Pulmonary Hypertension Unit

Targeted Medical Therapy

The information provided in these pages is a general overview of the medical therapies used in the treatment of pulmonary arterial hypertension. This is a summary and does not contain all possible information about this product or known side effects.
This information is not intended as individual medical advice and does not substitute for the knowledge and judgment of your PH Physician or nurse specialist.

Medical therapy in the treatment of PAH

One of the main goals of treatment is to prevent and even induce regression of the disease. Impact on patient quality of life is also a key consideration. It is desirable that the benefits outweigh the risks of treatment.

The therapeutic options will vary as the disease progresses and symptoms become more pronounced but it also depending on the classification of the disease.

Medical Therapies include:

Anticoagulants (Also known as blood thinners.)

Pulmonary hypertension is associated with pulmonary arterial thrombosis and a hypercoagulation.  Anticoagulation may reduce thrombosis and slow progression in some forms of the disease. Warfarin therapy has been shown to almost double three-year survival in primary pulmonary hypertension. For the patient, however, anticoagulation carries the inconvenience of regular INR testing and the possibility of a number of drug interactions.

Recommendations

All patients with primary or thromboembolic pulmonary hypertension should be treated lifelong with warfarin .The generally accepted INR IS 1.5-2.5
Warfarin should be considered in other types of pulmonary arterial hypertension where there is no contraindication, such as gastrointestinal haemorrhage, significant haemoptysis or liver disease with coagulation abnormalities.

There are possible serious side effects of anticoagulation therapy. The risk of bleeding is of major concern.

Points to note:

  • observe for prolonged or unusual bleeding
  • Bruising for unknown reasons
  • Fever, diarrhoea, vomiting.
  • Signs of internal bleeding-swollen or painful abdomen,
  • Joint pain or stiffness. a severe or long standing head ache.
  • Do not take medication-containing aspirin Ibuprofen containing drugs for example. Advil or Tylenol
  • Avoid sudden weight loss
  • Inform all health professionals know that you take warfarin
  • Use alcohol in moderation

Oxygen:

Hypoxaemia (low levels of oxygen in the blood) causes pulmonary vasoconstriction and may worsen pulmonary hypertension. Many patients with pulmonary arterial hypertension show significant falls in arterial oxygen saturation at night. This can be corrected with long-term supplemental oxygen therapy (2 litres/min oxygen from an oxygen concentrator). Some patients will find the need to use supplemental oxygen during the day or with activity.

Recommendations
Controlled oxygen therapy may be indicated for those patients with sustained nocturnal hypoxaemia where arterial oxygen saturations are below an average of 90% on air and are corrected on supplemental oxygen.
Your doctor may insist that you use oxygen during air travel; this is not necessary for all patients.
Remember that oxygen is highly flammable; so keep the tank and tubing away from any open flame.
It is recommended to keep away from high altitudes.

Diuretics:

Right heart failure gives rise to fluid retention which is improved by diuretics which assist the kidneys to eliminate water. Monitoring of renal function is necessary while taking diuretics in order to assess electrolyte levels. Disturbance of electrolyte balance can interfere with normal heart function.

Calcium Channel blockers:(CCBs)

Calcium antagonists cause pulmonary and systemic vasodilatation.
They are effective in the presence of vasoconstriction
The smooth muscle cells in arteries cannot constrict without cellular calcium, and calcium channel blockers assist the arteries in dilating by doing what the name implies.
A positive vasodilator response during heart catheterisation indicates patients who will respond to calcium channel blockers.
Only 10-20% of patients will fall in to this category of responders.
Long-term use of high dose nifidepine and diltiazem reduces pulmonary artery pressure and mortality with sustained improvement of symptoms.
Calcium antagonists cause systemic hypotension and their negative isotropic effect may be adverse when right ventricular function is already damaged.
In patients with left ventricular dysfunction diltiazem and nifidipine may worsen heart failure.

Bosentan (Tracleer)

Bosentan is in a class of medications called endothelin receptor antagonists. It works by stopping the action of endothelin, a natural substance that causes blood vessels to narrow and prevents normal blood flow in people who have PAH.

Bosentan may improve the ability to exercise and slow the worsening of symptoms in patients with PAH.

Bosentan tablets are also used for people who have digital ulcers ( ulcerations on their fingers) as a result of a condition called Scleroderma. Bosentan has been shown to reduce

The number of new finger ulcers that appear and can reduce the severity and frequency of their occurrence.

