for Nurses

2. What is the role of the nurse?

1What Is PAH?

What Is Pulmonary Arterial Hypertension?

Pulmonary arterial hypertension (PAH) is a rare subset of pulmonary hypertension (PH).¹

It is a progressive, potentially life-threatening disease affecting the pulmonary arteries of the lungs²
The World Health Organization (WHO) classifies PH into 5 groups. PAH is WHO Group 1³

WHO PH Clinical Classifications³

*CTEPH=chronic thromboembolic pulmonary hypertension

How Does PAH Affect the Right Side of the Heart?

PAH affects the blood vessels of the heart and lungs and is defined by its hemodynamic criteria, shown below.^4,5

In PAH, progressive increase in pulmonary vascular resistance (PVR) of > 3 Wood Units (WU) is accompanied by right heart dysfunction, which ultimately leads to right-sided heart failure.²

Hemodynamic Function: Normal vs PAH

Normal

The resting mPAP is 14 ± 3 mm Hg with an upper limit of normal of approximately 20 mm Hg^3,6

PAH

The PVR and pulmonary pressures are higher than normal due to obstructed or narrowed pulmonary arteries caused by endothelial dysfunction and vascular remodeling²

PAH is defined by^2-4*

Elevated mPAP: ≥25 mm Hg
Normal PAWP: ≤15 mm Hg
Elevated PVR: >3 WU

mPAP=mean arterial pulmonary arterial pressure; PAWP=pulmonary arterial wedge pressure; PVR=pulmonary vascular resistance.

*All values measured at rest.

How Common Is PAH?

PAH is a rare disease and affects 15 to ~52 people per million patients, in countries where studies have been conducted.^3,7,8*

*These studies were: the French Registry data from France based on 674 PAH cases from 2002-2003, estimating a prevalence of 15 cases/1 million per population and the hospitalization data from the Scottish Morbidity Record, which was based on 374 cases from 1986-2001 giving a prevalence of 52 cases per million population.

The average age of a newly diagnosed patient has dramatically changed since the 1980s.

^†Data are from both the Patient Registry for the Characterization of Primary Pulmonary Hypertension and the Registry to Evaluate Early and Long-term PAH disease management (REVEAL Registry). The Patient Registry for the Characterization of Primary Pulmonary Hypertension, initiated by the National Institute of Health in 1981. This multicenter, prospective registry included 32 medical centers in the United States with 187 patients with primary pulmonary hypertension enrolled from July 1981 to September 1985. Limitations include lack of standardized follow-up assessments; prospective studies are needed to validate findings. The Registry to Evaluate Early and Long-term PAH disease management (REVEAL Registry), a large, multicenter, prospective cohort registry that included 54 centers in the United States. 2,967 patients were enrolled between March 2006 and September 2007, all with newly or previously diagnosed World Health Organization group I PAH and pre-specified hemodynamic criteria by right-heart catheterization test. Limitations include lack of standardized follow-up assessments; prospective studies are needed to validate findings.

Approximately 80% of PAH cases are seen in women.^2,10

Nationally, approximately

2.8 years

is the mean time from symptom onset to PAH diagnosis, according to the REVEAL Registry. Most patients are in NYHA FC III at the time of diagnosis.^10,11

What Signs and Symptoms Should You Be Looking For?

Patients with PAH typically present with non-descript symptoms that mimic those of other more common disease states.^3,5

The diagnosis of PAH may be delayed due to these non-descript symptoms⁵
It is important to also note what their other complaints may be^5,12
The first symptom is usually dyspnea, which in the beginning may be exercise-induced but eventually worsens over time⁵

Clinical Presentation and Potential Causes

Syncope
Angina
Dyspnea
Edema

Characteristics of syncope to look for include:

Hemodynamic: Low blood pressure, drop in cardiac output¹³
Arrhythmic: Rare, but needs to be considered³

Angina is common in PAH. It may be indicative of disease progression.³ Characteristics of angina to look for include:

Increased myocardial oxygen (O₂) demand: Elevated right ventricular wall stress (volume, pressure)¹⁴
Inadequate O₂ delivery¹⁴

Characteristics of dyspnea to look for include¹⁴:

Decreased O₂ transport: Hypoxemia; Low cardiac output
Low diffusing capacity of the lung for carbon monoxide (DL_CO)
Low mixed venous O₂ saturation
Increased work of breathing

Characteristics of edema to look for include¹⁴:

Right ventricle (RV) failure
Tricuspid regurgitation
Non-adherence to diuretics³
Chronic deep venous insufficiency

What Are the Different Types of PAH?

