This story was sent in to the Aspergillosis Patients support group in 2001.
Mary Jo was an active member of the Aspergillus Support Group and she has suffered from Allergic BronchoPulmonary Aspergillosis (ABPA) for most of her life. This disease is caused by a non-invasive Aspergillus infection of the lungs. The fungus inhabits the air spaces of the lungs, living on the surface of the sensitive lung tissue causing inflammation but does not invade the lung tissue.
This means that although ABPA is not a rapidly fatal disease (i.e. unlike invasive aspergillosis, which needs rapid treatment), at the moment it is usually permanent.
The inflammation of the lung tissue can make breathing difficult, so steroids (e.g. prednisone) are used to control this. The antifungal drug itraconazole (Sporanox) has been recently shown to also help control the disease.
Part 1, early years, lung surgery and diagnosis
When I was a baby I had such severe eczema that my mother was afraid to even pick me up and hold me. The pediatrician told her that she could expect more problems with me later. His words proved to be very prophetic.
By the time I was 3 years old I had asthma. When I was 10 years old they took a chest x-ray and my pediatrician told my mother that I had “walking pneumonia.” Naturally that was really the beginnings of ABPA. Anyone out there reading this who has ever been told they have “walking pneumonia” can rest assured that that was when they can certainly date the beginnings of ABPA. About this time my pediatrician left our clinic to go back to school to become an allergist. He told my folks to take me to a general internist because of the problems I was having. I was considered a little young to be going to an internist, and I am sure I was the youngest patient he ever had, but it was a partnership that lasted until his retirement just a few years ago.
About this time the decision was made to send me to the Mayo Clinic. They thought it was really strange that such a young child had asthma and bronchiectasis since they considered bronchiectasis to be an old person’s disease. In those days the diagnosis of bronchiectasis was made by a procedure called a bronchogram as they did not use cat scans like they do now. This transpired in the late 60’s. My left lung was plugged up and did not clear and they decided to do surgery when I was 12. The upper lobe and lingula were removed. Subsequent doctors have been extremely critical of the medical care I got at this time. They have claimed that at that time a diagnosis could have been made and should have been made and that the surgery should never have been done. Surgery is almost never recommended for ABPA. Mostly because the plugging can and does develop anywhere. When I was a freshman in high school I began to develop plugging in my right lung. At that time my parents began to wonder if I would live to see age 20. They never said much to me about this but I certainly knew that they were very worried. At this time I remember asking one of my doctors what they were going to do when every bit of lung I had was plugged up and they had cut it all out. No one seemed to have an answer to that. When I look back at that time when I was about 15 or 16 my sister claims that there was enormous anxiety with my parents. She claims there was extreme worry about my future or possible lack of it and terrible frustration at the lack of diagnosis and what that meant in terms of my ability to function at that time and what it would mean for the future.
I do not seem to have noticed it as much as she. My brother claims he didn’t notice it either. When I was younger and they would put me into the hospital my sister would throw the most enormous hissy fits. I am pretty sure she thought I was never coming back. We would try to think of things to distract her. If I had been at the doctors while she was in school and if I had to go to the hospital my mom and I would quickly dash home to get a suitcase and then get out of the house before she got home from school because she would throw such fits it would be hard to get away from her. One year they even gave her a birthday present early but she ran after us as we drove away and chased the car down the street and threw her doll in the street and my dad had to chase her and bring her back into the house. By my sophomore year in high school the anxieties eased up because at age 16 I finally got a diagnosis. We now knew what was the matter with me. We also knew the prednisone was how it was treated. Ironically enough during these years my general internist had been treating me with prednisone. He did it out of desperation not really knowing that this was in fact the right thing to do. Probably he kept me alive. But he didn’t clear my lungs either. Because he would put me on quick tapers. He was concerned because I was developing into a woman at this time and he didn’t want to interfere with my growth or anything. He did it the best way he knew how to but he was really relieved to get a diagnosis too. From then on we were a little more aggressive using prednisone. This is 1972 into 1973.
Part 2 early college, inhaled antifungal drugs, systemic symptoms
By the time I was in college, fast tapers of prednisone weren’t doing it for me. I was having too many plugs and we decided to put me on alternate day doses. This seemed to work better for me. But like many people with ABPA I was restless and dissatisfied with that and wanted an alternative to prednisone.
At the time there were some studies being done using antifungal drugs. This is now the middle 70’s. My doctor and I decided to try inhaled Nystatin. We didn’t know at the time that it wasn’t effective against Aspergillus but we did it anyway. It helped a little but not a lot. It was very expensive and a lot a trouble, so eventually we abandoned it. I think it probably did help some but I didn’t know at the time that in later years, tests would show a positive reaction to Candida which is the organism that it mostly works against. We later decided to try amphotericin B. This is a much stronger drug and is more broad spectrum. Again it was a partial success. We had some trouble with me tolerating it as it is very harsh and has an unfriendly pH. We took steps to try to neutralize the pH but it really didn’t help. I used to get coughing jags from trying to inhale it. I couldn’t use it every day because my lungs needed to settle down from it. It was sort of like trying to inhale rubbing alcohol or something similar. Very harsh and irritating. Eventually we abandoned that too.
