Living With IPF - My Story


First, greetings to visitors and members of the Second Wind Organization, who may be visiting here via the new link up on their Member's Letters page. Just found it myself when I did a search on IPF and saw myself listed as "New". Excellent.

OK, we continue. Of special interest to me is my own particular troubleshooting problem on the road of life--Idiopathic Pulmonary Fibrosis, also known as IPF. Let me say here that I am revising this account post-transplant, and trying to include more detail than I had initially, so I apologize as it jumps between past- and present-tense as I go through the editing.

They tell me only 3 people in 100,000 suffer from this life-shortening disease, so I would assume that research-wise, we're pretty far down the list. We may, however, benefit from the knowledge gained from fighting other, more well-known pulmonary diseases, such as Cystic Fibrosis. In fact, as they map more and more genes and their role in disease cause and effect, perhaps they'll be able to one day give us an injection of "fixed genes" in a virus carrier that stops our immune systems from attacking our bodies, as it does in this and other auto-immune diseases. Hey, we can hope, can't we?

The Symptoms and the Diagnosis--December, 1994 to April, 1995

Maybe it will help some others if I share my own story here: I was diagnosed with Idiopathic Pulmonary Fibrosis (IPF) in April, 1995 at the age of 44, but only after fairly extensive diagnostic procedures were performed. The British call this disease Cryptogenic Fibrosing Alveolitis, if that helps you any in your searches. I'm a quarter English, by the way, on my father's side, and now I find out very recently from his sister, my Aunt Elinor, that not only did my father die in 1971 apparently from complications caused by IPF, but indeed both their grandfather and great-grandfather--my great-grandfather and great-great-grandfather--died from lung disease as well. In any case, my own experience has been as follows:

First, I began to feel shortness of breath in late 1994, right after Christmas. This followed a fairly serious bout a month earlier with a virus of some sort that I believe I caught at the Mercer County Community College Computer Show in New Jersey, where the hallways of the college were packed with people and there was absolutely no ventilation. Even in November, it was completely stifling. I even feinted towards the end of this roughly two-week long illness, which really shocked and upset Judy, my wife. (Also, right around that time, I had been at a National Sales Meeting in the Southwest, from which I returned with another cold or flu-like infection.) After roughly a month of waiting for this shortness of breath to go away, I was finally hospitalized in early February for a 4-day stay for what they (and I) thought, after seeing the X-Rays, was mycoplasmic pneumonia, for which I received 12 IV bags of E-mycin. But three weeks of follow-up X-Rays unfortunately showed that I wasn't getting any better. After my attending Pulmonologist suggested I go see a local thoracic surgeon for an open lung biopsy, I sought a second opinion from a leading Pulmonologist in the area recommended by my then-GP. At his urging, I then first had a bronchoscopy, where lung tissue was taken for analysis to see if I had Sarcoidosis, but which was inconclusive--no granulomas were found, which would have ruled in Sarcoidosis. However, their presence in the lungs can be very random, making it difficult with a Bronchoscopy to rule it out. Fortunately, however, we did rule out Cancer, although it was to come back to roost much later in a very ironic sort of way.

Then, in early April, I had a video-assisted thoracoscopy (instead of an open lung biopsy), where more lung tissue was taken that eventually confirmed that I had IPF. This is a procedure much like arthroscopic surgery, often done on the knees of football players, that I felt would be a lot less invasive than an open-lung biopsy, which I hear is a bear. In fact, I know of two sisters in their 50's to 60's who had IPF, whose brother also now has IPF, that unfortunately never fully recovered from their respective open-lung biopsies, and passed away within a month of each of their procedures.

I've created a page dedicated to the subject of the diagnostic procedures I underwent that explains them in more detail. You can access it by clicking here.

Living with The Disease that is IPF--April, 1995 to September, 1996

Me with IPF--Before the TransplantThis all started when I noticed that in the course of doing my job, which was as a dealer rep for a major pc networking software company, that I would get a bit winded when addressing large groups. Then I would be short of breath when carrying demo equipment. And lastly, when I brought in some wood for the fireplace insert (woodstove) at home, I had to sit down and pant for nearly five minutes. This was all in the January-February, 1995 timeframe. My boss Don said, "Better get it checked out", and my GP said, "Check into the nearest ER tonite!". So I did, as I described above.

