Niedokrwistość sierpowatokrwinkowa
Objawy

Niedokrwistość sierpowatokrwinkowa (SCD) jest dziedzicznym schorzeniem charakteryzującym się obecnością nieprawidłowych erytrocytów o kształcie sierpowatym, które ulegają przedwczesnemu rozpadowi (10-20 dni vs. 120 dni w normie), prowadząc do przewlekłej niedokrwistości i hipoksji tkanek. Główne objawy kliniczne obejmują nawracające kryzysy bólowe (VOC) spowodowane mikrozwężeniem naczyń, przewlekły ból, zapalenie palców (dactylitis), żółtaczkę, a także powikłania narządowe takie jak ostry zespół klatki piersiowej (ACS), udary mózgu, sekwestrację śledziony oraz priapizm. U dzieci obserwuje się dodatkowo opóźnienie wzrostu i dojrzewania oraz zwiększoną podatność na infekcje bakteryjne, zwłaszcza pneumokokowe. Diagnostyka i monitorowanie powinny uwzględniać ocenę funkcji narządów oraz profilaktykę powikłań zakaźnych poprzez szczepienia i profilaktykę antybiotykową.

Leczenie SCD opiera się na terapii modyfikującej przebieg choroby, w tym stosowaniu hydroksymocznika, który redukuje częstość kryzysów bólowych i potrzebę transfuzji, oraz profilaktyce infekcji u dzieci (penicylina od 2 miesiąca życia do 5 lat lub dłużej). Przeszczepienie komórek macierzystych stanowi potencjalne leczenie kuratywne, a nowoczesne terapie genowe oferują obiecujące wyniki w eliminacji objawów. Średnia długość życia pacjentów wynosi obecnie 42-47 lat, z różnicami płciowymi (mężczyźni 49,3 lat, kobiety 55 lat). Kluczowe jest stałe monitorowanie i kompleksowa opieka interdyscyplinarna, aby zapobiegać powikłaniom zagrażającym życiu, takim jak ostry zespół klatki piersiowej, udar mózgu czy sekwestracja śledziony, oraz zapewnić optymalną jakość życia pacjentów.

Niedokrwistość sierpowatokrwinkowa – objawy

Niedokrwistość sierpowatokrwinkowa (choroba sierpowatokrwinkowa) jest dziedzicznym schorzeniem krwi, którego objawy zazwyczaj zaczynają się ujawniać około 6. miesiąca życia. Nasilenie objawów jest różne u poszczególnych pacjentów i może zmieniać się z czasem, od łagodnych dolegliwości po poważne powikłania wymagające hospitalizacji12.

Niedokrwistość i zmęczenie

Niedokrwistość jest jednym z najczęstszych objawów niedokrwistości sierpowatokrwinkowej. Występuje, ponieważ nieprawidłowe sierpowate krwinki czerwone łatwo ulegają rozpadowi i obumierają. Podczas gdy prawidłowe krwinki czerwone zazwyczaj żyją około 120 dni, krwinki sierpowate giną w ciągu 10-20 dni, co prowadzi do niedoboru czerwonych krwinek1. Z powodu niedostatecznej liczby krwinek czerwonych organizm nie otrzymuje wystarczającej ilości tlenu, co powoduje zmęczenie, bladość, osłabienie i przyspieszone bicie serca23.

Ból i kryzysy bólowe

Powtarzające się epizody ostrego bólu, zwane kryzysami bólowymi lub przełomami naczyniowo-okluzyjnymi (VOC), są głównym objawem niedokrwistości sierpowatokrwinkowej. Ból pojawia się, gdy sierpowate krwinki czerwone blokują przepływ krwi przez drobne naczynia krwionośne do klatki piersiowej, brzucha i stawów1. Intensywność bólu jest różna i może trwać od kilku godzin do kilku dni. U niektórych osób występuje tylko kilka kryzysów bólowych rocznie, u innych – kilkanaście lub więcej14.

Ból może być opisywany jako ostry, intensywny, kłujący lub pulsujący i może wystąpić w dowolnej części ciała, a najczęściej dotyczy:54:

  • Klatki piersiowej
  • Pleców
  • Nóg i rąk
  • Stawów

42

Przewlekły ból jest również powszechny u pacjentów z niedokrwistością sierpowatokrwinkową. Jest to ból utrzymujący się dłużej niż 6 miesięcy i różni się od bólu podczas kryzysów67.

Obrzęk dłoni i stóp

Sierpowate krwinki czerwone mogą blokować krążenie krwi w dłoniach i stopach, powodując ich obrzęk. Jest to objaw nazywany zapaleniem palców (dactylitis) i często jest jednym z pierwszych objawów choroby u niemowląt i małych dzieci89.

