Should a Sickle Cell Trait Carrier Marry Someone with Sickle Cell Disease?

There is a hot topic over at the UKGreat Sickle Cell forum on FB. A gentleman posted this question: My genotype is SS and my fiance is AS. I love this girl very much because of her character. I intend to marry her. Should I go on? I need your candid advice.

I have very strong views about this as a sickle cell warrior. I have seen too many people with sickle cell get relegated to a lonely corner, dumped, and heartbroken just because of their genetic condition. I know many good men and women who are still single yet pining for a spouse even into their 40s and 50s. The fear of sickle cell is a strong thing, and I have to agree that not every person is up to the challenge of living and loving someone with sickle cell.

If you read my archives in the Love and Sex section, you will see that I struggled with my desire to have a soul-mate against my genetics, suffering many failed relationships and heartbreak in the interim. I had earlier ascribed to the notion of not wanting to have kids unless I was 100% certain that they wouldn't get sickle cell. It had been drummed into me as a child, that I had to look for an AA to marry...so that my kids would all be AS. But I realized a few years later, that there is another option. I could marry whoever I felt was the right man for me, and if our genetics didn't match up, then God, science, and modern medicine could help the rest of the way.

Because of this, I cannot advise this gentleman to dump his intended. If he loves her regardless of her having sickle cell...then I feel that he should go through with his plans. The only tricky part about it is the decision to have children. They both should decide if children are the end all be all. Not every couple has children...and yet, they still live wonderful and fulfilling lives.

If they do want children that bad, then they could adopt, have a surrogate egg where she carries the pregnancy, do genetic counseling, or selective in vitro fertilization (which is possible in the US and UK). In addition, with each natural pregnancy, she has a 25% chance of having a child with AS. This does not mean that 1 in 3-4 children will be AS while the others are SS...it means that with each pregnancy, she could have an AS child. I know a woman who had the same odds, had 3 children, and they all are AS.

Besides, the point of marriage is not to be a baby incubator, it is to share your life and love with someone else and have someone at your back who loves you and treats you well. Not everyone that gets married has children...and yet they still have very happy and fulfilling lives. We are living in a modern age, and the world is already overpopulated as it is. So choosing not to have children is not the end of the world.

Not everything in life is guaranteed. Even people with AS or AA can get diagnosed with a condition that has the potential to be more complex than sickle cell. And yet they still get to find love. Diabetes, Cancer, Heart problems, Hepatitis...it's a long list of medical issues that humans now face. Healthy people get viruses that kill within weeks--nothing is guaranteed! Anyone can have anything, so why is sickle cell the one that is the must shunned and feared? Bah!

In addition, if your parents had known about their phenotype, and chosen to break up, abort, or not have you, where would you be today? I consider my life as a blessing, and as the only sickle cell warrior in my family, I love my parents for having me. I love my life, and do not consider it a burden. 

So many sickle cell patients end up alone and lonely, without someone to share their life with because we in the community are even stigmatizing ourselves out of fear. We live our lives in fear of passing sickle cell to our offspring. Instead of wallowing in myths and fear, do your research! You can love someone with sickle cell, and to relegate this woman to a life without her love just because of her genetic condition----THAT is NONSENSE!

Sickle Cell Patients Go to the Hospital Repeatedly

Although this article is about a decade overdue, I am glad that there is now documented empirical knowledge that sickle cell patients are in pain almost every single day!

In eight states studied, researchers found that one-third of sickle cell patients who visited the hospital returned within 30 days in search of pain relief. Young people, between the ages of 18 and 30, were especially likely to seek care.

"I think it's not so much a failure of the hospital itself, but a failure of us being able to prevent these complications and being able to treat them," said Dr. George R. Buchanan, a pediatrics professor at University of Texas Southwestern Medical Center, who is familiar with the study findings.

Personally, and I'm sure most sickle cell warriors can attest to this, I return to the hospital because I AM STILL IN PAIN! If I wasn't hurting, I would never want to step foot in the ER. So it is a failure on the part of the health-care providers because they are so quick to discharge SC patients from the ER after the requisite 3 doses without proper medication to take at home. All they say is "Visit your Primary care doctor" but I can't go to the PCP because I am in pain. So I end up at home, getting sicker and sicker until eventually someone calls the ambulance or drags me back in the hospital. Even when admitted, doctors tend to discharge us before the pain is even resolved on a moderate level.

Anyway, it is a step in the right direction. This article was published in the mainstream U.S. News &World Report. Hopefully doctors will learn from this. 

Check out the full article HERE.

Sickle Cell and Ports, Catheters & IVs

Video blog posting about IVs, ports, and central access catheters

Do Not Call me a Sickler!!!

My first video blog.

Placing the Blame

Question: As the parent of a child living with Sickle Cell, I once blamed myself and felt guilty. That is no longer the case, however I do fear hearing the words...."Why did you give this to me".


Answer: I must confess, I did blame my parents...not that I have sickle cell--but that they were ashamed of me having sickle cell.  Although Nigeria has the highest population of people with sickle cell in the world, it was still somewhat 'shunned' and critiqued because of the lack of knowledge. There were myths that people with sickle cell couldn't have kids, would die young and no one wanted to marry someone with sickle cell.

