In The Beginning . . .
Today, Wednesday July 1, 2015, I am sitting in front of my computer after a day ill with vertigo. I have been resting and now feeling slightly better.
Today, Wednesday July 1, 2015, I am sitting in front of my computer after a day ill with vertigo. I have been resting and now feeling slightly better.
However, I want to write this blog about living with a primary immune deficiency, which in my case is the granulomatous variant of common variable immune deficiency. To abbreviate this I will use the initials CVID.
I am 73 years of age, and as I look back over the years I acknowledge that I have experienced considerable ill health. I was fostered and my foster mother took responsibility for me when I was two weeks old. My foster mother told me that I was a pale and sickly child and it was considered that I would not survive childhood.
I can recall as a child being in hospital for the removal of my tonsils and adenoids. I also recall that from the age of five years I seemed to spend a lot of time in doctor's surgeries, being treated for asthma, upper respiratory infections, sinusitis, and ear infections. Consequently I was always on the antibiotic that was available in the late 1940s. My foster mother must have had some concern about my health because we used to travel a considerable distance to doctors' surgeries, on the tram and then by walking. At that time we were living in a suburb that was basically all bush and trees, with a few houses along a beach line.
I was also a suffer of migraines, and had an episode of this almost every second week. This continued until I was in my mid-30s. Migraines were not properly diagnosed in the 1940s-1950s so I was considered to be having bilious attacks. Consequently I was always dosed up with cod liver oil. The first meal I had after the migraine was over was toast and vegemite, and this is probably why I love toast with vegemite so much!
As I continued through my teenage and early adult years I was continually in a doctor's surgery being treated for some bacterial lung infection. I would be treated with penicillin and in most times I was close to contracting pneumonia. I would try to make sure I was not in an environment were people were coughing or had influenza, but it seems to make no difference. When flu shots became available I would always make sure I received the vaccine each year, but still had infections. One of the reasons we moved from New Zealand to Queensland, Australia, was because of warmer weather, and an environment that we hoped would reduce the number of lung infections.
In November 1998, Beryl and I moved to Maryborough, Queensland, having purchased a lease on a motel in that city. We worked hard and in my view did a remarkable job of running the motel. We had casual help in cleaning, but we ran the front of house operation, did most of the cleaning, reservations and kept the financial records etc.
In early 2000s I had my appendix removed. Apparently I had had appendicitis flare-ups, but because of the amount of antibiotics I was taking the appendicitis cleared up, but left behind scar tissue which attached itself to the bowel. I am grateful that my GP made a correct diagnosis even though the surgeon did not think I had appendicitis but decided to operate since I had been admitted to the private hospital.
As I continued through my teenage and early adult years I was continually in a doctor's surgery being treated for some bacterial lung infection. I would be treated with penicillin and in most times I was close to contracting pneumonia. I would try to make sure I was not in an environment were people were coughing or had influenza, but it seems to make no difference. When flu shots became available I would always make sure I received the vaccine each year, but still had infections. One of the reasons we moved from New Zealand to Queensland, Australia, was because of warmer weather, and an environment that we hoped would reduce the number of lung infections.
In November 1998, Beryl and I moved to Maryborough, Queensland, having purchased a lease on a motel in that city. We worked hard and in my view did a remarkable job of running the motel. We had casual help in cleaning, but we ran the front of house operation, did most of the cleaning, reservations and kept the financial records etc.
In early 2000s I had my appendix removed. Apparently I had had appendicitis flare-ups, but because of the amount of antibiotics I was taking the appendicitis cleared up, but left behind scar tissue which attached itself to the bowel. I am grateful that my GP made a correct diagnosis even though the surgeon did not think I had appendicitis but decided to operate since I had been admitted to the private hospital.
In October 2004 Beryl and I organised a weekend down on the Gold Coast to attend our grandson's 21st birthday. Family had come over from New Zealand and other members of the family came up from Canberra. During the weekend I felt quite ill and noticed that I was passing dark urine. On the night of the 21st birthday party, which was held in Woodside, I became very sick and my body must have been in some form of shock. I had uncontrollable shaking, and could not get warm. We returned to the motel at Broadbeach, but I was unaware of travelling back there. The next day I felt a little better and we returned to Maryborough as had been planned.
On Monday morning, after cleaning the motel, I went to our GP and he said that I could either have a blood test and then go to hospital, or go to hospital and then have a blood test. I chose to have the blood test, and after this returned to the motel to continue working. By now, I was passing urine that was almost black. Around 2pm my GP called and told me to go straight to St Stephen's Private Hospital. I was on IV antibiotics for one week, with nil by mouth. I had a severe liver infection.
