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\begin{document}

\title{Management of respiratory problems in children on home invasive
mechanical ventilation}



\author[1]{Merve Nur Tekin}%
\author[1]{Nazan Cobanoglu}%
\affil[1]{Ankara University Faculty of Medicine}%


\vspace{-1em}



  
  \date{June 06, 2024}


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\selectlanguage{english}
\begin{abstract}
The management of respiratory problems in children on home invasive
mechanical ventilation (HIMV) is a complex and challenging task. In
recent years, with appropriate family education, these patients have
been able to be discharged from the hospital and continue their
treatment at home. The population of pediatric patients dependent on
HIMV has been increasing worldwide, presenting unique and varying care
needs. Management of these patients involves addressing ventilator
settings, monitoring respiratory status, ensuring airway safety, and
providing continuous support and education to patients and their
caregivers. Despite the completion of home settings and family
education, children on HIMV may encounter various respiratory problems
during home follow-up. Prevention and timely management of these
complications are crucial to improving patient outcomes. This article
summarizes the most significant respiratory problems in children on HIMV
and the management strategies for each problem are discussed,
emphasizing the importance of appropriate aspiration techniques, regular
monitoring, adequate training of caregivers, and a well-prepared
emergency plan.%
\end{abstract}%



\sloppy


\textbf{Introduction:}

The inability of the respiratory system to keep blood gas concentrations
within the normal range is called chronic respiratory failure. These
patients need external respiratory support and mechanical ventilators
are appropriate treatment option to improving the ventilatory capacity
of the respiratory system. While in the past, these patients were
followed up with conventional mechanical ventilators in intensive care
units when they needed external respiratory support. In recent years,
with the development of new technologies, airway is provided with
tracheostomy in prolonged intubations to avoid of complications of
intubation. After family education these patients can be discharged, and
their treatment is continued at an earlier time with home invasive
mechanical ventilators (HIMV). Because long-term invasive mechanical
ventilation in a hospital can have negative effects on a child's
maturation including social, psychological even emotional impacts. Being
in a hospital for an extended period can disrupt a child's normal
routines and interactions, potentially affecting their social skills and
emotional well-being. Additionally, being in a hospital environment
exposes the child to a higher risk of hospital-acquired infections,
which can further complicate their medical condition. As a result,
patients dependent on HIMV represent a growing population worldwide,
which have specific and variable care needs. The management of HIMV
patients involves addressing their ventilator settings, monitoring their
respiratory status, ensuring airway safety, and providing ongoing
support and education to both the patients and their families or
caregivers \textsuperscript{1}. Once all processes for home support are
in place, if clinical conditions allow; such as stable cardiopulmonary
status as demonstrated by heart rate, pulse oximetry recording, oxygen
support (possibly \textless{}40\%), absence of recurrent respiratory
infections and adequate weight gain; the child is a candidate for
discharge from home \textsuperscript{2}. Even if the patients discharged
with a HIMV are discharged after the completion of home settings and
family education, they experience various respiratory problems in their
home follow-up. In a retrospective observational cohort analysis of 228
children enrolled in a home mechanical ventilation program over a
22-year period in Southern California, found that 21\% mortality rate of
34\% due to progression of underlying conditions and 49\% due to
unexpected events; 19\% of these unexpected deaths were associated with
airway problems, such as tracheal hemorrhage, acute airway obstruction
and accidental decannulation \textsuperscript{3}. They also found that
pneumonia and tracheitis were the most common causes of non-elective
hospital readmissions in 40\% of patients receiving HIMV support during
their first year at home after discharge; half of these admissions
occurred in the first 3 months \textsuperscript{4}. Edwards et al.
examined severe emergencies in a group of ventilator-dependent children,
they found that the majority of admissions were associated with
respiratory problems such as increased secretions, infection,
atelectasis, and complications from tracheostomy \textsuperscript{5}.

Below, the management of respiratory problems in patients dependent on
home invasive mechanical ventilation will be addressed.

\textbf{1. \emph{Increased respiratory secretions and airway
obstruction}}

Airway obstruction is the most critical and life-threatening event that
can occur in patients with a tracheostomy tube or on home mechanical
ventilation. Immediate and effective action is crucial to clear the
airway obstruction to prevent the development of hypoxemia, which is a
condition characterized by low oxygen levels in the blood. Even a slight
contamination of small cannulas used in children can cause a significant
increase in airway resistance, as the smaller the inside diameter of the
cannula, the greater the risk of airway obstruction by secretions.

