Frequently Asked Questions about Rebekah

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What is Rebekah's disease?

Rebekah has a form of cancer called retinoblastoma. It is quite rare, striking one child in fifteen thousand, and usually appears in children between the ages of six to eighteen months. For more information, check out these links. She has one tumor in the left eye, and three in the right eye, which caused a retinal detachment leaving her unable to see. The detachment has corrected itself, and some sight has returned.

Has the cancer spread?

It has not. Her general good health and activity are the best sign of that, and a cytology scan of her spinal fluid (retinoblastoma, when it spreads, spreads on nervous fluid) came back with no sign of cancer cells. Other tests, including quarterly blood tests, various CT and MRI scans, as well as pathology reports, have continued to show no signs of spreading.

What is the treatment?

Rebekah was treated with directed proton radiation at the Harvard Cyclotron Laboratory in Cambridge, Massachusetts, under the direction of the Northeast Proton Therapy Center. She had treatments on Mondays, Wednesdays, Thursdays, and Fridays. There were twenty-two of these visits, which ended November 29, 1996. For each treatment, she was anesthetized and placed in position, then exposed to the proton radiation for a very brief period, less than two minutes. While she was in the treatment room itself for about in hour, most of that time was spent getting her into position (once for each eye) and preparing the equipment.

What is the expectation from the treatment?

Retinoblastoma is very vulnerable to radiation, and the doctors anticipate that the cancer will be completely eliminated. The tumors are shrinking, will continue to shrink in the weeks following the end of treatment, and any tumor tissue that has not been reduced should be inert. Dr. Shizuo Mukai, her ophthalmologist, does not feel that other treatment should be necessary, but he will examine her every three months until the age of three.

The outlook for her eyesight is very hopeful. While there is scarring from the retinal detachment, no tumors remain on the center of vision. She has shown considerable improvement in her sight since about the mid-point of her treatment.

Though successful on the tumors, the radiation also caused other damage to her eyes. In her right eye this became more than the body could correct, and her right eye was removed on October 6, 1997. The surgery went very well, but she suffered an infection in the eye socket that November.

What happens when an eye is removed?

When Dr. Mukai performed the enucleation, or removal, of the right eye, he placed a slightly porous, coral-like implant in the socket, which he attached to the muscles which control eye movement. He then wrapped conjunctiva tissue (a sort of skin) over the surface of the implant as an infection barrier. Typically, this would have healed in six to eight weeks and a prosthetic eye inserted to float atop it. A careful fit would allow the prosthesis to track with her good eye because of the implant's attachment to the eye muscles.

Rebekah had a difficult time with her enucleation. The major problem was the infection in November, 1997, which was followed by the discovery of a hole in the conjunctiva, exposing the surface of the implant. Without that covering, the implant is vulnerable to infection and might have to be replaced. Dr. Heidi Remulla has attempted to close this hole since December, 1997, using alloderm grafts and physical reduction of the implant's size. So far, while adequately healed to allow fitting of a prosthesis, there is still not a good covering of conjunctiva tissue. The primary reasons for this are the heavy doses of radiation to which this eye was subjected, followed by the further insult of a bad infection.

In June 1998 Rebekah had a prosthesis fitted by the Eric Jahrling, an ocularist. The prosthesis looks very good, and is indistinguishable from a real eye except when there are major differences in the pupil size (as in bright sunlight).

How much can she see now?

That is uncertain, because she can't tell us with words. She can definitely see to get through doorways and avoid furniture. She can see to reach for and pick up a Cheerio, but close observation shows that she has to feel for its exact location. An examination by Dr. Robert Scarpatti in October 1997, after the removal of her right eye, showed fairly good central vision but problems with her visual field, which are difficult to quantify. She is legally considered blind right now.

What caused this?

Retinoblastoma in both eyes has a genetic root. There is a defect in a gene pair on one of her chromosomes which has allowed these tumors to form. It seems that this is a new mutation, as there is no history for this or any related cancers in the family, and a DNA analysis found that only she has the mutated gene.

What are the side effects of her treatment?

In the short term, very little. She developed pink spots like sunburn on the sites the beam enters her skin, which faded very quickly.

In the middle term, she developed problems in March related to the radiation. Additional blood vessels began growing in her retinas, primarily in the right. This caused the retina in the right eye to detach again, and has the potential to bring in fluid which will increase the pressure in the eye resulting in a form of glaucoma. As of early April, the visible effects were subsiding and the retina starting to re-attach, but the pressure in her right eye was somewhat elevated. The pressure continued to rise, and had to be controlled with eyedrops, and then hemorrhages began to appear in the eye. The eye was removed when Dr. Mukai reported that he was no longer able to be confident of the state of the tumors because he had difficulty seeing them in a retinal exam.

In the longer term, the location of the tumors requires that the lenses of her eyes are in the radiation beam. This will cause cataracts within two years time. In addition, radiation kills bone growth cells, and she will probably develop small indentations on her temples as she grows. Of greatest concern is the fact that radiation increases the risk for other cancers. We are hoping that the use of proton therapy, with its ability to confine the radiation much more precisely, will reduce if not eliminate this risk.

How is she?

Rebekah is bearing up very well. She is active, playful, and interested. She walks and loves to talk. Much like her brother, she loves to tell stories. She likes toys that make noise or which she can move. Her favorite toys are her baby dolls, whom she mothers with great affection. She tends to name them after the people who gave them to her: thus "Baby Emily," "Baby Patty," and "Baby Cynthia."

She was not happy about the medical procedures she experienced four times a week. She had a central line IV for the administration of anesthesia, and although she didn't pull on it or play with it, she really disliked having anyone else do something with it. This included the daily flushing with a heparin solution and the connection for administering anesthesia. The central line was removed on December 10, 1996, to her, and our, great relief. During November 1997 she learned about being semi-permanently attached to an IV pole, and found her own ways to maintain her freedom with it.

Since her treatments concluded, Rebekah has been extremely happy. She attends twice-a-week day care at the Northford Early Childhood Development Center, where she adores the opportunity to play with other children.

She has developed a real awareness of medical procedures, in particular, IV's. When she is waiting for an examination underanesthesia, one is likely to hear an assertive childish voice call, "I don't want an IV!" And she has learned that if she is wearing her glasses, she can't be examined by an ophthalmologist, so she removes them with great reluctance.