A Little Bit of Normal is What We Needed

We are good! Many have been asking about how Macy is doing and how she is feeling.  She is good!  She says she feels like herself.  She is getting her color back some, but still looks pale to me.  However so does McKenna, lol.  It is winter in Indiana!  It can take a while for her hemoglobin to return to normal after her blood loss.  Her GI doctor said even though she had a blood transfusion, she will lack iron following all of this.  They placed her on a therapeutic dose until we return for the scope.  I had to reduce it some because it was causing pain in her abdomen.  She was getting 2 pills in the morning, followed by pain and bloating, and then 1 pill at night.  I reduced her to 1 pill morning and 1 at night, and the pains have gone away.  Her scope is scheduled for January 10th.  It will be early in the morning.  If she needs banding done, she will stay over night.  If nothing needs to be done, she will go home later that day. (Surgery #29) 

We've loved a little bit of normalcy lately.  We had Christmas with Mamaw and Papaw, and Christmas with the great-grandparents, aunts, uncles, and cousins. We went on a Christmas light scavenger hunt, and sat around in our pajamas watching movies and playing with new games and crafts.  It's help to take our minds off of things.  I wish it helped control my random crying when I talk about her, or the worry of a repeat.  I'm working on that. 

I've had a few people ask questions such as "what are the symptoms to watch for", "was she vomiting streaks of blood", "does she have cirrhosis of her liver", "what will the surgery in March do for her", and "why didn't they do the surgery from the beginning"????????  Great questions.....so here goes.......

There are no symptoms at all.  The only way you would ever know that this was a possibility of ever happening was to have a GI scope and take a look at her esophagus.  And no, she has never had a scope.  Even with all that she has going on, including Celiac Disease.  We skipped the scope and biopsy usually done for Celiac patients because we didn't want to put her through another surgery.  It was as decision made together with our GI doctor.  The only way to know you have a rupture is when it happens.  No, she was not vomiting streaks of blood, it was pure blood.  It was like something out of a horrible, horrible nightmare.  Pure blood.  If you Google "esophageal varices" you will most likely find info on people with cirrhosis of the liver (damage done to the liver, inability to function normally).  Macy has these due to portal hypertension (high pressure in her veins).  She was purposefully put in hypertension to force the veins to grow around her liver and get blood flow to her liver.  In March, she will have a vein taken from another part of her body and placed as her portal vein.  The portal vein is the main vein that carries blood to your liver.  Originally hers was the size of a thread, it is now larger, but will never be large enough.  Dr.  Superina couldn't do this during the first liver surgery because her liver was undersized and so were the veins in it.  If he were to reroute the blood to it, it would've been completely overwhelmed.  Therefore he slowly rerouted blood to the liver, giving it time to grow.  The liver is currently normal sized, and the veins in the liver are good sized as well.  Obviously the negative to this first procedure is that the portal vein still hasn't grown to full size (an most likely never will), and it has put her in portal hypertension for a long time.  Long enough to cause other problems such as these varices.  After the surgery in March, her liver should be functioning normally, with no more portal hypertension and no more risk for esophageal varices.   

So what can you pray for?  Pray for Macy to continue to stay healthy, with no complications until our scope on January 10th.  Pray for Macy to have a successful scope on January 10th.  Pray for health and safety until mid-March for her next livery surgery.  Pray for a successful liver surgery in mid-March with a normally functioning following.  Please continue to pray!  Thanks for continuing to support and follow our journey.  I don't respond to all of the love and prayers sent out way, but I read each and every one of them.  Thank you!!!!

We Are Home!

Home was calling Macy's name, and she is so happy to be home!  We are praying that Macy continues to recover at home, and we can all start to heal from this, physically and emotionally.  The emotional healing is going to take a lot longer for all of us than we thought.  We are understandably still on edge, and we are all worried about a recurrence of the bleeding.  We are really trying to stay close to home, and have a plan put into place if need be. 

