HEME
*Anemia
Decrease in the total number of RBC’s and or Hgb for age/sex.
Can be caused by decreased RBC production or increase in RBC destruction, increased RBC loss1
Clinical manifestations of anemia: tissue hypoxia, pale skin (no cyanosis b/c it happens slowly),
tachycardia, irritable, fatigue, dizziness/headache, depression, and slow thought process (often
seen in teenage girls)
Slower levels fall, better off you do (low RBCs over time, no cyanosis); falls fast= problem (cyanotic)
*Iron Deficiency Anemia
Most common type of anemia in children
Often happens around 12-24 months b/c parent will switch from formula to cow’s milk
Poor nutritional intake
Parent should give iron drops or vitamin (C) drops
Do not give iron drops with milk b/c it doesn’t absorb well GIVE WITH ORANGE JUICE
Best taken on empty stomach at bedtime with vitamin c
Stool change to tarry black with iron
Sources of iron: Spinach, organ meats, raisins, molasses, total bran cereal (without milk)
*Sickle cell anemia
Autosomal recessive – 25% will get it and 50% will carry the trait (both parents carry gene)
Sickle cell trait is protective of malaria (only positive)
Found on newborn screen
Triggered by emotional stress
Sickle and rigid shape + Fever and stress= crisis
These kids need vaccines b/c they commonly get infections
They are often put on prophylactic antibiotics starting at 4 months
Bone marrow will increase production of RBC’s so when you do a CBC RBC level will be high
These patients should not be at high altitude
Puberty may be delayed
Complications:
Cells clump and become vasoocclusive This causes the tissue to die and cause pain
o Vaso-occlusive crisis can occur in any organ (MI= crisis in heart; stroke= crisis in brain)
o #1 cause of stroke in kids (clumping)
Spleen becomes non-essential (garbage collector of clumps) occluded capillaries= no blood
flow spleen enlarges and can rupture, or cause spleen infarctions and spleen death
o Evaluate for fever and vaccines- susceptible to infections
Hand foot syndrome: 2-6 months, lose sensation in hands and feet b/c capillaries are so small
o Swollen, puffy hands and feet= can't do anything because it hurts
Acute chest syndrome: Vaso-occlusion in the lungs- potential lung transplant + BONE MARROW
TRANSPLANT (to make normal cells)- presents like pneumonia
Routine transfusion can cause an overdose of iron causing toxicity. We need to give chelating
agents (effects kidney). WATCH IRON LEVELS
,When a kid comes into the hospital one of the first things we ask is do they still have their spleen- if not
we don’t have to watch for enlargement and abd girth (spleen becomes non-functional and can be
susceptible to infection) spleen may need to be taken out
On a newborn you might feel the spleen (feels like jello cube under the skin)
Bone marrow increases production of RBCs but they are damaged so it isn't really helping
Delayed puberty= delayed physical growth
Treatment (hypoxic cells) (tx like MI)
1st!! Give pain meds- morphine for vasodilation
Then Give Oxygen (100% O2) for about 15-20 min.
Hydrate with IV fluids + whatever they want to drink
Re-oxygenate, rehydrate, Bed rest
Transfuse RBC’s (don’t give vaccines if you’re going to transfuse) to maintain Hgb over 10
o Chelating therapy with desferral (iron chelating agent) to prevent accumulation of iron in
tissues/organs (admin with transfusions)
Pain- Wong pain scale; morphine initially; switch to oxycodone, methadone, dilaudid; avoid activities
requiring mental alertness (aka driving)
PREVENTION!!
Frequent bed rest- reduce O2 demand
Don’t climb mountains (avoid high altitudes)
Avoid infection and sick people
Prophylactic penicillin
Give vaccines
Well hydrated – Tell them to drink 2 quarts of water a day
Avoid extreme heat or cold
Adequate nutrition
Parental education on management & assessment!!
*Aplastic anemia
Bone marrow failure- Need WHOLE BLOOD transfusions b/c your body doesn’t make anything
Can be born with it or acquired
Bleed easily and get infections because they don't have enough WBC
Congenital Aplastic anemia= Fanconi’s anemia.