Częste infekcje

Śledziona odgrywa ważną rolę w ochronie przed infekcjami. Uszkodzenie śledziony w przebiegu niedokrwistości sierpowatokrwinkowej zwiększa ryzyko rozwoju infekcji, szczególnie bakteryjnych, takich jak zapalenie płuc czy zapalenie opon mózgowo-rdzeniowych. Niemowlęta i dzieci z niedokrwistością sierpowatokrwinkową często otrzymują szczepionki i antybiotyki, aby zapobiec potencjalnie zagrażającym życiu infekcjom82.

Opóźniony wzrost i dojrzewanie

Krwinki czerwone dostarczają organizmowi tlen i składniki odżywcze niezbędne do wzrostu. Niedobór zdrowych krwinek czerwonych może spowolnić wzrost niemowląt i dzieci oraz opóźnić dojrzewanie płciowe u nastolatków810.

Żółtaczka

Żółtaczka, czyli zażółcenie skóry, oczu i błon śluzowych jamy ustnej, jest częstym objawem niedokrwistości sierpowatokrwinkowej. Pojawia się, gdy wątroba nie nadąża z filtrowaniem bilirubiny uwalnianej podczas przedwczesnego rozpadu krwinek sierpowatych211.

Problemy ze wzrokiem

Drobne naczynia krwionośne zaopatrujące oczy w krew mogą zostać zatkane przez komórki sierpowate. Może to uszkodzić siatkówkę – część oka przetwarzającą obrazy – i prowadzić do problemów ze wzrokiem, w tym do mroczków, niewyraźnego widzenia, pogorszonego widzenia nocnego, a nawet utraty wzroku812.

Powikłania i progresja choroby

Niedokrwistość sierpowatokrwinkowa może prowadzić do poważnych powikłań, które mogą zagrażać życiu. Osoby z niedokrwistością sierpowatokrwinkową często wymagają pilnej opieki medycznej lub są przyjmowane do szpitala z powodu powikłań, takich jak ostry zespół klatki piersiowej (ACS) czy przełom naczyniowo-okluzyjny (VOC)13.

Ostry zespół klatki piersiowej (ACS)

Ostry zespół klatki piersiowej jest najczęstszym powikłaniem niedokrwistości sierpowatokrwinkowej. Jest również najczęstszą przyczyną zgonów i drugą najczęstszą przyczyną hospitalizacji. Pojawia się, gdy komórki sierpowate tworzą skupiska i zatykają naczynia krwionośne w płucach. Objawy obejmują1314:

  • Nagły ból w klatce piersiowej
  • Kaszel
  • Gorączkę
  • Trudności z oddychaniem

13

Udar mózgu

Sierpowate krwinki mogą blokować naczynia krwionośne zaopatrujące mózg w tlen, zwiększając ryzyko udaru. Objawy udaru obejmują nagłą słabość, drętwienie po jednej stronie ciała, dezorientację lub trudności z mówieniem, widzeniem lub chodzeniem1415.

Sekwestracja śledziony

Sekwestracja śledziony występuje, gdy sierpowate krwinki czerwone gromadzą się w śledzionie, powodując jej powiększenie i bolesność. Stan ten może prowadzić do nagłego spadku poziomu hemoglobiny i może być śmiertelny, jeśli nie zostanie natychmiast leczony1617.

Priapizm

Priapizm to niechciany i czasami przedłużający się bolesny wzwód. Pojawia się, gdy sierpowate krwinki czerwone blokują naczynia krwionośne w penisie. Jest to stan nagły, który może prowadzić do zaburzeń erekcji, jeśli nie zostanie leczony1418.

Uszkodzenie narządów

Z wiekiem osoby z niedokrwistością sierpowatokrwinkową mogą rozwinąć różne i poważniejsze problemy medyczne, które pojawiają się, gdy tkanki narządów nie otrzymują wystarczającej ilości tlenu. Osoby z niedokrwistością sierpowatokrwinkową są narażone na zwiększone ryzyko udaru oraz uszkodzenia płuc, nerek, śledziony i wątroby1319.

Progresja choroby

Niedokrwistość sierpowatokrwinkowa to choroba postępująca, która może powodować postępujące uszkodzenia narządów w miarę upływu czasu, począwszy od dzieciństwa. Młodsi ludzie są zagrożeni postępującym uszkodzeniem narządów do czasu osiągnięcia wczesnej dorosłości20.

W ciągu pierwszych 10 lat po przejściu do opieki dla dorosłych następuje szybsze pogarszanie się uszkodzeń narządów, a wskaźnik śmiertelności wynosi do 20%. U 50% osób występuje nieodwracalne uszkodzenie przynajmniej jednego narządu do 50. roku życia20.

Dzięki wczesnej diagnozie i leczeniu w celu złagodzenia powikłań, osoby z niedokrwistością sierpowatokrwinkową mogą dożyć 50 lat. Niektóre osoby z tą chorobą mogą żyć znacznie dłużej13.

