Hematological Disorders (Peds)

classifications of anemias

1. decreased RBC production

-nutritional or bone marrow failure

2. increased RBC loss

-blood loss

3. increased RBC destruction

-sickle cell

management of hematologic disorders

1. treat underlying cause

2. occult blood in stole

3. may need multiple blood draws and heel sticks

- be honest and consider use of emla cream

4. allow rest periods

5. prone to infection -> teach hygiene, crowds and nutition

sickle cell anemia

1. a genetic disorder that causes RBCs to sickle to depranocytes (elongated) under stress

2. they vaso-occulde or get stuck together blocking blood flow

3. they then go through hemolysis or break apart easily (call this RBC destruction) leading to anemia

- common in african americans

- 1 in 4 chance of getting if both parents have

4. will have chronic anemia (Hgb 6-9)

causes of stress that lead to RBCs sickling...

1. dehydration

2. hypoxia

3. acidosis

diagnosis of sickle cell anemia

newborn screening

vasocclusive crisis

painful episode characterized by ischemia causing mild to severe pain that lasts minutes to days

-hard to control pain that is recurrent

-no ice= vasoconstricts more

Sequestration Crisis

large amounts of blood pool in the liver and spleen causing decreased blood volume and hypovolemic shock

signs of hypovolemic shock

1. hypotension

2. tachycardia

3. weak pulses

4. cool extremities

5. poor cap refill

-all are signs of poor perfusion

Acute Chest Syndrome (ACS)

1. check back and abd pain

2. fever

3. cough

4. dyspnea

5. tachypnea

6. retractions

7 declining O2 stats

-from blocked blood flow and inflammation in the lungs

CVA (stroke)

long term complication of unrelieved headache, vomiting, twitching, seizures, inability to move arm or leg, stagger walking, slurred speech and weakness

Factors that precipitate a crisis include...

1. dehydration

2. high altitude

3. cold

4. illness

5. physical strain on the body

Management of sickle cell anemia

1. no O2 unless hypoxic

2. regular blood transfusions for severe anemia (can cause iron overload depositing into kidneys)

3. exchange transfusions-> prevention of CVA

4. splenectomy-> decrease incidence of recurrent sequestrian crises; risk of infection

5. pneumococcal vaccines to reduce infection risk

6. adequate hydration to prevent sickling

7. PCA for pain management of vaso-occlusive crisis

Iron deficiency anemia

low iron-> low hemoglobin -> low oxygen carying capacity of RBCs-> tissue hypoxia

Pts at risk of iron deficiency anemia

1. premature -> supplemented at 2 mos

2. exclusively breastfed -> supplement at 4 months

3. toddlers-> switch to cows milk at 12 months; if do not have enough in diet become deficent

4. teens -> fad diets, poor eating habits, strenuous activities

5. girls-> lose blood during menstural cycles

Diagnosis of iron deficiency anemia

1. decreased hgb/hct

2. decreased ferritin

3. increased TIBC

manifestations of iron deficiency anemia

1. fatigue and activity intolerance

2. pallor and cool skin

3. brittle, spoon shaped nails

4. dizziness

5. headache

6. tachycardia

7. poor appetite

Iron supplements

1. given orally in 2 divided doses between meals

2. best absorbed on an empty stomach in an acidic enviornment

3. can take with fruit juice

4. do not give with milk products

5. liquid iron-> straw or syringe to back of mouth and brush teeth after.

6. check hgb at 1 month, treat with supplements for 3 months

IM and IV iron

IM= Ztrack, do not massage, can stain skin

IV= will give test dose to watch for allergic reactions

Iron stools

dark, greenish black stools

- if not present could indicate noncompliance

- hemocult monitored but not every time get a dark stool

If no improvement in Hgb check for...

bleeding or noncompliance

diet for iron deficiency anemia

1. iron fortified formula or cereal

2. meats (beef, chicken, fish)

