How to manage Von Willebrand's with other co-morbidities?

Question

Hello doctor,

I am a 55-year-old female, 5 feet, weighing 140 lbs, with a complex medical history including von Willebrand disease (types 1 and 2N), rheumatoid arthritis, end-stage osteoarthritis, severe widespread degenerative joint disease (DJD), hypermobile Ehlers-Danlos Syndrome (hEDS) with vascular involvement, hypertrophic cardiomyopathy, and COPD, currently undergoing pulmonary rehabilitation.

Over the past 10 to 14 days, I have experienced worsening joint instability, with increased joint fluidity, slipping, and dislocations. I have had recurrent falls over the past two years, which have become more frequent and resulted in increasingly severe injuries. On the last morning, I fell backwards hard nine times, experiencing confusion, dizziness, headache, and requiring maximum assistance to walk.

At the emergency department, imaging revealed my foot was fractured in four places along with multiple left-sided rib fractures. However, CT and MRI scans of the head, cervical, thoracic, and lumbar spine, as well as a CT angiogram of the chest, were all reported as grossly normal. I was admitted overnight for observation only.

During that time, my hemoglobin dropped from 13.1 to 12.1, and my RBC count decreased from 4.07 to 3.70, though no repeat imaging was performed per my request. My Von Willebrand Disease was not treated, and the hemophilia treatment center was not contacted. I was discharged home last week. At this point, I am seeking guidance on where the clinical focus should go next.

Please help.

Thank you.

Answer 1

Hello,

Welcome to icliniq.com.

I understand your concern.

Given your complex medical background, including Von Willebrand’s disease (types 1 and 2N), Ehlers-Danlos syndrome with vascular involvement, hypertrophic cardiomyopathy, COPD (chronic obstructive pulmonary disorder), and significant osteoarticular instability, the recurrent falls and injuries should raise urgent concern for systemic instability and occult bleeding risk.

The dramatic increase in falls with associated confusion, dizziness, and serious injuries (multiple fractures) strongly suggests either neurological compromise (e.g., autonomic dysfunction, vertebrobasilar insufficiency, or possible seizure activity) or vascular dysregulation. The drop in hemoglobin and RBCs (red blood cells) overnight, coupled with elevated PTT (partial thromboplastin time), raises concern for ongoing or delayed internal bleeding not fully appreciated during initial observation, especially in the context of untreated Von Willebrand’s disorder.

Your care now needs a multidisciplinary approach: urgent hematology referral (with coordination through your hemophilia treatment center) for bleeding risk assessment and possible factor replacement or DDAVP (1-deamino-8-D-arginine vasopressin) trial; neurology for further evaluation of recurrent falls, confusion, and possible syncope or seizure workup (including EEG (electroencephalogram) and tilt-table testing).

Cardiology for re-evaluation of hypertrophic cardiomyopathy’s role in exertional syncope, and rheumatology or orthopedics for mechanical joint instability related to hEDS (hypermobile Ehlers-Danlos syndrome) and RA (rheumatoid arthritis). In parallel, a home safety evaluation and physical therapy for gait stabilization with assistive device prescription (e.g., walker or wheelchair) are critical. You should not be managing this at home alone—request care coordination and possibly short-term rehab or inpatient evaluation through your primary physician or hospitalist for safe discharge planning and further diagnostic workup.

I hope this helps.

Kindly follow up if you have more concerns.

Thank you.