Why We Decided to Go With the Flo: Our Experience With Flolan for PH

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by Colleen Steele |

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My son Cullen was 8 when he was diagnosed with severe pulmonary hypertension (PH). His cardiologist in Washington state placed Cullen on Tracleer (bosentan) and Revatio (sildenafil). He also referred us to a PH specialist at Lucile Packard Children’s Hospital at Stanford (LPCH), in California.

The PH specialist was very concerned about Cullen and wanted to see him, but he also asked if we would agree over the phone to start him on continuous intravenous Flolan (epoprostenol GM).

PH shook our family like an earthquake, but the suggestion of starting Cullen on an invasive, and far more aggressive, third PH treatment felt like a seismic wave. It would require having a central venous catheter (CVC) placed into a large vein leading to his heart, which would emerge from a small opening in Cullen’s chest. I wasn’t emotionally prepared to make that decision quickly, so I asked if we could wait and make it at the specialist’s office during Cullen’s first appointment. The doctor compassionately agreed.

If you’ve followed my columns, you already know that we proceeded with Flolan. For patients and caregivers facing the same decision, I offer you our personal experience with the medication.

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Why triple therapy?

The oral PH treatments didn’t take long to improve Cullen’s breathing and oxygen levels, but echocardiograms continued to show that although he was feeling better, his heart remained in serious condition. Considering the severity of Cullen’s PH and its traumatic effect on his heart, the PH specialist felt that Flolan combined with oral treatments would lead to greater improvement.

Cullen struggled with a six-minute walk test during his first clinic visit at LPCH. It was disappointing, because a month prior, the oral treatments would have helped him complete it without incident. His medical team explained that when one PH medication increases, the other loses some of its effectiveness, causing a slight decline after three or four weeks. Flolan could improve Cullen’s activity level for many years without that decline. It could also delay the need for a heart and double-lung transplant.

The downside to Flolan

Catheter site infections and blood infections are risks associated with having a CVC and continuous infusion therapy. It is imperative to care for the CVC and the catheter insertion site, be hypervigilant about signs of infections, and immediately seek medical assistance if any issues arise.

Continuous IV Flolan runs on a CADD-Legacy ambulatory infusion pump, which is like an insulin pump but a bit larger. Patients and caregivers prepare the cartridges themselves daily by carefully mixing the prescribed dose of Flolan with diluent and then attaching the cartridge to the pump. The Mayo Clinic website describes this complex task in detail.

The process requires a lot of daily supplies, accumulating a lot of waste. In a previous column, I shared tips on organizing PH supplies at home. Cullen carried a backpack 24/7, which held the CADD pump and ice packs to keep the Flolan cold.

Most importantly, suddenly stopping Flolan treatment risks a PH crisis.

The pump and CVC insertion site also cannot get wet. In order to bathe, the pump must remain outside of the tub or shower and the CVC insertion site must be covered; we used a clear, bandage-like product called AquaGuard. For Cullen to swim, we purchased a dry suit.

Why did we agree to Flolan, and was it worth it?

As intimidating as all of this might sound, my husband Brian and I felt confident that we could handle the enormous challenge. We were well trained on everything we’d need, including CVC care, dressing changes at the insertion site, programming the CADD pump, and mixing Flolan. The hospital would not have released Cullen if they weren’t certain we could handle it all. It helped that we both were trained so that we could take turns with the responsibilities.

Triple therapy improved Cullen’s quality of life significantly. He had the option ‌to switch to Remodulin (treprostinil), which is easier to manage, but we stuck with what was working.

Keep the following in mind

Cullen did well on triple therapy for five years, but because of the severity of his PH, he declined and stopped responding to treatments. He received a transplant in 2014. But we know many patients who have done well on PH treatments for decades.

Cullen was placed on Flolan 14 years ago. Since then, other treatment options have become available that you and your doctor might also consider.

If you go with the Flo, just remember that training, routine, and organization are key to how comfortable you will be with this treatment.

Note: Pulmonary Hypertension News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Pulmonary Hypertension News or its parent company, BioNews, and are intended to spark discussion about issues pertaining to pulmonary hypertension.


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