Health and other forms of smoking

The fight against smoking continues. The industry is reinventing itself and creating new products that, despite their harmless appearance, are as harmful or even more so than conventional cigarettes.

Other forms of smoking

Francisco Casas, a pulmonologist at the San Cecilio Clinic, warns of this new epidemic of young faces and is very concerned about the general lack of knowledge surrounding vapers, hookahs and electronic cigarettes.

The number of young people who smoke has dropped by a third since the beginning of this century, and yet we cannot be happy. Why?

Because, although the prevalence of conventional tobacco consumption has been reduced, the consumption of other emerging forms of smoking has been increasing and now exceeds that of conventional tobacco among adolescents.

19.2% of young people between 14 and 18 years old smoke or consume some tobacco derivative. But vapers are the preferred choice for one in five.

Followed by hookahs, which are used by 14.3%, while 13.1% opt for conventional cigarettes.

It was thought that electronic cigarettes or vapers could be a less harmful substitute than tobacco, but this is not the case.

E-cigarettes are not harmless for active or passive smokers.

E-cigarette and vape smoke contain the same harmful constituents as conventional cigarettes: volatile organic compounds, hydrocarbons, heavy metals and may contain nicotine.

And they don’t help you quit smoking either?

They do not help you quit smoking or consume less cigarettes. In addition, these devices are the gateway to nicotine addiction.

What happens to our lungs when we vape?

In adolescents, lung development is slowed down, which usually forms in girls until the age of 18 and in boys until the age of 22.

In addition, it causes irritation and inflammation of the respiratory mucosa: chronic bronchitis, asthma, bronchiolitis and interstitial lung diseases.

Moreover, it destroys the alveoli and produces cellular mutations with the development of lung cancer.

Is it necessary to have a very strong habit to cause damage to the body?

As with conventional tobacco consumption, the number of cigarettes smoked daily and the number of years of consumption are factors involved in greater damage to the body.

However, there are factors linked to each individual that can make them more susceptible, such as genetic factors for lung cancer or asthma or chronic obstructive pulmonary disease.

They also have a very strong social component. A clear example is hookahs, which have become widespread in nightlife… Are they safe?

Not at all, they are even more harmful than conventional tobacco.

Is it true that a hookah session is equivalent to inhaling 200 times the smoke of a cigarette?

A single puff is almost equal to the volume of smoke inhaled by an entire cigarette.

The volume of smoke inhaled during a typical hookah session is approximately 90 liters, which is equivalent to 200 puffs, compared to the 0.5 liters inhaled when smoking a cigarette.

And yet there is more social permissiveness. Why?

Much of the blame for this permissiveness lies with society’s ignorance, and in particular, that of parents and educators. Sometimes we are carried away by the tobacco industry’s messages about its safety and supposedly lower addictive capacity.

After 30 years of prevention campaigns or restrictive laws, tobacco consumption has been reduced.

How many years do you think it will take now to minimize the impact of these new harmful habits?

I couldn’t tell you how many, certainly many, more than ten years and everything will depend on the messages that are transmitted to the general population and to young people in particular through the media and social networks.

It will also depend on the measures taken by public administrations to equate the consumption of these products with conventional tobacco, establishing the same prohibitions on their consumption by minors and for smoke-free spaces.

The active fight against the tobacco industry and its false messages is another of the battles.

Finally, tell a young person reading this what benefits they might notice when they quit smoking.

The first and most immediate thing you will notice is the decrease or disappearance of your cough; you will have a greater ability to practice sports and your oral health will improve.

In addition, tobacco accelerates cellular aging, specifically of the skin, and promotes hair loss, which will improve. Getting rid of nicotine addiction is another great advantage of quitting.

Source: Centro Andaluz Alfa 1, Ideal Granada

La entrada Other forms of smoking se publicó primero en Centro Andaluz Alfa-1.

On the occasion of the celebration of liver cancer day and to give it greater visibility, as well as raise awareness among the population about the knowledge of this disease in relation to others, we bring you this news.

