We Have a Schedule
I just talked to my contact at Temple - Fox Chase and she said the insurance company approval for my transplant went through, so Lisa starts prep work tomorrow, is scheduled to start her harvest on 8/5, and I'm scheduled to start my "conditioning" on 8/12. There are things that could delay this, but this is now a likely scenario. I also am going in tomorrow to talk to my oncologist for the results of my test and to get a calendar, etc.
While I must admit to some degree of nervousness as the actual transplant approaches, I know that in virtually every regard, sooner is better.
A Donor is Found
OK, here’s the latest. Monday, I went the dermatologist to have my flaky ears looked at because my oncologist was concerned that it might be a fungal infection. Both the young doctor I saw at first (Dr. Y) and the senior doctor he consulted with (Dr. S) were interested and bit confounded by what they saw. They said it doesn’t look like a fungal infection, but they didn’t like any of the other theories they came up with either. Dr. S said to Dr. Y that it was an “interesting presentation”. They took a couple of slices off one ear to send in for biopsy. They also said it was very unlikely to be skin cancer because it is symmetrical, as in on both ears. They also talked about cold damage, a discoid lupus (DLE) (as opposed to a systemic lupus (SLE)), and other things, but like I said, there were counter-indications for all of them. We’re waiting a week or two to get results from the biopsy.
The big news, however, is that I’ve got a sibling match for my bone marrow transplant and it’s my favorite sister, Lisa!!! I stopped by this morning and told her in person and she seemed delighted. I know I am quite relieved and happy. I discovered that I have been counting on a sibling match, realizing that I shouldn’t because there was a real chance of not having one. When FCCC called this morning, she said they found a match and it was some name like Joan. I said, “Who?”, and she looked again and said, “No, I mean Lisa” (she was looking at two different patients at the same time). For a second there, I thought I had a sibling that I was not aware of. She then said the oncologist, Dr. T wants to move along quickly so they wanted to schedule me for a battery (battery may be a particularly appropriate term) of tests some time next week. By the time I got to work, they had called and left a message that I was scheduled for tomorrow (Friday) from about 8:00 AM to 3:30 PM and the tests include a pulmonary function test (PFS), a MUGA scan (heart mechanics), a chest x-ray, and EKG, blood work, and a bone marrow biopsy, as well as a visit with a social worker and Dr. T. If the MUGA scan is to determine if I’m a Muggles, I can save them the trouble because I’m certain that I am (if that confuses you, don’t worry – it’s just a bad joke that readers of Harry Potter would get).
By the way, my weekly CBC showed my counts basically back to where they were before I got sick, with my platelets down a bit to 26K from 28K.
Thank you to everybody who has volunteered to get test as a donor for me. Finding a sibling match is really the best for my transplant, and the willingness of so many friends to be a donor makes me weep with gratefulness. One thing you could do is consider registering with the National Marrow Donor Program (NMDP) if you would consider donating to save the life of someone you don’t even know.
Another Feverish Weekend
Right now, we are waiting for the results of the HLA typing to see if there is a donor among my siblings. The last time we checked with Fox Chase Cancer Center, they said the results usually take 12 working days from when they got the blood samples, which would take us to this Thursday, July 22, and I think that is probably the earliest we would hear. After the donor is located, there is further testing of the transplant recipient (for those of you who are getting confused, that’s me in this case), getting approval for the transplant from the health insurance company, and then further testing of the donor before the harvest is started. It seems each of these things will take a week or so, so they said it looks like late August is the most likely time for the transplant to happen.
In the meantime, here is what happened last week:
· Wednesday, I went to see the doctor who originally drained my pilonidal cyst, and he said as far as he was concerned, I was done with it and he would not recommend doing anything further. I’ve healed well and he doesn’t even know if there’s much of a cyst left.
· On Thursday, I woke up with a slight sore throat and some achiness, but I went to work anyway, hoping they would just fade. They didn’t fade much, and I was already scheduled at 3:30 for my weekly hematology appointment to have a CBC. I told them about my achiness and sore throat, but my temperature was 98.9 and my hematologist, Dr. H saw nothing in my throat. My blood counts were very close to what they had been the 2 prior weeks. Dr. H also discussed the possibility of me being treated with Neulasta which is a G-CSF (granulocyte colony stimulating factor) drug to boost my white blood count (WBC). The advantages of taking Neulasta are that it should boost my WBC to decrease the likelihood of more infections, plus if it worked, it would tell us that if I were to get a serious fever, a G-CSF would be an effective treatment. He left the decision up to me, so after thinking about it for a few minutes, I decided not to go ahead with that right now. That night, I developed a fever of about 100.4.
