Barbara passed away on September 4, 2005.
Barbara’s personal journal: www.thebarbie.us
In November 2001, my wife’s (Barbara) GYN felt a lump in her neck. The lump did not show from the outside but could be felt by examination. Barbara didn’t do anything about it for a while but when she had some slight trouble swallowing she went to our family doctor. The family MD sent her for some tests [a barium swallow and thyroid ultrasound] that showed that her esophagus was being bent by something, and he sent her for an endoscope. Dr. Richard Moses examined Barbara and realized, without doing the endoscope, that it was not in her throat but outside of it. A follow-up scan showed that her esophagus and windpipe were being pushed aside (in a curve like a letter C) by some mass.
Barbara saw an ENT cancer surgeon at Temple Hospital, Dr. Daniel Kelley. They did some biopsies and found that the mass was malignant. They didn’t know the source of the mass but called it a “poorly differentiated carcinoma”.
On March 19, 2002, Barbara had a 9 hour surgery in which they took out a mass the size of a tennis ball, one internal jugular, some muscle and lymph nodes. She had some complications with blood clots and bleeding, and was in the hospital for a week.
The pathologist from Temple Hospital said that it is “most likely” thymic origin from the ectopic cervical thymic tissue present. Dr. Kelley said that this is extremely rare and that there is no set protocol for treatment yet. He recommended radiation, and said that the literature is unclear about the benefit of chemotherapy.
Because of the rarity of the diagnosis, we sent the slides to two other pathologists, one of which was Dr. Saul Suster, the recognized expert in the field. Both agreed with the diagnosis.
Dr. Kelley consulted with radiologists at Temple and with a local radiologist where we live. Since neither had any more experience with this type of cancer, Dr. Kelley said that she may as well be treated locally where it is more convenient.
Barbara went through 37 grueling radiation treatments. They had to fit her for a mask and lock her head back in an uncomfortable position. The treatment burnt her esophagus, trachea, and pharynx. She lost her voice for 4 months and some hair that was in the field of radiation. The surgeon said the burn in the esophagus is similar to the damage when people ingest drain cleaner. The pain was severe enough to stay on codeine since it hurt to swallow anything [water, saliva, ice cream].
She also had fatigue from the radiation. She describes the fatigue as “like having anesthesia for surgery and how you feel when they wake you up—but you don’t wake up. Maybe it is like you overdosed on pain killers or muscle relaxers, a very severe exhaustion. We need a new word; tired is an understatement. The body really needs the sleep, though, to repair the damage done. And you have no choice but to give in. It is a bigger force than anything I ever felt.”
For a period, her skin wouldn’t let up from itching and burning. It was constant near treatment number 25. The creams didn’t do anything and it would drive her crazy at times. She tried using a paintbrush to touch it. It was a sickly purple-red and made her miserable, as if she “had been stung by thousands of mosquitoes.” One day her skin progressed to a dry flake with a blackish color, like ash.
She is still getting tired and has periods when her voice is very hoarse and she can’t speak. Her hair is thinning, which may be because of damage to the thyroid, and she is seeing an endocrinologist. They want her vocal cords to be examined to see if they were damaged as well.
A recent PET scan showed that her left side of the neck, where she had the surgery, is clear. But something showed up on the right side now. They are watching her carefully and plan another PET scan to check the questionable item on the right.
Update on Barbara Neibauer 2003
Several months after completing radiation, Barbara’s CAT scan showed several small items in her right lung. The doctors suggested she wait until her next periodic scans to see if the items were still present. A CTA scan of her heart a few months later, however, revealed several more items and an enlargement of previous ones. This was confirmed that same week by a chest CT. At the advice of several physicians, Barbara saw Dr. Larry Kaiser at Penn. Dr. Kaiser suggested that she have the items removed so it could be determined if they were a metastasis of her Thymic carcinoma. On May 12, Barbara was admitted to Penn, where Dr. Kaiser removed the middle lobe of her right lung and wedges from the upper and lower lobe. The pathology confirmed that her Thymic carcinoma did indeed metastasize to her lung, but Dr. Kaiser was able to remove all measurable signs of the disease. The oncologists feel that the cancer may return, but the best course of action at this point is to watch Barbara closely with periodic scans, and deal with the future as it occurs.
Barbara’s scans in October 2003 showed some small “tumor activity” in the PET scan but a stable CAT. Most of the doctors said not to do anything about it at that time (one suggested she start chemo). She just had another set of scans (January 2004) which now show “increased tumor activity.” More spots lit up and the other ones were larger. It seems that she will eventually need something and we’ll have to make that decision in time.
Barbara started taking Octreotide in 3/2004 — three injections per day. After two months of treatment, the tumors seem stable (or even slightly smaller) on PET/CT scans. However, after four months on the drug, one tumor is over 40% larger and the others (even stable in size) show more activity in the PET scan.
Barbara is starting a new experimental drug now that the Octreotide seems not to be working. We’ve been working with Barbara’s doctors these past several months to getting approval from the Compassionate Use Committee and the FDA. All of the approvals just came in. One of Barbara’s doctors, Dr. Jerome Check, has written about the possible immunotherapy benefits from the progesterone antagonist Mifepristone.
After taking mifepristone for a few months, she got some mixed results on her scans. On the CAT scan, all but one tumor remained stable, but the one that grew increased about 25%. On the other hand, almost all of them showed increased activity on the PET scan, some significant increases. The oncologist had her pathology blocked tested and it was positive for the marker CD- 117 so, they decided she should try Gleevec. It is one pill per day.
After two days on Gleevec, her trigeminal neuralgia kicked in with terrible pains — two-hour bouts of intense pain in the face, like “someone is cutting my tongue and stabbing me in the side of my face while it felt like someone was sticking a screwdriver in the roof of my mouth over and over.” She has a high tolerance for pain and never had to take Demerol or any painkiller when they did the neck dissection and then when they took out the lung lobe. But now she increased her trigeminal medication and took demoral for the pain. She also stopped the Gleevec and after a couple of days, the trigeminal eased up somewhat. She is scared to death to start the Gleevec
again, but her docs want her to go back on it.
Meanwhile, two radiation oncologists have suggested she radiate the enlarged nodes as a temporary measure, either with or instead of the Gleevec. The trigeminal pains were so bad she would rather do the radiation than face them again.
Most recently, her eye doctor found that she has glaucoma in both eyes and has started her on medication. We wonder if the Gleevec caused this.