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.

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.

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.

Two Years + 165 Days: On Balance -- So So

Top Line: Susan’s numbers are mostly decent, but the GVHD persists, and she’s anemic

This past Monday, Susan saw Dr. Soiffer at Dana Farber. For a change, he was the only MD on her dance card. Nice not to have had the usual hectic dashing about.

Based on the results of her lab work, Soiffer felt she was “doing good” overall. Of course, that’s his objective, numbers-driven view. Susan’s subjective, how-I-feel-driven view is less rosy (though by no means dismal), as the GVHD-inspired usual suspects (mouth, eyes, skin) continue to loiter in the vicinity. However, she’s certainly not too miserable to get out and about a bit and enjoy some of her customary pastimes (you don’t need to be told what those might be, do you?).

The one significant issue (aside from the GVHD) is that Susan is anemic, and thereon hangs a tale. Her hematocrit and hemoglobin numbers are flirting with the levels where a transfusion would be indicated. However, Dr. Soiffer believes the anemia can be ascribed in large measure to the medications she is taking for her MAC condition (mycobacterium avium complex – as you may remember from some blogs ago . . . the thing that masqueraded as tuberculosis). He’s therefore reluctant to go the transfusion route, preferring to wait and see if Susan can get off the MAC medications by, say, February (that will be Dr. Marty’s call), and if that resolves the problem.

In the meantime, the plan is to boost her blood with iron pills and diet. Chicken liver (which Susan loves in the form of chopped chicken liver, and it’s easy on her impaired eating apparatus) happens to be an especially good source of iron, and spinach is no slouch, so perhaps she can add these to the protein shakes and smoothies she’s consuming for easy-to-ingest nutrition -- think “chicken liver and spinach chocolate protein shake.” Sounds yummy.

On another front, she needs to go back to MEEI (Massuchusetts Eye and Ear Infirmary) the end of the month to provide more blood so they can make more of the special, genetically-tailored, private stock serum for her eyes. Her next regular DFCI visit is scheduled for December 19th.

Two Years + 134 Days: More Good than Bad

Top Line: MAC is improving, mouth is improving (a sign of diminishing GVHD?), but Susan's blood work shows she is anemic.

The most recent visit to Dana Farber was last Wednesday (Oct 12). Dr. Soiffer was not available but Nurse Amy, who is more than qualified, pinch hit for him. Susan also saw Dr. Marty (Mr. Infectious Disease) about her MAC and Dr. Triester about her mouth. Usually, this range of MDs would require dancing around schedules and locations with separate visits to each. In this case, however, the mountains came to Mohammed, with Marty and Triester visiting Dr. Soiffer's examination area where Susan would see Amy. Very convenient and a lot less wear and tear and stress. It's nice to be a VIP. As usual, though, first stop, de rigeur, was Phlebotomy, and then . . .

Regarding lungs: Susan had had a lung scan the week previous to her DFCI visit and the coughing she had been experiencing, now greatly diminished but not gone, turns out to be asthma related. That's a prior condition, and she is responding well to the measures to control it. As for the MAC, it is much improved and Dr. Marty is pleased with her progress. There's no need to adjust the medications, or to see her again until January (barring any unforeseen development).

Regarding mouth: Clinically, Susan's mouth has definitely improved, which Dr. Triester attributes to a likely improvement in her GVHD. However, the mouth doesn't feel better, and that continues to make eating, and therefore adequate nutrition, difficult. The biopsy she had of suspicious tissue on the inside lip/gumline on her previous visit showed no malignancy (good), but rather, an uncommon condition (I can't spell the name; it must be very uncommon) that is sometimes seen with GVHD. It doesn't require treatment at this point and will likely resolve with the diminishment of the GVHD.

Triester speculates that the continuing discomfort may be due in part to this unpronounceable condition, but also to the fact that Susan hasn't had a regular dental checkup/cleaning in two years - that had been put on hold due to the GVHD-inspired state of her mouth and specialized treatments for it - but the progress is such that she is now cleared for a regular checkup and cleaning by a regular dentist. The attention of conventional dentistry may be all she needs at this point to feel as good as she tests.

Regarding recovery: With Dr. Soiffer being unavailable, it fell to Nurse Amy to interpret the morning's blood work and provide the overall assessment. Susan's weight is down, which is not surprising given how uncomfortable it is for her to eat. This substandard intake may be responsible, at least in part, for the most significant reading from the blood work: anemia. Susan's hemoglobin and red count are at levels that would normally indicate a transfusion. However, the anemia may be GVHD driven and without more information, the most appropriate treatment is unclear. Susan is to have some specialized blood tests, which can be done in NYC and the results made available to the Boston team. Once they understand the cause, they will be able to proceed accordingly.

