Nursing Care Plan (NCP) for Hemophilia
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Outline
Lesson Objectives for Hemophilia
- Understanding Hemophilia:
- Gain a comprehensive understanding of hemophilia, including its pathophysiology, genetic basis, and the specific factors (Factor VIII or Factor IX) involved in clotting.
- Recognition of Hemophilia Types:
- Differentiate between Hemophilia A and Hemophilia B, understanding the distinct characteristics and implications for clotting factor deficiencies.
- Assessment of Bleeding Risk:
- Learn how to assess and identify potential bleeding risks in individuals with hemophilia, considering factors such as joint pain, easy bruising, and prolonged bleeding after injuries or surgeries.
- Management of Hemophilia Complications:
- Explore strategies for managing complications associated with hemophilia, including the prevention and treatment of bleeding episodes, joint damage, and other related health issues.
- Patient Education and Support:
- Develop skills in educating patients with hemophilia and their families about the condition, emphasizing self-care, recognizing early signs of bleeding, and promoting a supportive environment for individuals with hemophilia.
Pathophysiology of Hemophilia
- Genetic Basis:
- Hemophilia is an inherited bleeding disorder caused by mutations in the genes that encode clotting factors, specifically Factor VIII (Hemophilia A) or Factor IX (Hemophilia B).
- Deficient Clotting Factors:
- Individuals with hemophilia have a deficiency or absence of clotting factors (Factor VIII in Hemophilia A, Factor IX in Hemophilia B), critical components in the blood clotting cascade.
- Impaired Coagulation Cascade:
- The deficiency in clotting factors disrupts the normal coagulation cascade, leading to inefficient blood clot formation and prolonged bleeding after injuries or trauma.
- Severity Grading:
- Hemophilia severity is often categorized into mild, moderate, and severe based on the residual activity of the clotting factor. Severe cases have less than 1% of normal clotting factor activity, while mild cases have 5-40%.
- Bleeding Episodes:
- Due to the impaired clotting process, individuals with hemophilia are prone to spontaneous bleeding into joints and muscles, leading to symptoms such as joint pain, swelling, and potential long-term joint damage.
Etiology of Hemophilia
- Genetic Inheritance:
- Hemophilia is primarily an inherited disorder caused by mutations in the genes responsible for producing clotting factors. The two main types are Hemophilia A (Factor VIII deficiency) and Hemophilia B (Factor IX deficiency).
- X-Linked Inheritance:
- The genes for clotting factors are located on the X chromosome. Hemophilia is more common in males because they inherit only one X chromosome, and if it carries the defective gene, they will develop hemophilia. Females, with two X chromosomes, are typically carriers.
- Spontaneous Mutations:
- In some cases, individuals may develop hemophilia due to spontaneous mutations in the clotting factor genes, even if there is no family history of the disorder. This accounts for a minority of cases.
- Carrier Mothers:
- Carrier females have one normal X chromosome and one with the mutated gene. While they may not experience bleeding symptoms, they can pass the mutated gene to their children. Sons who inherit the mutated X chromosome will have hemophilia.
- Rare Acquired Hemophilia:
- In very rare cases, individuals can develop hemophilia later in life due to the development of antibodies that inhibit clotting factor function. This acquired hemophilia is not inherited and is usually associated with underlying medical conditions or certain medications.
Desired Outcome of Hemophilia Nursing Care
- Prevention of Bleeding Episodes:
- The primary goal is to prevent and minimize bleeding episodes by ensuring adequate levels of clotting factors through appropriate treatments such as factor replacement therapy.
- Optimal Clotting Factor Levels:
- Achieve and maintain optimal levels of clotting factors to facilitate normal blood clotting and prevent spontaneous bleeding or bleeding following injury.
- Pain Management:
- Effectively manage pain associated with bleeding episodes, joint damage, or invasive procedures to enhance the individual’s quality of life.
- Enhanced Mobility and Function:
- Promote joint health and mobility by preventing and treating hemophilic arthropathy, a common complication. This includes early intervention, physical therapy, and joint protection strategies.
- Patient Education and Empowerment:
- Educate patients and their families about the importance of adherence to treatment plans, recognizing early signs of bleeding, and taking preventive measures. Empower individuals to actively participate in their care and advocate for their health needs.
Hemophilia Nursing Care Plan
Subjective Data:
- Irritability (infants)
- Pain or tightness in the joints
- Sleepiness or lethargy
- Double vision
Objective Data:
- Large or deep bruises
- Unexplained nosebleeds
- Blood in urine or stool
- Excessive bleeding from cuts or after dental work
Nursing Assessment for Hemophilia
- Clotting Factor Levels:
- Assess the individual’s current clotting factor levels through laboratory tests to determine the severity of hemophilia and guide treatment decisions.
- Bleeding History:
- Conduct a thorough bleeding history, including the frequency, severity, and duration of bleeding episodes. Explore any recent injuries, surgeries, or dental procedures that may have triggered bleeding.
