Hematologic and Immunologic Dysfunction Flashcards
The most common hematologic disorder of childhood
Develops slowly - children slowly adapt to it; not sudden thing identified - not realize until blood drawn; small things that add up: trouble concentrating/sleeping - smaller red flags
Not disease but indication/mainfestation of underlying process - figure out reason why anemic
Increased risk for infection: tissue hypoxic weakening body’s defense against infection
Decrease in number of RBCs or hemoglobin (Hgb) concentration below normal, or both
Decreased oxygen-carrying capacity of blood
Anemia - RBC disorders
Depletion of RBCs or Hgb, or both
Causes and physiology - Classification of anemias
Characteristic changes in RBC size, shape, or color, or a combination of these
Morphology - Classification of anemias
Sometimes defined as Hgb <10 or 11 g/dL; however, this cutoff may be inappropriate for children
Diagnostic evaluation - Classification of anemias
Hemodilution
Due to decreased RBC
Thinner blood
Decreased PVR - greater quantity of blood returning to heart
Increased cardiac circulation and turbulence
Murmur
Severe cases can lead to cardiac failure
Growth restriction
Children with this lack energy, easily fatigued, pallor - chronic thing - very slow to grow - deprived of iron cannot have the growth spurts - grow at slower rate
Nurse: need know what is causing anemia
Consequences of anemia in ped pop - Classification of anemias
decrease red blood cell production or side of the decreased red blood cell production The increased red blood cell loss or increased red blood cell destruction okay we need to know those three things and what they lead to some calm and sign common signs and symptoms of anemia your tachycardia, palpation, dyspnea, SOB, dizziness, lightheadness, diaphoresis, changes in skin color, looks fatigued - acting different - signs
Increased RBC loss
Bleeding: acute blood loss - nose bleeds; hemophilia
Increased Cardiac workload: notice in times of exercise stress or illness - see increased episodes of that to tachypnea, palpitations - red flag - figure out what is going on
Nurse: need know what is causing anemia
Diagnosis
Treatment of underlying cause
Supportive care
Therapeutic management of anemia
History and physical
ask all the normal questions you know when did the start what’s what have you noticed what things are different
CBC
we’re going to check that hemoglobin check that hematocrit look at those red blood cells
Diagnosis
Have to treat the underlying cause - do we need to have a transfusion; need to have a nutritional intervention;
transfusions in the Pediatric population hospitals
double checking for you before you give blood always going to double check it with another nurse
want to give that blood within 30 minutes of receiving it
make sure they have that ID
Ensure you’ve got all your supplies so as soon as it comes to your unit you’re ready to get it hooked up and going
scan your blood which is very helpful you still always going to check it with another nurse
going to get your vitals every 15 minutes and then you’re definitely going to watch for fever chills and irritability right those are pretty standard practices one difference especially with the younger much younger population is we don’t give it through that filter tubing
we pull it up through that tubing but we put it in a regular syringe and then just hook it straight up to some IV tubing and put it straight to the baby okay we don’t leave it in that filter tubing and we don’t hang it with any other fluids
can get fluid overloaded very quickly - kidneys are Half Baked
Transfusion after hemorrhage if needed
Nutritional intervention for deficiency anemias
Treatment of underlying cause
going to give IV fluids if we need to increase therapy if needed and we are going to have bed rest
need to be doing age appropriate care if they’re older we can show them their slide - should show them how their cells are different especially that later school age
if we have to start that IV give them some emela cream on there trying to make that not hurt as much - pick color want - give as many choices as can
Intravenous (IV) fluids to replace intravascular volume
Oxygen therapy
Bed rest
Supportive care
Generally preventable
Pathophysiology
Therapeutic management
Prognosis
Nursing care management
Iron deficiency anemia
Iron-fortified cereals and formulas for infants
Special needs of premature infants
Adolescents at risk because of rapid growth and poor eating habits
Generally preventable
Caused by any number of factors
“Milk babies”
Caused by inadequate supply of dietary iron
most common type in our pediatric population
not having enough hemoglobin and you have to have iron to produce hemoglobin so if you don’t have iron you’re not going to produce this hemoglobin
caused by an inadequate supply of dietary iron and can typically be preventable
if the baby is premature okay remember iron babies get their iron from Mom and that last trimester especially that last 4 weeks so if a mom is anemic if that Mom doesn’t have iron then they don’t have iron to give the baby so we always check our iron in our moms because I want our babies to get their iron