· Friday morning, the fever was still at 100.4 and my throat was even sorer, so I called Dr. H and when I went in, they recorded a fever of 100.4 also, and Dr. H said he saw redness in my throat he hadn’t seen before so he put me on an antibiotic even though he thought I probably had some virus and not an infection. My blood counts all seemed to change quite a bit in one day, with my WBC going up from 1.0 to 1.4 (the highest it’s been measured since 02/03), the platelets going down from 35 to 28 (the lowest it’s ever been measured), and my hemoglobin going up from 9.4 to 10.0. I’m glad to know my body still knows how to increase the WBC when needed. The last reading surprised me the most because the red blood cells I would think would be a little more stable, and there’s not an obvious reason why they would go up as there is for why the WBC would go up (to fight the virus/infection). Dr. H also had no explanation why the hemoglobin would jump. I wonder if the different testing machine could be why. He brought up the Neulasta possibility again, but I said I didn’t know how to evaluate the wisdom of going one way or the other. I think he sort of took this as another declination to do it now, so we’re not. He then sent me for a throat culture and blood culture to check for a systemic infection. I felt pretty bad Friday evening, but the fever broke a little before I went to bed.
· Saturday (yesterday), I felt a lot better, although still not great. I still and a sore throat and my right lymph gland had swollen so big, it was easy to see just by looking at me.
· Today, I feel even better and while the lymph gland is still quite swollen, my throat is not as sore.
I find that getting sick is a bit worrisome because I realize that my weakened immune system could probably be overwhelmed pretty easily. While it seems like things are kind of dragging here lately, my hematologist said that things don’t usually move this quickly for a bone marrow transplant, so I guess more patience is called for.
Some Good Things That Have Happened Already
On June 10, the day I got the definitive diagnosis that I had MDS, I got home from the doctor’s office at about 4:00 PM. I sat on our porch looking at our beautiful back yard on a beautiful day, waiting for my wife to get home so I could tell her. I was also feeling quite sad and teary and wondered how I was going to reach our four children (aged 27, 25, 22, and 20), of which the older two didn’t even live at home. Our 22 year old got home first, but I still waited for my wife to get home which she did within a few minutes. Within about twenty minutes after telling her the news, all four of our kids showed up and as we all sat on the porch, I broke the news to all of them and we had a good cry. We then all went out to dinner because no one felt like cooking and we felt like being together. The amazing thing was that I can’t remember the last time the six of us were together spontaneously on our porch or anywhere else. It was completely unplanned (by any of us). While I know a case could be made that something like that could be a mere coincidence that could be explained by probability, I also believe that a higher power really has everything under control and saw to it that we wound up together like that.
I have four brothers and a sister that have been so supportive and so willing to do whatever it takes to contribute to my wellness. I think they might even fight each other for the opportunity to be a donor and go through whatever that entails. They all say they feel lucky because at least they can do something. So many other people want to do something and to them I say if you just keep me in your prayers and keep positive thoughts in your head, you are doing more than you can imagine. I know it is frustrating for all my friends and relatives who want to do more, including my own parents and all my wife’s family, but I hope they all know that their love means more to me than words can express.
A few days ago, my sister wanted to have a prayer circle for me. At first, I was not really comfortable with the idea of being the focus of prayer circle and when I expressed this to my sister, she said we could make it about her. Anyway, she invited our parents, all our siblings and their spouses, and a few good friends of mine over to our house. Even though none of us had vast experience with prayer circles, we knew that we could all handle such an organic event and that however it turned out to be would be just what it was supposed to be. Indeed, it was a very special evening and I was deeply touched by the love and concern that was expressed.
I sent out a brief email to my friends and associates at work just so they would know what was going on and not be totally surprised when I wound up in a hospital going through chemotherapy and having a bone marrow transplant that kept me away from work for quite a few months. The responses I got were so sweet and thoughtful that I feel like weeping every time I think about it.
One of my brothers, who is involved in AA, mentioned to me one evening at a wedding that a whole lot of people in AA of whom I was unaware and whom I didn’t know were praying for me and keeping me in their thoughts. When I hear something like that, I once again feel almost overwhelmed by the amount of love and concern that has come my way – even from strangers.
