PEDS Ch.25-Hematological Function & Ch.30-Immune Function

***Nursing Assessment of the Hematological System

-analyzing the CBC is a critical starting point +high or low blood cell counts can indicate certain blood disorders +if erythrocytes, which carry oxygen via their Hgb molecules to the tissues, are small (microcytic) and pale (microchromic), they cannot carry as much oxygen because of a lower iron capacity. This low iron carrying capacity results in a child who tires easily and has tachypnea and tachycardia to compensate for the lower oxygen delivery. As hypoxia lingers, the child will not grow, develop, or increase in cognitive abilities as well as a child who has adequate oxygen saturation levels (93%-98%, supplemented, or up to 100% without supplementation). A component of the CBC known as the differential is the breakdown of the leukocytes or WBCs by type. These cells are the key to the immune response and the first line in the anti-inflammatory process. +In bacterial infections, neutrophils proliferate first to start battling the infective bacterial agent. Therefore, an increased neutrophil count is a key indicator that a bacterial infection is present and the immune response is beginning. If the number of lymphocytes is elevated, it usually indicates a viral infection. These smaller infectious agents are phagocytized by lymphocytes. + As the infection continues, monocytes begin to proliferate and transform into macrophages to continue the battle as the neutrophils and lymphocytes wane. With certain bacterial infections, the number of lymphocytes is low, overall, and atypical lymphocytes may appear on the report. These are the helper and killer T cells. The B cells are responsible for cellular immunity and make the immunoglobulins. When the leukocyte count is low, it indicates that the child has limited defenses and is susceptible to infection, as in a child undergoing treament for leukemia or a child with aplastic anemia. Thrombocytes (platelets) are essential in clot formation. Thus, a low laboratory thrombocyte count, as well as increased clotting times, can indicate petechiae, ecchymoses, purpura, or spontaneous to prolonged bleeding.

***Latex Allergy Manifestation

A child who has come into contact with latex can present with any of the symptoms of an allergic reaction: hives, wheezing, cough, shortness of breath, nasal congestion, rhinorrhea, sneezing, pruritus in the nose, palate or eyes, and hypotension.

***Bleeding Disorders: Disseminated Intravascular Coagulation Nursing Planning and Education

A child who is being discharged following DIC will likely continue taking heparin, low-molecular-weight heparin, or another anticoagulant at home for a period of time. Teach the child and parents how to take the medication, the side effects to monitor for, and what laboratory tests are required to monitor levels weekly or monthly, depending on therapy length. Instruct the child to slowly resume activities when the bleeding risk has subsided.

***Food Allergies Pathophysiology

A food allergy, or food hypersensitivity, is an IgE-mediated immunological reaction to ingestion of a food or food additive. About 4% to 6% of children in the United States are affected by food allergies. The most common food allergies acquired during childhood are allergies to milk, eggs, peanuts, tree nuts, fish, shellfish, wheat, and soy. Peanut, tree nut, fish, and shellfish allergies tend to be the only ones that continue into adulthood. There are two categories of food allergies: IgE-mediated and non-IgE-mediated. In IgE-mediated allergies, the IgE antibodies react to certain foods. In non-IgE-mediated allergies, other parts of the immune system react to the food. This reaction still causes symptoms but does not involve IgE. A person can have both types of allergies.

***Juvenile Idiopathic Arthritis Nursing Planning and Education

A primary goal for these children and families is to learn how to effectively manage pain and maintain mobility. Evaluate their knowledge about the disorder and the treatment regimen. Administer medications as prescribed to control inflammation and prevent disease progression. . Encourage the child to maintain joint range of motion and muscle strength via exercise (physical or occupational therapy). Swimming is a particularly useful exercise to maintain joint mobility without placing pressure on the joints. Teach families appropriate use of splints prescribed to prevent joint contractures. Monitor for pressure areas or skin breakdown with splint or orthotic use. Having two sets of books (one at school and one at home) allows the child to do homework without having to carry heavy books home. Health promotion includes educating the family about regular vision screening for the child to identify potential visual changes early and prevent further damage. Special summer camps for children with juvenile arthritis allow the child to socialize and belong to a group and have been shown to promote self-esteem in the child with chronic illness. Encourage appropriate family functioning and refer the family to support groups such as those sponsored by the American Juvenile Arthritis Organization.

***Aplastic Anemia (Fanconi Anemia) Manifestations

Acquired aplastic anemia presents in a manner similar to that of leukemia. It is associated with symptoms of pancytopenia, including excessive fatigue and weakness, lack of energy, and a change in behavior due to anemia; recurrent upper respiratory infections that never completely go away, febrile episodes, and lesions that remain erythemic, open, and unhealing because of leukopenia; and excessive bruising, petechiae, or epistaxis or gum bleeding as a result of thrombocytopenia.

***Latex Allergy Nursing Assessment and Diagnosis

All children who go to healthcare facilities need to be screened for latex allergy. Obtain the child's health history, making note of reported allergy to rubber items, such as gloves, or any symptoms of a reaction after exposure, such as hives, rash, coughing, wheezing, or shortness of breath. Additional questions and signs to look for in the health history include the following: •Swelling or itching of the mouth after a dental examination •Allergic symptoms after eating foods known to have cross-reactivity to latex (e.g., pears, peaches, passion fruit, plums, pineapple, kiwi, figs, grapes, cherries, melons, nectarines, papaya, apples, apricots, bananas, chestnuts, carrots, celery, avocados, tomatoes, or potatoes) On physical exam, assess the child for signs and symptoms of a reaction and implement appropriate interventions.

***Food Allergies Manifestations

Allergic reactions can involve the skin, mouth, eyes, lungs, heart, gut, and brain. Signs and symptoms include hives, flushing, facial swelling, mouth and throat itching, runny nose, and GI reactions, such as vomiting, abdominal pain, and diarrhea. In severe reactions, the tongue, uvula, pharynx, or upper airway may swell. Wheezing indicates that edema is occurring in the airway and is considered a serious sign of respiratory complications. Although it is rare, collapse of the cardiovascular system can occur.

***Anaphylaxis Pathophysiology

Anaphylaxis is an acute, immediate, and severe IgE-mediated response to an allergen. Any allergen, whether it is from food, latex, an insect sting, or drugs, has the potential to trigger a severe reaction that results in anaphylaxis. An anaphylactic response is an exaggerated allergic reaction in which the entire body is affected by an excessive release of chemical mediators. The response can be triggered by an allergen that has induced a response in the past or by one that has never evoked a response in the child before. Because anaphylaxis involves many organ systems and may be life-threatening, it is considered a medical emergency. Once contact is made with an allergen, a severe reaction usually starts within 5 to 10 minutes. B cells are triggered to produce plasma cells. The plasma cells secrete Igs. The IgE, mast cells, and eosinophils release a large amount of histamines and other inflammatory mediators, leading to tissue edema. Cutaneous, cardiopulmonary, GI, and neurological symptoms occur as a result. Plasma volume rapidly decreases from vasodilation, placing the body at risk for circulatory collapse. The child may need prolonged resuscitation, and there is a risk of death.

Anemias

Anemias are conditions in which mature erythrocytes are decreased in number or volume or impaired in function. In some conditions, this decrease in RBCs may be accompanied by an increase in immature RBCs, known as reticulocytes, in the circulation. Yet these cells are not as good at carrying oxygen as the mature ones. Sickle cell disease is a type of hemoglobinopathy, or disease of the Hgb, that shortens the RBC lifespan and causes the cell to become sickle shaped, which results in occlusions in the microcirculation. Beta-thalassemia, also known as Cooley's anemia, occurs as a result of a failure of the beta chains to form, which leads to rapid cell lysis. Aplastic anemia results from a congenital or acquired bone marrow failure in which no erythrocytes are produced in the bone marrow. The following discussion elaborates on each condition in more detail.

