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Rebekah Update

Location: [Home Page] [Our Children] Rebekah Update Thu, Oct 12, 2000 [Rebekah FAQ]


Rebekah!

Here's the story at the moment:

This page was last updated on Thu, Oct 12, 2000.

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The Current Status

Rebekah had a good retinal exam under anesthesia on Tuesday, October 3, at the Mass Eye and Ear Infirmary in Boston. For those of you who don't remember what all this is about (it has been a while, after all), Rebekah's ophthalmologist Dr. Shizuo Mukai uses a light and a lens to examine the back of Bekah's remaining left eye. He's looking for changes in the tumor areas and for signs of new tumor growth.

And the good news is, there was none of that! A good checkup!

These exams have been performed under anesthesia to date because of the difficulty young children have with keeping their eyes open when bright lights are shone in them, but Dr. Mukai suggested that we try our next exam in the office and see how she does. Rebekah, sitting in her hospital bed recovering from anesthesia and still wearing her IV, thought this sounded like a good idea. We'll see how she feels in February!

Please keep her in your prayers.

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[Eye Diagram]

The Story

Our fifteen month old daughter Rebekah lost her sight on Saturday, October 5, 1996. She was diagnosed with retinoblastoma, a cancer of the retina.

Rebekah was first examined by Dr. Philip Silverstone of Branford, Connecticut on October 5, who diagnosed elevated retinas in both eyes. He referred us to Dr. Craig Sklar of New Haven who examined her on October 7, and confirmed the diagnosis. Elevated retinas are caused by fluid or a growth growing behind the retina, pushing it out of shape and pulling it away from its blood supply.

On Wednesday, October 9, Rebekah was examined by Dr. Shizuo Mukai at the Massachusetts Eye and Ear Infirmary in Boston. This was a full exam under anesthesia. He and his team found fairly large tumors in both eyes, four in the right eye and one in the left. These tumors compete with healthy retina tissue for the blood supply, and that caused the retinal detachment which blinded her.

On Thursday, October 10, Rebekah was examined by Dr. Eric Grabowski, a pediatric oncologist, at Massachusetts General Hospital Cancer Center. From his discussions with us and review of the CT scans and spinal tap, he decided that the cancer was confined to the eyes, and that her life was not immediately threatened.

The Treatment

The options for treating this cancer are similar to those for other cancers: surgery, radiation, and chemotherapy, or combinations. Based on Dr. Mukai's and Dr. Grabowski's recommendations, we met with Dr. Eugen Hug, a radiation oncologist at The Massachusetts General Hospital (he has since moved to California). He and his colleagues with the Northeast Proton Therapy Center treated Rebekah with highly focused proton radiation from a cyclotron originally built for Harvard's physics department. The advantages of this therapy are that these tumors are highly vulnerable to radiation, it offers the hope that she will regain some degree of vision, and it avoids some long-term adverse affects of cancer therapies.

The proton radiation is significant. Traditional external beam radiation has the disadvantage that it irradiates tissues in addition to the tumors, which can cause other cancers. Proton radiation may have similar side affects, but because the beam itself is much more focused, the risks are probably reduced. The most remarkable thing about it is that they are able to determine the depth of maximum energy release, so that tissue beyond that area but still in the beam is not as severely irradiated. We are fortunate, because only two medical sites in the United States have proton beam equipment, and only the Proton Therapy team at Mass. General is using it for retinoblastoma.

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On October 17, we took the first steps toward her proton beam radiation therapy. She had a central line IV placed in her chest, which allowed her to receive fluids and anesthesia without having a needle placed in her each time she had a treatment. The anesthesia for this procedure is fairly light, and mostly sedated her without actually putting her fully to sleep, so she "woke up" better. Dr. Grabowski also took a sample of the fluid around her spinal cord (a lumbar puncture or spinal tap) which verified that the cancer cells had not spread via the nervous system fluid. Finally, she had a CT scan which Dr. Mukai used to determine the precise target for the proton beam, and the tissues to be avoided. The CT scan also showed that the tumors had not grown outside the globe of her eyes.

Rebekah was remembered in prayer several times during the Connecticut Conference Annual Meeting held at Center Church in Hartford, Connecticut, October 18-20. Attendees also signed a large wishes card which was delivered to us on Sunday evening. A photograph of people signing the card appeared in the November CONNtact, the Conference newspaper. Our thanks go to all the staff, delegates, clergy, and members of the Connecticut Conference for their prayers and care for us.

On October 23 Dr. Grabowski gave us the glad news that the cytology scan from the spinal tap was normal, confirming that the cancer has not spread. We were inexpressibly relieved. In addition, Dr. Mukai checked Brendan's eyes on October 25 and found that they are completely normal.

The treatments began on Thursday, October 24, at the Harvard Cyclotron Laboratory. She had them in the early afternoon. The entire treatment was on an outpatient basis. When we had to be in Boston overnight, we stayed with family, and otherwise she was home. There were twenty-two treatments in all.

Rebekah was not in any pain at that time, but she is a very independent child, and was occasionally frustrated by her inability to see where she was going. She would walk anyway, often throwing off a guiding hand, and became getting very good at finding her way about. She fed herself, but her hands had to be guided to her food initially. She become convinced that her fingers are more effective eating tools than silverware, which is difficult to argue, even now.

She was also capable of wrestling with her big brother and holding her own. She smiled and laughed, and was very glad when she returned home to her brother and parents. After a long day of anesthesia and procedures, she got fairly cranky, and was absolutely clear about her feelings on the subject! Her feelings may have something to do with the fact that she wasn't allowed anything to eat for six hours before the treatment.

Rebekah tolerated the treatments well. It helped that there was little in the way of immediate side effects: no nausea, no hair loss. She had more difficulty with the disruption in her sleep schedule (probably due to anesthesia), and would wake fairly often in the night. She ate very well. She did develop some red spots like sunburn on her temples, and some eye irritation, which faded over the couple of weeks after treatment ended.

The central line required "flushing" daily, and periodic changes of the dressing. She fortunately never tried to pull it out, but she greatly disliked having it cleaned, dressed, and flushed. Visiting nurses from Omni Home Health Care worked with us to teach us how to keep it clean and flushed, with limited cooperation from the patient.

Rebekah was prescribed a pair of shatter-proof glasses, she'd only leave them on less than 70 nanoseconds. The lenses are made of a material originally designed for NASA space suits! Obviously they were not to correct her vision, but rather to protect her eyes while we waited to find out how much vision would be restored. The optician suggested we put them on her before she wakes up. Of course, she wakes up earlier than we do...

Rebekah appeared in the New Haven Register on November 4 in a story by Lynn Fredricksen, with a photo by Mara Lavitt. The story has prompted a few calls, among them a personal call from US Representative Rosa DeLauro. We were frankly a little nonplussed at the attention.

After her last treatment on November 29, Dr. Mukai made another examination to assess her progress. The tumors continued to shrink, and the retinas in both eyes reattached.

Dr. Shizuo Mukai examined Rebekah on December 10 under anesthesia at the Massachusetts Eye and Ear Infirmary. The tumors were much smaller; the largest one was then 8 mm in diameter. Since the radiation worked well, he had the central line IV removed, which might otherwise have been left in as a means of administering chemotherapy.

Rebekah's eyesight also continues to improve. While it was uncertain exactly how well she sees, and whether she saw with her right eye at all, the difference from October 9 was remarkable. She could walk through a room without hitting furniture, she could pick up a toy she had dropped, she could even take a cup from our hands. Her spirits were extremely good, especially since the conclusion of radiation treatments and her return to day care. She was simply very happy to be able to play and be a little girl, and not be half-starved and sedated four times a week.

The Checkups

Rebekah's visit to Dr. Nathalie Azar at the Massachusetts Eye and Ear Infirmary for a vision examination on December 30 went very well. Bekah objected to having bright lights shone in her eyes (nobody said she was dumb). Dr. Azar told us that she was seeing equally well from each eye, which means no patching to force a lazy eye to work, but that she didn't think Bekah saw very well compared to normal. She compensated extremely well, but an adult experienced in sight who had her vision might be immobilized. She prescribed corrective glasses for slight farsightedness to give Rebekah the best possible vision she can have. This was intended to encourage her to try to focus her eyes where she might use hands instead.

We're very glad she didn't have to have an eye patch, because Brendan would have been terribly jealous that his sister could look like a pirate and he couldn't.

Dr. Shizuo Mukai examined Rebekah under anesthesia on January 7 in another check on the state of the tumors. Things are looking very good. He found that they have continued to shrink, which should continue for some time yet, and that no other tumors have grown. He will examine her again in March, and we expect every three months after that.

Rebekah was in the news a second time: this time in a front page story in the New Haven Register, again by Lynn Fredricksen. The story should be on-line when they get their archives working; follow the link to the Register, click Archives and enter Rebekah Anderson in the form. This story also includes a photo of Evelyn and Rebekah, though if truth be told, you can't see much of Evelyn. The November 4 story is available on-line, too, if you can convince the secure server of your credit card number, which I can't.

