This is now a discussion on sickle cell anemia. But before anything else, let's try to define what sickle cell anemia is. Sickle cell anemia is an anemia or a reduction in the number of red blood cells by reason of these cells having sickle.
Ang pasyente nagka-anemia o niubos ang iyahang red blood cell count kay ang iyahang red blood cell ni sickle. Unsa man ang sickling? Well, it actually means ang shape sa imuhang red blood cell becomes like a crescent moon. So kung makita ninyo dihan sa video, it's from a normal round, normally round red blood cell, nahimo siya into a crescent moon-shaped sickling cell.
Ang sickle cell anemia class is, first and foremost, it is an autosomal recessive condition. For a person to manifest an autosomal... recessive condition. Because it is recessive, it is required that the person must have two copies of the gene.
And for the person to have two copies of the gene, it is required that both parents must have the trait. Again, for a person to manifest a recessive condition, he has to have two copies of the gene, meaning to say homozygous angiahang pair. Let's take the case, for example, of a mother. A mother is a career, and a father being also a career.
So meaning, career, wala siya naka-manifest the trait because for a person to manifest the trait, you have to have two copies of the gene. So in the case of mama, ang usa perhaps is normal, and the other copy is abnormal. So in this case, usa ka normal, usa ka abnormal, on sa man si mama? Karira, naara siya yung trait, pero di ni mo makita na naa siya yung... sakit because again, a sickle cell anemia is recessive.
You have to have two copies of the gene. In the case of Papa, who is also a career, the redapita, then one copy must be normal and the other is abnormal. So career siya, ngano man, di siya mumanifest because again and again, for a person to manifest a recessive condition, you have to have two copies of the gene.
Kung si mama o si papa magkaanak o upat kabuok, ang ilahang anak most likely would be normal-normal, normal and abnormal, abnormal and normal, abnormal-abnormal. Sa upat nila kaanaklas, kung imong tanawon si number one, si number two, number three, number four, pila man sa ilahang upat kaanak ang mong manifest sa sickle cell anemia. Ang mumanifest. Si usara.
Ngano man. Because child number four has two copies of the abnormal gene. So kung recessive gani, you have to have two copies of the gene.
In this case, si number four na ay sickle cell anemia. Si number one. Unsa man si number one?
Si number one is normal. Dili siya career. Dipot siya mumanifest.
How about child number two and child number three? They are merely careers. like mama and like papa. In simple terms, let's simplify this. Moingon ka nga career ang usaka tao kay naasay trade, naasay usaka kupya sa trade, pero he or she will not be manifesting that because for a person to manifest, you have to have two copies of the gene because sickle cell anemia is a recessive condition.
Sickle cell anemia also class, aside from being a recessive condition, is an auto... Autosomal condition. Meaning to say, it is a defect involving your autosomes, not involving your sex chromosomes. What are autosomes? Autosomes class are chromosomes number 1 up to chromosome number 22. They are called your autosomes.
Because your chromosome number 23, the 23rd pair, is a sex chromosome. Meaning to say, kung naakay sakit or guba, from chromosome number 1, number 2, up to chromosome number 22, what is the class of the disease? Otosomal. So any defect from chromosome number 1 until chromosome number 22 is an otosomal condition. Example, if you have a defect in chromosome number 7, cystic fibrosis, otosomal.
If it's 1, up to 22. If you have a defect in chromosome number 10, here's Prong's disease. So what is it? Otosomal.
Because number 10 is one of chromosome 1 to 22. Right? Now, what if it has a defect in your sex chromosome? If it has a X-linked disorder or Y-linked disorder, then it is a sex chromosome condition.
It is not autosomal. So sickle cell disease, it's an autosomal recessive condition, which actually affects the beta chain of the hemoglobin of your red blood cell. It is a condition which affects the beta chain.
of the hemoglobin of a person. Now, moingon ganikag sickle cell class. This is a condition where ang red blood cell sa usakataw is lingin.
