The truth of the matter is...

  

You know what? I'm in a lot of pain right now (11.3.12) and writing this is the only way I feel I can distract myself. I was hospitalized because as I was walking to my doctors office I had to stop several times to gather myself by trying to control my breathing because I couldn't breathe and I was having pain at the same time. The social worker I just happened to run into suggested I go to the emergency room (ER) since the doctor I was going to visit was at jury duty and the person sitting as the replacement wouldn't have an open appointment till hours later.

I went to the ER and was treated with oxygen, a nebulizer treatment, and finally ketamine. The thing with treating my pain with ketamine is that I have to be admitted in the hospital in order to receive the treatment. Ketamine is the only drug that helps my pain now, not narcotics or transfusions. So my options are more than limited. It's frustrating to even think about it. To be honest it scares me to think about the reality of my fears. The options I have to treat my pain are really one thing: ketamine. And without it my fears have been--what will happen if I build a tolerance to it? Will I be left with no way to manage my pain?

The truth of the matter is that I'm in the hospital about seven months out of the year. That's NOT NORMAL!! It's at the point where my doctors have been discharging me home even though I'm still in really bad pain. They say I'm on too much ketamine and that's not good for my QT (refers to a group of disorders that increase the risk for sudden death due to an abnormal heartbeat.).

In spite of my pain I was told that I have to be discharged adding--there's nothing more we can do. Can you imagine that conversation? I know what she's about to say and once she tells me- imagine what I must have been feeling. I felt numb. I felt like OMG it's gotten to the point where I'm totally on my own?!? Then she asks me if I have any questions. I mean I know it doesn't matter what I say because once your told you have to leave whether your in pain or not-- what else is there to say? I knew no matter what I said things wouldn't change but I understood that she was just trying to give me something to quiet the hurt (I think). I appreciate her tact. I can't imagine being chosen to be the one to tell somebody that their pain can't be helped--to their face!.

When they  started discharging me in pain--I'll be honest--I was angry because all I could see was my pain and when your in pain things like conversations or decision making are ignored because all I can think of is God Please Make IT StOp--Take it Away! How could I see anything else? I've learned to digest the different phases of my disease so I stopped being angry at them, stopped being frustrated with everything and just pray and leave things to God. God will guide me and whatever needs I have. Especially when it comes to my disease. Whatever God allows I take in. When I think of the team of docs that work with me--I see how hard they have worked and continue to work, I think of the many meetings they had on my case, and I think of the consultations they've had with me--throughout the process keeping me informed on ideas for my pain management . Its been a long process of which I've always been included in.

I realize the work they've put into me and I thank them for it but where it ends for them it continues with me. I can't step away and say I've done  what i can. My pain is hard to control and being left with no options is a huge reality for me. For instance, my discharge. All that's in store for me is endurance.

So I've made the decision to just get what I can from my docs upon admission into the hospital. When they discharge me I’ll just continue to pray that whatever treatment I get will be sufficient. I'll just lay at home in pain and pray to God for his grace. I've been told I need to learn how to deal with the pain as it is. The thing is all my life I've had to endure the pain and there's no getting used to Pain!! Could you endure the pain of someone  pulling or ripping off your arm? That's how the pain feels sometimes. Or what about hundreds of knives being stabbed into your body? Another--not being able to walk or talk because of the pain. My family has had to carry me because of the pain. This is an even scarier journey I'm about to partake in and it seems I stand alone in this phase. I appreciate all the time and hard work the hospital staff has put in. I wish there was more they could do but they've done enough I guess. I wanted to blame them for the intense pain I've had to endure when discharged and at home but why? I thought they gave up on me but that's unfair.

It's hard for people to empathize with the pain I go through. Most don't understand. Some friends have walked away from me because of the pain they saw me in. I went along with the justification that I'm hard to be friends with because its true. In the past there were phases in my health back then. It went from the doctors treating me with pain meds, to their being frustrated with not being able to find an answer for my pain despite their efforts, and finally their frustration meant since they couldn't figure things out I needed to be discharged. I could always predict when the doctors would want to discharge me. Despite all this I would have months and months after discharge to live my life, enjoy my twenties, and be a good Christian.

