Cystic Fibrosis

A few weeks ago, I spent the morning with a reporter with The Wall Street Journal for a story on a young teen from Walnut Ridge who is suffering from Cystic Fibrosis. CF is a life-threatening genetic disease that primarily affects the lungs and digestive system. An estimated 30,000 children and adults in the United States (70,000 worldwide) have CF. In people with CF, a defective gene and its protein product cause the body to produce unusually thick, sticky mucus that clogs the lungs and leads to life-threatening lung infections and it obstructs the pancreas and stops natural enzyme from helping the body break down food and absorb vital nutrients.

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Chloe Jones, 14, is one of those people.

The story I worked on for the WSJ centers on Vertex Pharmaceuticals Inc. They have a 300,000-a-year cystic-fibrosis drug has ignited a legal battle where Arkansas' Medicaid program is restricting access to the expensive therapy. In a lawsuit filed in Arkansas federal court last month, three people suffering from the disease allege Medicaid officials have for two years denied them access to Kalydeco because of its cost. The plaintiffs allege state officials have violated their civil rights under federal law governing Medicaid, the government-run insurance plan for the poor.

Here is an excerpt from the story:

Karen E. Segrave for The Wall Street Journal CYSTIC Chloe Jones, 13, from Walnut Ridge, Ark., waits while wearing a vest used to clear her chest congestion in a hotel room in downtown Little Rock, Ark., on Monday, June 30, 2014 Jones and her family are trying to get access to the drug Kalydeco which is used in the treatment of Cystic Fibrosis, but the state of Arkansas is denying access to the drug which costs more than $300,000 per year.

Chloe Jones, a 14-year-old plaintiff in the Arkansas suit, spends several hours each day undergoing treatment and taking medications. One step requires her to spend 45 minutes, three times each day in an inflatable vest that puts pressure on the chest and loosens mucus in the lungs.

William Golden, medical director of Arkansas Medicaid, said that since Chloe's lung function was normal at the time her doctors prescribed Kalydeco, the state couldn't justify approving Kalydeco.

The state Medicaid agency denied her doctor's request for Kalydeco in June 2012, stating Chloe hadn't met the requirement of taking older medications for 12 months, according to a letter Medicaid sent her doctor. The state denied Chloe subsequent appeals in July and September of 2012, and in August 2013, according to attorneys at Sufian & Passamano LLP, a Houston law firm representing Chloe.

"They just don't want to pay for it," Chloe said during an interview in Little Rock, where she travels for treatment from her home in Walnut Ridge, Ark. "I feel like they don't care about what's wrong with me, that I'm not as important as everybody else."

Chloe's physician, Dennis Schellhase, says Chloe has now been taking all of the medications required by the state for close to a year and the hospital will soon submit another application for the drug with the state Medicaid program.

You can read more information on the Chloe from The Wall Street Journal by going to their on-line article.

Karen E. Segrave for The Wall Street Journal CYSTIC Aime (cq) Ledman shows just some of the medication her daughter Chloe Jones, 13, must take on a daily basis for her treatment of Cystic Fibrosis in a hotel room in downtown Little Rock, Ark., on Monday, June 30, 2014. Chloe Jones and her family are trying to get access to the drug Kalydeco which is used in the treatment of Cystic Fibrosis, but the state of Arkansas is denying access to the drug which costs more than $300,000 per year.

Karen E. Segrave for The Wall Street Journal CYSTIC Chloe Jones, 13, from Walnut Ridge, Ark., mixes up a batch of medication used during her treatment of Cystic Fibrosis in a hotel room in downtown Little Rock, Ark., on Monday, June 30, 2014. Jones has Cystic Fibrosis. Jones and her family are trying to get access to the drug Kalydeco which is used in the treatment of Cystic Fibrosis, but the state of Arkansas is denying access to the drug which costs more than $300,000 per year.

Karen E. Segrave for The Wall Street Journal CYSTIC Chloe Jones, 13, from Walnut Ridge, Ark., uses an  inhaler during her treatment of Cystic Fibrosis in a hotel room in downtown Little Rock, Ark., on Monday, June 30, 2014. Jones has Cystic Fibrosis. Jones and her family are trying to get access to the drug Kalydeco which is used in the treatment of Cystic Fibrosis, but the state of Arkansas is denying access to the drug which costs more than $300,000 per year.

Karen E. Segrave for The Wall Street Journal CYSTIC Chloe Jones, 13, (left) from Walnut Ridge, Ark., waits in the lobby at Arkansas Children's Hospital on Monday, June 30, 2014. Jones has Cystic Fibrosis. Jones and her family are trying to get access to the drug Kalydeco which is used in the treatment of Cystic Fibrosis, but the state of Arkansas is denying access to the drug which costs more than $300,000 per year.