Report on Baltimore IDF Conference

[Guest guest]

from Dale, Mom to Katy, CVID, age 19

Katy and I attended the 2nd semi-annual IDF conference in Baltimore the

19-21st of June. Here's my summary. It's purpose is to encourage you

and your family to mark your calendar and start making plans to attend

the 3rd national conference which will be June 23-25, 2005 at Disney's

Contemporary Resort, Walt Disney World, Lake Buena Vista, Florida!!!!!

This report will be slanted and biased by how I interpreted the

conferences. Sorry but I didn't take word-for-word notes -- just

listened and formed impressions. But thought especially if you've

never attended the conference you might enjoy hearing my version.

So, here goes:

Katy and I arrived on Wednesday night and checked into the Baltimore

Waterfront Hotel - a beautiful and luxurious hotel by our standards!

Our 15th floor room had huge windows overlooking Baltimore Harbor. I

attended a IDF volunteer's breakfast at 7:30 a.m. (4:30 California

time!) which was nice and let me say hello to Quentin Seals from Tenn

who used to be on this list. He and his wife and Matt were there. Matt

looks so good!!!! I also saw others who are active on the list -- so

I'll let them jump in and say hello. If I try to name everyone, I'll

miss someone and I don't want to do that. But Quentin said to tell

everyone hello.

At 10 o'clock Katy and I attended a D.C. briefing and attending the day

on Capitol Hill. Since that won't happen at the next conference -- I'll

skip that report.

Thursday night was the Opening Reception. The food was outstanding!!!!

If you've ever been around me -- you know I LOVE to eat. I thought

the food for this event was above and beyond nice! Or maybe I'd just

worked up a huge appetite at D.C. The event is just a huge hallway with

food every few feet and people crowded wall to wall eating and talking

and getting acquainted. I don't know why they can't find larger rooms

for this event -- but..... it was very well attended and very nice to

connect with people you recognize from previous years -- or meet

people. I gave out a lot of adresses and got to visit with lots

of people.

On Friday June 20, 2003 - Breakfast Symposium from 7 - 8:30 (I slept)

It was entitled: Treatment of Primary Immune Deficiency Diseases:

Findings from Two National Patient Surveys. The speaker was

Boyle, Ph.D., a IDF trustee (and other half of Marcia Boyles who

together were the founders of IDF).

From 7 a.m. to 10 p.m. there is both Child Care and a Youth Program.

The child care stays in the hotel, but has babysitting areas and

activities to keep the children entertained. The Youth program is for

the older and more independent child -- they did tours all over

Baltimore -- the aquarium, a baseball game, science museums, etc. I

didn't have a child attending, so don't know how it turned out this year

-- I know that two years ago, the parents and kids alike were SOOOO

pleased with Kiddie Corp -- the group in charge of the kids.

9:30 - 11:45 a.m. - OPENING SESSION

Speeches by Dr. Barr, Chairman, Board of Trustees

Marcia Boyle, Founder IDF

Dr. Buckley, Chairman of the Medical

Advisory Committee

Moran, President, IDF

Castaldo, Hereditary Angioedema

Association

Keynote Address: Strikas, M.D. Medical Officer with the

National Immunization Program Centers for Disease and Control and

Prevention.

His major speech was about the necessity of getting smallpox

vaccinations - which went over like a lead balloon. His whole focus was

on how important it is for entire communities to be safe by

vacinnations! Talk about someone being fed to the wolves -- I felt so

sorry for him. Somebody should have cued him into his audience.

Needless to say, that was a bust and I don't know that he'll ever know

why his speech bombed. (Everyone sat there politely frowning at him --

but no booing or hissing!)

11:45 - 12:45 Box lunch -- you grabbed your lunch and most people

headed to their rooms to catch a nap -- some sat around and talked and

some brave souls went for a quick shopping stroll on the waterfront.

(I napped)

1:00 - 2:30 there were specific diagnosis sessions: You could attend

only 1

COMMON VARIABLE IMMUNE DEFICIENCY AND IgG SUBCLASS

DEFICIENCY by Dr. Charlotte Cunningham-Rundles, Mt. Sinai School of

Medicine.

I was impressed by her great compassion for her

audience. She told that when she came to the 1st national conference 2

years ago -- she was so overwhelmed when she looked at her audience

because she had spent her life-time studying and learning about CVID,

but had never met more than a handful of patients. She encouraged us

to look around the room and had all the CVID patients lift their hand.

I got a lump in my throat thinking of how great it was to sit in that

crowded room and realize that we were not alone -- there were probably

300 attending that seminar! No new stuff was offered. So many people

attending are hearing the information that we share daily on Pedpid for

the very first time. I sat and talked with people between every session

trying to help them understand their diagnosis and answering questions.

So, this seminar didn't produce any new information for me -- but

just affirmed that what we are sharing on Pedpid really is the latest

and most up-to-date information. She spent almost as much time

answering questions as she did talking.

