for a specific radiologist who made it her point to tell me something was very wrong with my baby each and every time she saw me. She insisted that the baby had a complex heart condition(that I was told repeatedly by Children's Hospital Cardiologists that she did not) and that I also needed to do some research on Noonan Syndrome. That is what she believed the baby had.

I will never forget being in my hospital room with my OB and looking at the most awful xeroxed pictures of children with Noonan Syndrome. Keep in mind this information she brought me was from the 1970's. It was awful. We were devastated. It was also during these ultrasounds that we learned the baby had bi-lateral pleural effusions and would likely have a very difficult birth. We were told that if she were to be born alive, than she would not be breathing.

On August 17, 2006 I started bleeding heavily. I had the other 3 kids with me and made an appt. to see the doctor. With the kids sitting on the floor behind a curtain, I had an obstetrical exam and was told I had a chronic abruption and they were admitting me to deliver the baby. Bryan came from work in his uniform, my mother in law came and got the kids, and my Dad and stepmom, my mom and my brother came to wait in the waiting room for news on Brigitte.

My labor was AWFUL!!! The longest I had labored with any of the other kids was 4 hours....this was a nightmare. 18hrs later, with an obscene amount of doctors, residents, interns, nurses, respiratory therapists, etc........Brigitte Erica Kaiser was born. No crying to be heard. I didn't even get to look at her. She wasn't breathing and was immediately brought to the NICU where she would remain for the next 11 days.

It was during this 11 days that I realized that this was going to be a very long road. As a mom, I knew looking at her, that something wasn't right. First of all, she wasn't breathing on her own. That's a big give away!! Her ears weren't as high up as the other kids. Her eyes seemed to be further apart. For a preemie, she was HUGE. 7lbs. 3oz. Mostly all fluid built up in her tiny little lungs. She did indeed have a very complex heart defect. Those were the big ones to deal with for the time being.

We were finally able to bring her home 11 days later. She was so much tinier that before. She had a very difficult time eating. She slept 23 out of 24 hours. She was a sick little girl. We learned that she had Pulmonary Valve Stenosis, Pulmonary Artery Stenosis, and an ASD. We also got a clinical diagnosis of Noonan Syndrome in September of 2006. That radiologist that I came to despise each and every time I saw her.....wasn't wrong about anything. At my follow-up appointment I made a point to go and see her. I told her everthing I had thought about her...and made her promise me that she would continue to do what she was doing. She was a great doctor.

We were going to the cardiologist every week for a while. In November 2006, Brigitte was really starting to struggle. She wasn't waking, wasn't eating, and was losing a substantial amount of weight (4mo old and still only 7lbs). Her lips were blue. It was time for her first cardiac catheterization. That was done the Thursday after Thanksgiving. It was the first of many times I would kiss her goodbye and hope to see her alive after surgery. I still to this day can't believe how many times we have done this. She has been to the operating room over 30 times in 4years. We never would have imagined that then...its still hard to do now.

During the catheterization they tried to dialate the pulmonary valve with little luck. Her pulmonary pressures were exceedingly high. She was in congestive heart failure. We were brought back to her room and I knew something wasn't right. We were supposed to have gone home. Her cardiologist, Dr. Dave Fulton, came up and sat in the rocker next to me. Note to self: When a doctor makes small talk with you, its never good news. Brigitte needed open heart surgery ASAP. They were trying to sort out the details and if the surgeon he wanted was available, the surgery was going to be done in the morning. Brigitte had other plans. She decided to have some complications after the cath. She ended up having open heart surgery on December 8, 2006. At the time, we referred to it as her second birthday. We stopped doing that when we realized that she would end up with a million birthdays if we kept that up!!!

She was in surgery for over 6 hours, but the repair was done. She will likely need a valve replacement in the future, but we are hoping she can wait until she is in her teens before it becomes more of a need. Valves for children are artificial and don't grow with them. So although she would probably do better with a new valve now, it means open heart surgery every 5-7 years for a replacement until she is old enough to receive a donor valve or a valve from a pig. We'll cross that bridge when we come to it.

While recovering we decided to take advantage of some feeding issues she was having. She could projectile vomit like nobody's business!! Her reflux was out of control!! We left the hospital with a referral to GI who was going to see us after the holidays.

