Patient Guidance drafted 3/31/2020 by
Members of the Medical Advisory Board:
Frederick Askari MD PhD, Regino P. Gonzalez-Peralta MD, Tamir Miloh MD, Michael L. Schilsky MD FAASLD
Dear Wilson disease patients and family members,
In the face of this international emergency due to the novel coronavirus pandemic, we wanted to share the following information that we will hope you will find helpful at this difficult time. Our medical doctors have been receiving many inquiries from our patients, and hopefully this will address some of the frequently asked questions:
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Am I at greater risk for having a bad outcome from COVID 19 disease due to my having Wilson disease?
The Center for Disease Control (CDC) recently included in its high-risk categories for patients contracting COVID 19 infections patients with liver disease. We believe, but do not have evidence as yet, that patients with Wilson disease with well treated and controlled liver disease are not at higher risk than the community. This is an area of interest to the Wilson disease and larger liver disease community, and information is being gathered so that more will be known over time.
We do know that severe COVID19 infections appear to be associated with lower white blood cell counts, and in particular lymphocyte counts. While we don’t have good evidence yet that patients with lowered WBC (white blood cell counts, in particular a lower absolute neutrophil count and lymphocyte counts, both part of the white blood cell differential) may be at a higher risk if they get COVID 19 disease. From our experience with other viral infections they could also have a higher risk of more severe disease. If you are on other medications or have other conditions that lower your white blood cell counts this could theoretically increase your risk independent of your Wilson disease, and this should be discussed with your prescribing physician.
If you do have other high-risk conditions as defined presently by the CDC as older age (above 65 years), have serious heart conditions, diabetes or lung disease (moderate or severe asthma and chronic lung disease), renal failure and liver disease (those with decompensated cirrhosis have an increased risk per AASLD Guidance), morbid obesity (body mass index greater than 40), are immunocompromised or are undergoing cancer treatments you may be at higher risk for a severe illness if you get COVID 19. For individuals in this category, following the CDC recommendations about hand washing and physical distancing from others is very important. It is important for all of us to follow these recommendations so that we do not expose high risk individuals to infection from ourselves.
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Do I need to stop my medications or reduce my medications for my Wilson disease at this time?
No, you do not and should not stop your treatment for your Wilson disease.
It is important that your liver disease remain well treated at this time.
Please contact your treating physician before you make any of the following changes:
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Consider dosage adjustment if copper control is good and your dosage is greater than:
-1000 mg daily of trientine or d-penicillamine in adults
- 15 mg/kg of trientine or d-penicillamine in children
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Consider going to monotherapy with one medication if you are on a combination treatment (zinc and either trientine or d-penicillamine)
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If you have been treated for many years with any of the accepted therapies and a low copper status causes a reduction in your WBC, a dosage adjustment may be considered.
If you are on an experimental treatment for your Wilson disease, your principal investigator or study coordinator will notify you if there are any advisories from the study sponsor.
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What should I do to prevent coronavirus infection?
Standard measures apply – frequent washing of hands with soap and water (wash for 20 seconds minimum time) or with an approved sanitizing agent (ie Purell or others on the accepted lists). Avoid placing your hands near your face. “Social distancing” from others, 6-feet apart, is being recommended, and avoidance of any gatherings at all.
See CDC site for any other recommendations.
If a family member has symptoms or tests positive for the COVID19, then isolation or quarantine may be useful so that you remain uninfected. If you do not have the option for living separately from the infected individual, then careful decontamination of any common areas (bathroom in particular) is important and the affected individual should wear a mask as well.
If you are in a high incidence area for COVID19, consider all necessary precautions and isolate as much as possible. Though there is some controversy regarding whether everyone should wear a mask, this has not yet been recommended by the CDC, but recommendations have been changing as new information is available.
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What should I do if I think I have been exposed?
Please follow all the recommended rules being provided by the CDC and contact your local physician or local COVID hotline for recommendations. Recommendations are being updated frequently.