This is the normal red blood cell. Manaakay sickle cell or mawalaakay sickle cell ang atong red blood cell tanan parihos ramanta lingin o red blood cell. Kaso, class. ang person nga na ay sickle cell anemia or sickle cell trait, ang iyahang lingin nga red blood cell will change into a sickle shape, muraog sungkot, or a crescent moon shape.
Maunang itawag siyang sickle cell disease. Ngano mang ni-change man ang lingin into a sickling shape? Because of any of three reasons.
Kunsa man atong mga reasons nga pwedeng makapachange. Number one. pwede klas ang dehydration. When a child is dehydrated, ang lingin mahimong sikol. Number two, pwede klas nga ang bata na ay acidosis.
Meaning, kung mag-acidosis ka, ubos ang imong pH value, ang imong lingin mahimong crescent moon or musikol. Number three, pwede klas aside from dehydration, aside from acidosis, when there is a reduction in the levels of oxygen, up to the level of less than 60% O2 saturation. So kung munaog ang imong oxygen saturation, pwede di ay nga ang lingin mahimong sikol.
But then again, class, mahitabo rin yung sikling. Mahitabo rin yung transformation kung naakay sickle cell disease. Kita, class, bisan pag ihubak ta, bisan pag makubid ta, ang atong red blood cell lingin.
Ngano man, huwag man tayo sickle cell trait, huwag man tayo sickle cell disease. So sa sickle cell disease, ang lingin, mahimong, sickle. Kung na ay dehydration, acidosis, or a reduction in the oxygen level up to the extent of less than 60% O2 saturation.
So meaning to say, class, in sickle cell disease, whenever there is dao, dehydration, acidosis, or poor oxygenation, then, what do you see? There will be sickling. Okay? So, these are the three triggers that will cause the red blood cell to sickle.
Now, what are the characteristics if the red blood cell is sickle? Plus, if the red blood cell is sickle, they are fragile. They become even more fragile or short-lived. Meaning, easy to die. So, if your red blood cell dali mamatay.
Inig kamatay nila, niubos ang imuhang red blood cell. O, unsa man ang sakita, basta ubos ang imuhang red blood cell count. Anemia. Maunang itawag siya ang sickle cell anemia. An anemia that happens because of the sickling.
Kay the moment the red blood cell sickles, they become fragile, they become short-lived. Ideally, muabot mag-120 days ang kinabuhi sa red blood cell. Pero kani sila, basin 20 days lang, 30 days lang, dali mamatay. And if the red blood cell in a person is already dead, it's already dead, the red blood cell has died, it is a case of anemia.
It is an anemia because the red blood cells have sickled. It has sickled because of dehydration, acidosis, and decrease in oxygenation. And because many of your red blood cells have died.
Many of them have died. Once this is released, the red blood cell will be acquired by bilirubin. So what happens to the levels of bilirubin in the body?
There will be an increase in the levels of bilirubin. The so-called Tuana class, hyper-bilirubinemia. And what will be the manifestation of a person with increased levels of bilirubin? Magjondish.
Where can you first see the jondish class? In the whites of the eye. It's in the esclera. Pinakaunang mumanifest maklaro nga nagdag ang tao sa iyahang mata before pa makita ang pagdag sa tao diha sa iyang panit.
John Deas seems to appear first in the eyes. Done in the skin. I can see your problem into your eyes.
Dili sa panit. Awahina ng sa panit. Unag yun sa mata. Kay puti mangud ang mata.
Klaro kayo ang mancha sa mata. Klaro kayo ang pagyelo sa imuhang mata. So sickling again happens because of dao. And when there is sickling, it is short-lived. Dali mamatay.
Leading to anemia. And because inig kamatay, morilis o bilirubig, there will be accumulation. of levels of bilirubin causing jaundice in a person. And aside from that class, kung sa may mahitabo kung naatay si Kling, this will lead to a condition called vaso-occlusive crisis. Kung sa manang vaso-occlusive crisis, meaning to say, di na mo awas o tarong ang dugo while naa sa sulod sa imong ugat.
There will be an occlusion. In the blood vessel. That's why it's called vaso-occlusive.
