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Hem/Onc, Respiratory,
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Hematopoiesis
the production of RBC components
-typically at birth, all babies are hematopoietic in the bony cavities
-as a child ages, hemotopoiesis will move to include the central bones (spine, sternum, ribs, pelvis) which are more efficient and produce greater quantities
-key point: childhood cancer unlike adults has very little environmental influence
Blood
-4 basic components
plasma (liquid)
RBCs (carries oxygen)
WBCs (fights infection)
Platelets (clotting)
-RBCs are also called erythrocytes and make up almost ½ of your blood volume
they generate from bone marrow at a rate of 4-5 billion per hour
lifecycle of RBC is approx. 120 days
anemia
-number of RBCs of the Hgb and Hct concentration is reduced below normal value
-this condition reduces the oxygen carrying capacity of the blood, causing a reduction in the oxygen available for the tissues
-most common hematological disorder of childhood
infants/ toddler (too much milk and not enough iron rich foods) and adolescents (poor diet choices) are at highest risk
Patho:
-develops as a result of decreased production of RBCs or loss and destruction of RBCs
can be related to lack of dietary intake of the nutrients needed to produce the cells, alterations in the cell structure, or malfunctioning tissues (bone marrow)
anemia related to nutritional deficiency includes iron deficiency anemia, folic acid deficiency, and pernicious
may also result from toxin exposure (lead poisoning) or as an adverse reaction to a med (aplastic anemia)
blood loss may decrease RBCs from trauma or surgery
Consequences:
-if anemia has slow onset, most children will adapt and symptoms will be vague.
-hemodilution, MURMURS, cardiac failure, cyanosis, growth retardation/ FTT, stroke
Iron deficiency anemia
Risk factors:
-premature
-excessive intake of cow’s milk
-increased iron needs (blood loss), poor intake of iron (diet)
-GERD, malabsorption issues, colitis
Symptoms:
-tachycardia, pallor, fatigue, systolic heart murmur (due to ↓ blood viscosity), pica, weakness/fatigue, cognitive delay
Lab findings:
-↓RBC, H/H, reticulocyte count
-abnormal RBC, abnormal morphology
-stool guaiac to assess for hidden (occult) GI bleed
Anemia management
-treat underlying cause
-blood transfusion/ PRBC, supplemental oxygen, IV ferrous sulfate (can be painful and must be monitored during infusion)
-replace deficiency/ increase Vitamin C, limit cow’s milk, iron fortified cereals
-supplement for nutritional deficiency
Iron supplementation considerations/ education:
-give 1hr before or 2hr after milk, tea, or antacid to prevent decreased absorption
-GI upset is common at the start of therapy, will decrease over time
-if tolerated, administer iron supplements on an empty stomach
-give w/ vitamin C to increase absorption
-use a straw w/ liquid preparation to prevent staining of teeth
-use z-track method into deep muscle for parenteral injections. Do NOT massage after injection
-expect stools to turn a tarry green/black color if dose is adequate
-brush teeth after oral dose to minimize or prevent staining
-increase fiber and fluids if constipation develops
-to prevent toxicity, store no more than 1 months supply in a child-proof bottle out of reach of children
-allow adequate time for child to rest
-the length of treatment will be determined by the child’s response to the treatment. If Hgb levels are not increased after 1 month of therapy, further evaluation is warranted
-schedule universal anemia screenings at 12 months of age
Lead and anemia
-lead exerts toxic effects on the bone marrow, erythroid cells, nervous system, and kidneys
-the presence of lead in the bloodstream interferes w/ the enzymatic processes of the biosynthesis of heme
-the process results in hypochromic, microcytic anemia, and children may exhibit classic signs of anemia
-sources: paint (in homes built before 1978), soil, glazed pottery, stained glass products, lead pipes supplying water to the home, old painted toys or furniture, soil
Treatment:
-CHELATION THERAPY
treatment for lead toxicity
therapeutic management for high blood levels of lead involves chelation therapy (removal of heavy metals from the body via chelating agents), either orally or IV
Sickle Cell
-sickle cell disease = a group of disease in which abnormal “sick” hemoglobin (HbS) replaces normal adult hemoglobin (Hgb A)
-sickle cell trait: child has the gene but is asymptomatic
-sickle cell crisis: is the acute exacerbation of SCA
Sickle Cell Anemia:
-autosomal recessive
-African Americans, Mediterranean, Middle Eastern, Indian
-significant anemia, Hgb molecule is missing from the beta-chain, decreased life span of Hgb molecule, Hgb sickles when oxygenated
-sticky outer surface
Patho:
-as the cells sickle, the blood becomes more viscous bc the sickled cells clump together and prevent normal blood flow to the tissues of that area
-the sickle-shaped RBCs cannot pass through the smaller capillaries and venules of the circulatory system
-this vaso-occlusive process leads to local tissue hypoxia followed by ischemia and may result in infarction
-pain crisis results as circulation is decreased to the area. Pain may occur in any part of the body but is most common in the joints. Pain causes increased metabolic need by resulting in tachycardia and sometimes tachypnea, which leads to further sickling
Complications of sickle cell anemia
-vaso-occlusive pain crisis. Hemolysis follows sickling ad leads to further anemia. The increased activity of the spleen related to RBC hemolysis leads to splenomegaly, the fibrosis and atrophy
-jaundice, stroke, cold hands or feet, pallor
-headache, dizziness
-Acute chest syndrome: most common cause of death in children w/ SCA
-splenic sequestration: pooling of blood in the spleen leads to splenomegaly and abdominal pain
-reduced visual acuity related to decreased retinal blood flow
-chronic leg ulcers
-cholestasis and gallstones
-delayed growth and development
-priapism
-dactylitis = infarct of small joints
Precipitating factors of sickle cell crisis:
-anything that increases the body’s demand for oxygen or alters its transport
-trauma, infection, fever, physical stress, dehydration, hypoxia, extreme cold
Diagnostics and Management of SCA
Labs/ Diagnostics:
-cbc to detect anemia
-Hemoglobin electrophoresis separates the various form of Hbg and is the definitive diagnosis of sickle cell anemia or sickle cell trait
-Hgb ↓, ESR/Sed rate ↑, WBC ↑, bilirubin and reticulocyte levels ↑, peripheral blood smear reveals sickled cells,
-doppler to assess vascular flow
Treatment:
-rest
-oxygen
-HYDRATION, HYDRATION, HYDRATION
-analgesia for pain = w/ IV and move to oral as tolerated
-blood replacement for severe anemia = Hgb <7
-antibiotics for infection
-bone marrow transplant (95% cure rate) only matched related donor
-folic acid, daily treats anemia
-hydroxyurea daily for entire life span (still considered investigational) increased oxygenation to the cells
increased HgF reduces endothelial adhesion of sickled cells
Family education:
-early intervention for all fevers
-S/Sx of splenic sequestration
rapidly enlarging/firm spleen, severe left-sided abdominal pain, extreme paleness, rapid heartbeat, fatigue, weakness
-promote rest and HYDRATION
-S/Sx of acute chest syndrome
new cough, wheezing, tachypnea, hypoxia, new infiltrates on chest imaging
-medication compliance
-good resources for support
Aplastic anemia
-bone marrow fails to form elements of the blood
-pancytopenia which is a combination of anemia, leukopenia, and thrombocytopenia occurs
-typically, African American presents w/ a great decrease in RBC and a slight decrease in WBC and platelets
-Primary:
present at birth
fanconi syndrome: pancytopenia, hypoplasia of the marrow, patchy brown discoloration of skin from deposits of melanin, multiple other anomalies
-Secondary:
acquired
common causes = immune disorders, chemotherapy, anticonvulsants, leukemia or lymphoma, industrial and household chemicals, radiation, drugs, viruses
-most cases are acquired, but there are a few rare types of inherited aplastic anemias
-acquired aplastic anemia is thought to be an immune-mediated response
Treatment:
-immunosuppressive therapy to remove the presumed immunologic functions that prolong aplasia
-replacement of the bone marrow through transplantation (treatment of choice)
Hemophilia
-deficiency of one of the factors necessary for coagulation of the blood
-group of x-linked recessive (mostly males) bleeding disorders characterized by difficulty controlling bleeding due to deficiency in one of the coagulation factors
-factor VIII (Hemophilia A) = most common. 80% of cases
-Von Willebrand = inherited lack of von willebrand factor protein characterized by the inability of the platelets to aggregate
-classified from mild to severe
Manifestations:
-hemorrhage form any trauma (loss of teeth, circumcision, cuts, injections, epistaxis, etc.)