Regarding eyes: There was no need to see Dr. Dana at Massachusetts Eye and Ear. The serum continues to work. Susan's eyes are much improved although certainly not back to normal. Eyes will be added to her dance card for her next visit, scheduled for November 14th (as is the next posting to this blog, although we seem to be running some days late, of late).

Two Years + 105 Days: A Positive Turn

Top Line: Eyes improved, mouth improved (but there’s a but), skin no worse, MAC in check -- maybe GVHD is loosening its hold?

Susan and Bob made yet another pilgrimage last Wednesday to DFCI, the mecca of cancer cure, control, and care. First up (after the obligatory visit to Phlebotomy) was Dr. Villa, to address all things mouth. The good news here is that, based on comparative pictures, Susan’s mouth region looks better overall. This is important because it has been pain in this region that has made it very difficult for Susan to eat, and hence, to get adequate nutrition to promote recovery. The bad news is that there was one problem area – lesions on the gum – that needed exploring, via biopsy and stitches (big and continuing OUCH), to determine whether it is GVHD or something else. Results pending.

Next up was Dr. Soiffer, who pronounced himself satisfied with Susan’s overall condition. Her blood work results came back pretty much in acceptable range for all factors except bilirubin, which was somewhat elevated. Susan has had some swelling in the ankles, and Soiffer has put her on a water pill for this. He wants to see her again in 3 weeks.

Her final visit was with Dr. Dana at the Massachusetts Eye and Ear Infirmary for the usual battery of eye tests and an examination. Dr. Dana noticed, and Susan feels, improvement, such that he doesn’t need to see her again for 2 months, barring any unexpected developments. It seems that the tailored serum (ref. 8/15/11 post) is working – that, or her GVHD is improving, or both. You’ll know that her eyes are working because she actually drove to Hudson last week. Couldn’t have done that (at least not comfortably or safely) back when she couldn’t open her eyes.

She was not able to see Dr. Marty about her MAC condition, but here, she certainly continues to feel relief from the symptoms that led to her diagnosis. Some factors of the blood work done on Wednesday will enable Dr. Marty to determine whether the drug protocol he put her on needs any adjusting. Results there also still pending.

All in all, the improvements noted could mean that her GVHD is subsiding, but before proclaiming that a corner has been turned, let’s see what the story is after her next visit. Now, for a bit of trivia, to suggest how things might have improved: on the way back from Boston, Susan asked Bob to stop in Natick so she could check out a Container Store there – sounds like Susan of old. While in Natick, they happened upon a Jewish deli, where Susan ordered and consumed some kugel and soup (chicken, I presume), in spite of the sutures she’d received earlier in the day. That’s encouraging.

Two Years + 74 Days: Good News and Bad News

Top Line: The MAC is responding well to treatment, but GVHD continues, with skin and mouth about the same and eyes worse

The Siegels were in Boston this past Monday and Tuesday (Aug 8-9) for a round of tests and appointments. On Monday Susan saw Drs. Marty and Soiffer. The good news is that Dr. Marty pronounced himself pleased with Susan’s progress against the MAC attack. As you may remember from previous posts, MAC, or mycobacterium avium complex, an infectious pulmonary disease which in Susan initially masqueraded as tuberculosis, is a serious condition that requires a year-long treatment regimen. Getting the right selection and balance of the 3 (usually) antibiotics used in treatment is challenging to begin with, and generally requires some trial and error and periodic tuning thereafter. In Susan’s case the difficulty is compounded by her post-transplant status and GVHD, with the medications required for that. However, the protocol seems to be working at this point. Susan’s incessant cough is much improved. Even so, Dr. Marty is awaiting the results of certain blood tests to determine clinically whether more adjustment is advisable.

Dr. Soiffer continues to be concerned about the intransigence of the GVHD and the level of discomfort it is causing. Turns out, the MAC medications reduce the effectiveness of the main anti-rejection medication Susan is taking for GVHD, so Dr. Soiffer significantly increased its level. There’s been no noticeable improvement yet, but it’s not even quite a week. Let’s give it time. The good news is that Susan received a decent report card on her usual set of blood tests. It wasn’t “wellness” exactly, but there was nothing alarming.

Susan was also scheduled for an endoscopy late Monday afternoon, this to explore the reasons for difficulty she has swallowing. The procedure was scrubbed at the last moment because of concern about the pain involved, due to the GVHD-inspired condition of Susan’s throat. It may be rescheduled, but only with an anesthesiologist in attendance.

