Peds: Leukemia, Sickle Cell Anemia

what acid-base imbalance promotes sickling?

metabolic acidosis

will O2 help with SCA hypoxia?

no

should you suspect addiction if the SCA patient is watching the clock for the next pain medication dose?

no, never. they probably need a higher dose.

HSCT is not recommended for children with ALL during the first remission because

of the excellent results possible with chemotherapy

To reduce iron overload from chronic transfusion therapy, __________ therapy may be started

To reduce iron overload from chronic transfusion therapy, chelation therapy may be started

Sequestration crisis is

a pooling of a large amount of blood—usually in the spleen and infrequently in the liver—that causes a decreased blood volume and ultimately shock.

The invasion of the bone marrow with leukemic cells gradually causes

a weakening of the bone, a tendency toward fractures and severe pain

the most appropriate diagnosis for children with anemia is

activity intolerance r/t generalized weakness

one way to help prevent infection

adequate nutrition

SCA: Although the defect is inherited, the sickling phenomenon is usually not apparent until later in infancy because

of the presence of fetal Hbg (HbF)

best way to teach a child about their condition

on a doll

To reduce the negative connotation associated with the term crisis, it is best to say

pain episode

sx of bone marrow dysfunction-

peteciae, pain in joints and fatigue

Another serious complication is acute chest syndrome (ACS), which is clinically similar to

pneumonia

Leukemia occurs when stem cells in bone marrow

produce immature WBCs that don't function normally These cells proliferate & cause bone marrow to fill with abnormal WBCs

pro·lif·er·a·tion:

rapid increase in number

sudden sign of air embolus is

sudden difficulty in breathing

-Important to know- when cancer kids go home, teach families to

take temp once a day, if any temp diff in low or high tell them to come to the doctor immediately

In all types of leukemia, the proliferating cells depress the production of formed elements of the blood in bone marrow by competing for and depriving the normal cells of

the essential nutrients for metabolism.

fibrosis

the thickening and scarring of connective tissue, usually as a result of injury.

Multiple transfusions carry the risk for

transmission of viral infection, hyperviscosity, transfusion reactions, alloimmunization, and hemosiderosis

SCA: The most acute symptoms of the disease occur during periods of exacerbation called crises. There are several types of episodic crises, including

vasoocclusive, acute splenic sequestration, aplastic, hyperhemolytic, cerebrovascular accident, chest syndrome, and infection.

Most common & debilitating symptom experienced by patients with SCD is

vasoocculsive crisis -chronic pain can effect growth and development

Hydration is big! Need to keep blood flowing, no matter what drink more (sports, hot or cold days). If have crisis and want hydration during crisis and cant get oral can give through IV

yep

Hematopoietic stem cell transplantation has been used successfully for treating children who have

ALL and AML

Two forms of leukemia are generally recognized: what one do you need to know for the test?

Acute lymphoblastic leukemia (ALL) -Stem or blast cell- REMEMBER! Acute nonlymphoid (myelogenous) leukemia (ANLL or AML)

precautions for child with mouth ulcers

important to know- if you have ulcers, you will use soft toothbrush, put it under warm water. They are usually on a bland diet may need TPN

leukemia clinical manifestations to remember-

leg and joint pain, low grade fever, and easy bruising, anemia from infiltration of bone marrow, infection from neutropenia, bleeding from decreased platelets

Lumbar puncture for administration of chemotherapy and collection of cerebrospinal fluid may lead to a leak at the puncture site and low intracranial pressure. Some children may experience postdural puncture headache, which may be treated by

administering nonopioid analgesics and placing the patient in the supine position for 1 hour after the procedure.

Any pt dx with ALL leukemia- clinical manifestations are..

anemia, infection and bleeding

The most important prognostic factors for determining long-term survival for children with ALL (in addition to treatment) are

(1) the initial WBC count, (2) the child's age at the time of diagnosis, (3) the type of cell involved, (4) the sex of the child, and (5) karyotype analysis.

how often do SCA patients need to get blood transfusions?