- Joint Health and Mobility:
- Evaluate joint health and mobility, focusing on any signs of hemophilic arthropathy, such as pain, swelling, and limited range of motion. Assess specific joints commonly affected, such as knees, elbows, and ankles.
- Pain Assessment:
- Assess the individual’s pain related to bleeding episodes, joint damage, or procedures. Use a pain scale to quantify pain intensity and identify patterns or triggers.
- Psychosocial Assessment:
- Perform a psychosocial assessment to understand the impact of hemophilia on the individual’s mental health, coping mechanisms, and overall quality of life. Identify any emotional or psychological challenges.
- Treatment Adherence:
- Evaluate the individual’s adherence to prescribed treatment plans, including factor replacement therapy, prophylactic measures, and medications. Identify any barriers to adherence.
- Education Needs:
- Assess the patient’s and family’s understanding of hemophilia, its management, and potential complications. Identify educational needs and gaps in knowledge.
- Support Systems:
- Explore the presence of support systems, both within the family and the broader community. Assess the availability of resources and assistance for managing the challenges associated with hemophilia.
Implementation for Hemophilia
- Clotting Factor Replacement Therapy:
- Administer clotting factor replacement therapy as prescribed to manage and prevent bleeding episodes. Monitor levels regularly and adjust the dosage as needed.
- Pain Management:
- Implement pain management strategies, such as pharmacological interventions and non-pharmacological approaches, to address pain associated with bleeding episodes or joint damage.
- Joint Protection and Physical Therapy:
- Collaborate with physical therapists to develop and implement joint protection strategies and exercises. Encourage regular physical therapy to maintain joint mobility and prevent complications like hemophilic arthropathy.
- Education and Counseling:
- Provide ongoing education to the individual and their family about hemophilia, treatment options, and self-care practices. Include counseling to address psychosocial aspects and promote mental well-being.
- Emergency Preparedness:
- Develop an emergency plan for managing unexpected bleeding episodes. Educate the patient and caregivers on recognizing signs of severe bleeding and the steps to take, including when to seek immediate medical attention.
Nursing Interventions and Rationales
- Assess patient for signs of bleeding, cuts, scrapes, bruises, swollen joints
Patients often experience deep bruising from minimal contact or minor injuries. The deep bruising may lead to bleeding into joint spaces and vital organs.
- Assess patient for evidence of pain (non-verbal cues)
Patients may report painful joints or aching muscles; younger children may not know how to express pain and exhibit symptoms through guarding or irritability and fussiness.
- Provide passive ROM exercises
This is not advised during acute phase or with active bleeding, but encouraged when patient’s condition is stable to maintain or improve joint and muscle mobility
- Provide assistive devices as required
Physical deformity of joints may occur due to bleeds into joint, which may cause limited mobility. Provide assistance and devices as necessary.
- Initiate bleeding precautions per facility protocol
Small and simple injuries may cause excessive bleeding. Client should avoid blade razors (use electric razors), use soft-bristle toothbrush, and avoid blowing nose during nosebleeds
- Monitor coagulation tests; hemoglobin and hematocrit levels
Monitor for effectiveness of interventions and therapeutic levels of medication and supplements
Expect bleeding times to be reduced from the client’s baseline
- Anticipate need and administer blood products as required
Excessive blood loss may require transfusions; have O-neg blood immediately available in case of hemorrhage
- Avoid unnecessary invasive tests and techniques
Avoid heel or finger sticks and IM injections if possible
Be prepared for excessive bleeding with administration of vaccines (opt for subcutaneous routes if available).
Apply pressure to site for several minutes to allow time to clot
- Administer medications orally as available; administer replacement clotting factors as necessary
Replacement of clotting factors is the primary treatment for hemophilia; other supplements, antibodies and antifibrinolytics may be required
- Educate patient and caregivers on safety and prevention of injury
Prevention of injury is most important; patient should avoid physical contact sports and high-risk activities; use soft toothbrush, give appropriate (not sharp-edged) toys, use helmets and padding for sports, supervise playtimes
Evaluation for Hemophilia
- Clotting Factor Levels:
- Regularly assess clotting factor levels to ensure that replacement therapy maintains levels within the target range. Adjust treatment plans based on monitoring results.
- Bleeding Episode Frequency:
- Evaluate the frequency and severity of bleeding episodes over time. A reduction in the number of bleeding episodes indicates the effectiveness of the treatment plan.
- Joint Health and Function:
- Monitor joint health and function through regular assessments and imaging studies. Assess the impact of physical therapy and joint protection measures on maintaining or improving joint mobility.
- Pain Management Effectiveness:
- Evaluate the effectiveness of pain management strategies in reducing pain associated with bleeding episodes or joint damage. Adjust pain management plans as needed.
- Patient and Family Education:
- Assess the understanding of the patient and their family regarding hemophilia, treatment modalities, and self-care practices. Evaluate their ability to recognize and respond to signs of complications.
