Sickle Cell, Junior Year notes from a biology major who happens to be the specimen

Contents: 1. About me · 2. What it is · 3. Trait vs. disease · 4. How I manage · 5. Hydroxyurea · 6. The ER · 7. Resources · Appendix: my letter to professors and roommates

Jessie Bland. 21. Junior, biology major, at a state university here in Alabama. Plan: physician assistant school after graduation, then a job somewhere with a good hematology clinic, for reasons that will become obvious.

I have sickle cell anemia. They found it when I was born, through the newborn blood test, so I've never not known. I grew up in Birmingham with my mother, Irene, who put a water bottle in every lunch she ever packed me from kindergarten through twelfth grade. Other kids had juice boxes. I had a bottle of water with my name on it in permanent marker. I complained about it for twelve years. I now carry a liter of water everywhere I go, without being told, so I guess she won.

I put this page up because I get asked the same questions every semester: by roommates, by lab partners, by professors, by the girl I'm trying to impress who just watched me drink my fourth bottle of water before noon. Also because when I was a kid, the only things I could find about sickle cell were pamphlets with pictures of sad children on the front. I'm not sad. I'm busy. I have organic chemistry.

Standard disclaimer, which I mean: I'm a biology student, not a doctor. This is what I've learned from my hematologist, my classes and twenty-one years of field work. Talk to your own doctor.

2.1 The short version. Sickle cell disease is an inherited blood disorder. It isn't contagious. You're born with it or you aren't.

2.2 The mechanism. Your red blood cells carry oxygen using a protein called hemoglobin. Normal red cells are round, soft and flexible, so they can squeeze through the tiniest blood vessels. People with sickle cell disease have an altered form of hemoglobin, called hemoglobin S. Under certain conditions, especially when oxygen is low, it makes the red cells turn stiff and bend into a crescent, like the blade of a sickle, which is where the name comes from.

2.3 What follows from that.

• Blocked blood flow. Sickled cells are rigid and sticky. They jam up in small blood vessels, and the tissue downstream doesn't get enough oxygen. That causes sudden, severe pain called a pain crisis. Mine usually hit my lower back, my legs or my arms. A crisis can last hours or days. It's the main reason people with sickle cell end up in the hospital.
• Anemia. Sickled cells break down much faster than normal red cells, and your body can't keep up making new ones. So you're short on red cells all the time. That's the "anemia" part, and it's why I get tired faster than my roommate does.
• Infection risk. Sickle cell damages the spleen, which helps fight certain infections. So infections can get serious fast. For us, a high fever is a reason to go to the doctor or the ER right away, not a reason to sleep it off.
• Other complications. It can affect the lungs, the eyes, the kidneys, the brain and other organs. One serious problem, acute chest syndrome, can start with chest pain, fever and trouble breathing, and it's an emergency. This is why people with sickle cell see a hematologist regularly even when they feel fine.

2.4 Variability. Sickle cell is different for everybody. Some people have a crisis every few years. Some people are in the hospital every month. I've been somewhere in the middle. Nobody can tell you for sure how it will go for you.

This is the part everybody gets confused about, so here it is in one table. Genetics, my favorite class.

About one in twelve African Americans carries the sickle cell trait. It also turns up in families from Africa, the Mediterranean, the Middle East, India and parts of Latin America and the Caribbean. If two people who both carry the trait have a child, then each time, there's a 1 in 4 chance the child has sickle cell disease, a 1 in 2 chance the child has the trait, and a 1 in 4 chance the child has neither. My mother and father both have the trait. Neither of them knew it until I was born.

So here is my public service announcement: get tested. It's a simple blood test. Know before you start a family. Not so you'll be scared, just so you'll know.

Every one of these is about keeping my red cells from sickling, or catching trouble early. My hematologist and I worked these out over the years. Yours might be different.

4.1 Water. Dehydration makes sickling more likely. I drink water constantly. I carry a liter bottle to every class, refill it at every fountain on campus, and I know where every fountain on campus is. I am the reason the line at the fountain outside the biology building is long. Sorry.

4.2 Stay warm. Cold can trigger a crisis. The lecture halls here are air-conditioned to roughly the temperature of a meat locker, so I wear layers in August and carry a sweatshirt in my backpack year round. No swimming in cold water. No standing around at a December football game in a T-shirt to look tough.

4.3 No high altitude. Less oxygen up there. My friends go skiing in Colorado over winter break. I don't. I've made my peace with it. I hear the hot chocolate is overrated.

