Susan's Recovery Chronicle: October 2009

Tuesday, October 27, 2009

DAY +145: a Bit of Chop in the Water

Top Line: Susan is likely experiencing some GVHD, and it's being treated

Dr. Soiffer was able to see Susan's results today and to confer back and forth with Nurse Amy, who did see Susan yesterday. Here is the summary:

The good news -- the flow cytometry test, performed because Susan's neutracils and lymphocytes are out of balance, revealed no, repeat NO, CLL cells; in other words, the transplant is working
The other news -- Susan's persisting body rashes and facial blotching, along with a white coating in the mouth, are indicative of GVHD (Graft vs. Host Disease); normally this would be treated systemically with steroids, but see below
Also, Susan's liver enzymes are elevated again, which at this stage is another indicator of GVHD. She will have to go back on her hated liver pill, the one she believes was most responsible for her nausea; we shall see
Another old nemesis has returned as well -- CMV (cytomegalovirus) -- necessitating the re-introduction of Valcyte to Susan's meds regimen
Steroids, which would be indicated for some of her GVHD symptoms, can increase a proneness to infection, and CMV is an infection, which is why Dr. Soiffer is holding off on the steroids for the time being
Susan will have blood work done in NYC next week, and on the basis of those results the Boston team will decide if and how to adjust treatments
I will report via this blog as soon as we have Dr. Soiffer's assessment and direction, based on the blood work to be done; the date is uncertain at this point (maybe next Tuesday?)
Susan is scheduled to return to Boston in 2 weeks, on November 9, and the results of that visit will be posted late that evening

No, this is not the kind of glowing report we've been accustomed to the past few visits, but remember that GVHD is a common occurence and Susan is being watched closely and treated carefully.

Otherwise, how does Susan feel about being back in NYC and what has she been doing? The answers are "good" and "not much." The sojourn in Spencertown served its purpose very well, but it was time for a change of scene. Bob is now available seven days a week and he doesn't have to do all that driving. People can stop by to help out or just say hello when Susan is up to it. The Siegels have been able to eat out cautiously and sparingly. And Susan got to see her first movie since May -- they went to a 2PM showing of The Invention of Lying. There were four other people in the theatre, which was the idea. Of course, with these new test results, it will be prudent for Susan to curtail her already limited activity somewhat more.

Again, expect updates in a week or so after the next bloodwork, and then on November 9th after Susan's next Dana Farber visit.

Monday, October 26, 2009

"No News is" . . . No News

Susan went up to Dana Farber today, Bob driving, to see Dr. Soiffer. Unfortunately, Dr. Soiffer was out with what may be the flu. Susan did submit to her usual tests, but we need to wait for Dr. Soiffer to interpret the results. I'll post again when we know more. It may be tomorrow, it may be a few days.

Monday, October 5, 2009

DAY +123: Good Blood, Bad Tooth

Top Line: On the oncological front, Susan is progressing well; on the dental front, she's hurting after a tooth extraction today

Dr. Soiffer was not available today, but who needs or wants a doctor when there's a good nurse available. Nurse Amy was pleased with Susan's progress. Here are some details:

All Susan's tests/blood work looked good, except for the liver function, which is slightly out of range and needs to be watched but is not a cause for undue concern at its current level
Everything looking good includes potassium levels. Yea! No more horse pills, although a continued focus on bananas would be prudent
Susan was taken off Valcyte (prescribed to treat/prevent cytomegalovirus) in favor of a milder medication, so she may start to feel a little better on that count alone
Susan's rash, face and body, persists but its intensity and the discomfort it has been producing seem to be somewhat reduced; it is what it is and will be what it will be; continue to treat it with lotions and cremes
Susan is indeed cleared to eat at restaurants, provided they are bad restaurants with few patrons, but she must exercise caution, e.g., no salads, fresh fruits/vegetables (unless peeled/cooked), etc.
The good news/bad news story is that Nurse Amy cleared Susan to start using a toothbrush (about time! you would say if you'd been anywheres near her these past months), but the tooth extraction she underwent following her DFCI visit means it will still be a while before she can exercise her new permission
The next Dana Farber visit (and next planned update of this blog) will be Monday, October 26th

From DFCI, Susan and Bob went to see Dr. Maniscalco in Arlington, MA, in order to have an infected tooth (#30, for the orally fixated) removed. The less said and the less time spent thinking about dentist visits the better (no offense intended to Dr. Maniscalco, who seems to be very good). We hope Susan's recovery from this procedure will be no worse than such things usually are. Time will tell. As of this evening, it hurts.