***Bleeding Disorders: Disseminated Intravascular Coagulation Nursing Assessment and Diagnosis

Assessment includes observation of the clinical signs discussed earlier, as well as laboratory tests related to clotting time and blood cell counts, such as PT, PTT, and CBC (including platelet count). The diagnosis is confirmed when laboratory test results reveal thrombocytopenia, low fibrinogen levels, and elevated PT, PTT, and ***D-dimer levels.

***Human Immunodeficiency Virus (HIV) Manifestations

Children with HIV may be asymptomatic. Presenting symptoms include chronic diarrhea, failure to thrive, and delayed development. Later symptoms may include frequent infections, including symptoms of opportunistic infections. Infants with HIV develop illnesses and symptoms of AIDS disease within a shorter time frame than is seen in adults (i.e., the latency period is shorter).

***Aplastic Anemia (Fanconi Anemia) Pathophysiology

Aplastic anemia is a condition of complete bone marrow suppression or failure that produces pancytopenia, a condition in which all three blood cellular component (RBCs, WBCs, and platelets) are low or absent (Barone et al., 2015). The red marrow is replaced by a fatty yellow substance. This condition can be congenital or acquired. Congenital aplastic anemia, also known as Fanconi anemia, has an autosomal recessive gene transmission. Acquired aplastic anemia is believed to occur following an insult to the body that destroys the bone marrow, such as by one of the following mechanisms (Barone et al., 2015): •An autoimmune response •An infection •Exposure to chemicals: paints, plastics, petroleum products, shellacs, dyes, or benzenes •Pharmacological agents: chloramphenicol, antibiotics that suppress bone marrow, antineoplastic agents, sulfonamides, cimetidine, or anticonvulsants •Radiation The autoimmune process suspected in acquired aplastic anemia has been attributed to self-reactive T cells that obliterate the bone marrow blood-forming cells (erythropoietin, leukopoietin, and thrombocytopoietin). As a result, the rich, red bone marrow is replaced by a fatty yellow substance (Risitano & Perna, 2011).

***Aplastic Anemia (Fanconi Anemia) Nursing Intervention

As with the other anemia disorders, HLA-matched bone marrow or stem cell transplantation is the best option for a cure clinically Other treatments may include androgens and corticosteroids Monitor the child's absolute neutrophil and WBC counts for signs of neutropenia. Place the child on neutropenic precautions. With anemia, encourage the child to conserve energy and monitor oxygenation and tissue perfusion. Note any signs of tachypnea or tachycardia, which could indicate compensation for the poor RBC count. Provide thrombocytopenic care for petechiae, bruising, or bleeding, and avoid intramuscular injections and rectal assessment of temperature, which could cause deep tissue injury and hemorrhage.

***Bleeding Disorders: Hemophilia Nursing Planning and Education

At discharge, teach the family how to identify signs of bleeding and proper actions to take in response. Review with them the current plan of care for exercise and regaining motion in any joint that has had bleeding. Stress the importance of active range of motion over passive because it decreases the risk of bleeding due to overextending the joint. Suggest that they use exercises such as taping a reward at a challenging height and having the child walk the arm up the wall to reach it for an involved upper extremity joint. Educate the family on and evaluate their knowledge of prophylactic and treatment factor replacement and RICE intervention for active bleeding at home (Patient Teaching 25.1). Discuss growth and developmental milestones and provide anticipatory guidance as appropriate for the child's age level. If the child has mild or moderate hemophilia, include information on DDAVP. The National Hemophilia Foundation is a good source of information to help the family learn about the condition and resources. A detailed discussion of these topics is included later.Teach the family how to diligently assess for and identify the signs of intracranial, gastrointestinal, and renal bleeding. Intracranial bleeding may be indicated by a sudden severe headache, nausea and vomiting, dizziness, visual disturbances, changes in motor function or gait, or seizures. Any head injury needs to be evaluated. Vomit that has the appearance of coffee grounds or contains bright red blood denotes upper abdominal or stomach bleeding. Unexplained abdominal pain or swelling and stool changes that are black and tarry or bright red may indicate gastrointestinal hemorrhage. Urine with a red, bloody to an amber-orange character may be a sign of bleeding.One of the most frequent sites for bleeding in children with hemophilia is in the joints. A joint injury can lead to a severe deformity and a frozen joint. Movement is essential to maintain function and mobility. Therefore, once the bleeding is controlled, encourage parents to perform active range of motion exercises involving the affected joint. Active range of motion is better than passive because using passive could stretch the joint too far and cause synovial injury to recur. The injured joint is likely to be very painful, but stress to the parents the importance of the child engaging in activities that require the full range of motion in the joint. Having the child reach for a toy or stretch the hand high on the wall to retrieve a sticker, washable tattoo, or other appropriate reward can motivate the child to perform active range of motion in the shoulder or elbow joint. Playing with a ball and rolling it forward and back with the foot can assist the child in using full range of motion in the knee joint. Interdisciplinary collaboration with physical and occupational therapists can also help the child maintain joint motion. Provide the child and parents with anticipatory guidance related to helping the child meet upcoming developmental milestones. Discuss immunizations that the child will need to receive, and explain that they will need to be administered subcutaneously instead of intramuscularly to prevent muscle bleeding. For parents of children who are ready to begin standing, walking, and toddling, provide directions on preventing injuries. Encourage parents to allow walking only on intact carpeted areas, to pad or cover fireplaces, and to remove furniture with sharp corners to make any falls and bumps as benign as possible. Sewing padding in the pants to protect knees and hips may be needed to prevent hemorrhage while allowing the child to gain these skills. Once the child is old enough to participate in team sports, inform the parents of the risks of the child engaging in activities that involve contact or have a high likelihood of injury, such as baseball, soccer, and football. Suggest that they encourage the child to swim, golf, and participate in such activities as the scholar's bowl, chess team, and journalism club. When the child reaches puberty and needs to begin shaving, encourage the use of an electric razor. Teach the family that the child should take acetaminophen for pain and avoid nonsteroidal anti-inflammatory drugs, such as aspirin, naproxen, and ibuprofen, which inhibit platelet function.

***Anaphylaxis Manifestations

Clinical manifestations include pruritic urticaria (itchy hives), coughing, stridor, asthma attack, extreme anxiety, and loss of consciousness. Additional manifestations include lip, tongue, or palate pruritus and swelling; nasal pruritus; congestion; sneezing; stridor; tachycardia; and chest pain.

***Bleeding Disorders: Disseminated Intravascular Coagulation Manifestations

Clinically, the patient may experience peripheral thrombosis (positive Homan's sign), excessive bleeding from a venipuncture, oozing from old entry sites, petechiae, ecchymoses, purpura, epistaxis, gingival bleeding, gastrointestinal or pulmonary hemorrhage, tachycardia, hypotension, and muscle, back, or chest pain. Signs that may indicate an intracranial bleed or clot include a sudden headache, slurred speech, and asymmetrical movements

***Bleeding Disorders: Disseminated Intravascular Coagulation Pathophysiology

Disseminated intravascular coagulation (DIC) is a condition characterized by systemic coagulation throughout the bloodstream (due to thrombosis from stimulated thrombin) and consequent hemorrhage (due to fibrinolysis stimulated by plasmin), which uses up all the available coagulation factors. DIC is a secondary consequence in children that occurs because of some severe disease process or injury that is associated with hypoxia, acidosis, shock, burns, or sepsis and that is heralded by simultaneous bleeding and clotting. It is an emergency for an already ill child. DIC has many causes and may develop as a complication of any of the following: sepsis, shock, cancer, obstetrical conditions (placenta abruption, fetal demise syndrome, preeclampsia/eclampsia, or amniotic fluid embolism), burns, massive trauma, snake bites, heat stroke, or transfusion reactions (Marcdante & Kliegman, 2019; Porth, 2015; Schub & Balderrama, 2018). Regardless of the specific etiology, DIC involves the widespread formation of clots throughout the circulation, with many small blood vessels becoming occluded. The intrinsic and extrinsic coagulation pathways are activated and exhausted. Once the coagulation factors and platelets are consumed, bleeding results.