But even after these symptoms and the resulting diagnosis, I continued to work for another year and a half or so, on 20 mg. of Prednisone every other day--after an initial blast of 70 mg. per day for seven weeks, followed by a SLOW tapering process. All the while I was slowly deteriorating--my PFT results were down an average of 10% every three months. This was after starting out with 65% of predicted FVC and 35% of predicted Diffusion Capacity, both measured in late March, 1995. I gradually came to the realization that I was heading towards, if I was lucky, the inevitable double-lung transplant, which I initially wanted to put off as long as possible. But this reluctance passed as the disease grew more robust--by the end, I was praying for the call each day. The picture you see over on the right, as best as I can remember , was taken in the early summer of 1996, while I was still working, using a video snapshot via a "Snappy" that I borrowed from Norm at work. This was the problem--I never really looked that particularly "sick", but I was.

So--advice? Get the BEST Pulmonologist available. Forget your General Practitioner--even though mine, Dr. Narzikul, is a great guy and a big help--or even a non-star pulmonary specialist. Find the head of pulmonology at the best hospital you can reach, and get properly diagnosed. Unfortunately, we who get this and other similar lung and even liver diseases are usually a bit too late finding out about it, as the symptoms are only noticed after some 50% or more lung (or liver) function is already irretrievably lost. It's important not to ignore it, however--if you have, for example, Sarcoidosis, you can beat it with steroids and return to relative normalcy, so the trick is to distinguish what you have early on as best you can. Many avoid proper diagnosis, however, through either ignorance or denial, and that is definitely not a good way to go. The thing is, there are other worse things it could be, so it is imperative to get all this straightened out right away, then decide on a proper course of action--NOW! While you have the strength, because you will need it.

Nobody knows what really causes IPF--idiopathic means "we don't know", and cryptogenic means "we can't know"--so it's anybody's guess how to treat it. They know that hydrocorticosteroids like Prednisone help stave off the inflammation, but only somewhat. Reportedly, only 10% of patients taking Prednisone actually see a roughly 10% improvement in Pulmonary Function Test (PFT) results after taking it. This positive response is one of the only benchmarks known, and it generally means you may live for 8 or 9 years with the disease instead of 3 to 5 years (excluding lung transplantation, of course).

But if you're like the 90% of us who don't have this lucky response, you're probably panicking by now. Well, there is some hope available from an alternative point of view, but not much. For example, studies show that IPF patients have only 25% of what is a typical, i.e., "normal" Glutathione level in their blood. Glutathione is an anti-oxidant that can be augmented--allegedly--by taking it in pill form (not too efficient), or by taking L-Cysteine, which increases it's production in the liver (better). Best of all seems to be N-Acetyl-Cysteine (NAC), a pill taken in 600mg doses three times a day along with Vitamin C (I take 1 gram per dose). NAC not only allegedly helps raise the Glutathione level, but does many other good things too. All of these are common amino acids, and available at Great Earth Vitamin stores, for example. My feeling about all of this wavers between being solidly convinced that it works to having the sinking suspicion that it must have been what Steve McQueen was trying to do when he went down to Mexico to get unapproved drugs he thought he needed--Laetrile, I believe--then died anyway. But they probably can't hurt, and there is literature on the subject and experience in the field that shows it has relevance and is effective. It's certainly more effective than Actimmune. In fact, I have a copy of a study that claims that NAC may materially retard deterioration due to the disease. So did it help? I don't know--I was too paranoid to stop taking it to find out, but I and some other IPF transplantees all took it, and there are more of them than the number of IPF transplant failures I know about who didn't take it. I was also sent a copy of a study that claims that Colchicine alone may accomplish as much as Prednisone in treatment of IPF--which is to say, not much--so we went with that, too. My Pulmonologist had me taking 0.6 mg of Colchicine a day from nearly the beginning, which as he said, worked on animals, and although there isn't any human data yet, it's so benign that there isn't any reason NOT to take it.

