Yesterday Shelby had her upper endoscopy ("scope") at Rockford Memorial Hospital. She was wonderful! After talking things over with the anesthesiologist, we decided to not tempt the Versed (pre-med that really, really relaxes her that she had trouble waking up from when she had her tooth pulled) for such a short procedure and that I would go in with her while she fell asleep. She was scared, but did awesome. What a brave little girl she is! I helped hold her arms and legs still while the "gas" took effect. Its amazing how much you can change as a parent in 3 1/2 years of medical mayhem. I used to leave the procedure room or OR crying, haunted by the image of her struggling and fighting being put to sleep. Now I understand it as something totally normal that people do as they go under. Once she was asleep, I kissed her on the cheek, reminded Dr. Deutsch to have a nice, steady hand, and was on my way. Even though I can do that without much emotional outburst, I still don't like to be away from her long, so I scooted off to the cafeteria for a pop and bag of chips while Scott went to make some work phone calls and get a big 'ole cuppa Joe. Once back waiting for her I couldn't just sit, either. I have to pace. And look at the fish. Very relaxing. Fifteen minutes after they took her in Dr. Deutsch came out to give us the results.
Previously she had 1 small varix that stayed the same for about a 2 1/2 years. Now she has 4 varices, a red stomach, and a hemorrhoid. Varices are graded on a scale of 1 - 4 with 4 being the worst. Thank God Shelby has only one at a grade 2, one graded at a 1, and the other two aren't even big enough to get graded, but are there none-the-less. Her stomach is red due to the increased blood flow around it because the blood can't flow through her liver like it should. The hemorrhoid is, well, really, a varice. Hemorroids are veins that are taking on too much blood flow just like a varice. But is it just a hemorrhoid or something caused by her liver disease? Who knows! Its something to keep an eye on, too.
She woke up from the anesthesia crabby and upset because her I.V. was still in. It didn't take long for Shelby to get her way. She was thrashing around so much thus the I.V. started bleeding and she got it out. Trust me, I think she knew what she was doing. Smart little girl. Once that was out, she settled down, and was on to drinking and eating. We were able to leave the hospital 1 hour and 15 minutes after her procedure with her walking on her own. She didn't look like someone who just had general anesthesia. Off to McDonalds we went as promised.
I think the hardest part for her was not eating or drinking from the time she woke up until after the test. I used the promise of my special dark chocolate as an incentive to put on the medical bracelets at the hospital and, sure, enough, the little bugger remembered it as soon as she started to come to. She also remembered that she wanted pop and got a little irritated when the nurse mentioned a popsicle, but didn't return with one. Trust me, the nurse figured out why she was mad real quick! The popsicle arrived moments later.
While waiting to go in for her test, Shelby had great fun running through the halls while I counted to see how fast she could go. Mind you, this is a 3 1/2 year old in nothing but hospital slipper-socks, a pull-up, and a pj shirt. Very cute! Scott and I are pretty lax on what we let her get away with to entertain herself when doctors are involved. Race around the halls, great! Just don't run anybody over. Draw all over yourself with a pen, sure! Have fun! You know, stickers and the usual hospital tricks don't always work that well for "frequent flyers." RMH did update its pediatric outpatient area since we were last there and had an awesome fish tank and play area. Shelby did enjoy that a bit.
So many of you are probably asking "Now knowing about these varices, does it change anything for Shelby?" O.k. if you weren't asking it, we were. No, nothing changes. Now finding out her labs results today (which I'm trying to be patient about) could change things if they're still rotten like the ones on the 13th. Knowing about the varices just makes us worry more. Having a GI bleed is Shelby's biggest risk right now. A GI bleed would mean her vomiting blood or it coming out the other end. This would mean calling the ambulance and, most likely, spending some time in the hospital. She could need a blood transfusion or several. Sometimes bleeds aren't bad, but they are referred to as "life-threatening." Not good. Now will Shelby ever have a bleed? She might not. She could have horrible, grade 4 varices that would never bleed or she could bleed alot. There's nothing we can do with her that would change that fact. The doctor's at Children's Memorial take the route if it ain't broke, don't fix it - meaning, they don't try to treat the varices unless they're bleeding because the risk of doing it preventively can be worse that just waiting and seeing what happens.
Scott and I were actually expecting to hear this news with her last scope in November of 2004. We're not surprised that she has 4 varices because we know that her little body can only take so much of a crappy liver before it takes a toll on her. Those of you that know Shelby in person know that you can't tell from the outside (except for her large abdomen) that there is anything wrong with her, but her insides tell a much different story.
Continue to pray for her please and all the other children in the world that suffer due to disease, hunger, abuse, etc.
I''ll leave you with a funny conversation we had at the hospital.
Me: Shelby, these are the pictures Dr. Deutsch took of your insides. What do you think?Shelby: (Thoughtful pause) Ick!
Gotta love her! I'll update again when I hear on her lab results. Here's some pics of the girls from Christmas. (Click on any of the images to see larger.)