Whenever we see a medical professional that asks about our kids, my wife has taken to always saying that we have four kids and they’re great kids. We both smile because it’s become kind of an inside joke. WE DO HAVE GREAT KIDS! They have been so loving, supportive, and mature through all of this and I can’t imagine trying to get through it without them.
Most of all, I am grateful to my wife, who is also being very loving, supportive, and mature throughout all we are going through. While I know that the treatment will be hard on me, it’s not hard to imagine that in many ways, it will be harder on her. She will be the caregiver with everything that implies. She will have to watch me go through whatever it is I have to go through. I am glad to know that she will also have friends and family she will be able to turn to for love and support herself.
But most of all, I am grateful to my God, without whom I know I could not come close to getting through this. Lord, I know that you did not want or cause this to happen to me, and I know that you have brought, are bringing, and will continue to bring countless good things out of this situation. This is truly the miracle. I also know that to the extent that I can put my hand in yours and trust and follow you as a child does his parent, I will be able to get through anything with equanimity. If I appear strong, I know it is you that are the source of that strength. If others have figured out how to love me despite the obstacles I put in the way, I know that it is because they have figured out how to be vessels for your love. For all of these things and more, I thank you.
Bone Marrow Transplant Info
If you haven’t been there already (this is the same link as in the Introduction post) and are interested in a good primer on bone marrow transplants (BMTs), there is a book called Bone Marrow Transplants: A Book of Basics For Patients which is written by a lay person and the first chapter called "The Nuts and Bolts of Bone Marrow Transplants" is accessible online.
If you don’t want to wade through that much information, I will briefly explain BMTs as best I can. First a donor must be found. If one cannot donate their own marrow (autologous transplant), and I cannot because my marrow is diseased, then a matched sibling (allogeneic transplant) is the top choice. Finding a match involves doing a matching called HLA typing, which is done by drawing blood from the recipient and potential donors and then trying to match on all 10 antigens that they look for. There is a 25 to 35 percent chance that each sibling can match on all 10 antigens, meaning that since I have 5 siblings, the chances of finding at least one perfect match is around 75 to 90 percent.
There are some 200+ antigens that they don’t match and often don’t even know what they do and this is a big reason why they like sibling donors more than a MUD (matched unrelated donor), i.e. the other antigens are also more likely to match. However, if there isn’t a perfect match among siblings, they sometimes do a transplant with a one antigen mismatch, but may also go to the national bone marrow transplant registry which contains about 5 million potential donors. The donor then goes through more extensive testing.
Harvest from a donor is done in essentially one of the two following ways:
1. In a bone marrow harvest, a large needle is used to extract bone marrow from the top rear of the pelvis in several places and usually under a general anesthesia. There may be a little soreness for a day or two and the total marrow taken is about 2% of the total which the body replaces in four weeks.
2. A peripheral blood stem cell harvest which involves daily injections of a drug called neupogen for 4 days starting on a Thursday, followed by a harvest from the blood on Monday using a procedure not unlike the one I did for platelet donation where blood is drawn from one arm, processed by a machine that extracts the stem cells, and then replacing the blood in the other arm. The neupogen causes the donor’s bone marrow to produce so many white blood cells (20 times as much as normal?), that they and stem cells are forced into the blood stream. The marrow returns to its normal production rate when the drug is gone and the side effects can include achiness and maybe even a slight fever, but only for a day or two. The drug has been around for 15+ years and there seems to be no short or long term risk.
After a donor is found, the transplant recipient is “conditioned” for 6 days with chemotherapy and/or radiation to kill off essentially all of the bone marrow in the case of a full transplant, or much of the marrow in the case of a “mini” transplant. Since there is no marrow to produce all the blood parts, transfusions are required, and the donated marrow or stem cells are also implanted intravenously. The cells circulate and when they get to where the bone marrow belongs, they recognize that that is where they are to implant and do so. It then takes 2 or 3 weeks to see if the transplanted cells start producing blood parts.
Getting someone’s marrow (or stem cells, actually) means I wind up with someone else’s entire immune system. This is why the Graft Vs Host Disease (GVHD) is so common and serious, because the job of the immune system is to reject anything that doesn’t belong to it and in this case, nothing in the host will look like it belongs. To prevent the grafted marrow from rejecting the host, drugs are used to suppress immune system. This results in the patient being very susceptible to infections and diseases of all kinds, and for this reason, the 4 to 6 weeks in the hospital are in isolation in a special “clean” room where all visitors are required to wear masks and maybe gloves and other protective coating. Since the GVHD can be a problem for years, even when the patient goes home, precautions must be taken to try to prevent infection, including a mask and gloves when out in public. They say to expect to miss a year of work, although that can be longer or shorter. Working from home will be a possibility.
