What is sickle cell disease?

Sickle cell disease is a genetic condition that causes the red blood cells to change shape, harden, and not do their job as well as regular red blood cells. Although the severity of complications due to sickle cell disease can vary widely from one person to the next, it almost always has direct impact on educational needs. Sickle cell disease (SCD) directly affects education because it causes health, learning, and developmental problems, in four main ways:

1. The sickled cells don’t carry oxygen well to the brain, organs, and muscles; therefore, students with SCD have chronic anemia (low blood oxygen), causing poor stamina, slowed growth, and (for about half the students) problems with attention and learning.

2. Sickled cells can stick to each other and to blood vessel walls, restricting blood flow and making clots, causing severe pain and tissue damage in the brain, lungs, spleen, joints, or other body parts.

3. Sickled cells lower a student’s resistance to infectious illnesses; they can become sicker for a longer, and so may miss more school. Frequent school absences interfere with academic progress.

4. For some students, self-esteem, mood, or social relationships might be affected by difficulties with frequent pain and illness; learning or attention issues (in about half, not all, kids with sickle cell disease); slow growth and short stature; and bedwetting (because the kidneys do not concentrate urine).

How can kids with sickle cell disease stay safe and healthy in school?

Infection: The spleen does not work properly with most types of SCD. DO NOT give Tylenol or Ibuprofen for a fever. If the student’s fever is over 101 degrees Fahrenheit then his/her mother should be called and they should call their Hematologist. The 24-hour Hematology Clinic number is 612-813-5940. DO NOT wait to see if the fever goes down. It is very important that the student receive blood cultures and IV antibiotic. Blood infections are life threatening.

Cerebrovascular Stroke warning signs: If the student ever suddenly cannot see, speak, keep his/her balance, or move his/her arms & legs normally, call 911, then the parent, then the Clinic. This occurs very rarely among children and youth with SCD, but requires a FAST response.

Kidneys: Sickled cells can damage the kidneys. Consequently students with SCD cannot concentrate their urine and will need to use the restroom more frequently. Allow him/her to use the restroom as often as he/she requests. (If there are any concerns about frequency or duration of restroom use, call the Clinic nurse or psychologist.)

Fluids: Extra fluids can keep the sickle cells from clogging small vessels. We encourage students with SCD to drink ONE CUP OF LIQUID / 5 POUNDS OF BODY WEIGHT IN A 24 HOUR DAY. Allow the student to have a water bottle at his/her desk to maintain adequate hydration. (This will work much better than requiring him/her to use a hallway drinking fountain.) The student needs healthy snacks because his/her body has to work harder to make new blood cells.

Exercise: Like everyone else, students with sickle cell should exercise for a healthy life. We expect The student to fully participate in school activities including recess and physical education. However, The student should be allowed to rest if requested and she should be well hydrated before and during exercise. Never require him/her to continue exercise if she complains of pain, fatigue, or shortness of breath. During cold or windy weather she should be dressed appropriately including hat, boots and gloves. Modify P.E. expectations/grades for his/her stamina, energy, & endurance.

Swimming: The student can swim in an indoor pool if the water temperature is 80 degrees Fahrenheit or above. He/she should dry off immediately when leaving the pool and have a dry robe or towel on at all times. Sudden chills or temperature changes can trigger sickle cell pain. The student should be allowed to stop swimming on request. He/She should be well hydrated before and during swimming.

Transportation: If a student lives more than 2 blocks from the school we require a bus pick them up and transport them to school. The student should not stand out in the cold during winter (e.g. in a bus dismissal line, or waiting on the corner for the school bus to come).

Expect some uplanned medically necessary absences: Expect more health-related absences than for most students (medically excused tardy, absent, or early dismissal days). Make arrangements proactively to communicate with parents/student about assignments, missed lectures or tests. For a short absence, arrange time for the student to work with teacher or aide to make up missed information without penalty. If absent longer (with Doctor’s approval), arrange for someone to bring home assignments & work with the student at home; shorten or waive assignments if possible. Extend deadlines. Keep an extra set of books to study at home.

The student should have a Medication Administration consent form on file completed and signed by his/her Hematologist (Dr. Nelson).

The student also should have a “consent to release information” form on file so that the school can communicate with Students’s medical and psychosocial team (see below).

Pain management plan Ten basic steps for when a student feels sickle cell pain:

1. Drink a lot (water, juice, milk, soup, jello, ice, sherbet) & eat healthy snacks (protein).

2. Distract him/herself (concentrate hard on work, play, prayer, or breathing).

3. Rest in a quiet spot; relax on his/her own; use relaxation strategies.

4. Tell Parent (at home) or a teacher, coach, playground or lunchroom assistant (at school). All school staff should send the student to the school nurse if he/she complains of pain at school.

