Hemophilia
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Included In This Lesson
Study Tools For Hemophilia
Bleeding Precautions (Mnemonic)
Bleeding Complications (Minor) (Mnemonic)
Hemophilia Pathochart (Cheatsheet)
Clotting Cascade Anticoagulants Cheatsheet (Cheatsheet)
Recessive Gene Inheritance (Image)
Hemophilia (Picmonic)
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Outline
Overview
- Impairment of the body’s ability to control blood clotting due to deficiency in specific clotting proteins.
- X-linked recessive disorder (hereditary disorder)
- Carrier females pass to male
Nursing Points
General
- Types
- Hemophilia A (deficiency of factor VIII)
- Most common: 1 in 5,000-10,000 male births
- 80% of hemophilia cases
- Hemophilia B (deficiency of factor IX)
- 1 in 20,000 – 34,000 male births
- Hemophilia C (deficiency of factor XI)
- Less predictable, more complicated to treat
- Hemophilia A (deficiency of factor VIII)
- Clotting Cascade
- Missing coagulation factors prevent fibrin formation
- Hemophiliacs do not bleed more intensely
- They bleed for a longer time
Assessment
- Spontaneous bleeding – “bleeds”
- Epistaxis (nosebleed)
- Prolonged bleeding to trauma
- Frequent bruising
- Most common – Hemarthrosis
- Bleeding into joints
- Look for swelling, warmth & redness
- Not to miss!
- Bleeding in the brain
- Visual changes
- Headaches
- Change in LOC
- Slurred speech
- GI Bleed
- Hematemesis – throwing up blood
- Melena – black stools = upper GI bleed.
- Bleeding in the brain
- Normal PT and thrombin time, prolonged PTT
Therapeutic Management
- Goal of Therapy
- Replace missing clotting factors
- Prevent bleeding
- Prevent long term problems with joints
- Monitor child for signs of bleeding
- Medications
- Replace the missing factor
- Slow IV push
- DDAVP
- Increases the body’s production of clotting factor
- Useful in mild hemophilia A only
- Replace the missing factor
- Rapid treatment at home is best
- Parents learn to give clotting factors – IV push (usually via central line)
- Children can learn to self administer- 8-12 years.
Nursing Concepts
- Clotting
- Oxygenation
- Safety
Patient Education
- Educate parents on child safety
- Protective clothing
- Avoidance of contact sports
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ADPIE Related Lessons
Related Nursing Process (ADPIE) Lessons for Hemophilia
Transcript
Hey! In this lesson we are going to talk about Hemophilia in pediatric patients.
Okay, so the term hemophilia refers to a group of bleeding disorders. Patients with hemophilia aren’t able to clot when they have an injury because they are missing a certain clotting factor. It’s caused by an x-linked recessive gene. All this means is that females are carriers and pass the disorder onto males. Remember females have two XX’s which means the X without the gene takes over and females don’t have symptoms. Males have XY so if they have an X with the hemophilia gene they will definitely have the disorder.
There are several different types of hemophilia A, B & C. Hemophilia A is missing clotting factor VIII. Hemophilia B is missing clotting factor IX. Hemophilia C is missing clotting factor XI. A is the most common and accounts for 80% of hemophilia cases.
Your assessment of a patient with hemophilia is all about looking for signs of bleeding. Sometimes this will be obvious from a cut or injury. Other times the bleeding could be happening kind of silently. The most common place for bleeds are in joints. This is called hemarthrosis and the child is probably going to complain of pain in the joint. On assessment it will be red and warm. Other places bleeding can occur are in the brain and in the GI system. These can be life threatening so make sure not to miss symptoms like 1) Visual Changes 2) Headaches 3) Changes in their level of consciousness 4) Slurred speech. For GI Bleeds be on the lookout for 1) Hematemesis (vomiting blood) and 2) Melena (blood in stools- dark red or black).
In their blood work you’ll note a prolonged PTT. PT times and thrombin times will be normal.
Standard treatment of hemophilia is to replace the clotting factor that is missing. The clotting factors are given slow IV push. Depending on how severe the hemophilia is, some kids will have a central line placed to avoid having to start IV’s every time they need an infusion.
DDAVP is a medication called Desmopressin and it’s given IV infusion. It only works with mild hemophilia because it actually works by stimulating the body to release more of the missing factor, not by replacing the missing factor.
A really common problem that occurs with hemophiliacs are joint problems. Because they get bleeds there so often the joints the surrounding tissues can become damaged- so these patients may need physical therapy to make sure kids don’t end up with contractures.
The best way for hemophilia to be managed is at home. So parents are educated on how to either start IV’s or access central lines and give the factor. This makes sure kids get treatment as quickly as possible with minimal impact on their daily lives.
