Sickle Cell Boy died after doctor ignored Instructions

A schoolboy with sickle cell disease died after a junior doctor ignored instructions from his consultant to carry out a blood test, an inquest has heard.
Emmanuel Akinmuyiwa, seven,from Tyseley, collapsed and died during a blood transfusion after staff ‘missed opportunities’ to save his life.The boy suffered a cardiac arrest during the transfusion at Heartlands Hospital, Birmingham. It was carried outon February 11, four days after the youngster was admitted to hospital.But the coroner said that blood tests should have been carried out much earlier. If they were, it would have shown a transfusion was required far sooner – perhaps even on the day he arrived.Instead, there was ‘insufficient understanding’ of the boy’s condition and a ‘lack of urgency’before any action was taken.The schoolboy suffered from sickle cell disease, a hereditarydisorder that can cause red blood cells to mutate and restrict blood flow, Birmingham Coroner’s Court was told.The condition was spotted by a GP who referred him to the hospital.After he was admitted, Dr Helen Goodyear, a consultant paediatrician, set up a five-point treatment plan in which she asked for his haemoglobin, or hb, levels to be monitored.That meant taking blood tests to ensure it was still at a healthy level. If the figure fell below five, it would show a definite need for a transfusion, Dr Goodyear told the court.But her direct instructions were ignored by Heartlands staff, the court heard. The inquest heard how a junior doctor failed to take the tests when covering the weekend shift.It was only when another doctor took over four days laterthat a blood test was taken and a transfusion was finally requested. The doctor found the blood had clotted.After the request, there was still a four-hour wait for the correct blood type to arrive. The hb level was at just 2.7 grams when the transfusion was eventually performed.


The boy with his sister

Dr Goodyear said she was ‘horrified’ when she learned her treatment plan had not been followed through by a junior doctor without consultation. Senior coroner Louise Hunt gave the cause of death as cardiac failure caused by severe anaemia due to sickle cell disease.
Mrs Hunt said: ‘In my conclusion I do find a gross failure in not doing the blood test on the tenth (of February) when there was a clear clinical need and a clear direction to do so. ‘There were other failures – to provide a transfusion on either the eighth, ninth or tenth a failure by all staff to not recognise they had insufficient understanding of this condition and an appreciation of this very serious condition.’ Mrs Hunt ordered for a report to be sent to Clinical Commissioning Groups in the West Midlands and NHS England to ensure all trusts were better placed to treat patients with sickle cell disease. Emmanuel’s father Oluwasemilogo wrote a letter of complaint to Heartlands and spoke of his loss in a written statement read out in court. ‘The whole experience was very traumatic for us,’ he said. ‘He was our youngest child and only son. We had been trying for another child since 1997 and had to wait until 2005. We moved to the UK in 2006 to give our children a better life. ‘It’s impossible for us to have another child now due to my wife’s age. We are still struggling to come to terms with it and have constant recollections of his pain.’ Emmanuel was admitted to hospital complaining of a fever, leg pains and dizziness . his healthy hb ‘base’ level was 8.5 grams per deciliter of whole blood, but it dropped to 5.7 grams shortly after his admission. It was at just 2.7 grams when the transfusion was eventually performed. Dr Sarah Wilson, a consultant haematologist at Birmingham Children’s Hospital, which had been approached for advice on Emmanuel’s treatment, said transfusions should be performed when a patient’s hb count is more than two grams below their base level.


Nigeria: 50,000 children born with SCD annually.

The President of Sickle Cell Club, Mrs. Ayo Otaigbe, has revealed that about 50,000 Nigerian children are born annually with symptomatic Sickle Cell Anaemia, just as several of them do not survive beyond infancy.Otaigbe, who said this at the 2015 annual celebration of the Sickle Cell Club Lagos Nigeria, explained that the high death rate from the disease was due to lack of information on how toprevent or tackle it, as well as inappropriate care of patients. Adding, she said the disease was still not understood fully, which makes it unresponsive tosome drugs being marketed in the country.“More research is needed to find a rational therapy for managing the crisis, beyond theuse of analgesics, as sufferers still go through severe pain crisis. More importantly, we will continue to show and advocate that affected persons can live a normal and healthy life if they are well managed, thereby dispelling the myths, rumours and misinformation surrounding the condition,” she said.On the issue of stroke in sickle cell children known to impede mobility, she called on families to benefit from the Transcranial Doppler (TCD) ultrasound scan procedure which holds at the centre during week days.“Although Sickle Cell Disease is not only an Africa condition, over 80 per cent of affected children are born on the continent, yet Africa had shownindifference in addressing the issue,” adding that Nigerians should make a difference and promote funding that will makemeaningful research into the disease a reality.She said that the club had, through its activities, continuedto increase public awareness and knowledge of the disorder especially through the establishment of sickle cell clubs, partnering with the SCFN and other stakeholders. According to her, the focus for 2016 would be to consolidate thesuccesses recorded in 2015 and increase the level of awareness.

