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Pediatric Oncology for the Primary Care Provider Kate A

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1 Pediatric Oncology for the Primary Care Provider Kate A
Pediatric Oncology for the Primary Care Provider Kate A. Mazur, MSN, RN, CPNP Texas Children’s Hospital Advanced Practice Provider 1st Annual Conference Houston, TX February 8, 2014

2 Objectives Review the incidence of childhood cancer
Describe the most common childhood malignancies, with a focus on initial evaluation and diagnosis, red flags, and when to refer to specialist Analyze the unique precautions for patients with oncologic illnesses Through out the presentation, you’ll notice some text highlighted in red – these are the items that can be considered “red flags” or important take home points to help you to know when to refer to oncology Long term survivorship issues are hugely important for PCPs but earlier this conference Dr. ZoAnn Dreyer did an excellent presentation on this issue so I will not discuss it in this talk And I will also not talk about any treatment for this presentation b/c I think it’s beyond the scope of this talk and the treatment is usually managed by the oncology team and followed closely by them throughout the course of treatment

3 The Scope of the Problem
13,400 children ages birth to 19 years are diagnosed with cancer each year Cancer is the #1 cause of disease-related death in children Source: Surveillance, Epidemiology, and End Results Program, , Div. of Cancer Control and Pop. Sciences, NCI, 2006

4 The Good News… Overall 5 year survival rate across all cancers is now 80% Estimated 270,000 childhood cancer survivors in the U.S. However, two-thirds of survivors face at least one chronic health problem 25% of survivors face a late effect from treatment that is classified as severe or life- threatening I left this slide in here even though Dr. Dreyer presented survivorship in more detail but I think this is just very important to drive home. These children are being seen out in the community both before and after diagnosis into long term survivorship it is imperative that all survivors of childhood cancer receive ongoing monitoring and continued physical and psychosocial care throughout childhood, adolescences, and into their adult lives

5 Where do I start? Early diagnosis and initiation of treatment is imperative to improved survival Diagnosis of cancer starts with a thorough history and physical Most symptoms of childhood cancer due to either a mass, its effect on the surrounding tissues, invasion of the bone marrow, or secretion of a substance by the tumor that disturbs normal functions Most common presenting signs and symptoms of many malignancies include weight loss, failure to thrive, anorexia, malaise, fever, pallor, and lymphadenopathy Chief complaint is the most important clue

6 High Risk Patients Familial cancer pre-disposition syndromes
Li-Fraumeni Syndrome Familial adenomatosis polyposis Beckwith-Wiedemann Syndrome Family history of cancer Retinoblastoma Autoimmune diseases in patient or family Down Syndrome Neurofibromatosis Type 1 HIV/AIDS > 50 familial cancer predisposition syndromes have been described Li-Fraumeni – p53 mutation; breast CA, soft tissue sarcoma, osteosarcoma, leukemia, brain tumors, ACC, and others FAP – APC gene; both benign and cancerous tumors in small intestine, brain, stomach, bone, skin, and other tissues BWS – Wilm’s tumor, pancreatoblastoma, hepatoblastoma RB – RB1 gene; any eye cancers, CA of pineal gland, osteosarcoma, melanoma, and soft tissue sarcoma Autoimmune – bigger risk factor in adults; lymphoma, Cancers caused by viruses (cervical CA, liver CA, stomach CA) Downs – increased risk ALL NF-1 – chromosome 17 mutation; peripheral nerve sheath tumors HIV – non-Hodgkin lymphoma, Kaposi sarcoma

