Living With Primary Immunodeficiencies

IMPORTANT ELEMENTS FOR SUCCESSFUL COPING

In addressing the emotional aspects of living with the Primary Immuno- deficiency disorders, in my opinion, there are five very important elements in successful coping for the patients, parents and families.

1. Be knowledgeable about the disorder.
2. Maintain a positive attitude.
3. Accept limitations and changes in your lifestyle.
4. Have a support system.
5. Some people find having a healthy spiritual life is helpful.

1. BEING KNOWLEDGEABLE
Learn about your particular immunodeficiency disorder so that you can communicate intelligently with your doctor and nurse, your own family, and others involved with your family. Read, ask questions, learn. Here are suggestions from Dr. Ted Wymslo, Director of the Miami Valley Hospital Family Health for Caring magazine:

"TIMES HAVE CHANGED: IT'S OK to ASK YOUR DOCTOR QUESTIONS"

How to get the information you want from your doctor:

Write down questions before you go into the office.

Be assertive (that's different from aggressive) about raising
your concerns. If the conversation gets sidetracked, bring it back to your concerns.

Try to be specific in your descriptions of the symptoms or problems.

If the doctor is called out of the room while you are talking, help him/her to remember where you were in the conversation.

Do not be embarrassed to ask for an explanation of a medical term. Ask the doctor to explain it in simple terms until you are satisfied that you understand the explanation. Ask the doctor to draw a diagram or sketch that will make the idea clearer.

If you want more information, ask the doctor about pamphlets or books on the subject. Write or call local branches of national organizations related to your illness for literature.

Join a support group. Besides encouraging each other, care givers can help teach each other practical solutions to common problems.

Be considerate of the doctor's next patient. When you make the appointment, let the receptionist know that you may need extra time for discussion.

Ask, "What symptoms or signs of improvement should I watch for?What are the side effects of this drug? How does this disorder usually progress?

Bring along a friend or family member to help you listen and take notes for you.

Repeat anything vital that's been communicated. Make certain that you understand and have written down what it is that you are to do when you leave - especially having to do with drug dosage or dietary or activity restrictions.

Remember that there are some questions that your doctor simply cannot answer. There are unknowns.

Remember that you have options. You are entitled to a second opinion. If you find your doctor to be consistently uncommunicative and unresponsive to your questions, it may be time to seek another physician."2

HELPFUL PUBLICATIONS:

Several national organizations within IPOPI offer excellent publications explaining the primary immune deficiency disorders and treatment.

Living with Primary Antibody Deficiency

What are Primary Immune Deficiencies?

Travelling with Primary Immune Deficiencies

The History of Immunoglobulin Therapy

Information for IgA Deficient Patients

About...Subcutaneous Immunoglobulin Therapy

About...Intravenous Immunoglobulin Therapy: Information for Patients
Available from:
PiA (Primary Immunodeficiency Association of the U.K.)
Alliance House
12 Caxton Street
London SW1H OQS
United Kingdom Telephone: +44 207 976 7640 FAX: +44 207 976 7641

PATIENT AND FAMILY HANDBOOK
For the Primary Immune Deficiency Disease
Our Immune System (a children's book)
Available from:
Immune Deficiency Foundation
245W. Chesapeake Avenue Suite 206
Towson, MD 21204 USA Telephone: 410 321-6647 and 1- 800-296-4433

CONSENSUS DOCUMENT FOR THE DIAGNOSIS AND MANAGEMENT OF PATIENTS WITH PRIMARY ANTIBODY DEFICIENCIES
Available from:
The Royal College of Pathologists
The Royal College of Physician
The Primary Immunodeficiency Association (PiA)

TEN WARNING SIGNS
Available from:
The Jeffrey Modell Foundation Inc.
43 West 47th Street New York, N.Y. 10036 U.S.A.

UPDATE
Available from:
International Patient Organization for Primary Immunodeficiencies (IPOPI)
Alliance House
12 Caxton Street
London SW1H OQS Telephone +44 207 976 7640
United Kingdom FAX: +44 207 976 7641

2. MAINTAINING A POSITIVE ATTITUDE

Lemons or Lemonade?
A positive attitude will turn lemons into lemonade, and to get and maintain a more positive attitude we need to give ourselves opportunities to hear the good things too and not be consumed by the sorrow. We can learn from the positive experiences of others. If you are the person with primary immune deficiency you must try to do everything in your power to be as healthy as possible. This means staying on schedule with your treatment, following a good routine of getting necessary medical attention when symptoms first occur - don't put it off. It may mean being more aware of good nutrition and hygiene - beware of germs! Beware of people with germs!

