Sickle Cell Disease - knowledge is power!

Image Credits: Sickle Cell 101

Thanks to all who participated in our poll a few weeks ago.

Question was: Daddy has the Sickle Cell trait/disease. Mommy does not have the Sickle Cell trait/disease. Is it possible for them to have a child who has Sickle Cell disease?

The correct answer is YES!

A child can inherit Sickle Cell DISEASE even when both parents do not have hemoglobin S. If one parent has hemoglobin S, there are other interactions that could lead to Sickle Cell DISEASE. Here are some of the possible ways that a child can inherit the major types of Sickle Cell DISEASE.

1.) Parent 1 has hemoglobin S. Parent 2 has hemoglobin S. The child could inherit a type of Sickle Cell DISEASE referred to as hemoglobin SS.

2.) Parent 1 has hemoglobin S. Parent 2 does not have hemoglobin S. However, Parent 2 has Thalassemia. The child could inherit a type of Sickle Cell DISEASE referred to as Sickle Beta Zero Thalassemia. The child could also inherit another type of Sickle Cell DISEASE referred to as Sickle Beta Plus Thalassemia.

3.) Parent 1 has hemoglobin S. Parent 2 does not have hemoglobin S. However, Parent 2 has hemoglobin C. The child could inherit a type of Sickle Cell DISEASE referred to as hemoglobin SC.

4.) Parent 1 has hemoglobin S. Parent 2 does not have hemoglobin S. Parent 2 does not have hemoglobin C. However, Parent 2 has another type of abnormal hemoglobin (e.g D or E or O). The child could inherit a type of Sickle Cell DISEASE referred to as hemoglobin SD or SE or SO etc....

As you can see from the above, contrary to common knowledge that Sickle Cell DISEASE generally means that the person has hemoglobin SS, there are actually multiple types of Sickle Cell DISEASE.

• Hemoglobin SS is the most common form of the disease.

• Hemoglobin SS and Sickle Beta Zero Thalassemia are the most severe forms of the disease.

• Hemoglobin SC is a moderate form of the disease.

• Sickle Beta Plus Thalassemia is generally considered to be the mildest form of the disease.

• Hemoglobin SD or SE or SO are very rare and the severity of the disease in those rare cases, vary.

Important to know: Sickle Cell screening is critical. Unfortunately, the “Sickle Cell test” only screens for hemoglobin S. This means that you could do the screen for Sickle Cell, have negative results (absence of hemoglobin S) but still have a child with Sickle Cell disease from one of the other variations mentioned above if your partner has hemoglobin S. To be completely sure, you should request i) a Complete Blood Count (CBC) as abnormal results could potentially indicate Thalassemia and ii.) DNA testing or hemoglobin electrophoresis to find out the type of hemoglobin present in your blood.

Do you/your partner have hemoglobin S? Do you/your partner have hemoglobin C? Do you/your partner have Thalassemia? Know your genetic makeup. Knowledge is power!

Please note, the focus of this writing was solely on Sickle Cell DISEASE, not the Sickle Cell trait.

My Cancer Journey (Part 2)

"What did I loose in the whole process? Nothing really, except for a big chunk of my left breast."

My sister, Deanie took me to the second surgery in September 2007 and it was more of the same. The Surgeon was very nice as was the hospital staff. I was used to the routine and I felt confident that everything would be fine after this second surgery. I had the same post-op routine.

All I had to do was wait.

After two weeks I believe, I had follow-up tests and again I was told that not all the cancer had been taken out. A third surgery was scheduled for November. My daughter, Arlene took me to the third surgery. What I will always remember is that this time the surgeon walked me all the way to the theater. Quite a long walk, which one of the nurses offered to do but the doctor said no. What a guy!

As he wheeled me along that long walk, we talked about everything that was totally irrelevant to the surgery; things that I liked doing; what he liked; his family; mine; current news; anything that came to his mind. What a walk that was! It definitely put me at ease and the surgery seem like the furthermost thing on my mind.