Powikłania u dzieci

U dzieci z niedokrwistością sierpowatokrwinkową mogą wystąpić specyficzne powikłania, takie jak1121:

  • Zapalenie palców (dactylitis) – bolesny obrzęk dłoni i stóp
  • Opóźniony wzrost i rozwój
  • Zwiększona podatność na infekcje, szczególnie te wywoływane przez bakterie pneumokokowe
  • Sekwestracja śledziony – powiększenie i bolesność śledziony z powodu uwięzionych w niej krwinek czerwonych

1122

Różnice w objawach w zależności od wieku

Objawy niedokrwistości sierpowatokrwinkowej mogą się różnić w zależności od wieku23:

  • Niemowlęta i małe dzieci mogą doświadczać:
    • Gorączki
    • Bólu brzucha
    • Infekcji bakteryjnych pneumokokowych
    • Bolesnego obrzęku dłoni i stóp (zapalenie palców)
    • Sekwestracji śledziony

    23

  • Nastolatki i młodzi dorośli częściej rozwijają:
    • Owrzodzenia nóg
    • Martwicę jałową kości
    • Uszkodzenie oczu

    23

  • Dorośli zazwyczaj doświadczają:
    • Przerywanego bólu spowodowanego uszkodzeniem kości, mięśni lub narządów wewnętrznych
    • Powikłań związanych z uszkodzeniem narządów

    24

Sytuacje wymagające natychmiastowej pomocy medycznej

Niedokrwistość sierpowatokrwinkowa może prowadzić do zagrażających życiu problemów zdrowotnych. Należy natychmiast szukać pomocy medycznej lub dzwonić pod numer alarmowy, jeśli u osoby z niedokrwistością sierpowatokrwinkową wystąpią25:

  • Silny ból
  • Skrajne zmęczenie
  • Duszność
  • Zawroty głowy
  • Nieregularne bicie serca
  • Gorączka powyżej 38,5°C
  • Ból w klatce piersiowej, kaszel, gorączka i duszność (mogące wskazywać na ostry zespół klatki piersiowej)
  • Nagła słabość, drętwienie po jednej stronie ciała, dezorientacja lub problemy z mówieniem, widzeniem lub chodzeniem (mogące wskazywać na udar)
  • Priapizm (erekcja trwająca 4 godziny lub dłużej)

2514

Jakość życia i prognozy

Niedokrwistość sierpowatokrwinkowa to choroba przewlekła, która wymaga ciągłego monitorowania i leczenia. Jednakże dzięki postępom w medycynie jakość i długość życia pacjentów z tą chorobą znacznie się poprawiły26.

Leczenie niedokrwistości sierpowatokrwinkowej zazwyczaj ma na celu unikanie epizodów bólowych, łagodzenie objawów i zapobieganie powikłaniom. Obejmuje ono26:

  • Hydroksymocznik (Hydroxyurea) – zmniejsza częstość kryzysów bólowych i może zmniejszyć potrzebę transfuzji krwi i pobytów w szpitalu
  • Antybiotyki – dzieci z niedokrwistością sierpowatokrwinkową mogą otrzymywać penicylinę od około 2 miesiąca życia do 5 roku życia lub dłużej, aby zapobiec infekcjom
  • Przeszczep komórek macierzystych – może wyleczyć niedokrwistość sierpowatokrwinkową
  • Terapia genowa – osoby skutecznie leczone terapią edycji genów nie mają już objawów niedokrwistości sierpowatokrwinkowej

26

Średnia oczekiwana długość życia dla osoby z niedokrwistością sierpowatokrwinkową wynosi obecnie między 42 a 47 lat, ale przy właściwym leczeniu może być dłuższa. Osoby przypisane przy urodzeniu do płci męskiej mają średnią długość życia 49,3 lat, a osoby przypisane do płci żeńskiej – 55 lat2728.

Ważne jest, aby osoby z niedokrwistością sierpowatokrwinkową utrzymywały stały kontakt z lekarzem podstawowej opieki zdrowotnej. Pomoże to w zarządzaniu objawami niedokrwistości sierpowatokrwinkowej i zapobieganiu problemom w późniejszym życiu29.

