Day 4, April 29 – The Long, Good Friday

Okay, it isn’t Friday evening and I’m not writing this having just completed my appointments for the day. It’s Sunday and we are home. We were up at 3am on Saturday morning, took a taxi to Ronald Reagan Airport and arrived back in Seattle at about 10.30am. Melissa, Michelle’s sister, was there to take us home, and we fell in through the front door at about 3pm.
Needless to say the house was a tip – not because we’d had burglars in our absence, just because we left it that way. No matter. Michelle and I gave the kids dinner, put them to bed very early, and then had a romantic meal and sat up chatting about the week into the small hours with a nice glass of Columbia Valley.
Hahahahahaha. NOT!
Michelle fell asleep on the couch at about 7.45 while I flipped channels for a while before falling asleep myself about 9pm. But with the kids so tired, they were bound to wake up at 8.30am so we’d all have a pleasant lie in.
I think Sam came trooping into our room first at about 5.30am, closely followed by Madeline.
But none of this tells you anything about the fourth and final day which turned out to be the busiest. It’s inevitable during a week like this, one or more of the doctors you saw will want to do a follow-up. Dr. Perry in Rehabilitation had spoken earlier in the week about the possibility of getting me a Transcutaneous Electrical Nerve Stimulation (TENS) unit and, after having located one, wanted to bring it over to my room at 8.15 in the morning.
Well, I was fast asleep as 8.15 and neither Dr. Perry nor the nurses wanted to deprive me of a little sleep, so they let me be and rescheduled the visit for later in the morning. It was therefore a markedly more leisurely start to the day – none of the 5am wake up call with IV line insertion of Thursday, or 7am blood draw of Wednesday.
My first appointment was in the ultrasound department at 9am when my kidneys would be on view. Before then though I had the good fortune to eventually meet the wonderful Joy Bryant, a clinical research nurse, who had set up the whole visit. She more or less took control of the day, rescheduling a few appointments so I’d be sure to have a shower and some breakfast before four in the afternoon which seemed to be happening a lot.
Alkaptonuria carries a relatively high incidence of kidney stones but thankfully the technician at the ultrasound unit saw no sign of one – like I’d need a technician to tell me if I had a kidney stone!!!!!!! I was staying in a Belfast B&B about 12 years ago when I had my first and, mercifully, only kidney stone so far. It was 4am and try as I might to keep my cries of anguish to a minimum I failed. The landlord came running into my room and immediately drove me to the doctor’s office where I was given a very strong painkiller that somehow enabled me to get back to London in relative comfort.
I’m sure I’m embellishing the story a bit because it doesn’t explain what the doctor was doing in her office at 5am. Perhaps the landlord had phoned her and told her one of his guests was freaking out and she had to meet us at her clinic. Perhaps she liked to work late/early. Whatever the truth, I will never ever forget the pain…which, actually, is too mild a word.
Anyway, the kidney ultrasound went smoothly enough, the only problem being the very cold gel the technician spread over my sides and my yelping like a little puppy.
From the ultrasound I went to the Rehab clinic where an associate of Dr. Perry’s hooked me up to a TENS unit. A TENS unit is designed to create a feeling at the site of your pain that blocks the actual pain -or something like that. You have control over the strength of the stimulation and at what interval the pulses come, but basically it feels like a small electric shock is happening wherever you place the pads.
After some experimenting, I found a location, strength, and interval that seemed to work and I was very pleased to see it had a belt latch on the back meaning I can wear it when trying to play some golf this summer. It might be against the Rules but, let’s face it, I’m not going to be winning any prizes. Just to play a few holes and hopefully be given the opportunity to teach again would be absolutely fantastic. I helped coach First Tee kids at Lake Padden in Bellingham last year and can’t tell you how much I enjoyed it. I also helped the Director of Instruction teach a class of adult beginners which was equally enjoyable.
In addition to the TENS unit I also walked out of the Rehab clinic with a back cushion that pushes the lower spine out away from the chair a little thus preventing it from becoming too flat.
