Long-overdue update! Things are good.

jila's carnelian bracelet

Beautiful carnelian and amethyst bracelet the lovely Chela Gurnee gave to Jila. 🙂

Things are well! I feel guilty I haven’t updated here. Jila responded well to immunotherapy after all (all the way back in May 2013), and the farther we got away from having to think about a bone marrow transplant, the more I wanted to put it behind me. It was really traumatizing to face the possibility that she would not respond to the ATG and the fact that there was no match. It is still traumatizing, but I’ve got my sea legs now.

Today Jila is in remission, and her counts are strong, but still on cyclosporine. Her counts dipped when trying to wean last summer so the docs want to keep her on it for at least a few more months. Some patients are on this drug on and off for years. Some relapse. Some put the whole thing behind them as an isolated episode. We are hoping for this last outcome, naturally.

We’ve had a chance to catch our breath and get our lives together after 2013 threw us totally off the rails. And now I feel ready to start advocating and helping organize and develop resources for those needing bone marrow transplants. Obviously I hope Jila will never need one, but I want to do what I can to try to find one just in case. The worry that she will need one will probably never go away. So I’ll feel better if I keep myself busy trying to find a match for her, and the wonderful side benefit of this is that along the way this should help get matches for other patients.

I have no idea how to do this (i.e. organize a campaign and/or swabbing drives) and honestly have been blown away by what I’ve seen other parents accomplish, under unfathomable stress. I would like to help make a difference, especially because I feel so incredibly thankful and lucky that our outcome has been good so far. So I’m going to see what I can do. Stay tuned.

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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.

Counts on the rise, and heading north

We are headed to Milwaukee! After three solid weeks of improving counts the docs say we can get out of Dodge and chill out for a few weeks. We are now in the “wait and see/will it stick” phase. Jila will still need blood tests twice a week, transfusions, medications, etc. but she is solidly, strongly improving. As we pack up and move out of Ronald McDonald House and hit the road tomorrow morning my heart and thoughts will be with the other kids here and their families who are hoping and praying for the same. Again, Jila is not out of the woods (and if a transplant turns out to be needed down the road, we still don’t have a match), but today is undoubtedly great news.#swabforjila www.marrow.org

Great news today!

Jila’s blood counts are on the rise again! After a couple days downward they took a big jump again today, and we are getting discharged, whoo! We’ll be moving across the street to the Ronald McDonald House where I’ve had a room for the past week. Now that Jila will be joining me for the first time, they will give us a new “isolation” suite that is set up for immunocompromised kids. Unlike families in regular rooms, we’ll be allowed to eat in our room since it wouldn’t be safe for Jila to eat in the main dining areas.

The Logistics

The logistics of all of this have been rather mind-boggling, and I’m not exactly sure how we’re going to manage this next phase with Jila and I both at Ronald McDonald House. (Not complaining — I’m thrilled! But just thinking out loud about how to manage this.) The last time Jila was discharged (after the ATG treatment) her counts were so low they recommended we stay in our own hotel room with a kitchen, rather than Ronald McDonald House which has a shared kitchen. So we got a room at the Residence Inn Marriott and my awesome dad came to stay with us so I could grocery shop, pick up prescriptions, etc. (Also my brother-in-law Tim has been a huge help in picking up groceries and prescriptions during the times I’ve been on my own.) That was great — until we got readmitted two weeks ago to the hospital with expectations that a bone marrow transplant was imminent, meaning a likely couple months or more in the hospital.

Rather than keep paying for our hotel room (which also was a 25 minute drive away), we decided my dad would head back to Milwaukee and I got on a waiting list for a room at the Ronald McDonald House which is across the street from the hospital. Turtle and Jasper came out right away (again, thinking BMT was imminent) and after a few days trading off with Turtle staying at the hospital and me staying with Jasper at the Residence Inn, a room opened up at the RMHouse. So we moved into that last Thursday. When Turtle and Jasper went back to Albuquerque on Sunday I also returned the rental car which I didn’t really need anymore. So as of Sunday I’ve been flying solo.