Needless to say, that Christmas will go down in the history books. It was amazing. We were so thrilled and it became so apparent to us that we just needed to live life as if each day were our last. When you face struggles of this magnitude, I think it does one of two things to you. You either enjoy every moment and treat each of them as a gift....or you get angry. We chose to just be thankful and enjoy what we were given...for the time being.

We got through the holidays and were looking forward to getting some answers on the reflux piece of things. The first thing we did was get some films of her belly. She screamed...as she always did. We were then told to wait and someone would be with us shortly. Out comes the doctor....talking about the weather, the Patriots, etc. Small talk. Something was wrong. It turns out Brigitte had a malrotation of her intestines. They were twisting in knots inside. Not a good thing.

In March of 2007, two months post-op open heart surgery, Brigitte underwent major surgery to "re-design" her intestines. She also had a Gtube placed at the time to help her with some much needed weight gain. She rocked surgery once again. She is a superhero!!

Throughout the spring, summer and early fall...we were inpatient ALOT!!! Always for constipation, vomitting, and failure to gain weight....and multiple respiratory infections as well. We spent countless days at CHB (Children's Hospital Boston) doing testing, taking her to the OR for endoscopies, bronchoscopies, etc. All the while, she was getting worse and worse.

In November of 2007, Brigitte underwent another major surgery to help control the vomitting. It was called a Nissen Fundoplication. This basically meant that the surgeon was going to wrap her esophogus around her stomach and make a knot so she could no longer vomit. Sounds fun, huh? Brigitte will never be able to eat hard candy, hot dogs, grapes, cherry tomatoes, or soda due to the choking risk. She also cannot vomit, so anytime she gets a stomach bug etc....she needs to be admitted and have a naso-gastric tube placed down her nose and throat do releive the gas etc. Brigitte has since been able to "breakthrough" her fundo and can vomit small amounts...unfortunately, she can also still reflux and choke on liquids which leaves her with recurrent respiratory infections.

My hands are getting tired...as is my brain. So, I will leave you with a video that I made documenting her first year.

Make an on-line slide show at www.OneTrueMedia.com

Play On......

I may have mentioned a time or two before that music plays a big role in my life and how I cope with things. This week I bought George Strait's CD....with the song The Breath You Take.




Music plays such a huge part in how I cope. I know, I just said that, right?!?!? I spend countless hours in the car. Usually back and forth to Children's Hospital Boston, or to football, cheer or baseball. My car is my house. Or as Bryan so often refers to it "the rolling dumpster." LOL!!! It's so true though. You try having four kids in a car all the time.....add to it, one who pukes constantly. It's not a pretty sight!! Anyway, I digress. Sorry.

This song has hit home for me in so many ways. I know we have slowed our pace tremendously after the birth of Brigitte. Tremendously. I can honestly say, that we are a family that really does enjoy so much of the little stuff. Having said that....the last few weeks have taught me that I haven't slowed down enough.

September 8th will be a day I won't soon forget. Although in many instances, I wish I could erase it from my mind all together. It was 12 days after my son had major brain surgery. We were faced with our scariest night yet. We were losing her. Mine and Bryan's biggest fear. It was on our doorstep. We said our goodbyes. I kissed her goodbye. I took pictures. I made videos of her whispering "I love you, momma," because she was to weak to yell it in that loud boisterous voice we have all come to love and hate at the same time!!! I had to let her go from my arms and let Bryan hold her because I knew if something happened, he needed to hold her and love on her for the last time too. No parent should have to do this....especially not multiple times like we have.

I don't know what her future holds. I really don't. My gut tells me it isn't a life like my other kids are likely to live. My heart tells me, I can't ever let her go. I told her before they took her away from me that she needed to fight. That we had so much left to do. My heart told me that she has fought so hard for so long and that I couldn't blame her if she was done. I am so thankful that she decided that life is still worth fighting for. It blows my mind that despite all that she has gone through, that she believes that too. I don't think that I would. I think she is stronger than anyone I will ever meet. She knows that life on the other side will be so much easier. So much more beautiful. Instead she chose to come back and struggle because her mommy, daddy, brothers and big sister need her so much more than she needs us. For that, I will be FOREVER grateful.

Brigitte is home now. We go to Boston a few times a week. She even bought herself a ride back to CHB by ambulance the day after we got home. Maybe she's changing her mind. I don't know. All I know is that she is still here. Still fighting.