7 Leukemia Cancer of the bone marrow
Uncontrolled proliferation of immature white blood cells (“blasts”) Most common type of cancer in children and adolescents under 20 years of age (~30%) Incidence 4900 new cases each year Males > Females; Hispanics > Caucasians > African Americans Peak age 2-5 years Risk factors Down Syndrome: 14-fold increase Klinefelter Syndrome Fanconi anemia Immunodeficiencies: ataxia telangiectasia, Wiskott-Aldrich, Bloom syndrome Past exposure to chemotherapy or ionizing radiation Immature WBCs, such as myeloblasts, lymphoblasts, and monoblasts Does appear to be a race difference Note that none of these slides will list “causes” b/c the cause of the majority of childhood cancers is still unknown. I have listed some risk factors if any have been identified that may increase your risk of developing that particular malignancy Klinefelter syndrome – extra “X” chromosome; slight increase Fanconi anemia – genetic defect in cluster of proteins responsible for DNA repair Immunodeficiencies: r/t increased risk of infection Ionizing radiation is the ONLY environmental factor that has been proven to play a role in the development of childhood ALL. Lots of false information out there re. pesticides, etc; constantly being studied. Discovery of the link to ionizing radiation was made following the atomic bombs in Japan, where children who were exposed acquired an increased risk of developing leukemia. Leukemia almost certainly caused by some sort of virus or other infection, but has not been identified yet. Due to age at presentation (early school aged)

8 Most Common Types of Childhood Leukemia
Acute lymphoblastic leukemia (ALL) ~ 80% Acute myelogenous leukemia (AML) ~ 15% Chronic myelogenous leukemia (CML) ~ 5% Each main type of leukemia is named for the type of cell that is affected (a myeloid cell or a lymphoid cell) and whether the disease begins in mature or immature cells

9 Leukemia: Presenting Signs and Symptoms
Relate to the infiltration of the bone marrow History Fatigue Persistent fevers Bone pain Anorexia Weight loss Recurrent infections Physical Exam Pallor Bleeding, bruising, or petechiae Hepatosplenomegaly Lymphadenopathy Most of the presenting signs and symptoms relate to the abnormal blood counts May present with a limp or refusal to bear weight Recurrent infections – have a high index of suspicion for the kid that frequently returns with ear infections, for example Frequent nose bleeds; gingival bleeding when brushing teeth, etc HSM might cause abdominal distension

10 Lymphadenopathy: When to Worry
Supraclavicular, axillary, or epitrochlear adenopathy Adenopathy that persists longer than 6 weeks or is increasing in size Asymmetric lymphadenopathy Leukemia usually presents with generalized lymphadenopathy; localized LAD more likely to be infectious in origin Malignant nodes generally hard and nontender; infectious/inflammatory nodes usually tender, freely moveable, overlying erythema For localized cervical adenitis with inflammation and fever, treat with ONE course of antibiotics, if no response  refer DO NOT START STEROIDS I cannot stress this enough. It will muddy the clinical picture and ultimately cause a delay in the accurate diagnosis and treatment start

11 Leukemia: Initial Diagnostic Tests
CBC with manual differential, reticulocyte count, and peripheral blood smear Chem 10: Electrolytes, BUN, creatinine, Ca2+, Mg2+, PO4-, uric acid, LDH Hypocalcemia, hyperkalemia, hyperphosphatemia, hyperuricemia may indicate tumor lysis syndrome  oncologic emergency Elevated LDH (non-specific tumor marker) Bone marrow aspirate and biopsy is the only definitive diagnostic test Done by oncology service only Specify you want the differential on the order b/c this is not automatically done at all labs. Also, most labs do an automatic diff and often do not do a smear, so if the initial CBC is concerning, you may want to send a second sample to an alternative lab, such as at a larger hospital-based lab where you can get all of these components I’m going to review the CBC findings in the next few slides These electrolyte abnormalities may be indicative of tumor lysis syndrome which is an oncologic emergency. I won’t go into detail on this since TLS was covered by Brooke Bernhardt in the pharmacology pre-conference but if you see this, you need to either call the oncology team immediately or send the patient to the ER LDH is an indicator of tissue breakdown; cancer cells in general have a very high turnover rate so the destroyed cells lead to an elevation in the LDH LP if no contraindications such as elevated ICP (risk for herniation) BMA and LP ONLY to be done by the oncology service after referral ~5% of leukemia pts have CNS involvement at presentation

