Children With Special Healthcare Needs

1st Trimester March 2013 Continuing Education

Our agenda today

• System announcements• Children with special healthcare needs• Children with special psychiatric needs• (ALS) Strip o’ the month: Bradycardia• (ALS) Drug o’ the month: Atropine

Special thanks to:

The Alameda County (CA) Public Health Department

I. Introduction to Children With Special Health Care Needs (CSHCN)

CSHCN: Definition

CSHCN (Children with Special Health Care Needs) –Children who have or are at increased risk for a chronic

condition – physical – developmental– behavioral– emotional and who also require health and related services of a type or

amount beyond that are required by children generally.

From: Commentary in Pediatrics, Vol. 102, No.1, July 1998.

CSHCN: Epidemiology

• 12 million US children are considered “special needs,” which is 18% of all U.S. children

From: 1994 National Health Interview Survey on Disability

• Estimated that 25% of children treated in pediatric EDs have special needs

From: Pediatric Emergency Care, Vol 12, No. 3 June 1996

CSHCN: Health Care Realities

• Managed Care– Complicated home care financially

driven– Parents forced to provide

advanced care• Societal Changes

– “Family-centered care”– Disabled have right to be home

• Medical Advances– Portable technology– Improved techniques and

medications

CSHCN: Equipment

Technology-Assisted Children • Feeding catheters• Colostomies• Pacemakers• Glucometers• Nebulizers• Apnea monitors

• Tracheostomies• Ventilators/BiPAP• Central venous

catheters• CSF shunts• Vagal nerve stimulators

CSHCN: Important Points

• Assess and manage ABCs as with any other child

• Listen to parents/caregivers– They know problems and treatments very

well

II. Common Chronic Pediatric Illnesses: Pulmonary Disorders and

Airway Defects

Pulmonary Disorders and Airway Defects

Apnea• Definition

– Respirations cease for > 20 seconds or

– Respirations cease for < 20 seconds with cyanosis or bradycardia

• Causes– Obstructive, central, or mixed– Affects both premature and full-

term infants

Pulmonary Disorders and Airway

Defects Cystic Fibrosis• Overview

– Affects 30,000 Americans– Autosomal recessive disorder– Mucus builds up in lungs

• Signs and symptoms– Increased respiratory rate– Increased oxygen requirement– Paleness or cyanosis

• Management– Give active form of the abnormal protein product– Chest therapy with bronchial or postural draining– Antibiotics– Bronchodilators

III. Cardiovascular Defects

Cardiovascular Defects

Congenital Heart Defects (CHDs)• 1 in 1,000 live births• Two types

1. Acyanotic

2. Cyanotic

Cardiovascular Defects

Acyanotic Heart Defects• Account for the majority of CHD in children• Mixing of desaturated blood in the systemic

arterial circulation• Oxygen saturation is in the normal range• Generally septal defects, obstructions to the flow

of blood, and incomplete heart development.

Cardiovascular Defects

Signs/Symptoms of Acyanotic Heart Disease• Increased respiratory rate• Increased heart rate• Heart murmur• Signs of heart failure

– Rales on lung exam– Palpable liver edge– Swollen extremities

Cardiovascular DefectsTypes of Acyanotic Heart Defects• VSD (most common)

• Ventral Septal Defect– Defect in wall that separates

ventricles

• ASD• Atrial Septal Defect

• Patent ductus arteriosus• Fetal blood passage doesn’t close after

birth

• Obstructive lesions• Narrows the aorta or valves

Cardiovascular Defects

Cyanotic Heart Defects• Blood from arteries and veins mix in the heart • Typical oxygen saturation—70% to 90% on room

air• Palliative procedures often performed at birth• Caregivers/medical control may advise that you

avoid administration of O2 unless O2 saturation is below usual – Otherwise, never withhold oxygen!

Cardiovascular Defects

Signs/Symptoms of Cyanotic Heart Disease• Cyanosis• Increased respiratory rate, retractions• Increased heart rate• Poor perfusion• Diminished peripheral pulses• Poor feeding, sweats with feeds

Cardiovascular Defects

Types of Cyanotic Heart Defects• Hypoplastic Left Heart Syndrome• Transposition of the great arteries• Tetrology of Fallot• Tricuspid Atresia• Pulmonary Atresia• Truncus Arteriosus• Cardiac Arrhythmias

IV. Down Syndrome

Down Syndrome

Down Syndrome (Trisomy 21)• Chromosomal abnormality• Affects 1 in 800 births• Highest risk: women > 35 years• At risk for medical complications of multiple

systems

Down Syndrome

Signs/Symptoms of DS• Large tongue• Short neck• Obesity• Short stature• Loose ligaments• Epicanthal folds

