Shelby's clinic results

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.