Joint statement on the current coronavirus pandemic SARS-CoV-2 — COVID-19 in children and adult patients with Primary Immunodeficiencies (PID)

Joint statement on the current coronavirus pandemic SARS-CoV-2 — COVID-19 in children and adult patients with  Primary Immunodeficiencies (PID)

From IPOPI, ESID, INGID, APSID, ARAPID, ASID, CIS, LASID, SEAPID, J-PROJECT, IUIS

1st update, as of 2020, 11th March,

2nd update as of 2020, 3rd April).

Coronavirus – what is it?

In December 2019, a cluster of pneumonia cases was reported in Wuhan, Hubei
Province, China, linked to a novel coronavirus (SARS-CoV-2, leading to COVID-19 disease).1 Coronaviruses are common in many different animal species and it is rare that they infect people and spread between them, but it happens. Recent examples include Severe Acute Respiratory Syndrome (SARS-CoV-1—Cov, for coronavirus), and Middle-East Respiratory Syndrome (MERS-CoV). The SARS-CoV-2 is distinct from the previous two coronaviruses and causes COVID-19 disease.2

What is the current situation regarding COVID-19?

The situation is changing all the time and we advise you to monitor for the latest advice applicable to your area.

As of 2020 3rd April, over 1 000 000 cases of COVID-19 have been reported worldwide (181 countries). The countries with the highest number of reported cases are the USA, Italy, Spain, Germany, China, France, Iran and the UK.3

The spread and severity of this viral outbreak has demonstrated the need for a fast and comprehensive response from the public health sector. Beside the virus itself, one of the biggest threats is the overwhelming of the healthcare systems/hospitals due to the rapid spread and the lack of herd immunity in the general population.

How does it spread?

The transmission mode of COVID-19 is similar to previous coronavirus outbreaks spreading from person to person through:

Respiratory droplets spreading when coughing or sneezing
• Close personal contact with an infected person (shaking hands or touching)
• Touching contaminated surfaces and then touching eyes, nose or mouth with unwashed hands.4

Clinical symptoms due to COVID-19 infection

Human coronaviruses commonly cause mild to moderate illness in the general population. So far, the main clinical signs and symptoms reported in this outbreak include fever, fatigue, dry cough and runny nose. Some patients also experience aches and pains, myalgias, nasal congestion, sore throat and/or diarrhea. There has also been reports of transient loss of taste sense or sense of smell.

These symptoms are usually mild and begin gradually. Some people become infected but don’t develop any symptoms and don’t feel unwell. Approximately 80% of the affected people recover from the disease without needing special treatment.5

However, some patients might experience shortness of breath, requiring oxygen at the hospital. This can evolve into acute respiratory failure for which transfer to intensive care unit for non-invasive (face respiratory mask) or invasive (mechanical artificial ventilation) might be required. Some patients have experienced hyper-immune response mimicking hemophagocytic syndrome. A rebound at D+6 till D+10 may occur.

Should PID patients get systematically tested for COVID-19?

The situation is changing all the time and we advise you to monitor for the latest advice applicable to your area.

The test usually consists in a nasal swab, that is sent to a dedicated microbiology laboratory for detection of this virus (by polymerase chain reaction (PCR) method, within a few hours).

However, in light of shortage of manpower and availability of PCR, most national guidelines do not include or manage patients with chronic diseases, including PID, differently to other patients. At this stage, it is not recommended to have PID patients with no, or very limited, symptoms tested. Furthermore, in some cases, a negative PCR does not rule out infection (“false negative”).

Testing through detecting antibodies (IgG and IgM) against SARS-CoV-2 in the blood (“serology”) is not available on a large scale. This test will reveal whether the tested person has mounted a detectable antibody response after being infected with the virus.

For patients with PID who are not able to produce antibodies (such as patients with agammaglobulinemia or profound hypogammaglobulinemia), this test will not be useful. For the ones with other forms of PID (including the ones under Ig replacement therapy), this test might be of help.

Hence, it is advised to adhere to local and national guidelines for testing.

Treatments (vaccines & anti-viral medicines) tested for COVID-19

No medicine has yet demonstrated efficacy in treating or preventing COVID-19 but many are being studied in clinical trials.6

Hydroxychloroquine is an anti-infectious drug with anti-inflammatory properties. It is used to prevent malaria and to treat mild forms of autoimmune diseases such as lupus or rheumatoid arthritis.

It is well known that this drug has the potential to inhibit the action of the virus in the laboratory (in vitro testing) and its potential efficacy in preventing or treating COVID-19 is being carefully evaluated. There is however not enough evidence at this stage to ascertain its efficacy.

