Niemann Pick disease is a congenital disease involved with poor fat metabolism. It is a progressive disease that over time impairs the functionality of affected organs. The most affected areas include the brain, liver, spleen, nerves, bone marrow, and in severe cases, the lungs. Niemann Pick disease is rare, and although anyone can develop it, it is most common in children. The disease is rather fatal and there is currently no cure for it. Treatment administered is supportive and aimed at easing symptoms of the condition.[1] Niemann Pick disease has a wide variety of symptoms and they vary depending on the type one is suffering from.
Prognosis For Niemann Pick Disease
There are three major forms of Niemann Pick disease namely; Type A, Type B and Type C. For each type, the prognosis greatly differs depending on the severity of the condition. Type A is regarded as the acute infantile form, Type B is chronic but non-neurological, and Type C is considered a biochemically and genetically distinct form of Niemann Pick disease. Type A is more severe and most infants with the condition die during infancy. Type B is moderately severe but although affected individuals can live for a few decades, they require supplemental oxygen due to lung impairment. As for Type C, the life expectancy is variate for different patients depending on the effect of the condition on an individual. There are those who can live into adulthood while others die early during infancy.
Type A is more severe since it is a progressive neurogenerative disorder that often causes progressive neurological deterioration, and failure to thrive. So, most infants die early, say by the age of three years. Type B is less severe since patients do not experience neurological involvement with the disease. Type C has a different genetic and biochemical detailing compared to Type A and B. While many children with this form die before the age of 20, most usually succumb to the condition before they are 10 years old. For patients with delayed onset of symptoms, they have a likelihood of living longer, but regardless death is imminent before the age of forty.[2]
Lifestyle Changes For Niemann Pick Disease
Niemann Pick disease affects the ability to engage in various daily activities. The condition often starts in childhood years, and depending on the severity, children can either die early due to the complications associated with the disease or survive for several years. In one of the patients who have been diagnosed with the condition, the condition was identified when he was born. Due to the effects of the condition on the body, children may not be able to play as much as they had like or do things that they can love. Needless say, over the years of living with the condition, new symptoms arise which leads to limitations in various ways of life.[3]
It is advisable that parents join support groups that help with coming to terms with their children having the disease and how to help their kids out in leading a good life.
Adding to that, dietary modification can be considered, especially for patients with Type C Niemann Pick disease. A low-fat diet is recommended, but then again, it does not really improve on the metabolism process of fats nor slow down the disease progression. In cases where carbohydrate intake, in particular, disaccharides and oligosaccharides, was eliminated, it helped improve diarrhea, which is the main side effect of this type of the disease.[4]
Conclusion
Niemann Pick disease can be a fatal illness since it often results in death for affected individuals, who are often infants. The outlook for Niemann Pick disease is based on the type, its severity, symptoms and potential complications of the condition. Due to the adverse effects, the condition has on the body, patients are subdued to certain lifestyle modification in terms of limitations in doing various activities. In terms of dietary modification, it may be considered in instances where certain symptoms can be prevented by avoiding or eating certain foods.
- https://www.mayoclinic.org/diseases-conditions/niemann-pick/symptoms-causes/syc-20355887
- https://www.encyclopedia.com/medicine/diseases-and-conditions/pathology/niemann-pick-disease
- https://www.sanofi.com/en/science-and-innovation/living-with-a-rare-disease-jj-shares-his-story
- https://www.emaehrungs-umschau.de/english-articles/13-03-2019-dietary-carbohydrate-modification-in-niemann-pick-type-c/
Also Read:
- Is Niemann Pick Disease A Disability?
- How Do You Stop Niemann Pick Disease From Spreading?
- How Long Will It Take To Recover From Niemann Pick Disease & How Long Does The Symptoms Last?
- What Are The Ways To Prevent Niemann Pick Disease & Does It Reoccur?
- Who Is At Risk For Niemann Pick Disease & Is There A Blood Test For It?
