COVID-19 and PIDs FAQs

Updated May 11, 2020

This is a compilation of answers to questions received from IPOPI’s national member organisations via email, social media and during IPOPI’s COVID-19 webchats.

General prevention measures to avoid infection

  • Wash hands frequently (every hour) with 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/3 feet) with people suffering from acute respiratory infections
  • Avoid close contact (1 meter/3 feet) with anyone who has fever and cough
  • For extra precaution, avoid close contact (1 meter/3 feet) with other people when spending time outside your home
  • Avoid greeting people by shaking hands, kissing or hugging
  • Respect the confinement measures wherever these are applicable
  • People with symptoms of acute respiratory infection need to 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 healthcare provider

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

PID patients and COVID 19

How high is the risk for PID patients to be infected?

  • To date (11-05-2020), 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.
  • 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 COVID-19.

Are PID patients more likely to catch this infection and do they risk being more severely affected?

  • Validated data regarding additional risks for PID patients regarding the SARS-CoV-2 virus is not yet available.
  • To date (11-05-2020), 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.
  • In general terms, for PID patients with lung complications it is possible that the risk for an infectious disease is higher and that you might be more severely affected if catching the SARS-CoV-2 virus.
  • PID patients with underlying hypertension, diabetes or any heart disease should also be cautious as the risk for a more severe infection increases, as in the general population.
  • 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 regularly continue to take the 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.
  • Whilst immunoglobulin (Ig) replacement therapy provides protection against a wide range of infections, it does not guarantee immunity against COVID-19.
  • PID patients should be cautious, follow recommendations, and keep track of developments of COVID-19 in their region.

* SARS-CoV-2 is the virus that causes the COVID-19 infection.

Some PID patients have daily fever without necessarily having caught the virus. How can they avoid being put in isolation?

  •  Ideally these patients would be tested, show negative results, and thus avoid isolation.
  • However, in many parts of the world isolation/confinement is now recommended, meaning that even people who have not yet tested positive for COVID-19 are being advised to stay at home as a preventive measure.
  • To date (11-05-2020), 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.
  • As the situation is evolving rapidly in many places, PID patients 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..).

Is COVID-19 comparable to the swine flu pandemic in 2009 and the consequences it had for PID patients?

  • COVID-19 is an infectious disease, but it does not behave exactly like a classic airborne infectious disease, as for example the swine flu in 2009.
  • Some patients experience severe symptoms that require hospitalization (sometimes in intensive care units), caused by an abnormal immune response called hyperinflammation.
  • The immune systems response to the virus is unbalanced, which can lead to the severe symptoms of respiratory distress, subcutaneous skin symptoms, rheumatological symptoms features among others.
  • Due to the hyperinflammation it is believed that immunotherapy is a major component of the therapeutic arsenal for treating severe cases of COVID-19.

Symptoms

What are the clinical symptoms of a 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, tiredness, fatigue, dry cough and running nose.
  • Some patients also experience aches and pains, nasal congestion, sore throat, diarrhea and skin rash. These symptoms are usually mild and begin gradually.
  • In children and pre-teens, a form of vasculitis close to the already known Kawasaki disease has been recently described (see below).
  • 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.
  • Word of caution: Some COVID-19 patients might experience shortage of breath and require oxygen administration at the hospital. It has been reported that evolution to a more severe form requiring urgent medical care can be very rapid (within a few hours).

There have been recent cases with children presenting Kawasaki like symptoms, is this linked to COVID-19?

  • There have been some reports of children and young adolescents presenting a Kawasaki-like syndrome following a COVID-19 infection, indicating a link between the two.
  • Kawasaki disease is a rare pediatric inflammatory condition that mainly affects children up to 5 years old. It leads to inflammation (swelling and redness) in the blood vessels and infected patients may experience fever, rash, red eyes, peeling skin, severe diarrhea and joint pain. In more severe cases it can inflame the walls of the arteries and affect the blood flow to the heart. This can potentially be fatal.
  • The recently reported patients have been approximately 5-15 years old (older than the usual age group to be infected with this disease).
  • Many of these patients have tested positive for COVID-19, but do not show the respiratory symptoms commonly associated.
  • The relationship between these two diseases is still under investigation.

Transmission

How does the SARS-CoV-2 spread?

  • The transmission mode of SARS-CoV-2 is similar to the 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
  • Recent findings have also demonstrated that children may release virus in the stools up to 15 days after recovering from COVID-19. This means that frequent washing of hands should apply even after a patient has clinically recovered.

Why should people be quarantined when returning from risk areas, if they are not coughing and sneezing?

