Chapter 6 - The Long, Bumpy Road

Monday morning, Dec. 16th, 2002, was the day we met everyone, and got the first "glimpse" of what we were in for in the quest to figure out exactly what was causing Cyann to be so sick. Since there was a variety of symptoms, and nothing that really seemed to link everything in a nice, neat 'textbook' explanation, they called in a doctor/specialist for just about everything! There was an infectious disease specialist (for the rash, which was no longer there by Monday morning; once Cyann was admitted, they started her on a cycle of 3 different kinds of heavy-duty antibiotics to see what would work best. Turned out that Cipro, the same stuff used to treat Anthrax, was the key.); a dermatalogist (to determine if the 3 hemangiomas/"strawberry marks" she was born with were really benign or symptomatic of something serious); a nutrionist (to unravel the milk protein allergy); a genetics specialist; a gastroenterologist (Dr. Silber), and of course, a nephrologist, Dr. Haws. They even called in an optometrist to check out her eyes! Supposedly, certain metabolic diseases they were testing for could show symptoms in the eyes, like beginning cataracts or something. (He said that Cyann had beautiful, big, healthy eyes.)

At this point in time, there is no one test that is specifically to determine whether or not someone has ARPKD. Apparently, the doctors just work with the known symptoms, crossing off the possibilites of any other things it could be. I mean, there is no test to be taken that in the end you will hear, 'yes, you have tested positive for ARPKD.' The tell-tale signs of this disease are, obviously, cysts in the kidneys, for starters. But also, the enlarged kidneys (due to the number/size of the cysts inside), enlarged liver, and enlarged spleen. Which, as it turns out, was the "hard spot" on Cyann's left side that we kept feeling and the pediatrician said it was due to constipation! (We changed pediatricians/medical groups as soon as Cyann got out of the hospital!) But the 'clincher' symptom to go along with these is high blood pressure. And Cyann did not have high blood pressure when she came into the hospital. Only after they started filling her with fluids and going through all of the tests, did she start having hbp, and then it stuck around.

So at the start, she did not show 'classic symptoms' of this kidney disease. Therefore, they needed to rule out anything and everything else that could possibly cause all of the stuff that was going on inside her poor little body. They did urine and stool samples, another ultrasound, a bunch of blood tests, a CT scan, an MRI, and genetics/metabolics tests that had to be sent across the country for the results. That first night in the hospital when I was so scared of her having ARPKD, and now it was turning out that that was the best option to hope for. All of these other tests were to rule out much more serious things, such as a couple different types of cancer, or syndromes that I'd never even heard of, but which I learned were all terminable. I couldn't believe we were going through this.

On Tuesday, we got the results back from the blood test they took in the E/R. It came back positive, which at first was what I was hoping for, thinking it would mean that she didn't have the kidney disease. Of course, that was wrong information on my part, and in fact, I learned something that makes me cry to this day thinking about it. Usually, a person has a white blood cell count (WBC) in the 8,000-10.000 range or so. Then around 13,000, you are considered to have some kind of infection going on. Cyann's WBC was in the 40,000 range! The part that makes me cry still...remembering hearing that if we would have waited another 12 hours or so to bring her in, they wouldn't have been able to save Cyann. And remembering how we very nearly did almost not bring her in because we thought it was some kind of allergic reaction, and she had a doctor's appointment for the next day. It gives me chills to think about it now as I write this.

By the end of the week, it was looking more and more like the pre-diagnosis was correct. And I was learning to come to grips with that. (Sort of.) The final diagnosis would come after a liver/kidney biopsy, but that would have to wait until Cyann was completely free of infection. The biopsy was scheduled for March. In the meantime, all that was left was to wait for the reults of the genetics/metabolics tests that were sent across the country to John Hopkins. The idea was, that if any of those tests came back positive, then there would be no need for the biopsy to be done, because we would then already know what was going on. If all of those tests were negative, then the biopsy would go as scheduled.

As for everything else, Cyann seemed like a completely different baby. She was feeling a lot better, and happier. They told us we could go home as soon as we made arrangements for a blood pressure machine to be available for us to use at home to monitor Cyann's blood pressure ourselves on a daily basis. The problem was, those machines are expensive! But Scott and my dad got on Ebay and found one in Missouri. My dad, a retired OTR truck driver, was ready to jump in the car and go after it that afternoon, if it meant we could get Cyann home sooner. But then Dr. Haws told us about someone in Lake Havasu, much closer to home, that we could arrange to go pick up. The next problem was that we needed cables for the machine. Those had to be ordered and FedEx'd to us before we could leave the hospital. So although we were ready and able to take Cyann home on Fri. Dec. 19th, we couldn't leave until the cables arrived, on Tues., Dec. 24th, just in time for Christmas Eve.

The next obstacle was giving Cyann her Cipro at home. Originally, they wanted to put a 'pic' line so we would be able to give it to her at home through her vein. But, after 2 different techs and 5 excruciating tries, there was just no way to find a good strong vein left to get that needle into. As it was, the last place she had an IV in before going home was to have it in her head! But Dr. Haws came to the rescue once again, saying she would probably be alright with just the liquid form. And at 3pm on Christmas Eve, we brought Cyann back home. She was in such a good mood earlier that day, but as soon as we came into the house, she looked around and burst out crying!