Acute Lymphoblastic Leukemia (ALL) is a type of cancer that occurs when the bone marrow makes an excessive amount of immature lymphocytes. It is the most common type of childhood cancer. A child under the age of 15 years old has a 1:23,000 chance of developing ALL, with a peak age incidence between three and five years of age.1 There is no known cause, but environmental, genetic, viral and immunologic factors are all implied in playing a role in developing this type of disease.1 Systems impacted are immune, cardiovascular, musculoskeletal, respiratory, nervous, and lymphatic. This disease disrupts the normal amount of white and red blood cells in the body, while in turn reducing the body’s ability to defend against infections and diseases.
Patients with this diagnosis will have a complete blood count chart reading with averages reflecting decreased blood cell production. Most children will show the following values: white blood cell count (WBC) < 1000/CMM or >50,000/CMM, hematocrit (HCT) < 15% or >68%, and platelets (PLT) < 30,000/CMM or >1,000,000/CMM.2 The decrease in platelets can cause bruises. If cancerous cells are spread to the brain, then the patient can have headaches, nausea, and neurological dysfunction. There is a decreased tolerance for physical activity due to the alteration of the amount of red and white blood cells. Decreased oxygen carrying capacity impacts any cardiovascular intensive activity, impairing the child’s ability to maintain an elevated heart rate or blood pressure. Continuous spread of the diseased cells will irritate other visceral tissues and cause inflammation, which can impact the integrity of bone and muscle function.
Risk for development is highest in children under five years old. The risk then declines until the mid-20s and begins to increase again after age 50.3 Overall, about 40% of ALL diagnoses are in adults. The American Cancer Society found that the lifetime risk of getting ALL is less than one in 1,000 for the average person. The risk is slightly higher in Caucasian males.3 Other risk factors include exposure to X-rays before birth, radiation exposure, past treatment of chemotherapy, or having certain genetic conditions. Some of those conditions are listed, but not limited to, Down Syndrome, Neurofibromatosis Type 1, Bloom Syndrome, Fanconi Anemia, Ataxia-telangiectasia, Li-Fraumeni Syndrome, and Constitutional Mismatch Repair Deficiency.4
Diagnosis of this particular disease is done by a physical exam, followed by other diagnostic tests. A complete blood count is taken to examine the number of red blood cells and platelets, as well as the amount and type of white blood cells, hemoglobin, and hematocrit. Blood chemistry studies are also taken to check for electrolytes, fats, proteins, glucose and enzymes released into the blood by organs and tissues.4 Bone marrow aspiration and biopsies can also be administered to have access to a complete cytogenic analysis and immunophenotyping assessment. Both of these will examine blood and bone marrow cells to assess changes in chromosomes of lymphocytes and analyze the cerebrospinal fluid for myeloid cell changes.4
If a patient is diagnosed with Acute Lymphoblastic Leukemia, he or she will most likely have signs and symptoms including fever, easy bruising or bleeding, petechiae, and bone or joint pain.4 Other signs and symptoms that are prevalent in this population include painless lumps in the neck, underarm, stomach, or groin. Documentation of pain or feeling of fullness below the ribs, weakness, feeling tired, looking pale, and loss of appetite have also been noted.⁴ Once a patient is diagnosed, the disease cure rates are between 70% to 80% with remissions lasting a minimum of five years.5 Management of the disease and survival of children post-five year diagnosis is being correlated with increased post-therapeutic efforts by clinicians to maintain patient monitoring and education, while also having access to consistent pharmacological management.6
The pharmacological treatment of ALL is an extensive process that can from two to three and a half years.7 Treatment plans are determined by the interdisciplinary team based on the patient’s disease stage and current health status. The plans are normally broken into two main phases, Remission Induction and Post-induction. Both phases utilize different types of chemotherapy drugs.7 Phase 1, Remission Induction Therapy, typically lasts between four to six weeks. The typical drug regimen used consists of vincristine, L-asparaginase, and a corticosteroid (either prednisone or dexamethasone).7 During this stage, the patient is normally hospitalized and receives all treatments via intravenous administration.8-10 The end goal is to induce a complete absence of the cancer cells within the body.7 If by the end of the four weeks the cancer cells are still present, the patient will continue in this phase.4