Having a room at RMHouse has been a godsend on many levels. I can pop over for brief periods to do laundry, and best of all, eat. When I got the tour of the place I was told that volunteers made three meals a day, and I thought to myself, “Eh, I’ll make my own meals, no problem.” Well other than the occasional bowl of yogurt, I’ve totally not cooked at all. It’s incredible and moving and heartwarming and just plain nourishing to be there and witness what people do for each other in a time of need. Not having to spend energy on making meals for myself is something I didn’t realize would be such a blessing, but it is a huge one for which I’m incredibly thankful.

The tricky part will be figuring out how to get groceries so I can cook for Jila. Our doctor said even though her counts are better and she’s safer, he’d still rather that I prepare her food and that she should avoid buffets, which is how meals are served at RMHouse. RMHouse offers shuttles to grocery stores, but I can’t leave her alone (she’s 7 remember). I already have some groceries there (each family gets a section of the refrigerator and a locked cabinet) but I’ll need to replenish soon. I may be calling family later today to see if they might be able to come help again. (Heads up Kay and Reza!)

The Medical Situation

We have been back in the hospital for just over two weeks, since the day her distressing bone marrow biopsy results came back and the doctors told us we’d imminently have to start the process of bone marrow transplant. Then, two days after that, her counts started to rise. After several days of strong improvement the docs said it was a great sign that she had some healthy stem cells in her marrow that were starting to produce. The big question was (and is) whether those cells can keep it up and reproduce so that Jila once again can have a healthy immune system, not to mention other important things like platelets to control bleeding. As of today things are looking good.

After ATG therapy (or a bone marrow transplant for that matter), the first cells that are supposed to come back are white blood cells (WBCs) and a specific type of WBC called neutrophils, which are important for basic safety because they prevent bacterial and fungal infections, including life-threatening ones. Jila’s neutrophil count (called ANC, for absolute neutrophil count) was abysmally low before her turnaround two weeks ago, which is the main reason we’ve been back in the hospital. Now that her WBC and ANC counts have improved so much there’s less need for us to be in the filtered air and germ-controlled environment of the hospital.

We’ve been on the bone marrow transplant (BMT) unit which has considerably stricter rules/controls than “regular” hospital units. For example, parents are not allowed to eat or drink in patients’ rooms, nor use the bathrooms in the room; you have to be buzzed into the unit and wash hands immediately upon entering; they take the temperature of young children visitors (like Jasper) to prevent them coming in sick, etc.

Jila is still quite susceptible to viruses, so she’s not supposed to spend a lot of time in public for at least a couple months, and when she does she’ll have to wear a mask.

Another thing that probably won’t recover for a while is her platelet count. Platelets are the type of blood cell that help you stop bleeding by promoting clotting. They are one of the last types of cells to recover after ATG, chemo or bone marrow transplants. She has needed platelet transfusions about every 4-5 days since late April, and will likely continue to need them for at least a while. When she starts to maintain her platelet count and produce her own, that will be a great sign that the ATG treatment has really truly worked. I don’t even really know when to hope for that; I haven’t asked as I’ve just been focused on the immediate next milestone which is improvement in WBC and ANC.

I’m so incredibly happy for today’s good news. 🙂

Calling all Irish-Persians (aka Irish-Iranians)

Based on the genetic testing done on my daughter Jila and her extended family, it appears that the tricky part of her HLA typing comes from my dad Reza Pakroo who is Persian (aka Iranian; same diff), making Jila 1/4 Persian. Jila’s dad Turtle O’Toole is pretty much fully Irish. Ethnic/racial backgrounds make a big difference in finding a match, so if you are Irish-Persian or know any Irish-Persian folks, would you please consider doing a cheek swab test and joining the Be The Match bone marrow donor registry in the US, or an international registry?

Jila and Baba (her grandpa) Reza. Yes, this blondie is 1/4 Persian.

Jila and Baba (her grandpa) Reza, who is Persian. Believe it, this blondie is 1/4 Persian!