Ask a PH Specialist - Checklist
Though PH is generally a rare disease, seasonal allergies (or allergic rhinitis) are thought to affect up 30% of all adults. Typical symptoms of allergic rhinitis include nasal congestion, facial pressure, post-nasal drip, itchy and/or watery eyes, swelling around the eyes and sneezing. For some people seasonal allergies only become troublesome at certain times of the year depending on the environmental allergen that triggers the symptoms (e.g.: pollen in the spring). However, a large percentage of patients with allergic rhinitis has some symptoms throughout the entire year. In these cases, an indoor allergen may be responsible. In addition, patients with allergic rhinitis often have a predisposition to other related conditions, such as asthma and/or sinusitis.
As with any medical problem, establishing the correct diagnosis is key to determining proper treatment. It is important to remember that certain medications used to treat PH are associated with nasal congestion as a side effect. These include the endothelin receptor antagonists (Bosentan and Ambristentan) as well as phosphodiesterase-5 inhibitors (Sildenafil and Tadalafil). If nasal congestion occurs after starting a new PH medicine, for example, and there are no other symptoms to suggest allergy (such as itch eyes or a clear trigger), then it may be that your new PH medicine is responsible for the symptoms. If you suspect this, don't stop the medicine - rather, consult with your PH doctor first. As mentioned above, having allergic rhinitis may predispose to bouts of sinusitis, an infection of the sinus cavities within the head. Bacterial sinus infections may cause fevers, intense facial pressure, yellow or green drainage from one side of the nose, or upper tooth pain. If you notice these sorts of symptoms, you should call your physician immediately as other treatments may be required.
Assuming you have typical seasonal allergies, common approaches to treatment are aimed at rescuing inflammation in the nasal cavities. Trying to identify the trigger for the symptoms is very important, as this can help you know what to avoid, which might help improve the symptoms. Sometimes, allergy testing can help you figure out what your trigger might be. Assuming you have minimised exposure to any known allergens, nasal corticosteroids are the first line medicines used to treat allergic rhinitis. these act topically to reduce inflammation and congestion. In general, these medications are safe for use in patients with PH (a tip for use: point nozzle away from the middle part of the nose, as it can become easily irritated). Second, because the chemical histamine is involved in the allergic process, antihistamine medicines are also commonly used to treat seasonal allergies. Like nasal corticosteroids, we general feel these are safe for most PH patients; however, they can cause drowsiness. Though less commonly used, some types of rhinitis also may respond to a nasal spray called ipratropium bromide, also believed to be relatively safe in PH patients.
Types of medicines that are considered potentially unsafe in PH patients are those that act by constricting blood vessels. These are commonly referred to in a generic fashion as "decongestants", but more specifically include over the counter drugs pseudoephedrine (Sudafed), phenylephrine and the nasal spray oxymetazoline (Afrin). The first two drugs are often found in common brand name "cold and flu" or "sinus" preparations (e.g., Tylenol Sinus or Cold); there are also generic equivalents boxed as drug store brands. It is important that you always read the list of ingredients when buying a combination product. Some of these types of medicines have been shown to further increase the pressure within the pulmonary vessels as well as put a strain on the right side of the heart. On a case by case basis, these medications may be permissible for short term use, but you should always consult with your PH doctor first before taking any medications in this category.
Finally, remember that seasonal allergies alone should not cause shortness of breath. If you are noticing worsening breathing difficulty, especially if accompanied by worsening fluid retention (oedema) an/or light-headedness, these may be signs that your PH is worsening. If there is ever any question, you should always contact your PH doctor for guidance.
Answer provided by Jason S Fritz, MD - Assistant Professor of Clinical Medicine
Perelman School of Medicine at the University of Pennsylvania
Pulmonary, Allergy and Critical Care Division
Article first published in Pathlight Magazine Spring 2012 (PHA USA)
High Altitude and Pulmonary Hypertension
Overview - Increased altitude is associated with increased pulmonary artery pressures
Short term risks: variable blood pressure, increase in pulmonary hypertension, hypoxia (reduced oxygen supply), and arrhythmias (atrial fibrillation and other rapid heart rates) are possible.
Long term risks: progressive pulmonary hypertension and worsening hypoxia.
Supplemental O2: try to keep O2 saturation (amount of oxygen carried in your blood) above 90%. going over the high passes that are up to 3000 feet higher than the valleys in the mountains will likely cause more hypoxia and symptoms. Individuals will likely experience O2 desaturation with variable amounts of exercise at higher altitudes. Nocturnal O2 desaturation is common at higher altitudes above 6000 feet; the higher one sleeps, the more likely the need is for nocturnal (night time) O2.
1. Review with your doctor the advisability of going to high altitudes.
2. Monitor your O2 stats and try to keep it above 90%.
3. Limit your activities to very light effort the first few days.
4. Maintain good hydration and avoid alcohol as you acclimate to higher altitudes. You will still require fluid/sodium restrictions with PAH.
