CF TX Article,Teen Article,and Make a Wish Article

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Gift of life

Teen recuperating after lung transplant surgery 01/30/03

Coburn

Coburn The Edmond Sun

Shelton relied on her sense of humor to endure her recent lung

transplant surgery, she said. As a child, she learned to understand that a

physician’s ultimate goal is to help and not

hurt her. The 16-year-old Santa Fe High School student received a lung

transplant Dec. 20 at Integris Baptist Medical Center in Oklahoma City

and was released two weeks ago to recuperate at home. “Even though what

they might do is painful physically or mentally, whatever you do, you

can’t lose who you are and what you believe in and what you think is

right,†Shelton said. Her parents, and Mona Shelton, noticed when

was 4 months old, that she had been gaining water weight in her

legs and arms. “I was like a China doll in color,†Shelton said. Her

physician diagnosed her condition as cystic fibrosis. Cystic fibrosis

affects about 30,000 children and adults in the United States. The

genetic disease causes the body to produce an increased amount of thick

mucus that lines large organs such as lungs and stomach. Germs and

bacteria become prone to being trapped by the sticky substance.

Infections and absorption problems with nutrition are symptoms of the

disease. Mucus also obstructs the pancreas, preventing enzymes from

reaching the intestines to help break down and digest food, according to

the Cystic Fibrosis Foundation. Many children would die of malnutrition

before scientists began understanding cystic fibrosis, Shelton said.

Today, CF remains incurable. Neither does it go into remission, but it is

treatable. Passage of oxygen through the airways can be restored, Shelton

said, and enzymes can be taken to help patients digest and absorb food.

Cystic fibrosis is mostly a Caucasian disease. Usually one in 28 people

either are carriers or have CF. Shelton said she and her parents were in

disbelief when physicians advised them to consider a lung transplant. But

they realized the urgency of the matter, and was added to a lung

transplant list about 18 months ago. The organ transplant has given her

an extended quality of life. She understands how people can be squeamish

but encourages them to consider donating their organs when they die.

Also, donors can specify which organs they choose to give. “I’ve tried

most of my life to be normal and keep up with everyone,†she said. “I’ve

always tried to not bring attention to that I’m different and that I have

these conditions. I’ve just always tried to be a normal person.†Ballet,

swimming and horseback riding have enriched her life through her Campfire

USA membership. Shelton enjoys playing the viola as part of the Santa Fe

Orchestra. Baroque is her favorite style of music to be part of ensembles

performed during the school year. Still, misconceptions about CF have

left her disappointed about public reaction to disease. “When you cough

to loosen mucous and stuff, people think, ‘You have a cold. I don’t want

to sit by you,’†Shelton explained. Some fear the disease is contagious,

which is not true. People living with CF tend to have smaller bodies

without much fatty tissue. “People tend to look at you different because

you do look like you’re anorexic,†she continued. “A lot of people

automatically think, ‘Oh, she’s a teen-ager. She must be doing something

wrong. But you’re not and you can’t help it.†Edmond Santa Fe will

provide her with a tutor twice weekly until she returns to school. Her

goal is to return after spring break. Meanwhile, Shelton is adapting well

to a less structured schedule. And she has plenty of school work to

occupy her time. Being at home gives her time to work on time-management

skills, she said. Her morning usually begins at 7:30 a.m., when she

swallows her medicine, takes her vital signs and eats breakfast. After

lunch, she checks her blood sugar and occasionally goes outside when it’s

warm enough to be comfortable. “I walk around the block,†she said, “and

maybe during the afternoon — if my Mom’s home — we go and run errands.

But if I go out, I have to wear a mask.†The CF Walk-A-Thon is planned

this spring in Bethany and Shelton hopes to attend the major fund-raiser

for the Oklahoma City Cystic Fibrosis Foundation. She chooses to do most

of her studies during the afternoon, as well as play the viola in the

house. Agriculture studies is doing a lot to spark her interest. “You can

do homework when you want, but it’s kind of lonely because I’m here by

myself,†she said. Friends have called her to see how she’s feeling. Some

have paid her a visit. Shelton said she can have visitors as long as they

are not contagious with a cold or other contractible-type illness.

“There’s always somebody out there that loves you, and there’s always

somebody thinking about you,†she said.

--------------------------------------

                     DAILY MAIL (London)

                                January 30, 2003

HEADLINE: WE'VE GOT A DREAM

BYLINE: Alice

    FEMAIL has joined forces with the Make-A-Wish Foundation, a charity

which

makes dreams come true for children with lifethreatening illnesses.

    Since launching our appeal in December, we have been inundated with

responses from children hoping their dreams will come true, and readers

have

already pledged tens of thousands of pounds. But the more you pledge, the

more

dreams that can come true. Today, in Make-A-Wish week, ALICE ROBINSON

tells the

moving stories of six children for whom that will happen.

    Kirsty Loughlin

    KIRSTY LOUGHLIN, 13, has cystic fibrosis. She lives with her parents

,

46, a fulltime carer, and Gordon, 39, general manager for Cumbrian

Seafoods, and

brother , ten, in a cottage near Keswick in the Lake District.

