Hematological Disorders Flashcards
What is epitaxis and what is its treatment?
- Nose bleed
- Lean forward
- Pinch nose for 10mins
- Petroleum jelly can be used to moisten nares and prevent recurrence
What is the most common hematological disorder of childhood?
Anemia
What is anemia?
- Decrease in number of RBCs
or
- Decrease in hemoglobin concentration
- Result in either case is poor tissue perfusion
Relative hemoglobin levels for adult males and females?
- M = 14-18
- F = 12-15
- # s will vary by sources
- Children’s #s will be lower
TRUE or FALSE
The body cannot adapt to anemia.
FALSE
- If anemia ocurs slowly, the body can adapt
- ex. Move to denver from a low elevation
What are the basic s/s of anemia?
- Fatigue
- Pallor (pale)
What are some risk factors for Iron-Deficency Anemia?
- Diet high in milk
- Poor dietary intake of iron
- Blood loss
Why is too much milk a bad thing?
- It binds to iron and causes it to get excreted from the body reducing serum levels
What is a negative effect of cow’s milk on the GI system?
- it can cause microbleeds
Why should toddlers eat BEFORE they get their milk?
- So they get their nutrients from food before filling up on milk
What is the max amount of daily milk for toddlers and teens?
- Toddlers = ≤ 32oz
- Teens = ≤ 3 cups (24oz)
What is the treatment for iron-deficiency anemia?
- Treat the underlying cause
- Educate the family
What is our supportive care for severe cases of iron deficiency anemia?
- Intravenous (IV) fluids to replace intravascular volume
- Transfusion may be needed
- Oxygen
- Bed rest
What are the items to remember regarding iron supplements?
- Liquid admin for kids - tastes terrible
- stains teeth so give with straw and
- brush teeth immediately after
- Tablets for older kids
- Take supplements w/ vitamin C to help absorption!
What is the milk rule regarding iron supplements?
None for an hour or two before and after meal
What do we need to keep in mind for safety regarding iron supplements?
- IRON is extremely easy to overdose for kids
- Should be treated as a dangerous drug out of reach (even though its OTC)
- Hide grandmas iron pills!
What is Aplastic Anemia?
- Bone marrow failure!
- Pancytopenia with triad of
- Anemia
- Thrombocytopenia
- Leukopenia
Explain pancytopenia, each component and its s/s.
- Pancytopenia is the destruction of RBCs, WBCs and Thrombocytes (platelets)
- RBC destruction leads to poor oxygen perfusion
- s/s of hypoxia
- WBC destruction leads to susceptibility to infection
- s/s of poor immune response
- Platelet destruction leads to low clotting factor
- s/s unsual bleeding
What is the cause of aplastic anemia?
We don’t know. It is idopathic.
However, some cases that have caused:
- Overwhelming infection
- Radiation
- Industrial/household cleaners
What is the medical treatment for Aplastic anemia?
- Immunosuppression if autoimmune cause is suspected
- Bone marrow transplant
What are the key points regarding bone marrow transplant?
- Transplant (better success rate the earlier its done)
- 85% survival rate
What happens if you wait on a bone marrow transplant for aplastic anemia, what happens?
- reduces survival right to 70% as the child would to the human leucocyte antigen (HLA)
- body may now see it as “this isn’t me” and reject the new marrow
If both parents have the sickle cell anemia trait, what are the chances each child will have of getting it?
25%
What are the key points to keep in mind regarding sickle cell anemia?
- sickled cells can’t carry enough 02, leading to decreased perfusion
- sickled cells can stick together and cause blockages
When sickled cells stick together and create blockages, what happens?
- Tissue hypoxia
- Infarction (tissue death)
- Increased stress which causes even more sickling
SCA exacerbations can result in what 3 things?
- Vaso-occlusive crisis
- Sequestration crisis
- Infection
Describe the vaso-occlusive crisis of SVA.
- sickled cells cause blockages that result in a painful episode from hypoxia
- Leads to
- Acute Chest Syndrome (similar to pneumonia)
- crisis clogs chest
- leading cause for death in SCA
- CVA (Cerbrovascular Accident - stroke)
- Crisis clogs brain
- Infection
- Acute Chest Syndrome (similar to pneumonia)
Describe the sequestration crisis of SVA.
- crisis will clog the spleen or liver causing blood to pool in the organ and can cause vascular collapse
What is sometimes done to prevent a sequestration crisis of the spleen from occuring.
When/why else would this be done?
- A splenectomy would be done to prevent the sequestration crisis
- This would also be done with a spleen that has been shredded by SCA
What happens as a result of a splenectomy?
It leaves the pt @risk for infections
What is the treatment for SCA?
- Treatment aimed at reduce sickling
- Teach family stressors to avoid
- Blood transfusions/ exchange transfusion in severe cases
In some blood transfusions for SCA, why would Desferal/deferoxamine be given?
- Pt may have high iron serum levels due to xfusions.
- Desferal gets rid of excess.
