educator’s guide to sickle cell and school -...

Educator’s Guide to Sickle Cell and School

Educator’s Guide toSickle Cell and School


Introduction

Understanding Sickle CellWhat is Sickle Cell Disease?Other Facts About Sickle Cell DiseaseTypes of Sickle Cell DiseaseSickle Cell TraitCommon Characteristics of Sickle Cell DiseaseLess Common Complications of Sickle Cell DiseaseAnemiaInfectionPainful EpisodesCommon Triggers of Sickle Cell PainDecreased Urine ConcentrationGrowth and DevelopmentJaundice/Yellow Eyes

Tips for the EducatorHelpful TipsManaging a Student Through PainManaging a Student Through a Fever

What Can You Do To Help?What You Can Do as the TeacherWhat You Can Do as the PrincipalWhat You Can Do as the Guidance CounselorWhat You Can Do as the School NurseWhat You Can Do as the Physical Education

Instructor/Coach

Closing

Can You Pass the Test?

Table of Contents

ii

2234567788999

111414

16171819

21

23

24


Introduction

It is our hope that this book willprovide insight and/or information toschools that educate students withsickle cell disease. An important goalof the sickle cell team is to workclosely with the student, family, andschool to achieve school success. Ifthere are any concerns regarding thestudent, medically or academically,please contact the student’s parentfirst. We are more than happy to assistonce the parent has been notified.


UnderstandingSickle Cell


Sickle cell disease is a group of disorders thataffects the red blood cells in the body. Normalred blood cells are soft and round (like adonut) and travel through the body withoutany problems. The main job of red blood cellsis to carry oxygen. When exposed to certaintriggers, red blood cells affected by sickle celldisease can become hard, sticky and curvedin shape (like a banana). Sickled cells are notas soft and flexible so they may get stuck insmall blood vessels. If this happens, someparts of the body do not get enough oxygen.Pain or damage may occur.

Other facts about sickle cell diseaseOther facts about sickle cell diseaseOther facts about sickle cell diseaseOther facts about sickle cell diseaseOther facts about sickle cell disease

• It is genetically inherited, one gene passedfrom each parent

• It is usually identified at birth (throughmandatory newborn screening)

• It affects 1 out of every 400 African-Americans

• The disease also affects people ofMediterranean, Caribbean and Asiandescent

• The disease is characterized by chronicanemia, increased susceptibility toinfections and painful episodes

What is Sickle Cell Disease?

Sickled Cell

Normal Cell

2


• Hemoglobin SS Disease

(Sickle Cell Anemia)

• Hemoglobin SC Disease

• Sickle Beta Zero Thalassemia

(Sickle βββββ° Thalassemia)

• Sickle Beta Plus Thalassemia

(Sickle βββββ+ Thalassemia)

• Sickle Hereditary Persistence of Fetal

Hemoglobin (S/HPFH)

• Other types

Types of Sickle Cell Disease

Sickle cellaffects

1 out ofevery 400

African-Americans

3


Sickle cell trait is passed on from a child’sparents. Children who get one normalhemoglobin gene from one parent and onesickle hemoglobin gene from the other parentwill have sickle cell trait. A child born withsickle cell trait will have it all of his life but itwill never turn into sickle cell disease. Sicklecell trait is found in one out of every 10-12African-Americans. It can also be found inpeople of Mediterranean, Middle Eastern,Indian, Caribbean and South Americandescent. People with sickle cell trait usuallydo not have any medical problems. Thenormal hemoglobin that was passed on byone parent keeps them from having sickle celldisease. People with sickle cell trait shouldlive normal, healthy lives and have a normallife expectancy.

Sickle Cell Trait

4


• Anemia is a lower number of red bloodcells (oxygen carrying cells) in thebody. This results in a lowerhemoglobin level.

• Infection may occur due to the spleennot working properly. Sickled redblood cells get trapped in the spleencausing damage. The spleen normallyfilters bacteria out of the blood to helpreduce infection.

• Pain occurs when red blood cells“sickle” (change their shape) andbecome trapped in small blood vesselsin the body. Once trapped, bloodcannot flow normally. The area wherethe blood cells are trapped does not getenough oxygen, therefore pain mayoccur.

• Decreased urineconcentration causesfrequent trips to therestroom.

• Delayed growth anddevelopment is common inchildren with sickle celldisease.

Common Characteristics ofSickle Cell Disease

5


• Stroke occurs when there is decreasedoxygen to parts of the brain due to sicklecells blocking blood vessels.

• Gall stones happen more often in peoplewith sickle cell disease because sickle redblood cells break down much faster thannormal red blood cells. When these breakdown, they release bilirubin whichcollects in the gall bladder and can form asludge or gall stones.

• Aplastic Crisis is a sudden drop in thehemoglobin usually caused by a viruscalled parvovirus or Fifth’s Disease.

• Splenic Sequestration is an enlargement ofthe spleen and a sudden drop in thehemoglobin.

• Avascular Necrosis (AVN) is a change inthe shape of the hip or shoulder boneswhich can cause pain.

