TOGETHER WE CAN CURE THIS CANCER

ONCOLOGY SERVICE, WILLS EYE HOSPITA L PHILADELPHIA, PENNSYLVA N I A

W I N N I N G T H E B A T T L E O F L I F E A N D S I G H T

RETINOBLASTOMA

TOGETHER WE CAN

CURE THIS CANCER

Carol L. Shields, M.D. Barbra Bellis Neilly Stewart, R.N., M.S., M.P.H. and members of the Ocular Oncology Team Queen Warrick, Utrasound Technician Leslie Botti, Ophthalmic Technician Jeanette Soveral-Escalante Technician Kelly Ganzel, Patient Counselor Miguel A. Materin, M.D. Brian P. Marr, M.D. Arman Mashayekhi, M.D. Jerry A. Shields, M.D. Jeanne Zatavesky, Practice Administrator Photography by Sarah Harmon Support provided by The Eye Tumor Research Foundation, Incorporated, Philadelphia, PA Macula Foundation, New York, NY Black Patch Invitational Golf Tournament, Downingtown, PA Rosenthal Award of the Macula Society, Barcelona, Spain Paul Kayser International Award of Merit in Retina Research, Houston TX Mellon Charitable Giving from the Martha W. Rogers Charitable Trust . . . and generous donations from Michael, Bruce, and Ellen Ratner, New York, NY

Robert “Boomer” Kistler, Downingtown, PA Hess Foundation, Roseland, NJ IEW Construction Group, Trenton, NJ Carmella Brown estate, Hightstown, NJ and many others

TABLE OF CONTENTS

1. Oncology Service Wills Eye Hospital 4

A. Wills Eye Hospital 4

B. Oncology Service 5

2. Anatomy of the Eye 6

3. Retinoblastoma 7 A. What is Retinoblastoma 7 B. Examination 7 C. Diagnosis 8 D. Treatment 9 • Chemoreduction 9 • Thermotherapy 10 • Laser Photocoagulation 10 • Cryotherapy 10 • Plaque Radiotherapy 10

• External Beam Radiotherapy 10

• Enucleation 11 E. Prognosis 11 F. Genetic Counseling 12 4. Ocular Prosthesis 14 5. Future Appointments 14 6. Research 15 7. Funding 15

8. Frequently Asked Questions 17

9. Pearls for Patients 19

10. Worthwhile Websites 20

11. Funding 21

THE HISTORY ~

Wills Eye Hospital was founded in 1832 from the $116,000 bequest by Philadelphia Quaker merchant James Wills, Jr. As a medical specialty institution from its inception, Wills was unique from the start. The hospital quickly became recognized as a pioneer institution for the prevention and treatment of eye diseases.

INNOVATIVE DOCTORS ~

O ver the ye a r s, the ophthalmologists at Wills Eye Hospital became subspecialized into fields of cataract surgery, glaucoma, retina, pediatric ophthalmology, cornea, n e u r o - o p h t h a l m o l o g y, oculoplastics, and ocular oncology. Wills doctors have been recognized among the first in the United States to perform innovative procedures such as the placement of an intraocular lens following cataract surgery, new methods of laser photocoagulation for glaucoma and retinal diseases, application of a focal radiation device called a plaque to treat a cancer within the eye, and the implantation of a natural-appearing, artificial eye with movement following removal of the eye for cancer or infection. Wills doctors have been leaders in the development of improve d medical and surgery techniques, and they are regarded as prominent contributors in their subspecialties. Wills Eye Hospital is routinely ranked among the best eye institutions in the United States.

Worldwide, the Ocular Oncology Service at Wills Eye Hospital is

recognized as a leader in eye tumors from both clinical and

research perspectives.

ONCOLOGY SERVICE AT WILLS EYE HOSPITAL

Wills Eye Hospital has been an active leader in the field of ophthalmology

since 1834. It is the nation’s oldest and largest eye care facility.

THE ONCOLOGY SERVICE ~

The Ocular Oncology Service is the largest eye tumor service in the nation. It is one of the few departments in the world to specifically assist patients who are diagnosed with an eye tumor. Worldwide, the Ocular Oncology Service at Wills E ye Hospital is recognized as a leader in eye tumors from both clinical and research perspectives.