SEVERE COMBINED IMMUNE DEFICIENCY - by Dr. Buckley, Duke

University School of Medicine

I did not attend

X-LINKED AGAMMAGLOBULINEMIA by Dr. Miles, All Seasons Allergy,

Asthma & Immunology Center, Texas

I did not attend

CHRONIC GRANULOMATOUS DISEASE by Dr. Holland, National Institutes

of Health

I did not attend

SELECTIVE IgA DEFICIENCY by Dr. Lederman, s Hopkins

University School of Medicine

I did not attend -- but I think Russo did. Could you may fill us

in on what you learned there since so many of our kids are IgA

deficient. Any new information out there?

WISKOTT-ALDRICH SYNDROME - by Dr. Hans Ochs, University of Washington

School of Medicine

I did not attend.

HAE PERSPECTIVES, Dr. Bruce Zuraw, The Scripps Research Institute, Dr.

Alvin , Center for Blood Research, Dr. Konrad Bork,

Johannes-Gutenberg University, and Dr. Lorenza Zingale, Universita degli

Studi di Milano

2:45 - 3:45 p.m. Scientific Sessions (can attend only one)

IMMUNE GLOBULIN THERAPY - DR. MELVIN BERGER, Rainbow Babies and

Children's Hospital

GENETICS OF PID DISEASES - DR. JENNIFER PUCK, National Human Genome

Research Insistute, NIH

BONE MARROW AND STEM CELL TRANSPLANTATION - DR. LORI MYERS, Duke

University School of Med.

BLOOD SAFETY AND AVAILABILITY - DR. JONATHAN GOLDSMITH, Immune Defieincy

Foundation

GASTROINTESTINAL COMPLICATIONS OF PID by Dr. Lloyd Mayer, Mt. Sinai

School of Medicine

This is the one I chose.

+++++++++++Notes from this session are located at the end of this

document++++++++++++++++++++.

3:45 - 4:15 BREAK

4:15 - 5:15 Scientific Sessions (can attend only one)

IMMUNE GLOBULIN THERAPY (repeat)

GENETICS OF PID DISEASES (repeat)

BONE MARROW AND STEM CELL TRANSPLANTATION (repeat)

BLOOD SAFETY AND AVAILABILITY (repeat)

AUTOIMMUNITY AND IMMUNE DEFICIENCY - by Dr. Sleasman, M.D. All

Children's Hospital, St. sburg, Florida

++++++++NOTES FOR THIS SESSION ARE LOCATED AT THE END OF THIS

DOCUMENT+++++++

6:30 - IDF Celebration Banquet - good food, fun to sit around tables

and talk with new people. Speeches were centered on raising lots of

money to further research. Trying to drum up some big monetary

supporters. Thanks to all the corporations who funded the Conference.

then and I escaped to my hotel room and talked until

the wee hours (at least 'til Katy came home!)

SATURDAY, June 21, 2003

7-8:30 a.m. Breakfast Symposium - Innovations in IVIG Safety (I skipped)

7-8:30 a.m. HAE Breakfast Symposium (I skipped)

7a.m. - 6 p.m. Child Care and Youth Porgrams

9:00 - 10:15 General Session: Health Insurance: Making it Work for

You (I skipped)

10:15 - 10:30 Coffee Break

10:30 - 5:15 LIFE MANAGEMENT SESSIONS ( Attend one session in each of

the four time periods)

10:30 - 11:45

PERSON-TO-PERSON: LIVING AS AN ADULT WITH PID

PID IN THE SCHOOL SYSTEM

COPING WITH CHRONIC ILLNESS: STRENGTHENING YOUR FAMILY (ISSUES FOR

ADULTS with PID)

by Seymour, Minnesota State University

(I skipped) said it was GREAT!

COPING WITH CHRONIC ILLNESS: ISSUES FOR CAREGIVERS

CLINICAL TRIALS: ARE THEY FOR YOU? NIH

FINANCIAL PLANNING FOR CHRONIC ILLNESS

11:45-12:45 Box Lunch

1:00 - 2:15

PERSON-TO-PERSON: EXPERIENCES WITH IgG THERAPY

PID DISEASES IN THE WORKPLACE

COPING WITH CHRONIC ILLNESS: ISSUES FOR ADULT PID (repeat)

COPING WITH CHRONIC ILLNESS: ISSUES FOR CAREGIVERS (repeat)

++++++++THIS IS THE ONE I CHOSE - NOTES ARE LOCATED AT END OF

DOCUMENT+++++++++

CLINICAL TRIALS: ARE THEY FOR YOU? (repeat)

FINANCIAL PLANNING FOR CHRONIC ILLNESS (repeat)

2:30 - 3:45

PERSON-TO-PERSON; COPING WITH THE NEW DIAGNOSIS

PID DISEASES IN THE SCHOOL SYSTEM (repeat)