  • The virus does not only transmit from person to person through respiratory droplets spread for example when coughing or sneezing. As an example, the virus can also transmit if an infected person coughs while covering their mouth with their hand and then touches someone else who in turn touches their eyes, nose or mouth with unwashed hands.
  • There have been some reports of people who have tested positive for SARS-CoV-2 with only minor or no symptoms at all. In these cases, the infected person has still been able to transmit the virus to people who then become symptomatic, with minor or sometimes more concerning symptoms.
  • The fewer people who are exposed to an infected person the better. This is why quarantine for 2 weeks is usually recommended for people infected with SARS-CoV-2.

Is COVID-19 contagious before symptoms begin?

  • The data regarding the contagiosity is still scarce.
  • However, there have been some reports of people who have tested positive for SARS-CoV-2, showing only minor or no symptoms at all. In these cases, the infected person has still been able to transmit the virus to people who then become symptomatic, with minor or sometimes more concerning symptoms.

Can warmer weather reduce the number of COVID-19 infections?

  • Validated data for a seasonal behaviour of COVID-19 is not yet available.
  • As SARS-CoV-2 is a new coronavirus, the majority of the population is highly susceptible to infection. Even if some small studies point to warmth being unfavourable for SARSCoV-2, the contagiosity is likely to overwhelm any temperature effect on the spread of the disease.
  • Moreover, given that there are countries who currently experience warm temperatures and still see a rapid virus spread, other countries should not expect to see a decrease in cases with increasing temperatures.

Are there countries where patients who suffered from flu during the end of 2019 are being re-examined to see if they were in fact infected with COVID-19?

  • There are reports of patients who travelled to the Wuhan region in China and experienced more severe flu like symptoms after their return. In retrospect it is believed that they could have been suffering from COVID-19. There is not yet any solid data available, but it is being investigated.
  • There is also a case reported from a French hospital where previous pneumonia patients treated in December 2019 now have been retested. The results indicated that one of the patients was infected with COVID-19 in December 2019, one month before the first cases were confirmed in France.

Tests

What are serology tests and are they reliable?

  •  Serology tests are blood-based tests that can be used to identify whether people have been exposed to a pathogen by looking at their immune response. The serology tests look for the presence of antibodies. The antibodies detected by this test indicate if a person has had an immune response to SARS-CoV-2 (with symptoms or asymptomatic).
  • In contrast, the RT-PCR tests can only indicate if a person is currently infected with the virus and not if a person has been infected and recovered.
  • There are currently various serology tests and quick tests being made available online for people to do in their homes, but these tests need to be validated as they may not be reliable.
  • When validating a serology test the sensitivity and specificity are key indicators. This is to ensure that the test is sensitive enough to detect all patients who have had the COVID- 19 infection and that it is specific enough to only detect patients who has had the COVID- 19 infection.
  • Serology test rarely tests 100% for both sensitivity and specificity and it is likely that there will still be some false negatives and false positives. Due to this they need to go through numerous steps to ensure that they are validated for the market.
  • Please note that if you have tested positive with a serology test and already recovered, it may not mean that you are immune or noninfectious. We encourage PID patients to continue being cautious.

Can patients who do not produce antibodies use serology tests?

  • These patients are not good candidates to be assessed with serology tests.
  • So far there are no test for T-cells based immunity regarding COVID-19 available, but this may come in the future.

Prevention

What can people do to protect themselves from SARS-CoV-2?

  • 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) and the Centers for Disease Control and Prevention’s (CDC) 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 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/3 feet) with people suffering from acute respiratory infection
    – Avoid close contact (1 meter/3 feet) with anyone who has fever and cough
    – For extra precaution, avoid close contact (1 meter/3 feet) with all other people when going outside your home
    – Avoid greeting people by shaking hands, kissing or hugging
    – Respect the confinement measures wherever these are applicable
  • If you feel unwell and experience symptoms such as fever, cough and/or difficulty breathing, seek prompt medical assistance from your health care provider.

Should I wear a mask?

  • Masks can be effective if the person wearing it has the appropriate training for a good fitting mask, but if not used appropriately they can pose a risk for contamination. The mask needs to be replaced regularly. Guidance from the World Health Organization on the appropriate way of wearing masks includes:
    – Before putting on a mask, wash your hands (with alcohol-based hand rub or soap and water).
    – Cover mouth and nose with mask and make sure there are no gaps between your face and the mask.
    – Avoid touching the mask while using it; if you do, clean your hands with alcohol based hand rub or soap and water.
    – Replace the mask with a new one as soon as it is damp and do not re-use single-use masks.
    – To remove the mask: remove it from behind (do not touch the front of mask)
    – Discard immediately in a closed bin; clean hands with alcohol-based hand rub or soap and water.
  •  Some countries have taken measures for citizens to wear masks when spending time outside their homes and we advise to follow national guidelines. If you have symptoms you should wear a mask to protect people in your surroundings. If you are not able to wear a facemask (for example, because it causes trouble breathing), then you should do your best to cover your coughs and sneezes, and people who are caring for you should wear a facemask if they enter your room. If a shortage occurs, masks should be reserved for hospital staff and people experiencing symptoms.