12 Complete Blood Count: A Review
White blood cells/Neutrophils Responsible for fighting infection Red blood cells/Hemoglobin Carries O2 from lungs to blood tissues and CO2 from tissue to lungs Platelets Necessary for clotting and control of bleeding Most important screening tool for leukemia that is easy to obtain, relatively non-invasive, inexpensive, and can be done in an outpatient primary care setting Cannot understand leukemia without first understanding the basics of the CBC components and what is normal in order to identify when to be concerned for malignancy

13 CBC Findings in Leukemia
Leukocytosis - ~50% with WBC >10,000 and ~20% > 50,000 Elevated blasts and low neutrophils Anemia Thrombocytopenia 2 or more cell lines decreased  REFER 90% present with hemoglobin < 11g/dL, 45% < 7g/dL 75% present with platelets < 100,000/mm3 CBC is the most important screening tool for PCPs and can give you 90% of the information you need for the diagnosis

14 Lymphoma Tumor of the lymphatic system Incidence Risk factors
1500 new cases each year More common in adolescents and older teenagers, males, Caucasians Risk factors Immunodeficiency syndromes: Wiskott Aldrich, SCIDS, HIV/AIDS Autoimmune diseases: RA, SLE Some viruses: Epstein-Barr (particularly for Burkitt’s lymphoma) Since lymphoma is a cancer of the lymphatic system, which is responsible for defending the body from foreign invaders, most of the predisposing risk factors have to do with disorders that affect the immune system, either under-active (as is the case in ID or over-active as in AI) Wiskott Aldrich – X-linked, recessive disease; chronic thrombocytopenia, recurrent infections SCIDS – severe combined immunodeficiency syndrome; absence of functional T-lymphocytes HIV/AIDS – HIV pts are determined to have AIDS when they develop significant conditions and/or diseases in conjunction with the virus and often lymphoma is this determining factor Large correlation b/w autoimmune diseases and lymphoma – b/c as you know, in autoimmune disorders, the immune system sees its own tissues and attacks them as it would a germ or foreign invader. Lymphocytes (the cells from which lymphomas start) are part of the body’s immune system so the overactive immune system in autoimmune disorders may cause lymphocytes to grow and divide more often than normal and increase the risk of them developing into lymphoma cells Some viruses directly affect the DNA of lymphocytes, helping to transform them into cancer cells EBV – member of the herpes virus family, extremely common, responsible for mono infections and most cases of EBV are not serious. However, in pts w/compromised immune systems (i.e. HIV or AIDS) in which T-cells do not destroy infected B-cells, EBV-infected cells may become cancerous. Strongest correlation b/w EBV and lymphoma pertains to Burkitt’s lymphoma

15 Types of Lymphoma Non-Hodgkin’s Lymphoma – 60%
Lymphoblastic Anaplastic Burkitt’s Diffuse large B-cell Hodgkin’s Lymphoma – 40%

16 Lymphoma: Presenting Signs and Symptoms
History – 75% asymptomatic Unexplained fever for more than 3 days Weight loss of 10% within 6 months Drenching night sweats Malaise Anorexia Physical Exam – based on location of disease Head/Neck: supraclavicular or cervical adenopathy, jaw swelling, unilateral tonsillar enlargement Abdomen: splenomegaly, abdominal distention, jaundice Mediastinum: cough, chest pain, stridor Again, similar to leukemia, the lymph nodes are usually firm and are often described as “rubbery”. May be painless or painful if it has enlarged quickly

17 Lymphoma: Evaluation and Diagnostic Work Up
DO NOT START STEROIDS Blood work: CBC w/diff, liver and renal fxn tests, including alkaline phosphatase; ESR, ferritin, LDH may be elevated Imaging Studies CT scans (neck, chest, abdomen, pelvis) PET scan Bone scan Biopsy of affected node Required for definitive diagnosis Done under guidance of oncology team only CBC – might see cytopenias if there is bone marrow disease ESR, ferritin, LDH – non-specific, indicators of inflammation and cell turnover although XR can be performed quickly to determine presence of mediastinal mass, it won’t give you much other information about the extent of disease CT to evaluate for metastasis Positron emission tomography – only 70% of pts have disease that will show up on a PET scan. This is a nuclear imaging scan, where they inject a dye before the scan and it gets taken up by the malignancy which will then show up as a bright spot on the scan. However, the dye can also be taken up by infection or thrombosis so you may get false positives. So it’s really helpful to use the two imaging techniques in conjunction with one another to get the most information possible. Sometimes a bone scan is done if there is bone pain, an elevated alkaline phosphatase, or suspicion for metastatic disease but this may not be done with every patient Biopsy – only oncology service should perform this procedure

