Day 5 and I'm on my way (SCT)

[bailie]

Today (August 15th) it has been one year since my stem cell transplant. It has been an interesting and quality year. I have been very fortunate.

To recap, I was diagnosed by low platelets (48), low RBCs (3,75) and dropping WBCs (4.7) in November of 2013. I had two BMBs within two weeks which confirmed MDS (RAEB-2) and blasts between 10 and 19 percent. I was immediately prescribed Vidaza (7 days on with a 21 day break). I reacted very well to the Vidaza and blood counts all moved in a positive direction after the second round. Revlimid was added to the Vidaza for months 6, 7 and 8.

I went to the hospital on August 8th to get my 3-lumen Neostar put in place. On August 10th I started the Chemo Fludarabine. I felt fine the first two days and walking 5 miles a day at the hospital. The third day the chemo hit me hard and I could not walk a half mile. Fourth day was worse. Then I got the stem cells the night of August 14 at about !0:30 and into the morning of August 15th. As predicted by everyone, the first two weeks were difficult. I had no mouth sores ever. I did lose my taste for about a month and my sensitivity of smell increased dramatically.

My first year after transplant has been relatively uneventful. I gradually regained strength with only about two weeks of very mild skin GVHD. At about Day +100 I developed a dry cough which turned out to be one of the worst of fungal infections in my lungs. The infection was in an enclosed capsule that was removed by surgery. Doctors thought it was a complete success and it hasn't bothered me since. There was about an eight week recoup after surgery of just taking it easy. Since that time I have gained strength and endurance weekly. At about Day +230 I was diagnosed with a relapse which was a very rare Philadelphia+ AML with 33 percent blasts. That was a real "attention getter". I was immediately put on Vidaza and Sprycel (which would combat the Philadelphia chromosome). I reacted well almost immediately and after two cycles there was almost no sign of any problems. The doctors are "winging it" now because this is so rare and very little research. I am feeling almost normal with no GVHD and golfing 18 holes weekly. Last month I built a 135 foot long cedar fence along with several other small projects. I do get tired in the evenings and often go to bed before 8:00 p.m. and get up about 6:30 a.m. An interesting side note is that I relapsed with a different disease. The previous genetic markers/mutations (KRAS,SF3B1 and IKZF1) are gone and new ones (the Philadelphia, 9;22) appeared.



Next Monday I will have my 14th BMB. We check very closely to my situation. I will most likely continue on Vidaza and Sprycel for a while. The questions we have at this time are, "do we keep on Vidaza and Sprycel the rest of my life" or "do we back off to see what happens with the remission"? It is a tough decision because of almost zero research for my diagnosis. My doctors have been talking to others around the country for more information.

I would be happy to live several years with how I feel, but the odds are very slim. We know the Vidaza doesn't work forever, so we'll see. All in all it has been a very good year and I have no regrets about having had the stem cell transplant.

[rar]

I have moderate GVHD of the skin, mouth, and eyes. With Tac and 120 mg of prednisone it is getting better. I questioned whether the high dose of immune suppressants will increase my chances of relapse. His reply we can kill you with GVHD with too little immune, or kill you from a relapse with too much. It is a fine line between and we don't really know where it is, so we experiment. I don't know if he is joking. Even with my past severe GVHD of the gut I have no regrets with the transplant. If you trust your doctors then trust your doctors are working in your best interest.

Ray

[bailie]

I relapsed following 160 mg/day prednisone that was prescribed for my fungal infection in my lungs (they thought it was GVHD in the lungs). I don't know if it had anything to do with the prednisone. Turned out that one of the doctors said the prednisone was like pouring gasoline on a fire for my fungal infection. They tapered me from the prednisone really quickly. When they prescribed the prednisone they thought they were fighting GVHD, then it turned out to be a fungal infection. They quickly took me off the Tac at that time also.

What does GVHD of the eyes feel like? My eyes feel heavy in the evening, but show no outward signs of a problem. They just don't feel quite normal.