Relationship between the liver and AATD

AATD is the most common hereditary disease in Spain and in its most severe forms, it can cause damage to the lungs and liver.

Not all people with AAT deficiency suffer from liver problems, since in many cases it is mild and the patient does not even know they have it.

Alpha-1 Antitrypsin is a protein produced in the liver and its main function is to protect the lung from degradation and inflammation caused by infections or external agents.

When DAAT exists, this protein remains stagnant in the liver, which produces two consequences: it damages this organ and, by not passing into the bloodstream, it does not reach the lungs and leaves them unprotected.

The most common disease is cirrhosis, where severe scarring occurs in the liver; this can appear even in children and newborns due to ATT deficiency.

Diseases related to DAAT and life cycle

The development of hepatocellular carcinoma has been described in 2-3% of elderly ZZ individuals, or Wegener’s disease in 2-3%, and neutrophilic panniculitis.

This corresponds to 1 in 100 Pi*ZZ in the UK registry and 1 in 1,000 in the American registry.

Probability of incidence

Severe alpha 1-antitrypsin (AAT) deficiency is a complex monogenic disorder, with great variability in its clinical presentation, percentage of affected individuals and age of onset of diseases such as COPD.

Consequences of alpha 1 antitrypsin deficiency in the liver

Hepatic disease in ZZ homozygous individuals is of variable severity and is due to the formation of protein polymers, which cannot be excreted by the hepatocyte and accumulate inside it (90% in the Z mutation).

Up to 70% of ZZ newborns may present abnormal liver function tests, but only 10% develop prolonged neonatal colostasis and 2.5% of them develop childhood liver cirrhosis.

Consequences of alpha 1 antitrypsin deficiency in the liver

In adults, the risk of liver cirrhosis depends on sex and age, being more pronounced in ZZ men over 50 years of age (20-40%) who, in addition, have a higher risk of hepatocellular carcinoma, both in cirrhotic and non-cirrhotic livers. cirrhotics, and is independent of their history of hepatitis B (HBV) or C (HCV) virus infection.

Liver cirrhosis

Liver diseases linked to Alpha-11 Antitrypsin Deficiency are:

1. Chronic hepatitis
2. Cirrhosis
3. Liver carcinoma.

It is not yet known why some individuals with Alpha-1 Antitrypsin Deficiency develop liver damage and others do not.

Liver lesions are related to the retention and polymerization of AAT in the liver in those affected with the PiZ allele; Furthermore, it usually occurs in childhood.

It is diagnosed when there is liver dysfunction that cannot be attributed to any other cause.

INCIDENCE IN CHILDREN AND ADOLESCENTS

Frequency

The good news is that in most cases these alterations are stable during childhood and adolescence, without becoming serious. 

Only in certain cases can progressive liver damage occur that requires transplantation in children.

It is estimated that between 10-15% of children with the two defective ZZ genes develop clinical liver disease before the age of 20 years. 

Liver disease can also occur in adults and become severe in middle age.

Symptoms of Alpha 1 Antitrypsin Deficiency

Adult subjects with severe AATD usually present respiratory symptoms, but with early onset, starting at age 35 in smokers and after age 45 in non-smokers.

1. The most common symptom is dyspnea on exertion, which appears in 70-90% of patients.
2. Other common symptoms are cough (42%) and chronic expectoration (46%), related to the presence of bronchiectasis (23% of ZZ individuals).
3. Wheezing can be persistent or appear during exacerbations in up to 70-80% of cases.
4. Regarding the physical examination, there is no data that is specific to DAAT.

Recomendaciones

To finish, we only advise you on some simple guidelines such as quitting smoking, exercising daily, following a balanced diet, avoiding processed foods; and finally carry out periodic monitoring by medical experts.

Sources: Centro Andaluz Alfa 1, Alfa 1 España

La entrada Liver and AATD se publicó primero en Centro Andaluz Alfa-1.

For plasma donation awareness week, we have compiled some information that we think you might find interesting.