Down Syndrome

Conditions Associated with DS• Congenital heart disease

– VSD, ASD, AV canal• Orthopedic conditions

– Atlantoaxial subluxation• Neurologic Conditions

– Epilepsy• Airway and Respiratory problems

– Dental and speech abnormalities

V. Traumatically Disabled Children

Traumatically Disabled Children

• Unintentional injuries are the leading cause of morbidity and mortality

• Traumatic brain injuries– Risk of seizures – May need CSF shunt/feeding tube/wheelchair

• Spinal cord injuries– Difficulty regulating body temperature– Pressure sores are serious concerns– Disabled child may be unaware he or she is injured– High risk of abuse

VI. Neurologic Diseases

Neurologic Diseases

Causes of Seizures• Epilepsy• Traumatic brain injury• Genetic/metabolic defect• Congenital brain abnormality

– Including mental retardation

• Tumor

Neurologic Diseases

Generalized Seizures • Tonic clonic or grand mal

– Duration seconds to minutes– Most common type of seizure

• Absence– Vacant, blank stare– May occur many times a day

• Myoclonic– Infantile spasms– Difficult to control

Neurologic Diseases

Partial Seizures• Simple partial

– One part of brain involved– Child awake and aware– Involves one limb or one side of body– Can progress to generalized seizure

• Complex partial – Child unconscious– Affects one side of the body

• Psychomotor partial– Repetitive fine-motor activity– Most common; one part of the brain

Neurologic Diseases

Management of Seizures• Antiepileptic medications

– Most common treatment

• Home Valium per rectum– For frequent and/or prolonged seizures

– Can NOT give unless you are trained to do so and with approval of medical control

• Vagal nerve stimulators• Ketogenic diet

– For intractable seizures

Neurologic Diseases

Hydrocephalus• Excessive build-up of CSF

within the cavities of the brain• Causes

– Congenital hydrocephalus (occurs before birth)

– Acquired hydrocephalus (occurs after birth)

Neurologic Diseases

Management of Hydrocephalus• CSF shunts

– Ventriculoparietal shunt most common type

• Shunt complications– Shunt malfunction, obstruction – Infection

• Signs and symptoms of shunt malfunction– Headache– Nausea/Vomiting– Diminished mental status– Bradycardia, hypertension, irregular

respirations– “Sundown eyes”

Neurologic Diseases

Mental Retardation• IQ < 70• Non-progressive disorder• Cause is prenatal problem, brain injury, or genetic

syndrome• Requires special education

Developmental Delay• Results from prolonged illness or prematurity• Potential to “catch up”• Requires special education

Neurologic Diseases

Spina Bifida (Myelomeningocele)• Failure of the spinal cord to fuse during pregnancy• Characteristics depends on level of lesion

– Paralysis– Hydrocephalus– Delay in motor development– Loss of bladder function/UTIs– Normal intelligence– Considered to have latex allergy

Neurologic Diseases

Cerebral Palsy (CP)• Characteristics

– Damage to brain center controlling muscle control– Multiple types– Congenital or acquired

• Often occurs in very-low-birth-weight babies

– Hypertonic, contracted limbs– 50% have seizure disorder– Two-thirds have mental retardation

Neurologic Diseases

Cerebral Palsy• Management• Braces• Wheelchairs• Oral medications• Baclofen intrathecal pumps

VII. Hematology and Oncology Diseases

Hematology and Oncology Diseases

Sickle Cell Anemia (SCA)• An inherited hemoglobinopathy that

causes sickling of RBCs• Characteristics

– Pain,“vaso-occlusive crisis”– Splenic sequestration– Aplastic crisis– Sepsis

Hematology and Oncology Diseases

Hemophilia• An inherited disorder in which a factor needed for

clotting blood is either too low or missing• Incidence

– 15,000 in U.S. (mostly males)– 60% severe form– 15% moderate form– 25% mild form

• Characteristics– Prolonged bleeding– Factor routinely administered at home– Seemingly minor injury can be serious

X. Musculoskeletal Disorders

Musculoskeletal Disorders

Osteogenesis Imperfecta • “Brittle bone” disease• Incidence: 20,000–50,000 people in U.S.• Etiology

– Genetic disorder – Defective collagen synthesis– Multiple types, differing severity

• Characteristics– Bones fracture easily– Weak musculature– Growth retardation– Head disproportionately large for body

Musculoskeletal Disorders

Muscular Dystrophy• Cause

– Most common type is Duchenne’s

– Flaw in muscle protein – Muscle-wasting disease– Most are inherited

• Characteristics– Motor skills deteriorate– Cardiomyopathy– Shortened lifespan

XI. Tips for Chronic Conditions

Tips for Chronic Conditions

• Medical Identification Jewelry

• Parents trained in child’s care

• Often part-time home health care assistance

• DNR forms—Follow local protocols

• EMS Outreach Program• EMS Notification

Courtesy of the MedicAlert Foundation®. © 2006, All Rights Reserved. MedicAlert® is a federally registered trademark and service mark.