The story set off a media whirlwind. The AP wire picked up the story and the photo, and between 2:00 pm and 6:00 pm on Sunday 12/29, we had a phone call from the Boston Herald, and then visits from camera crews of WTIC-TV (Hartford Channel 61), WFSB-TV (Hartford Channel 3), and WTNH-TV (New Haven Channel 8). I know of twelve (12) newspapers that have carried the story in three states, including the two listed above: Meriden (CT) Record-Journal, Manchester (CT) Journal Inquirer, Lewiston (ME) Sun-Journal, Norwich (CT) Bulletin, Waterbury (CT) Republican-American, Portland (ME) Press-Herald, Springfield (MA) Union News, Connecticut Post, Lowell (MA) Sun, and the New London (CT) News. If we look a little glassy-eyed, that's why.

Let us know if you saw the story in your paper, and I'll put its name up in the list!

The reviews from here are, well, mixed. The word "miracle," which headlined the Register article, has done it. So all have jumped on the fact that my mother-in-law noticed that Rebekah could reach directly for a Cheerio on December 24, which she couldn't do before: and implied that her sight returned all at once on that day, which is simply not true. It doesn't seem to matter what we actually say, it still comes out sounding like Jesus healing the blind man outside Jericho. Click here for more reflections on this.

Rebekah returned to Boston for a day of checkups on January 29. She was first re-examined by Dr. Nathalie Azar of the Pediatric Ophthalmology Service at the Massachusetts Eye and Ear Infirmary. Dr. Azar had last seen Bekah in late December, and she was very impressed and pleased that Rebekah's eyesight has improved. While she is not able to quantify it, Dr. Azar did observe that Bekah can grasp objects with much more confidence and without missing them. While this certainly does not mean perfect vision, it is good that things can still get better. We will be seeing Dr. Azar about every three months, primarily to check for the cataracts which will eventually form due to the radiation.

Rebekah also went to see Dr. Eric Grabowski of the Massachusetts General Hospital's Pediatric Oncology service. Dr. Grabowski had not seen her since last October, and he was delighted with the improvement in her vision as well as the success of the treatment in reducing the tumors. He also checked for signs of tumor growth in other areas, and found no evidence of that. Over the next five to six years the primary danger is tumor growth in the original site, namely the eyes. After that time, recurrence in the eyes is less likely, but other cancers in other areas become more likely. For now, she is doing extremely well. We will be seeing Dr. Grabowski every three months, too.

Rebekah returned to the Massachusetts Eye and Ear Infirmary on March 11 for an examination under anesthesia by Dr. Shizuo Mukai. His primary interest was the growth or reduction in the retinal tumors, and he reported that there was no sign of recurrence or new tumors.

Unfortunately, he did see development of a problem related to the radiation therapy: increased blood flow in the right eye (which had had more tumors in it). This detached the retina again, and deprived her of the sight of that eye.

Dr. Mukai used a laser to cut off some of the unneeded blood vessels, but he could only do this in the very small area of the eye where the retina was still attached, about 15% of the total area. He said it was likely that the pressure of the eye's fluid would rise, and eventually become very painful. When this happens, the recommended treatment is removal of the eye. He scheduled Rebekah to be examined every two to three weeks to assess this condition, to use the laser to reduce the excess blood flow as possible, and to examine the tumors as well. The outlook for keeping the right eye at this point was not very good, but so far there were no signs of this condition developing in her left eye.

Despite this setback, we held the Rebekah Celebration on March 15, at which Rebekah, of course, was the star.

Dr. Shizuo Mukai examined Rebekah under anesthesia on Tuesday, April 1, at the Massachusetts Eye and Ear Infirmary. This visit, only three weeks after the previous one, was the result of seeing side effects of the radiation therapy on March 11.

Dr. Mukai found that the visible signs of the radiation damage were subsiding in both eyes, indicating that the condition was improving. The retina in the right eye was also beginning to re-attach, although comparatively little of it had done so in those three weeks (she cannot see from the right eye with most of the retina detached). The gain allowed him to seal off some more of the extra blood vessels to help prevent a painful buildup of fluid pressure.

Unfortunately there was some increase in pressure in the right eye, although not to a point where Rebekah seemed to sense it. He questioned us closely about her behavior and whether it seems that she is feeling pain, of which we had not seen the signs. The best indicator that things were going better, however, was that he placed Bekah back on the every three months schedule for examination under anesthesia. The lack of urgency suggested to us that he was confident that the crisis had passed.

Rebekah had two appointments on April 17, one to have her vision assessed by Dr. Nathalie Azar, and the other with her oncologist, Dr. Eric Grabowski. The appointments produced no surprises, and no alarms.

Dr. Azar's assessment confirmed Rebekah's lack of vision in her right eye, and found reduced sight in the three months since her last assessment. Because of the radiation effects, Dr. Azar had no treatment to offer, and suggested that we discontinue regular visits to her unless Dr. Mukai made another referral. The original reason for seeing her was to assess whether Rebekah required patching to bring a functional eye into use, and with her right retina detached, that was a moot point.

Dr. Grabowski found an active, nap-deprived, and somewhat grumpy child who had no desire to have her internal organs and lymph glands probed, and had a hard time with the exam as a result. It turns out that that difficulty was as informative as anything else, for he said, "If there were something wrong, she'd be easy to examine." So the cancer seemed well under control.

Rebekah had a clinic visit with Dr. Shizuo Mukai on Friday, May 2, at the Massachusetts Eye and Ear Infirmary. He was able to roughly assess the fluid pressure in her right eye, and found that it was still elevated. Because he could not use the same equipment as when Bekah is anesthetized, he could not tell us whether the pressure was higher or lower than when he examined her on April 1. He scheduled her for another exam under anesthesia in late May.

Her next examination under anesthesia by Dr. Shizuo Mukai at the Massachusetts Eye and Ear Infirmary was on Wednesday, May 28, 1997. He found that the tumors in both her eyes were still well reduced and calcified, and showed no signs of regrowth, nor were any new tumors visible. In addition, the radiation effects in her left eye were substantially improved, and he expected (and we confirmed) that she should have better eyesight with that eye.

Her right eye, however, was still having problems. The retina remained about 80% detached, the pressure was about 30% higher than normal, the first signs of a radiation-induced cataract had appeared, and he saw signs of some hemorrhaging as well. He did not expect that she would regain much useful vision in this eye.

He placed Bekah three eye drops, one of which was intended to reduce the pressure but could have triggered asthma if she were vulnerable to it. He told us that it was still possible that she could lose the right eye if the pressure did not go down.

Dr. Eric Grabowski, Rebekah's pediatric oncologist with the Massachusetts General Hospital, examined her on Thursday, July 24, 1997. His job was to check the possibility that cancer had developed elsewhere, and he found nothing to indicate that. Which made it a good checkup!

Along with a surface examination, Dr. Grabowski drew blood (a difficult business with a strong and determined child!) for later analysis. He also recommended that she have an imaging study by Magnetic Resonance Imaging (MRI) to confirm that no cancer has developed elsewhere. He said that imaging studies are routine for retinoblastoma patients, but that he is studying the utility of MRI scans versus the more "traditional" CT scans. We tentatively scheduled it for October, before her next visit to Dr. Grabowski.

The Surgery

On Tuesday, August 26, Rebekah returned to the Massachusetts Eye and Ear Infirmary for an examination under anesthesia (EUA) by Dr. Shizuo Mukai. In this exam, Dr. Mukai used both ultrasound and a visual examination of Rebekah's retina to determine the status of the tumors, as well as other tests of pressure, etc.

Bekah's left eye was doing quite well. The radiation-induced problems continued to pass off, and the tumors showed no signs of regrowth. This had been her only useful eye for some months due to the continuing retinal detachment in the right eye, and she had demonstrated the ability to see more than well enough to get around, find things, and play.

Her right eye, however, continued to suffer from radiation side effects, including elevated pressure, detached retina, and hemorrhaging. The latter was the biggest problem, because it made seeing the tumors more difficult, and it was getting worse rather than better. His recommendation was that he perform an enucleation, which is the removal of the right eye, and he was careful to ask about her vision with the left eye before making that statement.

After some thought, Evelyn and I decided to accept his recommendation and have the eye removed. She had not seen with this eye since early March or possibly late February 1997 due to the detachment, and that same detachment made it nearly impossible that she could regain vision in it. This eye also had the greatest volume and number of tumors, and its removal would also alleviate the danger of the cancer spreading. We did not like to do it, but we decided that it was necessary.

Dr. Mukai was scheduled to perform the operation in late September at MEEI. His office staff were thoughtful enough (and talented enough) to also schedule Rebekah for an MRI on the morning of the surgery. In addition, her oncologist, Dr. Eric Grabowski, was scheduled to be there for the surgery. The operation would be followed by an overnight stay at the hospital, with followup exams later in the week.