Since PAH is a diagnosis of exclusion, many tests are required to assess the probability of this disease.²

Causes of PAH may be idiopathic, heritable or drug-induced⁵
When a patient, regardless of age, seeks treatment for nonspecific complaints such as shortness of breath, lightheadedness, fatigue, edema, and chest pain, these symptoms should not be ignored or attributed to a deconditioned state, obesity, or smoking; PAH should be considered as a part of the differential diagnosis^3,5

What to Look For in a Physical Examination

Cardiovascular Findings on Physical Exam¹³

According to the 2015 European Society of Cardiology/European Respiratory Society (ESC/ERS) Guidelines, echocardiography should always be performed when PH is suspected and may be used to infer a diagnosis of PH in patients with multiple different echocardiographic measurements consistent with this diagnosis.³

Though the physical examination can provide information about the presence and severity of PH, there is an increasing reliance on diagnostic tests to make the diagnosis¹³
The findings of the physical examination may increase the suspicion of PAH, which will need to be confirmed by right heart catheterization (RHC) test¹³

What Is the Right-Heart Catheterization Test?

RHC is the only definitive procedure to confirm PAH and is recommended for all adult patients with suspected PAH.¹

The findings help distinguish PAH from pulmonary venous hypertension (PVH)¹⁴
RHC also reveals the amount of hemodynamic impairment²
In addition, vasoreactivity of the pulmonary circulation can be determined²

PAH Diagnosis^4*

The formal diagnosis of PAH is based on the following results from the RHC test.

1
Elevated mean pulmonary arterial pressure (mPAP): ≥25 mm Hg
2
Normal pulmonary artery wedge pressure (PAWP): ≤15 mm Hg
3
Elevated pulmonary vascular resistance (PVR): >3 Wood Units (WU)

*All values are measured at rest.

How Do You Assess Functional Class?

It is imperative to assess the patient's functional classification (FC) to help establish disease severity. WHO classifies patients according to their FC from I to IV (see table below).¹⁴

WHO FC

Class | Symptoms

I
Patients with PH in whom there is no limitation of usual physical activity; ordinary physical activity does not cause increased dyspnea, fatigue, chest pain, or presyncope
II
Patients with PH who have mild limitation of physical activity. There is no discomfort at rest, but normal physical activity causes increased dyspnea, fatigue, chest pain, or presyncope
III
Patients with PH who have a marked limitation of physical activity. There is no discomfort at rest, but less than ordinary activity causes increased dyspnea, fatigue, chest pain, or presyncope
IV
Patients with PH who are unable to perform any physical activity at rest and who may have signs of right ventricular failure. Dyspnea and/or fatigue may be present at rest, and symptoms are increased by almost any physical activity

As shown, the more severe the impairment due to symptoms, the higher the FC
It is important to monitor FC as it may improve or worsen over time⁴

Monitoring FC is important when developing a plan for the treatment and changes in the patient’s condition.¹²

It helps evaluate the plan and deciding when adjustments may be necessary³

The Importance of the 6-Minute Walk Test

The 6-minute walk test (6MWT) helps to address the severity of disease progression as an overall indicator of functional capacity.¹⁵

It measures the distance a patient can walk on a flat surface in 6 minutes and the shortness of breath and fatigue experienced during the activity¹⁵
Changing the configuration of the walkway significantly affects 6MWT performance¹⁶
The walkway needs to be consistent every time the patient is tested to ensure that the results attained are comparable throughout time¹⁵
Functional class and the 6MWT are two important measures of disease progression and treatment efficacy³
- Other measures can be utilized to assess treatment efficacy and disease status in these patients³

How Is PAH Managed?

As recommended in recent ESC/ERS guidelines, the overall goal of therapy should be achievement of a low risk status.³

Risk assessment in PAH determines the patient prognosis and response to therapy, and can aid decision-making with respect to the need to modify treatment³
The 2015 ESC/ERS guidelines recommend addressing the following key risk assessment questions at each visit³:

Is there any evidence of clinical deterioration since the last assessment?
If so, is clinical deterioration caused by progression of PH or by a concomitant illness?
Is RV function stable and sufficient?
Is the current status compatible with a good long-term prognosis, ie, does the patient meet the low-risk criteria?