18 Brain Tumors Can be malignant or “benign”
Much slower rate of treatment advances than other malignancies Unique challenges in children – brain still developing Incidence 3400 new cases every year More common in ages < 15 years Risk factors Little known Hereditary cancer predisposition syndromes (i.e. Li-Fraumeni Syndrome) I use quotes around benign, b/c this term isn’t really helpful in pediatric oncology and rarely used. This is b/c even the most low grade tumors can have devastating effects b/c of the nature of its location Brain is immature – much more likely to result in devastating late effects with huge morbidity associated with treatments for brain tumors, with the biggest player being radiation to the brain and what that can do later down the road to things like cognition, growth and development, hormones, etc

19 Most Common CNS Tumors Astrocytoma Brain Stem Glioma Medulloblastoma
Most supratentorial but can originate in cerebellum, brainstem, or hypothalamus Brain Stem Glioma Medulla, pons Medulloblastoma Cerebellum Ependymoma Ependymal tissue within the ventricular system, most commonly the fourth ventricle Astrocytoma – originate in astrocyte cells BSG – diffuse, infiltrating, very poor survival Medulloblastoma – highly cellular; arises from primitive neuroepithelial cells Ependymoma – predominantly arise from epithelial cells lining the ventricles in the brain Picture from The Children’s Hospital at Montefiore,

20 CNS Tumors: Presenting Signs and Symptoms
Depends on site and severity of disease as well as child’s age & development History Seizures Visual changes Headache Nausea/vomiting, often in morning Poor concentration or mental status change Physical Exam Hemiparesis Endocrinopathies Ataxia Cranial nerve deficits signs of increased ICP lethargy, academic changes, personality changes

21 Headache: When Further Evaluation is Warranted
Recurrent morning headache Headache that awakens the child Intense, incapacitating headache Changes in the quality, frequency, or pattern of headaches Presence or onset of neurologic abnormality Ocular findings such as papilledema, decreased visual acuity, or loss of vision Associated with vomiting that is persistent, increasing in frequency, or preceded by recurrent headaches Age 3 years or less DO NOT START STEROIDS increased severity, increased frequency children this young don’t usually complain of headaches these findings might suggest the need for a CT scan – do not wait until you can schedule one a few weeks down the road. Send to the ER to get one same day if you have a high index of suspicion for CNS pathology

22 CNS Tumors: Diagnostic Tests
Imaging studies are the only diagnostic tests MRI brain and spine CT often obtained first due to easy access LP if clinically indicated and safe to perform on the left – medulloblastoma as you can see, these aren’t always a solid mass. They can just appear as a sort of brightening, or what we call “enhancement”. The one on the right is more diffuse, that one on the left is more of a solid mass; sometimes you see a darkening in the center that usually represents some necrotic tissue. Sometimes if tumors grow really rapidly, then the tumor cells in the center might start to die as new ones form. Some CNS tumors have the potential to seed along the CSF pathway, so CSF cytology should be tested in patients with tumors such as medulloblastoma or ependymoma. However, and this is important – you MUST do the MRI BEFORE the LP to make sure it is safe to perform the procedure. If the MRI shows any evidence of midline shift on it, then there is a risk of herniation during the procedure and it must not be performed

