Starting DFMO with Update

We are en route on our way home.  We were in clinic a little longer than expected, so after getting back to the camper and hooking up, we got a late start. We will be making a stop in Ohio tonight soon to rest and then staying in Pittsburgh tomorrow night. It's been hard because we haven't had internet to really update, so I apologize. But I do have to tell you, the camp Host at KOA was a great lady and was wonderful to work with. Thank you Theresa for being a great host. 

Emily had her MIBG scan yesterday and did pretty well. Her head was really hurting from the way she was laying, but she did well. Yesterday we thought she would have to go to clinic after MIBG scan, but she didn't so it was nice to go back and hang out at the camper and nap and enjoy time with kids. 

We had heard from Shannon, the nurse practitioner who was wanting Emily to get GCSF, we couldn't understand why she wanted it.  So we waited to talk with her today about it. We did get preliminary results from yesterday's scans yesterday as well, but didn't have everything back. She asked that Emily be in clinic at 8 before scan to check counts and see how things looked. 

Today She had her PET scan today and as always rocked it. Thankfully her glucose was fine this time and didn't cause a delay like it did in January. Shannon called with her counts and thankfully her platelets looked good from her Wednesday transfusion, hemoglobin is dropping and her ANC on Wednesday was 210 today it was 0!  Shannon said Dr Sholler would really like for Emily to get GCSF/neulasta shot because her bone marrow right now is not making anything, it is less than 5%. She said this is very dangerous and they would feel better that she have it. She did state again, please take Emily in to the hospital if she has a fever over 100.4 since we are driving. PET scan went well and she handled it well. 

We went back to clinic after scan, and talked with Shannon. Scans are stable with some spots brighter/more density.   They feel there is more density because her VMA in January was 8.2 and it has gone up to 11.2. We should have results from PET scan Monday hopefully.  If they are negative like they were before, like we were told before we know there is still cancer in her body based on numbers and scans.  After talking with Shannon, we decided it was best for Emily to get neulasta. Emily's bone marrow is not making any cells and they could see that from the bone marrow biopsy/aspirates. Dr Sholler was notified of this and she too was concerned.  Shannon also explained that when looking to see if they were positive for MDS and NB, they only had less than 5% of her cells to look at them, so depending on how things go she may order another one when she needs to be back in 8 weeks. We are still waiting for the final results of the bone marrow and for the cellularity and the cytogenetics. We wanted to head home and have stem cells given back to her but we need to wait on the final results to know if giving her the cells would even work at this point. If we gave them to her and some things don't look good, it wouldn't work. The final results which we are nervous about, can tell us if she has pre leukemia or anything else going on in the marrow.

Stable is good. We honestly have adjusted to hearing stable because that is what we normally hear, but it's positive when there is no new disease. It's still heartbreaking when you just want to hear those 3 magical words. One day, we continue to believe, one day.  HOPE!  Right now and for a while she doesn't qualify for any therapy because her bone marrow has been hit to hard.  Shannon stated Emily should not have been on the chemo with the way her marrow looked, but who would have known since we didn't have a bone marrow since last year. It's scary to think, but this is why Emily had a neulasta shot before leaving the clinic today. 

We started DFMO in clinic today and Emily is happy to know that she is no longer on chemo for now. Her body/bone marrow needs a rest. Emily showed Shannon she could take 2 pills at the same time. We went over everything again, and when Emily needs to be seen in clinic at home to get labs and physical done. We also have to make sure that if Emily has to go in the hospital for anything that the doctors let Michigan know in real time what is going on because this all has to be reported to the FDA since the drug is provided by the FDA. Right row Emily will still continue to make 2 a week trips to clinic at home until her counts have recovered. It's most likely she will need blood come Tuesday when she has clinic. And again since her ANC is bottomed out, if any fevers we are to head to the closest hospital. Please hope for no fevers.

