Dr. M returned this evening  around 7pm to discuss our status, our next steps and his discussions now 24 hours after our first devastating meeting where we learned the diagnosis. We first discussed Zoe’s current state:

  • Her liver and spleen remain enlarged
  • Her blood count (hemoglobin) came up to 8.6 from 7.9, but her platelets remain low at 15 (15,000)
  • The ultrasound was able to determine that her abdomen is clear and, despite the enlarged liver and spleen she has no other indications of problems or tumors; this procedure also eliminated Neuroblastoma (a tumor on/near a gland as I understand it) as a potential source of her symptoms, leaving only HLH
  • The team of doctors with whom Dr. M consulted on Zoe’s treatment and diagnosis was unanimous in their opinion that Zoe has HLH
  • The team was unanimous in supporting the treatment regimen that Dr. M was to propose

Our next step is Chemotherapy. A nasty word for most adults, particularly those who have family members as I do who have undergone some form of it. It’s difficult to set aside the fear and uncertainty of something like chemo in order to see it as the necessary step for Zoe, but that’s what we must do. Chemo Therapy, a type of treatment using chemicals to combat disease, support the body, and suppress out of control bodily reactions to a disease. Movies and television and life itself imbue words with such negative power that it can be heartbreaking to even consider, but the reality of it is manageable when compared to the alternative — death.

Zoe’s treatment plan follows the agree upon international protocol also known as HLH 2004. This plan resulted from study primarily in European nations which, for whatever reason spend more resources studying HLH and have been more heavily involved in determining a treatment approach.

The Chemotherapy itself consists of 3 drugs, Dexamethasone, Cyclosporine-A, and Etoposide or VP-16. The side effects (or at least a portion of them) are as follows:

  • Dexamethasone: has a steroidal component, will likely cause her to be fussy, hungry and as a result of the hunger result in some weight gain
  • Cyclosporine-A: can cause excess hair growth (including back hair and facial hair, even in infants) and kidney problems
  • Etoposide or VP-16: the strongest of the 3 and the one that requires a central line (implanted IV); causes hair loss over time, can cause cancer (roughly 5% risk), can cause allergic reaction; the risk of cancer from Etoposide is not inconsequential, however Dr. M was adamant that the risks are far outweighed by the benefits.

After discussing the chemo plan, we discussed next steps. Zoe is to begin to receive the first two drugs within 24hrs, and will receive Etopicide once the central line is in. The central line is an implated IV line that is typically put on the left side so as to allow reasonable access to the heart. The line is inserted into a large artery so as to allow any medications to be rapidly dispersed into the bloodstream and allow easy blood draws for testing. This is used in lieu of an IV on an external limb for two primary reasons: first, some medications can destroy the smaller veins on limbs causing complications and preventing proper circulation of the medication, and second to allow less intrusive blood draws for the repeated tests that will be needed to determine platelet count and other statuses.

Next step is to be moved to the Hemo/Oncology ward on another floor where doctors are always available and nurses have specialized training to handle cases such as Zoe. The ward is currently overfull, with some patients residing in nearby rooms instead of that ward, however there is hope that we can move to our long term room in a few days. Around this same time Zoe should receive the central line, and soon after Maya (our 2 1/2 year old girl) will come in for a blood test to determine her viability as a BMT candidate.

We discussed risks again after talking about our next steps. This discussion was much more reassuring and indeed left Michelle and I with hope rather than despair. We’ve come to terms with Zoe’s status to what extent we can after a day, and have begun to try and find rays of hope in our discussions. We greeted Dr. M with our concerns when it came time to talk about the overall treatment, and he was as reassuring as I trust he could be. My statement to him was essentially, we need something to give us hope that this treatment can have a positive outcome. We need to feel that Zoe has a chance, however moderate, of coming through her pain and disease relatively unscathed. We recognize that she has terribly high risks (far more than single digit) of death and disability, but we want to hear her chances for success so there is something positive to hold on to through this.

Dr. M felt comfortable saying that Zoe has a better than average, better than 50% chance of coming through this completely unscathed. Yes, she will see doctors for the remainder of her childhood and yes, there remain chances of large and small complications — but there is also a solid chance for complete recovery and cure. This is what we needed.

The final part of our conversation of the evening was about the BMT, the procedure that had become our greatest fear. Dr. M described a few more details of the procedure, and we told him about a friend who, small world as it is, knows one of the team members on the Pediatric Bone Marrow Transplantation team at Duke. We’ll be put in contact with the team at Duke as we get closer to the BMT, which is estimated to be around the May/June timeframe (2-3 months). We asked after the mortality rate of BMT, and with caution Dr. M gave us the currently accepted number, which is 30% mortality or 70% chance of survival. Obviously an estimate, but one that we can stomach for now.

The factor to be most aware of here is that it is not the procedure that can kill Zoe, it is the aftermath. It’s the chance of infection once her immune system is essentially wiped out and before it can be rebuilt on the back of her sister’s healthy bone marrow. An simple viral infection spurred the expression of this disease, and the same thing can end it all. Frightening.