MPS and COVID-19 Concerns

Respiratory dysfunction: Pneumonia and ARDS is observed in COVID19 –Difficult intubation and ventilation at baseline: ER/ICU NEEDS TO BE AWARE
–Exacerbate Baseline Pulmonary Dysfunction in MPS
•Abnormal pulmonary function –less reserve
•Obstructive sleep apnea and baseline need for CPAP/BiPAP – know the settings
•CPAP/BiPAP use: can spread COVID19! Beware to caregivers/family

Cardiac dysfunction: Sudden cardiac death, myocarditis, prolonged QT (iatrogenic from experimental use of hydroxychloroquine) are observed in COVID19 patients
–Exacerbate baseline cardiac dysfunction in MPS patients
•Hypertrophic cardiomyopathy
•Valvular disease (stenosis or regurgitation)

Ophthalmologic: conjunctivitis observed
–Baseline corneal clouding, optic nerve involvement in MPS

Kidney failure: need for dialysis in some COVID patients
–MPS typically have normal kidney function

Liver/Spleen: Elevated LFTs observed and an indicator of “worse” outcome for COVID, synthetic dysfunction: hypercoaguable states, DIC observed in severe COVID causing bleeding/clotting issues

Neurologic: Seizures, Increased stroke due to hypercoaguable status of patient, hypoxic ischemic enephalopathy, necrotizing encephalitis, peripheral nerve damage and possible autonomic dysfunction
Monitor:
1. Seizure Disorders, could increase seizures
2. Hydrocephalus fluctuations – COVID-19 includes symptoms of headaches, vomiting, lethargy and sleepiness
3. Nonverbal patients may show increased irritability and behavioral changes.
4. Ensure protection of C spinal during intubation to avoid spinal cord compression.

–Although MPS patients have “normal liver function” we don’t know if organomegaly increases risk or, it may mislead clinicians that COVID-19 is causing abnormal liver activity.