SKILLS FOR MANAGING YOUR HEALTH CARE

MANAGING CHRONIC LUNG DISEASE

COMPLEMENTARY AND ALTERNATIVE MEDICINE: ASSESSING ITS ROLE IN PID

++++THIS IS THE ONE I ATTENDED - NOTES ARE AT END OF THIS

DOCUMENT++++++++++++

4:00 - 5:00

PERSON-TO-PERSON: PARENTING A CHILD WITH PID

PID IN THE WORKPLACE (repeat)

THE ROLE OF COMPLEMENTARY MEDICINE IN PID (repeat)

SKILLS FOR MANAGING YOUR HEALTH CARE (repeat)

MANAGING CHRONIC LUNG DISEASE (repeat)

7:00 - 10:00 IDF Extravaganza was incredibly wet!!!! The music was

loud, the crowd was cold and wet -- Once again, and I

decided a nice cozy chat in the hotel was preferable and much dryer!

The next IDF National Conference will be June 23-25, 2005 at DisneyWorld

in Florida. Save the date -- I think every family ought to attend at

least one of these Conferences. There are scholarships to help with

expenses - just notify IDF that you need the help and they will do what

they can.

Hope this will encourage some of you to attend next time.

In His service,

Dale Weatherford

NOTES - they made perfect sense when I made them almost a month ago --

now, I'm not sure what some of my comments mean. So -- next time --

you'll have to be there yourself!!!

GASTROINTESTINAL COMPLCATON OF PID by Dr. Lloyd Mayer, Mt. Sinai School

Of Medicine.

In the normal person the intestines must discriminate wisely what is

pathogenic and what is " normal " food. The gut is not helped by IgG

because enzymes destroy it. But IgA is primary. Every bite you take

is a huge assault by viral, fungal, parasites and bacteria and there is

more bacteria in your gut than there are cells in your body! Oral

tolerance means that our body must learn to not " reject " or " attack "

food. Our bodies learn that starting at birth. IgA learns to attach

to pathogens and then together they pass through the digestive track

without incident.

In a patient with CVID we see about 60% have chronic diarrhea, 40% have

malabsorption, 10% have splenomegaly (enlargement of the spleen) and 2%

have Irritable Bowel Disease including Chrohn's and ulcerative colitis.

NOTE: GI severity does NOT equal level of PID severity. No one knows

why they are not directly correlated, but they are NOT!!!!

IVIG does not aid GI problems because IgG does not affect gut anyway.

Lack of IgA is the major problem. But, CVID is much more common to

have GI problems than any other diagnosis. Most IgA patients have IgM

that can cover the gut also. IgM can compensate for loss of IgA in the

gut. Chronic Granulomatous patients see Irritable Bowel Disease more.

Bruton's most common GI problem is chronic viral infections. SCIDS

most common problem is malabsorption and chronic diarrhea.

What are some GI complaints that are most prevalent with CVID?

diarrhea

weight loss

abdominal cramping

bloating-distention

gas

bleeding (rare)

absess (especially peri-anal) fissures and fistulas

fatigue (from anemia)

One of the complicating factors is that antibiotics cause problems with

the GI tract.

Bacterial overgrowth can follow any antibiotic.

Most common intestinal infections are what to expect with CVID. You

don't usually see the rare ones like salmonella or shigella. Those

occur at a rate equal to the general population. You do see Giardia

lambia at a VERY high rate with CVID. It is the most common protozoan

infections and is now treated with Flagyl and we are seeing less severe

infections because of the available treatment.

When you have bacterial overgrowth, peristalsis is affected.

Peristalsis is the gentle action of waving the food down the intestinal

tract. The first sign of bacterial overgrowth is that fat is being

malabsorbed. If fecal fat is not being absorbed, it leads to A,D,E

and K vitamin malabsorption. Treatment is Cipro, tetracycline or

penicillin. But it has a tendency to recur.

We also have the heightened danger of autoimmune inflammatory diseases.

Pernicious anemia: causes atrophic gastritis, B12 deficiency, numbness

in hands and feet, and higher risk of stomach cancer.

Sprue or celiac: sensitivity to gluten. Causes a flattening of lining

that can be seen by endoscope. fat malabsorption, weight loss,

diarrhea, abdominal bleeding, fat in stool, bone loss.

CVID - usually not a true celiac because the immune system doesn't

respond. But this can be confirmed by biopsy.

Suggestion to cope with celiac: maintain gluten free diet, increase fat

in diet, use water soluable vitamins for A, D, E & K and don't use low

dose steriods.

COLON PROBLEMS:

Chrohn's is atypical

Irritable Bowel Disease is about 5.6% of CVID patients

Ulcerative colitis - atypical

diarrhea

rectal bleeding

abdominal pain

Very UNCOMMON is Nodular lymphoid hyperplasia which is an over

production of lymphocytes which swell the lymph nodes in the bowel wall.

If they get too big, they mash the lining of the intestines flat

causing malabsorption. Then the body may try to rid itself of the lump

and causing major irritation. Can lead to malignancy. But again --

very, very rare.

