6 diseases I would hate to have.

Recently I’ve been spending a lot of time studying different diseases, in particular genetic ones, and I am having this growing sense of fortuity and gratefulness that I was born without a genetic disease. In fact, given the high probability of some of them occurring, I find it amazing that I don’t have one. Some of them are not too bad, you know, treatable and all that, but some of them are pretty awful. The long and short of it is, if I had to choose a disease to be born with, the following are ones I would definitely not choose.

1. Being male. You might not think this is a genetic disease, but if you are unlucky enough to be born with 5-alpha-reductase deficiency, you might look like a normal female but then discover when you hit puberty that you start to grow facial hair, big muscles and little genitals that seem to come out of nowhere. Yes, it’s true: you are genetically male (46,XY chromosome) but phenotypically (how you look on the outside) female, at least until those hidden testicles start producing testosterone and turn you into a he-she. Gender reassignment is an option, but apparently this disease has a high rate of psychiatric suicide.

2. Werewolf syndrome (hypertrichosis). Yes, it’s just how it sounds: due to a genetic defect, thick dark hair starts to grow all over the body (except hands and feet), including all over the face, transforming the unfortunate sufferer into a real-life vision of the mythical creature. Sure you can get the hair removed, but waxing your whole body has got to be a bit of a pain.

3. Elephantitis. Not a genetic condition; it is caused by infection from a parasitic worm transmitted by mosquitos. It causes extreme edema (buildup of fluid in tissues) and most commonly causes extremely swollen arms, legs, breasts and genitalia. And believe me, guys, a 2-foot long penis is not cool.

4. Jumping Frenchman disorder. Originating in French-Canadian lumberjacks, this disorder involves hypersensitivity to being startled by unexpected noises or sights. We’re talking REALLY startled: they flail their arms, cry out and repeat words. Sometimes I think I have a mild form of this disorder as I have a very well-developed startle reflex, but apparently this is a hundred times worse.

5. Maple syrup urine disease. Not as sweet as it sounds, this genetic disease was named due to the maple syrup like odor of urine, due to an increase of certain amino acids being excreted in the urine. Because of a genetic deficiency of alpha-ketohydrogenase enzymes, the buildup of these amino acids causes toxicity to the brain and extreme mental and physical degeneration (seizures, etc.) leading eventually to death within a few years of birth. This disease is only common in people of Mennonite or Pennsylvania Amish descent though, which pretty much rules my family out.

6. Hirschsprung disease. It’s really not as difficult to pronounce as it looks, and if you don’t mind the treatment, I guess it’s not too crappy of a disease to have. Basically it results from a lack of nerve supply to the lower bowels, which causes a lack of the defecation reflex- even though the bowels are full there is no signal to go, so feces builds up and causes bowel obstructions. Treatment is either removing the part of the colon that is ‘aganglionic’, or more commonly, inserting a finger into the anal canal each day to stimulate the bowel to empty. Again, if you're good at putting up with crap, it's not too bad.

I could go on….there are thousands of these disease. The scary thing is that just like jumping Frenchman disease, many of us have mild forms of all sorts of things wrong with us- yet we live with them everyday and get on with our lives. We’re not perfect, none of us. This world isn’t perfect. We’re blessed to have the things we do have, especially our health, and remembering that should help us to be more compassionate to the people we encounter who do have some of these diseases.

