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Management of Idiopathic Pulmonary Fibrosis

Source: pharmacypracticenews

Idiopathic pulmonary fibrosis (IPF) is a progressive, debilitating, and often fatal interstitial lung disease characterized by scarring of the lungs with unknown etiology.1 This scarring causes the alveolar walls to become stiff and thick, preventing inflation, which reduces lung capacity and makes the exchange of oxygen and carbon dioxide more difficult. The decreased lung capacity and oxygen exchange increases breathlessness and decreases mobility and independence.2


Although the precise cause of IPF is unknown, there is evidence that risk factors including age and environmental exposures play a role. The disease tends to affect adults in their sixth or seventh decade of life.3 In addition, there is a strong correlation between smoking and IPF, as well as evidence of a detrimental effect of smoking on survival.4 Environmental exposures to certain types of manufacturing-related dust and genetic factors also are contributors.5

Prognosis and Disease Course

Common symptoms of IPF include dry cough, shortness of breath, fatigue, chest pain, and unintentional weight loss.2 Because these symptoms are nonspecific and are associated with other diseases, such as asthma, chronic obstructive pulmonary disease, emphysema, and heart disease, the diagnosis of IPF often is delayed.1 Thus, significant declines in lung function can occur by the time a patient is correctly diagnosed. It is imperative to start patients on FDA-approved therapies as soon as possible to preserve lung function, reduce the risk for acute exacerbations, and improve outcomes.1

The prognosis of patients with IPF is poor; median survival duration is less than 4 years.1 The clinical course of IPF can vary by patient, with some patients experiencing a slowly progressive course and others rapidly progressing. At diagnosis, there are no distinguishing clinical characteristics to indicate the clinical course a patient is likely to experience. Acute exacerbations with clinically significant worsening of lung function and/or dyspnea can occur at any time during the course of the disease and are associated with a high risk for mortality.1


Historically, the only treatment options available to patients with IPF were focused on symptom relief and not slowing the disease course. In 2014, the FDA approved 2 oral antifibrotic therapies for IPF: nintedanib (Ofev, Boehringer Ingelheim) and pirfenidone (Esbriet, Genentech) (Table).3 Current guidelines from respiratory societies recommend antifibrotic agents as first-line therapy for patients with IPF.6

In addition, patients can benefit from a holistic approach to care that includes pulmonary rehabilitation, education and support, vaccinations, and supplemental oxygen.2,7 Although there is no cure for IPF, medications combined with lifestyle changes can help to slow the decline in lung function, decrease respiratory symptoms, and reduce acute exacerbations.1

Table. Antifibrotic Agents Approved to Treat Idiopathic Pulmonary Fibrosis
MedicationDoseCommon AEsWarnings/
Counseling Notes
Nintedanib (Ofev, Boehringer Ingelheim)Recommended dose: 150 mg twice daily every 12 h with food Mild hepatic impairment (Child-Pugh A): 100 mg twice daily every 12 h with foodDiarrhea Nausea Stomach pain Decreased appetite Headache Weight loss High blood pressureHepatic impairment Elevated liver enzymes GI disorders Embryo-fetal toxicity Arterial thromboembolic events Bleeding events GI perforationIf AEs occur, consider a temporary dose reduction to 100 mg twice daily Pregnancy category D
Pirfenidone (Esbriet, Genentech)Titration dose: Days 1-7: 267 mg 3 times daily (801 mg/d) Days 8-14: 534 mg 3 times daily (1,602 mg/d) Days 15 and beyond: 801 mg 3 times daily (2,403 mg/d)Nausea Rash Abdominal pain Upper RTI Diarrhea FatigueElevated liver enzymes Photosensitivity reaction or rash GI disordersPatients who miss =14 d should restart with 2-wk titration regimen Take with food Pregnancy category C
AEs, adverse events; GI, gastrointestinal; RTI, respiratory tract infectionBased on references 8 and 9.