Tips for Chronic Conditions

• Assess and manage ABCs as with any other child

• Listen to parents/caregivers• They know problems and treatments very well

A Different Look

Why CSHCN Caregivers Call 9-1-1• 97 of 100 CSHCN families surveyed have sought

emergency care • 75 sought emergency care three or more times• CSHCN require EMS services because:

– Home health care equipment fails– Caregivers panic– No improvement with therapy– Child in respiratory or cardiac distress/arrest

A Different Look

Differences to Consider• Medical issues vs. equipment issues• Atypical baseline vital signs• May be smaller than same age peers• May be developmentally delayed

A Different Look

General Approach• Ask “What is normal for your child?”• Respect caregiver’s opinion on child’s condition.

– Many know as much as the doctors do about their child’s illness.

• Treat the child, not the technology.• Simple illnesses can be life-threatening .• Caregivers are experienced with the medical

system.

II. The ABCDEs:Interventions Using Special

Technology

AIRWAY: Tracheostomies

Airway

Tracheostomy• An artificial airway passed

through a surgical opening (stoma) in the anterior aspect of the neck and into the trachea

Airway

Tracheostomy Indications:• To bypass an upper-airway obstruction• To provide long-term mechanical ventilation• To facilitate clearance of excess secretions

Airway

Tracheostomy Types and Features

Single Lumen

Double Lumen

Fenestrated

Airway

Interventions:• Position of comfort

• Humidified air or O2

• Nebulized 1:1000 epinephrine, if protocols allow• If child is in extremis, consider endotracheal

intubation

Airway

Alleviating Respiratory Distress• Position• Suction• Oxygen• Repeat• An emergency tracheostomy tube change

may be necessary.

Airway

When to ventilate manually • Upon removal from ventilator• Consider before/after

suctioning or trach change• Signs of respiratory distress or

failure

Airway

Causes of Tube Obstruction• Improper airway positioning• Improper insertion of the trach tube

– Creation of a “false track”• Mucous plug• Failure to remove obturator after tube insertion

Breathing

Interventions• Disconnect patient from the ventilator• Began manual ventilation• Assess for chest rise, breath sounds• If no improvement, check for tracheostomy-

tube obstruction• If improved, consider ventilator issue• Prepare for transport

CIRCULATION: Central Venous Catheters

Circulation

Purpose of Central Venous Catheters• Administration of Medications• Delivery of chemotherapy• Nutritional support• Infusion of blood products• Blood draws

Circulation

Types of Catheters• Broviac, Hickman, Groshong

-Tunneled central venous catheters

- Proximal tip in the subclavian vein

- External access

• Port-a-Cath/Med-a-Port/PAS Port- Catheter system is completely beneath skin

• Percutaneous Intravenous Catheter (PICC)- Proximal tip in central vein

- Looks like a PIV

DISABILITY

Disability

Interventions • Position• Oxygen• Maintain body temperature• ALS: IV, fluids, IO• ALS: Consider inotropes for shock if

unresponsive to fluid resuscitation

Assessment of neurological status• Ask caregiver to compare child’s present

status to baseline

Disability

Cause of symptoms:• Shunt infection

– If child presents with a fever or redness along the shunt tubing, suspect a shunt infection

• Meningitis• Encephalitis

Disability

CSF Shunts• A CSF shunt is a catheter with one end in a

ventricle of the brain and the other end in the abdomen or atrium that drains excess CSF or bypasses a blockage of CSF.

Types:• Ventriculoperitoneal• Ventriculoatrial

Disability

Concern• The shunt could be damaged or

disconnected. This can result in increased intracranial pressure.

