Quick Update.

Quick Update-

Emerson spent a total of 20 hrs NPO waiting for his turn to go into the OR. It was 8pm by the time we were called down and we were all exhausted & cranky. At 9:20pm the Surgeon came out and said the procedure went well and Emerson was in the recovery room. They had removed his Broviak line and placed a PICC line (peripherally inserted central line). Close to 10pm a nurse walks through the doors. I thought she'd be telling me I could go in and see my baby. Instead she tells me Unit 1 is looking for us.. They had let Emerson wake up without us there and taken him back to his room where no one was waiting for him. I was LIVID. On top of my rage, Emie's poor eye lids got really swollen from fluids and he could hardly open them. It was a late, stressful night for us all and Emerson didn't sleep the best. The next morning went fairly routinely. His eyelids were still extremely swollen but we were told they should go down throughout the day. His stooling had begun improving a bit and they were not giving him as much replacement fluid. At about 2pm Emerson's pumps started beeping "downstream occlusion" which was weird because the flow had been perfect minutes earlier. The nurse came in and couldn't flush or draw back on his line. She thought maybe he'd clotted so she went to ask someone about potentially TPA'ing (clot busting med) his line. JP was asking the nurse if a clot would be sudden because our experiences in the past had not been like this. She said no and left the room. A short while later our nurse and the charge nurse came into our room. The charge nurse said that the sudden occlusion triggered a red flag for her. They went back and looked at what infusions Emerson had been receiving and noticed that our nurse had made a MAJOR error. She had been running two incompatible products through his brand new line and it was now completely blocked with crystallized medication....... I cannot begin to describe the anger, pain, hatred, sadness, and helplessness I feel. I understand that everyone is human and errors happen.. but it is hard to accept when that error happens on your baby. We had just been through the ringer with trying to get this line removed and a new one placed and not even 24 hrs later she ruins it.. I am still at a loss for words and I don't know if I will ever be able to look this woman in the face. They tried putting hydrochloric acid in the line to bust up the blockage and let it sit for 2 hrs. It didn't work.. So we tried once more letting it sit for 4 hrs. In the meantime we had to give Emie his meds he'd missed getting through his IV so at 11pm we had to wake him up to give him oral cyclosporine and his antibiotic. Then at 2 am two nurses and the bedside physician came in and turned the brightest lights on to take out the HCL acid. Again, it was unsuccessful.. My heart is still in a million little pieces. My poor babe doesn't deserve this.. They then came and woke me up at 3 am telling me I wasn't able to feed E past 6am because they wanted to leave him NPO incase he was going back into the OR.

So today started off really, really bad. The Oncologist came into our room before rounds with a bunch of different plans all depending on what the Infectious Disease (ID) Drs had to say.. He told us we may be going in to the OR today to get another Broviak line (tunnelled double lumen central line) put in or he wanted to look further into what was causing Es fevers by sending him to get a CT scan to rule out a fungal infection. Both involved fasting and poking.. two of my least favourite things. So we sat and waited for IDs phone call.

We were called into rounds still very unsure of where the day would take us. GOOD NEWS. Finally! The ID Drs did not think it would be safe to put a new line in until Emie has been afebrile for 48hrs bringing us to Friday. Our Oncologist was okay with this and also said we could potentially get a scheduled time and not be put on the red list. He also said they told him E did not need a CT scan because the likelihood of it being fungal were slim and he didn't need to be exposed to radiation. We also decided Emie didn't need IV access until Friday because his stooling/fluid needs (so far) have gone down and he's able to take all of his meds by mouth. This may change depending on how his pooping goes. So we spent the day not hooked up to anything! They came and removed the PICC line in our room without complications. Today is the first day E has been tubeless in 5 months. Emie also hasn't had a fever all day! Which meant we were able to spend an hour outside. It was absolutely glorious :)

So now we wait and hope Emie doesn't require an IV before Friday. They told us they hope to get him in for his line placement at 2pm. We are praying for no fevers, a quick OR visit, and that we get to go home in the near future.

Thank you to everyone who gave words of encouragement and sent prayers/positive vibes our way. They mean the world to us!

D

Deja vu

We were warned when Emerson was discharged on Aug 25th (Day +18 post transplant) to expect to be back in for a few bumps along the way. That being said, I didn't expect our first bump to be so soon..