After discharge from hospital I returned to working in our motel. We eventually sold the lease on our motel in April 2006, having bought a house in an Over-50s Residential Park in Hervey Bay six months earlier in anticipation of selling the lease on the motel.
After settling into our home in the village, my health started to deteriorate. I had been experiencing considerable pain in my left knee while I was still in the motel, and an orthopaedic surgeon had been giving me injections into the knee to relieve the pain. After we left the motel I returned to the orthopaedic surgeon for further examination as the injections were not working. An X-ray revealed that the top of the bone above the knee cap had broken off and it was decided to operate and insert an hemiCAP. This is a screw that was inserted into the bone which filled the hole caused by the bone breaking away.
I applied for disability pension and was granted this, and it was actually paid by the New Zealand pension system. Around the same time I started having lesions forming subcutaneously, and I was referred to a physician in Hervey Bay who diagnosed that I had sarcoidosis. Sarcoidosis responds to prednisone, and so I started taking a light dose of prednisone. This did not help at all, and in fact after one month I found that I was losing my eyesight.
As a result of this, I had to have my cataracts removed surgically and also with laser treatment as there was a build up of prednisone crystals that could not be removed by surgery. The physician had to rethink what was causing my subcutaneous lesions, which had been excised. Around the same time I was referred to a gastroenterologist and had an endoscopy. There were some small lumps removed, which were benign.
However, I was experiencing considerable weight loss, and I had lost 20kg. I had liver pain and a biopsy was taken of the liver, and I had lobular hepatitis. However, I continued to be unwell and the gastroenterologist said he could not help me any further and referred me to Wesley Private Hospital in Brisbane, under the care of an interventional gastroenterologist. I was in Wesley for a total of three weeks, and a considerable amount of tests were undertaken. One of my major problems was gut pain, but nothing showed up in the CT scans, ultrasound or MRI.
Although nothing showed up on the imaging it was decided that I would have a diagnostic laparoscopy on my gall bladder. This was done, and my gall bladder was full of stones. Another liver biopsy was undertaken while I was in Wesley, and again I had lobular hepatitis. The blood tests indicated I had chronic anaemia, renal impairment and hypogammaglobulinaemia. I was then referred to the clinical immunologist at Wesley and I was eventually diagnosed with granulomatous variant of common variable immune deficiency (CVID).
Correct Diagnosis At Last!
The immunlogist was absolutely convinced that I presented with an IgG4 deficiency: infections of lungs, liver, kidneys, sinus. This meant that I needed to undertake intravenous immunoglobulin replacement therapy. This replacement therapy is done every 28 days in the Maryborough Hospital, and will continue for the rest of my life. The IV infusion is a blood product, containing human antibodies. Unfortunately the effect of this therapy does not last for long, probably about a week or ten days if I am lucky.
I also receive an intramuscular penicillin injection during the infusion, and am also taking penicillin capsules, twice daily as a prophylactic. Over the years considerable damage has been done to my lungs, and I have bronchiectasis, COPD, as well as streptococcal pneumonia. I have lung and sinus infections almost every day. I also have pseudomonas.
The exact cause of my CVID is uncertain, although it is possible it is genetic. Granulomatous variant of common variable immune deficiency is very rare and as there is no register of CVID sufferers it is really a guess. Some say 1:100,000, but it is not certain. Some research has been done in recent years, and there has been some funding for research into PIDs in Canberra.
At the time of writing this blog I have had over 100 IVIg infusions. Over time I have had the amount of antibodies increased, and presently I have 27g of human antibodies in 400mls of maltose, infused at the rate of 240mls per hour. My infusion is done through a portacath which was inserted about four years ago. With accessing the portacath, changing bottles of the product (called Intragam P) flushing and a hep blocker, my time in the day clinic is about four hours. Intragam P is also nephrotoxic, which is a concern as I have renal impairment. I have my infusion every 28 days.
For me there is no cure. The damage has been done over the years and is irreversible. My specialists monitor any deterioration of my condition, and give me drugs to keep me comfortable. I have considerable pain, basically gut pain, and I take oxycodone (Targin 20/10 and 10/5) twice daily for this. I am reviewed every three to four months by a clinical physician in Hervey Bay hospital, and also every three to four months by my clinical immunologist in Wesley Hospital, Brisbane, a round trip of 700kms.
I visit my GP almost every month for oral antibiotics and morphine pain tablets. Fortunately all my doctors send my reports to each other and everyone seems to be up with the play. My GP has stated that I am the first patient with CVID he has had in his medical career. Recently when I was in emergency department at Hervey Bay hospital the ED doctor said he had never had another patient with CVID. Fortunately Beryl was with me in the emergency department and gave the ED doctor my immunologist's contact details, and the emergency doctor called him and got information on CVID and how to help me. I was transferred to a private hospital across the road from the public hospital, and my physician is linked with this hospital, so I was taken care of.
