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I'm fighting severe exhaustion tonight as Emily went to bed late last night, for me to wake her up early today to have a bath and breakfast  

and so that Emily would fall asleep in her MIBG spect scan. I'm also sorry if I haven't updated as much, but internet service is rather patchy hear at the Ronald McDonald House and we forgot our Wifi service.

Yesterday we went to the hospital, Helen DeVos for the first time. Such a beautiful and new hospital and lots of wonderful people. Emily's nurse Julie, is awesome, very attentive and great with Emily. Emily had her port accessed for the 2nd time ever since having it in order to get her labs and MIBG Injection.  It was a bit more traumatic than it was the very first time, but we were expecting it to be but she really flipped out. However once it was in she said it wasn't as bad as she thought but she still didn't want it to be messed with, but she did get an prize. We were also explained that she would need to be either accessed again the next day (today) or just leave it in, and Emily was adamant that it be taken out understanding that she would need to be accessed again today! But she would need to have an IV again for the scan today because of the CT contrast that would be needed. 

I didn't sleep well last night for many reason, but of course one of them being part of this and what Emily is going through and what options would and won't be available to Emily...This morning after Emily and I ate breakfast I received a phone call from my mom of news that I didn't want to hear. It was rather heart breaking and one that I didn't expect. She said she wanted me to hear it from her first and not someone else...my father passed away overnight and was found this morning... while my father is not one that I have been close to for many many years and we haven't talked in probably the last 7 years, it was still rather heartbreaking to know that he passed and that he never met my husband, that he never met Emily and Brianna and only met Jessie whens he was very young was hard.  I had hoped one day that he would get his act together and be a different person.. Although we were not close and biologically he is my father, and I considered my stepfather to be more of a father to me, it was still really hard to swallow, but yet at the same time I knew that I did try to make things work with him a while ago.   Anyway, I am not saying all of this to say I want anyone to say "I'm sorry" it is more because I want people to realize that anyone very close to you or family could be taken away from you tomorrow and you are only left with what you last knew of them, but love all those that are close to you. Anyway, this really didn't start the day off that great.

Emily took her 3rd accessing pretty well today, she did cry, but she did better with it.  The tape over the needle is hard, because some of it hits where they had to cut into her skin to place the port and she screamed with that, but overall we were so proud of how well she did today. Letting Emily go to bed late last night and getting up early, definitely helped with the scan today at 1pm. Within about 10 minutes of the scan Emily had fallen asleep, she can't move at all for this one since the CT scan is fused into the MIBG so she has to be in the same exact position.  But the falling asleep really helped her for the 90 minute complete scan.  The ladies in radiology were very easy to work with and were great with us.  I was so proud of her for doing so well with the scan, especially when prior she cried not wanting to have a scan.

After the scan we went to meet on the oncology floor with Dr. Sholler. It was awesome that we didn't have to wait long after the scan. Helen DeVos talks very highly of the spect MIBg and once Sholler came in and showed us the scans, we can completely understand why everyone speaks so highly of them.  So again in basic terms, Emily scans are stable...the spot in her skull, L4, illiac wings, femurs were still showing. They did see a spot on her spine in the T9, however they are not calling it new because with a regular MIBG scan it would be very hard to call because of everything around it that would show on the MIBG would not be MIBG Avid.  The 360 CT/MIBG scan was rather amazing because it showed everything and showed whether the spots that Emily has are either in the cortical bone or soft tissue, and they all showed cortical. There was an additional spot in her skull but she didn't call it a spot, she said that Emily has some sinusitis going on which showed that spot.  So we have 7 spots that Emily has instead of 6 (that constantly changes) that we need to worry about and that we want to one day get rid of.

