How Osteogenesis Imperfecta affects the Pulmonary System
Abstract
Osteogenesis Imperfecta is known to occur at the rate of 1 in 20,000 individuals. Research shows that it affects both males and females in equal frequency. It is also not subjective to a specific racial group. The deaths resulting from Osteogenesis Imperfecta depend on how severe the situation is. The study aims at discovering how OI affects the pulmonary system and what could get done to salvage the situation. Data on the disorder was gathered from various literature sources. Analysis of the data collected was carried, and it was evident that Osteogenesis Imperfecta does affect the pulmonary system resulting in various respiratory infections. Further research could get implemented to determine other systems that could be affected by OI.
Introduction
No situation is ever that serious until it hits closer to home. Over the past three years, I have been a volunteer at a Children’s home. And that is where I met Patel, a boy twenty years of age diagnosed with Osteogenesis Imperfecta at the tender age of twelve years. He has ever since been managing it, but the doctor’s recent visit might have changed all that. The doctor’s diagnosis informed that the disease was slowly creeping into his pulmonary system. With this diagnosis, I wanted to find out what would change. What is meant by the disease was affecting his pulmonary system? What new changes would I expect to see in him, and what signs show that the condition was getting severe by the minute? The write up below encapsulates my findings on the issue.
What is Osteogenesis Imperfecta?
How could I best describe Osteogenesis Imperfecta was my first point of concern. Osteogenesis Imperfecta could get characterized as a genetic disorder that mainly affects the bone. A person with this has improper bone formation meaning that their bones often fracture easily when subjected to mild trauma. Alternatively, the bones could fracture for no apparent cause. Various types of the disease are classified from type I to type XI. When one is born with type II or type X, their chances of survival decrease as they may not get medication enough to curb the disease. All the other types are non-lethal, and one may live there lives with it taking all the necessary precautions.
Symptoms of Osteogenesis Imperfecta
The symptoms of the disorder, as obtained from the research, were quite visible on observing Patel. They include; constant bone fractures before puberty that decreased exponentially after puberty, height inches shorter than his mates of the same age, brittle teeth, blue sclera, easy to bruise, and delayed motor skills. Other symptoms that could be present to a patient with Osteogenesis Imperfecta include; hearing loss, a prominent head, scoliosis, needs the assistance of a wheelchair to go about and the inability to twist their forearms either clockwise or anticlockwise.
There is a difference between the mild Osteogenesis Imperfecta and the severe kind. The mild one is often hard to diagnose and manageable when detected. The critical kind, however, is easy to diagnose and barely controllable when detected. The severe ones often type II and type X. With the two, the symptoms differ. They get characterized by stillborn birth or death of the child in the first months of his life. Also, one can have severe bone deformation. With the bone deformations associated with Osteogenesis Imperfecta, the disease may affect internal organs, leading to secondary problems. The problems arise from the various system infection, for example, the neurological system and the pulmonary system.
How Osteogenesis Imperfecta affects the pulmonary system
Osteogenesis Imperfecta affects the pulmonary system; as a result, the amalgamation of multiple factors. When a person has the disorder, their ribs and spine end up not developing typically. Contrary to the usual cases of development of the ribs and spines, there may be less space for lung expansion. If the lung cannot expand as required, it cannot perform its required function efficiently. Moreover, collagen, a vital building block of connective tissue in the lungs, could be affected. As collagen structure or function gets altered, the purposes of the lungs’ connective tissues also get disturbed. The disturbing effect displays itself by the lack of connection between various tissues in the lungs.
Once Osteogenesis Imperfecta affects the pulmonary system, the patient then develops breathing problems. Problems include consistent tiredness, lack of sleep, shortness of breath, or sleep apnea. It also guarantees that the patient is susceptible to respiratory infections. Respiratory infections include runny nose, cough, sinuses, mild flu, tonsillitis sore throat, sneezing, achy muscles, tuberculosis, and the common cold. The patient may also develop a restrictive pulmonary disorder. A condition best described by the reduction of the capacity the lungs can take. A condition commonly heard of in patients with severe OI, those with decreased chest volume, chronic bronchitis, or asthma. The state renders them liable to the development of lower respiratory infections. People with types II or III often succumb to death as the enquired respiratory complications result in pneumonia or even heart failure.
The care given to an Osteogenesis Imperfecta patient
There are various stipulated ways to care for an OI patient. General measures include preserving bone density, frequent monitoring of their hearing ability, physical therapy that will aid in the maintenance of bone strength, regular dentation checks, and the prevention of the development of cardiorespiratory complications. When applied efficiently, the patient could live peacefully with OI.
To prevent the development of severe cardiorespiratory complications, the caregiver could adhere to various stipulated guidelines. The caregiver should immediately respond to all respiratory infections by administering treatment. They should also treat any difficulties in breathing. Monitoring the functioning of the patients’ respiratory organs should be carried out yearly without fail, and physical therapy could be offered to the patient to encourage deep breathing. Moreover, the caregiver should ensure that the patient undergoes spine curvature monitoring and vaccinate regularly. The caregiver should also make an effort to treat underlying problems such as asthma, if any. Finally, the patient should not indulge in destructive habits such as smoking.
Conclusion
In conclusion, OI is a disorder that affects bone deformation. It makes the bones weak enough to fracture at any time despite there being no application of external pressure. Various types range from mild to severe. The mild OI types could easily get managed, whereas the severe one leads to death. OI could lead to secondary problems such as affecting the pulmonary system. It comes about as the lungs are not adequately formed to work at capacity. Once it touches the pulmonary system, it may lead to breathing problems. OI could be effectively managed or treated with the help of a caregiver.
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