3. wholegrain breads

4. vegetables

5. eggs

6. chickpeas

7. nuts

allergic reaction from blood transfusion

1. hives

2. itching

3. difficulty breathing

4. airway closure

hemolytic reaction from blood transfusion

1. blood in urine

2. flank pain

Thalassemia

Inherited Hgb defect that leads to destruction of fragile RBCs

-higher rates in mediterranean descent

early manifestations of thalessemia

1. pallor

2. poor feeding

3. enlarged spleen and liver

chronic signs of thalessemia

1. headache

2. activity intolerance

3. anorexia

4. chest pain

5. bone pain

6. delayed growth

7. enlarged head with flat nasal bridge if untreated

8. small stature

9. delayed sexual maturation

10. bronzed freckled complexion

Treatment of thalessemia

1. transfusions every 3-5 wks to keep hgb > 9.5

2. chelation therapy to prevent iron overload from frequent blood transfusions by removing metal from the body

3. splenectomy for fewer transfusions

-prophylatic antibiotics and vaccines

4. all families should be tested for the trait

considerations for chelation therapy

-side effects= GI upset, headaches, joint pain, renal dysfunction

-promote hydration while on therapy

Hemophilia/Von Willebrand Disease

Inherited deficiency of specific clotting factors causing prolonged bleeding

Manifestations of Hemophilia/Von Willebrand Disease

1. hemorrhage from trauma

2. excessive bruising

3. hematomas

4. hemarthroses -> internal bleeding into joints

-pain, warmth, swelling, decreased ROM

-may cause bone deformities from reccurent episodes

5. hematuria

complications and emergencies from hemophilia

1. bleeding in neck/mouth/chest-> concern for airway obstruction or aspiration

2. intracranial bleed-> headache, slurred speech, LOC changes

3. spinal hematomas-> can cause paralysis

4. GI bleed-> black tarry stools, decreased H&H, abd pain

Main treatment for hemophilia

replacement therapy of missing clotting factor

activity and joint care in hemophilia

1. exercise and physical therapy strengthens joints and prevents bleeding

2. do not do during an active bleed

3. do RICE for hemarthosis (joint bleeding)

what should be avoided in the care of hemophilia?

1. im injections if possible

2. heel sticks

3. aspirin and NSAIDS

4. invasive procedures

Safety and hygiene precautions for hemophilia

1. soft toothbrush

2. water flosser

3. electric razor

4. medical alert bracelet

5. noncontact sports

6. teach older children self-infusion

how to manage an active bleed with hemophilia pts

1. apply direct pressure

2. immobilize and elevate the injured area

3. after bleeding stops, start active rom

HIV

retrovirus attacks CD4 and T-lymphocytes, suppressing the immune system, increasing risk for infections and malignancy

-chronic by manageable with therapy

transmission of HIV

1. perinatal

2. sexual

3. IV drug use

4. blood or bodily fluid exposure

Manifestations of HIV

1. liver and spleen enlargement

2. parotitis (parotid gland swelling)

3. swollen lymph nodes

4. oral candidiasis and trouble eating

5. chronic diarrhea and n/v

6. failure to thrive

7. developmental delays

8. muscle pain

9. rashes

Diagnosis of HIV

- 18 + monthes = ELISA + western blot blood tests

- <18 months = PCR testing

- rapid test results in 30 mins; get confirmed with western blot or another rapid +

AIDS diagnosis

Below a certain CD4 level

associated with certain diseases: pneumocystis carinii pneumonia, lymphoid interstitial pneumonitis,recurrent bacterial infections, wasting syndrome, candida esophagitis, HIV encephalopathy, Cytomegalovirus, Mycobacterium avium-intracellular complex infection, pulmonary candidiasis, herpes simplex disease, cryptosporidiosis

antiretroviral therapy (ART)

drug combo to suppress virus at all stage of HIV cycle

-suppresses viral replication and prevent and prevents immune system deterioration

- CD4 count and viral load done routinely to monitor disease progression and response to therapy

- hard on GI system so check compliance

Management of HIV

1. pneumococal and flu vaccines to prevent infection

2. MMR/varicella only if not currently immunocompromised

3. can attend school or daycare; parents do not have to report

4. should follow standard precautions

5. educate safe sex, drug needles

6. nurses should always wear gloves

Nutrition for HIV

-High calorie, high protein

-may have nutritional issues due to GI symps and oral thrush