We are not Sickles, we are Warriors!!!

We are not Sicklers, we are Warrior. This was the Last Writeup from a Late Sickle cell Patient . Hammed Ajiboye

Sickle cell anemia is a terrible condition to afflict anybody. Its most obvious symptoms are yellowing of the eyes (jaundice) and pallor (pale skin) and painful episodes called Crises. It compromises the immune system and makes the sufferer susceptible to infections. However as terribleas the condition is it is not a death sentence and neither is itan excuse for the sufferer to engage in unproductive lifestyles or wallow in pity or self -contempt. With proper care and attention, sicklers can go on to live productive lives.


As a kid, I did not engage in physically exhausting activities like playing football which I longed for but which I knew would be too demanding. Fortunately I turned to books rather than to despair, I read hundreds of books. In the pages of those books, I discovered another life. Discovered that nothing was too impossible for me to do, and that like other people i had to work hard to get the things I constantly dream of. At other times though, I felt lonely and angry, angry at the all-powerful who could have prevented the constant pain I lived with just a word, angry at my parents for their mistake which brought untold pain on me, angry at myself for no justifiable reason. We sicklers feel a potpourri of emotions, joyful and happy now, distraught at the next minute. Mood swings are our constant partners. We long for the things others take for granted. Being able to go to bed without the fear of sudden headaches, back spasms or priapic pain. We don’t want to wake up in the morning and check our mucous membranes for signs of pallor. We have to pray fervently that we don’t develop ulcers stroke or heart failure. We want to be able to go for months without taking the annoying pain meds. We want to be sure that when we have headaches, it’s not the beginning of occlusive crises. We don’t want to meet new people and the first question they ask is ‘Are you a sickler?’ We are tired of prophylactics, laxatives, diuretics,and antibiotics. We despise the doctors pin prick to check Pcv, sick of the transfusions, weary of going of going to Clinic. We are weary of the negative body image, yellow eyes, tall,gaunt and lean like an ectomorph, with pale skin. The things others take for granted are what we long for. Talk about paradox. Sickle cell disease places limitations on what the sufferers can do and may at times dictate the life trajectory of the sufferer. The sufferer at times feels that he’s been sentenced to a lifetime of Folic Acid and Vitamin B- Complex and frequent visits to the hospital for this check and that check. Add in parents justifiable and at times unjustifiable paranoia over their children with constant reminders that you can’t do what your mates are doing even when you feel as fit as a fiddle. At such times one may feel let out of the fun and if left unchecked, the sufferer may turn into a broody, moody loner,keeping mostly to himself and isolating himself from the world around him. He may have feelings of rejection, pathos and despair about his medical state. But let the sickler out there beware, wallowing in contempt and self-pity, playing the blame game and complaining are negative emotions that wear out even the best of humans. There is therefore no excuse to add to your woes by engaging in these self-destructive habits. You can do anything you set your mind to do. Yet amidst all the trials and tribulations of living with sickle cell, we with the condition learn a vital lesson that those with ‘normal health’ learn too late. That for one to live productive lives, one has to learn to strike a balance in all things and that without sound health all other pursuits are a striving after the wind. Despite all the injections, transfusions and drugs we take on a constant basis, we appreciate each day and try not to endanger ourselves and our health. We realize that if one of us sneezes our entire families catch a cold. Yes the limitations of sickle cell may at times be overwhelming; our lives are an inspiration to others. These days, we do not see nor call ourselves sicklers, we call ourselves warriors and that is what we are, after battling and living with unimaginable pain, nothing fazes us anymore, and in our weakness, we find a new strength, a new will, a new resolve to go on and do exploits and so we find our strength in weakness . our lives are a testimony to the latest power of the human spirit to overcome great adversity and so like Victor Frankl, we overcome harsh conditions and endure the infantine taunts of those who tease us. Everyday we are hopeful, we believe in and enjoy life a little bit better. That which our friends take for granted, we are grateful for. That which people palpitate over is our normal routine. And so we push a little harder, love a little bit more and empathize better . we are not sicklers, we are warriors and we have overcome, we have fought the battle and we have won. If we have won the battle, then you can too. If a skinny kid who vacillates between hospital and home, pops pills daily, and has live with pain all his life can make something out of his life, then you my dear reader have no excuse whatsoever to whine or complain. If we have overcome, you too can and you will.