23 Solid Tumors: Evaluation of an Abdominal Mass
ALWAYS suspect a malignant solid tumor in a child with a palpable abdominal mass Avoid abdominal palpation as much as possible; palpate gently if necessary In younger children, often renal  neuroblastoma or Wilms’ tumor In older patients, may be related to leukemia or lymphoma with enlargement of the spleen or liver Obtain comprehensive GU and GI histories Associated symptoms of flushing, palpitations, diarrhea, failure to thrive, and fever could point to disseminated process such as neuroblastoma Complete exam with focus on skin, extremities (bone pain), neuro exam (Horner’s syndrome, spinal cord compression), organomegaly, measurement of abdominal girth Obtain diagnostic imaging STAT Usually start with abdominal ultrasound Newborns might be an exception to this. I would still have malignancy on your list of differentials, but an abdominal mass in a neonate is usually renal in origin and most commonly caused by hydronephrosis or multicystic kidney Once you have either felt the initial mass on your exam or if it is obvious just by looking at the patient (i.e. abdominal distention, prominent veins), then do not palpate the abdomen. This is also not the time for you to have every med student or resident come and feel the abdomen. If there is a malignancy, it could be encapsulated and pressing on it might break open the capsule, causing the tumor cells to spill out and spread all over the body Other possible malignant causes include rhabdomyosarcoma, rhabdoid tumor, hepatoblastoma, sarcomas, or an ovarian tumor Do not wait until the next available outpatient scan date that is a few weeks down the road. Send them to the ER if you have to. Imaging can include a plain film of the abdomen, chest XR, abdominal ultrasound, CT scan (include chest and pelvis in addition to abdomen), bone scan, MRI spine if neuro symptoms

24 Wilm’s Tumor Large, rapidly growing, vascular renal tumor
Second most common intra-abdominal malignancy in children Can be bilateral or unilateral Incidence 500 new cases/year Peak incidence 3 years of age Slight female predominance; African-Americans > Caucasians > Asians Risk factors Congenital anomalies: GU malformations, hemihypertrophy, Beckwith-Wiedemann syndrome, Denys-Drash syndrome Familial pre-disposition syndromes: Li-Fraumeni syndrome In the interest of time, I am only going to review the most common solid tumors of childhood BWS – an overgrowth syndrome Denys-Drash – involving genitourinary abnormalities

25 Wilm’s Tumor: History and PE Findings
Abdominal mass – smooth, firm, rarely crosses midline but 5- 10% are bilateral Diffuse abdominal distention with large masses Usually asymptomatic, but 25% have abdominal pain, vomiting, hematuria, hypertension Signs of thrombosis: leg swelling, prominent veins over abdomen Signs of hemorrhage into tumor (occurs rarely): anemia, fever, rapid abdominal distension parents are often the first ones to notice the abdominal mass or distension thrombosis occurs more often than hemorrhage

26 Wilm’s Tumor: Diagnostic Tests
Blood work – CBC, liver function, renal function Coagulation screen – may acquire Von Willebrand’s Abdominal ultrasound usually first test ordered – will reveal mass arising from within kidney Doppler US to assess patency of renal vein and inferior vena cava Abdominal CT There are no specific tumor markers for wilms, but initial blood work might consist of CBC, LFTs, renal function may be impaired, however, it is often normal studies have shown that as many as 8& of Wilms’ tumors pts develop Von Willebrand’s disease at diagnosis; so this should be evaluated for to determine whether treatment with DDAVP is necessary to correct coagulation prior to surgical intervention Doppler US b/c thrombosis can occur Abdominal CT further assesses the extent of the mass and can assess for smaller lesions in the contralateral kidney; can also assess for any liver metastases

27 Neuroblastoma Cancer of the sympathetic nervous system; derived from neural crest cells Second most common solid tumor of childhood and the most common extracranial solid tumor Incidence 650 new cases every year in the U.S. 90% cases in children < 5 years old Boys > girls; Caucasian predominance Risk factors Most cases sporadic Has been identified in other disorders of neural crest cells Neurofibromatosis Hirschsprung’s disease Beckwith-Wiedemann syndrome DiGeorge syndrome Neural crest cells are the cells that make up the sympathetic or peripheral nervous system that can grow in the sympathetic ganglia, adrenal medulla, among other sites median age is 2 years so these pts are somewhat younger than Wilms’ tumor patients