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  1. 12.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 Sickle cell anemia – Symptoms & causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/symptoms-causes/syc-20355876
    Symptoms of sickle cell anemia usually appear around 6 months of age. They vary from person to person and may change over time. Symptoms can include: […] Anemia. Sickle cells break apart easily and die. Typical red blood cells usually live for about 120 days before they need to be replaced. But sickle cells usually die in 10 to 20 days, leaving a shortage of red blood cells. This is known as anemia. Without enough red blood cells, the body can’t get enough oxygen. This causes fatigue. […] Episodes of pain. Periodic episodes of extreme pain, called pain crises, are a major symptom of sickle cell anemia. Pain develops when sickle-shaped red blood cells block blood flow through tiny blood vessels to the chest, abdomen and joints. The pain varies in intensity and can last for a few hours to a few days. Some people have only a few pain crises a year. Others have a dozen or more a year. A severe pain crisis requires a hospital stay.
  • #2 Sickle Cell Anemia: Symptoms, Causes & Treatment
    https://my.clevelandclinic.org/health/diseases/4579-sickle-cell-anemia
    Sickle cell anemia is a severe form of the inherited blood disorder, sickle cell disease. Symptoms are fatigue, infections and painful tissue damage. […] Symptoms change over time as your body makes more abnormal (sickled) cells. Common symptoms include: Fatigue from anemia: Babies with sickle cell anemia may seem unusually fussy and irritable. Frequent infections: The condition affects your spleen, which weakens your immune system so you’re more likely to develop infections. Pain: Sickle cell anemia causes tissue damage because your tissues don’t get enough oxygen. Tissue damage hurts, so you may have pain in your arms, legs, chest and back. It may start as an ache that gets worse or come on suddenly, causing excruciating pain. Painful swelling in hands and feet: This is one of the first symptoms of sickle cell anemia in babies. Yellow-colored eyes and skin from jaundice: Your liver filters red blood cells. In sickle cell anemia, fast-dying sickled cells release bilirubin that builds up in your system to cause jaundice.
  • #3 Sickle Cell Disease (SCD): Types, Symptoms & Causes
    https://my.clevelandclinic.org/health/diseases/12100-sickle-cell-disease
    Red blood cells die early with sickle cell disease, which can lead to anemia. Anemia occurs when you dont have enough healthy red blood cells to carry oxygen throughout your body. Anemia can cause severe fatigue, along with jaundice, irritability, dizziness and lightheadedness. […] Sickle cell disease can affect many parts of your body. Some of the effects are acute (they start suddenly) and some are chronic (they last for a long time). Sickle cell complications begin early and continue throughout your life.
  • #4 Sickle Cell Crisis: Symptoms, Causes, Treatment, Prevention
    https://www.webmd.com/a-to-z-guides/sickle-cell-crisis
    The most common sign is pain that might be dull, stabbing, throbbing, or sharp, and seems to come out of nowhere. How severe it is and how long it lasts varies with different people and different crises. Some people have crises here and there, while others may have them every month. […] You might feel the pain anywhere in your body and in more than one place, but its often in your: Arms and legs, Belly, Chest, Hands and feet (more typical in young children), Lower back. […] You may also have: Breathing problems (shortness of breath or pain when breathing or both), Extreme tiredness, Headache or dizziness, Painful erections, Weakness or a hard time moving some parts of your body, Yellowish skin color (jaundice).
  • #5 Signs and Symptoms of Sickle Cell Disease | Hematology-Oncology Associates of CNY
    https://www.hoacny.com/signs-and-symptoms-sickle-cell-disease
    If a person has sickle cell disease (SCD), it is present at birth. But most infants do not have any problems from the disease until they are about 5 or 6 months of age. Early symptoms of SCD may include: Painful swelling of the hands and feet, known as dactylitis, Fatigue or fussiness from anemia, A yellowish color of the skin, known as jaundice, or whites of the eyes, known as icteris, that occurs when a large number of red cells hemolyze. The signs and symptoms of SCD will vary from person to person and can change over time. Most of the signs and symptoms of SCD are related to complications of the disease. […] Pain episodes (crises) can occur without warning when sickle cells block blood flow and decrease oxygen delivery. People describe this pain as sharp, intense, stabbing, or throbbing. Severe crises can be even more uncomfortable than post-surgical pain or childbirth.
  • #6 Signs and Symptoms of Sickle Cell Disease | Hematology-Oncology Associates of CNY
    https://www.hoacny.com/signs-and-symptoms-sickle-cell-disease
    Many adolescents and adults with SCD suffer from chronic pain. This kind of pain has been hard for people to describe, but it is usually different from crisis pain or the pain that results from organ damage. […] People with SCD usually have mild to moderate anemia. At times, however, they can have severe anemia. Severe anemia can be life threatening. […] No matter the cause, severe anemia may lead to symptoms that include: Shortness of breath, Being very tired, Feeling dizzy, Having pale skin. […] Symptoms may include: Chest pain, Fever, Shortness of breath, Rapid breathing, Cough. […] A stroke occurs when blood flow is blocked to a part of the brain. When this happens, brain cells can be damaged or can die. The symptoms depend upon what part of the brain is affected. Symptoms of stroke may include: Weakness of an arm or leg on one side of the body, Trouble speaking, walking, or understanding, Loss of balance, Severe headache.