Coping, Phase I--Medical Leave of Absence, Pulmonary Rehabilitation and Supplemental Oxygen

Ironically, to me, what all this points to is that probably the agent of destruction in our bodies is the very thing that we suffer from the lack of the most--Oxygen! Since the Glutathione--an anti-oxidant--level is down, O2 does its oxidation thing, perhaps helping trigger the immune response that causes the cytotoxins to turn our alveolar cells into scar tissue, making gas exchange into and out of our bodies extremely difficult as time goes on. Believe me, I used to think of this every time I strapped on the old O2 when I hit the Nordik Track machine. Unfortunately, not long after that I had to strap on the old O2 all the time, as I was home on medical leave of absence and had to use supplemental oxygen full-time, at 2 LPM at rest and 6 LPM with exertion. They were shuttling 40-gallon liquid tanks in and out of here every six days then, which was soon to increase dramatically. Yikes. Nothing stays the same with this disease. You've got to keep moving.

A note of some interest--at my peak oxygen consumption rate near the end (late Spring, 1997), my oxygen company (HHCA) was delivering 10 M-60 tanks, 6 E-tanks, and replenishing four 40-gallon liquid oxygen tanks EVERY TWO DAYS! Their drivers were really the only people I saw outside of my family, a couple of guys I worked with for years, and the most selfless Dental Hygenist one could ever hope to know (Thanks, Carol!). They were my link to the outside world. We talked about the Eagles, the 76-ers, and bringing up dogs. Much more than a thoroughly professional group of oxygen delivery-type people, they were spirit-bolstering, joke-telling reminders of what life used to be like. As such, I want to thank Lindsey, Drew, Drew's Dad (who filled the tanks), everybody in Customer Service, Leo, Ron, and many others I've forgotten but will someday visit and take pictures of for the site. They literally helped save my life.

Judy and Me--11/96That's Judy and me in November, 1996, at Judy's Birthday party here at the house, after I was on Medical Leave of Absence and full-time supplemental oxygen for a couple of months. I took the nasal cannula off for the shot, as I did when I got my driver's license renewed earlier that month. You can see the Healthdyne 950 fingertip oximeter hanging from my neck. Things started to get pretty interesting not long after this. One of the next things I did was to sell the Nordik Track. I liked it a lot, but Cody from work was shopping for one, and my rehab people were talking treadmills, so I let it go. Big after-the-treadmill-sale surprise, however--all consumer-grade powered treadmills depend to some extent on gravity to augment their little pissant motors, and will not accommodate a zero degree elevation. The affordable ones only go down to three degrees, and you have to spend $1500 to get down to one-and-a-half degrees. Spend $2500, and you can get a flat treadmill. Nice, huh? Block up the back to level the thing out and burn out the motor, and you, your treadmill, and your extended warranty are on your own--not supported. One company makes nearly 90% of all the consumer-grade treadmills out there, which is a virtual monopoly--Icon Fitness, I believe. They make all the Space-Saver designs, etc. So for me, the thing sat waiting for the my post-transplant period, when I figured I could use it. At that time, just walking down stairs was tough, so I wasn't losing all that much. And while we're on the subject of relative disability, obtaining a suitable and reliable oxygen supply was the single largest logistics challenge I had to meet. That, and of course, trying to accomplish the daily tasks I used to take for granted--washing, dressing, drying, walking, standing, bending over, and of course--climbing stairs (my old nemesis). The days of 2 LPM at rest and 6 LPM with exertion were, at this point, long gone--for maybe six months. Although I used to hesitate to admit it, I had to sleep with an Oximizer (pendant-style) nasal cannula fed with 12 LPM. I found out after a subtle suggestion from Phil, my pulmonary rehabilitation guy, that during sleep your breathing becomes more shallow, and you need MORE, not LESS oxygen flow to keep your oxygen saturation level in the low 90's while you sleep. When I was secondary-infection-free, I could torque it back to 8 LPM, or even 7.