Be careful what you wish for

The last few days I have been thinking about a certain topic and today I had the chance to learn the lesson the hard way.
The Israelites, when they were wandering in the desert for 40 years, complained about their diet and God granted their request and sent them quail to eat. However, it was not without a cost. Psalm 106 says "He gave them what they asked for, but sent a wasting disease among them." I guess those Israelites thought they knew what they wanted, but it turned out to be a little more than they bargained for.
All of us humans are like that. We have this idea about what we want. It may even be a legitimate and good desire, and we cry out to God and pray for it. But sometimes the unfortunate thing is, he answers our prayers and gives us what we asked for, but at the cost of something. Until we learn to want what God wants, not what we want.
So I experienced this firsthand today. This week when I finished up my practicum Dr. G asked me if there was anything I wanted to see or do before I was done.
On my list of things to do before I die is 'see an autopsy', so I asked her if she could arrange it.
"No problem! I'll call the morgue!"
The pathologist was happy to have me and I took the stairs down to the cold crypts of the hospital basement and followed the dingy twisted hallways to the sign hanging from the ceiling that said 'Morgue.'
I came in and they got me scrubs and shoe covers and led me into the brightly lit room where autopsies were conducted. There was a whiteboard covering one wall that said 'Welcome to the Morgue' and then underneath it, '39 days without a maggot'.
Lying on the stainless steel gurney was a body, an older man with a nasogastric tube still hanging out of one nostril and a label around his stiff white toe.
"Shall we get started?" The pathology assistant asked.
I helped her take off his gown and using a ruler, measure him and his hair length and his scars and marks, etc. He had been dead a few days but was well preserved and cold and he looked as if he was just sleeping, except for his mouth which was wide open, and his sunken blue eyes, which stared at me where I stood.
He looked slightly familiar, but I thought, perhaps all old naked men look slightly familiar. He had several interesting scars down his chest, and looking at them I immediately deduced he had had open heart surgery, abdominal surgery, and looking at his leg I saw the scar where a vein graft had been harvested from. I felt his chest and through his cold rubbery skin I could feel the sharp tips of the wires that had been used to wire his breastbone together after his heart surgery.
We began to cut him open and dissect out his internal organs, looking for suspicious things. There was hardening of one of his lung lobes suggesting pneumonia. Stiff whitened areas of his major arteries suggested calcification leading to occluded blood vessels and heart disease. In his intestines there was a hard (probably cancerous) lump that had obstructed his bowels.
"Take your pick." the pathologist said. "He could have died from any of these."
Another body was brought in, this one a forensic case (involved in a criminal investigation), so she was accompanied by police officers who spent half an hour taking photos of her body and looking like they were going to throw up at the sight of the already dissected man on the other table.
The woman was freshly dead so her blood hadn't had much time to solidify and when her abdominal cavity was opened up, it was filled with bright fluid.
"Double glove." One of the pathologists told me. "This lady had hepatitis A,B and C. And who knows what else."
Single gloving is appropriate for emptying a bedpan as a nurse but not when you're wrestling to get a ribcage open and the shards of bone pierce through your gloves.
I took a few moments to go look at the chart of the man we'd been autopsying and suddenly I realized why he looked familiar. I knew him: he had been a patient of mine in the hospital. I suddenly had this eerie feeling, remembering him sitting up in bed and talking to me. What if he suddenly sat up from the table and started talking to me again?
One of the assistants took me for a tour of the morgue. He showed me the walk-in freezer that could accommodate nine bodies, and the ceiling-to-floor crypts labeled "Body parts" (he showed me the bags of thumbs and feet), "Stillborns", "Police case", and others. The smell was horrendous. I struggled to control my gag reflex and breath through my mouth.
Back at the table the assistant enlisted my help to 'string the bowels' (removing the tissues holding the intestines together so they could be laid out like a fat string of sausage). At one point I accidentally nicked the intestine and to my horror, the intestinal contents started to escape. There is really no nice way to describe it, so I'll leave it to your imagination.
I stood at the sink with the pathologist and he dissected each one of the internal organs in turn and discussed it with me.
"See the general crater-like appearance of these kidneys? This is an indicator of hypertension" (high blood pressure). "And this ovary? It's very tiny, which is likely because the woman was going through menopause."
He slipped one of the lungs into a metal basin hanging from the ceiling to weigh it.
"See how this one is twice the size of the other lung? She had significant pneumonia, and all this clear fluid oozing out of the tissues is pus."
As I was standing there a realization suddenly hit me. Some gross things in life I can handle. Some gross things I can put up with once and a while. But this is disgusting. I never, ever want to work in a morgue. In fact, I don't even want to be here right now.
It was several hours before I could take my leave. I tried to wash the formaldehyde smell out of my hands but I'm pretty sure it's there for a while. I walked back along the dingy hallways and thought, now I can cross that off my list. But strangely, there was no joy.
It reminded me of bigger, more important things in life. I often think I know what I want, or what I need. But really a lot of the time I have no clue. They say you should be careful what you wish for because you might actually get it. Personally I think I'm going to take that to heart and re-write my list-of-things-to-do-before-I-die tonight.