History

In 1994, a team of surgeons applied autologous plasma to the spongy tissue of a mandibular bone.
Autologous platelet-rich plasma (PRP) is a possible treatment for wound healing because it has components such as fibrin and high concentrations of growth factors (PRGF) that help in healing and bone regeneration.

Two years later, scientists Mark and Cols concluded that PRP increased the concentration of platelets (cells responsible for healing wounds) in the tissues, and regenerated the cells due to its enormous reserves of bioactive proteins.

As a result, the bone was able to consolidate much more quickly than with other treatments.

What is?

It is a liquid component of the blood that contains valuable information about the entire organism.

We could say that it is a biological treasure that transports essential elements, including blood cells, nutrients and, especially, proteins, which it distributes to all organs.

This unique vital liquid can potentially be the basis for the treatment of many diseases and, therefore, be key to improving people’s quality of life.

There are five types of cells in the blood:

1. Red blood cells
2. White blood cells
3. Platelets
4. Organic compounds
5. Electrolytes

What is plasma made of?

Plasma is a yellowish, almost transparent component of blood.

• Represents 55% of the total volume of blood.
• Water makes up 90% of the volume of plasma.
• Proteins represent 9% of the total plasma and are responsible for ensuring the proper functioning of our body.
• 1% are nutrients and gases.

Plasma proteins

The proteins found in plasma perform an important function in our system, and we mainly find:

1. Albumin: transports hormones and drugs and takes them to the tissues.
2. Coagulation factors: responsible, together with platelets, for stopping bleeding when we get a wound.
3. Immunoglobulins: serve to defend us from infections.

What is plasma used for?

In most cases, it is used to treat people who have suffered severe burns, or those who need a transfusion due to a serious accident or illness.

In general, it is a great discovery that saves the lives of thousands of people every day, and it is especially used for people with alpha-1 antitrypsin deficiency (AATD), immunoglobulin deficiency, autoimmune diseases and other rare diseases.

To treat these people, plasma is basically transfused from healthy people to patients to improve the immune response or the blood clotting capacity. To treat a patient with AATD for one year, between 130 and 1,200 donations are required.

DONATE PLASMA, SAVE LIVES

How much plasma does a person have?

Normally, a person weighing 70 kilos has about 3 liters of plasma.

Donation

Donation consists of extracting blood, separating the blood cells using a cell separator, retaining the plasma and returning the rest to the donor by the same means.

It is frozen at -40º until it is transfused to patients or manufactured as drugs.

How long does it take? About 45 minutes.

How often can you donate?

As it is mostly water, the donor’s recovery is very fast and donations can be more frequent.

You can donate every 15 days.

Although if you have given blood before, you must always wait a month for your next donation.

To donate plasma, the same requirements must be met as to be a blood donor:

1. Be between 18 and 70 years old
2. Weigh more than 50 kilos
3. Be in good health.
4. If you are a woman, you must not be pregnant.

What type of blood is most suitable?

All blood types are valid, although group AB is the universal plasma donor, compatible with all recipients.

We need everyone’s collaboration, it doesn’t cost anything, but you will get a huge reward for helping so many people improve their quality of life.

Source: Centro Andaluz Alfa 1

La entrada Plasma se publicó primero en Centro Andaluz Alfa-1.

Advances in the treatment of alpha-1 antitrypsin deficiency and future therapies

In June 2024, the meeting of the Spanish Network for Research on Alpha-1 Antitrypsin Deficiency (REDAAT) took place, during the 57th SEPAR Congress that took place in Valencia from June 6 to 8.

THE CURE IS THE FUTURE

Review of new strategies for future AATD therapies

On this occasion, Francisco Casas presented during the REDAAT meeting the “Advances in the treatment of DAAT and future therapies”.

During his presentation he reviewed the new strategies for the treatment of AATD, such as:

1. Neutrophil elastase inhibitors
2. Increasing exogenous AAT (existing IV replacement therapy with human AAT, advances in replacement therapy with human AAT and recombinant AAT (rAAT) by IV or inhaled route)
3. Increasing endogenous AAT (liver transplant and increasing AAT release by the hepatocyte)
4. Gene therapy
5. Treatment of liver disease.
6. Other strategies such as reducing lung inflammation with antioxidants or hyaluronic acid, and promoting alveolar regeneration with all-trans retinoic acid have failed.

