Trip had a follow up yesterday with Dr. Appel. She agrees that his skin has greatly improved and even thinks the eye bulging has gone down a little more, which hopefully means the mass in the orbital area is shrinking. She sent us to radiology for his skeletal survey. He was the perfect patient and almost knows how to position himself for the numerous “pictures” since he has been through it so many times.
Dr. Appel called last night to tell us the bone lesions look stable and a few are slightly improved. Of course we want all the lesions to be gone/healed, but we’ll take this. It means we can stay on methotrexate for the time being, which means we can count on him tolerating the drug well (not the hospital stay – the drug). She plans to give him two more rounds of chemo then do an MRI to check the existing mass and make sure the brain hasn’t been affected further. If things continue to improve on methotrexate, he will possibly continue it for the next 4-5 months and then hopefully move to an oral chemo for 6 months after that. We know at this point, we can’t count on anything to stay the same (especially treatment), but we hope this best-case scenario plays out to be true.
Last night I started on the mounds of medical bills that have been piling up since the beginning of the year. I was tired and didn’t want to look at them but just wanted to add them up and write a check. I’m thankful I sucked it up and went through them all because I found so many errors: wrong patient name/services, wrong company name that resulted in out-of-network claims, misapplied coinsurance. Those errors resulted in $2k overbilling that I would have missed if I had been lazy. Today’s lesson: always, always check and double check medical bills.
We are gearing up for Hike for a Cure in Austin on March 24. We have so many friends and their families participating and feel so blessed. The fund raising efforts have amazed me so far and give me hope that one day our efforts will lead to a cure. Together we will fund a CURE! http://www.active.com/donate/h4ac-texas
Wednesday, February 29, 2012
Thursday, February 23, 2012
Small Victories
Trip had a great birthday! Thanks for all of the happy wishes. Gaky was here for his birthday or we might have postponed it. I have strep throat and between trying to rest, work and make sure Trip didn’t get it everything was on Gaky’s shoulders. She got him a cake, bought him many presents, and made his birthday special.
We went in for blood work today. Everything is good except his hemoglobin, which probably explains why he is more tired than usual. We also had to get his monthly dose of Pentam, which is an antibiotic that he has to have because he’s allergic to the other two options. To get the medicine, we have to sit in a booth (phone booth size) and wear masks. His mask produces a gas that he has to breathe and my mask is to protect me from the carcinogen. This is usually 15 minutes of hard labor for me, as I have to hold his mask on and physically restrain him from ripping it off & running out of the booth. Today, he decided he was going to hold the mask on by himself. He almost held it the entire time, only cried half the time, and I never had to restrain him. I am so proud! He asked me yesterday, “Mom, what do 4 year olds get to do?” (probably thinking I was going to tell him he gets to start driving now). Today, I told him one thing is they learn they can do really hard things by themselves.
The FDA has given permission for another company to start making methotrexate, so hopefully the shortage scare is behind us. We go in for a follow up with Dr. Appel on Tuesday. We should get a skeletal survey then too. Hopefully it will show improvement so we can stay on methotrexate.
Friday and Saturday, Feb 24-25, Liam’s Lighthouse Foundation will be outside the Today Show to raise awareness for histiocytosis. Family, friends, volunteers will be holding posters of ones diagnosed with histiocytosis to draw attention to this rare disease. Tune in if you have time.
Today, I’m celebrating the small victories.
We went in for blood work today. Everything is good except his hemoglobin, which probably explains why he is more tired than usual. We also had to get his monthly dose of Pentam, which is an antibiotic that he has to have because he’s allergic to the other two options. To get the medicine, we have to sit in a booth (phone booth size) and wear masks. His mask produces a gas that he has to breathe and my mask is to protect me from the carcinogen. This is usually 15 minutes of hard labor for me, as I have to hold his mask on and physically restrain him from ripping it off & running out of the booth. Today, he decided he was going to hold the mask on by himself. He almost held it the entire time, only cried half the time, and I never had to restrain him. I am so proud! He asked me yesterday, “Mom, what do 4 year olds get to do?” (probably thinking I was going to tell him he gets to start driving now). Today, I told him one thing is they learn they can do really hard things by themselves.