[rar]

My eye problem is pain, vision that gets very fuzzy, light sensitivity. Eye doc says it is "dry eye", of course. It is getting better slowly. My whole body itchy rash is gone, my mouth is getting better. I had been down to 5mg pred every 3 days and as soon as I stopped I got the GVHD. They are concerned with the GVHD because last time I had it it was stage 4 that removed the lining from the gut with 2 months in the hospital. They told my wife that they see a lot of people who were not as sick as me die. My thoughts is to taper quickly to a low dose than taper more slowly. They say some have to remain on pred indefinitely. I think what I have fits the classic GVHD symptoms.

How long were you on pred? How rapidly did you taper? From the way you write it sounds like that maybe you didn't relapse but got a different condition?

Thanks for the response.

Ray

[bailie]

I was at the 160 mg and they decided I needed to taper quickly. I also had developed diabetes because of the prednisone. I tapered 20 mg every four days. When I got down to 20 mg the taper was to 10 and then zero. What really surprised them was that I never had any side effects from the prednisone except a great appetite. I think I felt the fast taper because I then started on the Vidaza and Sprycel at about the same time. I noticed that I was a little unstable walking and had to be careful. I was on the prednisone for about three months. My temporary diabetes tracked the prednisone almost directly and by the time I was off the prednisone the diabetes was gone. It was tricky adjusting my insulin to correspond to the prednisone taper. I was doing the finger pricking five times a day and my levels were all over from 45 on the low to over 500 at times. That made the prednisone taper a little more difficult. That time was the worst that I felt since about Day +12 after the SCT.

[traceyn11]

I wish you the best Bailie and hope you do get to live several more years with how you feel! You are such a supportive and informative member of this forum!

Tracey

[PaulS]

Hi Bailie - Congratulations on your anniversary - hopefully you will stay in remission for a long time. As you are in uncharted territory anyway - maybe the Vidaza will work longer than it might normally.

Your positive attitude is inspiring and I wish you all the best and many years filled with joy.

Paul

[bailie]

Thank you Paul and Tracey. I wish you the best. I had no pain going through the transplant process and I hope that neither of you will. My daughters stayed with me 24/7 for about three months and that really helped. My wife was there much of the time also, but she was in charge of our house and dog until I got home.

Ask questions as often ... we are here to help.

[bailie]

Just a quick update, I had an appointment yesterday to discuss a new mutation (Q252H) that has developed from the "Philadelphia chromosome" (9;22 translocation).This is very interesting. The nilotinib and previous dasatinib did not hold back this mutation leaving trace amounts of the cancer cells. So now I will be going back on Vidaza (7/28 days) and ponatinib (a "black box" chemo). So we'll see how that goes. My diagnosis (AML w/ Philadelphia chromosome) is extremely rare which makes this more interesting. There are no studies for my treatment and almost nothing in the literature so we are educationally "winging it". I have been feeling well and my CBCs and CMPs are about as good as any time in my life. Our insurance has been paying over $40,000 (not counting the cost of the BMBs) a month.

[Neil Cuadra]

bailie,

Being an "interesting" patient is less than ideal. I'm sorry they don't have more experience to know the ideal way to tailor your treatment.

My wife was also considered "interesting" to the hematologists at her treatment center. She was a topic of discussion at each of their team meetings. It was nice to have their combined expertise on our side, but there's no honor in being the first person with a particular combination of genetic abnormalities.

[bailie]

Thank you Neil, I always appreciate your perspective. My doctors have been reaching out to other doctors/experts around the U.S to get their input. There is just nothing out there that addresses my situation. It is "interesting".

[PaulS]

Hi Bailie - I thought I saw this post the other day but I was in a druggy stupor at the time and was hoping it wasn't so. I'm getting pissed at all the **** they keep throwing at you! Hopefully you'll continue to respond and to enjoy life. Your attitude is, as always inspiring. Are you having any symptoms or do you still feel relatively OK? Golfing?

Best to you and your family as well.
p.

[bailie]

Paul, I guess we all have our battles. It always amazes me how dissimilar they are. I guess that is why they really don't have a handle on these diseases. We try to extract as much as we can from each other, but that is a daunting task in itself. The more I learn, the more I realize how much I don't know. Same with the doctors. For me it is like trying to put together a 1000 piece puzzle with a few pieces missing. My thoughts have transitioned from this is mostly a science to this is as much art to now it is a lot of guesswork. That makes these diseases interesting, frustrating and many other things.

