“Sickle Cell is a hereditary blood disorder in which my body produces abnormal red blood cells. When my body begins to over produce these abnormal red blood cells it can be very painful”. Usually, that over simplified answer is enough to satisfy the inquirer’s curiosity and health class is over for the day. Some days I’m content with having a 5 minute conversation about a disease that has had an epic effect on my life in all areas of my life. Other days I just open up and begin explaining the real day to day problems that I have to deal with and the frustration that comes along with it. When I’m done with whichever response I’ve chosen to give in that moment. The response is always some version of the following: “Well, you don’t look sick. In fact you look very healthy to me.” Or “What does the government do to help? Aren’t there programs that can help you? I see commercials for Cancer Centers every day. Don’t they have something like that for you? Sadly…The answer to that question is always “No”. But then again the way that the government sees my illness and how I see it are very different.

The CDC defines Sickle Cell as a group of inherited red blood cell disorders. Healthy red blood cells are round, and they move through small blood vessels to carry oxygen to all parts of the body. In someone who has SCD, the red blood cells become hard and sticky and look like a C-shaped farm tool called a “sickle through”. In small blood vessels, they get stuck and clog the blood flow. That causes a depletion of oxygen to that part of the body (keep in mind this is a body in which its system is already operating on less oxygen than a normal one) can cause pain and other serious problems such infection, acute chest syndrome and stroke.

Types of SCD

HbSS

People who have this form of SCD inherit two sickle cell genes (“S”), one from each parent. This is commonly called sickle cell anemia and is usually the most severe form of the disease.

HbSC

People who have this form of SCD inherit a sickle cell gene (“S”) from one parent and from the other parent a gene for abnormal hemoglobin called “C”. Hemoglobin is a protein that allows red blood cells to carry oxygen to all parts of the body. This is usually a milder form of SCD.

HbS Beta Thalassemia

People who have this form of SCD inherit one sickle cell gene (“S”) from one parent and one gene for Beta Thalassemia, another type of anemia, from the other parent. There are two types of Beta Thalassemia “0” and “+”:

Those with HbS Beta 0-Thalassemia usually have a severe form of SCD.

People with HbS Beta +-Thalassemia tend to have a milder form of SCD.

There also are a few rare types of SCD:

HbSD, HbSE, and HbSO

People who have these forms of SCD inherit one sickle cell gene (“S”) and one gene from an abnormal type of hemoglobin (“D”, “E”, or “O”). Hemoglobin is a protein that allows red blood cells to carry oxygen to all parts of the body. The severity of these rarer types of SCD varies.

I define it as Complicated

Sickle Cell Disease is recognized by the United States government as a legitimate genetic disease. Thereby, granting those who suffer from it protection under the Disabilities Act. Therefore, making it illegal for hospitals, workplace and schools to discriminate against us. In addition, we are also eligible for government assistance programs like SSI, SSD, and Medi-care etc. Now that being said just because I’m eligible for these services, it doesn’t mean that it was easy to get them.

In order to automatically be approved for services. Like many before me, I filled out the application for Social Security Disability (SSD). Completed forms with the mandatory signatures, medical records and valid dates. Thus, proving that I met more than one of the following criteria:

1. Produce medical records citing that you have a disease that is recognized by the government as a debilitating illness.
2. Be hospitalized.
3. Be under a specialists/physicians care.
4. Prove that you cannot work due to your health.

After turning over 880 pages of medical records, six letters from my Hematologists and various specialists care for me. Explaining in tedious detail, the discrepancies in my work and school records. I was DENIED! Confused yet? I was too. I gave them everything they said they needed and it still wasn’t enough. I had to prove that I’d suffered from SCD before I was 16 yrs. old. Still not following yet? Let’s try to simplify this, Shall we?

Keep in mind that any person applying for SSD is only required to meet one of the criteria to receive services. The U.S. government recognizes Sickle Cell as a hereditary debilitating disease that patients are BORN with. Thus, acknowledging us as Genetically Handicapped. I was told that since I didn’t apply for SSD when my father died (I was 9yrs. old) or when I turned 25yrs. old and needed my own insurance. I had to show proof that I had Sickle Cell and a lifetime of crisis’, infections, and hospitalizations rendered me disabled and indeed handicap prior to my 16th birthday. So, my family hired an attorney, and scoured the Earth for every medical record, nurse, surgeon and doctor I ever had and went to court.