5. The higher you go the more your body is stressed by the thinner atmosphere.
6. The higher the degree of pulmonary hypertension and the higher you go the more symptoms and hypoxia you experience.
7. Other illnesses or conditions will also affect these changes with altitude (hypertension, heart failure, lung disease, atrial fibrillation, colds or flu).
8. If you are in a remote area and have problems it may take you a longer time to access assistance. Also, if you are trying to get to a lower altitude it may require you to first ascend a high pass before you can begin decent to a lower area.
See more on flying and PH by clicking here
Article courtesy of PHA USA Pathlight Magazine 2013
University of Colorado, Denver Medical Campus
Pulmonary hypertension is a rare disease that requires specialised medical care. Many local hospitals, physicians, nurses and emergency paramedics may not fully understand your condition. being educated and prepared will grant you peace of mind to worry less and live more.
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Create and Emergency Kit
Educate your Emergency responders
Order a Medic Alert Identifier
Preparing for Natural Disasters
Emergency Medical Services Brochure
Recommendations on Over the Counter Medications in Patients with PAH
When using non prescription, over the counter (OTC) medications, care should be taken as these agents may have a direct effect on the heart and lungs or may interact with medications commonly prescribed for patients with PAH.
Cold, flu, sinus, allergy, decongestant and headache medications frequently contain ingredients such as pseudoephedrine that have stimulant like properties. These medications cause vasoconstriction (narrowing or the blood vessels), and may worsen PH and increase blood pressure and heart rate. they may also cause palpitations and irregular heart rhythms. These medications may also contain significant doses of caffeine, which can have similar actions on the heart.
Therefore, all decongestants and medications that contain stimulants should be avoided in patients with PAH. They are marketed as tablets, caplets, liquid-gels, liquids or nasal sprays. As a general rule, if any ingredient is contraindicated in patients with high blood pressure, then it is contraindicated in patients with PAH. If there is uncertainty regarding the contents of specific formulations, ask a pharmacist for assistance. Medications that contain antihistamines (e.g., diphenhydramine, Benadryl, Claritin) may be used to treat cold symptoms, allergies, and hayfever, provided that they do not also contain decongestants.
Aspirin and medications classified as "non steroidal anti inflammatory agents" (e.g., Advil, Motrin, Naprosyn, etc.) may increase the risk of bleeding in patients taking blood thinners like Warfarin (Coumadin). These medications are typically found in analgesics (pain medications, this includes lotions and creams for inflammation), but may also be present in cold, allergy, and sinus medications. they should be used with caution and only for short periods of time. Large doses of acetaminophen (Tylenol) may cause liver damage, and may interact with blood thinners (Warfarin). Whether acetaminophen increases the potential for liver damage in patients taking Bosentan (Tracleer) or Ambristentan (Letairis) is unknown.
The use of herbal medications has become increasingly popular. Unfortunately, there is limited information on many of these products, and there are no established standards to regulate their production. As a general rule, a product marketed as "natural" should not be assumed to be safe. Like the OTC medications described previously, some herbal medications can affect heart and lung function, and interact with prescribed medications. The list of herbal medications is extensive, precluding a statement on each formula; instead, we will comment briefly on some of the more commonly used agents:
Ephedra (ma huang) contains ingredients with stimulant like properties. It should be avoided in patients with PAH. Ephedra, don quai, and St John's Wort may increase or decrease the actions of calcium channel blockers. St John's Wort and ginseng may affect digoxin concentrations.
Numerous agents affect the function of platelets (cells needed to clot the blood), which can increase the risk of bleeding in patients receiving blood thinners like Warfarin or Flolan (Epoprostenol). Examples of such compounds include garlic, ginkgo and ginseng. Some herbal medications can interact directly with Warfarin, either increasing (e.g., danshen, dong quai, papaya extract, vitamins A & E) or decreasing its effects (e.g., ginseng, St John's Wort, vitamin K).
Liver damage has been reported in patients using kava and Echinacea, and valerian may cause liver injury as well. Whether these compounds can increase the risk of developing liver damage in patients treated with Bosentan or Ambristentan is unknown. In summary, OTC medications and herbal therapies may be harmful, and should be used cautiously in patients with PAH. Herbal therapies should be viewed as drugs, not simply as "natural supplements." It is recommended that you discuss these drugs with your PH specialist prior to taking any of them.
