Jennifer Griffin

Parent Resource Coordinator

Alpha Resource Center

Santa Barbara, California

Sub-human

Objects of ridicule

Separated

Hidden

Recognized RIGHTS!!

Focused on INDIVIDUAL!!

Buzz words: Inclusion

Accessible

Adaptive

Supports

Services

Self-determination

Started in 1974 at a planning session for a

self-advocacy conference

Objected to the terms ‘retarded’ &

‘handicapped’

Wanted to be seen as “People First”

www.peoplefirst.org for a chapter near

you

Which led to…

What’s important to __________

What’s important for__________

What is working well? (needs to stay the same)

What isn’t working well? (needs to be different)

Planning Team - people in the individual’s life who are there on an ongoing basis focused on his/her needs, wants and goals

The Plan

Things I like/don’t likeThe people who are important to meWhat makes me happy/sadWhat makes me afraidThings I want to do/learnWhat is my home/school/work likeWhat do I need help with What are my health needsWhat people say about me at

home/work/school

Remember, when most people turn 18 they

can make their own decisions.

Parents can be the biggest obstacles even

when they say they want the best

The person at the center of the plan is the

child/adult with the disability, NOT the

parent/family member

Burger King story

Self-advocacy story

The ARC - www.thearc.org

Family Voices - www.familyvoices.org

Family Resource Centers - State Chapters

LGS foundation - www.lgsfoundation.org

Helen Sanderson Associates -http://www.helensandersonassociates.co.uk/reading-room/how/person-centred-planning.aspx

The Disability Rights Network -http://www.ndrn.org/index.php

Jennifer Griffin

Parent Resource Coordinator

Alpha Resource Center

jgriffin@alphasb.org

805-683-2145

4501 Cathedral Oaks Rd.

Santa Barbara, CA 93110

Wishing with

Make-A-Wish®

Objectives

• Mission and vision

• Eligibility criteria

• Steps to a wish

• Types of wishes

• Impact of a wish

• How to refer a child

OBJECT IVES

MISS ION

Our Missionis to grant the wishes of children

with life-threatening medical

conditions to enrich the human

experience with hope, strength

and joy.

Cole, 3, Hirschprung’s disease

I wish to go to cowboy camp

VISION

Chelsey, 18, autoimmune disease and

neuromuscular condition

I wish to meet my favorite basketball player

Our Visionis to grant every eligible child a wish.

• At the time of referral, the child

must be over the age of 2 ½ and

under the age of 18

• The child has not received a wish

from Make-A-Wish or any other

wish-granting organization

• The child’s medical condition is

currently life-threatening

(typically Progressive, Degenerative or Malignant)

ELIGIBILITY CRITERIA

Juliana, 7, leukemia

I wish to be a scientist

• Hematology/

Oncology

• Cardiology

• Neurology

• Nephrology

MEDICAL CONDITIONS

Most qualifying medical conditions

fall under one of the following

categories:

• Transplants

• Pulmonology

• Gastroenterology

• Urology

• Immunology

•Some medical conditions qualify alone.

Cystic Fibrosis Cancer

Pulmonary Fibrosis End Stage Renal Disease

Duchenne Muscular Dystrophy Hypoplastic Left Heart Syndrome

•Some medical conditions that have life-threatening

complications qualify.

Sickle Cell Disease – need for monthly transfusions

Epilepsy – intractable or refractory seizures

•Some treatments are considered life-threatening and

qualify.

Transplants

Ventilator dependent

ICD

MEDICAL CONDITIONS

Steps To A Wish

S T E P S T O A W I S H

1. Patient is referred.

2. Eligibility is determined.

3. Wish-granting volunteers are assigned

and meet with the wish child to

determine their wish.

4. Wish is approved.

5. The child enjoys their wish!

WISH TYPES

Daniela, 8leukemia

I wish to have a yorkie

Hunter, 7leukemia

I wish to meet John Cena

Sarah, 17tethered cord syndrome

I wish to go skiing

J’Len, 4leukemia

I wish to be

a police officer

IMPACT OF A WISH

A wish transforms the

lives of sick children

and their families for

years to come.Jazzmen, 16, Hodgkin’s lymphoma

I wish to have a royal Sweet 16

IMPACT OF A WISH

96%of wish families and health

professionals see emotional

improvements.

Jesus, 6, leukemia

I wish to be a charro

81%of parents believe their children are

more compliant with treatment.

IMPACT OF A WISH

Gavino, 4, leukemia

I wish to have a park in my backyard

IMPACT OF A WISH

74%of parents see the wish experience

as a positive turning

point.Jaeda, 18, cystic fibrosis

I wish to be a coast guard rescue swimmer

IMPACT OF A WISH

When children

feel better, they often

get better.

Juliana, 7, leukemia

I wish to be a scientist

Refer a child

Visit www.wish.org/refer

R E F E R

Recap

• Mission and vision - grant every eligible

child a wish

• 3 basic eligibility criteria – age,

diagnosis, wish history

• 6 steps to wish granting

• Types of wishes

• The power of a wish

• Refer a child

R E C A P

Pharmacology 101:

Anti-Epileptic Drugs

LGS Foundation Family

Conference

October 24, 2014

Columbus, Ohio

Michelle Welborn, PharmD

ICE Alliance

Objectives

• Understand the Absorption, Distribution, Metabolism

and Excretion of Drugs

• Understand Mechanisms AED Interactions and

Adverse Reactions

• Gain better understanding of how to avoid

interactions and adverse reactions and how to

discuss pharmacology of AEDs with healthcare

providers

A Delicate Balance

Excitation (lots of firing)

