What do guidelines and disease management programs (DMPs) mean for the patient?

Speaker: Prof. Heinrich Worth, Chairman of AG Lungensport eV (Germany) and Vice Chairman of the German Respiratory League eV.
Summary: Prof. Gratiana Steinkamp, as published in Alpha1 Journal 1-2018

COPD

The abbreviation COPD stands for chronic obstructive pulmonary disease and encompasses two distinct conditions: chronic bronchitis, characterized by a persistent narrowing of inflamed airways, and pulmonary emphysema, in which the supporting structures of the small airways are destroyed, leading to overinflation of the lungs. Alpha-1 antitrypsin deficiency can be considered a specific form of COPD, particularly in relation to emphysema.

Guidelines for COPD

In April 2018, experts from the German and Austrian pulmonary societies published recommendations on how to best treat COPD patients based on scientifically sound findings (evidence). These guidelines are developed using a standardized procedure. This includes reviewing and evaluating relevant scientific articles. The study results are assessed, and graded recommendations are derived from them. In addition to the guideline for physicians and other healthcare providers, a guideline specifically for patients is also being developed.

COPD diagnostics according to guidelines

For patients, the advantage of guidelines lies in their clear explanation of which examinations the doctor should perform and how to assess the patient's condition. To do this, the doctor determines the extent of bronchial narrowing, inquires about the patient's symptoms, and assesses the risk of an acute worsening (exacerbation).

The doctor asks the patient about risk factors such as tobacco smoke or inhaled pollutants, and gathers information about their medical history, comorbidities, physical activity, and current medication. The "AHA" signs – sputum production, cough, and shortness of breath – are particularly important. Questionnaires like the CAT (COPD Assessment Test) help to assess the symptoms more precisely. This test considers not only lung problems but also physical activity, sleep quality, and overall energy levels. Each of the eight questions has a score from 0 to 5 points. A score of more than 20 out of a possible 40 indicates that the disease is already significantly limiting the patient's life.

A pulmonary function test is used to detect persistent narrowing of the airways (obstruction). To assess hyperinflation, body plethysmography is performed. This device resembles a telephone booth, in which the patient sits during the measurement. Imaging diagnostics, particularly lung CT scans, help to pinpoint the exact location of the emphysema in the lungs.

Blood tests play a relatively minor role in COPD. However, the alpha-1 antitrypsin serum level should be measured in every patient at least once to rule out this condition. Blood oxygen levels and inflammation markers can also be important.
When COPD is diagnosed, the doctor should look for accompanying illnesses. This is because other organ systems are often affected, especially the cardiovascular system, the muscles, the skeletal system with osteoporosis, the metabolism with inflammation, and the psyche with anxiety or depression.

Once the results of these examinations are available, the doctor classifies COPD according to the severity of the symptoms and the acute worsenings/exacerbations using the letters A to D. Type D is the most severe and type A the mildest.

COPD therapy

The guidelines address not only drug treatment but also prevention, non-drug therapies, and surgical interventions. Prevention measures include not smoking, regular influenza and pneumococcal vaccinations, and access to clean, pollutant-free air at work. Non-drug therapies encompass physical exercise, physiotherapy, and nutritional counseling. Patient education also plays a crucial role. Surgical interventions are rarely necessary.
The most important pillar of COPD drug therapy is bronchodilators. They reduce bronchial constriction and, above all, hyperinflation. Their benefit is undisputed. Anti-inflammatory medications such as inhaled corticosteroids or the newer drug roflumilast are only beneficial for a smaller proportion of patients. Guidelines help physicians find the best combination of medications for basic therapy.

Additional medication is required to treat acute exacerbations. If the sputum is yellow-green, this may indicate a bacterial infection. Antibiotics are only helpful in this case, not in the case of viral infections.

Patient education

With a chronic condition like COPD, it's crucial that the patient is actively involved in their care. They should be able to recognize changes in their health and respond by taking appropriate measures and adjusting their medication accordingly. Patient education teaches them about the effects and side effects of medications, proper inhalation technique, and how to respond in an emergency.

The personal COPD action plan describes the symptoms in stable and unstable lung conditions and in the case of infection. The step-by-step plan for emergencies [DIA 22] details what the patient should do and in what order.
Experts have also developed special materials to provide comprehensive patient information, such as the book "I have my COPD under control!", which is reimbursed by health insurance companies.

