Retinoblastoma: Nursing

With retinoblastoma, retino- refers to the retina of the eye and -blastoma means arising from embryonic tissue. So, retinoblastoma describes a malignant tumor of the retina that arises from embryonic retinal cells; and it typically affects children younger than 5 years of age.Now, let’s quickly review the physiology of the retina, which is a thin layer of nerve cells that lie at the back of the eye.

This layer houses blood vessels, as well as nerve cells called photoreceptors, which receive light and convert it into neural impulses.

These impulses are then directed to the visual cortex of the brain, where they get translated into images.Now, the cause of retinoblastoma is rooted in genetics, specifically, inactivation mutations of a tumor suppressor gene called RB1, which can be the result of an inherited germline mutation or a spontaneous mutation.

Spontaneous mutations means that both RB1 mutations occur spontaneously in the somatic cells of the individual, and typically cause retinoblastoma in only one eye.

On the other hand, inherited mutations usually cause multiple tumors in both eyes. Lastly, risk factors for retinoblastoma include advanced paternal age, family history of retinoblastoma or a known RB1 gene mutation in one parent, paternal exposure to radiation, in addition to maternal exposure to diesel or gasoline exhaust during pregnancy.

Now, the pathology of retinoblastoma starts with mutations to the RB1 gene, which cause unregulated growth of retinal cells.

Over time, these cancerous cells form a tumor in the retina, which can then invade nearby structures. Left untreated, retinoblastoma can metastasize to the brain, opposite eye, lymph nodes, bone, bone marrow, and to the liver.

Other complications of retinoblastoma include blindness, retinal detachment, and secondary tumors, which are typically sarcomas.Typically, the clinical manifestations of retinoblastoma start by the age of one year in cases of bilateral involvement, and by the age of three years in cases of unilateral involvement.

The first symptom of retinoblastoma noticed by the parents is leukocoria, also called the cat’s eye reflex, meaning the pupil appears white instead of red as the light reflects off the tumor white surface.

Other signs and symptoms of retinoblastoma include strabismus, ocular inflammation, which can cause pain and a red eye, in addition to visible extraocular growth, decreased vision, restricted eye movements, and protrusion of the eyeball, called proptosis.The diagnosis of retinoblastoma starts with the client's history and physical assessment, followed by diagnostic tests.

These tests include red reflex examination using an ophthalmoscope, which will show leukocoria; direct ophthalmoscopic examination, which will show a retinal mass; as well as slit lamp examination may identify blood or tumor debris in the anterior chamber.

An ocular ultrasound can also be done to evaluate the size of the tumor; an MRI of the orbits and brain can reveal if there is tumor extension outside the eye; and genetic testing can also be done.

Finally, eye examinations for all newborns and children should be done at routine child care for early detection; and clients with a family history of retinoblastoma should undergo genetic screening to determine if frequent clinical monitoring for retinoblastoma is needed.

Now, the treatment of retinoblastoma aims at eradicating the tumor while preserving vision and offering good cosmetic results.

The mainstay of treating retinoblastoma is local or systemic chemotherapy, which is typically followed by radiotherapy. Other treatment options include laser surgery, cryotherapy, in addition to enucleation, which is the surgical removal of the entire globe of the eye.

A prosthesis can also be implanted after enucleation, for cosmetic reasons as well as preserving the shape of the orbit.

Finally, more than one treatment option can be used in order to get the best results. Alright, let’s look at the nursing care you’ll be providing for a client with retinoblastoma.

The priority goals of care include managing symptoms and providing supportive care for the child and family during treatment.

Begin by assessing the child’s pain using a pediatric pain scale like the FLACC scale, as well as other symptoms such as nausea and vomiting.

Administer the prescribed analgesic and antiemetic medications, and provide comfort measures including positioning and distraction.

Then, assess their baseline height and weight so their growth and development can be monitored during treatment. Be sure to confirm informed consent has been obtained for the prescribed treatments, provide care before and after the procedure, and closely monitor the child for adverse effects of the procedures.

If your client has received surgical management, provide routine postoperative care. Assess and continue to monitor their dressing for bleeding, provide routine dressing changes, and encourage the parents to participate in their child’s care.

If your client is receiving chemotherapy, monitor for side effects such as nausea and vomiting, changes in bowel habits, fatigue, and increased risk of infection.

Monitor closely for signs of infection like fever, and implement neutropenic precautions if indicated. Finally, assess your client and family’s psychosocial needs, and provide encouragement and support.

Collaborate with the child-life specialist to include developmentally appropriate medical-play therapy in the plan of care to decrease anxiety during treatment.

Lastly, provide the caregivers with referrals for home health, social services, community resources, and resources for genetic testing, as needed.Okay, let’s move on to client and family teaching.

Begin by explaining that the tumor developed in the light-sensitive area in back of the eye, and review what to expect during treatment.Teach them about common side effects of treatments like pain, nausea, vomiting, and fatigue, and instruct them to administer antiemetic and pain medications as directed, to provide small frequent meals whenever the client is hungry, and to encourage frequent rest periods as needed.

If the enucleation was performed, teach the caregivers to closely monitor the surgical dressing, to keep it clean and dry, and to keep it in place until their first follow-up visit.

Let them know that the child can bathe or shower, but remind them that the child’s head should not be put directly under water for a few weeks.

Advise them that once the eye socket has healed, their child will be fitted with a prosthetic eye to match the child’s natural eye.

Stress the importance by maintaining regular eye appointments as well as regular check-ups to assess growth and development patterns.

Finally, instruct the caregivers to contact their healthcare provider right away if their child develops any new symptoms, if the appearance of the eye or area around the eye changes, or if they notice a change in their child’s vision.

Alright, as a quick recap…. Retinoblastoma is a malignant tumor of the retina caused by mutations of a tumor suppressor gene called RB1.

Presenting symptoms can include leukocoria, strabismus, ocular inflammation, decreased vision, restricted eye movements, and proptosis.

Diagnosis involves physical assessment and tests like direct ophthalmoscopy for red reflex testing, ocular ultrasound, and MRI.

Treatment includes chemotherapy, radiotherapy, cryotherapy, laser surgery, and enucleation with the implementation of a prosthesis.

Priority goals of care include managing symptoms and providing supportive care for the child and family during treatment.

Client and family teaching focuses on understanding the disease process, the plan of care, management of symptoms,