In addition to learning how to give the medication parents and kids also need to know the following. They need to avoid contact sports! They should never give aspirin or NSAIDS for pain or fevers because they can cause bleeding too. Protective gear is really helpful, especially for accident prone toddlers and preschoolers. And they obviously need to feel pretty comfortable giving first aid.
Your priority nursing concepts for a pediatric patient with hemophilia are clotting, oxygenation and safety.
Okay- that’s it for this lesson. Hemophilia is pretty straightforward and really kids with hemophilia do really well and have a good life expectancy if they manage it properly. So let’s recap really quickly. Hemophilia is an x-linked recessive disorder where the patient is missing a clotting factor and therefore bleeds for longer when injured.
Hemophilia A is missing factor XII (8), Hemophilia B is missing factor IX (9), Hemophilia C is missing factor XI (11).
Your assessment of a patient with hemophilia is all about looking for signs of bleeding- the obvious ones and those less obvious like joints, brain or GI.
Treatment is just giving the patient an infusion of the factor that is missing and management is best when done at home- so parents and kids need to know how to prevent bleeds and then also how to administer their own clotting factor.
That’s it for our lesson on Hemophilia. Make sure you check out all the resources attached to this lesson. There’s a cheatsheet on clotting cascade if you need a refresher on the patho. Now, go out and be your best self today. Happy Nursing!
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Cirrhosis Complications Nursing Mnemonic (Please Bring Happy Energy)
Cleft Lip Repair – Post Op Care Nursing Mnemonic (CLEFT LIP)
Common Pathogens for UTI Nursing Mnemonic (KEEPS)
Common Signs of Parkinson’s Nursing Mnemonic (SMART)
Community Health Tool Nursing Mnemonic (MAP-IT)
Complications of Spinal Cord Injuries Nursing Mnemonic (ABCDEFG)
Complications of Thoracentesis Nursing Mnemonic (Patients Sometimes Bleed Internally)
Nursing Care and Pathophysiology for Heart Failure (CHF)
COPD management Nursing Mnemonic (COPD)
Cor Pulmonale – Signs & Symptoms Nursing Mnemonic (Please Read His Text)
Coronary Arteries – Location Nursing Mnemonic (I have a RIGHT to CAMP if you LEFT off the AC)
Cranial Nerve Mnemonic 01 Nursing Mnemonic (Olympic Opium Occupies Troubled Triathletes After Finishing Vegas Gambling Vacations Still High)
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Crohn’s Morphology and Symptoms Nursing Mnemonic (CHRISTMAS)
Cushings Assessment Nursing Mnemonic (STRESSED)
Cyanotic Defects Nursing Mnemonic (The 4 T’s)
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Diverticulitis Complications Nursing Mnemonic (Please Fix His Abscess SOon)
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Drugs that Cause SJS Nursing Mnemonic (I C NASA)
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Epiglottitis – Signs and Symptoms Nursing Mnemonic (AIR RAID)
Episiotomy – Evaluation of Healing Nursing Mnemonic (REEDA)
Evaluation of Irregular Moles Nursing Mnemonic (ABCDE)
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Factors That Can Put a Pregnancy at Risk Nursing Mnemonic (RIBCAGE)
Fetal Distress Interventions Nursing Mnemonic (Stop MOAN)
Fetal Wellbeing Assessment Tests Nursing Mnemonic (ALONE)
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Flu Symptoms Nursing Mnemonic (FACTS)
Fractures
GERD causes Nursing Mnemonic (Reflux Is Probably Mean)
Global Symptoms for Brain Tumors Nursing Mnemonic (HAS)
Gluten Free Diet Nursing Mnemonic (BROW)
Heart Failure – Right Sided Nursing Mnemonic (HEAD)
Heart Failure-Left-Sided Nursing Mnemonic (CHOP)