Faith

In a year or so I will be beginning my clinical clerkships- where I'm working full time in a hospital as a medical student and learning as I go. Clinical clerks are required to get a license, and if I want to do these practicums in BC, I have to get a license through UBC. I went onto their website the other day to look at the procedure and find out when I needed to apply for them.
Under no circumstances, it stated, does UBC grant electives to students who are not from a medical school in the following list. I read it carefully. Foreign medical students were allowed from Ireland, South Africa, USA, and several other countries. No Caribbean schools.
Now, I have no idea why they wouldn't allow Caribbean students to do electives here, but as I read that I had this distinct feeling: If God has called me to go to medical school, he has the responsibility to make sure that I graduate and get the appropriate licenses. I laughed to myself. Another opportunity for God to work miraculously!
I admit I felt slightly discouraged when I went to bed last night. It appears that this whole journey for me of going to Antigua has been a huge journey of faith. Actually, it started long before then. And at every step along the way, I have become more and more convinced of God's trustworthiness. I remember being 17 and applying to nursing school and being told it wasn't possible. Yet God had other ideas. When I wrote entrance exams I scored the highest marks in the history of the college and they made an exception and let me in. Am I that smart? Not really. Especially when it comes to math.
But you know what? God is that smart. The fear of the Lord, I read this morning, is the beginning of wisdom. Not all of us have a chance at being brilliant, but we all have a chance to fear the Lord. And 9 times out of 10, if I could choose wisdom over knowledge, I'd choose wisdom. (And the other 1 time would be when I was trying to show off.)
This morning I called the organization that licenses all physicians and students in BC, and I asked them if I could skip going through UBC and apply for a license through them. You'll have to send in an application, the woman said. You mean there's a chance? I asked. Yes, she said, you can apply. That's excellent, I told her.
I got off the phone and thought, if they will allow me to put in an application, then God can see that it gets approved.
The trick is: I've learned to let it go now. It's not that I don't care, because I do, but it's that I recognize that God's plans are way better than mine and as much as I like to think I do, I often have no clue what God is doing. Trusting him means putting everything on the altar, so to speak, and giving him permission to do what he wants with my life. Maybe that means I'll be a wealthy Canadian doctor. Maybe I'll be a poor African doctor. Maybe I'll never graduate from medical school and end up working in a florist's shop. (Actually, that is definitely my second choice in careers)
God can absolutely be trusted. I know that because I've seen him work miraculously, especially in the last few months, to provide things for me and make a way in a place that seemed impossible. I know that neither a licensing board nor a large sum of money nor any other thing is intimidating to God and can stand in his way. He is the one who decides what happens on this earth. God can be trusted.
Some people have huge mountains to be moved. I only have small ones! But I am watching God move them. And if any of my dear readers reading this today are feeling discouraged about something in their life, an unanswered prayer or an impossible situation, let me encourage you. God can be trusted! He will move your mountain for you!
"Now faith is the substance of things hoped for, the evidence of things not seen."