-excessive bruising from slight injury
-subcutaneous and IM hemorrhage
-hemarthrosis of any joint but especially knees, ankles, elbows = pain, swelling, stiffness, decreased ROM
-hematomas, even w/ minor injuries
-hematuria, tarry schools
-prolonged partial thromboplastin time (aPTT)
-platelets and PTT are often in normal range
Complications:
-intracranial hemorrhage
-airway obstruction
-shock
-joint deformity
-uncontrolled bleeding
Hemophilia Medication
-DDAVP (synthetic form of vasopressin that increases factor VIII) used in mild cases
-factor VIII, pooled plasma. Given IV and may require multiple doses
administer factor replacement by slow IV push
doses are based on the severity of the bleeding and the weight of the child
-corticosteroids = treats hematuria, acute hemarthrosis, and chronic synovitis
-NSAIDs = treats chronic synovitis; administer cautiously
Hemophilia management
-management of bleeding episodes
-administer injections Sub-q
-avoid unnecessary skin punctures
-venipuncture instead of skin and heel pricks
-monitor stool, urine, and NG fluid for blood
-DO NOT administer aspirin or aspirin containing products (acetaminophen okay)
-administer factor replacement
-teach child and family how to start IV and infuse factor
-observe for adverse effects = headache, flushing, alterations in HR or BP
-encourage rest and immobilize affected joints
-sports restrictions
-if external bleeding develops, apply pressure to the area until bleeding stops
-if it is inside a joint, apply ice or cold compresses to the area and elevate any injured extremities
Education:
-prevent bleeding at home
environment should be made as safe as possible to prevent injury
provide a safe home and play environment
set activity restrictions to avoid injury. Acceptable activities include low-contact sports (bowling, fishing, swimming, golf). While participating in these activities, children should wear protective equipment
used soft-bristled toothbrushes
-perform regular exercise and physical therapy after active bleeding is controlled
-maintain up-to-date immunizations
-wear medical identification
-observe for manifestations of internal bleeding and hemarthrosis
-control bleeding episodes using the RICE (rest, ice, compression, elevation) method
-participate in a support group
-administer factor replacement
Idiopathic Thrombocytopenic purpura (ITP)
-immune response following a viral infection that causes the body to produce antiplatelet antibodies
-acquired hemorrhagic disorder
-thrombocytopenia, minimal signs of bleeding (bruising, petechiae) normal bone marrow w/ normal or increased number of immature platelets (megakaryocytes)
-creation of antibodies against platelet antigens, leading to reduced platelet survival and impaired production
-most frequent occurring thrombocytopenia of childhood
-typically, under age of 10
-acute, self limiting: following parvo B19, measles, rubella, chickenpox, URI
-chronic > 12 months
-antibodies destroy platelets, which then lead to the development of petechiae, purpura, and excessive bruising
-ITP usually develops a few weeks after a viral infection and is most common in young children
ITP Assessment and Management
Assessment:
-petechiae, purpura, bruising
-inspect buccal mucosa
-ask for recent infection hx, immunizations UTD
Treatment:
-typically resolves w/i 1-2 months
-corticosteroids
-IVIG
-anti-D antibody: becoming the treatment of choice. Cheaper, one dose
-splenectomy for chronic ITP for children older than 5
-avoid aspirin, NSAIDs
-safety
-teach s/sx of serious bleeding
Thalassemia
-genetic disorder that is inherited via an autosomal recessive process
-children have reduced production of Hgb. Two types = alpha and beta with beta being more common. Beta disease can be divided into 3 categories
thalassemia minor = leads to mild microcytic anemia; often no treatment is required
thalassemia intermedia = child requires blood transfusions to maintain adequate quality of life
thalassemia major = to survive, child required ongoing medical attention, blood transfusions, and iron removal (chelation therapy). This form is also known as Cooley anemia.
Thalassemia major (Cooley anemia)
Patho:
-defective hemoglobin synthesis = beta globulin chains are reduced or absent
-RBCs become rigid and fragile and easily hemolyzed
-this leads to severe hemolytic anemia
-RBCs have decreased oxygen-carrying capacity → chronic hypoxia
-body increases RBC production to compensate → massive bone marrow hyperplasia and thinning of bony cortex
Complications:
-growth retardation, pathologic fractures, skeletal deformities, osteoporosis
-iron overload (hemosiderosis) caused by rapid RBC hemolysis and ↓ hemoglobin production
iron skin deposits in tissues leads to bronze skin pigmentation, bony changes, and cardiac dysfunction
-splenomegaly, liver disease, gallbladder disease, endocrine abnormalities, leg ulcers
-if untreated can lead to death before 5 years of age
Labs/ Diagnostics:
-H/H ↓, variation in size and shape of RBCs shown on peripheral blood smear
-bilirubin levels ↑, iron levels ↑
Management:
-monitoring Hgb and Hct and transfusing PRBCs at regular intervals
-monitoring iron levels
-iron chelation therapy to remove excess iron
-ensure families understand adhering to blood transfusion schedule and chelation therapy schedule is essential to child’s survival
Epistaxis (nosebleeds)
-short, isolated occurrences of epistaxis are common in childhood
-mucous membranes in the nose are very vascular and fragile, making nose bleeds common
-rarely an emergency
-cause of great stress to parents and child
Risks and Causes:
-trauma (picking or rubbing of nose, hit in the face, blunt injury)
-low humidity, allergic rhinitis, upper respiratory infection, foreign body
-medications
-rarely, but may be a result of an underlying disease (von willebrand, hemophilia, ITP, leukemia)
Nursing Care:
-have the child sit upright w/ the head slightly tilted forward to prevent aspiration of blood
-apply pressure to the lower nose w/ the thumb and forefinger for at least 10 min
-if needed, pack cotton or tissue into the side of the nose that is bleeding
-encourage the child to breathe through the mouth while nose is actively bleeding
-apply ice across the bridge of the nose
Medical treatment:
-nasal packing: gauze, special nasal sponges of foam, or an inflatable latex balloon is inserted into your nose to create pressure at the side of the bleed
-Cauterization: this procedure involves applying a chemical substance (silver nitrate) or heat energy (electrocautery) to seal the bleeding blood vessel
a local anesthetic is sprayed in the nostril first to numb the inside
Childhood cancer
-caring for child who has cancer requires compassion and competency
-nurse should provide individualized care and support to the child and the child’s family
-pediatric cancers have a low rate of incidence compared to cancer in adults. However, some neoplasms occur predominantly in children s/a Wilm’s tumor/ nephrobalstoma and neuroblastomas
Neoplastic disorders
-leading cause of death from disease in children past infancy
-occur much less often however than in the adult population
-almost half of all childhood cancers involve blood or blood forming organs: Leukemia and Lymphoma
Radiation
-radiation is dose-calculated and usually delivered in divided treatments
-radiation affects rapidly growing cells in the body
-cells that normally have a fast turnover can be affected in addition to cancer cells
-do not wash off marks on skin that outline the targeted areas
-avoid use of soaps, creams, lotions, and powders unless they are prescribed
-wear loose cotton clothing
-keep the areas protected from the sun by wearing a hat and long-sleeved shirts
-seek medical care for blisters, weeping, and red/tender skin
Chemotherapy
-use of drugs to destroy cancer cells
-this type of cancer treatment works by keeping cancer cells from growing, dividing, and making more cells
-provide an antiemetic prior to administration of chemo
-allow the child several food choices, including their favorite foods
-assess the mouth for mucosal ulcerations
-offer cool fluids to prevent dehydration and soothe sore mucous membranes
-some of the treatments can impair the child’s growth and development
