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My Son Has Wegener's Granulomatosis
My 28-year old adopted son was diagnosed in December 2013, with this dreaded disorder. Wegener's granulomatosis is a very rare and serious disease. In simple layman’s language, the disease involves one’s own immune system attacking oneself. From statistics, only 10 in a million per year will get this disease. And 97% of those affected are Caucasians, and mainly middle-aged. Based on the simple law of probability, my son who is dark skinned would not have any chance of contracting this disease. But fate would want it otherwise.
It all started with his nasal problem which led to some bleeding. On examination, the ENT specialist discovered massive growth around the hollow of the nasal passage. He took a specimen and sent for a biopsy. We were very worried for his condition which the doctor forewarned to be serious. So when the result came and it was found to be non-malignant, we were relieved and overjoyed.
Then the next appointment came, and the doctor performed an extensive nasal surgery on my son to clear off all the mess in the nasal passage. This time the doctor looked worried as the growth he removed was extensive, and he did not hide his concern over my son’s case. Another specimen was sent to the pathologist again. This time the results were bad. The pathologist suspected the disease called Wegener’s granulomatosis and suggested a further blood test called ANCA, to identify the disease. The final result confirmed that my son had the disease called Wegener's granulomatosis with polyangiitis.
Definition Of Wegener's Granulomatosis With Polyangiitis
This disease was previously known as Wegener's granulomatosis. It is named after Friedrich Wegener who described the disease in 1936. Wegener was accused of his Nazi past record, and the medical fraternity has since changed the name of the disease as “granulomatosis with polyangiitis” or GPA. However, for all practical purposes the term “Wegener’s disease” is conveniently and unofficially used to describe GPA. Often times it is also called “Wegener's granulomatosis” or simply “WG”. For this article I shall use the term “Wegener's granulomatosis”.
Wegener's granulomatosis is a very rare and serious disease which disorientate the immune system to attack its own organs. The main characteristic of this disease is inflammation of the blood vessels, narrowing them and restricting blood flow to the vital organs. This inflammation produces a special inflammatory tissue called “granuloma” that appears around the blood vessels. These granulomas will destroy the normal tissues causing severe damage to the vital organs. The main areas of attack are the respiratory system, the lungs and the kidneys.
My son’s first symptom was his chronic nasal problems, like running nose and blocked nose, which he was having for many years but he was adamant to seek treatment giving lame excuses that it was just a normal nagging problem. The first sign of the seriousness came with his nasal congestion and bleeding from the nose. That was when he consulted the ENT specialist and the subsequent diagnosis.
There is no known cause for Wegener's granulomatosis. As such, there is also no known preventive measures for Wegener's granulomatosis. However, it is not contagious. Early diagnosis and proper treatments may lead to a full recovery, although relapses may take place. Without proper treatments, the disease is fatal, most commonly from kidney failure.
Causes And Symptoms Of Wegener's Granulomatosis With Polyangiitis
There is no known viral, bacterial or other causative agents found to have caused Wegener's granulomatosis. The triggering event may be in the form of an infection, but no specific infection has been identified as the cause. Wegener's granulomatosis is diagnosed as an autoimmune disease in that the person’s immune system attacks itself instead of attacking external infectious agents. Proteins called antibodies are formed in the blood to defend the body against harmful external agents. However, in the case of the Wegener’s sufferer, these antibodies are directed against the white blood cells of the immune system. Such antibodies are called "auto-antibodies" as they attack one’s own body cells. Gradually, these "auto-antibodies" will bind to the blood cells forming tumor-like inflammation in the blood vessels. Hence the term “granulomas” is derived. This accumulation of tissues will restrict the blood flow to the various organs and tissues, resulting in the signs and symptoms of Wegener's granulomatosis.
In my son’s case, the symptoms appeared in the nasal passage, with great mass of dead tissue forming in the nasal passage, ultimately causing the nose to bleed. Different patient will experience different symptoms. My son’s symptom was one of the more common features. The nasal tissue growth has affected his hearing too. Other symptoms may include lung infection, weight loss, coughing of blood, fever, loss of appetite, joint pain, change in urine color, night sweats, and general feeling of weakness. Kidney disease is the most severe symptom of Wegener's granulomatosis. Patients who do not have kidney disease are termed as having "Limited Wegener's".
