Can't catch a break.

We were able to leave on a pass yesterday! Whoop! We got home shortly after 5 pm and began unpacking/wiping everything down we brought back. Emerson couldn't wipe the smile off his face. He wanted to be toured around the entire house and made sure we took him into each room. It felt good to be home. We had a good sleep all in our own beds and woke up today in a good mood. 

(All smiles before finding out his lines were blocked)

This morning we had to make a trip back to the hospital to draw a CBC off his Broviak line. We were also getting to test flushing his lines on our own so we can do it at home. The nurse came and tried to draw off both ports unsuccessfully .. They both flushed with a little resistance but we weren't able to draw off anything, not even the saline we had just flushed in. This is making them suspect that he has formed a fibrin flap over the ports that opens when fluid is pushed through but gets sucked against the opening when you try to draw off. We are going to administer the TPA into both ports today and let it sit for 24hrs. We were able to talk them out of calling lab down to poke him for the CBC and wait to try again tomorrow. I'm feeling a bit disheartened because I thought the Broviak line was going to solve all of our access issues and save him the agony of trying to get blood by poking him each time.. I don't understand why nothing can just go easily for us.. I just want one thing to go right..

(E- "I wanna go home") 

(Sick of these lunch dates)

I'm praying we find a donor match quickly because the wait so far has been extremely draining and we are running on fumes.. I just want this to be over and it scares me to know that we haven't even begun the hardest part. 

Needing positive thoughts sent our way that his lines are patent tomorrow and they can draw the blood work off of them.. My poor little babe just wants to be home where people aren't poking and prodding him 24/7.. 

D 

In&Out .. not the burger joint

When we were discharged from our initial stay I never would have imagined the rollercoaster of events that were to unfold..

Our First clinic day on Tuesday after the long weekend went well. His vitals were all normal and they were able to collect some (not all) of the blood they needed for his weekly check. We went home,  Emerson ate his first supper outside and tried his first corn on the cob :) The next day Emerson spiked a low grade fever of 37.9. We called the nurse on call and she told us to come in to get him assessed. They did a full assessment and there were no signs of infection anywhere besides his 4 big molars trying to cut through. They ruled out an infection and sent us home with permission to give him Tylenol as long as we check his temp prior to dosing him. On Saturday we noticed his thrush had come back with a vengeance. His poor little mouth was red&inflamed and his molar cavities were covered in thrush. Again we called the nurse on call and they had to try and work out a way to get Emerson into the hospital without having to go through and wait in ER. Luckily for us it was a gorgeous day and the emergency room was eerily empty. They took us straight through to an isolation room where we waited for the Hematologist to come and assess Emerson. He also could not find any other source of infection, his lungs were clear, ears sounded good, and had not been having any GI symptoms. He increased Emersons thrush medication back to the treatment dose and we were able to go home and monitor him closely for any fever or signs of infection. He was pretty fussy all weekend and wanted to be held most of the time. We thought it was just from him teething and the thrush.

On Monday we had our second routine clinic day. The blood draw was a bit of a challenge and they were not able to get enough blood for all the testing they wanted. They were happy waiting until our next clinic day to try again for the additional tests they wanted to run. We went home but were called a couple hours later by the nurse. Emersons hemoglobin level had come back as 54 (normal value is around 120). We rushed him in and the nurse assessed to see if there were any sites she could get an IV/draw a repeat blood test. She couldn't see anything. At this point Emerson looks like a pin cushion. He has bruises and poke marks covering his arms/hands/legs/feet. She had to page the  anesthetist to come with the ultrasound machine to try and find a site.. Even with that it was difficult to get a line but they were able to draw off enough blood to send down and get an IV. We were transferred up to our old room in Unit 2 where we waited for the test results to come back. His hemoglobin and dropped even further to 47. They discussed with us that Emerson had autoimmune hemolytic anemia. Emerson has no T-cells to tell his B-cells what to do. So in this case his B-cells have created an antibody against his own red blood cells. His body was destroying his cells faster then it could make them. The treatment for this is prednisone a steroid which will suppress his B-cells stopping the hemolysis. They started him on an extremely high dose through his IV. In this time they were also calling down to the blood bank to find matched blood to give him. His vital levels had been stable but with babies they are very good at maintaining "normal" until their little bodies give out and tank. The blood search was not going very good. Lab had to come and try and poke Emerson 3 more times (with multi pokes) to try and draw more blood for testing to get a proper blood match that had the same antibodies on the RBCs so his body wouldn't reject them. They were finally able to get a very small amount that they hoped would be enough for the testing. We sat waiting nervously watching his monitor for any change. They had been doing heel pokes to test his hemoglobin levels and the most recent one to come back was 43.. At this point his HR was elevated to 170 and his oxygen levels had dipped below 85 (normal is 100) twice. I was terrified, I had a disgusting feeling that at any moment he was going to tank. They drew another cap gas and it came back at 38.. The paediatrician came in and said we had to give Emerson unmatched blood because we couldn't wait any longer for a match. They consulted the PICU and had decided he would be safe to receive the transfusion on Unit 2 but had their team come up to closely monitor him through it. They drew up all the resuscitation drugs which normally are just in the room incase and hooked up the bagging unit incase he stopped breathing. I have never been so scared in my life.. They began the transfusion very slowly with no reaction. They gave the unit over 4 hours and Emerson had no signs of rejection (THANK GOD). They did another heel poke post transfusion and his hemoglobin had come up to 119. We got a couple hours of sleep and waited for a further plan.

