Pulmonary hypertension: real life stories☆☆☆★

“Never assume” 

Nearly 10 years ago, Grand Rapids, Michigan resident Nancy Frede and her family were riding bikes during a vacation—an outing that was a turning point in their lives. Her daughter, who was 4 years old at the time, was having trouble keeping pace with her brothers.

“She had always been physically fine, and this was inconsistent with her behavior as an active child,” Nancy relates. “We were shocked to witness her extreme fatigue and see her fingers turn blue. There had never been any indication that she had anything wrong with her.”

Alarmed, Nancy and her husband took their daughter to a series of physicians. Through the serendipity of a nurse who happened to be present at one physician's office and recognized the disease, the child was immediately diagnosed by specialists in St. Louis. A pediatric cardiologist performed a echocardiogram and detected an atrial septal defect, a condition that often causes PH.

“Imagine our shock,” Nancy says. “This doctor tells us that she has 5 years to live, and she needs a lung transplant. My other children were fine; no one had this illness.”

A nurse and case manager herself, Nancy knew there would be obstacles, but nothing prepared her for the ensuing battle with payors to get the best care. “I simply assumed that, with what I knew, I would be able to navigate the system,” she says, “but I can now say never assume anything with the care needed for pulmonary hypertension.”

She says anyone who is diagnosed or has a family member diagnosed with this disease needs to take a very aggressive, insistent stance with the system. Nancy emphasizes, “First, you need to make sure that everyone is on the same page with this disease. Parents or patients must know there are specialists who provide the individualized specialty care that is essential for pulmonary hypertension.” She says it is not enough to simply be referred to a cardiologist or pulmonary specialist. PH patients must get to that subspecialty, and that means a trip to a center of excellence, a “costly and emotionally upsetting experience for everyone” that drains the family pocketbook of $10,000 to $15,000 per year just for check-ups.

“Your insurance carrier is literally the difference between life and death. I have been a nurse triage person for many years, and I can tell you, never assume anything when attempting to get the subspecialty care and the expensive therapies required for treatment.”

Nancy says coordination between the physician's office and the insurance company is the linchpin to specialist. “The case manager can really be the critical first-line advocate for most appropriate care in this scenario.” The care program then can be coordinated with physicians at the local level for day-to-day routine care.

Nancy says, “Without this care, patients will progress in their disease and may incur thousands more in treatments, improper diagnoses, hospitalizations, and therapies. With proper, timely care, these costs can very often be avoided, and patients get the treatment they need to extend life.”

Nancy's daughter is now 13 and on Flolan, a continuous infusion pumped into a permanent, surgically placed catheter in the chest wall. She is doing very well, but the family's lives revolve around insurance choices and the effects of the disease. Nancy says, “It's difficult, but we try and do everything to give her the most normal life possible. We even go swimming; we use a lot of plastic wrap, tape, creative solutions to live normally. We tell other parents that kids are fragile, they must get the right care, and that parents are the kids' best advocate.”

Her message to case managers? “Be the connection, especially to the Pulmonary Hypertension Association (PHA). Physicians won't tell parents about PHA, and this is one of the single most influential sources for physicians, care centers, and information about this disease.” And when dealing with payors and other administrators, she cautions to “write down everyone's name, document every conversation each step of the way.”

And don't give up, “Because patients need you—you might be their only hope.”

A case of “tired muscles” 

For Melanie Brock, an attorney and 42-year-old woman with 2 children, the diagnosis of her PH 7 years ago was a series of tragic-comic events. “I had been taking prescription diet pills, and I began to have episodes of fatigue, finally to the point where I couldn't get out of a chair.”

Melanie had heard about the New England Journal of Medicine article citing the adverse medical events of the prescription diet aids and then sought treatment. “I had a terrible time trying to see a PH specialist. I went to several doctors, and each reported a more outrageous diagnosis than the one prior. Everything from job stress to outright psychosomatic problems were given as the cause of my condition. When a cardiologist performed a stress test (one definitive diagnostic tool for PH) but found no problem, he said I had tired muscles. However, I later found out that the echocardiogram was improperly reported!”

After researching on her own, Melanie finally found a PH specialist who diagnosed her condition as PH between Class 2 and 3, and she began treatment. Two years of valuable time had elapsed from the onset of symptoms to a proper diagnosis, with considerable upheaval to her family, profession, and personal well-being. She says, “A lot of unnecessary stress, time, and money was spent, and this can be avoided by proper referrals.”

The dynamics of Melanie's family have changed as a result of her diagnosis. She cautions patients, family, and friends about speaking frankly about the disease in front of their children. “One of my children heard something about me possibly dying. This makes a significant impact on a child, and both my children have had to be in therapy to cope with this situation. It is essential that everyone be mindful of how they discuss these extremely sensitive issues.”