Other drugs are also being carefully evaluated.


COVID-19 clinical trials at a glance

COVID-19 clinical trials listed on TranspariMED.

COVID-19 NIH clinical trials registry.

Living mapping and living network meta-analysis of COVID-19 studies

Precautions

Any respiratory virus that can be spread from person-to-person may be a risk for PID patients. Therefore, PID patients should be cautious and keep track of developments of COVID-19 in their region. Whilst immunoglobulin (Ig) replacement therapy provides protection against a wide range of infections, it does not guarantee immunity against coronavirus. The World Health Organization’s (WHO)7 and the Centers for Disease Control and Prevention’s (CDC)8 recommendations to reduce exposure to and transmission of COVID-19 include, but are not limited to, the list below.

  • The MOST IMPORTANT means to prevent infection are:

    • Wash hands frequently (every hour) with hand rub or soap and water for 20 seconds, (if not possible use alcohol-based hand rub), especially after direct contact with ill people or their environment

    • Avoid touching eyes, nose and mouth

    • Avoid close contact (1 meter) with people suffering from acute respiratory infections

    • Avoid close contact (1 meter) with anyone who has fever and cough

    • For extra precaution, avoid close contact (1 meter) with other people when leaving your home

    • Avoid greeting people by shaking hands, kissing or hugging

People with symptoms of acute respiratory infection should practice cough etiquette (maintain distance, cover coughs and sneezes with disposable tissues or clothing, and wash hands) and wear a respiratory mask if instructed by their local health care provider.

  • Masks should be used by people with symptoms, or when caring for someone with symptoms. They are generally not effective for preventing infection but should be used to avoid transmitting the virus to others.

  • For extra precaution, clean and disinfect frequently touched surfaces daily, including tables, doorknobs, light switches, countertops, handles, desks, phones, keyboards, toilets, faucets, and sinks.

  • Questions regarding daily life (school attendance, work, travel…) depend on the local epidemiological situation and the underlying PID and needs to be discussed with the PID expert physician.

  • If you feel unwell and experience symptoms such as fever, cough and/or difficulty in breathing, stay home and seek prompt medical assistance from your health care provider.

COVID-19 vaccine

There is currently no vaccine available for COVID-19. One or several vaccines should be available within 6-12 months.

COVID-19 in PID patients

To date (03-04-2020), global surveys aimed at collecting data on COVID-19 in PID patients do not point to an increased risk of COVID-19, especially not in its severe form, although a few cases have been reported.

However, certain PID patients might be at higher risk than others to catch this infection or a more severe course of the disease. In the absence of more precise data, patients with PID need to take extra care to prevent from getting this infection.

Research efforts are underway in order to monitor the cases of COVID-19 in patients with PID at a global scale.

Since the launch of the first survey 2.5 weeks ago, there are 11 cases reported (as of 2020, March 29th): 5 necessitating admission, one severe who evolved into ARDS. All kinds of PID diagnostic categories were found (phagocyte defects, combined immunodeficiency, antibody deficiencies, ALPS-like disease, and autoinflammatory disease). Almost all patients were aged <45 years. Typical presentation symptoms were as follows: upper respiratory symptoms, cough, fever. One child had diarrhea, vomiting and myalgia. One patient developed ARDS.

COPID19” is the more detailed second phase of the worldwide survey of COVID-19 in PID patients and is now available here: https://dsp.institutimagine.org/copid/connexion.php

Recommendations for PID patients

Patients with PID living in areas of high prevalence should take every precaution and adhere to local, regional and national recommendations (staying at home, teleconsultation, work from home, etc..).

Beyond the precautions mentioned above, we advise prompt phone contact with a doctor if an infection is suspected (should it be your PID expert, or your GP who should let your PID expert know about your condition in order to provide the best advice for each PID patient’s specific condition). Patients should always keep the details of their PID diagnosis and medical charts, medications, PID expert doctor and next of kin at hand, in case urgent medical care is needed.

PID patients with lung and/or heart complications, solid organ transplants’ recipients, recent recipients of hematopoietic stem cell transplantation or gene therapy, PID patients undergoing treatment for a cancer (malignancy), as well as patients under immunosuppressive or immunomodulatory drugs (for autoimmune or inflammatory or autoinflammatory complicating the PID course) should remain on their specific therapy until recommended otherwise by their PID expert physician. Immunosuppressive drugs (in particular corticosteroids), might limit signs of infections (fever and other clinical symptoms). It is this recommended to contact your PID expert physician in case of unexplained change in clinical status including your well-being.