Consequently, my condition was kept as a big secret in our family for most of my childhood and youth and the shame made me feel abnormal, weird, and different. My parents had often shared with me that they didn't know anything about sickle cell then, but even if they did...they wanted me so badly that it wouldn't have mattered to them. My mom also did tell me that she wishes that she had sickle cell instead of me...(that made me cry).

However, whenever I was sick, my parents were sick right along with me, and emphatically feeling my pain and crying with me. I hated being sick more because I would see how much my crises hurt them, and that just broke my heart.

I hope that your child never throws those words at you, but to me, I know that my parents felt so guilty and regretful already, and I never said it...mainly because I could tell how much they hurt when I was sick. Whatever happens, please make sure your child as an outlet to vent their frustrations with sickle cell. I would have given anything to have a support group growing up, of people just like me...because back then there was so much that I didn't know and so many emotions that I had that I couldn't share with others without sickle cell.

Sickle Cell & Pity

Here is a great question at the Sickle Cell Ning Forum.

Why is that when you go into a crisis people tend to give you weird looks and have pity for you? We don't need pity but support...

I think people offer pity what they do not understand something. Like when a loved one dies, friends go to the family to offer condolences, but really...what do condolences mean? Condolences won't bring the person back or ease the pain in the heart. But condolences send the message that I do care that you are hurting and that your heart is in pain. Conversely, to me, I don't see pity as a bad thing, I see it as people telling me that they do care that I am in pain. Plain and simple.

Pain is something that all humans can relate to because everyone has had pain one time or another in their lives. Sure, ours might be more all encompassing, and overwhelming, but pain is a shared human experience.

When people tell me they have diabetes, I don't give them looks of pity. However, when someone tells me they have an incurable cancer ---I pity that. It comes from my interconectivity as a human and my acknowledgment of human frailty. I understand that they are going through a struggle, and I empathize  with them. When I was younger, I used to hide from the pity and kept my sickle cell a secret because I didn't want pity. However, this just made it harder to deal with, since noone knew what I was going through, and when I needed support---I found it difficult to get any.

The kind of pity I hate the most is self-pity. That is the one that grates on my nerves. I hate to wallow in a 'woe is me' moment. Sure, I have sickle cell..but there are other people in the world going through much worse and are still smiling. I"m alive, I'm surrounded by people that love me, and I'm blessed to have survived so much. 

I feel that with more public knowledge and education, we will be able to have sickle cell viewed as a disease and not as a death sentence.  Perhaps when more people understand sickle cell, they realize that it's a condition with ups and downs, and when we are down, we do need more support. But at the center of it, we are just human, like everyone else.

So I guess on some level, pity is a weird form of compassion. I would rather have pity than have someone yell at me and call me a drug addict. I'm just saying...pity is the lesser of two evils. ;)

Nicosan Testimonial

One of my readers who has been on Nicosan for about a year now, has written this wonderful review that I would like to share with everyone. If anyone else has a Nicosan story that they would love to share; good, bad or indifferent; please send it my way. Also, if you would like to ask any questions about this, please leave a comment below or send me an email.




To whom it may concern,

I am an African-American male with Sickle beta Thalassemia and I have been meaning to write something on my experience with Nicosan to give other people trying to manage sickle cell more perspective. Currently , I am school and I hope to graduate soon as an RN. I only mention this to let everyone that I do have a medical background. However, my medical background isn’t supposed to convince you to take Nicosan. My main point is only to illustrate to you that I did EXTENSIVE research into the literature that’s available and am able to understand what the studies refer to.

So I have been taking Nicosan for about a year. I take my pills every day, and don’t skip any days. I usually take them with food because I also take a multivitamin which makes me feel nauseous if taken on an empty stomach. When I first heard of Nicosan I was extremely skeptical. I am very active with staying up to date on current research into Sickle Cell treatments. Hearing of any cure/treatment becomes one of those things that makes you afraid to hope. However, I looked at the early studies for Nicosan and they are very strong. It is very rare to find a double blind, randomized, prospective study. However, I had an issue with the fact that I found this over the internet. My thought was anyone can make claims over the internet and I wanted to speak with someone that actually takes it. Well, I did and I was convinced to purchase them. In a couple of weeks my Nicosan shipment arrives. Well, I still don’t take them because I’m still worried that it won’t work and they could be harmful. Well, I was finally told “what are you waiting for”. To be honest, the fear of disappointment was probably what was holding me back. So I started taking them and below I have written a little bit about my experience. I hope this is helpful.

Onset of effectiveness.
So my experience has been that you really do need to wait a little time to see a difference. The thing that makes what we are doing tough is that we aren’t looking for something to happen, we are hoping for something not to happen and the only the way to prove that is time. Initially, when I begun taking Nicosan I actually had these very small pains in places I normally didn’t. It was almost as if I was having a micro-crises, if that even makes sense. For example, I really can’t remember ever having pain in my hands, but when I first started Nicosan I did. However, these could be easily managed with Tylenol or Advil, but I didn’t take anything because I dislike frequently taking pain medication.