Acquired Aplastic anemia in children comes on slowly (drugs like sulfas or NSAIDs, chemicals like
benzine, lead, radiation, insecticides, viral infections (Hep, EBV))
Diagnosis: During bone marrow aspiration you don’t get good red bone marrow it comes out yellow
and fatty instead of deep red
Assessment:
Petechiae/prupura, bleeding
Pallor, weakness, fatigue
Tachycardia
Often die from transfusion reaction
Do bone marrow transplant in infancy (transplant ASAP)- EMERGENT, but finding donor takes time
HIGH MORTALITY
*β- Thalassemia
, Body kills its own red blood cells (bone marrow/spleen destroys Hgb); common genetic disorder
Severe hemolytic anemia (can only be fixed with repeated blood transfusion)- designated donor
o INCOMPATABILE with life No transfusions= death
o Transfuse on a regular basis + Chelation therapy (Desferal) to remove excess iron
Often in Mediterranean – middle east, Africa, Turkey
They need chelation b/c they will have an iron buildup
Aplastic crisis can happen after transfusion (due to not making bone marrow)- makes you weak,
drops blood count
Splenomegaly
Chronic hypoxia, severe fatigue
Facial feature of thalessemia (look different from rest of family)- bones thicken
o Prominent facial bones, chipmunk like mouth, prominent maxillary bones, protruding front
teeth, greenish/yellow skin tone, wide set eyes with a flattened nose
Folic acid deficiency, severe osteoporosis as kids
Susceptible to pathologic fractures (ex. trip on stairs and break leg)- super brittle bones
Delayed growth and sexual maturation
Congenital heart failure (myocardial fibrosis)
Fibrotic pancreas- turns into Diabetes M. type 1
Do a bone marrow transplant in infancy b/c they haven’t had any transfusions yet or antibodies
from other people’s blood
*ITP
Idiopathic = cause is unknown
Thrombocytopenic = decreased platelets
Purpura = excessive bleeding / bruising/ pinpoint bleeding (petechiae)
Hospitalized if platelets < 20,000
We don’t transfuse platelets b/c their body will just kill them (platelets disappear)
Only give platelets if they are ACTIVELY bleeding and need them to stop
Problem= very low levels causes bad internal bleeding- can bleed into your brain
Usually set off by a viral illness that triggers something (ex. URI, Varivax/MMR, EBV)
80% resolves spontaneously
DO NO HARM
Don’t do IM or rectal temps, no vaccines
No contact sports
Don't give NSAIDs- ex. Motrin or Advil (ibuprofen)
o Antiplatelets
No ASA
Monitor: guaiac stool, monitor neuro status, urine analysis for blood, monitor sx for blood (mm, sclera,
epistaxis, hematuria, stool)
*Hemophilia A
They bleed longer, not faster and easily
NOT a platelet issue it’s a FACTOR 8 issue Factor 8 is produced in the liver (can't clot)
X-linked inherited (so boys usually get it not girls)- females are carriers
In 80’s and 90’s many of them died of HIV from infusions
Be very careful to prevent injury
o Head trauma is dangerous- bleeding in contained space creates pressure
*Anemia
Decrease in the total number of RBC’s and or Hgb for age/sex.
Can be caused by decreased RBC production or increase in RBC destruction, increased RBC loss1
Clinical manifestations of anemia: tissue hypoxia, pale skin (no cyanosis b/c it happens slowly),
tachycardia, irritable, fatigue, dizziness/headache, depression, and slow thought process (often
seen in teenage girls)
Slower levels fall, better off you do (low RBCs over time, no cyanosis); falls fast= problem (cyanotic)
*Iron Deficiency Anemia
Most common type of anemia in children
Often happens around 12-24 months b/c parent will switch from formula to cow’s milk
Poor nutritional intake
Parent should give iron drops or vitamin (C) drops
Do not give iron drops with milk b/c it doesn’t absorb well GIVE WITH ORANGE JUICE
Best taken on empty stomach at bedtime with vitamin c
Stool change to tarry black with iron
Sources of iron: Spinach, organ meats, raisins, molasses, total bran cereal (without milk)
*Sickle cell anemia
Autosomal recessive – 25% will get it and 50% will carry the trait (both parents carry gene)
Sickle cell trait is protective of malaria (only positive)
Found on newborn screen
Triggered by emotional stress
Sickle and rigid shape + Fever and stress= crisis
These kids need vaccines b/c they commonly get infections
They are often put on prophylactic antibiotics starting at 4 months
Bone marrow will increase production of RBC’s so when you do a CBC RBC level will be high
These patients should not be at high altitude
Puberty may be delayed
Complications:
Cells clump and become vasoocclusive This causes the tissue to die and cause pain
o Vaso-occlusive crisis can occur in any organ (MI= crisis in heart; stroke= crisis in brain)
o #1 cause of stroke in kids (clumping)
Spleen becomes non-essential (garbage collector of clumps) occluded capillaries= no blood
flow spleen enlarges and can rupture, or cause spleen infarctions and spleen death
o Evaluate for fever and vaccines- susceptible to infections
Hand foot syndrome: 2-6 months, lose sensation in hands and feet b/c capillaries are so small
o Swollen, puffy hands and feet= can't do anything because it hurts
Acute chest syndrome: Vaso-occlusion in the lungs- potential lung transplant + BONE MARROW
TRANSPLANT (to make normal cells)- presents like pneumonia
Routine transfusion can cause an overdose of iron causing toxicity. We need to give chelating
agents (effects kidney). WATCH IRON LEVELS
,When a kid comes into the hospital one of the first things we ask is do they still have their spleen- if not
we don’t have to watch for enlargement and abd girth (spleen becomes non-functional and can be
susceptible to infection) spleen may need to be taken out
On a newborn you might feel the spleen (feels like jello cube under the skin)
Bone marrow increases production of RBCs but they are damaged so it isn't really helping
Delayed puberty= delayed physical growth
Treatment (hypoxic cells) (tx like MI)
1st!! Give pain meds- morphine for vasodilation
Then Give Oxygen (100% O2) for about 15-20 min.
