Supporting Loved Ones
A guide to give family members when they ask, "What can we do to help?"
Cystic fibrosis (CF) is one of the most common and serious genetic diseases in America. CF affects the respiratory (lungs), pancreatic, and gastrointestinal (GI) systems. It can also affect the sinuses, liver, spleen, and reproduction. It is a chronic disease that currently has no cure.
In the 1940's, CF was a fatal disease of early childhood. Today, thanks to advances in medical care, children can expect a much longer lifespan. Most children grow into adulthood. Many attend college, marry, and lead very productive lives. The median age of survival (half live longer, half die younger) is the late 30s.
Cystic fibrosis is present at birth, although it is not always found then. CF occurs because both parents carry a CF gene and each parent has given one to their child. It's important to remember that every child with CF has CF genes from both the mother and the father. The CF gene is common (one in 28 Caucasian Americans carries the gene) and marriages between carriers occur frequently (about one in every 400 marriages). Not every child from a family will have CF. Other children could receive a single CF gene from just one parent and become a CF carrier. Or, they could receive no CF gene and be free from CF.
Glands in the body that usually make thin, slippery secretions
(like sweat, mucous, tears, saliva, or digestive juices) make,
instead, thick, sticky secretions. These thick, sticky secretions
plug the ducts (small tubes) that carry the secretions either
outside the body or into hollow organs like the lungs or intestines.
This can affect vital body functions such as breathing or digestion.
In The Lungs
People with CF have thick mucus, which can trap germs in the lungs. Thick mucus is hard to clear, so the germs stay and grow in the lungs. Once these germs make a home in the lungs, there are more frequent lung infections. Frequent infections start a cycle of inflammation and infection, which becomes a chronic problem. Most people with CF die from respiratory failure.
In The Gastrointestinal (GI) System
The GI system is also often impaired in children with CF. Thick secretions block the pancreatic duct, so the enzymes used to break down food can't get to the small intestines. This is called malabsorption. It can lead to cramps; gas; bulky, foul-smelling stools; and poor growth.
Most people with CF have to work hard to stay healthy. Their daily care and treatments can take a lot of time. The treatment of CF varies with most children; but many will need to clear the thick secretions from their lungs. This is called airway clearance. There are several types of lung clearance therapies. Chest physiotherapy (CPT) involves clapping on the child's back to loosen lung mucus so that the child can cough it out. The Vest is a machine that is worn which vibrates the mucus loose. Devices like the Flutter® and Acapella also provide airway clearance.
Children with CF take many medicines. They may be on bronchodilators, steroids, anti-inflammatory medicines, or antibiotics. They may need to take antibiotics by mouth, intravenously (IV), or as a breathing treatment to fight lung infections. It's hard for some children to gain weight, so they must take pancreatic enzyme supplements with meals and snacks to help their digestion. Vitamins may be prescribed.
Care for the healthy CF child can take up to 1-2 hours a day. this involves taking the medicines and doing airway clearance. The amount of care goes up if the child is sick. At times, a child with CF needs IV medicines. These can be given at home. At other times, a doctor will admit the child to the hospital for 1-2 weeks for IV antibiotics and intense airway clearance.
SUPPORTING YOUR LOVED ONE WHO HAS A CHILD LIVING WITH CYSTIC FIBROSIS
By Siri Vaeth-Dunn
For many, the diagnosis comes after a long time of feeling frustrated and helpless. Many parents have watched their child go through many tests and procedures to find out why their child is ill and not growing well. For other parents, the diagnosis comes at birth, when the child is found to have an intestinal blockage. This often affects newborns with CF. Usually, there is no family history CF, so the diagnosis is a shock. Some may feel relief that they finally have a name for their child's symptoms. This relief is brief, as the reality of coping with CF sets in.
There is no way to prepare for the diagnosis of a chronic, life-threatening disease, for which there is still no cure. Families go through many emotions
- Denial - disbelief that their child has CF
- Grief - mourning the loss of their hopes that their child would be fine and that life would go back to "normal"
- Anger - asking why this has happened to their child and to their family. Feeling that "it isn¹t fair."