Threshold of Crises
So this factor is one of the biggest differences that I have seen with Nicosan. I am very careful with monitoring my activity level to prevent a crisis. As I have found that an ounce of prevention in worth a pound of cure with Sickle Cell. I can honestly say that I am able to do and be more athletic than I was before taking Nicosan. Although I didn’t do a “double-blind randomized controlled study” or anything, I did keep track of my workout. Before Nicosan I wasn’t able to perform cardiovascular workouts very well. I could maintain a low intensity cardio workout just fine, however, I kept my heart rate low to decrease the amount of oxygen I would need. After Nicosan, I was putting my heart rate higher without any problems. Any pain that I experienced I would just take motrin 400mg. This for me was a very big deal. Now please don’t think that I became a marathon runner by taking Nicosan, I still don’t do certain things like running because it causes me problems the easiest. Personally, I like the elliptical. However, the point I’m making is I am able to be healthier than I was before. I really don’t’ know what my true limit is because I never push it. That’s just a part of managing sickle cell. However, the psychological fear of pushing myself was very strong. This feature is characteristic of many people with chronic disease. Studies have shown that chronic disease affects every part of your life. As a consequence of reducing the “threshold of crises” it reduces my fear of being active.

Quality of Crises
So this area is very subjective. I want to make sure to mention that someone’s experience in this area could really vary. Over the year that I was taking Nicosan I never really had any episodes that I would consider big crisis. However, when I first started and when I got the flu I did have a crisis. Ordinarily, when I have a “real” crisis it lasts about three days, with the 1st day being the worst and it getting progressively less painful by the end. Generally, on the 4th day I don’t have to take any medication. However, after being on Nicosan they were different. The crisis for me actually lasted a little bit longer, however it was significantly less intense and painful. The crisis could basically be managed with less medication and it wasn’t as debilitating. This seemed kind of strange to me and really I can’t say that Nicosan was directly the cause because so many different factors affect the course of a crisis. However, it’s not something that I had experienced before taking Nicosan.

Frequency of Crisis
The frequency of crisis goes hand in hand with the threshold. If it is harder to have a crisis from taking Nicosan then hopefully I will have less of them. This really has been the case. I was free from any significant pain that required medication (including motrin or Tylenol) for almost a year until I got the flu. Plus, I was very active. I was working a lot and very stressed out from school. However, I also made sure to drink 3 liters of water per day.

Story of an ER Visit
My brother has sickle cell as well and I told him to take Nicosan. Well he had only been on it for a week and was on a long flight when he started having abdominal pains. Needless to say, they made him go to the ER by ambulance during a layover. So he called me. Every time a Physician spoke with him he called me three-way. I basically, informed the Attending physician, the resident, and everyone else who needed to know what Nicosan was. I informed them how it works in the body and directed him to look it up on the Cochrane review database (this is a peer reviewed database to evaluate research). Now remember, this is a medication that has orphan drug status in the US, but still isn’t FDA approved yet. Do you know what his discharge instructions said when they let him go? “Continue taking Nicosan as you were” They couldn’t find anything wrong with it. Now, they didn’t prove it worked or anything. But, they didn’t tell him not to take it. I personally, think that fact says a lot.

[Note]
I would like to sincerely thank Vixen for all of her efforts to help support others with sickle cell. Her blog and her willingness to put herself out there for something she believes in is incredible. I can’t remember how I happened to come across her blog, but I did and I would never have found Nicosan if it wasn’t for her. I’m not sure if everyone that visits her blog are really aware at how much of a “secret” Nicosan is. I have spoke with so many people in the medical field who have no idea that it exists and have never heard of it. Now, these aren’t just ordinary people, they are professors and researchers who still don’t know. I have even spoken with medical professionals who are from Nigeria who have never heard of it. So what Vixen is doing is huge. I think we all should appreciate what she has done because I have learned that it’s on us to manage our illness. I realized when I was a teenager that no one can carry my load and that means that they can’t manage it for me. No one can know what it’s like to be you, and because of that I don’t let anyone tell me I’m faking, exaggerating, not that bad, or not in pain.

Mission Statement

The blog is entitled Sickle Cell Can Kiss My A** and it is a positive and yet progressive platform for me, a sickle cell patient and advocate to explore all realms of thought into sickle cell. There is so much that sickle cell warriors go through (I refuse the term sickler, since having a condition like diabetes doesn't label one a diabeters, or cancer doesn't label one a canc-ler). Sickle cell warriors are in a constant fight on a day to day basis to keep ones head above water, deal with the pain, make the right decisions in life, deal with finances, medical bills and handle the backlash, negative vibes and criticism that having sickle cell brings.

But not everyone can have an attitude of gratitude; and not everyone that has sickle cell is a Christian. My life hasn't been easy with sickle cell, and yet, through all the adversities, I've been able to overcome and live my life to the fullest potential. I'm a registered nurse, have a wonderful family of my own, working on my Master's degree and holding down a full time job. All with sickle cell. My message is that sickle cell doesn't have to own you, you are not defined by your diagnosis and you can live a rich and fulfilling life with sickle cell.

