X-Post - Sickle Cell Disease and Malabsorption
I just kind of assumed that all WLS plans had been derailed, but she told me today that she was still thinking about it.
My limited insight is telling me that a malabsorptive surgery would be a very bad idea for her, but what do I know? I hope very much that her docs are on top of the situation but I also know better than to place any kind of blind trust in doctors. So I'm just curious. Anybody ever hear of anyone with sickle cell disease being approved for any WLS?
Anna, I'm about to leave my office and I don't have Internet access at home, but I will be in touch with you tomorrow. 
Anna, your friend needs to have a discussion with her doctors about pain management and learn as much as she possibly can before she proceeds with WLS.
I’m not sure how much you know about sickle cell, so please forgive me if you’ve heard this before. As you know, red blood cells carry oxygen to the different parts of the body. People with sickle cell disease have abnormal hemoglobin that can distort the structure and function of their red blood cells. The sickled blood cells are rigid, fragile and sticky. They don’t flow smoothly like normal blood cells. They have a tendency to break and clog up the blood vessels at the capillary level. The tissues on the other side of the blockage starve for oxygen. This results in pain and sometimes tissue damage. There's a long list of possible complications.
The number one reason for sickle cell patients to be admitted to the hospital is severe pain. This is called a sickle cell crisis. The frequency of pain peaks between ages 19 and 39 years. After the age of 19 years, more frequent pain correlates with a higher mortality rate. Any underlying cause should be searched for and corrected, but the majority of painful episodes have no identifiable cause. It can affect any area of the body, most commonly the back, chest, extremities, and abdomen, may vary from trivial to excruciating, and is usually endured at home without a visit to the emergency department.
FYI there is more than one type of sickle cell disease. The symptoms and severity can vary widely from person to person. Your friend would have to have a special blood test (genotyping) done to tell which variation of the disease she has.
The following information came from a Pain Management conference that I recently attended. I hope it is useful. I wish your friend all the best.
The most recent evidence based data shows effectiveness of various treatments as follows:
Strong support
– Penicillin prophylaxis (children)
– S pneumoniae vaccination
– Hydroxyurea
– Chronic exchange transfusion
– Iron chelation for chronic iron overload
– Stem cell transplant (high risk)
Less impressive
– PCA pump
– Folate supplementation
– H. influenzae vaccination, infuenza vaccination
– Erythropoietin
– Phlebotomy
– Exchange transfusion for Acute Chest Syndrome
– Antiplatelet agents for stroke prevention
Not helpful/harmful
– IV fluids (unless clinically dehydrated)
– Oxygen
Pain Management should be Multi-Modal!
(1) Psychological - include distraction, guided imagery, hypnotherapy, education, and psychotherapy. These therapies are noninvasive; cause few, if any, side effects; and provide patients with hope and a sense of mastery over their pain. Several studies have documented the ability of cognitive therapies to reduce pain in sickle cell crisis and in other chronic pain situations
(2) Behavioral - include relaxation, biofeedback, behavior modification, and deep breathing. These techniques replace anxious, distressed behaviors associated with illness with more adaptive behaviors associated with good health or "wellness." The most famous and commonly used of these techniques is the deep, rhythmic breathing technique used by pregnant women in labor. The most commonly used behavioral strategy in the authors' practice is the "pain-behavior contract." These contracts are increasingly being used as an alternative method of managing the discomfort experienced by adolescents in sickle cell crisis. These contracts help define limits and expectations and provide to patients (and their families) responsibility for adherence to treatment plans. Unfortunately, they are often used only in problem patients but could probably be used with great success in most adolescents and young adults--patients who need to have a sense of control.
(3) Physical - consist of hydration, heat, physical therapy, massage, transcutaneous electric nerve stimulation, and acupuncture.
