**Blue but hopeful**

Pulmonary hypertension is a devastating disease that in its primary form appears suddenly and without warning, mostly among young women. A local patient association is trying to increase awareness and raise funds to help victims.

If one had to dream up one of the most cruel and debilitating diseases to be diagnosed in young people, one would be hard put to beat pulmonary hypertension (PH). The narrowing of blood vessels in the lungs significantly reduces the amount of oxygen reaching the heart, so just climbing two steps is an immense effort. Unknown to the general public, it has been diagnosed in some 15 million people around the world. There are about 600 Israeli patients, 30 new cases a year and perhaps 500 more who have not yet been diagnosed.
It was first identified in 1891 by a German physician named Ernst von Romberg who noticed a condition during an autopsy that he called “pulmonary vascular sclerosis.” It took a whole century for an intravenous drug called epoprostenol to be developed and made available specifically for PH. Since then, five more drugs have been produced and approved as treatment, yet there still is no cure for the disease, which appears in several forms.
The World Health Organization has classified several PH groups.
Some are primary, while some are secondary, resulting from other diseases.
The most puzzling is idiopathic pulmonary arterial hypertension, which is a rare disease with an incidence of about two or three cases per million per year and a prevalence of about 15 per million. For some mysterious reason, the disorder appears usually in ones 30s, and women are three times more likely to get it than men. Children can get it even more rarely; when it occurs, the numbers of cases is more evenly split between girls and boys. About 30 percent of the victims are from the Israel-Arab community, and some Jordanians even cross the bridge to get treatment here.
As it can be triggered by a surge of female hormones, getting pregnant trigger could women who already have the disease. The inability to bear children and to function normally means that some couples divorce. There is no cure except a double lung transplant, but there are very few available organs; in any case, due to possible complications, the average survival rate after surgery is only about five years. However, there are prescription drugs and other treatments that can somewhat improve PH patients’ quality of life.
Another type can be inherited, induced by drugs or toxins, connective tissue disease or even induced by HIV infections, congenital heart disease, schistosomiasis from parasitic worms and sickle cell anemia.
Another group may result from valve problems in the heart, while yet another results from chronic obstructive pulmonary disease and other lung disorders. PH may also appear as a result of metabolic diseases involving the thyroid gland or Gaucher’s disease.
The various types of pulmonary arterial hypertension involve the vasoconstriction (tightening) of blood vessels connected to and within the lungs. This makes it more difficult for the heart to pump blood through the lungs, much as it is harder to make water flow through a narrow pipe as opposed to a wide one. Over time, the affected blood vessels become both stiffer and thicker, in a process known as fibrosis.
This increases the blood pressure within the lungs even more and reduces their blood flow.
In addition, the increased workload of the heart causes the muscle in the right ventricle of the heart to become less able to pump blood through the lungs, ultimately causing “right heart failure.” As the blood flowing through the lungs decreases, the left side of the heart receives less blood. This blood may also carry less oxygen than normal.
Therefore it becomes harder and harder for the left side of the heart to pump to supply sufficient oxygen to the rest of the body, especially during physical activity.
A WORLD Pulmonary Hypertension Day is held annually on May 5.
It was chosen because the date is the anniversary of the first documented death of a child, in Spain, from pulmonary hypertension over 30 years ago. In that case the disease resulted from toxic rapeseed (canola) oil. In this country, there is an Israel Pulmonary Hypertension Association (founded in the year 2000) that marks the day in May as well – this year on May 20. The association organized a “flash mob” (a group of people who assemble suddenly in a public place, perform an unusual act for a brief time and then quickly disperse) at the Weizmann City Mall in Tel Aviv.
Every participant wore blue-colored rubber gloves and blue lipstick, because among the symptoms of PH is cyanosis, in which the tips of the fingers and lips turn blue from an acute lack of oxygen.
Aryeh Copperman, a Bnei Brak resident and part-time teacher whose wife developed idiopathic arterial pulmonary hypertension nine years ago, is director of the association. “She was a talented hairdresser of women’s wigs, but now she can hardly leave the house,” he said. He joined the organization initially to get more information and as a volunteer to help his family and others. Today, he is the paid director of the association and more knowledgeable about the disorder than many general practitioners.
His family is well-known in Orthodox circles, as his uncle, Rabbi Dr. Yehuda Copperman, founded the Michlalah-Jerusalem College for Women decades ago.
The haredi (ultra-Orthodox) man, who was born in Liverpool and was three months old when his parents settled in Israel, gave an interview to The Jerusalem Post about pulmonary hypertension and its devastating problems.
“It’s the second marriage for both of us. Both of us are divorced, and we had a child together,” he said. “Today, she can’t even pick our child up, and it’s hard to move even from room to room,” he said with a pained expression. Suddenly, nine years ago, at the age of 35, his wife, then in her sixth month of pregnancy, did not feel well.
“My father is a cardiologist, and he saw the symptoms as a red light. She was taken to Tel Aviv Sourasky Medical Center for examination and tests, but no cause was identified. She was subsequently sent to a psychologist, because doctors thought it was all in her head. Nearly all pulmonary hypertension patients are referred to a psychiatrist or psychologist because the medical doctors can’t identify the problem. She was no exception,” says Copperman.