                 

                 

                             

Our first days home were amazing. The second we walked through our doors at home Emerson was more himself. His energy level was through the roof and he was so much more verbal/chatty. It was so so good to see him so happy and "healthy". We visited the Clinic a couple days after being home for a checkup and routine blood work. They noticed his cyclosporine (an immune suppressant drug to prevent graft vs host disease) levels hd fallen. Simple fix- we increased his dose. A couple days later we went in to check the levels and again they were dropping even with the increased dose. They increased the dose again. At this point he was taking the same amount as some of their larger teenage patients. It was getting harder and harder to make him take the med. Its not water soluble so they have to mix it in fish oil and the taste is horrendous. Most kids need gravol or another antiemetic before taking the cyclosporine because it is so putrid. We went in to check his levels once more and they had not budged.. Something wasn't right. With this huge dose of cyclosporine we noticed his stool begin to loosen also. The doctors made the decision to admit us back to Unit 1 on Sept 4th just so he could have the cyclosporine IV to get his levels back up. This meant packing up our day bag, all his meds, and enough toys every morning from 8-11am then go home and have to do it again for 8-11pm. It was exhausting. On tuesday morning while we were in getting his infusion Emerson spiked a fever. My heart sank and the lump in my throat was throbbing fiercely. I knew what the fever was from.. The night before Emerson had a stool that covered him from head to toe at 3am. We stripped him down and noticed that his central line caps had also gotten covered in poo. We cleaned them well and replaced the caps. Unfortunately one bacteria particle is all Emerson needs to get infected. The nurse drew blood cultures from both of his ports and they started him on heavy hitter antibiotics. 9 hours after the cultures were sent gram negative and gram positive bacteria had begun growing. We later found out he was positive for 3 separate bacteria.

The next morning Emie woke up okay but by the time JP had gotten to our room E took a hard turn south. He still had a fever and began shaking uncontrollably. He was extremely pale and tachycradic. We paged the doctors in stat. Emerson was having in septic shock. It was absolutely terrifying. The whole thing only lasted a couple of hours and then he fell asleep. The sensitivities (what antibiotic kills the bacteria) came back and we were able to switch Emie from 3 to 1 antibiotic. His fevers went down and he started to perk up as the days went on. He had repeat blood cultures drawn and finally on the third day they came back negative meaning that we were successfully treating the bugs.

                 

Throughout all of this Emerson's stooling increased and became very loose. The doctors started to worry that it was GVHD of his intestines so they made Emie NPO (only able to drink clear fluids).  If he did have GVHD his stooling would not improve with NPO. This caused me MAJOR stress.. My maternal instinct to want to nurture my babe was raging and seeing him screaming from hunger is the absolute worst. The next day they started TPN (nutrition through his central line). It helped keep him a bit more comfortable and less hungry. It was also reassuring knowing he was getting adequate nutrition and we were giving his guts a break. He was on the TPN for a total of 5 days. His stooling DID improve and they finally linked his loose stools to the antibiotic they had started.. This meant that we did not have to send him to the OR for a gastrointestinal scope and biopsy to ultimately rule out GVHD. It was a relief to not have to worry about GVHD on top of everything else and I thought it would be a simple fix to switch the antibiotics. However, there was not another antibiotic that would cover all 3 bacteria well enough and they take bacteriemia very seriously around here. So we were stuck with poop, lots and lots of poop.

                                

                               

Emerson was in a great mood once he started feeling better. He was very active and we spent a lot of time helping him walk around the room and follow him with his IV pole. His stooling was improving even on the antibiotic and we began talking about discharge. We also started him on oral Tacrolimus (a different immune suppressant). Its a very small volume compared to his cyclosporine dose and is water soluble so can be made to taste WAY better. Emerson even asks for more when he's finished! His first levels came back a little low so they increased the dose a very small amount. They repeated the levels a couple days after and they were where they needed to be! He's absorbing!!  Finally we could see the light at the end of the tunnel.

                                 

                                 

2 days before the end of his 14 day course of antibiotics was scheduled to end, Emerson spiked another fever.. It was deja vu all over again. They drew cultures from his line, got the lab in to poke his arm, and started him on an additional antibiotic. We were back at square one.. and I was completely, utterly defeated. I have found myself saying "I can't do this" more times then Id like to admit. But theres no "not being able to" because Emerson needs us and so we just keep on. Even though we feel like we cannot possibly take any more hits, any more pressure, any more pain, we hold our heads up and keep going.. 13 hours later a gram neg bacteria began growing from his line that resembled one from his initial infection meaning his line had infected with bacteria that was getting pushed into his bloodstream every time we uses it. They told us we would be removing his line, he'd need multiple peripheral pokes and to keep an IV in place for multiple days until they were certain his blood was cleared of bacteria and they could put in a new line. It was a lot to take in and I was emotionally unstable. I spent the night feeling very sorry for myself but the next morning had my first positive thought. I was going to ask for an IVAD port to be placed instead of another Broviak line. He may have needed a port placed later on as they are much safer while at home and keep infection rates down. Instead of having his lines hanging out of his chest there will be a little button right under his skin that they can connect a line to, draw off blood, and give infusions. It would be so nice for when we are at home because we would not have daily line care, dressing changes, and he could have a bath without having to tape up his entire chest! The downside to having a port placed so early post transplant is that he is still getting weekly blood draws for another two months. This would mean a peripheral poke every time which is painful. We had discussed it and it sounded like that was the plan moving forward. Until the culture came back the next day as a completely new bacteria.. The plan I had brought myself to terms with was thrown out the window. There was now hope we may be able to salvage the line!