5. Parent or school nurse checks for a fever; then calls the Hematology Clinic (and nurse calls Parent, if the student is at school). IF THERE IS A FEVER DO NOT GIVE PAIN MEDICATION (TYLENOL OR IBUPROFEN); CALL THE CLINIC IMMEDIATELY.

Parent should call the Clinic to help decide if unsure about sending the student to school.

6. After talking to Hematology Clinic, if there is no fever, school nurse (or Parent) provides Tylenol or Ibuprofen, warmth (heating pad set on low; hot water), massage, rest. The student should return to class ASAP. If he/she must stay home, it should be fairly boring — never more entertaining or enjoyable than going to school would have been.

7. If pain persists more than 2 hours, then nurse or Parent can give a narcotic analgesic. This medicine might make the student groggy. He/She might need to nap in the nurse’s office, then return to class later. If he/she is groggy she should be in school anyway, but Parent or nurse should notify the teacher, so the student is not required to take tests that day. The student should have time for re-teaching of class work taught on days when he/she is groggy or in pain. Note that medication can also help him/her be more alert, and learn better, when it controls distracting pain. Some students take hydroxyurea (a medicine that reduces the number and severity of sickle pain crises). Hydroxyurea may improve their alertness and learning due to better blood circulation. It does not cause problems with alertness or learning.

8. If pain persists, call Hematology Clinic again; the student might have to come in to the Clinic or the Emergency Department (E.D.). At the Clinic or E.D., The student might get IV fluids, medicines, or (as a last resort, respecting family’s religion), a blood transfusion. If pain improves, the student might be sent home.

9. Sometimes the student might need to be hospitalized (this is usually just for a few days). The student should return to school as soon as possible. There should be a plan in place for making up any work that is missed, or waiving some work. Pain and medications may make it impossible for the student to complete work during the absence.

Who are the student’s medical providers?

The number for the HEMATOLOGY CLINIC (Minneapolis) = 612-813-5940

Clinic Nurse/Case Managers (these expert “front line” nurses will answer questions when you call the Clinic, and help to decide whether or not the student needs to come to the Clinic or E.D.):

Liz McDonough, RN; Nicole Leonard, RN; Sue Purdie, RN 612-813-5940

(If none of these are available, any other Hematology-Oncology nurse can help.)

Medical Specialists will direct the student’s care when he/she comes to clinic:

Hematologist: Stephen Nelson, MD 612-813-5940

Hematology Certified Clinical Nurse Practitioners (advanced training to manage care for patients with SCD in collaboration with hematologist): Jane Hennessey, CPNP; Kristin Moquist, CPNP; or Kim Jacobson, CPNP. 612-813-5940

The psychosocial team will help take care of the student’s emotional, behavioral, self-advocacy, and learning needs as he/she grows up. These team members currently include:

Neuropsychologist: Karen Wills, Ph.D. (612-813-6224, fax 612-813-6385)

E-mail for Dr Wills is karen.wills@childrensmn.org

Dr. Wills evaluates the student’s brain function in relation to his/her school learning, emotional and behavioral adjustment; at parent’s reqest, serves as a liaison to the school IEP team and other agencies to help explain how the student’s medical condition and needs impact his/her educational and developmental progress, and participates in developing the “whole student and family” care plan for the student.

Social Workers: Jill Swenson MSW, or Melissa Adler MSW.

These team members assist with coordinating hospital services with services from outside agencies (e.g., Student Protective Services and school social work staff) to develop a care plan and to provide ongoing monitoring of compliance with the care plan.

If the student needs to be seen in the Emergency Department, CALL THE CLINIC

at 612-813-5940 for help with coordinating the student’s care and follow-up visits. This is the 24/7 number to call.

If the family ever has concerns about the quality of care that the student receives at Students’s Hospitals, they may wish to call Colleen Kulesa, Family Relations Liaison, at 612-813-7393, for help in discussing & resolving any problems, & to try to ensure they do not happen again

What is sickle cell disease?

Sickle Cell Disease (SCD) is a genetic abnormality that causes changes in the red blood cells (hemoglobin).  It is most common in people of African or Caribbean descent.  There are different subtypes of SCD.  This summary concerns Hb-SS, Hb-SC, and Hb-S beta zero thalassemia, three common subtypes that can cause serious medical complications.  (The sickle cell TRAIT, and the sickle cell disease type called Hb-S beta plus thalassemia, have not been shown to affect brain development.)