Are there additional prevention measures for PID patients?

  • To date (11-05-2020), 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.
  • 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.
  • PID patients with significant respiratory issues (severe asthma, bronchiectasis or chronic respiratory failure) should receive special attention (as for any risk of respiratory infection).
  • 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.
  • Keep in mind that it is always essential to regularly continue to take the 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 aware of the latest information on the COVID-19 outbreak in your region, for example provided by the World Health Organization’s (WHO), the European Centre for Disease Prevention and Control (ECDC) and by your national and local public health authorities.

What extra precautions should be taken for PID kids during this COVID-19 pandemic?

  • Validated data on COVID-19 in children is still limited but suggests that children with COVID-19 may have only mild symptoms. However, they can still pass this virus onto others who may be at higher risk.
  • Beyond the general precautions for all PID patients, the recommendations for your child will depend on the underlying PID. Thus, we recommend discussing it with your child’s PID expert.
  • Keep in mind that it is always essential for your child to regularly continue to take the PID treatment. Plasma Derived Medicinal Products (PDMPs), such as immunoglobulins (IVIG or SCIG) are safe and will protect you from many other infections.
  • If your child’s school has been suspended, we recommend following school guidance to ensure that your child can continue with their education.

Should PID patients reduce their IVIG frequency and/or increase the dose?

  • It is essential that PID patients continue to regularly take their treatment. Plasma Derived Medicinal Products (PDMPs), such as immunoglobulins (IVIG or SCIG) are safe. They will not protect you from COVID-19, but they will protect you from many other infections.
  • For patients with lung disease or a history of serious infectious diseases, especially COVID-19, it may be an option to increase the IVIG dosage. However, this should be personalised and agreed between the patient and the PID expert, not seen as a general approach.
  • There is no evidence to date that more frequent dosing of Ig in general will offer more protection.
  • 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.

Can hydroxychloroquine (HC) prevent people from getting infected with COVID-19?

  • Hydroxychloroquine is a group of drugs used to prevent and treat for example malaria, rheumatoid arthritis and lupus.
  • There is no validated data supporting that HC will help prevent getting infected with COVID-19.
  • HC has shown some efficacy in treating the first phase of the COVID-19 disease regarding the viral replication phase: data seem to point towards a shorter period of viral replication. However, experts have warned that the studies available so far are small and lacks enough rigor to be classed as evidence of a potential treatment. More time and testing are thus needed before the treatment can be determined to be safe and effective.
  • Clinical trials are currently under way to assess the efficacy and the tolerance of HC in COVID-19 patients.
  • Side effects of HC can be severe or even fatal. These are well-known and affect mostly the heart. Therefore, these side effects should be monitored closely by their health care professional.
  • Patients should only take HC if it has been prescribed by their treating physician.
  • PID patients should never self-medicate.

Does influenza vaccine protect against COVID-19 infection?

  • There is no proof that there is cross protection between influenza vaccine and COVID- 19.
  • In principle, it is usually beneficial to be protected against influenza because seasonal flu can lead to a higher incidence of bacterial lung infections. By getting vaccinated you reduce this risk.
  • However, recommendations regarding seasonal flu varies between PID categories (as there are live attenuated vaccines which might be contraindicated in some instances). Specialist advice should always be sought before receiving vaccinations.

Some countries have started to ease on confinement measures, should PID patients continue to stay at home as a precaution?

  • If a country has started lifting the confinement measures it is because their authorities has made a thorough risk assessment, concluding that it is safe for people to leave their homes.
  • In this case PID patients should follow their national guidelines but continue with recommended hygiene measures.
  • If you are in doubt, consult your PID expert.

Can antibiotic prophylaxis be useful to avoid COVID-19 infection?

  • Antibiotic prophylaxis is not believed to help avoid COVID-19 infection.
  • If you are already under a regular treatment, you should not stop it, but PID patients should not self-medicate. Please contact your PID expert for further advice.

Can vitamins help increase resistance against SARS-CoV-2?

  • Although keeping healthy diet is good practice, there is no evidence that taking vitamins will protect people from the new coronavirus.

Why are the recommendations not the same in every country?

  • This is a new virus and we are learning more and more each day. This pandemic includes various dimensions and until now many measures have been put in place as a response.
  • Decisions on health care policy are taken on a national level and for this reason guidelines regarding confinement, masks, tests and so on may vary slightly in each country.
  • For example, two countries with similar numbers of infected patients may have not have equally developed health care systems and therefore the measures may be different.
  • For treatments there is a much more standardized approach and health care professionals and researchers are collaborating all over the world to enhance the knowledge and generate validated data on the best treatment for this virus.

