As many of you already know, Golden has had a rough time of it lately. Early on the 8th of December 2007 we received a call from his pediatrician informing us that results from a blood test taken the day before revealed alarming information. We were instructed to immediately admit him to the pediatric emergency room at Beth Israel hospital in Manhattan.
Upon arriving Golden was subjected to a battery of tests which opened up the door to a host of possible disorders ranging from infection to disease. According to preliminary tests, Golden’s red blood cell and platelet counts were 10% their normal levels. In this situation, the imminent threat was heart failure and little guy was precariously close. Over the course of the next few grueling days Golden’s blood was stabilized through transfusions of blood and platelets. More tests were administered which were followed by less and less optimistic news as to what might be afflicting him. Finally, X-ray revealed lesions on his skull and right shoulder giving way to a likely candidate for what was the cause of Golden’s suffering. A rare and sometimes malignant cancer-like disease called Langerhan’s cell histiocytosis (LCH).
At this point Golden was transferred to the pediatric floor at Memorial Sloan-Kettering Cancer Hospital where he underwent further testing to confirm his diagnosis and to explore treatment options. The final diagnosis was high-risk multifocal LCH, with involvement of the skin, bones, bone marrow, liver, and spleen. Upon confirmation of LCH a medical port was implanted into his chest from which blood can be drawn and fluids(chemotherapy drugs, blood/platelets, antibiotics, etc.) can be transfused through and treatment began immediately consisting of chemotherapy and steroids.
After treatment began, Golden was switched to an out-patient status and discharged. He then received chemotherapy once a week, steroids daily, and typically attends the pediatric out-patient clinic at Sloan-Kettering three times a week for transfusions of blood and platelets and the occasional shot of GCSF(which inspires white blood cell production).
Golden underwent testing after 6 weeks of this treatment to see how the disease had responded. Happily, the tests revealed good news. No new lesions. The lesion in his right arm was healing and the ones in his skull were unchanged. His bone marrow tested negative for LCH! His transfusions needs had decreased. His liver and spleen were still enlarged, but smaller. His disease was categorized as active, better.
As per LCH III protocol, Golden began another 6 weeks of weekly Vinblastine and steroids. This round the steroids were reduced to 3 days a week. During this 6 weeks, Golden was often in Urgent Care with fevers and feeling pretty crummy. He also underwent an MRI to allay our fears that he could have new lesions in his head. The MRI revealed that not only were there no new lesions, but all four of the original ones were, in fact, healing! One was almost gone. Another ultrasound was done to check on the progress of his liver and spleen. The spleen was in normal range for size. The liver was still enlarged. His transfusion needs continued to decrease and we saw his platelets rise above 100 for the first time. He was getting better. Treatment was working.
It was decided, without hesitation, to start Golden on 9 months of maintenance therapy consisting of Vinblastine every three weeks, 5 days of steroids every 3 weeks and daily oral chemo, 6MP.
A few weeks into maintenance therapy Golden started to experience nausea and his counts began to slide down. He was put on an anti-nausea med and it was considered that the 6MP could be the culprit. To rule out toxicity the 6MP was discontinued. We waited another few weeks to see if his counts would improve. They did not. His liver and spleen also became enlarged again. An ultrasound and PET scan confirmed that his LCH was active and making a move on the reduced chemo.
After much debate among ourselves and with top LCH doctors from around the world, it was decided to start Golden on a regimen of high dose ARA-C and 2CDA immediately.
Golden’s blood counts got better after just one round of this new chemo. A very good indication that the treatment was working. Golden underwent a full body skeletal survey and an ultrasound of his liver and spleen after round two. These assessments revealed a slightly smaller liver and spleen, no new bone lesions and almost complete healing of all original bone lesions. More progress was being made.
Although we had hoped that Golden would only need two rounds of this risky high dose chemo, it was decided that he needed more to eradicate this disease from his body.
Round three was started in the end of July.
This new treatment proved to be very hard on our little boy. Round one left him shockingly thin. He weighed the same as he did when he was just 5 months old at his one year birthday, which was spent in the hospital. A g-tube was placed directly into Golden’s stomach to allow for tube feedings and to avoid further weight loss. Round two left Golden with an infection at the site of the g-tube that required additional time in the hospital. After round three Golden spent a week in the hospital with alarmingly high fevers, the source of which was never determined.
Thankfully, it was decided that Golden’s response to three rounds of high dose chemo was good enough to warrant a change in treatment. Round four and five were of 2CDA alone and in a much lower dose than in the previous three cycles. These rounds were done outpatient as well.
By cycle five Golden weighed 22 pounds and was crawling, cruising, babbling, playing, smiling and laughing everyday. He was a different child than from just three months earlier. We were so thrilled with his progress.
Golden has finished maintenance two. This was a 6 month phase to his treatment that started after cycle six. There were plenty of bumps in the road during those 6 months, but over all Golden came out healthier and stronger. During that time he learned to walk, talk (even talk back, “NO!”), eat (no more nursing), and sleep (through the night!). He had his first hair cut, took a 3 week vacation to California and turned two.
Golden is now a few months into maintenance three which is a year long phase. This treatment consists of two oral chemos only. He receives daily 6MP and weekly methotrexate. We take him into Sloan every two weeks for blood tests and a check-up. He continues to do great, is growing, learning and enjoying life. He even joined a soccer league. He’s a pure delight to both Bryan and I every day. We are continually amazed at how far he has come and how lucky we are to be his parents.