Trip was diagnosed with Langerhans Cell Histiocytosis in January of 2011. He has damage to his skull, bones, gums, skin, ears, and brain, specifically the pituitary gland. The skull and pituitary gland indicate central nervous system involvement, which means the LCH is more likely to return and there is a risk for brain lesions to develop.
The pituitary gland damage will not reverse but so far the only thing they think has been affected is vasopressin, which is the hormone that regulates fluid intake/output - basically dehydration. It’s called Diabetes Insipidus (which is nothing like sugar diabetes) and he will have that the rest of his life. The replacement hormone he receives is DDAVP by shot twice a day. The hormone tells his kidneys to hold onto fluid in his body, so he stops going to the bathroom and stops being so thirsty when his DDAVP is on board. He has two “breakthrough” periods a day, each for about 2 hours, when the medication wears off and he drinks and urinates constantly. We are very lucky he still has a thirst mechanism.
We have become pros at managing his DI and know that one day he will be also. We spent weeks in the hospital getting his dosage right, then weeks of getting daily finger pricks to check his sodium levels and writing down everything he drank and how much he urinated. We are glad those days are mostly behind us even though the DI will always have to be managed.
Trip originally received weekly chemo treatments of Vinblastine for 6 weeks and then moved to maintenance every 3 weeks. He was also taking a steroid, Prednisone, which helped with his skin. At the end of the 6 weeks he had an MRI and CT scan, which showed little improvement, but we still moved to the maintenance phase every 3 weeks. Unfortunately, the Prednisone falsely led us to believe the treatment was working because of the improvement in his skin.
Boy were we in for a shock… the Summer 2011, we learned that not only was there no improvement in the disease, it had spread to all parts of his little body. Still no risk organs were involved, but now we had major tumors around the orbital areas. We were terrified he would go blind from the pressure to his optic nerve. It was probably the lowest of the lows in our year of this horrific disease. We were fearful for his eyesight and for the lack of being able to get this disease under control.
We immediately changed protocols and went to a Vincristine and Ara-C combination. It was awful. The Ara-C produced flu-like symptoms and gave him rashes. If you know about having a port (surgical implant where the chemo goes into his body), you know that any time you get fever you end up in the hospital to ensure there is no bacterial infection – even if they think the fever is a chemo side effect. The rashes on top of the LCH skin issues were awful and impossible to treat. Trip powered through those six weeks only to learn again that that protocol didn’t work either.
At that point we were seeing an eye doctor once a week to be sure his eye sight wasn’t getting any worse, an ear doctor almost every two weeks, weekly visits to clinic, monthly visits to his Endocrinologist, chemo every three weeks and still no relief.
Our HemOnc in Dallas started us on 2-CdA and told us she was leaving, but leaving us with Dr. Appel who has taken a great interest in LCH. After starting the 2-CdA we were fortunate enough to get an appointment with the leading LCH expert in the U.S., Dr. Kenneth McClain, who is located in Houston.
We took a “family vacation” in September to Houston. We went a day early to go to Galveston so Trip could see the beach (although not a very pretty one!). We got to go on a dolphin tour, which he loved, and he got to swim in the ocean. When we got in the car to leave the beach and head to Houston for the night he was so thirsty even though he hadn’t broken through yet. We finally figured out he had been drinking the salt water while he was swimming!!! Talk about a bad idea in general, but especially for someone with DI. I’m glad we didn’t have any issues from that and happy we didn’t have to get his sodium levels checked… I imagine they were scary high!
We saw Dr. McClain at Texas Children’s. Just being with him gave us a little peace. He felt confident that 2-CdA would work for Trip’s LCH. He told us that he felt certain Trip would never need a bone marrow transplant, which made us breathe a little easier. He did say that most people with LCH experience 4 recurrences before the disease completely goes into remission. I asked if what we had been through thus far could be considered as recurrences, but we aren’t that lucky. He expects once the disease goes away, it will come back 4 more times… YUCK. We learned Dr. McClain is starting some great genetic research that we agreed to participate in and he told us we are one of five families that have stored cord blood, which could be very useful in his research. (At this point with the knowledge of the disease and where they are in using cord blood for treatments, it is unlikely that Trip’s cord blood will ever be useful because the assumption is he was born with the disease. If they used it, it would only be putting the disease back in his body. I still hold out hope for one day there could be something useful to come from it.) Dr. McClain sent us home with a little higher dosage of 2-CdA than he was getting and the name of the drug we would use if the 2-CdA didn’t work (Clofarabine).
Fast forward to January… Trip completed all six rounds of 2-CdA that his body can handle without doing long-term damage to his platelets. We thought we could see improvement in his eyes. They were still protruding but not nearly as much. He had another round of scans: bone survey, ultrasound, MRI and PET scans. We were hopeful, but not too optimistic given everything we had been through.
The results of those were “some improvement but still active disease” all over his body. The orbital mass is still there but not nearly as big, there are still bone lesions and the obvious skin and ear issues. The MRI shows parts of the brain that have been affected but so far there are no symptoms. Trip has MRI ND CNS LCH… got that?? It is Neurodegenerative Central Nervous System Langerhans Cell Histiocytosis and so far the neurodegenerative part only shows up on the MRI. There are some people with this condition that exhibit signs such as loss of coordination, loss of speech, slower development, etc., and there are some who only have the disease on the MRI and not apparent in day-to-day life. We will have to start testing Trip to get a baseline to watch as he grows to see if there is any deterioration in his condition.
So we have results, we should jump into Clofarabine, right?? Nope.
Our doctors in Dallas consulted with Dr. McClain and even though he still said Clofarabine now, they did not want Trip on a drug so toxic. Clofarabine has harsh side effects and more times that not lands kids in ICU. Our team here has had good luck using a less toxic drug, Methotrexate, for LCH, so they decided that’s the way to go for Trip and we will have the Clofarabine to fall back on.
So here we are now… Trip will get Methotrexate every two weeks, but it means hospitalization during the treatment instead of outpatient clinic like we are accustomed to. It is important that Methotrexate leave the blood, so Trip has to pass it through his urine. Not so easy in a child with DI. We are admitted and monitored by his HemOnc and his Endocrinologist. The Methotrexate is given over a 24-hour period and they expect it to leave the blood within three days.
Trip will have another round of scans in about 6 weeks to determine if this treatment is working. If it is working, it may mean less time on Clofarabine or hopefully it won’t even be needed. The less toxic the better after a year on chemo and all the other issues that he will have to overcome because of this disgusting disease.
I think that brings us up to date on his condition and where we are. I’ve heard from other LCH and “chemo” moms that writing is a way of therapy, so I hope they are right. I still question everyday what the lesson is in all of this. We are learning lessons everyday on this journey and I will try to share them here. My question still remains, couldn’t I learn these lessons without my child having to suffer from a rare and horrific disease? Maybe one day I’ll find the answer, but right now I’m pretty sure it’s simply because life isn’t fair. “Fair” is a place for corn dogs and ferris wheels.