On Monday, October 6, Dr. Shizuo Mukai reexamined Rebekah's eyes and performed an enucleation, a removal, of the right eye. We had accepted his recommendation for this procedure after the exam in August, and she did very well with the surgery and anesthesia. She spent one night in the hospital. The surgery was delayed two weeks by an ear infection of Rebekah's, and also illness in Dr. Mukai's house, and though we never wanted to do this, we are glad that we no longer wait for it.

Damage from the radiation therapy she had last year had caused retinal detachment, increased pressure and hemorrhaging in the right eye since February or March. The left was also affected, but those symptoms continue to improve while in the right they only worsened. At the August exam, Dr. Mukai was unable to clearly see the tumors and monitor their condition and activity.

After removing the right eye, Dr. Mukai quickly examined it and found no signs that the tumors had grown or spread beyond the eye. This confirmed the results of a Magnetic Resonance Imaging (MRI) scan performed on October 2, which also showed no signs of cancer elsewhere in her head. The MRI will be used in comparison with other scans over the course of years.

After the surgery, Rebekah showed most interest in sleeping, cuddled with her special blanket and a baby doll. At one point she woke enough to announce that "Baby is crying," and gathered the doll into her arms and went back to sleep. She appeared to be in no pain, and enucleation generally does not cause much pain after the operation.

Rebekah spent one night in the hospital after her right eye was removed, and spent most of that time sleeping. After so many anesthesias, both during her radiation therapy and examinations, we were quite surprised at how long it took her to get hungry and ready to play. But she decided for herself to take some extra time before going back to her active self.

On Wednesday, however, she was fully active and raring to go again, showing interest in food and playing happily with a new baby doll (called "Yellow Baby." She's not that interested in names). Evelyn reported that she was also demanding chocolate, which seemed like a good sign.

She wore a pressure bandage until Friday, October 10, when it was replaced with a simpler eye patch. She spent the week with her mother at her great-aunt and -uncle's house to be nearer the hospital in case of emergency. The pressure bandage was a great big thing, with which she hadn't bothered much, but she objected to its removal because of the adhesive's pull on her skin. She briefly wore an eye patch taped on the inside of her glasses, mostly to keep from catching a stitch in her eyelid on anything, and because the closed right eye looked rather bruised.

The stitch was removed on Friday, October 17, at which time everything looked very good. Dr. Mukai released her from wearing an eye patch, which accorded to Rebekah's desires. She really hadn't liked it. On the same day Dr. Mukai examined Brendan's retinas, which is a precaution taken with siblings because of the genetic roots of retinoblastoma. We were happy to hear that he found no sign of trouble. Rebekah also visited Dr. Eric Grabowski for her quarterly checkup, and received good reviews.

On Friday, October 24, Rebekah had a vision assessment exam by Dr. Robert Scarpatti, to whom we were referred by Connecticut's Department of Education and Services for the Blind so that her educational needs could be better understood. He was quite pleased with her vision, as his knowledge and experience of children with retinoblastoma led him to predict much poorer vision than she actually has. She definitely has a reduced visual field due to the tumors and the removal of her right eye, but she does have at least fairly good central vision. We are hopeful that she will be able to read print, though we intend to offer Braille to her if she finds that more useful. Dr. Scarpatti confirmed that for legal purposes, she is blind, but that definition apparently includes limited visual field.

Rebekah returned to Dr. Mukai's office in Boston on Friday, October 31 for a further post-operative checkup. Again things looked very good, and the appointment would have been quite brief, but Dr. Mukai had at last received the results of the DNA scans ordered a year ago. The confirm what we had expected but also hoped: that Rebekah is the only family member with the genetic mutation that is at the root of this cancer. Because it is possible for genes to vary in cells in different part of the body, this is not a blanket statement of "no risk," but it is a relief, particularly for our son.

In mid-November, Rebekah was treated for a penicillin-resistant post-operative infection in her right eye socket. Because the organism, a form of streptopneumonia, was not amenable to treatment with oral antibiotics, she received intravenous medications. The course of treatment ended just after Thanksgiving.

The infection became apparent on Tuesday, November 11, when she woke up from her nap at day care with the right eyelids severely swollen--and they had been unremarkable before her nap! We took her to Dr. Philip Silverstone, a Branford ophthalmologist, who prescribed an antibiotic, took a culture, and asked us to return the next afternoon. When we did, the swelling had actually worsened, and he advised us to bring her to her primary retinal ophthalmologist and surgeon, Dr. Shizuo Mukai in Boston. We also saw her pediatrician, Dr. Ferrin Holmes, that Wednesday night, who found no signs of infection in her ears, nose, throat, and lungs. He did increase the dose of antibiotic slightly.

On Thursday, November 12, Dr. Mukai saw her, and asked Dr. Heidi Remulla, an ophthalmic plastic surgeon, to look at her as well. They were concerned that the implant to which Dr. Mukai had attached the eye muscles might be involved in the infection, and fortunately it looked free of it. A major contributor to the problem is that she does not yet have her permanent prosthesis, which would fit well enough to allow the body's own mechanisms to prevent infection. The conformer which she has now allows air pockets to form in which bacteria can grow.

When Rebekah saw Dr. Mukai on Friday, November 13, he had received results on the cultures, which showed the infection was penicillin-resistant. He immediately admitted her to the Massachusetts Eye and Ear Infirmary for IV antibiotic treatment. He also sought the advice of Dr. Shaw Warren, an infectious disease specialist at Massachusetts General Hospital. She was prescribed two IV antibiotics, plus eyedrops and a topical ointment.

Throughout this Rebekah was mostly been her usual cheerful and decisive self. She rapidly figured out that ophthalmologists poke at her eyes when her glasses are off, so she resisted removing them when medical people were in the room. We noticed that she perked up a little with the beginning of antibiotics, but it was a subtle change, not obvious. Even when her eyelids were so badly swollen that they touched her glasses, she showed no signs of pain or discomfort. We have decided that for Rebekah, "Ow!" indicates displeasure, and not necessarily pain.

Rebekah has small veins, and even sedated (for a CT scan at MEEI on Friday, November 14) the anesthesiologist was unable to place a long-term IV port, so she was given a "peripheral" IV which needed occasional changing. Quite aside from the medical reasons for it, she kept pulling them out, sometimes two or three in twenty-four hours. Another consequence was that she had a saline solution running constantly to keep the needle clear of clots, and she got tangled in the tubing fairly often. A regular refrain was "I'm stuck!" which announced that she had reached the end of her tether, or that it has caught on some projection from beds, chairs, or her own feet. It slowed her down, however, not at all.

On Wednesday, November 19, we brought Rebekah to Yale-New Haven Hospital to get her closer to home (and for no other reason; the staff at MEEI were superb). Rebekah spent three nights at Yale-New Haven, most of which was spent making a way for her to go home. She arrived on Wednesday evening, and two specialists attempted to place a long-term IV line in her arm on Thursday, with no success. On Friday, November 21, Dr. Seashore, a surgeon, placed a central line IV near her clavicle. She had a variant of this last year during her radiation therapy.

With this IV in, we were able to bring her home on Saturday, November 22, where Pat O'Brien of NHS home nursing services instructed us in administering her medication. She received an antibiotic called vancomyacin over the course of two hours every eight hours. So we had an IV pole in her bedroom, and an IV pump, and IV bags in the refrigerator (this medication has to be refrigerated). Mercifully, we were able to time all this so that she slept through most of it. That meant, among other things, that she was not tied to an IV pole when she would rather be romping around.

Rebekah was examined on Tuesday, December 2, by Dr. Heidi Remulla, an ophthalmic plastic surgeon at Massachusetts Eye and Ear Infirmary; and by Dr. Shizuo Mukai, her retinal specialist and the primary physician for her retinoblastoma treatment. They were concerned with damage that may have occured before or during her recent post-operative infection, and whether she would be ready to have a prosthesis fitted soon.

Unfortunately, they discovered a tear in the tissue that was placed over the artificial coral implant. This tissue is part of the protection for the implant from infections, and it had to be repaired before she could be fitted with a prosthesis. The tear was not present when she had a CT scan on November 14.

Bekah had been scheduled to have the mold made for her prosthesis on December 9; instead, that OR time was given to Dr. Remulla and Dr. Mukai to repair the tissue over the implant.

The infection, fortunately, was controlled by her IV antibiotics. In the end, she spent from Friday, November 14, to Tuesday, November 18, inpatient at MEEI, and Wednesday, November 19, to Saturday, November 22, at Yale-New Haven Hospital. Home IV care followed until Friday, November 28.

Since the tear in the pericardial tissue wrapped around the implant had developed, Dr. Heidi Remulla placed a graft over it in an outpatient surgery on December 9 at Massachusetts Eye and Ear Infirmary. The graft was donated skin tissue which had had its genetic material removed to reduce the risk of rejection. It was intended to serve as a ladder for Bekah's own tissue to grow over the wound and heal it more permanently.

