multiple sclerosis and pulmonary arterial hypertension

 Overview

Multiple sclerosis (MS) is an autoimmune disorder affecting the central nervous system. The immune system attacks the nerves of the brain and spinal cord. Body movements may become slow or uncoordinated because signals from the brain to the muscles deteriorate, or arms and legs may feel numb because sensations from the extremities no longer reach the brain. The condition becomes progressively worse over time if not effectively treated. Although there is no cure, medications can help reduce inflammation and lengthen periods between attacks.

There are about 400,000 people in the U.S. living with MS. The condition is typically diagnosed anywhere from age 20 to age 50.*

Certain populations have an increased risk of developing MS. Whites are more than twice as likely as other races to develop MS, and women are affected almost twice as often as men.

Geography and climate may also impact an individual’s MS risk factor. MS is five times more prevalent in the northern United States, Canada, and Europe than in tropical regions.

Most people experience their first symptoms of MS between the ages of 20 and 40. While symptoms rarely begin before age 15 or after age 60, scientists have documented the initial symptoms of MS at almost every age. When symptoms occur, they can include:

• Dizziness, blurred or double vision
• Muscle weakness in the arms and legs
• Difficulty with coordination and balance
• Muscle stiffness and spasms
• Numbness, prickling or tingling sensations
• Difficulty with concentration, attention span and memory
• Fatigue
• Speech impediments
• Tremors

Unfortunately, there is no clear combination of symptoms or test results that can definitively say a person has MS. Health care providers use an array of testing strategies to determine if a person meets the criteria for a diagnosis of MS, including ruling out other possible causes of symptoms. Doctors will typically take a detailed past medical history, including detailed descriptions of symptoms and when they started. Then a physical and neurologic exam will be performed including a panel of imaging tests, such as magnetic resonance imaging (MRI). Typically, no one test is enough to confirm the presence of MS; a combination of positive findings is required.

Physicians often use the Revised McDonald Criteria in order to make a more efficient diagnosis of MS.

Other neurologic tests that may be completed are MRI, Visual Evoked Potential (VEP), and Cerebrospinal Fluid Analysis.

The following specialty medications are available at Albrighton Pharmacy, a specialty pharmacy for multiple sclerosis.                        

Ampyra® (dalfampridine)            

Aubagio® (teriflunomide)            

Avonex® (interferon beta-1a)             

Betaseron® (interferon beta-1b)            

Copaxone® (glatiramer acetate)            

Extavia® (interferon beta-1b)            

Gilenya® (fingolimod)        

Glatopa® (glatiramer acetate)                

Lemtrada® (alemtuzumab)            

Novantrone (mitoxantrone)             

Ocrevus™ (ocrelizumab)            

Plegridy® (peginterferon beta-1a)      

Rebif® (interferon beta-1a)             

Tecfidera® (dimethyl fumarate)             

Tysabri® (natalizumab) 

Zinbryta® (daclizumab)     

Financial resources are available to support your drug costs, including manufacturer and community programs. Albrighton Pharmacy specialists are available to help you find the right program for you.

• National Organization for Rare Disease | 1-877-247-4363
• Patient Access Network Foundation | 1-866-316-7263
• The Assistance Fund
• Good Days® | 1-877-968-7233

There are many organizations that support research and advocacy for MS. See below for a few of those organizations.

• National MS Society
• Multiple Sclerosis Association of America
• Consortium of Multiple Sclerosis Centers
• Multiple Sclerosis National Research Institute
• Multiple Sclerosis Foundation

High blood pressure in the lungs is called pulmonary hypertension (PH) or pulmonary arterial hypertension (PAH). PAH is a chronic and life-changing disease that can lead to right heart failure if left untreated.1

Although PAH is a rare disease, with an estimated prevalence of 15-50 cases per million, the prevalence of PAH in certain at-risk groups is substantially higher.2

1. https://phassociation.org/patients/aboutph accessed 7/12/2017

2. www.pah-info.com/How_common_is_PAH accesed 7/12/2017

The symptoms for all types of pulmonary hypertension (PAH) may be similar, and symptoms are usually more severe as the disease progresses. Symptoms of PAH may include:

• Breathlessness
• Chest pain (also called angina pectoris)
• Dizziness
• Fainting (also called syncope)
• Loss of energy (also called fatigue)
• Swelling of the arms, legs, ankles or abdomen (also called edema)
• Dry cough
• Raynaud’s phenomenon (chalky white or dusky blue fingers that may be painful and can sometimes be provoked by the cold)

In advanced stages of PAH, minimal activity may produce some or all of these symptoms. Patients in advanced stages may experience irregular heartbeat, a racing pulse, passing out and difficulty breathing at rest. Sometimes these symptoms mean you have another condition, but sometimes, these symptoms mean you have PAH.1

PAH can be difficult to diagnose in a routine medical exam because the most common symptoms of PAH, such as breathlessness, fatigue and dizziness, are also associated with many other conditions. If your doctor suspects that you have PAH, he or she will want to review your medical and family history, perform a physical exam and perform one or more diagnostic tests.

To determine if you have PAH, and what type, your medical team will schedule specialized tests. If your medical team suspects PAH as a result of one or more of the following tests, they will go on to schedule a right-heart catheterization, which is required to confirm diagnosis.