He suggested that your doctor check using UGI (barium), colonoscopy and

biopsy, stool studies for white blood count, cultures for bacteria and

parasites.

NOTE: Zinc deficiency if severe enough could mimic an Immune

deficiency. And severe malnutrition can produce an immune deficiency.

It cannot cause a " primary " immune deficiency -- but can cause the

antibodies to be lost in great enough numbers to cause a deficiency.

Overall -- I felt he did a good job with the presentation. It was

reassuring to know that all the GI problems that Katy has experienced

are " to be expected " with CVID. He encouraged us to be agressive in

treatment of bacterial overgrowth and parasites. He also emphasized

that CVID'ers don't usually catch the " rare " bugs. We usually go the

" common " route but get them at a much, much higher rate than the general

population.

AUTOIMMUNITY AND IMMUNE DEFICIENCY - by Dr. Sleasman, M.D. All

Children's Hospital, St. sburg, Florida

There is a definite link between autoimmunity and PID because when one

aspect of the immune system breaks down, we are designed so that other

facets begin to cover. That's great -- unless they try too hard to

cover and become agressive! Autoimmunity affects ALL systems and

causes inflammation and tissue destruction. These can include:

joints

skin

kidney

brain

lungs

organ linings

liver

muscles

GI

endocrine glands

blood elements

some common autoimmune states:

Rheumatoid arthritis

systemic lupus

Type I Diabetes

Irritable Bowel Disease

Hemolytic Anemia

MS

Nephritis

Inflammatory muscle diseases

Symptoms to watch out for and report to a doctor:

Persistent fever without a source

Chronic fatigue, malaise

Weight loss

Joint pain, swelling, morning stiffness

Rashes, skin ulcers, oral ulcers

Raynard Phenomena - vasospasm that cause white to red to blue color

changes in the skin

Shortness of breath

Jaundice, pallor

Bruising, bleeding

Muscle pain or weakness

Chronic abdominal pain, diarrhea

Hair loss

Swollen lymph glands

Urinary blood or protein

Depression or psychosis

Tingling or loss of sensation in extremities

Problems to watch for in any PID -- 1. infection. 2. cancer 3.

autoimmunity.

With CVID, 25% develop an autoimmunity. These include but are not

limited to:

Rheumatoid arthritis

SLE

Irritable Bowel Disease

malabsorption

gastric cancers

Hemolytic anemia

Bleeding problems with platelets

Muscle diseases

glandular disease - thyroid, diabetes, addison,

Selective IgA patients have to watch out for:

Rheumatoid arthritis

Type I Diabetes

SLE

Myasthenia Gravis - severe muscle weakness

Irritable Bowel Disease

Often see risk of Autoimmunity in relatives

No cure for Auto Immune Diseases - Band aides only

Sometimes high dose IVIG is recommended at a ratio of 1-2 grams per kilo

body weight

Sometimes prednisone, anti-inflammatory or immune supression druges are

used.

All 3 must be closely watched for adverse effects, including:

endocrine/metabolic, muscoskeletal/cutaneous, opthalmic,

cardiovascular, psychiatric, and immune supression.

NSAID - offers anti-inflammatory with weak immune suppression - so helps

symptoms but doesn't block future damage.

Cytotoxic drugs - chemotherapy - weak anti-inflammatory, potent immune

suppression, prevents end organ damage but...

adverse affects include cancer, infection, infertility, requires close

watch for toxicity.

Interferon B for MS - modulates the immune response

Hormones that regulate the immune system used in combination with chemo

may be a good option.

Monoclonal antibodies to B cells - block antibody production --

effective in lympho proliferative diseases and cytopenias.

Cytokine blockade - blocks receptors for inflammatory cytokines.

Infleximal - drug effective in adults - unknown in kids - causes immune

supression.

Good presentation until near the end when he lost me on all the

possible treatments that might be recommended and tried to answer

questions about each one. I got bogged down and overwhelmed with

information that I don't currently need. So.....

COPING WITH CHRONIC ILLNESS: ISSUES FOR CAREGIVERS

Coping with Chronic Illness: Pediatric and Family Issues by Tonya M.

Palermo, Ph.D. Rainbow Babies & Children's Hospital

Dr. Palermo was the only lecturer to give copies of her slides out.

THANKS!!!

I didn't make any additional notes because it was so easy to follow.