But lately, as you'll read below, the winter of '96-'97 was a secondary infection extravaganza, and as I was told, you can't really over-amp on O2 with a nasal cannula anyway, so as long as insurance was covering it, I decided to err on the high side. This gave me a margin that is mandatory for both transitioning into a sleeping position at night, and then into an upright position upon arising the next day. The heart rate plays a big role in all of this. But 12 LPM means a Y-hose-connected pair of 40-gallon liquid O2 tanks delivered twice weekly (initially), that at their maximum rate of 6 LPM each, tend to freeze up in 12-24 hours, so you need a SECOND set to alternate every half-day or so, to let the frozen set thaw out. Otherwise, your flow suffers, and a lot of O2 is wasted, or so it seems. Add to this a bedside (conventional E-size or larger) tank capable of 15 LPM as a backup, but mainly used as a 5-6 LPM add-on through a second parallel nasal cannula to support exceptional events like coughing attacks, which are routine events at night when getting into the horizontal position, and for added loads like telephone conversations when in the prone position, before the transition to an upright position has been made. Is all this clear? It worked, although it was a bitch to coordinate with the home healthcare people, but it supported me and played a big role in getting me to the Big Party. Good or bad, I used to say, it's only temporary. The only catch is, short of the BIG RIDE to the transplant center, for which one car was more-or-less properly outfitted, trips to anywhere at that time were nearly impossible, as even an E-tank will peter out pretty quickly at those flow rates. I could grit it out with a rebreathing mask at 6 LPM if I had to, and later with two portable tanks in parallel providing 12 LPM, but there was a lot of concentration required.

If you're unlucky enough that Prednisone doesn't adequately torque back the disease, you're probably going to get acquainted with Imuran, as well. Or any number of other combination therapies for halting the onset of the damage. Keep in mind that if Prednisone DOES work for you, I'm told that taking it every other day--if you can get away with it--somewhat reduces the "sleeping adrenal gland" side effect, not to mention lessening the possibility of more damaging side-effects like femural decay, glaucoma, etc. I actually like the stuff, but I do suffer from pseudo-diabetic symptoms like wooden-feeling feet, which I hate. I attribute this to the Prednisone.

If you're like I was, you probably also have some digital clubbing, or swelling of the finger tips and ends of your toes. It's another manifestation of oxygen deprivation at the extremities that you have to live with. It was maybe the first visible indication I had that something was different, but I thought at the time it was due to my over-aggressive guitar practicing. Probably made my foot problems worse as well.

Hoping, Phase I--Lung Transplant Evaluation Testing

So now, the big news was that in August, 1996, I went through transplant evaluation testing at the Hospital of the University of Pennsylvania, and having passed the tests, was listed for a double-lung transplant, which I was told would happen within about a year. I learned a few things during the testing, however. First, it took me over two hours to complete the PFT's--I was coughing constantly. My arterial gas number was really borderline, at around 87%, if I recall, and my Diffusion Capacity was REALLY low, at around 22%. I figured things were happening a little too fast,and that I was going to be in for a real fight. I had fairly high Pulmonary Hypertension (yet another reason I'm no longer working), so we took the conservative approach in order to preserve my heart. This was all, of course, both welcome and unwelcome news--welcome because failing admission to the program, it would have been Sayonara Baby in another year or two, and unwelcome because a lung transplant is definitely a major-league operation, and anything can happen. The numbers are not too bad--75%-80% survive the transplant, and 5% expire each year after that. So half pass on after 5 years. This is the across-the-board average, of course, and encompasses young and old, COPD, IPF, CF patients, and so on. Re-transplantation survival is something like 30%, so you don't get that option very readily. I would imagine it's more for pediatric cases and the like, although we were told it's reserved for isolated Bronchiolitis Obliterans cases. That's another story altogether. Let's hope thay get that one under control, eh?

So, what other semi-paranoid survival techniques did I employ, you ask? Well, for one, to help offset the effects of the steroids, I took Twin Labs' Cellmins--a Potassium, Magnesium, and Calcium supplement (one cap three times a day), as well as 42 grams of powdered whey protein (Rich Chocolate flavor) in a coffee and milk mixture each morning. The protein drink REALLY helped me (and still does) maintain at least a base energy level. Other miscellaneous vitamins I'm taking--Vitamin E (400 units once a day), CoQ10 in Twin Labs' Maxi-Life multivitamins (1 cap three times a day), Zantac to combat heartburn from the Prednisone (one tab twice a day), and timed-release Vitamin C with bioflavinoids (one 1-gram timed-release tab three times a day). I also took Claritin, to cut down on the post-nasal drip that was causing phlegm and resultant early-morning retching episodes. Seemed to work great. We now know that a big note here is to avoid Claritin-D (decongestant), if you have IPF. I tried it on the advice of a well-meaning Mother (mine), but after three days, was struggling for air. Seems that it constricts the blood vessels or perhaps something else in the lungs which is exactly what you don't want to do with IPF. Took almost a week to get over the symptoms, or so it seemed.