Please continue to send your thoughts and love that everything comes back with good results. Emily needs a rest from treatment/hospital visits/clinic. She wants to return to school, she wants to do some "normal" things!

Remember to hug and kiss your kids everyday and tell them how much you love them. Things can change quickly. Thank you to all for continuing to be a part of her journey and spreading awareness of childhood cancer. 

Neutropenic Here in Michigan

We are all exhausted ... Late night last night to early wake up to be at clinic this morning early equals not a lot of sleep!

Emily stresses a lot when she has to get accessed here in Michigan because a couple of times they accessed her a couple of times with no blood draw! But this morning it went ok ... We figured she would need platelets since she had quite a bit of bruising on her legs, arms and head. As figured her platelets were 18 even after getting 3 units of platelets on Friday back home. Bad news her ANC is 0 again. Neutropenic in Michigan is not where we wanted to be... EspeciAlly if she gets a fever, we head inpatient here which is not where we want to be.  So we are staying away from people and hoping to keep her fever free! Dad and I are happy we brought the camper because she doesn't have to be trapped in the renuncci hospitality room (most likely without kitchen) and can move around our camper and also go outside and not be around a lot of people! 

Today was a long day... We didn't think we would see Dr Sholler this visit because we knew she had a conference of some sort this week. So this morning when she walked in, we were surprised. She was leaving within the hour to catch a flight to south cArolina to talk about dfmo! After catching her up and what information she had, she wanted an additional testing done for MDS from her bone marrow aspirate and biopsys that she had this morning. We talked about the DFMO trial because this is where dad and I feel would be best for Emily right now. Emily's bone marrow needs a break from chemo, she wants her hair to grow back and she needs rest from the hospital. The 2-3 visits a week along with Inpatient stays right now isn't where we want to be. Emily has been battling neuroblastoma for over 3 years with lots of chemo, it's time to give her a rest. Don't get us wrong we are nervous about this change to compassionate trial of DFMO worrying over her progressing again or bone marrow not start getting better but dad and I agree it's time to see how she does. Dr Sholler agreed she felt this was a good move. 

So papers were signed for compassionate use DFMO trial today, hearing test done and now we move forwArd to scans tomorrow with MIBG and Friday PET scan.

As for hearing test results showed she has lost high frequency hearing and it is more severe in her left ear.  They didn't feel that hearing aids were needed at this time but said that she may need a personal FM system to help at school. We noticed it yesterday in the truck with a lot of background sound and Brianna talking , trying to talk with her she could not hear. 

At this point bone marrow needs to be negative to start dFMO trial.  The compassionate use means she is on a trial but it is not as strict as the one for NED! She did well with the bone marrow today, and had a little pain after but handled it well. She also did well with all pre meds for platelets and platelets well. She hAd some coughing from the platelets but we have noticed that quite a bit lately!

While we have been here blood pressure has been looking pretty good. It did go very low during bone marrow but was explained pressures normally go down with sedation and hers are a little more lower bc of her meds that she is taking but nothing they were concerned about!

Tomorrow is MIbG scan so hopefully she does well tomorrow and please continue to hope for stable or better. Wear your Emily gear tomorrow or Friday (and if you don't have gear, wear purple and pink to show your support of Emily! 

Thank you again for loving our girl and supporting us! It means so much to our family!! Please hug and kiss your kids and tell them how much you love them!

If you are interested in helping with a fundraiser for Emily, check it out here: http://loveofcharms.origamiowl.com/parties/emilyhubbelsfundraiser231675/collections.ashx

There is also a Facebook Page you can check out for the fundraiser and a place to ask questions : https://www.facebook.com/events/276371519190331/

Michigan

Sorry for the lack of updates but this too will be short...

We arrived in Michigan late last night. It is very cold here, 9 degrees and still lots of snow hanging around! We did being the camper this time because of Emily's lowered counts and being safe. Having her around of community of people isn't safe!

Today clinic, counts, Bone marrow and MIBG injection! Tomorrow Mibg scan and clinic and Friday pet scan and clinic.