Today, Melanie is doing well on Tracleer and Remodulin, very expense medications. She is also active with PHA and has been a respondent to their Q&A website. Her advice to case managers is to “become very familiar with the physician services and support lists” of PHA to get patients the right specialist at the right time. Do not forget that the diagnosis affects the whole family.

A tornado of emotions 

Formerly a practicing attorney in Seattle, Gail Boyer Hayes is well known in the world of PH. She wrote The Patient Survival Guide, now being edited for its third edition and available through the PHA. Gail's story is particularly compelling because she went from doctor to doctor for nearly 12 years before she was properly diagnosed in 1987. “This probably wouldn't happen today, but it is a vivid illustration of what can happen when egos, misinformation, and ignorance override patient care.”

“After the birth of my daughter in 1975,” Gail explains, “I knew I had a problem, and I went to doctor after doctor with the complaint that something was wrong with my lungs.”

Gail recounts an incredible list of diagnoses. She says she was told she hyperventilated on her treadmill and had low salt intake. One physician prescribed epinephrine, which caused her to pass out. When she reported this back to the physician, he dropped her case, saying she was difficult and noncompliant. She was told it was “all in her head” and that it was “the result of marriage problems.” Another suggestion was that she “have another baby to make her less anxious.”

When she was incorrectly diagnosed in 1983 as having a heart problem, she was near death and put on the heart/lung transplant list. Gail instinctively knew that this assumption was wrong and did not want the transplant. She was sent to a psychiatrist who she says described her “as being unable to accept my impending death.”

During the whole episode, she continued to believe the real answer was out there. “I cannot overemphasize the importance of correct diagnosis by the proper professionals, and yet this is the very factor that is blocked by our system to the detriment of untold numbers of patients who are out there and cannot receive the specialty care they must have.”

But it's not just the diagnostic process that is a barrier to PH care. The misunderstanding about the disease and the therapies used nearly equals the problem.

You're going to do what?! 

Pat Paton is legendary in the PH world as one of the founders of the PHA, an advocacy group she says is “so powerful that, when physicians and patients get together at the annual meeting, we are all on equal ground.”

Now a Florida resident—“the sea level does better for us”—Pat was diagnosed with PH 20 years ago and was 1 of the original participants in the Flolan (prostacycline) clinical trials. She describes the life-threatening effect of misinformation about the specific drug therapies for PH patients, suggesting how case managers can intervene.

“One of the problems case managers need to be aware of is that people who are on Flolan often have line infection problems with their catheter. I have been lucky enough to have had a successful therapy on Flolan, yet when I developed a line infection, my HMO was unfamiliar with Flolan.”

Patients on Flolan, Pat explains, survive on the continuously infused drug to lower pulmonary artery pressure and cannot be disconnected from the drug for more than a few minutes. Yet the life and death dependence on this drug is not widely known. She could not get her plan to allow a PH specialist visit to get her line fixed. She remembers, “I was referred instead to a cardiologist. He came into the examination room with a surgical tray, and my husband asked him what he was going to do. His reply was that he was going to remove the line. And then when asked what he was going to do next, he replied that in 2 or 3 months I should come back, and he would install a new one!”

Pat says she would not have made it to the car. She also says that case managers can be especially alert for potential patients who are undiagnosed. “Patients with pulmonary hypertension are difficult to distinguish from those with respiratory or cardiac problems. Case Managers are in the position to probe further when a patient with persistent problems incurs high medical costs but continues with unresolved problems.”

Pat says a telling feature of PH is when “a patient's complaints seem out of proportion to their physical symptoms. or the most part, we look good, and often because of this, it's hard to believe that we present with these severe symptoms.”

Pat says her pulmonary pressure is pretty good. She walks a mile a day and is active, she takes calcium channel blockers, and she has survived.

Never stop learning 

Sally Maddox was a lucky beneficiary of the innovative technologies that have been developed in the past 20 years. Sophisticated echocardiograms and cardiac catheterization now can swiftly measure pulmonary artery pressure.

“In 1990 I was in college and became out of breath playing basketball,” Sally recalls. “I suddenly felt so sick; it was terrible. I was only 20, but I was so nauseous, so fatigued, and I passed out for practically no reason. Luckily, I was referred to a cardiologist and had an electrocardiogram that proved to be abnormal. This prompted the doctors to order a heart catheterization, and the pulmonary pressure was identified.”

Next came a confirmed diagnosis at the Mayo Clinic in Rochester. Then her learning process began. “I spent time learning everything I could, and I discovered that there isn't a lot out there. PHA is probably the greatest single resource for patients, and I made the decision to get involved.”