Before you start Bosentan

Before you start Bosentan therapy, you will be required to have:

  • Liver function testing
  • A Full blood count.
  • A Urine pregnancy test if you are a female of child bearing years.

It is important to tell the pulmonary hypertension nurse specialist at the clinic any medical conditions which you have had or been treated for in the past:

  • A history of liver problems
  • If you are pregnant or think that you may be pregnant
  • If you are taking any medications like cyclosporine (following transplantation)

How to take this medication

Bosentan comes in tablet form and is taken by mouth. It can be taken with or without food twice a day, once in the morning and once in the evening.

It is important to take bosentan at the same time every day to achieve maximum benefit

Bosentan is typically started at the lower dose of 62.5mg and increased to 125mg after 4 weeks of treatment. During this time liver function tests will be carried out to ensure that there are no early signs of liver irritation.

It may take 1-2 months or even longer in some cases before the full benefits of therapy are experienced.

If you suddenly stop taking Bosentan your symptoms may get worse.

It is important to follow the directions provided by your nurse specialist and PH Physician on how to take your medications correctly.

Ask a member of the pulmonary hypertension team to explain any information that you do not understand.

Taking other medicines

Please tell your PH Physician or nurse specialist if you are taking or have recently taken any other medicines, including medicines obtained without a prescription. It is especially important to inform the PH team if you are taking:

  • Hormonal contraceptives (as these are not effective as the sole method of contraception when you take Tracleer).
  • Glibenclamide (for diabetes)
  • Cyclosporine A (a medicine used after transplants and to treat psoriasis), It is also important to mention any other drugs used to treat rejection following transplant.
  • luconazole (used to treat fungal infections)
  • Rifampicin (used to treat tuberculosis)

Potential Minor Side Effects

Bosentan may cause side effects in some people.

Patients are advised to contact the PH Unit and inform a member of the Pulmonary Hypertension team if any of these symptoms are severe and or do not improve

  • Headache
  • Flushing
  • Heartburn
  • Puffy, swollen ankles and or feet
  • Increasing shortness of breath

If you notice yellowing of the skin or eyes (jaundice), or if you have fever with vomiting/ nausea or abdominal pain,contact the pulmonary hypertension unit as soon as possible as this may be related to abnormal liver function.

On occasion some people do not tolerate Bosentan and can have more pronounced side effects. The following symptoms are uncommon, but if you do experience any of them you are advised to contact the Pulmonary Hypertension Unit immediately:

  • Significant swelling of the feet, ankles and lower legs.
  • Sudden weight gain
  • Increased shortness of breath
  • Fast, pounding or irregular heat beat
  • Dizziness
  • Fainting
  • Blurred vision

Female patients

Do not take bosentan if you are pregnant or plan to become pregnant because it may cause harm to the developing foetus.

If you are sexually active and able to become pregnant, you should not begin taking bosentan until a pregnancy test has shown that you are not pregnant.

If you are a woman of childbearing age, your PH physician or gynaecologist will advise you about the most reliable and appropriate contraceptive methods for you while taking Bosentan .

This drug may also make hormonal contraception (e.g., oral, injection, implant or skin patches) ineffective, therefore theses methods on their own are not reliable. It is imperative that if you use hormonal contraceptives you must also use a barrier method (e.g., female condom, diaphragm, contraceptive sponge or your partner must also use a condom).

Monthly pregnancy tests are necessary if you are of child bearing years.

It is important to arrange this with your GP on a monthly basis.

Contact the PH Unit as soon as possible if you miss a period or think that you may be pregnant while you are taking bosentan

Breast-feeding

You are advised to stop breast-feeding if you are taking Bosentan as it is not known if this medicine can pass in to breast milk. Tell a member of the pulmonary hypertension team at once if you are breast-feeding.

Male and Female patients

It is imperative that all persons taking Bosentan have monthly blood tests to monitor liver function as Bosentan can irritate the liver and cause the liver enzymes to become elevated. This can result in symptoms such as nausea, ,vomiting, fatigue, yellowing of the skin or eyes ( Jaundice) weight loss and pain on the right hand side of the abdomen. Regular blood tests are the only way to find potential liver issues before they becomes serious.

Liver function tests should be arranged with your GP on a monthly basis.

Always bring a copy of results to your clinic appointments.