-Don't like to do often, but during crisis need to protect kidney, spleen, etc -Any child at high risk for stroke b/c of sickling, get transfusions every 3-4 weeks to prevent risk of stroke

how to manage a SCA patient:

-Rest to minimize energy expenditure & to improve oxygen utilization -Hydration through oral & IV therapy -Electrolyte replacement because hypoxia results in metabolic acidosis (which also promotes sickling) -Analgesia for the severe pain from vasoocclusion -Blood replacement to treat anemia & to reduce the viscosity of the sickled blood -Antibiotics to treat any existing infection

when does fever for SCA become a concern?

38.5° C (101.3° F) or greater

4 phases of leukemia therapy

4 Phases 1.)Induction 2.)CNS prophylactic therapy -Prevents invasion into CNS 3.)Intensification (consolidation) -Eradicates residual leukemia cells 4.)Maintenance

A child diagnosed with lymphoma is receiving extensive radiation therapy. The MOST common side effect of this treatment is: A. fatigue. B. seizures. C. neuropathy. D. lymphadenopathy.

A

The nurses caring for a child are concerned about the child's frequent requests for pain medication. During a team conference, a nurse suggests that they consider administering a placebo instead of the usual pain medication. This decision should be based on knowledge that: A. this practice is unjustified and unethical. B. this practice is effective in determining whether a child's pain is real. C. the absence of a response to a placebo means the child's pain has an organic basis. D. a positive response to a placebo will not occur if the child's pain has an organic basis.

A

The primary consequences of leukemia in children are: I. Infection, from neutropenia II. Anemia, from decreased RBCs III. Vascular inflammation, from entanglement and enmeshing of RBCs IV. Bleeding, from decreased platelets a. I, II, and IV b. II, III, and IV c. I, II, and III

A

What are the most common signs and symptoms of leukemia related to bone marrow involvement? A. Petechiae, infection, fatigue B. Headache, papilledema, irritability C. Muscle wasting, weight loss, fatigue D. Decreased intracranial pressure, psychosis, confusion

A

what kind of vaccines are recommended for SCA patients ?

Administration of pneumococcal and meningococcal vaccines is recommended for these children because of their susceptibility to infection as a result of functional asplenia

aims of SCA therapy

Aims of therapy are to: -Prevent the sickling phenomena -Treat the medical emergencies of sickle cell crisis

what to do when a patient is clock watching

Analyze the drug and dosage and administer medica- tion on a regular schedule. Suggest changes to prevent rather than treat pain after it occur

A child who has been receiving morphine intravenously will now start receiving it orally. The nurse should anticipate that, to achieve equianalgesia (equal analgesic effect), the oral dose will be: A. the same as the intravenous (IV) dose. B. greater than the IV dose. C. one half of the IV dose. D. one fourth of the IV dose.

B

An important consideration when using the FACES pain rating scale with children is: A. that children color the face with the color they choose to best describe their pain. B. the scale can be used with most children, including those as young as 3 years old. C. the scale is not appropriate for use with adolescents. D. the scale is useful in pain assessment but is not as accurate when assessing physiologic responses.

B

What is appropriate mouth care for a toddler with mucosal ulceration related to chemotherapy? A. Lemon glycerin swabs for cleansing B. Mouthwashes with normal saline C. Mouthwashes with hydrogen peroxide D. Local anesthetic such as viscous lidocaine before meals

B

condition in which all the formed elements of the blood are simultaneously depressed

aplastic anemia

A child is status post-hematopoietic stem cell transplantation (HSCT) and is preparing for discharge home. Based on the nurse's knowledge of HSCT, which concepts are important to include in the discharge teaching plan of care? (Select all that apply.) A. Preparing the child to return to school within 6 weeks B. Keeping the child on a high-calcium diet C. Avoiding live plants and fresh vegetables D. Avoiding influenza vaccinations E. Practicing good hygiene

BCE

Definitive diagnosis: leukemia

Bone marrow aspiration or biopsy

8. Nursing care of the child with myelosuppression from leukemia or chemotherapeutic agents should include: A. restricting oral fluids. B. instituting strict isolation. C. using good handwashing. D. giving immunizations appropriate for age.

C

A child with sickle cell anemia develops severe chest pain, fever, a cough, and dyspnea. The FIRST action by the nurse is to: A. administer 100% oxygen to relieve hypoxia. B. administer meperidine (Demerol) to relieve symptoms. C. notify the practitioner because chest syndrome is suspected. D. notify the practitioner because child may be having a stroke.

C

leukemia etiology: what cause? what disorders make this more common?