We run TPN (total parental nutrition) through her central line 7 days a week for 12 hours a day. It's keeping her alive. Her bowel sounds are faint. We don't know what this bowel is going to decide to do. I have my theory. Let's just hope I'm wrong.

We are managing as best we can at home. The kids all know that life isn't the way it is supposed to be. We are just making the most of each moment the best we know how. We are also blessed to have so many people coming into our lives because of our struggles. There is still so much good out there. Londonderry is proof positive, just ask the Londonderry Wildcats.....and so many more of my friends who are determined to make this as easy as they can on us. We will never be able to show our gratitude the way it truly deserves to be shown. All I can say is that we count you in our blessings each and every night.

I should also let you know that Sean's follow-up wasn't what we had expected it to be. He is still continuing to have headaches and numbness in his hands and feet. He is going to have another MRI on October 12th and follow up with Neuro-Surg right after. Dr. Warf thinks he may need to have a second brain surgery. We have talked to Sean at length about this and he just wants his headaches to go away. Who can blame him. We are gearing up for this as we wait for those bowels of Brigge's to wake up!!!!

We'll get through. We always do.

“Being deeply loved by someone gives you strength, while loving someone deeply gives you courage.”

More Reality....

So it seems once again that the title of this blog is more than appropriate. We are always medically managing Brigitte to help her...to maintain her quality of life. Last night, we were truly Saving Brigitte.

On Tuesday night, Brigitte began getting sick at the football field. As I stood with friends, I said "Here we go, she's going to end up with a pneumonia." Little did I know that 24 hours later, Brigitte would literally be on her deathbed. She was dying and I didn't know it. I hadn't spent the night before hugging and kissing her. Hadn't spent the afternoon loving on her or sitting on the carpet playing baby dolls. Instead, I was so thankful when the bus had come to pick her up because I was going to get some quiet. I let her walk around the football field talking up everyone who would listen to her. All while talking with friends, I couldn't wait to get home and get all the kids to bed so I could rest. That was until 6pm and she walked to me vomitting. Not a terribly unusual happening, but I knew I was in for a long night.

I took her home, got her settled. Friends were good enough to bring Jake home later. Brigitte began to wretch and vomit more and more. She was up all night. I was buying time to get the kids on the bus and get her to the pediatrician. I decided to keep Sean home so as not to get a call from the school saying he had a headache while I was trying to deal with Brigge drama. So the bus came, we hopped in the car. I stopped at the gas station realizing Sean hadn't had breakfast...so I grabbed him a box of Wheat Thins and myself a Vitamin Water....meanwhile, Brigitte was tanking in the car.

Upon arrival at the pediatricians office we endured the nasty looks of those in the waiting room as my daughter's tounge was literally hanging out of her mouth while she wretched. The doctor immediately saw us....and immediately called for an ambulance. We were headed to Boston. The EMT's arrived and refused to transport her. We had to then wait again for a paramedic to come and off we went. Thankfully we were able to secure a direct admit and bypassed the ED all together. She got to the floor and was the sickest I had ever seen her. Her BP was dropping, as were her vitals. When Brigitte crashes, she crashes hard and she crashes fast. She had an XRAY which was alarming, and then a subsequent CT Scan which was worse. Within the hour she was prepped and headed in to the operating room.

The surgeon said "Pray for a miracle. She is a sick little girl, and things don't look good." She was showing signs of a necrotic bowel. We were told to expect her to lose most of her bowel along with some of her liver. They even talked about putting her on the transplant list if she met the criteria after surgery. We were told to pray....and so we did.

About 4 hours later we were told that she had a complete small bowel obstruction due to adhesions from a previous surgery. Her bowel was gray and dying......but after removing the blockage, they did see signs of blood returning and the bowel had started to pinken up. This was good news. This was our miracle.

She was transferred to ICU following surgery. She currently has a central line, 2 peripheral IV's, an Arterial line, Foley Catheter for urine, Gtube for drainage, a naso-gastric tube for even more drainage, and an epidural for pain. She has barely been awake since Tuesday night. She has a large bruise on her bowel that has the potential to further damage her organs. We won't know if it has caused a further blockage requiring removal of some of the bowel until she can begin getting fed again.....which could be up to a week away. She's alive, but very sick.

