Baby Genevieve

Saturday, June 12
So, yesterday was my birthday. Faith and I had a wonderful time at dinner with Blaise and her parents, and Ellie and her parents! I love being able to get together with parents when we all can understand pretty much exactly what everyone else has been through. The good news is, we will get to see them again tomorrow, as we all walk in the NSTAR Walk for Children's Hospital!
I mentioned a family in my last post that was coming up from Jacksonville, so that their little girl could get on Omegaven. I met Tiffany, the mom, today, and immediately all of my feelings from March of last year came rushing back, and I remembered everything I felt then, and was overwhelmed by how far Faith has come in that time... We talked for over 2 hours, and will hopefully get together once more before Faith and I go home.
As probably everyone who reads this blog knows, I believe everything happens for a reason. I didn't schedule Faith's appointments for this checkup, they were scheduled by Alexis, and I just bought plane tickets accordingly. When I found out the checkup was so close to the walk, I decided we might as well stay out a couple of extra days. And now, it just so happens that this family, whose story has been so similar to ours (and yet in many ways, so much more difficult!), comes to Boston on the day that we otherwise would have gone home!
Tiffany is here with Genevieve alone. Her husband, David, is home with Genevieve's twin sister, Gwendolyn, and her three older siblings, Jocelyn, Nathan, and Zachary. I remember how difficult it was to be here, in this huge strange city, by myself, with a sick baby... knowing that my son and husband were at home, going on with life without me. It was the most difficult, yet necessary, thing I have ever done.
I also clearly remember the costs of it all. Housing is not cheap, even in the patient housing ($30/night). Gen is in the NICU, so Tiffany can't just stay with her at the hospital, like I could when Faith was inpatient. And if you think groceries are expensive where you live, you should go shopping here sometime! On top of that, Tiffany did not have the "luxury" of flying commercial- they came on a Leer jet as a medical transport- so she had to pack pretty sparingly, and probably doesn't have a lot of the supplies she would have packed for when Gen is eventually outpatient, if she'd had room to pack them. (I was able to bring Faith's bouncy seat and a nice stroller, and we were fortunate enough to have a pack & play with a bassinet donated to us by one of our blog readers.)
I'm telling you all of this because Baby Genevieve and her family need help right now. They have been through so much, and they have so much of the journey still in front of them... I am going to copy their story into this post now, and I want to ask that if you have ANYTHING at all that you can give, anything from $5 to even being so generous as to donate a pack & play w/ bassinet for Gen to use when she is outpatient, please contact me, or go to one of their websites to make a donation: www.prayforgenevieve.com or www.brorsfamily.com

Genevieve Brors is a 3 month old little girl, born with gastroschisis. She is a twin sister to Gwendolyn and little sister to Jocelyn, Nathan, and Zachary, and daughter to Tiffany and David Brors.  Genevieve was at Wolfson’s Children’s Hospital in Jacksonville from when she was born on Feb 17^th until June 11^th . She is now at Children’s Hospital Boston.

This is her story, as told by her mother, Tiffany:

Genevieve has had a lot of complications in her short life.  She was born with gastroschisis (her entire intestine on the outside of her body).  A portion of her small intestine had died off and therefore was not attached to her stomach.  Because the actual intestine was not attached to anything at one end it did not develop normally and was shriveled and full of holes.  At 3 hours old, she had her first surgery to repair all of the holes in her shriveled intestine.

She spent 8 weeks with an ostomy bag (a bag to collect everything before it goes into the bowel) so that nothing would pass through her intestinal tract, allowing it to heal.  When Genevieve was 8 weeks old she had her second surgery, which reattached her intestine to her stomach and closed up her belly.  

Things kept going along well, then we would have a set back, then they would get better, then we would have a set back.  Finally we thought we were about ready to take her home.  She was eating well and going to the bathroom like she should!  We were so excited to have her coming home.

On Mothers’ Day I was there visiting with her and she was acting strange, crying a lot and just seemed uncomfortable.  It was just out of character for her, she is a normally happy alert baby who is easily comforted.  About 7pm I left and on the way out the door I mentioned to the nurse that she seemed gassy and to please ask the doctor for something to relieve her pain. By the time I got home Genevieve was almost dead.