The next morning just before lunch I had a look at his IV site. It was hard and didn't look right so I paged the nurse. The IV had fallen out.. The decision was made to put in his Broviak line early (a tunnelled central line catheter). There are risks associated with putting in the line early like an increased risk of infection and it clotting off but the benefits of not having to poke him a million times and having a secure way to administer medications/blood products outweighed the risks. Emerson was taken into the OR at 7pm and we were back in our room by 9pm. The line insertion went well. They did have to give him another unit of blood because his hemoglobin was back down to 67. Once back in our room on Unit 2 they administered his IV Ig and prednisone through his new line and also drew off more blood. His hemoglobin was back up to 130. While she was drawing off the blood she flushed both lines. The smaller of the two ports was not flushing well so they decided to administer TPA (an anti-clot drug) and let it sit in the catheter for 24 hrs. We checked the line again today and it is still not flushing so they are trying TPA once more. They told us that this sometimes happens in babies because their vessels are so small one of the ports might be resting against his vessel wall. This might cause us problems when it comes to transplant time because they need two functioning ports so we may need to think about another line being placed.. but we will cross that bridge when we get to it.

Now we are waiting for his prednisone dose to be decreased enough to go to an oral dose that we can go home on. They think that might be tomorrow or Saturday. We are also being educated on proper care/cleaning of his central line tomorrow. They mentioned that we may not be able to be "discharged" but will be able to go home on a pass because there is no procedure in place for admitting us without having to go through ER yet if the units are full.

Thank you again to everyone for the ongoing support. Please continue to share and follow in our journey and keep Emerson in your prayers.

D, xo

The lows are low, but the highs sure are high

On Wednesday Emerson had his bronchoscopy and bone marrow biopsy scheduled. He was put on the red list because his procedure was not planned meaning that there were a bunch of other babes who had been waiting months to get their own. They gave us hope that he would be done earlier in the morning because of his age and illness. Our Immunologist also needed it done before 14:00 because of her bone marrow teams working hours. They told us his last bottle could be at 2:00 the night before so we woke him up and fed him. He woke up at 6 am screaming wanting a bottle so I knew it was going to be a very long morning.. We distracted him the best we could but by 8 am I had had enough. I wanted an answer to when we were going to be scheduled into the OR and no one had an answer for me. I made them call down to the OR to see if he had been scheduled. He had not been and right now the earliest they'd get him in was 15:45 unless something opened up earlier. They were going to starve my baby "just incase" a room opened up earlier. I was shaking I was so angry. It is the hardest thing in the world to watch your baby cry in pain when you know you could end his suffering easily by giving him a bottle. My entire body hurt hearing him cry. All I wanted was an answer to a time so we could schedule to feed him 6 hrs prior to our OR time.. No one seemed to care and they told me it happens to babies all the time and I should suck it up. I might have said some words to the staff Im not proud of but I don't regret saying them at all. I think the entire policy&procedure that took place was absolutely ridiculous and flawed. We wrote up a complaint so hopefully no other families have to go through the day we went through.