Anesthesiologists and barbequed steak

Today I had my last day of my cardiology practicum and I felt sad that it had come to an end. In the morning I went to rounds with Dr. G and sat with all the surgeons discussing cases that had gone well or hadn't gone well. Dr. G and I went and saw a patient with serious endocarditis (an infection of the heart valves) who was waiting for surgery, and then I said goodbye to her and went over to spend the rest of the day with another surgeon watching pacemaker implants.
I had previously watched open heart surgery with Dr. H and he was a good deal kinder and gentler than Dr. K. However, while he wasn't brash and intimidating, I still felt trepidation approaching the OR. The nurses were happy to see me and the anesthesiologist started pimping me.
"How can you tell the difference between first, second and third degree heart block?"
I fumbled for an answer. We were walking down the hall together before the first case started, and suddenly we encountered two medical students. The young woman was obviously a student cause she had a brand new stethoscope and looked nervous. The man was older but I thought, well, medical schools take all types.
"Hey, Dr. P!" The man said to the anesthesiologist. "How come you've got a Calgary medical student with you?"
"I'm not from Calgary." I said. "I'm going to school overseas."
"Overseas?!" He gave me a funny look.
"Well, what's your name? Where are you from? Obviously you must be a mature student." He eyed me up and down.
(A mature student is one who is either a little older or has had extensive experience before coming to medical school.)
"I'm Heather." I stuck out my hand to shake his. "Thank you for calling me a mature student. But how old do you think I am?"
He rolled his eyes.
"Oh boy. Never answer that question if a woman asks."
"Well, I'll take it as a compliment that you think I'm mature." I said graciously. "But I'm probably younger than you think I am."
"Where are you going to school overseas?" He demanded.
"Before you ask me anymore questions, why don't you tell me what your name is?" I answered back. "I hardly think it's fair that you can quiz me on my age, occupation and nationality without answering some of my questions."
He gave me an astonished look. "Who do you think you are?"
"Who do you think you are?" I said right back, and then smiled. "I'm a student with Dr. H and I'll be in the OR today with him."
Dr. P started laughing.
"Heather, this is Dr. S and he is the head of anesthesiology at the hospital. He's the person who will be giving you a rotation here if you ever apply for one."
I could only smile wanly. "Pleased to meet you", and I walked down the hall with him staring after me, feeling like the world's biggest idiot.
Later when I was in the operating room the door suddenly opened and Dr. S stuck his head in.
"How's that lippy medical student treating you, Dr. H?!!" came a shout.
Dr. H looked at me surprisedly, because I had been quite demure up to that point.
"Where did you find that foreign student, anyway?!"
"He picked me up at immigration!" I shouted back.
The nurses started to laugh and Dr. S shook his head with a grin and closed the door again. Oh, Heather. You sure got lucky that time.
Dr. H was obviously amused and he offered to take me and another nurse for lunch. When we were on our way back up in the elevator I summoned up enough courage to pop the question.
"Dr. H, if it's not too busy this afternoon, can I scrub in on one of the cases and help out?"
"Sure! No problem." He said kindly.
He went to bring a patient down and I stood in the OR with the scrub nurse and she went over all the names of the surgical instruments with me and I tried to memorize them. Pick-ups. Toothed pick-ups. Snaps. Pott-Smith Scissors. Volkmann's retractors. Needle drivers. Dissecting scissors. Diefenbach blades. It went on and on.
We started the surgery and I stood and held tissue back with a retractor while Dr. H cauterized cut blood vessels. The smell of the sizzling flesh was just like barbeque and I envisioned myself gagging at a summer party next time I tried to eat steak. I swabbed blood and snipped the ends of his stitches and held guide wires for him.
I scrubbed in the next case as well and at the end he sutured the deep layers of fatty tissue and then gave me the fine silk thread to suture the final layer of skin. These were the same type of stitches I had failed to do with Dr. K and as I advanced the needle I tried not to let my fingers shake with nervousness. These particular type of stitches are called subcuticular stitches and they go parallel to the incision, just underneath the very top thin layer of skin. If done correctly, no stitches are visible on the outside of the incision, and more importantly, no puckering of the skin to leave a scar.
Dr. H watched me closely while I sutured, offering no comment, and as I pulled the last few ones through he said,
"You did a beautiful job", and then, "I'll leave you to finish up then."
He walked away, tearing off his gown and the scrub nurse handed me the instruments I needed while I finished with the incision, cleaned it up and then covered it in steri-strips and bandages.
He said I did a beautiful job. I helped transfer the patient to a gurney and washed up, and then floated down the hallway. Dr. H stopped me at the door.
"Thank you for coming today." He said. "It was a pleasure working with you. If you ever need a reference or would like a practicum or something, please don't hesitate to call me."
He said I did a beautiful job.
I changed into my clothes and went out to my car and as I drove down the road I felt a different kind of tears pooling in my eyes.
Over the last few years lots of people have asked me, why don't you just stay being a nurse? What makes you think it's going to be different being a doctor? I honestly haven't been able to give a clear answer, except somehow I believed that if God was calling me to go to medical school, which I truly feel he is, then he would make me love it.
When I woke up this morning my first thought was, yay! I get to go to surgery today! And I leapt out of bed. I wish I was going tomorrow. I wish I was going the next day. I wish I was going the day after that.
Success isn't about making piles of money or having a job with status or looking good (but you never know, I may have all of the above someday in the future). Success is about finding yourself in the center of God's will. I may have a negative balance in the bank and I may be just a lippy medical student and I may look ridiculous in my oversized green scrubs and 30-pound lead apron, but I know that where I am right now is in the center of God's will.
Now if only I could learn to hold my tongue around anesthesiologists, I might just be okay......