28 Neuroblastoma: Clinical Presentation
Can arise anywhere along sympathetic nervous system Signs and symptoms depend on location of primary tumor and presence of metastasis Two-thirds have primary site in abdomen, usually adrenal Thoracic region is the next most common primary site About 60% have metastatic disease at presentation due to vague initial symptoms and late presentation Bone marrow Bone Liver Skin Orbits these kids appear more ill than the Wilms’ tumor patients due to the high incidence of metastatic disease at diagnosis

29 Neuroblastoma: Presenting Signs and Symptoms
Firm, irregular, non-tender abdominal mass Hepatomegaly Abdominal pain Urinary obstructions Flushing Sweating Diarrhea Anorexia Malaise Site-specific symptoms from metastases to bone, skin, liver, or CNS Opsoclonus-myoclonus syndrome – “dancing eyes and dancing feet” I’m only going to review the s/s related to the most common locations of primary disease and metastatic disease neuroblastoma is one of the few causes of massive hepatomegaly flushing, sweating, diarrhea are related to tumor secretion of vasoactive instestinal peptide Bone – bone pain, limp Skin – blueberry muffin lesions which i’ll show you a picture of in an upcoming slide Liver – jaundice CNS – seizures, neuro s/s, cranial nerve deficits Horner’s syndrome – ptosis of the eyelid; meiosis=constriction of the pupil on the same side; anhidrosis=decreased sweating on that side of the face (sometimes parents will report that only one side of their face gets red when they are outside or have been running; it would be the unaffected side) opsoclonus-myclonus – consists of myoclonic jerks, ataxia, and jerky eye movements and is a unique paraneoplastic syndrome associated with neuroblastoma. If you see this, it should prompt a search for an occult neuroblastoma even in the absence of an abdominal mass

30 Neuroblastoma: Presenting Signs and Symptoms

31 Neuroblastoma: Diagnostic Tests
CBC – cytopenias Chem 10 Ferritin, LDH, uric acid Liver panel Urine catecholamines (VMA/HVA) CT chest/abdomen/pelvis Tissue biopsy for definitive diagnosis Bilateral bone marrow aspirate and biopsy to evaluate for BM involvement MIBG scan or bone scan to evaluate for metastasis ferritin, LDH, uric acid may all be increased, although these are very non-specific tests; increased in many cancers but also in non-malignant causes, just markers of inflammation and high cell turnover these tumors excrete catecholamines so these are elevated in about 90% of cases; these are used as screening techniques in the past, but abdominal ultrasound or CT is the current diagnostic procedure of choice

32 MIBG Scan as you can see, these can be very hard to read. An MIBG scan is another nuclear medicine technique just like the PET scan I talked about earlier. A dye is injected before the scan that will be taken up by catecholaminergic cells and areas of disease will then show up as bright spots on the scan. However, there are also some spots that always light up which is why additional scans are often needed and the MIBG is primarily used to evaluate the extent of disease and look for metastasis. You’ll see normal uptake in the salivary glands, myocardium, liver, and bladder

33 Osteosarcoma Tumor of the bone; usually at the end of long bones
Incidence 400 new cases/year More prominent during adolescent growth spurt; periods of rapid bone growth Males > females; African-Americans > Caucasians Risk factors Ionizing radiation Hereditary retinoblastoma Li-Fraumeni Syndrome

34 Osteosarcoma: Signs and Symptoms
Dull, aching pain, usually worse at night +/- soft tissue swelling, warmth May have vascularity over the mass Decreased range of motion Often long duration of symptoms prior to diagnosis; can be up to 6 months