Role of Science towards cure for Sickle Cell

Stroke is one of the most devastating complications of this sickle cell disease; it occurs in 5 to 10% of children affected. Since the early 1990s, ultrasound to measure blood flow in arteries of the brain (transcranial Doppler) has been standard practice in management of young people with SCD; those children with reduced blood flow have, until now, been advised to receive regular transfusions of non-sickling blood, to reduce their risk of stroke. However, a treatment regimen of chronic transfusions carries risk: besides unknown infection in the blood supply (deemed low) or bacterial contamination in blood (unusual), there’s a greater probability of developing iron overload , over time. Iron overload causes problems in the heart, liver, endocrine system and other organs.
Patients entering the TWiTCH trial was stopped early, in 2014, based on clear results for hydroxyurea being at least as good as transfusion in maintaining blood flow to the brain, as measured by transcranial Doppler. (This was the primary endpoint of the trial, which enrolled over 120 children between the ages of 4 and 15 years, all with severe SCD.) Ware said there was no significant difference in the incidence of strokes between the two treatment arms, and no significant problems observed from hydroxyurea on the study, which included follow-up through two years.
“This truly is one of the abstracts that can be defined as practice-changing,” Thompson said. She’s a pediatric hematologist and professor at
Northwestern University ’s Lurie Children’s Hospital of Chicago. and was
recently elected to an ASH leadership position. She fielded several questions on safety of this treatment at the press briefing.
“These are sick kids,” Thompson considered. Sickle cell disease causes strokes, life-threatening infections, pain, organ failure and early death. “There’s a growing body of evidence that this is a safe and effective drug,” she said. “Hydroxyurea is a helpful drug for many people who have sickle cell anemia,” she said. “But it requires careful discussion with patients,” she emphasized.
“We always tell patients, or families of children, that this drug was developed for cancer and is a form of chemotherapy,” Thompson said in a later interview. “We also share with them the mounting evidence of benefit to using hydroxyurea in ameliorating or preventing sickle cell complications without any increased risk of cancer. We have to be clear about it,” she said. “If there’s any perception of deception, that can cause problems.”
“There are concerns about fertility with hydroxyurea, especially in men,” Thomson said. “Also, although we always warn girls and women not to get pregnant while on this drug, it does happen,” she added “Sometimes, it’s not on the woman’s side, but by a man who’s on the drug. In theory this could cause problems, but so far we haven’t seen birth defects.”
“Hydroxyurea (HU) has been used in clinical trials for children with sickle cell disease who are as young as 9 months and there are no unique safety concerns from those studies,” Thompson wrote in a subsequent email. The
NIH guidelines for treating sickle cell disease strongly support broader use of HU including in children. “It is likely that the label indication will include children soon,” she added.
In a second presentation at the press meeting, Dr. Barbara Cappelli reviewed results from a large international registry of 1,000 HLA-identical sibling transplants performed for patients severe SCD. The perfectly-matched allogeneic transplants were carried out between 1986 and 2013, at 88 medical centers in 23 countries, Cappelli indicated.
According to the abstract (#541), which reads (as did others) slightly differently from the presentation, the median age at transplant was 9 years. Approximately half of the transplant recipients were female, and just over half of the procedures were performed since 2007. The reasons for considering transplantation (and entering the trial) included stroke, an SCD complication called acute chest syndrome, and having painful crises requiring hospitalization. At three years after transplant, overall survival surpassed 94%, Cappelli said.