For a while, I was taking Propulsid, which is kind of a laxative, that (literally, I guess) propels food through your body, so it hangs around less in your stomach area, where especially post-transplant, it can get kind of backed-up, fall into the lungs, and cause aspiration pneumonia. With me (pre-transplant), my pulmonologist thought it would help relieve some retching episodes I was having during morning and evening coughing spells. You know, nobody official seems to want to talk about this, but I've read that post-transplant, you lose your coughing reflex. (It turns out that for me, anyway, this is only partly true--you still have a gag reflex.) This is why Propulsid is apparently so important, to reduce the incidence of aspiration pneumonia, which would be prevented could you feel the food and cough it up. I can't wait for that feeling. Eeks. (It's not so bad.)

So, having stopped taking Propulsid, I used (and still use) a bowel maintenance routine consisting of a regular fiber supplement (orange-flavored Citrusel or Metamucil, which I like better), and a stool softener (Docusate Sodium--one in the morning and one at night). This all came out of an intestinal problem caused primarily by side effects of the antibiotic Biaxin, which caused some, shall we say, liquidity, which aggravated a little bleeding problem in the butt area. I tell you, what a ripple effect. I think you can trace a large measure of it right back to the Prednisone. The Propulsid was really kind of strong, and gave you little notice prior to water-closet time. And, I figured with everything else I was doing in that area, maintenance-wise, it had to go. And things were, overall, materially much better.

Coping, Phase II--Hard Drugs

Actually, this is what I consider a third phase of treatment. I incorporated 150 mg. a day of Imuran (an immune-suppressor also used as a post-transplant anti-rejection drug), and had to go to two 500 mg. Biaxin tablets a day, EVERY DAY, to stave off secondary--they felt like primary--infections that were filling my lungs with fluid and keeping my maximum oxygen saturation levels depressed up to 15 points or so. Formerly, the drill was I would recover from an infection after a 7- or 10-day antibiotic course, be OK for maybe two to three weeks, then get another secondary infection, take another course of Biaxin, and so on. Eventually, these intervals started to get shorter and shorter, until I was in an almost 50/50 situation--half the time I was sick, and half the time I was taking antibiotics. In addition, each time I would get another infection, it would be a little bit worse than the one preceding it. I'm sure that the added effect of the Imuran may have been contributing to this endless cycle. In fact, I was in bed for nearly all of January, 1997 until we came up with the "antibiotics all the time" approach. Actually, it was my Mom that first suggested that I just keep taking the antibiotics every day. I initially rejected the idea, for fear that the antibiotics would lose their effect. Then, interestingly, as I looked into it further, I learned from Dr. Kotloff, my consulting Pulmonologist at HUP, that Erythromycin--of which I understand Biaxin is a "second-generation" version--has anti-inflammatory qualities above and beyond its antibiotic qualities, and is even used as the primary treatment for a rare lung disease in Japan for that reason. I now credit this single change in my treatment with saving my life--it allowed me to stay alive long enough to get to the transplant. Be assured that I used to eat a yogurt every morning and take a "Super Acidopholous" three times a day--that's something like 10 billion organisms keeping my flora and fauna copacetic.

Hoping, Phase II--Improvisation Often Works, but Big Organizations Sometimes Don't