So here we are again planning for another long admission. This having been the scariest yet. We once again have to change the entire house around to accomodate her when she comes home. She will need to be kept away from as much illness, bacteria, etc. Its proving to be our biggest challenge yet.

Adding to this is that poor Sean is still recovering from major brain surgery less than two weeks ago.

I want to sit here and complain. But I can't. The nurse just walked in and said "The pain service will be in shortly, they got held up in the end of life room." Yes, such a place exists in our reality. So while I know that I have much to be unhappy about. I have so much more to be grateful for. We're going to get through this.

Brigitte is alive. Its all that matters.

Strength...

“Anyone can give up, it's the easiest thing in the world to do. But to hold it together when everyone else would understand if you fell apart, that's true strength.”

So there has been alot of talk about strength lately...mine, Bryan's, Sean's, Brigitte's, and Jake and Brenna's. Where do we get it? How do we do it?

I don't know the answers to either of those questions. What I do know is this. I didn't sleep at all last night. Tossing, turning, thinking. Worry, angst, control, sadness, laughter, tears....you get the picture. I went to bed early last night to get myself ready for all I have to do today. Including picking up Sean and Brigitte's medication for school, dropping off physical forms and having conversations with both school nurses about the kids, having a chat with the principal about tutoring for Sean, going to order glasses for Sean, getting a backpack for Brenna, getting Brenna a haircut, and then taking Brenna to cheer!! School starts tomorrow so there is alot to be done! Suffice to say with a normal week things would be crazy...but then there is Friday. Back to not sleeping.

I went to bed alone. Bryan was working another OT shift and wouldn't be home until midnight. Jake asked if he could come in and watch TV with me and how do you say no to that? Around 11:30 I sent him back to his bed and got myself as comfortable as I could. Bryan came home at some point because when I woke up at 3am he was there and so was Sean. Sean was cuddled up beside me as close as he could get. His head resting on my chest....soundly sleeping. I really had to go the bathroom, but it was going to wait. I needed to soak up this moment for as long as I could. What if it was the last time it ever happened? What if something goes wrong with the surgery and their are complications? All questions any parent would ask if their child was undergoing any procedure...big or small. Its just human nature. So I went back to taking in the sweet smell of lavender in his hair. Listening to his snore song. Watching his chest rise and fall. Holding him as close to me as I could. He gasped a minute and I jumped.

It was at that moment that I remembered that this isn't about me at all. This is about a 6yr old little boy who is undergoing brain surgery on Friday. I am not the strong one. He is. I am just his mom. I am just doing what I need to do to make this as easy on him as possible. We bought blankets and pillow pals. Picked out pajamas and word searches. We've got a bag ready to be packed. He is ready to do this. I am just there to pick up the pieces when he is out of surgery. To be there for him when he is scared walking to the operating room. To be there as they place a sweet strawberry scented mask over his little mouth and put him to sleep. I'll go back out to the hallway and break down with Bryan. Hug him. Beg God to keep him safe and get him back to me as the same Sean. Compose ourselves to go wait with family for the 4-5hour long procedure. Knowing the anesthesiologist is placing a tube down his throat. The residents are putting IV's and arterial lines in his little arms. While Dr. Warf makes a delicate 6-7inch incision down the back of his little neck and carefully proceeds to remove a part of his skull and brain. He will slowly wake up alone in the PACU and then they'll come and get us. Then we can begin to get him better and bring him home.

So, as I said. I am not strong. I am just his mom. Doing what every single mother out there should do for their child when they are sick and scared.

The moment....

Today I was at CHB with Brigitte for her psych appointment and for her first infusion of the new drug, Hizentra. She will get Hizentra once a week at home through two subcutaneous needles vs. getting IVIG once every three weeks via a peripheral IV. Her peripheral access is getting really bad, so we have been very lucky to get the go ahead to try this new drug.

Her appointment with Psych was at 10 and then the infusion was scheduled in the CAT/CR at 1pm. We had some time to kill in between and made our way to the cafe to check out the daily special.....lasagna or spareribs (YUCK!!!) So we headed back to Au bon Puke for some overpriced Corn Chowder and a tuna sandwich. How exciting. My mom and sister Alexis were kind enough to come with us today and served as great distractors!!