Cause remains unknown General theory is genetic changes Some show pattern of inheritance ***Children with Immune Disorders -- HIV/AIDS ****Downs & Klinefelter syndrome greater risk for developing certain cancers Environmental

how to numb the pain of a lumbar puncture

local anathetic 1 hour before procedure

how is leukemia medication given?

Chemotherapy -With or without cranial irradiation -Intrathecal (in the spine) meds for CNS involvement- b/c most meds wont cross the blood brain barrier

1. A child who is terminally ill with bone cancer is in severe pain. Nursing interventions should be based on knowledge that: A. children tend to be overmedicated for pain. B. giving large doses of opioids causes euthanasia. C. narcotic addiction is common in terminally ill children. D. large doses of opioids are justified when there are no other treatment options.

D

Children who develop moon face from short-term steroid therapy used to treat cancer may experience symptoms of: a. acute toxicities. b. decreased appetite. c. permanent facial change. d. altered body image

D

The most consistent indicator of pain in infants is: A. increased respirations. B. increased heart rate. C. clenching the teeth and lips. D. facial expression of discomfort.

D

The nurse is starting an intravenous (IV) line on a school-age child with cancer. The child says, "I have had a million IVs. They hurt." The nurse's response should be based on the knowledge that: A. children tolerate pain better than adults. B. children become accustomed to painful procedures. C. children often lie about experiencing pain. D. children often demonstrate increased behavioral signs of discomfort with repeated painful procedures.

D

The parent of a child receiving an iron preparation tells the nurse that the child's stools are a tarry green color. The nurse should explain that this is a/an: A. symptom of iron-deficiency anemia. B. adverse effect of the iron preparation. C. indicator of an iron preparation overdose. D. normally expected change caused by the iron preparation.

D

Treatment for the child with aplastic anemia will most likely include: a. administration of testosterone. b. administration of iron-chelating agents. c. irradiation. d. bone marrow transplant.

D

Hematopoietic Stem Cell Transplantation (HSCT): general information

Donors may be relatives or non-relatives Stem cells from umbilical cord blood This is tx option for child who has a relapse with acute lymphoblastic leukemia, achieves 2nd remission; transplant given when child is in remission

Newborn screening for SCA is mandatory in most of the United States. why?

Infants can be identified before symptoms occur Early diagnosis (before 3 months of age) enables initiation of appropriate interventions to minimize complications Family is taught to administer prophylactic antibiotics & identify early signs of infection and to seek medical therapy as soon as possible

Complications of Myleosuppression

Infection Hemorrhage Anemia

screening test for SCA

For screening purposes, the sickle-turbidity test (Sickledex) is frequently used because it can be performed on blood from a fingerstick and yields accurate results in 3 minutes.

what antibiotic works for SCA?

Give penicillin as ordered

what organs are most affected my Leukemia?

Highly vascular organs, such as the spleen and liver, are the most severely affected. CNS involvement -May cause increased ICP and changes in LOC

home instructions for SCA patients

Home instructions- encourage oral fluids, avoid cold (induces sickiling) , hot (cause dehydration), and provide adequate rest

most common race, age, and onset of leukemia:

It is more common in boys and Caucasians Peak onset between 2 and 5 years of age

how to teach parents about adequate hydration

It is not sufficient to advise parents to "force fluids" or "encourage drinking." They need specific instructions on how many glasses or bottles of fluid are required daily.

It is one of the forms of cancer that has demonstrated dramatic improvements in survival rates.

Leukemia

patho of leukemia:*******important to know

Leukemia is an unrestricted proliferation of immature WBCs in the blood-forming tissues of the body Proliferating cells depress the production of all formed elements of the blood in the bone marrow

what vaccine should not be given to a child on chemp

MMR vaccine

is the SCA gene dominant or recessive? what chance does a child have a getting it when both parents have the gene?