Engraftment rates were “excellent,” Cappelli said; in only 70 of 1,000 patients (7%) did the transplants not take hold of the patient’s marrow. Younger patients receiving transplants fared better than older, she indicated. With hundreds of transplanted patients out now more than 10 years (in some cases, over 20 years) after transplant, some patients might be considered cured of SCD. (Although Cappelli was reluctant to use the term “cure,” others did.)
The possibility of treating SCD by matched bone marrow transplantation is limited by several factors, Cappelli outlined. Only one in ten SCD patients has an identical sibling match, she noted; the option for transplant with such a high probability of a favorable outcome doesn’t apply to most SCD patients, who lack a perfect sibling donor. Second, many patients in developing countries lack access to healthcare facilities where bone marrow transplantation, a fairly standard procedure in modern medical centers, can be safely accomplished.
The drug works by enhancing hemoglobin’s affinity for oxygen, said Dr. Eleanor Ramos in a phone interview. She’s the chief medical officer of Global Blood Therapeutics, the study’s sponsor and product manufacturer. “We’ve observed that erythropoietin levels declined in patients who received the drug. Nothing is absolute, but this decrease in EPO strongly suggests that oxygen is being effectively delivered to tissues,” Ramos said. “This explains the observed changes in the red blood cells.” If this agent diminishes sickling and clogging of the small blood vessels, as happens in sickle cell disease, there should be less organ damage, she said.
The GBT440 study was designed to test various doses of the drug in healthy volunteers and adult patients with SCD. “So far, we have enrolled 22 adults who have sickle cell anemia in the trial,” she said. The reported findings include those 22 patients who’ve taken the drug at a set, tolerated dose for 28 days, along with 8 healthy research volunteers. (The study design calls for a 6:2 skewing in this phase.) The trial is now enrolling research subjects who will take GBT440 for as long as 90 days, Ramos said.
The protocol stipulates that everyone on the trial be monitored in a clinical research unit for the first four days of treatment. This enables researchers to watch for any potential untoward effects. It also permits close monitoring of how GBT440 is absorbed at each dose, when peak levels occur, and other aspects of its pharmacology. After the initial four days, patients can leave the unit and come back for periodic checks.
“The most common complaint we’ve observed is headache during the first week of the study, and those appear to resolve,” Ramos said. The headaches, considered grade 1 or 2 adverse events, have been mild or moderate, and have been reported by subjects receiving both the active drug and the placebo, she noted. Investigators report a total of four serious adverse events (SAEs) among the 30 subjects in this phase of the study. Three of those four were due to sickle cell crisis (a painful manifestation of SCD), and all occurred after discontinuation of the study drug (GBT440 or placebo). The other (fourth) SAE was an infection causing hemolysis; the patient was admitted to the hospital because he needed antibiotics, Ramos said. While it can’t be ascertained that the drug did not somehow contribute to that patient’s infection, patients with sickle cell disease are at increased risk; most lack functional spleens and are vulnerable to pneumonia and other infections.
“This drug is unique in that it’s a disease-modifying agent, that’s unrivaled in its ease of administration,” Ramos said. “It’s a single, daily dose oral agent,” she said. “It appears to be well tolerated.” The potential for relieving pain, and organ damage from low oxygen, is tremendous, she offered.
The ongoing clinical trial of GBT440 aims to enroll 128 patients. Results from the phase 1-2 trial will be available in 2016, Ramos said.
Finally, the Bluebird gene therapy abstract published by ASH (abstract #202) contains few details, and refers also to patients with another type of hemoglobin disorder, β-thalassemia (major), who received similar treatment. As reported at the meeting in Orlando, and consistent with information provided on the company’s website (“HGB-205 Study in beta-thalassemia major and severe sickle cell disease” and “HGB-206 Study in severe sickle cell disease”), as of mid-November 2015, a total of four SCD patients have received treatment with the lentiviral vector LentiGlobin BB305.