***Human Immunodeficiency Virus (HIV) Nursing Assessment and Diagnosis

During the health history, make note of any reports of the more common signs and symptoms: failure to thrive, recurrent bacterial infections, opportunistic infections, chronic or recurrent diarrhea (from GI infections and lactose intolerance), recurrent or persistent fever, developmental delay, and prolonged candidiasis. Look for risk factors in the medical history such as maternal HIV infection or AIDS, receipt of blood transfusions, sexual abuse, substance use or abuse, or participation in sexual behaviors without the use of a condom. Determine level of consciousness. Upon physical exam, note any presence of fever, plot growth on the standard growth chart, and assess for any growth or developmental delays. Inspect the oral cavity for candidiasis (thrush). Assess respirations and observe for work of breathing; auscultate for adventitious sounds. Palpate for enlarged lymph nodes or swollen parotid glands. Palpate the abdomen, making note of an enlarged spleen or liver. The incidence of HIV-2 in the United States is so low that the CDC does not recommend routine testing for HIV-2. Testing for HIV-2 in children is considered only if the mother had risk factors for HIV-2 or a known HIV-2 infection or if the child is exhibiting signs of HIV but the results of diagnostic tests for HIV-1 have been negative (Nyamweya et al., 2013). Therefore, the tests discussed here are for HIV-1. The tests used to diagnose HIV in children are different from those used in adults. In adults antibodies are measured, whereas in infants an aspect of the virus, not antibodies, is measured. Serum Ig levels may be unusually high early in HIV infection and then decrease. A child is diagnosed with AIDS when the child has developed an opportunistic infection, the serum shows signs of HIV antibody, and the CD4 count is 200 or less. The reverse transcriptase polymerase chain reaction, enzyme-linked immunosorbent assay, and CD4 counts are the common lab and diagnostic studies ordered to assess for HIV infection in infants and children. Refer to Table 30.4 for additional information about these tests.

***Bleeding Disorders: Disseminated Intravascular Coagulation Nursing Evaluation

Expected outcomes for a child with DIC include the following: •Intravascular coagulation is stopped, and the child returns to homeostasis. •The child has no embolism that causes permanent impairment. •If bleeding occurs, it is rapidly controlled and the child survives this life-threatening event.

***Human Immunodeficiency Virus (HIV) Nursing Evaluation

Expected outcomes for a child with HIV include the following: •The child has adequate respiratory function and remains well oxygenated and perfused. •The child has sufficient nutritional intake and hydration to maintain normal growth patterns and weight. •The child's skin remains free of preventable breakdown. •The child and family demonstrate appropriate coping methods related to the diagnosis and prognosis. •The child and family are able to adhere to the prescribed treatment regimens.

***Juvenile Idiopathic Arthritis Nursing Evaluation

Expected outcomes for a child with JIA include the following: •The child is able to move joints and complete activities of daily living with minimal pain and discomfort. •The child is able to demonstrate appropriate growth and development. •The child and family know how to recognize and manage the signs and symptoms of complications, such as avascular necrosis.

***Food Allergies Nursing Evaluation

Expected outcomes for a child with a food allergy include the following: •The child has not experienced harm from a hypersensitivity reaction due to the ability to successfully prevent or manage a reaction. •The child is maintaining adequate nutrition and hydration, as evidenced by achieving growth and development patterns appropriate for age.

***Latex Allergy Nursing Evaluation

Expected outcomes for a child with a latex allergy include the following: •The child and family have taken measures to reduce the child's exposure potential triggers of allergic reaction. •The child, family, and school personnel understand the signs and symptoms of a reaction and have created an emergency plan.

***Anaphylaxis Nursing Evaluation

Expected outcomes for a child with anaphylaxis include the following: •The child is able to maintain a patent airway and adequate oxygenation and perfusion. •The child has not experienced harm from a severe hypersensitivity reaction. •The child and family demonstrate appropriate coping skills. •The child is able to meet growth and developmental milestones appropriate for age.

***Aplastic Anemia (Fanconi Anemia) Nursing Evaluation

Expected outcomes for a child with aplastic anemia include the following: •The child experiences no sepsis, hemorrhage, or life-threatening anemic event. •Treatment is successful, and red bone marrow is produced. •For a child with Fanconi anemia, developmental milestones are optimized or mastered.

***Bleeding Disorders: Hemophilia Nursing Evaluation

Expected outcomes for a child with hemophilia include the following: •The child maintains normal PT, PTT, and coagulation factor levels. •The child maintains full range of motion in all joints. •The child engages in developmentally appropriate activities that offer a low risk of injury that could lead to bleeding. •The child and family demonstrate an understanding of appropriate risk prevention measures, medication administration, and identification of signs of various types of bleeding. •The child and family have a strong social support system in place.

***Sickle Cell Anemia Nursing Evaluation

Expected outcomes for a child with sickle cell disease include the following: •The child's oxygen saturation level is maintained at 93% or greater. •The child maintains adequate hydration. •Infection is prevented or treated effectively. •The child's pain is controlled. •Tissue damage is prevented or minimized. •If needed, the child is prepared effectively for a transfusion or exchange transfusion. •The transition to home care is seamless, with the family meeting the child's needs. •The child is emotionally sound and obtaining growth and developmental milestones.

***Sickle Cell Disease

Sickle cell disease is a group of diseases associated with the presence of an abnormal Hgb gene known as the Hgb sickle (Hgb S) gene, which results in sickle-shaped RBCs that tend to clump together, leading to occluded blood vessels, impaired oxygen delivery, pain crises, premature RBC death, and anemia.

***Common Triggers for Anaphylaxis

Food, such as nuts, shellfish, and eggs •Insect stings, such as by a bee or wasp •Drugs, such as penicillin and nonsteroidal anti-inflammatory drugs •Radiopaque dyes •Latex

***Bleeding Disorders: Hemophilia Pathophysiology

Hemophilia is a bleeding disorder that is usually inherited and involves a deficit in a coagulation factor, specifically, factor VIII (FVIII) or factor IX (FIX). In some cases, the patient may have a family history of the condition, such as a member who bled to death following a seemingly minor surgery or injury. In other cases, there is no family history of the disease—it just develops unexpectedly in an individual. Either way, the diagnosis is a matter of concern for the family.Hemophilia is a coagulopathy in which the child is born without an essential coagulation factor. Figure 25.10 illustrates the coagulation pathway or cascade that leads to the formation of a clot. Hemophilia A, known as classic hemophilia, is caused by a deficiency in FVIII in the coagulation pathway. It is the most common form of hemophilia and is an X-linked recessive disorder. Hemophilia B, or Christmas disease, is a FIX deficiency in the coagulation pathway. mother to son

***Human Immunodeficiency Virus (HIV) Pathophysiology

Human immunodeficiency virus (HIV) is a disorder caused by an infectious agent and is the most well-known acquired immunodeficiency disease. Children acquire HIV either vertically or horizontally. The mode of infection for an infant is usually via vertical transmission from the mother. The virus can be transferred in utero, during birth, or via breast milk. Horizontal transmission occurs from using nonsterile needles (as in IV drug use or tattooing) or from sexual contact. Acquiring HIV from transfused blood products has become very rare. Infants primarily acquire the virus through their mothers, whereas adolescents primarily acquire it through sexual activity or IV drug use. HIV causes a wide range of illnesses in children. Symptoms can range from none to mild, moderate, or severe. Acquired immune deficiency syndrome (AIDS), also known as acquired immunodeficiency syndrome, represents the most severe form of the illness. After a person contracts the virus, the blood does not exhibit any HIV antibody for 2 weeks to 6 months. During this time, the virus is replicating itself by infecting the CD4 (T helper) cells, causing them to become dysfunctional. The T helper cells demonstrate a decline in function even in asymptomatic infants and children who have not experienced large decreases in their CD4 cell count. Gradually, the number of CD4 cells declines, which weakens both humoral and cell-mediated immunity. When the number of CD4 cells is low, immune deficiency occurs and the patient becomes susceptible to opportunistic infections. Once a patient's CD4 cell count drops to less than 200 cells/µL and the patient has developed an opportunistic infection, he or she is diagnosed with AIDS