What to do if you forget a dose

Take the missed doseof Bosentan as soon as you remember it. However, if it is almost time for the next dose, skip the missed dose and continue your regular dosing schedule. It is imperative not to take a double dose to make up for a missed one.

Storage of this medication

Keep Bosentan medication in the container it came in, tightly closed, and out of reach of children. Store it at room temperature and away from excess heat and moisture (not in the bathroom). Return any medication that is out of date or no longer needed to your pharmacy.

If you have any queries about any of your medications, contact the Pulmonary Hypertension Unit at 01 8034420.

Sildenafil (Revatio)

What is Revatio?

sildenafilRevatio is a medicine called a phosphodiesterase type 5 (PDE5) inhibitor and it is used in the treatment of Pulmonary Arterial Hypertension (PAH). It can be prescribed for use on its own or added on in combination with other PAH therapies.

People with Pulmonary Hypertension have been found to produce too little of certain chemicals in the body. One such substance is nitric oxide. Nitric oxide helps to dilate the vessels in the lungs (pulmonary arteries) which are narrow and tight as a result of PAH. Revatio works by increasing levels of this substance in the body and helps the pulmonary arteries to dilate as a result. This in turn reduces the narrowing in the vessels and allows more blood to flow from the heart to the lungs.

Revatio has been shown to increase a person’s ability to walk further and reduce pressure in the blood vessels in the lungs (pulmonary arteries). Revatio can control the symptoms of PAH and hopefully impact positively on a person’s quality of life but it does not cure the disease.

How to take this medication

Revatio comes in tablet form and is administered orally.

It is a 20-mg tablet which is taken 3 times a day, morning afternoon and evening.

It can be taken with or without food however some people do complain of indigestion.

If this is the case, Revatio should be take with food to lessen discomfort.

It is important to take Revatio at the same time each day to achieve maximum benefit.

Before you start Revatio

At your clinic appointment, it is important to tell the nurse specialist of PH Physician if you have or have ever experienced any of the following.

  • Heart problems such as heart attack, stroke, irregular heartbeat, or chest pain (angina)
  • Problems with high or low blood pressure or blood circulation
  • An eye problem, glaucoma or problems with eyesight.
  • Severe liver or kidney roblems
  • Any disorders of the blood such as sickle cell anemia ,cancer of the blood cells or bone marrow

Precautions

The decision to commence Revatio will be made by your PH Physician as it is not a suitable therapy for all patients with Pulmonary Hypertension.

If you are taking nitrates for chest pain( angina) such as GTN Spray or nitroglycerine in tablet form, please inform a member of the Pulmonary Hypertension team.

Your physician needs to be informed if you are taking certain drugs for HIV, such as Ritonavir or antifuungals such as Ketaconazole or Intraconazole.

Always bring a list of your current medications with you to each clinic appointment to keep your PH Physician updated on any changes to medications. This will avoid any unnecessary drug interactions.

Potential Side Effects

As with any medication, some people may not tolerate Revatio and may therefore complain of the following symptoms.

  • Nosebleeds
  • Upset stomach
  • Trouble sleeping
  • Headache
  • Flushing

Very few patients have to stop taking Revatio due to side effects however be sure to inform a member of the Pulmonary Hypertension team if you are experiencing any new or troublesome symptoms while taking REVATIO. If you experience any sudden changes to your vision, contact the PH Unit as soon as possible.

What to do if you forget a dose

Take the missed dose of Revatio as soon as you remember it. However, if it is almost time for the next dose, skip the missed dose and continue your regular dosing schedule. It is imperative not to take a double dose to make up for a missed one

It is important to take your Revatio as directed by your PH Physician. Do not stop taking Revatio, decrease or increase doses without consulting the Pulmonary Hypertension Unit.

Remember: If you have any problems or queries pertaining to your medical therapy, contact the Pulmonary Hypertension Unit on 01 8034420.

Storage of this medication

Keep Revatio in the container it came in, tightly closed, and out of reach of children. Store it at room temperature and away from excess heat and moisture (not in the bathroom). Return any medication that is out of date or no longer needed to your pharmacy.

Return any medication that is out of date or no longer needed to your pharmacy.

If you have any queries about any of your medications, contact the Pulmonary Hypertension Unit at 01 8034420.

Sitaxsentan (Thelin)

Thelin is part of a Group of drugs known as Endothelin Antagonists.

It is used to lower the blood pressure in the blood vessels in PAH.