References
- https://www.medscape.org/viewarticle/721932
- https://www.augustahealth.org/blood-disorders/hemophilia-treatment-center
- https://cibd-ca.org/disorders-we-treat/hemophilia/types-of-hemophilia/
- https://www.mayoclinic.org/diseases-conditions/hemophilia/symptoms-causes/syc-20373327
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Transcript
We are going to focus on a care plan based on hemophilia. The pathophysiology behind hemophilia is that it’s a genetic bleeding disorder that is characterized by a deficiency in clotting protein. Patients with hemophilia experience longer bleeding times than others because their blood clots much slower. The amount of clotting protein available in the blood determines the severity of the hemophilia. It is categorized into three different levels. There’s mild, moderate, and severe complications of the disease including bleeding into the joints, hemorrhage into the central nervous system or the vital organs, and aspiration from bleeding until the air res. Some of the things we want to consider as nurses is we want to assess the patient for bleeding. We want to get those bleeding precautions initiated. We want to assess the stool and urine for blood, and we want to assess and manage pain. Our desired outcome for this patient population is that the patient is going to be free of infection. We’re going to manage and reduce the pain and inflammation, regain optimum mobility and prevent complications from bleeding.
These patients with hemophilia are very irritable during their events; that can mean a variety of things, but with this particular patient population, they are irritable. They also have pain and tightness in their joints. They always complain of pain or tightness, and that’s primarily because these patients are susceptible to blood pooling into their joints. They’re typically sleepy and lethargic. They have double vision. And some of the objective data that we might observe are deep or large bruises. These patients tend to have unexplained nosebleeds. They may have blood in the urine or stool, and they may have excessive bleeding from cuts or after dental work. So excessive bleeding. Okay. As nurses, there are a few interventions that we can do. The first thing we want to look at is we want to assess the patient for any type of signs of bleeding, whether it be by cuts, scrapes, bruises, or in it’s swollen joints. Deep bruising may lead to bleeding in joint spaces and other vital organs. So, we want to do an assessment of that. We want to assess for bleeding.
The next thing that we could do is initiate those bleeding precautions because these patients, if left in the right circumstances will bleed. So, we want bleeding precautions. A small injury that will cause a normal person no harm can make this patient bleed excessively. So we want to be careful of that. We also want to avoid any unnecessary invasive techniques. So this kind of leads into the blending precautions. We want to avoid heel and finger sticks, we want to avoid IM injections, if at all possible. Be prepared for excessive bleeding. When we administer vaccines, we can try to opt for sub Q routes when those are available as well. Be sure that with these patients, that we apply pressure to any site for several minutes to allow time to clot, because they take a lot longer to do that.
One of the treatments for hemophilia is more of a therapeutic and that is when we administer replacement clotting factors. So, that’s the next goal of ours; we want to replace clotting factors and that’s just an infusion that we can give IV. Replacing clotting factors is the primary treatment for hemophilia. Other supplements, antibodies, and antifibrinolytics may be required for this patient population. We’re going to monitor the coagulation tests. We’re going to look at their hemoglobin and hematocrit levels because we need to know if this patient is bleeding. We need to know where they’re bleeding. So, we want to do hemoglobin and hematocrit. We want to monitor the effectiveness of the interventions and the therapeutic levels of the medication; expect bleeding times to be reduced from the patient’s baseline. So, we’re going to also look at the PT, PTT, INR. Just like I said, different bleeding and coagulation blood levels that we’re going to monitor.
Finally with this patient, let’s focus on pain. We are going to assess the patient for evidence of pain, but very specific pain. We are looking at pain that is showing up in the joints or muscles. Patients often report very painful joints or aching muscles. And again, that’s due to the blood that pools, maybe they bumped their elbow somewhere. And where on the surface, you may not see anything internally. There could be blood pooling into those joints causing pain. So the key points that I want you all to focus on for hemophilia is that hemophilia is a genetic bleeding disorder characterized by a deficiency in the clotting proteins. The subjective data that the patient is going to present is they’re going to be irritable. They’re going to have some joint pain. They may be sleepy,lethargic, and they may have some double vision.
Well, we’re going to be able to see in the patient, our objective data that we’re going to have, is there is going to be some blood and urine or stool. They may have some large or deep bruises and some unexplained nosebleeds, small and simple injuries. We’re going to put them at a bleeding risk. I’m going to put them on bleeding precautions, because like I said, small and simple injuries can cause excessive bleeding, bleeding precautions, and we want to avoid any unnecessary lab sticks or insertions. We want to make sure we replace their clotting factors. Remember clotting factors are the only way to treat these patients. It’s the main treatment that these patients will receive. Remember that there are two different types of hemophilia and depending on which type A or type B it would depend on what clotting factor is needed. And lastly, we love you guys; go out and be your best self today. And, as always, happy nursing.
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Nursing Care Plans
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