Daily use of Biaxin worked (I was knocking on wood). My primary Pulmonologist--Dr. Meyer at this point--felt like we were kind of blazing a little research trail of our own, which seemed to validate an abstract from England he cited that described the anti-inflammatory qualities of E-mycin mentioned by my consulting Pulmonologist at HUP, so we have to share some credit here. However, had this not been a positive step, I'm sure I would have been knocked out by secondary infection. But that was not all--shockingly, I learned at my last monthly checkup at HUP, that they were all of a sudden claiming that their average waiting time had stretched out to a shade over two years. While I understand that this is not all that unusual at other transplant centers with large programs and numbers of people on their lists (like the University of Pittsburgh, Barnes, and so on), I was always told at HUP that they were a 9-12 month shop, and that this would play well with my possibly familial IPF, which had started to look like a very aggressive killing machine. But this, I now know, is not a localized problem. Not only are waiting lists growing, but more importantly, the number of donor organs are down, too. A deadly combination, for which I have yet to hear a clear explanation. But the net effect is the same--not good. Important advice if you have IPF--RUN, DO NOT WALK (well, if you can) to a transplant center for evaluation testing, and if you pass, list at two centers in different OPO (Organ Procurement Organization) areas. You do not have two years to wait in most cases. This is the single biggest point I can make to newly diagnosed patients. Nationally, the waiting period for the roughly 3,000 patients or so awaiting a lung transplant is two years or more, and IPF just does not wait around. Believe it.

But to HUP's credit, at their recommendation, at the end of March, 1997, we went to meet, give blood, pee a little, hock a loogie, and hopefully double-list at the somewhat nearby University of Maryland (in Baltimore), where amazingly, unmentioned in all of the Net resources I've seen, we found perhaps the most complete and professional lung transplant program we had seen (we looked at two). Maybe things were starting to look up, I thought. Then we got the good news--I would be listed at UMMC, and transfer all my accrued time from HUP (some nine months) over to them. I would also stay on HUP's list, thereby starting over (with the 90-day IPF credit UNOS grants), but while I would technically be double-listed, it might as well be considered an all-UMMC party. I did, however, plan to do post-transplant rehab at HUP (or a related facility) later, although this proved to be a non-option as UMMC (to their credit, I believe), wanted more-or-less total control over me for at least the first year after the operation. In any case, I thought it would be a good thing to keep the dual relationship alive. As it turned out, HUP really didn't play all that big a role after I listed at Maryland.

So, with that out of the way, what were the other little tricks I tried? Another antibiotic--CIPRO. While continuous Biaxin had eliminated the strong re-infections I was experiencing each time I would end a course, I started to see that old indicator--dramatic desaturation with routine exertion. Two-and-a-half days into a course 750 mg. of CIPRO twice a day later, I would sleep better, desaturate less, and have better peak sats at rest. Which let me turn down the O2 a little. This all becomes a full-time research job, I tell you.

The End Game--a Big-Time Balancing Act

Where were we? Well, by the end of the first 7-day CIPRO course, I felt pretty much the same as I did before the experiment. Since it targets organisms not attacked by Biaxin, and penetrates the lung really well, according to my Pulmonologist, there may have been some benefit there, after all, so I think it was worth it. But after that slight uptick on day two, I resumed the "wet cough" I had before. So we did a little Prednisone Pulse Bump--40 mg. a day for 5 days, tapering back down to 10 mg. a day over the following weekend. Let me tell you--this is where the whole deal is won and lost--with steroids. My lungs cleared up in 6-10 hours, I had a week of nearly uninterrupted, almost over-saturated sleep, and had enough energy to actually get my butt up and into the bathtub. I know that sounds like a it should normally be a no-brainer, but it wasn't. The only problem is, that when I went back to my normal dosage, the wet cough was back, and a lot of my extra energy had subsided. Oh well. I think it's significant, however, that relief came from an anti-inflammatory, and not an antibiotic. As such, I felt that I was pretty free from infection, which I think further justified taking Biaxin every day. The rub being, of course, is that they say that Prednisone dosages of over 10 mg. per day tend to retard healing of the transplant graft, so you can't maintain a comfortable level of steroids and keep your transplant center happy. Even so, I decided to try to negotiate a little higher daily "living level" of Prednisone, if they would let me. As it turned out, UMMC is a lot more tolerant of high Prednisone levels going into the transplant than a lot of other centers (well, mainly talking about HUP here), and do not match for CMV either, which I began to think was more of an exclusionary option rather than an insurance of success. Simply put, you prophylactically treat for CMV with Gancyclovir post-transplant anyway, so who cares about the match going in? Makes sense to me.