He was started on a new antibiotic and plans were made to ethanol lock his lumens (injecting alcohol into one line at a time and locking it in place for 24hrs) to kill any bacteria that might be growing inside the line. We started with his small blue lumen first. When we tried to get it out the next day the lumen was plugged... we couldn't get anything out. They made the decision to just push the ethanol into Emerson. It wasn't ideal but the amount was so small it shouldn't have an effect on him. They did blood sugar levels every 5 mins for 15 minutes just to be safe. He was okay and they were able to draw back blood. They ethanol locked his other lumen and began infusing through his small blue lumen. About an hour in, both channels on the triple pump and the syringe pump started screaming "downstream occlusion". His line had clotted off again. They took the ethanol out of the yellow lumen and TPA'd (clot busting drug) his blue lumen. The next day they drew a repeat set of blood cultures because Emerson was still febrile but acting like his normal self. The TPA was a success and both his lumens are working as they should now. His blood cultures also came back negative! So the only positive cultures he had was from the day he spiked his fever. The blood they drew from his arm also came back negative even from the initial positive making us believe the line was infected.

It has now been 8 days with an ongoing fever and negative blood cultures. They have no idea what is causing the fever. His stooling also increased AGAIN.. (most likely from the new antibiotics he's on) But of course it happened over the weekend and a new doctor was looking after us and brought up GVHD and wanted to make him NPO again.. JP had to warn me to cool it (I think he could tell I was about to go bananas). She agreed to not make him NPO but wanted to keep a close eye on his stooling. The only way to figure out where this fever is coming from is to remove his line. So we sit here and wait to be called into the OR. Emerson has been NPO since last night and is handling it like a beauty. I anxiously sit here and wait.. They can't give us a time but told us it would be in the early afternoon. I sit and wait to hand my baby over once again to the nurses, RT, and doctors in the OR.

                              

They plan to take out his line and we're hoping they will insert a PIC line (peripheral central line) or at least a longer, large bore IV that can be sutured in place. This will make the next few days easier on us with not having to worry about Emerson ripping out his IV and him needing to get poked a million times. Then once we have a few days of negative cultures we can discuss potentially going back into the OR to get a port placed.

In the meantime through all this we have reached day 52 post transplant. Nothing that had happened should affect his new immune system growing inside him. His 21 day chimerism test showed of the immune cells he has 100% are from the donor ( not just his old immune cells coming back), which is as good as we could hope and our immunologist has told us he's doing as well as could be expected on that front.

Thank you to everyone for the continued support. Please pray for strength for JP and myself to get over this road bump and for a smooth OR trip for Emerson.

We WILL get through this,
D

Grow Cells GROW! (Day -7 to Day +15)

I am still trying to process the fact that we are 15 DAYS POST transplant. What a journey it has been so far..

The days leading up to transplant

With each new day of chemo we could see Emerson slowly start to lose is "umph". He no longer wanted to stand or even sit on his own. I soaked up every second of cuddles I could. As nice as the cuddles were it was so hard to see the life draining out of him. One of the chemo drugs caused Emerson to break out in a nasty rash which we treated with benadryl. He was nauseous from time to time and all of the medications/ongoing norovirus infection have caused him to have persistent loose stool. Even though Emerson felt pretty lousy he was still in good spirits. His smile is the sweetest thing in this entire world. He barely fussed and was content watching the Bee Movie for the 500th time in a row. On our last day of chemo I received the most thoughtful gift from a dear friend. It was exactly what I needed to push through the next few days. It was filled with all of the "essentials" we would require for the next few days- the last day of chemo, our day of rest, and our "New Life" day. It was seriously the sweetest thing and I will cherish the memory forever. (Thank you, K xo)

(Mondayzzzzz)

My boys :)

Serious sweetness

E's last dose of chemo!