WHAT HAPPENS ON THE ONCOLOGY SERVICE ~

The Ocular Oncology Service was established in 1974, and since its inception, has been d e voted exclusively to the diagnosis and management of patients with eye tumors. Currently, the staff of the Oncology Service treats approximately 1,400 new patients with eye tumors each year. Some of these patients have benign conditions such as a choroidal nevus or conjunctival nevus. Unfortunately, approximately one-half of these patients have malignant, life-threatening conditions such as choroidal melanoma, choroidal metastasis, and retinoblastoma. Approximately 30% of all patients with uveal melanoma in the United States and nearly 50% of all children with retinoblastoma in the United States are managed on the Oncology Service. This amounts to approximately 500 new patients with melanoma and 120 new patients with retinoblastoma each year.

WHERE ARE THE PATIENTS FROM ~ Overall, eye tumors are uncommon. Most eye doctors rarely see a patient with an eye tumor, so affected patients typically travel great distances to seek modern treatment methods. Each week, doctors on the Oncology Service care for patients from many states in the

United States, as close as New York to as remote as Alaska and Hawaii. Patients have visited from every state in the nation. But the need for care extends far beyond the borders of the United States and patients from Europe, South America, Asia, Africa, and Australia are frequently treated on the Oncology Service. WHERE ARE THE DOCTORS FROM ~ The Oncology Service is world renown and doctors travel from “all corners of the earth” to visit and learn from this experienced team. Some doctors visit for a few weeks while others devote one or two years in fellowship training to perfect their skills to use in their home country. Over the past three decades, the Oncology Service has trained fellows from many countries including Canada, Mexico, Brazil, Argentina, Chile, Colombia, Venezuela, Germany, Italy, Belgium, Spain, Turkey, Iran, Egypt, China, Korea, Phillipines, India, Australia, and others.

TELL ME ABOUT THE NURSES AND TECHNICIANS ~

The Oncology Service has experienced nurses, technicians, photographers, and staff who are familiar with eye tumors. The nurses and technicians assist the doctor in the evaluation of the patient. The ancillary staff are well informed and provide a strong source of information and help for the family.

ANATOMY OF THE EYE

The eye is the visual organ of the human body. It is a complex organ, designed to focus light through the front portion of the eye (cornea), through the midportion of the eye (lens), onto the back portion of the eye (retina).

A cross section of the eye shows three basic layers of tissue. The outer layer is a white, tough, protective layer called the sclera. This white layer of the eye is readily visible to observers. The cornea is made of similar tissue to the sclera, but it is transparent and is important in focusing light into the eye. The middle layer of the eye is the uvea, or uveal tract. This layer provides blood flow and nutrition to the eye. The inner layer of the e ye is the retina. It is a very delicate transparent laye r, measuring only

approximately 200 microns in thickness. The retina is light sensitive and provides sight for the eye. The retina is comprised primarily of nerves and blood vessels that exit the eye at the optic nerve. The optic nerve leads directly to the brain. The most important cancer to arise from the retina is retinoblastoma.

The uvea is subdivided into 3 anatomic portions termed the iris (anterior part), ciliary body (middle part), and choroid (posterior part). The uvea is comprised of blood vessels and pigmented cells called melanocytes.

RETINOBLASTOMA

A. WHAT IS RETINOBLASTOMA ~

What is it ~

Retinoblastoma is the most common eye cancer in children. It is a life-threatening cancer of the retina within the back of the eye. Retinoblastoma is generally discovered in babies between the ages of 6 and 24 months, although it can be found at earlier or later ages. Retinoblastoma occurs in approximately 1 in 15,000 live births, and it is estimated to affect approximately 250 to 300 children each year in the United States. Worldwide, about 5,000 children develop retinoblastoma each year.

Who gets it ~

Retinoblastoma occurs equally in boys and girls and it is seen in all races. Retinoblastoma affects only one eye in about 70% of patients and it affects both eyes in 30% of patients. In some instances, there is a family history of retinoblastoma in a parent or relative, but in the majority of cases, there is no other member of the family affected with the tumor.

