Day +1471: It’s been a long time coming…

Superhero“It’s going to be a long time gone
And it appears to be a long
Appears to be a long
Appears to be a long time
It’s a long, long, long, long time…”

12/10/2014: I was going to wait until after my annual checkup and biopsy results to do another post but I realized it has already been nine months since I did a medical update here. My anxiety level is high tonight, anticipating tomorrow’s procedure and the seemingly endless wait for results. And the best result one can hope for is the equivocal “no evidence of disease” (NED); all unequivocal results tend to be bad news.

YES, the annual bone marrow biopsy is a big deal for me. Those close to me (aside from Michael) probably don’t realize this – my medical appointments are so frequent and varied that I may come across as blasé. DEFINITELY NOT THE CASE. Fellow travelers understand this well.

Having said that, I’ll move on to the boring, routine (for a cancer survivor) medical events, mostly for Michael’s and my record.

Dental

I closed the March 10th update with a pending trip to the dentist to have five cavities filled. I was mistaken – there were 8! The good news is that I had all eight done in one sitting on April 8th with neither sedation nor Novocaine/lidocaine. Yes, I’m incredibly impressed with myself! I was able to cancel the following week’s appointment and enjoy a day at home instead. My mouth continues to be extremely dry. I’ve been more diligent with fluoride gel, mouthwash, Biotene, and flossing but am likely to need work with every visit, one of the adjunct benefits of being a bone marrow transplantee. I postponed my next dental appointment until January when my new in-network dental insurance takes effect. I signed up for the wrong plan last year.

Gynecology

I had my annual gyn exam and PAP on April 1st, returning to my local gynecologist. The PAP was clear but I wasn’t happy with the exam in general. The gynecologist admitted he knew nothing about GVHD. The complete lack of coordination with post-transplant specialists is a continuing frustration with Hopkins. Some other facilities such as “The Hutch” in Seattle do a better job with this. I had a mammogram on April 23rd with normal results.

Otolaryngology/Otology/Audiology

On May 20th, an otologist diagnosed my intermittent left ear aches as probable temporomandibular joint syndrome (better known as TMJ). He recommended eating softer foods to reduce chewing for a few days when it flares.

ScopeAlso on May 20th, I saw my otolaryngology surgeon for a routine checkup. She said everything looked great and didn’t even scope me. I asked if anything could be done for the discomfort caused by the numbness and bulkiness in my neck below my chin. She arranged a same day consultation with a plastic surgeon, Dr. Boahene. He explained the procedure: “To remove the bulkiness, suck out fat/fluid through a pencil sized hole, then cut to remove excess skin.” Cuts are usually made behind the ears but he said he would use my existing scars. To help relieve tightness, he might cut muscles at the base of my neck. Recovery for bruising and tenderness would be about a month; total healing, a year. Unfortunately, insurance companies often see no difference between surgery for medical versus cosmetic reasons. They see just a number, a procedure code, but he will try to justify. I’m uncertain about having the procedure so keep putting it off. My transplant doctor suggests surgery could exacerbate GVHD. He said that my prior neck surgery could have caused the flare-up of skin GVHD on my back. He forgets the GVHD preceded my neck dissection by nearly a year. Anyway, I’m currently scheduled for surgery on January 16th but may change my mind again.

I had additional routine follow-ups with my otolaryngology surgeon on August 5th and November 11th. She scoped me both times and said everything looked great. The photo above shows her scoping me with an image of my tongue base on the screen. I’ll see her again February 10th. She said after three years with no evidence of disease we can lengthen the time between visits to six months or even a year.

My hearing test on June 2nd revealed mild hearing loss in the upper ranges. This could be genetic, chemo-related, or normal wear and tear. I return in a year for a recheck unless the hearing loss is interfering with life enough for me to want hearing aids.