***Food Allergies Nursing Assessment and Diagnosis

Immediately assess airway, breathing, and circulation to identify potential serious reactions. Once a child is determined to be stable, complete the rest of the assessment. Obtain a detailed food history and details about reactions, including the suspected food, quantity consumed, length of time between ingestion and symptoms, description of the symptoms, treatments received, and response to treatments. The detailed food history can help determine whether the reactions are a result of a food intolerance or a true food allergy. Make note of any reported GI symptoms, such as burning in the mouth or throat, bloating, nausea, and diarrhea. Identify potential risk factors such as a previous exposure to the suspected food, a history of asthma that has not been appropriately managed and controlled, or a report of eczema flare-ups related to food. Inspect the skin, observing for color and signs of a rash, hives, or edema. Auscultate the heart and lungs, making note of the heart and respiratory rates and any wheezing. Diagnostic testing involves allergy skin prick tests, RASTs, blood tests, IgE tests, and challenge tests. Challenge tests are done for suspected food and medication allergies. During a challenge test, a very small amount of an allergen is inhaled or taken by mouth. A physician, preferably an allergist, should be supervising the tests.

***Aplastic Anemia (Fanconi Anemia) Nursing Assessment and Diagnosis

In addition to observation of the signs described earlier, diagnosis of aplastic anemia is typically based on a CBC, with pancytopenia being a positive finding.

***Bleeding Disorders: Hemophilia Nursing Assessment and Diagnosis

In addition to observing for signs and symptoms of hemophilia-associated bleeding, monitoring laboratory values for prothrombin time (PT), partial thromboplastin time (PTT), and hemophilia factor levels is critical both in initial assessment and to determine whether coagulation factor replacement therapy is working. PT and PTT are both tests that measure the time required to form a clot (coagulate) and thus the presence and function of various clotting factors. Specifically, PT measures the time required for extrinsic clotting factors to perform their part in coagulation, which is normally 11 to 13.5 seconds in children. PTT measures the time required for intrinsic clotting factors, which include FVIII and FIX, to perform their part in coagulation, which is normally 25 to 35 seconds. Hemophilia factor assays measure the blood levels of specific coagulation factors that are deficient in hemophilia (FVIII and FIX). Increased PT and PTT and decreased levels of coagulation factors may indicate hemophilia.

***Juvenile Idiopathic Arthritis Pathophysiology

Juvenile idiopathic arthritis (JIA) is an autoimmune disorder characterized by inflammation primarily affecting the joints. The disorder was formerly termed juvenile rheumatoid arthritis, but only a few types of juvenile arthritis produce a positive rheumatoid factor. A child with JIA may experience periods of being healthy that alternate with periods of joint inflammation flare-ups. Milder cases of the disorder have been reported to resolve as children reached adulthood. Other children may experience a more severe form of the disorder, causing them to deal with joint inflammation throughout their adulthood.As the term idiopathic in its name indicates, the cause of this condition is unknown. Factors such as infection, trauma, and stress have been discussed as possible triggers, but it has proved difficult to scientifically demonstrate a causal relationship. Scientists have also suggested that there is a genetic component making the child more susceptible, and research continues to look for a possible connection with human leukocyte antigens. Twice as many females as males are diagnosed with JIA (U.S. Department of Health & Human Services, 2018a). Autoantibodies primarily target the synovial joints, resulting in decreased mobility, swelling, and pain. Normal synovial fluid acts as a cushion and helps lubricate cartilage, allowing for smooth joint movement. Once the synovium is inflamed, excessive thin, watery fluid is produced, diminishing the lubricating and cushion effects. Structures outside the joints may also become involved in the process, leading to further damage and deterioration. Potential complications include tendinitis, adhesions between joint surfaces, ankylosis of the joints, and soft-tissue contractures.

***Latex Allergy Pathophysiology

Latex refers to the protein in the sap of the Brazilian rubber tree (Hevea brasiliensis) and to the natural rubber products made from the sap. Latex can be found in many everyday household products, such as balloons, rubber bands, rubber balls, and bandages. The development of an allergy to latex products has become a common occurrence among children, healthcare workers, and the general population worldwide. Millions of consumer and commercial products containing latex are produced and are not required to include on the label whether the product contains latex. This lack of identification of latex content makes it difficult to completely prevent contact with the allergen. Gloves are an example of a very common item used in health care that contains latex and exposes patients and healthcare workers alike to this potential allergen. Many health institutions have now eliminated the use of latex gloves, but the gloves are still being sold in stores and used in other industries, so the risk of exposure remains high for those with an allergy. Children at the highest risk for latex allergy are those with spina bifida and those who undergo frequent medical procedures and surgeries in which they are exposed to products containing latex. Latex allergy and food allergy have a similar pathophysiology. A person can have a reaction from breathing in latex fibers in the air or from skin contact with a latex product. Not all reactions are a true allergy. There are three types of reactions to natural rubber latex: •IgE-mediated latex allergy: This type involves the immune system, is considered a true allergic reaction, and can be life-threatening. This type of reaction occurs when IgE antibodies react with latex proteins. •Cell-mediated contact dermatitis: This type causes skin inflammation but is not life-threatening. This type of reaction is more of a sensitivity to the chemicals used in the making of the product rather than to the rubber protein. The dermatitis can spread but usually resolves on its own. Four out of five people who develop an IgE-mediated allergy are reported to have had contact dermatitis first; thus, such a diagnosis should be taken as a warning sign and the child should be monitored closely for development of a true allergy. •Irritant dermatitis: This type is a common reaction but not an allergy. A red, itchy rash develops where the skin comes into contact with a natural rubber product. Healthcare workers who wear powdered gloves often develop this condition. Again, any contact dermatitis should be taken seriously and the person monitored for the development of a true allergy (AAFA, 2015).

***Juvenile Idiopathic Arthritis Nursing Assessment and Diagnosis

Note any history of irritability as an infant or a young child, because this may be the first sign of inflammation. Note any history of the child complaining of pain or not wanting to play or get out of bed in the morning. Ask whether the child has a history of fever. Assess for delayed growth and development and signs of current systemic disease, such as fever, rash, limping, or guarding of a joint or extremity. Inspect and palpate joints for edema, redness, warmth, tenderness and preferred position of comfort. Common lab results consistent with a diagnosis with JIA include mild-to-moderate anemia and elevated erythrocyte sedimentation rate.

***Anaphylaxis Nursing Assessment and Diagnosis

Note the child's level of consciousness. In the health history, obtain information about prior exposure to the allergen, including any medications the child received and the response to treatment. Symptoms seen on examination include the following: urticaria; edema of tongue; bronchospasm; laryngeal edema; facial edema (angioedema); hypotension; tingling of the palms of the hands, soles of the feet, or lips; light-headedness; and tightness of the chest (CDC, 2017). If prompt treatment is not received, these symptoms can progress to seizures, cardiac arrhythmia, shock, respiratory distress, and, ultimately, death (Priority Care Concepts 30.3). Diagnostic testing involves obtaining lab tests to check for elevated IgE and eosinophils levels in nasal and bronchial secretions. Check vital signs to assess for severe hypotension.

