Retinoblastoma cancer affects the retinoblasts. What is a retinoblast? It is an immature cell of the retina that multiply during gestation or when the fetus is still in the womb and early life, where it makes enough cells to create the retina. Now instead of maturing into cells that detect light, these retinoblasts continue to divide and grow out of control, forming cancer, thus the term retinoblastoma. It can occur in one eye or in both eyes. If left untreated, retinoblastoma grows, making the affected eye painful or worse makes the patient blind.

Pediatric Retinoblastoma Noticeable Symptoms

Pediatric Retinoblastoma noticeable symptoms include a white color in the center circle of the pupil when the light is shown in the eye, such as when taking a flash photograph. This often is the scenario where parents or anyone who see the picture of the child whose eyes have glint or a glare or some other whiteness in the pupil. Children and families fighting childhood cancer are fighting a good fight. Pediatric Retinoblastoma particularly is a rare malignant tumor that is often diagnosed early, once the symptom is noticed.

Pediatric Ophthalmology Specialist

The child is referred to a pediatric ophthalmology specialist, and the pediatric ophthalmologist will perform a fundus examination, once the child is diagnosed and confirmed with retinoblastoma the specialist will order tests to determine the size of the tumor and whether it has spread to other parts of the body. These tests may include an MRI or CT scan, bone marrow test or a spinal tap. And depending on the severity of the case some specialist will even do an ultrasound to help them make a proper accurate diagnosis, some cases the ophthalmologist may even decide that your child needs to be checked while under anesthetic so that they can have a better look at the retina.

Here are some important reminders for parents with kids who survived eye cancer.

Follow-through Doctors’ Appointments

Although medication has stopped, it is very important for parents to show up every doctor’s appointment for the medical team to continue to monitor the patient. The recurrence of cancer and side-effects of the treatments need close monitoring. Likewise, parents need to inform the doctor of any noticeable changes they see from their child after the treatment to prevent any misconceptions.

Keep Patient’s Medical Records Intact

All medical reports before, during and after the treatment must be kept in one place. This is for easy reference as the child grows. As an adult, medical check-up cannot be avoided year after year. With records handy, it will be easier for them to trace patient’s medical background. Some important information to be kept are the following:

• Pathology report from biopsies and surgeries (if applicable)
• Copy of operative report if surgery was undertaken
• Discharge summaries from hospital admissions
• List of medication given during chemotherapy (if applicable)
• List of dosage and types given during radiation therapy (if applicable)
• Names and contact information of doctors who gave treatment

Have Genetic Counseling

Most doctors would recommend the family to have genetic counseling. This will give the family a sound medical advice and calculated risks for the eye cancer survivor to develop second cancer later on. The counselor on genetics will review the family’s medical history. This is to predict likelihood that genes causing pediatric retinoblastoma might be inherited and passed on to other family members.

Undergo Genetic Testing

The genetic counselor might also suggest genetic testing for other children in the family. The results of the genetic testing should also be kept together with all other medical records.

One of the illnesses that affect children below six years old in the US is pediatric retinoblastoma which is reported to have 300 cases each year. This type of illness can impair a child’s vision and negatively impact their physical and emotional growth. Special care needs to be given when dealing with this kind of disease. Some ways you can give special care for kids with eye cancer include:

Hiring a Special Medical Team

Children diagnosed with pediatric retinoblastoma are given special medical attention by a team of physicians. It requires a team since treatment may vary depending on the response of the patient. The team is composed of medical field specialists such as:

Pediatric ophthalmologist – a physician who takes care of eye disease

Pediatric oncologist – a physician who gives treatment to children diagnosed with cancer

Ocular oncologist – an ophthalmologist who gives treatment to eye cancer

Radiation oncologist – a physician who administers radiation therapy

Counselling on Parenting Kids with Retinoblastoma

Parents not only need medical advice from physicians, but also help from psychotherapists so they can give the right special attention and care to their kid with eye cancer. In order for their child’s body to recuperate, parents need to regulate what their child eats and limit him or her from moving too much for a certain period of time as he or she undergoes treatment. It might help for parents to set a daily “alarm” to ensure that their child is taking prescribed medications on time.