Antifibrotic Agents


Nintedanib is a tyrosine kinase inhibitor that targets multiple growth receptors involved in the pathogenesis of fibrotic tissue remodeling in interstitial lung disease. The recommended dose is 150 mg orally twice daily with food.8 A dose reduction to 100 mg orally twice daily should be considered for patients with mild hepatic impairment or for the management of adverse events (AEs).8 The most common AEs are diarrhea, nausea, and vomiting. The nintedanib labeling includes warnings and precautions related to hepatic impairment, elevated liver enzymes, gastrointestinal disorders, embryo-fetal toxicity, arterial thromboembolic events, and bleeding events.8

In clinical trials, nintedanib resulted in statistically significant reductions in the annual rate of lung function decline, as measured by forced vital capacity (FVC), preserving lung function, and reduced the risk for acute exacerbations in the first year of therapy.8


Pirfenidone is an antifibrotic medication that is thought to have multiple effects. It has been shown to regulate important profibrotic and pro-inflammatory cytokine cascades in vitro and to reduce fibroblast proliferation and collagen synthesis in animal models.9 The recommended maintenance dose of pirfenidone is 801 mg 3 times daily taken with food, but patients will titrate to the full dosage over a 14-day period.9 The most common AEs are nausea, rash, and abdominal pain. The labeling for pirfenidone includes warnings and precautions about elevated liver enzymes, photosensitivity, rash, and gastrointestinal disorders.9

In clinical trials, pirfenidone was shown to significantly reduce the risk for lung function decline as measured by FVC and to preserve lung function.9

Role of the Specialty Pharmacist

At AllianceRx Walgreens Prime, due to the complex nature of IPF, specialty pharmacists play a critical role educating, counseling and monitoring patients. Educating patients about the disease and the importance of receiving all recommended vaccinations is essential.1 Specialty pharmacists can provide counseling and support related to healthy lifestyle changes, such as smoking cessation, improved nutrition, and increased exercise.1,59 Because the diagnosis of IPF often is delayed due to misdiagnosis, patients likely will have had declining lung function for quite some time. Thus, it is crucial for pharmacists to educate patients about the importance of starting antifibrotic therapy as soon as possible to preserve lung function, slow disease progression, and improve outcomes.1 In addition, specialty pharmacists can educate patients and monitor for AEs associated with antifibrotic therapies. Both medications approved for IPF have warnings and precautions associated with their use. Pharmacists can counsel patients about signs and symptoms to be aware of and when to seek medical attention. Pharmacists also can help ensure patients keep their appointments for laboratory monitoring. By supporting IPF patients in these ways, specialty pharmacists can help optimize outcomes for this population.

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Keeping pets protected during heatwaves this summer

We’ve been lucky to have been hit with some great weather this summer, however, some days have been exceptionally hot and causing us discomfort to the point where we don’t want to leave the house! Our pets can also struggle in this heat and if not looked after correctly, can lead to life threatening issues for them. 

Dr. Sarah Machell, Medical Director for Vetster, has shared her top five top tips to ensure that our pets can enjoy the summer days as much as we can.

Vetster, is a digital platform that connects licensed vets with pet owners virtually, with 24/7 online appointments, launches in the UK this summer.

1.  Provide adequate rest, shade, and ventilation

Coping with high temperatures and humidity is tough enough on its own, but it’s even more difficult for pets who are exercising in direct sunlight and don’t have adequate ventilation. Limit outdoor activities to early mornings and late evenings when it’s cooler. When walking your pet, choose shady routes off the pavement.  Ensure outdoor pets have shady, ventilated places to escape the heat.  Keep in mind that pets also rely on evaporation for cooling, regardless of if they sweat like horses or pant like dogs, and high humidity decreases the effect of evaporation. This means your pet needs you to keep an eye on the heat index for them as well as for you.  Be sure your pets have easy access to a steady supply of clean water. Pets are naturally wired to stay hydrated as long as they are healthy and avoid heavy exercise in the heat, so there’s no need to try to encourage your pet to drink more.  Just make sure the water supply is in easy reach and doesn’t run dry. 