  • #7 Sickle Cell Disease Symptoms & Complications | Vaso-Occlusive Events and Pain Crisis
    https://www.sparksicklecellchange.com/what-is-sickle-cell/symptoms-complications
    Increased risk for infections occur when damage to the spleen affects its ability to filter blood. […] Priapism occurs when sickled red blood cells become trapped in the penis causing painful, involuntary erections that can lead to erectile dysfunction over time. […] Dactylitis is a result of blocked blood vessels and causes painful swelling of the hands and feet, especially in young children. […] A catastrophic vaso-occlusive crisis from a severe trigger can lead all organs to fail due to sickling in the whole body. […] The underlying damage caused by acute complications can lead to more serious complications. […] Chronic pain is common among people with sickle cell and generally intensifies with age. […] Anxiety and depression affect about 35% of people living with sickle cell, which is approximately 5 times greater than that of the general population.
  • #8 Sickle cell anemia – Symptoms & causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/symptoms-causes/syc-20355876
    Some people with sickle cell anemia also have chronic pain from bone and joint damage, ulcers, and other causes. […] Swelling of hands and feet. Sickle-shaped red blood cells block blood circulation in the hands and feet, which can cause them to swell. […] Frequent infections. The spleen is important for protecting against infections. Sickle cells can damage the spleen, raising the risk of developing infections. Babies and children with sickle cell anemia commonly receive vaccinations and antibiotics to prevent potentially life-threatening infections, such as pneumonia. […] Delayed growth or puberty. Red blood cells provide the body with the oxygen and nutrients needed for growth. A shortage of healthy red blood cells can slow growth in babies and children and delay puberty in teenagers. […] Vision problems. Tiny blood vessels that supply blood to the eyes can become plugged with sickle cells. This can damage the portion of the eye that processes visual images, called the retina, and lead to vision problems.
  • #9 Symptoms of Sickle Cell Disease (Sickle Cell Anemia)
    https://www.webmd.com/a-to-z-guides/symptoms-of-sickle-cell-disease
    Sickle cell disease changes the shape of the oxygen-carrying molecules (called hemoglobin) found inside red blood cells. Red blood cells are usually round and flexible. This allows them to glide smoothly through your bloodstream. But when you have sickle cell disease, the abnormal hemoglobin creates stiff, sickle-shaped red blood cells. […] These events trigger the symptoms of sickle cell disease. […] Early signs in babies are: Extreme fussiness, Painful swelling of fingers and toes which is the definition of a condition called „dactylitis”, Yellow skin and whites of the eyes (also called jaundice or icterus). This is caused by the breakdown of hemogloblin released during the constant turnover of red blood cells. […] With sickle cell disease, you don’t have enough healthy red blood cells. This is a condition called anemia. Symptoms can include: Trouble breathing, Dizziness and lightheadedness, Fast heart rate, Fatigue, Irritability, Pale skin color, Delayed growth in infants, Delayed puberty in teens.
  • #10 Understanding Sickle Cell Symptoms | Sickle Cell Speaks
    https://www.sicklecellspeaks.com/understanding-sickle-cell/symptoms
    People with sickle cell, especially infants and children, are more likely to get infections. […] When sickled cells get stuck in the blood vessels, it blocks blood flow to the arms and legs. That causes swelling in the hands and feet. […] Sickle cell can affect the blood vessels in the eyes. That can lead to long-term damage, including loss of vision. […] Sickle cell ulcers are sores that usually start small and then get bigger. […] Symptoms include pain and problems with walking and joint movement. […] Because of their anemia, children with sickle cell may grow and develop at a slower pace than other children, and they may take longer to reach puberty.
  • #11 Sickle Cell Disease in Children – Stanford Medicine Children’s Health
    https://www.stanfordchildrens.org/en/topic/default?id=sickle-cell-disease-in-children-90-P02327
    Most children with SCD will start showing symptoms during the first year, often around 5 months. […] Symptoms can include: Anemia. This is the most common symptom. Having fewer red blood cells causes anemia. Anemia can make a child pale and tired. […] Yellowing of the skin, eyes, and mouth (jaundice). This is a common symptom. Sickle cells don’t live as long as normal red blood cells. They die faster than the liver can filter them out. The yellow color is caused by a substance (bilirubin) that is released when the red blood cells die. […] Pain crisis, or sickle crisis. When sickle cells move through small blood vessels, they can get stuck. This blocks blood flow and causes pain. This sudden pain can happen anywhere, but most often occurs in the chest, arms, and legs. […] Acute chest syndrome. This is when sickle cells stick together and block oxygen flow in the tiny vessels in the lungs. This can be deadly.
  • #12 Signs and Symptoms of Sickle Cell Disease | Hematology-Oncology Associates of CNY