Please send your love, thoughts for this to have stayed stable or better. Thank you for your love of Team Emily!

Update for the Week

We have had another busy week, but thankfully no inpatient stays for this week.

Tuesday, March 4th Update:

Emily had clinic this afternoon, and first time in a long time she didn't need any blood product transfusions. Everything is still low but not low enough to require anything. She is still neutropenic but hopefully by Friday when she has clinic again, she will be no longer be neutropenic. When she got there today her blood pressure was really high today. They gave her Hydralazine and checked her blood pressure every 15 minutes for hour and half and talked to nephrologist who requested that we up her dosage if enalapril. She has been getting headaches a lot which could be from how high blood pressures.  Next week they want us to meet with the nephrologist but they think she has chronic kidney disease from long term chemo that we will probably have to deal with long term or it could be short term. Insurance doesn't cover the blood pressure machine for home so they told us signs that we need to look for.  Other than that Emily seemed to be feeling so much better today and she is eating well.

Friday, March 7th Update:

Emily had clinic today and it was an all day visit today. Emily is still having high blood pressure! Thank you to some wonderful people who sent Emily a blood pressure machine to be able to see what her blood pressure is. They know who they are and we thank you so much! All of her counts are starting to come back up slowly except her platelets took a big drop from Tuesday. We did learn that she was positive for ParvoVirus PCR (but that was taken over a week and a half ago now) which helped in her counts dropping to zero along with the chemo. We have no idea how she would get this but Dr Gowda says it's in the air and because she is immunosuppressed she could easily get it! So along with the chemo, her counts dropped a lot.  He said they would normally give IVIG for that, which we did and antibiotics, which we did. I was worried wondering how do we know she is not still fighting it and he said that her counts are coming up, so we know she is past it. He said if her sister were to have it, they don't normally treat it, you have to let it pass.  Today her ANC was 1100 so we are finally out of the danger Neutrapenic zone and only In the precautions of being careful.  With that we are going to start her back on her two chemos. Dr Gowda did not want her to go on the thalidomide and Celebrex since it is supposed to drop your counts a lot and we didn't want to go back on full dose of the two she was on, so we are back to etoposide and cyclophosphamide! However instead of taking both of them everyday she will take 1 of them every other day.  We are currently working with Dr Sholler to get Emily in soon for scans, bone marrow to get her on compassionate use of DFMO. We really need to get Emily's bone marrow and platelets back up so that she can do other trials and platelets not be an issue. Julie, Dr Sholler's nurse, said she is working on it, so hopefully in a couple of weeks we will be on our way. As long as Emily has no progression she will be able to go on the DFMO for a while! We are looking Into a trial that is Dr Lucas' trial so we are waiting to hear back! This trial we are hopeful we could do is in Boston at Dana Farber, but again have to wait to hear back.

Today she did need to get platelets since they dropped so much. He again gave her 4 units since the one from this past Sunday lasted until today.  She continues to get Tylenol, Benadryl, Pepcid and solumedrol which have been helping in not having any reactions like she had before along with the platelets going slower.   Thank goodness!

Like I said before her blood pressure still remains an issue. Dr Bunchman who works with Dr Lo came to see us and he explained that he thinks this is a side effect that Emily will deal with for the rest of her life from chemo, radiation, MIBG radiation and more

Chemo. He called it Microangiopathy disease which is small blood vessels from treatment.  So he changed her medicine to lisinopril and upped the dosage a bit to help with her blood pressure. So starting tomorrow (since she had received her morning dose this morning) she will start the pill which should should last for 24 hours where the liquid was much faster acting and not staying in her system as long. Tonight she will just get her other dose and no longer take the enalapril!

We are hopefully her counts will continue to go up and she doesn't have to deal with the chemo side effects from doing them every other day. We are also hopeful to get her to Michigan soon so that we can get her on the DFMO and see her hair growing back and not seeing the nasty side effects she had! 