Never Give These 5 Foods To A Person Having Anemia

Anemia is not a very dangerous condition for most people, and it’s very common. But the symptoms can include serious fatigue, inability to concentrate, depression and a feeling that you just can’t handle any of your normal activities. In some really mild cases (for example, many women who just get anemic around their periods), doctors advise patients to simply eat some beef or other iron-rich foods whenever they feel anemic (beef is rich in iron and B12, which helps your body use iron) and/or take iron supplements. See a doctor* if you’re having these symptoms.

Note: This article is intended for people who have been diagnosed by a qualified health care professional, advised to treat themselves and advised on how to treat themselves. This article is not a substitute for medical advice.

Foods to avoid if you have anemia

While most people diagnosed with anemia are aware of many foods that can bring their iron levels back up to normal, it’s less well-known that some foods actually interfere with iron absorption. If you eat these foods alongside iron-boosting foods, you may end up doing yourself little good. If you have a history of anemia, knowing about these foods can help you prevent or lessen your bouts of it.
Do you need to give these foods up? Not necessarily, and certainly not entirely. What you need to do is: avoid having these foods in your stomach while you’re also digesting iron-rich foods or iron supplements. For example: don’t drink red wine within an hour to either side of the steak dinner you’re eating in hopes of boosting your iron levels. Additionally, you can counteract the iron-blocking effects of these foods by having some Vitamin C along with them.

Red wine.
The reservatol that helps fight heart disease and cancer seems to inhibit iron absorption. But white wine seems to help with iron absorption, so that’s an option.

Coffee can also keep you from absorbing iron into your system. Avoid it within an hour either way of an iron supplement or iron rich meal.

Black and green teas chelate with iron from plant sources, making it indigestible. Iron supplements are typically from plant sources. Tea doesn’t have this effect with meat iron {PDF link} sources, however. And in populations that drink a lot of tea, there’s no more anemia than in other populations, so there’s definitely still some research to be done in this area.

Soy proteins.
While one might argue that soy is rich in iron, non-fermented soy is also rich in phytic acid, which interferes with absorption of iron and other nutrients. Interestingly, fermented soy products (like soy sauce) can increase your ability to absorb iron. (Some sources actually recommend tofu for combating anemia because the phylic acid doesn’t completely block iron absorption, so you still get some iron from the soy. If you think tofu is your best available source for iron, be sure to eat it with plenty of vitamin C for best effect.)

You’ve probably heard that spinach is rich in iron and therefore great for anemia. It is rich in iron, but it’s never helped me with anemia at all. Turns out it has something called oxalic acid which binds with the iron and keeps your body from using it. Try broccoli, kale and other dark leafy greens instead.

Let’s Save Bakare Abidemi Abiodun

The Bakare we know is a young energetic man, a sport lover a red devil (Manchester United fan). That Bakare i know is a guru, a social media freak, a blogger and a networker. He loves reading and meeting new cool friends even though he is a shy type. A young Nigerian graduate who has been suffering with Sickle Cell Anaemia all his life is in dire need of financial assistance  to battle the disorder.


The young man, Abiodun Abidemi Bakare , according to Tosin Adesola ,
the Director of Sickle Cell Advocacy and Management Initiative, a non-
governmental organisation, urgently needs hips and knee replacement
surgery » and the body hopes to take him abroad for the operation as
soon as he is done with his physiotherapy here in Nigeria.
Bakare who was born with the disorder, has been going through a lot of
pain and discomfort as he struggled through school and his condition
became deteriorated as he was rounding up his final year examinations
at the Onabisi Onabanjo University and could not go for his mandatory
National Youth Service.


Adesola, herself a carrier of the disease, says she knows what the
young man is going through and is reaching out to kind spirited
Nigerians to urgently come to his aid.
Adesola talks about a new initiative her team has set up to raise the
money needed to take care of Bakare, called ‘N500 by 1m Campaign.’

We are a non-profit organization setup to give support and succour to people living with Sickle Cell. Since its inception in 2008, Sickle Cell Advocacy has touched the lives of sickle cell sufferers around the country through the monthly medical outreaches where we provide free medical consultation and free drugs to underprivileged children and adults with sickle cell thereby assisting in managing their health.
That’s why we need your N500 for the ‘N500 by 1m campaign. The N500 by 1m campaign is an initiative set up to encourage 1m Nigerians to donate N500 or more.

Sickle Cell Advocacy aims to provide urgently needed medication, life-saving surgeries as well as counseling and public awareness programs through the monies we generate from the campaign.

With your kind donations, we will be able to provide even more support to
improve the lives of those living with sickle cell in the country.
Sickle Cell is no longer a death sentence, with proper care and management they can live a well fulfilled and purposeful life, just like I have.

Why not join other Nigerians and I in this fight against sickle cell Anemia?
Please send in your N500 or more to either of these accounts.

Sickle Cell Advocacy & Mgt Initiative Account

Sickle Cell Advocacy & Mgt Initiative Account

Thank you all as we all  save Bakare.

What Is Iron-Deficiency Anemia?

What Is Iron-Deficiency Anemia?