Yesterday (Sunday) we surprised and delighted Susan. Cousins Leslie and Rudy drove up from Granby, Connecticut to visit. Susan came out on the front porch to greet them upon arrival and noticed a figure in the back seat, whom she assumed to be Leslie's father, Uncle Joe. Imagine her surprise and delight when who emerges from the back seat but Uncle Sonny, just flown up from Florida expressly to see Susan.

With Uncle Sonny there (our mother's older brother, for those who don't know) I could not not be there too, so an hour or so later Maria and I showed up, which was also a complete surprise to Susan. Thank God Susan's heart is almost as strong as her sense of design. With Cici there as well ("babysitting" Susan in Bob's absence, he and Charley then being en route back from visiting his mother in North Carolina), we had a very companionable circle of seven to enjoy the afternoon together.

I mention this because I'm pleased to be able to report that Susan looked good to me, particularly compared to the last two times I had seen her. True, she's lost 40 pounds, but that looks good off her. 41 would be too much, but down 40, she's the svelte little number Bob married back in 1980. Her energy level was decent, her voice was strong, her demeanor was perky. If you assumed she was simply partial to headscarves (or do we have a closet Muslim in the family?) and perhaps had had a bit too much sun, compromising her complexion somewhat, you would not know if you did not know.

The others needed to leave earlier, but Maria and I hung around until Bob got back around 7 last evening, having driven up from the City after the flight up from Charlotte. The poor man's been pushing. And he needs to leave early tomorrow morning for several days of business in the City. Susan's babysitter (she chose that term, not me; in my day, we used to call it a "lady's companion") is going to be Maria, who decided to preempt me in the rotation. She'll drive up tomorrow morning and spend a few days with Susan, shopping, organizing, arranging, re-organizing, shopping some more, re-arranging and generally doing the things those two love to do together, within the limits of Susan's energy. Susan will also get much better cooking out of the deal than she would have with me there.

Finally, while no firm decision has been made yet, Susan will probably return to NYC not too long after next weekend. No subways. Mask and gloves when she ventures forth. But hey, it's Manhattan.

So, all in all, a good report. Look for the next one sometime in the evening of October 26th.

Susan's disease

Chronic Lymphocytic Leukemia (CLL) is a slowly progressive form of lymphocytic leukemia, characterized by an increased number of B lymphocytes, one of 5 types of white cells in the marrow and blood (hence the concern when white cell counts are elevated). The role of B lymphocytes is to produce antibodies to help combat infectious agents such as bacteria and viruses. With CLL, B lymphocytes multiply unchecked and, in the absence of threats to combat, will attack healthy cells and tissue. It is common to experience enlargement of the lymph nodes and spleen as the disease progresses. CLL is the most prevalent form of leukemia and occurs predominantly after age 55, although Susan, ever the precocious one, was 50 when she was diagnosed.

Susan's diagnosis came in December, 1999, when routine bloodwork revealed an abnormally high white count. After evaluating the several world class cancer centers in the US, she settled on Boston's Dana Farber Cancer Institute (DFCI), affiliated with Harvard Medical School.

Susan was fortunate to be introduced by mutual friends to Dr. Lee Nadler, a Senior Vice President at DFCI and Professor of Medicine at Harvard Medical School. Dr. Nadler, who does not normally take on a patient load, agreed to become Susan's primary oncologist and has, over the years, arranged for her to see the appropriate specialists as needed, whether at DFCI or other institutions.

Susan underwent a regimen of chemotherapy from May through September of 2001 which provided her a strong remission lasting until the Spring of 2005, when elevated bloodcounts indicated that additional treatment was required. Tests revealed that the locus of the disease at that point was in her spleen, and she underwent a successful splenectomy operation in August 2005, enabling her to reestablish her remission.

Of course, there's a wrinkle. Over the past couple of years blood sample analyses have shown the presence of both Hodgkins Lymphoma and non-Hodgkins Lymphoma cells, suggesting that the disease has been morphing, muddying the waters as far as a clearcut diagnosis is concerned. There is no label for what Susan has (we are calling it a variant of CLL, for simplicity's sake). However, it doesn't really matter. The course of treatment at this point is the same: a stem cell transplant.