Hydrate with IV fluids + whatever they want to drink
Re-oxygenate, rehydrate, Bed rest
Transfuse RBC’s (don’t give vaccines if you’re going to transfuse) to maintain Hgb over 10
o Chelating therapy with desferral (iron chelating agent) to prevent accumulation of iron in
tissues/organs (admin with transfusions)
Pain- Wong pain scale; morphine initially; switch to oxycodone, methadone, dilaudid; avoid activities
requiring mental alertness (aka driving)
PREVENTION!!
Frequent bed rest- reduce O2 demand
Don’t climb mountains (avoid high altitudes)
Avoid infection and sick people
Prophylactic penicillin
Give vaccines
Well hydrated – Tell them to drink 2 quarts of water a day
Avoid extreme heat or cold
Adequate nutrition
Parental education on management & assessment!!
*Aplastic anemia
Bone marrow failure- Need WHOLE BLOOD transfusions b/c your body doesn’t make anything
Can be born with it or acquired
Bleed easily and get infections because they don't have enough WBC
Congenital Aplastic anemia= Fanconi’s anemia.
Acquired Aplastic anemia in children comes on slowly (drugs like sulfas or NSAIDs, chemicals like
benzine, lead, radiation, insecticides, viral infections (Hep, EBV))
Diagnosis: During bone marrow aspiration you don’t get good red bone marrow it comes out yellow
and fatty instead of deep red
Assessment:
Petechiae/prupura, bleeding
Pallor, weakness, fatigue
Tachycardia
Often die from transfusion reaction
Do bone marrow transplant in infancy (transplant ASAP)- EMERGENT, but finding donor takes time
HIGH MORTALITY
*β- Thalassemia
, Body kills its own red blood cells (bone marrow/spleen destroys Hgb); common genetic disorder
Severe hemolytic anemia (can only be fixed with repeated blood transfusion)- designated donor
o INCOMPATABILE with life No transfusions= death
o Transfuse on a regular basis + Chelation therapy (Desferal) to remove excess iron
Often in Mediterranean – middle east, Africa, Turkey
They need chelation b/c they will have an iron buildup
Aplastic crisis can happen after transfusion (due to not making bone marrow)- makes you weak,
drops blood count
Splenomegaly
Chronic hypoxia, severe fatigue
Facial feature of thalessemia (look different from rest of family)- bones thicken
o Prominent facial bones, chipmunk like mouth, prominent maxillary bones, protruding front
teeth, greenish/yellow skin tone, wide set eyes with a flattened nose
Folic acid deficiency, severe osteoporosis as kids
Susceptible to pathologic fractures (ex. trip on stairs and break leg)- super brittle bones
Delayed growth and sexual maturation
Congenital heart failure (myocardial fibrosis)
Fibrotic pancreas- turns into Diabetes M. type 1
Do a bone marrow transplant in infancy b/c they haven’t had any transfusions yet or antibodies
from other people’s blood
*ITP
Idiopathic = cause is unknown
Thrombocytopenic = decreased platelets
Purpura = excessive bleeding / bruising/ pinpoint bleeding (petechiae)
Hospitalized if platelets < 20,000
We don’t transfuse platelets b/c their body will just kill them (platelets disappear)
Only give platelets if they are ACTIVELY bleeding and need them to stop
Problem= very low levels causes bad internal bleeding- can bleed into your brain
Usually set off by a viral illness that triggers something (ex. URI, Varivax/MMR, EBV)
80% resolves spontaneously
DO NO HARM
Don’t do IM or rectal temps, no vaccines
No contact sports
Don't give NSAIDs- ex. Motrin or Advil (ibuprofen)
o Antiplatelets
No ASA
Monitor: guaiac stool, monitor neuro status, urine analysis for blood, monitor sx for blood (mm, sclera,
epistaxis, hematuria, stool)
*Hemophilia A
They bleed longer, not faster and easily
NOT a platelet issue it’s a FACTOR 8 issue Factor 8 is produced in the liver (can't clot)
X-linked inherited (so boys usually get it not girls)- females are carriers
In 80’s and 90’s many of them died of HIV from infusions
Be very careful to prevent injury
o Head trauma is dangerous- bleeding in contained space creates pressure