    Kirsty has two wishes: to see American singer Keys in concert,

and to

meet the Osbournes. Her mother says: KIRSTY has been poorly all her life.

    Four years ago she had an operation on her lung, and since then

things have

gone downhill.

    A year ago, she had to stop school because she just couldn't cope.

    She was always a very sickly baby. She didn't put on weight, and

always had

coughs and colds. She was diagnosed with cystic fibrosis when she was

nine

months old.

    Kirsty copes incredibly well, but it's heartbreaking to watch her.

She plays

the piano brilliantly and wants to be a musician. It's the one thing that

doesn't tire her out.

    Her name has been on the heart and lung transplant list for two

years, and I

carry a bleeper with me all the time in case the call comes. I pray that

Kirsty

will be given the chance for a new lease of life.

    She understands the enormity of the decision for the parents of the

dead

child.

    She said to me:'Mum, if I get the chance of lungs, I won't let anyone

down.'

Her dearest wish is to see Keys in concert. She would also like to

meet

the Osbournes - she thinks Ozzy is hilarious.

*****************************************

Patient Care® Archive

January 2003

Sexuality in teens with chronic illness

Advances in cancer chemotherapy, open-heart surgery, organ transplant,

antibiotic therapy, home care, and respiratory technology have increased

the number of chronically ill children surviving into adolescence. Some

of these teens become sexually active, presenting a challenge to the

primary care physician.1,2Like any teen, a chronically ill adolescent

risks pregnancy when she becomes sexually active. The repercussions may

be even more serious than in healthy teens, however. Unintended pregnancy

in a girl with heart disease may endanger her health. A young woman with

systemic lupus erythematosus may take teratogenic medications. Pubertal

development in teenagers with sickle-cell disease, cystic fibrosis, or

inflammatory bowel disease may be delayed. Very few chronic diseases are

associated with infertility in males or females, however. Morbidity may

be increased during pregnancy in patients with congenital heart disease,

diabetes, epilepsy, or systemic lupus erythematosus. These diseases may

adversely affect the pregnancy, and pregnancy may worsen the disease.

Diabetes mellitus increases the risk of spontaneous abortion, premature

labor, increased operative deliveries, and congenital abnormalities in

the fetus. The exact relationship between psychosocial problems and

chronic physical illness is not clear, although some studies state that

the adjustment of these teenagers is similar to that of their healthy

peers.The health-related considerations of sexual behavior in adolescents

with chronic disease are as follows. Genetic issues Adolescents with

certain chronic, genetically transmitted conditions such as cystic

fibrosis, beta thalassemia, and sickle cell disease require counseling.

This will empower them to make informed reproductive decisions. Fertility

Chronic disease may affect the fertility of the adolescent directly or as

a result of treatment. Cystic fibrosis causes impaired sperm and

fallopian tube motility resulting in infertility in boys and girls.

Survivors of childhood cancer such as Hodgkin's disease involving

subdiaphragmatic radiation are more likely to be infertile due to the

medications and treatment. Other diseases that might impair fertility in

girls are chronic renal failure and thyroid dysfunction.Pregnancy,

childbirth, and therapeutic abortion Adolescents who conceive may have

added risk with pregnancy and labor. Labor may acutely worsen cardiac or

pulmonary disease, for example. Girls with systemic lupus erythematous

may deliver infants with a discoid rash or congenital heart block.

Pregnancy may exacerbate a disease like asthma.Teratogenicity The

adolescent girl with epilepsy or cancer may be taking teratogenic

medications so classic fetal hydantoin syndrome is likely to be seen in

the fetuses of young women with epilepsy. Teens taking coumadin must be

switched to heparin since coumadin is teratogenic. Patients with cystic

fibrosis or rheumatoid arthritis may need antibiotics or

anti-inflammatory agents, some of which may be teratogenic.Contraception

Many misconceptions exist about the use of hormonal contraception in

adolescent girls with chronic disease. Medroxyprogesterone injection

(Depo-Provera) is an excellent choice for girls with sickle cell disease

and has shown to reduce the frequency of vasoocclusive crises. Girls with

surgically corrected congenital heart disease or diabetes may safely use

low-dose oral contraceptive (OC) pills. However OCs are contraindicated

in those with thromboembolic, pulmonary vascular, or cerebrovascular

disease. Certain adolescents with chronic illnesses such as spina bifida

may have latex allergy and should be advised against using nonlatex

condoms. Teens who are immunocompromised because of medical illness or

medical treatment need constant counseling about barrier methods of

contraception. REFERENCES1. Coupey SM, Alderman EM. Sexual behavior and

related health care for adolescents with chronic illness. Adolesc Med.

1992;3:317-329.2. Alderman EM. Reproductive health for girls with chronic

illness. In: Coupey SM, ed. Primary Care of Adolescent Girls.

Philadelphia, Pa: Hanley and Belfus; 2000: 127-135.

Sexuality of teens with chronic illnesses. Patient Care 2003;1.

Copyright © 2003 and published by Medical Economics Company at Montvale,

Becki

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