OUTLINE

*Physical, social and psychological functioning associated with chronic

health conditions

* Issues specific to PID

*Developmental changes in coping with illness

*Stress and burden of caregivers

*Impact on siblings

*Strategies to help children and families cope

PHYSICAL FUNCTIONING

*Capacity to perform age-appropriate activities

--Vigorous physical activities (running, playing, soccer)

--Self-care activities

--Roles (attending school, playing with friends)

SOCIAL FUNCTIONING

--Opportunities to interact with peers

--Ability to maintain normal peer relationships

--Specific social skills and social performance issues (how well fit in

with peers)

PSYCHOLOGICAL FUNCTIONING

*Psychiatric disturbances (e.g., depression)

*Psyhosocial problems

--Behavioral and emotional adjustment

--Low self esteem

--School performance problems

REACTION TO CHRONIC ILLNESS

*Wide range of responses to stress of chronic conditions

*Loss of normal, being different

*Loss of innocence

CHILD PSYCHOSOCIAL ADJUSTMENT

*Behavioral or emotional adjustment problems

--20% of children with chronic health conditions

*Prevalence of psychiatric mood disorders in children with chronic

health conditions is 9%

--About twice that of the general population of children and adolescents

*Many children have depressive symptoms but not clinically significant

depression

DEPRESSION IN CHILDHOOD

*Restlessness, irritability more common

*Physical symptoms (headaches)

*Decreased social activities (talking with others, playing games)

*Increased solitary behavior (playing alone)

PARENTAL CONCERNS

* " My questions are in relationship to mental health and PIDS. We have

had a long history of behavioral problems since infancy. We currently

have a diagnosis of probable early onset bipolar disorder (or some other

mood disorder) and I am wondering whether you have seen a higher

incidence of mental health issues than in other populations of

chronically ill children? "

--Parent of a 6 year old with CVID

WHICH CHILDREN ARE AT RISK FOR POOR ADJUSTMENT?

*Unclear relationship to condition severity (i.e., more severe illness

does not mean more adjustment problems)

*Longer duration of illness

*Girls acknowledge more distress than boys

*Functional status - impaired or not

*Parental adjustment

DEVELOPMENTAL CONSIDERATIONS IN ADJUSTMENT REACTIONS

*Toddler - safety, control, pain from procedures

*School-age child: participation in activities, school functioning,

parental protectiveness

*Adolescent: privacy and confidentiality, physical appearance, peer

relationships, social functioning

DEVELOPMENTAL TASKS OF THE CHILD WITH A CHRONIC ILLNESS

*Illness-related stressors: treatment regimen, hospitalizations,

altered physical appearance, school absences

*Typical childhood tasks: development of autonomy, identity formation,

establishment of peer relationships

EXAMPLE OF INTERPLAY OF ILLNESS-RELATED AND TYPICAL CHILDHOOD TASKS

*4 year old girl diagnosed with hypogammaglobulinemia treated with IVIG

*Extreme distress with infusions

*Recent history of separation anxiety

--refused to attend preschool, resisted sleeping alone

*Parents unsure of child's health or treatment status

PARENT PSYCHOSOCIAL ADJUSTMENT

*stress, burden

*role restriction

--the degree to which mothers feel the demands of the parenting role

control and dominate their lives, restrict their freedom, and hamper

their attempts to maintain individuality

*higher risk for depression

IMPACT ON SIBLINGS

*Siblings have their own emotional reactions

--worries about their ill sibling, guilt at normal sibling rivalry,

loneliness, and anger

*Concerns about parents' feelings

*Complaints about family communication

*Resentment and jealousy of added attention the ill child receives

DISRUPTION OF ROUTINE

*Anxiety about unpredictability of events

*If sibling gets sick, activities may be missed, or cancelled

*Anger at parents, sick child for ruining things

HELPING SIBLINGS COPE

*Keep the lines of communication open - both ways

*Acknowledge siblings' feelings, worries, and needs and give plenty of

chances to express them

*Be wary of minimizing concerns

*Remember that siblings want to talk about something other than illness

HELPLING SIBLINGS COPE

*Normalize sibling life - treat the sick child as normally as possible

*Redirect attention to the child tactfully when others focus only on the

sick child

*Spend special time with health siblings

SIBLINGS NEED BREAKS, TIME AWAY

WHAT MAKES PID DIFFERENT FROM OTHER CHRONIC HEALTH CONDITIONS?

*Onset may range from childhood to adulthood

*Several family members may have PID

*Exact prevalence of the disorders is unknown

*Relatively unknown in the public eye

CURRENT AGE OF PATIENTS WITH PID

Patient survey of 2,745 patients

5% were over 65 years of age

7% were between 55-64 years of age

13% were between 45-54 years of age

15% were between 35-44 years of age

10% were between 26-34 years of age

10% were between 18-24 years of age

10% were between 13-17 years of age

20% were between 6-12 years of age

10% were between 0-6 years of age

SPECIFIC ISSUES IN PID

*Financial and time commitments

*Pain/distress related to IVIG treatment

*Psychological difficulties (e.g., depression)

*Non-compliance with treatment regimen

*Organization/delivery of services may be fragmented

*Role constraints (e.g., missed school, work)