The main event Tuesday was a 5 hour stint at the Massachusetts Eye and Ear Infirmary involving a battery of tests preliminary to seeing Dr. Reza Dana, Director of Cornea and Refractive Surgery. Dr. Dana is the developer of a serum that can be effective in treating conditions like Susan’s. The treatment involves harvesting growth hormones from a patient’s blood as a basis for a serum, applied as eye drops, which can repair cornea damage and promote moister eyes. Susan’s eyes were so dry that she also received tear duct plugs to help keep what moisture she is producing in circulation around the eye. After Dr. Dana, she was sent to M.E.E.I.’s pharmacist for an explanation of how to manage the serum and what to expect. It’s kind of complicated. The serum, which requires frozen storage, should be ready sometime this week, but it can be several weeks after beginning the treatment before any relief is noticeable.

Finally, Susan saw Dr. Lehrman, who was covering for the vacationing Dr. Triester. The good news here is that the yeast infection opportunistically attacking Susan’s mouth (encouraged by the plethora of antibiotics she’s taking) is now much improved. However, the GVHD symptoms persist and she is uncomfortable. Eating continues to be difficult and often painful.

All in all, poor Susan is quite miserable, although some days are better than others. In particular, and ironically, her principal complaint at this point is her eyes, despite (or because of?) the plugs inserted when she was in Boston. She often can open them only with difficulty and pain, so she can’t read or do email and can watch television (or sort of watch) only sporadically and for short stints.

Susan does want you to know that she really appreciates the emails and cards she’s been receiving (as read to her by Bob or Charley) and apologizes that she can’t at this point respond back to you.

As usual, anticipate the next blog posting after her next visit to Dana Farber, scheduled for September 14th.

TWO YEARS + 48 DAYS: about MAC

Top Line: GVHD treatment on autopilot while the docs figure out the MAC attack

Susan an Bob were at Dana Farber yesterday to meet with Dr. Soiffer, her transplant doc and case manager, and with Dr. Francisco Marty, an oncological pulmonologist who is addressing the MAC (mycobacterium avium complex) issue.

The main purpose of the visit was to explain the additional medications Susan must now take and to get an early read on how her body is dealing with and responding to the new meds. At this point there are no issues, but it's early days and too soon to determine how effective this starter protocol will prove. Treating MAC, especially for a recovering transplant patient, is as much art as science, and will require continuous monitoring, primarily via blood work, with the possibility of minor to major adjustments in Susan's drug regimen. Once the right formula is in place (and it could even be this starter protocol), Susan should experience a notable improvement in her cough within 1-2 months. However, the medications will likely need to be continued for a year or more.

Susan is scheduled for a followup assessment in three weeks. At that visit she will also see an opthamologist at Mass General who will administer a serum that may help with Susan's eye problems. Yes, we said that tuning the GVHD treatments was being put on hold, but they must nonetheless be continued in a stable fashion (so as not to interfere with the interpretation and adjustment of the MAC treatments). This particular serum, however, has been determined not to have any potential conflicts with the current GVHD or MAC regimens, so it's worth trying now, given how uncomfortable Susan's eyes are.

Return to this blog in 3 weeks for an update on Susan's next DFCI visit.

TWO YEARS + 40 DAYS: add MAC to GVHD

Top Line: Susan's GVHD remains unchanged but she's now developed MAC, which must be dealt with first


Susan was to have visited Dana Farber (DFCI) today. However, her appointment was moved up to last Friday. Here's why: In addition to her GVHD symptoms, Susan has had a persistent cough for several months now. Her NY internist listened to her lungs and had her get a chest X-Ray, all of which proved negative, but the cough persisted.

She was referred to a pulmonologist who ordered a CT scan, which is more sensitive than an X-Ray. This revealed that she was not being hypochondriacal, but indeed had a lung problem that looked like tuberculosis. Samples were taken for cultures and Susan was put into quarantine, pending results. House arrest and no visitors. This transpired over the 4th of July weekend, of course, which introduced delays in diagnosis and treatment. Essential personnel (Bob and Charley, because they live there), were allowed in, but distances had to be observed (the parties confined to opposite ends of the living space), masks had to be worn, and there was frequent washing of hands, if not gnashing of teeth. The initial test came back positive for TB, but it was not a definitive diagnosis, as other mycobacterial lung conditions could look the same. For certainty, we needed to await the state's TB lab test results, which took a week or so.

While this was happening in NYC, Susan and Bob were in touch with Dr. Soiffer, who doubted it could be TB and mobilized the appropriate Boston docs. He had her come up this past Friday, the day her NY State lab results were due to be available, to meet with the team he'd assembled. Bottom line: it is not TB but MAC (Mycobacterium Avium Complex), also a serious condition requiring a year-long medication regimen, but, unlike TB, it is not contagious.