Telling sickle cell to kiss off (or kiss ass), is merely an expressive, a snub to the condition that threatens to overtake ones very existence. Though not the choicest of phrases, many people resonate with this survivor mentality and attitude. Sickle cell is not a walk in the park, it's not roses and cream, it's hard, it's a struggle and only the toughest survivors can cope without wallowing in self pity, depression or emotional lows.

Painting life pretty, ignoring the tough side, and trying to hide the pain and agony that we go through on a daily basis hasn't helped with public awareness--- it's only proliferated the mentality that sickle cell warriors are 'fakers, drug addicts, lazy, malingerers'. I don't bring sickle cell down, I don't make fun of my sickle cell warriors, I just talk about the other side of it...the real side of it. The hard side of it, that all sickle cell warriors go through but don't have anyone to relate with about it.

The public doesn't understand what we go through because NO ONE WANTS TO TALK ABOUT IT. If we don't talk about it, how will they understand? I started my blog because I couldn't find a blog that told the truth...a space that talked about the daily rigors of living with sickle cell. I've talked about relationships, work, sex, exercising, swimming, flying, fear of overdosing, hallucinations...the grittier parts of life that every sickle cell warrior goes through but no one wants to share. Other sickle cell warriors all over the world can relate to this, we are all fighting the same struggle and going through the same things.

There are many people isolated and stigmatized in the sickle cell community, in the USA, and in almost every tropical nation in the world. I've had emails from readers in Islamic countries, South American, Africa countries---even Indian and Asian populations have reached out to me, sharing their stories and building a community. Everyone has their mission, and perhaps the way I speak might rankle nerves; but I'm blunt, unorthodox, unconventional, and very happy to share my sickle cell testimony with the world.

My Nicosan Story

Here is a question that I got from a reader on Nicosan that I would like to address:

A friend of mine just started on Nicosan 12/12/08, due to lapse in medical coverage and not having monthly blood exchanges, he has been having severe pain every week, resulting in 2-3 ER visits/month since 9/08. Since taking it, the severity and frequency has been reduced, but not as much as we had hoped, and the other day had unexpected major crisis that landed him in ER for 3 days!! 1st time in 2 months. It has worked some,but this was disheartening to both of us, and I know most people are new to Nicosan, but if anyone could post about their experiences, is it working, a lot, a little, not at all? Just so we have something to compare it to? Thanks and I wish all of you "takers" well!!

I will leave this up for everyone to respond to.

This seems to be a trend that I've noticed from emails from other users of Nicosan. I had a friend that started taking Nicosan in December as well, but still had a major crises a few weeks later. He dropped out of it and went back to Hydrea. I felt bad when this happened, but each person has to find the best combination of meds and treatment plan that works for them.

I would love to hear the story of anyone that has been on this medication, even if it was only for a few short weeks.

Personally, (and you can read this in my archives), I started taking Nicosan late April, right before I went on vacation to Hawaii. I took it all vacation long, came home in May, started a new job and fell sick a few days later (Stress, strep throat, pneumonia). I was intubated on the ventilator---so yeah, it was a hard crises. I almost lost my faith in Nicosan right then and there, thinking it was a waste of time and money--or another drug peddled as a 'cure'.

Norio pushed me to continue with it, telling me that Nicosan wasn't to blame for my crises. "The manufacturer states that you should take it continuously for 30-90 days before expecting it to work right? So keep taking it regulary until then!" He theorized that my body was worn down prior to that, and the trip to Hawaii, stress of dealing with family, vacation, school and the new job had all contributed to me wearing down my body. "Now you are starting with a clean slate, your blood levels are perfect, and you will take better care of yourself, and you'll give the drug a chance to work right."

His adamant belief (and constant nagging), made me continue on taking the medication. I marked the days off on my calendar religiously, deciding to write days that I had pain, to days that I had no pain at all. Three weeks later, I realized that I hadn't had pain since I was in the hospital. I thought it was maybe because I had a new transfusion, my lytes were good etc. so I waited for the 3 month mark, when my next crises would normally hit before I believed.

3 months later...still no crises. Best of all, no daily pain. But I've had stretches of 4-5 months with no crises, so maybe it's just a fluke.

I hit the 6 month mark before I became a full believer in Nicosan. And I haven't even taken it regularly (I hate pills!). I would say I take it about 3-4 times a week...yet I haven't been hospitalized once since that last crises in May. I haven't even been to the ER!

So now, we are at the 9 month mark...and counting. My pain pills lie unused in my cabinet, I haven't filled a prescription since last year---I haven't even visited my doctor.

On May 8th, 2009, it will be one year since my last crises.

That is my Nicosan story. Now I want to hear yours! Leave it in the comment section or just email me.

Changing the Image

Okay sickle cell warriors, it's time for some frank talk. I'm sick and tired of hearing how lazy people with sickle cell are. I'm sure that the majority of us aren't...but for some reason, the public has the notion that we are just milking 'the system' for our pain, are too lazy to get a 'real job' and are drug addicts.