I had the surgery and the doctor again said that everything had gone well. My daughter is usually a happy-go-lucky person and she followed in the doctor’s footsteps. She talked about everything with me on our way home and she made me laugh a lot. We stopped for Chinese food at a restaurant on Riggs Road in Maryland. I followed the same post-op routines and I went to work as usual in the succeeding weeks. I looked forward to the follow up visit with my doctor. I was really prepared for any news whatsoever, good or bad. Finally, the day came, I saw the doctor and he told me that he seemed to have got everything out. Free at last, I thought.

"What did I loose in the whole process? Nothing really, except for a big chunk of my left breast."

I had not lost any sleep over the issue and I had not allowed myself to dwell on it for one minute. The doctor told me that I would need to do Radiology but not Chemotherapy. He suggested that to be on the safe side I should see an Oncologist to make sure everything was ok. He further advised me to enjoy Christmas and then schedule Radiology in the New Year. I had every intention of doing that. I saw the Oncologist as advised and he said that the prognosis looked good for a complete cure. He confirmed that there was no need for Chemotherapy. He suggested that I could take Hormone tablets if needed. He gave me a prescription which I never filled out. To this day I have not taken any hormone tablets.

I scheduled Radiology for January 2008. Sometimes I went once a week, sometimes twice. I had to exercise a lot and was given a 12 oz bottle to fill up with water and drink before every session. I was really worried when I started especially when I realized what Radiology entailed. Any remaining Cancer was literally being burnt out of the affected Breast. When the sessions started, the heat on the breast did not seem so bad but as the sessions progressed the heat was intensified. Sometimes I was very scared especially seeing how the heated area became very dark and swollen. A Cream was prescribed which I applied to the affected area. Every time I was assured that it was a normal reaction and that things were proceeding quite well. Everything healed up quite well and the sessions lasted for eight weeks. Following that, I had six-month mammograms for two or three years and it was determined that the Cancer seemed completely cured. After those initial years, I started having Mammograms on a yearly basis and to date everything has turned out fine.

In 2008 my Sister, Deanie went on the Susan G. Komen Cancer Walk on my behalf. In 2009 I started the first of five walks with Komen. From 2010-2013 my daughter Arlene, my son Brian, Deanie and my sister-in-law Sonia went on the Walk with me. We had a blast each time since we not only walked on my behalf but on behalf of others, friends and family who were survivors or victims of Cancer. We looked forward to that Walk every year.

What an experience!!! My kids told me that they were devastated when I gave them the news in 2007 and that they were “freaking out” according to my daughter. She added that the way I dealt with it helped her and her brother to cope. They both told me that not allowing it to get me down helped them tremendously since naturally they thought they were going to lose me. I thank the Lord every day for taking me through and out of it. I drew a blank and totally did not talk about it or think seriously about it when I was actually dealing with it. I told my children from the onset that I would handle it and that everything would be alright.

I never discussed it further with them except when they asked me questions about the treatment. Was that the right approach? I don’t think so. The name of the non-profit organization “Hematology and Cancer Untold” is quite apt since it depicts quite accurately my attitude and that of so many of us when dealing with Cancer. It is certainly an “untold” disease. People don’t usually discuss it, but in hindsight I feel that we should. Of course, I talked to one or two people in Church about it after the fact, but the majority did not know about it until 2014 when my family had a surprise birthday party for me. My son, Brian talked about how devastated he was to hear about my illness in 2007. Since that time also I have advised a lot of people about how to deal with it. I know that the most important thing is to be brave and strong and not be overwhelmed by the diagnosis. Also, the Cancer surgeries prepared me for the others I have had; Knee replacement, Thyroid and Back surgery.