Low-risk status is assessed through parameters such as 6-minute walk distance (6MWD), functional class, BMP plasma levels, right heart function, and hemodynamic parameters³

Treatment may include adjunctive and supportive therapies

These medications can help improve symptoms and allow the patient to improve walk distance⁵

Adjunctive therapies include⁵:

Anticoagulation therapy
Calcium channel blockers

Helps decrease blood clotting (also in CTEPH patients)

Supportive therapies include⁵:

Diuretics
Oxygen therapy

May allow for less stress on the right side of the heart

Currently, multiple therapies for PAH are approved by the Food and Drug Administration (FDA) that fall into 3 therapeutic pathways⁴:

1.
Prostacyclin pathway
2.
Endothelin pathway
3.
Nitric oxide pathway

The 2015 ESC/ERS Guidelines recommend following the PAH management algorithm when deciding therapy goals and treatment approach.³

PAH Management Algorithm^17,18

Many treatment options are available; therefore, it is important to consider which treatment pathway(s) should be targeted based on each individual patient³
Therapy goals and treatment approach are dependent on patient factors and guides whether a single therapy or combination approach will be taken³

2 What is the role of the nurse?

Nurses are an essential part of the health care professional team in the journey of a patient with PAH.

Since it is a chronic condition, patients require an interdisciplinary team to continually monitor disease progression, making the role of the nurse vital¹⁹

Roles include¹⁹:

Working directly with the physician and patient in monitoring treatment
- Improve patient adherence
- Reaching treatment goals
Provide follow-up and day-to-day care
Discuss care with the patient’s support system
Assist patients with obtaining medication approval and delivery
Managing fluid volume status¹²

Playing an Active Role in the Patient’s HCP Team

The 2015 ESC/ERS guidelines recommend that the PAH team collaborates with other professionals, including psychologists, psychiatrists, social workers, and patient associations.³

The multidisciplinary team is essential due to the nature of this disease and it will ultimately progress if not treated appropriately²⁰

A multidisciplinary environment can be provided in large-volume expert centers.²⁰

Most patients do not have access to multidisciplinary care centers that have specialized care²⁰
Many patients may live far away from specialized centers making it difficult to achieve optimal care²⁰
Patients who do not have access will require additional support and management from their local family health care providers, cardiologists, or pulmonologists²⁰
Patients who see physicians in multi locations require good communication and patient-held records for optimal patient management²⁰

What Do I Need to Communicate?

Patients may differ in their preferred way of learning; therefore, communicating information needs to match the individual's style.¹²

For patients to adhere to a treatment plan, they must understand basic information about the tasks for which they are responsible; if not, it can compromise patients’ agreement, motivation, and commitment²¹
Some patients may prefer to read the information at home rather than being overwhelmed during visits with health care providers²⁰
Some patients may need time to process the information before asking questions²⁰
Others may want immediate answers at appointments²⁰
Patients may benefit from attending patient support groups where they can receive social interaction and emotional support

This allows for patients with PAH to increase their understanding of the disease, while integrating peer support, promoting social interaction, and addressing patients’ emotional needs²²

How Do I Explain PAH to Patients?

Beginning the discussion of PAH with patients can be emotional and overwhelming for the patient as there are many questions the patient may have when they are diagnosed.

There is a need for more education on PAH²³

Teaching should include the following⁵:

Understanding the pathology of PAH
Early recognition of deteriorating symptoms and when and how to contact the health care provider
The adverse effects associated with medications and how these can be managed^24,25
Preparing and administering medication properly
Caring for infusion site (central line or subcutaneous) for those patients on continuous infusion. This can often be done in conjunction with the specialty pharmacy nurse

The nurse can educate the patient on⁵:

The unique characteristics of the medications, including side effect management
The constant self-monitoring needed to maintain medication effectiveness and the importance of remaining adherent to their medications
The responsibilities that the patient or family will assume with this condition, including frequent follow-up visits and knowing when to contact the office between visits

Awareness of a patient’s health literacy is an integral component in patient care, safety, education, and counseling.²⁶

Each patient may have a different level of ability to understand basic health information for a variety of reasons²⁷:

Emotionally unready to process the diagnosis
Materials not in their native language
Physical restrictions, such as poor vision or cognitive impairment

Patients have varying health literacy levels which can impact their ability to make informed health decisions²⁷
Patient health literacy can be easily overestimated²⁶ and care must be taken to make sure patients understand the information they are given

What Is Life Like for PAH Patients?