Facing my personal El Guapo

For those of you who haven’t watched ‘the three amigos’, El Guapo is a synonym for something that needs to be overcome. Another way to say it would be ‘facing my dragons’.
Either way you say it, today I had a chance to face my personal El Guapo. I wrote a few days ago about Dr. K, the cardiac surgeon who let me help out in surgery during the brutal day when I got yelled at and pimped by everyone. He is an incredibly brilliant and gifted surgeon, but aggressive, obnoxious, and intimidating. After getting told off for not suturing properly, I dreaded the thought of facing him again. When I saw him last week at the seminar I cringed and hoped he wouldn’t talk to me. Alas. Avoiding El Guapo was not my destiny.
Today my supervising doctor sent me to go watch open heart surgery with Dr. K. I had stayed up Monday night with a piece of chicken I’d thawed from the freezer and practiced suturing for hours. The needle kept slipping out of my tweezers and I picked it up again and again, getting the needle tangled in the thread when I tried to tie knots. As I approached the hospital I felt slightly sick. What would I say to Dr. K? Even though I’d worked hard, I didn’t feel like my suturing skills had improved.
When I walked into the operating room nursing station the head nurse shook her head when I explained who I was.
“This morning’s case is a very complicated case and it would be better if you didn’t come. You can return in the afternoon, if you’d like.”
I suddenly felt a sense of injustice. I was here to face my fears and learn how to be a good doctor. Just because it was inconvenient for the staff, I wasn’t going to back down.
“I was told to come today.” I argued.
“I’m sorry.” The nurse said.
Suddenly Dr. K came striding towards us, saying something to the nurse about something he was annoyed about.
“Dr. K!” I said quickly. “Can I watch your case this morning?”
He barely looked at me. “Sure. Whatever.” And walked away.
The nurse gave me a cold glare but I felt a thrill of victory. I ran after her to the change room and went in, changing into scrubs and tucking my hair under a net and my shoes into shoe covers. And I went to the OR.
Generally medical students just stand and watch unless they are in their 3^rd and 4^th year doing their clinical rotations. I found a stool and perched at the head of the patient, next to the anesthetist, who was very kind and explained things to me as she went along.
Dr. K breezed in with the surgical assistants and began the surgery. The patient was a complicated case as he had already had heart surgery and this was a ‘redo’ valve replacement, with one of his major arteries being stuck to the inside of his chest. I watched and asked the odd question and was asked the odd question in return. Dr. K kept making rude jokes and then laughing at me. I didn’t think he could see me blush under the mask, but perhaps he could read my eyes. I tried to keep my head up and I made the odd joke. Dr. K said something about my sewing skills and I said, “Hey, I’ve improved! I stayed up last night practicing on a chicken.”
The surgical assistant and Dr. K both turned incredulously. “A chicken?”
“Just a piece I thawed.” I explained. “It’s more like human tissue than a grapefruit, after all.”
An hour later or so, Dr. K spoke to me without looking up from the open chest cavity.
“If you stay for the next case”, he grunted, “You can scrub in and I’ll let you suture on the leg graft.”