Ear Actually more of a dermatology issue, but included here because it involved my outer ear – I had an infection in my right intertragic notch (see diagram) in mid-May. Dr. Patel, a Hopkins dermatologist, examined it during one of my photopheresis sessions and noted that it didn’t look like cancer. The antibiotic ointment she prescribed didn’t help, so she added a 10-day course of oral Keflex starting June 8th.

Side note: In general, since my bone marrow transplant, I don’t get that many more infections than before but when I do, they seem to linger an unusually long time. Fortunately, I’ve had nothing serious.

Dermatology

Because of cGVHD, I see my transplant doctor, Dr, Jones, quarterly instead of yearly. At my March 27th checkup, he told a visiting doctor that chronic GVHD occurs in 40%-50% of transplant patients but at Hopkins, they see less than 10% because of their post transplant Cytoxan protocol. He attributed my GVHD to trauma from “radiation and surgery.” I said I’d never had radiation, to which he responded, “But you did have surgery.” As usual, his memory was selective regarding the onset of my GVHD. I suspect the incidence of GVHD in Hopkins patients may be under reported but I do believe the Cytoxan helps. It’s quite amazing that I’ve had no systemic steroids or immunosuppressants and am faring no worse than my contemporaries from other facilities who’ve had both. I’m grateful to avoid the nasty side effects of those treatments. According to Dr. Jones, there is no cure for cGVHD, and despite many studies, one treatment has not been consistently more effective than another. Therefore, his goal is to minimize size effects while at least slowing the progression of the cGVHD.

While I was there, I again asked what might be causing the high cellularity in my marrow. Surprisingly, he admitted he honestly didn’t know – increased cellularity can be indicative of MDS but my last biopsy showed 100% donor and no chromosome abnormalities. He thinks that the reason might be inflammation from GVHD, particularly since my platelet count is also high. I’m anxious to see how my marrow has changed, if at all, over the past year.

My checkups on June 26th and September 25th were fairly routine. Dr. Jones said my increased fatigue was not surprising with cGVHD and treatments due to the breakdown of cytokines. My vitals and blood work are always good; every visit, he examines my mouth, eyes, and the active spots on my back and notes the absence of hair on my arms and legs and the dimpling of the skin on my limbs and abdomen. Each time, he pronounces, “I think it’s better.” Mostly, I coordinate my phototherapy treatments with my dermatologist, Dr. Sweren, and he bases them upon my feedback about how I’m responding.

By the way, my health insurance accepted the additional documentation provided by Dr. Sweren and ended up fully covering my photopheresis treatments. As I’ve said before, I’m very lucky to have great coverage, helped by being doubly insured through Michael’s military retirement and his current employment.

In mid-March, after two and a half months of extracorporeal photopheresis (ECP) twice a week, I felt a sharp pain deep in my left shoulder blade, an indication of where the next spot of skin GVHD would appear. I can feel the changes underneath before anything is visible on my skin. Sure enough, my left shoulder became dry and striated with “cigarette paper skin” and gradually became hyperpigmented with a pink center area, much like the spot on the middle of my back. It is also sore and itchy. My right shoulder is following the same trend, albeit more slowly. Wearing a bra is exceedingly uncomfortable. Underwires help relieve the pressure UVA1on my shoulders but are more aggravating to my abdomen. Home is never a problem since my main attire has always been loose-fitting loungewear. Unfortunately, I’m uncomfortable going publicly braless – because of my size (and the long-term effect of gravity on my DDDs), outer clothing fits very poorly. My compromise is to skip the bra and wear bulky sweatshirts for therapy or medical appointments and wear one when I go out socially. Winter is good for hiding under my coat when I shop and run other errands. It’s lucky I’m not very social!

After seeing no improvement by mid-April, I asked Dr. Sweren if I could have concurrent UVA1 and ECP therapies. He said that it wasn’t a problem medically but had never been done because insurance companies do not approve. However, his eyes lit up when I reminded him that the UVA1 also helped my seborrheic dermatitis, an entirely different diagnosis code. So, starting on April 28th, I became the first Hopkins patient to have both treatments ongoing. My new routine entailed 10 minutes (30 joules) in the UVA1 booth before beginning my photopheresis each time. I pass the time in UVA1 doing my neck exercises.