The diagnosis of PAH can be devastating to a patient, as many aspects of their daily life may be affected.

As the nurse, it is essential to communicate the impact of the physical restrictions that PAH can impose on patients²⁰

Patients with PAH often²⁰:

Have a decreased ability to carry out their daily activities, which may leave them feeling frustrated
Have financial burdens resulting from their reduced ability to work, which also may lead to their frustration and low self-esteem
Suffer from depression²⁸
Have impaired cognitive function, affecting verbal learning, memory, decision making, and motor function²⁹
May be limited in the ability to engage and self-manage their disease

In female patients with PAH that may become pregnant, a discussion regarding pregnancy and their disease is necessary.

Pregnancy in female PAH patients is associated with a substantial mortality risk³. Effective contraception is imperative for all women with PH of child-bearing potential³⁰
Patients who become pregnant should be informed of the high risk of pregnancy³
The potential need to terminate the pregnancy should be discussed³
Those who choose to continue their pregnancy should be cared for in a PH center of excellence in conjunction with a high-risk obstetrician³
Planned elective delivery and effective close collaboration between the PAH team and obstetricians is essential^3,30

The Discussion of Palliative Care

Since PAH is a progressive disease without a cure, end-of-life and ethical issues may need to be addressed.

Communication with patients with PAH is essential to allow for advanced planning and discussion of their fears, concerns and wishes³
Advanced directives may be recommended³¹
Prognosis should be discussed at the time of initial diagnosis, along with resuscitation status³

Cardiopulmonary resuscitation tends to have poor outcomes in patients with PAH
May increase chance of patients being in their preferred place of care at the end of life

Referral to specialist palliative care services should be used when planning advanced care³
Communication should be open and sensitive when discussing end-of-life options to patients³

3What Is optimal treatment for my patient?

How Does the Patient Receive Optimal Treatment?

Patients who are involved in the decision making regarding their disease are more engaged and motivated.²⁰

Patients who understand the benefits of being actively involved in decisions about their treatment and care are able to make informed decisions and to take ownership of their disease, which leads to improved outcomes³²

What Is Patient Activation?

Patient activation or patient engagement, is just one aspect of an individual’s ability to engage in their own care³³

It is an individual’s knowledge, skill, and confidence in managing his/her own health and health care³⁴
Patient activation can be thought of as having four stages^20,34

Stages of Patient Activation

1
Patient must believe that their role is important in managing their own health
2
Having the confidence and knowledge necessary to take action
3
Patient takes action to maintain and improve their health
4
Patient continues with self-management, even under stress

Patients may go back a stage or be in different stages for different aspects of their disease²⁰
Patients often have different attitudes when dealing with their health,³⁵ having an impact on the engagement process
Patients with chronic conditions who attended a self-management program reported significant improvements in patient activation³⁶
The nurse needs to stress importance of patient activation and engagement in the disease management of PAH patients²⁰

Further challenges arise when some patients may not wish to be engaged in the management of their disease.²⁰

For some patients, choosing not to engage may be a coping mechanism²⁰
The benefits of being an engaged patient should be communicated to these patients²⁰
Nurses need to keep emphasizing to their patients the need to remain engaged in the management of their disease²⁰

Importance of Patient Adherence

It is important to educate the patient both on the medications they have been placed on and the importance of filling prescriptions and adhering to the scheduled doses.

Medication adherence rates are approximately 50% in this population. Reasons for this may include^19,24:

Skipping doses
Not filling prescriptions
Taking medications at wrong times of the day
Interrupting therapy
High copayment³⁷
Medication side effects²⁴

Patient’s Self-Care: Physical Care, Mental Health

Patients should be counseled on their own physical care to prevent potential harmful situations.

