Idea theyre carrying . Well, it goes to the fact that sicklecell trait is asymptomat asymptomatic. Individuals that do carry the actual sicklecell gene are not have symptoms. They may exhibit in extremes like climbing higher altitudes or scuba diving, for instance. So you honestly have to be tested in order to confirm if you do carry the sicklecell trait. And for those who dont know exactly what sicklecell disease is, what is it . Well, sicklecell disease is unfortunately a, the most common genetic disorder in the world, and its the result of two affected hemoglobin genes that come together to form sickle hemoglobin, and as you know, hemoglobin is the protein thats found in our red cells that carries oxygen to all the surrounding tissues and organs, but in individuals who carry, who have sicklecell disease, unfortunately, during times of infection, when youre under low oxygen states, under stress, dehydration, those red cells actually take the appearance of a sickle shape and end up occluding or aclotting those vessels and oxygen and nutrients j that area and people end up having the most common symptom of pain related to sicklecell disease and other complications. Reverend newton, you are a former patient. I say that, because you had circumstantial cell. You were diagnosed it five yes. Age of 5. Yes. And you you lived with it for most of your life, until about nine years ago. Yes. Nine years ago i received a bone Marrow Transplant at the john hopkins hospital in baltimore and ever since then i have been cured of sicklecell anina. Your whole life changed . It did. I went from spending most of my time in the hospital, being on different forms of medication, various blood transfusions, platelet transfusions. If you can think of any symptom im one of those and can definitely say that now im postsicklecell anemia that i live a normal life. So bone Marrow Transplant in the cure for this disease . Yes. Well, it was for me, anyway. But it is the cure. Is that the cure, dr. Oneil . Bone Marrow Transplantation as well as gene therapy are the two main ways of curing someone of sicklecell disease. Uhhuh. Do doctors test for this when babies are born and do they need to . Right. So currently in the united states, all 50 states undergo newborn screenings. So sicklecell disease is one of the screening tests that we do. Uhhuh. The importance is identifying whether or not you do have sicklecell disease or trait at the time that your child is born and possibly repeating that test by the time the child is a year of age to just confirm if it is sicklecell trait or if your child does have sicklecell disease. You can be tested to determine whether you have the trait, but you really may not know you have the disease unless you develop symptoms. Talk a little more about what the symptoms are. So unfortunately, the signs and symptoms of a child with sicklecell disease usually presents itself around 6 months of age. And its the unfortunate handtofoot syndrome, where its essentially a pain syndrom hands and feet due to infarctions where the sicklecell is tracked in the surrounding tissue and bone. Unfortunately, that is the most common presenting symptom that an infant with sicklecell disease will present with. What about an adult . Well, with adults over time you would see evidence of a compensated anemia. So its not uncommon that a majority of our sicklecell patients will have hemoglobin levels that are very low. And at the point that they my require blood transfusions to at least provide more oxygen and energy and endurance during daily activities in life. So could you confuse that with just general anemia . A patient who suffers from anemia and is generally tired . Might that be confused with the sicklecell condition . Thats very true, but other values, and also doing a hemoglobin elektro foresis or hpoc would be something to discover whether or not the anemia is due to sicklecell disease or due to some other common reason for anemia. All right. Our topic this morning is sicklecell disease. Well be back in a minute with more about the disease, and be the importance of scd awareness. Stay with us. Here in vineland, new jersey, home of progresso, we love all kinds of chicken soups. But just one kind of chicken. Its white breast meat chicken every time. Because white breast meat chicken releases its flavor into the broth and absorbs more flavor from it. And in vineland, its all about flavor so if youre not going to make your own chicken soup tonight, do what we do. Make it progresso. Welcome back. Were talking about sicklecell anem anemia. Sicklecell disease this morning here on viewpoint, and patricia, pam la newton, im sorry. You had the disease before your bone Marrow Transplant and one of the problems that sicklecell patients have to deal with is blood transfusions. They have to constantly get them. You were telling me in the break how you had to undergo transfusions sometimes twice a month . . E. Yes. Not only just because of the extreme pain, sometimes my blood count would drop. So i would receive a transfusion, but sometimes my body wouldnt necessarily take the transfusion. So they would have to infuse me again. Uhhuh. Sometimes it would be once a month. Sometimes it would be twice a month. You know, but sometimes it would be over the period of maybe a year, monthly, just receiving transfusions. That must have been pretty difficult to deal with on a reg l lar basis. How did that affect your family life . It affected me because it required me to be in the hospital. Sometimes