***Human Immunodeficiency Virus (HIV) Nursing Interventions

Nursing care of an infant or a child diagnosed with HIV can be very challenging. The primary focus of care is on taking measures to recognize early signs of infection to avoid the development of HIV encephalopathy and AIDS. The number of children being diagnosed with HIV encephalopathy drastically declined after highly active antiretroviral drug therapy (HAART) was initiated. Care includes promoting normal growth and development; preventing infections; promoting compliance with the medication regimen; promoting nutrition; providing pain management and comfort measures; promoting respiratory and other organ function; educating the child, family, and caregivers; and providing ongoing psychosocial support. Instruct women with HIV who are pregnant on measures they can take to prevent transmitting the virus to the infant. Offer all pregnant women the opportunity to be tested and to receive any needed counseling services. If a pregnant woman is HIV positive, treatment depends on the stage of pregnancy. Once infants are born, they should receive treatment per the current guidelines. Breastfeeding is discouraged in mothers with HIV, so educate the mother on safe alternatives to breastfeeding (Avert, 2018). During delivery, protect the infant from the infected maternal secretions. After delivery, bathe the newborn as soon as possible and wash the eyes and face before administering prophylactic eye medication. Invasive procedures should be avoided if possible in the newborn. In 2017, the Department of Health and Human Services (DHHS) Panel on Antiretroviral Therapy (ART) and Medical Management of Children Living with HIV published updated guidelines for treatment (a link to the website and the document is located in the reference list at the end of the chapter). The treatment regimen prescribed should be based on consideration of patient characteristics (such as age and severity of illness), results of viral resistance testing, and characteristics of the proposed medications (DHHS, 2017 a-e; Van Dyke et al., 2016). The recommendations include the use of a combination of antiretroviral drugs. Often, this type of therapy is referred to as HAART and is described as an anti-HIV cocktail (Thompson & Shalit, 2014). Although there is no cure for HIV infection, treatment with HAART has improved survival rates, growth, neurodevelopment, and immune function (The Pharmacy 30.2). Before HAART became a treatment option, neurocognitive complications, such as progressive HIV encephalopathy, were devastating. A diagnosis meant a terminal prognosis, usually within 2 years (Lazarus, Rutstein, & Lowenthal, 2015). Aggressive prophylactic antibiotic therapy in combination with ART or HAART has been successful in preventing opportunistic infections (Capriotti & Frizzell, 2016). Currently there is no evidence to indicate that starting treatment earlier than 14 days of life improves outcomes, but it remains an option (DHHS, 2017d). The therapy is adjusted as needed. Many children with HIV experience failure to thrive, frequent diarrhea, and lactose intolerance, making it a challenge to maintain adequate nutrition and hydration. Treatments could include total parenteral nutrition, a special diet or formula, tube feedings, and vitamin supplements. Be aware of and avoid potential interactions between supplements and the child's prescribed antiretroviral medications. Antidiarrheal medications are not recommended for infants, but they may be prescribed for older children. Carefully monitor hydration status, skin turgor, and urine output.

***Food Allergies Nursing Planning and Education

Parents need much teaching and support, because having a child with a food allergy can cause anxiety and fear. Educate the parents, child, day care personnel, school teachers, friends, and others involved in the child's daily activities on the signs of an allergic reaction. Children with allergies are at risk for anaphylaxis, so teach parents and caregivers to be alert to the signs of an anaphylactic reaction. Refer families to the Food Allergy and Anaphylaxis Network for additional information and support. Teaching should include how and when to use the medications, especially during an allergic reaction. The family should have a written emergency plan in case of severe reactions. Dietary teaching should include how to read food labels, information about hidden allergens in food, and what foods are safe substitutes. A referral to a dietician may be helpful for the family to learn additional ways of avoiding the food allergens and how to manage the child's nutrition.

***Anaphylaxis Nursing Planning and Education

Priority education for the family is how to prevent and manage future episodes of an anaphylactic reaction. An emergency plan should be in place at home and at the child's school or day care center with up-to-date contact information. Teach the family, caretakers, and child, if age-appropriate, how to use injectable epinephrine, usually in the form of an EpiPen, in the case of allergen exposure. The child should carry the epinephrine at all times and wear a medical alert identification bracelet or necklace. Instruct the child and family to call emergency personnel (911) as soon as possible after the EpiPen has been administered. To help prevent future episodes, teach the child and family how to avoid known allergens. Immunotherapy (allergy shots) or desensitization treatments may be indicated for certain types of allergies, such as a hypersensitivity to stinging insects or a severe penicillin allergy. Families need to understand the treatments and what is involved; for example, desensitization typically requires the child to spend a few days in the intensive care unit while the treatment is being administered. Make referrals as needed to support groups, counseling, and other services.

***Sickle Cell Disease Pathophysiology

Sickle cell disease occurs when a person inherits two abnormal Hgb genes instead of two normal adult Hgb (Hgb A) genes, with at least one of the two abnormal genes being the Hgb sickle (Hgb S) variant. The Hgb S gene variant is an autosomal recessive trait, which means that the child with sickle cell anemia has received the gene from each parent. The sickling is due to Hgb within the cells crystalizing, and the RBC can revert to its usual discoid shape once the triggering condition has passed. Sickling can occur multiple times in the same RBC, until it breaks down or no longer returns to the discoid shape. Sickled RBCs have a propensity to adhere to each other and surrounding structures, and once there is enough of them, they begin to clog the vascular circulation, a process known as vasoocclusion

***Human Immunodeficiency Virus (HIV) Nursing Planning and Education

Teach parents and caretakers how to watch for signs of infection and when to call the healthcare provider. Counsel adolescents about the methods and risk of transmission and how to decrease their risk if they are sexually active or involved in risky behaviors, such as drinking alcohol or using IV drugs. Preventative measures and early treatment of infection are vital and include practicing good hand washing and hand hygiene, avoiding contact with individuals who are known to be infected, practicing good skin care, promoting a sanitary environment, receiving immunizations as scheduled, and following prescribed drug regimens. Regarding immunizations, it is important to keep on schedule. The administration of live virus vaccines depends on the CD4 count and level of immunosuppression. Children with HIV may receive the measles and varicella vaccines if they have mild-to-moderate immunosuppression (Kroger, Dutchin, & Vazquez, 2018). Families of children infected with HIV face many stressors. These include coping with the diagnosis of an incurable disease, costly treatments and healthcare bills, possibly more than one family member being infected (mother and child), public stigma associated with HIV, confidentiality issues, and the amount of time needed to devote to multiple medical appointments and hospitalizations. Parents and family members may experience anticipatory grieving, guilt, anger, or denial. The nurse can help support the family's coping by encouraging participation in support groups and allowing them to express their feelings using therapeutic communication. Educate the family regarding the importance of compliance with the HAART medication regimen. Work with the caregivers to develop a medication administration schedule that fits the family's routine. Educate personnel at the child's day care center or school regarding the disease, how it is transmitted, and how to handle having the child in the school setting. Eventually, as children with HIV get older, they will have to learn about their illness and how to care for themselves. Demonstrate acceptance of the child and model the behavior to others. Adolescents with HIV must deal with not only the growth and developmental tasks of adolescence but also the challenges of HIV stigma, compliance with the ART or HAART regimen, concerns about sharing HIV status with others, limited social support, worries about HIV transmission, transitioning from pediatric to adult health care, and living with a chronic illness (Evangeli, 2018). Provide children, adolescents, and their families with appropriate referrals and information regarding available resources, such as the Elizabeth Glaser Pediatric AIDS Foundation at www.pedaids.org.

***Latex Allergy Nursing Planning and Education

Teach the child and family about foods with a known cross-reactivity to latex. Parents should receive information about signs and symptoms of latex hypersensitivity and create an emergency treatment plan. Provide resource information, such as websites and the names of organizations for people with latex allergy.