A tracheostomy tube, while necessary for respiratory support, bypasses
the natural protective mechanisms of the nose and oral cavity, such as
ciliary clearance, filtration, humidification and warming of the inhaled
air. Consequently, children with a tracheostomy may encounter an array
of respiratory challenges, including an increased likelihood of cough,
susceptibility to pulmonary infections, and dryness of pulmonary
secretions. To safeguard the airways from irritation caused by dry air,
dust, or harmful particles present in the environment, the use of
appropriate filters becomes indispensable. Equally vital is the
implementation of humidification and frequent suctioning, essential
measures aimed at reducing the risk of crust formation, mucus plugs, and
potential tube blockages. By adhering to these meticulous care
practices, we can ensure optimal airway health and enhance the overall
well-being of children reliant on tracheostomy tubes for long-term
respiratory support \textsuperscript{6}. This vulnerable population,
emergencies related to the tracheostomy cannula occur more frequently
compared to adults, with accidental removal of the cannula and
aspiration being particularly prevalent. When encountering symptoms such
as fever, increased secretions, persistent cough, dyspnea, and
exhaustive forced breathing, close monitoring is essential to identify
potential complications. In such cases, employing pulse oximetry during
spontaneous inhalation of ambient air aids in timely intervention and
ensures adequate oxygenation for these young
patients\textsuperscript{7}. In patients requiring HIMV with ineffective
cough and/or neuromuscular disease with poor respiratory muscle strength
can be used a mechanical insufflation--exsufflation device to help
maintain airway clearance. Despite advanced home monitoring systems,
almost 20\% of the overall mortality rate are attributed to preventable
deaths related to tracheostomy accidents that like accidental
decannulation and mucous plugging of the tracheostomy tube
\textsuperscript{1}. In such situations, one of the caregivers must
always be awake and promptly remove the obstruction using an aspiration
device. Ensuring the patient receives adequate oxygenation is crucial
during this critical time.

According to the latest Canadian research study at a tertiary care
center that involved pediatric respiratory specialists and pediatricians
with specialist in tracheostomy tube care and home ventilation; in
patients experiencing excessive oral secretions, medical centers accept
a range of effective strategies for management. Among the approaches;
salivary gland botox injection, nutritional assessment, gastrostomy
feeding, humidifier thorough tracheal masks, enteral glycopyrrolate
administration, salivary gland duct ligation, and scopolamine
patches\textsuperscript{8}.

\textbf{2. \emph{Tracheal trauma and subglottic stenosis}}

Tracheal trauma, particularly erosion, results from unsupported
ventilator tubing pulling on tracheostomy canula and improper tracheal
aspiration \textsuperscript{9}. Flexible endoscopy can be used to check
that the location of the tracheostomy tube is centered in the trachea
and that the trachea is healthy. The American Thoracic Society (ATS)
statement ``Care of the Child with a Chronic Tracheostomy'' recommends a
control bronchoscopy every 6-12 months to evaluate the underlying airway
pathology, detect and treat complications, assess tube size and
position, and determine readiness for decannulation\textsuperscript{10}.
If complications are suspected, additional bronchoscopies can be carried
out.

Subglottic stenosis may occur due to the placement of the tracheostomy
tube above the airway. Other contributing factors include trauma from
prolonged endotracheal intubation before tracheostomy opening and
inflammation often associated with uncontrolled reflux. To avoid this,
correct tracheostomy care and proper placement are essential. With
appropriate endoscopic evaluation before decannulation, this
complication can be predicted and surgically corrected with cartilage
graft or segmental tracheal resection.

The aforementioned Canadian study evaluated several aspects, including
tracheostomy tube care, caregiver qualification, home monitoring,
speaking valves, medical management of tracheostomy complications, and
decannulation assessment. The frequency of tracheostomy cannula
replacement is most recommended on a monthly basis. Clinical indications
for more frequent tracheostomy tube changes were respectively; mucous
plugs (94.1\%), upper respiratory tract infection with increased
secretions (70.6\%), pseudomonas colonization (23.5\%), and younger age:
age \textless{}3 years (17.6\%), and \textless{}1 year
(11.8\%)\textsuperscript{8}.

\textbf{3. \emph{Granuloma formation}}

Granuloma formation is a condition that occurs when there is
inflammation in the area due to tracheal trauma resulting from not being
attentive to aspiration techniques. During the healing process, the
inflammation leads to the development of raised scar tissue on the
surface, which can cause narrowing or complete blockage of the airway.
Localized tracheomalacia may also develop due to inflammation at the
stoma site.