What causes it ~

The cause of retinoblastoma is unknown. It has not been related to nutrition, smoking, drinking, or any maternal problem during pregnancy. It is not related to environmental t o x i n s. It can develop in an otherwise h e a l t hy child despite a normal birth and early development.

What does it look like ~

Retinoblastoma develops from a single affected cell in the retina and it is invisible at its inception. Later, a small white nodule is

noted in the retina, but this is not visible to the parents and it does not affect the child at this point. With time, the tumor grows into a larger white vascular tumor that causes the child to painlessly lose vision and this can manifest with a drifting eye or an eye with a white or glassy-appearing pupil. At this point, the tumor is visible to the parents or grandparents and the child is typically taken to the doctor.

B. EXAMINATION

Once a child is suspected of hav i n g retinoblastoma, a complete eye and systemic examination is required.

Eye examination ~

The ophthalmologist who suspects retinoblastoma typically refers the child relatively urgently (within one or two days or weeks) to a retinoblastoma expert. The retinoblastoma specialist evaluates the child’s medical and family history, examines parents and family members, and then specifically focuses on the child’s eye examination. The eyes are checked with the pupils dilated. The doctor wears a headlight, called an indirect ophthalmoscope, and she/he uses a focusing lens to see all of the

details in the back of the eye. It is generally necessary to restrain the child to steady the head and body so that a reliable examination can be performed. Numbing eyedrops are placed on the child’s eyes for comfort. The child’s eyelids are held open by a lid speculum and soft cotton applicators are used to gently rotate the eyes to allow maximal visualization of the back of the eye. This is generally not painful to the child, but the child might cry.

Systemic examination ~

The pediatric examination is performed by a pediatrician or a pediatric oncologist. The systemic examination is based on how the eyes appear and what treatment is needed. It might include:

• Physical examination

• CT or MRI of brain and orbits • Blood tests

• Bone marrow analysis • Hearing and kidney tests

If on chemotherapy, the hearing and brain tests are important. The brain CT and MRI are usually performed twice yearly until age 5 ye a r s. These tests are done to evaluate for possible spread of the tumor as well as other related cancers.

C. DIAGNOSIS

How do we know it is retinoblastoma ~

The diagnosis of retinoblastoma depends primarily on findings seen on eye examination. The child with retinoblastoma may be found to have a crossed eye or a white pupil by the parents or a pediatrician and then referred to an eye specialist for an

e ye examination. On examination, retinoblastoma has a classic chalky-white appearance with prominent blood vessels. It is rarely necessary to biopsy the tumor for confirmation.

To better assess the tumor, an examination with sedation of the child in the operating room, called an examination under anesthesia or EUA, is usually necessary. During EUA, a complete eye examination with a detailed drawing of all tumors and related findings is performed. Other confirmatory tests like

p h o t o g r a p hy, ultrasonography, and fluorescein angiography are performed. These tests assist in planning the treatment strategy for the child. Fluorescein angiography uses an intravenous dye that discolors the child’s urine to a green-yellow color for one day.

If treatment is necessary, it is usually performed while the child is under anesthesia. Later, the doctors discuss the findings and treatment with the family. When the child recovers, the eyes might appear somewhat swollen and red for about 2 or 3 weeks. Future appointments are scheduled. If the child is in the midst of treatment, appointments are about 1 month apart. If the child is stable, then appointments are usually 2 to 6 months apart. Lifelong, all children with retinoblastoma should have an eye examination once or twice yearly.

D. TREATMENT

GOALS OF TREATMENT ~

The goals of treatment of a child with retinoblastoma are threefold

1. to save the child’s life 2. to save at least one eye 3. to protect the vision

There are several ways to treat retinoblastoma depending on the size, location, and multiplicity of tumors as well as the child’s age, status of the opposite eye, and systemic condition. The treatment methods include chemoreduction, thermotherapy, laser photocoagulation, cryo t h e r a py, plaque r a d i o t h e r a py, external beam radiotherapy, and enucleation. These are often used in combination.