The three drugs Susan will have to take are being phased in over the next week or so, and the protocol may change based on how well she can tolerate these particular meds, their potential interactions with her other meds, and how effective they prove to be. So, this is far from a walk in the park, but her case is considered to be a mild one, and in and of itself, the MAC isn't making her feel too bad, except for the near constant coughing.

Her GVHD problems persist, manifesting in the mouth, eyes, and skin, but trying new, and potentially more effective treatment for this complex of issues is on hold while her doctors establish and tune the treatment for her MAC. She will be going back up to Boston a week from today (July 18) and I'll report further then.

TWO YEARS + 5 DAYS: GVHD - THE ONGOING SAGA

Top Line: the good news - no lymphoma/leukemia detected; the bad news - Graft vs. Host continues unabated. As for the details, it's complicated

On Monday, June 6th, Susan and Bob travelled to Boston for her most recent examination by Dr. Soiffer. Breaking from tradition, where this blog has provided a relatively detailed account of findings and evaluations, today (almost two weeks after the fact - apologies), you'll see only a high level summary. One reason for this is that the details are complicated and explaining them (if I understood them) would be quite laborious, both to write and to read. Another reason is that what most of you reading this blog (if any of you still are) probably want to know is simply: how is Susan doing?

Here's how: Tests reveal no trace of the cancer. Nor is there any indication that the GVHD has attacked internal organs. That's good. That said, however (and without going over every test result), her "numbers," while not at alarming levels, indicate that Susan is not doing as well statistically as she has over the past year or so. This is doubtless a reflection of the Graft vs. Host problem, which in turn may be a consequence of various medication levels having been reduced over the past half year or so. Dr. Soiffer is adjusting the meds, so let's give that a bit of time to take effect, and we'll see. Speaking of seeing, he also wants to see her in another two weeks, which is increasing the visit frequency back down to one month. At least it's better than every week. Remember those days?

And just as a reminder, and without going into the details, Susan's GVHD manifests itself in unpleasant, uncomfortable, and unsightly ways in her mouth/lips, eyes, and skin. Continuing the topical treatments for these affected areas, along with an increase in the systemically targeted medications, will (we hope) lead to improvement and significantly less discomfort. As for prognosis, nobody can say how long this problem may continue. Frustrating, but that's how it is.

Finally, and very importantly, Susan has been receiving emails and other contacts from old friends and acquaintances with whom she has lost touch over the years but who somehow became aware of her condition. Score another one for social networking media. She wants you all to know that she has been delighted and heartened by receiving word from you, so keep those cards and letters coming. However, due to her fatigue (one blood result showed anemia) and her difficulty focusing her eyes for very long on a computer screen or letter, she has been slow to respond back. She does know that you know and care, she does care that you do, and she's doing her best to get back to everybody, but be patient if she doesn't get back to you promptly.

Next communique should be in a little over two weeks.

ONE YEAR + 314 DAYS: GVHD round 4 - Improving

Top Line: Susan is “doing well” overall, according to Dr. Soiffer, but it’s complicated.

Susan and Bob’s most recent Dana Farber visit was spread over two days (4/11 & 12). Monday began, as usual with a visit to the vampires, followed by an examination by Dr. Soiffer. Here are the salient points:

• clinically Susan is doing well even though she isn’t feeling all that well.
• Blood work results were good; slight elevation in white count but nothing alarming.
• Ascites has definitely diminished.
• With respect to the GVHD symptoms, mouth looks better, eyes about the same, skin continues to be very rashy, but since it’s not too itchy, leave it alone rather than add additional steroidal medications.
• Positive adjustments in medications: prednisone reduced by 33%, pills for ascites reduced from 3 to 2.
• Soiffer recommends, but does not insist upon, trying flaxseed oil for the eyes; Susan is considering.
• At next visit, which will be around the 2 year anniversary, Susan will have a flow cytometry performed. This is a technique for counting and examining microscopic particles (e.g., cells, chromosomes) in the blood and should reveal the status of the “markers” that define Susan’s disease. In other words, it should provide a quantifiable report card on Susan’s condition; however, if the results are not definitive, it may be necessary for Susan to undergo a bone marrow biopsy, which, to understate the case, is not pleasant.

Next, Susan was seen by Dr. Jacobs, her oncological opthamologist.