Surprise development--just as I got general permission to use alternating doses of 10 and 20 mg. per day of Prednisone, and I was on Day Three of this slight increase in my Prednisone protocol, I experienced an elevated heartrate of about 125 bpm, at my kind-of-normal working oxygen saturation level of 92%. And this was on one of the 10 mg. Prednisone days. So my Pulmonologist said, let's watch it overnight, and call me if it doesn't go down. He doesn't think it's caffeine or nerves, but we decide to watch it. Well, after a tough night of virtually no sleep, I saw that it jumped up 10-to-15 bpm to nearly 140 bpm, and the call goes in. And the inevitable "get into ER for an EKG" decision was made. From the EKG comes the conclusion--sinus ventricular tachycardia (SVT), and after an immediate initial application of intravenous saline solution, the tests began--an echocardiogram, an ultrasound of the legs (looking for blood clots), much blood drawn, and many visits with mostly cardiac specialists. They decided to keep me overnight, which turned into two. This was in early-to-mid April, 1997. Three bags of more intravenous saline solution on a pump, high flow levels of oxygen, and two nights of sleep lowered my heart rate to around 115 bpm, so I was free to go. The decision? SVT caused by dehydration due to the rapid breathing rate associated with IPF, and ingestion of more water at home than anything else causing (amazingly) dehydration, possibly affecting my electrolyte level (which actually tested good). But I learned that my cholesterol level was 130, so it wasn't a total loss of two days. Heart looked and sounded strong, no clots in the legs, hemmorhoidal symptoms mysteriously disappeared, although I was visited by both a proctologist and a colo-rectal surgeon (probably the clenched sphincter factor at the thought of surgery helped me there), and received an interesting presentation on the Transtracheal Scoop by an Ear, Nose, and Throat doctor. He thought it might, in my case, require up to four days in the hospital for fine-tuning for the flow I require, so I demurred. And my Pulmonologist has found in a recent study that it requires a lot of maintenance, and doesn't really provide any more relief than simple mask and cannula techniques. Also, I was told to drink 2 liters per day of GatorAde for rehydration, and self-administer Lovenox, a blood-thinning shot given twice-a-day subcutaneously. (This stuff is EXPENSIVE--thank God for Prescription Plans.) I hate shots, can't watch them given or taken, and all of a sudden I was puncturing my belly and leg fat like it was somebody else's body. Uncanny.

So I got released, and what happened? The worst sleepless night in two years, with a capped oxygen saturation level of 87%, and again the call went in. My doctor thought they probably should have stopped re-hydrating me after the second bag of saline. So I started taking two days of diuretic (Lasix)--one 20 mg. tab per day. I do, and I was all well again with the world. Except YOU try to drink 2 liters per day of GatorAde, along with the Citrucel/Metamucil commitment, and all the rest. All I seem to do is take medicine and drink. Oh, and give myself shots. And have a wicked time breathing. Hard to have an appetite with all that liquid in you.

Conclusions I take away from my brief stay in the hospital? Drop the NAC, drop the Cellmins supplement, cut back to only one Vitamin C and one MaxiLife with CoQ10 multivitamin a day, and see if my phlegm level and hemmorhoidal symtoms continue to improve, as they really did in the hospital. Also, I got a big wedgie foam pillow to sleep in an upright position, imitating the hospital bed, which was a God-send. I usually end up in the right place, but I tend to learn the hard way.

Coping, Phase III--Home Nursing Care

As I continued to slowly deteriorate, I did learn some things. First, my Pulmonologist's suggestion that I have a home nurse come in three days a week (M,W,F), was a big help, especially for my wife. Not so much for the work she offloaded, but for being another person to brainstorm with that understands what we're up against. Friends and family, although well-meaning, can often be more effort than relief, and we've been fortunate to get a great one--Noelle--young, aggressive, good on followup, and she even chased down the doctor for answers! She really was his eyes and ears in the field. Her first suggestion was a hospital bed in the master bedroom, and although I was against it, two weeks later (late April, 1997), I was in it, and it afforded me much needed sleep and my wife relief from sleeping next to a coughing machine. So Noelle would come in and take my vital signs, and most importantly, listen to my chest, and was instrumental in monitoring the delicate liquid input balance. We initially overdid it with the GatorAde and got into Pulmonary Edema problems again, and had to go to a Prednisone bump-up and Lasix combination, which fortunately pulled me out of what looked initially like a serious nosedive.