if it was during a time when school was in. At the time, im very athletic and playing basketball. I would have to leave school and go to the hospital and spend a few days, which required me to take off from those activities as well as take away from my schoolwork, and it also took away from me spending time with my family and i was very active in my church. So it took me away from my Faith Community as well. Are you now an activist . I wouldnt say im an activist, but what i would say is that although im cured, i dont i dont ever want to forget about those who are still in that present situation, because i know what it feels like to suffer with such a deadly disease. So i think that its my responsibility to reach out to the community, to raise awareness as much as i can, for all of my brothers and my sisters who are still going through the fight of circumstantial cell anemia. Dr. Oneil is it always a deadly disease . Well, i think the important thing is that with the gene therapy and bone Marrow Transplantation that there is hope for those individuals who do suffer from sicklecell disease. However, it is important to understand that even though we see sicklecell disease as a very painful disease it does affect other organs. So it can affect the heart. People with sicklecell disease can suffer strokes can have cognitive impairment. It can affect the lungs as well as the kizdneys and other organ as well. In the past one of the main focuses in treatment i understand is to manage the pain, but thats shifting now. How . Well, i think in the last maybe 7 to 10 years, there have been a number of pharmaceutical companies that have become deeply invested in attacking rare disorders, and one of those disorders happens to be sicklecell disease. So there is a new resurgence in the Clinical Research community on developing drugs that can actually be affective in managing sicklecell disease and all of its complications. Theres really only one drug that the fda approved for this . Correct. And that drug is called hydroxy areia, its lifesaving as far as identifying that if patients are started on hydroxy erenia, they can actually have an improved life span. Actually live longer. We no longer say that you only have up until age inside our mothers womb, were ut that hemoglobin usually shuts down to maybe undetectable levels or maybe 1 to 2 at the time they were born. Hydroxy arria actually keeps the hemoglobin levels up to help with avoiding sickle crises. Did you take that drug . Before the bone Marrow Transplant . I did, and we found that it was very helpful. However, my platelet count began to drop, which is one, could be one of the side effects of the drug. So as a precaution, i had to be taken off of the hydroxy arria and we found out when that happened that my pain crisis increased. So the drug is effective. All right. Got to take another break. Well continue our talk about sicklecell disease, right after this. Here in vineland, new jersey where progresso light soups are made, well never give up our cheddar, our cream, and especially our bacon. So we figured out a way to add rich ingredients like this into 22 light soups, all with 100 calories or less per serving. So if you want to eat light and not give up rich flavor, do what we do. Make it progresso. Welcome back. Were talking about sicklecell disease, ands reverend pamela newton, you received a bone Marrow Transplant about nine years ago. How long did it take you to find a donor . It took approximately one year. The initial step was to contact the National Bone marrow registry, however, we found that there was no match. So they began testing among my family members. Unfortunately i dont have any host siblings. I have four older brothers and we are all halfsiblings. When they tested them, they found that they, too, were a match. So what they did was after that was they tested my mom, who at the time was 65, and they decided that they would use her to be my donor. How tough is it, dr. Oneil, to find donors . Unfortunately, in circumstantial cell it is very difficult to find a full matched sibling who would be the ideal donor. And so what reverend newton has described is a halflobe transplant, using someone whos a partial match, but closely matched to you to be the possible, potential donor. Who does this disease affect most often . Ive always known that africanamericans suffer a disproportionate numbers but theyre not the only one . Oh, of course not. Sicklecell disease affects primarily people with sub sahara africa, the caribbean, mexico, africa, europe, india, asia. And it may be combined with other hemoglobinopathies as well. Such as beta and alpha, in which those particular side effects and complications could be even more compounding and devastat g devastating. Does that present a greater challenge when it comes to finding donors . Right. It really does. I think in the africanamerican population we do have to understand that maybe we wont we do not have a large number of families with whole siblings. So that limits the actual availability for the donor, and that brings to light the gene therapy, where you dont necessarily need a donor for establishing a cure for bone Marrow Transplant. Where theyre taking a virus and implanting that virus with the normal hemoglobinproducing gene to then just produce normal hemoglobin. We know there have been challenges finding, getting africanamericans to donate organs. Is it easier to convince a patient, a donor, to give bone marrow . Right. So than an organ . I