if the baby is premature they’re not getting the iron from that Mom or what iron they have gotten they’re going to burn through it very quickly so premature infants are almost always on an iron supplement
adolescents are also at risk because they’re rapid growth and they’re poor eating habits
breast milk is not a good source of iron there’s just a very very small amount of iron that is passed through breast milk
good amount of iron in the formula - not really worried about iron deficiency anemia
cows milk is a very poor source of iron and it can fill babies up very quickly all right and remember toddlers starting around a year are extremely picky so they don’t want to eat a lot of food they don’t like healthy foods necessarily and they just don’t eat a lot they would be happy just drinking milk but they can’t they’re not going to get their iron from that okay so what we need to do is we need to give them solids first and then give them milk after their solids
Pathophysiology
Increase in the amount of iron the child receives
iron supplement and typically this is an oral supplement that we can give
can cause staining on the teeth
take with ascorbic acid or vitamin C - that helps the absorption of it
not going to give it with dairy cuz that’s going to inhibit the absorption it likes the acidic environment
can cause dark colored stools and can cause constipation so we may need to start with a smaller amount and slowly work our way up to what they need
iron can be poisonous for kids so we need to be very careful about how parents store this we need to educate them on making sure kids do not have access to this
Therapeutic management
Diet
Iron supplementation
follow-up so usually a month after we’ve got this diagnosis when we started them on some type of supplementation - going to draw Labs again and see where their levels are - if it is not improving we need to figure out what is going on Administration - figure out why things not improving
Nursing care management
Autosomal recessive disorder
Clinical features
Pathophysiology of sickle cell anemia
has to do with the breakdown of red blood cells
have a decrease in oxygen carrying capacity
body is going through these red blood cells a lot faster than they should
have trouble keeping up a normal hemoglobin
do need transfusions especially
9% of African-Americans are carriers (have sickle cell trait)
40% of native Africans are carriers
If both parents have trait, each of their children has a 25% chance of having disease
Autosomal recessive disorder
chronic joint swelling aching bones
fatigue frequent infections they’re going to be small for their age either small kiddos okay and they’ll have enlarged spleens
Obstruction caused by sickled RBCs
The vascular inflammation
Increased RBC destruction
Abnormal adhesion, entanglement, and meshing of rigid sickle-shaped cells
Local hypoxia
Cellular death
Clinical features
Universal screening of newborns in the United States
do screen for on all newborns
Management
No cure (except possibly bone marrow transplantation)
Supportive care/prevention of sickling episodes
Frequent bacterial infections because of immunocompromise
common complications include stroke acute chest syndrome retinal damage nerve and organ damage
Bacterial infection: Leading cause of death in young children with sickle cell disease
Usual life span: Into the fifth decade
EDUCATION!
Diagnosis and management of SCD
Prevent sickling
goals are to avoid crisis relieve symptoms and prevent complications
issues the sickling of cells and it blocks the blood flow - leads ischemia that pain we want to prevent sickling we are going to have these kids rest and minimize their energy expenditure we are going to make sure they are always hydrated if they are in pain we are going to treat their pain
are going to have to replace blood product if needed these kids will be at an increased risk of iron buildup if they’re getting a lot of transfusion so it’s important that we test their iron levels
May avoid activities due to that fear of that pain and that crisis they may avoid doing something and this is going to delay their normal growth and development - pay attention to that when we do our education we have to educate parents and what we need to do to make sure they are developing appropriately for their age
Rest and minimize energy expenditure
Hydration
Electrolyte replacement
Analgesia
Blood replacement
Antibiotics
antibiotics - if the kids are under five they’re going to be on prophylactic antibiotics and typically we use penicillin for this - no cure our goal is to against support and prevent sickling bacterial infections - issues because they are immunocompromised bacterial infections are the leading cause of death and young children with sickle cell disease because we know this we can prevent we can teach ways to prevent getting sick and still lead a semi-normal life
Management
Treat the medical emergencies of sickle cell crisis
Possible prophylactic antibiotics for 2 months to 5 years
Monitoring of reticulocyte count regularly to evaluate bone marrow function
Blood transfusion: If given early in crisis, may reduce ischemia
Therapeutic management of sickle cell crisis
signs of a crisis
triggers
medical emergency -
Rest to minimize energy loss
