Nechama Rivlin looking through a graphic novel based on The Diary of Anne Frank..
(photo credit: Mark Neiman/GPO)
Earlier this month, the country mourned the death of first lady Nechama Rivlin. The people of Israel knew she died of lung disease – she was seen toward the end of her life carrying around a portable oxygen pump. Few likely knew exactly from what the first lady suffered.
Rivlin had a disease called idiopathic pulmonary fibrosis, or IPF. According to Prof. Mordechai Kramer, who treated Rivlin for the past 12 years, it is a very rare disease. Only around 800 people in Israel have it.
The Cleveland Clinic reports that in the United States about 50,000 people have IPF, and an estimated 15,000 new cases develop each year.
Doctors still do not know the reason a patient gets the disease, explained Miki Dror, head of the Israeli Fibrosis Society. The fibrosis scars your lungs, causing them to become stiffer and lose their elasticity. This makes them less able to inflate and take oxygen and therefore harder for the patient to breathe.
Over time, there is a progressive and irreversible decline in lung function, said Kramer. The average number of years from onset until full deterioration is three years, but Rivlin, for example, had the disease for 12 years, and some patients have it for less than one year.
Signs of the disease, he said, are shortness of breath, coughing and difficulty breathing while walking or exercising. He said that IPF cannot be diagnosed without a computed tomography (CT) scan.
“If you have unexplained shortness of breath, then you should ask your physician to do an X-ray and then a CT,” Kramer told the Post
However, Dror said that once one is diagnosed with IPF it can be a battle in Israel to receive proper health support.
There are only two drugs that work with IPF, she explained. They are not cures but they do help to slow down the onset of the disease. But the drug is only made available to people who have lost at least 20% of their lung function. Until then, Israelis are not eligible for the drug and cannot receive it at health basket rates.
Oxygen therapy is something that is recommended for people with IPF, said Dror, but it is not available to the Israeli public. Hyperbaric oxygen therapy increases the amount of oxygen your blood can carry, which temporarily restores normal levels of blood gases and tissue function to promote healing and fight infection.
And finally, as Rivlin did, patients eventually need full-time oxygen support.
“The Health Ministry allows a portable pump only if the person works,” Dror said. “If a person gets the disease and does not work, so he is already in his grave?
“Everything is money based,” Dror continued. “Because of that, these people do not have priority.”
Further, she noted that Rivlin was on the list to receive a lung transplant for two years. According to the National he National Organ Transplantation Center, in 2018 there were 1,138 Israelis waiting for an organ donation.
Kramer noted that in Rivlin’s case, the transplant was successful. However, within two weeks, she experienced heart failure and infection, which ultimately led to her death.
He noted that since the disease is considered an orphan disease – defined as a condition that affects fewer than 200,000 people nationwide, according to the US Food and Drug Administration – few companies are interested in researching new treatments.
“We need to push for further acknowledgment of IPF,” Dror told the Post
. “IPF does not get enough attention and we must wake the decision makers. The decision makers will be very appreciative.”
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