Pagkabarah sa imuhang ugat. Ngano mang nabarah ang imuhang ugat? Class, kung kani ang imuhang ugat, class, o ang red blood cell lingin, bisan pag magbangga na sila, muslide ra na sila against each other. Kay lingin man sila.
Pero class, o gi-compare na nimo kung nanakay sikling ang imuhang red blood cell, letter J, crescent moon. Munsa may tendency. Diba? Pwede siyang magkasangit-sangit. Pwede siyang magkabuhol-buhol.
So kung magklamping na na siya, magbuhol-buhol na na siya, that will lead to occlusion in the blood vessel. Maunag itawag siya o vasoocclusion. So vasoocclusion is a condition where the red blood cells have clumped with each other because of its abnormal shape.
So vasoocclusion happens. because simply of sickling. And what is the effect kung naa kay vaso-occlusion class?
Will the blood flow? Dili na. So there will be stasis of the blood.
Muungot ang dagan sa dugo, diha sa imong ugat. And when there is stasis, then the blood cannot proceed to the intended organs. Kung muadto ka sa lungs, di nakakalaho sa lungs, kaya na-traffic man ka. So there is stasis of blood, meaning to say there can be no oxygenation to the intended organs of the body so that this stasis could lead to ischemia. Ischemia is a condition where there is no more blood flow to the intended organs.
And now my question is, when there is ischemia to the organs, will there be metabolism on those specific organs? The answer is yes. And that kind of metabolism is known as anaerobic metabolism. And we all know that anaerobic metabolism will lead to the formation of a waste product. And that waste product is lactic acid.
And lactic acid can irritate the nerves, causing extreme pain. So a patient with a sickle cell crisis, or a patient with a series of sickling, or simply nga nag-sickle ang imong pasinti, ang sickling makakosong vaso-oklusyon. Ang vaso-oklusyon, makakosog stasis. Ang stasis makakosog ischemia. Ang ischemia makakosog anaerobic metabolism.
Anaerobic metabolism would produce lactic acid. And because lactic acid is irritating to any nerve, resulting in pain. Ang sintomas ni ay sa sickle cell crisis is extreme pain to that organ nga nagkabaso oklusyon.
Okay? So pain is a manifestation of a patient with Sickle cell crisis. Another question class might arise, when would a person begin to manifest sickle cell?
You cannot manifest it at birth. You do not see sickle cell symptoms at birth because the symptoms of sickle cell only begins when the child is at least six months. What is the manifestation of sickle cell at six months?
Remember, class, sickle cell disease is an autosomal recessive condition of the beta chain of the hemoglobin. Our problem is on the aspect of the beta chain. Kung imong tanawon ang hemoglobin class, ang hemoglobin sa fetus, ang hemoglobin sa fetus, unsang manday component ana, it is comprised of 2 alpha plus 2 gamma.
Inigabot ni mo ang 6 months class. After 6 months, the fetal hemoglobin, or the fetus, the hemoglobin of the fetus, will begin to transform into an adult form. So the hemoglobin now becomes an adult form.
That's the component in the adult form of hemoglobin. Right? 2-alpha and 2-beta.
Right? So now, what is the manifestation of 6 months? Because class, you all know that the problem on sickle cell disease only happens at the aspect of the beta chain.
Meaning to say, in 1, 2, 3, 4, 5 months, Naaba tay beta chain nga guba? Wala. Wala tay beta chain nga guba.
So wala tay sintomas sa sickle cell. Pero kung mugawas na ang imong beta chain kay adult na ang imong hemoglobin after six months, na nabatay symptoms of sickle cell? Yes, because naanaman tay beta chain. Again, sickle cell disease is an autosomal recessive defect of the beta chain of the hemoglobin. So there is no problem.
1, 2, 3, 4, 5. And the problem begins at 6 months after the fetal hemoglobin has been converted into an adult hemoglobin. And that is the reason why, class, our drug of choice for sickle cell is hydroxyurea. What does it do?