• Clinically, her eyes seemed about the same as last visit two months ago, although Susan feels they hurt a bit more. Dr. Jacobs did comment that Susan’s case was far from the worst she has seen and that the chronic GVHD which Susan is currently experiencing at a relatively mild and non-worsening level (as manifested in eyes, mouth, and skin) is actually an encouraging sign.
• Some background: the discomfort is due in large measure to dry eyes, to combat which opthamologists often insert plugs to block the tear ducts, thus keeping the eyes moist. There are four tear ducts altogether – upper and lower for each eye.
• Dr. Seedor, a NYC opthamologist working with Dr. Jacobs, inserted one plug (of a possible four) about two weeks ago as a “proof of concept.”
• When Dr. Jacobs examined Susan, she determined that the plug had come out (as can sometimes happen).
• Dr. Jacobs decided to insert 3-day dissolving plugs in all four tear ducts to judge whether this treatment was providing relief, and how much (to be determined by how Susan feels with the plugs vs. after the plugs have dissolved in three days). If the plugs turn out to be effective, then Jacobs and Seedor will confer about Seedor putting in four permanent plugs.
• Dr. Jacobs also gave Susan a sample of Lotemax, a prescription medication for dry eyes. Susan’s eyes are feeling better today, but it’s impossible to determine how much of the improvement is due to the Lotemax vs. the temporary plugs. After the plugs dissolve (which should be in another day or two) it will be possible to evaluate the effect of the Lotemax, as well as to determine whether to go ahead with permanent tear duct plugs.

On Tuesday Susan saw Dr. Treister for her mouth:

• Dr. Treister definitely felt that the condition of Susan’s mouth had improved since the last visit two months ago.
• He is having her continue with the mouth rinse she started as part of the clinical trial (see blog entries for 11/30/10 and 1/19/11), but at a reduced frequency, which is good news.
• However, there is no apparent improvement in the condition of the lips. Susan has found that OTC lip balms provide as much topical relief as does the medication Dr. Treister prescribed. He is switching her to a new, steroid-based medication which she is to use 2X/day while she may continue to employ OTC balms as desired.

The net: Susan’s “vital signs” with respect to the disease are good and her GVHD, which is at worst a moderate case, is stable to improving. We’ll take it. The next DFCI visit is scheduled for June 6th, just after the two year anniversary of the transplant procedure. You‘ll be able to read all about it here in the blog shortly thereafter.

ONE YEAR + 260 DAYS: GVHD round three

We interrupt this blog to bring you a just-breaking request from its subject, Susan.

In addition to friends and family with whom she is otherwise in touch, Susan has, with surprise and pleasure, gratefully received comments posted to the blog from people lost in her past and even a few total strangers. She would like to acknowledge this support but the blog does not require or capture your contact information. If and when you post comments, please feel free to include a contact email address or to reach out to Susan directly at SusanKargman@gmail.com. Thank you.

We now return to our irregularly scheduled blog posting.

Top Line: chronic GVHD persists (which is why they call it "chronic") as manifested in eyes, mouth, and skin, but it's not worsening.

While Monday offered Valentine's Day pleasures for most of us, for Susan and Bob it was another Boston grind: Dr. Soiffer, of course (preceded by the de rigeur pitstop at Phlebotomy so Soiffer would have numbers to cluck over). Also Dr. Triester for her mouth and Dr. Jacobs for her eyes. Lots of running around. At least the day was spiced up by the fact that the oncology people are now in their spanking new state-of-the-art digs, so there was the novelty of unfamiliar, and jazzy, surroundings. By the way, keep in mind that Boston's not all of it -- she regularly sees an internist and dermatologist in NYC whose observations and treatments need to be melded with those of the Boston docs.

Add it all up, and here's what's going on, in Susan's own words:
"Count: 22 pills (although one went down in dosage this visit) PLUS various drops, creams gels for mouth and eyes. Fun regimen."

But let's keep the long view in mind. Progress is being made, as a 9/11/10 photo of this glamorous woman suggests

Now, for the rundown of Monday's visit . . .

1. the SOIFFER segment: Susan's blood work was good, which is good, so they talked about the fact that chronic GVHD, even this far out, is not that unusual. It's impossible to predict how long it will last, but it could be many months, or it could be less (it's been, what?, three or four so far). It can't be "cured." It will run whatever its course turns out to be. However, it can be managed symptomatically (hence the doctors for eyes, mouth, and skin), with several of the indicated treatments being steroid based.

That introduces it's own set of problems, as steroids are not benign medications. It's a fine balancing act to provide enough steroid-based treatments, coordinated across several battlefronts (eyes, mouth, skin) to promote comfort and healing while limiting the complications which steroids entail. In fact, each specialist Susan is seeing would like to give her even more steroids but they can't, because in the aggregate she's at the fine edge between more harm than good. One of the side effects of these steroids is difficulty sleeping. File that thought.

The new news is that (1) Susan has an upper respiratory infection, which her internist is treating with antibiotics, but she feels kind of rotten as a result. And (2) Susan has not been sleeping. She either can't sleep so she stays up until 3, 4, or 5 in the morning, or she does manage to fall asleep earlier, say 11 PM or midnight, but then wakes up at 2 and can't get back to sleep. That makes her feel kind of rotten, too. Add in the GVHD, and you get the picture. Of course, adequate sleep is important to her recovery from GVHD. Dr. Soiffer prescribed a new sleep medication. We'll see how effective it is.

