NetWellness Cystic Fibrosis

Cystic Fibrosis

Patricia Joseph MS, MD — Tue, 25 Aug 2009 10:08:00 EST

Question: i am 61 years old my dr.says i have some signs of cystic fibrosis can this happen to an adult at this age?

Answer: Absolutely. Although most patients with "classic" cystic fibrosis are diagnosed very early in life, this is not always the case. There are variable degrees of disease severity and approximately 8% or patients with CF are diagnosed over the age of 15. Although uncommon, diagnoses much later in life&n . . .

Ask an Expert: Patricia Joseph MS, MD, Cystic Fibrosis

What`s the chance of a CF child?

Richard Shell MD — Tue, 07 Jul 2009 12:07:00 EST

Question: my boyfriend has CF , we want a child of our own. i have four children from previous relationshps two which have medical conditions see specialists regularly , i want to understand what the risk factor is to have a CF child, we are looking forward to adding another to the clan but i have to go for a . . .

Answer: I think the best answer would be to accompany your boyfriend to his next CF clinic appointment and have him ask his center to see if a genetic counselor could be available to speak with both of you during that visit. This might also be available though your obstetrician's office. You might also want . . .

Ask an Expert: Richard Shell MD, Cystic Fibrosis

My Son, CFand 260 lbs, Dehydration

John Heintz MD — Wed, 20 May 2009 19:05:00 EST

Question: my son diagnosed cf at 3 months..now 28 yrs old and 260 lbs tells me that being overweight helps him recover quickly from bouts of dehydration. he is a chef and subjected to very high temperatures in the kitchen..has on occasion collapsed and cramped up to the point of immobility...want him to see a . . .

Answer: Weight and hydration are separate subjects. Hydration and adequate sodium intake are critical in CF, and, especially in a hot environment, those with CF MUST take sufficient amounts of both. This can be accomplished with water, rehydration drinks, and salty snacks.

Weight is also importa . . .

Ask an Expert: John Heintz MD, Cystic Fibrosis

Cough syncope with cystic fibrosis

Patricia Joseph MS, MD — Wed, 13 May 2009 16:05:00 EST

Question: Our daughter is 21 and has Cystic Fibrosis and Cough Syncopede. The episodes are weekly and she passes out and coughs up large amounts of blood. What can we do to help her? The Dr`s that we have talked to have made no suggestions as to what we can do besides putting her feet up and pushing her hands . . .

Answer: Cough syncope is a very difficult problem. Basically, with severe episodes of cough (a coughing fit), some patients black out. Although several things can contribute to cough syncope, the basic problem in CF is one of prolonged cough episodes. During these events, the pressure in the chest . . .

Ask an Expert: Patricia Joseph MS, MD, Cystic Fibrosis

Cystic Fibrosis

Richard Shell MD — Fri, 13 Mar 2009 23:03:00 EST

Question: Can anyone please tell me how physicians/clinicians defined cystic fibrosis before the molecular nature was known?

Answer: Before the time of molecular diagnostic capabilities, a CF patient was diagnosed using a sweat chloride test. This measures the amount of chloride in the sweat and gives a value that can be diagnostic along with clinical findings of CF. I belive there is a very good description of this testing . . .

Ask an Expert: Richard Shell MD, Cystic Fibrosis

Adult cystic fibrosis

John Heintz MD — Sat, 03 Jan 2009 19:01:00 EST

Question: My mother is 72 and was just diagnosed with CF. She has been battling bronchitis and pneumonia all this last fall and into the winter. What is prognosis?

Answer: I cannot give you a prognosis, but can make a few general comments. First, diagnosis at 72 years old suggests relatively mild CF disease. With the diagnosis made, appropriate treatment becomes much more likely than without a correct diagnosis. Improved and continued good health will also almost cert . . .

Ask an Expert: John Heintz MD, Cystic Fibrosis

CF

Karen McCoy MD — Thu, 27 Nov 2008 11:11:00 EST

Question: What are the chances of cf if 1st sweat test taken on say day 1 shows abnormal, but 2 tests taken next day 1 hour apart & on different arms show borderline?

thanking you.

Answer: While impossible to quantify, there is a chance that the correct diagnosis is CF. This is a frustrating situation, which certainly does occur. Unfortunately the tests have to be repeated until a clear answer is found or alternative testing should be pursued.

Ask an Expert: Karen McCoy MD, Cystic Fibrosis

Possible late CF diagnosis?

Patricia Joseph MS, MD — Tue, 04 Nov 2008 23:11:00 EST

Question: I am a 22 year old male (5'10" - 190 lbs.), living in VA. I was diagnosed with Crohn`s disease/ colitis, acid reflux, and mthfr gene mutation when i was 16 years old. I am an athlete (football, etc.), and find myself having to battle to maintain my weight and physical abilities (such as endurance an . . .

Answer: The symptoms presented here could be attributed to a variety of causes. Certainly there are some symptoms consistent with cystic fibrosis. Cough, sputum production, and gastrointestinal issues are common. Patients with CF who are diagnosed later in life often have more subtle or atypical symptoms th . . .

Ask an Expert: Patricia Joseph MS, MD, Cystic Fibrosis

Specific colipase deficiency

Richard Shell MD — Thu, 23 Oct 2008 19:10:00 EST

Question: My 4YO was diagnosed with a specific colipase deficiency 2 years ago after chronic bouts of near constant yellow, oily, frothy diarrhea combined with poor growth and weight loss. She takes Lipram with every meal and snack and it has helped significantly.

It seems that I keep running into people . . .

Answer:

I belive the simple answer is to talk with your doctor and ask this question. I am not an expert in pancreatic disorders but pancreatic insufficiency is certainly a symptom, and sometimes a presenting factor, for cystic fibrosis.

A sweat test is an easy and painless test to obtain an . . .

Ask an Expert: Richard Shell MD, Cystic Fibrosis

Can cystic fibrosis (not)be inherited disease

Anne Matthews RN, PhD — Wed, 15 Oct 2008 14:10:00 EST

Question: Can Cystic fibrosis (not) be inherited disease Thanks...

Answer:

Yes, CF is always an inherited disease. Cystic fibrosis (CF) is a common genetic disease. CF affects the lungs and digestive organs. It is caused by a mutation (change) in a gene called CFTR. When this gene is mutated, it causes the secretions in the lungs and other organs such as the pancreas, b . . .

Ask an Expert: Anne Matthews RN, PhD, Cystic Fibrosis