Because hydroxyurea increases the number of fetal hemoglobin. Meaning to say, based on pag 50 anos na ka, 40 anos ka, 45, 46 pilay edad nimo, it does not matter so long as your hemoglobin is made in the form of fetal hemoglobin, you will be resistant to sickling. Simply because a fetal hemoglobin does not have the beta chain. So maunang ang atong tambal is hydroxyurea. Again.
Ang hydroxyurea promotes the formation of fetal hemoglobin in a person because we all know that a fetal hemoglobin is resistant to sickling. When a person has sickle cell disease, he manifests five major symptoms. And these five major symptoms class could be simplified as your 5S of sickle cell. Unsa maning 5S of sickle cell.
Number one. there could be swelling. Swelling of the hands and feet. The swelling of the hands and feet is otherwise known as your hand-foot syndrome. Nanuman class.
Because there will be inflammation to the parts of the hands and to the feet where there is no blood supply. So kung maunig imong kamot, kung maunig imong kamot, kung nagsikling ni diri na pita, Mudagan ba ang dugo? Padulong sa imuhang fingernails?
Padulong sa imong toes? Dili. So because there is no blood at this portion, there will be an inflammatory condition. The ischemia to the hands and to the feet can lead to an inflammatory condition.
Sa bisaya, mo hubag, mo inflamed, ang imuhang kamot o ang imuhang tiil. This is what we call as the hand, foot syndrome in patients with sickle cell simply because there is infarct. There is ischemia too. The hands and feet.
Number two, pwede kang magka splenomegaly. Splenomegaly. On sama ng megaly, nidako ang splen. So a patient with sickle cell is expected to have spleno Megali.
And ultimately, there can be spleen damage. Maguba ang imuhang spleen. Ngano na ama yung spleen omega-liser?
Basta naa kay sickle cell disease. Paynumduman lang tamuha. Onsa may reputation sa spleen. Onsa may atong nahibauan na function sa spleen.
Diba, class? Ang spleen is the graveyard of the cells. Lubnganan sa mga patay ng mga cell. Lubnganan sa mga nangaguba ng mga cells. The red blood cell should be cold.
If you're sick, you should take a rest. Is the shape of the red blood cell normal? It's not, right?
So the spleen is just right. That's the problem. The spleen will take out from our system any damaged cell.
And if it's in the blood of the spleen, it won't form a new one. It won't. It won't. It won't.
So what do you get? It won't be like the other one. But it will be like the red blood cell.
It's more loose. So go get hangtod ang imong spleen, ni burot. Ni dako ang imong spleen, spleno megali.
And kay grabe na siya kaburot. Guba na dayo na ang imuhang spleen such that a person will now be prone to infection. A person with a damaged spleen is a person who is at a very high risk for infection because the spleen plays an important role in the control.
of infectious process. Splenomegaly. What is S number 3? S number 3 is short-lived red blood cell.
Un sa may epik to, kung dali mamatay ang imuhang red blood cell, what will the person manifest? The person will have anemia. There is anemia kaya ang iyahang dugo pas-pas mamatay. Maunang niubos ang iyahang red blood cell count.
Because the red blood cells are short-lived. And remember that when the red blood cells are hemolyzed, when the red blood cells die, it gives up your unconjugated bilirubin. When the excretion of bilirubin is exceeded by the number by which bilirubin is produced, ang risultaan na class is magsklera ni mo kay magjondis.
So the person will manifest a jondis sklera. Kanosan na mahita mo? Kung ang paghimo sa iglalabay, bilirubin, is mas paspas kaysa paglabay mismo sa bilirubin, then there will be hyperbilirubinemia or jaundice which is first seen in the eyes. I can see your problem into your eyes. And number five, there could be sequestration.
Unsa man ang sequestration, pagpangimbargo. Kinsa may mangimbargo. So, iyang isequester ang mga damaged ng mga red blood cell in the body. Kuha dire, kuha dito. O niya, class.
Inig sikol, anaklas. Tanan man ni mo dugo ni sikel. Tibo, kaduguan man ni mo ang ni sikel.