Now I’m isolated/not social because I have no free time to be social between my hospitals admissions and my recouping period. I live with my father and his wife so I can get treatment from a specialized hospital nearby. I’m on disability because of how sick I've gotten that it keeps me from being able to work. I’m single, while all my peers are married and having families. I've seen a lot and endured a lot and lived a lot differently than most. Don’t feel sorry for me instead please Pray 4 me! I would die for the chance to get back into life but my life is here-now!- and I’m grateful for the amount of energy God spends on teaching me hard and necessary lessons of all sorts. Despite how I hate the control Sickle Cell Anemia (SS) has on my life, I Would Not Be The Woman I Am Today without it!

The truth of the matter is that it will always come full circle to me, my disease, my thorn, my problem. This disease chose me not the other way around. Sometimes I feel people forget that. Even my doctors. Though they're all great people I know if they lived a week in my worst pain they'd really understand and sympathize with Sickle Cell suffers in such a different light. God will guide me through what's to come, good or bad! This disease has directed everything in my life, I’m just along for the ride. God has a plan for me! God has a plan for YOU! Jeremiah 29:11-13.

A 2nd Transplant! Here we go AgAiN!

As many readers know I had a bone marrow transplant a year ago. It's a shame but for some reason my body rejected the bone marrow. The reason is unknown to myself and my doctor. I thought it was all over and all that hard work it took to get through it was for naught other than the lessons I learned from it. Anyway, I thought that was it but the doctors I worked with were so supportive of me that they approved for me to have a second transplant. Of course, I cannot use the same donor (my brother, Fola) for this next transplant. So though for the first transplant my father was excluded from being tested because of his age, they have decided to allow it for this transplant because they want me to not have to live with this pain anymore and they want to open up doors for more SS patients to be transplanted. My youngest brother Ayo will also be tested! 

It's crazy! It was a difficult process the first time around. I just have to have faith that God will get me through this as well no matter how tough it might get. Though it was a tough process, it went relatively well the first time to the surprise of my doctors and I. My biggest fear was that I would have a crises throughout the process but I only had pain at the beginning of it. My biggest fear this time around is that it won't be as easy. 

I know what to expect this time so if it doesn't go as easy I know how bad it can really get. Anyway, it will only exercise my faith especially if for a second time I reject my donor! I don't know if I can take another rejection. Before I get ahead of myself my father and brother have to be a match before any of that can be done. If neither my father or brother are a match then the transplant is not going to happen. I asked if we can go to a bank but since I'm the first transplant patient and its a new process they can only use family members. Once it begins to grow and more patients get transplants then a bone marrow bank can be used.

So I again ask for your prayers. Pray I find a match and pray the transplant is a success!!

Thank You! 

Ketamine

Pain management has been a challenge my doctors have had to deal with. The only way any of my doctors have known to tackle this challenge is by increasing my opiod use. This was the case until one of the best doctors I've had the privilege of working with Dr. Viscusi (pain team) began to revamp the way they treated my pain. Instead of just giving me increased doses of morphine they added to the roster Ketamine. Ketamine is an analgesic drug known to be used for different health issues. It has recently been known to be used as a pain reliever for crisis pain in few cases. Ketamine affects the sensitization of spinal neurons and so affect the development of neuropathic pain.

Ketamine is sometimes used for emergency surgery. As a random aside: I've been told it has been used as a horse tranquilizer. It's a pretty intense drug with huge side effects. Many people on Ketamine have to be monitored since it can cause psychotropic side effects like hallucinations. When on this drug a nurse or doctor is assigned through a 24hr/day period to monitor me in the event something happens or if I need an extra dose.

The highest level of opiod I've been on was 1200mg a day of Morphine. Ridiculous right? It's insane to confess that even at that dosage I still wasn't getting adequate pain relief. 1200mg a day was only as an outpatient. Once admitted I would also be maxed out on dilaudid PCA yet still not get pain relief. So Dr. Viscusi suggested to the team of doctors and nurses working with me that I begin treatment with Ketamine. I would become the first sickle cell patient to use this drug for pain management. My morphine dosage was dropped from 1200mg-600mg a day and about six months ago the pain team decided that those high levels of opiod were actually contributing to my pain as opposed to alleviating it. So I began a tapper of the morphine that went down 10% every three days as an inpatient.