35 Osteosarcoma: Diagnostic Tests
Diagnostic Imaging Plain radiograph of affected area – “sunburst pattern” MRI to further examine tumor boundaries, soft tissue component, relationship to joints, blood vessels, neurovascular bundle Chest XR or CT to evaluate for mets Bone scan to evaluate for skeletal mets Blood Tests Elevations in LDH, alk phos may be present Tumor Biopsy Necessary for definitive diagnosis Lungs are most common site of metastasis; XR is a lot faster, cheaper, easier to get than a CT but a CT is more sensitive for smaller pulmonary nodules that an XR might miss Again, there are no specific tumor markers for osteosarcoma and the LDH and alk phos aren’t tumor-specific; abnormalities do not distinguish b/w bone and liver etiologies

36 Supportive Care of a Patient with Cancer Infection Prophylaxis
Good hand washing!! Proper dental hygiene – daily brushing with a soft brush, use of chlorhexidine mouth rinse Avoidance of crowded, enclosed spaces Cleanliness of perirectal area – avoid constipation No rectal temperatures! Prophylaxis for Pneumocystis carinii pneumonia (PCP) from time of diagnosis until 6 months after completion of therapy – Trimethoprim- sulfamethoxazole, Pentamidine, Dapsone Viral and fungal prophylaxis may be indicated children receiving chemo are susceptible to acquiring infection from bacterial, viral, fungal, and protozoal organisms They are chronically myelosuppressed, and at times, severely myelosuppressed, as a direct result of the chemotherapy and have an increased exposure to nosocomial organisms many of these children have central venous catheters and may intermittently have mucosal breakdown secondary to chemo – 2 situations that interrupt the integrity of the physical defense barriers hyponutrition also plays a significant role in host susceptibility certain standards of care can be implemented to minimize the risk of acquiring serious infections in these childrens Avoid constipation just means encouraging a proper diet and liberal use of stool softeners if necessary and no rectal suppositories either; we want to avoid the possibility of a mucosal tear and entrance of enteric organisms PCP is an opportunistic infection that can be acquired in immunocompromised hosts Bactrim is usually first line. It is given twice daily on three consecutive days/week. Pentamidine is an inhaled or IV treatment, usually given monthly, that is used when patients can’t tolerate Bactrim or have a sulfa allergy. Dapsone can also be used, but this is falling out of favor b/c they have seen methemoglobinemia develop that has been linked to dapsone use just recently

37 Management of Fever in the Child with Cancer
Defined as a single temperature of ≥ 101°F or two temperatures ≥ °F taken 1 hour apart Do not give anti-pyretics! No rectal temperatures Every cancer patient who presents with fever should be considered septic until proven otherwise Prompt evaluation and management essential for improved survival Goal is to administer first dose of IV antibiotics within 1 hour of presentation you do not want to mask a fever b/c it is a sign that some sort of an infectious process is going on that we would want to be able to promptly recognize and treat since their immune systems are not working properly, they may not have the same signs or symptoms of an infection as a normal host would, so fever may be the only sign of infection

38 Initial Evaluation of Fever
Prompt tx essential – send to ER or urgent care center for immediate evaluation and management and call oncology service Evaluate for signs of septic shock Tachycardia – usually the first sign of shock. Ideally, aggressive treatment starts here. Check perfusion – delayed capillary refill; pulses weak and thready or bounding Hypotension – LATE sign Mental status changes; lethargy – OMINOUS Examine for focal signs of infection Often there are none Oral cavity, perianal area, skin, respiratory tract, abdomen (typhlitis) these kids can deteriorate VERY quickly. I cannot stress that enough. We have gotten very good at recognizing shock early and treating aggressively so often can avoid long ICU stays or starting pressors on these kids. A lot of things can cause tachycardia so many of us have started to disregard its significance. And many of those causes are reasons these pts could be tachycardic (anemia, anxiety, pain, fever) but now there is an evidence based table that corrects HR for fever and if the HR exceeds that number for the child’s age, we start treating them for shock. There are very few kids who will be harmed by fluid resuscitation or antibiotics. There are some exceptions, and those kids would be carefully considered and treatment modified accordingly. But the benefit far outweighs the risk. I won’t go into the signs of septic shock since I believe Dr. Patel did a separate talk on this subject