Keeping a Positive Environment

For kids undergoing treatment for eye cancer, pain is inevitable. They may feel restless or grumpy. Showing empathy and telling them words of encouragement would go a long way. They need to be reassured that it is going to be all right after the treatment. Patients can still play with other children, given that the game or activity does not require a lot of physical movement like board games. The family should give more time for laughter during meals and doing fun activities with the patient on a daily basis. Keeping a positive environment will boost the patient’s immune system and will have an impact on the kid’s recovery.

Symptoms of Pediatric Retinoblastoma

Symptoms of Pediatric Retinoblastoma include strabismus or crossed-eyes. This condition needs immediate help from an ophthalmologist. Other signs include poor vision, white pupil or change in size of the pupil.

Diagnosis of Pediatric Retinoblastoma

In early detection of kids’ eye cancer, a red reflex examination is performed by a pediatrician. More so, this is done after childbirth, before the baby is discharged from neonatal nursery as recommended by American Academy of Pediatrics. If there is an abnormal red reflex found, the child should be immediately referred to an ophthalmologist for funduscopic examination. After the examination is completed, fundus photography will also be performed to identify the size of tumor and stage. Another procedure to be done is the ophthalmic ultrasound that may show bright spots. MRI is also preferred over CT scan to reduce the risk of exposing the child to develop second cancer and avoid unnecessary radiation. Once pediatric retinoblastoma is confirmed, the patient shall be referred to see an ocular oncologist.

Treatment of Kid’s Eye Cancer

Managing kid’s eye cancer should be tailored-fit to the patient’s case. Factors to be considered include the location of the tumor, size, laterality, and vision prognosis. The patient should be referred to an ocular oncologist who is trained to treat retinoblastoma. Management of pediatric retinoblastoma is rather complex and there are three chosen methods for treatment; enucleation, focal therapy, and chemotherapy. A single treatment or combination may be used depending on the case of the patient and how the child responds to the treatment.

Hereditary

About forty percent of patients suffering from retinoblastoma have genetic defects which lead to multiple tumors affecting one or both eyes. It is commonly known as germline retinoblastoma or hereditary. These children are usually diagnosed before they reach 1 year old. And these patients may pass this condition to their future children. Patients diagnosed with eye cancer have higher possibility of developing other types of cancer.

Non-hereditary

About sixty percent of the patients have non-hereditary pediatric retinoblastoma. Mostly like, these patients have developed tumor in one eye only. On average, this tumor can be diagnosed on two-year-old children.

Retinoblastoma Treatment

Surgery is performed to remove the affected eye in cases of patients having advanced retinoblastoma. If only one eye is affected and is removed, above 90 percent of patients no longer need more treatment thereafter. However, if both eyes are affected, only one eye is removed, and the rest of the treatment will be focused on saving the other eye. If tumor has spread into tissues around the eyes or eye socket, chemotherapy is administered to patients after the surgery.

Chemotherapy uses strong medicines through injection to kill the cancer cells, stop their growth, and avoid growing more cancer cells. Periocular injection is a local treatment to focus on the affected eye. Injection can also be done in the blood stream, for the medicine to protect the body, preventing cancer cells to spread in other parts. Using chemotherapy alone cannot cure this eye cancer, and patients receive another treatment called focal therapy.

Focal therapy is a laser treatment therapy or cryotherapy while the patient is under anesthesia. This kind of treatment may be continued even after chemotherapy has been completed. In some cases, tumors developed in the eyes, if relatively small, are treated with focal therapy only.

Radiation therapy uses X-rays with high-energy or radiation, killing cancer cells or stopping their growth. This type of treatment is only reserved in case patients have not responded well to other therapies. One type is external radiation which uses a machine to deliver X-ray dose. Another is internal radiation which uses needles, wires or tubes to deliver radiation directly to the part of the body affected by cancer.

It is a malignant tumor that develops within the eye’s retina, which is the thin nerve tissue of the eye responsible in sensing light and transmitting images to the brain. This disease may occur at any age, but it commonly occurs during the early stages of childhood, usually before a child reaches the age of five. Retinoblastoma may either occur in only one eye or both eyes, but if left untreated, it may metastasize to other parts of the body.

The Four Stages Of Pediatric Retinoblastoma

In order for the physician to be able to plan the treatment for retinoblastoma, two factors need to be considered to determine the stage of the cancer: the size of the tumor, and its location.