Phase 2, Post-induction Therapy, is typically divided into three sub-parts, Consolidation, Reinduction, and Maintenance. Consolidation lasts approximately four to eight weeks and involves two different regimens: intrathecal therapy and systemic therapy.7 Intrathecal therapy is when the chemotherapy drugs are injected into the cerebrospinal fluid. Systemic therapy is when the chemotherapy drugs are injected into the circulatory system. Both forms contain similar drugs of methotrexate, L-asparaginase, cyclophosphamide, dexamethasone, and 6-mercaptopurine. Depending on the protocol, L-asparaginase can be substituted for doxorubicin.7 These drugs are given in the hospital via intravenous, intrathecal, or oral administration as a preventative therapy to eliminate the cancer that is within the central nervous system.7,9-13 Reinduction takes three to six months to complete. Its purpose is to reduce leukemic cell burden and eliminate any drug-resistant leukemic cells that might remain.7It consists of the same drugs listed previously, but at different dosages. Maintenance is the longest phase, lasting two to three years. This phase’s objective is to prevent the re-emergence of a drug-resistant leukemic cell clone. Its regimen consists of anti-metabolite therapy drugs, mercaptopurine and methotrexate.7 These drugs are taken at home via oral administration daily or weekly depending on the dosage.13,11 Not complying with the maintenance drugs increases the chances of a relapse. In the event of a relapse, a bone marrow transplant is commonly performed, and the maintenance phase is skipped.4
Currently, there is an experimental treatment option called Chimeric Antigen Receptor T-cell Therapy. It is a form of immunotherapy specifically used for patients who have B-cell ALL. It works by redirecting T-cell specificity and function so it binds to the CD19 antigen on the cancerous B-cells. The T-cells are removed from the patient’s blood, modified, grown in the laboratory, and then infused into the patient. Thus far, the clinical trials are showing encouraging results.4,14
Approximately 98% of ALL diagnoses attain remission after the Remission Induction phase. Research shows 85% of patients, ages one to 18, currently seeking treatment ages are expected to be long-term survivors. More than 90% of this group will survive at five years. Prognosis for relapse is dependent on the site of cancerous cells within the body and the time of original diagnosis.15 For those who relapse within 18 months, there is a 40% to 50% chance of survival. That statistic improves to 75% to 80% when relapse occurs after 18 months. The younger the child is when the diagnosis is made, the better chances of survival.15
Although chemotherapy has its benefits, it is also important to know the risks that are associated. These drugs not only kill the cancerous cells, but also attack healthy cells within the body. This can cause serious side effects like neurotoxicity, muscle atrophy, infertility, and liver toxicity. Other common side effects include, headaches, hair loss, impaired wound healing, nausea/vomiting, decreased appetite, stomach cramping, increased blood sugar levels, mouth sores, and low blood cell counts.8-13 Physical therapists cannot assist with the prevention of a majority of these side effects. However, they can minimize the impact these side effects have on how the body functions physically.
When a patient enters the physical therapy clinic for the first time and is given new patient paperwork, it is important to include quality of life, stress, and caregiver burden questionnaires. This will help the physical therapist understand not only where the patient is physically, but also where they and their families are mentally. Some high-quality questionnaires to include are the Caregiver Strain Index, Reintegration to Normal Living Index, Response to Stress Questionnaire, and McGill Pain Questionnaire. All of these questionnaires have excellent test-retest ability, internal consistency, and concurrent validity.16-19 For good practice and documentation, re-administer these forms sporadically throughout and at the conclusion of treatment.
During a physical therapy evaluation, physical dysfunctions that will be treated include severe fatigue, generalized weakness, and joint pain. Acute lymphoblastic leukemia should be initially treated with chemotherapy and other pharmacological options but in conjunction with strengthening and endurance training. The purpose of strength conditioning, functional capacity and endurance training will allow the patient to be able to complete ADLs, have peer to peer interactions, decrease the need for caregiver assistance, and improve overall patient quality of life. Sets of interactive obstacle course exercises with high repetitions, light loads/resistance, and low complexity will allow the patient to improve his or her endurance tolerance for daily play and self-care. Aquatic therapy is also a good option for patients in this population to help with non-weight bearing activities while increasing endurance and strength of the cardiovascular and musculoskeletal systems.
Treatment should be completed before chemotherapy begins in order to manage the extreme amounts of fatigue the patient will be experiencing. If physical therapy intervention is implemented after chemotherapy, the patient will need to have frequent rest breaks. The therapist also needs to be conscious of signs of distress and overwhelming fatigue. In order to address joint pain, therapists should implement gentle massage during sitting play, reduce weight bearing activities, and perform light stretches. To assess patient progress with improving functional capacity and endurance, therapy should be completed two times a week, ranging from four to six weeks in respect to the chemotherapy treatment schedule.