Disability

Causes of Complications:• Brain infection• Shunt obstruction (resulting in a

dangerous build-up of fluid in the skull)• Shunt malfunction• Peritonitis

Children with Special Healthcare Needs

• Cerebrospinal fluid shunts– Emergency care• Vomit, aspiration• Suction• O2• Assist breathing, intubate• Blood sugar• Treat seizures

Children with Special Healthcare Needs

Shunt Drainage Routes

Children with Special Healthcare Needs

External Shunt Infection

EXPOSURE

Exposure

Interventions• Assess neurovascular status distal to the

injury.• Gently place on a long-board splint. • Avoid taking the blood pressure of a child with

osteogenesis imperfecta. • Do not use a hare traction splint or MAST

trousers.

IMPORTANT POINTS

• Cover the child to maintain normal body temperature

• Respect the child’s privacy

• Carefully examine for injuries– Assessment may be difficult due to

developmental level– CSHCN are at high risk for abuse

• Report any suspicious injuries to the proper agency.

IMPORTANT POINTS

II. SAMPLE History:Feeding Catheters

Feeding Catheters

• Nasogastric Tube (NGT):– Catheter placed through the nose into

stomach– For supplementation in children who

cannot take enough by mouth– Short-term use– Can use to decompress stomach

Feeding Catheters

• Gastrostomy Feeding Tube (GT):– Catheter surgically or

endocopically placed into stomach or Jejunum

– Provides long-term nutritional support

Feeding Catheters

• Feeding Catheter Complications:– Gastric contents can leak, causing

irritation– Tube obstructed– Tube dislodged– Abdominal distention

I. Moving Children with Special Needs

Communication

• Challenges:– Language barriers– Is the person with the

child the primary caregiver?

– Assess child’s ability to understand.• Developmental delay• Visual/auditory deficits

Communication

•Management:– Use a soothing voice to provide comfort.– Explain each movement.– Ask the caregiver for a medical summary

card. Oftentimes, the caregiver may be too stressed to remember vital information.

Environment

•Challenges:– The scene and the child’s

response to that environment can be a great source of tension and anxiety.

– Multiple providers can create fear.

– Multiple voices can cause confusion.

Environment

• Management:– Limit the number of providers.– Ask one person to speak and interact

with the child.– Decrease chaos.– Keep the noise down.

TRANSPORT AND TRANSFER

Transport

•Challenges:

– Anxiety in child.– Child may resist being restrained.– Brittle bones and muscle contractures

can easily lead to injuries during transport.

– Do not pull on extremities!

Transfer

• Management– Make secure, firm contact– Suggest that family member move child.– Allow the child to lay in a comfortable

position.– Use padding around buckles and

contractures.– Do not pull on extremities!

Stabilization

• Secure and transport child in own special restraint device if:– No suspicion of cervical injury.– Child is not critically ill.– Device doesn’t impede assessment and

treatment.– Device can be properly secured in

ambulance.

Destination Decisions

• Challenges:– Parents’ confidence in EMS system*• 82% very confident in EMS care and home hospital• 77% uneasy/not confident in community hospital

care• 98% very confident in “home” hospital care

• Parents trust 9-1-1 but not 9-1-1 transport decisions!

*100 CSHCN families surveyed in 2000

Children with Special Behavioral and Emotional Needs

• Behavioral emergencies involving children present special challenges to EMS.

• Aggressive behavior may really be symptom of an underlying disorder or disability.

• Parents of mentally ill children often overwhelmed and isolated from community and social support network.

• Family may hesitate to call 911 because of fear of stigma or misinterpretation by EMS personnel.

EMS Response

• Volatile situations require shift from common EMS response to integrated community collaboration and adaptive “out-of-the-box” decision making.

• 911 call may be from a mother or school staff member desperate for help with “out-of-control” child.

• Immediate link to pediatric or mental health professionals may de-escalate the child’s psychiatric emergency and ensure continuity of care.

• Unfortunately many EMS system policies, procedures, guidelines, and training do not include these options.

Common Behavioral Emergencies in Children

• Major psychiatric disorders that may predispose to behavioral emergencies in children include – mood disorders (e.g., depression, bipolar disorder); – thought disorders (e.g., schizophrenia);– developmental disorders (e.g., autism); – anxiety disorders (e.g., posttraumatic stress

disorder); – other disorders such as attention deficit hyperactivity

disorder.

EMS considerations

• EMS can assist and advocate for child and the family during a behavioral emergency.

• Family of a child with behavioral problems lives in fear of restraints, hospitalizations, false accusations.

• Understanding emotional fatigue, physical exhaustion, and chronic life disruptions of families is is integral to addressing their needs.

EMS Considerations

• Families of children with psychiatric disorders often have competing fears: – Love for their child– Fear of a violent outburst by the child toward the

family– Fear of the violence that may occur if the child needs

to be restrained by the police or EMS providers.

