Von Willebrand Disease, A Simple Guide To The Condition, Diagnosis, Treatment And Related Conditions

By Kenneth Kee

This book describes Von Willebrand Disease, Diagnosis and Treatment and Related Diseases
Von Willebrand disease is the most frequent hereditary bleeding disorder.
Causes
Von Willebrand disease is produced by a deficiency of the von Willebrand factor.
The Von Willebrand factor induces blood platelets to clump together and stick to the blood vessel wall, which is required for normal blood clotting
The vWF is a multi-meric glycoprotein encoded by gene map locus 12p13.31
It is produced in the endothelium and kept in Weibel-Palade bodies.
It has 2 major functions:
1.It helps in platelet plug formation by drawing circulating platelets to the site of damage.
2.It attaches to coagulation factor VIII stopping its clearance from the plasma.
It can be congenital or acquired:
1.Hereditary - 3 types and many sub-types.
2.Acquired - also called pseudo-von Willebrand's disease or platelet-type;
It is often found in lymphoproliferative or myeloproliferative disorders and can also be linked with:
a.Solid tumors,
b.Immunological and cardiovascular disorders
Symptoms
This differs depending on the deficiency:
1.Bleeding tendency from mucosa such as:
a.Epistaxis,
b.Menorrhagia
2.Spontaneous bleeding such as:
a.Internal or
b.Joint bleeding (most severe of cases).
3.Blood clots during childbirth
4.Death
Diagnosis
Von Willebrand disease may be difficult to diagnose.
Low von Willebrand factor levels and bleeding should not always indicate that the patient has von Willebrand disease.
Tests that may be done to diagnose this disease are:
1.Bleeding time
2.Blood typing
3.Factor VIII level
4.Platelet function analysis
5.Platelet count
6.Ristocetin cofactor test
7.Von Willebrand factor specific tests
Treatment

Treatment may be DDAVP (desamino-8-arginine vasopressin), a medicine to increase von Willebrand factor level and decrease the chances for bleeding.
DDAVP is not used for all types of von Willebrand disease.
Tests should be performed to determine what type of von Willebrand the patient has.
If the patient is going to have surgery, the doctor may give the patient DDAVP before surgery to see if the von Willebrand factor levels increase.
The drug Alphanate (antihemophilic factor) is also approved to reduce bleeding in people with the disease who must have surgery or any other invasive intervention.
Blood plasma or certain factor VIII preparations may also be given to reduce bleeding.
Patients should be educated on the bleeding risk.
Advice is given regarding drugs that must be stopped such as non-steroidal anti-inflammatory drugs and antiplatelet drugs.
Minor bleeding disorders, such as bruising or a brief nosebleed, may not need any specific treatment.
Treatments to obtain homeostasis in vWD are:
1.Tranexamic acid and
2.Desmopressin or
3.Concentrates having either high-purity vWF alone or
4.Intermediate-purity concentrates having factor VIII-vWF
Tranexamic acid is an agent that fights fibrinolysis.
It can be given:
1.Topically,
2.As a mouthwash,
3.Orally or
4.Intravenously,
5.As a treatment for minor bleeding or
6.Given before surgery, either on its own or
7.As an addition to desmopressin or concentrates.
In women with menorrhagia, combined oral contraceptives or progesterone-containing intrauterine contraceptives often provide significant medical benefit.
Platelet transfusions may be useful in some patients with disease resistant to other therapies.
For prevention in major surgery or for treatment of severe bleeding episodes, vWF-containing factor VIII concentrates are the treatment of choice.
Patients with type 3 vWD and hemarthroses, epistaxis, menorrhagia and other bleeding risk factors need regular prevention with vWF.

TABLE OF CONTENT
Introduction
Chapter 1 Von Willebrand Diseas

• Language: English
• Publisher: Kenneth Kee
• Release date: Mar 11, 2018
• ISBN: 9781370729883