Most important to know- gene that determines hgb is dominant, takes 2 parents to give the child the disease. When both parents have it, 25% of child getting it

A condition in which bone marrow activity is decreased, resulting in fewer red blood cells, white blood cells, and platelets. Myelosuppression is a side effect of some cancer treatments

Myelosuppression- a side effect of some cancer treatments

leukemia: Problems of Drug Toxicity

N & V Anorexia Mucosal Ulceration- may need TPN for awhile Neuropathy Hemorrhagic Cystitis

Clinical features of SCA are primarily the result of:

Obstruction caused by the sickled RBCs Vascular inflammation Increased RBC destruction

Oral penicillin prophylaxis is also recommended by _ months of age to reduce the chance of pneumococcal sepsis

Oral penicillin prophylaxis is also recommended by 2 months of age to reduce the chance of pneumococcal sepsis

Peripheral blood smear (done with CBC) what are you looking for when leukemia is suspected?

Peripheral blood smear (done with CBC) -- Immature leukocytes -- Frequently low blood counts -- proliferated blast cells

what works well for vasooculsive pain?

Pharmacologic treatment -PCA pump -Hydration and pain management -Physical therapy Complementary treatment e.g., prayer, spiritual healing, massage, herbs, relaxation, acupuncture, guided imagery & biofeedback

3 main consequences of leukemia:

The three main consequences are (1) anemia from decreased RBCs, (2) infection from neutropenia, and (3) bleeding from decreased platelet production

how can a SCA patient increase the awareness of their disease?

The use of medical identification, such as a bracelet, is another way of ensuring awareness of the disease

skin care for patients with leukemia:

Provide meticulous skin care, especially in the mouth and perianal regions, because they are prone to ulcer- ation. Change position frequently to stimulate circu- lation and relieve pressure. Encourage adequate calorie/protein intake to prevent negative nitrogen balance.

what elements of the CBC will be decreased in leukemia?

RBCs, WBCs, platelets, plasma, stem cells decreased

a group of diseases collectively termed hemoglobinopathies

SCA

condition where adult hemoglobin is partially or completely replaced by abnormal hemoglobin

SCA

Aplastic anemia is

a condition that occurs when your body stops producing enough new blood cells

In children with recurrent life-threatening splenic sequestration, splenectomy may be a lifesaving measure. However, the spleen usually

atrophies on its own through progressive fibrotic changes (functional asplenia) by 6 years of age in children with SCA.

Leukemia:

cancer of the blood-forming tissues, is the most common form of childhood cancer. Leukemia is a broad term given to a group of malignant diseases of the bone marrow and lymphatic system

The vasoocclusive crisis (VOC), preferably called a "painful episode," is

characterized by ischemia causing mild to severe pain that may last from minutes to days

cause of acquired aplastic anemia

chemo drugs

-Watch out for any reactions to blood transfusion-

chills, fever, rash

The current treatment for SCD children who have experienced a stroke is

chronic transfusion therapy

what is anemia?

decreased oxygen carrying capacity of the blood

First, although leukemia is an overproduction of WBCs, most often in the acute form, the leukocyte count is low (thus the term leukemia). Second, these immature cells do not

deliberately attack and destroy the normal blood cells or vascular tissues

Oxygen administration is usually not effective in reversing sickling or reducing pain because the oxygen is unable to reach the clogged vessels. In addition, prolonged administration of oxygen can

depress bone marrow, further aggravating the anemia

when is the best time to give chemo?

early in the day

The spleen, liver, and lymph glands demonstrate marked infiltration, enlargement, and eventually

fibrosis

leukemia: Spleen, liver & lymph glands become infiltrated & enlarged eventually become

fibrotic

what should a patient with SCA avoid?

flying, hot or cold temperatures

Sickle Cell Disease is one of the most common

genetic diseases worldwide

how to determine CNS involvement in leukemia

lumbar puncture to evaluate CNS involvement

Vasoocclusive Crisis: symptoms

• Pain in area(s) of involvement • Manifestations related to ischemia of involved areas Extremities—Painful swelling of hands and feet, painful joints Abdomen—Severe pain resembling acute surgical condition Cerebrum—Stroke, visual disturbances Chest—Symptoms resembling pneumonia, protracted episodes of pulmonary disease Liver—Obstructive jaundice, hepatic coma Kidney—Hematuria Genitalia—Priapism (painful penile erection)

Sequestration Crisis: symptoms

• Pooling of large amounts of blood •Hepatomegaly •Splenomegaly •Circulatory collapse

clinical manifestations of SCA

• Possible growth retardation • Chronic anemia (hemoglobin level of 6-9 g/dL) • Possible delayed sexual maturation • Marked susceptibility to sepsis