I have had a few stints in hospital with pneumonia and lung infections which have required IV antibiotics. I have had four granulomas excised from my arm, basically immune tissue. As well as this, I have oral lichen planus, also caused by CVID. I still have bouts of lobular hepatitis and renal impairment. I have experienced loss of appetite for the last year, and have lost 10kgs in weigh. This weightloss is not a concern, as I am drinking sustagen and other nutritious food.
I had a left total knee replacement a few years ago, as the cartilage in my knee had been destroyed by CVID. I continue to have weird things happen to me physically, and most of these can be linked back to CVID.
I am very thankful that Beryl is extremely supportive and understands the impact that CVID has made on my life, and the implications for the future. We have tried to live our lives together normally, and grateful this is happening to a great extent. It does have some impact, particularly in terms of mobility as I get exhausted very quickly and unable to walk very far. Also there is the times when an ambulance has to be called in the middle of the night, and the stress and concern this places on Beryl. Also the uncertainty as to when the next episode will occur and emergency assistance will be needed. We know that there will be no improvement in the future, and we try to enjoy each day as it comes.
A few days ago I had another granuloma excised from my right arm. I am awaiting the histology of this, but I am sure it will be another piece of immune tissue.
A lot of my time is now spent resting particularly in the two weeks leading up to my next IVIG infusion. This is something that I have to come to terms with, and this time of resting is used for reading and studying for sermons and other involvement in my church. Fortunately, Beryl and I have active participation in our village and also in our church. Beryl plays outdoor bowls twice a week and indoor bowls twice a week. Beryl is also chairperson of the village social club, which arranged special events monthly in the village, eg the village dinner and also the village barbecue lunch held on the last Friday of each month.
Summation:
On Monday morning, after cleaning the motel, I went to our GP and he said that I could either have a blood test and then go to hospital, or go to hospital and then have a blood test. I chose to have the blood test, and after this returned to the motel to continue working. By now, I was passing urine that was almost black. Around 2pm my GP called and told me to go straight to St Stephen's Private Hospital. I was on IV antibiotics for one week, with nil by mouth. I had a severe liver infection.
After discharge from hospital I returned to working in our motel. We eventually sold the lease on our motel in April 2006, having bought a house in an Over-50s Residential Park in Hervey Bay six months earlier in anticipation of selling the lease on the motel.
After settling into our home in the village, my health started to deteriorate. I had been experiencing considerable pain in my left knee while I was still in the motel, and an orthopaedic surgeon had been giving me injections into the knee to relieve the pain. After we left the motel I returned to the orthopaedic surgeon for further examination as the injections were not working. An X-ray revealed that the top of the bone above the knee cap had broken off and it was decided to operate and insert an hemiCAP. This is a screw that was inserted into the bone which filled the hole caused by the bone breaking away.
I applied for disability pension and was granted this, and it was actually paid by the New Zealand pension system. Around the same time I started having lesions forming subcutaneously, and I was referred to a physician in Hervey Bay who diagnosed that I had sarcoidosis. Sarcoidosis responds to prednisone, and so I started taking a light dose of prednisone. This did not help at all, and in fact after one month I found that I was losing my eyesight.
As a result of this, I had to have my cataracts removed surgically and also with laser treatment as there was a build up of prednisone crystals that could not be removed by surgery. The physician had to rethink what was causing my subcutaneous lesions, which had been excised. Around the same time I was referred to a gastroenterologist and had an endoscopy. There were some small lumps removed, which were benign.
However, I was experiencing considerable weight loss, and I had lost 20kg. I had liver pain and a biopsy was taken of the liver, and I had lobular hepatitis. However, I continued to be unwell and the gastroenterologist said he could not help me any further and referred me to Wesley Private Hospital in Brisbane, under the care of an interventional gastroenterologist. I was in Wesley for a total of three weeks, and a considerable amount of tests were undertaken. One of my major problems was gut pain, but nothing showed up in the CT scans, ultrasound or MRI.
Although nothing showed up on the imaging it was decided that I would have a diagnostic laparoscopy on my gall bladder. This was done, and my gall bladder was full of stones. Another liver biopsy was undertaken while I was in Wesley, and again I had lobular hepatitis. The blood tests indicated I had chronic anaemia, renal impairment and hypogammaglobulinaemia. I was then referred to the clinical immunologist at Wesley and I was eventually diagnosed with granulomatous variant of common variable immune deficiency (CVID).
Correct Diagnosis At Last!