While at my hearing the so-called court “expert” (cardiologists making a decision regarding an SCD patient) accused me of falsifying lab records. Stating that my hemoglobin of 1.0 was impossible, my records were incomplete because they didn’t have tests and labs for the times I wasn’t sick/hospitalized. Therefore, he had no baseline to compare to. Finally, my favorite” functioning on opiates is actually much easier than people think”. Thus, making Morphine an invalid reason for not working. I won my case but, not after enduring the Twilight Zone cross-examination, insults regarding my medications and having to defend my social life. I mean how could I possibly go to the movies, my little sister’s b-day party, art classes and even college courses, if I’m so sick that I can’t work and I need government assistance? As if, me living my life as normal as possible somehow negates the fact that I do live with chronic pain and it prevents me from working in a traditional job setting.

After the yearlong battle and driving everyone around me crazy, I won. Even though, my story has a happy ending… so far. There are so many SCD patients who don’t know that they could be eligible for programs outside of SSI. Not only that, they don’t know that there are attorneys who will fight on their behalf with no upfront cost. Despite, how much the government recognizes us, as a population in need of their assistance, or how easy it tries to make signing up for these services. We still have to go out of our way to prove why we need their help. Complicated.

Unfortunately, this practice of acknowledging this horrible illness exists and then downplaying, ignoring, or flat out refusing to treat it’s patients is a very common practice in treatment centers, ER’s and hospitals across the country even, the world. Once again, everyone knows that the disease exists. They recognize that it causes extreme pain, attacks come on suddenly and that it’s treated with hydration, IV pain medications and sometimes blood thinners. However, 9 times out of 10 when a Sickle Cell patient goes in for medical care, they are met with negativity, disbelief, and prejudice. Often times the nurses and doctors assigned to care for them were most likely warned and not educated about Sickle Cell while in nursing or med school.

To add insult to injury, when medical facilities/hospital/treatment centers orientate their new staff; Sickle Cell Disease is the primary example used as a training tool on what to expect from “drug seeking” patients. According to a May 2012 study of drug seeking behavior in the ER titled:

The Clinician Impression versus Prescription Drug Monitoring Program Criteria in the Assessment of Drug-Seeking Behavior in the Emergency Department. Predictors for drug-seeking behavior are as follows:

1. Patient requests opioid medications by name.
2. Multiple visits for same complaint.
3. Suspicious medical history.
4. Symptoms out of proportion to examination.
5. Hospital site.

Unfortunately the so-called “Predictors” describe a typical Sickle Cell patients visit to the ER during a Crisis. For example, since I was born with this disease I know that Morphine is the only thing that helps, so far. Like most sickle cell patients my crisis occurs in the same places with little variation. Due to the unpredictably of a Sickle Cell Crisis, I may make anywhere from 1-5 visits to the ER in any given week, month or year. Then, add to that the random infections, ulcers, surgeries, PICC lines and Portacaths. I’m going to go out on a limb and say, I’m sure my medical history is as strange, as it is lengthily.

When I go to the ER I try my best to stay calm. That means no crying, screaming or yelling. I remain very calm and operate on the idea of “Let me help you help me”. Unfortunately, that means that I don’t fit into most people’s vision of pain and therefore, I get labeled as “Not being that sick and/or accused of lying about my pain”. By the time I explain myself, convince the powers that be that I really do need help and I get that help hours have passed. Clearly, going to the ER is a very complicated, tiring and stressful event. That is further complicated by people who allow their personal feelings to dictate the level care they give their patients.