Before you start Thelin

Before you start Thelin therapy, you will be required to have a

  • Liver function testing
  • A Full blood count.
  • A urine pregnancy test if you are a female of child bearing years

It is important to tell the pulmonary hypertension nurse specialist at the clinic any

medical conditions which you have had or been treated for in the past:

  • A history of liver problems
  • If you have raised liver enzymes detected by blood test
  • If you are pregnant or think that you may be pregnant
  • If you are taking any medications like cyclosporine (following transplantation)
  • If you are taking blood thinners –Warfarin. The dose of warfarin may need to be adjusted.

How to take this medication

The dose of Thelin is 100mg once a day.

It comes in tablet form and is taken by mouth and can be taken with or without food twice a day, once in the morning and once in the evening.

It is important to take Thelin at the same time every day to achieve maximum benefit

It is necessary to have liver function tests carried out every 4 weeks to ensure that there are no early signs of liver irritation.

It may take 1-2 months or even longer in some cases before the full benefits of therapy are experienced.

If you suddenly stop taking Thelin your symptoms may get worse.

It is important to follow the directions provided by your nurse specialist and PH Physician on how to take your medications correctly.

Ask a member of the pulmonary hypertension team to explain any information that you do not understand.

Potential Side Effects

Thelin may cause side effects in some people.

Patients are advised to contact the PH Unit and inform a member of the Pulmonary Hypertension team if any of these symptoms are severe and or do not improve

  • Headache
  • Flushing
  • Heartburn
  • Puffy, swollen ankles and or feet
  • Increasing shortness of breath

If you notice yellowing of the skin or eyes (jaundice), or if you have fever with vomiting/ nausea or abdominal pain, unusual tiredness or loss of appetite contact the pulmonary hypertension unit as soon as possible as this may be related to abnormal liver function.

On occasion some people do not tolerate Thelin and can have more pronounced side effects. The following symptoms are uncommon, but if you do experience any of them you are advised to contact the Pulmonary Hypertension Unit immediately:

  • Significant swelling of the feet, ankles and lower legs.
  • Sudden weight gain
  • Increased shortness of breath
  • Fast, pounding or irregular heat beat
  • Dizziness
  • Fainting
  • Blurred vision

Female patients

Do not take Thelin if you are pregnant or plan to become pregnant because it may cause harm to the developing foetus.

If you are sexually active and able to become pregnant, you should not begin taking bosentan until a pregnancy test has shown that you are not pregnant.

If you are a woman of childbearing age, your PH physician or gynaecologist will advise you about the most reliable and appropriate contraceptive methods for you while taking Thelin.

This drug may also make hormonal contraception (e.g., oral, injection, implant or skin patches) ineffective, therefore theses methods on their own are not reliable. It is imperative that if you use hormonal contraceptives you must also use a barrier method (e.g., female condom, diaphragm, contraceptive sponge or your partner must also use a condom).

Monthly pregnancy tests are necessary if you are of child bearing years.

It is important to arrange this with your GP on a monthly basis.

Contact the PH Unit as soon as possible if you miss a period or think that you may be pregnant while you are taking bosentan

 

Breast-feeding

You are advised to stop breast-feeding if you are taking Thelin as it is not known if this medicine can pass in to breast milk. Tell a member of the pulmonary hypertension team at once if you are breast-feeding.

Male and Female patients

It is imperative that all persons taking Thelin have monthly blood tests to monitor liver function as Thelin can irritate the liver and cause the liver enzymes to become elevated. This can result in symptoms such as nausea, ,vomiting, fatigue, yellowing of the skin or eyes ( Jaundice) weight loss and pain on the right hand side of the abdomen.

Regular blood tests are the only way to find potential liver issues before they becomes serious.

Liver function tests should be arranged with your GP on a monthly basis.

Always bring a copy of results to your clinic appointments.

It is also necessary to have a blood test done to assess your haemoglobin( full blood count) after one month on treatment and 3 monthly while on treatment.

What to do if you forget a dose

Take the missed dose of Thelin as soon as you remember it. However, if it is almost time for the next dose, skip the missed dose and continue your regular dosing schedule. It is imperative not to take a double dose to make up for a missed one.

Any queries pertaining to PAH medications should be discussed with a member of the pulmonary hypertension team.