Just wanted to take a quick moment and wish everyone a very Blessed & Merry Christmas! Scott is doing great recovering from his shoulder surgery. He's o.k. spacing out his pain meds to about every 6 hours or so until he moves the wrong way. January 3rd he begins physical therapy.
Riley is doing great, too. Thursday winter break began and she is having fun playing some new vtech smile games. We let them "open" one of their Christmas presents today - goldfish! One is orange and the other is white. Their names are Annie and Sandy. I would show you a picture, but our camera died this morning. Actually, its a known problem with the camera we have and they'll fix it for free. I just have to be without a camera for 2-3 weeks. Just in time for Christmas...
Shelby is doing o.k., too. She seems to be getting over her cold slowly, but surely. This coming Wednesday at RMH she'll be having an upper endoscopy (scope) to check for varices in her esophagus. Please say prayers for her. She was so excited when we left clinic on the 13th, exclamining "no more shots, no more x-rays, no more needles for a llllooonnnnggg time!" We'll, baby, I hate to tell you, but there will be needles this week. Its a fairly quick procedure, but the results could be the beginning of the long road to transplant OR she could continue her smooth sailing. Her repeat labs will be drawn while she's under the anesthesia, so at least she won't feel the needle. She'll probably wonder why the band-aid is there, though.
Please also continue to pray for our little friend Annika and her family.
I'm not going to gross anyone out with gory pics so don't worry. In fact we don't really even know what's under the gauze other than 4 little incisions. Scott's surgery went well. He was in for about 2 hours and the doctor fixed a little bit of everything. He is doing well right now with his polar care ice pad, 2 Norco pills, and a pain pump. What is polar care you ask? See the blue stuff and the cooler? Ice is in the cooler 
and is then pumped through the tubing to the blue pad wrapped around his shoulder. It keeps that shoulder nice and cold. The black stuff is the sling that his arm will have to stay in for quite some time. He is tired and sore right now, but hopefully we'll be able to keep on top of his meds to keep the pain under control. Here's crossing our fingers for a restful night!Please, please continue prayers for Annika. She went back into the OR today. Please read her mom, Moreena's, updates for more details.
Well, Shelby had clinic on Tuesday in Chicago. As I stated before, its the first time she's been to liver clinic there in 6 months. They were pretty amazed at how well she is still growing, how strong she is, and how active she is. Her liver and spleen continue to grow (that's not good), but we were told that 80% of her liver can be non-functioning and she can still compensate like she is. With so many spider veins on her face, they do agree with us that another upper endoscopy (scope) is in order to check for varices. I'll be scheduling that locally with Dr. Deutsch hopefully for January.
I did hear back yesterday, though, from the nurse that her "liver" numbers do not look as good as they have. Her bilirubin is up (the stuff that makes her look yellow) and her platelets are low at 75,000. Normal for a child her age is 150,000 - 400,000. Since she has a large spleen that "gobbles up" platelets, she is allowed by her transplant team to be o.k. as long as her platelets are above 55,000. She was at 134,000 in September. See the concern? Anyway, we are to watch her as she has a cold already and things like that can throw off a liver kid's numbers. If she doesn't improve or the cold goes away, but she isn't acting normal then we are to have her labs retested. Otherwise, they want her lab work redone in the first few weeks of January.
There is concern, too, to watch her for hepatopulmonary syndrome with her portal hypertension. I can't find a good link to a definition of hepatopulmonary syndrome that isn't full of medical mumbo-jumbo so here's my definition. When blood can't flow through a diseased liver like Shelby's, it can also effect the rest of her system. Hepatopulmonary syndrome would be when her lungs are affected because of the "block-up" in her system. If she had this, she would be re-listed for transplant as "curing" the diseased liver is the only way to get rid of the hepatopulmonary syndrome. She was tested back in March for pulmonary hypertension (sort-of similar to hepatopulmonary syndrome) with a saline bubble echo. That test came out o.k., but they still want to watch her closely for pulmonary problems because they can have no obvious symptoms.
Basically, once you start getting secondary diseases/syndromes that can't be managed with medication (like her osteoporosis) or medical procedures you need to take care of the primary problem, the diseased liver. Her team is watching closely for those types of issues with Shelby because she is not following the "textbook" path of progression of biliary atresia. Her Hepatologist said that she's never had a patient like Shelby. That's great as for the past 3 1/2 years she has exceeded all their expections, but it also makes it hard to know what's on the horizon for the progression of her disease. Right now, her biggest risk is a GI bleed from possible varices.
Today Scott is having shoulder surgery at noon at St. Anthony's. Please hold him in your prayers as he is having a bone spur removed, 2 cysts removed, and a possible tear fixed. It is to be outpatient, but he will be in surgery for 2-3 hours. On his FMLA paperwork his doctor listed him as totally incapacitated for 6 weeks! Yikes! He is nervous about the pain that will come with recovery, so please pray for him to be at peace with that.
Thank you once again for checking on our family and holding us in your thoughts and prayers.
