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

-->

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.