May is Cystic Fibrosis (CF) Month and Some Patients Are Living Past Middle Age Due to Treatment Advances

There is no cure for cystic fibrosis. That is the reason the Cystic Fibrosis Research Institute (CFRI) is very vocal and makes it clear that cooperation is needed from the CF community. The ‘community’ refers to CF patients, families, advocates, and the general public.

The term ‘share’, ‘support’, or ‘advocate’, may seem like a cliché. Be assured though that there is a serious message behind each plea for your help.

The Institute is a nonprofit incorporated in 1975 by families with CF children who, at the time, were not expected to live past their teens.

Although they lost their children to CF, many parents remained at CFRI hoping to save the lives of other children.

By the year 2012, CFRI was a million-dollar agency continuing to provide advocacy, education, and other services and programs to those affected by CF, including caregivers and family members.

You Can Help This Month and Every Month

Our readers are encouraged to help others by relating their own experiences with CF.

About CF

Over 40,000 people in the US are affected by CF with a worldwide total of approximately 105,000 patients. CF is a rare disease. It is progressive and it is a genetic (inherited) disorder. CF affects the digestive respiratory, reproductive, and endocrine systems.

The disease is no longer considered a childhood disorder as over half of the patients today are over the age of eighteen. People recently diagnosed have an average life expectancy of thirty to forty years.

The mission of the CF research institute is to be available to the CF community while continuing its dedicated research and support for a cure for this debilitating disease.

Mutations of the CFTR gene cause the production of thick mucus. The sticky substance accumulates and clogs passages of the digestive, respiratory, and endocrine systems, trapping harmful bacteria in the body.

The disease is inherited (autosomal recessive). Both parents must be carriers of the gene. The chances of inheriting the gene vary with each pregnancy. Details are available here.

Data from 2017 indicate that 1,700 CFTR gene mutations exist with 300 proven to cause CF while the balance may simply be ‘variants.’

About Infant Screening 

All U.S. states now include CF screening. Infants who are tested through newborn screening will either receive a diagnosis of CF or are labeled as CF/screen positive/inconclusive diagnosis.

CFRI urges parents to bring the infant to a CF Center to continue the diagnostic evaluation.

Symptoms of CF vary widely depending on age, mutation, or health issues. Primary systems include but are not limited to persistent cough, sinus infections, lung infections, or nasal polyps.

Meconium ileus is a serious primary symptom affecting approximately one out of every five infants with CF. It is a blockage of the intestine that may require surgery immediately.

Usually, infant screening covers common CF mutations. Cases may be identified at a later date suggesting the need for genetic tests that search for a larger range of mutations.

Patients with lung disease that has significantly advanced are candidates for double lung transplantation which is considered to be an option for prolonging a CF patient’s life.

Visit CFRI’s website to view all options and join, donate, share, participate, advocate, and help others: Cystic Fibrosis Research Institute – Research – Education – Advocacy – Support (cfri.org)

 

Rose Duesterwald

Rose Duesterwald

Rose became acquainted with Patient Worthy after her husband was diagnosed with Acute Myeloid Leukemia (AML) six years ago. During this period of partial remission, Rose researched investigational drugs to be prepared in the event of a relapse. Her husband died February 12, 2021 with a rare and unexplained occurrence of liver cancer possibly unrelated to AML.

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