*Uncertainty about the future

QUALITY OF LIFE ISSUES

*Physical, social, and mental well being more impaired than healthy

patients and patients with other chronic conditions such as diabetes or

asthma

--roles at school, work, home, with friends

--general health

--impact on parent personal time

--physical functioning

CURRENT PHYSICAL LIMITATION

Of 2,647 patients reporting

42% said they had NO physical limitations

28% said they had SLIGHT physical limitations

21% said they had MODERATE physical limitations

9% said they had SEVERE physical limitations

CRITICAL INTERVENTION PERIODS

*Predictable stress points (e.g., at diagnosis)

*Developmental transitions (e.g., beginning school)

*Day-to-day stresses

TYPES OF INTERVENTIONS

*Education

*Social Support

*Stress Management

*Counseling

EDUCATION AND ADVOCACY

*Patient education

*Educating important members of your community (school, work)

*Understanding your legal rights

*Obtaining patient advocacy

MAKING HEALTH-RELATED DECISIONS

*Obtain information from a variety of expert sources

*Talk to people you trust. Remember, opinions from friends and family

can be misinformed and emotionally charged

*Consider long-range goals

*Take notes during meetings with medical team so you don't have to rely

only on your memory.

SOCIAL SUPPORT INTERVENTIONS

*Help patients form friendships with others who have similar health

conditions

--acquisition of illness knowledge

--improve disease management skills

*Help patients establish positive peer relationships in a non-disease

oriented environment such as school, work, etc.

SPECIFIC PARENTAL CONCERNS IN THE SCHOOLS

*Misperception of child's illness

--fear of contagion

*Dealing with missed school days

*School performance

*Sick classmates

*Infection precautions

PARENTAL CONERNS

" Her teachers make snide remarks about her being absent so much. Other

kids make nasty remarks. What happened to compassion in people?? --

parent of 11 year old with selective IgA deficiency

POTENTIAL ROLE OF TEACHERS AND NURSES

*Facilitating child adjustment

*Educating classmates concerning childhood chronic illness

*Normalizing illness-related tasks

*Promoting age-appropriate independence and functioning

PARENT RESOURCES

*Websites

*Educational materials

*Children's books

*Summer camps

STRESS

*Any change in your body or health

*Common experience in patients with illness, treatments, life with

chronic health condition

SYMPTOMS OF STRESS

*Headache, backache

*Muscle tension

*Fatigue, weakness

*Stomach upset

*Constipation, diarrhea

*Teeth grinding

*Hypertension

*Easily distracted

*Cold hands and feet

*Susceptibility to cold or flu

*Tics, tremors

*Sleep disturbances, insomnia

*Weight gain or loss

*Indecisiveness

SUGGESTIONS FOR IMMEDIATE STRESS REDUCTION

*Do something physical

-- Exercise, go for a bike ride, jump rope

--relax (breathing, yoga, massage)

-- get away physically or mentally

*Do something fun

SUGGESTIONS FOR LONG-TERM STRESS REDUCTION

*Take care of your body (eat right and regularly, sleep, regular schedule)

*Organize your life

*Set realistic goals

*Prioritize

*Keep a sense of humor

*Consult a professional

LEARN TO IMPLEMENT EFFECTIVE COPING STRATEGIES

*Communicating and expressing emotions

*Focusing on positive aspects

*Managing information

*Seeking support

*Take control of what you can, let go of what you can't

KEEP A SENSE OF HUMOR

WHAT CAN YOU DO TO HELP YOUR CHILD TO MANAGE OR PREVENT STRESS

*Promote good lifestyle choices

--sufficient sleep

--eating habits

--realistic schedule/activities

*Build in " down time "

*Model good coping and stress management

*Seek professional help when needed

NONPHARMACOLOGICAL PAIN OR STRESS MANAGEMENT

*Distraction

*Self-hypnosis

*Imagery

*Relaxation

*Deep breathing

ADHERENCE WITH TREATMENT

*Medication

--pills

--using inhalers

*Procedures

*Other regimen tasts

POTENTIAL BARRIERS TO ADHERENCE

*Misunderstanding of important

*Attitude or concerns

*Worries and expectations about illness

*Unable to swallow pills

STRATEGIES TO INCREASE ADHERENCE

*Make sure child understands treatment -- directly from health care provider

*Increased monitoring

*Cues and reinforcement

*Tailoring the regimen to child's routines

*Teaching specific skills (e.g., pill swallowing, coping strategies for

procedures)

SPIRITUALITY

*May start to ask questions about the meaning of life, the purpose of

suffering, the existence of God, and the effectiveness of prayer.

*Spirituality is not a religion; your spirituality is your unique

philosophy of life, your view of the universe

CHILDREN'S SPIRITUALITY

*Can offer home, meaning, direction, acceptance, understanding

*Children trust what parents tell them about God and take every word

literally

*Communicate beliefs that will sustain your child.

RECOGNIZING SPECIAL RISKS FOR STRESS AND ADJUSTMENT PROBLEMS

*Difficulty managing the essential coping tasks such as misunderstanding

the realities of diagnosis or treatment

*Not being able to develop skills to accomplish health care

responsibilities.