• Intraocular Retinoblastoma. The cancer cells are found unilaterally or bilaterally, but does not affect the tissues surrounding the eyes or other parts of the face.
• Extraocular Retinoblastoma. The cancer cells have started spreading to areas outside the eye. The cells may be confined only to the tissues surrounding the eye, or it may have already spread to other parts of the body.
• Trilateral Retinoblastoma. Sometimes, children with bilateral retinoblastoma may have a third tumor that develops in the pineal gland of the brain (the gland that produces melatonin, which is the sleep hormone). This tumor may cause nerve disorders and must be diagnosed as early as possible, because treating it may require a different approach.
• Recurrent Retinoblastoma. From the name itself, it means that the cancer cells have returned or progressed after treatment has already been done, and it may recur either in the eye or somewhere else in the body.

Common Signs And Symptoms Of Pediatric Retinoblastoma

• White Pupillary Reflex (Leukocoria). The most common early symptom of the disease. It is diagnosed by shining a light in the eye and the color of the pupil is checked. If the color is red (blood vessels in the back of the eye), the eye is normal; but if the color is white or pink, the eye has retinoblastoma.
• Lazy Eye (Strabismus). A condition in which one eye is facing a different direction than the other. Though this may have other possible causes in children, it can also be caused by retinoblastoma.

According to recent studies, 6% of newly diagnosed retinoblastoma were inherited from the previous generation, while 94% were sporadic, which means the family history of the patient is negative for the disease. This is only one of the three different classifications of children’s eye cancer, with the other two being bilateral or unilateral, and heritable or non-heritable.

Other Classifications of Pediatric Retinoblastoma

• Unilateral vs. Bilateral Retinoblastoma. The classification of pediatric retinoblastoma when either only one eye (unilateral) or both eyes (bilateral) are affected. According to research, bilateral retinoblastoma may manifest in children at a much earlier age than unilateral retinoblastoma. And in the total number of cases, two-thirds of pediatric retinoblastoma are unilateral, and only one-third of the cases are bilateral. Unilateral retinoblastoma cases are usually not inherited while bilateral cases are almost always inherited.

• Heritable vs, Non-heritable Retinoblastoma. The classification of pediatric retinoblastoma that identifies whether or not the disease has a chance of being passed on to the next generation. Usually, those patients with retinoblastoma who inherited the disease from their parent/s have a chance of passing it on to their future offsprings, and this covers an estimated 40%
  of all cases of retinoblastoma. On the other hand, 60% of those cases are non-germinal, which means that the disease was acquired sporadically and is at a greater chance of being non-heritable.

Even though a child will inherit the gene from either one or both parents, they will still have a chance of not having the disease but that chance is very slim since their risk of developing retinoblastoma is at 90%.

Preventive and Treatment Measures of Pediatric Retinoblastoma

As of now, there are no other preventive measures for retinoblastoma except for having the child’s eye examined as early as possible for a higher chance of being able to treat the disease and save the child’s vision.

For the treatment, it highly depends on the severity of the disease and whether or not it was inherited genetically. There are both non-surgical and surgical treatment methods for pediatric retinoblastoma.

Non-surgical Methods:

• Chemotherapy. The treatment used to shrink the tumor cells and is usually the first choice of treatment before other options are considered.
• Cryotherapy. Another non-surgical treatment method used for retinoblastoma when the disease is not yet very severe and the patient may be able to keep at least part of their vision. This method is done by freezing the cancer cells, preventing it from metastasizing.
• Laser Therapy. One of the last options used when treating retinoblastoma since it has a high risk of putting damage to healthy cells and tissues, and the possibility of triggering other tumors outside of the eye in cases of genetic retinoblastoma.

Surgical Method:

• Enucleation. Also known as eye removal, is done when the disease is very severe that there is a high risk of it spreading to other parts of the body, or the chance of saving the child’s eyesight is zero.

For children where the tumor is confined to the eye and has not spread systemically or into the orbit or brain, the cure rate is high. Other children with the genetic form of retinoblastoma, the risk is high for developing subsequent malignancies, most commonly sarcomas. While those children with the heritable form of the disease who were exposed to ionizing radiation at age less than one year this risk is greater. Genetic counseling in families with retinoblastoma is an important aspect in their care and should be coordinated with a medical geneticist or genetic counselor that is part of the retinoblastoma team.