Dr. Shizuo Mukai also examined Rebekah's left eye while she was anesthetized, and found no indication of tumor growth, infection, or worsening radiation trauma.

Just to make sure we stay on our toes, Bekah had a couple of high fevers after this surgery. Because she is was antibiotics, however, her pediatrician, Dr. Ferrin Holmes, diagnosed a virus--which was a relief, but did seem like adding insult to injury.

We returned to Boston on December 16 so that Dr. Remulla could check the condition of the graft. It remained in place, though it was too early to know if the pericardial tissue would grow over it.

Thursday, January 15, was marked by several things: the last quarterly payment of income taxes for 1997 (oof!), an ice storm in Connecticut (not as bad as the previous week's in northern New York and New England, but bad enough), and two appointments for Rebekah in Boston. We were very glad to have the company of Matt McCaffrey, who was very good to drive us up and back. We were especially grateful for his skills on the icy lanes of Interstate 91!

The first visit was a post-operative follow-up with Dr. Heidi Remulla at the Massachusetts Eye and Ear Infirmary. She was looking for the condition of a graft placed over the implant in her right eye socket, and the progress of healing a rip in the conjunctiva tissue over that implant. She brought in Dr. Peter Rubin, the head of eye orbit plastic surgery at MEEI, for another opinion.

They felt that Bekah was progressing well with this. The graft was in very good shape after five weeks, and showed no signs of holes or melting. The conjunctiva had not grown over, and in one corner had receded slightly, but this did not worry them much. They seemed to feel that it will take time, and afair amount of it. Dr. Remulla asked us to return in a month.

She also said that it would be a while before Bekah could have a prosthesis fitted.

The second visit was to Dr. Eric Grabowski, Rebekah's oncologist at the Massachusetts General Hospital, for her quarterly check-up. He found no signs of other tumors, but abundant evidence that she is two. Early in the visit he said, "Hello, Rebekah" a couple of times, with no response. Then he said, "Well, maybe later."

Bekah said, "Maybe not."

One of the uncertain issues for Dr. Grabowski was the follow-up procedure. He said that there is no agreement among specialists in retinoblastoma on what tests are required in the months or years after treatment. So where he told us last summer that he thought an annual MRI would suffice, he wanted now to schedule one for spring--six months after her last one. We expect this sort of thing to change fairly often as he and his colleagues study the matter.

Rebekah's quarterly examination under anesthesia came around on March 10, 1998, and brought her back to the Massachusetts Eye and Ear Infirmary under the care of Dr. Shizuo Mukai. He reported that he was very pleased with how her remaining left eye is looking. The tumor is smaller than it was in December, and radiation side effects are continuing to pass off. He did see some radiation damage on the retina, which he described as similar to scarring. This is no surprise, of course, but the eye's continued progress is very encouraging.

Dr. Heidi Remulla examined the right eye socket and its recovery from a very bad infection and ripped tissue last November. The graft she placed in December, remarkably, showed no signs of melting or tearing, a thin layer of conjunctiva tissue covered most of it, and it appeared to be well nourished with new blood vessel growth. She did find two small holes that had developed outside of the graft and which had again exposed the marble-sized implant beneath. These were small enough that she was able to correct it with some sewing, and did not require a new or additional graft.

She was pleased enough with the situation that we hoped to be fitting a prosthesis in six to eight weeks. Dr. Remulla wanted to take a look in the clinic again soon, but this was the first time since the infection that the prosthesis had been in the describable future.

It should be noted that, at this point, Rebekah had a great deal of this figured out. She didn't mind visiting her great-aunt and -uncle for a night, but she knew what happens at the hospital and doesn't like it. "Can I listen to your heart, Rebekah," she was asked, and "No!" she replied. She even resisted the bracelet, which I think she associated with her lengthy hospital stay last fall. But my favorite is this impassioned cry:

"No IV!"

She knows.

Rebekah had a follow-up appointment with Dr. Heidi Remulla at the Massachusetts Eye and Ear Infirmary on March 26, 1998, two weeks after Dr. Remulla had examined the healing of a wound over the implant in her right eye socket. At the time, Dr. Remulla saw that that wound was healing, but that a new opening had appeared beyond the limits of the previous one. She repaired these by sewing. Unfortunately, in the follow-up visit, she saw that these had not held, and that the tear in the conjunctiva was even larger. Rebekah needed an additional graft in this new area. That surgery was scheduled for late April.

Among other things, it meant that she would not be fitted for a prosthesis for about four months.

On March 31, 1998, Rebekah had an MRI study at the Massachusetts General Hospital, as part of the general follow-up of her condition. The results of the study were not immediately available.

On Thursday, April 23, Dr. Heidi Remullah performed surgery to attempt to repair the hole in the conjunctiva tissue over the implant in Rebekah's right eye socket. That tissue protects the implant from infection, and it was damaged by the severe infection she had last November. A graft Dr. Remullah inserted in June was still holding, but the original wound had continued to expand beyond the edges of that graft, and the implant had been exposed once more.

This procedure started rather late and went rather long. Dr. Remullah scraped away some amount of the implant material so that she could place the graft over it and then bring together conjunctiva tissue over that, to encourage healing of the wound. To provide some additional protection for it, she placed temporary sutures in Bekah's eyelid to keep it closed. We expected that those would come out the next week.

It was a very long day. We arrived at 8:30 am, and didn't leave the hospital (Mass Eye and Ear, of course) until 6:00 pm. Bekah was a long time perking up after the lengthy anesthesia. To add insult to injury, she came down with a cold over the weekend.

The next week, however, she seemed to be her happy self and was merrily playing around the house. With occasional cries of distress, which were: "I have a runny nose!"

Bekah had two more appointments on May 21, and the news was good. We were given risen to hope that she would have her prosthesis at last in just a couple of weeks.

The first visit was with her oncologist, Dr. Eric Grabowski. These have generally not been very trying visits, except for drawing blood, and this one was no exception to either rule. Dr. Grabowski was quite pleased with how she was doing, and with the results of the MRI scan taken at the end of March. Even the blood drawing went pretty well; Bekah cried and protested, but she was able to calm herself down when she realized that thrashing around would hurt. She even said goodbye nicely!

The second appointment was with Dr. Heidi Remulla, the eye plastic surgeon who had been working to repair the tissue over the implant in Bekah's right eye socket. That eye had been stitched shut since the surgery on April 23, and Dr. Remulla left the stitches in until the next examination under anesthesia on June 9.

The prognosis was that this repair would successful, because Dr. Remulla arranged for the ocularists, the Jahrlings, to attend this EUA and take a mold of the eye socket. We scheduled a visit to their offices on June 10 for the entire day, to fit and match the color of her left eye (they actually paint the prosthesis to the correct colors). We were really looking forward to having this completed, as you can imagine.

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Prosthesis

Bekah had an examination under anesthesia on June 9 at the Massachusetts Eye and Ear Infirmary, and it went very well indeed. It was a busy morning, but the exam went quickly and she came out of anesthesia very nicely. Best of all, there was no recurrence of tumors and they were able to take the mold for her prosthesis.

In order:

Dr. Shizuo Mukai examined her left eye, and found that the tumor showed no signs of regrowth. It was about 10% of the size it was when discovered in October 1997. He saw no signs of new tumors either, although she was developing a slight cataract. The last is a side effect of radiation therapy. Dr. Mukai scheduled his next EUA for September.

Dr. Heidi Remulla looked at the situation in her right eye socket, on which she had performed repair surgery six weeks before, placing an alloderm graft over the exposed implant and drawing conjunctiva tissue over that to further close it. She found that the conjunctiva had again pulled back, exposing the alloderm. The latter, however, was in good condition, and she decided that a prosthesis could be fitted, as long as it was shaped to prevent pressure on the alloderm graft. She scheduled a look at Rebekah in the clinic in about a month's time.

Mr. Eric Jahrling, from Jahrling's ocularists, took a mold of the right eye socket which would be used to prepare a prosthesis. Bekah would spend the better part of the next day in their clinic having it precisely fitted and the color matched to her good left eye. At last!

On June 10, we went to the ocularist's office: Jahrling's, on Staniford St., which is about two blocks from the hospital. Eric Jahrling was the one to work with her (this is a family business; his brother and a cousin, I think, also work from that office).

The process began pretty simply. Bekah sat with Evelyn on a typical ophthalmologist's chair while Eric Jahrling painted a small iris to match her remaining left eye. I had assumed that he would start with a full globe, but he actually began with by choosing a small disk with a hole in the center, like a somewhat thick washer, as a color match. With that as a base, he used very fine brushes and a set of paints to color it exactly. He also used a circular file to widen the hole in the center to match the width of her pupil--he did all this working under a light which is designed to match the color and brightness of an overcast day. It took some time, but less than I'd anticipated.