PID patients with significant respiratory issues (severe asthma, bronchiectasis or chronic respiratory failure) should receive special attention (as for any risk of respiratory infection).

Keep in mind that it is always essential to continue the regular treatment for your PID.

Plasma Derived Medicinal Products (PDMPs), such as immunoglobulins (IVIG or SCIG) are safe and will protect you from many other infections.

For everyone, including PID patients, we strongly recommend you to keep up with the latest information on the COVID-19 outbreak in your region, for example provided by the World Health Organization (WHO), the European Centre for Disease Prevention and Control (ECDC) and by your national and local public health authorities.

National guidelines provided by national health authorities should be followed (the epidemiological situation and the management might differ from one country to another).

We want to stress that your PID expert can you give you the best personalized advice.

Patients can visit the IPOPI website to have full access to the FAQ here: https://ipopi.org/covid-19-and-pids-faqs/

 

Plasma Derived Medicinal Products (PDMPs), including Immunoglobulins

According to a statement from Plasma Protein Therapeutics Association (PPTA) there is no risk of transmission of SARS-CoV-2 by PDMPs.9

For PID patients who are on Ig replacement therapy, there is no evidence to date that more frequent dosing of Ig will offer more protection. Whilst Ig replacement therapy provides protection against a range of infections, it does not guarantee immunity against coronavirus.

For PID patients whose condition does not require to be under regular Ig replacement therapy, there is no need to start Ig replacement therapy since no antibodies targeting COVID-19 is expected to be contained in the existing preparations.

There is no recommendation to give immunoglobulins to the general population to protect or treat people against COVID-19.

Decline in plasma supply

The COVID-19 outbreak and associated confinement and movement restriction measures will impact supply of blood and plasma collection and may affect medicinal product circulation and supply.

As the plasma necessary to produce PDMPs is either collected from plasma donors (apheresis plasma) but also from blood donations (recovered plasma), this will almost inevitably impact the access to these life-saving therapies, although it may take a few months before PDMPs shortages start to be observed (it usually takes 7-10 months from the time plasma is collected from a human donor to reach the patients).

Numerous countries have reported significant drops in blood collection and a similar development is expected for plasma collection.

Various PID stakeholders are currently taking measures to react to this development on both national and regional levels so that PID patients are prioritized in case of any supply tensions or shortages associated with the COVID-19 outbreak.

About IPOPI

IPOPI is the leading advocate for primary immunodeficiencies’ patients worldwide working in collaboration with patients, doctors, politicians, regulators, pharmaceutical industry and other relevant stakeholders. IPOPI is the Association of national PID patient organisations currently representing 68 countries. More info: www.ipopi.org, Facebook, Twitter


About ESID

The European Society for Immunodeficiencies (ESID) is a non-profit organization whose main objectives are to facilitate the exchange of ideas and information among doctors, nurses, biomedical investigators, patients and their families concerned with primary immunodeficiency diseases and also to promote research on causes, mechanisms and treatment of these disorders. ESID was established as an informal group in 1983 and became a society in 1994. More information: www.esid.org, Twitter


About INGID

The aims of INGID are to improve and extend the quality of nursing care of patients with primary immune deficiencies, and to increase the awareness and understanding of primary immunodeficiencies amongst nurses. More info: www.ingid.org


About APSID

The Asia Pacific Society for Immunodeficiencies (APSID) works to provide PID care, education and research for PID patients, through collaborative infrastructure and various APSID Working Parties. A group of over 60 Asian paediatricians and scientists interested in Primary Immunodeficiency met in Osaka, April 2015 and pledged to establish APSID with the following missions: To care and cure patients with primary immunodeficiency (PID), To share PID experience so as to promote collaboration & education, To improve PID management through understanding its genetics & pathogenesis and To advocate and advance the care of PID patients through engaging governments, patient organizations & industry.

About ARAPID

ARAPID is the Arab Society for PID. Its purpose is to bring together the English-speaking east region of the Arab world, closer to the French-speaking west region, to better serve PID patients from the Arab world who are united by consanguinity, etiological profile of PIDs and culture (awareness).