I continued ECP for a total of 7-1/2 months, missing only a few sessions due to vacations, illness, or holidays. Some sessions went smoothly but with increasing frequency, the ECP machine halted with annoying alarms due to the flow rate being too slow. The nurse suggested that the problem might be with my high platelet count combined with other unknown blood properties but I suspected a problem with the port. Finally, on August 13th when the flow rate remained zero after many attempts, I decided to take a break from ECP. When the effect of a treatment is gradual and subtle, one way to find if it’s working is to stop and see if the condition worsens. As you know from Michael’s post on August 25th, I had the port removed. The problem turned out to be a loop in the internal line. The surgeon offered to go through my groin and straighten the line or to replace the port with a new one but I wanted to be free of it altogether. I knew I could get a new one if I decided to resume ECP. So far, I haven’t. I don’t think it was helping. Michael is less certain.

Beginning August 20th, I increased my UVA1 exposure to 50 joules per session (15 minutes, 9 seconds) and have remained at this level twice weekly. I’m thinking of trying to cut back to once a week after I see Dr. Jones tomorrow and Dr. Sweren on December 16th.

Hematology

Backtracking a bit, you may recall that my ferritin level remained high after transplant; although gradually dropping, my count was still double the high end of the reference range. My transplant doctor and hematologist were unconcerned but I kept reading about others on marrowforums.org who were being treated for the same issue, either with drugs or phlebotomy. I definitely didn’t want drugs but I had an idea. After photopheresis, a small amount of blood is left in the bottom of the collection container. The nurse pours this into a bag and infuses it via the connected line. I decided to skip having that bit of blood returned each time to reduce my ferritin level. The nurse said she’d had some others do the same.

The slight blood loss each time caused my red counts (RBC, hematocrit, hemoglobin) to drop. When the counts dropped slightly below normal in mid-May, I resumed having my blood returned. They continued to drop, then increase, then drop again, never low enough to warrant a transfusion or to be terribly concerning but worrisome to us nonetheless.

At my routine checkup on June 25th, my local hematologist, Dr. Bahrani, reiterated what Dr. Jones said about the treatments and GVHD causing fatigue. He also said the treatments were most likely the reason for my slightly lower blood counts. When my extreme fatigue continued through the summer and my counts did not recover, I saw Dr. Bahrani again on August 26th. He ran more blood tests and called me with results on September 1st – my ferritin had dropped to 13 (below normal). He recommended getting an iron infusion, which I had on September 16th, and advised getting a gastroenterology checkup and colonoscopy to rule out internal bleeding.

I saw my gastroenterologist on October 16th. Since I wasn’t due for a colonoscopy for several more years, he opted for a blood test to rule out celiac disease and a stool sample to check for occult blood. Fortunately, both tests came back negative. I had my blood work rechecked with Dr. Bahrani on October 27th, and all primary counts were back within normal ranges. I’m still tired and lacking stamina but not nearly as much as before. I no longer need daily naps.

Ophthalmology

My eyes continue to be my greatest source of frustration post transplant. The Thermalon mask that I mentioned in a prior post does help. I resumed using Restasis nightly as of May 13th. It seems less irritating if I use the drops right before the bedtime hot compress (mask). Beginning on November 18th, I started using Restasis before my morning compress, as well. Oddly, it’s intolerably irritating if I put drops in both eyes but not so bad if I do only one eye. The pain/irritation is worse when I focus at a distance – perhaps the untreated eye compensates for the other and reduces the strain. Whatever the reason, I try to do anything that helps. When I’m having an especially bad eye day, I use the mask more frequently. I clean my eyelids and lashes with Ocusoft before and after each compress application. I also use preservative free over-the-counter eye drops many times a day.