Such teachings can include⁵:

Avoiding over-the-counter decongestants
Smoking cessation
Avoiding secondhand smoke
Avoiding overexertion
Avoiding food high in potassium if on potassium-sparing diuretic or in patients with chronic kidney disease
Updating vaccinations
Maintaining ideal body weight

There is often a significant impact on the psychological functioning of patients³
The nurse should have close links to colleagues who work with those problems such as psychiatry, clinical psychology, welfare and social work³

Patient’s Support System: Involving the Caregiver

Caregivers play a key role in supporting patients with PAH in the management of their disease, along with every day physical and emotional needs.^23,38

Caregivers can be:

A health care professional
Family and friends
Support group

Caregivers benefit from receiving appropriate information on PAH.²³

They need to understand the challenges of the disease, how it will impact their daily life and how they may be able to help the patients²³
To encourage caregivers to be engaged, they also need to be provided with timely information and support^23,38
Most caregivers reportedly receive either limited or no written information about PAH from health care providers at diagnosis, which may lead to many unanswered questions³⁸
Caregivers are often more proactive than patients at obtaining information from a range of sources³⁸
By spending time with patients on a daily basis, caregivers are in a strong position to help and encourage patients to engage in self-management of their disease²⁰
By attending clinic appointments with the patient, family members can learn about PAH and contribute to the patient’s individualized treatment plan¹²

Role of Support Groups

Patients with PAH have a progressive condition which can cause them to have anxiety and/or depression.⁵

Support groups can offer short- or long-term support to help patients manage anxiety or depression.⁵

Psychosocial patient support should include⁵:

Allowing patients to verbalize their fears
Assisting with acceptance of realistic perceptions and expectations
Incorporating family, friends, caregivers in their disease management
Encouraging patients to participate in support groups, which can be in person or online such as:

4 Additional Resources

Patients increasingly seek information regarding PAH via the internet²⁷ and it is vital that this information is correct and that health care providers, especially nurses, are aware of sources of high-quality information.

Additional links are provided below to assist with further learning about PAH in regards to the disease, management and patient care education