2. the TRIESTER segment: The condition of Susan's mouth is about the same to slightly improved, but Dr. Triester observed what might be a yeast infection so he has added an anti-fungal medication to the rinses and creams she is already using for her mouth and lips.

3. the JACOBS segment: there appears to be some modest improvement in Susan's eyes.

On the dermatological front, while she is still rashy, she is not itching as much as she once was. So, she may be a little less uncomfortable with respect to GVHD symptoms, but we don't know, because the upper respiratory infection and sleep deprivation are masking how she would otherwise feel. As the antibiotics and the new sleeping pills do their job, We think and hope that, with respect to GVHD, she'll feel somewhat better.

Dr. Soiffer's scheduled her next visit for April 11th, almost two months out. I take that as a good sign. Tune in then for the next update,

ONE YEAR + 232 DAYS: GVHD round two, continued

Top Line: Susan’s been experiencing some GVHD for a few months now, but it’s gradually improving.

Susan and Bob did the Boston thing two weeks ago (Jan 5), preceded by a dermatology visit in NYC on Jan 4 as part of the same cluster of care. (Apologies for this belated report.)

Here’s the skinny:
Susan is continuing to experience a return of Graft vs. Host, as described in the November 30 post. You may remember that this is a common occurrence in transplant patients. In its severe form, which tends to happen earlier in the transplant experience, it can be very dangerous. When it occurs this long after the procedure it is less likely to be life threatening, but it can be quite uncomfortable. However, it is also an indication that the transplant is working, which is good.

Without being specific, because the details get icky, Susan’s GVH is manifesting in her mouth and eyes as well as in pretty extensive rashing, particularly around the torso. Rest assured, Susan’s condition is being followed assiduously by her doctors, Dr. Latkowski for her skin, Dr. Jacobs for her eyes, Dr. Triester for her mouth, and, of course, her primary transplant oncologist, Dr. Soiffer. The good news is that the various symptoms seem to have peaked and are finally starting to abate. The study being conducted by Dr. Triester, the one where Susan was given one of two medications currently under review, has concluded and she is now being given both medications, along with topical salves for her lips. Otherwise, it’s various steroidal products along with a number of lotions and ointments for eyes and body rash.

As those of you who are in closer touch with Susan will know, the GVHD has slowed her down, as she oscillates between uncomfortable and very uncomfortable, but it has not stopped her. She’s not always up to it, but she does get out some to do the usual things she likes to do (i.e., shop), although she does need to be very careful with what she eats – Bob’s been enlisted as her taster – as anything at all spicy and even certain unspecified ingredients that you or I might not notice can cause real distress. But she’s coping . . . and, I think, gradually improving.

I promise to blog more promptly after her next DFCI visit.

ONE YEAR + 182 DAYS: Let Us Not Be Rash

Top Line: a Touch of GVH, manifesting as rash, but not serious

It wasn't bound to happen, but it's not surprising that it has. And don't worry. We've got your back. Also your eyes, mouth, and front. That's the official assessment from the good Dr. Soiffer, per Susan's visit to DFCI yesterday. In short, she has developed a mild to moderate case of GVH (graft vs. host), which has manifested as a body rash, mouth sores, and inflamed and glassy eyes . In case you don't remember what GVHD is, you can scroll down to the bottom of the blog where you'll find a description of the disease, the transplant procedure, and the risk factors in doing the transplant.

The condition isn't fun, but it isn't debilitating. Maria and I visited with Susan and Bob this past Saturday in Spencertown, and she was up to hosting a small family gathering (Leslie, Rudy, and John Henri Richardson, Nate and Callie, Charley Siegel, and us). With her body covered, as it was, you wouldn't have known.

That said, the condition has to be treated and watched. So, here's the summary from the DFCI visit: blood work results were basically good, except for a slight elevation in the liver numbers. Dr. Soiffer had Susan see an oral oncologist on staff, who prescribed a lip cream and is deciding between one of two different mouth rinses that are currently being evaluated in a clinical study. Susan will need to be enrolled in the study in order to receive the treatment, and that registration (which may require another trip to Boston next week) is currently in process. The good news about this study is that it does not compare the treatment to a placebo but rather pits the two medications against each other, head to head. So, Susan will be getting something, whatever it is, that is expected to work.

