Susan's Recovery Chronicle: 2012

Monday, November 5, 2012

3 YEARS, 5 MONTHS, 4 DAYS: Goin' Pretty Good

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Top Line: Chronic GVHD persists, but MAC is vanquished, meds are beginning their downward adjustment, and Susan feels mostly decent most of the time.

It was two weeks ago (10/22) that Susan made her most recent Boston pilgrimage. Life (no excuse) and Hurricane Sandy (subject and blogger both without power) conspired to delay this posting, but now, here it is. Had there been truly urgent news, I would have found a way. Fortunately, the ship of recovery continues to sail on an even keel.

Susan and Bob actually stayed over in Boston that Sunday night, given a 10:30 start of appointments and a very packed day. The rounds began for her at MEEI (Mass Eye & Ear Infirmary, but she only needed the “eye” part), where she had blood drawn to centrifuge into the serum base for her designer eye drops. These are the ones that have to be kept refrigerated, so some attention to logistics is required, but they really do provide relief. One of the minor impacts among so many horrendous ones attributable to Sandy, is that the drops, although ready, are being held frozen up in Boston until the Siegels are confident that UPS is again delivering normally without risk of delay (and spoiling of the frozen drops).

Then it was over to Dana Farber and phlebotomy for more blood. She wasn’t able to make it there in time for her 11:30 slot, so she lost her place in the queue, had to wait some, and the domino effect scrunched her subsequent appointments. She was late for her 12:30 with Dr. Soiffer, bumping up against his 1PM commitment, with blood work results just beginning to trickle in (wordplay – blood/trickle – intended, as always).

Soiffer was pleased with how Susan looked upon examination, but their meeting was abbreviated and the information available to Soiffer was incomplete. From what he could see, the numbers were decent except for some anomaly on kidney function, which will have to be monitored. Susan’s to check it again in NYC in a month (now, two weeks).

The good news outcome here was a net reduction in medications: based on good liver function levels, one liver med was eliminated altogether (although another was increased some), and her prednisone dosage was reduced from 10 to 7.5 mg. And in the good news vein, note, it had been 4 months since her last DFCI visit, and her next visit will be scheduled for about 3 months out. Remember when she was going up every week, and then every 2 weeks, and so on. This is goodness.

Next, it was over to Brigham & Women’s Hospital to see Dr. Triester, the oral oncologist. If you remember, in the previous blog posting, we mentioned that Susan was afflicted with a verruciform xanthoma, an uncommon, but not unheard of, fellow traveler with GVHD. This visit Dr. Triester removed it. The biopsy results have come back negative. Good. Susan is now decidedly more comfortable in the oral region, tolerating a wider variety of foods, and eating with a better appetitie. Also good.

By the way, and to round out the day, she also received her flu shot while she was in Boston. Remember George Burns, who famously said (to paraphrase): At my age, when I bend down to tie my shoes, I look around to see if there’s anything else I can do while I’m down there.

Finally, and unrelated to Boston or Susan’s recovery, she has developed a squamous cell carcinoma on her face. Unlike a melanoma, a squamous cell carcinoma is usually not a cause for concern if it’s caught and attended to early. She’s been planning to have it removed and biopsied here in NYC. However, in yet another instance of Sandy’s reach, the NYU Medical Center Hospital, out of which her dermatologist works, has been shut down temporarily, postponing Susan’s attention to this matter. Of course, she’ll reschedule for the earliest possible opportunity.

Check back in about 3 months, folks for the next status update.

Wednesday, July 11, 2012

Happy Anniversary 3.1.09

Top Line: with the MAC brought under control, meds levels readjustment led to a rocky GVHD patch, but the protocol has finally been tuned to where Susan is feeling decent.

First, two Dana Farber visits and a big anniversary have slipped by without having been blogged. Mea culpa. Mea maxima culpa. If I were my sister, I’d dock my pay and put me on notice. I’ll try to be even more wordy than usual in this posting to compensate for the shortfall.

So, what did you miss while the blogmeister was asleep at the switch? Disregarding chronological order, let’s celebrate (a day late, a dollar short, and after the fact) the third anniversary of T-Day. Don your party hats and grab your noisemakers. Now, look silly and make noise in recognition of this milestone. The further in time Susan is removed from the date of her transplant procedure, the more auspicious it is for her long-term health, chronic GVHD notwithstanding.