He then began working with a wax model of the prosthesis. He had already prepared its reverse side from the mold he'd taken the day before; now he was working to match the "height" of the front of the eye above the eyelashes. He did this by applying and shaping small bits of wax to the front of the model, and then inserting it in the socket to compare with the left eye. Then he'd remove it and make the indicated changes.

Bekah, I must say, did not enjoy this part. I don't know if the back of the socket was tender, but I'm pretty sure the eyelid was where it had been sutured for four weeks. She was initially fairly cooperative about having the model inserted and removed, but resisted more as the work went on.

We then left, as the model needed to be turned into an actual prosthesis. So we spent some time at Quincy Market (which is not far from there), and had lunch at the Union Oyster House--yum!

When we returned, he inserted the prosthesis to check its fit and alignment. The pupils, after all, need to be pointed in the same direction! He made some more adjustments, and then we had another break. I think this is when the final coating was put on it, but I'm honestly not sure.

When we returned from some juice and frozen coffee, the finished prosthesis was ready. Rebekah was not at all happy about having it inserted this time, and fought it pretty hard--she'd had no nap, and been pretty good about things for the better part of the day, but now she'd had it. So when the Jahrlings went to take her picture (they keep one of all their patients) they got a wailing face.

Eric Jahrling's comment was, "Another happy customer!"

The prosthesis has been no trouble to us at all. It has shown no signs of falling out, although we have frightened our day care providers by giving explicit instructions on what to do if it does! Bekah shows no interest in playing with it, which is best for keeping it in place. She doesn't like having it cleaned, which consists of squirting water from a bulb across it; she may not have nerves on the surface of the prosthesis, but the ones in the eyelids have the same reflexes!

Brendan was very happy that she finally had her fake eye. He said it made her look much more beautiful. I think that the long weeks of a clear plastic conformer and then the sponge-padded sutures were pretty hard on his sense of what's right. So even though he was bitterly disappointed that she can't see with the fake eye, he certainly approves of its presence.

As for Rebekah, she was explaining that her baby doll didn't like her (the doll's) fake eye last week. So I asked, "Do you like your fake eye, Bekah?"

She thought a moment, and then said, "I think my fake eye makes me look very pretty. I like it."

Rebekah had two checkups on July 14, 1998: one with Eric Jahrling to look at her prosthesis, and the other with Dr. Heidi Remulla to see how her right eye socket is healing. It was a very positive visit.

The morning appointment was with Eric Jahrling, the ocularist who made Rebekah's prosthesis. This was a standard follow-up visit so that he could check the fit and appearance, and take a look at how dirty it had become in a month. It was an early appointment in case he had to do a lot of work over the course of the day, but he didn't: everything looked fine, and we won't need to return there for six months.

In the afternoon Dr. Heidi Remulla, the plastic surgeon, examined the right eye socket to check its healing. She was very, very pleased--the wound was much smaller than when she had repaired it in June. She told us that she had recently read an article that retinoblastoma patients seemed to have somewhat more difficulty with this kind of implant than others, and that to see this healing was very reassuring. She will next see Rebekah at her exam under anesthesia by Dr. Mukai in September.

All of which made for a long day, but the news was very good--and the dinner some good friends brought to us was very good, too, and a great help to two parents tired from the drive.

The Duckling Day

Rebekah had her quarterly checkup with a pediatric oncologist at the Massachusetts General Hospital in Boston on August 13, 1998, and she was just fine.

Like a lot of things that happened around us that summer, there were some odd occurrences. I've tried to tell the story more-or-less in order. It might make sense...

Bekah's oncologist is Dr. Eric Grabowski, and she had this appointment for at least two months. We made it for midafternoon so that we would be able to take Amtrak from New Haven to Boston and avoid the long, tiring, all-too-familiar drive. With our vacation the previous week, however, I didn't do anything about train tickets until the day before the appointment, when I drove to Union Station while on other errands downtown and bought them.

I had no sooner done so, and was in fact in the car on the way home, when Evelyn answered a phone call at home. It was the department secretary at MGH Pediatric Oncology, and she was sorry but Dr. Grabowski was on vacation and she hadn't been able to persuade the computer to tell her what appointments he had until now, and did we want to reschedule or see another doctor? Evelyn said she'd need to talk to me, and she paged me as I pulled into the yard.

We decided that, although we generally prefer the consistency of seeing the same doctor, we had another errand in Boston to accomplish and we'd keep the appointment. Evelyn called them back and so it was set.

We didn't see Dr. Alison Friedman, a new member of the staff, until about 3 pm, an hour late, which isn't surprising given the problems they were having with retrieving appointment information. The visit was remarkable mostly for Bekah's cooperation. She didn't volunteer anything, but kept her resistance to occasional grunts--well, emphatic grunts. The quarterly blood drawing was accompanied by shrieks of protest, but with much shorter duration than usual, and no great struggling. She forgave it rapidly, too, and stood very nicely for her weighing and measuring, which is a first! She also, of course, made sure that her baby doll, Emily, got a bandaid, too.

A swanboat on the pondThat accomplished, we set out on the day's other errand: following the ducks in Robert McCloskey's Make Way for Ducklings. It made for a thoroughly delightful afternoon for two young children and their parents. MGH is close to the Esplanade and the Longfellow Bridge, which is the backdrop behind McCloskey's pictures of the Mallard's nest on an island in the Charles River (I think the island was cut in two for a marina entrance). From there we followed the book's route across Storrow Drive (using the Arthur Fiedler footbridge, as Michael the policeman wasn't available to stop traffic for us), up Mount Vernon St., and down Charles St. to the Public Garden. We found Mrs. Mallard and the ducklings marching along there in a larger-than-life statue along the walkway; Brendan and Rebekah both enjoyed having their photograph taken on Mrs. Mallard's back. We just made it to the swan boats for a ride around the pond--Brendan was disappointed that there were no ducks visible on its small island, and sadly, we had neglected to bring food for the ducks--and had supper at Durgin Park. Rebekah was simply overjoyed that there were ducks to see! From there we walked along the harborside to South Station for our 8:30 pm train back to New Haven.

Now that's the way to do a doctor's visit! The kids thoroughly enjoyed the day, were happy and cooperative with us and each other, and just generally fun to be around. Brendan hiked about with almost no complaints, and Rebekah walked a fair amount of the route herself, riding in her stroller for the rest.

The day produced a newsletter article as well, which you'll find here: "Redemption and Ducklings."

Dr. Shizuo Mukai examined Rebekah's left eye on Tuesday, September 22, and found that the tumors had remained inert; there was no sign of new tumors or regrowth. The radiation side effects also seemed to be passing off, because he didn't mention them. The exception was that he noted a developing cataract, which would eventually need to be removed.

Since she had turned three years old, Dr. Mukai placed her on a schedule of retinal exams every four months rather than three. He also said that, at this age, he no longer expected to see tumors in an exam, which was some comfort--and also somewhat chilling in retrospect.

While Bekah was anesthetized, Dr. Heidi Remulla examined the right eye socket, and unfortunately found that the implant was still exposed in places. She said that the center was thickly covered, but that there are tears at the edges. She asked us if she could take Bekah's case to a conference of plastic surgeons and seek their advice on further treatment.

My impression was that she believed that replacing the implant would probably be necessary and was hoping that one of her colleagues would suggest another option. We hoped so, too.

Implant Surgery

Bekah had a follow-up Magnetic Resonance Imaging scan on Thursday, October 8, 1998; it was the primary means used by her oncologist at that point to look for tumor growth in other possible locations. The results were generally good, but Dr. Grabowski was curious about a slight change at the end of the optic nerve. He thought it was probably scarring, but was glad to have an opportunity to have it biopsied.

In addition, Brendan had a retinal exam that day with Dr. Mukai at Massachusetts Eye and Ear Infirmary--fully awake, because he was a good and brave boy--and he did fine. Dr. Mukai said he didn't need to take a look at him again for two years. Brendan has never been suspected of having retinoblastoma, and the genetic scans don't show the same defect Rebekah has, but with this disease one checks siblings.

The distressing news the previous week came from Dr. Heidi Remulla, the plastic surgeon who had been working on closing the wound in Bekah's right eye socket. After conferring with several colleagues, she came to the conclusion that there was a "non-clinical" infection remaining, which was the reason it wasn't healing. That meant the implant had to come out. To replace it, she recommended using some of Bekah's own tissue rather than an artificial substance, and because she had not performed this on a child, she referred us to a surgeon at a Rhode Island Hospital/Hasbro Children's Hospital in Providence named Dr. Migliore. Sadly, this meant that the entire post-surgical recovery before a prosthesis had to be repeated, and her first prosthesis might be discarded.

On the plus side, we followed the medical visits with a wonderful trip to Virginia over the long weekend to celebrate the baptism of our lovely goddaughter Claire Elizabeth Bickers.

On October 22, 1998, we met with Dr. Michael Migliori, an ocular plastic surgeon in Providence, RI, so that he could examine Rebekah's right eye socket and discuss with us a plan for resolving the problems she had had with exposure of the artificial implant.