How does SCD affect the body and brain?

Sickling of blood cells: Sickle cell disease (SCD) is a recessive genetic difference in the gene for globin, part of the hemoglobin (Hb) molecule within red blood cells, which carries oxygen throughout the body.  When oxygen level within the blood cell drops, the sickle hemoglobin (HbS) forms long-chain molecules, making the blood cell more dense, less pliable, and elongated or “sickle” shaped.  These long, rigid, “stickier” cells do not move through the blood vessels as smoothly or quickly as normal cells.  The sickled cells are more likely to get stuck on the blood vessel walls, and form clumps or clots within the tiny capillaries.  A higher count of sickled blood cells, compared to normal cells, is associated with more severe complications of SCD (see below).

Chronic anemia: The body and brain do not receive sufficient oxygen from the blood.  Less oxygen is carried by each blood cell and less blood gets to some areas of the body due to narrowed vessels. More severe chronic anemia (measured by the hematocrit) is associated with lower IQ in children with SCD.  IQ in HbSS decreases slightly with age, even in children with normal MRIs, an effect not seen in children without HbSS.  This may be due to the effect of chronic anemia (hypoxia) interfering with normal brain development.

Vascular damage: The blood cell membranes are damaged by mechanical strain, and in turn cause damage, as they move through blood vessel linings.  They clump with each other and with platelets.  Blood vessel linings become narrower. More damage to the blood vessels decreases healthy blood flow to the brain and increases risk of stroke.

Increased blood volume and pressure: The body compensates for narrowed blood vessels by increasing the pressure and volume of blood flow in other vessels.  This can be visualized as increased cerebral blood flow volume (CBFV) in the carotid, middle cerebral, and anterior cerebral arteries, for example, on Transcranial Doppler (TCD) testing.  The compensatory increase in CBFV  is associated with increased risk of silent or overt ischemia/infarct (stroke).  Increased CBFV (as measured by abnormal TCD values) is associated with lower IQ even in children without evidence of strokes. Brain cells starved of oxygen do not function or develop properly, and the increased CBFV may not sufficiently compensate for needed oxygen especially given the high metabolic demands of developing brain.

Acute chest syndrome: Pain and oxygen loss due to acutely diminished blood flow in the lungs; may be associated with acute or chronic hypoxia, with brain consequences.Starving the brain of oxygen reduces energy and learning ability.

Pain crises: Pain in joints, hands, feet, or other areas of the body associated with HbSS-related vascular changes.  Can occur “out of the blue” but is most commonly triggered by dehydration, extreme temperatures, or sudden change in body temperature.  Pain crises may cause increased absences or, at least, distract children from learning and can affect mood and effort in the classroom.  Pain in the hands may affect writing and other fine motor activities.  Body pain may affect participation in PE and sports.

Other physical damage that affects learning (apart from direct effects on the brain): Diminished blood supply to the spleen makes it harder for the body to fight infections, so children with SCD become ill more easily and take longer to recover; this is especially severe in children withsplenectomy (removal of the spleen).  Frequent infectious illness may contribute to school absences even in children who do not have frequent pain.

Diminished blood supply to the bones can cause an “avascular necrosis” in which bone deteriorates, necessitating surgeries such as hip replacement, in young children.

Diminished blood supply to the tiny vessels of the eyes (retinas) can cause blindness or low vision.  In some cases, visual changes can happen suddenly, so teachers should be aware of this possibility in a child with sickle cell disease.

Diminished blood supply to the heart can cause cardiac arrhythmia, which further impair the child’s energy and blood circulation.

Normal blood cells remain in the body about 10 days, but sickled blood cells die off in 2 or 3 days, and must be constantly replaced.  The body has to work hard to make new blood cells all the time.  Therefore children with sickle cell disease may have to eat and snack more often than other children, but may not gain weight or grow normally, even when they eat a lot.

Dehydration, extreme temperatures, extreme physical exertion, and extreme emotional stress, can contribute to increased sickling and pain crises. Children need to stay very well hydrated and avoid extreme temperatures, especially temperature changes.

