Sickle Cell Disease
Leukemia/Lymphoma
CNS Tumors/ Neuroblastoma
Grab Bag
End of Life Care
100
Sickle Cell Disease (SCD) is a hematoglobinopathy, a genetic condition caused by a mutation in normal DNA that determines hemoglobin production. Normal Hemoglobin is absent but this is present in it's place.
What is Hemoglobin S?
100
This type of leukemia results from abnormalities in differentiation and proliferation in the lymphoid cell lineage (75-80% of childhood leukemia) versus this type of leukemia which results from abnormalities in differentiation in the myeloid or megakaryocytic cell lines ( 15-20% of childhood leukemia).
What is ALL? (Acute Lymphoblastic Leukemia) and …What is AML? (Acute Myelogenous Leukemia)
100
This is the average age for Neuroblastoma which is a solid, soft, mass that presents mostly in the abdomen. It is also known as the “silent tumor” since it presents with widespread metastasis at diagnosis in 60% of patients.
What is 2 years old? (Almost 90% of patients are diagnosed before 5 years of age.)
100
This is a malignancy in a small, round blue-cell. 35% of these tumors are found in the head and neck region, which resection is usually possible and radiation and chemotherapy are both used for most patients.
What is Rhabdomyosarcoma?
100
Palliative care is focusing on this aspect of care, instead of the curative goal of conventional treatments.
What is symptom management?
200
In patients with SCD, this organ has a low-oxygen environment and suffers massive infarction during the 1st 6 to 12 months of life, leaving the patient at higher risk for pneumonia, bacteremia, and osteomyelitis. To assist with this, these patients are generally on prophylactic PenVK.
What is the spleen?
200
This type of lymphoma is often characterized by the presence of binucleate or multinucleated giant cells. The classic Reed-Sternberg cell has a bilobed nucleus with two large, prominent nucleoli that give the cell an owl’s eye appearance. The presence of this cell is critical in establishing the diagnosis. Staging is dependent on organ involvement and ranges from lymph nodes, spleen, lung, bones, liver, and/or bone marrow.
What is Hodgkin’s Lymphoma?
200
This is the stage for NBL with any primary tumor with dissemination to distant lymph nodes, bone, bone marrow, liver skin, and/or other organs.
What is Stage 4?
200
This disease is most commonly found during the adolescent growth spurt and is distinguished from other bone tumors by the production of osteoid substance. The patient may have pain that is dull, aching, and constant and is often worse at night.
What is Oseosarcoma?
200
This can occur as a result of immflamation, tumor progression, or immunologic disorders. During the terminal stage, this most likely occurs due to infection but treatments should be discussed with the family and depends on the goals of care.
What is fever? ( Treatment with antibiotics may prolong the dying process without increasing comfort or decreasing suffering. This should be discussed and decided on by the patient, family, and medical team.)
300
Pain crisis is the hallmark of SCD and the most common reason patients seek medical care. These are two of the three factors that pain results from in this disease process.
What is a) ischemia secondary to the occlusion of blood vessels by sickled RBC’s, b) damage to the vascular endothelium, or c) inflammation?
300
More than 85-90% of Lymphoblastic Lymphomas are derived from the immaturity in this type of cell. Approximately 70% of these patients will present with a mediastinal mass which may cause symptoms including dyspnea, stridor, wheezing, dysphagia, and swelling of the head and neck.
What is the T cell?
300
This is a disorder of the posterior lobe of the pituitary gland that results in decreased or absent production of antidiuretic hormone (ADH). Due to the lack of ADH, excessive water is then excreted in the urine. Treatment of this disorder may include monitoring of the sodium levels and the use of the medication DDAVP (synthetic vasopressin).
What is Diabetes Insipidus?
300
Retinoblastoma is a rare intraocular tumor that may be inherited. The treatment is multimodal and requires both a pediatric oncologist and a pediatric ophthalmologist. However, this is primary form of treatment.
What is enucleation? (Enucleation is removal of the eye, leaving the eye muscles and remaining orbital contents intact.)
300
Besides reducing anxiety, and alleviating pain, this drug class can also decrease the sensation of air hunger which may be present as the natural progression of death occurs.
What are Opiates? (Morphine)
400
This is responsible for 25% of deaths in sickle-cell patients. It is the rapid deterioration in respiratory function and is caused by the occlusion of the vessels of the lungs with sickled cells.
What is Acute Chest Syndrome?
400
These are two of the prognostic factors that influence the prognosis and are included in the determination of treatment of ALL.
What are a.) Age at diagnosis (children 2-10 years old are likely to have the most favorable prognosis) b.) Initial leukocyte count (less than 50,000/mm3 most favorable) c.) Speed of response to treatment?
400
Certain types of brain tumors can diminish or block CSF, causing increased intracranial pressure (ICP) which can include this triad of symptoms.
What are morning headache, morning emesis, and lethargy?
400
This tumor differs from Osteosarcoma because of its radiosensitivity. It does most frequently occur in the bone. PNET (primitive neuroectodermal tumor) is also in this family of tumors.
What is Ewing’s Sarcoma?
400
“Gasping” respirations are the last respiratory pattern prior to terminal apnea and may last for a few breaths or continue for days. This is the primary cause of this type of breathing.
What is severe hypoxia? (This may be very distressing for the family to witness but it is believed that the patient is now rendered unconscious and is not experiencing discomfort or distress.) Sharing this with the family may be comforting to them.
500
This is the result of the trapping of sickled RBC’s in the spleen. The consequence is an enlarged spleen and a rapid, severe drop in hemoglobin with a compensatory rise in the reticulocyte count. The platelet count drops sharply as platelets become trapped in the spleen as well.
What is Splenic Sequestration?
500
This syndrome is a potentially fatal metabolic condition, that occurs when the rapid breakdown of a large number of malignant cells are releases intracellular metabolites into the extracellular circulation. This condition may present hyperkalemia, hypophosphatemia with subsequent hypocalcemia, and hyperuricemia.
What is Tumor Lysis Syndrome? PUP’s up /Ca+ down (to help remember)
500
An increased level of this adrenal gland hormone may result in the following which are symptoms of NBL – hypertension, flushing, periods of sweating, weight loss, and irritability. A urine collection of this same hormone is included in the initial work up for the disease.
What is catecholamine?
500
This is a large, rapidly growing, vascular tumor which may present with an asymptomatic abdominal mass, hematuria, and sometimes hypertension.
What is Wilm’s Tumor?
500
During end of life care, if the patient is showing signs of decreased strength, dizziness, shortness of breath, and tachycardia, this treatment may be an option to relieve these symptoms.
What is a PRBC transfusion?
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