Part 2 — Treatment, Side Effects, Restrictions and Implications for School


Although metastases may not be present when a patient is first diagnosed with Ewing's, they can occur at any time. Treatment of only the original tumor in the bone does not keep metastases from showing up and usually leads to only short-term survival. Therefore, treatment of Ewing's sarcoma starts with chemotherapy. A positive response to chemotherapy usually predicts a better chance of survival. The side effects of chemotherapy include fever, increased risk of infection and increased risk of bleeding. The specific side effects seen depend on the drugs used and the timing of treatment.

Treatment of the bone where the cancer started depends on the size of the tumor. For small tumors, the bone can be removed surgically. It can then be replaced with either a piece of metal that is shaped to look like the bone (similar to the joint replacements used in older people with arthritis) or a bone that is the same shape as the removed bone. Large tumors are commonly seen in the pelvis.

Ewing's tumors will frequently break out through the surface of the bone, and involve a substantial amount of adjacent soft tissue. The involved tissue can include muscle, nerves and blood vessels. The tumor must be removed completely, along with those invaded structures. If so much tissue must be removed that the patient can no longer effectively use that extremity, radiation therapy can be used to kill the cancer. Although this cancer responds to radiation, there is some evidence that the cancer is less likely to come back if it is removed surgically, rather than irradiated. For those reasons, amputation may be indicated.

Side effects of chemotherapy

In addition to surgery, people with Ewing's sarcoma usually must have chemotherapy treatments. Like other cancers, a combination of chemotherapy drugs (very strong medications that are used to treat cancer) are commonly used to treat Ewing's sarcoma. The side effects depend on which drugs are used to treat the patient. Common side effects include:

Friends treating cancer together.
  • nausea
  • vomiting
  • fatigue
  • low blood cell counts
  • susceptibility to infection
  • hair loss
  • low bone mass

Physical, dietary and other restrictions

If the child's doctor believes that the affected bone could break easily, restrictions on activity might be needed immediately after diagnosis. After treatment, continued limitations might be needed, depending on the therapy chosen. Irradiated bone is more likely to break, so children treated in this manner might have lifelong restrictions on their activities.

For those patients treated with surgery, the degree and duration of activity restriction depend on the manner in which the bone was put back together. For example, if the surgeon used another bone (whether grafted from another site in the child's body or from a donor), it is likely that activity will be limited for several months while the bone heals. During this time, the child should remain mobile, to promote proper healing of the bone. Crutches or a walker can be used if directed by the medical team for support and stability.

Other physical restrictions might be necessary as a result of the chemotherapy treatments, rather than the cancer itself. The possible side effects of chemotherapy depend on the medications used. These medications are very effective against the tumor, but can affect many other tissues. One commonly affected tissue is the bone marrow, which produces all the cells in the blood. There might be a decline in the numbers of red blood cells, platelets and white blood cells. The effects of these declines are delineated below.

Red blood cells (erythrocytes) contain hemoglobin, which carries oxygen throughout the body and provides energy. If there are fewer of these cells to carry the oxygen, the patient might be tired and easily fatigued. Physical activity could be restricted in these circumstances. Rarely, a blood transfusion might be necessary to restore adequate oxygen transport.

Platelets help stop bleeding and bruising when we are cut or are hit. If chemotherapy causes a drop in the level of platelets in the blood, the patient will develop bruises more easily and will bleed more when cut.

White blood cells (leukocytes) are needed to fight infection. If the white blood cell count is low, there might not be enough of these cells to fight off an infection. The patient should avoid any setting where exposure to germs is likely, such as malls, football games, grocery stores and theaters. If an infection does occur, the patient must be watched very closely, and could require hospitalization for intensive therapy with intravenous (IV) antibiotics. Any infection in a person with a low white blood cell count can be deadly. The best intervention is to prevent infections before they occur. Many infections are carried on the hands, by germs placed there when someone coughs, sneezes or touches a surface with germs on it. Remember, everything has germs on it. All people should:

  1. cover their coughs
  2. cover their sneezes
  3. wash their hands

Implications for school

Best friends support each other.

