A Simple Guide To Mucoviscidosis (Cystic Fibrosis), Diagnosis, Treatment And Related Conditions

By Kenneth Kee

This book describes Mucoviscidosis (Cystic Fibrosis), Diagnosis and Treatment and Related Diseases

Cystic Fibrosis (Compendium of Medical Poems by Kenneth Kee 2016)

Cystic fibrosis is an inherited recessive genetic disease
Sticky mucus blocked the lungs and airways with ease
It also blocks ducts in the pancreas and digestive tract
As a result the intestines cannot fully absorb proteins and fats

This can result in vitamin deficiency and malnutrition
There will be bulky stools, intestinal gas and constipation
There is also pain or discomfort in the abdomen.
The sticky mucus can cause all forms of digestion problem

Sweating will cause the loss of salt resulting in dehydration
CF also causes infertility in men by blocking the secretions
This disease can make it harder for women to get pregnant.
An early diagnosis and treatment of the illness is very important

Treatment is with inhalers and medicine to open the air tube obstruction
Pancreatic enzymes are given to help the protein and fats digestion
A rare complication is chronic lung obstruction
There may also be poor absorption and nutrition

-An original poem by Kenneth Kee
Cystic Fibrosis is well known throughout the world for its health complications resulting from the production of mucus that is much thicker and more sticky from the exocrine glands in the lungs and digestive system.

This results in blockage of the ducts of the digestive system and airways with abnormal widening (cyst formation) and scarring of the organ tissues (fibrosis) seen especially in the pancreas.

Cystic fibrosis is named due to the cysts and fibrosis in the pancreas.

A better name for this disorder would be mucoviscidosis because of the thick and viscous mucus.

From ancient times, children around the world have been afflicted with mucoviscidosis (cystic fibrosis) that leads to shortened lifespan.

In medieval Europe, these children were thought to be cursed by witches.

Salty skin was a sign of an imminent disease without known cause or cure.

“Woe to the child who tastes salty from a kiss on the brow, for he is cursed and soon will die.”

High levels of salt in the sweat of patients with mucoviscidosis indicated an abnormality in electrolyte transport from the sweat gland.

It is suggested that sweat ducts in these patients were not permeable to chloride.

The faulty chloride channel is believed to be situated in the apical membranes of the lung surface or glandular epithelium to explain the respiratory and systemic organ failure linked with mucoviscidosis.

Mucoviscidosis (cystic fibrosis) is an inherited recessive genetic disorder that causes thick, sticky (viscous) mucus to accumulate in the lungs, digestive tract, and other secretary glands such as salivary, sweat, sinuses, liver and sex organs.

Mucoviscidosis is caused by defects in the cystic fibrosis gene, which codes for a protein transmembrane conductance regulator (CFTR) that functions as a chloride channel and is regulated by cyclic adenosine monophosphate (cAMP).

Medicines used to treat patients with mucoviscidosis (cystic fibrosis) may be pancreatic enzyme supplements, multivitamins (fat-soluble vitamins), mucolytics, antibiotics (inhaled, oral, or parenteral), bronchodilators, anti-inflammatory agents, and CFTR potentiators (e.g., ivacaftor) and correctors (e.g., elexacaftor, lumacaftor, tezacaftor).

Cystic fibrosis transmembrane conductance regulator (CFTR) potentiators are the first available treatment that acts on the defective CFTR protein, the underlying cause of cystic fibrosis.

There is no cure for mucoviscidosis

Better medical and surgical treatment methods have improved the mean survival age to 37 in recent years.

TABLE OF CONTENT
Introduction
Chapter 1 Mucoviscidosis (Cystic Fibrosis)
Chapter 2 Causes
Chapter 3 Symptoms
Chapte

• Language: English
• Publisher: Kenneth Kee
• Release date: Feb 11, 2021
• ISBN: 9781005805388

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

A

Simple

Guide

To

Mucoviscidosis

(Cystic Fibrosis),

Diagnosis,

Treatment

And

Related Conditions

By

Dr Kenneth Kee

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

Ph.D (Healthcare Administration)

Copyright Kenneth Kee 2021 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 Mucoviscidosis (Cystic Fibrosis), Diagnosis and Treatment and Related Diseases which is seen in some of my patients in my Family Clinic.

(What You Need to Treat Mucoviscidosis)

This e-Book 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 autobiography account of my journey as a medical student to family doctor on my other blog: http://afamilydoctorstale.blogspot.com.

This autobiography 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.

My diagnosis and treatment capability has improved tremendously from my continued education.

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

Mucoviscidosis (cystic fibrosis)

Woe to the child who tastes salty from a kiss on the brow, for he is cursed and soon will die.

