Cystic Fibrosis Treatment Options

An Autosomal Recessive genetic disorder, Cystic Fibrosis is notorious to involve major organs such as lungs, pancreas, intestines and the liver. Additionally but rarely, it can involve heart, spleen, reproductive organs, bones, gallbladder, nose and sinuses. Therefore, for Cystic Fibrosis Treatment, early diagnosis with pre-natal screening is extremely essential.

Before the treatment options are discussed, it must be known that there is no definite cure for Cystic Fibrosis. Efforts can just be made to improve the lifestyle of the patient and cure the symptoms. Therefore, psychological support is vital and the parents and the patient should be taken into confidence. Additionally, patient should be counseled on maintaining a good diet and healthy lifestyle because these efforts are imperative in improving the patient’s condition.

Since Cystic Fibrosis commonly involves the lungs, the first treatment option is directed towards the treatment of airway infection. Therefore, broad-spectrum antibiotics should be started on Cystic Fibrosis patients and they remain on this treatment for all the times because these patients are always in need of prophylaxis against infections. Also, Cystic Fibrosis patients eventually develop bronchiectasis and start to produce a lot of sputum so mechanical techniques should also be employed to expectorate the sputum. New devices have been introduced in markets that have the ability to amalgamate a cough assistive phase. Eventual treatment for worsening lung function lies in lung transplant.

Often, newborns with Cystic Fibrosis are found to have intestinal obstruction. In this case, it is mandatory to go for prompt surgery otherwise it will create gangrenous perforation which is life threatening for the newborn. Patients with Cystic Fibrosis usually have pancreatic insufficiency by birth, hence they are given the lacking digestive enzymes because these enzymes assist the duodenum to absorb nutrients effectively.

While discussing Cystic Fibrosis treatment, the most commonly seen non-lung complication should be definitely discussed: Diabetes. Cystic Fibrosis usually presents with a mixed Type I and II Diabetes and therefore, experts have given it a totally different entity of Cystic Fibrosis Related Diabetes (CFRD). Previously, treatments with oral anti-diabetic drugs have been tried but they did not have any promising results so the definite treatment lies in Insulin.

Due to the loss of ciliary motion in different parts of the body, the other two common complications seen with Cystic Fibrosis are infertility, infection of nose and sinuses. In nose, there may be development of nasal polyps and subsequently, there may be very little air flow. In these cases, surgery is indicated and complete washout of sinuses is also recommended because formation of polyps carries the high risk of sinus infections. For infertility, specialist centers need to be contacted. Cystic Fibrosis female patients have seen improvement in fertility status after assisted reproduction technology and males have seen benefit from Testicular Sperm Extraction.

Another complication that Cystic Fibrosis carries along with itself is the development of osteoporosis. For patients who develop it are recommended to increase their intake of Calcium and Vitamin D. Bisphosphonates have shown a promising effect. Patients often develop poor growth due to multiple systemic issues. For them, feeding with an external nasogastric tube is indicated.

Finally, it must be said that Cystic Fibrosis is a disease that involves multiple organs of the body and every patient may show variation in symptoms. Therefore, for effective Cystic Fibrosis Treatment, the patient should be kept up in regular follow-up of a physician who can treat the condition with expertise. Worldwide, Cystic Fibrosis care centers have been established which take to task all the complications that a patient comes with and deal them effectively. Enrolling patients in these programs is an obligatory part of their management.