Once upon a time there lived a perfectly normal family until one day…..

In January of 2001, Haley had a sore throat and a fever. At the doctor’s that day, her lab work showed liver abnormalities, and her spleen was enlarged. She was admitted to the PICU and very quickly worsened. Our doctor was using scary words like “unstable” and “critical”. She was diagnosed with pneumonia and developed respiratory failure. At that point our local hospital was past its comfort level with this critically ill 7 year old child, and we were transferred to Duke via air ambulance. We left around midnight on a Thursday, and that trip remains one of the most surreal experiences of our journey. Here I was on a bitter cold, yet beautifully clear January night first flying, then riding in a screaming ambulance through dark, deserted streets, with some man I had never met before keeping my daughter alive by forcing air into her lungs with an ambu bag. I remember thinking, through my panic and fear, that I would never be the same person again. Some experiences are so profound; you know in an instant they have changed you forever.

Duke: The place we learned about PICU’s, vents, and hospital chapels…..

Once at Duke, the doctors were quite sure we were dealing with a malignancy…it was the only thing that explained her profound bone marrow suppression, her deficient immune system, and her enlarged liver and spleen. A bone marrow biopsy was done and a diagnosis of Acute Myelogenic Leukemia (AML) was made for 48 hours…then the flow cytology of the bone marrow aspirate showed no evidence of cancer. Lots of prayers of Thanksgiving that day! Haley was extubated after 6 days and steadily improved. She was given IV steroids for her lungs and they probably masked her liver problems…but not for long.

“She won’t live her whole life with that liver….”

We were sent home and everything seemed fine…except it wasn’t, stubborn liver numbers that wouldn’t go down, nagging fatigue and just a parent’s sense that things were still not right. Every week those liver numbers were a few points higher until suddenly she was in sub-fulimant liver failure with an AST of 2000, ALT of 1950, Bili of 13, and a PT of 20. She was immediately admitted to our local hospital and given Fresh Frozen Plasma to correct her clotting factors, so a liver biopsy could be done. After biopsy, we finally had our diagnosis…Autoimmune Hepatitis (AIH). She responded well to the steroids, but developed gallbladder problems, and was transferred to Cincinnati Children’s Hospital where they felt she had an “overlap syndrome” of Primary Sclerosing Cholangitis (PSC). Serial liver biopsies were done, and the hepatologist there said her liver “crumbled” as he extracted a piece with a needle. He said she could not “live her whole life with this liver.” He said he expected her to need a transplant by age 12 or 13. We were devastated; we couldn’t believe what he was saying. This sort of thing happened to other people!

Atlanta: Our new transplant team…and maybe our new home…..

We decided to explore our transplant options and ended up in Atlanta…mostly because of their living related donor program. Her liver has responded fairly well to treatment and her Hepatologist says she will not need a transplant because of her current state of liver disease, but because of the damage that was done to her liver while this disease simmered and we had no idea she was sick. She has cirrhosis of the liver, and that damage is irreversible. She also has “shunting” through her spleen as her body tries to compensate.

Lightning strikes twice….

2002 was fairly uneventful except for some pretty significant joint pain. We kept charts of her pain and she often required strong narcotics to control it. She had to stop all extracurricular activities and was unable to attend school. We even tried IV Remicade treatments, and then all of a sudden the pain just stopped. We still have no idea why.

In January of 2003, she started running fevers. She ran a fever of 104-105 every day for 30 days. During these 30 days, we spoke to or saw her doctors every day. In fact, our local doctor got to where she would call me when she got into the office to see how Haley had been the previous 24 hours since we had talked. We went to Atlanta twice that month. She was initially diagnosed with CMV and had I PICC placed and was started on Ganciclovir, but instead of getting better, she worsened and we were sent to Atlanta for admission and workup of” fever of unknown origin”. Once again, they scared us to death with the cancer talk, this time they were worried about lymphoma. This time the bone marrow biopsy came back perfectly normal right away and we all breathed a sigh of relief. She was a bit of a mystery to the doctors with her fevers, swollen lymph nodes, and odd rashes. As we were discharged, a tentative diagnosis of Systemic Lupus Erythematous (SLE) was made. But lupus could be very mild and was only worrisome if it affected the heart, lungs, or kidneys. So we thought we were “safe” from another horrible diagnosis.

SLE: Not just rashes and joint pain……

Haley never rebounded from that hospitalization and she developed a strange new symptom – her eyelids swelled. Our local doctor ordered a CT scan to check out those swollen eyes and they discovered fluid around her heart and both lungs. Soon after she began sleeping 14-18 hours a day and coughing at night. That earned us another Atlanta admission. She was admitted on May 7th, and we stayed for 6 long, horrible weeks. She had 3 cardiac drains placed, 3 pulses of IV Solumedrol at 900 mg a pop, a bout of Shingles that caused her bladder to shut down, a dose of the chemo drug Cytoxan, and her liver couldn’t take the stress of all of that so it shut down too and she had an episode of hepatic encephalopathy. All in all, she spent 14 days in PICU, 4 of those on a respirator. She was discharged on 6/18 on 19 oral meds. We are down to 16 now.

As for right now, we trust in God and take things one day at a time……And today is a good day.