*Having emotional reactions and distress that are out of control

*Not being able to maintain good relationships with others

*Difficulty handling medical treatment procedures due to anxiety or fears

*Depression

*Previous or coexisting family problems such as a family crisis apart

from health concerns

GOALS OF INTERVENTION

*Mastery of anxiety and fears related to the illness and its management

*Developmentally appropriate understanding of the illness and compliance

with treatment regimens

*Integration of the illness into family life

*Successful adaptation to important systems, such as school and peers

(Do you now see why 1 hour sessions just are not long enough to cover

this!!!!)

Then she passed out a page labeled Parent Resources

Books:

Children's story books about coping

- The Magic Tree by T. Obinkaram Echewa

- The Worry Stone by nna Dengler

- and the Terrible, Horrible, No Good, Very Bad Day by Judith

Viorst

- Peeling the Onion by Orr (for older children)

Children's storybooks about illness

-Little Tree: A Story for Children with Serious Medical Problems by

Joyce Mills

Washington, D.C., Magination Press, 1992

-What about Me? When Brothers and Sisters Get Sick. by Alan kin,

Washington, D.C., Magination Press, 1992

Parent resource Books

-Brothers, Sisters, and Special Needs: Information and Activities for

Helping Young Siblings of Children with Chronic Illness and

Developmental Disabilities by Debra Lobato, 1990

- The Intelligent Patient's Guide to the Doctor-Patient Relationship:

Learning How to Talk so Your Doctor will Listen. By Barbara Korsch,

M.D. and Caroline Harding, 1998

-A Child in Pain: How to Help, What to Do. By Leora Kuttner, Ph,.D. ,

Hartley & Marks Publishers, 1996

-Spinning Inwards by M. Murdock, Shambhala Publications Inc. 1987

(resource book on using imagery with Children)

Websites

School Issues

- Rainbow Babies & Children's Hospital -

http://www.rainbowbabies.org/families/schooloutreach.asp

-Bandaides and Blackboards: Chronic Illness in the classroom:

http://www.faculty.fairfield.edu/fleitas/contenta.html

Advocacy

-Center for Patient Advocacy: http://www.patientadvocacy.org

Support

-National Parent-to-parent support and information system:

http://www.iser.com/NPPSIS-GA.html

Compiled by Tonya Palermo, Ph.D. for the IDF National conference 2003

COMPLEMENTARY AND ALTERNATIVE MEDICINE: ASSESSING ITS ROLE IN PID

I attended this one because it was presented by a researcher at NIH

who is examining the role of complementary and alternative medicine in

PIDS. So I was very interested in learning.

Okay, the man from NIH who was supposed to speak had a death in the

family and couldn't show. One of his assistants did his speech and then

talked about her own research. HIS specialty was with alternative

treatments that include supplements, etc. HER specialty was with stress

management. This became obvious as she spoke because she was not

aware of any contraindications for PID using supplements that boost the

immune system, which made this speech very dangerous for those who did

not realize that THERE ARE CONTRAINDICATIONS for people with IMMUNE

DEFICIENCY. She spoke as if we were a typical audience not affected by

PID. She repeated over and over that she had not had time to review

PID and was not sure of the interactions. So, I'm going to type my

report as she reported it -- but I do so with GREAT concern that you

realize that this was NOT her specialty and she had not researched the

possibility of interactions that could cause autoimmune reactions. I

really wanted to hear from the gentleman who was scheduled to talk to

see if he could give us some guidelines on what was okay to try and what

was not. This did not happen.

COMPLEMENTARY and ALTERNATIVE MEDICINE (CAM)

Complementary meaning that we use non-traditional sources in addition to

traditional medicine.

Alternative Medicine meaning that we use non-traditional sources instead

of traditional medicine.

These include:

Manipulative and body based (Chiropractic or Accupuncture)

Biological (Dietary Supplements or herbs)

Energy based (Reiki, Magnets, Qi gong)

Alternative (Homeopathy & Naturopothy)

Mind-Body (Yoga, Prayer or Meditation)

Several things that have caused an increased interest in CAM:

Conventional Medicine has " pushed " the use of CAM because:

of it's failure to yield cures

adverse effects of orthodox regimes

lack of practitioner's time

dissatisfaction with the technical approach

fragmentation of care by using specialists

CAM has pulled into it's net because:

media reports of dramatic results

belief that CAM is natural

focus on spiritual & emotional well-being

therapist takes time with patients

In 1998 29% of adults used some form of CAM therapies

Women & college graduates used as follows: 10% used herbals, 8% used

chiropractic, and 6% used massage therapy.

HIV patients were using: imaging, high dose vitamins, massage,

relaxation, herbs and accupuncture.

1/3 of U.S. doctors are including CAM in their recommendations.

