Diagnosis, treatments and milestones

As I talk with more friends many are asking me if we ever figured out exactly what Jila has, and I realize my posts here and on FB are so spotty I might not have been clear about that. In a nutshell they are treating it as Hepatitis-Associated Aplastic Anemia (HAAA), which essentially means that it’s aplastic anemia (i.e. bone marrow failure) which was preceded by unexplained hepatitis (i.e. liver inflammation). In most cases of HAAA the hepatitis resolves with or without treatment (as it did with Jila, with treatment) but then develops into aplastic anemia 2-3 months later. This is pretty much exactly what happened to Jila.

They don’t know what caused it. Most cases of aplastic anemia are idiopathic, meaning the cause is unknown. We have no family history of anything like this and she had zero health issues up until last summer.

It started as hepatitis in June 2012 but negative for all known hepatitis viruses, and negative for markers of autoimmune hepatitis (AIH). Even though she tested negative for AIH, it acted like an autoimmune issue (i.e. the body seemed to be attacking itself), so when it didn’t resolve on its own after 6 months, they treated her with the standard therapy of prednisone (steroids) starting in December 2012. She responded well, with a few ups and downs, and her liver inflammation was essentially gone by March 2013.

But her blood counts had also been kind of odd from the beginning, and when her second spike of hepatitis peaked in November 2012, her platelets started dropping a lot as well. As soon as they started the steroids in December 2012, her blood counts went up fast. In fact her blood counts rebounded quite quickly and strongly to the steroids, while it took a bit longer to start seeing meaningful results with her liver enzyme levels. But they responded too, just a little bit more slowly, and once that response started she continued to improve pretty solidly until her levels hit normal in March 2013.

Right at that time her platelets started dropping again. She had follow-up liver and bone marrow biopsies in April, and her liver looked great; fully healed. Her bone marrow, however, was at 5-10% cellularity, meaning that most of the stem cells in her marrow that make blood cells (there are three types: WBCs/white blood cells, RBCs/red blood cells, and platelets) had somehow been destroyed. This in turn meant that her damaged marrow could not produce enough blood cells to live.

At that cellularity level, she was diagnosed with Severe Aplastic Anemia (there’s “regular,” Severe and Very Severe).

We discussed treatment options. The preferred treatment (and statistically the most successful) is a bone marrow transplant from a matched sibling. Jasper had already been typed and we knew he did not match.

The next best treatment (this is the one Jila had) is called ATG/cyclosporine therapy, which is an immunosuppressive drug regime. First, 10 days inpatient with anti-thymocyte globulin (ATG) given via IV 8-10 hours a day. The ATG kills off the suspected rogue white cells that are believed to be responsible for attacking the bone marrow (and previously, the liver), which should then allow the remaining bone marrow to rebuild its supply of stem cells, which can then make blood cells again.

Starting at the same time as the ATG, patients start taking cyclosporine, which is an immunosuppressive drug often taken by transplant patients to fight organ rejection. The cyclosporine basically “turns off” certain white cells (T-lymphocytes) so they stop attacking stem cells in the bone marrow. According to our wonderful doctor, Dr. Michael Grimley, the cyclosporine kind of keeps a lid on the white blood cell production and somehow helps “train” them so the new white blood cells don’t make the same mistake as before and attack the body’s own cells as if they were foreign invaders.

Patients typically take cyclosporine for at least 6 months, and from what I understand it’s not uncommon to be on it a year, two, or longer. Every patient is different in terms of being able to wean off of cyclosporine without relapse.

Jila did the ATG treatment starting on April 26 for 10 days. She showed almost no improvement until June 1 — she really had us all sweating — but starting then, her counts have been rising steadily ever since. From mid-May to late June she daily took a drug called neupogen (aka G-CSF, for granulocyte colony-stimulating factor) to boost her neutrophil counts which were dangerously low, but hasn’t had any since July 1 and her neutrophils are holding steady without the drug (yay). Neutrophils are important in fighting bacterial and fungal infections (and after this experience I know more about fungal infections than I ever wanted to know).

Of the three types of blood cells (WBCs, RBCs and platelets), the WBCs are expected to rebound first and platelets last. There seems to be some variation here but several times the docs and nurses have said the platelets are the last to start climbing. So I have always had this in my mind as an important milestone.

Jila’s platelets started climbing on their own just a week ago, right around July 15. After she got the ATG she had been needing transfusions about once every 5 days, then it started to extend recently to about a week, then last Thursday for the first time, the counts were higher than they had been on Monday—no transfusion needed! And today (Tuesday) they’re a bit higher still. So we’re all excited to know her marrow is now producing platelets too.

I’ve been told over and over that there are typically a lot of bumps in the road with aplastic anemia and blood counts naturally go up and down a fair amount, so I’m always careful not to get attached to expectations. A virus could set her back; her marrow might get tired again; who knows. Her counts could go down again and she might need more transfusions.

But it’s also possible she has really turned a corner and won’t be looking back. I find myself peeking back just out of caution and fear but am trying to just let myself enjoy the hope that we won’t have to go back around that corner ever again. If we do we do. I hope we don’t.

Jila in the grass

Jila was allowed to play in the grass for the first time last week.