                                          Ketamine:

It was extremely tough to get off the Morphine since I've been taking large doses of it for years. I went through intense withdrawal. I kept asking myself how do drug users do this but in time I was finally completely off it and Dr. Viscusi suggested my not being medicated with any other opiod but instead manage my pain at home with alternative drugs such as Butrans (also help with withdrawals), nortryptaline, and Lyrica. As an inpatient include those drugs and an infusion of intravenous ketamine. The highest level of Ketamine I've been on is 40mg.

Since I'm no longer on opiods I've been finding it difficult to control my pain at home so the pain team has prescribed me with nasal ketamine. Once again I'm the first SS patient to be prescribed it. In the 2yrs (or more) I've been on Ketamine I never knew that there was a nasal spray for it. I was told that it is used in cases of people who have severe migraine's. Though I do have migraines I use it not for that but for my pain crises (attacks). Since my discharge from the hospital about a month ago I've used it once and I've remained out of the hospital which is a victory for me since recently I've been returning back to the hospital a week or two after my discharges. So staying out though its been only a month is a real victory. I only use the nasal ketamine when my pain is at its worst and I think I may have to go to the hospital. The less I use it the more likely it is to work when I do use it. So my goal is to keep from using it too much because if my body gets too conditioned to the use of this drug I fear that there are no more options for me out there. I was lucky Dr. Viscusi knew about this drug but my options are getting less and less. I don't know why my pain is as extreme as its been since graduating from college but I fear that if the pain that I go through scares me as it does now then what am I to expect in 5yrs or 10yrs? Thank God for Ketamine. If I wasn't on it then imagine what dose of Morphine I may be on now.

A step at a time, a day at a time!

Eugene Viscusi, MD, director of Acute Pain Management at The University of Thomas Jefferson Hospital

Dr. Viscusi is one of the best doctors I have ever had the pleasure of working with. I am now 31 yrs old and since I was a baby, starting with the hand foot syndrome and jaundice, I have been fighting every year til now with this disease. In all these years I have had many encounters with doctors and nurses that were good & bad. Those doctors that were bad were individuals that made me feel small, were in-compassionate, frustrated that they didn't understand why the disease was affecting me the way it was and as a result would get frustrated with me. 

However, the good doctors and nurses were patient, and understanding. They took into account that my form of the disease wasn't my fault and instead doubting/disregarding me and my input--would work with me in trying to stabilize my many hospitalizations. Dr. Viscusi is the latter. He's not perfect but I just wanted to write a blog in his honor because no matter the ups and downs I truly respect and am grateful for how hard you work on my behalf. I know on my end that I too can get impatient and scared but you always match me with assurance and thoughtfulness. Dr. Viscusi just has a lot of wisdom. He knows what kind of medication to give me for that specific symptom. Unlike doctors I've had in the past that write Rx for the sake of writing them. I've been given medications that have totally shut my body down. Docs have written huge and uneffective doses that would leave me lethargic at times. For instance, I used to take 450mg of morphine three times a day in addition to taking other pain meds! However, Dr. Viscusi saw how uneffective it was and how it was actually hurting me and adding to my pain--instead of easing it. So they tapered me off of  it while I was in the hospital. Though I went through intense withdrawals, it was later obvious to everyone that because I was off of the morphine, my spirit/demeanor was lifted. So, on doctors orders, I am no longer taking narcotics~across the board. If it wasn't for Dr. Viscusi I'd still be on those large doses feeling weak and tired all the time and I would still be in a lot of pain.

Dr. Viscusi has always been open and honest with me about my care. He's always been willing to take some risks to get me to a point where he can ease my pain. Ketamine is an analgesic drug used in operations to keep the patient sedated. Well Dr. Viscusi wanted to try using that drug for my sickle cell pain! He was open about what the side effects were and the fact that this was an experimental trial. This drug has never been used with sickle cell patients before, so I would be the first. I was fine with that. I'm always open to new ways of dealing with my pain! Plus, I trust Dr. Viscusi! Well it worked! Ketamine helped my pain (didn't cure it) and I still use it till this day! No other Doctor has been willing to work so hard for me and take risks for me like Dr. Viscusi! I am truly grateful for him and so I wanted to take the time out to show him how grateful I am for having him as my doctor! All the work that you do does not go unnoticed, ThaNk yOu!!!