***Aplastic Anemia (Fanconi Anemia) Nursing Planning and Education

The discharge instructions for a child receiving immunosuppressive therapy with ALG or ATG should focus on preventing serum sickness with corticosteroids. Because corticosteroids are foul tasting, instruct parents to try different methods for making them more palatable to the child, such as adding a flavored syrup to them, giving a strongly flavored chaser after administering them, and placing them as far back in the buccal space as possible for liquid preparations and on the tongue for tablets. If a prepubertal male child is receiving androgen therapy, prepare the family for the pubertal changes and short stature that occurs from epiphyseal growth plates sealing as a result of the treatment; refer them to an endocrinologist for guidance, if needed. Help them learn about using safety razors for shaving the facial hair that develops with puberty. Pancytopenia indicates a need to assess tissue perfusion and conserve energy. Teach the child and family leukopenia precautions to protect the child from infections. In addition to the infection prevention measures covered earlier for sickle cell disease and beta-thalassemia, neutropenic precautions are initiated when the absolute neutrophil count drops below 1,000 and become very strict when it drops below 500. Have the child wear a face mask when in public; avoid raw fruits, vegetables, and meats; remove live plants from the household because of mold spores; avoid contact with ill people; and observe closely for wounds that remain open and unhealed. Provide thrombocytopenia home care instructions on managing epistaxis, observing for signs of gastrointestinal bleeding, using a very soft toothbrush or cloth to clean the teeth and prevent gingival bleeding, avoiding rough housing or horse play, and avoiding monkey bars or other areas that would place the child at risk for a fall that could lead to internal hemorrhage. If congenital birth defects are present with Fanconi anemia, a multidisciplinary plan of care is needed, including speech therapy, audiology, physical therapy, and occupational therapy. Provide care for sensory disorders, and provide resources to help the family learn to use sign language to communicate with a deaf child. Teach the family about hearing aid care and replacing batteries. Instruct parents to keep batteries out of reach of a toddler because ingesting them would result in a medical emergency. If the child is visually impaired, provide instructions to the family on assisting with corrective lenses or functional training for blindness. Encourage the child and family to use facilities that specialize in educating students who have sensory deficits, such as state schools for the deaf and blind. Provide teaching on maximizing orthopedic function, preventing contractures, and using assistive devices to potentiate activities of daily living. Teach the family how to work on strengthening the child's core and assisting with gross motor, fine motor, and language development. Provide exercises and activities that they can incorporate in their home care activities.

***Sickle Cell Disease Manifestations

The first manifestation of sickle cell disease is often a vasoocclusive crisis. Vasoocclusion produces a painful crisis, which is the most frequent cause of hospitalization in children and adolescents with sickle cell disease. The vasoocclusion robs the tissues of oxygen and results in ischemic infarcts at the site of the occlusion, ensuing in cellular death, which is extremely painful and can occur anywhere the blood flows. Figure 25.4 shows the areas of involvement in a child with sickle cell disease. In children younger than 5 years of age, the vasoocclusive crisis frequently affects the bones in the hands and feet, resulting in dactylitis (Fig. 25.5; hand-foot syndrome). The infarct of the bone marrow spaces produces edematous, erythematous extremities. The dactylitis is very painful and can lead to ulcerations of the tissues similar to venous and arterial insufficiency ulcers.Another common area for vasoocclusive crisis in toddlers and preschoolers is the brain, where a cerebral vascular infarct can occur, producing neurological tissue injury. The child may have a stroke or silent cerebral ischemia (SCI) that may be unrecognized or produce debilitating paralysis, aphasia, seizure, headache, facial droop, and even death. The nurse must pay careful attention to neurological assessment of these children and seek emergency assistance for them when any suspected alterations are noted. Children with sickle cell disease are at risk for SCI and chronic anemia for impaired cognition. Transfusions and exchange transfusions are part of the emergency intervention for SCI. Similar to dactylitis, the peak age of SCI occurs between birth and 5 years. Acute chest syndrome is a complication of sickle cell disease that occurs as a result of sickling occlusion in the pulmonary vessels, emboli from infarcted bone marrow, or an infectious change that damages the lung tissue. This condition results in chest pain, hypoxemia, dyspnea, tachypnea, cough, fever, wheezing, radiographic infiltrates, and respiratory distress. The onset can be subtle or sudden respiratory compromise. It is very serious and should be treated as a medical emergency. Acute chest syndrome mimics pneumonia, but the chest radiograph shows infarcted infiltrates rather than empyema or purulent areas. The coronary vessels are susceptible to infarcts just as the brain and lungs are. Children may have infarcts that kill tissue in the cardiac muscle. The infarct is caused by sickle cell obstruction, not plaques, as in atherosclerosis. The gastrointestinal organs also may be affected by vasoocclusion and hemolysis. The spleen absorbs the damaged and lysed cells and slowly become fibrotic and nonfunctional, a condition known as asplenia. This damage produces a splenic sequestration that is chronic and leads to increased infection risk because the spleen stops filtering infectious agents and trapping debris. The genitourinary tract is also impacted by vasoocclusion. Males may have painful erections, a condition called priapism. The infarct occurs in the penile circulation, and blood is unable to flow in or out of penis. The priapism is not related to sexual stimulation. Repeated priapisms can result in erectile dysfunction. The testicles and ovaries are also susceptible to the poor circulatory flow and can sustain damage, causing infertility. Hemolysis and infarcts in the kidneys can produce inefficient glomerular filtration and renal tubular dysfunction, resulting in the inability to concentrate the urine. Enuresis develops because the urine output is greater than bladder capacity. Unlike other cases of enuresis, restricting oral fluids before bedtime is not advisable. Hydration is essential to maintain the blood flow. Teach parents to get the child up to go to the bathroom periodically to prevent bedwetting. The altered filtration results in hematuria. The RBCs are not reabsorbed and are excreted in the urine. The urine color is amber to red tinged. Another complication is retinal detachment due to repeated infarcts from vasoocclusion. Retinal ischemia leads to serious retinal disease and visual acuity changes, ending in retinal detachment.

***Juvenile Idiopathic Arthritis Nursing Interventions

The focus of nursing care is on promotion of a normal lifestyle by managing the inflammation, pain, and mobility issues (resulting from pain and swelling in the joints) of the disorder. The medication regimen includes NSAIDS, corticosteroids, and antirheumatic medications. Encourage parents to promote the child's sleep by providing a warm bath at bedtime, warm compresses to affected joints, or massage. Refer the child to a specialist in pediatric rheumatology.

***Anaphylaxis Nursing Interventions

The focus of nursing management for children with anaphylaxis is on supporting the airway, breathing, and circulation. Determine whether the airway is patent and breathing and circulation are sufficient. This is a medical emergency, and therefore the child should be treated with IV antihistamine, IV epinephrine, and IV corticosteroids (Capriotti & Frizzell, 2016). The focus of therapeutic management is to assess and provide ongoing support of the airway, breathing, and circulation. The initial goal is to maintain a patent airway. Sometimes endotracheal intubation is needed to meet this goal. After establishing a patent airway, administer epinephrine as ordered. Administer supplemental oxygen as needed. If bronchospasms occur, administer a bronchodilator inhalation treatment. Administer IV fluids to provide volume expansion and corticosteroids to prevent late-onset reactions. Monitor the child for at least 2 hours after an anaphylactic reaction.

***Latex Allergy Nursing Interventions

The focus of nursing management is to prevent exposure to latex products. Once exposed, remove the allergen and cleanse the area with soap and water. Assess the child for reaction signs, and prepare to intervene as needed, such as by resuscitation, if the reaction is severe. Latex allergy should be documented as an alert on the child's chart, identification band, medication administration record, and physician's order sheet.

