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What is Hemophilia A
a hereditary blood disorder associated with a deficiency in coagulation Factor VIII
How do you get hemophilia
It is a recessive disorder where you mom is a carrier and passes it on to her sons
S/S of hemophilia
- bruise easily
- excessive bleeding for losing teeth, minor lacerations and injections
When a child has severe hemophilia what will you see?
- bleeding that occurs in the muscles and joints (hemarthrosis), especially the knees. Reoccurrence of this bleeding in the same joints will cause:
- swelling, pain, bleeding and stiffness
Dx of hemophilia
after circumcision cuz there is prolonged bleeding
but that can be missed so it will be delayed till toddler years since most bleeding occurs in muscles and the joints and we cant see that
will have to wait till they fall and bleed too much
Labs to test for hemophilia
PT, PTT, Fibrin levels, Platelet count
Goal for managing Hemophilia
prevention of excessive bleeding and tissue damage by supplying the body with additional factor VIII which is missing or ineffective
Why aren't blood transfusions given to patients with hemophilia any more?
HIV and Hepatitis risks
What is given to help hemophiliacs? How?
Recombinant Antihemophilic Factor
It is reconsitituted with sterile water and given by IV
Therapies for Hemophilia are starting to be given
prophylactically to delay joint problems
What is given to kids when they are bleeding and how?
Desmopressin Acetate via intranasal spray
What is given to hemophiliacs for nose bleeds and oral clots
Amicar or Cyklokapron
How do you treat bleeding for the hemophiliac?
With a near drowning you will find that the child has
- swallowed a lot of water
- brain damage
Swallowing large amounts of fresh water causes
hyponatremia and cerebral edema
Respiratory support for a child that almost drowned cuz they have rales, rhonchi, are at risk for pulmonary edema, atelectasis and pna
- suction the oropharynx
- Oxygen by mask or bag valve mask
How do you support a childs circulation that almost drowned?
- pulse ox
- cardiac monitor w/assessments regularly
- cover with warm blankets
How do you do a neuro eval on a child that almost drowned?
- check pupil reflexes
- check response to verbal and tactile stimuli
- do they move extremities
How do you decrease ICP in a child that almost drowned
elevate HOB to 30
What fluids will you give if a child almost drowned in fresh water?
How do you help a child recover from acidosis that almost drowned?
How do you remove the fluids swallowed after a near drowning?
gastric tube to decompress
When will you see an infection on a kid that almost drowned?
Infants in an emergency
- crying can be stressful for parents
- paci as comfort
- let parent hold infant as much as possible
- reunite parents with infant asap
Toddlers in an emergency
- wont like being restricted and will push limits
- remove restrictions/restraints as soon as safety permits
- don't understand time....so tell about procedure right before
Pre Schoolers in an emergency
- Need time between explanation of procedure and completion
- Don't use words like stick or cut...scares them
- Include parents in treatments....but don't have them restrain the child
- May blame themselves for problem/injury
School Age in an emergency
- can understand cause of the illness
- can understand simple explanations and make decisions about their own care
- stress importance of injury prevention behaviors
Adolescent in an emergency
- still may not be emotionally mature
- age of risk taking...but can be fearful of death
- consider themselves invincible
- privacy is important so approach them as you would an adult
General poison information to gather....
- was the child found ingesting substance
- what time did it occur
- how did the event occur
- substance involved
- where it occurred
- any previous episodes
- any other medical problems or allergies
Priority management with poisoning
ABC...then prevent and treat shock
Treatment for Tylenol ingestion
- Mucomyst IV or PO
- IV fluids
- Diet-sodium restricted, high calorie and protein
Treatment for aspirin ingestion
- induce vomiting
- Sodium Bicarb for acidosis
- Vitamin K
What does charcoal do?
Treatment for ingestion of Corrosives....draino, detergents, cleaners
DONT INDUCE VOMITING, LAVAGE or GIVE NEUTRALIZERS...call poison control and follow their recommendations
- maintain airway
- dilute with water or milk
- flood external burns with water
- endoscopy to assess burns
Treatment for ingestion of Hydrocarbon...gas, lighter fluid, paint thinners, turpentine, furniture polish
- DONT INDUCE VOMITING!!
- support ventilation
- admin O2
- IV fluids
Describe gastric lavage
- orogastric tube
- mixed with NS or 1/2 NS
- protect airway...may gag and vomit
- charcoal after lavage
Benefits of charcoal
- it binds to the toxin and passes it through the GI system
- most effective if done w/in 60min of ingestion
- can repeat admin to prevent reabsorption from biliary tract
Tricks for administering charcoal to a toddler
- have child sit on parents lap and give w/ oral syringe
- mix with chocolate milk
- put in a decorated container so child cant see
if refuses....give via NG
When do you use Ipecac? What does it do?
Acetaminophen, ASA, toxic plants, iron containing products
induces vomiting within 30 min and eliminates 30% of ingestion
Who doesn't get Ipecac?
a person with an altered mental status
Primary assessment in an emergency situation
- #1 Airway
- #2 Breathing
- #3 Circulation
** ABC then go to injury**
Airway assessment in an emergency
- positioning for air entry
- audible sounds
- airway obstruction
Breathing assessment in an emergency
- increased or decreased work of breathing
- nasal flaring
- use of accessory muscles of respiration
Circulation assessment in an emergency
- skin color
- cap refill
- rate and strength of peripheral and central pulses
Secondary assessment during an emergency
- Obtain a history regarding injury
- by whom
- how...fast, far fall, gun, knive
- mechanism of injury....circumstances
What happens when a baby gets cold?
- oxygen consumption increases
- glucose consumption increases
How do we help a baby that cant get warm?
- Radiant Warmer for initial stabilization
- Place in a flexed position
- Skin to Skin contact/Kangaroo Care
What is phototherapy for hyperbilirubinemia?
- baby placed under a fluorescent light
- eye patched
- exposed skin...uncovered in isolette
Things to monitor on a baby getting phototherapy
- look for rashes....turn frequently and NO LOTION
- watch hydration
- monitor for hyperthermia
- promote nutrition
- assess for bili stools
green, loose, increased frequency
used for hyperbilirubinemia treatment at home...allows infant to be held, changed and fed during therapy
What's difficult about a baby with hyperbilirubinemia?
You cant hold them a lot so it impairs bonding.
Cant even hold during feeds or cares
What will a baby with NEC present with?
- abdominal distention (shiny/tight)
- bowel loops
- decreased bowel sounds
- gastric retention
- blood stools
- bile stained emesis
Classic sign that a baby has NEC
- pneumatosis Intestinalis
- *air in bowel wall....shows up on x ray
What do I assess for on a patient with NEC
How do I prevent NEC with early detection?
- Look at I&O's
- measure abd girth
- assess gastric residuals
- assess for vomiting
- OB test
**when initiating or increasing feeds, frequently assess them
How do I treat a baby with suspected or actual NEC?
- IV hydration
- IV antibiotics
- Bowel resection if needed
- Assess for signs of sepsis
- Watch for signs of impending bowel perf