I have no complaints with the way I feel. I have periodically had joint pain (left hip and left shoulder) that is interesting. It feels like "bone on bone". The pain will really slow me down for about four days and then will completely go away. I have been trying figure whether it might be GVHD, the chemo (nilotinib) or something else. I will be switching to a different chemo (ponatinib) next week that doesn't have the "joint pain" listed as a side effect. The trade-off is that it is a "black box drug" that lists heart attacks and blood clots as a significant problem. I am not golfing now mainly because we have been having record breaking rain in the northwest.

I am hoping that you are starting to turn a corner with your situation. I am with you mentally knowing what you are feeling. Progress is our goal and keep the goal in mind.

[bailie]

Update, I just finished 30 days in the hospital with induction chemo following a relapse in early April. Still feeling weak from the stay in hospital. Will start 10 day cycle of Dacogen on Monday in preparation for a DLI in middle of June.

[JordanN]

Hi, Bailie,

I just wanted to wish you all the best with the Dacogen treatment and DLI coming up. You have been through so many ups and downs with this crazy disease and somehow you find the way to keep going and keep on managing an unbelievably difficult condition. You have my respect, admiration, and sincere wishes for success in your upcoming treatments.

I worked for a short time at OHSU and the VA in Portland many years ago- I hope they are giving you a room with a fantastic view of Mt. Hood!

[bailie]

Jordan, thank you for the encouragement! As far as the view that you mentioned. We have talked about it often. I thought the same thing as I walked into my room. For the first two days I thought "this is terrific"! Then the chemo hit. I didn't care about the view and very seldom wanted the blinds/curtains open to see the view. It surprised me that I had those feelings. The Flag/Ida seems to hit me very hard.

Thank you for your comments. My situation has my doctors baffled in that there are so many atypical aspects. 1) At Day +210 when I initially relapsed, I relapsed with a leukemia completely differently than my pre-transplant diagnosis (very rare). 2) My pre-transplant mutations did not show up after relapse. 3) I had a new mutation characteristic of CML (the "Philadelphia chromosome" and not MDS nor AML (very rare).

[Hopeful]

Thinking of you bailie. Hang in there! You are incredibly strong and have such a great attitude. Hope things turn for you soon!

[Cheryl C]

I'm very sorry to read that you've relapsed again, Bailie. You are brave and always so confident and positive. I wish you success for the DLI coming up and I'll be watching for your posts.

[Sue&Dave]

Bailie - I echo the sentiments of all our fellow posters. Best to you - keep the faith, dig deep when necessary but don't forget to lean on those you can count on. I will be praying for you.

[maggiemag]

Unbelievable, Bailie! Another relapse? Well the bad news is that you are unique; the good news is that you are unique. All along your journey you have shown us incredible courage, attitude, and also a great deal of knowledge that you put to use. Stay determined! You have faith in your medical team, and know they want to cure you for good this time. I send you my best, my admiration and my prayers for a speedy recovery from the DLI, although I now forget what that stands for.
Mags

[bailie]

Thank you everyone. My status is getting down to fewer choices which is somewhat daunting.

Maggie, A donor leukocyte infusion (DLI) (which I will receive from my German donor) is a possible strategy for managing a patient in relapse. In this procedure, the patient receives a boost of immune cells from the original donor's blood. In certain circumstances, it may be extremely effective in controlling recurrent cancer in a patient. This ability of the donor cells to control or eliminate the residual malignant cells has been called the graft-versus-tumour effect. Their are drawbacks however with a DLI primarily GVHD which is unpredictable. The success rate for the DLI is about 15-20 percent. If it fails the situation will be difficult.

[lisa3112]

Good luck ballie! Sorry to hear. Good luck with the DLI. You are very strong and I believe your positive outlook will get you through this 👊

[Cheryl C]

Bailie - I wonder if this info could be useful in helping protect you from infections if you decide to go through with the DLI? I just received it from my brother yesterday and it looks interesting. I'm prone to infections myself due to a low WCC and hypogammaglobulinaemia.

https://www.ncbi.nlm.nih.gov/pubmed/27940189/
https://mail.google.com/mail/ca/u/0/...c44bbbf6703fa6