Heart Failure-Origin Nursing Mnemonic (Left – Lung|Right – Rest)
Heart Sounds Nursing Mnemonic (APE To Man – All People Enjoy Time Magazine)
HELLP Syndrome – Signs and Symptoms Nursing Mnemonic (HELLP)
Hemodynamics
Nursing Care and Pathophysiology for Hemorrhagic Stroke (CVA)
Hemorrhagic Stroke Risk Factors Nursing Mnemonic (HATS)
Hiatal Hernia Symptoms Nursing Mnemonic (Her Belly Really Hurts Following Dinner)
High Pressure Vent Alarms Nursing Mnemonic (Kings Eat Big Cakes)
High Risk Behavior Nursing Mnemonic (HEADSS)
Hypercalcemia – Signs and Symptoms Nursing Mnemonic (GROANS, MOANS, BONES, STONES, OVERTONES)
Hyperglycemia Management Nursing Mnemonic (Dry and Hot – Insulin Shot)
Hyperkalemia – Causes Nursing Mnemonic (MACHINE)
Hyperkalemia – Management Nursing Mnemonic (AIRED)
Hyperkalemia – Signs and Symptoms Nursing Mnemonic (Murder)
Hypernatremia – Causes Nursing Mnemonic (MODEL)
Hypernatremia – Signs and Symptoms 2 Nursing Mnemonic (FRIED)
Hypernatremia – Signs and Symptoms 2 Nursing Mnemonic (SWINE)
Hypernatremia – Signs and Symptoms 3 Nursing Mnemonic (SALT)
Hypertension – Nursing care Nursing Mnemonic (DIURETIC)
Hypertension- Complications Nursing Mnemonic (The 4 C’s)
Hypocalcemia – Definition, Signs and Symptoms Nursing Mnemonic (CATS)
Hypoglycemia – Signs and Symptoms Nursing Mnemonic (TIRED)
Hypoglycemia Management Nursing Mnemonic (Cool and Clammy – Give ‘Em Candy)
Hypoglycemia symptoms Nursing Mnemonic (DIRE)
Hypokalemia – Signs and Symptoms Nursing Mnemonic (6 L’s)
Hyponatremia- Definition, Signs and Symptoms Nursing Mnemonic (SALT LOSS)
Hypoxia – Signs and Symptoms (in Pediatrics) Nursing Mnemonic (FINES)
Hypoxia – Signs and Symptoms Nursing Mnemonic (RAT BED)
IADLS (Instrumental Activities of Daily Living) Nursing Mnemonic (SCUM)
Increase MAP Nursing Mnemonic (VAK)
Inflammation- Signs and Symptoms Nursing Mnemonic (HIPER)
Insulin Mnemonic (Ready, Set, Inject, Love)
Interventions for Aphasia Nursing Mnemonic (PROP)
Intra Uterine Device – Potential Problems Nursing Mnemonic (PAINS)
Intrarenal Causes of Acute Kidney Injury Nursing Mnemonic (TONIC)
Leukemia – Signs and Symptoms Nursing Mnemonic (ANT)
Levels of consciousness Nursing Mnemonic (Never Carry Dirty Socks Or Smelly Clothes)
Lidocaine Toxicity – Signs and Symptoms Nursing Mnemonic (SAMS)
Low Pressure Vent Alarms Nursing Mnemonic (Cake Everyday)
Lymphoma – Signs and Symptoms Nursing Mnemonic (NURSE For Pete’s Sake)
Management of Glomerulonephritis Nursing Mnemonic (Please Help Deliver Diuretics)
Management of Lyme Disease Nursing Mnemonic (BAR)
Management of Pressure Ulcers (Pressure Injuries) Nursing Mnemonic (SKIN)
Manic Attack – Signs and Symptoms Nursing Mnemonic (DIG FAST)
MAO Inhibitors Nursing Mnemonic (TIPS)
Medication Classess for IBD Nursing Mnemonic (Sometimes I Can’t Answer)
Medications for Pancreatitis Nursing Mnemonic (Please Make Tummy Better)
Medications to Prevent Seizures Nursing Mnemonic (Pretty Little Liars Forever)
Meningitis Assessment Findings Nursing Mnemonic (FAN LIPS)
Mnemonic for Organ Systems (MR DICE RUNS)
Multiple Sclerosis Symptoms Nursing Mnemonic (DEMYELINATION)
Murmur locations Nursing Mnemonic (hARD ASS MRS. MSD)
Nursing Care and Pathophysiology of Myocardial Infarction (MI)
Myocardial Infarction Nursing Mnemonic (MONATAS)
OB Non-Stress Test Results Nursing Mnemonic (NNN)
OLD CARTS Mnemonic (OLD CARTS)
Oral Birth Control Pills – Serious Complications Nursing Mnemonic (Aches)
Pain Assessment Questions Nursing Mnemonic (OPQRST)
Nursing Care and Pathophysiology for Parkinsons
Asthma
Pediatric Gastrointestinal Dysfunction – Diarrhea
Pharmacokinetics Nursing Mnemonic (ADME)
Planning Community Health Interventions Nursing Mnemonic (PRECEDE-PROCEED)
Pneumonia Risk Factors Nursing Mnemonic (VENTS)