I was ecstatic and under my mask I’m sure he could tell I was grinning.
“Anyone who tries as hard as you,” he continued, “ought to be given a chance.”
By the time the next case came around I was feeling trepidation. Would I be able to suture after all? I watched the beginning of the case from my vantage point and there was a new surgical assistant who was very nice and explained things to me.
“I’m going to scrub in for this case.” I told her when she asked.
“Oh, this is probably not a good case for a beginner.” She said nicely. “But on another day you might be able to scrub in when we have time to teach you.”
“Oh, she’ll be fine.” Dr. K spoke up, waving his forceps at me. “She’s scrubbed before and she knows what she’s doing.”
Oh dear.
I went into the hallway and scrubbed my arms with iodine soap and rinsed them under the tap, opening the door with my shoulder and holding my clean hands up in the air. One of the nurses gave me a sterile towel and then draped a gown around me and tied it in the back, and then slipped surgical gloves over my hands. I took my place at the bedside next to Dr. N, the surgical assistant, keeping my hands up and trying not to fog up my glasses when I breathed. O God, please help me get it right.
For cardiac bypass surgery, it is common to take out a surface vein in the leg called the great saphenous vein and use it as a graft in the heart, turning it backwards so as to reverse the valves. One of the surgical assistants had made long cuts down the length of the man’s leg and had dissected out the vein and removed it, and it was lying like a fat worm on a sterile sheet while he closed the leg back up together.
He put the needle grippers and tweezers with needle attached into my hands and showed me where to put the first stitches. As I started to put the needle in I had to still my shaking hands. I fumbled with the instruments for a while and managed to get the stitches in.
“Do you know how to knot?” He asked me.
“Sort of.” I started to do it the way I had seen Dr. K do it and my hands got all tangled up. Finally I put down the needle grippers and began to wind the thread around the tweezers and knot it by hand the only way I knew how.
“Oh, you do it that way.” The surgical assistant said. “That works too.”
I finished suturing one cut and then I did a second one and used the stapler to close them up.
“That looks good.” He complimented me.
Let me tell you, that one little line made my day. I felt like I had slain my dragon right then and there.
“Stand closer.” Dr. K ordered me. “You might as well stay scrubbed in and help me.”
And he let me assist for the rest of the afternoon. At one point a suction tube came loose and sprayed blood in the air and all over Dr. K’s front. He looked up at me.
“Is there blood on my face?”
I smiled. “No, you look good.”
The nurses started to laugh. “She said you look good, Dr. K!”
“You’ll do fine here.” He said with a grin.
I stayed 11 hours and when we finished I stapled the man’s chest closed, swabbed up the blood and then covered his incisions with smooth white bandages. It felt wonderful.
I’ve always said I won’t be a surgeon. I didn’t want to spend the rest of my life staring at 4 square inches of a person’s body. But there’s something about rising to the challenge that appeals to me. I don’t know if I’ll be a surgeon, but I do know that avoiding my El Guapo, whoever or whatever he may be, is not my destiny.
“For God has not given us a spirit of timidity, but of power, and love, and a sound mind.”