The moment came while I was standing at the soda fountain filling up my cup with diet pepsi that I swore off weeks ago. Since Sean's diagnosis, it has been my go to drink when I am not overinduldging myself with Kahlua Mudslides on the rocks. Don't Judge!!! I found myself becoming completely overwhelmed right at that very moment. It was high noon, and the place was mobbed with people. Here I am stopped dead in my tracks with tears rolling down my cheeks.

What happened? I guess what happened was that I realized my life has become Children's Hospital Boston. My sister had just made the comment "Shannon, your like famous here. You know everyone." I think it finally had sunk in that I have spent so many days living, breathing that hospital that even the staff at Au bon Pain knew me. We ran into countless nurses, doctors and friends just in our short venture from Fegan 10 to the Main Lobby, all with a ready smile and a big hello for Brigitte!! She is a face you don't forget. She is the one who always has the ability to make someones day. They love her. What made it so real today was that we happened to run into Brigitte's geneticist, Dr. Amy Roberts. We exchanged pleasantries, and then got down to the nitty gritty of Brigitte news when we both realized we were having a very intense conversation about Brigge in the middle of Au bon Pain as if it were nothing. Dr. Roberts was the first to realize "Lets have this conversation somewhere else." Me: "Are you free Friday, we'll be on the 3rd floor Surgical waiting area waiting for Sean." Her: "Perfect, when I get a break, I'll come up and we'll finish."

Seriously???? Sean is going to be laying on an OR table with his head cut open and I am going to be multi-tasking taking care of Brigitte business in the waiting room. This is our reality. I totally lost it.

I quickly composed myself, wiped my eyes with my T-shirt....and headed to the cashier. Proceeded to pay, sat down with Mom, Alexis and Brigge....got on with our day.

I guess what I am trying to get at is that emotion comes at different times. Often, inopportune times. Sometimes you have to just go with it. Let yourself have that moment. Acknowledge it and move on. For as you know, life goes on with you or without you. Whether your home and able to weep privately or standing in the middle of Au bon Pain with a tray full of corn chowder. If you don't have these moments, something is wrong with you. I have decided that these little moments are what continue to remind me that this isn't a dream, nor is it a nightmre. Its reality and we just need to make the best of it. Even if it means multi-tasking in the surgical waiting room.

Sean and his Chiari

Wow, guys!! It really has been a long time, hasn't it!! So much for me being better about this, huh?!?!?

Well another day, another diagnois. This time, another child.

We recently found out that Sean (my 6 year old) has a Type 1 Chiari Malformation. He will be undergoing major brain surgery this Friday at Children's Hospital in Boston.

Shock and disbelief do not do our feelings justice. We had just started to get ourselves on a good path, and then WHAM! Out of the blue this happens.

Bryan and I had a long conversation last night about how we are truly feeling about this. I came to the conclusion, that honestly, I just don't know how to feel. When it comes down to it, I am so angry. Angrier than I have ever been about anything that Brigitte has gone through. This in itself, is leaving me so angry. Why am I not more upset about things that Brigitte goes through? Have we just learned to deal with the fact that this is her life and none of it is such a big deal anymore? How sad is that for her? Or is it that we just have never realized the full impact all of this has on her on a daily basis because she hasn't been old enough or verbal enough to tell us how she really feels, unlike Sean?

Sean is surely telling us how he feels. He is so angry. He is so sad. He is so scared. Why shouldn't he be? He is about to endure one of the worst things in his lifetime.

My question is why does this keep happening to us? Why my kids? Does it make me a bad person to question these things? I continue to wonder what I have done that someone feels so strongly in punishing my children for something I have done. Surely, they have done nothing to deserve this. Maybe I am looking at it from the wrong angle. I know that I should be looking at it from the perspective that someone is using me to show the world how it should be done. That you can get through trials and tribulations with grace and dignity. That you should always look at things knowing it could always be worse. We are the family that comes together and just does what needs to be done whatever the days calling may be. I keep telling myself that there has got to be some wonderful thing that is going to happen to us. Maybe we'll hit the lottery? Maybe we'll be the millionth customer at Hannaford and win a years worth of free groceries? This is what I tell myself when I walk into my upstairs bathroom that still doesn't have a sink after I broke it 3 years ago with my hair dryer....I put a big hole in the porcelain. This is what I tell myself when Sean and Brigitte sneeze and cough all night because of the rugs that so desperately need replacing in our bedrooms. This is what I tell myself when Wells Fargo wants 10k for our new mortgage modification that I just don't have. This is what I tell myself when Bryan works his 3rd 16hr shift of the week and is only going to get 3 hours of sleep for the umpteenth time this month.