Please continue to keep Emily in your thoughts and love. And remember to hug and kiss your kids every night and tell them you love them!

 #neuroblastomasucks #emilyupdate #emilysjourney #emilyhubbeldotcom

Home

Home!!!

Everyone is exhausted but Emily is happy to be home.   #emilyhubbeldotcom #emilysjourney #emilyupdate #neuroblastomasucks 

Checking In

Last night was an extremely long night. I think I got about an hour of sleep total from watching her and keeping up with high blood pressures! The IVIG they check vitals every half hour and every hour hour increase if her body/vitals can handle it! They started at 8.5 and by 30 minutes she should have been able to go to 16.5 but during the first 15 she had a little high blood pressure so they keep it at 8.5 and then the next half hour checked it again and it was down a little bit. They went to 12.5 instead of 16.5 and she went up a little but not too high...next half hour tried to go to 16.5 but her blood pressure went up so they decided to put it back to 12.5 and leave it there and give her blood pressure medicine to bring it down.  Overall the rest of the night she handled the transfusion well. It went for 11.5 hours at a very slow rate.  It finished by 11 and then they checked counts. Port is still bring funny right now and still having a hard time getting the blood to draw but was able to get what she needed to send it off. 

Dr Lo came by this morning and said that she felt like at this point Emily didn't need to go home on any blood pressure meds but that she would follow her when we are clinic. She also reviewed her kidney ultra sound, bladder and that nothing was of concern but could tell where the tumor was removed that kidney was a little smaller but wasn't causing any problems!

Remember yesterday her platelets were 23 and they gave 4 units last evening, after the draw today after IVIG they were 15. Crap what is going on. Hemoglobin dropped from 9 to 8.2, so hopefully she won't need blood again anytime soon. 

so they ordered pre medications and started those. Platelets came and they went to start them but checked vitals and Blood pressure was too high (146/96)to start platelets so they gave her hydralazine and after a little while blood pressure came down and she could start.  The team did call Dr Lo and she would like for Emily to go home on enalapril. Taking a dose in the morning and in the evening and they are also checking with critical care to see if they can bring a blood pressure machine to the house. 

Platelets have now finished and blood pressure is up again now that done and they have redrawn labs to check to see where her counts are before they send her home tonight. As long as everything looks ok they will send her home on another medicine to the already other hundred that she is taking and keeping an eye on her blood pressure. 

So now we wait! Please continue to keep Emily in your thoughts and that we make home tonight with no other issues.  And that everything looks ok to at least give her a break and she come back Tuesday!  We are ready to get home to be with daddy and Jessie will be there tonight and Brianna is coming back from nanny's tonight. Emily is more than ready and wants to be home!

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#neuroblastomasucks

Thank you Carla and Morgan for the shirt, Emily loves it!

Very Long Day at Hotel VCU

Overnight Emily continued to have high blood pressures but thankfully no fevers. Blood draws at 5 in the morning tend to be rough as well because her port has been very positional so drawing blood has been tough! So the past two nights she has had to come back and draw again each time waking Emily and trying to get her to move around to get the blood draw!

Platelets didn't go up much today either 23 only to 26! She was given 2 units yesterday so they decided to give her a full 4 units today. Her body since that anaphylactic reaction has been eating up the platelets. WBC .4 so she is starting to go up, just really taking a while. They did say the CMV virus they were checking for came back negative but the other one has not come back yet!

Today has been a rough day. Actually it's been a rough week, we all just need a vacation away from work and everything, ugh something would be nice. 