CHEMOREDUCTION ~

Chemoreduction is a method of using i n t r avenous chemotherapy to reduce retinoblastoma to a small size so that the residual tumors can be eradicated with focal treatment methods like thermotherapy or c r yo t h e r a py. Chemoreduction is used in nearly all children with bilateral retinoblastoma and about 25% of children with unilateral retinoblastoma. This technique invo l ves delivery of intrave n o u s chemotherapy each month for 6 months. The delivery takes about 2 days of each month and is usually performed in Philadelphia at Children’s Hospital of Philadelphia. Each c h e m o t h e r a py session is coupled with an examination under anesthesia so that treatment of the tumor scars can be done the same day.

Retinoblastoma before chemoreduction

THERMOTHERAPY ~

Thermotherapy is a laser method in which the residual tumor is heated for 5 to 15 minutes to a temperature that kills the cells. It is focal so that the surrounding tissue is unaffected. It typically leaves no external scars on the eye, but some children might show an irregular pupil after treatment.

LASER PHOTOCOAGULATION ~

Laser photocoagulation is a method to treat a small retinoblastoma by closing the blood vessels to the tumor with heat. This causes no external scars on the eye, but leaves a small scar on the retina.

CRYOTHERAPY ~

Cryotherapy involves the use of a probe the size of a pencil placed on the eye to deliver a focal freeze for about 1 minute through the wall of the eye into a tumor inside the eye. Surrounding tissue is usually unaffected from this treatment. It typically leaves no external scars on the eye, but the eye is often swollen for 2 or 3 weeks.

PLAQUE RADIOTHERAPY ~

Plaque radiotherapy is a method of giving focal x-ray treatment to a small part of the eye by using a piece of metal, called a plaque. The plaque has implanted radiation and it is sutured temporarily onto the eye directly over the retinoblastoma. The child stays in the hospital for several days (usually 3 to 7 days) while the radiation plaque is in place. The radiation dose to the retinoblastoma is quite focal and limited to the eye itself with little radiation elsewhere to the body. After the correct dose is given, the plaque is removed in the operating room and the child is discharged. Eye drops will be prescribed to be

used 3 times a day for 3 weeks. The eye heals well over a few weeks. Long term concerns include vision loss from cataract or retinal swelling. We have not seen radiation related second cancers following plaque treatment. EXTERNAL BEAM RADIOTHERAPY ~ External beam radiotherapy is a method of x-ray treatment to the entire eye by a radiation m a c h i n e. This is available at only a few experienced centers worldwide. It takes about 4 weeks (Monday through Friday) in which small doses of radiation are given to achieve the final dose. External beam radiotherapy can cause the eye to feel irritated, dry, look red, and the patient might loose the eyelashes temporarily. This generally resolves, but the child is also at risk for long term problems like vision loss from cataract or retinal swelling. There is also a risk for radiation related second cancers, especially in children with bilateral retinoblastoma.

Child with left prosthesis.

ENUCLEATION ~

Enucleation is a method of removing the entire eyeball. The eyelids and muscles of the e ye remain. This is used for eyes that h ave large tumors or eyes that have d e veloped painful glaucoma. All other methods of treatment are considered before advising enucleation, but many patients have life-threatening large tumors that necessitate enucleation. Fo l l ow i n g enucleation, an implant is placed in the empty orbit and allowed to heal for 1 or 2 months. Then, an artifical eye (prosthesis) is made to match the remaining eye. The cosmetic appearance is generally outstanding with a natural appearance and comfortable fit as well as some movement of the eye. Protective lenses made of polycarbonate are advised to be worn at all times in the form of glasses during the day or goggles during activities or sports.

Recommended reading for vision in one eye is a book entitled “A Singular View. The Art of Seeing with One Eye“ by Frank Brady, available online at www.amazon.com.

E. PROGNOSIS

What is the prognosis ~

Fo r t u n a t e l y, most children with retinoblastoma survive and lead a good long and productive life. In the United States, nearly 98% of children survive, but not so in less advanced countries where about 50% of children die from tumor spread. Long term ocular and pediatric examinations are advised for the child.