Depending on the impact of ALL and current disabilities of the child, the need for certain medical equipment, such as lower extremity orthotics, crutches, or a functional wheelchair, might arise. Decisions are most often determined by the presence of musculoskeletal weakness, fatigue, or joint pain as a result of the cancer and pharmacological treatment. These pieces of equipment can help a child become more functional when traveling short or long distances, increasing their quality of life.
Referrals are made to the primary care physician and the oncologist in order to discuss any new findings that might be suggestive of metastasis or further bodily system dysfunctions. Occupational therapists will be referred to for improving fine motor tasks and activity of daily living functions. Physical therapists will monitor the impacts of medical treatment and assess the possibility of developmental delays. Depending on patient presentation, further referrals to any of these specialists might be warranted: hematologist, pediatric surgeons, radiologist, neurologist, social worker, dermatologist, psychologist, nutritionist, and child-life specialist.4
It is clear to see how a cancer diagnosis affects the patient, but it is also important to understand the emotional, financial, and relationship strains it can put on the family. As a parent seeing their child go through the pain that comes with diagnostic and treatment procedures, the experience can lead to an overwhelming amount of guilt.20 Simultaneously, siblings may feel as if their life is being disrupted and have concern for their sibling’s survival. This can lead to siblings feeling as if they have to fight for their parents’ attention, which may cause tension between family members.20 Family finances are also affected during this time. A cancer diagnosis comes with costly diagnostic tests/exams, hospitalizations, pharmacological treatments, traveling fees, and other hidden medical fees.21
Studies show that most families incur thousands of dollars of debt during cancer treatments, due to the western model of healthcare combined with well below average government/state insurance coverage.21 Research suggests that families could benefit from psychosocial support during this time, whether from relatives, friends, or mental health professionals. Seeking support might increase the family’s understandings and help gain a feeling of control over their situation. Eventually, as months of treatment pass by, a feeling of normalcy will begin to occur. However, the conclusion of treatment can bring on a new mixture of feelings and fears. Families might have joy that this process is over and their child is healing, yet continue to feel anxious over the accumulation of debt, relapse, growth developmental delays, or other treatment implications.20 It is important to remind patients and their family to reach out for help during these times as well.
Works Cited
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2. Seattle Children's Hospital powered by Mayo Medical Laboratories. Seattle Children's Hospital. https://seattlechildrenslab.testcatalog.org/show/CBC-No-Diff. Accessed July 13, 2018.
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12. Cleveland Clinic Cancer. Cyclophosphamide. Chemocare. http://chemocare.com/chemotherapy/drug-info/Cyclophosphamide.aspx. Accessed July 13, 2018.
13. Cleveland Clinic Cancer. Mercaptopurine. Chemocare. http://chemocare.com/chemotherapy/drug-info/Mercaptopurine.aspx. Accessed July 13, 2018.
14. NCI Dictionary of Cancer Terms. National Cancer Institute. https://www.cancer.gov/publications/dictionaries/cancer-terms/def/car-t-cell-therapy. Accessed July 13, 2018.
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16. Caregiver Strain Index. Rehabilitation Measures Database. https://www.sralab.org/rehabilitation-measures/caregiver-strain-index. Accessed July 13, 2018.
17. Reintegration to Normal Living Index. Rehabilitation Measures Database. https://www.sralab.org/rehabilitation-measures/reintegration-normal-living-index. Accessed July 13, 2018.
18. Response to Stress Questionnaire. Rehabilitation Measures Database. https://www.sralab.org/rehabilitation-measures/response-stress-questionnaire. Accessed July 13, 2018.
19. McGill Pain Questionnaire. Rehabilitation Measures Database. https://www.sralab.org/rehabilitation-measures/mcgill-pain-questionnaire. Accessed July 13, 2018.
20. Miedema B, Easley J, Fortin P, Hamilton R, Mathews M. The Economic Impact on Families When a Child is Diagnosed with Cancer. Current Oncology. 2008;15(4):173-178. doi:10.3747/co.v15i4.260.
21. Sneha L, Sai J, Ashwini S, Ramaswamy S, Rajan M, Scott J. Financial burden faced by families due to out-of-pocket expenses during the treatment of their cancer children: An Indian perspective. Indian Journal of Medical and Paediatric Oncology. 2017;38(1):4-9. doi:10.4103/0971-5851.203493.