So, kuha una ni spline ang imong dugo. So a person with sequestration will die of hypovolemia. Magka-hypovolemic siya kay wak na'y dugo ang tao.
Wak na siya'y dugo di kay nagdugo siya. Wak na siya'y dugo kay gibargo. Gi-sequester ni spleen. And these are the 5S of sickle cell.
Swelling, splenomegaly. With spleen damage Your short-lived red blood cell Causing anemia Your sclera turning yellow Or jaundice Because of the excessive levels of bilirubin Where the production of bilirubin is higher Than the rate it is excreted And sequestration Causing severe hypovolemia Lastly class How do we manage sickle cell disease? Not only that What is sickle cell disease? Delikado ang ilahang kinabuhi. Kaya di sila pwede ma-expose to dehydration.
Di sila pwede ma-expose to acidosis. Di sila pwede ma-expose to low oxygen levels. Delikado ang ilang kinabuhi.
So what are we hoping for people with sickle cell? Let's just give them hope. Let's give hope to patients with sickle cell.
Lahirin yung YouTuber. Let's give hope. Let's give hope to patients with sickle cell.
H, you hydrate? Natural. Kung dehydration ang kos, tambal, hydrate, either IV fluid or increase oral fluids.
Common sense man siguro na class. Kung ang hinongdan dehydration, they hydrate. Kung ang hinongdan poor oxygenation, they give oxygen.
Diba? So you hydrate, you give oxygen. And what is a very significant symptom of a patient with sickle cell crisis? A person is in...
Pain. So letter P. Pain management. So what can we call this?
We can call this acetaminophen. Okay? We can call this morphine. But take note, class.
Watch out for morphine, right? Because morphine is an opioid analgesic. And an overdose of your opioid analgesic can lead to respiratory depression. How do you know depression is respiratory?
System. If the respiratory rate... Obos. Less than 12 to 20 cycles per minute.
So a person taking opioid analgesic like morphine may be overdosed as manifested by respiratory depression. Dapat ang nurse andam. Dapat ang nurse alert pag counter anak.
Kung sa ganito ihatag ni nurse, basta na ay respiratory depression from overdose in opioid analgesic? Naloxone. Narcan.
Your antidote for opioid. toxicity. So pain management. Importante ni class ha? You need to relieve the patient of pain.
Nga naman, when a person is in pain, his demand for oxygen increases. When the demand for oxygen increases, then pwedeng muubos ang iyahang oxygen. And you know that ang pag-ubos sa oxygen is the reason for sickling.
So kung magsickling, mu pain na po. Kung magpain na po, masikling na po, kaya nga no, gamit sa kusigin. Nga, magpain na po. And you would notice that it seems to be like a cascade. Siya, naka-kos-aning, nani-kos-niyang, nani-kos-niyang, hangtunwa na yung undang.
That's why you need to manage pain with acetaminophen or morphine. Otherwise, there will be a cascade of pain and sickling in patients with sickle cell disease. And letter E. Tanawag itong kus-klas.
Dehydration. acidosis. Poor oxygenation.
Unsa may pwede natong mabuhat para mawagtang ang acidosis. We need to manage the electrolyte levels of the patient. So class, give hope to patients with sickle cell disease. You give hydroxyurea, you give hope, and another H is to hypertransfuse your PAK.
Red blood cell. Mag-abuno tag dugo sa pasyente nga packed red blood cell ra. Di ka mo hatag ug fresh whole blood.
Ang imong ihatag kay red blood cell ra gyo nga tos pasyente. But make sure that prior to giving your packed red blood cell, before hyper transfusion of packed red blood cell, na-address na nimo ang dao. Na-address na nimo si dehydration, acidosis, and poor oxygenation. Otherwise, ang 1,000,000 katuniladang apak red blood cell ngayong mong ihatag, musikil ragihapon to og bargohon ragihapon sa spleen.
Sayang ang dugong. Okay? So let me end this class by saying that in sickle cell, we give hope to patients with sickle cell.
Thank you for watching.