Since 1996, Sally's health has been stable. She got married, has a full-time job as a teacher, and plans to finish graduate school in the next year. She says that, despite the fact there are still bad days, she knows she is one of the lucky ones. She reports, “I'm now running a support group for patients and their families, and we all wish that case managers could come to these meetings to learn what we have to go through. I think the more they can learn, the better equipped they will become to help us get the care we need.”

Sally says patients have to be their own best advocate for care and that professionals who can act in their behalf are incredibly valuable in improving quality of life. “Half this battle comes from the stress of trying to cope with everything, and stress itself can kill a patient. The more case managers can learn to help us, the more success stories we will hear.”

A child's perspective 

For adults with PH, the scenario is very different than that of a child like Sarah Rossi, who is now 10 years old and described what it was like to “get sick”: “First I had a headache and my stomach hurt; I felt like laying down. Then I saw all these twinkly things in my eyes. We went to a lot of doctors, and then they found what I have.”

Liz Rossi, Sarah's mom, said that diagnosing the PH was an anxiety-ridden trial. The reasons clinicians gave for her normally very active and athletic daughter's fainting and fatigue were “too much syrup on the waffles” and “asthma, maybe diabetes.” She says her other 3 children were completely healthy. But when Sarah repeatedly passed out during soccer, there was instant panic. “You're given things in life and you don't know why, and I made the decision that I would tell other people to consider—you can hide or you can make a difference.”

Liz says, “We were lucky; the doctor who finally diagnosed Sarah said, ‘Ten years ago, it would be you and me and a box of Kleenex, but today there are treatments.’” Sarah is now on calcium channel blockers and doing well.

Liz says that “everyone, absolutely everyone that Sarah comes into contact with” needs to know about PH and what to expect. “The school needs to know, the local emergency medical service needs to be alerted as to emergency care, her friends, their parents—no stone is left unturned in the information chain of Sarah's contacts.”

When asked who her favorite person is, Sarah says, “Nurse Nancy because she took me to get a candy bar when I was in the hospital. I really like her.” But Nurse Nancy, Liz explains, was part of providing the crucial link in helping parents cope with this devastating experience. Taking Sarah out of the consult room allowed a serious discussion to take place without exposing her to that tense environment.

“I have seen parents who are such basketcases; they come to the PH clinics with that ‘deer in the headlights’ glazed expression,” Liz reports, “and their kids look the same. If I could tell any caregiver any message, I would say this: these kids will never get better until the parents get better. That might be one of the most important things any health care provider can offer.”

Heart lung transplantation 

Beyond the pharmaceutical therapies, transplantation is another option, particularly in patients for whom current drug therapies fail. Beatrice Y. Grimes, RN, transplant coordinator and case manager at Xantus Health Plan in Tennessee, recommends other case managers consult the United Network for Organ Sharing. This group provides relevant information on how members are listed on the organ waiting list, the criteria for receiving organs, and survival rates.

Grimes says the wait typically may be several months to 2 years or more. However, of critical importance is a letter of agreement for the evaluation and transplant fees/process between the patient's payor and the transplant center. She emphasizes the need to put these affairs in order because, once someone becomes a candidate for transplantation, she should “get on the list as negotiations may be long and drawn out.”

Case manager impact on outcomes 

At the September 2002 Medical Case Management Conference, Alan Wright, MD, addressed the audience and emphasized the new role of case managers with high-risk, high-cost cases like PH. Wright, senior science officer of TheraCom, said the role of the case manager, “typically responsible for case oversight of patients with excessive use of plan resources,” can become the “liaison to ensure access to the thorough and attentive clinical experts that are necessary in these cases. …Today's case managers must get their payor clients to realize that the right medical care at the right time is essential. This not only reduces medical risk, it vastly improves patient outcomes.”

Ultimately, this approach can save the payor tremendous, unnecessary expense. When timely and appropriate PH care is provided, “It will reduce risk in nearly every case, and then you have improved outcomes and you save lives,” Wright concluded.

At University Hospitals in Cleveland, PH Coordinator Kathleen Hague, RN, BSN, says she relies on case managers to help manage the red tape for patients who need rehab. “We have a PH clinic here. Our patients who experience medical events, such as a stroke, need to be transferred to rehab, but the allowable per diem is usually just $200, and this never covers the costs of these patients and their drug regimen.”

Choices in life and death 

The patient stories reported here are all positive, but many patients who make the brave fight with PH do not get a happy ending. Although diagnosis is vastly improved, patients who are uninsured or have other compromising social or physical factors do not have the option of getting the best care. In other cases, the long-term prognosis is grim, despite all efforts at care. Patients who have progressed to Class IV when diagnosed may live 3 to 5 years, often less.

In any case, PH is a progressing disease, and the families it affects need support, counseling, and advocacy to make end-of-life decisions in a compassionate and caring environment.