The FDA has given permission for another company to start making methotrexate, so hopefully the shortage scare is behind us. We go in for a follow up with Dr. Appel on Tuesday. We should get a skeletal survey then too. Hopefully it will show improvement so we can stay on methotrexate.
Friday and Saturday, Feb 24-25, Liam’s Lighthouse Foundation will be outside the Today Show to raise awareness for histiocytosis. Family, friends, volunteers will be holding posters of ones diagnosed with histiocytosis to draw attention to this rare disease. Tune in if you have time.
Today, I’m celebrating the small victories.
Sunday, February 19, 2012
Home Sweet Home
We were discharged from the hospital are home catching up and resting. Trip feels “free” again and even did some breakdancing so the doctors & nurses could see how ready he was to leave. The Methotrexate has not been too hard on him other than spending the few days in the hospital and being hooked up to machines. He has been nauseated but finally ate a little when we got home. Hopefully we’ll be back to our normal until we go back in 10 days (assuming his skeletal x-rays show signs of improvement).
His endocrinologist added a test while we were there to see if he is producing cortisol. He was on high-dose steroids for a big part of last year, so we weren’t able to test. We have been supplementing him with a small amount of hydrocortisone since he came off prednisone. The test proved his adrenals are not making cortisol, so we will continue to have to supplement the rest of his life. We have a shot that we have to keep with us for emergency situations and have to give him stress doses when he’s sick or goes under for scans or surgery. This of course can be and has been managed, but was still a disappointment to learn that his body isn’t doing its job in that area either.
We are humbled to have so much support from all of you. Every time we feel sad or overwhelmed, we are reminded how much support we actually have and how many people are in our corner.
I am so thankful my son came home from being in the hospital for days and still felt like going outside to play. I am thankful my family can have dinner together and can sleep under the same roof tonight. I am thankful that I can shower in my shower and sleep in a comfortable bed. I am thankful I have a full pantry to go to when I get hungry tonight. My lesson is not to ever take the luxuries in my life for granted. There is always something to be thankful for.
His endocrinologist added a test while we were there to see if he is producing cortisol. He was on high-dose steroids for a big part of last year, so we weren’t able to test. We have been supplementing him with a small amount of hydrocortisone since he came off prednisone. The test proved his adrenals are not making cortisol, so we will continue to have to supplement the rest of his life. We have a shot that we have to keep with us for emergency situations and have to give him stress doses when he’s sick or goes under for scans or surgery. This of course can be and has been managed, but was still a disappointment to learn that his body isn’t doing its job in that area either.
We are humbled to have so much support from all of you. Every time we feel sad or overwhelmed, we are reminded how much support we actually have and how many people are in our corner.
I am so thankful my son came home from being in the hospital for days and still felt like going outside to play. I am thankful my family can have dinner together and can sleep under the same roof tonight. I am thankful that I can shower in my shower and sleep in a comfortable bed. I am thankful I have a full pantry to go to when I get hungry tonight. My lesson is not to ever take the luxuries in my life for granted. There is always something to be thankful for.
Saturday, February 18, 2012
Breakdown
We saw Dr. Appel on Tuesday and asked about Methotrexate. At the time, they were having meetings on how to best “conserve” the drug. She admitted he is a candidate to get bumped if the supply does dwindle more, but thought that we could get one more round in. What they have explained to me is that are conserving and not rationing, meaning they will make sure kids that need the drug are admitted on the same day based on their prescribed amounts so that nothing in a vile is wasted – basically a logistical issue for them. They aren’t withholding it from any kid at this time. They think the media has blown it up a little but think that might help in getting the supply started again (with a much bigger price tag).
The new treatment plan is for Trip to have another skeletal survey in two weeks before his next dose is due to see if the lesions in his bones are improving any. (She wants to hold off on the MRI and the PET due to the amount of radiation exposure unless it is needed.) If the bones show improvement we will continue another two rounds of Methotrexate and then have the PET and MRI. If there are new lesions on the bones or growth in the existing lesions, we will go immediately to our toxic friend, Clofarabine.
