Day +113

Raul has now gotten three doses of the Etoposide, and will have his fourth on Friday. I have not noticed any side effects from it yet other than that his BP increases a bit while it is running (we expected it to decrease). He has also actually been more happy most of the time since being on the steroids, though occasionally gets extra grumpy. The steroids have made him not sleep as well the past few days. Yesterday I met with genetics and we sent of the testing to see if his HLH is genetic. The results will take anywhere from a couple weeks to two months.

Raul has not needed platelets in a week now; he has not gone this long since early October! He has still been needing blood more frequently, though. And he has not needed GCSF in a month, as his counts have stayed in normal range. He is still needing dialysis four days a week, unfortunately. We had hoped to go down to three, as his BUN is better, but he has just been retaining too much fluid. His Bilirubin has stayed down as well, so his eyes are almost back to normal color (very little yellow left)!

We got the results of Raul's engraftment check from his bone marrow biopsy. That showed 26% engraftment, which was similar to the CD15 of 24% we got from his blood (his CD3 was 97%). The good news was that his skin biopsy showed 18% donor cells! Anything above 15% is considered good, and even below that they believe that patients will see benefits. I was sort of surprised that his number was so good since his other engraftment numbers had dropped!

Raul has really been enjoying getting out of the room, and out of the hospital, lately! We have gone to the Ronald McDonald House on several passes, and last weekend even went to a model train museum. Some of the displays were too high for Raul to see from his stroller, but he liked that one of the lower sets had horn and whistle buttons that he could push! We have also gone out of the room to walk around the hospital the past few nights, which he had refused to do for a long time. Raul has been cooperating nicely this week for school, and seems to even be enjoying it some. He definitely seems to be having more energy! 

Day +108

After a few stressful days, we finally have a plan. We thought the plan was set last Saturday, but it changed several times over the past week, and we are now back to where we started! Raul's lumbar puncture has been clear so far, which is good news. His preliminary bone marrow biopsy results came back this morning, and they did see some hemophagocytes. Not a lot, but they were there, so we will begin the Etoposide (chemo) today to treat the HLH. He will get it twice a week for two weeks, then once a week for at least six weeks. Many cases of HLH do not have positive bone marrow biopsies, but our team only wanted to begin treatment if Raul did, as some of the other criteria he met was not as definitive. The good news so far from the biopsy was that he did have all the types of cells that he should have. The sample was 80-90% cellular, which is more normal than his last at home that was 95% (hypercellular). This time around the fact that he is on chemo will not necessarily keep us in the room like before transplant, it will just depend on his counts.

This morning Raul's platelets were the highest they have been since transplant! He got platelets on Wednesday and Thursday because of surgery, but he has gotten them multiple days in a row many other times and never gotten this high! His Hemoglobin has also been a bit better this week, and his BUN/Creatinine continue to be improved. Unfortunately his weight has been getting very high now that his diarrhea and fevers are improved and he isn't losing as much fluid from those. He has only had one low grade fever (100.4) and one real fever (103.4) in the past week! His Bilirubin is now down to 3.5 as well, which is much better, and he is not looking very jaundiced anymore.

We had a great night last night! We went to the Ronald McDonald House for Bingo, and Raul got a few awesome prizes. He talked almost non-stop for the few hours we were out, and sat up the whole time as well! We think it was the most normal he has acted since transplant! It was so fun to get to hear him talk, joke, and play again. And not a single whine, even while we were eating dinner. He still talked for quite a while after we got back to the hospital, too. 

Days +104/+106

Day +104
As usual, the plans changed again today. Raul did not begin the Etoposide (chemo), as they decided he is now healthy enough to get a bone marrow biopsy and spinal tap now that his BP and fevers are better. He will get these on Wednesday, along with the skin biopsies we delayed last week. They are not questioning the diagnosis, as he already meets enough of the criteria without those tests, but they do not want to give chemo before the tests as it could affect the results. Raul is getting the steroids once a day, and has not had a fever since they started! Because of scheduling, we are going to be starting dialysis around 7 tonight, which is much later than usual! Raul will not be happy to be woken up to get weighed at the end!

Day +106
Today Raul had his blister test, skin biopsies, bone marrow biopsy, and lumbar puncture. The preliminary testing from the lumbar puncture is normal (as we expected), but there are some tests that will take longer to come back. We should have the first set of preliminary tests from the bone marrow biopsy tomorrow, but more will take longer, including the engraftment information.

This time Raul's blister test took approximately 40 seconds. This surprised me as his thighs have what I consider to be "good" skin. I do not have the exact times from his two other blister tests, but the one in September they said was almost immediate as well. Usually with RDEB it takes several minutes, but the test results do seem to vary a lot, even within the same patient. Tonight Raul is having dialysis, so it has been a busy day!

Day +102

Day +100 is usually thought of as an exciting day, as that is when people can go home if they have not had too many complications. Unfortunately, it was just another day for us here in the hospital. We have had a stressful week with many changes in plans, dealing with the lack of answers for Raul's issues, and now some not so great news/answers.