So, the options and what was available from Dr. Sholler.  Sholler is amazing and she was great with Emily and Emily took to her very well! She is very gentle, personable and easy going with Emily, and us which made things go a lot easier.  The hospital has a much slower pace than what we have been used to, so it made the experience much easier on us and not so stressful. Dr. Sholler knows her stuff and could answer questions with ease and we felt comfortable with her.  She didn't rush us and try to get us to hurry or make a decision with what she had thrown at us, she sat there and answered all of our questions and walked through everything step by step and even writing it out. Emily does qualify for 3 of Sholler's trials. TPI-287 with Irinotecan and Temozolomide, Nifromax with Cyclophosamide and topetecan and then DFMO with Etoposide.   We spoke to her and she stated that she felt that Emily would be eligible for CH 14.18 Antibody Therapy should we be able to clear more of her disease, but that she felt that she was eligible for the HU 14.18 for relapsed/refractory disease and wasn't sure why we were told she was not.  So, they were looking to see if we wanted to go that route could that get that opened for us there. She walked through everything with us and then said she didn't want us to make a decision there, but that how about we come back in the morning after we have had some time to sit on it and and we can discuss it further after we have had some time to think about it. So we will meet with Sholler at 9AM tomorrow to talk about things and answer any other unanswered questions.

So with scans 2 weeks ago and we are still sitting at stable, Dr, Sholler still feels that shooting for NED and looking for a NED Party is possible. She again used the word that we have HOPE.  We are no where near close to this journey being over and traveling just appears to be a bit more than we had really thought, however like she said if we don't do anything because she still has disease in her bones, that yes it could still spread and we don't want that.

Doug and I have had some time to talk about things, look over the paperwork that Sholler has given us and once we make a decision on what we would like to do after making the decision with her we will update. 

We will be flying home Saturday through the Wings of Mercy again.  We have to get the rental car back by 5 pm tomorrow night and the Ronald McDonald House said they would bring us back to the RMH.  However our flight to head home we need to meet the pilot at Holland, MI instead of Northern Air where we came in, so we are waiting to hear if the RMH can take us there, because if not we are going to have to see if we have any other options.  But we do need to get home and get Brianna home and also I will have a funeral to attend.  We are ready to be home, its been a long and exhausting week.  We are overwhelmed with decisions and we miss the kids.   The Ronald McDonald House too is very strict here, so if we have to head back here at all, we will look to stay at the Renucci House instead of here.

Remember to hold all those that you love close and near and dear to your hearts and always tell your kids how much you love them and hold them every night.

We are in Grand Rapids, Michigan

Long overdue journal entry...here it comes...I know when I start receiving emails, text messages and facebook messages asking how Emily is doing that it has been a while since I updated, and I apologize.

Since we got home from Philly, Emily has done well, The port finally started to feel better and where they took the broviac out wasn't feeling as sore. When we got home we started working Helen DeVos to get an appointment set up. We didn't want Emily not being on treatment for too long so we wanted things to move quickly. Thankfully, Dr. Sholler was able to get us in March 7th for CT scan fused with MIBG on March 8th. They also wanted to do a bone marrow biopsy but because Emily had already had one at CHOP on the 23rd and no treatment after that, they were able to use those results, which were negative. Thursday Emily will have her MIBG scan and then afterwards we will meet with Dr Sholler to talk about the scan and talk about the trials that they have to offer.

Thursday Emily had a clinic visit at home and we were able to talk with Dr. Gowda for a little while. This was also Emily's first time having her port accessed. They used Emla cream and overall she did fine with the port being accessed, I think it was just the fear and anxiety of it all. I have to be honest it is so weird not having to flush her lines daily which I have done for the last 14 months and doing weekly dressing changes, which I have also done for the last 14 months. Dr.Gowda also didn't understand why CHOP didn't have any other trials that Emily would qualify for and didn't understand why Emily didn't qualify for the HU 14.18 Antibodies. Antibodies/Immunotherapy is supposed give children an additional 30% EFS (Event Free Survival Rate) and this is what we want for Emily. Dr. Gowda looked over the trial and couldn't see why she didn't qualify for it, so he was going to check with CHOP and see what he could find out. He did print out stats on the temolozide and irenotecan chemo that they wanted to put Emily on and really the numbers were not all that great, however if it works, then it works. Dr. Gowda indicated to us that he worried about putting Emily on any type of Phase 1 trials right now when she has a great quality of life and no problems with anything except for a tad bit high on her thyroid levels and we agreed with him. He also indicated to us that after we visited with Dr. Sholler that we really needed to make a decision on what to put Emily on because he didn't want her off treatment too long and she progress. This too we agreed with. So, although we are making the trip to MI, it doesn't mean that we will go with any of the treatment that they have, but we wanted to have options if they are better for Emily than just the chemo.