Treatment/vaccine

Is there any anti-viral medicine to treat SARS-CoV-2 available?

Is there any vaccine available?

When can we expect a date by which a vaccine will be available for PID patients?

  • Due to the various clinical trial phases a vaccine needs to go through it is not expected to be available until 2021.

Why does it take so long to develop treatments and/or vaccines?

  • It is only possible to develop a treatment or a vaccine with reliable data, i.e. from a well-designed clinical trial. Clinical trials are research studies aimed at evaluating a medical intervention. They are the primary way for researchers to find out if a new treatment is safe and effective in people.
  • Clinical trials for developing and testing a new drug (including vaccines) normally go through three to four phases.
  • During phase I an experimental treatment is tested on a small group of often healthy people (20 to 80) to judge its safety and side effects and to find the correct drug dosage.
  • During phase II more people are included (100 to 300) and the emphasize is on effectiveness. The aim of this phase is to obtain preliminary data on whether the drug works in people who have a certain disease or condition. These trials also study safety, including short-term side effects. This phase can last several years.
  • In phase III further information about safety and effectiveness is gathered, studying different populations and different dosages and using the drug in combination with other drugs. The number of participants usually ranges from several hundred to about 3,000 people (usually less when it comes to rare diseases).
  • After phase III the regulatory agency (e.g. the FDA) will take a decision to approve, or not approve, the experimental drug or device.
  • A phase IV or Post-Marketing Surveillance phase can take place after approval. During this phase the aim is to find out more about long term benefits and side effects. Sometimes, the side effects of a drug may not become clear until many people have taken it over a longer period of time.
  • It is also important to differentiate between a drug that has already been tested to treat something else and that now is being repurposed for COVID-19 and a new vaccine where the scientists needs to start from the very beginning.

Are there ongoing clinical trials that investigate if intravenous immunoglobulin (IVIG) can be a potential coronavirus treatment?

  • Yes, there are some clinical trials (e.g. in France, the US, Spain, China) who have started or soon will start investigating if IVIG can treat adults with respiratory failure as a result of COVID-19 infection.
  • So far there is not enough validated data available to establish if it is efficacious as a treatment or not, but there are pharmaceutical companies announcing that they will have early results from a clinical trial this summer.
  • IPOPI is closely monitoring this development and will continue to emphasize the importance of PID patients having prioritized access to IVIG.

Can PID patients take the vaccine for COVID-19 when it becomes available?

  • Recommendations will vary between PID patients and specialist advice should always be sought before receiving vaccinations.
  • Currently there are many on-going clinical trials with live vaccines and after approval there will be different vaccine strategies made available. This should include one appropriate for PID patients (depending on which type of PID).

Will the future licensed treatment or vaccine be distributed globally from the start? When will it be available in specific countries?

  • This will depend on various factors such as the production capacity of the individual companies and the company’s distribution channels. Hopefully many companies will try to distribute worldwide but this can vary.
  • There cannot be a worldwide release as each product needs to be nationally approved. In some countries it may be approved faster than in others which may lead to a quicker distribution.
  • World supply will take time.

What are the risks of participation in clinical trials for COVID19 anti-viral treatments and vaccines for PID patients?

  • Most patients with PIDs should not receive live-attenuated vaccines.
  • The risks will vary depending on the PID and the comorbidities. Specialist advice should always be sought before participating in a clinical trial.

What is the difference between convalescent plasma and hyperimmune immunoglobulin?

  • Convalescent plasma is the plasma obtained from donors who have recently recovered from COVID-19. At this stage it is an investigational product that has been used in the treatment of COVID-19 under some specific circumstances, in some countries.
  • Treatment with convalescent plasma is not a new approach. It has been used in the past to treat other diseases and it has not always been proven efficient.
  • However, it is important to note that although promising, convalescent plasma has not yet been shown to be safe and effective as a treatment for COVID-19. Therefore, it is important to study the safety and efficacy of COVID-19 convalescent plasma in clinical trials.
  • The viral inactivation in convalescent plasma is not as extensive as for hyperimmune immunoglobulin which is fractionated with 2-3 antiviral steps, similar to IVIG.
  • Hyperimmune immunoglobulins are a type of plasma derived medicinal products manufactured in the same way as regular immunoglobulin (IG) therapies, but from the plasma of donors who have developed high titres of specific antibodies. Hyperimmune immunoglobulins therefore contain much higher titres of specific antibodies than regular IG therapies and are entirely different products.
  • In the case of COVID-19 there is currently no hyperimmune immunoglobulin available. Several companies are working on the development of such therapies. The production process would involve using the plasma of many donors who have recovered from COVID-19 and therefore have high titres of COVID-19 antibodies. Once collected, this plasma would then be transported to manufacturing facilities where it will be fractionated into the final product in a similar fashion as regular immunoglobulin therapies. This is a long production process, often lasting between 7-10 months. The clinical trials for this treatment will start earliest in July 2020.