- Depression - feeling deep, long-term sadness about the diagnosis
- Fear -worrying that their child's health will decline quickly; fearing that their child will die; fear of the unknown.
Besides the shock of diagnosis, parents new to CF may be overwhelmed by all there is to learn to give daily care, including respiratory therapies, medicines, and nutrition supplements. They may feel pressure to become CF experts too quickly.
Parents vary in their reactions to diagnosis. They have varied ways of coping. One parent may want to learn all about the disease while the other may be too depressed to learn more. CF takes a lot of time and money to treat. Some parents may have to rethink their work, daycare, vacation, school, insurance, travel, and finance plans.
Use these ideas as a guide when your loved ones ask what they can do to help.
- LEARN. Ask if there is anything you can read to learn about CF. Use the internet to learn more. The Cystic Fibrosis Foundation (www.cff.org) and the Boomer Esiason Foundation (www.esiason.org) are good sites.
- GIVE EMOTIONAL SUPPORT. Ask what you can do to give support. Accept the pain and sadness they may be feeling. They may be dealing with hope, denial, grief, and fear.
- DO NOT EXPOSE THE FAMILY TO ILLNESS. For those with CF, there is no such thing as "just a cold." Viruses can have a much bigger impact. They can lead to more severe infections for the child with CF. When you visit the family, wash your hands when you enter their home. If the family with CF is coming to your house to visit, and someone in your house is ill, don't wait until they get there to tell them. Call them. Give them the option to stay home. Everyone in the family needs yearly flu shots.
- LEARN CF CARE. Daily CF care varies and may include enzymes, antibiotics, vitamins, and other medications. Most children will need respiratory therapy, like the Vest or chest physiotherapy (CPT). Ask questions. Have your loved ones show you how to give enzymes, medications and respiratory therapy. Maybe you could care for the child and let the parents rest. Don't neglect routine care like check-ups and immunizations.
- GIVE TO CF ORGANIZATIONS. Participate in CF fundraisers. By joining the search for a cure, you give hope to the family and lessen their sense of aloneness.
- SPEAK OUT ABOUT CF. Most people have not heard of CF or know little about it. Teach others about CF and the need to find a cure. Teach others about infection control. Involve others in CF fundraisers. Be aware of government funding for medical research at the National Institutes of Health and Public Assistance programs such as Medicaid, Maternal and Child Health Programs, and Adult CF Programs. Join the CF Foundation's Public Policy Alliance (http://www.cff.org/legislative_action/what_is_the_public_policy_alliance/).
- BE SENSITIVETO THE TYPE OF STORIES YOU SHARE WITH THE FAMILY. Families hearing about people who have died from CF does not help. Lung transplants may extend the life of a CF patient, but they are not a cure for CF. What you can do is check your license to make sure you are an organ donor. While parents must hope for the best for their child, they live with the fear of the worst. Think about the impact of a CF-related story before you share it.
- TREAT KIDS WITH CF THE SAME AS OTHER KIDS. Children need to be treated "normally." Be sensitive to their special needs and fears, but don't be too careful. Children with CF need limits, just like their non-CF peers.
- EVERYONE'S CF IS UNIQUE. Some may have mild lung disease, others may be hospitalized often for lung, sinus, liver, and GI problems. Many things decide the course of the disease for each person.
- PAY ATTENTION TO SIBLINGS. The brothers and sisters of children with CF often feel left out, due to the extra care that the child with CF needs. Some feel jealous of the time parents spend with the other child doing treatments and going to appointments. Give special time to the siblings to help them cope with their doubts and fears.
- WHEN THE CHILD WITH CF IS IN THE HOSPITAL, ASK HOW YOU CAN HELP. Call before you visit, in case the child or parent is trying to rest. If you are sick, don't visit. Don't bring your kids, if they are sick. Ask about special precautions before you visit. Wash your hands when you enter the room. Offer to stay with the child so the parent can rest. Arrange a play date for the sibling, who may be feeling left out. Help with shopping, washing, and cleaning.