Finally, I think about “cool chick” Enitan Metzger so many times and I never cease to be amazed by her strength, courage and tenacity. She had been suffering all her life from Sickle Cell and yet she did not complain. She took it all in her stride every day of her life. What has always been foremost in my mind was what she said to me one particular day when we were teaching at the Annie Walsh Memorial School. “Ar see den foot yar day art me tiday. Ar nor bisin sef, den day do den yone, me day do mi yone.” In other words: “My legs hurt today, but I am not going to pay them any mind. They are doing their thing and I am doing mine.” That is the attitude I used to determine my altitude in dealing with Cancer and any other adversity for that matter. I’m sure all Cancer survivors have their specific and personal reactions to it.

Whatever, it is, I advise you not to let Cancer own you and bog you down with undue concern and worry. You must own it, control it, handle it and you will be the better for it. When I see or hear about people suffering from Cancer and fighting for their lives, I say, “You go guys, that’s the way to do it. However, I take my hat off to all non-survivors. They lost the battle, but they fought hard. May we hold them in our embrace forever and may their struggles continue to inspire us. As I look through the eyes of all the kids at St. Jude’s, Children's’ Hospital I say to them, “Go on, boys and girls, fight for your lives. Just do it!” I salute them for their strength, courage and bravery. I understand their pain and suffering since I have walked a mile in their shoes.

Dedicated to my Family, Dorothea Williams, my Cancer Surgeon, all the other doctors, nurses and hospital personnel at Sibley hospital, Community Radiology Associates, WRA, my “Cool Chicks” Selina Pratt, Rosaline Robison, Elizabeth Boyle, Enitan Metzger, and my beloved Mom, Matilda George whose extreme bravery and courage in the face of so much adversity will forever sustain me.

My Cancer Journey (Part 1)

I looked at my sister and thought…”if I don’t come out of this I will never see her again or my children or the rest of my family."

In June or July 2007 I went for a routine Mammogram at Community Radiology in White Oak, Maryland when I was told that there was an abnormality. I was asked to redo the test at which time I got the same prognosis. I did an ultra sound and was informed that the results would be sent to the lab for further analysis.

A few days later I was told that I had breast cancer and had to discuss options with my primary doctor. That day and subsequent days, what went through my mind was that I had not done a mammogram in 2006. Anyway, I decided from that moment onwards that I was not going to worry unduly about the situation.

I saw my primary care physician in Washington, D.C. and he said to me: “Oh, you don’t have to worry about it. You’ll be fine. I’m going to send you to the “Breast man,” his exact words which I will never forget. Oh, boy was that reassuring! He referred me to a breast cancer surgeon in Washington, D.C and I made an appointment to see him. The Surgeon was middle aged, very handsome and very charming. He put me at ease from the moment I saw him. By that time my test results had been sent to him and he pulled them up on his computer and looked them over. He showed me the initial test pictures as well as the ultra sound pictures and then proceeded to explain the status to me.

The breast cancer was in its initial stage and it looked like a little needle point on my left breast. I was surprised at how calmly I accepted the diagnosis. I put it to the furthermost part of my mind and heart. I believe this was how I made sure that I maintained my sanity. At no time did I dwell on it or feel sorry for myself. I swept it off the rug and kept it below the rug because most of all I did not need sympathy from anyone. Somehow or other I did not feel sad, just numb. I remember trying to lighten my mood by saying to myself, “And the fun has just begun.” Who did I tell about the diagnosis? I told my sisters, and Dorothea Williams my close friend, who is like a sister to me. Why didn’t I tell other people? 1) As much as I am a people person, I always prefer to keep my personal business private and I do not usually pry into other people’s business. 2) We don’t usually talk about Cancer.