Lung rehabilitation

The guidelines recommend rehabilitation for patients in COPD groups B to D. The goal is to improve well-being, functional capacity, and quality of life. Unfortunately, in a large study of 6,000 patients, only 7 (%) received rehabilitation.
After three weeks of inpatient rehabilitation, most patients have benefited significantly. However, if they don't make changes in their daily lives afterward, these improvements are quickly lost. In many cases, there is still a lack of sufficient support, such as local pulmonary exercise programs.

DMP (Disease Management Program)

Disease Management Programs (DMPs) are structured treatment programs for chronically ill patients, based on treatment guidelines developed by experts. This ensures the quality of care and aims for long-term cost reduction.
When a patient enrolls in such a Disease Management Program (DMP), they can be assured of being examined and treated according to current guidelines. Structured patient education is also covered, and in some German states, smoking cessation programs are included as well. Access to exercise therapy and rehabilitation is easier through a DMP. The program outlines the key drug classes for both long-term and on-demand therapy, allowing physicians to determine which medications are appropriate for each patient. Overall, patients benefit significantly from more intensive care provided by physicians and specialized professionals.
The benefits and success of such disease management programs (DMPs) are monitored and evaluated. Quality indicators and targets have been defined for this purpose and are continuously measured. For example, emergency treatments and acute deteriorations, as well as the proportion of patients who smoke, should decrease over time.
The speaker has formulated the 10 commandments of COPD management for treating physicians. They summarize the most important measures for diagnosis, therapy and monitoring of treatment success [DIA 46].

Summary: Prof. Gratiana Steinkamp, as published in Alpha1 Journal 1-2018.

Health policy: The fight for an Alpha-1 guideline

In the first half of 2018, we focused more on health policy. It's a vast field in which we still have much to learn and need to become much more involved. Who is familiar with the German Alpha-1-GuidelineNo one? No wonder, because they don't exist. We are – as so often – part of COPD, although AATD is known to also lead to liver or skin disease. The effects of AATD even occur in children, sometimes with severe courses and in very rare cases requiring transplantation – all areas not covered by COPD.

But how do you proceed, who do you approach? Our Health Minister, Mr. Spahn, directly? Then we received help from Mr. Alexander Wilke (from the Institute for Evidence-Based Health and Care Management (IfGV).) very right, who together with us a Position paper wrote.

Knowing that it is virtually impossible to create our own guideline, we would still like to at least in the COPD guideline We will also contribute our points, and we will fight harder for this in the future. But we don't just want to contribute to the COPD guidelines, but also, for example, to the... Guideline for the treatment of bronchiectasis (European Lung Foundation: Bronchiectasis).

Both guidelines mandate testing for AATM – there is sufficient data to support this.

Incidentally, there is an international... Alpha-1 guideline.

The position paper, entitled „Opening of the DMP – Part COPD for patients with alpha-1 antitrypsin deficiency in Germany“ was a start, but it didn't stop there, because in March Mr. Wilke and I presented at the Joint Federal Committee (GBA) We presented our concerns in Berlin and were met with a great deal of understanding.
Our arguments were taken to the GBA subcommittee and discussed there; at the beginning of April we received a very positive response. The committee members advocated opening the COPD disease management program (DMP) to patients with alpha-1 antitrypsin deficiency.
According to Mr. Wilke, we have already achieved a great deal – the door is open. In my opinion, everything takes far too long.

On April 24th, Alpha1 Global organized a meeting at the European Parliament. Approximately 30 of us participated (Alpha1 experts, patient representatives from various countries, and 1-2 EU parliamentarians). Patient representatives from six countries reported on their experiences with Alpha-1 antitrypsin deficiency in their respective countries.

This also showed that there is still much to be done to be heard by health policymakers and even more to achieve a better situation.

On April 25th, the company CSL introduced a Alpha-1 Awareness Day (European Alpha-1 Awareness Day) was held with a roundtable discussion. This also consisted of patient representatives, Alpha-1 experts, and representatives of other organizations, and aimed to deepen the understanding of this rare genetic disorder. (EU Roundtable on Alpha-1 Antitrypsin Deficiency (AATD)).

What good did that do? – I don't know. But in any case, raising awareness is important, and we see part of our responsibility as constantly drawing attention to ourselves and our illness.

„"Constant dripping wears away the stone"“

Eventually everyone will know about Alpha-1 Antitrypsin Deficiency – we're working on it.

Marion Wilkens, as published in Alpha1-Journal 1/2018.