General Summary for Schools (Minneapolis Children’s Hospital Sickle Cell Clinic)

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