About ASID

The African Society for Immunodeficiency (ASID) is a PID focused scientific society. Its main objectives are to improve PID awareness and care within Africa and has been working on addressing continental African PID peculiarities. ASID strives to support African patients through collaborating with national and international patient groups and works with national societies and other relevant authorities to achieve its objectives. ASID also collaborates with international PID societies and alliances, and the industry to promote better PID care and research. Please visit website: www.asid-africa.org

About CIS

The Clinical Immunology Society (CIS) is based in the United States but has members from around the globe. The mission of CIS is to facilitate education, translational research and novel approaches to therapy in clinical immunology and to promote excellence in the care of patients with immunologic/inflammatory disorders.

About LASID

The Latin American Society for Immunodeficiencies (LASID) is a vibrant and inclusive international society. This is the home of all professionals dedicated to the field of Primary Immunodeficiencies aiming to develop and perfect the education, scientific research, and health care within this medical specialty. LASID’s mission comprises the following: To increase awareness in Primary Immunodeficiency Diseases (PIDD) at all levels all over the continent, to develop diagnostic capabilities to reach as many as possible patients and to favor the development of centers providing appropriate treatments for PIDD patients.


About SEAPID

South East Asia Primary Immunodeficiency Network or “SEAPID” is a regional NGO – the South East Asian network of Primary Immunodeficiency Experts. It was established in Bangkok, Thailand on 26th January 2015, following an accord reached by experts from the six South East Asian founding countries, namely, Indonesia, Malaysia, the Philippines, Singapore, Thailand and Vietnam.

About IUIS Inborn Errors of Immunity Committee (IEI)

The IEI Committee consists of experts in all aspects of primary immunodeficiencies. Its missions are: to provide an up-to-date classification of all primary immunodeficiency diseases (IEIs), to assist with the identification, diagnosis and management of patients with these uncommon conditions, to support diagnostic and therapeutic guidelines developed by national societies and others, to assist healthcare providers, to promote awareness, diagnosis and treatment of IEIs in all regions of the world, to produce ad hoc reports on any aspect of IEIs, to assist in the welfare of patients with these conditions.

About J-PROJECT

The J-PROJECT is an Eastern and Central European network that aims to increase awareness of PID disorders among physicians and to improve medical training in this domain. This physicians’ education campaign was started in March, 2004, and since then it has established itself as a successful awareness program for PID disorders.

More resources:

Short videos by Prof. Kate Sullivan, member of the medical board of the Immune Deficiency Foundation (IDF)

https://youtu.be/ydk-Q959RJY (posted on 2020, March 3rd)

https://youtu.be/3DUKPXgYaYc (posted on 2020, March 10th)

https://youtu.be/A7AIZ0HVOBA (posted on 2020, March 18th)

1 European Centre for Disease Prevention and Control. Infection prevention and control for the care of patients with 2019-nCoV in healthcare settings. ECDC: Stockholm; 2020. Available at: https://www.ecdc.europa.eu/sites/default/files/documents/nove-coronavirus-infection-prevention-control-patients-healthcare-settings.pdf

2 European Centre for Disease Prevention and Control. Q & A on COVID-19. 31 March 2020. Available at: https://www.ecdc.europa.eu/en/covid-19/questions-answers [Accessed 03-04-2020].

3 Johns Hopkins University. Coronavirus COVID-19 Global Cases by the Center for Systems Science and Engineering (CSSE) at Johns Hopkins University (JHU). 02-04-20. https://www.arcgis.com/apps/opsdashboard/index.html#/bda7594740fd40299423467b48e9ecf6. [Accessed 03-04-2020].

4 The World Health Organization. Q&A on coronaviruses (COVID-19). 9 March 2020. Available at: https://www.who.int/news-room/q-a-detail/q-a-coronaviruses [Accessed 03-04-2020].

5 Ibid.

6 Inserm. Launch of a European clinical trial against COVID-19. 22 March 2020. Available at: https://presse.inserm.fr/en/launch-of-a-european-clinical-trial-against-covid-19/38737/ [Accessed 03-04-2020].

7 The World Health Organization. Q&A on coronaviruses (COVID-19). 9 March 2020. Available at: https://www.who.int/news-room/q-a-detail/q-a-coronaviruses [Accessed 03-04-2020].

8 Centers for Disease Control and Prevention. Prevention & Treatment. 18-03-2020. Available at:

https://www.cdc.gov/coronavirus/2019-ncov/about/prevention-treatment.html [Accessed 03-04-2020].

9 The Plasma Protein Therapeutics Association. New Coronavirus (SARS-CoV-2) and Plasma Protein Therapies. Updated March 13, 2020. Available at: https://www.pptaglobal.org/media-and-information/ppta-statements/1055-2019-novel-coronavirus-2019-ncov-and-plasma-protein-therapies [Accessed 03-04-2020].

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