Other things we found out along the way:
• Dr. P at HUP would probably use stem cells from bone marrow instead of from circulating blood and so would require a bone marrow harvest instead of a stem cell harvest from the blood. Dr. T at T/FCCC would probably use stem cells from the peripheral blood. While there are some indications that the latter can include a higher incidence of chronic GVHD, Dr. T feels there are advantages that outweigh that risk, and Dr. P says it’s a minor detail and that higher risk of chronic GVHD may not be all bad.
• Dr. T would do a “mini” bone marrow transplant (bone marrow transplant or BMT is a term that seems universally to include stem cell transplants). He says that recent studies have shown it to be just as effective with fewer and less severe side effects because of the reduced level of chemotherapy. A mini BMT means they don’t kill all of the bone marrow, but leave some bad cells to be cleaned up by the transplanted immune system.
• More GVHD may not be all bad because it means the immune system is doing its job in fighting foreign entities. If it weren’t doing that well, it also wouldn’t be as effective in getting rid of the diseased marrow. In fact, I saw some statistics that show that if the donor is an identical twin (syngeneic transplant) where the marrow and stem cells are essentially identical, the incidence of recurrence of the disease can be 3 times higher than with a matched allogeneic transplant.
• There are not a lot of restrictions for visitations in the hospital. They don’t want overnight stays or sick people, but that’s about it.
• Dr. T is concerned about my pilonidal cyst and thinks it should be excised before a BMT, but I don’t think he realized how extensive the surgery is and that the recovery could be 6 weeks or more. I’m not sure I can afford that kind of delay. He will consult with my hematologist and pilonidal surgeon about that.
• Dr. T also thinks that the scaling on my ears is a fungal infection and he wants me to see a dermatologist about getting it treated before the BMT.
• Males receiving female stem cells seem to have more problems than others, but I don’t know if that will affect my sister’s candidacy
• The transplant recipient ends up with the unsuspecting donor’s DNA, at least in the blood and maybe other bodily fluids. This means I could wreak criminal havoc with impunity because the DNA is not mine.
The Transplant Becomes a Bit More Urgent
So, on June 10, everything had changed. It looked like a bone marrow transplant was going to be needed in the following 6 months or so. My hemoglobin count fell below 10 for the first time (it went from 11.1 on 6/1 to 9.8 on 6/10), my WBC was at .9, and my platelet count had actually risen to 60 from 38. Two weeks later, on June 24, I had another CBC and the counts had all dropped to their lowest ever – WBC at .8, platelets at 30, and hemoglobin at 9.5.
My hematologist, Dr. H was clearly a bit alarmed at the apparent acceleration in the decline of the blood counts, and said he would like to push or prepone (it’s an actual word meaning to schedule for an earlier time as opposed to postpone) my transplant to happen by the end of July. This was hard to hear at first. While I knew a bone marrow transplant was in the near future, facing it in only weeks made it a lot more real. After chewing on it for a while, I realized that sooner was better than later for a lot of reasons. First, there’s less waiting. Also, for my wife who teaches fourth grade and my son who’s planning to go into the Peace Corps in January, an earlier transplant means that by the time school starts and certainly by January, I should be home from the hospital and we’ll have some idea of how well the transplant took.
Dr. H said there are two places in the Philadelphia area where bone marrow transplants are done – at the Hospital of the University of Pennsylvania (HUP) in downtown Philadelphia and at the Temple – Fox Chase Cancer Center (FCCC) in Northeast Philadelphia. He says he has worked with both places and they are both good. I decided that I wanted to visit and have initial consultations with both places so I could make a choice based on something, so I set up appointments for the following Monday and Tuesday (6/28 and 29).
Myelodysplastic Syndrome (MDS) is a disease of older people. Most people are over 70 years old when they contract the disease and approximately 90% of those who get the disease are 60 or older. This is significant because the only treatment known that can cure it is a bone marrow transplant and these are rarely done in people over 55 years old. I am 52 years old, so I’m still a candidate.
I wondered whether my disease is considered cancer since I have a malignant growth of cells, and if it is not cancer, why is it not. Dr. H explained that cancer involves tumors of some sort. Leukemia is not a cancer. It has something to do with body parts that constantly renew themselves (like blood) and those that don’t (like lungs, bones, breasts, prostates, etc.). By definition, I don’t have leukemia because the blasts in the marrow have to be 15% to be leukemia, and mine are at 5%. However, it’s just a definition – my blasts would increase over time to be 15% and then I would have developed an Acute Myeloid Leukemia (AML) that is very difficult to treat.
