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Lee Shapiro, M.D., FACP, The Center for Rheumatology, LLP, Albany, New York Robert Simms, M.D., Boston University School of Medicine Carol Feghali-Bostwick, Ph.D., Assistant Professor of Medicine, University of Pittsburgh Thomas A. Medsger, M.D., Jr., Gerald P. Rodnan Professor of Medicine at the University of Pittsburgh School of Medicine

 

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Your Medications: A Guide to Better Understanding

In previous articles in "Scleroderma Voice," we have encouraged you to become better educated about your disease, and to keep an accurate accounting of your current medication list, allergies, surgeries, and annual diagnostic screenings.

Knowledge is Power!

Becoming better informed and more organized will help you be a more effective partner in your healthcare. Knowledge is power, and we certainly want you to be as empowered as possible.

To that end, we are providing yet another way to help you engage in an open and healthy dialogue with your physicians. Because persons with scleroderma may see several physicians for a variety of health issues, the list of medications that one takes can be confusing to say the least. It is our goal to take as much of the mystery out of your medication list as possible, and to raise your medication IQ.

Understand What You Are Taking and Why

During an appointment with your physician, a new medication may be prescribed for you. Due to all the other issues that may be discussed during that short and hectic period of time, you may leave the doctor’s office wondering exactly what this new medication is supposed to do for you. Based on my conversations with patients, this scenario is more common than you may imagine.

After returning to the quiet of your home, you can start to absorb the information from your recent office visit, and review your doctor’s instructions.

You may ask yourself: Which one of my symptoms was this prescription intended to treat? How soon will I see an improvement? What side effects should I look for?

To help address these questions you may have, we have decided to offer the following guide as a tool. We sincerely hope it will help to clarify some of the issues.

Disclaimer

This list is provided for informational purposes only, and is not to be taken as an endorsement of any drug by the Scleroderma Foundation or its Medical Advisory Board.

Many of these drugs are prescribed by doctors, but have not been proven useful and should be used only with caution and supervision.

Please keep in mind, not every medication is appropriate for every patient.

A review of your present medication list—including over-the-counter and herbal preparations —taking into account your symptoms and unique medical history, will be necessary to help you and your physician make informed decisions about changes to your medication regime.

This Medications Guide Will Be Updated As Needed, Here On Our Website

The Scleroderma Foundation plans to publish an updated version of this guide each year in our magazine, Scleroderma Voice.

Also, we will maintain this medications guide on this page of our website. The online guide will be updated on an ongoing basis as required.

The Scleroderma Foundation will inform you about any new developments regarding drug breakthroughs on our website and in our publications as we become aware of them.

Can't Afford Your Prescriptions? Look into Patient Assistance Programs (PAPs)

If you can’t afford to buy your prescription medications, most drug makers offer a limited supply of free prescription medication to eligible patients through Pharmaceutical Manufacturers Patient Assistance Programs (PAPs).

For more information, ask your doctor or your pharmacist, or look in the PhRMA (Pharmaceutical Research and Manufacturers of America) directory. To get the directory, call 800-762-4636 or visit www.phrma.org.

Web Resources

Needymeds.com – A resource for finding Patient Assistance Programs and other assistance with the cost of healthcare.

You can also search online at www.helpingpatients.org, a new website created by PhRMA and 48 of its member companies, designed to help providers and patients find Patient Assistance Programs.

The searchable database at www.rxassist.org helps you find PAPs by company, brand name, generic name and drug class. It includes eligibility and application instructions.

At www.benefitscheckup.org, thanks to the National Council on the Aging (202-479-1200), persons 55 and over can get a personalized report of programs that can help save money on prescription drugs.

 

Scleroderma Medications Guide

Raynaud's Phenomenon

Calcium Channel Blockers

nifedipine (Procardia®, Adalat®)
amlodopine (Norvasc®)
isradipine (Dynacirc®)
diltiazem (Cardizem®, Dilacor XR®)
nicardipine (Cardene®)
nisoldipine (Sular®)
felodipine (Plendil®)

Action

Relax blood vessels

Most Common Side Effects

Low blood pressure, constipation, nausea, may worsen swallowing difficulty, lightheadedness, headache, leg edema, palpitations, irregular heartbeats

Angiotensin II Receptor Antagonists

losartan (Cozaar®)
valsartan (Diovan®)

Action

Block constriction of blood vessels

Most Common Side Effects

Diarrhea, dizziness, cough, muscle pain

Others

prazosin (Minipress®)
doxazosin (Cardura®)

pentoxifylline (Trental®)

Action

Relax blood vessels

Unclear

Most Common Side Effects

Low blood pressure
 

Nausea, dizziness

Gastrointestinal Symptoms

Gastroesophageal Reflux Disease (GERD)