The term “anemia” usually refers to a condition in which your blood
has a lower than normal number of red blood cells. In a person with
anemia, the blood does not carry enough oxygen to the rest of your
body. The most common cause of anemia is not having enough
iron. Your body needs iron to make hemoglobin. Hemoglobin is an
iron-rich protein that gives the red color to blood. It carries oxygen
from the lungs to the rest of the body.
Iron-deficiency anemia usually develops over time if your body
doesn’t have enough iron to build healthy red blood cells. Without
enough iron, your body starts using the iron it has stored. Soon,
the stored iron gets used up.
After the stored iron is gone, your body makes fewer red blood
cells. The red blood cells it does make will have less hemoglobin
than normal.
Iron-deficiency anemia can cause fatigue (tiredness), shortness of
breath, chest pain, and other symptoms. Severe iron-deficiency
anemia can lead to heart problems, infections, problems with
growth and development in children, and other complications.

What Causes Iron-Deficiency Anemia?
Blood Loss
When you lose blood, you lose iron. If you don’t have enough iron
stored in your body to make up for the iron loss, you’ll develop
iron-deficiency anemia.

In women , low iron levels may be due to blood loss from long or
heavy menstrual periods or bleeding fibroids in the uterus. Blood
loss that occurs during childbirth is another cause for low iron
levels in women.

Internal bleeding (bleeding inside the body) also may lead to iron-
deficiency anemia. This type of blood loss isn’t always obvious, and
it may occur slowly. Some causes of internal bleeding are:
A bleeding ulcer, colon polyp, or colon cancer

Regular use of aspirin or other pain medicines, such as nonsteroidal anti-inflammatory drugs (for example, ibuprofen and naproxen)
Urinary tract bleeding

Blood loss from severe injuries, surgery, or frequent blood drawings also can cause iron-deficiency anemia.

Poor Diet
The best sources of iron are meat, poultry, fish, eggs, and iron-fortified foods (foods that have iron added). If you don’t eat these foods regularly, or if you don’t take an iron supplement, you’re more likely to get iron-deficiency anemia.
Vegetarian diets can provide enough iron if the right foods are
eaten. For example, good non-meat sources of iron include spinach
and other dark green leafy vegetables, certain types of beans, dried
fruits, and iron-fortified breads and cereals.
During some stages of life, such as pregnancy and childhood, it
may be hard to get enough iron in your diet. This is because your
need for iron increases during these times of growth and

Women of childbearing age are at increased risk for iron-deficiency
anemia because of blood loss during their monthly periods. About 1
in 5 women of childbearing age has iron-deficiency anemia.

Inability To Absorb Enough Iron
Even if there’s enough iron in your diet, your body may not be able
to absorb it. This may be due to intestinal surgery or diseases of
the intestine, such as inflammatory bowel disease or celiac
disease. Prescription medicines that reduce acid in the stomach also
can interfere with iron absorption.

What is the treatment for Iron-Deficiency Anemia?
Treatment for iron-deficiency anemia will depend on the cause and
severity of the condition. Treatments may include dietary changes
and supplements, medicines, and surgery. Severe iron-deficiency
anemia may require treatment in a hospital, blood transfusions, iron
injections, or intravenous iron therapy.
Eating a well-balanced diet that includes foods that are good
sources of iron may help you prevent iron-deficiency anemia.
Taking iron supplements (as your doctor prescribes) also may lower
your risk for the condition if you’re not able to get enough iron
from food.

Signs of a Sickle Cell Pain and Crisis in Babies


How do you know your angel is in pains, he/she cant say “mom, am getting hit by a crisis” but as a mother here are Signs of a Sickle Cell Pain and Crisis in Babies

1. Continuous and Persistent Crying:
Crying can be indicative. Once you eliminated all the other possibilities of the crying (diaper, feed, sleep, comfort), and nothing seems to work, your child may be having sickle cell pain.

2. Swelling : Hands, feet, and limbs may seem bigger than usual or swollen and puffy.

3. Jaundice: Yellowing of the skin and eyes. You should monitor your baby’s eyes daily. The sclera (the white part) may be yellow. The deeper the yellow, the more likely a crisis. Some eyes may not be yellow, but just a ‘change’ in the eyes.

4. Warmth: The affected extremity may be warmer than the other one. For example, the foot, leg, or thigh may have a warmer temperature than the other one that has no pain. Also may occur in knees, arms, pelvis or any joint.