Storage of this medication

Keep Thelin medication in the container it came in, tightly closed, and out of reach of children. Store it at room temperature and away from excess heat and moisture (not in the bathroom). Return any medication that is out of date or no longer needed to your pharmacy.

If you have any queries about any of your medications, contact the Pulmonary Hypertension Unit at 01 8034420.

Ambrisentan (Letairis)

Ambrisentan is licensed for the treatment of PAH with Class I, II and III symptoms

to improve capacity for physical activity and to delay time to clinical worsening .

Treatment is initiated at 5 mg once a day with or without food and increased to 10mg once a day if tolerated. Tablets should not be split, crushed or chewed but swallowed whole.

Potential Side Effects

On occasion some people do not tolerate Letairis and can have more pronounced side effects. If you do experience any of the above mentioned problems you are advised to contact the Pulmonary Hypertension Unit to seek further advice.

Observe for the following:

  • Swelling of lower limbs
  • Sudden weight gain
  • Nasal congestion
  • Hot flushes or facial and neck flushing
  • Headache
  • Increased shortness of breath
  • Palpations
  • Stomach pain

If you notice yellowing of the skin or eyes (jaundice), or if you have fever with vomiting/ nausea or abdominal pain, unusual tiredness or loss of appetite contact the pulmonary hypertension unit as soon as possible as this may be related to abnormal liver function.

Female patients

Do not take Letairis if you are pregnant or plan to become pregnant because it may cause harm to the developing foetus.

If you are sexually active and able to become pregnant, you should not begin taking until a pregnancy test has shown that you are not pregnant.

If you are a woman of childbearing age, your PH physician or gynaecologist will advise you about the most reliable and appropriate contraceptive methods for you while taking Letairis.

This drug may also make hormonal contraception (e.g., oral, injection, implant or skin patches) ineffective, therefore theses methods on their own are not reliable. It is imperative that if you use hormonal contraceptives you must also use a barrier method (e.g., female condom, diaphragm, contraceptive sponge or your partner must also use a condom).

Monthly pregnancy tests are necessary if you are of child bearing years.

It is important to arrange this with your GP on a monthly basis.

Contact the PH Unit as soon as possible if you miss a period or think that you may be pregnant while you are taking bosentan

Breast-feeding

You are advised to stop breast-feeding if you are taking Letairis as it is not known if this medicine can pass in to breast milk. Tell a member of the pulmonary hypertension team at once if you are breast-feeding.

Male and Female patients

It is imperative that all persons taking Letairis have monthly blood tests to monitor liver function as Letairis can irritate the liver and cause the liver enzymes to become elevated. This can result in symptoms such as nausea, ,vomiting, fatigue, yellowing of the skin or eyes ( Jaundice) weight loss and pain on the right hand side of the abdomen.

Regular blood tests are the only way to find potential liver issues before they becomes serious.

Liver function tests should be arranged with your GP on a monthly basis.

Always bring a copy of results to your clinic appointments.

It is also necessary to have a blood test done to assess your haemoglobin( full blood count) after one month on treatment and 3 monthly while on treatment.

What to do if you forget a dose

Take the missed dose of Letairis as soon as you remember it. However, if it is almost time for the next dose, skip the missed dose and continue your regular dosing schedule. It is imperative not to take a double dose to make up for a missed one.

Storage of this medication

Keep Letairis medication in the container it came in, tightly closed, and out of reach of children. Store it at room temperature and away from excess heat and moisture (not in the bathroom). Return any medication that is out of date or no longer needed to your pharmacy.

Any queries pertaining to PAH medications should be discussed with a member of the pulmonary hypertension team.

Inhaled Iloprost (Ventavis)

Ventavis is a member of a family of drugs known as prostanoids. It is an inhaled prostacyclin which is administered using a specially designed nebulizer.

How does it work?

Patients with pulmonary hypertension have a decrease in many chemicals which typically circulate in the blood. One such substance is prostacyclin which dilates the pulmonary arteries and helps to increase blood flow from the heart to the lungs. However in PAH the level of prostacyclin is decreased, this further adds to the problem of narrowing in the pulmonary arteries. Ventavis helps to increase his level of prostacyclin through inhalation. Thereby increasing dilation of the pulmonary arteries and increase blood flow from the heart to the lungs and reducing symptoms of PAH.

How to take this medication

Ventavis is contained within a glass ampoule or vial.

The solution is drawn up and inserted in to a nebulizer chamber.