Biopsy Results Of My Son's Tissue Sample
My Son's First ANCA Test Results
My Son's Second ANCA Test Results
Tests And Diagnosis
Wegener's granulomatosis is one disease that has no specific test to definitely confirm the presence of the disease in the patient. It is more of a process of elimination to rule out all the other possible diseases, and when none is present, then Wegener's granulomatosis will be the most probable determination. The first diagnosis is through biopsies of the affected tissue. Then the other comprehensive tests will include blood tests, urine tests for protein and red blood cells and to rule out any kidney disease, CT scans, chest and sinus X-rays to detect any abnormalities resulting from lung and sinus inflammation and any lung disease and MRI profiling. The diagnosis of Wegener's granulomatosis is confirmed when both abnormal cellular formations, called granulomas, and vasculitis in a biopsy of tissue involved with the inflammatory process are detected.
My son had to undergo all these tests, including TB and HIV tests. After all these tests were confirmed negatives, that the doctor confirmed the diagnosis as Wegener's granulomatosis with polyangiitis, with the crucial cANCA test which showed positive.
The ANCA blood test is the closest the diagnosis can get to determine Wegener's granulomatosis. ANCA is short for anti-neutrophil cytoplasmic antibodies. The presence of ANCA is seen in 90% of WG patients. ANCA is a specific type of antibodies that attack the patient’s own white blood cells causing inflammation and are also present in HIV patients. As a measurement of progress of Wegener's granulomatosis, the ANCA test is the main tool. A decrease in the levels of ANCA in the blood relates to recovery, while an increase will signify a flare-up.
My Son's Medication:Prednisolone
My Son's Medication:Conpanzole
My Son's Medication:Osteocap
My Son's Medication:Calcium Carbonate
Treatment And Medication
There is hope for full recovery following proper treatments within a few months. Maintenance therapy will continue for another 24 months. As the Wegener's granulomatosis is a very serious disease, life-long medical monitoring is mandatory.
The following medications are prescribed for WG patients.
Corticosteroids. Medications such as prednisone or prednisolone, are used to suppress the immune system and as initial treatment. Corticosteroids are used to inhibit the inflammatory reaction to a variety of inciting agents.
Immune suppressant medications. These are cyclophosphamide (Cytoxan), azathioprine (Azasan, Imuran) or methotrexate (Rheumatrex, Trexall), to counteract the body's immune reaction. These are powerful and toxic drugs and may have serious side effects. These medications are generally called chemotherapy treatment, administered through intravenous under close supervision of the medical doctors.
Complementary medications. Because of possible side effects and risk of infections, other drugs are also prescribed to counter these risks. Some of these are listed below:
Sulfamethoxazole-Trimethoprim (Bactrim, Septra) to prevent lung infection.
Bisphosphonates (Fosamax) to prevent bone loss (osteoporosis) associated with prednisone use.
Folic acid, a synthetic form of the B vitamin folate, to prevent sores and other signs and symptoms associated with the depletion of folate from methotrexate use.
My Son’s Treatment And Medication
My son’s main medications are as below:
Prednisolone, a form of steroid for treating a wide range of inflammatory and auto-immune conditions.
Cyclophosphamide, a chemotherapy drug administered intravenously (IV). This drug has severe and life-threatening adverse effects such as leukemia, bladder cancer, hemorrhagic cystitis, and permanent infertility. For autoimmune diseases, doctors often substitute less-toxic drugs, but for my son’s condition, the doctor prescribed this drug. The treatment protocol is for 6 doses. The first 3 doses have been given fortnightly each time. The next 3 doses will be administered once in three weeks. So far my son has responded positively, without any side effect symptom, except once when he vomited.
Complementary medications are conpanzole (containing 40mg of pantoprazole) to prevent gastric problem. Another drug he is taking is osteocap (containing 0.25 mcg) for absorption of calcium; plus calcium carbonate (500mg) to strengthen his bones against osteoporosis. In addition, the ENT doctor who performed the initial nasal surgery also prescribed a number of antibiotics for prevention of infection.