You've all heard my rants about public perception and my efforts to educate those I come in contact with. But standing on my soap box will only educate so many. We all have to join together to change the face of sickle cell.

One of my nursing friends who works in Georgia commented that she had worked with many sickle cell patients, and she made a few generalizations which provoked another one of my soap box tirades. But sitting back listening to what she was saying, I realize that although she was overly judgmental and uneducated regarding sickle cell, the sicklers that she had come across had created a bias in her.

"They just go from hospital to hospital seeking drugs. Some lay in the bed for days, sometimes weeks and don't even brush their teeth. They act like princesses, requesting all kinds of food and pain meds around the clock."

Yes darlings, I did set her straight, but at the same time, I had to listen to what she was saying. She's been a nurse for 15 years, and has taken care of scores of sickle cell patients...yet the majority had this 'victim' mentality.

We are not victims. We are survivors. Stop laying in bed having a pity party...it's just a disease. You aren't dead yet, pain is just another fact of our lives, so please, don't whine and complain that it's the end of the world.

Personally, I hate it when people pity me, it makes my skin crawl. So I do everything in my power to avoid that, and draw strength from within, living my life the best way that I can.

I'm in school, have a demanding job, maintain a good relationship and travel constantly. If I can fulfill my dreams...so can you. Having sickle cell is not the end of your world...so please warriors, no more playing the Victim card. It's so not us.

Yes, we fall sick alot, yes we visit the hospital several times a year...but that doesn't mean that we have to enjoy it. Do whatever you have to do to get out of that hospital bed and back to your wonderful life. Being in the hospital is not a vacation or a walk in the park, it's sheer purgatory and you should be doing your best to get out. Whether it's walking, washing up, eating healthy, hydrating etc...do your best to get better and back to the real world.

Live your life and please be a sickle cell warrior and example wherever you are. Do your part to change the face of sickle cell.

Thank you.

I got an A!

Hey, I just got my grades for this last semester. I rocked out y'all! My GPA is 3.9 and I'm still banging out straight A's. Oh yeah, oh yeah *jumping up and down*

Anyway, in the spirit of giving, sharing and all that Jazz, I'm going to host the sickle cell presentation I created for my final class project, the one that earned me a resounding A+. The premise of the project is that we were supposed to identify a vulnerable population and develop an assessment tool for them. I created a sickle cell questionnaire...send me a message if you want that, and wrote a paper...and a PowerPoint presentation. It was only supposed to be one teaching tool and the paper, but I kinda went overboard...y'all know how passionate I can be.

Okay without further ado...

Sickle Cell Disease Teaching Presentation

View SlideShare presentation or Upload your own. (tags: sickle cell)

Variations of Sickle Cell

Since sickle cell is a mutation of a strand of chromosomes, there are several different strains or variations. Here are the five most commonly documented:

Sickle cell trait: The person is carrying the defective gene, HbS, but also has some normal hemoglobin, HbA. Persons with sickle cell trait are usually without symptoms of the disease but have mild anemia. Under intense, stressful conditions, exhaustion, hypoxia (low oxygen), and/or severe infection, the sickling of the defective hemoglobin may occur and result in some complications associated with the sickle cell disease. This affects 1 in 12 African Americans.

Sickle cell anemia: The person has most or all of the normal hemoglobin (HbA) replaced with the sickle hemoglobin (HbS). It is the most common and most severe form of the sickle cell variations. These persons suffer from a variety of complications due to the shape and thickness of the sickled cells. Severe and chronic anemia is also a common characteristic for children with HbSS. This is what I have.

Sickle cell - hemoglobin C disease: The person has both HbS and HbC. This is often referred to as HbSC. Hemoglobin C causes red blood cells, called target cells, to develop. Having just some hemoglobin C and normal hemoglobin, this person might be mildy anemic. These persons often suffer some of the complications associated with SCD, but to a milder degree. Vaso-occlusive crises (the flow of blood is blocked because the sickled cells have become stuck in the blood vessels), organ damage from repeated sickling and anemia, and high risk for infection are all similar traits for HbSS and HbSC.

Sickle cell - hemoglobin E disease: This variation is similar to sickle cell-C disease except that an element has been replaced in the hemoglobin molecule and is often seen in Southeast Asia populations. Some persons with hemoglobin E disease are without symptoms. However, under certain conditions, such as exhaustion, hypoxia, severe infection, and/or iron deficiency, some mild to moderate anemia may occur.

Hemoglobin S-beta-thalassemia: This involves an inheritance of both the thalassemia and sickle cell genes. The disorder produces symptoms of moderate anemia and many of the same conditions associated with sickle cell disease. While this disorder more often has milder symptoms than sickle cell disease, it may also produce exacerbations as severe as those of sickle cell disease.

The Sickle Cell Drug Addicts

I'm a nurse in a NYC public hospital and we have a cohort of patients w/SC who I see in the ED at least everyday or every other day. The hospital has a SC clinic but when it closes the patients come to the ED. They don't have to wait and everyone knows them. So for the next 6-8hrs they are given high doses of Dilaudid/Benadryl (at least three rounds) and are released. Okay, the question - are they addicts? I mean I KNOW SC is a horrible disease and reading your blog only drives that home for me. But honestly as a nurse my compassion is waning. The patients wait at the desk 2-3hrs on the dot after the last dose saying they need the next. Seeing them on a daily recurring basis only decreases my compassion as well. I really would like to get your take.

