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Acute Chest Syndrome and Sickle Cell

We all have heard about the dreaded Acute Chest Syndrome (ACS). Some of us have had it, and some sickle cell warriors have died from it. But what exactly is acute chest syndrome?

What is ACS? Acute chest syndrome (ACS) is a pulmonary illness defined by a new infiltrate on chest radiograph in combination with at least 1 clinical sign or symptom (chest pain, cough, wheezing, fast respiration rate, fever). Fever and cough are the most common presenting symptoms in children, while chest pain, shortness of breath, and chills are common in adults.

Many people state that it feels like you can’t get any air, as if you are suffocating, and struggling for every precious breath. It feels to some like an elephant sitting on your chest, and the pressure is compressing your lungs and making breathing an impossibility. Of all the complications, having your breath stolen from you, having every second a struggle for oxygen, this is by far the scariest, most panicky situation to ever be in.

Recurrent episodes are thought to contribute to chronic lung disease, pulmonary hypertension, and cor pulmonale.

Physiology: During a sickle cell crises, the sickled cells have problems passing through the tiny vasculature of the lung tissue. This causes a blockage which leads to injury which then starts the inflammation, hypoxia, and lung injury cycle. This cycle continues on and on similar to what goes on in Acute Respiratory Distress Syndrome until large parts of the lung is injured and unable to process oxygen. Eventually if this process continues, the patient will be completely unable to breathe on their own and may even need to be intubated and mechanically ventilated (which is what happened to me).

What causes Acute Chest Syndrome? ACS is associated with all genotypes of sickle cell but occurs most frequently in patients with homozygous (SS) disease. The main causes of acute chest syndrome are pulmonary infections (like pneumonia, bronchitis, RSV, CMV, flu etc.), emboli (like fat clots or blood clots), and rib infarctions which is caused by splinting or rib breakage during a vaso-occlusive crises.

The other possible causes is what is called iatrogenic. Excessive narcotic use and excessive hydration have been proposed as 2 possible iatrogenic causes of ACS; however, both have limited supporting evidence. It has been suggested that atelectasis secondary to hypoventilation and poor respiratory effort with narcotic use might lead to ACS. In addition, it has been suggested that patients admitted for sickle cell pain crisis may develop pulmonary edema and subsequently ACS if hydration strategies are too aggressive. This is why alot of physicians err on the side of caution when prescribing fluids and narcotics to sickle cell patients.

How do you treat Acute Chest Syndrome? Treatment involves supportive care, incentive spirometry, broad-spectrum antibiotics for the infection, bronchodilators (to expand the airway and improve oxygenation), and early blood transfusion when indicated.

In short: Acute Chest Syndrome is a medical emergency, a serious complication that can be life-threatening. 4.6% of patients with ACS die from respiratory complications, and ACS can increase your length of stay by 5 days. Please, please my dear warriors! Prevent this from happening to you as much as possible. Use your incentive spirometer, because this helps to improve oxygenation, improve blood flow to lung tissue and expand the alveoli. The incentive spirometer has to be your best friend when you are admitted, regardless of whether you are in pain or not. The recommendation is 10 breaths every hour when awake, between the hours of 8am to 10pm. I have a habit that whenever I press my PCA button, I use the incentive spirometer.

If you are having any problems breathing during your sickle cell crises, be sure to mention the possibility of acute chest syndrome to your doctor, nurse or advocate to bring it to their attention. Early identification and aggressive treatment has given better outcomes and most people with ACS recover fully.

Educate yourself and be well.

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Sickle Cell Warrior

Sickle Cell Warrior

Tosin is dedicated to perpetuating healthy and positive messages about sickle cell. Although sickle cell is a subject often taboo in the communities this condition is most prevalent, Tosin’s message is that sickle cell is not something to be ashamed of and you can live a rich and fulfilling life with sickle cell. Sickle cell warriors are the most amazing people in the world, with a great fortitude for compassion, willpower and strength.

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12 Comments

  1. leslie
    leslie July 22, 12:37

    Help. My boyfriend was hospitalized with pneumonia- they said 3 days ago they are treating for both pneumonia and acs..but they kept saying it got worse. the first day he was in the emergency room intebated so they could give him enough meds for the crises. not today they say he has fluid in his lung..it has been 5 days since hes been in there. Like hes still breathing with the respirator- yesterday 50%..

    Im just really worried. Any helpful info you give would help trememndously.