-unfortunately, chemotherapeutic medications disrupt the cell cycle of not only cancer cells but also normal rapidly dividing cells
-this results in a significant number of adverse effects
-the cells most likely to be affected by chemotherapy are those in the bone marrow, the digestive tract (especially the mouth), the reproductive system, and hair follicles
Adverse effects:
-skin breakdown
-pain
-constipation, anorexia
-mucous membranes ulcers (mucositis)
-bone marrow suppression
-alopecia
Leukemia
-classified by the type of WBCs that becomes neoplastic and is commonly divided into two groups: acute lymphoblastic leukemia (ALL) and acute myeloid AML)
-ALL is most common childhood malignancy
-increase in the number of immature WBC that infiltrate bine marrow, organs, and tissue
-bone marrow infiltration causes crowding of cells that would normally produce RBCs, platelets, and mature WBC
-metastasis to spleen, liver, lymph nodes, CNS, testes, ovaries, GI tract, kidneys, and lungs
ALL:
-most common form of cancer in children. Overall cure rate is over 70%
AML:
-generally, the higher the WBC count at diagnosis, the worse the prognosis. Common ALL has BEST prognosis
-commonly occurs in children btwn 2-10yrs (85%)
-it is more common in white children than in other races
-more common in males
-higher incidence in pts w/ Trisomy 21
Leukemia Patho
-unrestricted proliferation of immature WBCs in the blood-forming tissues of the body
-cellular destruction takes place by infiltration and subsequent competition for metabolic elements
-although leukemia is an overproduction of WBCs, it often causes low leukocyte counts
-liver and spleen are the two most affected organs
-in ALL, abnormal lymphoblasts are fragile and immature, lacking the infection-fighting capabilities of the normal WBC
-the growth of lymphoblasts is excessive and the abnormal cells replace the normal cells in the bone marrow causing MASSIVE metabolic needs
Leukemia manifestations/ diagnostics
Manifestations:
-anemia = the bone marrow becomes unable to maintain normal levels of RBCs, WBCs, and platelets, so anemia, neutropenia, and thrombocytopenia result
-infection (neutropenia)
-low grade fever
-pallor
-increased bruising and petechiae (anemia)
-listlessness
-enlarged liver, lymph nodes, and joints
-abdominal, leg, and joint pain
-headache, unsteady gait, pain,
-enlarged kidneys and testicles
-manifestations of increased intracranial pressure
Diagnostics/ Labs:
-anemia (low blood count)
-thrombocytopenia (low platelets)
-neutropenia (low neutrophils)
-leukemic blasts (immature WBCs)
-blood smear (Immature WBC)
-lumbar puncture to evaluate CNS involvement
changes in behavior or personality, headache, irritability, dizziness, persistent N/V, seizures, gait changes, lethargy, or altered LOC may include CNS infiltration
-bone marrow aspiration and biopsy (most diagnostic)
Leukemia Management
-prepare child and parents for procedures: LP, bone marrows, surgery
-education and treatment side effects: N/V, alopecia, anorexia, moon face, neuropathy, mucosal ulcers
-relieve pain
-prevent complication of myelosuppression: infection, hemorrhage, anemia
-therapeutic management of the child w/ ALL focuses on giving chemotherapy to eradicate the leukemic cells and restore normal bone marrow function
-nursing care of children w/ ALL focuses on managing disease complications s/a infection, pain, anemia, bleeding, and the many adverse effects related to treatment
-many children require blood product transfusion for the treatment of severe anemia or low platelet levels w/ active bleeding
-chemo, radiation, stem cell transplant
Lymphomas
tumors of the lymph tissue (lymph nodes, thymus, spleen), account for about 10%-15% of cases of childhood cancer
-may be divided into two categories = Hodgkin disease and non-Hodgkin lymphoma which includes more than a dozen types
-Hodgkin disease tends to affect lymph nodes located closer to the body’s surface, s/a those in the cervical, axillary, and inguinal areas, whereas NHL tends to affects lymph nodes located more deeply inside the body
-3rd most common group of malignancies in children
-Hodgkin Lymphoma: more prevalent in children 15-19 years of age
-Non Hodgkin Lymphoma: more prevalent among children less than 14 years of age
Hodgkin Lymphoma
-neoplastic disease originating in the lymph system
-primary involvement is the lymph nodes = palpate for enlarged lymph nodes; they may feel rubbery and tend to occur in clusters (most common sites are cervical and supraclavicular)
-often metastasizes to the spleen, liver, bone marrow, lungs, and other tissue
-better survival rate!
-malignant B lymphocytes grow in the lymph tissue, usually starting in one general area of lymph nodes
-presence of REED-STERNBERG cells = differentiates Hodgkin disease from other lymphomas
-as the cells multiply, the lymph nodes enlarge, compressing nearby structures, destroying normal cells, and invading other tissues
-cause of Hodgkin disease is still being researched, but there appears to be a link to Epstein-Barr virus infection
-Hodgkin disease is rare in children younger than 5 years of age and is most common in adolescents and young adults; in children 14yrs and younger, it is more common in boys than girls
Classification and Diagnosis:
-enlarged cervical or supraclavicular lymphadenopathy, cough, abdominal pain, anorexia, discomfort
-Ann Arbor Staging System
classification A: asymptomatic
classification B: temp greater than 38 degrees celsius, night sweats, unexplained weight loss of 10% or more over the last 6 months
-lymph node biopsy
presence of REED STERNBERG CELLS
-bone marrow aspiration and biopsy
Management:
-radiation therapy
-chemotherapy (alone or w/ radiation) dependent on severity of disease
-good long term prognosis
Non-hodgkin’s lymphoma
-occurs more frequently in children
-disease is usually diffuse rather than nodular
-cell type undifferentiated or poorly differentiated
-dissemination: occurs early, often, and rapidly. Children w/ NHL are usually symptomatic for only a few days or a few weeks before diagnosis bc the disease progresses so quickly
-mediastinal involvement and invasion of meninges (cough, dyspnea, orthopnea, facial edema, or venous engorgement may indicate mediastinal disease in the child w/ NHL. This is an emergency requiring rapid treatment)
-NHL tends to affects lymph nodes located more deeply w/i the body. NHL spreads by the bloodstream and in children is a rapidly proliferating, aggressive malignancy that is very responsive to treatment
Wilm’s Tumor/ Nephroblastoma
-malignancy of kidney or abdomen
-typically, unilateral
-common in ages 2-5
-metastasis is rare
Manifestations:
-mass is usually found by caregivers during routine bathing/dressing of their child
-painless, firm, nontender abdominal swelling or mass
-fatigue, malaise
-weight loss, fever, hematuria, HTN
-manifestations of metastasis include dyspnea, cough, SOB, and chest pain
-IF A TUMOR IS SUSPECTED DO NOT PALPATE OR INFLICT TRAUMA TO THE ABDOMEN
-depending on stage and size of tumor treatment will include surgical removal, chemo, and/or radiation
Labs/ Diagnostics:
-BUN, creatinine
-CBC, urinalysis
-abdominal ultrasonography
-abdominal and chest CT scan
-inferior venacavogram (rule out involvement w/ the vena cava)
-bone marrow aspiration (rule out metastasis)
Neuroblastoma
-malignancy of the adrenal gland, retroperitoneal area, head, neck, pelvis, or chest
-toddler years w/ 95% prior to age 10
-50% of all cases have metastasized before diagnosis
Assessment:
-palpable abdominal mass
-weight loss, constipation, anorexia
-diaphoresis, HTN
-manifestations of metastasis include jaundice and dark pigmented nodules on the extremities, dyspnea, edema of face and neck, ill appearance, bone pain
Labs and Diagnostics:
-CBC and coagulation studies
-urine catecholamines
-skeletal survey
-skull, chest, neck, abdominal CT
-biopsy of tumor
Management:
-surgical removal of tumor
-chemotherapy/radiation for residual disease, prevent reoccurrence
-infection prevention
Retinoblastoma
-congenital, highly malignant tumor that arises from embryonic retinal cells
-unilateral or bilateral
-may metastasize to the brain, opposite eye, lymp nodes, bone marrow, and liver
-it may be noticed if a pupil appears white when light is shined into the eye, sometimes w/ flash photography. Eyes may appears to be looking in different directions