If you are the care giver, you must try to keep yourself healthy physically and mentally. Trying to be perfect and all things to all people is exhausting and not conducive to optimism. Make time for yourself. If you feel good about what you are doing for yourself, you will have a more positive attitude.

One of the biggest problems for care givers is fatigue, and this damages your spirit of optimism. "The widespread belief that not getting enough sleep increases susceptibility to infections and possible other illnesses appears to be confirmed by recent studies.

Sleep stimulates the production of various cytokines and other protective components of the immune system, and in laboratory animals, sleep deprivation is associated with depressed immune system function. In humans, healthy slow-wave sleep is associated with a marked increase in certain immune system activities, and a decrease in those components associated with fatigue and drowsiness.

Stress is a major cause of insomnia and can also depress immune system function. Since lack of sleep itself is stressful, this can set up a vicious cycle. Chronic sleep deprivation is common and insidious, since it may take months for symptoms to appear".5

Parents of young primary immune deficiency patients commonly suffer from loss of sleep and find it difficult to remain emotionally strong when suffering from fatigue. It is normally the mother who is home all day with the sick child and many times, siblings, caring for them along with household responsibilities, trips to the doctor, grocery, etc. Then at night she may not get enough sleep due to a coughing, feverish child. The husbands normally are away at work all day but provide some relief during the evening hours. When a husband realized how exhausted his wife was, he hired a sitter for their children and took his wife to the mountains for a weekend alone. The young mother spent the entire week-end sleeping. She returned home refreshed physically and emotionally.

On the Sunday morning of Mother's Day, a father took their early rising children to a park at 5:30 A.M. to allow his exhausted wife to sleep. What a wonderful Mother's Day
gift!

Since neither parent has the opportunity to make up the sleep loss during the day, it is important for them to find a way to take turns with the caregiving at night during an illness, to allow the other to get needed sleep. In situations where there are more than one family member with primary immunodeficiency this is especially stressful. Being a care giver for a sick child is a joint effort. A single parent has a special need for additional help, especially if that parent needs to maintain a job outside the home. In the U.S., the Family and Medical Leave Act provides parents with a ( limited) paid ' sick leave' to care for sick children. Recurring or lengthy illnesses, however, may make it necessary for a regular care giver to help the single working parent.

Besides finding ways to catch up on sleep, there are other things you can do to care for yourself. Delegate household jobs so that everyone is helping, pay attention to your diet to make sure you are getting proper nutrition, make time for physical activity such as jogging, bicycling, or walking, and do something pleasurable for yourself - read, write or sketch in your personal journal, plant a flower bed, etc. Ladies, take a leisurely bath by candlelight, invite someone for tea in the afternoon, watch a humorous TV movie, etc. And for men, it might be good for you to attend a sports event with men friends or pursue a hobby. It will not be easy to find the time or to feel guiltless taking the time for yourselve, but you need it and you deserve it.

The mother of two sons with agammaglobulinemia writes in Caring magazine:

"How to Develop Optimism"

Identify when you are engaged in negative self talk.

Ask someone to tell you when you speak negatively.

Use positive, empowering phrases several times daily. (Tell yourself you CAN do this job or handle this infusion, you CAN ask for help, you CAN say wha you need.) Try to be around positive people.

Don't put yourself down when you make a mistake

Don't give up. Change takes time!

Though it may not be possible to control the situations in our lives, we can control what we say to ourselves about them. If we tend to focus only on the negative, it will stamp out the positive. As a parent, my attitude is being copied by my children. My children are learning patterns of coping and dealing with life based upon what I do and say every day around them. Parents have a remarkable opportunity to mold a child's approach to life, and a parent's optimistic attitude will be modeled by a child, giving him or her a wonderful asset."3 However, avoid unrealistic, insincere or forced positive remarks.

Charles Swindoll writes about attitude,"The longer I live, the more I realize the impact of attitude on life. Attitude, to me, is more important than facts. It is more important than the past, than education, than money, than circumstances, than failures, than successes, than what other people think or say or do. It is more important than appearance, giftedness, or skill. It will make or break a company...a church...a home. The remarkable thing is we have a choice every day regarding the attitude we will embrace for that day. We cannot change our past...we cannot change the inevitable. The only thing we can do is play on the one string we have, and that is our attitude...I am convinced that life is 10% what happens to me and 90% how I react to it. And so it is with you...we are in charge of our attitudes."

3. ACCEPTING LIMITATIONS AND CHANGES IN YOUR LIFESTYLE

Part of the adjustment means making some changes and some sacrifices. The family routine may be upset occasionally. Siblings may need to miss an anticipated activity because the parents need to care for the sick child. The working parents may need to rearrange their work schedule or work related activities in order to be care givers during an illness. Social life may be disrupted. But these inconveniences can be worked out successfully based on family unity, love, and understanding. The disappointments can be balanced with other enjoyable activities at another time. Adults with primary immunodeficiency may need to adapt to a different work environment that provides flexibility in work hours and a healthier work place. In this era of high tech, many people are working at home with computers. Young adult patients preparing for life's work must be realistic about their health and may want to consider professions that offer flexibility and a healthy environment.