Although Susan has been fortunate enough to be essentially asymptomatic all these years, her white cell counts have gone up as high as 320,000, only to be brought back down to a reasonable level through chemotherapy. To put that number in perspective, a healthy individual's count usually ranges between 4,000-10,000. The fact that she doesn't look sick and generally feels fine does not mean that she doesn't have a serious and progressive illness.

From the beginning, Dr. Nadler had indicated that at some point the disease would advance to where the best course of action would be a transplant procedure, which is dangerous but potentially curative. That point is now.

Susan's Transplant Procedure

RATIONALE for the TRANSPLANT

While the right protocol of chemotherapy can produce a remission in patients with blood cancers, these remissions are almost always temporary, not curative. The number of remissions a particular patient's disease may enable him or her to realize is very limited - sometimes only one, sometimes several - but in general any successive remission(s) is less robust and of shorter duration. The time comes when the best approach is to go for a cure, which is what a successful blood and marrow stem cell transplant can offer. The transplant entails significant risks (as outlined below), but Susan is at the point where the benefits of another remission (if successfully achieved) might be fleeting and leave her less able to tolerate the transplant at some future date.

BLOOD and MARROW STEM CELL TRANSPLANTS: the SHORT COURSE

Blood, despite its homogenous appearance as a red fluid, is actually a very complex medium that contains many different factors, all of which are generated by a person's bone marrow. In a blood cancer like Chronic Lymphocytic Leukemia (CLL) these components of the blood become unbalanced. A person with CLL produces an ever increasing quantity of white cells in the blood over time, particularly B lymphocytes.

White blood cells , including lymphoctyes (B lymphocytes, T lymphocytes, et al.) act as the body's defense against infection and other foreign bodies, and so they naturally increase to combat such threats, but when the threat has been eliminated, the white cell count returns to its normal level. To oversimplify, elevated levels of white cells, pumped out by malfunctioning bone marrow, with no disease or infection to neutralize, begin attacking healthy tissues and systems. This is the case when a person has CLL.

If the bone marrow which produces the white cells can be restored to its proper functioning, then the white cell count will return to normal. A blood and marrow stem cell transplant can achieve this by largely destroying the sick person's marrow through chemotherapy and/or radiation and then replacing it with healthy marrow. To be more accurate, it is not the physical marrow which is destroyed and replaced but rather stem cells within the marrow responsible for the production of red cells, platelets, and the various kinds of white cells, including the lymphocytes. Therefore, the procedure is most accurately called "Blood and Marrow Stem Cell Transplantation" or Stem Cell Transplantation, although it is often referred to loosely as a Bone Marrow Transplant since the diseased marrow is being regenerated via the healthy stem cells of the donor.

There are two basic kinds of Transplants: autologous and allogeneic. In the autologous version, the patient's own stem cells are harvested during a period of strong remission when healthy stem cells predominate. The blood or marrow with the healthy stem cells is then frozen for later use when the disease has returned in force. In the allogeneic version, the healthy stem cells are provided by a donor whose tissue type closely matches that of the recipient. Susan will be having an allogeneic transplant.

In fact, there are two types of allogeneic transplants - the full or standard transplant (myeloablative transplant) and the so-called "mini-transplant" or "reduced intensity transplant" (non-myeloablative transplant). The standard transplant involves very high doses of chemotherapy and/or radiation in the conditioning phase (the week prior to the transplant), which is very hard on the recipient's system. The mini-transplant involves less intensive conditioning and is better tolerated by older patients (roughly 55 years old and up; Susan, like Jack Benny, is 39 . . . for the 22nd time). The standard transplant works by destroying the diseased bone marrow nearly completely, but with very significant stress on the recipient's system. The mini-transplant works by relying on the donor's immune cells, whose efficacy is bolstered by a drug protocol, to destroy the remaining cancer cells. Susan's medical team has determined that the mini-transplant is the most appropriate procedure for her.

The week or so prior to the actual transplant is referred to as the "conditioning phase" and is used to destroy as many of the cancer cells as possible as well as to suppress the patient's immune system to minimize rejection of the donor's cells. This is done via chemotherapy and/or full body irradiation, and can be very taxing. The actual transplant procedure is similar to a blood transfusion but takes several hours to accomplish.