While concerned about my blood counts, Dr. H is positive about the BMT. He says I’m young and otherwise healthy (some people with MDS have had chronic infections, illnesses, and transfusions that have already wracked their bodies by the time they submit to a BMT), and that with a matched allogeneic (from a sibling) transplant, which we expect to get, the odds are good. Understand that by good he means greater than 50%, which is certainly not great, but it’s good enough for me. I feel like with a positive approach, good medical team, and the love and prayers of family and friends, it’s in the bag.
A Brief History
For years, I had been doing platelet pheresis donations. This started when we lived in Delaware and for more 3 years, I did about 5 donations a year at the Blood Bank of Delaware. After moving to the Philadelphia area, it took me a while to get set up to do donations at the Red Cross here. In November of 2000, I did my second or third platelet donation at the Red Cross, after which they told me I couldn't do any more donations until I had gotten my counts checked which I did right away.
For the next 6 months or so, I tried to get information from the Red Cross about what my problem was until they finally said I should go see my doctor. I went to my primary care physician and he said I did have a low platelet count and I should see a hematologist. I finally got to my hematologist (I’ll call him Dr. H) in July of 2001 at which time my hemoglobin (part of the red cell count) was 12.8 which was low normal, my white blood count (WBC) was 1.7 thousand (should be in the range of 4.5 to 10.5), and my platelet count was 103 thousand (should be in the range of 200 to 450).
After that, I saw Dr. H every 4 to 6 weeks for a complete blood count (CBC). My hemoglobin was pretty steady, declining slightly until December of 2003, when it was at 11.9. In that same period, my WBC and platelet count declined steadily, reaching 1.0 and 52 respectively. Between December of 2003 and May of 2004, the counts were at 10.5 (hemoglobin), 1.0 (WBC), and 37 (platelet). During this entire time, I felt fine and was completely symptom free. Dr. H was clearly most concerned about my white blood count because the white blood cells are the ones that fight disease and infection. He told me if I ever had a fever of 100.5 or higher, I should call and maybe get admitted to a hospital because the white blood cells could easily get overwhelmed. For 3 years, I never had to call and had only 2 brief treatments to see if they had any effect (folic acid and vitamin B-12).
He also did a bone marrow biopsy and aspiration in July of 2001 and every summer after that. From the first 3, Dr. H could make no definitive diagnosis, and said his best guess was that I was developing a Myelodysplastic Syndrome (MDS). He also indicated that it was possible that I would spontaneously remit.
In May of 2004, things changed. On May 27, I had my fourth bone marrow biopsy and since the results take 2 or 3 weeks to come in, I set up an appointment with my hematologist to follow up on June 24 (my wife and I were vacationing at the Jersey shore during the week of June 12 – 19). On Memorial Day, May 31, I felt pretty bad and at 11:30 PM, I found I had a fever of 101.5, so I called the hematologist’s office. When the doctor on call discovered that I had no other symptoms than a fever (like coughing, etc.), she said I didn’t need to come in, which was a relief to me.
The next day (Tuesday), I called Dr. H and told him about the fever (it had subsided a bit by morning) and that I was quite sore in my coccyx area. I didn’t think it was from the bone marrow biopsy (which was also in that area) because I could see the puncture and it looked fine. My best guess was that I was bruised following a fall while cleaning up after a wedding. He saw me that day and said he thought I might have an infected pilonidal cyst, so he put me on an antibiotic, sent me to a surgeon, and set me up for another appointment on June 10. On Wednesday, I saw the surgeon who confirmed that I had an infected pilonidal cyst and he drained it, leaving the cyst there. If you want to know a bit more about pilonidal cysts (and be warned, you may not), click on this link for pilonidal cyst .
On Thursday, June 10, I went back to Dr. H, thinking I was getting another CBC (complete blood count) and a check on my infection. What I didn’t expect and got was the results of my bone marrow biopsy which showed that I definitely had MDS and that my blood counts were falling fairly quickly. He told me that I needed a bone marrow transplant in the near future. While I knew somewhere inside that I might get this news, it still hit me pretty hard because I knew that while the bone marrow transplant was my best option, it is a very risky procedure.
Here ends the brief history. I will follow with more details and more recent history in subsequent posts.