Rebekah behaved herself fairly well, although she wouldn't put her chin in the device ophthalmologists use to get a magnified view of the eye, and in any case the exam was quite quick. Dr. Migliori explained that tissue exposed to radiation tends to shrink or recede, and that therefore there was not a lot of flesh available to use to cover the implant (which needs some sort of tissue over it). He recommended that he remove the artificial implant in place, which probably had a low-grade infection in it, take some dermisfat from around her hip, and shape that into an implant which he could then stitch to the eye muscles.

This would have several advantages: no rejection problems, no need to force tissue over it (since it would be living tissue), and it could grow with her and not force her to wear an excessively thick and heavy prosthesis. He was quite certain that removing the current implant would resolve the low-grade infection which Dr. Remulla was convinced exists.

While we were there, we called Dr. Eric Grabowski, who had left a message for us that he wanted to confer about Bekah's MRI scan from three weeks before. He told us that a comparison of that scan and one six months before showed a very slight thickening of the optic nerve in the right eye. This is not a common indication of tumor growth, but he asked if Dr. Migliori would take a biopsy of that during the procedure, which he agreed to do.

Rebekah had surgery on Thursday, November 19, to replace the artificial implant in her right eye socket. After nearly a year of attempts to keep that implant covered with tissue, her doctors at the Massachusetts Eye and Ear Infirmary (Drs. Heidi Remulla, eye plastics, and Shizuo Mukai, retinal specialist) concluded that infection had involved the slightly porous implant. Being artificial, antibiotics could not penetrate it sufficiently to eliminate the infection, so the implant itself needed to be removed.

The repeated tearing of the conjunctiva tissue had left relatively little to use over a new artificial implant, so Dr. Remulla recommended replacing it with one created from her own tissue. As she had only performed this procedure once on an adult, she referred us (with Dr. Mukai's support) to Dr. Michael Migliori, an eye plastic surgeon in Providence, RI, who had been one of her instructors and a classmate of Dr. Mukai's. He performed the surgery at Hasbro Children's Hospital in Providence. Dr. Remulla came down to assist.

This made hospital number four for us, and for the first time one where we did not have family (or our own home) nearby, so we stayed at the Ronald McDonald House located at the edge of the hospital's property. We arrived Wednesday evening to discover that a group of Southwest Airlines employees were preparing a massive Thanksgiving dinner (with all the trimmings) for the guests. We were really very kindly welcomed, and the house was very well set up for families and their children.

On Thursday morning we went to Hasbro for the surgery, and found all the staff to be very skilled, kind, and attentive. The hospital itself is very new and unbelieveably well equipped for its young patients. The fact that it has a nursing school associated with it meant that we had even more staff attention.

Dr. Migliori successfully removed the old implant and fashioned a new one from tissue he removed from Bekah's left hip. He attached all four muscles, and also took a look at some scar tissue on the optic nerve which has looked somewhat different on Bekah's October MRI. He took a biopsy of it for her oncologist, Dr. Eric Grabowski, but didn't think it looked alarming at all. As we've heard nothing, we assume the result was negative.

Bekah took a long time recovering from this procedure. The surgery lasted about an hour, and Bekah woke fairly quickly, but she didn't eat the rest of the day as she felt pretty nauseous. I had to run her security blanket back to the Ronald McDonald House to wash it after one episode. She stayed overnight and was pretty upset when she had to go home on Friday with a bandage taped over her eye.

At home she stayed pretty quiet and unhappy for the better part of three days. She finally started acting more like herself on Tuesday, but still didn't feel up to participating in her nursery school's Thanksgiving party. We decided to keep her home from day care until after she'd had a followup checkup -- the holiday actually made that easier.

Bekah had a good Thanksgiving, though she didn't eat nearly as much as her big brother (I'm not saying anything about what her father ate), but she felt well enough to insist on coming outside for the Thanksgiving croquet tournament until the cold and rain drove her in (long, I might add, before the game ended).

On Tuesday, December 1, we returned to Providence for the follow-up visit to Dr. Migliori. He removed the stitch which held the right eyelid closed and was quite pleased with how everything looked. We scheduled another visit for just after New Year's, and shortly after that she would have her regular exam under anesthesia by Dr. Mukai. We arranged for the Jahrling's prosthetics firm to have someone present to take an impression of the socket for a new prosthesis.

Rebekah had an examination under anesthesia at the Massachusetts Eye and Ear Infirmary on Tuesday, January 12, 1999. The tumors were still well reduced and inactive, which was the most important news of the day.

Dr. Shizuo Mukai did the examination of the left eye, and told us that the radiation-induced cataract there had reached the stage where it needed to be removed. This is a fairly simple procedure, but any surgery in an eye which has retinoblastoma tumors carries some risk that some active cancer cells may escape the eye's orb and travel elsewhere in the body. Because of this concern and because of her age, Dr. Mukai recommended against implanting an artificial lens; a new glasses prescription will need to compensate.

The surgery was scheduled for early February. Though there was no real haste, both her vision and Dr. Mukai's ability to visually inspect the tumors required the cataract to be removed, and we didn't see any real sense in waiting.

In the right eye socket, things were going fairly well. The new implant appeared very healthy. At a follow-up appointment with Dr. Michael Migliori the previous week, on January 7, 1999, he noticed that some skin appeared to be growing on the implant -- this isn't too unusual, as the tissue was taken from just below the skin -- but skin, desireable as it is on the surface of the body, doesn't work well on an implant. Skin sheds dead cells, which could build up and feed infection behind the prosthesis. So he asked Dr. Heidi Remulla to scrape the skin cells away, which she did. She decided to take another look at the situation when Dr. Mukai removed the cataract.

Eric Jahrling of Jahrling's Ocular Prosthetics came in and took a mold of her right eye socket for a new prosthesis. We had been scheduled to have the new one made over the next two days, but he felt that fitting over the skin scraping could be painful or damaging to the implant, so we had to reschedule that set of appointments. As with everything else, that turned out to be two weeks later.

On the positive side, Bekah had relatively little difficulty coming out of anesthesia. She even responded promptly to a nurse's bribe, sitting bolt upright and taking juice at the offer of removal of her IV!

I have to include one other story. Bekah had her quarterly appointment with her oncologist, Dr. Eric Grabowski, in December. That went well, but the story happened in the waiting room. Another mother had a baby there, quite possibly who had been recently diagnosed with retinoblastoma (though I'm not sure of that). She and Evelyn were talking about Rebekah, who was playing around the room, and the woman said that Bekah managed very well, and Evelyn agreed that she did.

Bekah chimed in and said, "I can do anything."

Cataract Surgery

On Tuesday, February 2, 1999, Bekah had cataract removal surgery at the Massachusetts Eye and Ear Infirmary in Boston under the care of Dr. Shizuo Mukai. The surgery went well, and examination of her retina and the right eye socket both had good results. She and Evelyn stayed another night with family in North Reading, Mass, so that she could have more time to recover from anesthesia before the long drive home.

The cataract was the result of her radiation therapy in 1996; because of the size and location of the tumors in her eye, the radiation beam had to include the eye's lens, which then caused the lens to cloud -- which is what a cataract is. The surgery removed the lens through an incision in the cornea. Rebekah will still be able to see, but she will need a glasses prescription that can compensate for the missing lens. That will follow in about a month.

The day began somewhat badly -- we were scheduled to arrive at the hospital at 6 am, so I had set an alarm the night before to wake me at 4:40. Unfortunately, it was a digital alarm, and I set it for 4:40 pm. Oops. My uncle wakened us just after 6, to our great consternation. We phoned the hospital, learned that if we were there by 7 we'd be okay, and headed for the car. Oh, yes, we did dress along the way...

We were at the hospital precisely at 7, and brought Bekah up to the pediatric floor, from which pediatric day surgeries were being handled that day. Bekah was not happy about the eye drops, and pretty disappointed that we didn't have enough time for her to try out the newly renovated playroom. There were a lot of parents and children around, more than I can remember seeing before. When Bekah and I arrived on the surgical level, they guided us to wait in the recovery area -- the preop section was filled with adults and there were three other children waiting with a parent in recovery!

Bekah was grumpy to all medical personnel as usual.

Dr. Mukai came up to talk to us after the surgery, and told us that he would like to check the healing in a couple days, and again in two weeks. Two weeks after that we would make some arrangements to get her a new glasses prescription, but we hadn't settled where to do that. We had seen Dr. Nathalie Azar at MEEI before, but she was expecting a child soon and it wasn't certain she would be available in a month!

Dr. Heidi Remulla also looked in on Rebekah during the procedure to check the new dermisfat graft in her right eye socket. It looked very good, with no new skin growth over it, so we were all set for our visit to the Jahrlings' for her new prosthesis the next week.

Bekah wore a patch and an eye shield called a Fox shield (to keep her from poking at it) for the next two days, but she did not complain or pull at it. I explained about coming back to Boston with her on the train in two days, and she actually brightened up at the prospect of a train ride.