Who is at risk for spread and death ~

The prognosis of a child with retinoblastoma depends on several factors, most importantly the results of pathology. Children with retinoblastoma that has invaded into the optic n e r ve, choroid, sclera, orbit, or anterior chamber require chemotherapy as they are at greatest risk for metastasis (spread) and death. Those without invasion do not require chemotherapy.

F. GENETIC COUNSELING

There are several important aspects of the genetics of retinoblastoma. Retinoblastoma is classified into hereditary or nonhereditary types. Hereditary retinoblastoma patients are at increased risk for pinealoblastoma, second cancers, and transmission of the genetic trait to their children.

Hereditary retinoblastoma includes: 100% of children with bilateral retinoblastoma

100% of children with familial retinoblastoma

10% of children with unilateral retinoblastoma

Nonhereditary retinoblastoma includes: 90% of children with unilateral

retinoblastoma

IMPORTANT LIFE-THREATENING CONCERNS FOR CHILDREN WITH

BILATERAL OR HEREDITARY RETINOBLASTOMA

Metastasis

This can occur, usually within one year from diagnosis, and primarily depends on the pathology findings. Fortunately, only 2% of patients develop metastasis. If the tumor i n vades into the optic nerve or choroid then chemotherapy is advised to prevent metastasis.

Pinealoblastoma

This is a brain cancer that can occur in 8% to 10% of children with bilateral or familial retinoblastoma. MRI or CT of the brain is important to monitor for this tumor, especially up to age 5 years as most pinealoblastomas occur before this age.

Second cancers

Children with bilateral or familial retinoblastoma have an increased risk for second cancers elsewhere in the body such as in the bone, soft tissue, and skin. Lifelong monitoring for second cancers is advised.

Table 2.

Chance of having another child with retinoblastoma if there is a family history of retinoblastoma

Affected Normal

# Eyes Affected Parents Child Sibling

Both eyes 40% 40% 7%

One eye 40% 40% 7%

The chance of having another child with retinoblastoma depends on two factors: 1. if the affected child has the tumor in one

or both eyes and

2. if there is another family member with retinoblastoma.

The following tables will give you an idea of the chances of your having another child with retinoblastoma depending on these factors. Generally, if there is another family member with retinoblastoma, the parents have a higher chance of carrying the gene (trait) to pass on to future babies. If there is no family history of retinoblastoma and the child has the tumor in only one eye, then the chances of passing on the trait to future generations is much less.

Table 1.

Chance of having another child with retinoblastoma if there is no family of retinoblastoma

Affected Normal

# Eyes Affected Parents Child Sibling

Two eyes 6% 40% 1%

OCULAR PROSTHESIS

What is a prosthesis ~

When an eye is removed (enucleation), the surgeon places an implant into the socket that remains buried in the tissue for life. About 6 weeks after surgery when the tissue is healed without swelling, an ocular prosthesis (artificial plastic eye) is fitted by an ocularist (artist who specializes in making the artificial human eye) to cover the buried implant. The prosthesis is a removable device and resembles a thick contact lens. The prosthesis fits comfortably and provides a natural appearing eye that matches the opposite eye.

How long can a prosthesis be worn ~

A prosthesis can remain in the socket for life, but it should generally be cleaned daily while bathing or showering by rinsing the surface using clean warm water. A warm washcloth can be used to wipe off debris without removing the eye. Occasionally, it should be removed to clean off mucous or debris. Most parents remove a child’s prosthesis at home for cleaning every 1 to 3 months. If the child is seen by an ocularist or an eye doctor, then it can be cleaned at their facility. Sometimes a prosthesis feels dry and teardrops are necessary for lubrication.

Should the prosthesis be replaced ~

During the child’s lifetime, the eye socket will g r ow and the prosthesis may need to be reshaped or even completely replaced to better fit the socket. This will be determined by the ophthalmologist or ocularist. In general, the prosthesis should be checked annually by the ocularist.