Finally, not only is Sickle Cell a complicated topic when dealing with the government, and getting care in an E.R. but most of all in personal relationships. The Sickle Cell community is largely made up of only child, single parent households. One reason is once a child is born and it’s discovered that they have a hereditary disease. The parents usually make the decision not to have any more children. It may sound extreme but, if both parents have the trait then with every pregnancy they will have a 25% chance of having another baby with SCD. Unfortunately, “normal” marriages have a 50/50 survival rate. Add to that, a child that is incredibly sick, hospital stays/bills and the overall stress and strain that accompanies regular marriages. The Sickle Cell community has one of the highest divorce/single parent household rates in the country. Making the parent/child relationship the first, in a long line of complicated relationships to come. When a child is born with a genetic disease that’s passed on from the parent, there is a mutual guilt that both parties feel toward the other. The parent feels guilty because they gave their child a disease that causes them immense pain that they can’t fix. So, they sit by the bed and do their best to be strong and positive. When they actually are terrified, angry with themselves, and helpless to take their baby’s pain away.

On the other side of the coin lies the child. Who sees through their parent’s happy facade and knows that under their fake smiles lay tears and hopelessness. As the child grows up the guilt doesn’t fade away. If anything, it increases. Simply, because the child wants to go into the world to discover, learn, be adventurous, hopefully start a family of their own, basically…GROW UP. By doing what comes natural, they are seen as being irresponsible, selfish, stubborn, in denial and ungrateful. Why? Not because their parents don’t want them to grow up and be self- sufficient. They just want to control the way they go about doing it. For example, when I went to college I did what every normal college student does in their first year: drink, party, stay up/out all night, procrastinate, get side tracked and discover body art. Looking back what I did wasn’t so bad. In fact, it was quit tame compared to what most college freshmen do to claim their independence. Unfortunately, my mother didn’t see it that way. What I thought of as harmless fun and even a rite of passage if you will. My mom saw as putting my life endanger, stupid, disrespectful and ungrateful. Of course, I saw her as being controlling and crazy. That is until she explained herself many years later.

She told me that she never makes a decision without first asking the questions: “Is this best for Marissa? How will this affect her overall health? Of course, if the answer is negative to either question than she doesn’t do it. For example, when I got my tongue pierced she cut me off completely and basically disowned me. What I saw as harmless piece of jewelry, in a questionable place, as being another form of self- expression. She saw as a life threatening act, that basically through her life’s work away, with a note reading F*CK YOU MOM. Sound a little dramatic? I thought so too but, not to her. From her point of view I might as well have played Russian roulette. When I pierced my tongue, I’d just been released from a 3 month stay at Cedars Sinai. Recovering from a failed blood transfusion that left me unable to receive any blood products outside of Platelets. Due to expected prolonged hospitalizations, I had a greater chance of organ failure, blood embolisms, and more susceptible to infections in and out of the hospital. Unlike traditional ear piercings, those in other body parts never fully heal thus, leaving small open wounds in the body. According to my mother “You might as well have had a blinking Vacancy sign tattooed on your forehead. To let passing germs and viruses know they’re welcome to set up shop in your body!” All of the years she spent making sure I had the best healthcare possible. Teaching me about my disease and its pathology. Most importantly, she trained me not only to communicate my needs to doctors and nurses alike but to protect myself above all else. By piercing my tongue I’d ignored her life’s work and worst I put myself in harm’s way. Now I not only consider how my actions may impact my health but, how it’ll affect her and anyone else that cares about/loves me.

As complicated as the Parent/Child relationship is…finding and maintaining healthy, positive and supportive friendships and relationships are even more so. First of all, like most people who aren’t patients or related to one, they don’t know what it is. So, the first thing a patient must do is not only educate them on the disease but, train them about their own personal care. Bottom line if one goes into crisis while out with their friends, those people have to know how to take care of them. From the first day I started school every teacher, classmate and their parents knew I had Sickle Cell. They knew my limits, what to look for if I was falling into a crisis, who to call and what to do until I was picked up or taken to the hospital.

When, I was in high school and my friends began to drive that training became more intense, extensive and much more serious. When I was 16yrs old my friend Nicole and I were shopping when I went into an intense crisis that came on quick and with a vengeance. Even though, my mother was only 20 minutes away at home, my hospital was across the street from the mall so; we went straight to the ER. Nicole called my mother and quickly turned to her training to help me. Like everyone else, she was instructed to:1- call Adrienne (or designated person if she was out of town) and my doctor.2- Take notes: Write down when the crisis started, where/how intense the pain was, what pain meds I took how many, what dose and time. Did the meds seem to be working or not. 3- Once in the ER: Share notes with the medical staff and tell them allergies, what medications I’m on and what I take in the hospital.4- take notes on the staff: Their names, what medications they order and when they’re given. Most importantly, what they say/do that’s even remotely unprofessional or discriminatory.