I called the hospital when I got home just to check on her and I couldn’t get a hold of anyone.  It took about an hour and the doctor called us to say that she was really sick and they were calling in the surgeon immediately.  She said to get there as soon as possible.  Surgery called us shortly after to get permission to take her back to the operating room over the phone because they were pretty sure she wouldn’t live long enough for us to get there and sign it.  When we got to the hospital the priest was baptizing her and doing her last rites.  We were in a daze.  They rushed her into surgery and left us by saying that they didn’t expect her to live through it so say good-bye. I was absolutely devastated.

Genevieve made it through that surgery alive with only a 10% chance.  Her intestine had twisted and died inside her.  It caused her to get a deadly infection called NEC that basically kills the affected tissue and starts decomposing it while you are still alive.  It is horribly painful.  She was too sick and swollen to put what was left of her intestine back into her body so they put it into a plastic bag called a silo on the outside of her body.  She was tremendously sick but she was still alive against all odds.

Her surgeon came to check on her the next morning and noticed a few areas that didn’t look very good on the intestine that was in the silo.  She scheduled Genevieve for her 4th surgery the next day to investigate those areas.  The next morning she was taken back to surgery.  Her surgeon thought there would be a few inches of not so good tissue that needed to be removed. We were not so lucky.  When the surgeon began to investigate her bowel she found that most of it was dead.  It was so bad that the surgeon herself called us during the surgery and told us that she was in even worse shape that the previous surgery where she only had a 10% chance of surviving it.  Her odds of surviving we so low that she couldn’t give us a percentage for survival.

When she had finished salvaging what she could of Gen’s intestine we all had a meeting.  The news was devastating.  Genevieve didn’t have enough intestine left to survive and what was left was sick.  The surgeon had to sew live tissue to dead tissue because of where the bowel had died.  She was 100% sure that it was going to leak stomach acid into her abdomen so she left her cut open and put a vacuum over the wound to pull out the acid so that she wouldn’t start digesting all of her internal organs. 

Her liver was also failing because she had been on IV nutrition for so long.  IV nutrition is not designed for long term use and causes acute liver failure.  She needed a liver and bowel transplant if she even made it through the night.  She said that we couldn’t even go day by day because it was minute by minute with her.  She was deathly sick.

But Genevieve hung on.  We were counseled to make her comfortable and let her pass because the life of a transplant patient is hard and she didn’t have much of a chance anyway, and because we have 4 other children to care for.  Genevieve refused to give up.  She was not ready to die.  We agonized over that decision and decided that if Gen was willing to fight for her life then so were we.

Within a couple of days Gen was fighting against the ventilator but her doctor didn’t want to take it out incase she needed another surgery.  It was really bothering her though so she grabbed it and pulled it out herself.  Everyone was amazed that she was breathing perfectly on her own.  Three holes opened up in her intestine, but her surgeon expected that and the wound vac was keeping her from getting an infection.  Gen was healing so aggressively that her belly wall actually started to close under the wound vac.  Her body is so incredible!

Anyway, over the last two weeks in Jacksonville, some amazing things happened.  She was able to have 2 drains removed from her, she is breathing on her own, she came completely off antibiotics, the wound vac was replaced by an ostomy bag, and one of the holes in her intestine closed on its own.  All of the doctors were astounded at her progress.  The only problem with all of this was that she was still on the IV nutrition that was causing her liver to fail.  Everyday her liver got worse because of it.

The doctors in Jacksonville did their best to minimize the damage and only gave her the lipids (what causes the damage) 2 times a week.  However, this wasn’t enough calories for her to grow and develop, so it was a balancing act to keep her alive on the medicine that was killing her. 

Thankfully, there is a different kind of medicine for the IV food (called Omegaven) available at Children’s Hospital in Boston, and it has been shown to reverse the liver damage in over 200 children with similar conditions.  After three appeals to the insurance company, they finally approved us for a transfer to Boston for 30 days. On June 11^th, Genevieve and I transferred to Boston.

The costs for transport, long-term hospital care, relocation, medicine and transplant are astronomical! Just the one medicine (Omegaven) is $75.00 a day.  So far we have been paying for minimal expenses in dealing with all of this but even now it is adding up.  We have 4 other children who are out of school and we have to pay for care for them because we have to spend so much time at the hospital.

We were commuting an hour each way to the hospital sometimes several times a day while Gen was in Jacksonville. My husband’s mother passed away on Memorial Day, so we have had to buy plane tickets to Chicago and deal with those expenses.  The list goes on and on.

Still tube free, and staying that way!