Then I remind myself that complaining and wishing doesn't make the present any easier to deal with. That we just have to get up another day and keep doing what we are doing. That my kids are smart, beautiful, honest, respectful, loving and more importantly, ALIVE. That we are doing a good job despite how hard we struggle. That once again we will get through this trial and be better off on the other end.

At the end of the day, it really is hard to believe in all of that. I have to though, its what keeps me going.

Pray for Sean!!!

ENJOY.....

Fear......

Sorry, again for the delays in posting. I really am going to try to be better about it. Right now, I have posters to print, a house to clean, laundry to fold, and bags to pack. I have to get all this done in the next 36hours or so....as life as we know it begins back again at our second home, Boston Children's Hospital. So, having said all that, like I said, I am trying to get better about blogging, but I don't know when to fit it in.

Oh, I glazed over the "back at CHB again part"....yes, that is my brain trying to ignore it. Can't though....its happening. Friday morning we have to be in bright and early. She is having another MRI of her brain. Due to some recent developments...well, not so recent, just recent that someone has decided to listen to that "crazy mother with the 4 kids!!" They have a bed booked for her to spend the night due to the length of the scan and the amount of images they want to get. It seems as if "we may have greatly underestimated the damage to her brain" (neuro's words, not mine)...and he wants this done ASAP. I might also add that he is working her up with a strong suspicion for Mitochondrial Disease and the possiblity that she may have suffered a stroke. Hence the title of this entry...FEAR.

I am usually the one giving all the comforting words to all the family and friends when they hear the scary words that often come out of my mouth about Brigitte. Don't GOOGLE it. Don't read about it. Don't worry, she'll be fine.

I'm still doing all of these things, except the one person who is usually telling me not to do those things, the one person who keeps this semi-calm person from getting themselves worked up before we know answers, the one person who always tells me "Don't worry until I get worried," is now worried himself. So the fear sets in.

I think people often forget that Brigitte's illnesses....many of which we don't know the full extent of yet, are life-threatening. Those that aren't, have the potential to be life-altering. We live in a constant state of "What if?" and "When?" Just when we think that things settle down and we can begin to plan, they change again. Often these fears go unspoken. Nobody wants to ask the questions. They just look at you, smile and say "well, at least she's cute!" This is less than helpful. We don't get statistics or guesses because there isn't another Brigitte to compare to. She is her own little mess.

So it leaves us worrying. The more and more people diagnose her with things, the more fear I have. I'd be lying if I said I wasn't afraid she might die. She might. I will bring her back into that hospital on Friday morning, let them intubate her for the "I can't even count that high" time.....and we will sit and wait. Hoping she doesn't come out with vocal cord damage ( a risk of intubation....its gotta happen sometime, right?) Hoping she tolerates the procedure. Hoping we don't get devastating news on Tuesday when we go back for the results.

We live in a constant state of fear. Some days are easier than others. Most suck. Some may say this is all very dramatic. Might be. I can tell you that those who think that, have never endured their child being sick. Really sick. It doesn't leave you. You will never understand it no matter how hard you try.

Sean and I went on his Field Trip to Squam Lake last week. The pollen had been terrible in this area for a few days, and then the smoke from the fires in Quebec moved in. We ended up having to leave early because he was having alot of trouble breathing. He's my asthma and allergy guy for those of you who don't know. Anyways, his teacher had the kids write Thank You's to the chaperones. This was Sean's:

Dear Mom,

Thank you for coming on my field trip with me. The show was cool. That was the best field trip ever. I wish we could stay for the rest of it. But I was sick but not that sick like Brigitte.

Your son Sean

He is only 6 years old. This is not the first time he has written about Brigitte and how sick she is. He obsesses about dying. Where people are buried. Does it hurt? He often says "When Brigitte dies, will I get to see her again?" He thinks she's going to die. This breaks my heart. This makes me terribly afraid. I am often more afraid of how this all affects them more than it does Brigitte. Physical scars are one thing, but emotional ones are completely different. You should see him with her. He literally smothers her each time he hugs her. It's as if he is afraid that he can't get enough of her. I feel that way sometimes too. Look for a post soon of some pictures we had done on Brigge's trip. Your heart will smile when you see how much love these kids all have for each other!