We were able to schedule a flight through Wings of Mercy and everyone through the organization were/is amazing. We wanted to leave Tuesday, March 6th before we needed to be here, but the closest that she had was Sunday March 4th, so we worked with what they had. So, we had to make sure Brianna could stay with my parents and Jessie went to her dads a day early. We were told that Helen DeVos does not allow siblings into their clinic because of risk of infections, which we think this is great most clinics should do this.

Emily on the plane

Thanks Mom and Brian for taking Brianna and taking the van from the airport. Wings of Mercy is an amazing organization and the pilots were great. Very friendly. This was Emily and mine first time on a small plane, and we both did fine, no problems. I was a little worried about my anxiety with the flight, however overall it wasn't bad, there was moments and mostly I was trying not to be too nervous and let Emily see that. It took about 3 hours to get to MI, however if we were to drive it is about 775 so approx 13 hours away. We got into the FBO airport and someone took us over to the main terminal to pick up our rental car and then head to the Ronald McDonald House. This would be our first time staying at a Ronald McDonald House and they were able to confirm that we should have a room with no problem (nothing like Philly) but we also had a room at the Renucci House if there wasn't anything at the RMH.

The plane that we flew on to head to MI

Emily enjoying time with Sue and JoJo in the pool!

Emily's bestest friend Sue and JoJo made the trip (6 hour trip) to come visit Emily and Doug and I and they got a hotel and made sure the hotel had a pool. Emily was so excited to be able to come to Michigan and meet up with Sue, she has missed her and then get to get in the pool. Thank you Sue and JoJo, Emily absolutely enjoyed the day and being in the water.

We did get a call today from Helen DeVos that they had scheduled Emily for an EKG on Wednesday as well, because if we decided to go with one of their trials that some of the trials would require an EKG, so we were ok with that. We are anxious to hear what they have a available here, and everyone from facebook when we noted about being in MI has been so friendly and kind. Thank you all so much for your kindness and welcoming us to MI. I think at this point we are not nervous about anything, we are just anxious to get things into the next mode and know what we are doing.

Overall, Emily is doing great. Her hair is coming in quickly and she is so excited about that. She said she can't wait until I can put pigtails in her hair. She is enjoying going to school once a week for 3 hours (next week they will offer it twice a week) and she is so happy to be around other kids. Emily is so excited to learn and be around other kids. She is starting to gain weight and she is almost at her weight of 30 pounds where she was when she begin this whole journey 14 months ago, so it is so nice to start to see meat on her bone and not see just the bones. She is thriving and we are really happy about that, and so ready to be past this journey.

Thank you to everyone who has been so helpful and continues to be. The meals that have been coming from Fairy Godmother Project, have been amazing and ever so helpful. The very friendly people and the fundraisers that have been started in my mom's area and even by some local friends are so wonderful. We are very thankful to everyone who has been so kind. The gift cards continue to be of so much help and we are so thankful to all of the wonderful people who are just so wonderful to our family and send some and continue to do so. We can not tell you how much help they are! My cousin Mandy sent me a message and said someone wanted to send Emily a special present that she will pick up when we get home and we can't wait to see the special present for her. We will be sure to take a picture and share. Thank you Tricia and Lynn for making the Team Emily bags and when we get home we can start making some bags and we will be able to take them up to the hospital. We are still collecting coupons to make bags for families and will continue to do so until we can get a huge stash of items, but we are so excited to be giving back to newly diagnosed families. Thank you Sue and JoJo for always sending huge amounts of coupons and for bringing a huge stash of things for the bags. Giving back is excited and makes us feel good for all the amazing things that people do for us. I am sorry if you have emailed or sent messages and I have responded, life has been busy, but I will try to get back to you all soon. Thank you again for all you have done and continue to do, it means so much and we constantly appreciate all that you do.