-radiation, chemotherapy, laser therapy, cryotherpay
-for severe disease, removal of eye is necessary
Osteosarcoma/ Ewing’s
bone cancer, usually of the femur
-osteosarcoma usually peaks during adolescence and overlaps w/ growth spurts
-ewings sarcoma occurs prior to 20yo and is more common in caucasians
Manifestations:
-nonspecific bone pain that is often mistaken for an injury or growing pains
-weakness, swelling, decreased movement or limping
-anemia, fever, or unexplained weight loss
-inability to lift a heavy object
Labs/ Diagnostics:
-CBC
-Xrays, CT of primary site, bone biopsy, CT of the chest and bone scans to evaluate metastasis
Management:
-provide emotional support
-explain possibility of sterilization it indicated
-administer chemotherapy as indicated/ prescribed
control N/V w/ antiemetics prior to treatment and manage adverse effects of treatment
provide teaching about observing for manifestations of infection, maintaining good hygiene, and bleeding precautions
-limb salvage procedures may be performed = includes course of chemo to shrink tumor and then total bone and joint replacement after the tumor and affected bone are removed
-child may receive chemo preoperatively and postoperatively if limb amputation must occur
fitting of temporary prosthesis may occur immediately after surgery
-encourage joining support groups, make referrals to mental health counseling, PT, and OT
Rhabdomyosarcoma
-cancer of skeletal muscle
-most common soft tissue cancer in children; most common in children under 10yo
Manifestations:
-can cause pain in local areas related to compression by the tumor
-physical findings are based on affected area
-CNS: impaired cranial nerve function, stiffness in neck, decreased HR and respiratory rate
-Orbit: unilateral proptosis, ecchymosis of conjunctiva, strabismus
-Nasopharynx: stuffy nose, nasal obstruction, epistaxis, palpable neck nodes, visible mass (late)
-Paranasal sinuses: pain, nasal obstruction, discharge, sinusitis, swelling
-Middle Ear: chronic otitis media, pain, sanguinopurulent discharge, facial paralysis
-retroperitoneal area: usually no findings, abdominal mass, pain, intestinal or genitourinary obstruction
-perineum: visible superficial mass, bowel or bladder obstruction
-extremity: pain, palpable fixed mass, lymph node enlargement
Diagnostics:
-CT scans or MRI of the primary site
-biopsy of tumor if possible
-LP to evaluate metastasis
Management:
-Chemo, localized radiation, surgical biopsy
-Complications include skin desquamation and myelosuppression
HIV and AIDS
-HIV is a viral infection in which the virus infects a specific subset of T-cells and CD4 T cells causing immune dysfunction which leads to organ dysfunction and a variety of opportunistic illnesses in a weakened host
the principal modes of HIV transmission to the pediatric population are mother-to child transmission (perinatally or breast milk) and adolescent risky behaviors s/a sexual activity and IV drug use
-In the U.S. the number of AIDS new cases and deaths in children below 13 years of age is sharply decreasing yet increasing in adolescence
HIV
-children acquire HIV either vertically or horizontally
-vertical transmission refers to perinatal (in utero or during birth) transmission or via breast milk. Vertical transmission may also be referred to as mother to child transmission
-Horizontal transmission refers to transmission via nonsterile needles (as in IV drug use or tattooing) or via intimate sexual contact
Pediatric Manifestations:
-lymphadenopathy
-hepatosplenomegaly
-recurrent URI, sinusitis, or otitis media
-oral candidiasis
-chronic or recurrent diarrhea
-failure to thrive
-developmental delay
-parotitis
Vaccine association:
-DO NOT administer live vaccines to the immunocompromised child w/o the expressed consent of the infectious disease or immunology specialist
-immunosuppression is a contraindication to vaccination w/ live vaccines
Defining conditions for AIDS
-pneumocystis carinii pneumonia = major complication
-lymphoid intestinal pneumonitis
-recurrent bacterial infections
-wasting syndrome
-Kaposi sarcoma
-candidal esophagitis
-HIV encephalopathy
-CMV disease
-mycobacterium avium-intracellulare complex infection
-pulmonary cadidiasis
-HSV
-cryptosporidiosis
HIV and AIDS management
-balanced diet, high in calories and protein
-TPN if rx
-good oral care
-non pharm methods of pain relief
-assess/treat pain
-excellent hand hygiene
-prevent infection
-administer meds
antiretroviral meds
antibiotics
IVIG
Education:
-lifelong illness and treatment/meds
-identify situations requiring medical care: headache, fever, lethargy, warmth, tenderness, redness at joints and neck stiffness
-avoid high-risk behaviors
-identify stressors and make referrals in community
-safe practices using needles/syringes
-discuss w/ adolescents’ various routes of transmission
Pediatric respiratory differences
-since respiratory illnesses account for most pediatric admissions to general hospitals, nurses caring for children need to have expert intervention skills in this area
-children have significantly higher metabolic rates than adults. Their resting RR are faster and their demand for O2 is higher. Adult O2 consumption is 3-4L/min while infants is 6-8L/min
-in any situation of respiratory distress, infants and children will develop hypoxemia more rapidly than adults
-early intervention is critical
-most pediatric cardiopulmonary arrests begin as respiratory failure or respiratory arrest
Pediatric upper airway
-the upper respiratory tract includes nose, pharynx, adenoids, tonsils, epiglottis, larynx, and trachea
pharynx, larynx, and trachea are smaller w/ less capacity than adults
-child’s airway is shorter and narrower
-infant’s airway is 4mm wide (adult is 20mm)
-trachea grows in length rather than diameter in first 5 years of life
-cartilage that supports the trachea is more flexible
-newborns are obligatory nose breathers
Pediatric lower airway
-25mm alveoli at birth and not fully developed
-alveoli do not begin to increase in size and complexity until 8 yrs
-it is not until 5 months of age that the smooth muscles lining the bronchioles will react to irritants w/ bronchospasm
-children under 6 years of age use the diaphragm to breathe bc their intercostal muscles are weak and immature
Airway resistance:
-when edema, mucus, or bronchospasm is present the capacity for air passage is greatly diminished. A small reduction in the diameter of a child’s airway (from the presence of edema or mucus) will result in an exponential increase in resistance to airflow
-the child’s airway is highly compliant, making it quite susceptible to dynamic collapse in the presence of airway obstruction
Poiseuille’s law:
-if radius is halved, resistance increases 16 fold, resistance increases 16x in an infant
Pulse Oximetry
-noninvasive measurement of O2 sat of arterial blood
-appropriate probe site must have good circulation. Can be placed on fingertip, toe, earlobe, or around the foot w/ a clip or band
-LED light should be on top of the nail if using toes or fingers
-especially on neonates and small children site should be inspected/rotated per facility policy (4-8hrs) to prevent pressure necrosis in infants who have disrupted skin integrity or poor perfusion
-Fowlers or semi-fowlers facilitates respiration = use when possible to maximize ventilation
Respiratory infections of upper airway
-the major passages and structures of the upper respiratory tract include the nose or nostrils, nasal cavity, mouth, throat (pharynx) and voice box (larynx)
-respiratory system is lined w/ mucus membranes that secrete mucus
Causative Viral agents:
-RSV
-nonpolio enterovirus, A, B, C, D, coxsackie viruses, adenoviruses, parainfluenza, human meta-pneumoviruses
Causative Bacterial Agents:
-group A beta-hemolytic streptococci
-staphylococci
-haemophilus influenza type B, chlamydia trachomatis, mycoplasma organisms, pneumococci
Manifestations:
-fever, loss of appetite, vomiting, diarrhea, abdominal pain, nasal congestion, nasal discharge, cough, sore throat, adventitious breath sounds
-Meningismus = a constellation of signs and symptoms (headache, neck stiffness) suggestive of meningitis, characterized by meningeal irritation w/o objective findings.