Working with the set of doctors and nurses that I work with has in many ways become a family. As many know, families have their ups and downs but in spite of it they remain family. Well in spite of the hard times I do appreciate you all and want to thank you all (PURPLE TEAM, PAIN TEAM)!!!

In My Weakness I Found Strength

You know every day this summer I got up and felt alone. I had no one there with me. At those moments where your thoughts and mind go--u know?  I'm in my room thinking and wondering when will this all end? Will this end? I did this transplant because God told me to & I hoped that with this would be the cure. Never did I think that the transplant process was not for my disease but for my self.

I put all my hopes, prayers, and will into this transplant being a success and curing me but in the midst of it all I've found that the transplant has cured me but not of my disease but of my self. I've always been such a driven person and used my situations as my push or start ups to life's challenges. How could I have forgotten that the biggest challenge of all was to be my inner will. For so long I have relied on people, friends, family to be my launching point but God has been teaching me over and over again with it all going over my head until now. My launching point needs to be God. And needs to be me. In the end when things are at its finest of times and hardest of times family won't be there nor will my friends but my Savior--Eloi (will be there) and I alone will be there.

All my life all I've wanted was to feel wanted, loved, appreciated like anyone else would but all my life God has put me in situations where I've felt unloved, unappreciated,or unwanted. Why? I guess you can ask God that but if you ask me then its because God wants my character, my core, my everything to be nothing but humbled. Having this disease and living with the pains that it presents is the hardest. I've been learning and continue to learn that God definitely has a plan for me, a plan to prosper me and not to harm me but to give me a sense of direction and drive that will get me through life, it's challenges and the surprises that come on its way. I can no longer rely on friends or family to be my point of direction. God is teaching me that He alone is my point of direction and that as life goes on family and friends may disappoint me but Never will My God disappoint me. He is my ROCK & my everlasting strength so No, this transplant was not a bone marrow transplant but God's ways of spiritually transplanting my fears and concerns with his spirit of peace and serenity.

You know going through this transplant process has been so much more than an eye opener. It has opened up my life and the people in it. I've learned that some people are there for a season and others for the long haul. Though I wish those that were here for the season were here for the long haul I am truly grateful for the time that God has put you in my life. I pray that another season comes and that when it does we'll both be in a place where we can maybe be there for one another for the long haul.

My strength comes at the time of my life when no one else is there to help--it's just God & me and we lock arm in arm and walk right through whatever the challenges that present itself.

I've learned that though I do need friends and family by my side I can survive without them. If they can survive without me then I can survive without them and our lives can move on as it is destined to. Though I wish we could survive together. I've learned that survival means you need to live your life. So go and live the Life that God has called you to. Whatever the needs and desires God will provide--just trust in him and it will be okay--but in every single weakness look long and hard because there lies the strength you never thought existed.

Interview with National Heart, Lung, and Blood Institute (NHLBI)

I was invited with a few core others to do an interview with the Acting Director Dr. Shurin of The National Heart, Lung, and Blood Institute (NHLBI) at the National Institutes of Health (NIH). It was a short interview but informative. The meeting consisted of about three or four representatives of the sickle cell community who posed questions pertaining to sickle cell disease and the strides NHLBI is making to further research, awareness, education & the policies that will make them all happen.

*Has there been a surveillance project identifying the needs of the sickle cell population?

Dr. Shurin’s response: a Survelance Project has been established to develop guidelines to assure the needs of the sickle cell community are met. The details of those guidelines have not yet been published but will be once everything is established.

Here is a snippet of what NHLBI is doing with this project:
(follow this link to read the rest of the article, http://www.nih.gov/news/health/feb2010/nhlbi-18.htm)

NHLBI, CDC Launch Surveillance and Research Program for Inherited Blood Diseases
Six States to Study Sickle Cell Disease and Thalassemias in National Pilot Project
Medical researchers are developing a new surveillance system to determine the number of patients diagnosed with a family of inherited blood disorders known as hemoglobinopathies, including sickle cell disease, thalassemias, and hemoglobin E disease.
The National Heart, Lung, and Blood Institute (NHLBI) of the National Institutes of Health is funding the four-year pilot project, which will involve the Centers for Disease Control and Prevention and six state health departments, to create ways to learn more about the extent of hemoglobinopathies in the United States.
… The research will also help describe the demographic characteristics of individuals with these conditions as well as their geographic distribution. Researchers will also examine the existing health care resources available for patients with hemoglobinopathies.