Well, I am going to call it a night. We are getting up early to meet with Sue and JoJo for breakfast before they head back home. Remember to love and kiss your kids every night and tell them how much you love them. Love you Jessie and Brianna! Good Night.

Broviac is Out / Port is In / Chemo Is on Hold

This morning I was awake early and couldnt go back to sleep and Doug was awake as well, and then fell asleep. Doug and I talked yesterday after leaving the hospital and the more we sat on it the more we felt like there is more treatment options out there and sitting with no trial but just to try these drugs on Emily and move forward just wasn't what we wanted to see for Emily. So this morning at 545 am I emailed Dr Sholler and explained the results from the MIBG scan we received and what Dr Mosse said. Dr Sholler replied by 853 am when we were bringing Emily to surgery. She explained that the two chemos were a good suggestion although they do have quite a few trials out there that Emily would qualify for. Here comes that word, Sholler has "HOPE" for us. She further explained that they would be happy to see her and see what option would be best for Emily.

Surgery went well today, Doug and I were both nervous about the day. We didn't tell Emily about the upcoming surgery, because her anxiety gets to her and she gets really upset. At 4 years old she understands a lot, but there are just somethings that at 4 years old it is best not to tell her. We were glad that Dr. Mattei was well enough from his previous surgery that he was able to do the broviac removal and place the port in. This port opens more options for Emily as in "quality of life" and more of what she can do, and this is what we feel is best for Emily. After the removal and insert, she would have a bone marrow biospy/aspirate complete to check for disease in her marrow.

Emily did well, she is in pain and her chest is hurting, but she is happy that she can now go swimming. She looked down at her chest and said "mommy my line is gone." I said, "Wow that is great, you can go swimming now!" She was so excited that she said "Yes, and she couldn't wait to tell Daddy that she has a cool line now (no line, but called a port)". We are happy that she is happy with this decision and that she can now do more things. I am sure the first time it is accessed maybe a little bit traumatizing, but I think we can work through it and all will be fine. However, no more dressing changes for Emily which she is excited about and no more nightly flushing. When Dr. Mattei came out and said she was done with the removal and insert, we were happy to hear she was ok and then she was having the bone marrow done. Dr. Mattei did give us a prescription for of tylenol with codeine because he said that she will probably be in pain for a few days. After getting back to the camper today, both Emily and I fell asleep for about 4 hours together. She is laying back down again with Daddy while I write this update tonight.

While waiting for Emily is surgery, we received a call from the social worker at CHOP that she knew that we had some medicine (chemo's and antibiotics) to pick up for Emily, but that the Timidor (temolozide) was not completely covered by our insurance and that it was quite a bit out of pocket. I asked her how much we were talking about and she said $500 per cycle. OUCH. I explained to her that Doug and I are putting these chemos and antibiotics on hold right now until we get a second opinion since CHOP is stating there are not any clinical trials available for Emily right now. While, we still may be coming back to these, right now before we rush into this, we are checking to see what else may be available. I did ask that the social worker to continue to look into this and see if there was something that we could do about the $500 and how they could help should we decide to come back to this. But Emily is stable right now, and we have a little time to see if there is anything else out there that can help, however Doug and I don't like the thought of Emily being off treatment for too long, so we are already moving toward with Sholler and getting what is needed! Being off of treatment for too long, allows for the disease to move forward and this is what we don't want. The social worker said she would email the team, which I then also emailed the team and explained that we want to wait before moving forward and making the wrong decision. As we were walking back from Emily waking up from general anesthesia, we ran into the nurse practioner, so Doug explained to her what we were doing and she understood and felt that it was good idea to get other opinions.