This has been an extremely challenging week for our family outside of Trip’s disease and related medical issues. We had to make a gut-wrenching decision that ultimately broke my heart. It took my focus off of Trip and put me in a place where I had to call for backup. We were in the hospital yesterday when I called my mom in panic mode. Within 7 hours of that phone call she was hugging me and reminding me what is good, true and important in life. She was making my son happy and giving me comfort. What greater blessing is there in life than having loving, steadfast family members?
My lesson for the week is to keep my eye on the ball.
While Trip was hooked up receiving chemo yesterday and I was having my breakdown, he grabbed my hand, kissed it and hugged me. He said, “Mom, I’m going to do everything I can to take care of you.” He gives me peace, comfort and hope even when I should be giving it to him. He’s a beautiful child inside and out and I’ll be forever grateful that he is mine.
We will hopefully get to go home tomorrow if the Methotrexate is out of his system. His birthday is Tuesday, so I’m looking forward to celebrating his 4 years of life and spoiling him rotten just because I can. I’m so thankful we will be at home and together on his birthday.
The new treatment plan is for Trip to have another skeletal survey in two weeks before his next dose is due to see if the lesions in his bones are improving any. (She wants to hold off on the MRI and the PET due to the amount of radiation exposure unless it is needed.) If the bones show improvement we will continue another two rounds of Methotrexate and then have the PET and MRI. If there are new lesions on the bones or growth in the existing lesions, we will go immediately to our toxic friend, Clofarabine.
This has been an extremely challenging week for our family outside of Trip’s disease and related medical issues. We had to make a gut-wrenching decision that ultimately broke my heart. It took my focus off of Trip and put me in a place where I had to call for backup. We were in the hospital yesterday when I called my mom in panic mode. Within 7 hours of that phone call she was hugging me and reminding me what is good, true and important in life. She was making my son happy and giving me comfort. What greater blessing is there in life than having loving, steadfast family members?
My lesson for the week is to keep my eye on the ball.
While Trip was hooked up receiving chemo yesterday and I was having my breakdown, he grabbed my hand, kissed it and hugged me. He said, “Mom, I’m going to do everything I can to take care of you.” He gives me peace, comfort and hope even when I should be giving it to him. He’s a beautiful child inside and out and I’ll be forever grateful that he is mine.
We will hopefully get to go home tomorrow if the Methotrexate is out of his system. His birthday is Tuesday, so I’m looking forward to celebrating his 4 years of life and spoiling him rotten just because I can. I’m so thankful we will be at home and together on his birthday.
Saturday, February 11, 2012
Shock
A “chemo” mom friend sent me a text this morning asking if Trip would stay on Methotrexate with the shortage. WHAT??
I ran to the Internet to pull up this not-so-reassuring article: http://www.nytimes.com/2012/02/11/health/policy/supply-of-methotrexate-a-cancer-drug-may-run-out-soon.html
Since Trip isn’t a kid with ALL whose life would be immediately at risk if he stopped receiving the drug, my guess is we may get pushed to the low priority list. Just when we felt like we were on the right track.
I will ask Dr. Appel on Tuesday what the chances are we will get bumped. Until then I will hope they find a way out of this for all kids’ sakes that need this drug. We might end up on toxic Clofarabine after all. Seems so hard to be a cheerleader for poison.
Today’s lesson should be not to panic before all the details are known. I’ll try to remember the lesson after Tuesday.
Today's gratitude is that for a mom who thought of us when hearing that news piece.
I ran to the Internet to pull up this not-so-reassuring article: http://www.nytimes.com/2012/02/11/health/policy/supply-of-methotrexate-a-cancer-drug-may-run-out-soon.html
Since Trip isn’t a kid with ALL whose life would be immediately at risk if he stopped receiving the drug, my guess is we may get pushed to the low priority list. Just when we felt like we were on the right track.
I will ask Dr. Appel on Tuesday what the chances are we will get bumped. Until then I will hope they find a way out of this for all kids’ sakes that need this drug. We might end up on toxic Clofarabine after all. Seems so hard to be a cheerleader for poison.