Classmates and peers of the student should be educated about Ewing's sarcoma. They will have many questions about their friend with the disease, including decreased mobility, hair loss, weight loss and frequent absences. A common reaction from parents and teachers is to keep quiet about these issues, fearing that discussing them will cause problems for the child with Ewing's. In fact, the opposite is true. The child's classmates want to know what is going on with their friend. If they are not given accurate and truthful information, they will fill in the gaps in their understanding with stories, whispered tales, half-truths and other misinformation. However, providing factual and accurate information is the best way to help other kids understand what their friend is going through and encourage their support.

Clearly, this process must be carried out with openness and sensitivity, and certainly with the permission of the student and parents. Education of classmates and peers can be done by the hospital teacher where the child receives treatments, by the school nurse, or by another knowledgeable person. Frequently, the student will want to be involved in this process. After all, the student can provide the best insight into the impact of the illness.

A student with Ewing's sarcoma will likely have significant absences from school as a result of surgery, chemotherapy, radiation therapy, medical procedures, laboratory tests and medical appointments. Infections, which may be serious, will also interfere with school attendance. Typically, the most active part of the treatment for Ewing's sarcoma lasts about a year. The teacher will be required to show a great deal of flexibility and creativity to ensure the student does not fall behind.

Full-time homebound education is not recommended for the student with a chronic illness. It removes the student from the school environment and precludes the opportunity for normal social development.

On the other hand, intermittent homebound schooling, which provides educational support at home or in the hospital, can augment traditional school attendance and provide continuity for lessons and other academic activities when the student cannot attend school.

Acommodations might be needed for school.

Modifications and accommodations will be necessary to create a physical and learning environment that is appropriate for the student with Ewing's sarcoma. For example, the student might need a wheelchair or another walking aid. Consequently, the seating arrangement would need to be modified, the student might need to leave classes early to avoid traveling through congested halls or could be given additional time to travel between classes. In these circumstances, the student might benefit from a Section 504 Plan or perhaps an evaluation to determine whether being classified as Other Health Impaired (which would lead to an IEP) might be indicated. Both of these plans would help the school provide the support needed by the student.

If the health status of the student changes, teachers and classmates should be informed of these changes and their effects. For example, if the child's tumor spreads to the lungs, the therapy will change drastically and could involve additional drugs with new side effects, hazards and restrictions on activities. The hospital school teacher can help explain the student's condition and how it will affect activities at home and school. Ongoing, effective communication between the family, school and hospital will result in better understanding and will promote optimal support for the student.

KU Kids Healing Place

During treatment for Ewing's Sarcoma, there are many challenges for the child and family. In addition to the essential medical treatment for Ewing's, it is important to care for the psychological, social and spiritual needs of the child and family. This type of care, called palliative care, previously had been reserved for only the sickest patients. However, research has demonstrated that palliative care helps maintain normalcy throughout treatment, recovery and life. Today, every child with Ewing's sarcoma receives the benefit of palliative care, along with all family members. The KU Kids Healing Place (KUKHP), through the Department of Pediatrics at the University of Kansas Medical Center, focuses on treating all aspects of the child and family, ensuring that each receives the care needed to maintain psychological, social and spiritual well-being during treatment for Ewing's sarcoma and on into life. The entire treatment team will work to ensure that the needs of the child are recognized and addressed both in the hospital and in the community, including school, sports, worship and activities. KUKHP partners with school professionals, peers, clergy, coaches, scout leaders and many others to ensure that the child continues to grow and achieve in all environments.

For more information, see:

Part 1 — Introduction and Symptoms


National Library of Medicine/National Institutes of Health

Contributed by:

Kim Templeton, MD
Department of Orthopedic Surgery
University of Kansas Medical Center

Kathy Davis, MSEd, PhD
Associate Professor
Project Director, Connected Kansas Kids
Director, KU Kids Healing Place
University of Kansas Medical Center