Don't get me wrong, I don't let this fear consume me. We make the most of every single day. We enjoy the good when it is good....and always say that the worst could be worse. We are not sitting by letting all the good pass us by. Its just that the bad never leaves us.......sometimes this is a blessing. It allows us to really soak up the good and appreciate it.

So what is the moral of this story? I don't really know. I guess it is that writing helps with our fears. If we keep them all bottled up inside they grow and grow. It helps me to write all of my crazy stuff here for all of you to read. It helps Sean tell a story about how he is really feeling. I think you can learn alot about people and what they write. We are all afraid of something. What are you afraid of? Do you talk about it? Do you write it down?

You might want to. It really helps.

Reality.....

So, did you figure out where she was happiest?? Yep, it was in the pool. Which is where we spent a good deal of our time. That was awesome.

Todays lesson is all about reality.

How so you say?? Well, perfect example happened yesterday. Brigitte gets home PT and OT services since her medical fragility has not allowed her to be in a clinic setting. Thankfully, she has progressed to a point that we are making the transition to outpatient therapies in July. This will allow for more and/or different therapies to take place. It is also an opportunity to take Brigge out of her comfort zone. Currently, she feels very safe at school and very safe at home. We venture to new places and well.....all hell breaks loose. Which is why Disney was a partial disaster! She will have an opportunity to take place in pool therapy and also some animal integrated therapy in which both will help with the sensory issues we are seeing so many problems with.

Part of the problem has been brought on by us. Yes, I said us. Her family. The very people who love and care for her so tremendously. We have created a bit of a brat. I do remember making this prediction a long time ago.....it has come to fruition ten-fold. We have done everything for her. We also laugh at her bad behavior. I will share a Disney story here.

On our last night before leaving we decided that we had had enough of buffets, characters etc. We spent the afternoon at the pool and were sick of eating "pool food." We were craving good food, but were to exhausted to trek on out and see what we could find. We decided to settle at Il Mulino at the Swan. Easy Peasy. Pasta and wine does wonders for everyone, right?? What kids can't find something they like at an Italian restaurant. It was perfect. Add to the fact that it was EXPENSIVE...that meant there was no need for reservations!!! Most families on property were not eating here, but at the cafeteria. For those of you who know my children, we can really take them everywhere. They are very well behaved and we are always complimented on their behavior. So who would figure this would be a problem?? Not I. Not Bryan. It was apparent that the hostess felt otherwise. Lets just say she wasn't thrilled to see us and it was even more apparent by the table she chose to seat us at. I will add that we were not the only family in the place.....but we were the only family with more than 2 kids. Things started out relatively well. Bread was on the table, kids had drinks, dinner was ordered. No complaints. All I was thinking "I'll show that hostess." Ok, Ok.... I was really thinking "hmmmpfh....I'll show that bitch!!!" Well, Brigitte was about to do it for me. At the top of her lungs Brigitte proved the hostess completely right by screaming "FUCK!"...repeatedly. Yeah, good times. As mortified as I was, all I could do was laugh. The kids laughed even harder. I think Bryan was choking on his breadstick. Good Times.

We are part of the problem. A big part. Brigitte's therapist brought me back to reality yesterday. I was telling him how demanding Brigitte can be. She had me thinking the other day "I can so see why parents shake their children." She drives me to the edge of every cliff, every single day. The kids get sick of hearing her and give her what she wants. Worse though, all of us have given her everything she wants because the majority of her life has been so difficult. We have all done what we could to help her. Make life a little bit easier.....on all of us, honestly. So there I was, feeling responsible for partly creating this monster!! For allowing it to go on even though I fully knew a long time ago what the implications would be. Jon, her PT, reminded me "Shannon, she almost died.....multiple times. None of you know what her future holds and you all do what you can for her to make her difficult life a little bit easier and love her as much as you can. There is no fault in that."

He is absolutely right. I know this. I have thought about it constantly. It is what keeps me up at night. I know the kids think about it. Its hard to know what Bryan thinks about it. If the gray in his hair and his blood sugar told the story....I'm sure it would say it is his biggest worry too. The problem is, nobody talks about it. It is the big white elephant in every room.