She was discharged with a few new medications, putting her up to 10 meds in the a.m. and 6 meds in the p.m.  Our current plan for follow-up has changed a little from my previous post.  Our plan is to return in 4 weeks for another scope.  If everything looks good she will be outpatient and we will go home that day.  If any banding is needed, she will spend the night and be discharged the next day.  We are not sure about the plan following that, but she might possibly have another scope between then and March.  In March she will have the big liver surgery, the Rex Shunt. 

Please continue to send your prayers this way.  Please pray for Macy's physical and emotional healing.  Please pray that the banding surgery done on Friday keeps her esophageal varices under control, and she is safe.  Please pray for McKenna.  We try our best to always make her feel our focus and commitment to her as we also try to focus on the health of Macy, but in all honesty I know we have flaws in our methods. Please pray for Jeremy.  He is back at work, but understandably worried while he is away.  Please pray for me.  As most mothers do, I don't feel worthy of anyone's focus when my children and family require it to.  I want their prayers to be first, however I really need them this time.  I am not handling this well at all.  I am told all the time that I am such a good mother, so strong, and just like Wonder Woman.  I try to live up to those comments and typically do a good job at just moving on and keeping a focus on our goals. I'm not able to do that yet.  I am breaking down and losing it when I talk to my mom on the phone, or simply talking with Jeremy at home.  I have so many fears, so many questions, and so much that I keep praying about.  I am not sure what exactly I need right now, but I know for sure that I need prayers. 

Another Bump...Maybe A Boulder!

Today has been the hardest and scariest day I have ever had.....maybe comparable to the day Macy was born.  The day my baby was born, I didn't know if she would make it.  I didn't today either.  I start crying just thinking about what we all went through this morning.

Macy woke up as usual at 5:30 a.m., turned her alarm off, and rolled back over in typical teenager style.  I walked in her room to wake her up, she hopped out of bed, and I laid down on her bed.  That's how we roll.  I'm just there to make sure she gets up and stays up, and then we head downstairs. Nothing seemed out of the ordinary as she headed to the bathroom, however I did hear her do a little cough/throat clearing.  Apparently when she got into the restroom she felt extremely nauseous.   The nausea caused her to vomit immediately, and it was all blood.  She yelled from the bathroom for help.  I am not exactly sure if I can clearly recall the events that took place after that, but I remember her vomiting over and over blood for about 5 minutes.  McKenna was woken up and told to get dressed.  Jeremy and I dressed at one point, and we were out the door on our way to Riley. Macy had stopped vomiting at that point. 

While in route I paged our transplant surgery team at Lurie in Chicago and the GI on call at Riley.  I spoke to the GI on call in the car, and she was heading down to the ER to meet us when we arrived.  Macy started vomiting again at one point during our car trip, and again was all blood.  A very large amount.  McKenna was sitting in the back with her and clearly very scared. 

When we arrived at Riley they were amazing!  They got her into a room, and within what seemed like minutes had IV access and labs drawn.  Soon after that, another access line in order to give her a blood transfusion.  Her hemoglobin was clearly dropping from all of the blood loss, but surprisingly she was holding her own with her stats.  As they did all of that, the GI surgeon was on his way in, and they were clearing an OR.

At this point, they knew what was going on.  It was esophageal varices.  Essentially what that means is, she had extremely dilated veins in her esophagus, and they burst and bled.  This can be very common in Abernethy patients that are in portal hypertension, yet extremely life threatening.  So due to the liver problem that Macy is currently being treated for, these bleeds can occur.  Once they got into the OR, they were able to locate and stop the bleeding.  They did a banding procedure on these varices and it greatly reduced the dilated veins.

At this point, she needs to be watched closely following the surgery, so we are here through the weekend.  Eating and drinking needs to be monitored closely as well due to the surgery being in the esophagus.  At first she was told no food or fluid orally at all today.  Then they switched her to clear liquids.  She was so happy to finally have something to "eat".  She had broth, jello, juice, and a popsicle.  She will be able to move onto soft foods hopefully tomorrow, and will do that for a few days. 