Contact your local provider or hotline and they will determine if you need screening. Often, they will ask you questions about whether you have fever, cough, loss of smell and taste or other symptoms. If they feel you need testing for coronavirus, they will likely send a prescription to a locally designated site or laboratory that will schedule testing at a at a specific time. There are beginning to be other testing options, such as drive through testing and more rapid testing platforms that will help local facilities turn around the time for results more rapidly. More information on this should be available from your local health centers. Current turnaround time for test results of hours is improved from the original 2-4 days and is improving daily.
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If I have a fever and am not yet sure I have the virus, is there anything I should take?
There are no medications or antivirals available yet that have undergone careful clinical trials that can prevent or mitigate infection. Many agents and biologics are under investigation for COVID19. Some of these treatments are aimed at reducing the viral entry into our bodies, reducing the virus replication and others aimed at reducing the bodies inflammatory response to the virus that sometimes can be harmful to us. We do not recommend taking any additional medications or supplements without your physician’s guidance.
If you are symptomatic and would like to treat a fever, acetaminophen is safer than non-steroidal medications (NSAIDS, ibuprofen, naproxen, and the like). In adults, if you have an impaired liver, do not take more than a total of 2 grams of acetaminophen per day (2 grams = 2000 milligrams or 4 of the extra strength tablets with 500 milligrams each of acetaminophen in a 24-hour period). In children, the safe dosage is 10-15 mg/kg body weight of acetaminophen every 4-6 hours as needed up to 5 doses per day. Initial concerns regarding the lack of safety of NSAIDS and COVID19 disease are not confirmed clinically, however it is a good idea if you have any liver impairment or renal dysfunction to avoid NSAIDS in general.
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Should I wear a mask to prevent coronavirus infection?
The current CDC recommendations are to wear a mask to prevent infections of others if you are coughing or sneezing and think you may be infected however recommendations are subject to change.
Only very specific types of masks may actually prevent uptake of viral particles (see World Health Organization and CDC site links below). These masks are not comfortable to wear for long periods of time. Indeed, if used and disposed of incorrectly, they may even expose wearers to a higher risk of infection. Many providers will wear these masks and other gear as part of the personal protective equipment (PPE) needed when seeing patients with or with suspected coronavirus infections. Hand washing and distancing are your best protections and are more practical to do than using this equipment on an everyday basis.
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I have heard that some medications used to treat hypertension will potentially increase my risk of infection. Is this true?
There was a concern that medications used for hypertensive patients to lower blood pressure that fall into the class of ACE (angiotensin converting enzyme) inhibitors or ARB (angiotensin receptor blockers) may increase coronavirus uptake in patients. This so far is just speculation. If you are on a medication that falls into these categories, there is a recommendation to continue these medications unless recommended by your physician. You should check with your primary care physician or prescribing physician if you have further concerns about these medications.
Resources:
https://www.cdc.gov/coronavirus/2019-ncov/index.html
https://www.who.int/emergencies/diseases/novel-coronavirus-2019/advice-for-public
At this difficult time, we hope that you all will follow the recommendations to be safe and careful. In this way we can protect ourselves, our families and other members of our communities. We will get through this trying time together.
Michael L. Schilsky MD FAASLD
Director, Yale Center of Excellence for Wilson Disease
Frederick Askari MD, PhD
Director, University of Michigan Center of Excellence for Wilson Disease
Regino P. Gonzalez-Peralta MD
Director, Florida Hospital Center of Excellence for Wilson Disease
Tamar Miloh, MD
Director, University of Miami Center of Excellence for Wilson Disease
Dr. Michael Schilsky is the chair of the Wilson Disease Association medical advisory board and is the Professor of Medicine; Medical Director, Adult Liver Transplant at Yale-New Haven Transplantation Center.
Dear Wilson disease patients and family members,
In the face of this international emergency related to the novel coronavirus pandemic, I wanted to share the following information that we will hope you will find helpful. Our medical doctors have been receiving many inquiries and hopefully this will address some of the frequently asked questions:
-
Am I at greater risk for having a bad outcome from the nCovid19 virus due to Wilson disease?