(4) Pharmacological - Which opioid to use or whether it makes any difference is unclear. At equipotent doses, most opioids produce the same salutary effects and induce the same side effects. In the past, meperidine was the most commonly prescribed opioid for sickle cell crisis pain because of its euphoric effects and the mistaken belief that meperidine produced less biliary spasm and less respiratory depression than morphine, which is untrue. Meperidine should be avoided in patients with sickle cell anemia and perhaps all children because it has a toxic metabolite, normeperidine, which is excreted in the urine and causes seizures if it accumulates in the blood. This is most likely to happen in sickle cell patients in crisis because they require high opioid doses for several days; have impaired renal function; and are often taking NSAIDs, which reduce renal blood flow and filtration rates.
You're welcome. Someone close to me died young from sickle cell complications. He suffered most of his life. Being an advocate for these patients is a passion of mine. A lot of times they go to the hospital and the staff assume they're junkies because the normal dosages of pain medicines just don't work for them. There is a lot of misinformation out there.
If there's a sickle cell center of excellence in the area where your friend lives, she will receive better care through their facility. I would highly advise her to get a Health Care Power of Attorney so that someone she trusts can make decisions for her and be assertive about getting her proper care should she be unable to do so for herself.
Children's hospitals seem to be the most in tune with taking care of sickle cell patients. That might be another good resource for her to check into as well.
I'll pass on your info to my friend. I would imagine that every system in the body is at least indirectly affected by this horrible disease, and because of that, proper nutrition would be crucial. That's why I wonder if a malabsorptive surgery might be a terrible idea. I wish her insurance covered VSG.
Yes
Complications of Sickle Cell Anemia
Anemia: Because the sickle cells are rigid, when they clog the flow in the blood vessels, they break apart. This causes a low blood count, or anemia.
Hand-Foot Syndrome: Swollen hands and feet are often the first signs of sickle cell anemia in babies. The swelling is caused by sickle-shaped red blood cells blocking blood flow out of the hands and feet.
Splenic Crisis: The spleen normally filters out abnormal blood cells and helps fight infection. If it becomes clogged with sickle cells it is unable to perform this function. This also causes a sudden drop in hemoglobin and can be life threatening if not treated promptly.
Infections: Due to damaged spleen. Pneumonia is the number one cause of death in young children with sickle cell anemia.
Acute Lung Syndrome: The sickle cells stick together and block the flow of oxygen in the tiny vessels in the lungs. This is a life-threatening condition similar to pneumonia. Multiple episodes may cause permanent lung damage.
Pulmonary Arterial Hypertension: Damage to the small blood vessels in the lungs makes it hard for the heart to pump blood through the lungs. This causes blood pressure in the lungs to rise. Shortness of breath and problems with breathing are the main symptoms.
Delayed Growth and Puberty in Children: A shortage of red blood cells causes the slow growth rate. Adults who have sickle cell anemia often are slender or smaller in size than other adults.
Stroke: Two forms of stroke can occur in people who have sickle cell anemia. One form occurs when a blood vessel in the brain is blocked. The other form occurs when a blood vessel in the brain bursts.
Eye Problems: Sickle cells also can clog the small blood vessels that deliver oxygen-rich blood to the eyes. This can cause serious problems, including blindness.
Priapism: Males who have sickle cell anemia may have painful and unwanted erections. It happens because the sickle cells block blood flow out of an erect penis. Over time, priapism can damage the penis and lead to impotence.
Gallstones: When red blood cells die, they release their hemoglobin. The body breaks down this protein into a compound called bilirubin. Too much bilirubin in the body can cause stones to form in the gallbladder.
Ulcers on the Legs: Sickle cell ulcers (sores) usually begin as small, raised, crusted sores on the lower third of the leg. The cause of sickle cell ulcers isn't clear. Some heal quickly, but others persist for years or come back after healing.
Multiple Organ Failure: Multiple organ failure is rare, but serious. It happens if the patient has a sickle cell crisis that causes two out of three major organs (lungs, liver, or kidney) to fail.
Jaundice: Sickle cells don’t live as long as normal blood cells. When they break down, bilirubin is released into the bloodstream. The liver, which filters the blood, is overwhelmed by the rapid breakdown of red blood cells. In people with dark skin, jaundice is visible mostly as yellowing of the whites of the eyes.
Bone necrosis: Over time, after being restricted of blood flow, bone tissue dies. This is a serious condition because the dead areas of bone do not function normally, are weakened, and can collapse.