The solution is then changed from a liquid in to an aerosol mist by a special machine called a nebuliser. Breathing in the mist carries Ventavis deep in to the lungs, where it can work most effectively on the vessels that are narrowed helping to reduce symptoms. It is important to discard any unused Ventavis solution that is not usde in one inhalation session.

Most people will have 6 inhalation sessions spread throughout the day.

One dose is administered every 3 hours. Ventavis is taken at night to allow for undisturbed sleep.

One inhalation session will usually last about 8-10 minutes depending on the person’s technique and breathing pattern.

Sometimes people experience fatigue towards the end of the day and hence the last nebulizations or two may take longer than usual.

Before you start Ventavis

If you think any of the following may apply to you, it is important to tell your PH Physician or nurse specialist:

  • f you are allergic (hypersensitive) to iloprost or any of the other ingredients of
  • Ventavis.
  • Had or have a history of stomach ulcers, injuries, or other bleeding disorders
  • If you have had a stroke within the last 3 months
  • A history of a heart attack within the last six months
  • Severe changes in heart rate
  • Have a History of chest pain, angina or coronary artery disease.
  • A heart defect, faulty heart valve or congenital heart defect.
  • If you are pregnant or breast-feeding
  • If you have infection of the lungs, severe asthma, or other lung disease as this
  • may make inhaling the medication difficult.
  • A history of low blood pressure

Taking other medicines

 

Please tell your doctor or nurse specialist if you are taking or have recently taken any other medicines, including medicines obtained without a prescription. It is especially important to tell your doctor if you are taking. In particular medicines used to treat high blood pressure or heart disease or effect blood clotting such as aspirin or warfarin

Pregnancy:

Do not take Ventavis if you are pregnant or plan to become pregnant because

Ventavis may cause harm the developing foetus.

If you are sexually active and able to become pregnant, you should not begin taking Ventavis until a pregnancy test has shown that you are not pregnant.

If you are a woman of childbearing age, your PH physician or gynaecologist will advise you about the most reliable and appropriate contraceptive methods for you while taking Ventavis.

This drug could make hormonal contraception (e.g., oral, injection, implant or skin patches)less effective, therefore theses methods on their own are not reliable.

It is imperative that if you use hormonal contraceptives you must also use a barrier method (e.g., female condom, diaphragm, contraceptive sponge or your partner must also use a condom).

Contact the PH Unit as soon as possible if you miss a period or think that you may be pregnant while you are taking bosentan

Breast-feeding

Ventavis must not be given to women who are breast-feeding, since it is not known whether the active substance is passed on through breast milk. Ask your doctor or pharmacist for advice before taking any medicine.

Newborns, infants, and pregnant women should not be subjected to Ventavis in the room air. These persons should not remain in a closed room where Ventavis is being administered to a patient.

Precautions

Be sure to ventilate or air the room in which you have taken your Ventavis treatment.

Ventavis dilates the blood vessels from the heart to the lungs and can cause a drop in blood pressure. This may cause dizziness or light-headedness in some people.

Do not drive or operate any tools or machines if you feel these effects of low blood pressure.

Do not let Ventavis solution come into contact with your skin or eyes.

If it does, rinse the skin or your eyes immediately with water.

Do not drink Ventavis solution. If drinking accidentally occurs, drink plenty of water and contact your doctor.

Return any medication that is out of date or no longer needed to your pharmacy.

What to do if you forget a dose

Take the missed dose of Ventavis as soon as you remember it. However, if it is almost time for the next dose, skip the missed dose and continue your regular dosing schedule. It is imperative not to take a double dose to make up for a missed one

It is important to take your Ventavis as directed by your PH Physician. Do not stop taking Ventavis, decrease or increase doses without consulting the Pulmonary Hypertension Unit.

Potential side effects

Ventavis causes vaso dilation of the blood vessels going from the heart to the lungs and as previously mentioned it can cause a drop in blood pressure

Some people can experience the following:

  • Low blood pressure on starting Ventavis or on dose increase
  • Dizziness or fainting
  • Flushing of the face and neck
  • Headache
  • Nausea
  • Vomiting
  • Diarrhoea

If you experience any problems taking Ventavis or have any queries pertaining to your drug therapy or overall condition, it is important to contact the pulmonary hypertension Unit on 01 8034420

Subcutaneous Treprostinil (Remodulin)

Remodulin is a class of drug known as a prostacyclin and it is indicated for use in patients with PAH who display Class II-IV symptoms.