Silver nitrate is the most recommended first-line agent for the
treatment of granulomas, while other recommended agents include
ciprofloxacin-dexamethasone otic suspension, topical steroid ointments,
frequent dressing changes, or topical antibiotics \textsuperscript{8}.
One study noted the choice of cuffless cannulas to ensure the
possibility of using speaking valves and to reduce tracheal trauma. In
the presence of excessive leakage, a new larger cannula was used if the
exhaled tidal volumes could not be controlled \textsuperscript{2}.

When granulation tissue is detected, patients should be referred to head
and neck surgery. If there is no response to medical treatment, the
granulation tissue can be removed with optical forceps or potassium
titanyl phosphate or laser coagulation. To prevent the recurrence,
intralesional steroid injection can be performed \textsuperscript{6}.

\textbf{5. \emph{Hemorrhage}}

Hemorrhage is the presence of blood during tracheostomy aspiration. In
some severe cases, bleeding may occur within the tracheostomy tube even
without aspiration. Such bleeding should be considered a serious
condition and requires appropriate medical intervention. These bleedings
can be caused by tracheitis or vulnerable granulation tissue on the
trachea. Tracheitis is a bacterial infection of the tracheal mucosa.
Frequent aspiration may be required due to increased secretion after
infection, which may irritate the tracheal mucosa and cause bleeding.
Systemic antibiotics are used in the treatment, and it is recommended to
increase hydration. It is also important to use an appropriately sized
and correct length suction catheter to avoid tracheal trauma during
aspiration \textsuperscript{6}. There are three techniques for
aspiration: ''Shallow aspiration'' is the superficial insertion of a
catheter into the very core of the tracheostomy tube to remove
secretions from the opening. The ''pre-measured technique'' is a method
that is neither deep nor shallow, in which the farthest side holes are
inserted to a certain depth where they emerge from the end of the
tracheostomy tube. ''Deep aspiration'' is the insertion of the catheter
until it meets resistance and then gently withdrawing it before
aspiration is applied. Damage and inflammation of the tracheal
epithelium are known to occur when deep aspiration is routinely
performed, and there are many recommendations for its discontinuation.
Pre-measured technique is recommended to minimize airway injury.
Determining the exact depth of insertion in the pre-measured technique
is crucial to prevent epithelial damage or insufficient aspiration.
Marking can be made on a demonstrative catheter using a tracheostomy
tube of the same size to measure insertion depth \textsuperscript{6}.

\textbf{6. \emph{Tracheocutaneous fistula}}

Long-term tracheostomies often lead to epithelialization of the stoma
with mucocutaneous overgrowth in the artificial lumen at the stoma site
and tracheocutaneous fistulas \textsuperscript{11}. There is the direct
relationship between tracheostomy dependence times and the probability
of developing a tracheocutaneous fistula. This situation can be
explained by the need for more time for mucocutaneous overgrowth and
squamous epithelialization. In studies, the conclusion that this
critical time point is 24 months has come to the fore. The percentage of
children who develop tracheocutaneous fistula ranges from 13\% to 57.3\%
in the literature. Despite the longstanding and widespread use of
tracheostomies in medical practice, a standardized approach for
decannulation still does not yet exist among different practitioners.
Clinicians should develop a standard decannulation protocol to
decannulate patients as soon as appropriate to reduce the risk of
developing tracheocutaneous fistula \textsuperscript{12}.

\textbf{7. \emph{Tracheoesophageal fistula}}

Tracheoesophageal fistulas develop when the trachea opens into the
esophagus after tracheal lacerations. These rare but serious
complications can occur with chest trauma, tracheal intubation or
percutaneous dilatation tracheostomy. It can be seen after rigid
tracheal intubation, respiratory failure emergencies or esophagectomy.
Tracheal lacerations can also be caused by a variety of factors,
including inexperienced physicians, improper stylet use, cuff
overinflation, or coughing with a closed expiratory valve. Tracheal
lesions caused by the cuff can be of two types, longitudinal and
circular, due to cuff overinflation or cuff decubitus in the tracheal
wall.

Diagnosis of tracheal lesions can be confirmed by radiological findings
or bronchoscopy. Treatment approaches range from conservative
non-surgical treatment to surgical interventions and carry a high
mortality rate. The choice of treatment varies depending on the location
of the tracheal injury and the patient's respiratory status. In cases of
severe respiratory failure, mechanical ventilation may restrict recovery
and alternative approaches such as bypass or multi-tube use may be
necessary \textsuperscript{13}.