Dr. Superina's office in Chicago has been in direct contact with our GI team here.  The current plan is to return in 6 weeks for another scope and possible banding surgery if needed (same thing we did today).  We are no longer "waiting to see what happens" with Macy in regards to her next liver surgery.  It will now be in March, or sooner if need be for some reason.  Once we have the Rex Shunt in March.  Her liver will essentially be fixed.  No more portal hypertension and no more chances of having any esophageal varices. 

Macy is doing good.  I'd love to say she is in high spirits, but she hates the hospital life.  She gets  really grumpy while we are here, and she just wants to get home.  She's also very rattled from what happened today.  She admitted to me that she feared for her life.  It just breaks my heart to hear her tell me that.  No child should ever have to face all that she has faced. 

McKenna came to visit tonight.  She was in good spirits.  I worried about her all day.  It was such a scary event this morning, and I am sure she didn't know exactly what was happening, but that it was bad.  I asked the counselor at school to check on her and see if she was okay.  She was doing fine when she checked on her, so that calmed my mind a little. 

Jeremy and I had a very emotional day.  I'd love to say that we held each other while we cried, but for some reason we had a very hard time looking at each other today. Every single time we did, we cried.  I had to leave the ER room a few times because I just couldn't control it.  I am normally very good at holding it together, but I just couldn't today.  We both lost it when we told her goodbye as they took her to the OR.  I am so big on not frightening her, but then again she needs to understand that it's okay to cry when we are scared.  As we hugged her and kissed her with tears in our eyes, she cried too.  It was her first and only cry today.  She is constantly showing her toughness and her ability to overcome, but it was nice to see her let it out for a moment.

I will never get that vision out of my head of her vomiting this morning.  I don't want to let her out of my sight.  It took me 10 years to stop checking on her in the middle of the night as she slept, and now I feel like I want to sleep in her bed.  I kept saying this morning, "what if that would've happened while she was at school", "what if that would've happened if she were home alone or babysitting McKenna"????  It scares me to death, and I am sure it does her too.  I worry about what this event may do to her long term.  Her anxiety has been elevated following surgery.  We asked for Child Life to come chat with her twice today, once before surgery and once after.  I think it helped a little bit.

Please continue to pray for all of us, especially Macy!  We don't ask for help, and we don't take it when offered.  I know it may sound crazy, and I tell myself we should take people up in their offers.  However,  we are a very close family unit that leans on each other a lot.  It was really hard for Jeremy and McKenna to leave tonight, it's hard to have our family sleep in two different places, but we know it's necessary.  So the time we have together, even if it is just to stare at the walls in the hospital, means a lot to us.  McKenna worries just as much about Macy as Jeremy and I do, and she is actually happy and excited to come to the hospital. So prayers is all we ask for at this time.  Just pray!

Uncharted Territory

First of all, thank you all for the many prayers and well wishes we received for our family and mostly Macy.  It means the world to have so much support!  Thank you Mamaw for staying with McKenna and loving her while we couldn't be there! 

It was a whirlwind of a trip to and from Chicago. Are you wondering why we travel to Chicago when we have Riley and Peyton Manning Children's Hospital right here in Indy?  The liver condition that Macy has is extremely rare.  When I say rare, I mean like 35 diagnosed cases in the world when she was diagnosed in 2008.  A case study from 2014 that I read indicated that since the first discovery in 1793, only 80 cases have been described.  Which I am sure many cases early on went undiagnosed, and some still do.  Nevertheless, extremely rare.  We found another girl who had this same condition, and her mother told us about Dr. Superina in Chicago who claimed he could fix this.  Everyone else told us that she needed a transplant.  So off to Chicago we went, and that's why we keep going back. 