PROBLEMS USING CAM

(and this is vitally important for PID)

INCONSISTENT PRODUCT AND PRACTICE STANDARDS

you don't know what's inside those bottles!!!!!

no one regulates purity, or content.

bottles claiming to have a certain amount have varied

LOTS OF CLAIMS/FEW RESEARCH RESULTS

In a normal NIH trial there would be:

Preclinical - Phase I, Phase II, Phase III, obtain FDA approval, and

then marketing would begin.

In CAM it's already been marketed and none of the above has been

accomplished. So, NIH has thrown away all the pre-clinical phases and

just tried to test the results.

CAM approaches for PID

1. to improve physical and emotional well-being

2. to amerliorate disease symptoms and side effects of treatments

3. enhance deficient immune system (THAT'S THE DANGEROUS ONE!!!!!!!!!!)

One of the best things PID can do is to take responsibility for one's

own health

1. increase mobility - exercise, yoga

14% of all US deaths are attributed to lack of activity and poor diet.

sedentary lifestyles are linked to 23% of deaths from major chronic

diseases

physical activity reduces risk of death from heart problems

reduces development of diabetes and high

blood pressure

reduces development of colon and breast cancers

helps build healthy bones and muscles,

joints, keeps healthy weight

helps older adults to become stronger and

avoid falls

reduces feelings of depression and anxiety

Walk as fast as you can but still be able to talk or whistle. Good goal

to keep in mind so as to not over-do or under-do.

One way to decrease pain is accupuncture, accupressure and massage

Accupressure produces opiate-type response in brain same as pain killers.

Best used for pain and for nausea post-chemo

Then she began to talk about her specialty and really warmed up to the

subject.

Her specialty was Coping Effectiveness Training.

This helps to remove frustrations, depression, improves sleep, helps

medications to work better, and gives more energy.

With PIDS stress relief is absolutely necessary (DUH!)

Negative emotions produce physiological processes. Health behaviors

become negative and cause social isolation.

In what is called the NUN Study. NIH has determined that thinking

positively increases life span. They asked each Nun as they enter the

convent to write their biography. A group of researchers rated the

biographies for positiveness or negativeness and set them aside. Many,

many years (I wish I had written it down) they discovered that the

positive thinking nuns were in better health and lived longer than the

negative thinking ones.

Her technique asks us to examine our stress. She called this general

stress and to write down and discover specific stressors.

Then look at the specific stressors and determine if they are changeable

or unchangeable. If they are changeable - you need to employ

problem-focused strategies and stressed problem-solving and negotiating

skills. But if they were unchangeable -- you need to employ emotional -

focused strategies such as relaxation, reframing and positive growth.

Some of these emotional strategies are:

reframing is putting a different frame on the same picture using

positive words instead of negative. The glass is half empty or the

glass is half full depending on the frame you put on it. Is this

failure a set-back, or one more posibility ruled out to help you reach

your goal?

acknowledge and share the beauty and the positive. (take time to smell

the roses) (look for the good side of things)

use positive accounting at the end of the day (instead of saying " I

didn't get this, or this, or this done -- say instead -- I DID get

this done)

look for lessons learned and positive moments (well, at least I learned

not to try THAT again!)

use humor (sometimes it's just a choice on whether to cry or laugh)

identify sources of positive meaning in life (focus on personal value

and goals)

We can focus only on the weeds in the garden, or we can plant flowers!

When these CET stratagies are employed they have seen: less burn out,

less stress, and positive growth.

And NIH has found that employment of these CET strategies enhance immune

function with a heightened resistance to infection.

They took a group of people through CET training and a group of people

who had not had CET training and introduced a rhinovirus by nose spray.

The group with CET training resisted the " germ " at a much higher rate

than the control group without CET training.

She then shared the following warnings and reports:

DSHEA definition of all oral supplements keeps the following out of the

control of FDA. So THERE IS NO CONTROL!

Right now, oral supplements can make outlandish claims as to their

effectiveness without any legal consequences. Everyone is rushing

around trying to get Truth in labeling required and Truth in claims

required just like Food or Drugs. The following are at this point

exempt from ANY REGULATIONS:

vitamins

minerals

herbals

amino acids

any other diatary substance

Ginsing is one example that NIH looked at. Some bottles contained half

the amount of active ingredient claimed, others had 3 times the dose.

Some were contaminated (BIG WARNING TO US), There is no safety, no

standardization, and no report of drug interactions with other common

drugs.

For example, St. 's Wart blocks birth control pills, cancer drugs

and blocks absorption of many drugs. No one knows what it does with IVIG.

To get more information regarding CAM, please visit www.NCCAM.NIH.gov

That's my report. I was disappointed not to get more information about

probiotics and other herbal supplements. The speaker did encourage us

to write the above government agency and tell them our questions.

Because their clients are the public and if the public wants certain

research to be done -- they need to request it. You can also contact

your representative or Senator to ask for more research to be done

toward certain subjects. Essentially our day on Capitol Hill was to

request Congress to instruct NIH to spend every penny that had been

alloted on PID on research for PID and nothing else.