  2. Sickle Cell Warrior
    Sickle Cell Warrior Author July 26, 07:38

    @Leslie, I’m sorry to hear that your man has been so sick. Just hang in there, be supportive and be there for him. He is already on the mend and will get off the ventilator

  3. Latonya Chambers
    Latonya Chambers August 05, 10:31

    my daughter, Kennedy just got back from Vanderbilt Children’s Hospital from having chest pains & trouble breathing, she’s better now but this summer has been tough

  4. Sickle Cell Warrior
    Sickle Cell Warrior Author August 08, 02:27

    @Latonya, I’m glad to hear that your daughter is better.

  5. earlyce
    earlyce September 02, 03:45

    hi my son nelson has been having chest pain for the last 3 days how can help him?

  6. T.J. Sickle cell Natural Welllness
    T.J. Sickle cell Natural Welllness September 19, 23:23

    Chest complications, And pain crises can also occur, when exposed to cigarettes… May we all stay away from cigarettes, even if its second hand smoke..

  7. Renee
    Renee September 20, 13:48

    I was just recently discharged from the hospital last Thursday with pneumonia. I was a bit confused about the incident because I usually experience the problem after travelling from 1 extreme temp to another. This time I did not do that so I took notes when ever the docs would tell me something. Apparently, as sickle cell warriors we get ischemia in the lung which then makes it easy for us to catch pneumonia. They said there is nothing that they could do. However, a nurse practitioner informed me that warriors should be getting a PFT and Echocardiogram every year.
    At this time I don’t know what else I should be doing because I take the necessary precautions, I have antiseptic wipes and sanitizers wherever I go, I stay away from ppl who are coughing, etc. Now, I will start with the PFTs and the Echos. The docs and I many not know what to do at this point but I trust God. He allowed this disease and if He wants he can take it away. God knows, so I’m not worried. I’m just going to keep doing what I have been doing.

  8. Sickle Cell Warrior
    Sickle Cell Warrior Author October 11, 22:48

    @Renee, the NP is right. We have to do an echocardiogram every year to catch if we have pulmonary hypertension early. There are many respiratory conditions due to the damage of Acute chest, and I do believe this is why we are more susceptible to pneumonia. Have you gotten your Pneumovax?

  9. Victoria
    Victoria January 20, 03:54

    my son has been battling with pneumonia since november last year, he’s taken several antibiotics and been admitted to 3 different hospitals in abuja, Nigeria but he keeps on having relapses after about two weeks of feeling better. I am so confused and I don’t know what to do. Please help.

  10. YARKEYA
    YARKEYA July 07, 21:57

    I HAD ACS IN 2008, I WENT IN FOR LEG PAIN AND 2 DAYS LATER I COULDNT TALK OR LIE DOWN FLAT BECUASE IF I DID I COULD NOT BREATH AT ALL I WAS GASPING FOR AIR.. I HAD NO SYPTOMS PRIOR TO THE SHORTNESS OF BREATH NO CHEST PAIN OR ANYTHING, LUCKLY I HAD A BLACK HEMATOLOGIST THAT ONLY DEAL WITH SICKLE CELL ANEMIA, I HAD TO GET A PIECE OF MY RIGHT LUNG REMOVED FROM THE DAMAGE AND FLUID AND A CHEST TUBE PUT IN, THE PAIN SUCKED SOOO BAD AFTER MY SURGERY I HAD TO GET A EPIDURAL TO NUMB ME WHICH WAS GRRRREAT. IN THAT SAME HOSPITAL STAY I HAD TO GET A LIVER BIOPSY DONE WHICH THEY DONT PUT U TO SLEEP FOR THEY GIVE U MEDS TO WHERE UR NOT SUPPOSE TO FEEL IT BUT I FELT EVERYTHING EVEN THE CUTTING SO THEY STOPPED AND I WAS PUT TO SLEEP, I ALSO HAD TO GET A A-PHERISIS WHERE THEY DRAIN ALL YOUR BLOOD AND GIVE YOU ALL NEW BLOOD,THAT WAS MY LONGEST AND SCARRIEST HOSPITAL STAY AT 34 DAYS. BUT gOD HELD MY HAND THRU THE WHOLE ORDEAL I I MADE IT THRU OKAY,THANK THE LORD!!! SO WHEN I GET CHEST PAIN SCREW THE HOME MEDS I HEAD STRAIGHT FOR THE ER…BETTER SAFE THAN SORRY!!!

  11. jesu
    jesu April 06, 01:49

    My daughter also started a pain to her chest,pain killers takes the pain away for couple of hours and it’s back again.
    she’s breathing fine, but I just can’t stand the pain she’s feeling so am taking to the hospital .

  12. Sickle Cell Warrior
    Sickle Cell Warrior Author April 17, 21:02

    @Jesu, if your daughter EVER complains of pain in the head or chest, or any breathing problems, please take her to the hospital ASAP. This is a medical emergency and can lead to death. I’m so glad you took her in. I hope she is doing better now.

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