Second Half of May 8 lecture-sorry it is late

Blood
White blood cells
Red blood cells
Platelets

Anemias

Sickle cell

White-three granular
Basophils-contain Heme and ???
Eosinophils-allergies and parasites
Neutrophils-most abundant

Two agranular
Monocytes-most efficient
     Macrophage-eat debris
Lymphocytes-two types
T-cells recognize foreign antigens, stop the immune response
B-cells produce antibodies

Trigger to produce more red blood cells
Hypoxia-----kidneys produce erythropoietin—stimulate bone marrow to produce RBC

Hematology

Red blood cells 4 types
Type          Antigen         Donor
_________________________________
A              A           A and O
B                B           B and O
AB             A and B      A, B, AB and O
O                None          O

Rh factor- D antigen-have it or don’t.
Negative has none
Positive has

Can’t mix Rh factors

When mother has negative and fetus positive-can have mixing in utero
Can get injection called RhoGAM to prevent antibody formation
In utero can have baby’s cells attacked by antigens-erythroblastosis fetalis

Blood types come from parents- Mother A+; Father B+-baby cannot be O (unless there is another man in the equation)

Blood transfusion reactions:
Can have reaction up to one week to ten days later-usually not as severe
What do you need to know when giving blood?
VS:      temperature
     Rash-need to look before gives blood,

Can’t microwave blood.
Blood can only be held for 30 minutes

If patient is having a reaction:
Stop transfusion
Make sure line is patent with normal saline-can flush with the blood –Y if not a SOB problem

Kids that get transfusions:
Anemia-sickle cell
Leukemia

Premedicate: Tylenol and Benedryl because you know they will have reaction

If Jehovah’s Witness the court can intervene and the child can be given blood in life threatening situation. The child becomes the ward of the court for the blood transfusion. Not considered child endangerment if it is a religious decision. Auto transfusions are not acceptable. When drawing from central line, usually do waste draw and put it back after the draw, can’t on JWs; must waste it.

Most common hematologic problem in peds

When does newborn run out of iron reserves: 5 months but may not show till around 9 months. Happens when baby is switched over to cereal and don’t cut back on milk (Mild babies-doughy, pasty, and lethargic), drink several bottles of milk a day put on cow’s milk without iron. Usually is treated with iron fortified food and cutting back on milk.
What happens to kids that continue to be low on oxygen?
Increased RBCs, small child, and slow brain development

Hemophilia-blood coagulation disorders
Secondary to Factor 8 (A)-most common or Factor 9 (B or Christmas disease)
How do you get it?
X-linked recessive from Mother
Mother carrier-has two X chromosomes and are protected from the disease.
Replacement used to be whole blood now just the factors

Most common sites of injury are joints
Most hemophiliacs are expected to live normal, full life, before blood screening died from AIDS
One 1:10,000 births
Picked up when? Prolonged bleeding from circumcision or oral mucosa or bruising over bony prominences.
If they come into hospital with bleeding in joints the #1 complaint is PAIN. Need lots of pain relief. Then swelling and mobility problems.
Where it bleeds can lead to complications:
Intracranial bleeding
Renal impairment

Will have increased reactions to blood products
Normal bleeding times but have increased PTT and to confirm the dx have functional assays of blood to determine lack of factor.

Pain med: Tylenol, not ASA or NSAIDS
Use ice on joints not heat-heat will increase bleeding.

Von Willebrand’s affects women, may not know it, can have long menstrual periods and bleeding with surgery.

Sickle cell anemia
Complication: clogging of circulation secondary to shape of cell

Causes of crisis:
Dehydration-fluid replacement. May get one and a half to two times the rate of fluids instead of usual maintenance rate.
Acidosis
Fever
Hypoxia-oxygen. Unlike respiratory kid, will often stay on O2 with sat of 100% to prevent sickling

Complications:
Heart failure
Infection from tissue infarction
Pan foot-Circulation cut off to feet
Kidney disease
CVA
Osteomyelitis
Lungs

How does the person get the disease?
Autosomal recessive-need trait from both parents
25% chance to get disease, 50% home free, 25% carriers

RBCs normally last 120 days, Sickle cells-30 to 60 days
Sickle cells (Hemoglobin S) increase when fetal hemoglobin (hemoglobin F) runs out at about 6 months.
Treatment now is with Hemoglobin F (fetal) Does not sickle.

Things you need to know in hydration:
Avoid cold and vasoconstriction during sickle crisis-can increase pain.
Avoid altitude-low O2 secondary to increase in atmospheric pressure.
Sickle cells originated in Africa, the mutation resisted infection with malaria.

Usually on low dose prophylactic antibiotics from about the time of birth-PCN, can be on it up to around age 6.
Folic acid-promote red blood cell production

Crisis causes pain. Can cause loss of blood flow to extremities that leads to ischemia and causes pain.

Not all the cells in the body sickle at the same time.