• Priapism is a painful, unwanted erectiondue to trapped sickle cells in the penis. Itis treated like a painful episode, withfluids and pain medicines.

Less Common Complicationsof Sickle Cell Disease

6


Most children with sickle cell disease will beanemic. This means that the child will have alower amount of red blood cells in the bodyand have a lower hemoglobin level. Normalred blood cells (without sickle cell) usuallylive 120 days. Sickle red blood cells only live10-20 days. Being anemic may cause thechild to tire more easily and need to restmore frequently.

InfectionChildren with sickle cell disease (especiallysickle cell anemia and sickle beta zerothalassemia) are at higher risk of infectionthan people who do not have sickle celldisease. Infection is the leading cause ofdeath in young children with sickle celldisease. It is very important for infections tobe treated quickly. Some common infectionschildren with sickle cell disease may getinclude meningitis (infection of the spinalfluid), pneumonia (infection of the lungs),osteomyelitis (infection of the bone) andsepsis (infection in the blood).

Anemia

7


One common occurrence in children withsickle cell disease is pain. Pain can happen atany time of the day or night. It may last a fewhours, few days or even a few weeks. Thepain can range from mild to severe and isdifferent in each child. Sickle cell pain can beanywhere in the body but the most commonsites are the arms, legs, back and stomach.Some pain can be treated at home or duringthe day at school but sometimes the painbecomes severe enough to needhospitalization.

Painful Episodes

Common Triggers of SickleCell Pain:

• Exposure to extreme hot or coldtemperatures

• Dehydration (extra fluids are needed toprevent dehydration which can causepain)

• Over-exertion• Infection• Stress• High altitudes• Shivering (if wet or cold)

8


Children with sickle cell disease urinatefrequently because they are not able toconcentrate their urine. Occlusion from sicklecells may cause small amounts of damage tothe kidneys, therefore causing the inability toconcentrate the urine. More fluid intake isrequired to prevent sickling, therefore morerestroom breaks are needed.

Children with sickle cell disease may bethinner and smaller than other children theirage. The child’s metabolism is usuallyincreased due to the chronic anemia causinghim to burn off more calories and put on lessweight. Children will usually “catch up” inthe later teen or early adult years.

Jaundice/Yellow EyesChildren with sickle cell disease sometimeshave yellowing of the eyes. Red blood cells inthe body break down quickly in thesechildren. As this occurs, bilirubin is releasedcausing the white part of the eye to turnyellow. The yellow color may come and gobut some children may always have yelloweyes. This is not a contagious condition orone that requires medical intervention.

Decreased Urine Concentration

Growth and Development

9


Tips for theEducator


Tip #1: Collaborate with the student’s parent.• Always include the family in

educational planning.• Call the family with any questions or

concerns.• Help the family advocate for the

student’s educational success.

Tip #2: Discuss with the parent and studenttheir wishes for disease disclosure.

• Do not assume the student wants otherstudents to know about the disease.

• Always obtain parent/studentpermission to share information withthe student’s classmates.

Tip #3: Assist with missed classroominstruction.

• Follow established educational plans.• Allow additional time to complete

missed assignments and exams.• Coordinate with the family on how

they can help the student to catch up.• Consider asking the school to establish

a 504 or IEP plan.

Here’s How You Can Help

(continued on next page)

11


Tip #4: Hydration• Encourage frequent trips to the water

fountain.• Allow a water bottle at the student’s

desk.

Tip #5: Bathroom privileges• Provide an open pass to the restroom.• Remember that students with sickle

cell disease cannot concentrate theirurine, therefore needing frequentbathroom breaks.

Tip #6: Activities• Allow the student to participate in all

activities, as he/she is able.• Allow rest during activities as needed.• Modify activities to include, instead of

exclude the student from his/herpeers.

• Swimming may cause painfulepisodes due to cold water. Providean alternate activity for the student asneeded.

• Allow extra fluid intake during timesof increased physical activity.

Here’s How You Can Help (cont’d)


1 2


Tip #7: Nutrition• Offer a well-balanced diet (there

should be no food restrictions relatedto sickle cell disease unless instructedby the parent).

• Encourage extra fluid intake,preferably avoiding caffeine-containing drinks, to preventdehydration.

Tip#8: Transportation• Assist the parent in establishing bus

transportation to and from school ifneeded.

• It is important for the student not towalk long distances in the cold orheat.

Tip#9: Fostering Self-Esteem• Encourage the student’s strengths and

assets.• Provide positive reinforcements as

much as possible.• Encourage creativity and imagination

instead of emphasizing limitations.• Treat student as normal as possible.

Here’s How You Can Help (cont’d)

1 3


• Encourage fluids.• Allow rest.• Escort the student to the school nurse.• Dispense pain medications as instructed

by the parent.• Apply heating pad or warm pack to

painful area.• Notify the parent of the student’s

condition.• Provide emotional support.• Utilize relaxation or distraction

techniques.

• Notify the parent immediately.• Monitor temperature.• Give Tylenol as directed by the parent.• Encourage extra fluids.