In the immediate post-transplant period, the patient's immune system is severely compromised and he/she needs to be kept in a protected environment (first the hospital and then a kind of half-way house) and closely monitored. Within a month or so of the procedure, normal blood function is re-established to a degree sufficient to allow the patient to return home, but the environment needs to be very carefully managed to minimize infectious agents. Chronic Graft Versus Host Disease (GVHD - see below for "Risks") can be a significant risk for a year or more after the procedure.

RISKS

The conditioning procedure for an allogeneic stem cell transplant may produce a variety of unpleasant side effects, from mild to quite severe, but these are generally manageable and not considered life threatening. Because it involves a less rigorous conditioning, the "mini" version of the allogeneic transplant (the kind Susan is having) generally causes fewer and less difficult complications. However, there are still three major risks to consider with an allogeneic transplant :

1. Disease or infection.
The conditioning process essentially destroys the patient's immune system, leaving him or her virtually defenseless against disease or infection in the pre- and immediate post-transplant period. Therefore, it is extremely important that the patient's environment be carefully controlled to minimize contact with infectious agents. Measures include filtering the air in the room, requiring visitors to wear masks, ensuring frequent and thorough handwashing, eliminating raw foods (e.g., fruits and salads) from the diet, not allowing flowers or plants (which well-wishers might send) into the environment, and so forth. The medical team will also provide the patient with antibiotics and antimicrobial drugs as a preventive measure. It generally takes a month or so for the donor's stem cells to promote formation of enough blood and immune cells in the recipient for him or her to leave the protected environment.

2. Graft Versus Host Disease (GVHD)
The allogeneic transplant works largely through an effect called "graft versus tumor" (GVT), where the donor's lymphocytes identify the patient's cancer cells and destroy them. However, the donor's lymphocytes may also attack the patient's normal cells, which they might recognize as a foreign body and so a threat to be neutralized. When this happens, it is called Graft Versus Host Disease (GVHD), a complication that carries a definite mortality risk. The careful tissue matching that is done to ensure donor and recipient compatibility reduces the likelihood and potential severity of GVHD, but it cannot eliminate the risk.

The more complete the tissue matching (it is never 100%, except with identical twins), the healthier the recipient is going in, and the younger the recipient, the milder the case of GVHD is likely to be. Nevertheless, the majority of recipients will experience some degree of GVHD, despite the prophylactic drugs that the medical team administers.

GVHD occurs in two forms -- "acute" and/or "chronic." The terminology has nothing to do with the degree of severity, but rather refers to the timing. Acute GVHD occurs within the first three months, but its likelihood of occurrence diminishes somewhat with time. Chronic GVHD is what develops at some point after the period for the acute phase has passed. The risk for chronic continues for a year or more after the transplant procedure. A person who has developed and been cured of acute GVHD is still at risk for chronic, as is someone who has been symptom free even a year out.

3. Host Versus Graft Effect
Since the patient's immune system is not entirely destroyed by the conditioning regimen, it's possible for the remaining cells to reject the transplanted donor stem cells, in which case the transplant is unable to gain traction and do its work. Again, careful tissue matching between donor and recipient will reduce the likelihood of this effect to any worrisome degree, but some level of risk remains.

SUSAN'S MEDICAL TEAM

Dr. Lee Nadler - Lead oncologist and case manager - DFCI
Linda Drury - Physician's Assistant to Dr. Nadler - DFCI
Dr. Robert Soiffer - Lead transplant specialist - DFCI
Amy Joyce, Nurse Practitioner for Dr. Soiffer
Dr. Michael Grossbard - Local (NYC) oncologist and case manager - Roosevelt Hospital

Blood and Marrow Stem Cell Transplantation - a pamphlet

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Transplant Pamphlet

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http://www.scribd.com/doc/15894155/Transplant-Pamphlet?secret_password=14dot1c2s1fyv9g8j4k2

Susan's Recovery Chronicle

Tuesday, October 27, 2009

DAY +145: a Bit of Chop in the Water

Monday, October 26, 2009

"No News is" . . . No News

Monday, October 5, 2009

DAY +123: Good Blood, Bad Tooth

Background Information

Leukemia and Lymphoma Society-Home

Dana Farber Cancer Institute - Home

Blood and Marrow Stem Cell Transplantation - a pamphlet

National Bone Marrow Transplant Link

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Susan's disease

Susan's Transplant Procedure

Blood and Marrow Stem Cell Transplantation - a pamphlet