Our special gift

Exactly what we needed!

New Life Day!

We had a pretty low key morning. We were all anxiously awaiting any news from the lab about the stem cells (quality/quantity) and when we would start the transfusion. We were told that the quality was extremely good but there were quite a bit of the donors RBCs that they were not able to remove. They do not screen donors for blood type and Emersons donor was not a match to him. This meant that to be safe we would have to give the stem cells in two batches to reduce the total number of RBCs in the unit. Shortly after lunch, our room filled with nurses and equipment needed for the transplant. You could feel the excitement of our family in the air. The first cells entered Emies body at 2:16 pm. It is mind blowing to think that such a small amount of cells can do such a big job, the total quantity infused was only 30 mL of stem cells! They are transfused into Emies blood stream and know exactly where to go and what to do. Those cells will start dividing and growing creating an entirely new immune system. What an extraordinary process. Emerson tolerated the transfusion well and there were no complications. We ended the day with a nice supper to celebrate what an extraordinary day it had been. Emerson now has a second birth day :)  They started the second transfusion early the next morning, again, with no complications.

lazy morning watching flix

All "dressed" up for his big day :)

"No more photos mom"-E

The box carrying Emies new immune system

New life entering his body

<3

The perfect end to our day

The days following

The bad: The side effects from the week of chemo slowly began showing up. Emerson has not left our arms. Some days even lifting his head up has been too much. He developed blisters on his bottom that have been a nightmare. We have a whole pharmacy worth of things we need to put on his bottom to keep it safe from infection until his cells grow enough to heal it. On day +8 he began losing his hair. It has only thinned so far and he still has a pretty good chunk on the top of his head :) He has needed two RBC transfusions and one platelet transfusion which he had an allergic reaction to.

The rash that covered his back, shoulders, and face

Shedding baby hairs everywhere

The good: He has only required tylenol for pain management! He has also not required any additional nutritional supplementation. We were told that 99% of children require some from either an NG tube or TPA (nutrition through his central line). I was so nervous of Emerson potentially needing these because I have no experience with them. So for him to keep eating like a champ has me singing Hallelujah. He also did not get any mouth/throat/intestinal mucositis! After transplant our dr also told us we were allowed on "pass" for hour long walks outside. It had been 19 days that Emie had not left his room. We couldn't wipe the smile off his (or my) face the whole hour! 

That smile melts me

Soo HAPPY!

Looking for les petits oiseaux

On our first walk to the duck pond

Pure bliss

This wittle face!

Over the last few days Emerson has officially "engrafted". This is incredible news! It means the cells have made his body their home and are GROWING! His white blood cells and AGC (total neutrophil  count) have been in the normal range for over three days and they have stopped giving him GCSF ( a drug to stimulate his body to pump out immune cells). As great as this all sounds we are in no way out of the woods. Now we have to be extremely cautious in looking for any signs of acute graft vs host disease (GVHD). This can show up in his skin (rash), liver (bilirubin levels), and intestine (persistent diarrhea). These can show up any time in the next 100 days. Emerson is on a medication called cyclosporin which is an immune suppressant and helps prevent GVHD. It is not a very pleasant drug and makes E feel pretty lousy the entire time its infusing (2 hrs twice a day). We have been trying to get it to a "therapeutic level" in his body. This means they give an amount and test to see if the level is where they need it to be with blood tests. If its too low they increase the dose and vice versa. They overshot the amount they needed to increase Es dose a couple days ago resulting in his body having too high of a level. He has not tolerated this well at all. He has broken out into large red blotches that fade and appear in a new spots. He's been fussy and lethargic. There is also nothing we can do but make him comfortable until his body metabolizes the drug down to where it needs to be. Aside from that, Emerson is doing extremely well. They are switching most of his medications over to oral doses we can go home on. The last of these switched will hopefully be Monday with the cyclosporin as long as we can get the levels where we need them. If all goes to plan we should be HOME mid next week!!!! PLEASE GOD let us be home next week!! Once home we will be under the same isolation as pre-admission and we will need to make multiple trips a week back up to the hospital for clinic visits but at least we will be home.

REUNITED and it feels sooo good! - Our puppies were able to come home for a short bit while Emie was admitted to the hospital. We walked them up to the duck pond so Emie could see them. He was SO excited :)

With each new day we are one step closer. Closer to discharge, closer to having our fur babies home for good, closer to being able to get aunty/uncle hugs, closer to playing with friends, closer to having a working immune system.