Today’s lesson should be not to panic before all the details are known. I’ll try to remember the lesson after Tuesday.
Today's gratitude is that for a mom who thought of us when hearing that news piece.
Friday, February 10, 2012
“Mom, what’s Diabetes Insipidus?”
Yesterday we went to the hospital to get Trip’s blood counts and for an eye exam. His counts were great, which is good news but typical on Methotrexate. They are unlikely to go down on Methotrexate compared to the rest of the chemotherapy treatments he’s had. He did great on his eye exam and can even see a little better than I can with my contacts (which isn’t hard). As soon as the doctor entered the room, she exclaimed, “Oh his eye looks even better than the last time!” What a relief!! News we’ve been hoping to hear for so long. The bulging in his eyes has decreased to where he finally is getting a little crease in his right eyelid. The small victories mean so much these days since we went so long without any good news. We don’t have to go back to her until after the next MRI… big change since we were going every week not long ago.
This morning, Trip popped up from watching Tom and Jerry and out of the blue asked, “Mom, what’s Diabetes Insipidus?” We don’t shy away from talking about his disease in front of him, but I was surprised he remembered that specifically and while doing nothing. I tried to explain it so a 4 year old could understand it and told him his body doesn’t know how to hold onto his peepee like it should and it’s what makes him thirsty when his medicine wears off. He sat there for a few seconds to let it absorb and said, “You know what I think it is? Diarrhea.” (He said this because when his medicine isn’t onboard and he consumes large amounts of fluid, he sometimes ends up with diarrhea. He HATES getting diarrhea. It has the potential to ruin his day but typically stops once his shot is given.) I told him diarrhea is definitely a symptom of DI and that he’s one smart kid to even ask. I asked him if what I told him made sense to him and if he had any questions. He simply said, “No, Mom. I’ve got it now.”
Lesson of the day: Never underestimate how smart my kid is and how well he can take honesty even when I perceive it as bad news.
Trip had a very hard time last year when he was first diagnosed with needles, blood, doctors, getting accessed for treatment, etc. He would threaten to break the hospital down with his “magic” sword. It got to the point to where his doctors suggested he go to play therapy. It’s just like therapy for adults but the kids use toys to express how they are feeling. He goes once a week (as long as there aren’t any other more important appointments) and works for 30 minutes. He has missed the last two weeks because of treatment and another appointment. I can see a decline in his attitude when he doesn’t get to go. He has done play that indicates a feeling loss of control, which is totally understandable. Recently he has started doing death play. Colson thinks it reflects what we do at home – pretending to be injured and he saves us or vice versa, but his therapist thinks this is a little more serious than just being the hero. She said what he is doing in there is wonderful and the type of work he needs. I hope she is right and he does feel relief from the sessions. He seems to enjoy them, but a cardinal rule is that I ask him no questions about his session, not even if he had fun. I have no idea how he made the leap from his disease and what he is going through to death. I thought that was a very adult subject that wouldn’t occur to him, but once again he surprises me with his perception.
We go in Tuesday for more blood work and a follow up with the doctor. They want to admit him for treatment again on Thursday and hopefully out by Sunday. These hospital visits sure are going to make the weeks go by fast. We are looking forward to a weekend of doing nothing and everything he wants at the same time.
This morning, Trip popped up from watching Tom and Jerry and out of the blue asked, “Mom, what’s Diabetes Insipidus?” We don’t shy away from talking about his disease in front of him, but I was surprised he remembered that specifically and while doing nothing. I tried to explain it so a 4 year old could understand it and told him his body doesn’t know how to hold onto his peepee like it should and it’s what makes him thirsty when his medicine wears off. He sat there for a few seconds to let it absorb and said, “You know what I think it is? Diarrhea.” (He said this because when his medicine isn’t onboard and he consumes large amounts of fluid, he sometimes ends up with diarrhea. He HATES getting diarrhea. It has the potential to ruin his day but typically stops once his shot is given.) I told him diarrhea is definitely a symptom of DI and that he’s one smart kid to even ask. I asked him if what I told him made sense to him and if he had any questions. He simply said, “No, Mom. I’ve got it now.”