Pneumothorax Signs and Symptoms Nursing Mnemonic (P-THORAX)
Possible Infections During Pregnancy Nursing Mnemonic (TORCH)
Post-Partum Assessment Nursing Mnemonic (BUBBLE)
Postpartum Hemorrhage (PPH)
Preeclampsia: Signs, Symptoms, Nursing Care, and Magnesium Sulfate
Pregnancy Outcomes Nursing Mnemonic (GTPAL)
Probable Signs of Pregnancy Nursing Mnemonic (CHOP BUGS)
Process of Labor – Mom Nursing Mnemonic (4 P’s)
Process of Labor – Baby Nursing Mnemonic (ALPPPS)
Promotion and Evaluation of Normal Elimination Nursing Mnemonic (POOPER SCOOP)
Prostate Nursing Mnemonic (FUN)
Proton Pump Inhibitors
Pulmonary edema treatment Nursing Mnemonic (MAD DOG)
Pupil Reactions Nursing Mnemonic (PERRLA)
Reactivation of Herpes Zoster Nursing Mnemonic (FICA)
Reasons for a Bronchoscopy Nursing Mnemonic (Please Assess His Weird Bronchoscopy Results)
Reasons for Chest Tube Nursing Mnemonic (Don’t Ever Fail)
Restrictive Lung Disease Causes Nursing Mnemonic (PAINT)
Rheumatoid Arthritis Assessment Nursing Mnemonic (RHEUMATOID)
Risk Factors for Cholelithiasis Nursing Mnemonic (5-F’s)
Risk Factors for Osteoporosis Nursing Mnemonic (ACCESS)
Safety Check Nursing Mnemonic (MADLE)
SBAR Communication Nursing Mnemonic (SBAR)
Schizophrenia
Scleroderma Symptoms Nursing Mnemonic (CREST)
Seizure Causes Nursing Mnemonic (VITAMIN)
Seizure Documentation Nursing Mnemonic (TDOC)
Senile Dementia – Assess for Changes Nursing Mnemonic (JAMCO)
Shock – Signs and symptoms Nursing Mnemonic (TV SPARC CUBE)
Nursing Care and Pathophysiology for SIADH (Syndrome of Inappropriate antidiuretic Hormone Secretion)
Signs of Osteoarthritis Nursing Mnemonic (OSTEO)
Sprains and Strains – Nursing Care Nursing Mnemonic (RICE)
SSRI’s Nursing Mnemonic (Effective For Sadness, Panic, and Compulsions)
Stages of Fetal Development Nursing Mnemonic (Proficiently Expanding Fetus)
Stages of Hepatitis Nursing Mnemonic (PIP)
Steps in the Nursing Process 1 Nursing Mnemonic (ADPIE)
Steps in the Nursing Process 2 Nursing Mnemonic (AAPIE)
Steps In The Nursing Process 3 Nursing Mnemonic (SOAPIE)
Steroids – Side Effects Nursing Mnemonic (6 S’s)
Stoke Assessments Nursing Mnemonic (FAST)
Symptoms of Hyperthyroidism Nursing Mnemonic (SWEATING)
Symptoms of Hypothyroidism Nursing Mnemonic (MOM’S SO TIRED)
Symptoms of Nephrotic Syndrome Nursing Mnemonic (NAPHROTIC)
Symptoms of Wernicke’s Encephalopathy Nursing Mnemonic (COAT)
TB Drugs Nursing Mnemonic (RIPE)
Toxicity Sepsis- Signs and Symptoms Nursing Mnemonic (The 6 T’s)
Tracheal Esophageal Fistula – Sign and Symptoms Nursing Mnemonic (The 3 C’s)
Traction – Nursing Care Nursing Mnemonic (TRACTION)
Transient Incontinence – Common Causes Nursing Mnemonic (P-DIAPERS)
Trauma – Assessment (Emergency) Nursing Mnemonic (ABCDEFGHI)
Trauma – Complications Nursing Mnemonic (TRAUMATIC)
Trauma Surgery – Medical History Nursing Mnemonic (AMPLE)
Treatment of Sickle Cell Nursing Mnemonic (HOP to the hospital)
Triage Nursing Mnemonic (START)
Two pathways of the peripheral nervous system Nursing Mnemonic (SAME)
Types of Anemia Nursing Mnemonic (Always Introduce Special Patients)
Types of Hemorrhoids Nursing Mnemonic (Pie)
Ulcerative Colitis – Assessment Nursing Mnemonic (MADE 10)
Umbilical Cord Vasculature Nursing Mnemonic (2A1V)
Vascular Disease – Deep Vein Thrombosis Nursing Mnemonic (HIS Leg Might Fall off)
Vascular disease – Raynaud’s symptoms Nursing Mnemonic (COLD HAND)
Vasospasm Therapy Nursing Mnemonic (Triple H Therapy)
VEAL CHOP Nursing Mnemonic (Fetal Accelerations and Decelerations) (VEAL CHOP)
Vitamins – Fat Soluble Nursing Mnemonic (All Dogs Eat Kibble)
Vitamins – Water Soluble Nursing Mnemonic (Birth Control)
Walkers Nursing Mnemonic (Wandering Wilma Always Late)
Who Needs Dialysis Nursing Mnemonic (AEIOU)