No, they aren't addicts. Think of the worst pain you have ever had in your life. It could be a broken limb, labor, migraine or even the shock like twinge you get once a while when you turn your head the wrong way. Okay now multiply that pain by 1000. And imagine that it's unrelenting, constant and totally all consuming. Picture that pain not only in the affected limb but all over your body. Imagine the pain immersing every single one of your body cells, coating them like fire while a million men are drilling into your bones. You can actually feel every single cell in your body screaming in pain, you can feel with every breath the pain gets worse and worse and worse and worse.

That was just a short snapshot of what a pain crises feels like, in fact, that was just a mini-compilation in words. In actuality it's so much worse. You've been on all kinds of pain meds all your life, in fact one might say you've built a tolerance to them. See tolerance is not the same as addiction. Addiction occurs when you crave the medication but you don't have any pain at all, Tolerance occurs when you've been on pain meds for a while and your body is accustomed to the dose, so now, a dose that you consider 'high' is actually barely scraping a few shards off the pain.

At the most, the Benadryl zonks you out enough that you can ignore the pain, but it's still there like a beastly monster, waiting for an hour or so just to pounce and devour you in waves of agony all over again. Your tolerance is to the level that the Dilaudid dose just helps to take the edge off, the edge that would otherwise have you screaming and rolling on the floor in hysteric misery.

Unfortunately for you, your prescription ran out, or you don't have a high enough dose to cover the immense pain that you are feeling. The clinic is closed. You were just in the ER yesterday, but got discharged still in pain. You don't want to go back to the hospital, but you have to, because right now at this very moment, you feel like if you don't get some help, you are going to die. It's a fine line between wanting to live and wanting to die, and that's the determination that makes you go to the hospital.

The nurses and doctors remember you from the day before. The doctor tells you that you were just here yesterday and should see your primary care physician. The nurse tells you to stop messing around, she's busy and doesn't have time to deal with fakers or addicts. You try to carry on a brave front, but inside you are a roiling mass of despair. The pain doesn't even let you focus, it doesn't let you express what you really want to say. All you can do is grip your sides and pray to God for mercy.

You watch the clock, still hurting but too proud to ask for more meds. The dose the harried nurse gave you was small and didn't do much. You smile and say thank you, gritting your teeth and sweating profusely in the effort to remain civil even though this fiendish torment is owning you, claiming you, torturing you. The hands on the clock creep slowly onward until finally you realize it's almost time for another dose. Perhaps this time you will find relief from the anguishing waves of pain. Perhaps.

Alas, your wishes are in vain as this dose barely does anything more than the last did. You know you should ask for more pain meds, but the look the nurse gave you when she injected the so called 'large' dose was enough to keep you quiet. You don't want to risk rejection and apathy from the people that are supposed to show compassion to you. So you go inward, into your happy place and become zombie like, trying to float outside of your body so that the pain doesn't get you. The nurse thinks you are sleeping, and tells another, "she's just drug seeking". Those words reached you and caused your heart to break some more. Now it's not just about the physical pain, but the emotional abuse you've gotten from the hands of the 'helpful' has just made you realize how bleak your situation is. No one understands.

Finally after the last dose, the nurse and doctor concur that you are well enough to be discharged. Your pain is still there, the underlying problems aren't solved, in fact they weren't even addressed. You are about to be discharged because you got the requisite three doses of Benadryl/Dilaudid and the ER 'can't do more to help.' Because everyone knows that three doses is enough to fix you. It's enough to fix sickle cell pain and let you go home. Besides, they need the bed for sicker patients. You protest, faintly at first, then louder, "But I'm still in pain! I'm still hurting!" Your pleas fall on deaf ears and icy demeanors. You are practically shoved out the door, paperwork appearing instantly, your IV taken out and the CNA unceremoniously comes to the room and tells you she needs to clean the bed.

The pain is still intense, you can barely walk---in fact, you find yourself falling as your knees buckle down. Your hands reach forward and prevent you from landing on the floor. Your joints protest as you use them, the cold hitting you and making everything a thousand times worse. You drag yourself by sheer force of will outside, forcing yourself to just keep breathing, keep living, keep fighting. Go Vixen, go! Just take another step, just crawl one more inch.

Tomorrow is another day. Perhaps tomorrow you will find some relief from this all consuming, all encompassing agony. Perhaps tomorrow someone will throw the rule book away and treat each patient on an individual basis. Perhaps tomorrow the doctor at the clinic will give you a higher dose for your oral pain meds. Perhaps tomorrow you will come across someone that actually understands, or at the very least, has some fuckin' compassion.

But today, all you have is you. You and your pain.

Connecting With Someone With Sickle Cell

I just got off the phone with Milan, a reader from the blog who is the absolutely most fabulous chick I've had the pleasure of meeting with sickle cell. She's like a young version of me, all spunk, feisty, full of determination, attitude, sass and heart. She's actually what I wish I was when I was her age; she's so smart and self-aware.