MRI abnormalities:  Ischemia (diminished blood flow, with diminished oxygen, glucose, and other nutrients, to certain regions of body and brain), infarct (tissue death due to severely diminished or interrupted blood flow), and atrophy (brain tissue is lost or fails to develop, so the brain is smaller than normal): In some cases, such as increases in blood flow through basilar arteries of the brain, those other vessels are fragile and not well adapted to the increased blood flow, so they can burst under the pressure, causing strokes.  Sometimes sickled cells or cell-platelet aggregates can block tiny vessels, interrupting blood supply to tissue “downstream” from the clot.  Because of the vascular anatomy of the brain, OVERT strokes with clinically obvious manifestations (changes in sensory and motor function visible on neurological exam; seizures, coma, other major changes in consciousness) tend to occur in anterior cortical regions.  SUB-CLINICAL or SILENT strokes (areas of dead tissue, visible on MRI) tend to be smaller and most likely to occur in anterior deep white matter, in the border zone of the Anterior and Middle Cerebral Artery distributions.  There may also be MICRO-INFARCTS too small to be visible on MRI scans but associated with under-development of the corpus callosum in children with HbSS.

From 10-15% of children with HbSS will have overt strokes by age 15; another 20-25% will have “silent strokes”; the rate of micro-infarcts cannot be measured with current standard neuroimaging.

Peak age for stroke appears to be ages 2 to 4 years, but recurrent stroke is common and can occur at any age.  Massive stroke (basilar artery bleeds) becomes more common in ages 10 to 14 years, associated with progressive occlusion of the anterior arteries.  Anterior infarcts (ACA/MCA border zone in particular) are more common than posterior infarcts at all ages, however it is possible for infarcts to occur anywhere.

Localization effects are uncommon except with massive strokes in older children – usually the effect is a global decrease in function.  Diminished anterior corpus callosum size is associated with lower scores on measures of attention and executive function, independent of IQ.  Greater amount of tissue loss associated with infarct is associated with lower IQ.  These changes in the brain may occur with no “gross evidence” of stroke, that is, no change in speech or movement; but they may show up as impairment of IQ, memory, or other neuropsychological test results and as a struggle to learn in school.  It is important to screen every child with SCD to assess neuropsychological function and help to determine whether more expensive and invasive medical tests, such as MRI/MRA, are needed.

Karen E. Wills, Ph.D.,

Pediatric Neuropsychologist

Children’s Hospitals and Clinics of Minnesota

Karen Wills is the Director of The Sickle Cell Disease Program for Learning Assessment and Neuropsychological Evaluation (SCD-PLANE)

This post is an excerpt from a letter sent to the Minnesota Department of Education two years ago because of misinformation found on their website regarding sickle cell disease.

This year’s event showcased a broad range of speakers, all from Minnesota. The event started at 8 am with continental breakfast and networking by members, attendees and speakers.

The first speaker was Bonnie Jean, an author and parent advocate of the Pacer Center . The second speaker was Wendy Ringer also a parent advocate of Pacer. Elizabeth McDonough of Hematology/Oncology, Children’s Hospitals and Clinics of Minnesota took center-stage for a very interesting round table discussion about expectations from the provider’s standpoint. The fourth person to speak was Dawn Lorenz of Psychological services, Children’s Hospitals and Clinics of Minnesota. She is their insurance and reimbursement officer and gave a very informative talk on the ‘behind the scenes’ of healthcare payments and insurance company technicalities. The final speaker was Abdulahi Sheikh of Minneapolis Urban League. He discussed the cultural issues surrounding immigrant and minority health in Minnesota.

Other highlights of the conference include Dr Karen Wills of psychological services Children’s Hospitals and Clinics of Minnesota who gave a brief overview of SCD PLANE: Program Fro Learning Assessment and Neuropsychological Evaluation, a program of the Childrens’ Hospitals and Clinics of Minnesota. Mr Martin Uko gave the position statement at the end of the presentations and after discussions and deliberations. Dr M.Obinna Nwaneri of Fairview Southdale Hospital and University of Minnesota Physicians talked about the plans of starting an adult sickle cell disease program and clinic at the Fairview University Hospital in the coming months. Dr Steve nelson was present at the event.

The event was broadcast live on Twitter and Facebook with minute-by-minute updates all through the event. President Barrack Obama visited Minneapolis today and this may have been a distraction as some of the expected families made last minute cancellations. However attendance was impressive.

To view photos, follow this link: Conference Photos

About Sickle Cell Disease Advocates of Minnesota, SCDAM
The Sickle Cell Disease Advocates of Minnesota (SCDAM) was formed to improve the quality of life for people living with Sickle Cell Disease by creating awareness, providing support and finding resources. We have operated as a volunteer-based organization since 2003.

History of the Organization: SCDAM began as a support group for parents of children and family members of people living with sickle cell disease in 2001. After several meetings and realizing the limited support that sickle cell disease patients in the Twin Cities get, Mr. Emmanuel Benson, Michelle Amankwah, Fred Amankwah and Martin Uko decided to form SCDAM. They became the founding members and SCDAM was incorporated in the state of Minnesota on Feb 3, 2003. The present leadership includes a physician, Dr Osita Nwaneri who has chaired the board since May 2008.