Other treatments available include the following;
Rituximab (Rituxan) which is a drug aimed at reducing B cells which have to do with inflammation. Rituximab may prove to be as effective as cyclophosphamide, and may even be better for treating relapsed cases.
Plasmapheresis or plasma exchange, which replaces the blood plasma of the patient. This treatment may help the kidneys to recover.
Surgery for those with kidney failure, possibly kidney transplant.
Other experimental drugs such as mycophenolate mofetil (CellCept), infliximab (Remicade), intravenous immunoglobulin, deoxyspergualin and antithymocyte globulin.
Prognosis And Reference Links
With new and more effective drugs, WG patients now have better chance of recovery and to live a normal life. In the past, 80% of untreated WG patients died within a year, and another 10% died the second year. Approximately 50% of WG patients will have a relapse within 2 years after stopping the medication. The relapse may occur at any time. As such, it is most important that WG patients maintain regular check-ups for the rest of their lives.
A Final Personal Observation
This is my personal observation and opinion regarding this very rare disease, specifically in relation to my son’s condition. It is not based on any scientific research but purely on my personal belief, observation and analysis. As I have often suggested in many of my wellness articles, our mental condition has a great influence in our well-being and health. If we habitually generate negative mental thoughts, such as anger and resentment, we are like injecting poison into our system, gradually making our own bodies sick. Such people have negative aura and negative vibrations. Likes attract likes. And a negative attitude attracts negative forces. Over time, our bodies become a welcoming home for disease-bearing germs and viruses. The end result? We succumb to serious diseases. The strength and health of our body immune system depend very much on our mental foundation. If our minds are flooded with negative emotions and influences, our body systems will not function smoothly; every organ is negatively affected, and our blood circulation will likewise be restricted. The immune system gradually weakens and malfunctions. Wegener's granulomatosis is the manifestation of the body immune system malfunctioning or gone crazy, to attack its host, instead of attacking foreign agents.
It is my personal belief that one area of the prognosis is the ability of the patient to discard the negative and dark side of life, and to focus on the bright side and positive aspect of life. People close to the patient will have to provide the right positive environment for the patient to heal himself mentally. A lot of tender loving care will be needed. May I go a step further; a spiritual reawakening will certainly help the patient to recover completely and to lead a full, happy and healthy life. My belief is that the patient will definitely do well if he embarks on the three-prong therapy, namely, effective medical treatment, positive mental attitude, and unwavering spiritual rejuvenation. In this time of traumatic condition, spiritual fellowship support will help immensely.
In the final analysis, the patient will need to be awaken to take charge of his life. While help and support from all avenues are a great contributing factor, the patient himself must make the positive effort to want to recover to live a useful and a healthy life.
After Nearly 2 Years!
Update On My Son's Condition And Progress
As at to date, 16 July, 2015, my son is recuperating. The past 1 year has been an excruciating experience. He was in ICU three times, and survived!
The first was because of pneumonia, and he recovered after 9 days. Subsequently he was discharged but not after a minor surgery to drain out excess fluid from his right lung. He never fully recovered, and after a few weeks he got worse, and had to be admitted to the hospital again. Apparently the infection of his lungs has gotten worse, and he was coughing terribly, coughing out fluid. He was operated on his lungs and sent to ICU again to recover. This time another nine days again. He came out with an "open window" wound on his right lung. This "open-window" wound required daily dressing at the clinic even though he was discharged. This went on for another few weeks, but he did not recover. Then he developed fever and coughs. He was again admitted to the hospital and this time his condition deteriorated due to severe infection on both lungs. He was admitted to ICU again for the third time. Various types of strong antibiotics were administered. This time, he was there for 11 days. Each time in ICU we thought he was a goner, but each time he survived. This was a miracle, three times over! At present my son is recuperating but still has the intermittent coughs. Apart from that he seems to be stable. Let's hope for the best.
My son has been given a course of Rituximab and will be given the second course in due time. In addition, he has also consulted a homeopathy physician and is taking supplementary medication prescribed by the physician. (This update is on Oct 8, 2015, which is incidentally, my son's 29th birthday.)
