20 Participants Needed

Symdeko for Cystic Fibrosis

HH
Overseen ByHeather Hathorne, PhD
Age: Any Age
Sex: Any
Trial Phase: Phase < 1
Sponsor: University of Alabama at Birmingham
No Placebo GroupAll trial participants will receive the active study treatment (no placebo)
Breakthrough TherapyThis drug has been fast-tracked for approval by the FDA given its high promise
Approved in 3 JurisdictionsThis treatment is already approved in other countries

What You Need to Know Before You Apply

What is the purpose of this trial?

This trial aims to explore how certain drugs might help individuals with specific genetic mutations linked to cystic fibrosis (CF). Researchers are testing treatments such as Symdeko (a combination of tezacaftor and ivacaftor), Ivacaftor, and Orkambi to assess their impact on the function of CFTR, a protein involved in CF. Individuals with cystic fibrosis and a mutation that might respond to these drugs could be suitable candidates, particularly if they have managed the condition without recent severe flare-ups. As a Phase 1 trial, the research focuses on understanding how the treatment works in participants.

Will I have to stop taking my current medications?

The trial does not specify if you need to stop your current medications, but it excludes those taking medications with significant drug interactions with CFTR modulators. It's best to discuss your specific medications with the study team.

Is there any evidence suggesting that this trial's treatments are likely to be safe?

Previous studies have used the medications Symdeko, Ivacaftor, and Orkambi to treat certain types of cystic fibrosis (CF). Symdeko has been tested in over 1,000 patients and is generally well-tolerated, with most people not experiencing serious side effects. It is approved for patients aged 6 and older with specific gene mutations. Ivacaftor aids CF patients with a different type of mutation, and research shows it improves lung function and is safe for many. Orkambi combines two drugs to assist patients with two copies of a specific mutation. Studies in both young children and older patients have shown it is generally safe.

These treatments have been tested in various patient groups, and results indicate they are mostly well-tolerated. Prospective trial participants should remember that while side effects can occur, they are often manageable.12345

Why are researchers excited about this trial's treatments?

Researchers are excited about these treatments for cystic fibrosis because they offer targeted approaches based on specific genetic mutations. Symdeko and Orkambi are both CFTR correctors, which help correct the malfunctioning protein caused by cystic fibrosis mutations. Ivacaftor, on the other hand, is a potentiator that enhances the function of the CFTR protein, especially for mutations similar to the wild type. Unlike standard treatments that may not account for individual genetic differences, these treatments are tailored to the patient's specific mutation, potentially leading to more effective and personalized care. This precision in targeting the underlying genetic causes of cystic fibrosis is what makes these treatments stand out.

What evidence suggests that this trial's treatments could be effective for cystic fibrosis?

In this trial, participants will receive one of the following treatments: Symdeko, Ivacaftor, or Orkambi. Research has shown that Symdeko, a combination of tezacaftor and ivacaftor, can significantly improve lung function in people with cystic fibrosis. Studies have found that it enhances lung performance, lowers sweat chloride levels, and improves quality of life. Ivacaftor alone has also proven effective, with evidence showing it reduces lung issues and hospital visits for cystic fibrosis patients. Clinical trials have demonstrated that Orkambi, which combines lumacaftor and ivacaftor, benefits patients with certain genetic mutations by improving lung function and reducing disease severity. All these treatments aim to enhance lung health and overall well-being in cystic fibrosis patients.56789

Are You a Good Fit for This Trial?

This trial is for individuals with Cystic Fibrosis, aged 6 or older, who have specific CFTR mutations that might respond to certain drugs not yet approved for their condition. They must be on a stable CF treatment and able to give consent. People can't join if they have severe liver or kidney disease, had an organ transplant, are currently in another drug study, smoked recently, or take medications that don't mix well with the trial drugs.

Inclusion Criteria

I am 6 years old or older.
My CFTR mutation could improve with specific approved treatments.
My cystic fibrosis lung treatment has not changed recently.
See 2 more

Exclusion Criteria

I cannot use CFTR modulators due to conditions like advanced cirrhosis or ESRD.
I am not taking any medications that interact badly with others.
You have smoked cigarettes within the last 6 months.
See 4 more

Timeline for a Trial Participant

Screening

Participants are screened for eligibility to participate in the trial

2-4 weeks

Treatment

Participants receive off-label CFTR modulators such as Symdeko, Orkambi, or Ivacaftor based on their CFTR mutation response

32 weeks

Follow-up

Participants are monitored for safety and effectiveness after treatment

4 weeks

What Are the Treatments Tested in This Trial?