more common in young pts w/ systemic infections (flu, pneumonia)
URI management
-ease respiratory effort
-promote rest and comfort
-prevent spread of infection
-reduce temp if uncomfortable
a fever is a body’s defense mechanism to fight bacteria and viruses which usually like to live w/i certain “normal” temperature ranges. Elevated temps help fight infection so treat if the child is uncomfortable
-promote hydration
-provide support and reassurance
Education:
-use a cool-air vaporizer to provide humidity
-rest during febrile illness
-maintain adequate fluid intake. Infants may be given commercially prepared oral rehydration solutions, and older children may be given sports drinks
-administer meds using accurate dosages and appropriate time intervals
-develop strategies to decrease the spread of infection.
strategies include performing good hygiene; covering the nose and mouth w/ tissues when sneezing and coughing; properly disposing of tissues; not sharing cups, eating utensils, and towels; and keeping infected children from contact w/ children who are well
-seek further medical attention for the child if manifestations worsen or respiratory distress occurs
Pediatric factors that affects resistance to organisms
-immune system
-day care
-malnutrition
-anemia
-preterm birth
-allergies
-secondhand smoke exposure
-cardiac anomalies
-cystic fibrosis
-healthy children tolerate short-term decreases in hemoglobin levels and O2 sat w/o serious consequences, but ill children, especially those w/ anemia (having decreased oxygen carrying capacity) lack the necessary reserve capacity to preserve tissue oxygenation
Nasopharyngitis (common cold)
-self limiting virus that can persist 10-14 days
-caused by numerous viruses
RSV, rhinovirus, adenovirus, influenza, parainfluenza viruses
-fever will be managed w/ age and condition
-managed at home
-treatment is supportive (antipyretics, nasal spray, cool mist, over the counter cold meds)
-antihistamines are largely ineffective and not recommended (drying)
-parenteral reassurance = usually want some type of medicine (Antibiotics contraindicated)
-the common cold is referred to as a viral URI. Colds occur more frequently in the winter. They affect children of all ages and have a higher incidence among children who attend daycare and school-age children.
Tonsilitis
-tonsils are lymph type organs that filter organisms from the resp and GI tract
-infants have underdeveloped tonsils thus rarely get tonsilitis or strep
-often occurs w/ pharyngitis = viral or bacterial but most are viral, immature immune system (younger children)
-kissing tonsils (4+)
when tonsils touch at the midline, the airway may become obstructed. Also, if the adenoids are enlarged, the posterior nares become obstructed
the child may breathe through the mouth or may snore
-must distinguish btwn GABHS and viral to prevent unnecessary antibiotic treatment
Acute streptococcal pharyngitis (Strep throat)
-group A beta hemolytic infection (GABHS)
-risk for rheumatic fever especially if not treated
-sudden onset of sore throat, headache, fever, sometimes abdominal pain, vomiting, diarrhea, tongue red and swollen, sandpaper rash to the trunk (scarlitina), cervical lymphadenopathy. Sometimes tonsils can have a white patchy “exudate” on them - “strep breath”
-diagnosed by rapid strep and/or throat culture rapid antigen testing to determine GABHS infection
-antipyretics for pain and fever
-oral penicillin is mainstay of treatment administered for at least 10 days. Erythromycin for penicillin allergy. IM penicillin is not recommended unless noncompliance is suspected (painful)
-a bacterial sore throat most often occurs w/o nasal symptoms
inflammation of the throat mucosa (pharynx) is referred to a pharyngitis
a sore throat may accompany nasal congestion and is often viral in nature
group A streptococci account for 15% to 30% of cases, w/ the remainder being caused by other viruses or bacteria
Complications:
-peritonsillar or retropharyngeal abscess
-retropharyngeal abscess may progress to the point of airway obstruction, hence requiring careful evaluation and appropriate treatment
-peritonsillar abscess may be noted by asymmetric swelling of the tonsils, shifting of the uvula to one side, and palatal edema
-additional complications include rheumatic fever
Rheumatic fever
-condition that can inflame or make the heart, joints, brain, and skin swell
-rheumatic fever is an inflammatory disease that can develop when strep throat or scarlet fever isn’t properly treated
-rheumatic fever is thought to be an immune response to an earlier infection. As the body’s defense system, the immune system is important in fighting off infections
Symptoms:
-fever, painful and tender joints = most often in the knees, ankles, elbows, and wrists
-pain in one joint that migrates to another joint,
-red, hot or swollen joints
-chest pain, fatigue or slightly raised painless rash w/ a ragged edge
-heart murmur, jerky uncontrollable body movements (sydenham chorea) = most often in the hands, feet, and face
-outbursts of unusual behavior, s/a crying or inappropriate laughing that accompanies sydenham chorea
Strep management
-considered contagious from onset of symptoms until 24 hrs after 1st dose of antibiotics
-discard toothbrush after a minimum of 3 doses of antibiotics
-saline gargles
-orthodontic appliances, pacifiers must be sterilized
Tonsillectomy
Absolute:
-peritonsillar abscess
-airway obstruction
-tonsils requiring pathology
Relative:
-three or more tonsil infections per year
-persistent foul breath/taste caused by chronic tonsilitis
-chronic tonsilitis
Contraindications:
-cleft palate = tonsils help minimize air escape during speech
-acute infections at the time of surgery = risk of bleeding
-uncontrolled systemic diseases
-poor anesthetic risk
Post-op care:
-WATCH FOR SIGNS OF BLEEDING!!