Thursday, June 10
Today was our CAIR appointment here in Boston. Faith and I have been here since Tuesday night, and now we are done with appointments for this trip. We had appointments with Dr. Rodriguez (motility specialist who did Faith's endoscopy and antro-duodenal manometry study back in March/April of last year) and Dr. Puder yesterday. They were very impressed with her progress.
Dr. Rodriguez actually remembered us (or pretended very well that he did) and he explained that unfortunately it's pretty common with a gastroschisis kid for them to have motility problems pretty much forever, and for the gut to completely shut down when they get sick for at least the first few years. He anticipates that she will gradually get better about that, and most likely when she is older it will be more like IBS symptoms, rather than complete gut shut-down. He wasn't too happy with her growth though, and expressed concerns that one of the things that contributes to poor intestinal health is poor nutrition/malnutrition... but he pretty much left it up to whatever CAIR wanted to do, just leaving us with a strong suggestion to get Faith onto a nutritional/formula-type drink that she would actually drink.
Dr. Puder pretty much agreed. He didn't have much to say (there's really not much to say with how well she's doing), but was very curious about her development, as usual. She showed off some tricks for him and Danielle and Alexis, and gave Danielle hugs, but recognized that Dr. Puder is, in fact, a doctor, and therefore could not be friendly to him. She has a real issue with anyone who is easily identifiable as a medical professional. Danielle and Alexis wear normal "street" clothes, so I don't think she knows who they are... but anyone wearing a white jacket or scrubs is pretty much the most evil person in the world, as far as Faith is concerned. They ordered labs, just a basic CBC, LFT's, and a vitamin panel. It'll be a couple of weeks until the vitamin labs come back, but I will be interested in those results.
Getting her labs drawn was an adventure, as usual. She has very little peripheral access, and what access she does have is just little tiny veins that blow easily. They had to stick her in each arm, and each stick involved actually sticking her, and then digging the needle all around inside her arm to actually get the vein. Unfortunately, Faith is really observant, and she knows what is coming as soon as the phlebotomist pulls out the little blue rubberband thing, and freaks out accordingly. It took two phlebotomists and both of Faith's arms to get all the blood they needed for the labs, and an assistant and me to hold her still enough for them to actually draw the blood. I would wistfully remember the ease of having a central line to draw blood from, except that to prevent infection we didn't actually draw from it... and life without a central line is infinitely easier!
We (Faith and I) met a new Omegaven family for lunch today before our CAIR appt. They are from NY, and little Bradley has Microvillus Inclusion Disorder, just like Sam and Bo. Bradley is a little cutie, and it was great to meet them! I enjoy being able to talk to new families and help them or answer questions, just like people did for us a year ago when we were in those shoes.
CAIR was a long appointment, as usual... our appointment was at 1:30, but we were there early, since we walked over with Bradley and his family and their appointment was at 1:00. We got taken back to a room right away, and proceeded to sit there for a LONG time waiting for someone to come in. Faith played on the rolling doctor's chair, practiced walking while holding my hands, and made friends with Jacob, another little Omegaven graduate we know from an online support group. She also talked and practiced signs with me, and then finally the nutritionist came in and started quizzing me on Faith's eating and what's gone on since September.
The nutritionist wasn't all too happy with Faith's growth... I explained that she was sick back in March and had lost a pound and was still sort of recovering from that, and that we had stopped g-tube feeds but that Faith was only drinking water. She left to go get us some flavored drink samples for Faith, and Julie (the nurse) came in and asked about how everything else has been. She also wasn't that happy with Faith's growth.
Apparently Faith dropped a few curves on the growth chart since we were last here. The nutritionist pretty much wanted to put Faith back on her tube feeds... I said I didn't want to do that until after we tried having her drink the Pediasure they brought us. She tried it right there in the room, and really seemed to like it- she has almost had an entire bottle already! She only has to drink 1 1/2 bottles of it a day to equal the amount of formula she was getting through her tube overnight, so I think we will easily accomplish that (we'll probably give her 2 bottles a day). I am optimistic that she will grow just fine on this...
We waited a little more, a few other people popped in to say hi and talk about how big she is and how awesome she looks, and then a GI doc I had never met came in with Julie to wrap things up. Julie mentioned that the nutritionist thinks we should consider restarting the tube feeds, but that it would be ok to try the Pediasure first and just watch her weight closely. Faith has her 18 month checkup with our family doctor next week, so we will have her weighed there and report back to Boston.
At the end, Alexis came by, and I was able to tell her what I really thought about it all. I'm not sure if I've ever really clarified that the CAIR appointments are sort of just a technicality, honestly I'm not entirely sure why we even still go to them, other than to get the input of the nutritionist... But, I have trust issues with doctors who *aren't* Dr. Puder (or our family doc), and actually the CAIR doctors have told us stuff before that was contradictory to what Dr. Puder had told us, so I've never actually really followed with the CAIR doctors have told us. From the start, I've sort of smiled and nodded my way through CAIR appointments, listening to whatever they said, but knowing all along that I would just keep doing whatever I was doing, or whatever Dr. Puder and I had already talked about doing.
I think am kind of a "naughty" sick kid parent, because I am not afraid to do whatever I think is best, without clearing it with the doctors first... but you know what, it's worked! I got Faith off all of her meds, I got her off TPN, and now I've gotten her off her tube feeds, without much input from the doctors at all... usually I've just told them what we've done after the fact...
So basically, I explained to Alexis that they had mentioned possibly having to re-start tube feeds, but that I WON'T be doing that, unless it's clear that having her eat and drink 100% isn't going to work. I'm not going to only give her a week to prove herself, either. We have a scale of our own, so I will weigh her once a week if I need to. But I don't really think I need to weigh her every week to know that she's doing ok! So what if she's below the 3rd percentile? So was I as a baby, and so was her big brother.
Anyway, I forgot to bring up her apparent bacterial overgrowth, so we didn't discuss any solutions for that... We briefly talked about it yesterday, but didn't come to a decision... I would like to try probiotics and see if they help take care of it.
We didn't really talk about taking her tube out... the general consensus is that it's safer to leave it in, even if we're not using it, than it is to take it out and have to put a new one in the next time she gets sick and stops tolerating feeds. We will be back in Boston for our next checkup in December, and we will probably discuss taking her tube out a little more seriously at that point.
Now that we are done with appointments, we have a couple of days to just have a little fun... I think we will go out shopping tomorrow (I need some new summer shoes) and then we will be meeting Blaise and Ellie's families for dinner. Not sure what the plan will be for Saturday... and then Sunday is our fundraising walk, and Monday we are headed home!
Hopefully somewhere in there, we will meet another new gastroschisis/short gut family, who should be getting to Boston to put their little girl Genevieve on Omegaven in the next couple of days. This family's story is very familiar to me, as it is really close to what we experienced! Honestly, one of Faith's doctors in Denver actually told me that he was surprised that she never got NEC, he said of all the complications that happened, that would have been the one they would have actually expected... The weird thing about this family is that I actually was told about them by one of my "normal" friends at home, who has nothing to do with gastroschisis or short gut!
I love it that I have been able to use our experiences (which seem SO negative) to help other families going through the same type of difficulties! I really LOVE the bible verse from 2 Corinthians at the top of the blog, and the blessing of helping these families makes me feel like we are living out God's will, and helps me to realize the big reason for it all!