Now speaking of GVHD, you may remember that Susan had developed MAC (mycobacterium avium complex – say it fast five times) a tuberculosis-like disease, for which she was being treated with a drug protocol that interacted in somewhat unpredictable ways with her usual meds regimen, requiring a bit of trial and error to get all the factors tuned properly.

More specifically, doses of her two major GVHD medications – prednisone and tacrolimus – had to be roughly doubled during her treatment for the MAC attack. When she was pronounced ready to discontinue the MAC stuff, her docs reduced her other meds back to more or less pre-MAC levels, with the intention to reduce dosages further over time as her condition allowed. For those concerned with the timeline, this was the first unblogged DFCI visit and took place a couple/few months back.

Then came the anniversary. But even before the anniversary, Susan was not doing well. Resurgent GVHD, manifested primarily in the mouth, eyes, and skin, was making her most uncomfortable. Jump forward to about 3 weeks ago when Susan next went up to Dana Farber, and the decision was made to double the dosages for prednisone and tacrolimus. This has brought her back to higher levels than Dr. Soiffer would like, since the goal is to be gradually weaning her off this stuff, but it has also brought significant improvement in how she feels.

The current thinking is that as her body acclimates to all the changes in medication protocols she’s been through, she will be able to resume the path of medication reduction. The GVHD will, we hope, quiesce, even as the number and dosage levels of the drugs she takes are reduced. Do keep in mind that it is “chronic” GVHD, so she will continue to be subject to flareups. However, these generally diminish in frequency and severity as time passes (no guarantees, of course), and that’s why the Third Anniversary is a big deal.

One more thing: Part of Susan’s travails with the state of her mouth have to do, not with GVHD per se, but with the growth of a lesion called verruciform xanthoma. This is an uncommon, but not unheard of, fellow traveler with GVHD and is usually benign, though it can become quite uncomfortable. Dr. Treister (remember him, the guy who handles oral issues for transplant patients?) has decided to excise the lesion (and, of course, biopsy it) in an upcoming visit that Susan is working on scheduling.

More to follow when there’s more to say.

P.S. In case you haven’t figured it out (I know I wouldn’t have), the perhaps obscure 3.1.09 in this blog entry’s heading -- “Happy Anniversary 3.1.09” – indicates that it is 3 years, 1 month and 9 days after the June 2, 2009 transplant procedure.

Sunday, January 15, 2012

TWO YEARS + 217 DAYS: Looking Better

Top Line: Susan's docs all feel she is making good progress

The most recent Dana Farber visit took place on Wednesday, January 11th. Susan saw Dr. Soiffer, her transplant case lead, as well as Drs. Marty and Dana for her MAC and eye condition respectively.

Let's keep it short and simple this time. All three felt Susan is coming along nicely. The main issue is when Susan can be weaned off of the MAC medications. Dr. Marty thinks this can happen in about 3 months. It is important because those medications have an uncertain interplay with Susan's other drug regimens (esp. the anti-rejection and GVHD meds), which themselves may now be candidates for reduction, but not until the MAC issues are removed from the equation so that her underlying, MAC-free status can be assessed.

It would certainly be nice to go from 30 to 3 pills a day. Those are made-up numbers, but you get the point: as her medication protocol can be reduced it is (1) indicative of real progress, and (2) makes daily living simpler and less difficult.

So, objectively, the measures that the doctors look at and their physical examination of Susan all indicate an improving condition, and subjectively, Susan is feeling somewhat better and less tired than she had been. Don't mistake this for normalcy yet, but we'll take it. Check back in a month for the next update.

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

Monday, November 5, 2012

3 YEARS, 5 MONTHS, 4 DAYS: Goin' Pretty Good

Wednesday, July 11, 2012

Happy Anniversary 3.1.09

Sunday, January 15, 2012

TWO YEARS + 217 DAYS: Looking Better

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

Followers

Blog Archive

Susan's disease

Susan's Transplant Procedure

Blood and Marrow Stem Cell Transplantation - a pamphlet