FUTURE APPOINTMENTS

The child will require close follow up of the eye by the retinoblastoma specialist during the first 6 years of life. When the child is an infant, an examination under anesthesia (EUA) will be scheduled every 1 to 6 months depending on the severity of the tumor and the age of the child. When the child is about 5 years old and mature enough to sit steady for an examination without sedation, then the examinations will be done in our office. Before leaving the hospital after each visit, it is important to schedule the next appointment. If appointments are frequently missed, the child might be at serious medical and visual threat.

Following treatment and for the remainder of her/his life, any child with retinoblastoma should have an eye examination twice a year. Also, a qualified medical examination by a pediatrician, pediatric oncologist, or primary care physician is advised lifelong.

Eye protection is important ~

All children with retinoblastoma should we a r p r o t e c t i ve polycarbonate glasses at all times and p o l ycarbonate goggles during activities to shield the eyes from injury. These are ava i l a b l e at most stores that sell eyeglasses.

RESEARCH

The Oncology Service is involved in every aspect of research from the clinical features of eye tumors to treatment alternatives, genetic c o n s i d e r a t i o n s, study of tumor DNA, and other facets of eye tumors. Much of this work is published in peer-reviewed medical journals. In fact, the Oncology Service team publishes over 60 medical reports in medical journals each year on various aspects of eye tumors and cancers. For a complete list of publications please refer to w w w. e yecancerinfo.com. This enormous amount of work has an ultimate goal: for improvement in patient care. Thanks to the generous support of research donations, these achievements are possible.

Regarding retinoblastoma, there is a tremendous amount of ongoing research being performed on clinical, genetic, and basic science aspects of retinoblastoma by the members of the Oncology Service at Wills Eye Hospital in collaboration with Children’s Hospital of Philadelphia and many other centers worldwide. Researchers are constantly searching for better ways to detect retinoblastoma at an earlier stage, even when it is barely visible to the human eye, in order to save the eye and vision. Improved methods of genetic analysis are underway. The search

for better therapies continues. There have been great strides in retinoblastoma research and this will continue in the 21st century.

FUNDING

Support for research is provided by The Eye Tumor Research Foundation, Incorporated, Philadelphia, PA Donation from Michael, Bruce, and Ellen Ratner, New York, NY

Mellon Charitable Giving from the Martha W. Rogers Charitable Trust

Macula Foundation, New York, NY Black Patch Invitational Golf Tournament, Downingtown, PA

Rosenthal Award of the Macula Society, Barcelona, Spain

Paul Kayser International Award of Merit in Retina Research, Houston TX

And many more, including patients and families.

The Eye Tumor Research Foundation PO Box 832 Philadelphia, PA 19105-0832 www.etrf.org T-SHIRTS SWEATSHIRTS HATS TOTE BAGS UMBRELLAS BEACH TOWELS COFFEE MUGS

CANCER WRIST BANDS AND OTHER ITEMS

available for purchase from

The Eye Tumor Research Foundation. Inscribed on each item is

“Support eye cancer research”.

Proceeds go directly to retinoblastoma research at the Foundation.

Your support is greatly appreciated

MONEY CONTRIBUTIONS FOR EYE TUMOR RESEARCH

CAN BE MADE TO:

FREQUENTLY ASKED QUESTIONS

. . . ABOUT RETINOBLASTOMA (RB)

1. What is rb?

Retinoblastoma (reh-tin-oh-blast-oma) is a childhood eye cancer arising from immature retinal cells in one or both eyes. The tumor can d e velop in the human fetus, newborns, infants, or young children.

2. How is rb found?

It causes a yellow-white glow in the pupil or a crossed eye.

3. What does the white pupil indicate?

This is called leukocoria and it is actually the tumor that is visualized inside the eye through the pupil.

4. Why does the eye drift?

Rb can decrease vision and this will cause the eye to drift or become “lazy”.

5. Can rb cause pain?

No, rb is usually painless, but some patients with glaucoma develop pain.

6. What causes rb?

It is caused by a mutation in chromosome 13. All humans have two copies of a normal “tumor suppressor” gene on chromosome 13 in every cell in their body that protects them from rb and other tumors. When both copies of this gene malfunction, then rb develops.