Variations in Anatomy and Physiology of Hematological Function

The hematological system incorporates all of the components of blood: -erythrocytes (red blood cells, RBCs) -leukocytes (white blood cells, WBCs) -thrombocytes (platelets), as well as the plasma, or liquid portion, that carries the cellular components +Erythrocytes transport oxygen and carbon dioxide through the hemoglobin (Hgb) molecules within these cells. Hgb is composed of two sets of polypeptides (the alpha and beta chains, which form the protein), globulin, and heme, which is composed of four atoms of iron per molecule. A key variation in children is the presence of fetal Hgb until 4 to 6 months of life. At this time, it is replaced by adult Hgb or one of the variants. +WBCs are responsible for infection control and immunological regulation. The WBCs are granulocytes and agranulocytes. Granulocytes include neutrophils, the most abundant of the granulocytes. Neutrophils are primarily the first line of defense that battle or phagocytize bacterial invaders. The other two types of granulocytes are eosinophils, which function in allergic reactions, and basophils, which have function in parasitic infections and a protective function in creating pathways for the phagocytes to move to sites of inflammation. The agranulocytes are lymphocytes and monocytes. Lymphocytes are small, less powerful phagocytes and are the first responders in viral infections. During a bacterial or immune-regulated process, lymphocytes become T cells and B cells. Monocytes, which are the most powerful phagocytizers and which become macrophages, phagocytize viral and bacterial infections. In addition, monocytes have a longer life than do neutrophils and lymphocytes. +Thrombocytes and other clotting factors in the plasma, such as fibrinogen, factors I, II to V, VII, VIII, IX, and X, are the key to clotting. These platelets and coagulation factors aggregate to seal tissues from injury and to protect from hemorrhage by sealing blood vessels

***Sickle Cell Anemia Nursing Interventions

The key to treatment of sickle cell disease is addressing the triggers of sickling—the conditions that cause the erythrocytes to change shape from discoid to crescent or sickle. Therefore, take measures to prevent hypoxia, infections that produce fever and acidosis, and dehydration. Other interventions for sickle cell disease include pain management, medications to increase fetal Hgb (hydroxyurea) or bind iron (deferoxamine), bone marrow transplantation, and providing psychosocial support to the child and family. These are discussed in detail later. To help prevent hypoxia, maintain the child's oxygen saturation levels at 93% or above. Also, prepare patients for surgical procedures that can result in hypoxia. Before surgical procedures that may lower the oxygen tension, administer a transfusion of packed RBCs. The aim of this transfusion is to have a higher concentration of non-sickle than sickling cells. Keep the percentage of sickle cells in the circulatory flow below 30% and the Hgb volume greater than 10 g/dL to lower the risk of the child having a vasoocclusive crisis. Patients may need transfusions at other times, as well. In some situations, the patient receives a transfusion simply to correct the blood Hgb concentration and volume. Other times, the patient may need an exchange transfusion to remove the sickled cells and replace them with normal HgbAA cells. Transfusions are also used in treating strokes, priapisms, and, in some cases, acute chest syndrome, splenic sequestration, and other complications of sickle cell disease. To prevent infections, make sure the child with sickle cell disease receives prophylactic antibiotics and regular vaccines to protect from illness, particularly when asplenic and exposed to infections. Early treatment is necessary for any signs of infection. Viral illnesses, many of which may be prevented with immunizations, can trigger an aplastic crisis, which is an autoimmune response following a viral infection that stops bone marrow production. Hyperhemolytic crisis is also associated with an infectious process that causes the RBCs to break down excessively. Hydration is very important because it helps prevent vasoocclusion by moving the RBCs through the vessels and vascular bed, which prevents sickled cells from aggregating and clogging the microcirculation. During a vasoocclusive crisis, providing intravenous hydration with an isotonic solution increases the hydrostatic pressure and volume and improves blood flow. Prevent dehydration by maintaining the child's fluid intake at1,600 mL/m2 or at 1.5 times the normal fluid needs for age and weight. If using the Holliday-Segar formula for fluid requirements, calculate the child's fluid needs and then multiply it by 1.5. Pain management for sickle cell disease should focus on general comfort measures and medication (How Much Does It Hurt? 25.1). Apply warm compresses using devices such as a heat therapy pump to help vasodilate the painful areas for 15 to 20 minutes. A systematic review revealed that cognitive behavioral therapy, acupuncture, biofeedback, massage, and aquatic therapy have been studied as nonpharmacological therapies for pain management in patients with sickle cell disease. The most significant intervention was cognitive behavioral therapy (Williams & Tanabe, 2016), which is a form of psychotherapy that changes how individuals view and cope with pain. Nonsteroidal anti-inflammatory drugs are also used as an adjunct to potentiate the effects of opioids in pain control. However, they have side effects that may increase the risk for heart complications and stroke, as well as gastrointestinal bleeding. In 2017, hydroxyurea, also known as hydroxycarbamide, was approved by the U.S. Food and Drug Administration (FDA) as a medication for treating children with sickle cell disease. The recommended initial daily dose for children 2 years of age and older is 20 mg/kg. The benefits of hydroxyurea for treating sickle cell disease are that it increases the level of fetal Hgb and reduces the incidence of vasoocclusive crisis, promotes splenic function, and reduces the incidence of stroke and acute chest syndrome (Hankins et al., 2014; Nottage et al., 2014). The Baby Hug study tested, over a 2-year period, the effects of hydroxycarbamide/hydroxyurea on infants with sickle cell disease who were 9 to 18 months of age. The statistically significant findings revealed decreased pain and dactylitis in the study participants. The fetal Hgb level was increased. The adverse effect of hydroxyurea is neutropenia, but it reverses if the medication is stopped for a week. Monitoring the absolute neutrophil count and WBC levels is a key nursing intervention for this therapy, in addition to the earlier measures for the prevention of infection.Deferoxamine therapy may be used if hemolysis results in high levels of free iron. This chelating agent given by subcutaneous infusion binds the iron and makes it excretable in the urine. Instruct the child and parents to report any side effects, such as urinary changes (especially urinary tract infections), oliguria, dark color of the urine as a sudden change, blurred vision or halos around lights, swelling of the throat or difficulty breathing, signs of infection (especially bladder and urinary tract infections) or infection at the insertion site, and diarrhea or watery stools. Also inform the patient and family that the child should take vitamin C when taking deferoxamine to maximize the iron-binding capacity of this medication. The only curative therapy for sickle cell disease is a bone marrow transplantation, specifically autologous stem cell therapy with a human leukocyte antigen (HLA)-matched sibling donor.

***Bleeding Disorders: Hemophilia Nursing Interventions

The mainstay of treatment is factor replacement. For patients with severe hemophilia A, the recommendation for prophylactic treatment with FVIII is about 3 times weekly of 20 to 50 U/kg. Children with mild or moderate hemophilia A may get prophylactic treatment but are more likely to get factor on demand with injury. FVIII on-demand treatment varies in dose depending on the type of bleed: for joint hemorrhages, 40 to 50 U/kg every 12 to 24 hours; for large muscle hemorrhages, intracranial bleeds, or other severe hemorrhages, 50 U/kg continuously to intermittent infusions every 8, 12, or 24 hours . Another source calculated that the dosage of FVIII of 1 U/kg would increase the plasma factor concentration 2%, that a child with severe bleeding needs an increase in plasma factor concentration of 80% to 100%, and that a child with moderate bleeding needs an increase in plasma factor concentration of 40%. Preparing the factor for administration depends on how it is supplied. Because the factor is very expensive, if the ordered dose is slightly less than a vial when reconstituted, contact the prescriber and request authorization to give the entire vial to avoid waste. At one time, the factor was obtained from large pools of human plasma. Many donors would sell their blood for use in producing the factor. During this time, those with hemophilia were greatly affected by infectious outbreaks of HIV and hepatitis B, C, and D. New procedures in producing the recombinant factor for replacement have reduced the viral transmission.factor replacement, the patient is likely continuing to hemorrhage as a result of the inhibitors, which are immunoglobulin G antibodies. The coagulation is achieved by administering agents that produce clotting in spite of the factor deficit, such as factor VIIa, activated prothrombin complex concentrate . Although factor replacement is the priority to stop bleeding, treating hemorrhages also includes local measures. Teach the family and child the mnemonic RICE. "R" is for rest. The child should stop using the joint that is bleeding. "I" refers to the need to ice the area, which causes vasoconstriction and slows the blood loss. "C" indicates compression. Applying pressure to the site of the bleeding helps to slow and stop the bleeding to the area. "E" stands for elevate the injured area, which also helps to slow the blood loss. Teach the family to have ice packs, gallon bags of frozen alcohol and water in a 50/50 concentration, or frozen vegetables in the freezer ready to use at all times. Ace bandages may be used to apply compression, as well as direct hand compression. School nurses must also be prepared to care for the child at school and have the needed supplies to treat bleeding episodes. Gloving is important with any body fluid exposures. Another key treatment is desmopressin acetate (DDAVP), which increases the FVIII level to three to four times the circulating level in those with mild or moderate hemophilia A (The Pharmacy 25.5). Teach patients how to administer this medication intranasally. The child must clear the nasal passages before dosing. DDAVP is a synthetic antidiuretic hormone. It causes the body to retain water and may cause hyponatremia, hypertension, and congestive heart failure. Teach patients using this medication to report inadequate urine output or if there is a urinary or respiratory tract infection while using it. Some patients may be placed on a fluid restriction, especially when at risk for water intoxication. For dental procedures, administration of aminocaproic acid helps with clot formation by blocking fibrinolysis. The child's dentist should plan and supervise the shedding of deciduous teeth and administer aminocaproic acid to the child ahead of time. The child may also be given factor replacement and be scheduled for deciduous teeth pulling to control bleeding with this common preschool or school-age occurrence.