Blood tests check the oxygen levels in the blood, they observe liver and kidney function, and they identify whether the patient has collagen vascular disease, thyroid problems, signs of infection or HIV antibodies. One test, the brain natriuretic peptide, helps to assess the strain on the heart and may also be used to monitor response to treatment.

Chest X-rays can reveal an enlarged right ventricle or pulmonary arteries. Chest X-rays can also show signs of emphysema or scarring (interstitial fibrosis) of the lungs.

An electrocardiogram checks the electrical impulses of the heart. Electrodes are attached to the patient’s skin, and a recording of these impulses is made. However, an ECG alone is not enough to indicate a PAH diagnosis. If your doctor performs an ECG, he or she will also perform one or more additional procedures to identify PAH.

In this procedure, electrodes are placed on the patient’s skin, and a sonogram of the heart is taken. This painless procedure is often used to make a preliminary diagnosis by estimating the pressures in the right heart and assessing how well the heart is functioning. Other heart conditions that produce symptoms similar to PAH may be diagnosed with an echocardiogram. In addition, an echocardiogram may be used to monitor a patient’s condition.

These tests measure how much air your lungs can hold, how much air moves in and out of them and the lungs’ ability to exchange oxygen. These tests may be performed to potentially identify its cause.

During this test, a patient will be asked to perform an exercise, most commonly a six-minute walk. The purpose is to identify the patient’s exercise tolerance level.

This diagnostic tool tests for blood clots in the lungs by producing a picture of air and blood flow to the lungs. A small dose of radioactive material is breathed in and another small dose is injected via a blood vessel into the lungs. The doctor will review the images that are produced to evaluate the health of the lungs.

If the results of initial tests point to PAH, your doctor will schedule a right-heart catheterization (commonly referred to as a “right heart cath”). Right-heart catheterization is one of the most accurate and useful tests to get a definitive diagnosis for PAH. This is the only test that directly measures the pressure inside the pulmonary arteries, and it should be done in all patients at least once to confirm a patient’s diagnosis of PAH. During the test, doctors insert a catheter (a thin rubber tube) through a large vein in the patient’s groin or neck. They then pass the catheter up into the patient’s heart to measure the blood pressure in the right side of the heart and lungs.

This test is used for patients who have already been diagnosed with PAH to determine how much their pulmonary blood vessels can relax over a brief period of time. Its main purpose is to screen for patients who might respond favorably to calcium channel blockers, a form of medication. The test can also help determine the patient’s prognosis. With a right heart catheter in place, the patient is given drugs that relax the pulmonary arteries. The test drug is given to the patient in higher and higher doses, pausing at each dose to see how the patient reacts. Once a significant response occurs or the side effects become intolerable, the test is considered complete. 2

1. https://phassociation.org/patients/aboutph/symptoms-of-ph/ accessed 7/12/2017

2. Nhttps://phassociation.org/patients/diagnosis/ accessed 7/12/2017

While there is currently no cure for PAH, different treatment options are available to help you manage your disease and feel better day-to-day depending on your type of PAH. Your doctor may prescribe medication as part of your treatment. The following specialty medications are available at Albrighton Pharmacy for the treatment of PAH.

Adcirca® (tadalafil)            

Adempas® (riociguat)             

Flolan® (epoprostenol)             

Letairis® (ambrisentan)             

Opsumit® (macitentan)             

Orenitram® (oral treprostinil)            

Remodulin® (intravenous treprostinil)            

Revatio® (sildenafil)             

Tracleer® (bosentan)             

Tyvaso® (inhaled treprostinil)             

Uptravi® (selexipag)             

Veletri® (epoprostenol)             

Ventavis® (iloprost)

Financial assistance coordination may be available to support your drug costs including manufacturer and community programs. Albrighton Pharmacy representatives are available to help you find a program that might work for you 

• Caring Voice Coalition (CVC)
• Good Days
• PSI-Patient Services, Inc.
• Pan Foundation (IPF only, not PH)
• Flolan (epoprostenol) GSK manufacture assistance
• Adcirca (tadalafil) United Therapeutics manufacturer assistance
• Adempas (riociguat) Bayer manufacturer assistance
• Letairis (ambrisentan) Gilead manufacturer assistance
• Opsumit (macitentan) Actelion manufacturer assistance
• Orenitram (oral treprostinil) United Therapeutics manufacturer assistance
• Remodulin (intravenous treprostinil) United Therapeutics manufacturer assistance
• Revatio (sildenafil) Pfizer manufacturer assistance
• Tracleer (bosentan) Actelion Pathways
• Tyvaso (inhaled treprostinil) United Therapeutics manufacturer assistance
• Uptravi (selexipag) Actelion Pathways
• Veletri (epoprostenol) Actelion Pathways
• Ventavis (iloprost) Actelion Pathways

There are many organizations that support research and advocacy for pulmonary arterial hypertension. See below for a few of those organizations.

• Pulmonary Arterial Association
• PHAware
• National Heart, Lung, and Blood Institute (NHLBI)
• National Organization of Rare Disorders (NORD)

• Centers for Medicare and Medicaid
• What is Medicare
• Medicare
• Food and Drug Administration for patients
• The Voice of the Patient – Pulmonary Arterial Hypertension - A series of reports from the U.S. Food and Drug Administration’s (FDA’s) Patient-Focused Drug Development Initiative

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