Updates : 25 December 2015 and 24 April 2016
Today is Christmas Day. I thought it auspicious to run an update on my son's health progress. He was given another dose of Rituximab a few weeks ago. Apart from the conventional Western medical treatment, he has been to the acupuncturist and also tried moxibustion. We have a machine used by my wife for many years and now my son is also using it. It is called the "electro static" health equipment. We also have an alkaline water dispenser and my son is also drinking the alkaline water periodically. Alkaline water is supposed to aid maintaining a healthy body.
My son's current chronic problem is his periodic coughs. Apparently the very aggrassive MRSA bacteria are still lying latent in his lungs. So he needs to be extra careful and vigilant in his personal hygiene.
Latest update : 24 April 2016. We received good news that the sputum test has shown negative bacteria invasion. This means at least although he coughs, there is less or no risk of bacteria contamination.
You are invited to read the sequel to this article about the "Spiritual Upliftment Of My Son".
Sequel To This Article
You are invited to read the sequel to this article about the "Spiritual Upliftment Of My Son".
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Justin Choo (author) from Malaysia on July 10, 2016:
Thank you my friend, Eddie, for the positive encouragement.
Eddie Leong on July 09, 2016:
I will throw every herbal treatment I can against an auto-immune disease. There are many types of auto-immune disease and as of today there are often said to no cure. The only recourse is to hope that non-conventional remedies will help.
I have researched on virgin coconut oil and how drinking many tablespoon has helped in supposedly chronic cases.
A new stemcell technique called CRISPR/Cas System is being progressed in USA and the West. This involves snipping off segments of the cell's genome. This is showing much promise for treatment of diseases.
I am in contact with a stemcell expert in USA on something else and can ask him about whether there is any research done on Wegener disease.
Keeping attacking the problem. Nothing to lose.
Justin Choo (author) from Malaysia on July 09, 2016:
Thanks for sharing your health regime. You can read my articles on Moringa and SSG too.
Eddie Leong on July 09, 2016:
I am a great believer in Urine Therapy. Have be a UT user for 15 years, in very good health, have not been to see the Doc for many years now.
UT may or may not be the answer but it is my own urine that helps to greatly boost my own immune system. Lately I have started on Moringa powder, am eating a few Sabah Snake Grass leaves daily. Also have started to use Virgin coconut oil.
I believe whatever may be the disease and whatever may be cause.. genetic, infection, etc. it is our immune system that will determine whether we survive or get well.
Justin Choo (author) from Malaysia on July 12, 2015:
Great to hear that you are one of the survivors. Yes, this illness will hang over my son's head for the rest of his life. His greatest hurdle is to change his attitude and improve on his personal hygiene. There is a limit that we as parents can help him, and take care of him. But in the final countdown, he has to take control of his life. He is coming to 30. Now he is living with us. He is recovering but still coughing. Both his lungs were attacked, and he was in ICU three times. The fact he survived so far is itself a "miracle".
Don, thanks for the sharing. It gives greater hope for my son. Take care and be healthy.
Don Bobbitt from Ruskin Florida on July 12, 2015:
Great article. I was attacked with this insidious disease in 1993 and it almost took me out.
In fact, it did damage my kidneys to the point that I needed to have a kidney transplant in 1996. Luckily, the disease has not reoccured since then.
And, I agree with your suggestions. I am confident that my positive attitude during the worst of the disease, and my personal changes to my diet were significant in the quality of my life since those days,
Good Luck to your son. This disease will hang over his head for the rest of his life and only he can fight it successfully.
Justin Choo (author) from Malaysia on August 30, 2014:
Good afternoon Linda,
Thank you for your visit and comment. Yes, I do hope they find an effective cure soon.
Linda Crist from Central Virginia on August 30, 2014:
First, let me say that I am so sorry that you and your son have had to learn about Wegener's the hard way. Next, I want to thank you for sharing such a personal story. Few know about the disease and you did an excellent job of defining it and describing the journey of diagnosis and treatment. I was aware of Wegener's but learned a lot from your article. Having been diagnosed with a serious illness many years ago, and overcoming it thankfully, I know the fear all too well. But I also know that for every day that passes there is a scientist in a lab looking for a cure. We need hope above all else.