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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
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
Resources
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