- DON'T SMOKE. People with CF work daily to slow lung damage. Do not expose a family coping with CF to cigarette, cigar, or pipe smoke. Second-hand smoke is bad for all. For people with CF, it's worse.
- SHOULD YOU BE TESTED TO SEE IF YOU CARRY CF? CF is a genetic disease. If you are related by blood to someone with CF, it is possible that you can carry the gene. If you're thinking about having children, knowing your status makes a difference. Also, health outcomes are improved with early diagnosis.
- CF IS A LIFE-LONG DISEASE AND COPING CHANGES OVER TIME. People tend to offer help right after diagnosis. Coping with CF is like riding a life-long roller coaster. The best help is long-term help.
Cystic Fibrosis Foundation 6931 Arlington Road Bethesda, Maryland 20814 www.cff.org
Cystic Fibrosis: A Guide for Patient and Family by Daniel M. Orenstein.
Third Edition. Published by Lippincott-Raven Publisher. 2004.
Glossary of terms
The removal of mucus secretions from the lungs by coughing or other methods.
Something that stops swelling
Medicines that kill bacteria (not viruses)
Medicine that opens and relaxes the lungs to aid breathing.
Someone that has one CF gene instead of two. They do not have CF but can give it to their child. For a child with CF, each parent either has CF (two CF genes) or is a carrier (one CF gene).
Chest clearance therapies
Treatments to clear lung mucus (chest physiotherapy, the Vest™, the Flutter®, Acapella™, etc.).
Chest physiotherapy (CPT)
Treatment to break up and loosen lung mucus so that it can be coughed out.
Lasting a long time. CF is a chronic disease. The opposite of "chronic" is "acute".
Tubes or pathways for secretions. Ducts are found in organs, organ systems, and glands. In CF, thick mucus can clog ducts and block secretions.
Enymes help to break down foods during digestion. In CF, mucus can block the tube that carries enzymes from the pancreas to the food. People with CF may take extra enzymes to help digest their food.
Relating to the stomach and intestines
The basic unit of heredity. Genes decide a big part of what people are like (eye color, looks, height, health). CF is caused by a defect of a gene. If the Dad's sperm has a CF gene and the Mom's egg has a CF gene, the child will have CF.
Having to do with genes (See "Gene"). A trait passed on from one family member to another.
Viruses or bacteria
A cell, group of cells, or organ that makes a secretion for use in the body
A secretion of certain glands. Hormones manage body functions like growth, maturing, and heart rate. Hormones are not affected by CF.
Shots needed to protect from illness
Stopping the spread of illness by washing, cleaning, avoiding sick people, etc.
The swelling of body tissues due to irritation or injury. Inflammation is a process by which the body's white blood cells and chemicals protect us from infection and foreign substances such as bacteria and viruses. Inflammation occurs with an infection.
Something that blocks the flow of food or feces in the intestines
Poor uptake of nutrients from food for use by the body. In CF, mucus can plugs the ducts that carry the enzymes and hormones used in digestion. The body can't digest food as well so doesn't get the nutrients from the food. The body needs nutrients for health and growth. A common symptom of CF is failure to thrive.
The middle point in a line of values. Above and below the median are an equal number of values. In "1 4 5 9 12", "5" is the median. Two numbers are above the 5, and two numbers are below it.
A thin, slippery fluid made by mucus membranes and glands. In CF, mucus is often thick and sticky.
Pills, fluids, snacks, and drinks that give the body extra nutrition.
Long gland-like organ found behind the stomach. The duct part of the pancreas secretes enzymes into the intestine to help break down food. In CF, mucus may clog the ducts and block digestion. The other part of the pancreas contains endocrine tissue, which makes the hormone insulin. Insulin controls how the body uses and stores sugar.
Pancreatic Enzyme Supplements