For more information on pediatric psych issues and EMS response:

• http://www.acphd.org/media/109325/meeting_challenges_pediatric_behavioral_emergencies.pdf

Rhythm O’ the Month• Bradycardia

Dysrhythmias Originating Dysrhythmias Originating in the SA Node in the SA Node (2 of 10)(2 of 10)

NormalQRS

NormalPRI

Upright and normalP Waves

SA nodePacemaker Site

RegularRhythm

Less than 60Rate

Sinus Bradycardia

Rules of Interpretation

Sinus Bradycardia - Causes

• Decrease in sympathetic tone on the AV node (increase in parasympathetic tone)

• Pressure on fontanels in infants• Intracranial swelling• Glottic irritation from ET tubs, gagging,

emesis• Disease of the SA node• Hypothermia, Hypoxia

Sinus Bradycardia

• Administration of digitalis, propranolol (Inderal), verapamil, and quinidine

• Common in acute inferior AMI - Involves the right coronary artery which supplies the SA node with blood

Sinus Bradycardia in Children

• Life threatening, especially in newborns and infants.

• Often a respiratory issue– Newborn• Dry, stimulate, suction first• Then oxygen, ventilation• CPR if heart rate remains under 60

– Babies/children• Treat respiratory issues unless child has known cardiac

issue

Sinus Bradycardia

Treat the patient, not the monitor!

Bradycardia is also common during sleep, rest and in trained athletes!

Sinus Bradycardia

• Ultimate clinical significance…..– Decreased heart rate/BP which leads

to decreased CARDIAC OUTPUT

Sinus Bradycardia

• Treatment Modalities– Asymptomatic – pulse and adequate

BP (>100 systolic)

Routine Medical Care

IV, O2, monitor

Position of Comfort

Sinus Bradycardia

• Symptomatic– Hemodynamic Instablity (BP <100

systolic)• Syncope, hypotension, altered mentation• Chest pain, palpitations, diaphoresis• Difficulty in breathing• Poor skin vitals and perfusion

Symptomatic Bradycardia - ALS

• Do not delay pacing while getting ready for Atropine

• Pace at 70. – Increase MA until pulse matches.

• While pacing, consider Versed, 2.5 mg slow IVP.

• If necessary, Dopamine to maintain SBP 90-105 and pulse >60.

Sinus Bradycardia

• Hypotension– Leads to decreased cardiac output

– Palpitations• Because of SA node’s increased relative

refractory period permits refractory firing

Sinus Bradycardia

– Chest Pain• Heart disease already exists• Coronary blood flow is decreased

Sinus Bradycardia

• Bottom Line: TREAT THE UNDERLYING CAUSE TO ABOLISH THE DYSRHYTHMIA AND INCREASE THE RATE

Drug O’ the Month• Atropine!

Atropine Indications

• Symptomatic bradycardia. • NO LONGER USED: asytole or

PEA• Nerve agent exposure• Organophosphate poisoning

Adverse Reactions

• Dry mouth, hot skin, intense facial flushing• Blurred vision or dilation of the pupils with

subsequent photophobia• Tachycardia• Restlessness• May cause paradoxical bradycardia if the

dose administered is too low or pushed to slowly

Contraindications

• Acute MI• Myasthenia Gravis• GI Obstruction• Closed angle glaucoma • Known sensitivity to atropine,

belladonna alkaloids or sulfates (NOT sulfa)

How Atropine Works

• Increases firing of the SA node.• Increases conduction through the

AV node.• Opposes the action of the vagus

nerve.• Blocks acetylcholine receptor sites• Decreases bronchial secretions.

Atropine Dosage

Symptomatic Bradycardia• Adult: 0.5 mg IV/IO every 3 to 5 minutes to a

max dose of 0.04 mg/kg. Don’t delay pacing for Atropine.

• Peds: Oxygen/ventilation first. Then Epi! If that doesn’t work then 0.02 mg/kg (min of 0.1 mg/dose; max of 0.5 mg/dose). – Repeat once in 5 minutes

Dosage

Nerve Agent or Organophosphate Poisoning

• Adult: 2 mg IVP repeated if needed every 5 minutes until symptoms dissipate

• Peds: 0.02 mg/kg IV/IM every 5 minutes as needed until symptoms dissipate

Special Children, Special

Care

Questions? If you are watching live, feel free to type in the text box to your right.

If you are watching the recording, or the Powerpoint, feel free to email afinkel@silvercross.org or call 815-300-7130.