    https://www.hoacny.com/signs-and-symptoms-sickle-cell-disease
    Brain imaging and tests of thinking (cognitive studies) have shown that children and adults with hemoglobin SS and hemoglobin S0 thalassemia often have signs of silent brain injury, also called silent stroke. […] Sickle cell disease can injure blood vessels in the eye. The most common site of damage is the retina, where blood vessels can overgrow, get blocked, or bleed. […] People with SCD can have problems with blood vessels in the heart and with heart function. […] In adolescents and adults, injury to blood vessels in the lungs can make it hard for the heart to pump blood through them. This causes the pressure in lung blood vessels to rise. Symptoms may include shortness of breath and fatigue. […] The kidneys are sensitive to the effects of red blood cell sickling. SCD causes the kidneys to have trouble making the urine as concentrated as it should be.
  • #13 Sickle Cell Anemia: Symptoms, Causes & Treatment
    https://my.clevelandclinic.org/health/diseases/4579-sickle-cell-anemia
    Sickle cell anemia can cause serious and sometimes life-threatening complications. For example, people with sickle cell anemia often need emergency medical care or are admitted to the hospital because they experience complications like acute chest syndrome (ACS) or vaso-occlusive crisis (VOC). […] Acute chest syndrome (ACS) is the most common complication of sickle cell anemia. It’s also the most common cause of death and the second most common cause of hospital admission. It happens when sickled cells clump and clog blood vessels in your lungs. Symptoms include: Sudden chest pain. Cough. Fever. Trouble breathing. […] As you get older, you may develop different and more serious medical problems that happen when organ tissues don’t receive enough oxygen. People with sickle cell anemia are at increased risk for stroke and lung, kidney, spleen and liver damage. […] Thanks to early diagnosis and treatment to ease complications, people with sickle cell anemia may live into their 50s. Some people with the disease may live much longer.
  • #14 Sickle Cell Disease – Symptoms | NHLBI, NIH
    https://www.nhlbi.nih.gov/health/sickle-cell-disease/symptoms
    Fever. If you have a fever of more than 101.3 degrees Fahrenheit, or 38.5 degrees Celsius, seek attention from a healthcare provider and treatment with antibiotics right away. […] Chest pain, coughing, fever, and shortness of breath. These symptoms may be suggestive of acute chest syndrome requiring treatment in a hospital, where you may receive antibiotics, oxygen therapy, and/or a blood transfusion. […] Sudden weakness, numbness on one side of the body, confusion, or trouble speaking, seeing, or walking. Call 9-1-1 if you’re showing any of these warning signs of a stroke. […] Priapism. Having an erection that lasts for 4 hours or more is an emergency.
  • #15 Signs and Symptoms of Sickle Cell Disease | Hematology-Oncology Associates of CNY
    https://www.hoacny.com/signs-and-symptoms-sickle-cell-disease
    Many adolescents and adults with SCD suffer from chronic pain. This kind of pain has been hard for people to describe, but it is usually different from crisis pain or the pain that results from organ damage. […] People with SCD usually have mild to moderate anemia. At times, however, they can have severe anemia. Severe anemia can be life threatening. […] No matter the cause, severe anemia may lead to symptoms that include: Shortness of breath, Being very tired, Feeling dizzy, Having pale skin. […] Symptoms may include: Chest pain, Fever, Shortness of breath, Rapid breathing, Cough. […] A stroke occurs when blood flow is blocked to a part of the brain. When this happens, brain cells can be damaged or can die. The symptoms depend upon what part of the brain is affected. Symptoms of stroke may include: Weakness of an arm or leg on one side of the body, Trouble speaking, walking, or understanding, Loss of balance, Severe headache.
  • #16 Sickle Cell Disease in Children – Stanford Medicine Children’s Health
    https://www.stanfordchildrens.org/en/topic/default?id=sickle-cell-disease-in-children-90-P02327
    Splenic sequestration (pooling). The spleen becomes enlarged and painful when sickle cells get stuck and build up there. Fewer red blood cells are able to move. This can cause a sudden drop in hemoglobin. It can be deadly if not treated at once. […] The symptoms of SCD may look like other disorders or health problems. Always see your child’s healthcare provider for a diagnosis. […] Most children will start to have symptoms during the first year of life, often around 5 months.
  • #17 Sickle Cell Disease – How Sickle Cell Disease May Affect Your Health | NHLBI, NIH
    https://www.nhlbi.nih.gov/health/sickle-cell-disease/health-effects
    People who have sickle cell disease usually also have mild to moderate anemia. At times, they can have severe anemia. Severe anemia can be life-threatening. […] Aplastic crisis: An aplastic crisis is usually caused by a parvovirus B19 infection, also called fifth disease or slapped cheek syndrome. […] Splenic sequestration crises: When red blood cells get trapped in the spleen, fewer are available to circulate in the blood. This can lead to severe anemia that causes a pain crisis.
  • #18 Signs and Symptoms of Sickle Cell Disease | Hematology-Oncology Associates of CNY
    https://www.hoacny.com/signs-and-symptoms-sickle-cell-disease