***Juvenile Idiopathic Arthritis Manifestations

The typical symptoms seen in JIA involve manifestations of joint inflammation, such as pain, redness, warmth, stiffness, and swelling (Fig. 30.4). Inactivity increases the stiffness and pain, and thus people with JIA tend to be very stiff in the morning after a night of sleep and decreased activity. Other forms of the disorder can involve the eyes and other organs. JIA is classified according to the number of joints involved and the presence of systemic manifestations, as shown in Table 30.8.

***Food Allergies Nursing Interventions

Therapeutic and nursing management should be focused on avoiding and alleviating the child's immunological response to food allergens. Medications used include histamine blockers and epinephrine. An important first step is to determine the difference between a food intolerance and a true food allergy. Food intolerance can occur when certain foods or additives cause an abnormal physiological, but not immunological, response. An example is milk allergy, which is often misperceived as lactose intolerance. Determining the difference helps ensure that the child receives appropriate treatment.

***Bleeding Disorders: Disseminated Intravascular Coagulation Nursing Interventions

Treatment of a child with DIC is focused on maintaining oxygenation and tissue perfusion, correcting acidosis, and replacing depleted coagulation factors. As ordered, administer intravenous platelets, fresh frozen plasma, and cryoprecipitate. Administer heparin or low-molecular-weight heparin to keep the coagulation factors that are being replaced from activating and continuing the hemorrhage. Nursing care should include ongoing assessment of patients for signs and symptoms of microvascular clot formation and possible emboli; altered mental state; sudden hypoxia; shortness of breath; hemoptysis, hematemesis, or other types of hemorrhage; tachycardia related to volume depletion; and hypotension. Monitor the results of laboratory studies such as platelet count, PT, and PTT. Assist with therapy and treatment of the underlying disease that triggered the process of DIC.

***Bleeding Disorders: Hemophilia Manifestations

When a child is born with an abnormally low or absent level of a coagulation factor, the ability to clot is impaired. The deficiency factor results in spontaneous or traumatic bleeding. These children can have hemophilia that ranges in severity from mild (>5% to ≤40% FVIII or FIX activity) to moderate (1% to 5% FVIII or FIX activity) to severe (<1% FVIII or FIX activity), based on the amount of factor produced and used (Zappa, McDaniel, Marandola, & Allen, 2012, p. e141). Hemorrhages in a child with hemophilia are typically contained within the tissues and commonly occur in a joint when the synovial capsule sustains friction that damages capillaries, resulting in an acute joint bleed. In a child with severe hemophilia, activities as mild as throwing a ball, walking across a grassy lawn briskly, and lifting and tugging on the shoulder joint may cause bleeding. In a child mildly affected with hemophilia, more intense trauma, such as a fall or bumping into a wall while walking at school, is required to trigger a bleed. Obviously, cuts, falls, and injuries that open the flesh result in a prolonged and excessive bleeding episode. The acute bleed is associated with swelling, pain, redness, and, eventually, ecchymotic or purpuric coloration. After the bleeding is controlled, the joint is swollen and painful, with limited movement, a condition called hemarthrosis.

***Sickle Cell Anemia Nursing Planning and Education

When planning the discharge of a child with sickle cell disease, teaching the family to maintain hydration levels is very important. The child needs to be given the required amount of fluid per day in milliliters and cups. To help ensure that the child takes in the full required amount, the daily total can be divided by the number of hours the child is awake to provide an hourly intake goal. For example, if a child is to have 1,674 mL in a day and is awake about 12 hours per day, divide the total by 12 hours, which results in an hourly intake goal of about 140 mL. The family also needs to have an idea of the variety of forms the fluid can take and their approximate volume. A single popsicle is 90 mL, and a double or twin popsicle is 180 to 200 mL. An 8-oz cup is 240 mL, and a 4-oz cup is 120 mL; thus, a small bowl of soup is about 120 mL. In particular, the family needs to know how many 4-oz cups (14 cups) and 8-oz cups (7 cups) should be consumed daily.Review with the family their plan for getting medications filled and proper administration of the medications. Discuss the use of analgesics at home, including the importance of safely storing narcotics out of the child's reach, of only parents administering them, and of protecting them from unprescribed use. Teach the family the purpose of each medication, side effects to watch for, and when to notify the prescriber about issues. Teach patients and families to avoid potential triggers for hypoxia, such as high altitudes and planes that are not pressurized. Teach the family to get the child immunized, including annual influenza vaccines and a pneumovax every 5 years, and to avoid exposing the child to crowds when cold and influenza viruses are rampant. Teach the child to use a hand sanitizer and wash hands frequently. Stress that the child should avoid touching the mouth, nose, and eyes unless the hands have just been washed. Educate the family to seek medical attention promptly when the child has a fever of 100.5°F to 101°F (38.1°C-38.3°C) or greater or demonstrates signs of an infection, such as diarrhea, vomiting, sore throat, cough, or congested nose or chest. Inform the family of the signs of a stroke or splenic sequestration and when to go to the emergency room versus the primary care provider. Urgent signs and symptoms include severe pain not relieved by home medications, chest pain and congestion that is getting rapidly more severe, inability to speak, drooling, inability to move one side of the body, seizure activity, and altered level of consciousness or orientation.

***Nursing Assessment and Diagnosis of Pediatric Immune Function

allergies, immunosuppressive issues, lymph nodes, neuro in check QQQQ

***Sickle Cell Anemia Nursing Assessment and Diagnostics

•Hgb electrophoresis: ᴑIs the most diagnostic test for sickle cell disease ᴑDetermines the types of Hgb present and the percentage of each in the circulating blood (i.e., Hgb A, Hgb F, and Hgb S) ᴑGuides treatment by determining the percentage of the variant Hgb •Sickledex: ᴑIs used for routine screening before surgery to prevent a sickle cell crisis in an undiagnosed patient when under anesthesia ᴑCannot differentiate between the disease state and the trait •Complete blood count: ᴑIs used as a diagnostic tool to visually detect the presence of sickled RBCs ᴑIs not optimal for detecting the condition, because the cells may not be visualized, although present ᴑAllows identification of RBC morphology In the United States, the prevalence of multiracial descent makes identifying those at risk for having sickle cell disease challenging. For that reason, all patients should have a sickle cell screen before surgery and the results documented to alert the healthcare team to potential surgical complications associated with this condition. For example, hypoxia, which occurs commonly in the surgical setting, can trigger a sudden and severe crisis in an individual with sickle cell disease. Knowing ahead that a patient has this risk factor allows the nurse and other members of the healthcare team to take extra measures to prevent this complication.