Raul's engraftment this time showed his CD3 at 97% (which is slightly lower, but still good), and his CD15 down to 24%. This does not mean his transplant has failed, but it does mean his engraftment has dropped. With the transplants for EB, it is believed that even lower percentages of donor cells can be beneficial. We will check his numbers again in a month. If his CD15 was to drop to 0, with the CD3 still being higher, then a boost of cells may be beneficial to him.

Dr. Tolar stopped by last night to go over all this, and at that time the plan was that we would wean his CSA over 10-14 days instead of 8-10 weeks. However, they got some new test results back later last night, and now we will be keeping the CSA at a therapeutic dose. There is literature about going both ways: stopping the CSA vs. increasing suppression, so this change does not mean we are giving up on the donor cells!

Unfortunately, the new labs that came back last night gave Raul enough met criteria to be diagnosed with HLH (Hemophagocytic lymphohistiocytosis). This is an uncommon, life threatening, condition in which the body's immune system does not work properly. It can become overactive and end up destroying good cells and tissues in the body, which can lead to problems with the bone marrow and organs. Because of this Raul has persistent fevers, low platelets, low hemoglobin/hematocrit, very high ferritin, very high triglycerides, enlarged liver, enlarged spleen, and a high Soluble CD25 level. Due to the risks we will not be checking his bone marrow or spinal fluid, as it would not change the treatment. His kidney failure is a separate issue.

The treatment for this will include continuing the CSA, taking Decadron (a steroid), and taking a chemotherapy called Etoposide. The Decadron starts at a high dose, which is tapered over 8 weeks. After that he will get pulse doses every two weeks for three days. For the Etoposide he will get two doses each of the first two weeks, once weekly for six weeks, and then every two weeks. We do not know the exact length of the continuation therapy, but likely at least several months. There is no guarantee that the treatment will be successful, but the doctors are hopeful that it will be. Sometimes a bone marrow transplant is used to treat HLH, but Raul would not be a candidate for another transplant at this time due to his kidney failure.

Raul is beginning the steroid today, and will begin the chemo on Monday, as that needs to be dosed an hour before dialysis. I am hopeful that this steroid will perk him up like the last one we tried did, as it would be great to have him feeling a bit more like himself! They do not expect him to have too many side effects from the low doses of the chemo, thankfully. And since the chemo is a low dose, it should not kill off the rest of his donor cells, though I would assume that is always a possibility. 

Day +99

Today is day +99, and we have been in the hospital 120 days! Raul only needed blood twice in the past week, which is great! He has still needed platelets more often, though. His fevers are about the same. WBC/ANC still within normal range. Bilirubin still high, both direct and total. Because of his bilirubin he had another ultrasound today, which is generally unchanged. His liver and kidneys are still enlarged, and his spleen is borderline. The ultrasound basically just showed chronic problems with his liver and kidneys, but nothing specific that would help explain the high bilirubin. He also had a repeat chest CT yesterday, which was similar to the past ones, and showed some micronodules.

We have stayed at 60 ml/hr for Raul's formula with the baby food green beans mixed in. It is a little hard to tell if that is helping, but I think it is. He is still getting a little dehydrated from the amount of stool, so has been very thirsty! His BUN has been much better the past week, and we are hoping to go to only having to do dialysis three days a week. Today is the first time in three months that we have gone two days without dialysis! We have started weaning Raul's CSA, which will take 8-10 weeks, so hopefully that will help his kidneys too!


Two nights ago Raul's blood pressure stayed low after dialysis, and we just assumed they had pulled too much so he got a fluid bolus, which helped minimally. However, his BP never really rebounded, and has stayed pretty low since last night even without dialysis. Last night he got several fluid boluses, and when that didn't help we added back more antibiotics. Today they were concerned he was septic, though thankfully his BP has not dropped any lower, so if he is it is not as severe as last time. He is also still acting like his normal self.

We did hold off on his +100 day skin biopsies, as we did not want to cause his BP to drop any lower. His engraftment studies were sent off, so hopefully we will know about those at the end of this week or early next week. His low BP also contributed to our decision to skip dialysis today, as his BUN was okay and they likely would not have been able to pull much fluid anyway. His BP has stayed low but stable, though they are considering adding a medicine to raise it anyway even though it is not dangerously low at this point.

We are now trying to get Raul to sit at his table for school

Day +96

This morning when I woke up it was -10 degrees (-28 with windchill), and it has only gotten a little better as the day has gone along! Raul got two passes this week to go out of the hospital. Today we bundled him up and went to visit the fire station, where we got a nice tour. Raul talked to the fire fighters, and even asked them about their axe and radio. His legs were hurting him a lot, but he was glad we went! They invited us to come back any time. He is now napping after his busy morning.

We got up to his full amount of formula a few days ago, but then had to back off a little, though not enough that he would have to go back on TPN. We have also added baby food green beans to his formula to help with his stool. This has been our biggest issue lately, but we are hoping it will improve and we won't have to go back to TPN. It has also made him a bit dehydrated, so we haven't been pulling fluid the past few dialysis runs.