All of this sounds very intense and a lot to put on a teenager. However, had she not been able to handle it, she wouldn’t have been my friend. Let alone someone I could go out with by myself. Thank GOD she was capable! That trip to the ER was the first time I was treated as a lying, drug-seeking teenager. Despite the fact, that I had just been there3 months prior and was treated right away, no questions asked, and with respect. Somehow in three short months between my 15th and 16th birthday, I suddenly stopped being a child in pain and was mysteriously reborn as a lying addict. Neither of us could believe the way we were being treated. First, I was told my friend couldn’t be in the room with me because they needed to see if our stories “MATCHED”. We were being interrogated as if we were serial killers or worst (we ignored them). Then, I was told that I wouldn’t be treated until they were “SATISFIED” that I was actually in a painful crisis. That meant a series of unnecessary, invasive, derogatory tests and questions. For example, I was asked if I was sexually active. The answer was “NO” and I had never been. The doctor’s reply “Well we’ll order a pelvic, just to be sure”. As if that wasn’t humiliating enough, they started quizzing me on everything from what my disease is, what Morphine is and how does it work and why are IV’s necessary for my treatment? To whom I knew on staff in the Pediatric Unit. Reminding me that if I failed their test or refused to co-operate then, I’d be banned from the hospital forever! I was so hurt and shocked I started crying uncontrollably. That’s when Nicole kicked into high gear. She wouldn’t allow them to do the pelvic; she reminded them that I was a minor and that their behavior was beyond inappropriate. Then, she recorded everything that happened and started calling HR and reporting them (while they were still in the room, lol). When my mother arrived they tried to paint Nicole as threatening, out of control and dangerous. Of course they lied about the way they were treating me. Thanks to Nicole we had a detailed written record of what happened and a lot of people got into a lot of trouble. Somewhere suspended, while others were either transferred or fired. I was very lucky that day to have her with me. She saved me from being traumatized by the pack of psycho doctors and nurses.

I wish I could say that all of my friends and family trusted that I was really sick. Let alone took me to the hospital, stayed and fought that hard for me. She was 1 of 4 friends in my life that really stepped up to the plate and took care of me. 1 of 3 that never resented me felt that I owed her or blamed me for getting sick. A majority of people I once called “friend”, would stay away from me when I was sick. Then, when I got out they would pretend like it didn’t happen and we would pick up right where we left off. The rest would scold me for drinking and going to parties. Basically, blaming me for not preventing my crisis. Eventually, those people would stop speaking to me in protest. Stating that I was too much trouble to deal with. I’ve been told that I make myself sick and then expect them to drop whatever they were doing to take care of me. Basically, I was a sympathy whore who used a so-called disease to get over on people and make them feel sorry for me. Of course they were right because if I cared about myself then, I would never travel, dance, drink, be up late or even walk in order to prevent being hospitalized. Now that I’m older I have a better understanding of a “Friend “and I choose them more carefully. It doesn’t take away the pain that comes along with being judged and ridiculed by people that don’t even know what Sickle Cell is or how it affects those of us who suffer from it.

If finding and keeping a friend seems like a challenge. Imagine being an adult and trying to have relationship, marriage or children. I’ve only had 2 major relationships in my life. 1 in college and 1 after.

My first date with both of them was in the hospital, lol. They both did their best to support me and care for me. Unfortunately, fear got the best of them and they just disappeared. No breakups. They were there one day and gone the next with no notice. Years after the fact I was told that one couldn’t deal with the idea of me going to the hospital, dying and leaving him with our 3 children and a mortgage? WTH?!?! We never even discussed kids let alone 3 of them. Nor did he ever express that concern to me so, we could have least tried to work through it. The other had a problem with me caring for him when he was hospitalized. He was a deputy and retired from the military he thought he was going to swoop in and save me. Once he realized I didn’t need saving and it become more apparent that he did, he was gone. For the most part it is very difficult to find a life companion when you’re sick. Most people don’t want the responsibility. Others don’t want to risk having children with the disease. Since I know my health is a huge burden. I often found myself going out of my way to be the perfect girlfriend. In hopes that it would off-set the inconveniences related to my health. Complicated, Complicated, COMPLICATED!!!