I must say that when I was using it I felt that my lung secretions looked “cleaner” and were less brown looking, but during this time I had never really been able to successfully reduce my prednisone dose and that was my goal when I set out to do this. So it was a mixed bag. It was also a very expensive drug. Highly perishable and sort of difficult to work with. Eventually I just quit using it.
Now we are getting into the early 80’s. For years I have contended with multiple symptoms i.e.:
Muscle and joint aches and
Low grade fevers
I felt that I had exhausted everything that traditional medicine had to offer and it was time to look at alternatives.
I chose an “alternative allergist” and it was a good move. First of all he never turned out to be particularily radical. He was horrified that I had allergy shots for 10 years as a child. Yes they did make me worse, but had I had a diagnosis during those years we would never have done that either. Allergy shots are a big no no for ABPA and don’t ever let anyone tell you differently. This guy did a lot of very complete allergy testing and I had been to other allergists. I found out that I cross react to every mold, yeast and fungi on the face of the earth. He seemed to have a very good understanding of mold allergies in general and I really learned a lot. He learned a lot from me too. He couldn’t help me but it was a good experience. Later my other pulmonologist who disapproved of him had all the testing he did on me repeated and got exactly the same results. So everyone got educated that time around. We are now about 1985.
Part 3 ovarian cyst, and deflazacort
In 1985 I had emergency surgery for an ovarian cyst. I don’t know how long I had it but I had no symptoms at all until I bent over in pain one day and did not straighten up until after the surgery. The cyst was the size of a grapefruit and I lost my right ovary and tube. I always regretted not having it cultured for fungus because you just never know. I feel that in terms of my overall health I descended into a downward spiral at this point in time. I had certainly always been in very poor health but for some reason I became a lot less functional after this incident.
As it got to be towards the 90’s it became clear to me that I was not going to be able to work much longer and that I was going to have to start thinking about disability. I had always had fairly poor attendance and I could only work and not do much of anything else. My family did all my housework and shopping for me and when I got home from work I would take a bath and put on my nighttie and eat a little supper and crawl into bed. On weekends I would just vegetate.
One day in the mail I got a note from my brother along with a clipping from the Rochester paper. It stated that the Mayo Clinic was about to start clinical trials on a drug that was supposed to be “prednisone with no side effects.” I decided that I would move heaven and earth to have that drug and so began one of the strangest adventures of my life.
The name of the drug was “deflazacort” and they were already using it in Europe. I already had a doctor in the pulmonary department at the Mayo Clinic. He did everything he could to get me into the study but it was in the allergy department and they did not want ABPA. Our next step was to get the FDA and the drug company to release it to us to just use individually and ignore the study. Again we hit a brick wall. To release a drug on a compassionate use basis the drug needs to be substantially different than anything else already available that is already FDA approved. We could not establish this, since deflazacort was already of the same class of drugs as prednisone. Since the drug was already being used in Europe it seemed that it would be a good idea to try to get some and bring it here. You see I was desperate. If it were everything I wanted it to be I would be able to take very large doses with little risk of side effects and probably continue working and maybe function better than I ever thought possible. Isn’t that everyone’s dream come true? I was ready to inconvenience anyone I had to,and I was ready to ruin myself financially if necessary. After all, if it worked it would certainly pay for itself. I hired an attorney who specialized in international law. She made a search of foreign doctors who went to American medical schools and who were now practising in their native countries. She wrote to them all. We got one response from a very kind Italian pulmonologist. He prescribed deflazacort for me. The American Express Company had it filled and it was sent by private courier to me. It was hand walked through customs by the courier. And yes. This was very expensive.
When I got the drug I had instructions for taking it. I went to see my doctor and we made the transition from prednisone to deflazcort. When the allergy dept. got wind of this they were very eager to know how much I was taking and how I was feeling etc. My doctor discussed it with them. Do you think that was the right thing for me to have ok’d that? Or should I have told them to go jump in a lake?
I stayed on deflazacort for about a year. I couldn’t tell any difference at all between it and prednisone and neither could my doctor. I really don’t know what happened with the Mayo Clinic study but I doubt that it ever panned out. As far as I know it never got FDA approval. Had it worked out we would all be on 100 mg of deflazacort a day and going out dancing every night and in vigorous health. This is now 1991.