Na+ and Ca++ inside cell

GLUTAMATE RELEASE

Inhibition (balancing the

firing)

Cl – inside cell; K+ outside

cell

GABA RELEASE

Stomach to Brain – Pharmacokinetics

and Pharmacodynamics of AEDs

Pharmacokinetics =

What body does to

drug

Pharmacodynamics =

What drug does to

body

Pharmacokinetics =

What body does to

drug

Pharmacodynamics =

What drug does to

body

Pharmacokinetics of AEDs

• Absorption

• Distribution

• Metabolism

• Elimination

Useful Terms• Tmax : time to maximum drug concentration

• Cmax: maximum drug concentration

• AUC: amount of drug under the

time/concentration curve

• Half life (t ½) : Time it takes for ½ of drug to

be eliminated from body

• Steady state: Absorption = Elimination

Clinical Pearl

• Common question – how long will it take for the drug to get out

of his system?

• It typically takes about 5 half lives to clear a drug from the body

after discontinuation of the drug

• “ Steady state” pharmacokinetics occur in the same amount of

time

Example:

Phenobarbital t ½ = 2 – 7 DAYS

Lamotrigine t ½ = 13.5 HOURS

Clinical Pearl:

How long does

drug stay in

system?

PharmacokineticsC

on

cen

trati

on

Day 1 Day 2

Adapted from Cloyd JC, et al. Pharmacotherapy. 2000;20(8 Pt 2):139S-151S.

Time (h)

Immediate-release (tid)

Zone

of

Seizure

Control

Trough: SeizuresExtended-release (qd)

0 8 16 24 32 40 48

Peak: Side Effects

Clinical Pearl: What if she throws

it up????

< 30 minutes – repeat all doses

30-45 minutes – half doses

45 minutes – 1 hour – repeat of drug

is dosed once a day; do not repeat if

drug is dosed twice a day or more

> 1 hour – don’t repeat

Clinical Pearl:

What if she

throws up?

Bioequivalence of Drugs:

FDA Accepted Parameters

• Single dose of reference drug and test drug given to healthy adults in a crossover design. Bioequivalence accepted when the 90% confidence interval of the ratios

– AUC

– Cmax

– Tmax

• The bioequivalence interval falls between 0.8 and 1.25 (log-transformed data)

41

Testing for Bioequivalence

0.8 1.251.0

0.8 1.251.0

0.8 1.251.0

Test product low

nonequivalent

Test product high

nonequivalent

Test product

bioequivalent

42

Bioequivalence : Generic/Generic

0.8 1.251.0

0.8 1.251.0

0.8 1.251.0

Brand Product

Generic #1

Generic #2

Clinical Pearl: Making

Decisions About Generics

•If you must use generics, should be the same generic

manufacturer each refill

Clinical Pearl:

Should we use

generics?

Drug Interactions Mechanisms

• Inhibition of Absorption

• Enzyme Inhibition

• Enzyme Induction

• Additive Pharmacodynamic Effects

• Antagonistic Pharmacodynamic Effects

Pharmacogenomics

• Genetic variability (also known as polymorphism)

influences metabolism

• 1/15 Caucasian or people of African descent have

exaggerated responses to standard doses of beta

blockers

• 1/5 Asian people are poor metabolizers of drugs

dependent on CYP2C19 enzyme for metabolism

(phenytoin, phenobarbital)

Inhibition of Absorption

• Binding to cations such as aluminum, magnesium, iron,

calcium (multi-vitamins, supplements)

•pH dependent absorption – pH in stomach changed by

drug or food (dairy, acidic fruits or vegetables)

•Full or empty stomach ?

Enzyme Inhibition

• Resource to check for drug

interactions

www.drugs.com/drug_interacti

ons.html

• Use with caution and consult

prescriber or pharmacist

Enzyme Induction

• Enzyme inducers increase the activity of certain

metabolizing enzymes, thereby decrease effect of

drugs dependent on these enzymes for metabolism

– Carbamazepine, phenytoin, primidone (Mysoline),

phenobarbital

Pharmacodynamic Interactions

• Antagonistic Interactions

– Giving drugs that can decrease seizure threshold

to person with epilepsy

• Propofol (anesthetic)

• Certain high dose antibiotics

• Aminophylline (bronchodilator)

• Cyclosporin

• Oral contraceptives

• Stimulants

• Anti-psychotics

Pharmacodynamic Synergy

• Multiple Drugs for LGS – Should I take off all drugs and start

all over?

• Make all changes under supervision and agreement of

neurologist

• Consideration of continued need of AED should be made as

new drugs are added

• Weekly pill box

• Know what can be crushed or broken

• Disguise taste when possible

•Watch carb content when on keto diet

(good time to switch from liquids)

•Follow through with monitoring blood

levels when appropriate

•Have a sick plan

Clinical Pearl:

Practical Tips

Food and Herbs that Alter Drug

Metabolism

• St John’s Wort

• Milk Thistle

• Garlic

• Ginseng

• Licorice

• Grapefruit

Herbal Pharmacodynamic

Interactions

• Herbs that can decrease seizure threshold

• Gingko biloba

• Star fruit

• Star nise

• Sage

• Ephedra

• Eucalyptus

• Pennyroyal

• Shankhapusphi

Conclusion

• Understanding pharmacology concepts may help

facilitate discussion with healthcare providers and

make informed decisions

• Herbs are not necessarily benign and may interact

with AEDs – any use of herbal therapy should be

discussed with neurologist before use

• Patients with LGS require multiple AED therapy, and

utility of drug may need revisiting before new drug is

added

Questions?