So the moral of the story is, don't hide behind your reality.

Here is a picture of our Disney reality. I LOVE it. It is one of my favorites!!

Honesty.......

Todays lesson is all about honesty. So with that being said, it has always been my intention that this blog will tell it all like it is, no mincing words or fake pictures!! Just real life. Our life. Which hasn't been all that easy on so many levels. I promise to not sugarcoat things.....or people. Keep in mind though, these are my feelings. MINE. Not anyone elses. I will not apologize for them. They may change with the weather. I may feel something today, and tomorrow could be just the opposite. I can guarantee that someone will read into what I say too literally and take offense. Just remember this blog is not about you...it is about me and about Brigitte and how I am surviving trying to raise my 3 older kids and keep her alive all at the same time. If you feel "guilty" about something I may say or do...thats on you!! I might even lose a few friends or even family members along the way. Again, nothing new...it happens. I'm over it. I'm done apologizing for my life and how I live it. I've done it for far to long...and I always seem to be the one apologizing even when I haven't done anything wrong. Not doing it anymore!! I have a job to do. It's mothering my four children the best way I see fit. Trust me....sometimes I really suck at it. I fed them cake for breakfast every day last week because I didn't have the energy to go to the grocery store. Your probably wondering what they ate for dinner then?!?!?!? That would be McDonalds, Burger King, Wendy's, and Dunkin Donuts. I did manage to get a salad in them on Friday at the Pizza Wars. BONUS!!! Me, I don't eat that stuff. Lg. Diet Coke was sufficient for my 3 squares a day. I went shopping yesterday, so don't go reporting me yet. Last nights dinner was steak tips, zuchini and summer squash and they even ate rice cakes for snack. Brigitte's even had about 6 strawberries today!!! Whoot!!! Whoot!!!! When I'm on, I'm on!! When I'm off, I'm waaaaay off. They'll live. I hope. I'm sure there will come a day when they are forwarding me the bills from their therapist. It all gets blamed on the mother anyways. I will feel ok telling them "I did the best I could and I loved you," even if I was feeding you cake for a week!

My intention when I started this blog a few weeks back was to update you live from Disney. Welllllll, we all know that didn't happen for a myriad of reasons. The biggest being the computer issues we had, but the other being that this "wish trip" wasn't all we thought it would be....or all I thought it would be. I am settling in back at home....slowly. I think they call it Post Disney Depression...even though when we were in Disney, I wanted nothing more than to be back home. GO FIGURE!!!!

I spent months planning for this trip. That was probably my biggest mistake. I had heard so many wonderful things that happened for Wish Families on their trip.....none of which happened for us. I don't want this to come across as complaining. Really, I'm not. Just consider it a lesson for all who go after us. Wish family or not. Let it happen.

Brigitte had been having alot of issues prior to us leaving. Things we have always seen, but haven't been able to deal with because all of her medical issues have been front and center for so long. These are behavioral in nature, and things that we won't be able to fix..... but will just make tolerable. She is having a hard time with noise. Loud and soft. She hates crowds. She bangs her head on the floor and hits herself now. Lots of "hand flapping" with over stimulation. Word retrieval....bigtime problem!!! Had we been focusing on these issues all along, maybe the trip wouldn't have been so bad....maybe we wouldn't have encouraged it to be a wish at all. Not beating myself up about it. Again, I do the best I can!! We are at a point where not alot can be done medically unless we make some major, major decisions...so lets focus on the things we can help right now. Neuro issues taking center stage! For those of you following over from the CarePage, if your wondering where her weight is, its down.....waaaaay down. 11.9 kilos at last glance. Ummmmmm...haven't we been there for about 2 years??? Yeah, I thought so too!! I know, I know. She'll get there. She will. When she is ready!

In a nutshell, despite all of the "issues" we experienced.....we really did enjoy ourselves. After figuring out what worked and what didn't. There was more of the "didn'ts" than the "dids"....but when it did, it really did!! Those are the memories I will keep in my heart. The others, not to be forgotten, just hidden away with alot of the other unpleasantries that come with "Saving Brigitte."

More updates to come. Soon. I PROMISE!!! It just has to wait until my dog stops puking and having diarrhea. Thats what I woke up to at 5am. If its not the kids its the dog. If its not the dog, its the husband.


I leave you with a few pictures...you can see where she was happiest!!