Genetic & Congenital Diseases

What It’s Like To Live With Sickle-Cell

The blood disorder, which primarily affects people of African descent, shows the ways in which racial prejudice can cause the health care system to break down.

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When Damian Jackson was a young man, some lawyers helped his family out. “I admired them,” he remembers. “They were smart, and they helped people out. I thought it would be a good thing to go into if I could.” Now, Damian’s a litigator with close to two decades’ trial experience and around a hundred jury trials to his name.

Damian’s desire to help others out doesn’t stop when he leaves the office. The Philadelphia man spends his free time speaking up for sickle-cell sufferers, helping them access the health care they need during a crisis, and working to raise awareness of a disease that’s largely misunderstood by medical professionals.

Damian Jackson has had sickle-cell disease his entire life.

Damian says he’s lucky. After all, he’s had fifty years of pretty good health. This could be attributed to a balanced diet, enough rest and regular exercise. Or perhaps it’s due to his parents, whose experiences with Damian’s elder sister, also diagnosed with the genetic condition at birth, shaped the way they raised him.

However, despite his good fortune, the defense attorney hasn’t escaped his share of crises – the debilitating bouts of pain experienced by sickle-cell sufferers. Crises are caused when oxygen-carrying red blood cells become distorted, forming a sickle, or crescent shape. The pain is immense, and during his last crisis Damian was unable to do anything else than reach for the phone and dial 911, before spending three days in the hospital.

But these episodes have been rare, and despite a few aches and pains, Damian just gets on living the best life he can: lifting weights and running on the treadmill each morning, going to the movies and spending time with his wife of fifteen years, Dionne. He’s recently taken up golf, and started going to yoga classes.

Damian’s softly spoken and polite as he explains how sickle-cell disease has shaped, but not overtaken, his own life, and how important it is to raise awareness of the condition within the medical community.

Sickle-cell disease varies in its symptoms and severity. How has it affected you?

I am very fortunate that my case has been mild for the most part throughout my life. Thankfully – I’m knocking on wood as we speak – I haven’t had a crisis situation in about fifteen years. That’s very unusual. I’ve had other issues with my sickle-cell, some aches and pains where I think, okay, I need to sit down for a while, but not a full-blown crisis where I’ve had to go into the hospital and be under a doctor’s care.

I know some people who have the disease who are totally disabled, they cannot hold down a full-time job, they can’t do most things for themselves because they have such frequent crisis situations that it affects their day-to-day living.

I did have a wound on my ankle this past year that was very slow to heal, doctors attributed the slow healing process to my sickle-cell, believing there wasn’t enough oxygen getting to that area. That’s a side effect but not something I’d consider as a crisis.

What happens in a crisis?

Normally a red blood cell is circular, but with a person with sickle-cell, theirs are in the shape of a sickle. What happens is from time to time, these cells will start to clot in a certain area, on a blood vessel, and that’s what causes the pain. This is known as a crisis, and it’s an episode of pain, often very severe. Normally it would cause you to receive intravenous pain medication, and be in the hospital for several days, possibly weeks.

What triggered your last crisis?

I’ve always try to say what factors triggered a crisis—was I running myself ragged, did I do anything extra—and I’ve never been able to come up with a cause. I’ve talked with others, and repeatedly people will say I wasn’t doing anything out of the ordinary, it just happened. It’s really unfortunate what happens with sickle-cell it just happens.

My last crisis was about fifteen years ago. Every person who has sickle-cell has an area on their body where they have their crisis, and for me it’s always been my back. I was in such excruciating pain I couldn’t do much more than call 911 and the emergency rescue had to come and take me from my apartment to the hospital because I couldn’t walk at that point. I was in hospital for three days.

Is your good health down to your lifestyle, or is it simply luck of the draw?

I attribute it to the way I have lived. I was diagnosed at birth, and my older sister has sickle-cell disease too, so my parents knew from an early age that there were certain things they could do early on that could potentially help me, like getting enough rest, having a proper diet, things of that nature. Since I had those at an early age they have benefited me throughout my lifetime.

I try to maintain good eating habits, eating lots of fruit and vegetables, I don’t sleep a lot, I never have, but I do try to monitor that and make sure I don’t run myself ragged.

Is there any stigma associated with sickle-cell disease?

One of the big problems with sickle-cell disease is that you’ll have an episode of crisis and go to the hospital and run into a doctor who’s not familiar with you. And you tell them that you’re in pain, and doctors are automatically apprehensive about giving you pain medication. So they might not medicate you enough to relieve you enough, which means you’ll experience even more pain.

Couple that with the fact that SCD primarily affects people of African descent, so you have these African Americans going into the ER seeking pain medication and you get a doctor who’s not familiar with the disease. They can’t give you an objective test like taking your temperature to see how much pain you’re in, and they’re somewhat apprehensive. They have whatever stereotypes built in so they give you the least amount of medication possible, then they want to put you back out of the hospital onto the street.