\textbf{8. \emph{Respiratory tract infections and ventilator associated
pneumonia}}

Respiratory tract infections (RTIs), including tracheobronchitis and
pneumonia, are prevalent among children with tracheostomy tubes and
often lead to frequent hospital admissions. In a study conducted in
Taiwan, 139 children who received HIMV support for more than 3 months
were followed and found that the most common reason (47.7\%) for
re-admissions after discharge was infections, the most common infection
type was respiratory tract infection. They thought that the reason for
this was the negative psychosocial effects of caregiver family members,
limited home care resources and financial burden \textsuperscript{14}.
Borges et al. observed hospital readmissions and found that the most
common cause was respiratory infection, which was the most common
prolonging and aggravating factor for
hospitalizations\textsuperscript{15}. Kun et al. found that pneumonia
and tracheitis were the most common causes of hospital readmissions in
the year following the initiation of HIMV \textsuperscript{4}.

Ventilator-associated pneumonia (VAP) is a serious complication with
high morbidity and mortality in intensive care units. In studies
conducted on patients dependent to HIMV, the incidence of VAP was found
to be lower than in intensive care, but the risk increases as the
duration of ventilator support is prolonged. Although VAP performed in a
home environment rarely causes death, it often requires hospitalization.
Treatment of bacterial tracheobronchitis or pneumonia should be based on
the clinical picture and recent cultures and sensitivities.
Additionally, vaccinations should be up-to-date for children receiving
chronic ventilator support. High-risk children are recommended the flu
vaccine, and antiviral drugs may be considered for flu treatment or
prophylaxis. Palivizumab is recommended for reducing serious respiratory
infections caused by respiratory syncytial virus in high-risk infants.
To prevent respiratory viral infections, families should be trained to
reduce exposure through handwashing and to limit time in high-risk
environments \textsuperscript{16}.

Appropriate aspiration techniques should be used to prevent respiratory
tract infections. There are two techniques for absorbing secretions from
the airway. ''Sterile technique'' involves using a sterile catheter and
sterile gloves for each aspiration procedure, ensuring that the catheter
placed in the tracheostomy cannula does not encounter any contaminated
area. This method is traditionally used in hospitals, with the
''modified clean technique'' (using non-sterile gloves and sterile
catheters) being used in recent years. In contrast, the ''clean
technique'' for aspiration in the home environment is more common and
useful. In clean technique, all caregivers should wash their hands
before and after each aspiration procedure to avoid contamination.
Alcohol or disinfectant solution is an acceptable tool when water and
soap are not available. After aspiration is complete, the main catheter
should be flushed with clean water until secretions are cleared
\textsuperscript{10}. Exposure to tobacco smoke also causes RTIs through
a variety of mechanisms and avoidance of tobacco smoke exposure is
recommended \textsuperscript{17}.

Also, there is a high aspiration rate in early readmission and recurrent
pneumonia. Improper care by nurses, such as inadequate feeding methods
for example feeding in the supine position, may contribute to a higher
aspiration rate. In addition, nasogastric tube feeding is thought to
cause aspiration pneumonia because it facilitates bacterial colonization
and reflux in the upper gastrointestinal tract. In one study, the
prevalence of nasogastric tube feeding was found to be higher in the
early readmission group compared with oral and gastrostomy feeding
tubes, suggesting that this may increase the incidence of aspiration.
Prior to discharge, caregivers should be provided with appropriate
training, knowledge of aspiration techniques to prevent contamination of
the aspiration catheter and to prevent food aspiration. Also, training
in feeding methods such as proper head height and proper feed rate
control is recommended. Providing adequate training may help reduce the
risk of aspiration and related complications in long-term ventilator
dependent patients \textsuperscript{18}.

In addition, local wound infection is usually a self-limiting but
possible event. This is often associated with inadequate secretion
management leading to premature failure of maturation sutures. If these
sutures become infected and fail too soon, it can result in direct
access from the wound site to the cervical neck and chest. Rapid
detection allows for home wound care, preventing more serious
complications.