4. HAVING A SUPPORT SYSTEM
HOW FAMILY AND FRIENDS CAN HELP

Grandparents and other relatives as well as friends and neighbors should understand the immune deficiency disorder your child or spouse has and what that means to your family. However, it is not unusual for some relatives to avoid discussing it, preferring to remain detached from your family's situation, and some relatives may never reach a level of understanding and emotional support you need and desire. But you should not allow your hurt, disappointment and anger with them to build up to the point that it damages the family unity, and becomes another stress in your life. Accept your relatives as they are, forgive them for not being willing or able to cope with your problem, and turn to your friends instead. Their interest and loving support are therapeutic, and their willingness to help in practical ways provides both physical assistance and emotional strength when you need it.

It helps to have a group of willing friends to call upon for help so that one person is not called too often. And don't feel guilty that you aren't doing anything in return. You will no doubt have an opportunity later to help them in some way, if it will make you feel better, but remember, a good friend does not require repayment.

HOW SUPPORT GROUPS, E-MAIL AND THE WEB CAN HELP

Each country that is a member of IPOPI (International Patient Organization for Primary Immunodeficiencies) has its own immune deficiency organization that provides literature, newsletters, and occasional local meetings. These meetings for patients and their families offer an excellent opportunity to learn from guest speakers and to get acquainted with each other.

Communicating with others in similar situations can be very helpful in many ways. Just knowing there are other people experiencing your same challenges can be comforting, and talking with them in person or through E-mail can be helpful in practical ways as well as emotionally. IPOPI members gain strength from each other in sharing their personal experiences and their knowledge about medical treatments, hospitals, physicians, and information about current research.

Reported gains from self-help groups:

* Acceptance of me as an O.K. Person
* The feeling that I'm not alone
* Knowing that I'm not lazy, crazy, or neurotic
* Validation that it's not "all in my head"
* New friendships, networking
* Reinforcement for efforts toward positive action
* Help with goal setting
* Improved communication skills
* Help in becoming better informed about my disease6

There are more than 10,000 health-related web sites alone and thousands more on-line support communities. For Web surfers and E mail readers, a good place to start is the IPOPI web site where you will find excellent information just for primary immunodeficiencies, a guest book, a Chat room, and a Forum for interactive communication with others going through the same thing you are experiencing.

Places to go for medical information:

American Association of Immunologists:
http://glamdring.ucsd.edu/others/aai

Health World (provides free access to Med Line):
http://healthy.net

Immune Deficiency Foundation:
http://www.immunedeficiency.org

International Patient Organization for Primary
Immunodeficiencies (IPOPI)
http://www.IPOPI.org

the Jeffrey Modell Foundation)
http://academic.mssm.edu/peds/modell/guidelin.html E-mail address: Modell@AOL.COM

Journal of Immunology:
http://ji.journals.at-home.com/JI

Medaccess: http://www.medacess.com

Medinfo:http://www.medinfo.org

National Institute of Allergies and Infectious Diseases
http://www.niaid.nih.gov/publications

The National Organization for Rare Disorders (NORD)
http://www.nord-rdb.co
Email address: orphan@nord-RDB.com
(provides rare disease information through the Rare Disease Database on the World Wide Web)

OncoLink: http://www.oncolink.upenn.edu/

Yahoo's Health section
http://www.yahoo.com/Health

5. KEEPING A HEALTHY SPIRITUAL LIFE

Having a sick child or a sick spouse or partner, or being the immune deficient patient, is a heavy burden, too heavy to carry by yourself. Bernie S. Siegel, MD, retired general and pediatric surgeon in the U.S. who writes about humanizing medical care, said he thinks life "is like an enormous gift that is handed to you that may be hard to carry, and you may need some help carrying it."7

Don't forget your spiritual needs. Whatever your religious faith, reach out to connect with a higher power for renewed strength, guidance and peace.

In Living With Chronic Illness, author Cheri Regester, who has personal experience living with a chronic congenital disease, says "...illness and troubling questions it raises can and do challenge, reform, and even enrich religious belief, and that this renewed faith, in turn, aids endurance." She continues to say," I find that my faith is a very heali thing for me, because I know it's always there, it's a constant and it's not going to go away".9

An adult with primary immunodeficiency recommends finding a spiritual path you connect with, and believes that the power of spirit and mind should not be underestimated..