Bekah, her grandfather and I went back to Boston on Thursday, February 4, 1999, for a follow-up checkup with Dr. Mukai at MEEI. She did very well, and Dr. Mukai reduced her eyedrops and freed her from the infamous Fox shield.

The Fox shield is a metal version of the old pirate's eyepatch: it's intended to protect the eye from damage while it heals. The shield is perforated so that the wearer can see out -- but for about thirty-six hours Bekah also had a medical eye patch over her eye as well. That left her unable to see.

Did that stop her? No. Bekah continued to make her way around, and get angry at walls for being in her way. It was particularly startling at home, because she could get around the house very well indeed, feeling for objects that she knew she'd encounter soon. She moved more slowly than usual, but not uncertainly.

Which made me wonder how well she sees ordinarily, with so little difference between one eye and no eye. I simply couldn't figure out whether she saw very badly and had wonderful coping skills, or saw reasonably well and had wonderful coping skills.

By the end of this visit, however, Bekah had the patch off and her glasses on again (safe from ophthalmologists) and the skin irritated by the tape which held the Fox shield on could begin healing.

Rebekah had her new prosthetic right eye matched, sized, and presented on February 9, 1999. It looks great.

We went up Monday (2/8) to stay overnight, as the first appointment was at 8 am. We had an interesting adventure on the way up: we saw a car/tractor-trailer collision on Interstate 290 in Auburn, MA, just about four or five cars ahead of us. We stopped to help, and between the highway getting closed for a medical helicopter landing, police interviews, and cleaning up the road, we stayed there for about an hour and half. As far as we know, there were no critical injuries. We were just glad to have not been part of the crash as well!

On Tuesday at 8 am, we began the process of getting a fake eye. Eric and Joyce Jahrling of Jahrling's Ocular Prosthetics in Boston, MA, used the first appointment to adjust the size and match the color with Bekah's left eye. Rebekah was completely uncooperative. She screamed when the wax model was inserted or removed, and she refused to look at Eric Jahrling when he wanted to check the colors (instead, she'd close her eyes). After an hour and a half, they decided that the fit was okay, but she'd have to come back to check color again at 11:30.

We spent the time in a bookstore in Quincy Market (not far away), where Rebekah found some Winnie-the-Pooh stuffed animals, and chose a baby Roo to take home. To our complete surprise, with Roo along, she became the complete charmer for Eric Jahrling on our return to the ocularists, and he was done in about twenty minutes.

After lunch I had to take a train back to New Haven, and Evelyn and Bekah returned for the final fitting. Evelyn reports that she was more cooperative than the first session, but not as much as the second. In any case, when it was over, she had a splendid new right eye.

On February 18, 1999, Rebekah had a good checkup with Dr. Shizuo Mukai yesterday in his clinic at the Mass Eye and Ear Infirmary. Dr. Mukai examined her left eye to verify it was healing properly after he removed a cataract from it two weeks before. It looked very good to him, and he was even able to get a glimpse of Bekah's retina during the office visit, and that looked good, too.

Over lunch before our appointment, we mentioned to Beth Arnold, the social worker attached to the retinal service, that we were looking forward to Rebekah getting a new prescription, as her eyesight was noticeably worse after the cataract removal. Beth found Dr. Mukai in the cafeteria as well, who promptly turned at the doctor next to him and asked her to see Bekah after he did. She happened to be Dr. Naoko Kohara, a pediatric ophthalmologist, and she was able to see her later in the afternoon and get an objective examination of the eye and determine roughly what her prescription should be.

It was important to Dr. Kohara, however, to also get a subjective examination, that is: to get some information from Rebekah herself about what she could see. That wasn't happening. By the time we saw Dr. Kohara, Rebekah had had dialating eyedrops, which she hates, had a retinal exam, which involves shining bright lights into her eye in a dark room, which she hates, then had another one, which she didn't like any better, and then had an objective prescription exam, which involves _more_ shining of lights into the eye in a dark room, which she...

In any case, Rebekah was in no mood to look through lenses or identify pictures for Dr. Kohara. So we scheduled a return to Boston next week for a follow-up exam, to hopefully result in a usable prescription. Dr. Kohara said that it was quite important to do it soon, so that Bekah will be accustomed to using all the vision she has.

Rebekah was pretty tired after all this, and fell asleep pretty quickly the second time I tucked her in on the train ride home.

Rebekah visited her oncologist, Dr. Eric Grabowski, at the Massachusetts General Hospital on March 18, 1999. This was a regular quarterly exam, and things looked good.

Bekah did not, however, feel particularly helpful. She did have a cold, and that makes her more tired and grumpy than usual. So do physicians. She resisted the exam with varying degrees of emphasis -- mostly lots of "No!" but occasional struggles as well. Sigh.

Dr. Grabowski has said in the past that the best indicator of her health is her ability to resist. Well, she's healthy!

He also noted that the bones of her skull were growing symmetrically, and that she was getting taller as her long bones start a growth spurt. We don't know exactly how tall she was, because she pretty successfully resisted that measurement.

One very bright note was that since she had an exam under anesthesia next week, Dr. Grabowski did not draw blood for tests. His colleagues at the Mass Eye and Ear would do that next week. We were all -- medical staff included -- pretty grateful to avoid that!

On Tuesday, March 23, 1999 Rebekah had an examination under anesthesia by Dr. Shizuo Mukai at the Massachusetts Eye and Ear Infirmary. The exam had quite an agenda:

All of these went well. The eye had healed, and the tumors remained unchanged. Dr. Mukai did find, of all things, a hair growing on the implant, but did nothing about it and would confer with his colleagues about whether anything needs doing. And he also did the objective examination, though we did not receive the results to give the prescription to an optician.

As for Rebekah, she turned on the charm. She emerged pretty happily from her bed (at 5:45 am) and played nicely at the hospital. She cooperated for the nurses and doctors listening to her heartbeat, and after we had told the anesthesiologist which hand to put her IV in, chipped in herself:

"Yes, you need to put the IV in this hand," as she held it up, "so that I can suck this thumb," switching hands. The anesthesiologist nearly melted, and Evelyn and I just gazed at each other with a "who is this child and what happened to Rebekah?" expression.

She even woke well from the anesthesia, with no nausea or great distress. We were out of the hospital by 10:45!

Evelyn and Bekah then headed back to stay with my aunt, while I took the train home to spend the night with Brendan.

On Wednesday, March 24, 1999, Evelyn and Bekah returned to Boston for a visit to Jahrlings' Ocular Prosthetics. This time monster-daughter appeared. The visit was simply to do a quick check of the prosthesis' look and fit, and I gather it went a little longer than anticipated. Eric Jahrling actually stuffed cotton in his ears so that he could do his work without ear damage!

By the time I saw her later that morning, she was a happier child.

Rebekah has had two exams under anesthesia (EUAs) by Dr. Shizuo Mukai at the Massachusetts Eye and Ear Infirmary. The first was on March 23, and the more recent one on Tuesday, July 27. Rebekah's left eye continues to look good, with no new tumor growth. At the July appointment he did find exposed sutures from her cataract removal in January, which he removed. Her next EUA will be in November.

Bekah at these last two appointments has been exceptionally cooperative. She is thoroughly aware of the procedures, and though she definitely does not like being anesthetized, does not resist very much. The result has been, mercifully, a much quicker recovery time after the procedure. At this last one, she took a few sips of juice and then said, "And now I can have my IV out!" Persuaded that she should have a little more liquid, she wolfed down a popsicle and repeated, "Who's going to take my IV out?" So, one of the nurses took it out!

Rebekah also visited Dr. Michael Migliori at his office in Providence on June 8. Dr. Migliori performed the surgery to rebuild her right eye socket last December. This will be the last follow-up visit with him unless there are problems. As he said, "You don't need to come all this way to have me tell you things look good!"

On June 17, Bekah made her quarterly visit to Dr. Eric Grabowski at the Massachusetts General Hospital. She isn't certain yet that she wants to be cooperative for this visit (which mostly involves standard physical examination stuff: stethoscope listening, ear examining, and abdominal prodding), but she was better than she has been. Her Waterloo is the weighing and measuring part, which for some reason she really resists. In any case, she remains in good general health. We are scheduled to have the second of her yearly MRI scans done in early fall.

In other news, Bekah got a new glasses prescription this spring to compensate for the cataract removal, which has been a great success. The fitting was something of a trial, however. She refused to try on her new glasses, insisting that the old ones were fine. Evelyn at last gave up and took her back to the car, but refused to leave until she had them on. After some time, Bekah donned the glasses and announced, "They hurt." Evelyn said that was because she hadn't let the optician fit them. Bekah thought briefly about this and said, "Okay. Let's go do that." So in they went again and Bekah was completely charming.