39 Fever: Diagnostic Work Up
CBC with differential – risk-based management based on neutrophil count Blood cultures – all CVC lumens and peripheral; important to have prior to first dose of antibiotics Urinalysis/urine culture, if feasible based on clinical status – no catheterization Additional cultures of any potential sources of infection – skin lesions, stool sample if diarrhea, mouth sores Do NOT I & D any skin lesions Chest XR if respiratory symptoms – obtain portable or delay until after initial antibiotics Do NOT perform LP until oncology service is consulted the overall theme is that we do not want to delay the start of antibiotics for these patients if an LP seems indicated initially, contact the pediatric oncologist immediately for assessment. Evaluation for increased ICP may be necessary by imaging in some patients prior to lumbar puncture

40 Empiric Therapy of the Febrile Patient
DO NOT wait for culture results Broad-spectrum antibiotics must be started immediately; will tailor antibiotic choice when culture results known Antibiotics chosen based on individual institution’s local microbial prevalence and antibiotic susceptibility patterns Regimen also based on risk criteria – high risk if ANC < 100, infant ALL, AML, in any phase of leukemia tx other than maintenance (induction at highest risk), < 7 days from last chemotherapy, focal signs of infection, or concern for sepsis Admission often indicated Anti-virals and/or anti-fungals may be added as clinically indicated Septic shock treated aggressively with normal saline fluid boluses and triple antibiotics I’m going to skip this slide since this was covered in Brooke Bernhardt’s talk on oncologic emergencies these patients will usually need more than one IV access so these interventions can be done simultaneously

41 Immunizations and the Cancer Patient
Cannot assume adequate immunization even if patient’s vaccines are up to date prior to diagnosis NO LIVE VACCINES Siblings or household contacts should not receive the oral polio vaccine All other vaccines may continue as scheduled, but will need to check titers at completion of treatment to ensure adequate immunity. Boosters or full re-immunization may be needed after completion of therapy Highly recommend seasonal influenza vaccination in patient and household contacts this is b/c all the chemo they get wipes out their immune system. Assume they are susceptible to everything. This is why herd immunity is so important. To protect patients like this from getting a potentially devastating disease that their immune systems will not be able to handle pts receiving chemotherapy or who are otherwise immune compromised, should not receive live virus vaccines – specifically the MMR and varicella vaccines. Also includes oral polio and the nasal spray formulation of the influenza vaccine (oral polio is a live vaccine; the injection is inactivated) Oral polio vaccine is contraindicated in household contacts b/c viral shedding may occur for 8-12 weeks and may lead to paralytic poliomyelitis in an immunocompromised patient so inactivated polio vaccine should be given instead. All other vaccines are safe to be administered to household contacts, included the other live vaccines such as MMR and varicella b/c transmission of disease from these vaccines does not occur may continue as scheduled, however, this sort of comes with a caveat – the inactivated vaccines pose no special risk to immunosuppressed children, but they pts may not respond adequately to the vaccine so we often hold all vaccines until therapy is completed with the exception of the flu vaccine! whether immunization occurred prior to or during chemotherapy since there is the potential loss of protective antibody titers as the result of immune suppressive therapy

42 References Howlader N. et al. SEER Cancer Statistics Review, , National Cancer Institute. Bethesda, MD, based on November 2012 SEER data submission, posted to the SEER web site, April 2013. Hastings, C. (2002). The Children’s Hospital Oakland Hematology/Oncology Handbook. St. Louis, MO: Mosby, Inc. Kline, N. & Tomlinson, D. (2005). Pediatric Oncology Nursing. Heidelberg, Germany: Springer Publishing. Lowry, A., Bhakta, K., & Nag, P. (2011). Texas Children’s Hospital Handbook of Pediatrics and Neonatology. McGraw-Hill Publishing. Pizzo, P. & Poplack, D. (2005). Principles and Practice of Pediatric Oncology, 5th edition. Philadelphia, PA: Lippincott, Williams & Wilkins. Zorc, J. (2013). Clinical Handbook of Pediatrics, 5th edition. Philadelphia, PA: Lippincott, Williams & Wilkins.

43 Questions??

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