Antacids

Gaviscon, Maalox, Mylanta, Rolaids, Tums

Action

Neutralize stomach acidity

Most Common Side Effects

Diarrhea, constipation

H-2 Blockers

cimetidine (Tagamet®)
ranitidine (Zantac®)
famotidine (Pepcid®)
nizatidine (Axid®)

Action

Inhibit stomach acid secretion

Most Common Side Effects

Mental confusion (cimetidine only), diarrhea, headache, dizziness

Proton Pump Inhibitors

omeprazole (Prilosec®)
lansoprazole (Prevacid®)
esomeprazole (Nexium®)
rabeprazole (Aciphex®)
pantoprazole (Protonix®)

Action

Inhibit stomach acid secretion

Most Common Side Effects

Diarrhea, headache, dizziness

Others

sucralfate (Carafate®)

Action

Coat esophagus & stomach; forms protective barrier

Most Common Side Effects

Constipation

Gastrointestinal Symptoms, continued

Improve Swallowing

GI Stimulants

bethanecol (Urecholine®)
metoclopramide(Reglan®)


erythromycin

octreotide acetate (Sandostatin®), an injectable medication

Action

Stimulate intestinal muscle contractions, may improve heartburn

Can be helpful in very severe cases for improving the motion of the bowel

Most Common Side Effects

Abdominal cramping, diarrhea, dizziness, headache, drowsiness
(Reglan only)

Gastric irritation, nausea

Low heart rate, cardiac arrhythmias, nausea, diarrhea, pain at injection site, expense

Gastrointestinal Symptoms, continued

Relieve Constipation

Bulking Agents

calcium polycarbophil (FiberCon®, Fiberall®)
psyllium (Metamucil®)

Action

Soften stool

Most Common Side Effects

Abdominal bloating, flatulence

Softening Agents

docusate calcium (Surfak®, Sulfolax®)
docusate sodium (Colace®)

Action

Soften stool

Most Common Side Effects

Mild abdominal cramping

Others

lactulose (Cephulac®, Cholac®)

polyethylene glycol (MiraLax®)

Action

Make bowels move

Most Common Side Effects

Abdominal cramping, flatulence

Nausea, abdominal bloating, cramping, flatulence

Gastrointestinal Symptoms, continued

Small Intestine Dysfunction: Bacterial Overgrowth/Diarrhea

Broad Spectrum Antibiotic use is the mainstay of treatment for this complication. There are many potential approaches to this therapy. Example: Antibiotics are given in 2–3 week courses followed by a 1–2 week drug holiday. Generally a few cycles of this treatment can allow for quiet periods of a few months to a few years. However, some persons may require almost continuous antibiotics.

Alternating antibiotics and increasing the antibiotic-free period will decrease the development of resistant strains of bacteria.

Note: Prolonged use of Broad Spectrum Antibiotics may be complicated by superinfection.

Broad Spectrum Antibiotics

Examples include:
tetracycline
ampicillin
metronidazole (Flagyl®)
vancomycin, ciprofloxacin (Cipro®)
amoxicillin/clavulanate (Augmentin®)
clarithromycin (Biaxin®)
azithromycin (Zithromax®)

Action

Decrease bacterial overgrowth

Most Common Side Effects

GI upset, diarrhea, nausea, and vomiting, prolonged use may be complicated by superinfection

Joint & Tendon Pain

Non-Steroidal Anti-Inflammatory Drugs (NSAIDs)

flurbiprofen (Ansaid®)
fenoprofen (Nalfon®)
nabumeton (Relafen®)
diclofenac (Voltaren®, Cataflam®)
diclofenac & misoprostol (Arthrotec®)
diclofenac sodium (Voltaren®)
etodolac (Lodine®)
ibuprofen (Motrin®, Advil®, Nuprin®)
indomethacin (Indocin®)
ketoprofen (Orudis®)
ketorolac (Toradol®)
meloxicam (Mobic®)
naproxen (Naprosyn®, Anaprox®,
Aleve®)
oxaprozin (Daypro®)
piroxicam (Feldene®)
suldinac (Clinoril®)

Action

Suppress inflammation

Most Common Side Effects

GI upset, stomach ulcers, impaired kidney function, liver inflammation, confusion

COX-2 Inhibitors

celecoxib (Celebrex®)

Action

Suppress inflammation

Most Common Side Effects

Headache, edema, rash

Analgesics

acetaminophen (Tylenol®)

tramadol (Ultram®)