Kenneth Kee

Medical doctor since 1972.Started Kee Clinic in 1974 at 15 Holland Dr #03-102, relocated to 36 Holland Dr #01-10 in 2009.Did my M.Sc (Health Management ) in 1991 and Ph.D (Healthcare Administration) in 1993.Dr Kenneth Kee is still working as a family doctor at the age of 74However he has reduced his consultation hours to 3 hours in the morning and 2 hours inthe afternoon.He first started writing free blogs on medical disorders seen in the clinic in 2007 on http://kennethkee.blogspot.com.His purpose in writing these simple guides was for the health education of his patients which is also his dissertation for his Ph.D (Healthcare Administration). He then wrote an autobiography account of his journey as a medical student to family doctor on his other blog http://afamilydoctorstale.blogspot.comThis autobiography account “A Family Doctor’s Tale” was combined with his early “A Simple Guide to Medical Disorders” into a new Wordpress Blog “A Family Doctor’s Tale” on http://ken-med.com.From which many free articles from the blog was taken and put together into 1000 eBooks.He apologized for typos and spelling mistakes in his earlier books.He will endeavor to improve the writing in futures.Some people have complained that the simple guides are too simple.For their information they are made simple in order to educate the patients.The later books go into more details of medical disorders.He has published 1000 eBooks on various subjects on health, 1 autobiography of his medical journey, another on the autobiography of a Cancer survivor, 2 children stories and one how to study for his nephew and grand-daughter.The purpose of these simple guides is to educate patient on health disorders and not meant as textbooks.He does not do any night duty since 2000 ever since Dr Tan had his second stroke.His clinic is now relocated to the Buona Vista Community Centre.The 2 units of his original clinic are being demolished to make way for a new Shopping Mall.He is now doing some blogging and internet surfing (bulletin boards since the 1980's) startingwith the Apple computer and going to PC.The entire PC is upgraded by himself from XT to the present Pentium duo core.The present Intel i7 CPU is out of reach at the moment because the CPU is still expensive.He is also into DIY changing his own toilet cistern and other electric appliance.His hunger for knowledge has not abated and he is a lifelong learner.The children have all grown up and there are 2 grandchildren who are even more technically advanced than the grandfather where mobile phones are concerned.This book is taken from some of the many articles in his blog (now with 740 posts) A Family Doctor’s Tale.Dr Kee is the author of:"A Family Doctor's Tale""Life Lessons Learned From The Study And Practice Of Medicine""Case Notes From A Family Doctor"

Book preview

Von Willebrand Disease,

A

Simple

Guide

To

The Condition,

Diagnosis,

Treatment

And

Related Conditions

By

Dr Kenneth Kee

M.B.,B.S. (Singapore)

Ph.D (Healthcare Administration)

Copyright Kenneth Kee 2018 Smashwords Edition

Published by Kenneth Kee at Smashwords.com

Dedication

This book is dedicated

To my wife Dorothy

And my children

Carolyn, Grace

And Kelvin

This book describes Von Willebrand Disease, Diagnosis and Treatment and Related Diseases which is seen in some of my patients in my Family Clinic.

(What The patient Need to Treat Von Willebrand Disease)

This eBook is licensed for your personal enjoyment only. This eBook may not be re-sold or given away to other people. If you would like to share this book with another person, please purchase an additional copy for each reader.

If you’re reading this book and did not purchase it, or it was not purchased for your use only, then please return to Smashwords.com and purchase your own copy.

Thank you for respecting the hard work of this author.

Introduction

I have been writing medical articles for my blog http://kennethkee.blogspot.com (A Simple Guide to Medical Disorder) for the benefit of my patients since 2007.

My purpose in writing these simple guides was for the health education of my patients.

Health Education was also my dissertation for my Ph.D (Healthcare Administration).

I then wrote an autobiolographical account of his journey as a medical student to family doctor on his other blog http://afamilydoctorstale.blogspot.com.

This autobiolographical account A Family Doctor’s Tale was combined with my early A Simple Guide to Medical Disorders into a new Wordpress Blog A Family Doctor’s Tale on http://kenkee481.wordpress.com.

From which many free articles from the blog was taken and put together into 800 eBooks.

Some people have complained that the simple guides are too simple.

For their information they are made simple in order to educate the patients.

The later books go into more details of medical disorders.

The first chapter is always from my earlier blogs which unfortunately tends to have typos and spelling mistakes.

Since 2013, I have tried to improve my spelling and writing.

As I tried to bring the patient the latest information about a disorder or illness by reading the latest journals both online and offline, I find that I am learning more and improving on my own medical knowledge in diagnosis and treatment for my patients.

Just by writing all these simple guides I find that I have learned a lot from your reviews (good or bad), criticism and advice.

I am sorry for the repetitions in these simple guides as the second chapters onwards have new information as compared to my first chapter taken from my blog.

I also find repetition definitely help me and maybe some readers to remember the facts in the books more easily.

I apologize if these repetitions are irritating to some readers.

Chapter 1

Von Willebrand Disease

What Is Von Willebrand Disease?

Von Willebrand disease is the most frequent hereditary bleeding disorder.

This is the most frequent hereditary coagulopathy disease in humans.

Incidence

Incidence is as high as 1-2% in the general population on random screening.

Worldwide incidence is about 125 per million with between 0.5 and 5 per million being severely affected.

The majority of patients have mild disorder.

It is more frequent in females.

It is more serious with blood type O.

What are the causes of Von Willebrand