We did reply back to Sholler and would like to have a meeting with her to discuss our options available for Emily. Their social worker is working to get authorization and tomorrow I need to get our Express Scripts (prescription) information to give to them. We don't want to wait to long and want to get this show on the road, so we wait to hear back for the appointment to be made and Sholler will send out a jet to pick us up to get us there. We will continue to keep everyone updated on where we are with things.

Please send us our thoughts and love that we make the right decisions on getting Emily the best possible treatment available. Doug has been doing a lot of reading about the trials that Sholler has sent us that is available, so he is reading into them to see what is best.

Sending our girls at home, Jessie and Brianna, much love and thoughts. We miss you girls. We should be leaving here Saturday sometime, staying her making sure Emily is ok. Make sure you hug and kiss your kids each night and tell them how much you love them. Sending our love to RJ and Joey who lost their fight to NB. We have to find a cure, because this has to stop happening. We will keep fighting to find the cure.

MIBG Results 2-22-12

I know everyone is looking for an update on how Emily's scans turned out...we were both very nervous about the results and especially with Emily complaining of pain in her right hip! The results came back with written terms: " whole body images demonstrate multiple foci of increased tracer uptake in the following areas: the right side of the skull, the lumbar spine, pelvic bones bilaterally, proximal and mid shaft of the femurs bilaterally. Compared to the prior study of 1/10/2012 , the intensity of some lesions has decreased." This means that Emily's scans are stable and some of the lesions are not as bright. Dr Mosse seemed surprised by the results but happy.

This means Emily is not progressing and is not regressing but the intensity of some are lighter. While there is nothing to celebrate as of yet, we are ok with the results and just happy to have Emily here with us and enjoying life.

So, what's next...we still have a long road ahead of us...we don't have an end in sight (I asked) and what is next is low dose chemo. Low dose chemo until something else comes open. Currently Emily does not qualify for any trials, mainly because she is not progressing and because they don't have any clinical trials that are open. So we are on a bridge right now... Low dose chemo it is... Emily will start on a antibiotic tomorrow that is to help with the major side effect which is diarrhea and then come Monday she will start to different chemos at home. Irinotecan and temozolomide. Emily will do two rounds of this which is 21 days per cycle. So the first 5 days will be an oral liquid which is the irinotecan (but we are awaiting approval from the insurance on this one since they normally do it by IV ...if not approved then Emily will have to go to the hospital for two weeks everyday Monday thru Friday to receive the irinotecan) and the temozolomide which is in pill form. After the 5 days she will have 2 weeks of of no chemo and then start the second round. After the second round we will go back which is approximately 45 days for scans to see if the chemo is working and re-evaluate.

We are still nervous about what if this does not work... Dr Mosse said this works for some and they are on it for years but then others it does not work. We asked about hair loss and she said no but on the side effects it says the most common effects are decrease in number if red and white blood cells and platelets made in bone marrow, diarrhea and hair loss. So we are going to question that. Emily will only have to go once a week at our home hospital for count checks.

Tomorrow is surgery day...removal of broviac line and insertion of port. This will work out well with the chemo that Emily is being put on that it will not need to be accessed as much. So ready for emily to be able to enjoy the pool, taking a bath and just normal things and not be left out of the summer activities...this is why our hope would be to sometime take the girls to the great wolf lodge in Williamsburg!!! She will also have her bone marrows checked for disease while she is under tomorrow. She will not be inpatient but we have to be around for any problems and also to get the chemo to take home with us. They want the chemo to start Monday.

Doug and I are still talking about getting a second opinion, if you will, from Sholler in michigan on this and if we are going in the right direction. Doug and I want to do what is right for emily and rid her of this horrible disease and move forward. We also asked Mosse about Sloan but she didnt feel it was a good option for Emily right now.

So we charge in...we move forward... Ok with the results but our goal is still to be Ned... The thought of having to keep Emily on a chemo for long term just to not progress is not something we want...no end in sight, but we move forward and just happy she is happy and not in any pain....

Will update after surgery tomorrow on how she is doing.