    Males with SCD can have unwanted, sometimes prolonged, painful erections. This condition is called priapism. […] When red cells hemolyze, they release hemoglobin. Hemoglobin gets broken down into a substance called bilirubin. […] There are a number of ways in which the liver may be injured in SCD. […] Sickle cell ulcers are sores that usually start small and then get larger and larger. […] Sickling in the bones of the hip and, less commonly, the shoulder joints, knees, and ankles, can decrease oxygen flow and result in severe damage. […] Children with SCD may grow and develop more slowly than their peers because of anemia. […] Pregnancies in women with SCD can be risky for both the mother and the baby. […] As in other chronic diseases, people with SCD may feel sad and frustrated at times.
  • #19 Sickle Cell Disease – How Sickle Cell Disease May Affect Your Health | NHLBI, NIH
    https://www.nhlbi.nih.gov/health/sickle-cell-disease/health-effects
    The sickled cells that give the disease its name can lead to pain throughout the body and serious damage to organs such as the heart and kidneys. […] Pain crisis: This is also known as a sickle cell or vaso-occlusive crisis and can happen without warning when sickled red blood cells block blood flow. […] Chronic (long-term) pain: Chronic pain is common and is defined as any pain lasting longer than 3 months. […] Delayed growth and puberty: Because of anemia, children who have sickle cell disease may grow and develop more slowly than their peers. Full sexual maturity may be delayed. […] Infections: The spleen is important for protection against certain types of infections. With sickle cell disease, a damaged spleen raises the risk of certain bacterial infections and additional vaccines are recommended as protections from these infections.
  • #20 Disease progression in sickle cell disease
    https://ourironwill.com/progression/scd
    Sickle cell disease can cause progressive organ damage. […] Sickle cell disease is a progressive disease that can result in multiple organ damage over time starting in childhood. […] Younger people are at risk of progressive organ damage by the time they reach their early adult years. […] Within the first 10 years of shifting to adult care, there is more rapid worsening in organ damage with a death rate of up to 20%. […] 50% of people have irreversible organ damage affecting at least one organ by the age of 50. […] Iron overload from multiple blood transfusions can add additional stress and damage to the vital organs. The goal for people living with sickle cell disease is to get and stay ahead of progressive organ damage.
  • #21 Sickle Cell Anemia: A Parent’s Guide for the School Age Child | Texas DSHS
    https://www.dshs.texas.gov/newborn-screening-program/sickle-cell-disease/more-about-sickle-cell/parents-guide
    Children with sickle cell anemia are well most of the time, but certain complications can occur which are very serious and sometimes fatal (cause death). It may be hard to believe that a healthy looking child has a life threatening disease, but this is true when a child has sickle cell anemia. […] Anemia exists when the number of red blood cells in the circulation is below normal. Sometimes anemia is referred to as a low blood count. Anemia is common in all individuals with sickle cell disease. Even though a child’s usual blood count is quite low, this causes few if any real problems. The blood count in a child with sickle cell anemia can drop suddenly. When a child who is already anemic has a drop in the blood count, this is potentially serious. The symptoms of a very low blood count are headache, irritability, unusual sleepiness, lethargy, rapid heartbeat, and pale color.
  • #22 Sickle Cell Disease (SCD) Clinical Presentation: History, Physical Examination
    https://emedicine.medscape.com/article/205926-clinical
    Aplastic crisis is a serious complication caused by infection with parvovirus B19 (B19V). […] Splenic sequestration occurs with highest frequency during the first 5 years of life in children with HbSS, but can occur at any age in individuals with other sickle syndromes. […] As HbS replaces HbF in the early months of life, problems associated with sickling and RBC membrane damage begin. The resulting rigid cells progressively obstruct and damage the spleen, which leads to functional asplenia. This, along with other abnormalities, results in extreme susceptibility to infection. […] During childhood and adolescence, SCD is associated with growth retardation, delayed sexual maturation, and being underweight. […] Hand-foot syndrome, or aseptic dactylitis, is a common presentation in children younger than 5 years. This condition is caused by infarction of bone marrow and cortical bone in the metacarpals, metatarsals, and proximal phalanges. Hand-foot syndrome is usually one of the earliest clinical manifestations of SCD.
  • #23 Sickle Cell Disease (Anemia): Symptoms, Treatment, and Life Expectancy
    https://www.medicinenet.com/sickle_cell/article.htm
    Some features of sickle cell anemia that can occur at any age include: Fatigue, Anemia, Pain crises, Bone infarcts. […] Infants with sickle cell anemia do not develop symptoms in the first few months of life because the hemoglobin produced by the developing fetus (fetal hemoglobin) protects the red blood cells from sickling. This fetal hemoglobin is absent in the red blood cells that are produced after birth so that by 5 months of age, the sickling of the red blood cells is prominent and symptoms begin. […] Infants and younger children can suffer signs and symptoms such as: Fever, Abdominal pain, Pneumococcal bacterial infections, Painful swellings of the hands and feet (dactylitis), Splenic sequestration. […] Adolescents (preteens and teens) and young adults more commonly develop the following symptoms: Leg ulcers, Aseptic necrosis, Eye damage.