Back in my room, Joy saw to it that I had time to eat my breakfast, and asked the clinical photography department, with whom I had a 10.30 appointment, if they could do their thing in my room rather than have me go to them.
Ten minutes later the photographer was at the door. She taped some blue material to the wall and had me stand in front of it facing her. She took a few shots face-on, and had me turn through 90 degrees in both directions. She then came in for a few close-ups, capturing the whites of my eyes (which can be discolored) and my ear lobes (which already are a bit). She took a close-up of my hands and fingers, and was gone in ten minutes which gave me time for a quick snooze.
But Dr. Siebel walked in. He handed me a copy of ‘The Hunger Games’ which we had spoken about a couple of days before and which he had bought for me while out shopping the previous evening. I don’t recall ever having been given a present by any other doctor in my 40 long years, but that’s what NIH doctors are like (disclaimer: NIH doctors are not obliged to give their patients gifts).
A doctor from the multiple sclerosis department was the next to visit. I think the combination of MS and AKU was something of a talking point, and they wanted to investigate, even though the NIH visit had nothing to do with MS.
I was looking forward to my next appointment. Any time you get a chance to visit Dr. Perry you say ‘which room?’ She had compiled a print-out with 34 exercises and stretches she wanted me to practice at home. Some I had done before, some were new to me.  She also had me demonstrate each of them to make sure I was able to execute them correctly. She gave me a few other back pain ‘toys’ to try out too.
I was in Dr. Perry’s office for two hours making a total of five with Wednesday’s three.
And with that I was done. I returned to the room, packed up my gear, had a final round of vitals testing (blood pressure improving all the time), and one last conversation with Dr. Siebel who had been such a marvelous help and encouragement throughout.
I phoned the Bethesda Court Hotel and ordered the limousine. It turned up half an hour after it said it would, so I had a chance to do some stretching on the benches at the entrance – yes, I got several looks from passers-by.
When I got back to the hotel, Michelle and the kids were there having spent the day at the Treasury seeing how money is made. We hit the town and found a burger joint where we wolfed down the biggest, fattest burgers and fries I’ve seen in a while.
We were in bed by 9pm and seven hours later sat in a taxi en route to Ronald Reagan National Airport. As the wheels came up, we looked down and, directly below us almost, was the Pentagon – one last, cool landmark to add to all the rest.
My thanks go to Dr. Siebel, Dr. Perry, and Dr. Rosing for their expertise, and Dr. Introne in particular for the ground-breaking work she is doing on behalf of AKU patients around the world. Without her research, there would be no hope of ever finding a cure (or an effective treatment that reduces its effects at least) for AKU. I also want to mention all the nurses and technicians that made the visit so memorable. The NIH is an unbelievable institution, and if there are any AKU patients out there that haven’t yet been part of Dr. Introne’s study, I recommend it without hesitation, and encourage you to sign up. The more data Dr. Introne can gather the better.


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Day 3, April 26th – The ‘I hate Cardiac CT Scans’ Day

Forgive me; I suspect tonight’s entry will be considerably shorter than the first two. Fact is, I am spent; exhausted; utterly drained; or, as English readers would say, completely knackered. Today’s schedule involved only two procedures, neither of them particularly pleasant.
I was up at five to prepare for a 7am cardiac MRI appointment. The nurse put an IV line in and I was taken in a wheelchair on a 20-minute ride into what seemed like the bowels of the hospital. I was half-asleep and not really paying attention, but I’m pretty sure we went underground. We passed some building work, must have turned 25 corners and ended up in a place where everyone seemed to have a different accent. ‘Wow, we must have gone a long way,’ I thought.
It felt quite weird to be so far from the rest of the hospital. I thought this must be where the screaming goes on, where people’s cries for help can’t be heard. The MRI staff though were just as expected – friendly and professional, even at seven in the morning. Cris got me ready while Marcia helped me through all the paper-work. I kept asking her inane questions because I liked listening to her brilliant accent. She sounded like Rosemary the receptionist/dispatcher at the police station where Number One Super Guy Hong Kong Phooey worked as a mild-mannered janitor…’Hello, hello?”