• Fever of 101.5 degrees F or greater• Moderate to severe pain• Paleness• Excessive fatigue• Slurred speech• Inability to use an arm or leg• Vision changes• Shortness of breath

Managing a Student ThroughPain

Managing a Student Througha Fever

Notify the student’s parent immediately for:

1 4


What Can YouDo To Help?


• Help the family maintain a comprehensiveassignment notebook with clearinstructions.

• Identify when a 504, IEP (other healthimpaired), and/or homebound instructionis needed.

• Communicate frequently with the family.

• Clearly explain the parent’s responsibilityto pick up and turn in homework whilethe student is out.

• Be familiar with sickle cell disease and itscharacteristics.

• Allow the student to have water breaksand/or a water bottle at his/her desk.

• Provide additional passes to the restroomand school nurse as needed.

What You Can Do as theTeacher

Communicatefrequently withthe family.

1 6


Encourage staff involvement in learningabout sickle cell disease.

Collaborate with parents and teachers inthe development of a 504 or IEP.

Designate a staff person to monitor thestudent’s 504 or IEP.

Communicate with the parent yourschool’s willingness to assist in thestudent’s physical health and intellectualdevelopment.

What You Can Do as thePrincipal•

•

•

•

17

(Sample 504 plan)


What You Can Do as theGuidance Counselor

Facilitate staff development by hosting aneducational session on sickle cell disease.

Evaluate students with sickle cell diseaseto determine the need for a 504 or an IEP.Often the parent(s) may not be aware thestudent qualifies for special education or504 accommodations. If a teacher comesto you with concerns about a student,assess the student’s absences and daysspent in the nurse’s office.

If an IEP or 504 evaluation process isrequested by the school or the parent(s),coordinate with school representatives tosee that this process is timely.

Designate a staff person to monitor thestudent’s 504 or IEP.

Provide emotional support to the studentwith sickle cell. Welcome the student backwhen there has been a prolonged illness athome or in the hospital.

Provide educational resources, such asinformation about summer school, afterschool sessions and tutoring to assist andenhance the student’s learning.

•

•

•

•

•

•

1 8


Become educated about sickle cell diseaseincluding its characteristics andtreatments.

For every student with sickle cell disease,maintain a plan that includes informationon warning signs, complications, “asneeded” medications, routinemedications, things to do in an urgentsituation.

Communicate with the parent for anyquestions or concerns.

Ask the student what he/she usually doesat home for treatments or things that workbest for him/her.

Have all necessary paperwork in place toadminister medications as needed.

Monitor response to pain managementinterventions.

What You Can Do as theSchool Nurse

•

•

•

•

•

•


1 9


Coordinate educational sessions for otherschool staff members.

Notify the parent for any painful episodes,fevers of 101.5 degrees F or greater,increased lethargy, increased paleness orany other concerns.

Provide necessary treatments for painfulepisodes:

-Rest

-Increased fluids

-Heating pad

-Pain medications (as instructed by the parent)

-Notify parent for further directions

School Nurse (cont’d)

•

•

•

2 0


What You Can Do as thePhysical EducationInstructor/Coach

Be familiar with sickle cell disease and itscharacteristics.

Encourage participation in all activities astolerated.

Communicate with the parent about thestudent’s ability to fully participate inactivities and note any restrictions.

Allow the student to rest as needed(children with sickle cell disease may tiremore easily than others and prolongedstrenuous activity may lead to a painfulepisode).

Provide an alternate activity that includesother students when the scheduledactivity is too strenuous for the studentwith sickle cell disease.

•

•

•

•

•


21


Be supportive if the student is unable tocomplete the scheduled activity.

Allow frequent use of the water fountain(children with sickle cell disease maybecome dehydrated quickly).

Avoid exposure to extreme temperatures(hot, cold, damp).

Physical EducationInstructor/Coach (cont’d)

•

•

•

2 2


Many students enjoy academics andgo on to graduate, attend college, andpursue a career or vocation. Yourrole as a teacher, mentor, or friendwill assist the student in becoming asuccessful adult. With your support,the information in this booklet willhelp a student with sickle cell diseasesucceed.

Closing

2 3


FUN QUIZ

1. Sickle cell is a disease that affects the redblood cells.

True False

2. What are ways you can assist with thestudent’s educational success:

a. Allow bathroom privilegesb. Communicate with the student’s

parentc. Create a notebook with

classroom assignmentsd. All of the above

3. Circle the common characteristic(s) ofsickle cell disease:

a. Mild to moderate painb. Contagious diseasec. Anemiad. Impairs cognitive abilitye. Infection

4. Student’s with sickle cell disease can graduate and lead normal lives.

True False

Can You Pass the Test?

Answers: (1) True, (2) d, (3) a,c,e, (4) True

2 4


Blank


Children’s Mercy Hospitals and ClinicsHematology/Oncology

2401 Gillham RoadKasnas City, Missouri 64108

816.234.3000www.childrens-mercy.org

This publication is supported by funds fromthe Missouri Department of Health and Senior Services

educator’s guide to sickle cell and school -...

Documents