09 August 2010

Some Reasons Why Bennett May Have More CF Complications Than Other Kids With CF

We are still learning alot about Cystic Fibrosis and Bennett's CF in general.  They weren't kidding when they first told us that CF is very hard to predict.  Every person's cystic fibrosis disease affects them differently - due to genetic, enviromental and physical reasons. 

However, studies do tell us about trends, which helps us anticipate risks that one's CF exposes them to.  For example, Bennett's earliest clinical symptom of CF, his Meconium Ileus, has been researched thoroughly and places Bennett at risk for a number of factors.  This may be why Bennett seems to have more CF Complications than other children with CF.

Bennett's History of Meconium Ileus puts him at risk for Liver Disease
Findings suggest that patients with intestinal obstruction (such as meconium ileus or meconium peritonisis) are at greater risk for the development of liver cirrhosis.1  The frequency of meconium ileus or its equivalent is significantly higher in patients with cystic fibrosis and liver disease than in patients without liver disease.2  (Therefore, Bennett is on the medication Ursidol to prevent liver disease from forming.)

Bennett's History of Meconium Ileus puts him at risk for Surgical Complications and Intestinal Obstruction
Scar tissue can build up one day causing another obstruction in Bennett's bowel.  Therefore, Bennett is at risk slightly for a higher incidence of meconium ileus equivalent and a significantly higher rate of surgical complications. The risk of surgical complications is highest in those presenting with complicated meconium ileus and those undergoing resection as Bennett did.3

Bennett's History of Meconium Ileus (particularly because it required surgery) may put him at risk greater issues with malnutrition.
One study showed clear association of Meconium Ileus with malnutrition in CF.  The study observed poor growth among Meconium Ileus patients was not because of poor dietary intakes, but was related to surgical treatment for Meconium Ileus and poor essential fatty acid status. 4 5

Bennett's History of Meconium Ileus may put him at risk for a more severe CF symptoms.
Several studies have suggested that Meconium Ileus is a risk factor for lower pulmonary function. 6
Meconium ileus may be an early indication of a more severe phenotype of cystic fibrosis. This was suggested by the significantly lower pulmonary function found in children with a history of meconium ileus compared to age- and sex-matched children who did not have meconium ileus.7  Meconium Ileus children have worse lung function and more obstructive lung disease than those without Meconium Ileus. Such abnormalities are accompanied by reduced lung volume. Meconium Ileus is a distinct CF phenotype with more severe pulmonary dysfunction.8

However, Studies Conflict On Whether Meconium Ileus Puts Bennett At Risk For An Early Death 
"Patients with CF and Meconium Ileus presented a poor nutritional status at diagnosis and a lower survival rate compared to the general CF population."9 
"We conclude that babies presenting with Meconium Ileus have no worse long-term outcome than those presenting symptomatically later in infancy, despite having undergone invasive procedures in the newborn period."10
"Long-term outcome is similar in CF patients who present with Meconium Ileus and those who do not."11
"These findings suggest that patients with cystic fibrosis and those without Meconium Ileus do not have major intrinsic differences and that the previously poor outlook in patients with Meconium Ileus has improved greatly."13
"These results suggest that adequate initial nutritional and medical management of Meconium Ileus allows further similar nutritional status and PFTs compared with other early-diagnosed symptomatic CF patients. In this study, Meconium Ileus did not represent an additional risk factor for the patient's life."12 

* Analysis of risk factors for the development of liver disease associated with cystic fibrosis., 2, 7

* Clinical outcome of cystic fibrosis presenting with or without meconium ileus: a matched cohort study., 12

* Clinical and genetic comparisons of patients with Cystic Fibrosis, with or without meconium ileus., 13
* Does presenting with meconium ileus affect the prognosis of children with cystic fibrosis?, 10

* Effect of meconium ileus on the clinical prognosis of patients with cystic fibrosis., 5, 9

* Long-term outcome after neonatal meconium obstruction., 3, 11

* Long-term pulmonary outcome after meconium ileus in cystic fibrosis., 6

* Longitudinal pulmonary status of cystic fibrosis children with meconium ileus., 8

* Meconium ileus and its equivalent as a risk factor for the development of cirrhosis: an autopsy study in cystic fibrosis., 1, 2

* Nutritional status of patients with cystic fibrosis with meconium ileus: a comparison with patients without meconium ileus and diagnosed early through neonatal screening., 4


  1. This is like a medical journal! I'm sure researching is good therapy for you.

  2. Wow. Very interesting. Our daughter had MI at birth. Even though I am sure this studied carefully...there still is a lot of hope. Madeline (18 months) has had NO pain, blockages, infections, or other CF complications since she was born. She is not on any laxatives, etc. You never know Bennett might be the same down the road. Thanks for sharing your research....


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