Daily life

Should children with PID stay home from school in countries with high COVID-19 prevalence?

  • Several countries have closed schools to avoid further spread of the virus. Decisions are being made based on the local epidemiological situation and the situation is changing constantly.
  • In countries where schools are open or re-opening, the first recommendation is to monitor the latest advice applicable to your area and to always seek advice from your child’s expert physician in case of doubt.
  • A general recommendation, in countries where confinement is not required, would be that PID patients continue to go to school, but that the school should ensure that the parents are informed immediately if any of the teachers or classmates present (even mild) symptoms. School attendance will therefore depend on the local epidemiological situation and the underlying PID and needs to be discussed with the PID expert physician.
  • If your child goes to school, please ensure they follow appropriate hygiene measures carefully and to monitor and follow your countries national guidelines.

In parts of the world were countries started to lift the restrictive measures, should paediatric PID patients return to school and adults to work?

  • If a country has started lifting the confinement measures it is because their authorities has made a thorough risk assessment, concluding that it is safe to return to school and work if appropriate hygiene measures are put in place.
  • If nothing else is mentioned this also includes PID patients, but we encourage PID patients to seek advice from their PID expert if they are in doubt.
  • Please ensure to follow appropriate hygiene measures carefully and to monitor and follow your countries national guidelines.

What can people do to cope with possible psychological stress and anxiety?

  • Enforced movement restrictions can induce feelings of stress and anxiety for people.
  • Remember that it is OK not to be OK but try your best not to panic and keep cool headed. This is especially important for parents with regard to their children.
  • Try to eat well and sleep enough.
  • Maintain phone and video contact with family and friends.
  • If you are in confinement, try your best to keep your brain and body active during this period. Have a routine. Download an exercise app, listen to music, re-read your favorite book, take an online course or clean your home.
  • Take breaks from social media and from watching, reading and listening to the news during parts of your day.
  • If you feel like you need support, seek advice from a psychologist or follow the advice from your national health authorities.
  • More information on how to cope with stress and anxiety available here.

Are there additional precaution measures for PID patients who cannot stay at home during this period?

  • For PID patients without possibility to isolate, we strongly recommend following guidelines for hygiene: washing hands every hour with soap and water (alcohol based hand rub if soap and water is not available), avoid touching eyes, nose and mouth, avoid public transport, avoid closed spaces, avoid close contact with other people when outside your home.
  • For extra precaution, clean and disinfect frequently touched surfaces daily, including tables, doorknobs, light switches, countertops, handles, desks, phones, keyboards, toilets, faucets, and sinks.
  • Some countries have taken measures for citizens to wear masks when spending time outside their homes and we advise to follow national guidelines. Masks can be effective to prevent from spreading the disease if the person wearing it has the appropriate training for a good fitting mask, but if not used appropriately they can pose a risk for contamination. The mask needs to be replaced regularly. Guidance from the World Health Organisation on the appropriate way of wearing masks includes:
    – Before putting on a mask, wash your hands (with alcohol-based hand rub or soap and water).
    – Cover mouth and nose with mask and make sure there are no gaps between your face and the mask.
    – Avoid touching the mask while using it; if you do, clean your hands with alcoholbased hand rub or soap and water.
    – Replace the mask with a new one as soon as it is damp and do not re-use singleuse masks.
    – To remove the mask: remove it from behind (do not touch the front of mask)
    – Discard immediately in a closed bin; clean hands with alcohol-based hand rub or soap and water.
  • We encourage PID patients to try their outmost to follow the confinement measures
    wherever these are applicable.

Access to hospitals/specialists/treatments 

Should patients continue to go to the hospital to receive their immunoglobulin (Ig) treatment?

  • It is very important for PID patients to stay on their Ig treatment, even though it might be administered at the hospital. Generally speaking, it is therefore better to continue receiving the treatment in the hospital rather than to postpone it.
  • Some countries have put in place special measures to enable patients who usually get their treatment in the hospital to instead, temporarily, get it at home.
  • For PID patients who have this possibility; temporary switching to home treatment is advisable to avoid visiting the hospital and potentially getting exposed to the virus. Home treatment can either be administered intravenously (IVIG) or subcutaneously (SCIG) depending on the country.
  • For PID patients who do not have this possibility, clinics should provide a space for PID patients on a dedicated time after the clinic has been closed and properly disinfected. The PID patients should be treated in separate rooms while receiving their treatments.
  • PID patients should follow the local health care professional advice in terms of prevention. If a shortage arises and a PID patient cannot access their treatment, then self-confinement and all other preventive measures should be strictly enforced.

Some patients have been switched from receiving IVIG therapy at the hospital to home administration. What preventive measures should be taken by nurses to minimalize the risk of contaminating the patient in their home?