Parents would do everything they can to try and keep kids from becoming ill or feeling pain. Though parents describe the day of their child’s cancer diagnosis as “the day their world fell apart”. When a child is diagnosed with chronic illness, it’s ordinary for parents to feel guilt and sadness. Anger is also common. You may feel angry toward your partner, the world or even, at times, toward your child. These feelings are normal.

Addressing your child’s medical condition directly is the best way to move forward. According to Norberg, A.L., Lindblad, F., and Borman, K.K. (2005), A study on parents of children with cancer, found those parents who took action and focused on the problem experienced lower levels of anxiety and depression than parents who denied or avoided the situation.

COPING WITH STRESS AND ANXIETY

There are parents who cope well with the stress and anxiety exerted by their child’s diagnosis and treatment. They usually reach within themselves to find strength and skills to traverse the experience. Parents should be aware of the effects such as trauma for the family, adjusting expectations and parenting styles according to their changeable needs. This is not one of those ordinary experience though. Most of these families undergo anxieties and short tempers, as well as periods of genuine calm. Most of them do survive the condition together and frequently emerge to make the family unit stronger and more understanding of one another.

DEALING WITH ANGER

Anger is a common reaction to retinoblastoma, but this cancer is mediocrity’s accountability. The cause of the genetic changes that spark eye cancer in children is unknown. When a child suffers, we usually result to find something or someone to blame most especially when we are not familiar on the illness the child is suffering. Parents would sometimes often unload their anger or rage on medical staff, their partners, through themselves, friends and relatives, and even their children. Unraveled rages can be especially destructive in times of crisis, especially when experienced by a child with cancer or their siblings. Be aware that you are more possible to have a short temper when you are under stress. Be upbeat in reducing or omitting stress, and know how to manage your anger or even to set aside your anger entirely, as this will lessen the risk of undesirable outbreaks.

COMMUNICATE AND VENT OUT

Communication is a great tool. If you have a difference with your child’s doctors, better to address and discuss your concerns immediately. By this, you are well composed and prepared to talk openly and deliberately compared to rush talk, tense or outburst once you bump into hallways. It is important to ask the doctor or nurse to explain things that you do not understand or things that you have discussed before to explain to you once again.

People respond more positively and calmly when you ask questions properly or discussions are not argumentative. Avoid to irritate the person, might be the doctor or nurse, when you have discussions. Talk to them early to avoid unnecessary misunderstanding and anguish. Make sure that when you talk, you take your time, especially when you’re talking to your child’s doctors or even your partner when you are both composed. The risk of discussions sinking into a slanging match is high when either of you is very anxious.

REACH OUT TO RETINOBLASTOMA COMMUNITY

Try to connect with other family. Sharing of experiences, discussions and small talks will help you feel relieved and the fact that you are not the only one experiencing this challenge is a great relief. Reaching with other families of children who have the same condition and same situation having diagnosed with retinoblastoma, and build relationships with them like read other children’s stories, talk with parents while waiting on clinics, join an online retinoblastoma community, join or start a retinoblastoma / cancer support group.

STAYING ACTIVE AND POSITIVE

Seek constructive ways to release your tension before it fall over into anger that will not only affect others but also yourself. Activities such as exercise, or dancing each day helps release tense and anxiety. Walking or running is another way to release tense. Some household chores like gardening, cleaning of clutter, home arrangement and even cooking and baking is a big help. Writing down your daily experience or video blog is also helpful working through anger and anxiety.

Releasing your feelings through voice in a safe environment like beach or climbing a mountain often eases their power over you. Try to breathe fresh air, it helps cease stress. Breathing exercises can rapidly reduce anxiety and stress. Solely taking a deep in breath and slow, regular out breath helps calm the body and focus the mind. Run-through this easy technique when you are already calm, to upkeep you in moments of crisis. Listen to soft music with a gentle rhythm is also a very powerful way to relax the body and mind.

EYE Screening

Our eyes are the window of our soul. A baby can only first see just a light, second colors, and then third faces. It is how important the eyes are. It is one of the most sensitive and critical part of an infant and it should be given a priority through eye screening. Eye specialists use new technologies to detect eye problems especially in infants who are observed to have difficulties in focusing on something and are easily agitated by too much light. Eye screening can detect genetic or acquired form of eye disease.