4.4 Rest. I don't pull all-nighters. Ever. Exhaustion and stress are both triggers for me. I study early and in pieces, which, it turns out, is also just the right way to study. I'm in bed by midnight even during finals. My roommate Shane thinks this is the strangest thing about me, out of everything.

4.5 Don't overdo it. I play intramural basketball, but I sit out when I need to, I drink water at every time-out and I don't play in the heat of the afternoon.

4.6 Shots. Flu shot every fall, first week it's offered at the student health center. My hematologist also keeps me up to date on the pneumonia vaccine. Infections are a bigger deal for us.

4.7 Folic acid. My hematologist has me take it every day, to help my body make new red cells.

4.8 Know the warning signs. I go to the doctor or ER right away for a fever over 101, chest pain or trouble breathing, a crisis that won't let up with what I can do at home, a bad headache that won't quit, or any sudden weakness or numbness. My hematologist gave me the list. Get yours.

4.9 Regular checkups. I see my hematologist in Birmingham every three months, and more often for blood work. I schedule them around exams.

In 1998 the FDA approved a drug called hydroxyurea for adults with sickle cell anemia. It's the first drug approved specifically to cut down on sickle cell crises. In a big study, adults taking it had about half as many pain crises as the people who didn't take it. From what I understand, it works by getting the body to make more fetal hemoglobin, the kind babies have, which doesn't sickle, and that keeps red cells from sickling as easily.

My hematologist and I talked about it for a long time, and I started it in the summer of 1998, the summer after my freshman year. It isn't for everybody, and it means getting blood counts checked regularly so the doctor can watch for side effects and adjust the dose. That's a decision for you and your hematologist, not for a web page.

Results so far (n = 1, so read accordingly): my freshman year I was in the hospital four times for crises, including one week in October that cost me a midterm. Since I started hydroxyurea I've been admitted once, for three days last March. I still have bad days. I still have pain. But I've had more good semesters than I ever expected. My sophomore GPA was my best yet, which I would like my mother to know is on the internet.

I'm going to be frank here, because nobody else seems to be.

When you have sickle cell and you're in a crisis, you need pain treatment, and you need it soon. And sometimes you sit in an ER waiting room for hours, in the worst pain of your life, while people who came in after you get called back first. Sometimes the staff don't know much about sickle cell. Sometimes a doctor who has never met you looks at a young black man asking for pain medicine and decides you're there to get high. That has happened to me. It has happened to almost everyone I know with sickle cell. It's real, and it's wrong.

Most of the nurses and doctors I've had have been good, some of them great. But I've learned to speak up for myself, calmly. Here's what I do:

• I carry a letter from my hematologist in my wallet with my diagnosis, my usual treatment plan and her office number. I hand it over at the front desk.
• I say it plainly, every time: "I have sickle cell disease. I'm having a pain crisis." Then I describe where it hurts and how bad, on the one-to-ten scale. I don't exaggerate and I don't play it down.
• I ask them to call my hematologist.
• I bring somebody with me when I can, my roommate or my mother, to keep track of things and to speak up when I can't.
• I stay polite, even when I'm hurting and angry. It isn't fair that I have to. But it works better.
• If something goes wrong, I write it down afterward, times and names, and I tell my hematologist. Sometimes she calls the hospital. Things have gotten better at the ER near campus since she did.

If you're a nurse or doctor reading this: thank you for what you do. And please, when someone with sickle cell tells you they're in pain, believe them.

• Sickle Cell Disease Association of America. National organization with local chapters in a lot of cities. Information for patients and families, education, and support groups. Find the chapter nearest you.
• A sickle cell camp, if you have a kid with sickle cell. The summer camp I went to at ten was the first place I ever met someone else my age who had it. We compared water bottles.
• A hematologist, and a comprehensive sickle cell center if there's one near you.
• Your campus disability services office. If you're a college student with sickle cell, register with them before you need them. They can help arrange things like make-up exams after a hospital stay.
• My letter. The letter I give my professors and roommates every semester. Print it, borrow it, change it to fit you.

Contact: [email protected]. I answer questions from other students with sickle cell, from parents, and from roommates. I don't answer "can you do my genetics homework." (Shane.) Visitors: since January 1999 Last updated: Tuesday, September 7, 1999 (new semester, new letter) Not medical advice. One patient's notes. See your hematologist.