7. Who is at risk for rb?

Those with a known family history of rb are at risk to have children with rb. However, most new cases of rb have no family history.

8. Is rb hereditary?

It can be, especially if the child has bilateral rb.

9. Can something in pregnancy cause rb?

No, there are no known pregnancy-related causes for rb.

10. Is there something in the environment that causes rb?

No, there are no known environmental causes for rb.

11. Can rb be detected with amniocentesis?

Sometimes, but the genetic mutation is not always visible.

12. If a child has rb in one eye, can it develop in the other eye?

Yes, if there is a family history of rb or if the rb in the one eye is multiple.

13. What are the side effects of treatment?

This depends on the treatment. Some children have hair loss with chemotherapy, others have redness around the eyes with radiation, and others have eye swelling with cryotherapy.

14. Do I need to see a pediatric oncologist instead of my regular pediatrician?

It is important to follow with both physicians.

15. How can we afford the treatment?

Most insurances cover the costs of treatment. There are additional sources listed in this brochure. Any suggestions or additions you may find would be greatly appreciated!

16.Can this cancer spread to other parts of the body?

Possibly it can spread to the brain and rest of the body, but this happens only in advanced rb.

17. What might the vision be?

The vision depends on many factors, but most children can see enough to walk and play without great difficulty. But remember, the goals of treatment are to first save the child’s life, then save the eye and vision.

18. Will my child have delayed development?

Some children with hereditary retinoblastoma are delayed in walking, speech and other facets of development. However, most children are not delayed.

19. Will my child be socially withdrawn?

No, most children adapt well to the diagnosis and the visual handicap, especially with family support and education.

20. Why must rb children wear protective glasses?

Eye protection at all times is advised for all children with rb to shield the eye(s) from i n j u r y, especially during sports. This is particularly important for children with one remaining eye.

21. Can a child with rb attend a regular school?

Yes, but if both eyes are affected and the vision is reduced, the child might need a visual assistant during school. Public schools provide this service for visually handicapped children, but it is important to ask your school system about this plenty in advance.

22. Can an eye transplant be done after the eye is removed?

No, transplant of the whole eye is not currently possible.

23. Is there research for rb?

Ye s, much research is being conducted worldwide for rb. Philadelphia is a major center for rb research. Newer and better ways to detect and treat rb are being investigated. Studies into the gene that causes rb are underway.

Donations to the Eye Tumor Re s e a rc h

Foundation, PO Box 832, Philadelphia PA

1 9105-0832 from fa m i l y, gra n d p a re n t s,

re l a t i v e s, and friends continue to make these

discoveries possible. In fact, some childre n

themselves have used their birthday and

g raduation parties as a fundraiser for rb

PEARLS FOR PATIENTS

Ronald McDonald House

3925 Chestnut St. Philadelphia, PA 19107 (215) 387-8406

www.philarmh.org

Ronald McDonald House

550 Mickel Blvd. Camden, NJ 08103 (856) 966-1190

www.ronaldhouse-snj.org Hosts for Hospitals

Stay with a nearby volunteer family (610) 660-6667

www.hostforhospitals.org

ASSISTANCE WITH FLIGHTS TO PHILADELPHIA:

Corporate Angel Network (CAN) Nationwide public charity flights for cancer patients using available seats on corporate jets

CAN, Inc.

Westchester County Airport, One Loop Road, White Plains, NY 10604 (866) 328-1313 info@CorpAngelNetwork.org www.CorpAngelNetwork.org PEDIATRIC ONCOLOGISTS AT CHILDREN’S HOSPITAL OF PHILADELPHIA Anna Meadows, MD

Children’s Hospital of Philadelphia (215) 590-2299

Anne Leahey, MD

Children’s Hospital of Philadelphia (215) 590-2299

OCULARISTS TO FIT ARTIFICIAL EYE Legrand Associates in Pennsylvania (800) 523-4314

(215) 545-0442 www.legrandeyes.com

Kelly and Associates in Pennsylvania (215) 567-1337

www.slpeye.com

Strauss Eye Prosthetics in New York (585) 424-1350

RETINOBLASTOMA INFORMATION IN MEDICAL TEXTBOOKS AND MEDICAL WEBSITES

Ocular Oncology Service, Wills Eye Hospital www.eyecancerinfo.com

Atlas of Intraocular Tumors by Shields and Shields www.eyecancerbook.com

Diagnosis and Management of Intraocular Tumors

by Shields and Shields www.amazon.com

RETINOBLASTOMA DONATIONS AND RESEARCH

Eye Tumor Research Foundation www.etrf.org

PO Box 832

Philadelphia, PA 19105-0832 (215) 928-3105

T- s h i r t s, swe a t s h i r t s, hats, tote bags, and other items available for purchase.