Caring for Someone with PAH

5references

Hoeper MM, Bogaard HJ, Condliffe R, et al. Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol. 2013;62(25 Suppl):D42-50.
Lai YC, Potoka KC, Champion HC, et al. Pulmonary arterial hypertension the clinical syndrome. Circ Res. 2014;115(1):115-130.
Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J. 2016;37(1):67–119.
McLaughlin VV, Archer SL, Badesch DB, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association developed in collaboration with the American College of Chest Physicians; American Thoracic Society, Inc.; and the Pulmonary Hypertension Association. J Am Coll Cardiol. 2009;53(17):1573-1619.
Ross CA. Pulmonary Arterial Hypertension: Early recognition and treatment can make a lifetime difference for your patient. J Nurse Pract. 2007;3(6):404–409.
Kovacs G, Berghold A, Scheidl S, et al. Pulmonary arterial pressure during rest and exercise in healthy subjects: a systemic review. Eur Respir J. 2009;34(4):888-894.
Peacock AJ, Murphy NF, McMurray JJ, et al. An epidemiological study of pulmonary arterial hypertension. Eur Respir J. 2007;30(1):104-109.
Humbert M, Sitbon O, Chaouat A, et al. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med. 2006;173(9):1023-1030.
Rich S, Dantzker DR, Ayres SM, et al. Primary pulmonary hypertension. A national prospective study. Ann Intern Med. 1987;107(2):216–223.
Badesch DB, Raskob GE, Elliot CG. Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry. Chest. 2010;137(2):376-387.
Palevsky HI. The early diagnosis of pulmonary arterial hypertension: can we do better? Chest. 2011;140(1):4-6.
Archer-Chicko C. Nursing care of patients with pulmonary arterial hypertension. In: Yuan JXJ, Garcia JGN, Hales CA, et al, eds. Textbook of Pulmonary Vascular Disease. 1^st ed. New York, NY: Springer Science+Business Media;2011:1531-1558.
Rich JD, Rich S. Clinical diagnosis of pulmonary hypertension. Circulation. 2014;130(20):1820-1830.
McLaughlin VV, McGoon MD. Pulmonary arterial hypertension. Circulation. 2006;114(13):1417-1431.
Crapo RO, Casaburi R, Coates AL, et al. ATS statement guidelines for the six-minute walk test. Am J Respir Crit Care Med. 2002;166(1):111-117.
Barnett CT, Jacmann JS, Moore NC, et al. The effect of walking path configuration on six-minute walk test performance and gait variability: a pilot study. Gait & Posture. 2015;42(3):S54.
Maron BA, Galiè N. Diagnosis, treatment, and clinical management of pulmonary arterial hypertension in the contemporary era: a review. JAMA Cardiol. 2016;1(9):1056-1065.
Galiè N, Corris PA, Frost A, et al. Updated treatment algorithm of pulmonary arterial hypertension. J Am Coll Cardiol. 2013;62(suppl 25):D60-D72.
Tartavoulle TM. Evaluation and management of the adult patient with pulmonary hypertension. J Nurse Pract. 2011;3(5):409-416.
Graarup J, Ferrari P, Howard LS. Patient engagement and self-management in pulmonary arterial hypertension. Eur Respir Rev. 2016;25(142):399-407.
Gerwing J, Indseth T, Gulbrandsen P. A microanalysis of the clarity of information in physicians’ and patients’ discussions of treatment plans with and without language barriers. Patient Educ Couns. 2016;99(4):522-529.
Rahaghi FF, Chastain VL, Benavides R, et al. Shared medical appointments in pulmonary hypertension. Pulm Circ. 2014;4(1):53-60.
Guillevin L, Armstrong I, Aldrighetti R, et al. Understanding the impact of pulmonary arterial hypertension on patients’ and carers’ lives. Eur Respir Rev. 2013;22(130):535-542.
Stewart T. Facilitating pulmonary arterial hypertension medication adherence: patient-centered management. Adv Pulm Hypertens. 2010;8(4):228-231.
DeSmedt RH, Denig P, Haaijer-Ruskamp FM, et al. Perceived medication adverse effects and coping strategies reported by chronic heart failure patients. Int J Clin Pract. 2009;63(2):233-242.
Dickens C, Lambert BL, Cromwell T, et al. Nurse overestimation of patients’ health literacy. J Health Commun. 2013;18(suppl 1):62-69.
Parker RM, Ratzan SC, Lurie N. Health literacy: a policy challenge for advancing high-quality health care. Health Aff (Millwood). 2003;22(4):147-153.
Poms AD, Turner M, Farber HW, et al. Comorbid conditions and outcomes in patients with pulmonary arterial hypertension: a REVEAL registry analysis. Chest. 2013;144(1):169-176.
White J, Hopkins RO, Glissmeyer EW, et al. Cognitive, emotional, and quality of life outcomes in patients with pulmonary arterial hypertension. Respir Res. 2006;7:55.
Bonnin M, Mercier FJ, Sitbon O, et al. Severe pulmonary hypertension during pregnancy: a mode of delivery and anesthetic management of 15 consecutive cases. Anesthesiology. 2005;102(6):1133-1137.
Fenstad ER, Wordingham SE, Swetz KM. Pulmonary hypertension and palliative care: what, when, where, and why? Advances in Pulmonary Hypertension. 2016;15(1):26-31.
Ellins J, Coulter A. The Health Foundation, Picker Institute Europe, Oxford, United Kingdom. How engaged are people in their health care? Findings of a national telephone survey. November 2005.
https://www.picker.org/wp-content/uploads/2014/10/How-engaged-are-people-in-their-health-care-....pdf. Accessed November 27, 2018.
Carman KL, Dardess P, Maurer M, et al. Patient and family engagement: a framework for understanding the elements and developing interventions and policies. Health Aff (Millwood). 2013;32(2):223-231.
Hibbard JH, Stockard J, Mahoney ER, et al. Development of the patient activation measure (PAM): conceptualizing and measuring activation in patients and consumers. Health Serv Res. 2004;39(4 Pt 1):1005-1026.
Eriksson M, Lindström B. Antonovsky’s sense of coherence scale and the relation with health: a systemic review. J Epidemiol Commun Health. 2006;60(5):376-381.
Turner A, Anderson JK, Wallace LM, et al. An evaluation of a self-management program for patients with long-term conditions. Patient Educ Couns. 2015;98(2):213-219.
Waxman A, Chen SY, Boulanger L, et al. Factors associated with adherence to phosphodiesterase type 5 inhibitors for the treatment of pulmonary arterial hypertension. J Med Econ. 2013;16(2):298-306.
PHA Europe. The impact of pulmonary arterial hypertension (PAH) on the lives of patients and carers: results from an international survey. http://www.phaeurope.org/wp-content/uploads/International-PAH-patient-and-Carer-Survey-Report-FINAL1.pdf. Updated 2012. Accessed November 26, 2018.

This MPR/PulmonologyAdvisor Fact Pack is produced as a basic reminder of important information for healthcare professionals. Readers are advised to consult manufacturers and specialists if questions arise about specific products, treatments, or diseases. The publisher and editors do not assume liability for any errors or omissions. MPR, PulmonologyAdvisor, and Fact Pack are registered trademarks of Haymarket Media, Inc.