  • #24 Sickle Cell Disease (Anemia): Symptoms, Treatment, and Life Expectancy
    https://www.medicinenet.com/sickle_cell/article.htm
    Symptoms in adults typically are intermittent pain episodes due to injury of bone, muscle, or internal organs. […] The life expectancy of persons with sickle cell anemia is reduced. Some patients, however, can remain without symptoms for years, while others do not survive infancy or early childhood. Nevertheless, with optimal management patients can now survive beyond the fourth decade. […] Most patients suffer intermittent pain crises, fatigue, bacterial infections, and progressive tissue and organ damage. Impaired growth and development are the result of the physical and emotional trauma that is endured by children with sickle cell anemia. […] Causes of death include bacterial infection (the most common cause), stroke or bleeding into the brain, and kidney, heart, or liver failure. The risk of bacterial infections does diminish after three years of age. Nevertheless, bacterial infections are the most common cause of death at any age. Therefore, any signs of infection in a person with sickle cell anemia must be reviewed with a doctor to prevent damage and save lives.
  • #25 Sickle Cell Disease – Symptoms | NHLBI, NIH
    https://www.nhlbi.nih.gov/health/sickle-cell-disease/symptoms
    Know when to seek emergency medical care. Sickle cell disease can lead to life-threatening health problems. If you think that you or someone else is having a serious symptom, seek medical care or call 9-1-1 right away. […] Symptoms of sickle cell disease vary from person to person and can change over time. How the disease affects your body affects the type of symptoms you have. […] Early symptoms include a yellowish color of the skin (jaundice) or whites of the eyes (icterus) that occurs when many red cells undergo hemolysis, extreme tiredness or fussiness from anemia, and painful swelling of the hands and feet, known as dactylitis. […] Serious symptoms of sickle cell disease are emergencies and need treatment right away. Seek care or call 9-1-1 if you or someone else is experiencing severe pain, fatigue (extreme tiredness), shortness of breath, dizziness, and irregular heartbeat.
  • #26 Sickle cell anemia – Diagnosis & treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/diagnosis-treatment/drc-20355882
    A blood test can check for the form of hemoglobin that underlies sickle cell anemia. […] Management of sickle cell anemia is usually aimed at avoiding pain episodes, relieving symptoms and preventing complications. […] Hydroxyurea reduces the frequency of pain crises and might reduce the need for blood transfusions and hospital stays. […] Children with sickle cell anemia might receive penicillin from about 2 months old to 5 years old, or longer. This medicine can help prevent infections, such as pneumonia, which can be life-threatening to children with sickle cell anemia. […] A stem cell transplant can cure sickle cell anemia. […] People who are successfully treated with gene editing therapy no longer have symptoms of sickle cell disease.
  • #27 Sickle Cell Disease: Signs and Symptoms
    https://www.health.com/sickle-cell-disease-symptoms-7970516
    The average life expectancy of a person with sickle cell disease is 52.6 years. People assigned male at birth have a life expectancy of 49.3 years, and those assigned female at birth have a life expectancy of 55 years. […] Yes, symptoms of sickle cell disease may worsen with age. You may experience additional complications such as pulmonary hypertension (high blood pressure in the lungs), leg sores, gallstones, and kidney damage. […] Sickle cell disease can damage all major organs of the body including the heart, lungs, kidney, spleen, liver, and brain.
  • #28 Symptoms and Signs of Sickle Cell Disease: Treatment
    https://www.emedicinehealth.com/sickle_cell_crisis/symptom.htm
    People who have sickle cell disease have a reduced life expectancy. Adults with the most severe forms of sickle cell disease have a life span that is 20 to 30 years shorter than people who don’t have the condition. The national median life expectancy of people who have sickle cell disease is 42 to 47 years. A report from Blood, the Journal of the American Society of Hematology, showed that some people with mildly symptomatic sickle cell disease may live longer lives with proper management of the disease, but more study is needed.
  • #29 Sickle Cell Anemia Symptoms: What You Need to Know
    https://www.bmhsc.org/blog/sickle-cell-anemia-symptoms-what-you-need-to-know
    Sickle cell anemia symptoms often appear during infancy. […] Most babies show symptoms at some point during the first year of life, usually around 5 or 6 months of age, Gault says. […] Some of the most common symptoms children with sickle cell disease have include jaundice, fatigue and swollen hands and feet. […] Anemia may cause someone to feel tired, cold, dizzy and/or irritable. […] Pain is another common symptom of sickle cell disease sickle cells stick to blood vessel walls, slowing or stopping blood flow and oxygen to the organs and tissues. […] We call this a pain crisis, and its serious, Gault says. A pain crisis can happen anywhere on the body and last hours or even days. […] Other complications of sickle cell disease include acute chest syndrome, chronic pain, eye problems, heart problems, splenic sequestration crisis, and stroke. […] One of the best things patients with sickle cell disease can do is maintain a relationship with their primary care provider, Gault says. Keeping in touch will help manage sickle cell anemia symptoms and prevent problems later in life.