Interventions

  • Symdeko
Trial Overview The trial is testing Symdeko in patients with Cystic Fibrosis who have certain genetic mutations. It's designed to see if this medication can help even though it's not officially approved for these particular mutations.
How Is the Trial Designed?
3Treatment groups
Experimental Treatment
Group I: SymdekoExperimental Treatment1 Intervention
Group II: OrkambiExperimental Treatment1 Intervention
Group III: IvacaftorExperimental Treatment1 Intervention

Symdeko is already approved in United States, Canada, European Union for the following indications:

🇺🇸
Approved in United States as Symdeko for:
🇨🇦
Approved in Canada as Symdeko for:
🇪🇺
Approved in European Union as Symkevi for:

Find a Clinic Near You

Who Is Running the Clinical Trial?

University of Alabama at Birmingham

Lead Sponsor

Trials
1,677
Recruited
2,458,000+

George Solomon

Lead Sponsor

Trials
1
Recruited
500+

Published Research Related to This Trial

In a study of 101 cystic fibrosis patients treated with elexacaftor-tezacaftor-ivacaftor, participants reported significant improvements in respiratory symptoms, sleep quality, and overall well-being within just a few months of treatment.
Patients experienced a notable reduction in treatment burden and expressed renewed physical strength and self-confidence, leading to better life planning, although some had concerns about body image and treatment dependency.
Patient perspectives following initiation of elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis and advanced lung disease.Martin, C., Burnet, E., Ronayette-Preira, A., et al.[2022]
Elexacaftor/tezacaftor/ivacaftor, a triple-combination therapy, showed potential benefits for a 37-year-old cystic fibrosis patient with chronic lung allograft dysfunction, including improved pancreatic function and quality of life, despite no change in lung function as measured by forced expiratory volume.
The treatment was well-tolerated with no significant side effects, although careful monitoring of renal function and immunosuppressant levels is necessary, indicating that this therapy may be beneficial for select patients awaiting lung retransplant.
Elexacaftor/Tezacaftor/Ivacaftor as a Bridge to Lung Retransplant in a Recipient With Cystic Fibrosis.FitzMaurice, TS., Nazareth, D., Iyer, K., et al.[2022]
In a study of 48 adults with cystic fibrosis, treatment with tezacaftor-ivacaftor (TEZ/IVA) led to significant improvements in lung function (FEV1 increased from 66% to 72%) and reduced annual acute exacerbation rates after 6 months.
TEZ/IVA was found to be safe and well-tolerated, with only 6% of participants experiencing adverse events, indicating its potential as an effective treatment option for adults with cystic fibrosis.
Real-world data on the efficacy and safety of tezacaftor-ivacaftor in adults living with cystic fibrosis homozygous for F508del and heterozygous for F508del and a residual function mutation.Vincken, S., Verbanck, S., Braun, S., et al.[2023]

Citations

Real-World Outcomes of Ivacaftor Treatment in People with ...Improvements in the standard of care have prolonged survival for pwCF; however, the median age at death was 30.8 years in 2018 in the US [13] ...
Real-world impact of ivacaftor in people with cystic fibrosis ...Despite this, the ~42% reduction in rate of PEx and ~30% reduction in hospitalisations demonstrate the efficacy of ivacaftor in terms of ...
KALYDECO® (ivacaftor) Studies & ResultsKALYDECO is a prescription medicine used for the treatment of cystic fibrosis (CF) in people aged 1 month and older who have at least one mutation.
Data From Follow-Up Study of KALYDECO™ (ivacaftor ...Data From Follow-Up Study of KALYDECO™ (ivacaftor) Showed Durable Improvements in Lung Function and Other Measures of Disease in People with Cystic Fibrosis Who ...
LONGITUDE: An observational study of the long-term ...LONGITUDE is an observational, registry-based cohort study using data from the UK CF Registry to evaluate outcomes of ELX/TEZ/IVA in people aged ≥6 years.
Safety Profile & Side Effects*KALYDECO is not approved and is not effective in people with cystic fibrosis (CF) with two copies of the F508del mutation (F508del/F508del) in the CF gene. In ...
Safety Profile | KALYDECO® (ivacaftor)KALYDECO is indicated for the treatment of cystic fibrosis (CF) in patients ≥1 month who have ≥1 mutation in the cystic fibrosis transmembrane conductance ...
8.kalydecohcp.comkalydecohcp.com/isi
Important Safety Information | KALYDECO® (ivacaftor)KALYDECO is indicated for the treatment of cystic fibrosis (CF) in patients ≥1 month who have ≥1 mutation in the cystic fibrosis transmembrane conductance ...
Efficacy and safety of ivacaftor in patients with cystic ...Ivacaftor is used to treat patients with CF and a G551D gating mutation; the KONNECTION study assessed the efficacy and safety of ivacaftor in patients with CF ...
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