We think his dry weight has likely increased again as well. We are now discussing trying peritoneal dialysis for him, as that would be every night and we would not see such extreme fluid shifts and super high BUNs as we do with doing hemodialysis four days a week. In people without kidney failure, our kidneys are working 24/7, not just 12-16 hours per week! If it worked, this could also be done at home, which would be a huge bonus for when we go back to MA! We are looking at doing the catheter for the peritoneal dialysis next week, as he will have to go to the OR anyway for some day +100 tests.

Raul's labs remain pretty much the same. His bilirubin goes up and down, but isn't going quite as high on the spikes. He is still needing platelets and blood pretty regularly, but can go a couple days in between. WBC/ANC have stayed at good levels and he has not needed GCSF since December 27th. His fevers are still happening 1-2 times a day usually, but not always as high as we had been seeing at his worst. He has been acting much more like himself lately, and been talking a lot more, which is great! We are working on trying to get him out of bed and out of the room more often to help build up his strength. 

Day +93

Tonight's Music Therapy

Day +92

Well Raul's bilirubin is back up a bit, so it seems like it may just bounce around for a while before truly going down. Raul got blood the past four days (though one infusion was stopped soon after starting due to a fever), and thankfully his hemoglobin is finally up to a normal level today! He is now getting EPO three times a week during dialysis, so hopefully that will help keep it up. And his Platelet count only dropped from 26 yesterday to 25 today, which might be a post-transplant record for him! Still a couple fevers a day, but not constant like it used to be. He will also be off TPN as of tonight, which is very exciting!

The past couple days he has been in a pretty good mood and been more talkative. He has been sitting up in bed more often, and worked really hard in PT yesterday. He is back to school after winter break, and not thrilled about that, but he liked that his teacher came back up today to visit Pablo from Backyardigans with us! They had a story time with Pablo in the library, and Raul tired out before the stories, but did get to visit with Pablo (unhooked from his pole even!)!

Day +90

Thankfully Raul's bilirubin is heading back down! It was 7.5 yesterday and 6.3 today. We aren't sure why it went back up or why it is now coming down, but I'm just glad it is coming down! Raul continues to just have 1-2 high fevers a day, and they come down easily with tylenol. This afternoon he had a fever that appears to have come down without even getting tylenol, which hasn't happened in ages! He is definitely feeling better without so many high fevers!

Raul's feeds are up to 55 ml/hr tonight, and his ultimate goal is 70 (though 60/65 could be okay). His TPN has been lowered for tonight, and should be stopped entirely tomorrow. Yesterday they lowered the calories but did not lower the fluids, so today his weight was crazy high since he is now getting over 1,000ml of formula a day too. His weight was up to 19.6 kg, and his dry weight has been 18kg. We are thinking his dry weight may have increased again a little, so we just pulled off 1kg today as he is scheduled for dialysis tomorrow too, and we will see how he does at that weight. In the past when he has gone above 18.6 he has had breathing problems, but amazingly had very few issues this time (breathing a bit fast, and needed a small amount of blow by oxygen briefly overnight). He was definitely puffy, though.

Raul was able to go two days without needing platelets, but was borderline today and got a nosebleed, so he will get them tonight. He had been doing well with fewer blood transfusions, but the past few days he has been very low. His hemoglobin was 6 Saturday when he got blood, then was only just over 7 yesterday morning so they tried to give blood but he spiked a fever so they had to stop it. Because he was only able to get a small amount before his fever, he had to get more today as he was back down to 6.3. Hopefully this time it will last longer! 

Day +88

As you can probably tell from the photos, Raul is more jaundiced. His bilirubin was up to 12.5 yesterday. It may be related to the aHUS, but we are hoping it will improve once we get him off the TPN. His formula is now up to 35 ml/hr, so we are making progress thanks to the GJ tube! He is still throwing up even though we are venting the G portion of his tube, but at least he isn't throwing up his formula or meds. anymore. We also started him on a medication to help his motility from his stomach. Thankfully, his diarrhea has only increased a little even with the formula and the motility medication!

This past week he has gotten two blood transfusions, and three platelet transfusions. This is still a lot, but much better than it had been for a while! I believe we are going to start giving him EPO to help cut down on the blood transfusions, as when you have kidney failure the kidneys do not make it (or do not make enough) on their own. Raul hasn't needed GCSF in a week, which is great! His counts came back as we had hoped when we stopped a few of his antivirals, so now we are going to start one back to help keep down his BK virus levels. Raul's weight was up .8kg today, which was his highest weight since starting dialysis, but we were able to pull all the excess weight off during dialysis.

Raul has been pretty happy and talkative the past couple days, particularly in the morning. It is great to see him act more like himself during these times! He also has been having fewer fevers, which has been amazing, as the high fevers really make him feel crummy. He had a true fever Thursday at 4pm, then not until Friday at 4pm, and then today around 2pm. And thankfully when he does have them they are now going down pretty quickly with the IV tylenol.

Day +87/Happy New Year