---This Surveillance is key to identifying where patients are, what may be lacking in that area and how we can measure


*How is prioritizing since this is an old disease but there doesn’t seem to be an urgency unlike with other disease?

Dr. Shurin’s response: We do need more research however; the reimbursement level is low for people to be treated & tried in these clinical trials. Congress is important to keep the pressure on & not just on the physician’s. Sickle Cell Disease Guidelines which aim at helping make SCD a priority. What the NHLBI Has Done Up to Now

Additional Information I found on the topic:


2008 new structure and innovations in SCD program
In March 2008, after a rigorous program assessment, extensive public input, and advice from the National Heart Lung and Blood Advisory Committee, the NHLBI announced a comprehensive and innovative restructuring of its research SCD program. As a result, the NHLBI is moving forward with the following innovations to its SCD portfolio:
• Basic science: Support for basic research will be expanded through funding of investigator-initiated grant applications and through NHLBI-initiated RFAs focused on the pathophysiology of SCD, the biology of pain in SCD, fetal hemoglobin switching, and genetic modifiers of disease expression and progression.
• Translational and clinical research: The NHLBI reconfigured the Comprehensive Sickle Cell Center (CSCC) program into a Basic and Translational Research Program (BTRP) with dedicated training components.
• Participation in clinical research: The scope of clinical research trials will be broadened to allow a greater number of people with SCD to participate in NIH-sponsored clinical research trials.
• Community outreach: In partnership with the Sickle Cell Disease Association of America and other patient advocacy groups and professional organizations, the NHLBI will develop evidence-based guidelines for the care of people with SCD across the lifespan that can be used by health care practitioners throughout the world.
• New resources for medical care: The NHLBI is in the process of creating resources that will improve the medical care that is provided to individuals living with sickle cell disease.

2009 workshop on awareness and educational needs of SCD community

In September 2009, the NHLBI held a workshop to help inform NHLBI program staff of the awareness and educational needs that the sickle cell community would like to see addressed on a national scale - the SCD Awareness and Education Campaign Strategy Development Workshop. This workshop brought together individuals with sickle cell disease, community based organizations that serve the sickle cell population and representatives from the health care community. The goals were to determine where there are gaps in sickle cell awareness and education, what approaches could be taken to help close these gaps, and what partners can help the NHLBI address the health communication needs of the sickle cell community.

2009 - present
The result of all these deliberations is the current major effort at NHLBI in leading the development of a set of evidence-based guidelines for the management of sickle cell disease. The guidelines will be principally aimed at primary care practitioners and their patients to help manage the ongoing processes of the disease. The guidelines will also be valuable to hematology specialists and their patients. The process also will identify areas where additional research is needed to obtain evidence that can inform practice
*Do enough people with sickle cell anemia participate in the clinical studies available to help aide in its success?
There is an issue with having minorities participate in clinical studies which are needed to further research success. Volunteers can participate in studies by visiting: http://clinicaltrials.gov/ct2/results?term=sickle+cell+anemia


*Who is involved with putting together these guidelines? Are any sickle cell patients involved in speaking up about the needs of the community from the patient’s perspective and with insights only sickle cell patients can give?

Dr. Shurin’s response: the table of people involved in this project consists of mainly administrators however; an employee associated with the project has sickle cell and can add the patient perspective.


*In asking this question I was hoping to hear that mainly different walks were involved with putting the guidelines together namely, physicians, nurses, administrators, patients, family members of SC patients, social workers that work with the SC population, etc. However, that was not the case. My hope is that in the end the guidelines truly cover the vast majority of problems, needs, and issues sickle cell patients face. When they become available & I read them, know that I’ll share my views on them. I look forward to your comments on them as well.

I did ask: Do you know if new steps are taken to help ER’s restructure the way they treat & care for SC patients ie. Waiting time, pain management?


Dr. Schurin's response: SC patients will need to speak to their hospital administrators and work together on making those changes. It’s difficult to work on this issue as a whole because each hospital runs differently & have different policies so the more effective means would be to work with the hospital social worker who advocates for you. That would make a load of difference with the administrators of the hospital.

What can be done to create a greater pool of donors in the blood bank for Bone Marrow Transplant?