The immunlogist was absolutely convinced that I presented with an IgG4 deficiency: infections of lungs, liver, kidneys, sinus. This meant that I needed to undertake intravenous immunoglobulin replacement therapy. This replacement therapy is done every 28 days in the Maryborough Hospital, and will continue for the rest of my life. The IV infusion is a blood product, containing human antibodies. Unfortunately the effect of this therapy does not last for long, probably about a week or ten days if I am lucky.
I also receive an intramuscular penicillin injection during the infusion, and am also taking penicillin capsules, twice daily as a prophylactic. Over the years considerable damage has been done to my lungs, and I have bronchiectasis, COPD, as well as streptococcal pneumonia. I have lung and sinus infections almost every day. I also have pseudomonas.
The exact cause of my CVID is uncertain, although it is possible it is genetic. Granulomatous variant of common variable immune deficiency is very rare and as there is no register of CVID sufferers it is really a guess. Some say 1:100,000, but it is not certain. Some research has been done in recent years, and there has been some funding for research into PIDs in Canberra.
At the time of writing this blog I have had over 100 IVIg infusions. Over time I have had the amount of antibodies increased, and presently I have 27g of human antibodies in 400mls of maltose, infused at the rate of 240mls per hour. My infusion is done through a portacath which was inserted about four years ago. With accessing the portacath, changing bottles of the product (called Intragam P) flushing and a hep blocker, my time in the day clinic is about four hours. Intragam P is also nephrotoxic, which is a concern as I have renal impairment. I have my infusion every 28 days.
For me there is no cure. The damage has been done over the years and is irreversible. My specialists monitor any deterioration of my condition, and give me drugs to keep me comfortable. I have considerable pain, basically gut pain, and I take oxycodone (Targin 20/10 and 10/5) twice daily for this. I am reviewed every three to four months by a clinical physician in Hervey Bay hospital, and also every three to four months by my clinical immunologist in Wesley Hospital, Brisbane, a round trip of 700kms.
I visit my GP almost every month for oral antibiotics and morphine pain tablets. Fortunately all my doctors send my reports to each other and everyone seems to be up with the play. My GP has stated that I am the first patient with CVID he has had in his medical career. Recently when I was in emergency department at Hervey Bay hospital the ED doctor said he had never had another patient with CVID. Fortunately Beryl was with me in the emergency department and gave the ED doctor my immunologist's contact details, and the emergency doctor called him and got information on CVID and how to help me. I was transferred to a private hospital across the road from the public hospital, and my physician is linked with this hospital, so I was taken care of.
I have had a few stints in hospital with pneumonia and lung infections which have required IV antibiotics. I have had four granulomas excised from my arm, basically immune tissue. As well as this, I have oral lichen planus, also caused by CVID. I still have bouts of lobular hepatitis and renal impairment. I have experienced loss of appetite for the last year, and have lost 10kgs in weigh. This weightloss is not a concern, as I am drinking sustagen and other nutritious food.
I had a left total knee replacement a few years ago, as the cartilage in my knee had been destroyed by CVID. I continue to have weird things happen to me physically, and most of these can be linked back to CVID.
I am very thankful that Beryl is extremely supportive and understands the impact that CVID has made on my life, and the implications for the future. We have tried to live our lives together normally, and grateful this is happening to a great extent. It does have some impact, particularly in terms of mobility as I get exhausted very quickly and unable to walk very far. Also there is the times when an ambulance has to be called in the middle of the night, and the stress and concern this places on Beryl. Also the uncertainty as to when the next episode will occur and emergency assistance will be needed. We know that there will be no improvement in the future, and we try to enjoy each day as it comes.
A few days ago I had another granuloma excised from my right arm. I am awaiting the histology of this, but I am sure it will be another piece of immune tissue.
A lot of my time is now spent resting particularly in the two weeks leading up to my next IVIG infusion. This is something that I have to come to terms with, and this time of resting is used for reading and studying for sermons and other involvement in my church. Fortunately, Beryl and I have active participation in our village and also in our church. Beryl plays outdoor bowls twice a week and indoor bowls twice a week. Beryl is also chairperson of the village social club, which arranged special events monthly in the village, eg the village dinner and also the village barbecue lunch held on the last Friday of each month.
Summation:
The above sums up what is happening to me in terms of my immune deficiency and the effects it is having in other parts of my body. I hope it also gives some insight into other effects this is having on us emotionally and psychologically. People often say to me that I am looking well. This comment is quite unhelpful, because most of the time of do not feel well, and also probably experiencing considerable pain. Other people feel that the treatment and drugs I am taking will make me better. Again, this is unhelpful because these drugs are prescribed to make daily living more comfortable. I am constantly feeling unwell with joint pain, gut pain, kidney pain, sinus infections, headaches, breathing problems and fatigue.
