Hematology Pediatric Round 1


Hematology system allows for oxygen transport, minimizes hemorrhage and helps fight infection. Blood transports substances needed for cell metabolism, regulates acid/base balance, protects against infection and injury.

Blood components- plasma, cellular elements.

Plasma - water, solutes,
Cellular elements - cells proteins.

Blood components pic.jpeg
Cellular elements - erythrocytes (RBCs), leukocytes (WBCs), thrombocytes (platelets).

Prior to birth the blood cells are produced in the liver and spleen.
After birth new blood cells are produced from stem cells in the bone marrow (hematopoiesis).

Red Blood Cells - (RBCs) -
Supply tissues with oxygen, most abundant cells in blood.
Remain in circulation for 120 days then removed by the spleen.
RBCs do not replicate, new ones are produced by the bone marrow.
Their biconcave shape and small size allows them to reach all cells.
Each RBC has an oxygen carrying protein hemoglobin.
Hemoglobin allows RBCs to transport 100 xs more oxygen than could be transported in plasma alone.

RBC count peds pic.jpg

White Blood Cells (WBCs) -
Remove debris and defend against invaders.
The circulatory system delivers the WBCs to the site of injury or infection.
Invasion of foreign body first stimulates infection and then an immune response.
Lymphocytes are responsible for the immune response.
Monocytes, macrophages, neutrophils, eosinophils and basophils are responses to inflammation.
* CBC with differential measures the WBC levels*
WBC peds pic.jpg

Platelets
Disc shaped cytoplasmic components
Not cells
Live for 10 days then removed by the spleen
Facilitate blood coagulation by adhering to injured vessel wall and release biochemicals.

Pediatric platelet count pic.jpg

Anemia
Not a disease
Decrease in the oxygen carrying capacity of blood.
Most common blood disorder of children.
Prevalence over 13% in children under 5.
Microcytic, normocytic and macrocytic describe the size of RBCs
Hypochromic, normochromic and hyperchromic describe color and hgb content
Size differences are related to production problems
Color differences are related to hgb content.
Hgb peds pic.jpg
Anemia caused by:

*Production decrease (erythropoieisis)
Due to nutritional deficiency (iron, folate, B12, copper)
Due to bone marrow failure (aplastic anemia, ALL, parvovirus, CMV, malignancies)

*Destruction of RBCs increased (hemolysis)
Due to hemoglobiopathies (sickle cell, thalassemias)
Or drugs, toxic substances or infection

*Increased red blood cell loss
Due to acute blood loss (epistaxis, hemophilia, DIC)

Signs & Symptoms are related to the cause and severity- mild <11, mod <7, severe <3.
Pallor
Tachycardia
Headache
Fatigue
Shortness of breath
Muscle weakness
Irritability
Lack of interest in play
Anorexia
Murmurs
Low grade fever
Yellowish skin (blood destruction)
Grayish look suspect resp. because of pallor
Hair falls out, nails are brittle and spoon shaped
Spleen and/or liver enlargement
Cool skin, decreased peripheral pulses
These children can be underweight, normal weight or overweight.
If not resolved physical and developmental delays may occur.

Diagnosis:
history, clinical symptoms and labs. ? recent blood loss, diet

Nursing Diagnosis:
Ineffective tissue perfusion
Imbalanced nutrition
Deficient caregiver knowledge
Activity intolerance

Nursing Outcomes:
Child will maintain an adequate Hgb
Child will consume minimum RDA iron each day
Caregiver can describe feeding guidelines
Child will be able to engage in age appropriate play without fatigue

Nursing Interventions:
Correct underlying problem, treat the cause!
Caregiver education - Why adequate iron is necessary
Dietary and supplemental education
Breastfeeding (or iron fortified formula) till 4-6 months (more bioavailable) then 1mg/kg/day of iron
Age 4-6 months 2 or more serving of iron fortified infant cereal
By 6 months 1 feeding per day vitamin C rich fruit /day.
After 6 months pureed meats
Children age 1-5 no more than 24 oz. cows, goats, soy milk per day.
Consumption of iron rich foods
Cook acidic foods in iron pan
Give exact amount of iron supplement, more is not better
*Keep iron locked up to prevent overdose
Avoid side effects by taking with or after meals
Stools tarry green color
Be aware constipation may occur
***Liquid iron may stain teeth use straw, or dropper or syringe
Avoid fluoride, antacids, coffee, tea, milk, dairy, bread within 1 hour, interferes with absorption

*Interesting alternative*
  • Scientists in India have experimented with adding iron to inexpensive candies. These were given to at risk 3-6 year old children once a week with improvement noted.
  • Iron deficiency- adolescent rapid growth and poor diet, preg. Adol. Esp. needs more, most common inadequate intake. School age child blood loss. 3rd world country hook worms.





Dietary sources of iron:
Meats: Lean beef, veal, pork, lamb, poultry, liver
Seafood: Clams, oysters, shrimp
Grains: Whole grain bread, fortified cereals, brown rice, enriched pasta, tofu, soybeans
Vegetables: Spinach, okra, sweet potatoes, winter squash, white potatoes
Fruits: Tomato, prune juice, prunes, raisins, figs
Legumes: Dried peas, dried beans, legumes
Miscellaneous: Molasses, egg yolk, nutritional yeast


Case Study: Anemia

Super Nanny has done a good job teaching this family where to start to address the child's anemia. It is your job to explain the mechanism. People will only change their behavior when there is a logical reason. If you don't explain the link between diet and food these changes will not last.