Neutrophil elastase inhibitors (NE)

They offer an alternative to AAT augmentation therapy that does not rely on intravenous or nebulized routes.

One such drug is alvelestat (Mereo), which has recently completed a phase 2/3 trial, known as ASTRAEUS, of reducing the biomarkers desmosine and Aα-val360, in urine.

Another NE inhibitor, PHP-303 (pH Pharma) has completed phase 1 trials, with a promising safety profile.

AAT Increase

It is based on the derivation of human plasma donations.

It is an effective and safe treatment, but it depends on human donors, it is expensive and its administration is intravenous in a day hospital, and requires about 2 hours for its infusion.

The synthetic production of AAT or NE inhibitors could reduce this limitation; in addition, the treatment until now has been intravenous, but only a minority of the infused AAT reaches the lung.

On the other hand, an inhaled route can improve efficiency, while its administration is less invasive to administer. In addition, dosing escalation strategies vary in the literature, with equivocal evidence between regimens [5,14,17].

Finally, this therapy only addresses lung disease, that is, approaches aimed at treating liver disease are required if we want to treat all patients adequately.

The current status of drug development for AATD is summarized in the following table.

SPARTA Trial

The SPARTA trial of 60 mg/kg versus 120 mg/kg human AAT is awaiting results, which are anticipated to be available in 2026.

Another novel strategy is the administration of human AAT subcutaneously (ClinicalTrials.gov Identifier: NCT04722887), which will allow for home administration via infusion pump.

Given the limited availability of human AAT, research is underway to synthesize functional AAT, called recombinant AAT (rAAT).

This also increases the potential to create a maximally efficient AAT protein.

INBRX-101 (Inhibrx) is a rAAT Fc fusion protein that has completed Phase I trials showing normal serum levels of AAT in 24 patients with AATD, with an acceptable safety profile.

Inhaled AAT

Eliminates the need for intravenous access and allows for administration directly to the site of interest, which is the lung.

Less drug is needed to achieve the same lung tissue concentration.

An inhaled human AAT product (KAMADA-AAT, Kamada), has completed Phase 2 trials.

Results included significantly higher functional AAT levels in epithelial lining fluid (ELF) at doses of 80 mg and 160 mg.

Corrective drugs

AAT-correcting drugs aim to change the shape of the misfolded dysfunctional AAT protein, thereby improving its function.

In addition, they also prevent the polymerization of AAT in the liver, which is the main cause of AATD liver disease.

Trials of these drugs are in the early stages [33–35]. VX-864 (Vertex) showed a significant improvement in functional AAT levels versus placebo.

(Mean difference +2.2 to +2.3 μmol/L across 3 doses, p < 0.0001 in all 3 cases) in a phase 2 trial of 44 participants.

ADVANCE Research

Gene therapy has the potential to act by altering DNA or RNA to produce functional AAT instead of misfolded proteins.

The ADVANCE study is a phase 1/2 trial of ADVM-043 (Adverum), which uses a viral gene transfer vector to deliver a functional gene to the liver of 6 patients.

Functional human AAT is thus expressed, but serious adverse events have been reported.

Nucleotide editing therapies

The development of nucleotide editing therapies is a very active area: BEAM-302 (Beam) is described as a “liver-targeted lipid nanoparticle formulation of base editing reagents.

It has reportedly been able to correct alterations in the SERPINA1 gene in mouse models, with increases in serum AAT and a reduction in liver polymers.

Similarly, Intellia has developed NTLA-3001, a CRISPR-mediated targeted gene editing therapy.

Wave Life Sciences and GSK have collaborated to develop WVE-006, an RNA editing oligonucleotide that offers to correct single nucleotide mutation at the mRNA stage.

Fazirsiran (Takeda)

It is described as an RNA interference therapy, which causes degradation of AAT RNA, thus reducing AAT production from hepatocytes, polymerization and subsequent liver disease.