  • All nurses or health care professionals that visits PID patients in their homes need to be free from COVID-19 symptoms.
  • More detailed guidelines (concerning masks, gloves, aprons etcetera) may vary depending on the country and the resources.

What is recommended for PID patients in countries with less developed medical systems?

  • PID patients living in areas of high prevalence should take every precaution and adhere to local, regional and national recommendations such as working from home if possible, avoiding public transport, avoiding closed spaces (e.g. shops) with many people etc.
  • Practice social distancing and be especially careful when touching places such as: mobile phones, keys, light switches, doorknobs, elevator buttons, shopping bags etc as the virus can live on these surfaces and the infection may spread.
  • Try to eat well and sleep enough.

Keep in mind to:

– Wash hands frequently (every hour) with 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/3 feet) with people suffering from acute respiratory infections
– Avoid close contact (1 meter/3 feet) with anyone who has fever and cough
– For extra precaution, avoid close contact (1 meter/3 feet) with all other people when going outside your home
– Avoid greeting people by shaking hands, kissing or hugging
– Respect the confinement measures wherever these are applicable

What should a patient do if they are confined in an area where there is no PID expert available?

  • PID patients should continue their regular treatment, follow guidelines concerning hygiene and follow national guidelines.
  • PID patients should avoid unnecessary travel if not advised in their region. If a trip is necessary and the patient risks not being able to return home, they should ensure that they have a contingency plan prepared for that situation.
  • PID patients can get in touch with their national patient organisation and/or IPOPI who may be able to provide the contact details to a local PID expert.

What should patients do if the hospital is overwhelmed or closed?

  • PID patients should follow their local health care professional’s advice.
  • If there is an immunoglobulin shortage and a PID patient cannot access their treatment, then self-confinement and all other preventive measures should be strictly enforced.

What should patients do if they cannot go to the hospital to access their immunoglobulin (Ig) treatment because they or their family members have COVID-19 symptoms?

  • It is very important for PID patients to stay on their Ig treatment and the solution will depend on the local circumstances.
  • If a PID patient or their family members experience symptoms and it restricts them from going to the hospital, they should consult their PID expert doctor to see if the treatment can be administered in the patient’s home or if the patient can switch to subcutaneous treatment for a limited period.
  • IPOPI monitors the access to Igs and if you experience problems we encourage you to share this information with us by emailing julia@ipopi.org

In case of infection

What is the recommended treatment for a PID patient infected with COVID-19?

  • There is currently no data pointing to whether PID patients are at higher risk of more severe disease from COVID-19.
  • The majority of the infected people will experience a mild form of the disease.
  • If a PID patient gets infected with COVID-19 the symptoms should be treated.
  • PID patients should have prompt phone contact with their local healthcare professional if an infection is suspected and follow their advice (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).

What should a PID patient infected with COVID-19 do if they face breathing difficulties?

  • PID patients who suspect that they are infected with COVID-19 should have prompt phone contact with a doctor (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).
  • Immediately get medical attention if you experience any of the following symptoms:
    – Trouble breathing
    – Persistent pain or pressure in the chest
    – New confusion or inability to arouse
    – Bluish lips or face
  • Please note that this list may not include all warning signs. Consult your medical provider for any other symptoms that are severe or concerning.

Is there any anti-viral medicine to treat SARS-CoV-2 available?

Are there treatments that seem more promising than others?

  •  It is too early to say. There are currently approximately 1500 ongoing trials with over 150 in phase 3 (usually designed as randomized double-blind trials aiming at assessing the efficacy and the tolerance of a new drug or combination of drugs versus conventional therapy). Some are coming closer to approval, but more testing is needed before a treatment can be recommended.
  • It is only possible to develop a treatment or a vaccine with reliable data, i.e. from a well-designed clinical trial. Clinical trials are research studies aimed at evaluating a medical intervention. They are the primary way for researchers to find out if a new treatment is safe and effective in people.
  • Clinical trials normally go through three to four phases.
  • During phase I an experimental treatment is tested on a small group of often healthy people (20 to 80) to judge its safety and side effects and to find the correct drug dosage.
  • During phase II more people are included (100 to 300) and the emphasize is on effectiveness. The aim of this phase is to obtain preliminary data on whether the drug works in people who have a certain disease or condition. These trials also study safety, including short-term side effects. This phase can last several years.
  • In phase III further information about safety and effectiveness is gathered, studying different populations and different dosages and using the drug in combination with other drugs. The number of participants usually ranges from several hundred to about 3,000 people (usually less when it comes to rare diseases).
  • After phase III the regulatory agency (e.g.the FDA) will take a decision to approve, or not approve, the experimental drug or device.
  • A phase IV or Post-Marketing Surveillance phase can take place after approval. During this phase the aim is to find out more about long term benefits and side effects. Sometimes, the side effects of a drug may not become clear until many people have used it over a longer period of time.
  • It is also important to differentiate between a drug that has already been tested to treat something else and that now is being repurposed for COVID-19 and a new vaccine where the scientists needs to start from the very beginning.