 2. Be wary of paws on the hot pavement! 

When the weather gets extremely hot, so does the pavement—asphalt, in particular. If you’re taking your dog out for a walk, try to remember that they don’t have shoes to protect against the heat. Even though paw pads are extremely tough, hot surfaces can burn them. Consider using padded booties for their paws to create a barrier between paw pads and the hot concrete.  Better yet, try to steer clear of the streets and walk on the cool grass instead. If there’s an opportunity to wade through some clean water or catch the spray from a sprinkler, that’s even better. Remember that pavement retains heat and you still need to be aware of the risks when you go for walks in the evening.

3. Look out for signs of heatstroke 

Heatstroke is a very serious condition and one to look out for in your pets. As a pet parent, it’s important to be aware of your pet’s fitness level and avoid overexertion when the weather’s too hot or humid. Less athletic dogs, dogs with underlying illnesses, and brachycephalic breeds are at higher risk for developing heatstroke, so keep an extra close eye on them. Heatstroke is life-threatening, but it can be avoided if you take action to cool your pet when they show early signs of heat stress. If your dog doesn’t want to keep walking, lies down in the shade, or digs up cool dirt to lie in, those are clues they’re getting too hot.  Excessive panting that doesn’t improve after a short rest is another indicator. Get out of the heat and offer water to keep the threat of heatstroke from escalating.  Splash down hot ears, paws, and bellies with water to achieve more rapid cooling.

4 Never leave your pet unattended in a hot vehicle 

Heatstroke can happen in the blink of an eye—it cannot be stressed enough that you should never, ever leave your pet unattended in a vehicle. This is true even if you leave the windows down for fresh air or if you think you’ll only be gone for a few minutes. Studies show that even if the outdoor temperature is 72℉ (22°C), a car can rise up to 117℉ (47°C) in only an hour. Imagine how quickly a car can become dangerously hot when outdoor temperatures are a balmy 86℉ (30°C). Even if you’re leaving your car unattended for a minute or think that leaving a window open will help – the life-or-death gamble you’re taking isn’t worth it.

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Why dosage matters in menopause treatment

By Rizvan Faruk Batha MPharm, PGDip GPP, IPresc, MRPharmS, Superintendent Pharmacist of Specialist Pharmacy.

Bioidentical Hormone Restoration Therapy (BHRT) is an alternative option to traditional synthetic HRT, using bioidentical hormones to treat hormonal conditions in both men and women. BHRT is compounded medicine and put simply, means that the individualised ingredients are mixed together under the direction of a qualified prescriber’s prescription to meet the tailored needs of a patient.

For women going through menopause, compounding treatments are often prescribed later down the line, when a woman has been unable to settle on the appropriate dose of HRT with a General Practitioner, often experiencing severe side effects from either too much or too little HRT. In short, compounded menopause medication offers another route for those patients where the licensed preparation is not appropriate or hasn’t worked.

Patient-led care plays an important role in compounded menopause medicine because the patient is involved throughout the process; during the consultation with the prescribing practitioner, and with the pharmacist developing the customised dosage.

One of the biggest challenges faced by compounding pharmacies is the drugs being classified as unlicensed. For drugs to be licensed, it involves research and clinical trials to assess the efficacy, quality, and safety of the medicines, and because of this process, more often than not it is the recommended route to prescribe licensed medicines. That being said, prescribing unlicensed compounded menopause medicines may be necessary, especially when it comes to the patient’s specific need and interests and where licensed medicines have been unable to satisfactorily meet the needs of the patient or are unsuitable for them.

Utilising compounded menopause medicine as a method to enhance patient care could be beneficial for the many patients that need specific dosing or formulation requirements, but sadly more often than not we see delays to patient needs and treatment, as the rise of mass manufactured licensed medicines have grown in popularity. Compounding menopause medicine could offer huge potential for many, but prescribing practices have moved towards evidence-based medicines because of the responsibility imposed on prescribers for prescribing compounded therapies. Clinicians need to understand that even licensed products are not safe or effective for all patients particularly if the product is being used in a population that were not part of the original clinical trials for the drug.

It is important to know that compounding pharmacies and pharmacists in the UK are also regulated and licensed by the General Pharmaceutical Council (GPhC). The GPhC set standards for pharmacists and pharmacies to meet to remain on their register with the aim to protect the public and give them assurance that they will receive safe and effective care when using pharmacy services.