It is typically used in conjunction with other PAH medications.

It is a very potent vasodilator, causing dilation of the pulmonary arteries (blood vessels going from the right side of the heart to the lungs) but also dilates the blood vessels in the general circulation.

This causes widening of the blood vessels and increases blood flow from the heart to the lungs while reducing the resistance against which the heart has to pump against.

As with all PAH treatments, the aim is to reduce symptoms associated with activity and or at rest and try to improve quality of life.

How is this medication given?

Remodulin is administered by means of a small infusion pump which is connected to a very fine bore catheter (tubing) which is then connected to a very fine needle and this needle is inserted in to the fatty tissue of the abdomen.

The point on the skin where the needle is inserted is referred to as the infusion site. Typically these infusion sites are changed every 5-7 days or but can be kept for longer periods if the site is not causing problems.

ut15Each time that a person changes an infusion site, the new site is inserted in a different part of the abdomen.

By rotating the sites where drug is delivered, you can limit the problems associated with discomfort.

Treatment with Remodulin means that the person will receive a continuous infusion of drug 24 hours a day, 7 days a week.

The infusion is never discontinued or stopped unless by the PH Physician or Nurse Specialist.

Potential side effects

Remodulin works by dilating the blood vessels in the lungs and also those in the general circulation. As a result of this some people can experience side effects when treatment is initiated and when the drug dose is increased.

The following are a list of symptoms than can occur:

  • Low blood pressure
  • Pallor,
  • Dizziness
  • Flushing of the face and neck
  • Nausea, and or vomiting,
  • Diarrhoea,
  • Discomfort at the infusion site; this can vary between patients, in some case it can be
  • mild and others can experience pain.
  • The infusion site can look red around the area ( erythema) and can feel warm or hot to touch. Ice packs, topical analgesia (creams applied directly to the surface of the skin and anti-inflammatory agents are used regularly to treat site discomfort.

Precautions

Abrupt withdrawal or sudden large reductions in drug delivery of Remodulin may result in worsening of PAH symptoms and should be avoided.

To avoid potential interruptions in drug delivery, the patient must have immediate access to a backup infusion pump, subcutaneous infusion sets, spare pump batteries and vials of drug.

Remodulin may lower blood pressure therefore it is important to tell your PH Physician if you are taking any of the following:

  • Water tablets (diuretics)
  • Tablets used to treat high blood pressure such as Nifidipine, Captopril
  • Vasodilators such as Nitrates used to treat angina
  • Blood thinners

Do not drive or operate any tools or machines if you feel these effects of low blood pressure.

Do not let Remodulin solution come into contact with your skin or eyes.

If it does, rinse the skin or your eyes immediately with water.

Breast Feeding

It is not known whether Remodulin is excreted in breast milk or absorbed systemically

(in to the general circulation) after ingestion.

Because many drugs are excreted in human breast milk, caution should be exercised when Remodulin is administered to nursing women.

Storage of this medication

Keep unopened Remodulin vials stored in a cupboard at room temperature and out of reach of children. Once a vial of Remodulin is opened, store it in the fridge and use within 30 days. Return any medication that is out of date or no longer needed to your pharmacy. If you have any queries about any of your medications, contact the Pulmonary Hypertension Unit at 01 8034420.

Intravenous Treprostinil (Remodulin)

Remodulin is infused through a catheter which is surgically placed under the skin.

This indwelling central venous catheter remains in place for prolonged periods, possibly years, and the patient’s ability to accept and care for the catheter is a huge consideration before treatment can be considered.

Intravenous Remodulin works in the same way as Remodulin which is administered

via the subcutaneous route.

Potential side effects

The side effect profile for Intravenous Remodulin is the same as the symptoms patients experience on subcutaneous Remodulin. However as intravenous Remodulin is infused directly in to the blood stream, symptoms can be more pronounced.

Sympotoms can include:

  • Facial and neck flushing
  • Headache
  • Nausea
  • Vomiting
  • Diarrhoea or loose stool
  • Low blood pressure
  • Dizziness
  • Jaw pain

It is important to rememer that each person responds in a different way to intravenous Remodulin and therefore not all patients will experience all of the above symptoms when treatment is initiated. Typically symptoms are more pronounced when treatment begings and when doses are increased.