I usually look forward to MRIs of which I usually have one a year to check my brain for new multiple sclerosis lesions. I can lie still and sleep for an hour, oblivious of the loud and repetitive sounds going on all the time.
But this one was different. Cris issued breathing instructions throughout the entire procedure, asking me through the headphones either to ‘take a breath, blow it out, stop breathing (for eight seconds)’, or ‘take short, shallow breaths (for 20 seconds)’. Obviously I had to remain conscious and do as Cris instructed or the images would not have been want Dr. Introne wanted. Cris also pushed gadolinium, which is used as a contrast agent, in through the IV line from her chair in the room next door.
The hour went by very slowly and my back was in a lot of pain which made it the most unpleasant MRI I’ve ever had. That was no one’s fault, of course. I just had to stay awake and my back would have hurt no matter what position it was in. Whatever, I was not unhappy when Cris announced we were done and I could breathe normally and move about a bit. When I came out of the tube, I immediately brought my knees to my chest which felt wonderful. But Cris still needed to help me up off the table.
She took me back to the waiting room where I watched the ESPN highlights of last night’s Real Madrid v Bayern Munich Champions League semi-final (I don’t think Chelsea will beat the Germans in the final) then promptly fell asleep. I think I had been asleep for an hour when Marcia came and collected me for the Cardiac CT Scan – ‘hello, hello, wakey, wakey, anyone there?’
Things hadn’t gone well so far this morning, but they were about to get worse. Marcia discovered my IV line was no longer any good. She tried pumping some new liquid into it to flush it but felt a lot of resistance. She had to take it out and put another in on the other arm. This one didn’t work either so she went to the radiology department to find someone who outs IV lines in all day.
This lady found a vein and inserted a line without any problem. She then covered it with some tape to hold it in place. Just as she got up to leave she said ‘That’s going to hurt – not the needle, taking the tape off.”
Of all the pokes, prods, pills, clinics, procedures, surveys, scans, and tests I’ve ever had in a hospital or doctor’s office, it is the removal of the IV that I fear most. I think I can tolerate discomfort pretty well, but I dread the moment the nurse tries ripping the tape off my arm. It is excruciating…that is to say, bloody painful.
Anyway, I got sort of comfortable on the moving bed and went into the scanner. Again I was asked to alter my breathing pattern, then after a while was given a pill that dissolved under my tongue and made my blood vessels show up better. It also produced an almighty headache which I was told usually took three minutes to disappear but which stuck around for at least an hour. As well as the headache-inducing tablet, an X-ray dye contrast agent was pumped in through the IV and produced a very unpleasant burning sensation predominantly in my ears.
Again, I was thrilled when the technician said we had finished. He walked me back to the waiting room from where a porter took me back to my room. It was 2pm, and to be honest I felt awful. I hadn’t eaten all day, the headache was still present and I was very tired. Back in the room I fell asleep for two hours. I woke up to find Michelle and the kids in the room. Had it been anyone else, I might have asked them, as politely as I could manage, to leave. But I hadn’t been with them for a couple of days and was very excited to see them now.
The thought of the IV removal still lingered in the back of my head though, and I sort of froze when the nurse came in to remove it. ‘Be gentle with me,’ I pleaded.
As luck would have it, she had found some adhesive-remover and took the tape off without too much bother. I wouldn’t want to have it done every day, but it was 1000% better than  expected and another reason why I love the NIH.
The nurse left and Dr. Introne came in with Dr. Siebel to give me the wrap-up (I have some more tests tomorrow, but Dr. Introne won’t be here).
Together the doctors took me through their findings, explaining the results of the urine test, eye exam, blood test, EKG, pulmonary function test, skeletal survey, and Dexa scan in a great detail. It was revealing and very useful, if a little overwhelming. The vast majority of the results were encouraging said Dr. Introne, but my blood pressure was still a concern. (Fortunately, tonight I have had two very much improved readings.)