bleeding can occur up to 10 days post op (especially when those scabs come off) (frequent swallowing, tachycardia, pallor, hypotension, clearing throat frequently)
citrus juice and brown or red fluids should be avoided; the acid in citrus juice may irritate the throat, and red or brown fluids may be confused w/ blood if vomiting occurs
-pain reduction w/ combination opioids to increase comfort to allow for drinking
-soft/liquid diet
-monitor for difficulty breathing related to oral secretions, edema and or bleeding
-discourage from coughing, clearing the throat, blowing the nose
-administer liquid analgesics or tetracaine lollipops as prescribed
-cool water, crushed ice, popsicles (non citrus), fruit juices
-milk, ice cream, and pudding, are NOT recommended bc milk products coat the mouth and throat, causing the child to clear their throat, and increase the risk of bleeding
Influenza
-caused by orthomyxoviruses
A, B, C, D (within each subgroup are mutations of proteins)
-peculiar affinity for the epithelial cells of the respiratory tract
-destroys the epithelium while causing edema
-alveoli also become distended
-occurs primarily in winter months. Children w/ chronic heart or lung conditions, diabetes, chronic renal disease, or immune deficiency are at higher risk for more severe influenza compared to other children
Manifestations:
-mild, moderate, or severe
-dry throat and nasal mucosa, dry cough
-flushed face, loss of energy, sudden onset of fever and chills
-poor appetite, stomach pain, vomiting, headaches, myalgia (body aches), eye pain (photophobia, tearing)
-severe cases can cause viral pneumonia or seondary bacterial pneumonia
bacterial infections of respiratory system commonly occur as complications of flu, severe pneumococcal pneumonia in particular. Otitis media and myositis also
acute myositis is particular to children. A sudden onset of severe pain and tenderness in both calves causes the child to refuse to walk
-lab testing is a throat or nasal swab
Flu management
-promote increased fluid intake
-rest
-uncomplicated flu only requires symptomatic management
-teach family s/sx of respiratory distress
-tylenol or motrin
-oseltamivir (Tamiflu) and others decreases flu symptoms if given w/- 24-48hrs from onset of symptoms
does not “cure” flu
helpful for both types A and B
a lot of GI side effects for children
-droplet and contact precautions
-educate and encourage flu vaccinations annually
Vaccinations:
-inactivated virus recommended annually for children 6 months +
-children 6 months - 8 years require 2 doses wen it is their first season being vaccinated
-live virus contraindicated in those w/ hx of Guilian-Barre, heart, or lung disease, diabetes, kidney failure, immunocompromised, respiratory conditions, or severe allergies to chicken eggs
-some flu vaccines are attenuated = live vaccine that has been weakened (using only a single gene) or the virus is inactivated yet produces an immune response from the host
-offer the best defense against flu and it potentially serious consequences and can also reduce the spread of flu to others
Otitis media
-most prevalent disease of early childhood: before 24 months
-most bacterial cases of OM are preceded by viral URI
-passive smoking increases risk
-SES and extent of exposure to other children are two greatest risk factors
Management:
-AOM is the most common reasons for antibiotic use in the ambulatory setting
-a great deal of resistant OM
-focus on pain management for mild cases
-use of steroids, decongestants, and antihistamines for AOM not recommended
-tympanostomy tube placement and adenoidectomy for chronic recurring OM
-can cause scarring of the tympanic membrane w/ recurrent infections which can lead to hearing loss. Can be very painful
Infectious mononucleosis (BV)
-spread through the saliva “kissing disease”
-incubation is 30-50 days
-acute, self-limiting disease common amongst adolescents
-the spot test (monospot) is most commonly used diagnostic test = in early stages may be unreliable. An EBV titer is most reliable at any point
-check the spleen!
Complications:
-splenic rupture
-Guilliain-Barre syndrome
-aseptic meningitis
Manifestations:
-headache,
-fatigue, sore throat, hepatosplenomegaly
-chills/fever, loss of appetite, puffy eyes, sore throat, cervical lymphadenopathy, exudative pharyngitis, rash
Management:
-rest!
-Tylenol and Motrin
-warm salt water
-check CBC
-no contact sports until spleen has returned to baseline
-during the acute phase, if tonsillar or pharyngeal edema threatens to obstruct the airway, then corticosteroids may be given to decrease the inflammation
Croup (laryngotracheobronchitis)
-general term applied to symptoms of hoarsness, resonant cough “barking” “croupy”, varying degrees of inspiratory stridor
-can affect the larynx, trachea, bronchi
-viruses are most common cause
RSV, parainfluenza, influenza types A&B
-usually worse at night
-children btwn 3 months and 3 yrs of age are the most frequently affected
-the inflammation and edema obstruct the airway, resulting in symptoms. Mucus production also occurs, further contributing to obstruction of the airway. Narrowing of the subglottic area of the trachea results in audible inspiratory stridor
Management:
-self-limiting
-supportive therapy (humidified cool air)
-oral steroids
-encourage fluids. Popsicles
-IM steroids for severe cases
-Racemic Epi for severe cases
-most severe cases are hospitalized = IV fluids, O2 therapy, aerosolized treatments, etc
-explain to parents that the effects of racemic epinephrine last about 2 hrs and the child must be observed closely as occasionally a child will worsen again, requiring another aerosol
Epiglottitis
MEDIAL EMERGENCY!!
-obstructive inflammatory process
-obstruction is supraglottic
-responsible organism is typically H. influenza (vaccines (Hib) have assisted w/ the decline of this disease)
-can be either bacterial or viral in origin
-abrupt onset that can progress quickly
-mainly seen in children ages 2-5
Assessment:
-very sore throat
-tripod positioning = sitting up, chin thrust out, mouth open, tongue out, drooling to increase free movement in the pharynx
-drooling, difficulty swallowing
-inspiratory stridor
-high fever
-hoarseness, mild hypoxia, respiratory distress, lateral neck x-ray is diagnostic
Management:
-prevention of progressive respiratory obstruction
-protect the airway = can be edematous
-do not agitate
NPO, IV or IM steroids, cool/humidified oxygen, antibiotics, droplet precautions/ isolation
-be prepared for intubation or tracheostomy
Epiglottitis nursing alert
-three clinical observations
abscence of spontaneous cough
drooling
agitation
-when epiglottis is suspected, the nurse SHOULD NOT attempt to visualize the epiglottis w/ a tongue depressor or take a throat culture, but should seek immediate, emergent medical attention
-throat inspection should only be done when a doctor that is comfortable w/ endotracheal intubation is present and ready
-allow the child to assume a position of comfort. Do not place child in a supine position, as airway occlusion may occur. Provide 100% oxygen in the least invasive manner that is acceptable to the child
-if the child w/ epiglottitis experiences complete airway occlusion, an emergency tracheostomy may be necessary
Infections of lower airway
-the bronchi and bronchioles of infants and chidren are narrower in diameter than the adult’s placing them at increased risk for lower airway obstruction
-lower airway obstruction during exhalation often results from broncholitis or asthma or is caused by foreign body aspiration into the lower airway
-in any situation of respiratory distress, infants and children will develop hypoxemia more rapidly than adults
bronchitis
-inflammation of the large airways (trachea and bronchi)
-associated w/ URIs
-viral agents are primary cause
-mild, self-limiting disease
-illness might begin w/ a mild URI. Fever develops followed by a dry, hacking cough that might become productive in older children. The cough might wake the child at night. Auscultation of the lungs might reveal coarse rales. Respirations remain unlabored
Assessment:
-persistent, dry, hacking cough (worse at night) that can last for weeks
-productive w/i 2-3 days
-fever
-wheezing
-rhonci
-resolves in 5-10 days
Management:
-symptomatic treatment
-analgesics, antipyretics, and provide increased humidified air (cool mist vaporizer)
-recovery is typically 5-10 days
-adolescents w/ bronchitis should be screened for tobacco and marijuana use
Bronchiolitis
-most common infectious disease of the lower airway
-maximum obstructive impact and bronchiolar level
-children from birth -2 yrs; rarely after 2 yrs
-the severity of disease is related inversely to the age of the child
-usually viral = most often RSV but also adenovirus, parainfluenza human metapneumovirus, and Mycoplasma pneumonia
-RSV infection causes necrosis of the respiratory epithelium of the small airways plugging of the lumens w/ mucus and exudate. The small airways become variably obstructed; this allows adequate inspiratory volume but prevents full expiration. This leads to hyperinflation and atelectasis
Assessment:
-labored respirations, poor feeding, pharyngitis
-possible eye or ear infection
-cough can last weeks, retractions, tachypnea, listlessness, apneic spells, poor air exchange, poor breath sounds, cyanosis
-increased nasal drainage, wheezing, intermittent fever
Management:
-supplemental O2 for O2 sat < 93%
-cool, humidified air
-meds as prescribed
-bronchodilators not recommended
-nasopharynx suctioning
-maintain airway
-nasal pharyngeal washings: positive identification of RSV can be made via enzyme-linked immunosorbent assay (ELISA) or immunofluorescent antibody (IFA) testing
-adequate fluid intake as tolerated otherwise IV fluid until acute phase has passed
-maintaining adequate oxygenation
-most infants are managed at home w/ close observations and adequate hydration
-hospitalization is required for children w/ more severe disease