Part 4 Disability
In May of 1991 I applied for Social Security Disability. I just couldn’t hold it together anymore and I was nearly killing myself trying to work. My attendance had gotten bad and I was just about on the verge of complete collapse. A friend of mine who has lupus and multiple chemical sensitivities gave me the name of a good lawyer. She had already been through the process. He was a very good lawyer but it took from May 1991 when I first applied until July 1992 when I actually received some money to settle my case. You definitely need a lawyer to get you through the process. My applications, re-applications and appeals were all denied and it got so far that I actually had a hearing. What was the problem? Certainly I was sick enough and I certainly had a well documented case going back many years.
Part of the problem was that my lung functions were still actually fairly decent despite years of wear and tear. So I couldn’t qualify on that alone. The problem mostly turned out to be that there is no government listing for ABPA so I needed to qualify under equivalents. The day of my disability hearing was one of the worst of my life. I was going on trial to prove that I was too sick to work. That just made me feel sicker yet. The big surprise was that it turned out ok. Right before the hearing an elderly gentleman came up to me and introduced himself. He was the doctor that SS hired to be at the hearing. Almost as soon as the judge started the hearing he stood up and announced that the judge should find me disabled. Apparantly he had already made up his mind from reading the records. He then told the judge what equivalents to use. Asthma, Bronchiectasis, and Myasthenia Gravis ( to cover fatigue etc.). One thing that my lawyer and I really underestimated was how much weight would be given to the side effects of prednisone. We had emphasized the disease mostly but the doctor gave great weight to the side effects, and that surprised us a little. Since he considered it medically necessary for me to be on it he included the Myasthenia Gravis equivalent to cover my effects from prednisone.
The hearing did not last that long. I really don’t know why they bothered at all since the decision was mostly made from paperwork they already had and it costs money to have a hearing like that. After the hearing the doctor asked to speak to me. My lawyer looked a little nervous but I said ok. He then commented on some of the medical care and treatments I had received ( as always critical of the surgery I had done) and then he proceeded to tell me that most people have a lot of trouble getting diagnosed. He really knew a lot about ABPA. He told me about a medical student at the medical school where he taught who developed ABPA. They diagnosed him with tuberculosis and then proceeded to nearly kill him. So that is a sample of what can happen in an ideal setting. After all, this student had dozens of first rate doctors looking after him. Suffice it to say that I was really relieved to have this settle.
Part 5 odd rashes, sporanox and some final thoughts
In the fall of 1993 I noticed that my skin was really going crazy. I have always had skin problems like eczema and acne but this was really different. It started out as hives. Then after the attack of hives, it would subside. However a few of the welts would remain. Not all of the welts would remain out of each episode though, only a few. This happened all over my body except for my face and scalp and hands. The remaining welts then would harden and raise and they were the funniest looking nodules. They almost looked a little like warts. But not all the remaining welts turned into nodules either. Some of them turned into open sores in little red spots. I looked like I had the measles or chicken pox or something. This was not the first time I had a weird skin problem. As a teenager I began to develop funny looking blood blisters on my hands. We never did figure out what caused that and I still get them to this day. With that in mind I didn’t hold out too much hope for getting answers on this situation but went in to see a dermatologist nevertheless. She biopsied two of the nodules and sent them in. They came back negative for every culture she ordered – this time including tests for fungi. She didn’t know what to do. The constant itching was driving me insane. After some frustrating visits she finally told me that she was positive it was somehow connected to my disease but that she just couldn’t define it and she didn’t know what to do for me. She took a large grocery store sack and filled it full of samples of creams and soaps etc., and gave it to me and wished me luck. One more thing for me to have to live with.
Fast forward to winter of 1997. For months I had been looking at advertisements in magazines for Sporanox. The drug company was touting it as a cure for toenail fungus. I knew that it killed aspergillus and they must have thought it reasonably safe if they were advertising directly to consumers. My doctor and I decided it was time to give it a try. He gave me a prescription and told me to start out slowly to make sure I tolerated it. We decided to try 1 100 mg capsule a day. I regret to say that I lasted exactly 3 days on that dose. I developed severe headaches from it, so it looked like it was going to be a failure. One morning a week or so later I got out of bed and looked down at my legs. My skin had really started to clear up for the first time in about 4 years. I thought I was imagining it so I had my folks look and they both were astonished. Only 3 pills and it was really improved. I began taking the capsules apart and dividing up the beads into gelatin capsules so that I could take small doses and my skin almost completely cleared up. My doctor was surprised and continues to this day to give me small amounts of Sporanox to keep my skin clear. Unfortunately I have never noticed much change in my lungs but I doubt that I ever take enough to do much that way.
This about wraps it up but I will just say a few more things.
The most important thing is to have doctors who genuinely care about your welfare. If they don’t have all the knowledge, there is nothing to stop them from looking things up, but you can’t substitute anything for a caring doctor.