Finally at 14:00 they told us we would be going to the OR within the next hour (E had been without food for 12 hours). We got his stroller/cover ready to walk him down, met with the anesthetic team/RT/surgeons and did our time out. I was able to walk with Emie into the OR suite and comfort him while we fell asleep with the sleeping gas. The two procedures were to take about an hour if everything went smoothly. We sat and waited watching the board to see if his name had changed colors to "case closing". At the two hour mark our Immunologist came in and told us they had worked on the IV for the first hour of the case... He ended up getting a central line (a big long IV close to his heart) put in. She said her procedure went very well and they were just starting the bronchoalveolar lavage (they flush a bunch of saline into his lungs and suck it up to see what bacteria are growing). Another hour went by and the Respirologist came out. The bronch also went well and they were happy with the fluid return. He was doing well in the recovery room and would be able to go back to our room on Unit 2 instead of the Pediatric Intensive Care Unit. We were able to go back and see him. The nurse was holding him and he was pissed (hangry).. We transferred him back to our room but were told we couldn't feed him until 1900 because they had to use freezing on his vocal cords.. Thankfully he fell asleep for a bit and woke up just before 1900. Just like that the day from hell was over and ...

It was DISCHARGE  day :) !!

Emie woke up smiling and ready to start the day. We began organizing and packing our belongings up. The resident came and removed the central line and E was free! We were just waiting on Immunology & the Dietitian to come talk with us. The Dietitian gave us a booklet of dos and don'ts for Emerson's new diet which is similar to what not to eat while pregnant but to the extreme. No berries/ fruit you cannot peel, no probiotic dairy products, no beef jerky/smoked meats, no baked goods made with over ripe fruit such as banana bread :(, and the list goes on. We waited a couple hours for immunology before walking ourselves down to the clinic to get things going. They gave us our emergency contact numbers incase E spikes a fever while at home and the information about our weekly clinic visits. We were now home free! We loaded up E in his "bubble" and made our escape. I have never seen him so excited to be at home. I don't think he has wiped the smile off his face since Thursday. We have been enjoying having some "normal" days without vital signs and IV lines :)

Now we wait to hear about Emersons blood results to get in and for him to be added to the donor pool. Pray that he finds a match quickly and that we are not re-admitted due to fever.

I will update on Tuesday with how our first clinic day goes.

D

My 1st Mothers Day

Never in a million years did I think I'd be spending my 1st Mothers Day in a hospital room. I've been struggling to stay positive today.. and I feel really guilty about it. My husband let me sleep in and Emerson woke up smiling. I was also gifted a photo that the hospital staff made for Emerson to give me. So I should have no reason to be having a little pitty party for myself. We have also received an inconceivable amount of love and support from friends/family over the past couple days. So much so that it has brought JP and myself to tears numerous times. I don't know what we did to deserve so many amazing people in our lives.

Even with all this love/support my heart is heavy.. I'm sad/mad that I am stuck here in this hospital room (day 9 and counting..), that this is how I am spending my first mothers day, that my baby is sick and will not get better for awhile, that everything I was excited about (My moms wedding, Emie's 1st birthday party, my return back to work..) has been cancelled and the only thing I have to look forward to is finding a BMT donor quickly so we can start the long scary road to recovery. Im still struggling with questions like, "Why me and my family?". My little boy (or any little babe) does not deserve any of this.. I know these thoughts and feelings do me no good and that I need to stay positive and strong to carry my little boy through this but it is so hard.. This burden feels so heavy and the road to health feels too long.. Ive started feeling down less frequently then when E was first diagnosed. I have more good days and happy times then sad and down but every once in awhile, when Im sitting alone, the sadness begins to seep in and consume my thoughts. I know this journey is not going to be easy and having so many people supporting us through will make us strong. So, thank you again to everyone for their kind words. They are what has kept us going these past few days.