Can heparin be used to treat COVID-19 infection?

  • One of the specific features of this infection is that it may lead to blood clots in some organs such as the lungs and the kidneys. This can possibly be treated with heparin as it is a blood thinning / antithrombotic drug.
  • This is not a treatment against the virus itself, but against one of the many complications that we learn more about each day.

If a PID patient is infected with SARS-CoV-2, should the general practitioner or local hospital get in contact with the patient’s immunologist?

  • Yes, if an immunologist is available, they should be contacted.
  • Not only may the immunologist have additional ideas and advice, but without them being informed there will be no possibility to collect new data. Collecting data is vital to improve the situation for the future.
  • IPOPI is currently participating in the launch of a 2nd global survey on SARS-CoV-2 and PIDs, working jointly with the International Union of Immunological Societies and other medical societies.
  • Help us by disseminating this survey to your national medical advisers and all national doctors who manage PID patients in your country: https://www.surveymonkey.com/r/67RBPNZ?

If a PID patient with bronchiectasis gets the virus, what is the protocol to follow in a country where knowledge on PIDs is low?

  • The bacteria in the lungs might create a more severe reaction if a PID patient is infected with SARS-CoV-2, therefore additional antibiotics that would normally be prescribed for a person with bronchiectasis might be an option to try for protection quality. It would be advisable to address this already existing bacteria to possibly reduce the severity of the symptoms caused by the COVID-19
  • PID patients should have prompt contact with their local healthcare professional if an infection is suspected and follow their advice (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).

Should special measures be taken for certain PID patients with COVID-19 infection?

  • The majority of the patients will only have a mild form of the disease and so far, there is no data pointing to whether PID patients are at higher risk of more severe disease from COVID-19.
  • Special measures for specific primary immunodeficiencies (CVID, XLA, CGD, WAS, AT, IgG Subclass, IgA def etc.) should be advised by the patient’s PID expert doctor.
  • All PID patients with COVID-19 should monitor the symptoms closely, keep their PID expert doctor informed about their status and follow their advice.
  • All PID patients should 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.
  • For PID patients who have tested positive for COVID-19, it is recommended to perform a second screening after the patient has clinically recovered, as it may be that some PID patients might struggle with clearing the infection. These patients may remain positive longer and risk remaining a source of infection to their environment.

Are there any COVID-19 antibodies in the existing immunoglobulin treatment for PID patients?

  • No antibodies targeting COVID-19 is expected to be contained in the existing Ig preparations.

If a patient with agammaglobulinemia or hypogammaglobulinemia/CVID recovers from COVID-19, will they develop specific T-cells for COVID-19? If yes, how well are they protected from another COVID-19 attack?

  • There is a belief that T-cells might play a role in the immune response against SARSCov- 2 and as these functions very well for XLA patients it is possible. However, it is too early to say how this will affect these patients as the data is not yet available.
  • There is currently no evidence that people who have recovered from COVID-19 and have antibodies are protected from a second infection.

What should PID patients do if someone they live with gets infected?

  • If someone in your family gets infected the likelihood that the disease will be transmitted to the patient increases, despite properly following hygiene measures.
  • It is encouraged to clean and disinfect high-touch surfaces daily in household common areas (e.g. tables, hard-backed chairs, doorknobs, light switches, phones, tablets, touch screens, remote controls, keyboards, handles, desks, toilets, sinks)
  • In the bedroom/bathroom dedicated for an ill person: consider reducing cleaning frequency to as-needed (e.g., soiled items and surfaces) to avoid unnecessary contact with the ill person.
  • As much as possible, an ill person should stay in a specific room and away from other people in their home, following home care guidance.
  • More information available at the Center for Disease Control and Prevention website

Future challenges

What does it mean that a country has reached or exceeded the peak of the virus outbreak?

  • It is misleading to speak about a peak as it is often associated with an expected decline. Instead, based on current epidemiological data across the world, we should refer to it as reaching a plateau where the level of contamination continues to be high but does not increase.
  • There may still be a high number of new cases each day, but when reaching the plateau these numbers will not increase nor decrease for an extended period of time.
  • Due to drastic confinement measures there are now a number of countries who have reached this plateau. Depending on the confinement exit strategies put in place, this may be followed by a decline in infections.
  • It is important to understand that de-confinement measures may lead to a further increase in infections and that patience and caution will be needed.

Do people who recover from COVID-19 create antibodies and become immune?