For her eyes, Susan still needs to see an oncological opthamologist (which will happen at DFCI if she returns next week, otherwise in NYC), and for her body rash, her own dermatologist (who did an oncology rotation at DFCI). So, there are still open issues, but a plan is in place to address them. In the meantime, please realize that while her condition needs to be treated and followed, it is not considered life threatening. She is uncomfortable but not miserable. It is not the "yechy-ness" of old.

Susan will need to return to DFCI in about a month as part of the study to assess the efficacy of whichever mouth rinse is prescribed for her. Hopefully by then the rash, and the GVH, will have subsided, if not disappeared entirely. You can read about it here when it happens.

ONE YEAR + 125 DAYS: Looking Good

Top Line: Progressing Nicely

Susan, accompanied by her ever faithful chauffeur and spouse, Bob, was in Boston yesterday to see Dr. Rob Soiffer, her ever faithful oncological transplant specialist. The tests run indicated no issues worth mentioning, Susan's outward appearance and her self-assessed condition were good, and Dr. S. pronounced himself satisfied. He swapped out one medication for a milder variant and indicated that he planned, over time, to wean Susan off most of the drugs she currently takes. A good marker for her progress is that Dr. Soiffer scheduled her next appointment for two months out. Remember back when it was every week?

As for this blog, is anyone still out there? At this point, the blog's audience, if any remnant of it remains, realizes that Susan has been doing well these last several months. Accordingly, blog entries are becoming briefer and more widely spaced. Soon they may become so brief and so widely spaced in time that they will, for all practical purposes, cease to exist, and that will be a good thing. Nevertheless, Susan's ever faithful blogster will remain at his post blogging until there is absolutely nothing left to blog because Susan has been pronounced officially "cured." And if these posts have already become, or will so become soon, something akin to the problematic sound or lack thereof of the proverbial tree falling in the forest with no one to hear (or, in this case, see), so be it. . . .

. . . all of which is to say, in typically long-winded fashion: "back in two months with the next update, whether you tune in to read it or not."

ONE YEAR + 55 DAYS: Coming Along

Short and Sweet: Susan had a good visit with Dr. Soiffer yesterday. There are a few test results that were not available on a same day basis, but what lab work did come back was decent. From a quantitative perspective, Susan is doing well at this point, and those of you who have seen or talked to her recently know that in general she's been feeling pretty good.

As you may remember, the further out from the transplant Susan gets, the less likely it is that she will have complications or setbacks. So what's happening (or not happening) now is goodness. However, while increasingly less likely, problems can still arise, so the appropriate position is "guardedly optimistic."

DAY +365: Happy Anniversary!

Top Line: a good report at the 1 year mark

Hard to believe, but it was a year ago today that Susan received her transplant. For those fond of strolling down Memory Lane, I have dug into the archives to append the blog from that day (see below). As for today, here's how things stand:

Susan was to have gone to Boston yesterday for a liver biopsy, among other things, but she "called in sick" with a sore throat, so those procedures were postponed. By last night she was feeling better and today Susan and Bob drove up for her scheduled appointment with Dr. Soiffer. Her consultation came after the usual round of vital-signs-taking and blood tests, all the results of which are not yet available, but based upon what Soiffer did see, he pronounced her "doing very well at this point," especially given her rocky history over the past year.

To be more specific: those test results that were available by this afternoon were all good, including those that indicate how the liver is doing (e.g., bilirubin). The liver, as a potential locus for GVHD, has been, you'll remember, an ongoing concern, with a secondary manifestation of severe ascites. One of the rationales for the liver biopsy scheduled for yesterday was continuing ascites. I'm delighted to report that the ascites is now so diminished that Soiffer doesn't see a need for the liver biopsy.

The most interesting aspect of the day was the spate of re-vaccinations which Susan needed to have administered. A year ago, when she lost her marrow, she lost her vaccine-provided immunities. Today, like an infant, she was at the point where she was ready for her standard set of childhood vaccinations. Happy Anniversary and Happy Birthday, Susan!

As those of you who have had recent contact with Susan outside of this blog will know, after what seemed an interminable period of halting and uncertain progress, with two fair-to-good days followed by a really Y**** day, Susan has strung together an impressive number of good days. While she appears painfully thin, or fashionably slender, she has actually gained back 4 pounds recently and is now eating with a pretty good appetite. Woohoo! It's a far cry from cheering when she managed to down half of a grilled cheese sandwich.

The one year mark is often thought of as a watershed (informally, of course, as there's no clinical, or magical, boundary that separates day 364 from 366). But transplant patients who make it this far are seen as moving into clearer, less dangerous waters. No guarantees (when ever are there) but the progress is indisputable.

We'll wait, of course, for the remaining test results, but absent surprises, Susan's free to cruise along unmonitored until her next DFCI visit on July 9th. Until then . . .