Dr. Introne and Dr. Siebel were in the room for maybe an hour and, just before leaving, were joined by Dr. Douglas Rosing, head of the Cardiac Consultation Service at the National Heart, Lung & Blood Institute of the NIH. He and two of his staff had come to talk blood pressure.
When all the doctors had left (about 6pm), I was able to have a shower at last, have some lunch (followed an hour and a half later by dinner), and sit down with Michelle and the kids who had spent the day in Bethesda, Sam doing his homework in preparation for his return to school. Tomorrow they are off to the Treasury to see how money is made. I told Maddie to stuff as many $100 bills into her pockets as she could.
‘But you can’t Daddy,’ she said. ‘They’ve got cameras.’

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Day 2, April 25th – The Pee in My Bag Day

This is hardly the place to go all political on you, but ever since landing in the States ten and a bit years ago I have had a problem with the ‘system’. In the UK, where I was born and lived until I was 30, I was able to take advantage of nationally-funded healthcare which basically meant any time I went to see my doc about a sniffly nose, broken leg, demylinated nerves (multiple sclerosis) or discolored urine (alkaptonuria) it was free. We paid for it with taxes of course, but basically it was great. Market-driven healthcare just seems wrong somehow.
A brilliant neurologist told me last year that while America may well have the finest doctors and medical specialists in the world, it has the worst healthcare system. I was lucky to meet and be treated by some amazing physicians in Britain so I’d add an asterisk to the first statement, but I tend to agree with the second.
Here’s the thing though…whatever system gave the world the National Institutes of Health is alright by me. Yesterday’s suspicions about this place being the best medical institution I’d ever been in were confirmed today with another series of tests that ran like clockwork – Rolex clockwork.
It started when a nurse walked into the room at 7am to draw more blood -apparently they didn’t find what they were looking for in the 14 vials they took out of me yesterday! Six more came out this morning after which I had a few minutes to get ready for a Dexa scan in the Nuclear Medicine Department on the first floor.
Katrina, the technician, explained the scan would measure  the density of my bones which is useful info as people with  AKU are prone to osteoporosis. The scan took quarter of an hour and was totally painless. And Katrina was so personable she made the whole thing rather fun. From there I went across the corridor to Radiology where two X-Ray specialists did a full skeletal survey. That took half an hour as there were plenty of images they needed to get, and from several different angles. But again, it was pain-free.
After returning to my room, I did have time for a five-minute shower but sadly the breakfast on my table had to remain uneaten as I was whisked off to Rehabilitation and what was undoubtedly the highlight of the day.
Make no bones, Dr. Monique Perry is the kind of doctor you want to see. Not only does she obviously know physiatry and rehabilitation medicine like the back of her hand, she is gentle, funny, sensitive, professional, and above all caring. My appointment with her lasted three hours during which we discussed my medical history in some detail, she tested the range of motion in my shoulders, elbows, fingers, wrists, hips, knees, feet, legs, and arms, I took a six-minute walk around the department during which she observed my gait, ability to maintain a straight line (rather, inability) and the amount of puff I was producing (plenty), and she gave me numerous suggestions for exercises that will improve my range of motion, strengthen muscles around joints,  and hopefully decrease the pain.
I was almost sad to leave Dr. Perry, but I still hadn’t eaten and my time in rehabilitation had overrun by an hour. Knowing I had a two o’clock appointment with the echocardiogrammer (the wavy red line underneath it tells me that isn’t a legitimate word), I hurried back to my room to have as much of my breakfast (which had now been sat on my table for four hours) as I could cram in.
But halfway there I got a call from phlebotomy asking me to go back and have more blood drawn. The cardiac department had requested some tests that Dr. Introne hadn’t, but I went back nonetheless. Fortunately I was called through to cubicle No. 15 (I think I said there were 12 yesterday. Well, there are 15) fairly quickly but then disaster happened. Because my 24-hour urine analysis was still going on I had to take a pee bottle with me wherever I went. I had ‘performed’ twice since leaving my room earlier in the morning so had a good amount of urine sloshing around securely (I assumed) in my bottle.