If you thought the parent/child relationship, friendships and intimate relationships were complicated it’s nothing compared to co-workers and bosses. A large part of Sickle Cell patients having to be on government assistance (outside of insurance) is that it’s very hard to get and keep gainful employment. Since most people are unaware of Sickle Cell they find it very difficult to understand how it affects the people who have it. Often times we are seen as being unreliable because you never know when a crisis will flare up. Or if you will be hospitalized or simply have to stay home for a few days. Due to the fact that we have chronic pain and take large amounts of pain medications. We are often labeled as drug addicts. Most people don’t understand how we’re able to function when we’re in crisis. Once again the idea that one should be in bed if they are suffering from pain that requires narcotics to control comes into play. After all how can someone have pain, pop pain medications and continue to work or do any physical activities be honest about their health? Due to this ignorance we are labeled as drug addicts and often fired. Therefore, many Sickle Cell patients are self- employed and fairly successful. The fact of the matter is most of us having a good work ethic and wants to work. Unfortunately, due to ignorance and lack of understanding often times we are seen as unemployable. COMPLICATED.

Finally the most complicated and most important relationship is the one I have with myself. After all I’m the constant in all of the relationships described above. My thoughts, feelings and actions dictate all of my relationships from beginning to end. How I view myself is the largest factor in all of my relationships. It not only dictates the type of people I attract and allow in my life. It is the driving force in how I treat them and how I allow myself to be treated and for how long. Having chronic illness I have no choice other than to be a leader. Especially, when it comes to dealing with my doctors, nurses in and out of the hospital. At a very young age my mother began to teach me to trust my instincts and standby my decisions. That meant that I not only had to believe in myself but, I had to without question or hesitation tell people what I needed and speak up when they did something wrong. At 2yrs. old my mother taught me to place my hand over possible I.V. site and say “NO!” to the nurse attempting to start the I.V. That was my first lesson in not only guiding my nurses but also in knowing me. As you can imagine having a 2yr. old telling an adult that’s been put in charge of caring for them say “NO” and prevent them from doing their job can be a little off putting. Naturally the nurses thought I was just afraid of being stuck like most people. When in actuality I wasn’t. However, I was aware that for whatever reason (be it I was having pain in that part of the body or I knew that the veins they were looking at didn’t work) I had to intervene in order to prevent a possible disaster and get the medication I needed. That was also the first step in a long line of incidents in which I had to not only believe that what I wanted was indeed what was best for me at the time and convince the doctors and nurses to follow my lead. In actuality I had to have the same confidence, determination and belief that what I wanted was best. Just like a doctor in there but without the schooling and white coat. Doctors are capable of carrying for patients because they know that they have about 5 minutes to establish a relationship with their patient. In which, the patient trusts the doctor enough to follow his lead. Doctors can give all of the orders they want but without the trust of the patient it’s of no use. In my situation I know I have to get the doctors to follow my lead without feeling like I’ve stepped on their toes. The way I do this is by being calm, respectful and educated about my disease and an expert on me. Just because I’m more than capable of caring for myself and teaching everyone in my life to the same. There are times when I just want to forget the 37 yrs. of experiences and training that has made me who I am. I find myself wanting to fade into the background and allow everyone else to make decisions on my behalf. I get tired of having to educate people who have been to medical school about my disease. It’s very frustrating to not only have be the patient, my own advocate (and other peoples too) but the educator, doctor and nurse. On top of all the other hats I wear daughter, sister, God mother, cousin, niece, friend and sometimes therapist. When I get overwhelmed and I want to stop all of it. So I can just walk the earth like Caine in the Kung Foo movies. Then, I remember that I was put on this earth for a reason (I’m still working out the details) and that roaming the planet trying to find myself is a luxury I don’t have. COMPLICATED!

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