  • Explain the role between blood, hemoglobin and iron.
  • What physical symptoms can anemia produce? Name some corresponding nursing diagnoses.
  • What is a good hemoglobin level? A bad one?
  • Who is at risk for this disorder?
  • What is the treatment and corresponding nursing interventions? Be specific in covering teaching topics.
  • Reiterate where sources of iron can come from.


Hematology Round 2
Sickle Cell Anemia






Three types:

Sickle Cell Anemia

Sickle Cell Hemoglobin C

Sickle Cell Thalassemia disease



Incidence and Etiology:

Autosomal recessive disorder - If both parents do not have the gene the child will be a carrier or will not have the gene

Carriers - Sickle Cell Trait (SCT), 1/14 African Americans carriers

Traced back thousands of years, children with SCT survived malaria epidemics



Pathophysiology:

Sickle cell gene has sickle hemoglobin, hemoglobin S

This abnormal hemoglobin aggregates in the RBC causing it to elongate into a cresent or sickle shape.

These cells are stiffer and less able to change shape.

This results in them being unable to pass through microcirculation causing vaso-occlussion, pain and infarction.

These cells are destroyed or pool in the spleen and only survive 10-20 days.

Thus anemia.

Sickle cells pic.jpg



Sickling of RBCs:

Hypoxemia - decreased oxygen tension of blood

Increased Hydrogen ions (decreased ph)

Increased plasma osmolarity (increased solutes in blood)

Decreased plasma volume (increased viscosity)

Low temperatures (vasoconstriction)



Manifestations:

First 6 months of life asymptomatic because hemoglobin F (fetal) doesn't sickle, as hemoglobin S replaces F sickling occurs.

Pallor, irritibility, fatigue, shortness of breath, jaundice,

* pain * can occur in any body organ or joint especially during vaso-occlusive crisis



Sickle cell pic.jpg

Vaso-occlusive crisis:

Accumulation of sickled cells in a vessel causing obstruction, if not reversed infarction can occur.

Pain can last several hours to several weeks

Painful crisis - bone marrow ischemia, deep pain, lumbosacral, spine, knee, shoulder, elbow, femur

Hand-Foot Syndrome - tender, warm, swollen hands and feet

Acute chest syndrome - infection, pain in chest, infarction, respiratory distress

11-20% can experience cerebral vascular accident (CVA), stroke, before age 18 (preceded by headache and decreased school performance)

Priapism - obstruction of penile veins, persistent erection, urinary retention



Sequestration crisis:

Mortality rate of 50%

Excessive pooling of blood in the liver and spleen - as blood leaves circulation results in shock

This can be fatal as the spleen can hold 1/5 bodies blood supply

Treatment for repeated sequestration crises removal of spleen.







Aplastic Crisis:

Decrease in erythropoiesis, despite shortened life of sickle RBCs and need for increase.

Often preceded by infection, human parovirus B19



Decreased blood flow to organs (eyes, GI, kidneys, spleen, heart, brain, lung) can result in blindness, GI upset, abdominal pain, enuresis, immune suppressed, rheumatism, paralysis, pnuemonia.

* Children with SCA and SCT can have vaso-occlussive episodes and hypoxia caused by shock, exercising at high altitudes, cold temperatures, or undergoing anesthesia.



Diagnosis:

In newborn metabolic testing
Lab work as a result of symptoms


Nursing Diagnosis:

Acute Pain
Ineffective tissue perfusion
High-risk for infection
Ineffective coping of child and family

Outcome Identification:

Child will report relief of pain
Child's anemia will improve
Child will be afebrile
Caregivers are aware of resources



Nursing Interventions, Planning And Implementation:

Pain management around clock, not prn
IV, hydration
Sequestration crisis results in the administration of RBCs
Spleenectomy if frequent sequestration
Prevention of sickling by avoiding infection, acidosis, dehydration, constricting clothing, exposure to cold, high altitudes
Immunizations
Prophylactic oral penicillin until age 5
Blood transfusions ( watch for iron overload)
Heating pad, not cold to painful areas
Activity prn
Passive R.O.M. exercises
IV fluids
frequent handwashing
blood administration per protocol
Possibly oxygen administration
Resource identification, coping assistance

Family education:

Why sickling occurs?
Importance of good nutrition, handwashing, prevention through antibiotics and immunizations
Maintaining hydration
What to look for: fever, pallor, pain, enlargement of spleen
School needs to be aware
Sports take care with injuries and hydration

Here is your patient for today. Isn't she beautiful. Katelin has sickle cell disease. She is a bright active young lady.


Katelin is doing a paper in her life science class about Sickle Cell Disease, while she is pretty adept at handling her disease, she has some questions about the actual physiological cause and mechanisms. Explain to her why the cells sickle and why that causes problems.

Explain to Katelin how she can best manage her disease with her athletics. She often is outside for these events, what implications does this have on her care?

Katelin wants to be a nurse. We need nurses! Teaching is a huge part of a nursing career. Teach Katelin about the physiology behind her disease.

When discussing management of her sickle cell disease during a crisis, what might you expect Katelin to say?

Her friend has had a stroke and a spleenectomy. Is this common for this disease? Explain why? What are some other complications which can occur?

Is there a cure? The promise of stem cells from cord blood







Your sickle cell patient today:











Splenic Sequestration located at
http://youtu.be/-8AKGLgSFfw

Fox News 5 Reports on Sickle Cell located at
http://youtu.be/4dq7ieN3xqw

Cord blood Stem Cell Transfusion located at
http://youtu.be/SGpQI2yvBcM

Sickle cell crisis
https://youtu.be/8lLCEs1DMbo
https://youtu.be/Y3eWxtKUE90