Hydration through oral or IV therapy
Electrolyte replacement
Analgesia for pain (tend to be undermedicated)
Blood replacement for anemia
Antibiotics for infection
Treat the medical emergencies of sickle cell crisis
going to be a lot of pain - biggest red flags
blood in their urine
irritable
tired
Pain in tongue, Palms of their hands and their nail beds
signs of a crisis
Infection - is a big one
cold exposure
high altitudes
over exertion
dehydration
things that cause cellular oxygen deprivation - not getting enough oxygen to ourselves it can increase our risk of having a Sickle Cell crisis
triggers
get these kids to the hospital
going to have them rest
going to minimize energy loss
blood replacement for that anemia - going through those red blood cells a lot quicker - cells don’t carry the oxygen like we need
decrease excessive exercise so we still want them to be active we still want them to be exercised energy - don’t want them to be in restrictive or tight clothing
encourage socialization - go to school but we have to be aware of their risk for infection - if they have a temperature we want them to be immediately evaluated
medical emergency -
make sure they’re well hydrated
another thing is we want these kids to always be immunized that’s a big education thing - not delayed in immunization schedule
give them hydration - oral or IV depending on how severe they are
Hydration through oral or IV therapy
treat their pain
Analgesia for pain (tend to be undermedicated)
antibiotics if they have an infection - prophylactic antibiotics we’re going to monitor everything
Antibiotics for infection
transfusions - remember watch for that iron - giving a lot of transfusions - teach the parents and the children maintain as high of the state of oxygenation as possible
Blood transfusion: If given early in crisis, may reduce ischemia
A group of hereditary bleeding disorders that result from deficiencies of specific clotting factors
In 80% of cases, the inheritable pattern is X-linked recessive
Identification of a specific factor deficiency allows for definitive treatment
Hemophilia A
Hemophilia B
Von Willebrand disease
Hemophilia
hereditary bleeding disorder
has to do with increased blood loss
need different factors depending on what type they have
almost all cases we can do blood testing to check these kids to see if they have it we are also going to focus on what type
how much normal clotting activity they do have
after a platelet plug develops at a bleeding site the lack of clotting Factor prevents a stable fibrant clot from performing - we will have some delayed bleeding
A group of hereditary bleeding disorders that result from deficiencies of specific clotting factors
“Classic hemophilia”
Deficiency of factor VIII
Hemophilia A
Also known as “Christmas disease”
Caused by deficiency of factor IX
Hemophilia B
Deficiency of von Willebrand factor and factor VIII
Von Willebrand disease
History of bleeding episodes
X-linked inheritance
Laboratory findings
Diagnostic evaluation of hemophilia
do a lot of different blood tests such as a PTT time
will check for certain factors
see bleeding into the joints - they’ll have bruising
is also familial
Overt prolonged bleeding
Hemarthrosis
Ecchymosis
History of bleeding episodes
Low levels of factor VIII or IX, prolonged partial thromboplastin time
Normal: Platelet count, parathormone level, and fibrinogen level
Laboratory findings
Prevent bleeding
Recognize and control bleeding
RICE
Prevent the crippling effects of bleeding
Support the family and home care
Genetic counseling
Replace missing clotting factors
Mild-to-moderate hemophilia: Patients live near-normal lives
Control of symptoms and limiting joint damage improve quality of life
Care management of hemophilia
prevent bleeding as much as possible
keep a safe environment
good dental hygiene
padding furniture
we’re going to rest ice compress and elevate
get to the hospital if they’re not stopping bleeding
greatest risk are going to be that spleen if the spleen gets hit pretty hard - cause hypovolemic shock
any head injury - worried about intracranial bleeding
repeated joint injury - can lead to decrease range of motion
increase this plasma levels of clotting factors it helps prevent those deformities caused by the repeating bleeding into that muscle joint in organs
be very very diligent with these kids
mild to moderate hemophilia patients do live near normal lives - controlling the symptoms and limiting that joint damage - improve their quality of life and a lot of that is going to be around the education of parents and then once the kids get older we can start to educate the kids a little bit more remember that adolescence does and those years in QOL
Safe environment
Dental hygiene
Prevent bleeding
Aggressive replacement therapy with factor concentrate
Home infusion
Replace missing clotting factors
Inherited disorder
clotting disorder it is almost always inherited however there are some cases that are not
lots of bleeding that we’re having trouble stopping
Intervention: Rice - rest ice compress Elevate administering clotting factors and support for that child and parent
Child has a defect/deficiency in the von Willebrand factor protein (helps with clotting!)