infant w/ tachypnea, significant retractions, poor oral intake, or lethargy can deteriorate quickly, to the point of requiring ventilatory support, and thus warrants hospital admission
RSV
-spreads rapidly… isolate infants
-most common cause of hospitalization in children less than 1 year of age
-severe RSV is a significant risk factor for later development asthma
-passed through secretions
-prevention: synagis which is a monthly injection given at the onset of season (usually october) until end of season (April) for preemie infants less than 6 months
-recent FDA approval of a vaccine
-wheezing that is unresponsive to bronchodilators
-severe cases will often lead to pneumonia
-the infant might appear air-hungry, exhibiting various degrees of cyanosis and respiratory distress, including tachypnea, retractions, accessory muscle use, grunting, and periods of apnea
-cough and audible wheeze might be heard. The infant might appear listless and uninterested in feeding, surroundings, or parents
-infants and young children w/ RSV tend to have copious secretions
-inverse severity relationship to age
Pneumonia
-primary disease or complication
-can be viral or bacterial
-causative agent is either inhaled directly into the lungs or picked up from the blood stream
-lobae pneumonia = affects entire lobe
-pneumonitis = inflammation of the lung that is not caused by a pathogen, but it is still a type of pneumonia
-vaccines are out primary prevention against bacterial pneumonia (PCV-13) ages 6wks to 71 months
-children w/ bacterial pneumonia are more apt to present w/ toxic appearance
-CXR: varies according to child age and causative agent. In infants and young children, bilateral air trapping and perihilar infiltrates (collection of inflammatory cells, cellular debris, and foreign organisms) are the most common findings
Manifestations:
-fever typically higher than 101.4
-chills, cough, tachypnea/dyspnea, crackles or rhonchi, dullness to percussion
-nasal flaring, chest pain (shallow breathing), pallor to cyanosis, irritability, lethargic
Aspiration pneumonia
-this is a particular concern for the child who has difficulty swallowing due to paralysis, weakness, debility, congenital anomalies, neurologic conditions (CP), absent cough reflex, or the child who is forced-fed when crying or breathing rapidly
-food, secretions, inert materials, volatile compounds, or liquids enter the lungs and cause inflammation and chemical pneumonitis
-S/Sx are similar to those of infectious pneumonia
-treatment is the same as infectious pneumonia
-major focus on PREVENTION
pneumonia management
-supportive
-frequent repositioning to avoid pooling of secretions and creating new pockets of infection
-supplemental oxygen if needed
-fluids
-antibiotics/ antifungals (unless viral)
-chest physiotherapy
cupping of hand, one layer of clothing, various positioning
-nebulized bronchodilators
-hospitalization for children less than 1 month, those w/ chronic illness, severe manifestations of illness or immunocompromised
-pneumonia is often perceived by the public as a bacterial infection, so most parents will need an explanation related to treatment of viral infections
Pertussis
-known as whooping cough
-incubation period 5-21 days
-caused by Bordetella pertussis
-spring and summer months
-in the past, primarily occurred in children less than 4 years
-in recent years, a large increase in children 10 years and older, making us question the length of vaccine
-now recommend Tdap (booster) at age 11
-highly contagious and life threatening to infants
-children, and infants especially, may appear very ill, vomit following cough fits, and appear exhausted
-infants younger than 12 months of age may not have the strength to have a whoop (cough)
-in infants, cough may be minimal or absent, w/ the main symptom being temporary pauses in breathing
Manifestations:
-dyspnea, apnea (infants)
-persistent, overwhelming cough followed by a whooping noise
-common cold manifestations = runny nose/ congestion, sneezing
-severe cough starts in 1-2 weeks (coughing fits) whooping sound on inspiration
-pneumonia, seizures, encephalopathy, symptoms last 6-10 weeks and longer
-eyes may appear bloodshot due to excessive coughing
Pertussis management
-prevention = DTap vaccine schedule (5 injections) and Tdap booster at 10-11
-azithromycin, erythormycin, calrithromycin
-treatment typically does not shorten the span of the coughing spells
-humidity is helpful for coughing spells
-plenty of fluids
-oxygen
-hospitalization for infants or moderate to severe cases
-babies and children under age 7 will always get DTap. Children over age 7 and adults will always get the Tdap vaccine
The DTap vaccine contains full strength doses of all three vaccines. The Tdap vaccine provides a full strength dose of tetanus vaccine and smaller doses of diphtheria and whooping cough to maintain immunity
COVID
Manifestations:
-fever, cough, SOB, muscle aches, sore throat, headache, N/V, diarrhea, loss of taste or smell
Management:
-frequent hand hygiene
-maintain distance of at least 6ft from others when indicated
-wear facemask when indicated
-avoid touching face, mouth, and eyes
-obtain covid-19 vaccination according to current CDC guidelines
-the following vaccines are currently available for children >6 months old
-due to the novelty of this virus recommendations are constantly changing as science is obtained and updated
-administer antivirals or monoclonal antibodies as indicated
MIS (multisystem inflammatory disease)
-the covid infections inflames the blood vessels, digestive system, skin or eyes
-very rare but a very serious complication
-can happen up to 2 months post covid infection
-most often happens in children btwn 5yrs and 11yrs
-treatment consists of limiting swelling and inflammation = may include antibiotics, steroids, IVIG
Foreign body aspiration
-highest risk in ages 1-3 years
beads, small magnets, legos, small game pieces, marbles, etc.
hot dogs, peanuts, popcorn, gum, seeds, round candies
small lithium or cadmium batteries = esophageal or tracheal corrosion
-ALWAYS consider a foreign body as a cause of respiratory distress
-usually will have SUDDEN onset
Assessment:
-laryngotracheal = dyspnea, cough, stridor, hoarsness (decreased air entry)
-bronchial = paroxysmal coughing, wheezing, asymmetric breath sounds, dyspnea
-nasal = purulent, nasal drainage, foul odor
-small children are prone to insert things into ears or nose w/ a cold, runny nose or ear pain in efforts to make it feel better
Management:
-CXR
-endoscopy is the mainstay of diagnosis and management
-bronchoscopy for objects in the larynx and trachea
-when a child is choking, a blind finger sweep is NOT indicated = may lodge item further into airway
Tobacco exposure
-parental or family smoking is a cause of morbidity in children
-increased rate of emergency room visits and higher rate of SIDS
-secondhand smoke places the child at great risk for respiratory illness, reduced performance on pulmonary function tests, asthma
Asthma
-considered on of the leading causes of hospitalizations among children
-hyperresponsiveness to variety of factors, illness, cold, exercise, allergens
-body releases histamines and leukotreines which causes inflammation
-4 main categories = intermittent, mild persistent, moderate, and severe persistent
-chronic childhood inflammatory disorder of the airways that results in intermittent and reverisible airflow obstruction of the bronchi and bronchioles
-causes school absences and is considered one of the leading causes of hospitalizations among children
-the obstruction occurs bc the mast cells release histamines and leukotrienes which causes inflammation or airway hyperresponsiveness. The different types of asthma include intrinsic (no prior hx of asthma), extrensic (exposure to an allergen), exercise induced, and medication induced
Asthma triggers/ manifestations
Triggers:
-Allergens
indoor = mold, cockroach antigen, dust, dust mites, animal dander
outdoor = grasses, pollen, trees, shrubs, molds, spores, air pollution, weeds
irritants = tobacco smoke, wood smoke, odors, sprays
exercise
-cold air or changes in weather or temperature
-environmental change (ne home or school)
-infections/ viruses (colds)
-animals = dogs, cats, horses, rodents
-medications = aspirin, NSAIDs, antibiotics, beta blockers
-strong emotions = fear, anger, laughing, crying
-conditions = GERD, tracheoesophageal fistula
-foods = nuts, eggs, dairy products
Manifestations:
-hacking cough, SOB, decreased O2 sat
-prolonged expiratory phase, auduble wheezing, mucous production
-restlessness and apprehension, tripod position
-speech = short, panting breaths
-sitting retractions
-inaudible breath sounds or crackles = severe obstruction
-severity ranges from long periods of control w/ infrequent acute exacerbations in some children to the presence of persistent daily symptoms in others
Asthma diagnostics/ management
Diagnostics:
-pulmonary function tests (most accurate test for diagnoses and its severity)
-CXR, skin testing for allergens, peak flow
Management:
-goal is to maintain normal activity levels
-prevention of exacerbations
-EIA typically present in endurance sports. Obesity exacerbates it. Exercise is advantageous for those w/ asthma. Appropriate prophylactic treatment usually permits full participation
-Breathing exercises = strengthen the respiratory musculature and develop more efficient patterns of breathing. Help prevent overinflation during times of exacerbation and improve cough
-Hyper sensitization
when there is evidence of a relationship btwn asthma and unavoidable exposure
symptoms occur all year
symptom control is difficult w/ drug therapy due to multiple meds or compliance
Drug Therapy:
-step wise approach
-metered-dose inhaler (always use w/ a spacer)
-nebulizer
-diskus
-two categories (broncho dilation)
long term control meds (prevention) = inhaled corticosteroids, cromolyn sodium, long acting B2 agonists, methylxanthines, leukotriene modifiers
Quick relief meds (rescue) = B2 agonists, anticholinergics, systemic corticosteroids
Status athmaticus
MEDICAL EMERGENCY!!!