Lesson of the day: Never underestimate how smart my kid is and how well he can take honesty even when I perceive it as bad news.
Trip had a very hard time last year when he was first diagnosed with needles, blood, doctors, getting accessed for treatment, etc. He would threaten to break the hospital down with his “magic” sword. It got to the point to where his doctors suggested he go to play therapy. It’s just like therapy for adults but the kids use toys to express how they are feeling. He goes once a week (as long as there aren’t any other more important appointments) and works for 30 minutes. He has missed the last two weeks because of treatment and another appointment. I can see a decline in his attitude when he doesn’t get to go. He has done play that indicates a feeling loss of control, which is totally understandable. Recently he has started doing death play. Colson thinks it reflects what we do at home – pretending to be injured and he saves us or vice versa, but his therapist thinks this is a little more serious than just being the hero. She said what he is doing in there is wonderful and the type of work he needs. I hope she is right and he does feel relief from the sessions. He seems to enjoy them, but a cardinal rule is that I ask him no questions about his session, not even if he had fun. I have no idea how he made the leap from his disease and what he is going through to death. I thought that was a very adult subject that wouldn’t occur to him, but once again he surprises me with his perception.
We go in Tuesday for more blood work and a follow up with the doctor. They want to admit him for treatment again on Thursday and hopefully out by Sunday. These hospital visits sure are going to make the weeks go by fast. We are looking forward to a weekend of doing nothing and everything he wants at the same time.
Tuesday, February 7, 2012
Throw a fit, give thanks and write.
We got discharged from the hospital last night but not before a small fit was thrown… by me. From a HemOnc perspective, Trip reacted as expected to the Methotrexate and his levels dropped to .06 – safe enough to be released. We were all so happy, so we packed up and waited for the discharge orders. Then the oncology fellow waltzes in and says, “Endocrine won’t release him until he’s on his home DDAVP routine.” HUH? I explain that his actual endocrinologist that was on call over the weekend and I worked out the details of how to manipulate his DDAVP dosage and that she has a lot of trust with my knowledge of his DI. I was told that doesn’t matter and that the doctor on call now is calling the shots. “YOU MEAN THE DOCTOR THAT DIDN’T EVEN STOP BY TO SEE HIM TODAY??!?!?!” That was the beginning of the fit.
I demanded to see the doctor for a full explanation of their reasoning and the goal they want Trip to achieve before going home. I explained my understanding and agreement with his endocrinologist’s perspective. This is the problem with hospital stays and having DI. DI is extremely subjective and every doctor has a different method of dealing with it. When Trip is in the hospital for any length of time, we might see 3 different endocrinologists with 3 different opinions (basically the reason we were in the hospital for weeks last year).
Not sure if the doctor heard about my fit and got scared or was already at home sipping on a martini, but he/she never showed up. About an hour later, the nurse came in and said we were getting released.
Lesson #1 for the day: Throw a fit. ☺ Actually, the lesson is to stand up for what I know is best for my son. My beautiful, intelligent, RN cousin told me she always encourages her nurses to go with the mom’s intuition because they know their child better than anyone. Good, solid advice. Trip’s endocrinologist knows everything we’ve been through and knows how I treat his DI, and she also encourages me to question everything. I’m happy I did.
When we were leaving the hospital Trip literally ran out with his mask on. We got looks like “that kid is on chemo?” and I loved them because to me it meant he was feeling good. When we got home, he ran around the island in the kitchen yelling, “I’m free! I’m free! I’m free!” and giggling. It was wonderful to see. Later the three of us got in the bed and wrestled I gave thanks for those moments… our little family was under one roof enjoying each other and my son felt good and happy! Lesson #2 for the day: Give thanks constantly for every little thing. Gratitude makes what I have enough and brings me joy.
Lesson #3 for the day: Write. The writing thus far has been so therapeutic and has brought people to me that encourage me. It has given me much perspective on the day-to-day events and a way to process the feelings. I didn’t realize how much better I would feel in doing it and how many people are out there cheering us on. THANK YOU TO ALL. The encouraging words make all of this a little more bearable.