\textbf{9. \emph{Barotrauma, pneumothorax and pneumediastinum}}

Air leaking problems such as pneumothorax, pneumomediastinum, and
subcutaneous emphysema are more often classified as early complications
of tracheostomy. Because of the anatomical differences between children
and adults, children have traditionally been thought to be at high risk
for these complications. These complications can be detected on
postoperative chest radiographies (x-ray), and many centers routinely
perform chest x-ray after tracheostomy for this
reason\textsuperscript{19}. Pneumothorax is one of the most common
results of barotrauma, often associated with mechanical ventilation. In
pediatric intensive care units, studies have shown that barotrauma and
pneumothorax are linked to the duration of mechanical ventilator support
and hospital stay. However, patients who experience pneumothorax have a
significantly higher mortality rate compared to those who do not. This
highlights the importance of careful monitoring and management of
mechanical ventilation to minimize the risk of barotrauma and its
potentially severe consequences. There are some opinions that argue that
the incidence of barotrauma in intensive care patients is related to the
underlying condition rather than ventilator settings, but there are not
enough studies with this \textsuperscript{20}.

For children needing HIMV, ATS recommends regular maintenance of home
ventilators and related equipment as specified by the manufacturer. Care
should be taken to ensure that the actual ventilator settings seen on
the ventilator monitor are consistent with the prescribed settings
before the hospital discharge. Twenty-four hours a day service and
telephone support should be available for mechanical breakdowns or
malfunctions \textsuperscript{1}.

\textbf{10. \emph{Accidental decannulation}}

Decannulation refers to the unintentional or voluntary removal of the
tracheostomy cannula from its proper position. In the context of
patients on home mechanical ventilation, involuntary decannulation is a
critical and life-threatening respiratory problem. Decannulation is the
most frequent (6\%) late complication of tracheostomy in a Canadian
cohort with 30 years of follow-up \textsuperscript{21}. The incidence of
accidental decannulation, as a late complication of tracheostomy, was
found in a Korean (2.7\%) and a Brazilian study
(5.7\%)\textsuperscript{22,23}.

There are some studies about various methods for maturing an infant's
tracheostomy stoma to prevent accidental decannulation and
complications, such as creating a permanent fenestration in the trachea,
using flaps to suture the tracheal opening, and employing the
`starplasty technique'. The `starplasty technique' appears to be the
most effective in reducing major complications but may result in a
tracheocutaneous fistula after decannulation, necessitating
reconstruction. These procedures are generally reserved for long-term
tracheostomy cases or when there's a concern for accidental
decannulation \textsuperscript{6}.

The latest ATS statement on caring for a child with a tracheostomy at
home recommends that two adult caregivers be trained in the care and
replacement of a tracheostomy tube \textsuperscript{1}. A study by
Tolomeo et al. examined caregivers of infants discharged from the
hospital with a tracheostomy. Seventy percent of caregivers reported
that the second caregiver attended the child's medical care frequently
or very often, and 50\% of those surveyed believed they could not take
adequate care of the patient at home without another trained caregiver
(24). In making this recommendation, ATS places high value on patient
safety and caregiver quality of life, while placing low value on the
increased use of resources required for training multiple caregivers.
The following pieces of equipment must be available for home use when
caring for a patient in home mechanical ventilation: ventilator, spare
ventilator, batteries, aspiration equipment (portable), a self-inflating
bag and mask, heated humidifier, supplemental oxygen for emergency use,
pulse oximeter and nebulizer \textsuperscript{1}.

When a tracheostomy cannula becomes dislodged or removed accidentally at
home, it can lead to immediate airway obstruction, compromising the
patient's ability to breathe. This situation requires urgent
intervention and can be life-threatening if not addressed promptly.
Patients on home mechanical ventilation are at a higher risk of
involuntary decannulation due to their need for prolonged respiratory
support and the presence of a tracheostomy tube.

Hypoxemia can lead to serious complications and even death if not
promptly addressed. Therefore, in cases of accidental decannulation or
any other event that obstructs the airway, it is essential to act
quickly and appropriately. Caregivers, family members, and anyone
responsible for the patient's care at home should be trained to
recognize the signs of airway obstruction and know how to respond
appropriately. For patients with tracheostomy tubes, reinsertion of the
cannula by trained individuals should be attempted, if possible.
However, if unsure or unable to reinsert the cannula safely, it is
essential not to force it, as this could worsen the situation. Instead,
focus on maintaining a clear airway until professional medical help
arrives.

Regular monitoring, education, and having a well-prepared emergency plan
are essential components of managing patients on home mechanical
ventilation to mitigate the risks of airway obstruction and ensure a
prompt response to any respiratory emergencies \textsuperscript{25}.

\textbf{Conclusion:}

To optimize the care of pediatric patients on HIMV, a comprehensive
approach encompassing preventive measures, continuous monitoring, and
prompt intervention for respiratory problems is essential. Through the
implementation of effective management strategies, healthcare
professionals can enhance the quality of life and outcomes for this
vulnerable population of children dependent on HIMV.

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\end{enumerate}

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