Cystic fibrosis is a genetic disorder resulting from an improper copy of a gene. The gene produces a protein necessary for water and salt transmission in a variety of body tissues.
As a result of the mutation in the gene, mucus produced by the body is thick and sticky instead of thin and slippery. The thick mucus clogs vital air passages, leading to chronic breathing difficulties and susceptibility to lung infection. In the digestive tract, mucus blocks ducts in the pancreas, preventing vital enzymes needed for the digestion of food from reaching the intestinal tract. This incomplete digestive process can result in malnutrition.
Cystic fibrosis is not the same in every patient. It varies in severity and symptoms. Some patients may have serious lung involvement while others suffer primarily from the interruption of the digestive process.
What are the symptoms of cystic fibrosis and how is it diagnosed?
Symptoms considered to be highly suggestive of cystic fibrosis are: very salty-tasting skin; persistent coughing and wheezing or shortness of breath; recurrent colds, pneumonias and sinus infections; excessive appetite but no weight gain; foul-smelling, greasy, bulky stools. Due, in part, to the 1,000 mutations of the CF gene, symptoms vary from person to person.
Early diagnosis of cystic fibrosis is very important because it can minimize the damage done to the body by the disease. The "sweat test" - which measures the amount of salt in sweat - is the standard diagnostic test for those with symptoms. A high salt level indicates CF.
How common is cystic fibrosis?
Cystic fibrosis affects approximately 30,000 children and adults in the United States. One of the most common genetic disorders, it affects one out of every 20, or five percent, of Caucasian Americans who “carry” the gene for CF. The incidence of the disease is about 1 in every 3,200 live babies born. The gene is less common in other ethnic groups with a lesser carrier frequency.
The CF gene is recessive, therefore, any child with CF had to inherit the gene from both parents, even though neither of them may have had any symptoms of the disease.
How is cystic fibrosis treated?
Although there is no known cure for cystic fibrosis, proper therapy started in early childhood and maintained through life can keep the disease under control, minimizing lung damage, maximizing weight gain, and making a productive life possible. CF varies greatly in severity from person to person, so treatment must be tailored to the individual. Therapy is most frequently aimed at reducing bronchial obstruction and lung infection through a regimen of postural drainage, aerosols. and antibiotics. Enzyme therapy is used for digestive inefficiency along with nutritional supplements and extra nutritious foods.
What assistance does the Pharmaceutical Services for Adults with Cystic Fibrosis program provide?
For eligible applicants, the program provides:
Reimbursement for up to $500 toward the cost of the deductible on the client’s health insurance policy;
Financial assistance, up to an individual budgeted allowance, for the purchase of items that are medically necessary for the treatment of cystic fibrosis, including:
Medical equipment and supplies,
How do I know if I’m eligible for the Pharmaceutical Services for Adults with Cystic Fibrosis program?
To be eligible for the program, a person must:
Have a diagnosis of cystic fibrosis verified by a doctor,
Be a New Jersey resident,
Be 18 years or older, and
Have an individual annual income less than $37,929/year (for the year 2004-2005).
How do I apply?
People who meet the eligibility requirements should:
Call the New Jersey State Organization of Cystic Fibrosis (NJSOCF) office at (973) 595-1003 for an application package or
Download application forms and instructions from the Organization’s website (www.njsocf.org)
If I have health insurance, how do I get assistance with the uncovered portion of prescription medications?
Once accepted into the program, NJSOCF provides the client with a monthly budget for allowable prescription medication.
To obtain reimbursement if paying out of pocket for co-pays on medications, forward receipts for insurance co-payments to NJSOCF for reimbursement, or
Prescriptions can be ordered through the Cystic Fibrosis Pharmacy in Florida (1-800-741-4427) and sent directly to you, or obtained at your local Pathmark Pharmacy. If medications are ordered from one of these pharmacies, NJSOCF will be billed directly by the pharmaceutical provider for the insurance co-payments on the medications.
What if I do not have insurance?
The NJSOCF will pay for allowable prescription medications up to your individual budgeted allowance.
Medications can be ordered through the Cystic Fibrosis Pharmacy in Florida (1-800-741-4427) and sent directly to you, or they can be obtained at your local Pathmark Pharmacy. NJSOCF will be billed directly, up to your budgeted amount.
The NJSOCF will link you to all patient assistance programs sponsored by pharmaceutical companies that market certain costly drugs (i.e., Tobi, Pancrease, Creon) that will exceed your individual budgeted allowance.
How do I get reimbursed for my insurance deductible?
Submit an explanation of benefits from your insurance company showing that you have met your yearly deductible.
You will be reimbursed up to $500 annually.
How do I obtain equipment or home IV’s that my insurance company will not cover, or will only partially cover?
NJSOCF will order the needed equipment (i.e., nebulizer, pulse-ox unit, etc.) from approved vendors and will be billed the difference directly after insurance payments, or the full amount if not covered by insurance.
To obtain home IV’s, a social worker must call NJSOCF to make arrangements. NJSOCF will contact a home care service company and arrange for the initial nursing visit, equipment and antibiotics. NJSOCF will be billed directly from the home care service company.
How do I use the Pathmark food card?
NJSOCF will provide a monthly budget for nutritious food.
You are initially required to select a Pathmark store for food shopping.
Present your card at Customer Service to obtain an “accounts receivable invoice.” Bring the invoice to the register when you check out.
The food card is to be used for nutritious FOOD items only, not to exceed your budgeted allowance. Any taxable items, such as household products, will not be accepted by the plan.
Pathmark will bill NJSOCF directly.
Can I purchase nutritional supplements through the program?
NJSOCF provides a monthly budget for nutritional supplements.
Nutritional supplements, including vitamins (i.e. Adeks, Mephyton, SCANDISHAKE ®, SourceCF products, etc.) can be ordered through NJSOCF and shipped directly to you.
Selected items from Pathmark, i.e., Ensure, Boost, energy bars, etc., can be purchased with your Pathmark food card.
If I attend college out of state, am I still eligible for program benefits?
Yes. But note that you must show proof of New Jersey residency, and file a State of New Jersey tax return.
Where can I find more information on CF?
Website addresses are provided as a resource. Listing of websites does not indicate endorsement or agreement with all materials posted.
Janice D. Ibezim, RNC, CPM, MPA
Department of Health/Division of Family Health Services
Program Management Officer (PMO)
Office of Cancer Control (OCCP) Program
New Jersey Cancer Early Education Detection (NJCEED) Program
New Jersey Commission on Cancer Research
Department of Health P. O. Box 360,
Trenton, NJ 08625-0360 Our Locations