Inscribed on each item is “Support eye cancer research”.

Proceeds go directly to retinoblastoma research.

American Cancer Society www.cancer.org

(800) 227-2345

National Childhood Cancer Foundation www.curesearch.org

Candlelighters Childhood Cancer Foundation www.candlelighters.org (800) 366–2223

The Little Rock Foundation www.tlrf.org

(877) 220-2500

National Federation of the Blind www.nfb.org

(410) 659-9314

National Association for the Visually Handicapped www.navh.org (888) 205-5951 National Eye Institute www.nei.nih.gov (301) 496-5248

Chat room for siblings of disabled children www.sodapoponline.org

A Singular View. Seeing with One Eye www.amazon.com

WORTHWHILE WEBSITES SUPPORT AND INFORMATIONAL

SOCIETIES, FOUNDATIONS, AND BOOKS

FINANCIAL SUPPORT

General Assistance programs provide food, housing, prescription drugs, and other medical expenses for those who are not eligible for other programs. Contact your state or local Department of Social Services. Hospitals that receive Hill-Burton funds are required by law to provide some services to people who cannot afford to pay for their hospitalization.

(800) 638–0742

www.hrsa.dhhs.gov/osp/dfcr/obtain /consfaq.htm

Medical costs that are not covered by insurance policies sometimes can be deducted from annual income before taxes.

www.irs.ustreas.gov

Medicaid (Medical Assistance) a jointly funded, Federal-State health insurance program for people who need financial assistance for medical expenses.

www.hcfa.gov/medicaid/medicaid.htm The Patient Advocate Foundation (PAF) is a national nonprofit organization that provides education, legal counseling, and referrals to cancer patients and survivors.

(800) 532-5274

www.patientadvocate.org

Patient Assistance Programs are offered by some pharmaceutical manufacturers to help p ay for medications. To learn whether a specific drug might be available at reduced cost through such a program, contact a medical social worker.

The State Children’s Health Insurance Program (SCHIP) is a Fe d e r a l - S t a t e partnership that offers low-cost or free health insurance coverage to uninsured children of low-wage, working parents.

(877) KIDS-NOW www.insurekidsnow.gov

Check with independent sponsors such at your work place. Some employers have contributed to travel costs as a charitable donation.

Community voluntary agencies and service organizations such as the Salvation Army, Lutheran Social Services, Jewish Social Services, Catholic Charities, and the Lions Club might offer help.

Fundraising is another mechanism to c o n s i d e r. Contact your local library for information about how to organize fundraising efforts.

WORTHWHILE WEBSITES

REFERENCES

Brady FB.

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Shields JA, Shields CL.

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Philadelphia: WB Saunders, 1992. www.eyecancerbook.org

Shields JA, Shields CL.

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Philadelphia: Lippincott Williams and Wilkins, 1999.

www.eyecancerbook.org

Shields JA, Shields CL.

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Philadelphia: Lippincott Williams and Wilkins, 1999.

www.eyecancerbook.org

Shields JA, Shields CL.

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Philadelphia: Lippincott Williams and Wilkins, 1999.

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DePotter P, Shields JA, Shields CL.

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Philadelphia: JB Lippincott Co, 1995.

Tasman W.

The History of Wills Eye Hospital 1832-2002.

Ocular Oncology Service Wills Eye Hospital

840 Walnut Street – Suite 1440 Philadelphia, Pennsylvania 19107 215.928.3105

www.eyecancerinfo.com www.etrf.org

www.eyecancerbook.com

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