Action

Relieve pain

Most Common Side Effects

Risk of liver & kidney damage

Esophagus irritation & sedation

Narcotics

acetaminophen/propoxyphene (Darvocet-N®)
acetaminophen/hydrocone (Vicodin®)
acetaminophen/oxycodone (Percocet®)

oxycodone hydrochloride (OxyContin®, OxyIR®)

Action

Relieve severe pain

Most Common Side Effects

Addiction possible, constipation, dizziness, drowsiness, nausea, vomiting




Sedation, low blood pressure, nausea, vomiting

Pulmonary Fibrosis/Alveolitis

Immunosuppressants

(none proven in controlled trials;
the following have been used)

cyclophosphamide (Cytoxan®)




azathioprine (Imuran®)



cyclosporine (Neoral®, Sandimmune®)


mycophenolate mofetil (Cellcept®)

Action




Suppress immune response


Suppress immune response

Impair lymphocytes


Impair lymphocytes

Most Common Side Effects



Bone marrow damage, hair loss, nausea, diarrhea, bladder inflammation, increased cancer risk, increased infection

Nausea, pancreatitis, increased infection, increased cancer risk, anemia

Tremor, headache, hypertension, nausea, vomiting, kidney & liver injury, infections

Tremor, headache, hypertension, chest pain, GI hemorrhage, nausea, vomiting, anemia, fever, infection, sepsis, leg edema

Pulmonary Arterial Hypertension

Patients diagnosed with pulmonary arterial hypertension should be referred to a pulmonary hypertension center for experienced evaluation and management of this serious scleroderma complication.

Endothelin Receptor Antagonists

ambrisentan (Letairis®)

 

bosentan (Tracleer®)

Action

Act on blood vessels

 

Act on blood vessels

Most Common Side Effects

Potential risk of liver injury, edema, and birth defects

 

Potential liver injury, headache, flushing, edema, anemia, male infertility, birth defects

Prostaglandin Derivatives

epoprostenol (Flolan®)





iloprost (Ventavis®) inhalation solution



trepostinil (Remodulin®)

Action

Act on blood vessels




Act on blood vessels


Act on blood vessels

Most Common Side Effects

Headache, flushing, jaw pain, diarrhea, foot/bone pain, potential serious infection associated with central line catheter in the chest wall

Flushing, increased cough, low blood pressure, headaches, nausea, spasm of jaw muscles, fainting

Headache, flushing, pain at infusion site

Calcium Channel Blockers

Can be helpful to a small proportion of patients; see Raynaud’s section

Action

See Raynaud's section

Most Common Side Effects

See Raynaud's section

Phosphodiesterase Type 5 (PDE5) Inhibitor

sildenafil citrate (Revatio®)

 

tadalafil (Adcirca®)

Action


Act on blood vessels

Act on blood vessels

Most Common Side Effects


headache, dyspepsia, flushing, nosebleeds, insomnia

headache, stomach upset,
back pain, muscle pain, nasal stuffiness, flushing, pain
in arms or legs, dizziness, or vision change

Renal Crisis/New Onset Hypertension

ACE Inhibitors

captopril (Capoten®)
enalapril (Vasotec®)
lisinipril (Prinivil®, Zestril®)
quinapril (Accupril®)
ramipril (Altace®)
fosinopril (Monopril®)
benazopril (Lotensin®)
moexipril (Univasc®)
trandolapril (Mavik®)

Action

Block constriction of blood vessels

Most Common Side Effects

Skin rash, dry mouth, taste disturbances, fluid retention, drowsiness, lightheadedness, diarrhea, cough

If taking corticosteroids, monitor your blood pressure frequently, and report changes to your healthcare provider immediately.

Warning About Corticosteroids and Renal Crisis

Corticosteroids, such as prednisone and medrol, have been used to treat several complications of systemic scleroderma, such as pulmonary fibrosis, pruritis (itchy skin), and joint pain.

Recent findings indicate that corticosteroids can be implicated in precipitating renal crisis.

Therefore, the medical community has concluded corticosteroids should be used cautiously, in a carefully screened patient population.

For example: to manage patients who also have myositis (muscle inflammation).

Anyone taking corticosteroids should monitor blood pressure frequently, and report changes to the healthcare provider immediately.