Our morning started off a little rough with the nurses taking labs, and then coming back for more because they missed a couple.  They told us that they didn't need a urine sample, so Macy went to the bathroom, and then they said she did.  It was all kind of a mess, which if you know me well you know I am not about disorganization.  I didn't let it consume my worries though, because my focus was on Macy.  All my worries and prayers were for her.  This was surgery #27 for our girl, and it never gets easier.  Never! 

The surgery was supposed to last about 1-2 hours.  I calmly prayed and read my Kindle for the first hour and half.  By the 2 hour mark I was standing and staring toward the door.  By 2 hours and 30 minutes, I had enough and asked the receptionist for some news.  Fully expecting her to say that Macy was in recovery and they would be coming to get us soon.  "She's still in the operating room."  Ummmmm, what?!?!?!  She said someone should be giving me an update.  They called the front desk to speak to me.  I have horrible flashbacks about getting phone calls in the waiting room from Macy's first surgery, the surgery that my baby came out of with a tracheotomy.  Anyway, they informed me that she went to sleep peacefully, and they had finished the biopsy, and venogram through her neck area.  They needed better pictures and were currently going in through her abdomen.  She was doing very well though.  I sat for 5 minutes, and then was back up pacing.  The surgery took 4 hours.  Double what it was supposed to be!  Oh my nerves!

Macy had a lot of pain following surgery and said she felt like she was having trouble breathing.  It continued in the recovery room, and then also when they moved us into extended recovery.  Her numbers were good, her hemoglobin was good, and she sounded great.  The doctor informed us that when they went through her abdomen, some blood leaked around her spleen, and was probably irritating her diaphragm.  It can cause pain from her spleen area all the way into her left shoulder.  It was positional as well, and she was unable to lay on her side.  She was eating well, and wanted to start going for walks down the hallway.  Once we started going for walks and having her sit in a chair instead of the bed, her pain decreased some.  However in the evening and overnight she had a few times of sharp pain in her abdomen and chest, which seemed like muscular spasms.  It happened quickly, scared the crap out of her, and then would go away quickly.  She said the only areas hurting her today are the two incision sites, the other pain has gone away.  We made it home this afternoon, and we are so excited for our own beds.  However she is afraid to sleep in her room alone.  She's afraid she'll have those pains overnight and no one will be with her to help her.  So I'm going to stay with her tonight again.  Poor girl!

We saw our surgeon today.  They called us and asked us to come early because he was heading to a transplant after he saw us.  I honestly don't know how to categorize the news we got, and I haven't quite figured out my feelings about it all yet.  Her images were good, and things looked pretty good from the imaging.  She had good pressures in her portal vein, good veins and flow inside her liver, and her labs were pretty good.  The concerns were that she still doesn't have a large portal vein that took over, and her body created another shunt (area that carries blood in the wrong direction).  She has a shunt that is running back toward her spleen and toward her heart.  So her pressures look good because not all of the blood that should be going to her liver is going to her liver, it's escaping to other areas.  This backup of blood is causing her spleen to enlarge (which it has been larger for a while), and her platelet count to continually decrease.  It is the lowest it's ever been at 66.  The consensus is that she needs another surgery, but the question is when.  In our surgeon's words, "we are in uncharted territory".  He's done this second surgery a few times for Abernethy patients, but all patients react differently, and the timing of when it needs to occur is different for each patient.  I worry about when the right time is....I hate pushing things to the limit.  I don't like to watch things slowly get worse instead of better.  However, I don't want to push her into surgery.  Miracles happen, and she herself is an amazing miracle.  Macy knows how to surprise us, so let's pray for a miracle!

We will be followed with labs, and will return to Chicago in June for a CT scan for another check on her liver.  Until then we are watching her closely, especially with the low platelet count.  We are watching for any abnormal bleeding and her energy levels.  Prayers are appreciated as we head into another round of the unknown.  Thanks again for always checking in on us!