This morning her blood pressure was really high before Dr Gowda visited and were all over night. With blood pressures still being an issue, dr gowda said he is not ready to let her go home yet! So they worried that with high blood pressure that her kidneys were having some problems especially since her creatinine levels have remained elevated but not crucially high. Etoposide can cause kidney issues as well as years of chemo (like she has had), so they contacted Dr Lo a Nephrologist! However Dr Gowda feels the high blood pressure is the overload of all the fluids, But they also don't want to rule anything out! So they got a Nephrologist to be a part of Emily's team and to see what's going on. She ordered an ultra sound of the kidney and some extra labs overnight to check somethings. Blood pressure remained high and so they gave her IV hydralazine and checked it in 15 minutes and her blood pressure was still high and then gave her isradipine (pill) and after awhile it was still high so another dose a little bit higher of hydralazine. After half hour it was checked again and now she had extremely low blood pressure and very high heart rate and extremely lethargic. Then time for a bolus to bring the blood pressure back up! By this time Emily had moments of ups and then extreme downs and when she finally came down off the medicine she seemed to be feeling like a "glucose low".  During all this they were able to give her platelets but watched her closely (we were upon the 4 hours and we really didn't want to lose platelets that a donor took time to donate) and even the ultra sound was being done around this same time. Emily has been very agitated throughout all this not talking much and feeling very annoyed. During this time we also had bloody lose stools. So all of this adding up made for a very nervous mom watching all this and seeing her moods up and down. Thankfully this morning she felt up to hanging out for 2 hours in the playroom with Sue, myself and Brianna! See picture....

Dad slept today after not sleeping all day yesterday and of course night from working, I hated that when he called after waking up to give him the update of the day.  Never fun to wake up and hear the news that Emily has had a rough day and all that has been happening. Then being frustrated with the doctor on too much medicines so going from one extreme to the other, was hard! Thank you to Sue for bringing Brianna up for the day and getting breakfast and lunch. Poor Brianna hasn't had anything stable with Emily being in the hospital and having a hard time with dad going to work, and where she will be for the night! She was really good today and nice to have her here hanging with us. Tonight Nanny also came for a visit and then daddy got here with toys for both of the girls. Thank you nanny for taking Bri for the night and thank you sue and Jojo for holding down the fort. I feel so bad that we couldn't have any down time to visit and see Jojo and wish we could just take some time off and go spend time with them. 

Emily hasn't seem to have had anymore loose bloody (and they were not just tinged but all red and obviously blood) so we are thinking she had some kind of gi thing going on or tear that couldn't clot because her platelets were so low and then has not had anymore since she received platelets! I know we were concerned with hemoglobin dropping again but they did a stat hemoglobin check and it was from 10 to 9.

For the past 24 hours Emily is starting to eat better, so thank you dad for making sure she had her Mac and cheese because she has been eating that and chicken noodle soup like crazy!

Tonight about 1110pm we finally got the IVIG going. The hope is that whatever antibody or whatever that is eating up the platelets the IVIG will help it. The biggest concern with IVIG is blood pressure so it Is being checked what seems like every 15 minutes. They bump it if she can tolerate it but they are only bumping it at halves to not overwhelm her body! At first her blood pressure went up in first 15 minutes but has gone down within next half hour! So so far she is tolerating and for that I am happy! 

Emily is very agitated with people, doctors, noises and just anything out of the normal. I feel so bad, dad and I both do because of how she is feeling! Everything she is going rough and putting her through more hell when this was supposed to be less!

The plan is to get this done with no high or low blood pressure because if goes " low we may have to either do fluids and if it goes high will have to do meds.  It will probably be about 10-15 hours to complete so I am not sure when the blood draw will be completed to check counts and everything else and we are not sure on discharge. I know that Dr Low is to come back in the morning and review Emily and see what is going on. She will determine if she will need to go home on blood pressure meds tomorrow and review kidney ultra sounds. But for us, We just want to make sure she is safe when she heads home and not worry about blood pressures and anything else. If discharge tomorrow, we will have clinic on Monday morning to get blood counts and check in with Nephrologist!

Please continue to keep Emily in your thoughts and love. We are all completely exhausted and want to be home. I'm so tired tonight and won't be sleeping much to keep an eye on her. 

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