Tuesday, June 2, 2009

DAY ZERO: Out with the Bad Cells, In with the Good

The donor stem cell's, now Susan's (top)
Susan and Nurse Karen doing nothing (left)

The deed is done. The infusion began at approximately 10:15 today and was finished about an hour and a half later - turned out to be briefer than expected. And yes, for the major inflection point in Susan's disease, it was an underwhelming event. Nurse Karen simply hooked up the bag with the stem cells (the red bag in the picture above) and that was it. No drumrolls, no fireworks display, just a steady drip like any other coming from a bag through an IV line. If Susan did not know it was happening, she wouldn't have been able to tell.

Susan's team of 5 doctors came in at one point during the procedure, nodded sagely, clucked a bit, squabbled mildly and made up (just to show they were on the job), and then left without doing anything or offering any useful information or advice. Dr. Soifer (Susan's lead transplant specialist) stopped in a bit later to say hello and said everything was looking good.

Susan has no appetite but Nurse Karen says that's par for the course. I'll continue to nag her about her applesauce and fluids. Otherwise, she definitely looks better than she did yesterday and feels somewhat better - if she were eating and drinking, she'd feel better still, but that's a fine balancing act between nutrition and nausea.

Susan's room is a little like Grand Central - people are constantly popping in and out. In just the past hour we've had Nurse Karen bearing ice chips, Carol the Patient Care Coordinator with documents to sign, and Tammy the Social Worker for no discernible reason, but she was very sweet and she did leave her card.

And finally, Susan says "Hi. I'm glad it's over, but the real part is just starting." Couldn't coax more of a statement from her for the record at this point, but she says she may have more to say later, so stay tuned.

DAY +314: Still Working to Understand the Issue

Top Line: Internal bleeding (now resolved) has delayed plan

Susan and Bob went up to Dana Farber Monday, where they expected to meet with the gastroenterologist to get to the bottom of the ascites/liver issue. Susan was looking and feeling a bit peaked, though, and the blood work (which is always done first upon arrival) revealed a significantly depressed hematocrit, a sign of internal bleeding. Dealing with this put all other plans on hold.

An endoscopy was performed and it revealed bleeding ulcers, both esophogeal and gastric. Susan was hospitalized, put on Nexium intravenously, and given 4 units of blood. Today she was feeling much better -- nothing like a jolt of fresh blood to perk a body up -- and actually ate a good breakfast.

This afternoon they performed a needle biopsy to get a sample of the ascites fluid. The doctors are going to consider the results of the various samples they have taken and bring that information into an examination and consult on April 26th with the gastroenterologist being tasked to get to the bottom of Susan's condition. Paracentesis to drain the ascites fluid is also a likely treatment item when she returns on the 26th. As I write, she should be in the process of being released to return home to NYC, but with a number of adjusted and new medications.

Tune back in late on April 26th, when I expect to be blogging the results of Susan's next DFCI visit.

DAY +306: Working to Understand the Issue

Top Line: No alarming outcomes but the persistant ascites an issue

The good news is that there was no bad news resulting from Susan's visit to Dr. Soiffer on Monday. However, she continues to be something of a medical mystery. Ascites, the buildup and retention of fluid in the abdomen, is usually a byproduct of some kind of liver issue. Susan's ascites continues and seems to be associated with her maddening pattern of two or three good days followed by a bad day (remember "yech"?). So, what's the liver issue? What's going on gastrointestinally speaking? What's root cause? Nobody knows.

As Soiffer explained it, all the various tests she's had over the past several months -- endoscopy, MRI, liver ultrasound, et al. and ad infinitum -- seem to point in different, mutually exclusive directions. It's a puzzlement. He has enlisted his most preferred gastroenterologist, someone who was desired but not available in an earlier go-round on this matter, and this guy, we hope, will be able to figure it out. He has ordered an echocardiogram, to be done this week in NYC, and then a return visit to DFCI next Monday when Susan will undergo another paracentesis to remove excess fluid that's accumulated due to the ascites. Based on outcomes, combined with all previous data points, he will, we hope, have a better understanding of Susan's condition than has been hitherto achieved and will prescribe the appropriate course of action.

Meanwhile, let's keep this all in perspective. It's now more than 300 days since the procedure. The further out she gets, the better the prognosis for a lasting cure. While there's been some GVHD, which is the greatest threat to a transplant patient, it has not been particularly virulent. Over time, if the conflict hasn't escalated, the foreign and native armies just get worn out from fighting and decide to get along, intermingle, intermarry, until, ultimately, they can't tell themselves apart from each other.

Susan is experiencing more good days, days where she feels almost like a civilian, than bad. She's getting things done, getting out of the house (except on bad days). Let's see what this new doctor comes up with. I'll report back, via this blog, after her visit to him next week.