Patients with Wilson disease are not thought to be at higher risk than the community at large unless they specifically have lowered white blood cell counts due to their disease or treatment, in particular we focus on the absolute neutrophil count and lymphocyte counts (part of the white blood cell differential). If you are on other medications that lower your white blood cell counts this could may increase your risk independent of your Wilson disease.
If you do have other high-risk conditions as defined by the CDC (Center for Disease Control) – older age (above 70 years), have heart disease, diabetes or lung disease, you may be at higher risk for a severe illness if you contract the coronavirus. For individuals in this category, following the CDC recommendations closely is very important. It is important for all of the rest of us to follow these recommendations so that we do not expose high risk patients to infection from ourselves.
-
Do I need to stop my medications or reduce my medications for my Wilson disease at this time?
No, you do not. Unless you have been on high dose treatment (15 mg/kg or higher) or combination therapy (zinc and a chelating drug, d-penicillamine or trientine) or have been treated for many years and have a lowered white blood cell count. If you are on an experimental treatment, your principal investigator will notify you if there are any advisories from the study sponsor.
-
What should I do to prevent coronavirus infection?
Standard measures apply – frequent washing with soap and water (wash for 20 seconds minimum time) or with an approved sanitizing agent (ie Purell or others on the accepted lists). Avoid placing your hands near your face. “Social distancing” from others, 6-feet apart, is being recommended, and avoidance of crowds and any meetings or gatherings.
-
What should I do if I think I have been exposed?
Please follow all the recommended rules being provided by the Center for Disease Control.
Contact your local provider and they will determine if you need screening. Often, they will ask you questions about fever, cough or other symptoms. If they feel you need testing for coronavirus, they will likely send a prescription to a local laboratory that will schedule you for the testing at a specific site and at a specific time. There will soon be other options, such as drive through testing which you may access for this. More information on this likely will be available from your local health centers. Current turnaround time for test results of 2-4 days will likely improve with time.
-
If I have a fever and am not yet sure I have the virus, is there anything I should take?
There are no approved medications or antivirals yet that can prevent or mitigate infection. Some agents are in clinical trials at this time. If you would like to treat fever, acetaminophen is thought safer than non-steroidal medications (ibuprofen, naproxen, and the like). If you have an impaired liver, do not take more than a total of 2 grams of acetaminophen per day (2 grams = 2000 milligrams or 4 of the extra strength tablets with 500 milligrams each of acetaminophen in a 24-hour period).
-
Should I wear a mask to prevent coronavirus infection?
The current recommendations are to wear a mask to prevent infections of others if you are coughing or sneezing and think you may be infected. Only very specific types of masks may prevent particulate uptake (see linked WHO information and CDC information below), and these are not comfortable to wear. Many providers will wear these masks and other gear as part of the personal protective equipment needed when seeing patients with or with suspected coronavirus infections. Hand washing and distancing are your best protections and are more practical to do than using this equipment on an everyday basis.
-
I have heard that some medications used to treat hypertension will potentially increase my risk of infection. Is this true?
There is speculation that medications used for hypertensive patients to lower blood pressure that fall into the class of ACE (angiotensin converting enzyme) inhibitors or ARB (angiotensin receptor blockers) may increase coronavirus uptake in patients. This so far is just speculation. If you are on a medication that falls into these categories, there is not as yet a recommendation to stop the medication unless recommended by your physician. You should check with your primary care physician or prescribing physician if you have concerns about these medications.
Resources:
https://www.cdc.gov/coronavirus/2019-ncov/index.html
https://www.who.int/emergencies/diseases/novel-coronavirus-2019/advice-for-public
At this difficult time, I hope that we all will follow the recommendations to be safe and careful. In this way we protect ourselves and members of our communities. We will get through this trying time together.
Michael L. Schilsky MD FAASLD
Chair, Medical Advisory Committee