think i think youre right about that. In the typical sense, when were talking about a bone Marrow Transplant, where we are honestly where, extracting peripheral blood. Not actually doing the bone marrow. In some institutions they may actually extract bone marrow. Its actually taking the blood and those stem cells and using the stem cells to introduce into the recipient for the bone Marrow Transplant. So it may be a little bit easier to versus donating an organ. Reverend newton, were you optimistic that youd find a donor . Well, because of the severity of my case, and myprognosis, i was not optimistic at first. However, because im a woman of faith and i have a, a large support system, who were praying for me, and encouraging me, and also with the help of the doctors, such as dr. Oneil and other doctors at the university, my Optimism Level began to rise. l hematologist to treat this . Ideally. As children age, the likelihood of them actually being seen by a hematologist is very very slim or very unlikely. Is there a shortage . There is a shortage. Of hematologist, who are willing, actually, and manage sicklecell patients. So family practitioners, other internists and other doctors who may be interested in taking care of sicklecell disease can also be advocates in managing adults with sicklecell disease. All right. Weve got to take another break, were ut well have final thoughts about sicklecell, and what you need to know to protect you and your family, right after this. Welcome back to our discussion about sicklecell disease. Dr. Oneil, there are a lot of misperceptions about thiss clear them up for this. What are are they . Clear them up . Well, its important to know that individuals with sicklecell trait will develop sicklecell disease. If you choose to have a child that you and your partner are tested to confirm what are the odds of your child, of i dont are child having sicklecell disease. It issals a important to, to know that sicklecell trait or sickle trell disease does not contribute to other common medical conditions, like hypertension, hithyroid problem and coronary artery disease. You recommend everybody guess tested . Everyone should be tested. If its not to it is to at least clarify if you are carrying one of those abnormal hemoglobin genes, because that information is going to be important for your children and your childrens children, to determine if they may be carriers of the sicklecell trait. So thats the most important thing. Reverend newton you dont have children now. I dont. At one point youd totally given up on that . Yes, i did. I knew what i went through as a child suffering with sicklecell and i didnt feel i could subject my child to go through what i went through. However, now that im all cured im waiting for the lord to send me somebody and then possibly in the future have one child. Have one child, yes. Well, we certainly wish you well. Thank you. And were so glad that you are doing so much better. I know that its important that patients like reverend newton be advocates for themselves, and to be informed. I think that pamela is a, or reverend newton, im sorry, is a wonderful example of how someone can survive such a devastating disease, and live to actually tell millions of people that you can be cured of this devastating disease. So its a blessing to sit next to her. Yeah. Thats what youve been doing, though. Youre not just living your life. You are talking about this. You are telling other s. You have informed your family. Youve educated yourself. Yes, and also, everyone i come in contact with, i make it a point to, you know, bring this subject up. You know, i encourage them if they havent been tested to get tested. You know. Even if you think you have been techted, but theres a slight the possibility that you havent, i always recommend that you get tested. Dr. Oneil, the statistics show the most patients, 65 to 70 are economically disadvantaged. Whats the story behind those numbers . A i think the unfortunate thing is sicklecell disease afflicts people in thirdworld countries. Predominantly here in north america, africanamericans, people of hispanic and latino descent. One in three nigerians have sicklecell disease. So it is an epidemic and i think we have to understand that if we dont necessarily recognize how devastating this disease is, it will be soon. All right. Reverend patricia dr. Patricia oneil of Howard Universitys center for sicklecell disease and reverend pamela newton, thank you both so much for being with us and were so happy about your renewed life. Yes, yes. And we wish you well and good luck. Thank you. Finding that man and having that baby all right. Thank you both. And thats viewpoint. Thank you for joining us, im pat lawson muse. Stay with us. News 4 is next. Enjoy your sunday. Mmm yoplait its snack time oh, look yoplait original now has 25 less sugar. Time to taste it. How is it . It tastes good congratulations yoplait you did it yoplait news4 today starts now. Seven hours and counting until the redskins home opener and the beginning of the kirk cousins era. Well tell you how the new starting quarterback plans to keep cool under pressure. Plus a hundred people waking up away from home after a massive apartment fire. How one resident got all the residents out in time. Good morning. Welcome to news4 today on this sunday. Im adam tuss. Im angie goff. Well check in on the forecast in one second, but we have some breaking news coming into the newsroom right now. A major part of the g. W. Parkway is shuwn