08-06-08

Some additional information on yesterdays results: when Brandon was hospitalized in December for low counts, we told his doctors of his recent excessive drinking and wetting (he was consuming 120+ ounces of water a day; a child his age and weight should we drinking 33 ounces of fluid a day). We were aware that a child diagnosed with LCH could develop Diabetes Insipidus (DI): A rare disease that arises as a result of posterior pituitary dysfunction; it is caused by inability of the kidneys to conserve water as they filter waste from the blood, leading to frequent urination and pronounced thirst.

They ran urine specific gravity tests and an MRI to check for DI. The results showed that Brandon's urine was border-line diluted for DI. His MRI showed that his pituitary gland was fusiform shaped and enlarged by 50%. These results were non-conclusive as his urine could be diluted due to the increased volume of water, and some people naturally have a fusiform-shaped pituitary gland. Dr. Wood recommended radiation of the pituitary before full-blown DI set in to prevent future progressive involvement, which can be life threatening. Our concerns were obviously radiation on his brain, and the risk of a secondary malignancy.

Dr. Wood spoke to Dr. Ken McClain, the leading LCH doctor in the nation, possibly the world. Unfortunately, there is not a National Protocol for treating the onset of DI, prior to DI becoming full blown. Dr. McClain recommended Cytosar (ARA-C, Cytarbine), which is a chemotherapy drug that enters the central nervous system. Cystosar has long been recognized as an effective systemic chemo drug for treating leukemia. The intent is for the Cytosar drug to enter the pituitary gland and prevent DI from progressing and still be effective in fighting LCH. The side effects of Cystosar are nausea, vomiting, fever, reddish eye discoloration, loss of balance/coordination. There is no negative effect on major organs such as the heart, lungs, kidneys, liver, etc. The treatment would be 100 mg/m2 of Cytosar for 5 consecutive days, every 28 days. Day 1 would be given in clinic and then Days 2 -5 would be given at home. Also, Cytosar will likely reduce RBC, Hemoglobin and Platelet counts. After a 6 month period the treatment plan will be reassessed. Brandon’s current treatment plan of Methotrexate, 6MP would cease immediately. However, Dr. Wood would still like to give Brandon 3.5 ml of Vinblastine around day 14 of the 28 day cycle. This is so we continue to remain aggressive with fighting the LCH and Brandon has typically responded well to Vinblastine. However, becoming neutropenic from the Vinblastine is a possible side effect due to the drop in WBC. Dr. McClain also did not support treating DI with radiation due to the possibility of more severe long-term complications and the sensitive nature of radiating the pituitary gland. Also, secondary malignancy is a strong possibility. A follow-up MRI on the pituitary gland will still be given in mid-January ’08 to see if the pituitary stock appears thickened or damaged, which would indicate that DI exists. While this plan was experimental, we were in favor of it as it did not include radiation.

So in conclusion, once DI develops, it is a life-long condition. In a child with LCH, it can effect the entire pituitary gland and be fatal. We caught the DI in the onset stages. It was likely noticed within a few days time, possible hours of onset. Prior to this, this was not believed possible. Brandon participated in an experimental treatment and was successful. He will likely be written up in medical journals, and his treatment may become the new protocol for LCH patients developing DI. We are so impressed with our medical staff, and feel blessed by their diligence! Thanks to them and God, we have our dear boy, and he will go on to live a long, fantastic life!