Thallassemia-Greek word meaning anemia by the sea
Group of Genetic blood diseases. Life long and often life threatening illness. About 100,000 people born every year with the severe form of the disease.
Two main types- alpha and beta depending on the part of the O2 carrying protein
Beta most severe
Affects: South East Asia, Chinese and Pilipino ancestry, Prevalence in Italian, Greek and Mediterranean population

Can result in fetal or new born death

What is it?
Hereditary hemolytic anemia
Deficiency in the synthesis of the hemoglobin  poly peptide chain

Happens in response to anemia:
Overproduction of erythropoietin- erythroid hyperplasia, expanded marrow space, enlarged liver and spleen and severe anemia
Liver, cardiac and infectious complications

Hemochromatosis: excessive iron in the tissues
Needs lots of transfusions to stay alive; the iron is deposited in the tissue secondary to the transfusions. Can be toxic

Because of wide marrow space have increased risk of bone deformities and fractures.

Jaundice secondary to RBC breakdown
CHF (due to increased work load) and cardiac arrhythmias

Treatment:
Deferoxamine (Desferal): a chelating agent
Binds to iron and eliminates it from the body.
Need frequent treatments.

ITP:
Idiopathic thrombocytopenia purpura: no known cause, low platelets in blood, bleeding.
May be caused by virus that damages platelets.
Usually Self-limiting
Viral illness---drop in platelets---bleeding.
Platelet count may be as low as 20 thousand.
May be on Prednisone for anti-inflammatory effect, IgG can help elevate platelet count
Most go into remission.
Adults can get

Leukemia: cancer of the blood and blood forming elements
Main point: production of abnormal WBCs that don’t do their job, crowd out the working cells.

Problems:
Frequent infections.

If low RBCs: anemia
If low platelets: bleeding

Test: bone marrow to diagnosis
CBC
Will see high blast content on count. Then bone marrow to dx.

See handout of effects of chemotherapy drugs-poisoning your body (If cancer does not kill them the drugs may)

Cause death of rapidly growing cells-child full of rapidly growing drugs—how does the med know the difference.
One effected area is the hair follicles-loss of hair – alopecia
Hair may grow back different

Nausea and vomiting: very common
Pre-medicate and don’t push food.
Let them eat what when they feel like it.

Some chemo agents target the bladder and the heart: can have effect for entire life

ALL-the most common in children- acute lymphocytic leukemia
AML-acute myelogenous
Chronic and acute
Leukemia in children has higher cure rate than in adults.
Now about 80% cure rate.
Used to do chemo and then wait out remission and hope
Now:
Give drugs and once in remission do lumbar puncture and do intrathecal intensive to hit central nervous system even if no signs.
Do maintenance for longer: two to three years. Chemotherapy at intervals during that time.
Cures them.
Problems: more problems, Infertility, infections in PICC lines, hearing loss from Gentamicin as part of triple antibiotics


Low platelets: don’t give hard toothbrush

Hodgkin’s disease: survival rates good particularly in early disease. Seen primarily in young adults, but can appear in children.
Painless but possible movable lymph nodes, fever, weight loss, malaise
May be treated with radiation and chemo, depends on stage.

Non-Hodgkin’s: more highly malignant. The whole lymphoid system is lymphoid tissue. Aggressive and wide spread involvement but usually a quick response to treatment.  Does not have to be fatal.

Neuroblastoma:
22 mos average age.
Solid tumor
Originates from sympathetic nervous system tissue.
Primary site is abdomen.
Most common extra cranial solid tumor (Brain tumor most common solid tumor)
Approx 500 new cases/year

Abdominal-altered bowel/bladder function
Often metastasis prior to dx

Surgery:
Tumor resection
Chemo
Radiation

Stage 1 &2 good survival
Stage 3-4 10-30% survival

Rhabdomyosarcoma:
Usually toddler/preschool
Particularly seen in head or neck as hard non-tender mass


Retinalblastoma: eyes
Can be congenital
Can be present at birth or can developed later up to two years-may or may not be hereditary
Can see whitish glow-(cat’s eye reflex) in eye/eyes
Can see with ophthalmoscope for final diagnosis
Try to preserve function of eye and vision and get rid of tumor
Advanced stages may have enucleation done esp. if optic nerve involved
90% survival rate
Increased chance of secondary tumors in lungs-lung cancer

Wilms' tumor
Separated from kidney sits on top of kidney
Never want to palpate abdomen if suspect because the tumor can seed and cause metastasis-pain, HTN signs
Major surgery needed, need to remove kidney-usually unilateral; may need to follow up with chemo and radiation
Pretty good prognosis unless metastasis

Osteogenic sarcoma- usually more localized.
Mets even with amputation
Femur most common site, followed by knee, tibia and humerus
Adolescent boys-may have pain and ignore it, have fx that finds the tumor

Rotationplasty: resect the tumor including the knee joint with the knee rotated 180 degrees and re-transplanted to the thigh creating a shorter leg with the ankle joint now the knee joint

Supposed to allow for a normal life.

All the stuff that can go along with poss amputation: body image would be big.

Ewing’s sarcoma:
Much poorer prognosis and is generalized to bones in body. Diffuse

Brain tumors:
Second category of tumor-about 5% of tumors
Depends on what portion of brain what type of symptoms that you will see
Children can’t tell you symptoms.
Nausea, vomiting, gait disturbances (hard to tell in very little kids)
Look for signs of increased intracranial pressure.
May go to surgery have tumor removed and have shunt put in.
Where it is and how treated leads to outcome

Usually don’t radiate the brain of children under 3 years because the brain is still developing

Test question:
How do you treat brain tumors in general?
Radiation, chemo and surgery if operable

Some may be inoperable, may be in brain stem or wrapped around structures.