I demanded to see the doctor for a full explanation of their reasoning and the goal they want Trip to achieve before going home. I explained my understanding and agreement with his endocrinologist’s perspective. This is the problem with hospital stays and having DI. DI is extremely subjective and every doctor has a different method of dealing with it. When Trip is in the hospital for any length of time, we might see 3 different endocrinologists with 3 different opinions (basically the reason we were in the hospital for weeks last year).
Not sure if the doctor heard about my fit and got scared or was already at home sipping on a martini, but he/she never showed up. About an hour later, the nurse came in and said we were getting released.
Lesson #1 for the day: Throw a fit. ☺ Actually, the lesson is to stand up for what I know is best for my son. My beautiful, intelligent, RN cousin told me she always encourages her nurses to go with the mom’s intuition because they know their child better than anyone. Good, solid advice. Trip’s endocrinologist knows everything we’ve been through and knows how I treat his DI, and she also encourages me to question everything. I’m happy I did.
When we were leaving the hospital Trip literally ran out with his mask on. We got looks like “that kid is on chemo?” and I loved them because to me it meant he was feeling good. When we got home, he ran around the island in the kitchen yelling, “I’m free! I’m free! I’m free!” and giggling. It was wonderful to see. Later the three of us got in the bed and wrestled I gave thanks for those moments… our little family was under one roof enjoying each other and my son felt good and happy! Lesson #2 for the day: Give thanks constantly for every little thing. Gratitude makes what I have enough and brings me joy.
Lesson #3 for the day: Write. The writing thus far has been so therapeutic and has brought people to me that encourage me. It has given me much perspective on the day-to-day events and a way to process the feelings. I didn’t realize how much better I would feel in doing it and how many people are out there cheering us on. THANK YOU TO ALL. The encouraging words make all of this a little more bearable.
Monday, February 6, 2012
Is having no answer an answer?
Trip’s Methotrexate infusion finally finished yesterday. So far so good. He doesn’t seem to have much of an appetite, but that is normal for a chemo week. When the infusion finished and the machine beeped he yelled in delight, “Mom, we’re finished! We can go home!” He is used to the machine beeping and then we get to go home. Not this time. We have to flush the Methotrexate out of his body and no one really knows how long that will take. Leukemia patients get more of a dosage, so it might not take the three days it takes them to clear it but he has DI so it might take longer if he can’t pass the fluid as quickly as he should.
I tried to explain to him that the beep this time doesn’t mean we get to go home. Should my answer to him be, “Trip, with this chemo protocol we have to wait until the Methotrexate clears your blood. Because you have Diabetes Insipidus it is a little harder to manage because your kidneys don’t release fluid like they should. We have to wait and see how long it will take.” or should it be “We get to leave when they say so.”
My defeat in not knowing how to answer him led to a non-answer. Maybe my brain is fried or I’m just tired. Luckily he’s a trooper and goes with the flow other than occasionally telling the nurse, “I’m taking you down, Jennifer!” as Alvin says on Alvin and the Chipmunks. My lack of an answer to him didn’t greatly impact his demeanor.
I slept at home last night so I could get up this morning and work early and Colson stayed with Trip. On the way home I had a chance to meltdown in private. Seems like there is a big black cloud hanging around our friends. We have an LCH buddy who is fighting for his life after a bone marrow transplant last week. We have friends who were forced to say an early goodbye last weekend to the newborn they have been preparing for 9 months to take home. We have yet another friend who begins his third fight against cancer this week in hopes of staying around for his wife and kids. It’s almost too much for a heart to take. I don’t have an answer for any of us when I so desperately want one for all of us.
Why are these things happening to them, to us, to decent people who try to make a difference in this world? “It is the way it is.” Is that an answer? “I don’t know.” Is that an answer? Regardless, my demeanor in front of Trip needs to be as his was to me – no impact just because I don’t get an answer. I need to be strong and comforting to him even when I’m confused and need comforting myself.
My lesson of the day: accept that there aren’t always answers that make sense. This lesson may have to be re-learned over and over again because putting this into practice is harder than knowing what the lesson is.
Wednesday, February 1, 2012
2011 Catch Up
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.
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.
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