Skin Fibrosis

Immunosuppressants

(none proven in controlled trials;
the following have been used)

cyclophosphamide (Cytoxan®)
 

   

cyclosporine (Neoral®), Sandimmune®)
 

D-penicillamine (Cuprimine®, Depen®)
 

methotrexate (Rheumatrex®, Trexall®)
 

mycophenolate mofetil (Cellcept®)

Action


 

Suppress immune response
 

Impair lymphocytes
 

May inhibit collagen cross-linking

Suppress immune response

Impair lymphocytes

Most Common Side Effects


 

Bone marrow damage, hair loss, nausea, diarrhea, bladder inflammation, increased cancer risk, increased infections

Tremor, headache, hypertension, nausea, vomiting, kidney & liver injury, infections

Bone marrow damage (rare), kidney damage, mouth sores, skin rash, nausea, diarrhea

Liver inflammation, nausea, mouth sores, skin rash
 

Tremor, headache, hypertension, chest pain, GI hemorrhage, nausea, vomiting, anemia, fever, infection, sepsis, leg edema

Sjögren's Syndrome

Prescription Drugs

pilocarpine hydrochloride (Salagen®)




cevimeline hydrochloride (Evoxac®)

Action

Improves dry mouth symptoms in patients with Sjögren’s

Most Common Side Effects

Dizziness, headache, flushing, urinary frequency, nausea, sweating, hypertension, tachycardia



Headache, rhinitis, diarrhea, nausea, sweating, urinary frequency

Over-the-Counter Products

Dry Mouth:

Salivart®



Biotene Oral Balance
Moisturizing Gel®
Biotene Dry Mouth Gum®
Biotene Dry Mouth Toothpaste®
Biotene Dry Mouth Mouthwash®
Optimoist®
Salix Lozenges®

Dry Eyes:

Artificial tears®

Action

 

Saliva substitute, alleviate dry mouth







 

Provide tear-like lubrication

Most Common Side Effects

 

No significant side effects observed








 

 

Mild stinging, temporary blurred vision

Reactive Depression

Selective Serotonin Reuptake Inhibitors (SSRI's)

sertraline (Zoloft®)
paroxetine (Paxil®)
fluoxetine (Prozac®)
fluvoxamine (Luvox®)

Action
 

Improve symptoms of depression and peripheral pain; Prozac may also improve Raynaud's

Most Common Side Effects
 

Headache,insomnia, sleepiness, nervousness, diarrhea, nausea, constipation (Paxil® only)

Tricyclic Antidepressants

amitriptyline (Elavil®)
nortriptyline (Pamelor®)
imipramine (Tofranil®)
clomipramine (Anafranil®)

Action

Improve symptoms of depression, may improve restorative sleep

Most Common Side Effects

Drowsiness, dry mouth, blurred vision, orthostatic hypotension, weight gain, constipation

Other Treatments

bupropion (Wellbutrin®)
venlafaxine (Effexor®)
 

nefazodone (Serzone®)
 

trazadone (Desyrel®)

Action

Improve symptoms of depression

Improve depression

May improve depression-related insomnia

Most Common Side Effects

Headache, insomnia, dry mouth, high blood pressure, nausea


 


Sedation, nausea, orthostatic hypotension

Localized Scleroderma

Medications

(none proven in controlled trials;
the following have been used)

hydroxychloroquine sulfate (Plaquenil®)

methotrexate (Rheumatrex®, Trexall®)


prednisone (Deltasone®, Cortan®)


phenytoin (Dilantin®)


potassium p-aminobenzoate (POTABA®)


D-penicillamine (Cuprimine®, Depen®)


PUVA (phototherapy)

Action




Unclear


Suppress immune response

Suppress inflammation

Unclear


Unclear


May inhibit collagen cross-linking


Decrease skin thickening

Most Common Side Effects




Seizures, leukopenia, thrombocytopenia

Liver inflammation, nausea, mouth sores, skin rash


Fluid retention, GI irritation


Dizziness, confusion, nausea, vomiting

Skin rash, nausea, diarrhea

Bone marrow damage (rare), kidney damage, mouth sores, skin rash, nausea, diarrhea

Burning, itching, nausea, skin aging

Skin Itching (Pruritis)/Dryness

To relieve dry itchy skin, it is necessary to moisturize often. Also, you must protect your skin from harsh detergents, hot water, and the cold winter air, all of which will rob moisture from your skin. You may want to consider using a humidifier in the home during the cold winter heating months to replace much-needed moisture into the air. Remember that not everyone will have the same response to every product. You may have to try a few products until you find one that will work for you.

Over-the-Counter Skin Lotions
KeriCream®
Lanalor®
Lubriderm®
Eucerin Moisturizing® creams and lotions
Nivea Moisturizing®
UltraDerm®
Alpha Keri Bath Oil®
Penederm alpha hydroxy cream®
Aveeno bath oil & moisturizer®

Action
Moisturize skin

Most Common Side Effects
No significant side effects observed

Antihistamines (Prescription)

diphenhydramine (Benadryl®)
hydroxyzine (Atarax®)

 
 

Colchicine

Action

Block histamine response, thereby decreasing itching

Reduce inflammation associated with calcinosis

Most Common Side Effects

Drowsiness, dry mouth

 
 
 

Diarrhea

 

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