NURSING MANAGEMENT

i. Nursing Diagnosis

        Fibrodysplasia ossificans progressiva (FOP) is a rare genetic disorder that causes the formation of bone in muscles, tendons, and ligaments. 

Nursing diagnoses for FOP may include:

        - Impaired mobility related to decreased range of motion due to joint stiffness and bone formation.

        - Risk for falls related to impaired mobility and altered gait due to bone formation.

        - Risk for skin breakdown related to immobility and pressure on bony prominences.

        - Anxiety related to loss of independence and fear of worsening symptoms.

        - Chronic pain related to bone formation and muscle strain.

ii. Nursing Care

Nursing care for FOP may include:

        - Promoting mobility: Encourage the patient to engage in range of motion exercises to help maintain joint  mobility and prevent further stiffness. Assist with mobility aids as needed, such as crutches or a wheelchair.

        - Monitoring skin integrity: Regularly assess the patient's skin for signs of pressure ulcers, particularly in areas bony prominences. Implement preventive measures, such as turning and positioning the patient regularly and using pressure-relieving devices.

        - Pain management: Develop a pain management plan in collaboration with the patient and healthcare team. This may include pharmacological interventions, such as nonsteroidal anti-inflammatory drugs (NSAIDs) or opioids, as well as non-pharmacological interventions, such as heat or cold therapy.

        - Patient education: Provide the patient with information about the disease, including its progression, potential complications, and strategies for managing symptoms. Teach the patient and their family members how to perform range of motion exercises and skin care.  

        - Emotional support: Patients with FOP may experience anxiety and depression due to the impact of the disease on their mobility and independence. Offer emotional support and referral to mental health professionals as needed.

 

iii. Health Teachings

Health teachings for FOP may include:

        - Importance of early diagnosis: Educate patients and their families about the importance of early diagnosis of  FOP. Early detection can help prevent complications and improve quality of life.

        - Avoidance of trauma: Explain the risk of flare-ups triggered by trauma or injury. Teach patients and their families to avoid activities that may cause trauma or injury, such as contact sports.

        - Maintenance of mobility: Encourage patients to maintain mobility through regular exercise and stretching.Provide guidance on appropriate exercise and physical therapy techniques.

        - Prevention of falls: Teach patients and their families about fall prevention measures, such as using mobility aids and ensuring the home environment is safe and accessible.

        - Skin care: Educate patients and their families on the importance of skin care to prevent pressure ulcers. Teach proper skin care techniques, including regular skin inspections and use of pressure-relieving devices.

        - Pain management: Discuss pain management options with patients and their families. Provide education on the use of medications and non-pharmacologic pain management techniques, such as heat or cold therapy.

        - Genetic counseling: Consider referral to genetic counseling for patients and their families to discuss the risk of  passing on the condition to future children.

References: 

Wayne, G. Impaired Physical Mobility Nursing Care Plan. 2018. Retrieved from Impaired Physical Mobility Nursing Diagnosis & Care Plan - Nurseslabs

Qi Z, Luan J, Zhou X, Cui Y, Han J. Fibrodysplasia ossificans progressiva: Basic understanding and experimental models. Intractable Rare Dis Res. 2017 Nov;6(4):242-248. doi: 10.5582/irdr.2017.01055. PMID: 29259851; PMCID: PMC5735276.