Assessment
Nursing interventions
Von willebrand’s disease
Epistaxis
Gum bleeding
Easy bruising
Excessive menstrual bleeding
Assessment
Similar to hemophilia (RICE)
Administering clotting factors
Support for child/parent
Nursing interventions
autosomal recessive Disorder - so both parents must be carriers
individuals of Mediterranean African and Southeast Asian descent
have a reduced or no production of one of those synthesis of hemoglobin and remember that is the protein that carries that oxygen in the red blood cells
reduced production and absent production we have what’s called beta thalassemia intermediate
beta thalassemia minor we really need intervention maybe a transfusion here there and maybe signs of anemia is very most common
Beta fallacy the major which we can have some significant issues with that one
symptoms - 3 to 6 months; anemia; splenomegaly which will also show us jaundice in a swollen abdomen; growth restriction; chipmunks
nursing interventions blood administration is the most common; we have to keep up those blood supplies - we have to give them those hemoglobin; monitor for iron overload; Do some genetic counseling; up to date on all of their immunizations including the flu vaccine; common for these kids to have a spleenomectomy and get that spleen taken out if that does - report any signs of infection because now they are at an increased risk of sepsis
too much iron
Autosomal recessive disorder (both parents must be carriers)
Reduced production of 1 of the Beta-globin chains in the synthesis of hemoglobin
Assessment
Nursing Interventions
B-Thalassemia major
it can cause this hemochromyostasis with that we can get arrhythmias pericarditis cirrhosis and diabetes and also hypothyroidism
too much iron
More common in individuals of Mediterranean, African, S.E Asian descent
Autosomal recessive disorder (both parents must be carriers)
Anemia
Hepatosplenomegaly
Growth Restriction
Chipmunk Facies
Assessment
Blood administration
Monitor for iron overload
Hemochromatosis
Education
Nursing Interventions
Issues with lead poisoning is that kids that have lead poisoning are not usually symptomatic
instead of absorbing iron the body absorbs the lead instead so it appears as if the child has anemia related to iron deficiency however they’re getting enough iron
three main things are worried about is neurologic system anemia that’s what’s going to cause the renal system
can lead to cognitive and behavioral problems: aggression hyperactivity impulsivity delinquency long-term trouble in schools developmental delays lower IQ and it can also lead to physical growth and reproduction issues
Most common between 6 months- 6 years
Peaks around 2-3 years
Common causes
Risk screening starting at 6 months
Clinical assessment
Blood lead level greater than 5mcg/dl requires intervention
Treatment- separate the child from the lead source!
If blood levels are higher than 45mcg/dl, pharmacologic treatment is necessary
Chelation therapy
Prevention
most common between 6 months and 6 years - peak around 2 to 3 years - start screening at 6 months and the initial screening talks about asking about how old the house is and what are potential risk factors in the house like parents jobs and if we do have to worry about some cultural things again such as Folk remedies
at a higher risk we can do additional screens and we can do them earlier clinical assessment
Common symptoms: failure to gain weight developmental lags behavioral changes Pica
Super high levels - we’re going to have to have a pharmacological intervention and that’s going to be your chelation therapy
have to educate parents find out where they work
Toys especially if they were they’re from a different country
Put food on - not have lead - prevention is huge
Lead poisoning
bloody noses - this is common
severe it could mean some kind of iron underlying disease such as vascular moralities leukemia
we have to remain calm keep the child calm
put some ice on top of the nose while we’re still holding pressure and that can cause those capillaries to get smaller and increase that clotting have them sit forward for 10 minutes and then let go
can cauterize those little capillaries in there if kids have allergies they will be at increased likelihood of having these bloody noses
Isolated and transient epistaxis is common in childhood
Recurrent or severe episodes may indicate underlying disease
Vascular abnormalities, leukemia, thrombocytopenia, clotting factor deficiency diseases (von Willebrand disease and hemophilia)
Care Management
Epistaxis
Remain calm; keep child calm
Bleeding usually stops within 10 minutes after nasal pressure
Have child sit up and lean forward
Apply pressure to the soft lower part of the nose
Evaluate further if bleeding continues
Care Management