You know what’s coming.
The phlebotomist asked what I had in my bag. Strangely, but as it turned out luckily, he also had a peek. He then looked at me strangely and queried my method of urine collection. I didn’t follow him so had a look myself, and saw that the lid had come off. To cut a long and unpleasant story short, we did manage to get it back in the bottle with the lid safely shut. But I’d lost crucial minutes. By the time I got back to the room I surely wouldn’t have time to eat much.
The porter was already waiting for me when I arrived. But she kindly allowed me a few minutes to finish my yoghurt parfait after which we walked the 100 yards or so to 5NE and the echocardiogram room. Typically for NIH doctors and technicians, Mike was very friendly and while he checked my aortic valve for possible signs of calcification, we debated who was the greatest golfer of all time. He said Jack Nicklaus, I said Tiger Woods. Mike is a very persuasive fellow so by the time I left I was agreeing with his theory that, while Woods had played the best golf ever played (1997 Masters, 2000 US Open), Nicklaus had the better record over a longer period of time which had to count for something. He’s right, it does.
Following the echo, I moved across the floor to a pulmonary function testing lab where I was asked to blow hard…harder….HARDER! into a tube a few times. It took 20 minutes at the end of which I felt extremely light-headed.
It was 4pm and I got back to the room feeling beyond tired. But before I could snooze, Dr Introne wanted a word. Most of the results she had seen so far were encouraging, but she was concerned about my blood pressure which had been high for each of the five tests I’d had so far. She tested it herself and discovered that it was indeed beyond what she felt comfortable with. She called a cardiac doctor and within a few minutes I was given some Norvasc, otherwise known as almodipine, a calcium channel blocker used to lower blood pressure. Two hours later my pressure was tested again, but it hadn’t budged. Same again a few minutes ago.
The nurse is now calling the doc to see if another dose might be a good idea. Thing is, I really don’t feel like I have high blood pressure. But as Dr Introne told me, you don’t really ‘feel’ high blood pressure; there aren’t any symptoms as such.
It’s a little concerning, but after the day I’ve had, I’m way too tired to let it keep me from sleeping.
Tomorrow morning I have a cardiac MRI at 8am and a cardiac CT at 12pm. I’m told I’ll be in the MRI department (not the tube itself) for four hours. So I’ll be sure to take the Tiger Woods book I’m reading. I look forward to being impressed by this facility and its staff once again.
And the system? Well, as Tiger is fond of saying, it is what it is.

(By the way, the nurse just came in and said the almodipine is having the desired effect and my blood pressure is where Dr. Introne wants it. Which is nice.)

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Day 1, April 24th – The 14 Vials of Blood Day

As I take the last bite of my lightly-spiced (cajun) chicken breast dinner – which I ordered by phone and which was bought to my bedside in no time at all along with a delicious Caesar salad – I can reflect on what has been a productive, even enjoyable, first day at the National Institutes of Health in Bethesda, Md.
I’m only a quarter of the way through this four-day battery of tests – designed to give Dr. Wendy Introne a better understanding of the condition – and it might all go downhill from here, but so far the experience has been entirely positive (that said, the nurse just took my blood pressure and left the room with the ‘hmm, we might have a problem here’ look on her face.)
The day started early with a 6am wake-up call back at the Bethesda Court Hotel where my wife and two children are staying while I’m here. The hotel’s limousine took me the three miles to the NIH and, after passing through security and taking the shuttle bus to the main Clinical Center (Building 10 of 66 – yes the NIH campus is enormous), I went through Admissions where I think I signed more forms than when refinancing my house. It was still before 8am so I was probably among the first to be admitted which meant the staff were very friendly. I imagine after twenty admissions and several hundred requests to ‘sign here please’ the day starts to drag.
I was taken to my room on the fifth floor of the building and immediately welcomed by the staff nurse who showed me around, explained my schedule and introduced me to Dr. Stephan Siebel, an associate of Dr. Introne who is conducting research here at the NIH that will hopefully lead to an effective treatment for AKU.
