good sunday morning. welcome to "viewpoint." i'm pat lawson muse. sickle-cell disease is the most common genetic disorder in the country. an estimated 3 million people carry the sickle-cell trait. for those who suffer from the disease, it can be pretty tough and pretty painful to live with. september is sickle-cell awareness month. joining me this morning here on "viewpoint," dr. patricia anneal, the center for sickle-cell disease and reverend be pamela newton, a are toer sickle-cell patient now cured of the disease. thank you both for joining us. >> thank you. >> thank you. >> dr. o'neil, this is a very common disease. if it's so common, why is it so many carry the trait and have no

idea they're carrying? >> well, it goes to the fact that sickle-cell trait is asymptomat asymptomatic. individuals that do carry the actual sickle-cell 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 sickle-cell trait. >> and for those who don't know exactly what sickle-cell disease is, what is it? >> well, sickle-cell disease is unfortunately a, the most common genetic disorder in the world, and it's the result of two affected hemoglobin genes that come together to form sickle hemoglobin, and as you know, hemoglobin is the protein that's found in our red cells that carries oxygen to all the

surrounding tissues and organs, but in individuals who carry, who have sickle-cell disease, unfortunately, during times of infection, when you're 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 sickle-cell 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 sickle-cell 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 i'm one of those and can definitely say that now i'm postsickle-cell 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. o'neil? >> bone marrow transplantation as well as gene therapy are the two main ways of curing someone of sickle-cell disease. >> uh-huh. >> 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 sickle-cell disease is one of the screening tests that we do. >> uh-huh. >> the importance is identifying whether or not you do have sickle-cell 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 sickle-cell trait or if your child does have sickle-cell 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 sickle-cell disease usually presents itself around 6 months of age. and it's the unfortunate hand-to-foot syndrome, where it's essentially a pain syndrom hands and feet due to infarctions where the sickle-cell is tracked in the

surrounding tissue and bone. unfortunately, that is the most common presenting symptom that an infant with sickle-cell disease will present with. >> what about an adult? >> well, with adults over time you would see evidence of a compensated anemia. so it's not uncommon that a majority of our sickle-cell 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 sickle-cell condition? >> that's 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 sickle-cell disease or due to some other

common reason for anemia. >> all right. our topic this morning is sickle-cell disease. we'll be back in a minute with more about the disease, and be the importance of scd awareness. stay with us.

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welcome back. we're talking about sickle-cell anem anemia. sickle-cell disease this morning here on "viewpoint," and patricia, pam la newton, i'm sorry. you had the disease before your bone marrow transplant and one of the problems that sickle-cell 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 wouldn't necessarily take the transfusion. so they would have to infuse me again. >> uh-huh. >> 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, i'm 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 wouldn't say i'm an activist, but what i would say is that although i'm cured, i don't -- i don't 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 it's 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. o'neil 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 sickle-cell disease. however, it is important to understand that even though we see sickle-cell disease as a very painful disease it does affect other organs. so it can affect the heart. people with sickle-cell 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 that's 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 sickle-cell disease. so there is a new resurgence in the clinical research community on developing drugs that can actually be affective in managing sickle-cell disease and all of its complications. >> there's really only one drug that the fda approved for this? >> correct. and that drug is called hydroxy areia, it's life-saving 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 mother's 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. we'll continue our talk about sickle-cell disease, right after this.

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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 don't have any host siblings. i have four older brothers and we are all half-siblings. 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. o'neil, 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 half-lobe transplant, using someone who's a partial match, but closely matched to you to be the possible, potential donor. >> who does this disease affect

most often? i've always known that african-americans suffer a disproportionate numbers but they're not the only one? >> oh, of course not. sickle-cell 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 african-american population we do have to understand that maybe we won't -- 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 don't necessarily need a donor for establishing a cure for bone marrow transplant. where they're taking a virus and implanting that virus with the normal hemoglobin-producing gene to then just produce normal hemoglobin. >> we know there have been challenges finding, getting african-americans 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 you're right about that. in the typical sense, when we're 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. it's 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 you'd find a donor? >> well, because of the severity of my case, and myprognosis, i was not optimistic at first. however, because i'm 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. o'neil 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 sickle-cell patients. so family practitioners, other internists and other doctors who may be interested in taking care of sickle-cell disease can also be advocates in managing adults with sickle-cell disease. >> all right. we've got to take another break, were ut we'll have final thoughts about sickle-cell, and what you need to know to protect you and your family, right after this. ♪

welcome back to our discussion about sickle-cell disease. dr. o'neil, there are a lot of misperceptions about this's clear them up for this. what are are they? clear them up? >> well, it's important to know that individuals with

sickle-cell trait will develop sickle-cell 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 don't are child having sickle-cell disease. it issals a important to, to know that sickle-cell 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 it's 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 children's children, to determine if they may be carriers of the sickle-cell

trait. so that's the most important thing. >> reverend newton you don't have children now. >> i don't. >> at one point you'd totally given up on that? >> yes, i did. i knew what i went through as a child suffering with sickle-cell and i didn't feel i could subject my child to go through what i went through. however, now that i'm all cured i'm 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 we're so glad that you are doing so much better. i know that it's important that patients like reverend newton be advocates for themselves, and to be informed. >> i think that pamela is a, or reverend newton, i'm 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 it's a blessing to sit next to her. >> yeah. that's what you've been doing, though. you're not just living your life. you are talking about this. you are telling other s. you have informed your family. you've 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 haven't been tested to get tested. you know. even if you think you have been techted, but there's a slight the possibility that you haven't, i always recommend that you get tested. >> dr. o'neil, the statistics show the most patients, 65% to 70% are economically disadvantaged. what's the story behind those numbers? >> a i think the unfortunate thing is sickle-cell disease afflicts people in third-world countries. predominantly here in north america, african-americans, people of hispanic and latino

descent. one in three nigerians have sickle-cell disease. so it is an epidemic and i think we have to understand that if we don't necessarily recognize how devastating this disease is, it will be soon. >> all right. reverend patricia -- dr. patricia o'neil of howard university's center for sickle-cell disease and reverend pamela newton, thank you both so much for being with us and we're 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 that's "viewpoint." thank you for joining us, i'm pat lawson muse. stay with us. news 4 is next. enjoy your sunday.

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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. we'll 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. i'm adam tuss. >> i'm angie goff. we'll 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