Back in mid-March, I contacted my ophthalmologist, Dr. Prescott, via patient portal to let her know the medroxyprogesterone drops were not helping and to ask about tacrolimus (an immunosuppressant) eye drops. The vet prescribed these drops for my cat when he had severe eye ulcers but I couldn’t find much information about human use. She replied:

“I do not recommend tacrolimus for dry eye, though I have used it with some success on one patient with very severe atopic disease. Since it is off label, and still so new, I am concerned with possible side effects and long term effects.


“…I think it is worth having an evaluation with Dr. Hessen [a Wilmer optometrist], who is wonderful! I have seen great results with the PROSE lenses, so it is definitely worth exploring. They are about $5,000/lens, but insurance may cover them. I will send Dr. Hessen a note and put in a referral request…”

I procrastinated and finally scheduled an appointment with Dr. Hessen for June 27th. Unfortunately, I didn’t realize she was pregnant – her office called me to cancel on June 21st because she had her baby sooner than anticipated. I could’ve scheduled with another doctor but wanted to await her return because she came so highly recommended from multiple sources. Her specialties are dry eye, autoimmune diseases, and scleral lenses.

I was shopping for new frames at the local optometrist’s office on May 14th, and they offered to do a vision test. Dr. Butterworth took a photograph of the back of my eyes and discovered a hemorrhage in the left eye. He said it could be from many different causes – strain, coughing, etc. He forwarded the results to Wilmer but I forgot to ask about it when I saw Dr. Prescott.

I saw Dr. Prescott again on August 1st. She noted that in addition to dry eye, I have anterior and posterior blepharitis (inflammation). Anterior blepharitis affects the outside front of the eyelid where eyelashes are attached. Posterior blepharitis is caused by dysfunction of meibomian glands within the eyelids that secrete oils to help lubricate the eye. The crusty eye yuck is from blepharitis. She advised me to continue using Lotemax steroid drops, gel, and ointment twice daily as well as the OTC eye drops, Restasis, and mask.

At my next follow-up on October 27th, Dr. Prescott measured my vision at 20/40 with glasses. That was with great effort and much squinting on my part to read the eye chart. She mentioned that Dr. Hessen was back at work and again suggested I consult her about scleral lenses. I called Dr. Hessen’s office the next day and was lucky to get an initial consultation on October 29th due to a cancellation.

Dr. Hessen is personable and excellent at explaining things. She took as long as I needed to answer my questions and Schirmer Testreiterated I could contact her via patient portal if I thought of more. My eye pressure was tested and found to be slightly high, 22 in both eyes, probably because of the steroid drops. Normal pressure is 10-21 mmHg. I had a Schirmer test on both eyes to measure tear production. As you can see in the diagram on the right, normal range is 15-25mm. My result was 1mm. That is very bad but the tech said any result above zero is hopeful because it means that tears can be produced. It may be possible to increase production or at least keep the tears being produced in the eye longer.

Dr. Hessen discussed three possibilities for treatment:

  1. PROSE lenses (prosthetic replacement of the ocular surface ecosystem) – large hard lens with a significant learning curve for insertion. They must be inserted and removed daily. $5600 each – insurance may cover. A side benefit of the lenses is correction of near or distance vision (but not both). More info: http://www.bostonsight.org/Home
  2. IPL (intense pulse light for dry eye) – 5 treatments (once a month for five months), about $500 each, may require additional maintenance treatments. Insurance won’t cover. More info: http://dryeyenews.com/what-is-ipl/
  3. Lipiflow – one treatment, about $2000, may need to be repeated. Insurance won’t cover. More info: http://www.lipiflow.com/why-lipiflow/lipiflow-a-different-approach/

I need to do more research on these and write a separate post for those interested. I’m scheduled for a PROSE lens fitting on December 29th. Dr. Hessen says she has had a 100% success (i.e., significant improvement) rate for patients who are able to get past the learning curve and tolerate wearing the lenses. I have concerns about my patience level, having tried “normal” contact lenses when I was young and not liking them. However, this time I have more incentive – better vision rather than vanity.