We spoke for an hour and I must say, talking to someone else that has sickle cell is one of the most liberating experiences that you can have. I heartily recommend it to everyone that has sickle cell. We go through our lives in isolation, rarely coming across people that really get what it is to be like us, to go through what we go through. Sure we do come across people that "understand" but unless actually you have sickle cell, you have no idea!

Milan and I connected on so many levels, and we've actually been through alot of similar experiences. I think talking and sharing with her opened me up to alot of issues that sickle cell warriors go through that I haven't even begun to address on here, like how to deal with a doctor that is butting heads with you, ignorant medical professionals, isolation in white communities and when insurance gives you the boot.

So, for meeting people with sickle cell, the internet is such a valuable resource. Don't be afraid of using it. Try Myspace, Facebook, Yahoo groups or forums. Try your doctor, he might have another patient with sickle cell that they can connect you with. Don't be afraid to pick up the phone, dial the number and actually talk live with another sickle cell warrior.

Dealing with Sickle Cell Without Using Drugs

My daughter who is 12 years old (has SS)is thinking about taking Hydroxyurea instead of getting monthly blood transfusions. If Hydroxyurea is not right for sickle cell people what other thing can they take or do if they don't want the blood monthly? Do you know?

First of all, know that I'm not a licensed medical professional. My views on Hydrea are just from my personal experiences and beliefs about it. It's shown to be effective for some people just not all. Also, it only drops your incidence of having a crises by what...30-50%; which when added to the side effects is not enough for me.

Keep in mind that not everyone has side effects, and my hematologist recommends Hydrea strongly to all his clients with sickle cell. This is the only FDA approved drug that is presently used in the US to control sickle cell. There are other options though.

1. There are non-pharmaceutical options like Pro-Arginine.
2. Diet modifications like being vegetarian, avoiding overly processed and fried foods and bulking up on foods rich in thiocynate like carrots, beets and green leafy vegetables. I highly recommend buying and incorporating Back to Our Roots in your life.
   
3. Stress relieving activities like pilates and yoga, as well as a regular exercise regimen.
4. Hydrate, hydrate, hydrate! Drink as much water as possible. With sickle cell, you can never drink enough water. I have a friend that is 48 with sickle cell, has never been hospitalized a day in his life. His secret? Water. He drinks 2 gallons a day! So drink up.
   
5. Rest. This is one of the points that I struggle with especially with my busy schedule. Try to get at least 6-8 hours a night. The most rejuvenating sleep is between 8pm and midnight. However, your body has to be in REM (which takes about 90 minutes from when you actually fall asleep). So try to go to bed early, sleep those hours and wake up feeling refreshed and rejuvenated. I'm a night own by nature and by my job so this is a hard one for me.
   

Those are the main precepts that I recommend for your daughter. I would also highly recommend doing some research into a bone marrow transplant. She might be able to be cured from this, so check with your docs if she's a good candidate.

I Have Bad Veins

One of the commonalities of sickle cell warriors is that fact that after a zillion hospital admissions and ER visits, eventually, our veins go into hiding from all the trauma. The medications that help us also help to scare the beejesus out of our veins as well as all those blood draws! My hands are scarred up from IVs and I don't even remember a time when I had good veins in my arms.

I'm down to my last good vein, and even that is getting hard and sclerosed. So in the near future if I keep getting sick, I'm going to have to put a longterm catheter in...yuck! These are the options for access that have been most frequently presented to me.

Porta-Cath: Also called a port, this is a closed access device that is placed under the skin usually on the chest wall. It's an outpatient procedure done under general anesthesia, and once you get it, the port can be accessed periodically as needed with a big needle that fits right in there. When you go home, they take the needle out and you just have the port still in your chest covered by skin. It's the most preferred for those on long term therapies, has the lowest risk of infection and is not detectable unless you are bare chested. It can be in you for months to years and will need period flushing with heparin (usually every month or so) to keep it from clotting up. I'm wary about having a lump on my gorgeous chest, but I guess you gotta do what you gotta do.

PICC Line: This is used for more short term IV management than the Port. It's inserted in the hospital by a certified nurse done in a sterile environment through one of your arms and threaded to the superior vena cava close to your heart. It can be in for a maximum of 6-8 weeks. They usually don't want to keep it longer because it has a risk of causing infection especially endocarditis. I've had 3 PICC lines all in the last 2 years and just have small scars that look like mosquito bites on my arms to show for it. Sometimes they leave it in after discharge for long term antibiotics or chemo.

Triple Lumen Catheter (TLC): This is in for the length of your hospital stay. It's usually placed in the larger veins of the femoral or jugular. If it's in your groin (Femoral) area, it has a high risk of infection, after all, your urinary & rectal tract are only a few inches away! Getting it in your neck is a real pain (I've had both). It may have 1-3 ports on it, which means you can get blood, some IV fluids and still get your pain shot through the other port. It has to be taken out at discharge though.