In this case, in the phase 2 trials, liver biopsies were taken from 15 patients with PiZZ AATD and liver disease at baseline and at 12 weeks, with a median reduction of -83.3% in AAT.

Serum liver enzymes were also reduced in all patients, and fibrosis regression was observed in the majority. The safety profile is good.

Ongoing trials

Phase 3 trials in larger samples are ongoing.

Another RNA interference therapy, belcisiran (Dicerna), is also in phase 2 trials.

Future therapeutic therapies AATD

En la siguiente figura se resumen las diferentes dianas terapéuticas en la DAAT.

Key points

1. Currently, IV AAT therapy remains the only disease-modifying and well-tolerated pharmacological intervention available for AATD.
2. In the short term, the use of chaperones could be a promising treatment, as well as new molecules that interfere with RNA, inhibitors of AAT or neutrophil elastase, and inhaled AAT that may improve efficiency while being easier to administer.
3. In the long term, there is great potential for gene therapy:
   • The use of dual-function vectors that inhibit Z-AAT gene expression and promote M-AAT biological activity is a viable approach for the correction of pulmonary and hepatic manifestations related to AATD.
   • Co-administration of AAT viral vectors and capsid-specific Treg cells is a way to avoid immune responses, improving their efficiency and reducing adverse events resulting from their administration.
   • Gene editing strategies, including the use of RNA silencers and CRISPR-Cas9, are very promising for the correction of specific mutations in the SERPINA1 gene.
4. Strategies to recreate whole organs for transplantation, or repopulation with hepatocytes expressing corrected AAT would be very useful, as they would allow simultaneous treatment of lung and liver disease associated with AATD.
5. Homogeneous and robust evaluation criteria for all these therapies are needed, which assess the relationship between the biological activity of AAT:
   • Rate of progression of lung parenchymal and extracellular matrix degradation.
   • Inflammatory state of the lung.
   • Clinical, functional and imaging efficacy.
6. It is necessary to identify new biomarkers that evaluate the levels of disease progression and response to AATD treatments.

Reference sources

1. McElvaney NG, Burdon J, Holmes M, et al. Long-term efficacy and safety of alpha1 proteinase inhibitor treatment for emphysema caused by severe alpha1 antitrypsin deficiency: an open-label extension trial (RAPID-OLE). Lancet Respir Med. 2017 Jan;5 (1):51–60. doi: 10.1016/S2213-2600(16)30430-1
2. De Soyza J, Pye A, Turner AM. Are clinical trials into emerging drugs for the treatment of alpha-1 antitrypsin deficiency providing promising results? Expert Opin Emerg Drugs. 2023 Dec;28(4):227-231. doi: 10.1080/14728214.2023.2296088.
3. Campos MA, Kueppers F, Stocks JM, et al. Safety and pharmacokinetics of 120 mg/kg versus 60 mg/kg weekly intravenous infusions of alpha-1 proteinase inhibitor in alpha-1 antitrypsin deficiency: a multicenter, randomized, double-blind, crossover study (SPARK). COPD: J Chronic Obstructive Pulmonary Dis. 2013 Dec;10 (6):687–695.
4. LLC GT. A study to evaluate safety, tolerability and pharmacokinetics of two different doses of Alpha1-proteinase inhibitor subcutaneous (human) 15% in participants with Alpha1-antitrypsin deficiency. 2021. Available from: https://classic.clinicaltrials.gov/ show/NCT04722887
5. Miravitlles M, Dirksen A, Ferrarotti I, et al. European Respiratory Society statement: diagnosis and treatment of pulmonary disease in α 1 -antitrypsin deficiency. Eur Respir J. 2017 Nov;50(5):1700610.
6. Sandhaus RA, Turino G, Brantly ML, et al. The diagnosis and management of alpha-1 antitrypsin deficiency in the adult. Chronic Obstr Pulm Dis. 2016 Jun 6;3(3):668–682. doi: 10.15326/jcopdf.3.3.2015.0182

Source: Centro Andaluz Alfa 1

La entrada Future AATD therapies se publicó primero en Centro Andaluz Alfa-1.