  • There is currently no evidence that people who have recovered from COVID-19 and have antibodies are protected from a second infection.
  • However, based on experiences from MERS-CoV and SARS-CoV-1 (previous coronaviruses), it is possible that patients who recover from SARS-CoV-2 will develop long-term, but not life-long, antibodies.

When will we know more about the possible increased risks for PID patients and the consequences of the disease for them?

  • IPOPI is currently participating in the launch of a global survey on SARS-CoV-2 and PIDs, COPID19, working jointly with the
  • International Union of Immunological Societies and other medical societies.
  • This is the 2nd part of the survey (launched 31-03-2020), gathering more detailed information.
  • So far there are only a handful PID patients documented in this survey, but the data is still being collected and the numbers will most likely increase.
  • Help us share this survey with your national medical advisers and all national doctors who manages PID patients in your country:

Is there any action taken to improve knowledge on the impact of SARS-CoV-2 for PID patients?

COVID-19 will most likely become a common disease, what does that mean for PID patients?

  • It’s not the first SARS virus outbreak, there were two before in the previous years, one called SARS-CoV and the other MERS-CoV, and there will likely be other SARS virus outbreaks in the future. It is not yet possible to say if this will be the same kind of viral infection, but we will need to live with this current virus in the next years.
  • It is likely that the vaccine will be available in 2021.

How long can a person be protected by antibodies received with convalescent plasma treatment?

  • Convalescent plasma is the plasma obtained from donors who have recently recovered from COVID-19. It is important to note that although promising, convalescent plasma has not yet been shown to be safe and effective as a treatment for COVID-19. Therefore, it is important to study the safety and efficacy of COVID-19 convalescent plasma in clinical trials.
  • There is not yet enough validated data on the protection given by convalescent plasma, but a person receiving it should not be protected by antibodies for more than 1 month.

Some world regions are entering the seasonal flu period, will they have to fight the flu and SARS-CoV-2 at the same time?

  • Yes, both viruses will circulate.
  • If available, it is recommended to take the flu vaccination to ensure protection from one of the two viruses.

Plasma supply

Will there be shortages of immunoglobulin due to possible decrease in plasma donations?

  • The COVID-19 outbreak and associated confinement and movement restriction measures may impact supply of blood and plasma collection and the medicinal product circulation and supply.
  • New national guidelines on masks may also pose a potential risk for plasma collection as donation centres may not be able to fully operate if they face mask shortages.
  • As the plasma necessary to produce PDMPs is either collected from plasma donors (apheresis plasma) or from blood donations (recovered plasma), this will almost inevitably impact the availability of these life-saving therapies. It may take a few months before PDMPs tensions 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.
  • IPOPI and other PID stakeholders are taking measures to react to this development.
  • IPOPI, together with the Platform of Plasma Protein Users (PLUS), are currently in the process of reaching out to every member state in the European Union to highlight the fact that plasma collection centres should be considered critical infrastructure organisations and remain open and functioning during this pandemic.
  • The European Centre for Disease Control and Prevention (ECDC) has released a report has classified blood and plasma (among other substances) as “essential substances of human origin” (SoHO) and state that all precautionary measures possible needs to be taken in order to maintain a safe and high quality supply of these. IPOPI is now included in the list of stakeholders consulted by the ECDC to make sure that the voice of PID patients is considered. The 1st update of the report was released on 29 April, still mentioning plasma as a SoHO.
  • IPOPI has released an open letter with a call to authorities to ensure sufficient blood and plasma supply during and after the COVID-19 outbreak. National member organisations are encouraged to translate this letter and use as a tool when advocating in their national setting.

Are PID stakeholders reacting to a possible drop in plasma donations?

  • Yes, and we encourage our national member organisations to advocate in their national settings.
  • IPOPI, together with the Platform of Plasma Protein Users (PLUS), are currently in the process of reaching out to every member state in the European Union to highlight the fact that plasma collection centres should be considered critical infrastructure organisations and remain open and functioning during this pandemic. Currently all plasma centers in Europe are open (Austria, Germany, Czech Republic, Hungary).
  • The European Centre for Disease Control and Prevention (ECDC) has released a report classifying blood and plasma (among other substances) as “essential substances of human origin” (SoHO) and state that all precautionary measures possible needs to be taken in order to maintain a safe and high quality supply of these. IPOPI is now included in the list of stakeholders consulted by the ECDC to make sure that the voice of PID patients is considered. 1st update from 29 April.
  • IPOPI has released an open letter with a call to authorities to ensure sufficient blood and plasma supply during and after the COVID-19 outbreak. National member organisations are encouraged to translate this letter and use as a tool when advocating in their national setting.
  • Latest updates from the Plasma Protein Therapeutics Association (PPTA) available here

IPOPI’s response to the COVID-19 pandemic

What is IPOPI’s roadmap to tackle the COVID-19 pandemic for PID patients if it rebounds?

Translations of the FAQs

French; Spanish