My mother was my best friend and biggest champion. She had a great faith in God and her motto was to trust in Him and just live one day at a time. One time when I was having a particularily bad time she told me not to live one day at a time, but rather just live one minute at a time. And I leave you with that thought.
Appendix A prednisone
Most of us have a love/hate relationship with prednisone. I think this is prefectly normal. We need it to stay alive. It reduces inflammation in our lungs, helps clear plugs and thus minimizing damage to our lungs, and it helps lower our IgE and lessen allergic reaction. Unfortunately it also has numerous side effects. It can affect any part of the body. In my case it has caused weight gain, cataracts and easy bruising. It can cause osteoporosis and other skeletal problems, high blood pressure, ulcers, thin skin, delicate blood vessels, diabetes, cataracts, glaucoma, mental disturbances and many other problems. You should be in close contact with your doctor when using this drug. It also wouldn’t hurt to look up all of the side effects in a drug reference book because you may develop something unusual that it would help to be aware of. For the most part prednisone should be used as sparingly as possible. The most sparing way to use it is a quick taper, when you are on it for about a week or so and the first few days are high doses which are rapidly dropped until you go off it. If that can’t manage you properly then the next thing to consider would be alternate day doses. This is thought to minimize adrenal suppression. After that, more aggressive schedules would be daily doses and divided doses, which would be more than one dose during a day. The closer the doses are together the more you have an enhanced therapeutic response but the side effects are more pronounced too. As a teenager I was always put on quick tapers because I was still growing up, but by the time I was in college we switched me to a steady alternate day dose because the quick tapers weren’t doing it for me and I had grown up a little more. Now I take daily doses.
If and when you taper you should work closely with your doctor. You should be given a tapering schedule to follow. This should be done slowly. To discontinue prednisone abruptly could result in going into shock and or dying. I am not joking. If you experience severe weakness while tapering you should contact your doctor, but first bump up your prednisone dose to compensate for what might be adrenal suppression. Anytime you are on prednisone you should wear medical identification for a period of approximately 2 years after you have gone off it. For most of us that means continuously wearing medical ID. This is very important in case you are in an accident or develop a sudden illness. Medical personnel need to know if they should assume that you are adrenal suppressed. They can compensate for this by giving you IV steroids. To not have this information available could result in your death. This is all information given to me over a number of years by various doctors.
Appendix B Bronchiectasis
I suppose many people wonder how they have come to have two lung diseases for the price of one. How does bronchiectasis happen?
When we develop plugs in our lungs from ABPA they lodge in our bronchial tubes. The mucus becomes jammed in there quite tightly and this causes the affected bronchial tube to literally stretch out of shape. It can almost look like a little balloon. You may be able to clear the plugging out eventually but the bronchial tube never really resumes its original shape. It has been permanently damaged. This can happen in many different areas of the lungs and I have had many different plugs in many sections and I have fairly extensive bronchiectasis. Most people with ABPA develop bronchiectasis from repeated plugging episodes. The best thing to do to minimize this is to use prednisone as best you can to keep the plugging to a minimum. The more bronchiectasis you develop the more problems you are going to have. These problems include decreased lung functions and increased susceptibility to infection. The loss of elasticity from the stretching makes it harder to breathe efficiently. Most of us have asthma too so that is a double whammy. We also are more prone to infection because of this because the lung’s ability to clean itself is impaired by the distension. That is because it is hard to cough stuff out of the affected areas and these areas are more likely to accumulate mucus.
At a certain point some people can evolve from this into pulmonary fibrosis and end stage lung disease but this is not necessarily true in any particular case. I am now 45 years old and have had bronchiectasis since at least age 10 and no one has ever told me that I am going in that direction. My lung functions are certainly impaired but I am still in an upright position most days.
I have had severe bronchiectasis going back to childhood and I am infection prone because of this. I take antibiotics approximately once a month to manage this. You may also find some blood in you sputum occasionally due to this. However any time you cough up any significant amount of fresh blood you should be seen immediately on an emergency basis since this could be something more serious than just small amounts of usually drier blood that can go with having bronchiectasis. I believe that in my particular case episodes of pleurisy and chest wall pain are also connected to having bronchiectasis as well as being attributable to the inflammation and exascerbations of ABPA. Anyone out there who has received a diagnosis of bronchiectasis can probably be certain that this reflects the disease activity of ABPA for a period of time that is probably more likely to be a period of years and not weeks or months. These are things that I have been told by various doctors over a number of years.
14th July 2002
It is with great sorrow that we have to report that Mary Jo died recently from complications of the illnesses that she so eloquently wrote about in these articles. She wrote these articles and was an active member of the patients discussion group management team in her efforts to try to help people in a similar circumstances and in that she found much success – we will miss her.
The Aspergillus website team