5. Fever: This often the last sign for crisis or pain. A fever greater than 101°F is a solid indicator. A fever means your baby may have an infection, which does lead to pain/crisis. Most crisis also have a fever component attached in almost all babies.

6. Withdrawal and Sensitivity to Touch : If you baby reaches for you, but then pushes you away or doesn’t want to held, or touched, she/he probably is having some sort of pain. They want your comfort, but you holding them may be causing more pain. So if you notice this mixed message, they probably have pain.

7. Mood: Really irritable

8. Lack of Appetite: If your baby is not eating or drinking milk as she usually does, then this can be another sign. This can lead to dehydration which can lead to a pain crisis.

9. Lack of sleep : If you are on a regular sleep scheme and your baby cries all night long, and does not sleep

10. Movement : The baby does not want to move, and if they move, they will cry. If a toddler, you may notice limping.

11. Color: If he/she has paleness or lighter coloring especially in the palms of hands and feet, inside of mouth…Also may have black or blue areas on lips, toes, fingers or skin. This is from diminished oxygen to tissues because of low blood count

12. Lethargic or Limp: Just not moving, walking, or acting as usual.

13. Intuition: Over time you will develop the intuition that will just kick in and tell you. My mom would just look at me and ‘know’ that I was in pain. It is your mothering instinct taking over. If your gut is telling you something is wrong with your baby…then take him/her to get checked out by a doctor.

Approach to the Vaso-occlusive Crisis in Adults with Sickle Cell Disease

The vaso-occlusive crisis, or sickle cell crisis, is a common painful complication of sickle cell disease in adolescents and adults. Acute episodes of severe pain (crises) are the primary reason that these patients seek medical care in hospital emergency departments. Frequently, however, the pain is incompletely treated. Despite advances in pain management, physicians are often reluctant to give patients adequate dosages of narcotic analgesics because of concerns about addiction, tolerance and side effects. It is important to recognize a pain crisis early, correct the inciting causes, control pain, maintain euvolemia and, when necessary, administer adequate hemoglobin to decrease the hemoglobin S level. The family physician and the hematologist must work together to treat acute pain episodes promptly and effectively, manage the long-term sequelae of chronic pain and prevent future vaso-occlusive crises.
The vaso-occlusive crisis, or sickle cell crisis, is initiated and sustained by interactions among sickle cells, endothelial cells and plasma constituents.
Vaso-occlusion is responsible for a wide variety of clinical complications of sickle cell disease, including pain syndromes, stroke, leg ulcers, spontaneous abortion and renal insufficiency.
Epidemiologic data indicate that 5.2 percent of patients with sickle cell disease have three to 10 episodes of severe pain every year.
In most patients, a pain crisis resolves within five to seven days. A severe crisis may cause pain that persists for weeks to months.
When a vaso-occlusive crisis lasts longer than seven days, it is important to search for other causes of bone pain, such as osteomyelitis, avascular necrosis and compression deformities. When a recurrent bone crisis lasts for weeks, an exchange transfusion may be required to abort the cycle.
A vaso-occlusive crisis most commonly involves the back, legs, knees, arms, chest and abdomen. The pain generally affects two or more sites. Bone pain tends to be bilateral and symmetric. Recurrent crises in an individual patient usually have the same distribution.
Predicting when a vaso-occlusive crisis may occur can be difficult. A number of factors, however, including dehydration, infection and cold weather, are known to precipitate acute crises. In many patients, the exact cause cannot be determined.
Patients need to be aware of the factors that can precipitate vaso-occlusive crises. These patients are particularly susceptible to dehydration because of a reduced ability to conserve water secondary to a defect in renal concentrating ability. They should be counseled to wear warm clothes in cold weather, drink adequate amounts of fluids in hot weather and avoid exercising to the point of fatigue and dehydration. Avoiding mountain climbing or air flights in an unpressurized cabin (noncommercial flights) above 10,000 feet
Avoiding exposure to extreme cold, exercising to exhaustion or using drugs that can lead to acidosis (e.g., acetazolamide [Diamox])


The visual analog scale consists of a horizontal line labeled from zero to 10, with zero indicating “no pain” and 10 indicating “worst pain possible.”  The patient circles the number that indicates the overall intensity of the pain. The visual analog scale has been found to be a clinically useful objective parameter for titrating narcotic analgesics and planning hospital discharge.
The verbal pain scale can be used if a patient is unable to provide a written response to the visual analog scale. The patient is asked to report pain intensity verbally on a scale of zero (no pain) to 10 (worst pain possible).
The pain relief scale compares the degree of pain relief that has been achieved with the degree of pain the patient had on the previous day and/or the first day of hospitalization. The degree of pain relief is based on a scale of zero to 100 percent.
The patient can also be asked to mark the area of pain on a scaled body drawing. This technique is useful for determining the extent of involvement and for distinguishing the pain of vaso-occlusive crisis from pain caused by other complications, such as joint infection.