History of nursing in Spain

In 1977, the specialties law separating Pulmonology from Cardiology was approved in Spain.

Since then, both specialties have been established separately, making great advances in medical research.
Nursing, in Spain, has been practiced as such since 1857 when the practitioner’s career could be studied as a regulated free education.

In 1924, the National Health School was founded, and its purpose was to train doctors and create a health nursing body that would act as auxiliary personnel, Health Technical Assistants, known as ATS.

Already in 1952 the laws were reformed and the titles of Practitioners, Midwives and Nurses were unified.

In this way, it gave rise to the recent title of Diploma in Nursing, with the recognition of a DUE university degree and the profession of Nursing, with its own body of knowledge.

Respiratory Nursing

• In respiratory diseases, the nursing role is key in the appropriate approach to the patient.
• Respiratory nursing focuses its care on promoting the highest degree of respiratory health in the population.
• Respiratory nurses are advisors in managing the care of patients with respiratory problems.

That is, they evaluate and implement new ways of caring for respiratory patients and are experts in their field with extensive knowledge of respiratory complications both in the hospital environment and in the community.

For some authors, respiratory assessment is an essential element in the basic training of nursing students.

However, the reality is that general nurses express doubts regarding basic aspects of this, such as measuring respiratory rate or the technique for performing blood gases.

Currently, there is no recognized specialty as respiratory nursing in our country, despite it being a need expressed on numerous occasions by the group of nurses who provide care in the pulmonology environment.

Respiratory system

The respiratory system is a complex anatomical system that includes the lungs, airways, various parts of the central nervous system related to the control of ventilation, respiratory muscles and the rib cage.

The assessment is a systematic process that includes clinical history and physical examination that consists of:

• Recognize changes in lung status.
• Determine interference in daily activities.

Therefore, nursing staff must assess whether there are significant changes in these aspects; In addition, if there are factors that affect the respiratory control mechanisms

Among the characteristics to be evaluated we can mention the frequency, volume, rhythm, ease and efforts, respiratory sounds and chest movements in addition to the presence of cough and expectoration in the patient.

Importance of Respiratory Nursing

Respiratory nursing plays a fundamental role in many areas of patient care, such as the following:

1. The hospitalization floor
2. Functional testing laboratories
3. Specialized consultations
4. Patient care in the face of smoking
5. lung transplant
6. Bronchoscopy
7. sleep unit
8. Ventilation
9. Home care.

In general, there are a variety of roles and responsibilities, depending on the environment and patient needs.

For example, they assess respiratory status and needs with tools such as pulse oximetry and chest x-rays.

They also develop and implement care plans in collaboration with other health professionals.

On the other hand, they administer medications and treatments such as oxygen therapy and bronchodilators.

Another very important aspect is to educate patients about their condition and how to prevent complications.

They also monitor progress and adjust care plans accordingly.

As well as providing emotional support to patients and their families.

Chronic respiratory diseases

On the other hand, it faces the care of chronic patients, which requires, in many cases, complex treatments such as:

1. Non-invasive mechanical ventilation (NIMV)
2. Treatment with continuous airway pressure (CPAP)
3. Home respiratory therapies.

It is known that this type of treatment affects the patient and their families, as it involves a change in lifestyle and requires monitoring by specialized personnel.

Specific treatments

Likewise, currently, the route chosen for the treatment of obstructive respiratory diseases is inhaled.

Given the growing appearance of new devices and the importance of correct technique for their administration, constant updating by nursing staff is essential, where it plays a fundamental role in patient education.

Conclusions on respiratory nursing

For all these reasons, the existence of groups, as well as training and discussion forums, are essential.

The objective is to promote the growth of respiratory nursing and give value to the role of the specialized nurse.

Nurses are essential in the care and monitoring of patients with respiratory pathologies, being a reference for family members and caregivers.

Source: Centro Andaluz Alfa 1, Consejo General de enfermería, dialnet, sogapar

La entrada Respiratory Nursing se publicó primero en Centro Andaluz Alfa-1.