Cystic Fibrosis (Compendium of Medical Poems by Kenneth Kee 2016)

Cystic fibrosis is an inherited recessive genetic disease

Sticky mucus blocked the lungs and airways with ease

It also blocks ducts in the pancreas and digestive tract

As a result the intestines cannot fully absorb proteins and fats

This can result in vitamin deficiency and malnutrition

There will be bulky stools, intestinal gas and constipation

There is also pain or discomfort in the abdomen.

The sticky mucus can cause all forms of digestion problem

Sweating will cause the loss of salt resulting in dehydration

CF also causes infertility in men by blocking the secretions

This disease can make it harder for women to get pregnant.

An early diagnosis and treatment of the illness is very important

Treatment is with inhalers and medicine to open the air tube obstruction

Pancreatic enzymes are given to help the protein and fats digestion

A rare complication is chronic lung obstruction

There may also be poor absorption and nutrition

-An original poem by Kenneth Kee

What is Mucoviscidosis (cystic fibrosis)?

Mucoviscidosis (cystic fibrosis) is an inherited recessive genetic disorder that causes thick, sticky (viscous) mucus to accumulate in the lungs, digestive tract, and other secretary glands such as salivary, sweat, sinuses, liver and sex organs.

Mucoviscidosis (cystic fibrosis or CF) is a serious genetic disorder that induces severe damage to the respiratory and digestive systems.

This injury often happens from an accumulation of thick, sticky (viscous) mucus in the organs (Mucoviscidosis).

The most often involved organs are:

1. Lungs

2. Pancreas

3. Liver

4. Intestines

Mucoviscidosis (cystic fibrosis) involves the cells that produce sweat, mucus, and digestive enzymes.

Normally, these secretions are thin and smooth like olive oil.

They lubricate various organs and tissues, stopping them from getting too dry or infected.

In people with mucoviscidosis (cystic fibrosis), a faulty gene induces the fluids (mucus) to become thick and sticky (viscous).

Instead of working as a lubricant, the fluids obstruct the ducts, tubes, and passageways in the body.

This can lead to life-threatening disorders, such as infections, respiratory failure, and malnutrition.

It is important to get treatment for mucoviscidosis (cystic fibrosis) right away.

Early diagnosis and treatment are essential for improving quality of life and increasing the expectancy of life.

About 1,000 people are diagnosed with mucoviscidosis (cystic fibrosis) every year in the USA.

While people with the disorder need daily treatment, they can still have a relatively normal life and work or attend school.

Screening tests and treatment methods have become better in recent years; so many people with mucoviscidosis (cystic fibrosis) can now live into their 40s and 50s.

What is the cause of Mucoviscidosis (cystic fibrosis)?

Cause

Mucoviscidosis (cystic fibrosis) is one of the most frequent chronic lung diseases in children and young adults.

Mucoviscidosis (cystic fibrosis) happens as a result of a deficiency in what is termed the cystic fibrosis transmembrane conductance regulator gene, or CFTR gene.

This gene regulates the movement of water and salt in and out of the body’s cells.

A sudden mutation, or change, in the CFTR gene induces the mucus to become thicker and stickier than it is supposed to be.

This abnormal mucus builds up in various organs throughout the body, such as the:

1. Lungs

1. Intestines

3. Pancreas

4. Liver

It also increases the quantity of salt in the sweat.

Many different defects can have an effect on the CFTR gene.

The type of defect is linked with the severity of mucoviscidosis (cystic fibrosis).

Mucoviscidosis (cystic fibrosis or CF) is produced by a defective recessive gene which is passed through the families.

The defective gene is passed on to the child from their parents.

In order to have mucoviscidosis (cystic fibrosis), a child must inherit one copy of the gene from each parent.

If they only inherit one copy of the gene, they will not develop the disease.

However, they will be a carrier of the defective gene, which means that they may pass the gene on to their own children.

Risk factors for mucoviscidosis (cystic fibrosis)

Mucoviscidosis (cystic fibrosis) is most frequent among people of Northern European descent.

However, it is known to happen in all ethnic groups.

People who have a family history of mucoviscidosis (cystic fibrosis) are also at a higher risk since it is an inherited disorder.

It induces the body to produce abnormally thick and sticky (viscous) fluid called mucus that builds up in the air passages and blocks the breathing tubes of the lungs.

The thick sticky mucus also can obstruct the ducts in the pancreas (the organ that helps to break down and absorb food through its enzymes).

This prevents the digestive enzymes made by the pancreas to reach the small intestine.

Without these enzymes the walls of the small intestines cannot fully absorb fats and proteins.

This leads to vitamin deficiency and malnutrition since the nutrients cannot be absorbed.

Bulky stools, intestinal gas, severe constipation, and pain or discomfort in the abdomen will result.

The sticky mucus leads to blockage of the air tubes in life-threatening lung infections and serious digestion problems.

The disease may also involve the sweat glands which is very high in sodium.

Sweating will induce the loss of a large quantity of salt resulting in dehydration and imbalance