Breast Feeding

It is not known whether Remodulin is excreted in breast milk or absorbed systemically

after ingestion. Because many drugs are excreted in human breast milk, caution should be exercised when Remodulin is administered to nursing women.

Precautions

Abrupt withdrawal or sudden large reductions in drug delivery of Intravenous Remodulin can result in abrupt worsening of PAH symptoms and should be avoided at all costs.

To avoid potential interruptions in drug delivery, the patient must have immediate access to a backup infusion pump, infusion sets, spare pump batteries and vials of drug.

Remodulin is a vaso dilator and can cause a drop in blood pressure.

This may cause dizziness or light-headedness in some people.

To reduce the risk of dizziness and lightheadedness, get up slowly when rising from a sitting to a lyying position.

Do not drive or operate any tools or machines if you feel these effects of low blood pressure.

Do not let Remodulin solution come into contact with your skin or eyes.

If it does, rinse the skin or your eyes immediately with water.

Remodulin may lower blood pressure therefore it is important to tell your PH Physician if you are taking any of the following:

  • Water tablets (diuretics)
  • Tablets used to treat high blood pressure such as Nifidipine, Captopril
  • Vasodilators such as Nitrates used to treat angina
  • Blood thinners

Line Infection

Intravenous Remodulin is administered through a permanent indwelling central venous catheter which is surgically inserted under the skin. This catheter provides direct access to the blood stream and as a result can lead to line infection ( sepsis). To avoid or lessen the risk of infection, follow the nurse specialist`s advice on proper handling of the medication and cleaning of the line.

Signs of infection:

  • Soreness at the catheter site
  • Redness around the catheter site
  • Heat
  • Fever
  • Chills
  • Clammy
  • Low blood pressure
  • Lightheadness Report and signs of infection immediately to a member of the pulmonary hypertension team.

Intravenous Epoprostenol (Flolan)

flolanDue to the nature and course of the disease, people with pulmonary hypertension will require more intensive medical therapies as symptoms become more pronounced.

Intravenous Flolan is FDA approved and indicated for clinical use in the long-term intravenous treatment of Idiopathic Pulmonary Arterial Hypertension or Pulmonary Arterial Hypertension associated with collagen vascular disease (the Scleroderma spectrum of patients).

Potential side effects

Intravenous Flolan is infused directly in to the blood stream, terefore symptoms can occur very quickly and be more pronounced.

Sympotoms can include:

  • Facial and neck flushing
  • Headache
  • Nausea
  • Vomiting
  • Diarrhoea or loose stool
  • Low blood pressure
  • Dizziness
  • Jaw pain

It is important to rememer that each person responds in a different way to intravenous Flolan and therefore not all patients will experience all of the above symptoms when treatment is initiated. Typically symptoms are more pronounced when treatment begings and when doses are increased.

Precautions

Abrupt withdrawal or sudden large reductions in drug delivery of Intravenous Flolan can result in abrupt worsening of PAH symptoms and prove fatal.

To avoid potential interruptions in drug delivery, the patient must have immediate access to a backup infusion pump, infusion sets, spare pump batteries and vials of drug.

Flolan is a potent vaso dilator and can cause a drop in blood pressure.

This may cause dizziness or light-headedness in some people.

To reduce the risk of dizziness and lightheadedness, get up slowly when rising from a sitting to a lyying position.

Do not drive or operate any tools or machines if you feel these effects of low blood pressure.

Do not let Flolan solution come into contact with your skin or eyes. If it does, rinse the skin or your eyes immediately with water.

As mentioned, Flolan may lower blood pressure therefore it is important to tell your PH Physician if you are taking any medication which can further drop blood pressure.

  • Water tablets (diuretics)
  • Tablets used to treat high blood pressure such as Nifidipine, Captopril
  • Vasodilators such as Nitrates used to treat angina
  • Blood thinners

Line Infection

Intravenous Flolan is administered through a permanent indwelling central venous catheter which is surgically inserted under the skin. This catheter provides direct access to the blood stream and as a result can lead to line infection ( sepsis). To avoid or lessen the risk of infection, follow the nurse specialist`s advice on proper handling of the medication and cleaning of the line.

Signs of infection:

  • Soreness at the catheter site
  • Redness around the catheter site
  • Heat
  • Fever
  • Chills
  • Clammy
  • Low blood pressure
  • Lightheadness Report and signs of infection immediately to a member of the pulmonary hypertension team.

 

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