Another thing we also learned at that point was that eating almost anything desaturated me at least 5 points, which could last up to an hour, and some foods are really worse at this than others. So we managed to reach a concensus--Noelle (our nurse), my wife, and I--to have a spaced rotation of small snacks, with as many calories and nutrients as we can manage. Meals as we know it, however, were largely out. Especially dinner. Dinner was killing me, and as things got worse, it got worse. Now, a snack is a half of a grapefruit (this was pre-transplant--grapefruit juice multiplies the effect of Cyclosporin), a small yogurt, an Ensure, a "junior" portion of Cream of Wheat, a small bowl of peach sections, and so on. But I had to be careful to space them out over an hour apart, or I would get cumulative oxygen desaturation, which put increased demands on my auxiliary oxygen supply, which I was consuming at the highest rate of any other customer of my home healthcare company as it was.

As for the ever-present phlegm problem, it assumed a predictable pattern--induceable expectoration before the evening snack, then a second round before the bedtime bathroom run, and then a pretty lengthy (and messy) bout in bed right before "lights out". Who said IPF is characterized by a non-productive cough? All I can say, there was a terrific amount of what I would have to say most closely resembled dog drool in those lungs, and I was doing everything I could to get it out. It's when the stuff turned some color other than clear that I put in for more antibiotics.

The last few weeks were also been marked by another course of CIPRO and a return to an elevated Prednisone level. The single greatest challenge became trying to successfully taper back down the Prednisone level after elevating it to alleviate a congestion or oxygen desaturation problem. A lot rode on this, and it was truly a team effort. The problem is, just when you get used to one level, lowering it really throws a wrench into the works. The problem is, as with many drugs--the more you use, the more you need. And each time you bump the level, you can only come back down part of the way back to where you were before, so your steroid consumption level just keeps going up as the disease progresses. I had to (and still do, frankly) use Valium to get to sleep at night, and even it is up against it with Prednisone, which is a powerful opponent.

At this point, after a Christmas, a New Year, an Easter, a Memorial Day, and hot weather--still no call from the transplant center. But at least it appeared that transportation was kind of getting worked out. Judy diligently pursued 3 or 4 different avenues, but we never knew what we would actually do if and when we got the call. After 13 months on the list, things were not moving very quickly. So we hoped. And I prayed a lot to Saint Anthony (I call him Big Tony) for all the caregivers that had given so much to get me so far. To let them down after such an emotional investment would have been too much to bear. But on an upbeat note, we were at that point past Preakness Week, and hotel rooms would be more readily available in Baltimore should you-know-what happen--maybe God had a plan, I thought.

Hoping, Phase III--June 9, 1997--The Call!

It happened; it finally happened, and at a time when it looked like it never would. Words still cannot describe the sense of overwhelming obligation I feel towards my donor and his (or her) family, the personnel at Lankanau Hospital here in Pennsylvania, and of course the entire team at the University of Maryland. To say that they saved my life is simply the truth, but beyond that, they saved the lives of many, many people that worked so hard for so long on my behalf. If this account helps others in the same position that I was in to continue hoping for help that can be there, then I feel that I have at least contributed a little towards relieving the great debt that I owe. In many ways, this entire experience has been a miracle, and a gift.

For the entire transplant story, including the final days of the disease, the call, and the recovery, click here. Judy and I spent most of the entire months of June and July, 1997 in Baltimore, MD, as (sort of) the guests of the University of Maryland Medical Center (UMMC), and their Thoracic Transplant Organization. I promise that there a lot pictures there! Sorry there aren't more here, but I didn't get a digital camera until a little too late in the process, and I didn't want to leave a legacy of pain and suffering behind, either--especially if things didn't work out. Look out, though--there's a big shot of THE SCAR.

Resources on the Net

Originally, when I first started to look, there wasn't a whole lot of information up on the Net on all this, but there is a lot more now, or maybe the search engines are just more finely tuned. I know that my website itself gets maybe 10-30 hits per day, especially since I put those little fine-print keywords on the botom of my homepage--a little webmastering trick there. In any case, the following is an early list I used to get some information, but you can probably do a lot better yourself by now.

For information on organ transplants and becoming an organ donor, click here.

To get back to the WA3FLE homepage, click here.

Copyright © 1997-2006 by Roger W. Stevens. All rights reserved.

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