Retinoblastoma (Rb) in Children

One type of eye disease is Retinoblastoma. This is a rare disease mostly occur in children ages 2 years old and below and it is commonly known as Pediatric Retinoblastoma. It is where a cancer cell is progressively mutating to kill the healthy cells which develops into a tumor in the retina and will spread further on other parts of the body like the brain, spine, skull, and bone marrow.

Can Retinoblastoma be treated?

Retinoblastoma is treatable but is dependent on how early the cancer is detected. Also, the parents play a very important role in communicating to their doctor or specialist of all family histories and backgrounds especially if the same happened to a relative or a member of the family. If it is already a genetic disease they suspect to be passed-on to their child, early screening like Prenatal Screening for retinoblastoma is done with the parent’s approval of the test. This is a very sensitive kind of test so if the parents decided to discard this option they are advised to do Newborn screening immediately after giving birth. This is to check if gene mutation is inherited by their child and also to monitor the progression of the disease. Regular eye check-ups is then necessary especially if it is a hereditary type of retinoblastoma.

Forms of Retinoblastoma

There are two forms of Retinoblastoma: Heritable and Non-heritable.
Heritable retinoblastoma is when there is a family history that the disease already occurred. Second, if there is a mutation or change in the RB1 gene. In this form, a tumor maybe developed not only in one eye or both eyes are affected.

Non-heritable retinoblastoma developed much later in the child’s life compared with a child that has a heritable form of retinoblastoma. Most cases occur when the child ages 5 years old and below. This is caused by an isolated genetic mutation in which both parents don’t have the said gene. The cancer appears only in one eye of the patient and if treated earlier, have a higher chance of recovery and non-occurrence of the cancer. Physicians still don’t know the cause of a non-heritable retinoblastoma that develops to children.

Treatment for Retinoblastoma

Retinoblastoma treatment is a journey, not only to the child but to the parents as well. There are different ways in treating retinoblastoma. It depends on what stage the prognosis of the doctor is and what type of treatment should the child undergo. Here are some of the tests available for the parents to choose from:

• Eye Screening & Eye Examinations
• MRI (Magnetic Resonance Imaging)
• CT (Computerized Tomography) Scan
• RB1 Gene Test
• Ultrasound Exam of the Eye
• Fluorescein Angiography
• Fine Needle Biopsy
• Cytogenetics and Gene Expression Profiling
• Tests for Metastases
• Chemotherapy
• Focal Therapy

Continuous tests and examinations should be done even after a child undergoes a therapy and was cleared by the doctor since there is a tendency for the cancer to recur. Parents should be very observant of the symptoms of retinoblastoma or other types of cancer in order to address the child’s concerns or to ease the pain or discomforts they feel as early as possible.

If you or a loved one have suffered injury, or even death, due to pediatric retinoblastoma, call Dellecker, Wilson, King, McKenna, Ruffier & Sos, LLP as soon as possible. We’ll work to earn you the compensation you deserve.

Proving Pediatric Retinoblastoma Malpractice

In order to be pediatric retinoblastoma malpractice, four conditions must be proved:

• A patient/doctor relationship existed: The accused doctor must have been in a patient/doctor relationship with the patient.
• Breach of duty of care by the medical professional: Doctors owe a high duty of care to their patients, a level of treatment equal to similarly trained professionals in their field. If the doctor does not meet this standard, a breach of the duty of care occurs. This breach of a duty can either be an act or failure to act.
• Causation of injury to the patient: This breach of the duty of care must lead to the patient’s injury. For example, if the doctor improperly diagnoses pediatric retinoblastoma and the patient is injured due to this negligence, this is causation.
• Damages to the patient: Damages must be shown to have occurred, including pain and suffering, medical bills, lost wages, and more.

Your Rights

If you, your child, or a loved one has been injured as a result of pediatric retinoblastoma malpractice, you may be entitled to monetary compensation. You’ll need an experienced lawyer on your side, because hospitals, doctors, and insurance companies often have great legal teams.

Call us or come in for a free consultation. We have the expertise and knowledge you need.

Monetary Damages

In a case of pediatric retinoblastoma malpractice, like all cases of medical malpractice, you may be entitled to monetary damages. These damages include compensation for physical, mental, and emotional pain and suffering, medical bills, and lost future earning potential.