“But finally it was diagnosed, and she had to abort the fetus, as pregnancy is very dangerous with the condition.”
The Israeli association was based in Herzliya and not very active at that time. “Now that I am director, it is located in Beit Shemesh and very active. We have 300 members but think there are many more sufferers.
We want to reach all of them and to increase awareness among both the general public and physicians. Early diagnosis is crucial for reducing the severity of the disease.”
Pharmaceutical companies and individuals give the association financial support so it can give members financial assistance for the purchase of medications and medical equipment. The association is recognized under Section 46 of the Israel Income Tax Act, enabling donors to benefit from a 35 percent deduction as a result of their contribution.
Another function of the association is advocacy work leading to the inclusion of medications and new treatments in the Health Ministry’s basket of subsidized drugs and health services.
“We gather relevant information concerning the disease and disseminate it to patients and their families. We also run a website in Hebrew, English, Arabic and Russian at www. phisrael.org.il offering information on a broad array of topics relating to the disease,” said the director. The association also operates a phone line for help 24 hours a day, at 1-800-747574.
Other activities include organizing support groups for patients and their families, convening yearly conferences including lectures by physicians and other specialists, and providing a framework for social interaction and mutual support and sponsoring social events, which help patients forget their sorrows and problems for a while. The association also runs a special support group for couples on coping with problems in their intimate relationships.
THERE ARE about 60 children with various types of pulmonary hypertension, most of them treated as outpatients at Petah Tikva’s Schneider Children’s Medical Center. “The disease harms many organs in the body, but especially the lungs and heart,” said Copperman.
Many patients of all ages need oxygen. There are oxygen tanks and oxygen-producing machines for homes lent by Yad Sarah, but these are not mobile. If one wants a small device that produces additional oxygen on the go, Yad Sarah doesn’t have them. They plug into a vehicle’s cigarette lighter to produce oxygen and cost NIS 20,000. Some people can’t afford even the minimal copayments for medications that are included in the health basket.
Some of the available drugs are administered only intravenously.
All of the drugs have some side effects, said Copperman. Two of the drugs approved for expanding the blood vessels in the lungs are sildenafil (known popularly as Viagra) and tadalafil (Ciali) – both conventionally prescribed for men for erectile dysfunction. For pulmonary hypertension, the generic drugs have other names: Revatio and Adcirca. The pills, also taken by women, increase pulmonary artery vasodilation and lower pulmonary arterial pressure and pulmonary vascular resistance. But they only relieve the symptoms. “These two drugs are the cheapest; there are others that can cost NIS 80,000 a month,” sighed Copperman. His wife receives an injection that costs NIS 40,000 monthly. “I learned how to give her shots at home,” he said.
It is very important to exercise as much as possible to improve fitness, even though this is very difficult for patients. Most victims breathe in oxygen from tanks when they’re asleep. He knows of 15 patients who underwent double lung or heartlung transplants, but some of them did not succeed. “One woman went through two transplants, but they failed, and she died.”
Pulmonary hypertension is a round-the-clock disorder.
“There is never any vacation from it. The National Insurance Institute gives a monthly stipend of only NIS 2,500 for a patient who is 100% disabled.
They can’t work, and it isn’t enough to cover all expenses.” Contacted about this, NII director-general Prof. Shlomo Mor-Yosef said he is familiar with the disease, but since the amount of money the NII gives is determined by law, he has no leeway in paying out more. Some of the four public health funds, said Copperman, are more helpful to victims and families than others.
Fewer than 10 local physicians are specialists in the rare disease. “One woman went to study the disease in the US. We hope she will return and practice in Beersheba’s Soroka University Medical Center, as there is no other specialist in it there now,” said the association director.
Pulmonary hypertension experts at the Henry Ford Hospital in Detroit recently reported that a yoga exercise program tailored for such patients could be very helpful.
Called Yoga for PH, the 40-minute program includes three yoga exercise levels and a nutrition and lifestyle discussion. It is available for free download on iTunes and Googleplay. The program was developed by pulmonologist Dr. Rana Awdish, who also practices yoga.
“These yoga exercises are modified specifically for the needs of pulmonary hypertension patients and can be done safely regardless of the patient’s level of physical fitness,” Awdish stated. “We all recognize the importance of exercise. But for these patients, routine activities like walking up stairs can be challenging, so convincing them they can have success exercising can be challenging.
“What we’ve created are a series of gentle, low-impact exercises that can be performed in the comfort of their own home, sitting in a chair or standing. These are designed to improve balance, strength, reduce stress and calm the nervous system.
Even patients who require oxygen can perform these exercises,” Awdish explained.
As transplants do not seem to be the ultimate answer for patients, researchers from Israel, England, the US and other countries point to stem cells as a possible solution. “It will surely take years, but they have shown in mice studies that stem cells to create healthy new blood vessels [angiogenesis] could be the answer. Clinical studies on patients will take time, but at least there is hope,” Copperman concluded.