Since then, Bekah reports that the new glasses really do help her see better. She frequently wears them while sleeping, as she doesn't like the delay in putting them on in the morning. I can't really detect much change in what she does, but she has been so good at compensating for so long that I'm not sure what she now does visually that she hadn't done before.

On Thursday, October 28, 1999, Rebekah had her quarterly appointment with her oncologist, Dr. Eric Grabowski, and got a good report. She was also a good girl, and got a bribe. 8-{)

This was actually a dual purpose visit and a long day of medicining for the entire family. I actually typed it aboard the train on the way home.

In any case, Rebekah and I took the train to Boston Wednesday evening to join Evelyn, who was volunteering at the Mass General Hospital and Mass Eye and Ear Infirmary as a chaplain during her sabbatical. Brendan stayed home where Evelyn's parents looked after him (thanks, Mom and Dad!). We then piled into the car for the short trip to my Uncle Dan and Aunt Nancy's house (thanks, Uncle Dan and Aunt Nancy!) to stay overnight before the appointments Thursday.

Fortunately, Bekah did not have an early appointment that required us to face rush hour traffic, but she did have an anesthesia scheduled for the afternoon, so we woke early to try to give her something to eat to tide her over. Unfortunately, she has learned the "routine" of anesthesia too well. She simply didn't believe that she was really allowed to eat the morning she had anesthesia, even if it was going to be later in the day, so she ate very sparingly indeed.

At 11 am we had safely negotiated the in-the-process-of-rebuilding interchange from I-93 to Storrow Drive and were waiting for Dr. Grabowski. He saw us late, but with his usual quiet politeness and attentiveness. Rebekah cooperated very well for her height and weight measurement, and fairly well for Dr. Grabowski's examination. He noted that she has grown a full inch since he saw her in July, and has gained weight as well -- everyone who saw her today has noted how tall she's getting.

The second appointment was a Magnetic Resonance Imaging Scan (MRI), also at Mass General. For this Rebekah behaved, if anything, even better than she had for the less involved appointment with Dr. Grabowski! She was subdued, but still willing to chat, and far more responsive with the nurses in MGH's day surgery clinic. Of course, the relaxing drug she was given before the anesthesia may have helped...

As seems usual with the MRI, there was a delay. A trauma case had to be taken immediately ahead of her, and we did a good deal of waiting. The scan itself took much longer than we'd expected as well, and I had to run down to South Station to exchange our 6 pm tickets for 8 pm. It was, in fact, at about 6:00 that we actually left the hospital, Rebekah having consumed some apple juice and an orange popsicle, and riding on my shoulders to the T station.

Incidentally, she received the bribe when Evelyn joined us at the Rte 128 station (where she left her car preparatory to returning to Boston a couple of days later): she had successfully found a toy store where she purchased three Star Wars: The Phantom Menace action figures that Bekah had requested -- Anakin Skywalker, Obi-Wan Kenobi, and Queen Amidala. We'd told her she'd get two figures if she was very good, and so three was a surprise.

I should mention that she wanted to know why she didn't get them all. Ah, well.

Bekah returned to Boston on Monday, November 22, for a follow-up blood test. When she visited Dr. Eric Grabowski in late October, he ordered blood drawn for routine tests while she was anesthetized for her MRI scan. A day later, he called and told us that the blood test showed a low white cell count, which could have been related to anesthesia, so would we have it drawn again while she was awake? We did, at our own pediatrician's, and it came back still low.

Dr. Grabowski remained fairly unconcerned, as Bekah had been recovering from a cold and he though that the most likely reason, but he still wanted it repeated, so Bekah and I stepped from the train platform in Boston's South Station and made our way to the pediatric oncology/hematology clinic at Massachusetts General Hospital, where Evelyn met us (she'd been working as a chaplain at MGH and MEEI during her sabbatical). Rebekah was exceptionally well behaved for the blood drawing, and fairly shy for meeting Evelyn's colleagues in the chaplain's office.

On Tuesday morning, November 23, we returned to the Massachusetts Eye and Ear Institute for her periodic eye exam under anesthesia by Dr. Shizuo Mukai. The traffic into Boston that morning was dreadful, so we were somewhat late, and Dr. Mukai's first patient had been unable to come in because of fog on the south shore, so Rebekah got in very quickly. She was very, very well behaved for it all, and for the first time didn't cry as the anesthesia mask was placed over her mouth. Some of this was due to a dose of verced, which is a relaxing medicine they have taken to giving her in the last year. I think her increasing weight had them giving her a stronger dose this time, because she was relaxed beyond belief: at one point she leaned rather unsteadily on her mother's shoulder and sighed, "I have such a good feeling!"

Dr. Mukai found nothing of concern in the exam, and the next was scheduled for March.

Bekah came out of the anesthesia very well, too, and we were out of the hospital by 12:30, much to our surprise -- Bekah's longer procedures in the past have meant much longer recovery times. We decided to catch the 1:45 pm train from Boston (Evelyn had to meet us at Route 128 station further south, where she left her car). I worried how likely it was that I'd be able to save a seat for her two days before Thanksgiving, and I was right to worry! The train was packed, but I managed it, though not without a couple of unhappy looks...

Rebekah had an appointment on January 28, 2000, with Dr. Robert Scarpatti, a family optometrist in Farmington, CT, used by the State of Connecticut's Board of Education and Services for the Blind to do assessments of those who receive their services. It was a fairly short, non-traumatic visit, and we think some good help will come out of it.

Bekah saw Dr. Scarpatti two years ago, so things had changed a good deal since then. Dr. Scarpatti hadn't known, for example, about her cataract removal a year before, and he was very attentive to evaluating the effectiveness of her glasses prescription. He found that she would definitely benefit from stronger lenses and began making them up for her; since the state would pay for them we didn't know how long that would take. He also got Bekah to try a magnifier she could use for looking at words and pictures on paper called a Jupiter loupe. She definitely preferred using it for looking at his pictures and so he ordered one of those, too.

Bekah tried using a monocular, but didn't seem very impressed by it, and Dr. Scarpatti recommended trying it again in six to twelve months to see if she would like it any better then.

Rebekah was quite cooperative (if somewhat closed-mouthed) for the exam, but we discovered an interesting problem: she couldn't identify line drawings of a house or an umbrella because the simple drawings didn't look like what _she_ identifies as those objects. She could distinguish one from another, however, so Dr. Scarpatti got her to compare cards he held up with cards she had in front of her, and that seemed to work pretty well.

All in all, a good visit -- and no anesthesia. 8-{)

We made the trip to Boston again on Thursday, March 9, to visit Rebekah's oncologist, Dr. Eric Grabowski, at the Massachusetts General Hospital. We made the trip in some style aboard Amtrak's all-electric Acela Regional service, which cut 55 minutes from the journey.

Rebekah was initially uncertain at the doctor's office. She happily hopped on the scale, but took a great deal of persuasion to stand for her height measurement and postively kicked through her blood pressure measurement (note that she didn't scream -- we warned her that if the nurse couldn't hear she'd have to do it again!). After some time quieting down, though, she happily chatted with Dr. Grabowski and allowed him to do his checks quite freely. In fact, she giggled a lot as he checked those underarm lymph nodes. He also won her over by deciding that he could wait on a blood test until her exam under anesthesia in three weeks.

Dr. Grabowski was pleased with how she is doing, and told us that she has passed the time from diagnosis when a tumor of the pituitary region (known as "trilateral retinoblastoma") is most likely to appear. In other words, that risk is now comparitively low, which is very good news. There will be other risks for other tumors to come, but this is a very important one to be past.

She will be having another MRI study this summer as well as her continued follow-up EUAs with Dr. Mukai.

And in other good news, Dr. Grabowski said that her latest ear infection had pretty well cleared up.

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For more information

Some informative if somewhat frightening links:

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Email Notification by PageWatch

When I first posted this page last October, I updated it nearly every day for a few weeks. As diagnosis became treatment, the updates slowed, and now that treatment has turned to checkups, new information is posted only once every month or two. So I invite you to use the PageWatch service, which is free; after you submit the form below, you will receive a confirming email, and after that you will receive email a day or two after I update the page. Cool, huh?

One caveat: though PageWatch is free to you (and me), the folks at MicroMat need to eat, so part of the email message to you will be advertising for somebody else. Such is life in America.

To be notified by Email when this page is updated enter
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And there is an alternative. I send email to a long list of folks every time she has a checkup; it usually goes out a couple days before I update this page. If you aren't on that list and would like to be, let me know via email at Bekahnews@computerseraph.com. On the subject line, or in the body of the message, type "Subscribe to Bekah News". And on the list you'll go!

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Eric Anderson
5 Brainerd Dr.
Portland, CT 06480-1517
(860) 342-0774 (home)
(860) 342-1155 (Eric's office)
ESAnderson@computerseraph.com

http://www.computerseraph.com/Trains/Bekah.htm -- Revised: 12-Oct-00
Copyright © 1999 Eric S. Anderson
ESAnderson@computerseraph.com

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