So even though compounded medicines are ‘unlicensed’, there is a lot of due diligence exercised by the pharmacists to ensure the products meet the safe and effective care criteria. This is generally demonstrated through the purchase of medical grade active and inactive pharmaceutical ingredients (with certificates of analysis and safety data sheets), trained staff, following and updating SOPs, audits, traceability of ingredients and products during recalls, as well as continuous learning and error reporting being supported in the pharmacy.

Ultimately, how compounded menopause medicine is viewed will depend on the knowledge and experience of the patients and professionals involved. Although the medicine is unlicensed it is important to understand that experienced clinicians and pharmacists involved in the process of making the decisions are regulated, and patients are consistently monitored during their response to the medicine. If compounded menopause medicine was integrated in the healthcare system, it could change and improve the quality of life of many patients suffering with debilitating menopause symptoms, and we hope as a pharmacy that in the future we will see a shift in how compounded medicine is viewed.

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We need more than investment in services to solve the mental health crisis.

By Lea Milligan

It’s now widely recognised that there is a crisis in mental health. In September, a staggering 1.6m people in the UK are waiting for mental health treatment on the NHS, and 1 in 4 people globally are impacted by a mental illness at some point in their life.

This crisis is only being exacerbated by the increasing cost of living, global conflicts causing generational trauma and the ongoing fallout from COVID-19.

Lack of equitable access to services, treatment and resources means the most vulnerable in society continue to suffer disproportionately.

Everyone assumes that addressing this crisis requires more doctors, more appointments and more mental health treatment centres. Whilst one answer is, yes, we absolutely need more of these, it’s not the only answer.

More services are not the sole solution: We also need more research.

Mental health research is the secret weapon that often is overlooked by policy makers.

  • Research shows us where to focus resources, so that we don’t just blindly throw money and time at the parts of the system that are not working.
  • Research helps move people through the system faster, giving them more effective treatments and faster diagnoses.
  • Research reduces the number of people requiring acute care by providing effective prevention strategies and intervention methods.

Research speeds up access to services:

The EnCAMHS project, sponsored by Greater Manchester Mental Health NHS Foundation Trust, is working to improve and refine the referral process to CAMHS (Child and Adolescent Mental Health Services). By mapping and raising awareness among professionals of the different types of support available for children and young people, the EnCAMHS team are hoping to reduce waiting lists and speed up access to care for the most urgent cases.

Research improves treatments:

I have seen firsthand the incredible breakthroughs in treatments that have come from targeted investment in research.

MQ researcher Dr Colette Hirsch from King’s College London developed a new treatment for anxiety and depression called cognitive bias modification for interpretation (CBT-I). New treatments such as this help provide a greater range of treatment choices, more important now than ever because, according to the World Health organisation, the pandemic triggered a 25% increase in anxiety and depression worldwide.

Dr Jennifer Wild from Oxford University has developed a new treatment for PTSD in health care workers, with a 90% recovery rate. This is an unprecedented success rate for a group highly vulnerable to traumatic stress.

And Dr Ethel Mpungu has developed a community-based therapy for people living with both depression and AIDS in rural Uganda. The 578 participants of her study remained symptom free from depression 12 months later. Her work has been internationally recognised and, due to its success and cost-effectiveness, it is being rolled out across other African nations, improving the lives of millions of people.

Research can help us prevent mental ill-health in the first place:

For example, the IDEA (Identifying Depression in Early Adolescence) project has created a prediction model to identify the young people who are most at risk of developing depression in later life. This tool can be used to identify the most vulnerable before depression can take hold, getting them timely interventions that can prevent a lifetime of mental ill-health.

This Mental Health Awareness Week (9-15 May), plenty of people will be talking about smashing stigmas, reducing loneliness and the need for more mental health support. And, of course, these are absolutely important. Vital even. But don’t forget about research.

Because it is only through research that we can truly make progress.

Lea Milligan is CEO of MQ Mental Health Research, a charity that funds world-class research and innovation to create better mental health care.

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