Pain from a vaso-occlusive crisis is often undertreated because of concerns about narcotic addiction and tolerance, perceived drug-seeking behavior, excessive sedation, respiratory depression and lack of specific findings on the physical examination.
Physicians often fail to prescribe narcotics appropriately and tend to overestimate opioid dependence in patients with pain crises. Yet the incidence of opioid analgesic addiction in patients with sickle cell disease has been reported to be no higher than 3 percent.
Many drug regimens have been effective in the treatment of acute pain in sickle cell disease. Pain management should follow the three-step “analgesic ladder” recommended by the World Health Organization for the treatment of cancer-related pain.  The choice of analgesic and the dosage used should be based on the severity of pain in the individual patient.
Patients with mild pain can often be treated at home with oral fluids and nonnarcotic analgesics. Patients can also be started on acetaminophen with or without codeine or oxycodone (Roxicodone), depending on pain severity. Nonsteroidal anti-inflammatory drugs can be used unless they are specifically contraindicated because of peptic ulcer disease, renal disease or hepatic dysfunction.

STOP the PAIN before it STARTS!!!!🚫

When we are in pain our first option and last resort can be to go in the medicine cabinet, grab our prescription with our pain pills in them, and take a couple of our pain medicine to relieve the pain and discomfort we are experiencing. Everyone with sickle cell knows that once a crisis has set in motion it may be a little too late for you to try any other methods to relieve the pain you are feeling. At this point the most important thing is to take something before the pain spirals out of control and brings us more pain that is just too unbearable to handle. However when it comes to taking pain medicine we are aware that it can put stress on our bodies and cause damage to our organs over an extended period of time. With that in mind, the idea is for us to cut down on the number of crisis we have so we can eliminate the amount of medicine we take and the number of pain crisis we go through altogether. No one enjoys being in pain or having that groggy feeling you get from constantly taking pain medicine. So here are a couple of lifestyle changes you can incorporate into your everyday life to get you on the right track of feeling better and help you stop the pain before it starts.
Start by eating healthy -The benefits of eating healthy have shown to cut down on significant health problems like heart disease, hypertension, and diabetics. And you can do this by cutting back on a lot of sugary foods in your diet, watching your salt intake, and putting more fruits and vegetables in your diet.
Work exercise into your daily routine – Next to eating right I say exercise is one of the best things you can do for yourself to improve your health. The body responds to movement and exercise helps to keep your body functioning at a good level because it builds the body up and makes it strong to fight off infections to ensure you stay healthy. Another benefit of exercising is it improves the flow through your lung passages so that you are getting an adequate supply of oxygen through your body which could mean fewer crises. There are many different forms of exercising but aerobics and cardio exercises I think are the best for you. They get the heart pumping and circulating blood throughout the body so that your blood cells move more fluidly throughout the body. Also cardio exercises are less strenuous on your joints and less likely to leave you sore afterword’s or cause any strains to your muscles. If you are unable to perform any of those exercises than I say get out and walk at least 15-30 minutes.
Avoid smoking and drinking – Smoking decreases the amount of oxygen you are getting to the lungs and into your body which causes your cells to deoxygenate faster and become sickled. Consuming to much alcohol dehydrates you and breaks down your red blood cells at a rapid pace and your body cannot keep up to make new ones which will eventually cause you to have a lower hemoglobin until eventually you end up in a sickle cell crisis.
Hydrate yourself – We can’t stress the importance of water. It is life’s essential ingredient. It helps provides lubricant for the blood cells moving throughout the veins and delivers oxygen to the body. Water is essential for all of life on earth and many living things can’t survive without it.
We may not be able to totally stop our bodies from going into a sickle cell crisis but I think if you start doing these things you will significantly see a change in the number of crisis you are experiencing. So from one sickle cell warrior to another, stay healthy and take care of your body.

Creating an avenue for all SCD warriors accross the world!