It’s a while since I have been an in-patient in a hospital but I don’t remember the admissions process ever being so 1) friendly, or 2) efficient. I immediately felt comfortable; that I was in good hands. It was great to be in a hospital room where every nurse or doctor that came by knew exactly who I was and why I was here – none of this ‘So what’s your name again?’ or ‘You’re here because of Alkaptowhatnow?’
Dr. Siebel took me into an adjoining room where we chatted for an hour about my medical history and what each of the 16 tests scheduled for the week would involve. He also explained exactly what AKU is and why it, and other metabolic disorders, are so rare. I flatter myself when I say I understood most of it…okay, some.
After the consultation with Dr. Siebel, I met Dr. Introne for the first time, and we chatted a little more about the condition and what steps I was taking to minimize its effects. The staff nurse then returned to take all my meds away (they are given to you as and when needed) and begin my 24-hour urine analysis. I have to pee into a bottle then put that bottle in the fridge at the bottom of my bed. I just hope I don’t wake up in the middle of the night a little dozy and go in search of a midnight snack.
A porter then arrived to take me down to phlebotomy and, again, I was a little taken aback, not to say utterly impressed, with how smoothly everything worked around here and how well my schedule was being adhered to.
Before having my blood drawn, I had an EKG which lasted all of two minutes. There was some discomfort when the sticky nodes were removed from my arms, chest, and legs, at the end of the procedure, but I suspect it was nothing like putting one of those hair removal patches on your legs and ripping that off.
I returned to the waiting room, took a ticket, and sat down expecting a long wait. But my number was called about four minutes later. At home it usually takes an hour to have my blood drawn even if I’m the only patient in the building. Today, I was shown into one of the 12 blood-work cubicles where a nurse took 13, maybe 14, vials of blood out of me, the process taking no more than five minutes.
From there, I went back to my room where I ordered some late breakfast – yoghurt parfait, banana bread, tea – and had a snooze.
At 1pm I was taken up to the National Eye Institute on the 11th floor for a consultation with Dr. Brooks. The staff nurse had warned me the eye clinic is one of the busiest places in the hospital so to be prepared for some long wait times. I anticipated an afternoon of clock-watching.
But again, I’d hardly had time to sit down before a member of Dr. Brooks’s staff was calling my name. He did some routine tests before informing Dr. Brooks I was ‘ready for him’. Now came the longest wait of the day – about ten minutes during which I watched CNN and learned what a scoundrel John Edwards was/is.
Dr. Brooks carried out some visual acuity tests and recorded various other data before taking me down the hall for some Fundus photography which isn’t nearly as Fun as it sounds. Wikipedia defines Fundus photography as the ‘creation of a photograph showing the interior surface of the eye, including the retina, optic disc, macula, and posterior pole’. Basically, it consists of a technician shining an incredibly bright light into your eye and taking a quick picture in the split second before you pull your eye away complaining about the brightness. Okay, it wasn’t quite that bad, but after a dozen pictures you are left with a dark patch in front of your eye that takes about 15 minutes to fully disappear.
From the fun of the Fundus, I went to another room for an OCT of the optic nerve head. We all know that OCT is Optical Coherence Tomography, but did you know it can determine the optic disc margin automatically? Me neither.
After having my pupils dilated with drops, the insides of my eyes photographed with an absurdly bright light, and my optic disc margin determined automatically, I had a hard time seeing straight for an hour. I had to walk hard against the wall on the way back to my room for fear of walking into people or falling down several flights of stairs.
I did eventually make it back safely and fell right back to sleep on my very comfy Advanta bed which I can maneuver every which way. When I woke I was delighted to discover I had visitors – my wife and kids who had been to the Natural History Museum in DC. The kids showed me the crystals they had bought in the gift store, and told me about the T-Rex, tarantulas and termites they had seen. Tomorrow they’re off to the zoo while I have a CT Scan, Dexa Scan, skeletal survey, echocardiogram, rehab consultation, and pulmonary function test.
Sounds like a fun day for everyone.

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