Update on Emerson- We are still on cruise control until E's bronchoscopy and bone marrow sample on Wednesday. Yesterday Emerson lost his 3rd IV. Because he's been such a difficult poke they've put in orders to finish the last couple of days of antibiotics orally. Our last dose will be tomorrow morning (YAY!). Not having the IV in has been great. E's able to move around a bit again (as long as he doesn't touch the floor). He pulled himself up to standing a few times yesterday which is something we haven't seen in weeks. The IV was also giving E a bit of fluid so now the nurses are on us to make sure he's getting enough orally. Es appetite has improved since being in and feeling better but some days are still a struggle to get him to eat/drink. The nurse weighed him this morning and he was down a bit of weight again. I was surprised because he's been eating way more then he had pre hospital admission. We will have to keep a close eye on it and hope that its just due to the antibiotics and stress of being in a place away from home. E's nose is still pretty snotty. The nasal swab came back positive for 2 common cold viruses. There is no treatment to these so he will likely be battling a cold/runny nose up until his transplant. Now we wait patiently for the next couple days to go by as quickly as possible so we can GO HOME!!

No news is good news :)

Happy Mothers Day to every momma out there <3 I hope your day is filled with family & love. Give your babes an extra squeeze for me today

D, xo

The Beginning

Our world was turned upside down Friday May 1st.

How it began.. Emerson and I had caught a nasty virus at the start of April. Mine lasted 2weeks but his never went away. He was later diagnosed with pneumonia and put on antibiotics. After a repeat cxr his pneumonia had improved but not resolved so our family physician suggested we take him to children's hospital to get looked at. Thursday afternoon we were looked at in ER. We had blood work drawn that showed Emersons neutrophil level to be zero which was worrisome. They changed our antibiotic and sent us home adding that if he spiked a fever to bring him in ASAP. Overnight he spiked a fever so we brought him in the next morning. We were rushed through ER and put in an isolation room where we waited. I thought our worst case scenario was we would be admitted for IV antibiotics and fluid...

When the hematologist walked in the room with an entourage of residents I knew something was wrong. She began to explain that they had overlooked Emersons lymphocyte level on the previous blood work. It had also come back extremely low. They drew a million more tests and swabbed every part of him. We waited what felt like an eternity. The hematologist came back and sat down.. She told us they were extremely suspicious that Emerson had a rare genetic disorder called Severe Combined Immunodeficiency (SCID). She began explaining what it was and the treatment involved. All I heard was "wahhhwah wahh".. My head was spinning and I felt like vomiting.. I didn't believe her. My baby was healthy, he's been in the 99th percentile for height and weight since he was born. He's the happiest baby I know. There was no way he could have something so wrong with him. 

We were admitted to Unit 2 and started IV antibiotics. There we waited.. The tests they drew in ER were what we needed for the official diagnosis and they wouldn't be back for a few days. We were stuck waiting.. Praying and hoping.. I had convinced myself that they were wrong, he didn't fit the SCID picture with failure to thrive.. So when the immunologist met with us Monday I was not prepared for what she had to say. She  began showing us numbers all leading to the diagnosis. She told us Emerson had SCID and began going into detail about his treatment plan.. I felt like I was dreaming, I had an odd feeling like I was floating high above looking down on us. These things only happen in the movies.. I remember thanking the lord for our healthy baby boy when he was born.. 

We have a very long and scary road ahead of us. For now we are to stay at the hospital until he is done his 10day course of antibiotics (Sunday) and his bronchoscopy (tues/wed). As long as he's "healthy" we are allowed to go home. Our family has been working around the clock to make home as safe as possible for Emerson. Our puppies have taken a year long vacation back to SK, all our house plants have found new homes, everything has been professionally cleaned, & HEPA filters have been installed (thanks Aunty&Uncle xo). At home we will be put on isolation so only grandmas will be allowed in, no other visitors. Once home we wait for the genetic testing to come back and are put in the pool to find a bone marrow transplant donor (1-3 months). They tell us our chances of a match are good but are not promising anything. If you would like to help out us/another family in need you can sign up to become a bone marrow donor at www.blood.ca under stem cell donor. It is as easy as a cheek swab. Once a match is found we will be admitted to start chemotherapy and then the transplant. We will be in the hospital for 1-3 months while Emerson recovers from the chemo and gets healthy enough to go home. At home we will stay on the isolation precautions for 6-9 months or until his immune system begins making Cells. 

We have a long road ahead of us and the worst is yet to come.. We really appreciate all the kind words of support from friends/family. We are truly lucky to have you all in our lives. Please continue to pray for our little boy that he stays healthy up till his transplant. 

D, xo