Those are the 3 main ones that I've come in contact with. My personal favorite is the PICC line, it's right in the middle for efficiency, ease of use and it's relatively fast to put in. A skilled nurse can do it in under an hour. So next time they mention any of these options, you are now in the know.

Sickle Cell & Cold

Today I was asked a question by a friend, "Which do you like better, hot or cold?" My answer was a vehement HOT! Not just because it's so miserable being cold but mainly because when I'm cold for a period of time (like more than 20 minutes), I put myself at a higher risk of getting pain.

This is unfortunately why I don't do anything that drops my core body temperature. Stuff like dancing in the rain, kissing in the rain or even getting in the ocean will make my joints hurt like crazy shortly therafter. Sometimes, it doesn't even take 20 minutes, I can feel the cold start seeping in from a walk through the chilly snow to my house and I know I'm going to hurt!

So I invest in lots of thermals, thick socks, gloves, scarves, coats and layered outfits. I rarely go out in the winter with just a sweater and jacket, I have to have some leggings and tights on as well, and if I have any even leg warmers. For some reason my top half feels fine, it's the extremities that do me in.

Dress warmly peoples---it's hella cold out there.

10 Things Good About Sickle Cell

Okay, I'm not here just to whine and complain. So in the spirit of giving thanks, I'm listing the top 10 reasons I can think of why it's great to be a sickle cell warrior.

1. You get a free "get out of work" pass from school, work or home whenever you are feeling tired, down or sick. This can also be applied when you are feeling kinda okay but want to spend the day painting your nails, eating bonbons and reading a good book.
2. You get lots of pain pills, so if you are ever broke, you can sell them off the street for ten bucks a Vicodin (I've never done this btw!)
3. You've learned the fine art of balancing. Some people take decades or lifetimes to learn this but you have learned to listen to your body, pace yourself and rest whenever your body demands. In essence you are actually alot healthier than the type A's who just run, run, run until they stroke out.
4. You get lots of presents, flowers and teddy bears several times a year. I have a whole collection of stuffed animals I don't know what to do with.
5. You don't have to search for a cause or something to volunteer for, you already have your life's cause imprinted in your DNA.
6. You lose weight whenever you are sick. So it's like your own insurance policy against becoming overweight. When you get better, you can eat all you want to get back to your 'ideal' body weight.
7. You get reassurance on a regular basis that your friends and family love you. You don't feel like you are taken for granted. You don't feel underappreciated or not recognized.
8. You have a legalized reason for getting narcs. The immediate rush and relief you get with IV meds is enough to make you want or crave to be sick. Okay...I know we shouldn't admit it, but we are human. Narcs feel good. Narcs feel really, really good!
9. You can buy lots of warm sexy coats and cute sweaters every year and claim it as a medical expense off your taxes.
10. You learn to love life and every moment that you are well, because you've learned that in the blink of an eye it will all dissipate. So you enjoy the moments that you have!

Pain Can Kiss My Ass

Meet Elise...isn't her attitude so fuckin' incredible?

Transmission of Sickle Cell

Okay, this is for all the ignorant peeps out there. No offense, but why the fuck do you think that you can get sickle cell from your boyfriend/girlfriend? It's not an STD, it's not contagious---it's an inherited disease. Inherited! This means that if your parents weren't on the ball with getting their HbS screening done before they got pregnant with you, then one of their presents to you is the disease.

You can't get it from kissing, or from having sex with someone with SCD. You can't get it from even interacting with them---or a blood exchange. (Although why someone with anemia might be giving blood is an anomaly anyway!) It's a DNA flaw, pure and simple, a protein on the DNA chain that got substituted wrong. So unless you are creating DNA (Mothers!) it's not something to worry about.

If you don't know your HbS type, have your doctor check for you before you decide to get pregnant. And check your sperm donor's man's HbS type as well. Here's a simple chart to give you the logistics. Remember, this is the probability per pregnancy, not per child. This means that for every time you get pregnant, you have the chances. Some people think that it means that if they have four kids, only one with have sickle cell...not so! Here's the breakdown.

AA + AA
Very, very good. None of your kids will have anything to do with Sickle cell.

AA + AS
You have a 25% chance with every pregnancy of having a child that is a carrier of the trait. No biggie. Just be sure to educate your children on their HbS before they start having sex.

AS + AS
You have a 50% chance of having a child with the trait, a 25% chance per pregnancy of having a child with no trait and a 25% chance of having a child with the disease. This is what happened to my family.

AA + SS
Moving up! This is for someone with SCD, like me, having a child with someone that isn't a carrier of either the trait or disease..(Like Norio!) You children will just have AS, just be carriers of the sickle cell trait. They'll be fine! They just have to watch their SCD parent live with it.

AS + SS
Not so good. With every pregnancy you have a 75% chance of having a child with sickle cell disease and a 25% chance of having a child that's a carrier. You might wanna test your fetuses asap to check. Or consider some other alternatives to having a child together...like adoption or an egg donor. Trust me, this isn't something you want to pass to your children.

Okay, I hope that clarifies it. You cannot get sickle cell from kissing, sex or swapping bodily fluids. You can get other STDs and HIV however, so make sure you USE PROTECTION!