Our Mission: The SCDAM exists to improve the quality of life for people and families affected by Sickle Cell Disease by identifying resources, providing support and creating awareness through education about this condition in the state of Minnesota and beyond and support and advocacy of affected families

Our Vision: To become an organization with sufficient resources to support the needs of people and families living with sickle cell disease in the state of Minnesota and to have a community that is aware of sickle cell disease, strategies to prevent it and adequate services to support those affected.

About The Third Annual Minnesota Sickle Cell Conference

The theme of this year’s conference is focused on healthcare finances and navigating the healthcare system to optimize sickle cell disease care. It is entitled: “PAYING FOR YOUR CARE, GETTING THE CARE YOU DESERVE”. Many patients and families have numerous difficulties obtaining optimal care for sickle cell disease because of the poor knowledge of insurance companies and the often beaurecracy-laden reimbursement procedures. Procedures and care known to be standard of care in other parts of the country still require ‘special approval’ by many HMOs. This has become a barrier to care for this vulnerable population.

We have brought together a group of experts in two broad areas with the goal of enlightening attendees on effective ways to access care for sickle cell disease and stress-free access to financial support for the care of persons affected by sickle cell disease. The sessions will include round-table discussions as well as question and answer sessions. At the end of this half-day meeting, participants will know what happens behind the scenes before, during and after clinic visits. How are the fees paid? What can we do to ensure the best communication and rapport with our providers? What other help and assistance is available to ensure that my child attainsfull potential in school?

Speakers at the conference include resource ersons from Minneapolis Urban League, PACER Center and Children’s Hospitals and Clinics of Minnesota. Registration is free. We have been raising funds for the conference through Facebook causes, “Sickle Cell Advocacy: Let’s Do This” on Facebook.

About Sickle Cell Disease

Sickle Cell Disease is a recessively inherited condition characterized by chronic severe bone pains, anemia, recurrent infections and complications of the major organs of the body. Common complications include splenic infarction, renal failure and stroke. Minnesota researchers are beginning to expose adverse effects of poor brain bloodflow of the disease on school performance. This condition is much more common in African Americans, native Americans, Hispanics and peoples of Mediterrenian ancestry. It is diagnosed at birth through newborn screening.

There is no known cure however researchers have successfully performed bone marrow transplant on a handful of children with very severe disease and most of them have done well and no longer experience symptoms. The standard of care however still remains regular physical and blood checks by a hematologist, ultrasound of the head from 3 years of life to look for early signs of strok potential, and hydroxyurea treatment to prevent symptoms. Other newer drugs developed in Nigeria have not been approved in the USA and are not available here.

Sickle Cell Disease can be prevented if individuals and the community as a whole take testing seriously. Testing for sickle cell disease carrier state before having children is one way of ensuring that more children are not born with this disease. The test is a simple inexpensive blood test that can be performed in most doctors’ offices.

Contact:
M Osita Nwaneri, MD
Chairman SCDAM
P.O.Box 16238,
Saint Paul MN55116
www.scdam.org
Telephone: 1-888-667-2326 (1-888-MN-SCDAM) Extensions 301-309
Email: osita@scdam.org, martin1@scdam.org,
twitter @sickleadvocate
Donate to SCDAM on our website or mail a check to P.O.Box 16238, St Paul, MN55116
Facebook: Become a fan of Sickle Cell Disease Advocates of Minnesota
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The researchers for this study were: Arianna Lund and Stephen Nelson of the Pediatric Hematology Department of the Children’s Hospitals and Clinics of Minnesota, Minneapolis and St Paul, Minnesota,USA.

This poster was presented at the Florida PASS Sickle Cell Conference, in Ft Lauderdale in March, 2009.
You can join Dr Nelson and other experts here for more detailed discussion of the research findings and its implications.

Dr Nelson gave an update to families during the bi-annual sickle cell update of the Hematology Department of Children’s Hospitals and Clinics of Minnesota on  9th of May 2009 at Children’s Hospital Minneapolis. The title was A Report from the Annual National Sickle Cell Disease Scientific Meeting. It was well attended. At that meeting, SCDAM also addressed the participants and new volunteers joined our advocacy group. The next family Seminar will hold on the 10th of October 2009. The Title is Home Strategies for Pain Control. What Can we do at home?

Download zipped poster here: Weather and Sickle cell Disease