- Must be infused over several hours
Blood xfusions can decrease sckling episodes. Which pts may need them every 3 wks for the rest of their lives?
- Those with a history of CVA or ACS
What is important to keep in mind regarding hydration and a pt in SVA crisis?
- Give them 1.5x’s normal maintenance dose to help clear the blockages
Pain is a huge issue with SCA. What are the key points regarding medication?
- want to start meds as soon as possible,
- give PCA if they’re old enough
- want them to feel in control
- nsaids, motrin, ibuprofen and opioid med is typical
What is our education to parents who are concerned about their child getting addicted to opioids?
- behavioral addiction is incredibly rare
How is Beta Thalassemia, “Cooley’s Anemia” acquired?
inherited from parents
What is happening in Beta Thalassemia?
- Red blood cells hemolyze and become non-functional
- Body makes more RBCs to compensate
- Inside the bone marrow AND
- Outside the bone marrow (extramedullary hemotopoiesis)
How does Beta Thalassemia affect oxygenation?
Constant creation and destruction of RBCs and not enough oxygen-carrying hemoglobin to serve the body well
Simply put, what is Beta Thalassemia and what does the process lead to?
- Constant descruction and production of RBCs result in poor perfusion
- Process leads to
- Brittle bones, hepatosplenomegaly from the process
- Delayed growth
- Facial deformities
What are the key points of Hemophilia to keep in mind?
- X-linked recessive pattern
- Only males get it
- Males/Females can carry
- Factors VIII and IX help create clots
- Don’t do things that make ya bleed
What are the key points to know for Von Willebrand Disease?
- Type of Hemophilia
- VW protein helps for clots
- VW Disease results in not enough or dysfunctional VW protein which leads to excessive bleeding
What is the treatment of Von Willebrand Disease?
- Goal: minimize episodes of bleeding
- Tx: DDAVP (desmopressin), which stimulates the release of VW factor
Symptoms of hemophilia may not occur until what age?
6mos
What are some s/s of hemophilia?
- Frequent and larger bruising and nosebleeds
- Hematuria
What is the challenge with pts with hemophilia?
- Internal, non-visible bleeding
- Need to know the s/s
What is most important regarding a head injury for a hemophilic pt?
Monitor for ICP
What is Hemarthrosis and what are the s/s?
- Bleeding into joint spaces
- S/s include warmth, pain, bruising, and decreased movement
What is a target joint?
- Hemarthrosis in the same joint on 4 different occasions w/in 6 months
What is the treatment for Hemarthrosis?
- RICE
- Analgesics
- Nsaids work really well, but need to be careful as nsaids can reduce production of platelets
- ROM exercises after bleeding stops to prevent contractures
- These are done by the pt, not us
- We encourage it when appropriate
- Physical therapy
What is the treatment for Hemophilia?
- Primary therapy is replacement of missing clotting factors
- Desmopressin (DDAVP) - for mild cases
- Increases Factor VIII activity
- Transfusions
- if loss of too much blood
- Prompt intervention to reduce complications
- Exercise and physical therapy
- No cure, just control of symptoms
What do we educate re: activity for our pts with hemophilia.
- physical activity is important, they just need to be safe
- No contact source
How does weight relate to hemarthrosis?
- Too much weight caues xtra stress on joints and increases incidence of hemarthrosis
What hygeine warnings do we give for pts with hemophilia?
- soft toothbrush
- No metal razors
TRUE or FALSE
Aspirin is ok for people with hemophilia.
FALSE
- Asprin increases risk for bleeding
- We also need to educate the pt that aspirin is also in things like alka-seltzer and pepto bysmal
What is Idiopathic Thrombocytopenic Purpura (ITP)?
- An aquired hemorrhagic disorder that is characterized by:
- Thrombocytopenia (destruction of of platelets - PLT<20K
- Purpura (advanced peticea)
- NORMAL bone marrow
The cause of ITP is unknown, but it usually follows after a ….
viral infection
What is the treatment for ITP?
- IVIG
- Prednisone - to help body settle down and minimize platelet destruction
What is IVIG
- A super-concentrated and very diverse collection of antibodies against many possible infectious organisms your body might encounter.
- Administered via IV
Explain Acute vs Chronic ITP.
- Acute usually follows a viral infection and is self limiting
- Chronic will also resolve itself but will last 12 months or longer
What is a sign of DIC, why is it so important to recognize and how is it treated?
- Systemic bleeding all over
- Can be internal, external at puncture points, etc
- The next step is death
- Treated by tending to the underlying cause
Overstimulation of clotting has 4 major effects. What are they?
- Bleeding
- Organ damage
- Anemia
- Tissue hypoxia
All kids should be screened for lead poisoning by what age?
12mos
What are the CNS s/s of lead poisoning?
- Hyperactivity,
- impulsiveness,
- lethargy,
- irritability,
- loss of developmental progress,
- hearing impairment,
- learning difficulties
What is the treatment for lead poisoning and how does it work?
- Chelation therapy
- Uses special drugs that bind to metals in your blood and is then excreted through urine