Dr. Shurin's response: By making this none to your readers that there is a need. The more people donate the better pool of donors there will be for transplants.

I had other questions like, why there have been so many Sickle Cell Center closings; How to better educate doctors and nurses that work in the ER’s. However, we weren’t able to get to those. I want to encourage my readers to not only become aware of the issues presented here but to stand up and be apart of the solution. We can start “together” by encouraging those we know to donate to the bone marrow bank, volunteer for those clinical studies & holding the administrators in your varying hospitals accountable--to take notice of the needs of SC patients. We can have success but we need to not only rely on institutions like NIH or NHLBI but hold ourselves accountable as well.

Join me in the fight.

Trust

It's amazing to me how hard it is to find a doctor who is truly invested in your care and overall well-being. I've seen so many doctors-- I couldn't even give a number to the amount of doctors I've seen. When it comes to my care I am very involved. You'd think many would be involved in their care but most patients just go along with whatever the doctor says without really asking questions or voicing concerns. My approach to my care is that of trust, communication, and dialogue.

Trust to me goes both ways. I need the trust of my physician so that I can get the best possible care. If my physician doesn't trust me then there's limitation to understanding and believing what I share with them about my health. For instance, when I share what I believe is affecting my pain--if my doctor doesn't listen/trust me then no matter what I say the doctor will dismiss my concerns and not take into account my insight into my health. Then there's my trust in my doctor. Trusting in my doctor is the key for me in feeling comfortable. If I trust my doctor then I know that the doctor has my best interest at heart.

Having open communication with both my physicians and nurses means making sure that all the doctors and nurses involved are on the same page. During one of my hospitalizations I was put on a specific medication by one doctor in the morning but by afternoon another doctor decided to remove me from that medication. The next day the doctor who initially had me placed on that drug asked me what happened since he was unaware that a change had been made. I was left having to explain to him that another physician did not feel as though the medication I was placed on was helping me so I was taken off that drug. Not long after--I was placed back on that medication. I shouldn't have been put in that situation and I shouldn't have been compromised like I was. Sudden changes like that can have an adverse effect therefore pushing any progress I had back (which means increased pain).

As a result of this experience I've been very adamant with all my physicians involved about communication so that such a thing does not happen again. Now, before any changes are made I ask to make sure that all parties are not only aware of upcoming changes but are in agreement on the best plan of action for my health. Aside from it being important for the nurses and doctors to be on the same page I continuously advocate that I be made aware of any and all changes to my medications, treatment, tests etc. so that I can understand the reasoning behind such changes and so that I can voice concerns that may arise. When it comes to sickle cell anemia many in the health care system agree that we the patient know our bodies better than some physicians. The best doctors that I work with are the ones that don't pretend to have all the answers but are the ones that are humble enough to admit that I go through this all the time and I know my body better than they do. The doctors that ask what do you think, what dosage should I start with, what works best for you, are the best because its not about pretenses but about quality of care and pain management. In the same breathe the best patients are the ones that admit that they don't have all the answers and are willing to work with their physician. It is a collaborative effort treating sickle cell anemia and no one person has all the answers. That is why I play such an integral role in my care. From knowing why a certain blood test is taken to the dosage of a medication--I make sure that I am aware of what is entering and leaving my body. If I don't take my health seriously then who else will? If I don't fight to be heard then who endures all the pain? If the doctor makes all the decisions with no input from the patient then who lives with the ramifications? The answer to all of these questions and many more is, I do!

Whenever I'm placed on a new drug that is unknown to me I do research until I am completely comfortable & knowledgeable about that drug. This is my life. I take it very seriously. I want to encourage you to do the same and make sure that you are a part of your treatment & care. Upon being discharged from the hospital during one visit I was discharged on a new drug that I've never heard of and so I began my research. When the doctor came in to do their discharge summary I had a lot of questions pertaining to that drug & the physician shared how impressed she was with my diligence. It didn't even occur to me that that was what I was doing until she said that. So it got me thinking and it encouraged me to share with you all how important it is not to be a "yes" man when it comes to your health. Many people treat doctors like God-whatever they say goes. If you have a question, ask it. If you have some hesitations then voice it. If your physician doesn't want to work with you then maybe it's time to start looking for someone who'll want to hear those questions & concerns because they should want the best for you. You are your best advocate so Advocate!