-considered prolonged severe asthma attack
-respiratory failure and death
-distended neck veins, wheezing, labored breathing, nasal flaring, tachycardia, tachypnea, hypoxia, risk for cardiac and respiratory arrest
-respiratory distress despite the vigorous use of sympathomimetics (albuterol and epinephrine)
-often w/ other conditions s/a pneumonia or viral illness
-administer O2, improve ventilation
-IV magnesium sulfate = potent muscle relaxant
-Heliox via nonrebreathing mask to decrease airway resistance and WOB
-Ketamine = smooth muscle relaxant decreases airway resistance
-in children who have more severe asthma, the NAEPP recommends the use of the PEFR to determine daily control
Cystic firbosis
-respiratory disorder that results from inheriting a mutated gene
-characterized by mucus glands that secrete an increase in the quantity of thick, tenacious mucus, which leads to mechanical obstruction of organs (pancreas, lungs, liver, small intestine, and reproductive system); an increase in organic and enzymatic constituents in the saliva; an increase in the sodium and chloride content of sweat; and autonomic nervous system abnormalities
-autosomal recessive trait (both parents carry trait for cystic fibrosis)
-multisystem involvement
increased viscocity of mucus gland secretion causing obstructions
elevation of sweat and chloride electrolytes
increase in enzymatic constituents of saliva
autonomic nervous system abnormalities
-at present all states include testing for cystic fibrosis as part of newborn screening
Cystic fibrosis manifestations
GI:
-meconium ileus
bowel obstruction that occurs when the meconium in your child’s intestine is even thicker and stickier than normal meconium, creating a blockage in a part of the small intestine called the ileum
-abdominal distention, vomiting, failure to pass stools, rapid dehydration
-large, bulky, lose, frothy stool
-voracious appetite (early), loss of appetite (late), weight loss, marked tissue wasting, growth failure, thin extremities, sallow skin, deficient in fat-soluble vitamins (A,D,E,K), anemia
-thick secretions block ducts and cause cystic dilation, degeneration, diffuse fibrosis
-prevents pancreatic enzymes from reaching duodenum
-impaired digestion/ absorption of fat, steatorrhea
steatorrhea is the excretions of abnormal quantities of fat w/ the feces owing to reduced absorption of fat by the intestine
-impaired digestion/absorption of protein, azotorrhea
azotorrhea is the excessive discharge of nitrogenous substances in the feces or urine
-sweat gland dysfunction
-failure to thrive
-increase weight loss despite increased appetite
Pulmonary:
-wheezing
-dry, nonproductive cough (early)
-increased dyspnea
-obstructive emphysema
-over inflated, barrel shaped chest
-cyanosis
-clubbing
-recurrent bronchitis and pneumonia
Reproductive:
-delayed puberty in girls
-thick cervical secretions possibly making it difficult for sperm motility
-sterility in boys
-tenacious seminal fluid/blocking of the vas deferens
-child w/ cystic fibrosis often has barrel chest
Respiratory progression (cystic fibrosis)
-progression follows chronic infection
-bronchial epithelium is destroyed
-infection spreads to peribronchial tissues, weakening the bronchial walls
-peribronchial fibrosis ensues
-O2/CO2 exchange decreases
-chronic hypoxia
-pulmonary HTN
-cor pulmonale = pulmonary heart disease, is the enlargement and failure of the right ventricle of the heart as a response to increased vascular resistance or high blood pressure in the lungs
-pneumothorax
-hemoptysis
the coughing up of blood or blood-stained mucus from the bronchi, larynx, trachea, or lungs
-sweat, tears, and saliva have extremely high sodium and chloride levels
Cystic fibrosis diagnostics
-positive sweat chloride testing = abnormally high sodium and chloride found
-absence of pancreatic enzymes
-newborn screening
-CXR = patchy atelectasis
Cystic fibrosis management
Respiratory:
-airway clearance therapies
-bronchodilators
-physical exercise
-aggressive treatment of infection
-aerosolized antibiotics
-home IV antibiotic therapy
-yearly tune ups, good daily chest PT, lung transplant
-all children who have pulmonary involvement require chest physiotherapy w/ postural drainage several times daily to mobilize secretions from the lungs. Involves percussion, vibration, and postural drainage, vigorous pulmonary hygiene for mobilization of secretions is critical to prevent infection
GI:
-replacement of pancreatic enzymes (w/i 30 min of eating meals)
-high-protein, high-calorie diet: as much as 150% recommended daily allowance
-laxatives
-pancreatic enzymes (sprinkles)
must be administered w/ all meals and snacks to promote adequate digestion and absorption of nutrients
-vitamin supplements = fat soluble
-relief of intestinal obstructions
-reduction of rectal prolapse
-treatment of chronic GERD
Cystic fibrosis prognosis
-expected life expectancy 35-40 years
-progressive and incurable
-transplantation
increases life expectancy
availability of organs
Family support
-emotional needs of child and family: counseling, support groups
-need for multiple treatments a day
-frequent hospitalization
-home care
-implications of genetic transmission of disease
-continual adjustments w/i family must occur
-children are frequently hospitalized, and this may place an additional strain on the family and its finances. Some people carry the defective gene, but never develop cystic fibrosis
Apnea
-absence of breathing for longer than 20 seconds
may be accompanied by bradycardia
-ALTE (acute life-threatening event)/ BRUE (brief resolved unexplained event)
-in newborns may be related to = hypoglycemia, hyperbilirubinemia, infection, hypothermia, prematurity
-correct cause if possible
-home apnea monitor
-when infant is noted to be apneic, gently stimulate him or her to take a breathe again. If gentle stimulation is unsuccessful, then rescue breathing or bag-valve-mask ventilation must be started. Poorly defined term that is used to describe an acute change in infants breathing, appearance, or behavior
-symptoms are brief typically less than a minute do not confuse w/ SIDS
BRUE (brife resolved unexplained event)
will usually include the following:
-apnea or change in breathing. Typically w/o effort or occasionally will struggle due to obstruction
-color change… cyanosis, pallor
-change in muscle tone… usually limp, rarely rigid
-chocking or gagging
-altered LOC
Majority due to:
-GERD
-neurologic episodes = seizures, bleed, infection
-less frequent = cardiac disease, upper airway obstruction, metabolic disorder
-DON’T FORGET CHILD ABUSE