D

Day -9 to Day -6

The time finally came to say goodbye to our friends on Unit 2. We had our last day with them on Tuesday. We had to go in for his routine check as well as dressing change, blood work, IVIG, and we ended up TPAing his lines again. It was a very long day..

Wednesday Day -9

The day we have been waiting for, praying for, stressing over, and dreading..

We didnt have to show up to Unit 1 until 8pm so we made the most of our last day of freedom. In the morning we took Emerson for a quick hike in the Paskapoo Slopes near our home. We came home for lunch and a nap. Then we took Emerson to the park to enjoy the slides and tire swing. We loaded our bags into the car and made the trip to our new "home". We were greeted on Unit 1 by his nurse who did a quick assessment. Then we were left on our own for a while to unpack and get E settled in. He made his way to the door and won over the hearts of all the nurses on the Unit with his contagious smile. Such a little heartbreaker already. Throughout the night they gave him another dose of IV antibiotics and an antiemetic drug to prepare him for the next day of chemo. Emerson slept awful the first night. I think he was unsure of his surroundings and kept thinking he had visitors coming in to play.

Thursday Day -8

Emerson woke up at his usual time smiling and ready to start the day. He was very good natured and active. It is extremely hard to keep him occupied and happy in our room. He was given his first dose of chemotherapy around 2pm. The drug that he received was a subcutaneous injection (much like a vaccine poke). He's now also on a new anti fungal through his central line, a drug to prevent liver damage, and an antiemetic drug. He tolerated the first dose of chemo well. He went down for a nap shortly after and woke up in a decent mood. We played for a bit and then had supper. He was extremely hard to get down to sleep at bedtime and partied until 10pm. I didn't realize how hard it was going to be to see him get the chemo for the first time. The nurse came in wearing a crinkly blue gown to protect herself and told us from now on we have to protect ourselves too when changing his diaper. It blows my mind that we can be injecting something so toxic and vile into my poor little baby.. Throughout the day I noticed many other nurses in the blue gowns going into other rooms and my heart weighed heavy. The thought that every little babe in this unit is as sick or sicker then my little guy is unbearable. I am overwhelmed with sadness thinking about all the other families going through this. I had a talk with another momma who's little one also has SCID and has already undergone chemo. She told me I have to stop thinking of it it in such a negative way. She said, its not "poison" its a "life saving regime". It helped a lot to hear that and Im trying to think of it more positively, but its really really hard.

Emerson has also developed horrible diarrhea. Stool cultures have been sent to rule out a new source of infection. The most likely cause is one of his new medications (3 of them can cause diarrhea). We've been going through 8 sleepers throughout the day.. poor little buddy.

Friday Day -7

Emerson was his cheerful self when he woke up. He was pretty happy throughout the day but we've noticed he's started to be fairly moody from some of the medications he's on. He goes from super happy to full out throw himself on the ground tantrum in the blink of an eye. He had his second dose of the same chemo drug around the same time. He napped after and woke up in a bad mood. I could tell something was wrong but couldn't find what he wanted. He started to wail as if he was in pain. I was frantically trying to find out what was wrong and asking the nurse if there was anything we could give him. While she was seeing if he could have another dose of tylenol he finally gave in and started taking his bottle.. I still don't know if he was just hungry (Id tried to give him his bottle prior to him finally taking it) or if he was in pain and decided to sooth himself with his bottle.. I broke down. It was the absolute worse feeling thinking he was in pain (that we caused) and we couldn't do anything about it. I cannot wait for this to be over.. the sad part is we haven't even started the "hard" drugs.

We got Emerson settled and he seemed to be in better spirits after his bottle. He was still extremely moody but at least he wasn't crying. As I was sneaking out of the unit the code bell rang. Swarms of staff flooded into the room and I could hear them shouting for the emergency equipment.. Again I broke.. That poor family.. thats poor baby. No one should have to go through this.

Emerson received his first dose of Ativan (to prevent seizures that another medication we're giving him can cause) at 8pm. In 99% of kids it relaxes them and makes them sleepy. Well, not for Emerson. He was wired- bouncing off the wall wired. He stayed up until almost 11pm. I hope it was a one time thing!

Saturday Day -6

Emerson woke up in a great mood and had a bit of breakfast with JP. They began giving him more fluids and have talked to us about maybe starting a stool replacement because he's been having so much diarrhea. Today the plan is to put in his foley catheter (protects his bum from the nasty chemo drugs) and to start the worst of the chemo drugs later in the evening. I hope he tolerates it well and if not I hope we can better manage his pain.

Please pray for my little guy as he goes through this great battle. Thank you to everyone for the words of encouragement, they are greatly appreciated.

xo, D