How do you think sickle-cell disease would be treated if it was a Caucasian disease?

I point to Tay-Sachs disease which does affect Caucasians but it’s even more rare. But when someone with this presents at the hospital the medical community has a greater understanding, and they’re not stigmatized as drug seekers or anything else.

A friend of mine with sickle-cell was in Texas, and went into crisis pain. This person is in the medical community, they were actually working at the hospital where they went into the ER. The hospital staff knew who they were and they still treated them the same way just because they were an African American, presenting with sickle-cell pain. The doctors actually refused to give adequate pain medication and medical treatment until my friend’s home physician got in touch with the doctors and explained what was going on. I thought that was unbelievable. They wouldn’t take the word of their own colleague, who was a medical professional, because they had a lack of understanding.

Sickle-cell patients just want to get pain relief, and if you can stem the tide of that pain early, we can be out of your hair in a much shorter time.

Sickle-cell patients just want to get pain relief, and if you can stem the tide of that pain early, we can be out of your hair in a much shorter time. Unfortunately the medical community doesn’t understand that, and with trying to piecemeal the pain away, they just extend the whole process.

Usually if I go into the hospital where doctors and staff aren’t familiar with me, I know I can call someone in the hospital administration and talk to them, and they’re going to exercise a certain amount of power on the doctors who are treating me, so I can get what I need. But your average patient—who will also be in pain which affects their ability to advocate for themselves—isn’t going to be able to do that.

As carriers, did your parents ever express guilt over passing the sickle-cell trait onto you?

They’ve never expressed it but I believe it is there. Just certain conversations I’ve had with my mum over the years. She’s never come out and said “I feel so guilty you have the disease” but she has said she will always worry about us. When we were younger, if you catch a cold, a head cold, a bad cold that can sometimes trigger a crisis, your body gets dehydrated, your immune system will get distressed. As small children, going to elementary school, we would get sick a lot. So my mom has said in the past she would worry, sometimes she couldn’t sleep at night sometimes when we were sick because she was so nervous and so worried about us.

Is this same concern a reason you have chosen not to have children?

I didn’t want children. I never wanted to bring somebody into this world who would have to deal with the pain that I went through. I thought, I can’t go through the mental gymnastics while I’m dating and have somebody fill out some type of genetic card before we got serious, I just hoped I would meet someone who shared those same views, and if we did in fact want children we could possibly adopt or do something else. My wife has never wanted children either, but for her own reasons.

How did you approach talking about SCD with your wife, when you were dating?

Sickle-cell’s a lifelong disease. If you’re going to be with your life mate they’re going to need to know how to handle it. That was one big thing I talked about with my wife, when we were dating, that if I ever go into crisis and I need you to speak for me, this is what you need to do. I educated her so that she would know if in fact that time would come and I couldn’t advocate for myself that this is what I’d need.

Sickle-cell’s a lifelong disease. If you’re going to be with your life mate they’re going to need to know how to handle it.

Fortunately, I’ve been mostly healthy since we’ve been married, but I have had pneumonia once, she had to step in and be there for me for that,

How much awareness is there of sickle-cell disease within the African American community?

Most members of the African American community either have a family member or know someone with sickle-cell disease, so there is awareness. When my sister was diagnosed my mom really had no clue what the disease was or anything else. She told me about talking to her friends and very few of them knew anything about it until she spoke about it with one of her friends who actually had the disease as well, but back then it was so stigmatized that nobody really spoke about it.

What advances would you like to see for sickle-cell patients?
I’d like to see much greater awareness. Besides the doctors being apprehensive about you receiving the pain medication, a lot of doctors don’t learn about SCD in medical school. I have heard from doctors that they maybe spend a few minutes on SCD throughout their whole educational career, unless they go into haematology. So many doctors are ignorant of the disease.

I also think something that isn’t emphasized enough is within the sickle-cell community is to really think hard about family planning.

People date and fall in love, and a lot of people don’t know they have the trait. Or one might have the disease and the other the trait. But by then, they’re already in love, and about to get married. At that point, what do we do? Because the person with the trait, and the disease link up. Most likely, depending how many children they have there’s a greater chance they will have a child with the disease and not just the trait. So that’s something people really need to think about.

How  do you reconcile living with a reduced life expectancy?

You can’t plan for everything. But I try to live as normal a life as possible and really not dwell on that. I try to eat right, exercise, spend time doing the things I enjoy outside work, and really hope for the best. I’ve understood from an early age that the life expectancy for somebody with sickle-cell wasn’t very promising. But with the advances in medicine and staying on top of things, being an advocate for my own personal healthcare, I think I’ve done all I can do to make sure I have the longest, best life I can.

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