Sharon Jones RN, MSN, CCMPulmonary Hypertension Nurse Navigator

Christiana Care Hospital

Disclosures

Consultant for Actelion Pharmaceuticals

Distinguish Pulmonary Hypertension from similar disease states.

Define the responsibility of the case manager relating to the PH patient hospital stay and transitions of care.

Describe case management collaborative strategies that can be used to promote best practice pertaining to the PH patient.

Increased blood pressure in the pulmonary vasculature

Not the same as systemic hypertension

Not the same as CHF Many types and causes Pulmonary Arterial Hypertension

has specific diagnosis criteria and treatment regimens

World Health Organization Classification:

Group 1 – Pulm Arterial HypertensionGroup 2 – PH due to left heart diseaseGroup 3 – PH due to chronic lung disease and/or hypoxemiaGroup 4 – Chronic thromboembolic pulmonary hypertension (CTEPH)Group 5 – PH due to unclear multifactorial mechanisms, i.e., sickle cell disease, sarcoidosis, chronic renal failure

Statistics of PAH

“Rare Disease”

Affects all ages, races & ethnic backgrounds, more common in women, non Hispanic blacks & people age 75 or older

2-10 cases per million in the US

16-74 cases per million globally

Mean age at diagnosis is 50

Global prevalence hard to estimate due to lack of diagnostic capabilities & access to care

Predominate Etiologies of PAH

30-56% Idiopathic

15-30% Connective Tissue Disease

10-43% Congenital Heart Disease

Other PAH Etiologies

Drug & Toxin Induced-Methamphetamines, “Fen-Phen”

HIV Portal Hypertension Schistosomiasis

Symptoms

Dyspnea Fatigue Chest Pain Near Syncope Syncope Edema Right-sided second heart sound

(S2) Sternal heave Jugular vein distention

Diagnosis of PAH

Best diagnosed by a PH Specialist

Best treated in a PH Center

Average time to diagnosis 2.8 years

Must be diagnosed by right-heart catheterization

Diagnostic Tests for PAH

Echocardiogram Right Heart Catheterization * Electrocardiogram Chest X-Ray CT of the Chest Ventilation Perfusion Lung Scan Pulmonary Function Tests Sleep Study Blood Work

* Gold Standard of Diagnosing PAH

PAH Treatments

No cure, only supportive care:

OxygenC-Pap/Bi-PapOral Drug TherapyInhaled Drug TherapyContinuous SQ Drug InfusionContinuous IV Drug Infusion

Other Treatments

Edema management-i.e., diuretics

Anti-coagulants CTEPH patients who get a

pulmonary thromboendarterectomy may cure PAH (only curable form)

PH & The Case Manager

Know Hospital Policy

Identify Hospital PH Experts

Know PH Patient Designated Units

Get Basic PH Education

PH & The Case Manager

Oral Drug Therapy

Revatio (Sildenafil) Adcirca (Tadalafil) Letairis (Ambisentan) Opsumit (Macitentan) Tracleer (Bosentan) Adempas (Riociguat) Orenitram (Treprostinil)

Inhaled Therapy

Ventavis (Iloprost) Tyvaso (Treprostinil)

Special Inhalation Devices Patient taught by specialty pharmacy

nurses to administer drug & care for inhalation device at home

Hospital nurses must be specially trained to care for patient in case the patient is unable to administer the meds via the device

Subcutaneous Drug Therapy: Remodulin (Treprostinil)

4 hour half-life Patient manages drug mixing,

CADD pump & cassette changes Drug should NEVER be stopped

unless under supervision of PH specialist

Hospital nurses must be specially trained to care for patient

IV Drug Therapy Remodulin IV-central line

Veletri (Epoprostenol)

3-6 MINUTE Half-Life Potential for rebound PH/death if

stopped Central line Patient self-management at home Hospital nurses must be specially

trained

Drug Therapy Considerations Most if not all need a prior

authorization from the patient’s insurance company

Letairis, Tracleer, Opsumit, Ventavis & Adempas have drug company oversight (patient/prescriber)

Revatio & Adcirca are the only PAH drugs available from a retail pharmacy per patient’s drug plan

Specialty pharmacy (mail order) per patient’s drug plan

Patient Assessment: PH doctor, PH drugs prescribed, specialty pharmacy used

Know hospital formulary & specialty pharmacist Patient can only use 3 days of their own

medication, then hospital/facility must provide Share information with the treatment team Notify PH Specialist of patient admission Obtain patient records Listen To The Patient!!!

Drugs started in the hospital cannot be stopped

Specialty pharmacy nurses teach pt/family inhaled, IV/SQ meds starting in the hospital

Drug enrollment forms (per hospital policy designee)

Prior authorizations (per hospital policy designee)

Must have knowledge of ICD-9 codes & testing to complete prior authorizations for PAH drugs

CM ResponsibilitiesCM Responsibilities

Transition of Care

Drug therapy determines disposition Cost of drugs: $14,000->200,000/year Cost prohibitive for facilities & Hospice Patient/family must be totally

independent with drug administration prior to discharge on IV/SQ therapy

Inhaled therapy teaching can be started a few days before discharge

Disposition: Home Any route of drug If new start, must have home

supply delivered prior to discharge or samples given

IV/SQ patients given CADD pump prior to discharge by specialty pharmacy & pt is changed to home pump

Home Care

Home care can follow up on fluid management and dietary concerns-need to coordinate visits with SP if they are involved

Specialty pharmacy nurse continues patient teaching of inhaled, IV/SQ meds

Specialty pharmacy does blood pressures on new Adempas patients for drug titration

Facilities

Short term vs Long term care Inform facility of PAH drug therapy Some facilities allow short term

stays if on oral therapy Facility must supply drug in most

cases Inhaled, IV/SQ drugs cost-

prohibitive & staff are not trained

Hospice Involve PH specialist in hospice

decision Treatment team & family should

decide whether to stop PH drugs due to ramifications

No coverage for PAH diagnosis Must have hospice approved

diagnosis Arrange for supply of PAH drugs if

needed

CM Collaborative Strategies Case manager is patient’s liaison with

hospital treatment team, PH specialist, & specialty pharmacy

Communicate often with pt’s PH team Keep patient/family informed of

transition plan Obtain previous records & have

mechanism to send hospital records to PH specialist

Best Patient Outcomes

Communication & Teamwork!!!

Resources

Pulmonary Hypertension Association: www.phassociation.org/

PH Central: www.phcentral.org

American Heart Association: www.americanheart.org

Sharon Jones, RN, MSN, CCM:shjones@christianacare.org

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