In the meantime, Dr. Hessen wants me to wean myself off Lotemax (which I’ve done) because high eye pressure puts me at risk for glaucoma. She said to use HOT compresses at least twice a day, as hot as possible short of burning my lids. I’ve increased the time I heat the Thermalon mask from 20 to 35 seconds in the microwave and am getting more of the gunk out, presumably helping unclog the glands. I also wear the hot mask for 10-15 minutes at a time instead of 5 minutes. She also said to continue using preservative free OTC drops and Genteal ointment and to take an oral supplement of 2000-3000mg daily of Omega-3, either fish oil or flaxseed oil. I’m taking fish oil capsules, two 1500mg capsules every night.

Miscellaneous

Beginning July 18th and lasting 7 weeks, I had a tickle in my throat and chronic cough, accompanied by hoarseness (no other symptoms of illness, aside from fatigue). I suppose it may have been allergy. I treated it with Benadryl and Sudafed.

I had my flu shot on September 17th. Happily, insurance covered it this year.

On September 21st, I was treated at Patient First for a urinary tract infection, my first in many, many years. I used to be able to get rid of UTIs by drinking lots of cranberry juice but this time, I also needed a week of Cipro antibiotic.

I’ve had more frequent bouts of nausea in the past six months. I don’t know why. It comes on very suddenly but usually abates if I eat something or take a 0.5mg Ativan. The low dose of Ativan also helps on the rare occasions I have trouble sleeping.

That’s about it for now. I hope to have another update soon with results from my annual bone marrow biopsy.

5 Comments so far

  1. Wow. Your issues sound so overwhelming Karen. You have every right to have high anxiety levels. I found that the drops (3 different one) I was using to lower my eye pressure (52 and 37) was causing my dry eye to be horrific. I stopped the pressure/glaucoma drops and my dry eye is much better! I take an oral medicine for the pressure and it seems to be working. I told them they have to treat the pressure without drops as I cannot stand the dry eye while using them. Hopefully you will get some relief. I agree the eye problems are Number 1 in the irritation level. I’m happy a lot of your tests were good! Let me know how your biopsy turns out. Hugs Lor

  2. Lori Patrick on December 18th, 2014 at 12:25 pm
  3. Oh, Lori, my issues are nuisance level – nothing life threatening! I can’t imagine enduring what you’ve been through this year. Hugs to you, too.

  4. Karen on December 18th, 2014 at 1:00 pm
  5. I cannot wait to read the good results of your biopsy, there is nothing blasé about it! You do such a wonderful service for all transplant patients by documenting your appointments/challenges for all to read.

    Chronic cough and tickle and something to try. When Mike was admitted recently for induction they suggested trying meds for acid reflux for his chronic cough and it has worked wonders. So many docs suggested allergy meds, but it never really went away until now. He did see a pulmonologist just before admission who thought he had a mild case of asthma, but the reflux meds really did help.

    Looking forward to your update and thanks so much for sharing a complete picture.

    Hugs to you and Mike,
    Mike and Debbie

  6. Debbie W on December 18th, 2014 at 11:32 pm
  7. Forgot, the lines are from a Crosby, Stills & Nash song.

  8. Debbie W on December 18th, 2014 at 11:36 pm
  9. First I want to say I love the sweatshirt! You are a superhero! Eight cavities filled and no drug. Superhero!

    Have you ever thought about writing a book about your journey? Others on the journey will find it helpful I would think. You have great notes to use. And you have wit and humor. It would be inspiring.

    Looks like you are auditioning for the “Blue Man Group”! Great picture! 🙂

    Your progress has been remarkable. Keep up the good work! And continue your research. I think it has made a big difference in your care. You know what is out there and you make good valid suggestions to help you. Superhero!

    Long Time Gone-Crosby, Stills, & Nash

    Love,
    Cathy 🙂

  10. Cathy Johnson on December 30th, 2014 at 12:57 pm

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