Ask a PH Specialist Children and PH in the newborn
Question: What is PPHN (persistent pulmonary hypertension of the newborn)?Answer: PPHN is a condition in which the pulmonary vascular resistance (PVR) does not drop at birth; PVR is the resistance to flow which must be overcome to push blood through the lungs. The prevalence of this condition is estimated at one to two cases per 1,000 live births. It primarily occurs in babies born .34 weeks gestation, although prematurely born infants can also suffer from it. As part of normal fetal development, the PVR is very high to allow blood to preferentially flow to the placenta instead of to the lungs. This occurs because the lungs are not participating in gas exchange so the fetus relies on its motherfs placenta to enrich blood with oxygen. With the first few breaths at birth, the lungs open up, the blood vessels dilate, the PVR begins to drop dramatically and blood flow is now directed to the lungs as the sole source of oxygen. This transition occurs gradually in the course of the first few days to two weeks of life.
However, in certain situations, this decrease in PVR does not occur and PPHN results. PPHN is unique from other types of PH in that this is a developmental or adaptive problem which occurs in the newborn period. The mechanism of PPHN can be understood in two broad types: Underdevelopment. This is a condition of hypoplasia, or undergrowth. As a result, there are fewer blood vessels in the lungs, and the PVR is naturally higher to start. There is some ability of the blood vessels to widen after birth, but itfs limited. These infants have PH at birth which does not naturally improve.
Vascular constriction and maladaptation. The lungs and blood vessels are normally developed in these infants, but the blood vessels themselves are constricted or narrowed. Itfs thought that this occurs in response to abnormal levels of hormones in the body. This is typically seen as a reflection of birth events (delivery after 40 weeks gestation, meconium aspiration syndrome, infection such as Group B Streptococcus) or with congenital heart lesions, which the extra blood flow through the pulmonary circulation can stress the pulmonary blood vessels (premature closure of blood vessels, pulmonary venous abnormalities). The most common cause of PPHN is meconium aspiration syndrome.
Risk factors for PPHN are male sex, black or Asian maternal race, abnormalities in maternal body mass index (overweight [BMI > 27] or underweight [BMI < 20]), diabetes, asthma, cesarean section, late preterm gestational age, large for gestational age status (LGA) and infection. Prenatal exposure to medications such as selective serotonin reuptake inhibitors (SSRIs, commonly used for depression treatment) have also been implicated in PPHN development; the effect of non-steroidal anti-inflammatory medications such as ibuprofen on PPHN is controversial.
Infants with PPHN will usually present within the first 24 hours of life with trouble breathing and low oxygen saturation levels. As mentioned above, they may also have symptoms to suggest other associated conditions. Tests such as chest X-rays, bloodwork and echocardiograms will be done to evaluate the infants thoroughly. The echocardiogram is the test used to make the diagnosis of PPHN, and it will show signs of elevated right ventricular pressure and abnormal shunting of blood through persistent fetal circulation vessels.
Treatment is generally aimed at supporting the heart and lungs as the PVR may continue to fall slowly. This consists of oxygen, intubation/mechanical ventilation, antibiotics, intravenous fluids, sedation and blood pressure support medications. In more difficult cases, inhaled nitric oxide may be needed to assist with PVR reduction. The use of medications commonly used in PH (sildenafil, bosentan, prostacyclins) are occasionally considered in these settings. For severe cases of PPHN, extracorporeal membrane oxygenation (ECMO) is used to maintain oxygen delivery to the body until the PVR falls.
Infants who survive PPHN will need to be followed by pediatric subspecialists such as pulmonologists and cardiologists. Many of the infants who survive PPHN will remain on some PH therapy upon discharge from the neonatal intensive care unit and will need to be managed by a PH specialist. Other specialists who may need to see PPHN survivors are developmental specialists and neurologists as there is an increased risk of developmental delays.
Answer provided by Nidhy Paulose Varghese, MD, Assistant Professor of Pediatrics, Division of Pulmonary Medicine Baylor, College of Medicine, Texas Children's Hospital
For more information on Children and PH click here
Pulmonary hypertension (PH) is a disease characterized by high blood pressure in the lungs that affects the vessels responsible for transporting blood from the heart to the lungs, which are called pulmonary arteries. It is a rare, life-threatening disease, with a higher incidence among women and older people.
In addition to experiencing symptoms such as shortness of breath, fatigue, dizziness, chest pressure or pain, swelling in the ankles, legs and abdomen, bluish color of the lips and skin and irregular heartbeat, patients are also more predisposed to the development of other medical conditions.
Want to learn more about 11 pulmonary hypertension related diseases?
Among these diseases is pneumonia. Pneumonia is the cause of death in patients who suffer from pulmonary hypertension in seven percent of cases since the lungs cannot tolerate pulmonary infections.
But how do pulmonary hypertension and pneumonia relate?
Patients diagnosed with pulmonary hypertension have their heart and lungs compromised and are more susceptible to the development of additional lung disorders. This is why patients are advised to get a pneumococcal pneumonia vaccine and yearly flu vaccines to avoid suffering from pneumonia.
Interested in learning more about PH and pneumonia? Discover more here.