Awesome news!

Wednesday, June 2
We're not using Faith's g-tube anymore! We stopped about a week ago, with the exception of one night, before we headed to a Rockies game and didn't want her to get dehydrated from being outside all day. But she is doing awesome, drinking at least 20 oz every day, and eating like a pro!
She amazes people with how fast she can wolf down a banana, and she LOVES meat! She can polish off half a cornish hen, or an entire chicken breast, all by herself! She regularly eats at least as much (if not more) than her big brother at meals. Not such a big fan of veggies though... we might have to try giving her some green smoothies and seeing if she'll drink them...
So, not using her tube means she's no longer getting *any* formula. And while the strong anti-formula side of me wants to do a happy dance, she's also almost exclusively drinking water... she's lactose intolerant, so we have to give her Lactaid milk, but she really doesn't seem to be a big fan of it... So the worrier side of me wonders what the nutritional implications are for a toddler who doesn't drink anything but water, and won't eat her veggies... I think we will try a flavored formula when we are in Boston next week, to see if she will drink it, and if she will, we might switch her from her baby g-tube formula to big girl flavored, drinkable formula! (not that her Neocate isn't drinkable, but if you smelled it, you'd understand why she won't drink it!)
I'm looking forward to our week in Boston, to seeing our Boston friends again, and to walking in the NStar Walk for CHB! Please consider donating to our team, the work they do at Children's Hospital Boston, and the support they give to families there is absolutely amazing!