With the Surgeon’s help I scheduled a whole gamut of tests which were mandatory and during those tests someone was always either sticking me here or poking me there. The most painful of all those tests for me was the Biopsy. For that test, something like a Stapler was used to get a piece of the affected area for further testing. “Ouch, I said, during the biopsy. “that really hurt”; but I took it all in my stride. I later discovered that it was the most pain I would feel throughout the entire preliminary tests. All those tests further confirmed the initial prognosis that it was breast cancer in the early stages. The Surgeon asked me what I wanted to do; i.e. start with a Lumpectomy [to remove all the cancer from the breast] or have a complete Mastectomy [cut off the entire Breast]. Naturally, I felt that the latter option was too drastic, so I opted for the former – A date was set for the Surgery at Sibley Memorial Hospital, Washington, D.C., August 24, 2007.

The days leading to the surgery were as normal as possible for me. I went to work as usual. At the time I worked from 11 pm to 7 am taking care of a 90 year old lady. I had specifically accepted that job a year earlier [2006], to allow me to be one of the principal care givers to our Mom. So, after work each morning I went to my Mom’s house and performed the daily duties. I got her breakfast ready, took her clothes our and got them ready for her, helped her to take a bath, made her hair, read the daily guide and scripture for the day, prayed together and had breakfast together. If she had any appointments, I took her there. We also took walks as often as she could. This was a fun time for both of us because we spent a lot of time together. We talked about every subject under the moon; and we laughed a lot.

At no time did I tell her that I had Cancer. She had enough problems of her own. She had suffered three Strokes from which she recovered very well. She had severe arthritis and both knees hurt her tremendously. Furthermore, she was awaiting Bi-lateral Knee surgery. Somehow, my Mom’s life and mine had always been so intertwined. Since I was the first born, I seemed to have inherited all her ailments. I was also suffering from severe arthritis at the time. Her bilateral Knee replacement surgery was first scheduled for August 24, the day of my Surgery but it got postponed to the next Friday, August 31, so I had my first cancer surgery one week before her Knee replacement surgery.

Finally, it was the day of the surgery. It was supposed to be an in and out procedure. My sister, Deanie took me to Sibley Hospital. On the way we talked and laughed a lot as though we had no care in the world. I appreciated her so much that morning. She helped tremendously in keeping me sane and calm. All the staff who greeted us and prepared me for Surgery were extremely nice. They also helped a lot in keeping me calm. I seemed to be in some kind of stupor in which I was not thinking about the situation or what was going to happen. The surgeon came and spoke to me and made sure I was comfortable. All pre-surgery motions were carried out. My sister was still in the room as I was prepped for surgery.

"When the Anesthesiologist came in, introduced herself to me and explained what she would do was when the gravity of the situation dawned on me."

When she held my hand and injected it, I looked at my sister and thought…”so if I don’t come out of this I will never see her again or my children or the rest of my family. The floodgates opened then, and tears were streaming out of my eyes and down my face. The Anesthesiologist saw that and continued reassuring me that everything would be fine. Soon I was unconscious and had the surgery. I woke up in the recovery room and was given the usual ginger ale and graham crackers. My sister was there when I woke up. The Surgeon came and told me that everything had gone well. The staff gave me the post-op directions and forty-five minutes later I was ready to go home. There was bandage and dressing around my left arm and around the left breast but what struck me most was the little discomfort that I felt. After the surgery I went home, rested and went to work at night. I followed all the post op directions; [avoid any type of strain of the left arm; keep the dressing on for a couple of days and keep the area dry]. Except for a little stiffness at times there was hardly any real pain in the breast.

I made an appointment for a follow up with the surgeon. He had told me that what would really determine the success of the surgery would be if the “realms” were clean; i.e. [the area surrounding the cancer spot from the breast to the armpit]. After two weeks I saw the surgeon again and unfortunately, he told me that the realms were not clean. I had to do more x-rays and ultra sounds to confirm that. I went to WRA, Washington Radiology Associates and was told that the Cancer was gone. I went again to Community Radiology Associates and was told that the realms were indeed not clean. Finally, I did some more testing at Sibley hospital and got confirmation that the Cancer was still in the Breast. Another surgery was scheduled for September.

Read more about my story in the blog, My Cancer Journey (Part 2).